WR - Senior Paeds Notes 2nd Ed UPDATED JAN 2019 - Anggelos Edit

Wayne Robinson, MBBS Class of 2015
Wayne Notes
Senior Paediatrics
nd
2 Edition
Notes Prepared and Created by:

Wayne Robinson (Class of 2015)
Compiled and Bookmarked by:

Anggelos
Wayne Robinson, Class of 2015 - 1
Paediatrics
Asthma Notes
Sources: Nelson’s, GINA
September 2014
DEFINITION
GINA - A heterogeneous chronic respiratory disease that leads to inflammation of the airway. It is characterized by
1. Wheeze 2. SOB 3. Chest tightness 4. Cough. There is variation in symptoms over time, in intensity and expiratory
air flow
• Chronic inflammation results in airways hyperresponsiveness (AHR) to provocative exposures
• Management aims: 1. Reduce proinflammatory environmental exposures and 2. DAILY anti-inflammatory
medications (ie. corticosteroids) and 3. Control any comorbidities that may worsen asthma
AETIOLOGY
• Cause not fully determined

• Combination of environmental exposures and inherent biological and genetic vulnerabilities
• **Causal environments: Inhaled allergens, viral RTIs, chemical/biological air pollutants/irritants eg. tobacco smoke.
o Exposure to these in predisposed host result in prolonged pathogenic inflammation and aberrant repair -->
Lung dysfunction develops.
o This abnormal growth and development in early life leads to abnormal airways at mature age
GENETICS
• More than 100 gene loci linked to asthma.

• Loci contain proallergenic, proinflammatory genes esp. IL-4 gene on chromosome 5
• Other genes: ADAM-33 metalloproteinase gene, genes on chromosome 5q31
ENVIRONMENT
• Injurious/severe LRTI of the airways that manifest as *pneumonia and *bronchiolitis are risk factors for
persistent asthma.
• Other infections/Microbes
• Allergens – Inhalant allergens > food allergens
• Irritants/Pollutants e.g. Tobacco smoke
• Stress
RISK FACTORS (Must Know)
POINTS
Approximately 80% of all asthmatic patients report
disease onset prior to age 6!!
- BUT only a minority of children who experience
recurrent wheezing go on to persistent asthma
- Maternal asthma is the single most important risk factor for asthma development
2 main types of childhood asthma: 1. Recurrent wheezing 2. Chronic asthma
PATHOGENESIS
Airway obstruction in asthma results from numerous pathologic processes:
• In small airways, airflow is regulated by smooth muscle encircling the airways lumens – bronchoconstriction
of these bronchiolar muscle bands restricts and blocks airflow (Parasympathetic system stimulates bronchoconstriction)
• ALSO, a cellular inflammatory infiltrate AND exudates containing mainly eosinophils, can fill and obstruct
airways AND induce epithelial damage and desquamation into the airways lumen.
• ALSO, helper T lymphocytes (CD4) and other immune cells produce proallergenic, proinflammatory cytokines
(IL-4, 5 and 13) that mediate the inflammatory process.
***KNOW THIS: So, hypersensitivity and susceptibility to environmental triggers may lead to:
1. Airways inflammation -> obstruction
2. AHR -> obstruction
3. Oedema -> Basement membrane thickening -> subepithelial collagen deposition -> obstruction
4. Smooth muscle AND mucous gland hypertrophy -> mucus hypersecretion -> obstruction
ALL CONTRIBUTE TO AIRFLOW OBSTRUCTION (LOOK AT EACH AND JUST OBVIOUSLY SEE HOW)
CLINICAL MANIFESTATIONS AND DIAGNOSIS
***NOTE FROM LECTURE: The most likely diagnosis in children with recurrent wheezing is asthma, regardless
of the age of onset, evidence of atopic disease, precipitating causes, or frequency of wheezing
MOST COMMON CHRONIC SYMPTOMS:

• Intermittent DRY COUGHING
• EXPIRATORY WHEEZE
Other symptoms in older children/adults:

• Shortness of breath
• Chest tightness
**NB**: The respiratory symptoms are WORSE AT NIGHT!!
Others are subtle/nonspecific: Limited physical activity, general fatigue (may be due to sleep disturbance)
• ***MUST ASK ABOUT PREVIOUS BRONCHODILATOR USE IN HISTORY!!:

o Symptomatic improvement with treatment supports asthma diagnosis!
o BUT lack of improvement with bronchodilator/corticosteroid therapy is inconsistent with asthma and
should prompt consideration of “ASTHMA-MASQUERADING CONDITIONS”
• ***MUST ASK ALL Triggers***: Physical exertion, hyperventilation (laughing), cold or dry air, airway
irritants/allergens (incl smoking at home, pets etc), respiratory infection (induce airway inflammation – eg. RSV,
rhinovirus, adenovirus, influenza, parainfluenza, mycoplasma pneumoniae, chlamydia pneumoniae).
ENVIRONMENTAL HISTORY IS VITAL
• **Ask about risk factors: SEE RISK FACTORS ABOVE AND OTHER DIFFERENTIALS OF WHEEZE
From lecture: HISTORY FOR A WHEEZING PATIENT:

o Age of onset o Greasy stool (cystic o Number of siblings
o Course of onset (Acute vs. fibrosis)? o Occupation of inhabitants
chronic) o **Eczema at home
o Cough o Choking o Pets
o Shortness of breath o Triggers o TB exposure
o Cyanosis o Cold air o Worms
o Chest pain o Allergic rhinitis o ***Family history of
o **Exercise-induced o Weight loss Atopy/Asthma
symptoms o Recurrent infections o ***Past use and response
o Postnasal drip o Birth history to bronchodilators
o Snoring o Environmental history o Food allergies
o Spitting up o Smokers at home o Co-morbid conditions
History suggestive of asthma

o Intermittent episodes of wheezing Patient dx w/ asthma but medication seem not
o Seasonal variation to work. Use the following algorithm:
1. Check compliance (when and how [technique]
o Family history of asthma and/or atopy
meds are taken)
o Good response to asthma medications 2. If compliance OK --> check triggers
o Positive asthma predictive index 3. If triggers absent --> check dose
4. If dose OK --> re-evaluate dx
**History suggestive of a diagnosis other than asthma
o Poor response to asthma medications/bronchodilators
o History of neonatal or perinatal respiratory problems
o Wheezing since birth - congenital abnormality
o Associated with feeding or vomiting
o History of choking associated with cough & SOB
o Poor weight gain
o Recurrent ear or sinus infections
o Wheezing with little cough - Mechanical cause of obstruction- small airways, airway malacia
o Symptoms vary with position (TM)
o Progressive dyspnea, tachypnea, exercise intolerance & failure to thrive suggest interstitial lung disease
Examination (See Notes on wheezing for general wheezing examination)

• ***Chest findings are often normal!! – Ask for deeper breaths -> May elicit wheezing
Inspection: - BARREL-SHAPED chest, possible signs of resp. distress if severe exacerbation

- HARRISON’S SULCI/GROOVES - horizontal groove along the lower border of the thorax corresponding to the costal insertion of the diaphragm, ass w/ chronic asthma
- ALLERGIC SHINERS
***DURING EXACERBATIONS:
• Expiration prolonged
• Expiratory wheezing
• Decreased breath sounds – most commonly in RIGHT LOWER POSTERIOR LOBE!!
• Crackles or rhonchi sometimes – result from mucus hypersecretion and inflammatory exudate in airways
***SEVERE EXACERBATIONS
• Even longer expiration
• Inspiratory AND expiratory wheeze
• Laboured breathing
• Poor air entry
• Respiratory distress – Tachypnoea, SSR, SCR, ICR, nasal flaring, accessory muscle use (e.g. Diaphragm)
In extremis -> Airflow may be so limited that wheezing cannot be heard

DIFFERENTIAL DIAGNOSIS
[**CLASSIFY: Upper vs. Middle vs. Lower respiratory tract conditions**]
[ALSO: Extraluminal compression vs. Intraluminal obstruction vs. Intrinsic change in airway dimension]
Also depends on the age group (see lecture)
Other common causes of intermittent chronic coughing --> GER, rhinosinusitis

SEE TABLE BELOW - * = More common ones
DIFFERENTIAL DIAGNOSIS OF CHILDHOOD ASTHMA
UPPER RT CONDITIONS MIDDLE RT CONDITIONS LOWER RT CONDITIONS
- Allergic rhinitis * - Laryngotracheobronchomalacia * Viral bronchiolitis *

- Chronic rhinitis * - Laryngotracheobronchitis (e.g., Gastroesophageal reflux *
- Sinusitis * pertussis) * Bronchopulmonary dysplasia (chronic lung disease of
preterm infants)
- Adenoidal or tonsillar - Chronic bronchitis from environmental
hypertrophy tobacco smoke exposure * Pneumonia
- Nasal foreign body - Vocal cord dysfunction * Pulmonary oedema (e.g., congestive heart failure)
- Foreign body aspiration * Causes of bronchiectasis:
Cystic fibrosis
- Laryngeal web, cyst, or stenosis Immune deficiency
- Vocal cord paralysis Allergic bronchopulmonary mycoses (e.g.,
- Tracheoesophageal fistula aspergillosis)
- Vascular ring, sling, or external mass Chronic aspiration
compressing on the airway (e.g., tumor) Immotile cilia syndrome, primary ciliary dyskinesia
- Toxic inhalations Bronchiolitis obliterans
Interstitial lung diseases
Hypersensitivity pneumonitis
Pulmonary eosinophilia, Churg-Strauss vasculitis
Pulmonary hemosiderosis
Tuberculosis
Medications associated with chronic cough:
Acetylcholinesterase inhibitors
β -Adrenergic antagonists
Angiotensin-converting enzyme inhibitors
Sickle-cell crisis (Acute chest)
LABORATORY FINDINGS
1. PULMONARY FUNCTION TESTS (Spirometry and Peak flow)
POINT: ‘Forced expiratory airflow measurement’ helpful in diagnosis and monitoring efficacy of therapy
A. Spirometry is helpful as an objective measure of airflow limitation
o **Valid spirometry measurements depend on a patient’s ability to perform a full, forceful, prolonged expiration,
usually feasible in children > 6 yrs. old. Reproducible spirometric efforts are an indicator of test validity; if the FEV
1 (forced expiratory volume in 1 sec) is within 5% on 3 attempts, then the highest FEV 1 effort of the 3 is used
Normative values for FEV 1 have been determined for children on the basis of height, gender, and ethnicity
o Because asthmatic patients typically have hyperinflated lungs, FEV 1 can be simply adjusted for full expiratory
lung volume — the forced vital capacity (FVC) — with an FEV 1/FVC ratio.
o Generally, an FEV 1/FVC ratio < 0.80 indicates significant airflow obstruction
BUT these measures alone are NOT diagnostic of asthma as numerous other conditions can cause airflow reduction
Bronchodilator response to an inhaled β -agonist (e.g., albuterol) is > in asthmatics than nonasthmatics; an
improvement in FEV 1 ≥ 12% or > 200 mL is consistent with asthma.
IMPORTANT TABLE (NELSON’S)
B. Peak expiratory flow (PEF) monitoring devices provide simple and inexpensive home-use tools to measure
airflow and can be helpful in a number of circumstances. Patients must practice over 2-3 weeks to determine a “personal
best”, preferably at times when they a not symptomatic
• Peak flow rate monitoring can be accurately performed by most patients > 5 years
• ** PEFR < 80% predicted for height/patient’s personal best should trigger the administration of an inhaled
short-acting beta2 -agonist
• A PEFR < 50% of the patient’s personal best should trigger both administration of an inhaled short-acting
beta2 -agonist AND immediate medical attention
SEE MY FULL NOTES ON PEFR
NOTE WELL: PEFR and FEV1 are different. Forced expiratory volume over 1 second (FEV1) is a dynamic measure of
flow used in formal spirometry. It represents a truer indication of airway obstruction than does peak flow rate. Although peak
flow rate usually correlates well with FEV1, this correlation decreases in patients with asthma as airflow diminishes.
Also FEV1 is timed while Peak Flow is not
2. Radiology
The findings of chest radiographs (PA and lateral views) in children with asthma often appear to be normal, aside
from subtle and nonspecific findings of hyperinflation (flattening of the diaphragms) and peribronchial thickening
CXR:
• Hyperinflation (Flattened ribs and diaphgram, Increased number of ribs over hemidiaphragm, Right diaphragm same level as left)
• Flattened hemi-diaphragms
Lateral chest findings:
• Peribronchial cuffing 1. Increased retrosternal air
• Atelectasis
Chest radiographs can be helpful in identifying abnormalities that are hallmarks of asthma masqueraders (aspiration
pneumonitis, hyperlucent lung fields in bronchiolitis obliterans), and complications during asthma exacerbations
(atelectasis, pneumomediastinum, pneumothorax).
TREATMENT **MUST SEE ENTIRE UHWI OFFICIAL EMED DOCUMENT**

• SEE GINA GUIDELINES
• SEE SUMMARY OF THE STEP UP AND DOWN APPROACH FROM A CONCISE SOURCE!!
• MUST SEE UHWI OFFICIAL EMED DOCUMENT AND GINA FOR MANAGEMENT OF EXACERBATION
UHWI Document is on next page - Anggelos
***ASTHMA IS A CHRONIC CONDITION THAT IS OFTEN BEST MANAGED WITH DAILY ICS
CONTROLLER MEDICATION as monotherapy or with adjunctive therapy***
***NOTE WELL: Remember patient education AND control of environmental factors AND comorbidities (eg.
GER, rhinitis, sinusitis etc.)***
NELSON’S: During initial patient visits, a basic understanding of the pathogenesis of asthma (chronic inflammation and AHR underlying
a clinically intermittent presentation) can help children with asthma and their parents understand the importance of recommendations
aimed at reducing airways inflammation. It is helpful to specify the expectations of good asthma control resulting from optimal asthma
management. Explaining the importance of steps to reduce airways inflammation in order to achieve good asthma control and
addressing concerns about potential adverse effects of asthma pharmacotherapeutic agents, especially their risks relative to their
benefits, are essential in achieving long-term adherence with asthma pharmacotherapy and environmental control measures.
GINA (GLOBAL INITIATIVE FOR ASTHMA) GUIDELINE
Based on GINA guidelines, must use this table to determine level of control, which will determine when to
“Step-up” management, using the table on the next page
**NOTE: For BOTH severity and control, the 5 factors considered are:
1. Daytime symptoms
2. Nighttime symptoms/awakenings
3. Interference with normal activity
4. SABA use for symptom control
5. Lung function (FEV1, PEFR and FEV1/FVC ratio)
GINA (GLOBAL INITIATIVE FOR ASTHMA) GUIDELINE
RE: BRONCHODILATORS
• Short-acting beta 2 agonists: Salbutamol (aka albuterol), terbutaline

• Long-acting beta 2 agonists: Salmeterol, Formoterol
• Anti-cholinergics: Ipratropium bromide (Adrovent)
RE: TREATMENT OF INFLAMMATION AND HYPERREEACTIVITY
• ICS: Budesonide, beclomethasone, fluticasone

• Oral steroids: Prednisone [KNOW SIDE EFFECTS OF STEROIDS]
• Methylxanthines: Theophylline, aminophylline – now rarely used in children
• Leukotriene receptor antagonists: Montelukast, Zafirlukast
•Complications of steroid therapy – Put in place by Anggelos
1.CNS – Sleep disturbances, Mood swings, Behavioral changes Anxiety, Depression, Euphoria, Psychosis
2.EYES – Blurred vision, Retinopathy, Glaucoma, Cataract
3.ORAL - Thrush
4.RESPI - Hoarseness
5.CARDIO – HTN (due to Na retention), Edema
6.GASTRO – Decrease appetite, Vomiting, Peptic ulcers, Hematemesis, Colitis
7.MSK – Muscle weakness
8.BONES – Osteopenia, Osteoporosis,
9.SKIN – Hyper/Hypo-pigmentation, Rash, Acne, Easy bruising, Increase hair
MANAGEMENT OF EXACERBATIONS
SEE UHWI EMED MANUAL (See Next Page) + GINA PAGE 21 OF 32
GINA: EXACERBATION SEVERITY (MUST KNOW!)
Paediatrics
Breastfeeding and General Infant Feeding
Sources: Kaplan USMLE Step 2 Review Book (For the theory), Nurse Pauline Lovindeer lecture (For practical)
November 2014
**THIS IS A COMMON OSCE COUNSELLING STATION AND MCQ TOPIC**
GENERAL
• Most can breast feed immediately after birth and all can feed by 4–6 months
• Feeding schedule should be by self-regulation; most establish by 1 month
WHO Definition: Exclusive Breastfeeding: Feeding infants with breast milk ONLY (for the first 6 months of life) giving
no other food or drink, NOT EVEN WATER.
- ONLY exceptions:Money MOVS = Minerals or Medicines - ORS - Vitamins - Syrups or Drops - Anggelos
- Breastmilk alone is sufficient for the first 6 months of life
- After which appropriate and adequate complementary foods should be introduced gradually
- Breastfeeding should be continued up 2 years of age preferably BUT can go beyond 2 years if desired
BENEFITS/ADVANTAGES OF BREASTFEEDING
Separate into MATERNAL and BABY/CHILD
Immune Functions of Milk
-Ig-specific, antigen binding

-Lactoferrin deprives baceria of iron
-Bifidus Factor supports lactobacillusbifidus
-Oligosaccharides block antigens binding
to mucosa in the genitourinary tract
-Nucleotides promote maturation of gut
Mnemonic by Anggelos
CONTRAINDICATIONS = VACSS - Anggelos
NB: MASTITIS IS NOT A CONTRAINDICATION FOR BREASTFEEDING
1. Viruses
A. CMV, HSV– (If lesions on breast)
B. HIV– (Unless there is no other option, in which case the patient should exclusively breastfeed. Using feeds
in this case will cause irritation of the gastric mucosa and increase chance of virus transfer)
C. HBV– (BUT Mothers with HBV infection are free to breastfeed their infants after the neonate has received
the appropriate recommended vaccination against HBV)
4.Acute maternal disease if infant does not have disease (Tuberculosis, Sepsis)
5.Cancer of the Breast
6.Substance abuse
7.Specific Drugs:
Absolute contraindications Relative contraindications
Antineoplastics Neuroleptics
Radiopharmaceuticals Sedatives
Ergot alkaloids Tranquilizers
Iodide/mercurial Metronidazole
Atropine Tetracycline
Lithium Sulfonamides
Chloramphenicol Steroids
Cyclosporin
Nicotine
Alcohol
OTHER IMPORTANT POINTS
• **Foremilk- the milk released at the beginning of a feed is watery, low in fat and high in carbohydrates
RELATIVE to the creamier hindmilk released as the feed progresses
The nutrient content is relatively independent of maternal diet except for fluid intake and some vitamins
- Carbohydrates - lactose, oligosaccharides
- Fat - LCFAs, fat soluble vitamins
- Protein - Whey 70%, casein 30%, immune proteins
- Minerals
COMPARISON OF BREAST MILK TO COW MILK.

Note: Do NOT give infants cow milk prior to 1 year. Reason: Fe-deficiency anemia with early introduction (< 1
yr) of cow’s milk
COMPONENT HUMAN MILK COW MILK
Water/solids Same Same

Calories 20 kcal/oz. 20 kcal/oz.
Protein 1–1.5% (whey dominant) 3.3% (casein dominant)
Carbohydrate 6.5–7% lactose 4.5% lactose
Fat High in LCFAs High in MCFAs
Minerals Iron better absorbed Low iron and copper
Vitamins Diet dependent, low in K Low in C, D
Digestibility Faster emptying Same after 45 days
Renal solute load Low (aids in renal function) Higher
***SO compared with cow’s milk, breast milk has a lower renal solute load as well as:
- Equal calories (20 kcal/oz.)
- Higher carbohydrates
- Lower protein, but higher whey %
- Lower calcium
- Lower PO4
- Lower renal solute load – aids in renal function
BREAST ANATOMY AND FUNCTION
- Alveoli are small sacs made of milk-secreting cells

- ** Prolactin makes the cells produce milk
- ** Muscle cells contract and are acted on by oxytocin
Note: The difference between small and large breasts is fat content.
- The internal duct structure is the same
Muscle cells
{ Oxytocin makes them
contract
Prolactin Milk secreting cells

{ Prolactin makes them
secrete milk
- Prolactin is secreted after the feed to Ducts
produce milk for the next feed Larger ducts

- Baby sucking stimulates the release of prolactin
in the blood
Nipple
- More prolactin is secreted at night
- Suppresses ovulation
Areola
- Most prolactin is in the blood 30 mins after
the feeding and produces milk for the next feed Montgomery's glands
Alveoli
Oxytocin Reflex Supporting tissue
and fat
- Oxytocin works before or during feed to make milk flow
- *** Oxytocin reflex can be stimulated before actual sucking by thinking lovingly of baby, sounds of baby, sight
of baby, confidence
- *** The reflex can be hindered by worry, stress, pain, doubt
- Makes uterus contract
Inhibition of breast milk

Note: The presence of milk in the breast, ie. a breast full of milk acts as an inhibitor and stops the secretion of further milk
**BREAST ATTACHMENT**
WATCH THIS 1st https://www.youtube.com/watch?v=y--syZR0u1E
Baby Reflexes
1. Rooting- when something touches the lips, the baby opens mouth and puts tongue down and forward
2. Sucking- When something touches the palate the baby sucks
3. Swallowing Reflex- as the mouth fills with milk, the baby swallows
GOOD ATTACHMENT:
- Taken much areola and underlying tissue into the mouth

- Stretched the breast tissue to form a teat and the nipple forms about 1/3 of the teat
- Therefore the baby is suckling from the breast NOT solely the nipple
- Tongue cupped round nipple
Points:
- More areola is above the top than below the bottom lip
- The baby’s *mouth is WIDE OPEN
- Lower lip turns outwards
- *BABY’S CHIN TOUCHES THE BREAST
A. **HOW TO ACHIEVE THE ATTACHMENT**
1. Move the baby across the nipple until the mouth opens wide
2. Nipple should point to the baby’s nose
3. When baby opens wide, push baby's head onto the breast. Baby’s lower lip aims way below the nipple so
he gets the tongue under the larger ducts
B. POSITIONING THE BABY
• Baby’s head and body in line (ear, shoulder and hip in the same plane).
• Baby held close to mother’s body (chin touching the breast)
• Baby facing the breast, nose to nipple, nipple points to roof of the mouth for attachment.
• Support the baby comfortably. Newborns- support whole body, older baby maybe just head and shoulder.
NAME DISCRIPTION NOTES

Cradle Hold Same arm supports while drinking from the The cradle hold often works well
same breast. for full-term babies who were
delivered vaginally.
Cross Cradle/Over Hold Support your baby's head with the crook of your Especially useful for young infants
arm. Instead, your arms switch roles. who have not figured out how to
breastfeed yet
If you're nursing from your right breast, use your
left hand and arm to hold your baby. Rotate his
body so his chest and tummy are directly facing
you. With your thumb and fingers behind his head
and below his ears, guide his mouth to your breast.
Football/Clutch Hold Tuck your baby under your arm (on the same Works well for large breasted
side that you're nursing from) like a football mothers and those that need to
or handbag. avoid the baby being on their
abdomen as in a C-Section.
Position your baby at your side, under your arm.
She should be facing you with her nose level with
your nipple and her feet pointing toward your back.
Rest your arm on a pillow in your lap or right

beside you, and support your baby's shoulders,
neck, and head with your hand. Using a C-hold,
guide her to your nipple, chin first.
Reclining Hold Nurse while lying on your side in bed and the Recommended when a mother has
baby is under the breast while you support the too much milk or the flow of milk is
baby’s head with hand opposite the breast. too fast
Ask your partner or helper to place several pillows Because one is reclined backwards
behind your back for support. You can put a pillow or lying down, gravity will help
under your head and shoulders, and one between your milk come out slower
your bent knees, too. The goal is to keep your back Therefore, less chance of baby
and hips in a straight line. gagging on excess milk
With your baby facing you, draw him close and

cradle his head with the hand of your bottom arm.
Or, cradle his head with your top arm, tucking your
bottom arm under your head, out of the way.
Table placed by Anggelos
HOW OFTEN TO BREASTFEED
• Encourage breastfeeding on demand

• Whenever the baby wants for as long as the baby who is well attached w a n t s
• NOTE: Wake the baby if he sleeps too long
• Typically, the baby can be fed every 2 - 3 hours
• By size:
o At least 6 - 8 feeds to a LARGE BABY (i.e. about every 3 - 4 hours) in 24 hours OR
o 10 - 12 feeds (i.e. every 2 - 2.5 hours) to small or jaundiced babies in 24 hours
OTHER IMPORTANT POINTS

It is best to use cup feeding
• Practice **rooming-in allow mothers and infants to remain together 24 hours a day instead of bottle feeding as
bottles can cause nipple confusion.
• Give no artificial teats or pacifiers (soothers) to breast-feeding infants
• Foster the establishment of breast-feeding support groups and refer mothers to them on discharge from the hospital or
clinic
FORMULA FEEDING
• Infant formulas. Formula feeding is used as asubstitute for or to supplement breast milk
• Most commercial formulas are cow-milk-based with modifications to approximate breast milk.
• They contain 20 calories/ounce.
Specialty formulas (soy, lactose-free, premature, elemental) are modified to meet specific needs.
• Formula versus cow milk - Fe-deficiency anemia with early introduction (< 1 yr) of cow’s milk.
• Advanced feeding— Stepwise addition of foods (one new food every 3− 4 days)
SOLIDS
• Iron-fortified cereal only at 4-6 months
• Step-wise introduction of strained foods (vegetables and fruits), then dairy, meats (6-9 months; stage I and II)
• Table foods at 9-12 months
• Foods better saved for year 2:

o Egg whites
o Chocolate
o Nuts
o Citrus
o Wheat products
o Fish
**NO HONEY in first year of life – infant botulism
Storage of milk to the left

- Anggelos
COMPLICATIONS OF BREASTFEEDING
Use the mnemonic SELCOM - Anggelos
Problem Prevention Treatment

Sore nipples - Position baby properly so Seek advice from doctor or
nipples are not damaged. midwife
This usually happens because baby - Some signs apart from pain
is no well positioned. that nipples are being hurt are
wedged or pale nipples after
feeding.
Engorgement Breastfeed frequently, 8-12 - Use a hot, moist towel or take a
times in 24 hours hot shower before nursing your
This occurs when more milk is baby.
being produced than is expressed. Express your milk when - Hand express some milk to
This can cause your breasts to feel feedings are missed allow for easier latching.
hard, painful, hot and appear taut or - Massage breast before and
shiny Wean your baby gradually during breastfeeding and
pumping.
- Use a breast pump as necessary
to express milk if your baby is
having problems latching or if
you only nurse on one side per
feeding.
Low milk supply Make sure to prevent being Prevention is the treatment
anxious, stressed, having
doubtful thoughts. Keep baby
close for bonding.
Crack nipples Same as sore nipples Same as sore nipples
Oral thrush - Washing hands carefully Seek advice from doctor

after nappy changes and immediately
This is an infection caused by using separate towels will
Candida fungus. Usually seen with help prevent the infection
cracked or damaged nipples. It spreading.
looks like white creamy patches or - Wash and sterilise any
film anywhere in the mouth. dummies, teats or toys your
baby puts in their mouth.
Mastitis - Talk with your physician and
determine if antibiotics are
This is an infection of the breast necessary.
and is a serious complication. May - Rest.
have flu like presentation with a - Breastfeed frequently and try to
fever BUT THE BREAST IS keep breasts as empty as possible.
PAINFUL. Your baby will not get ill from the
milk.
- Wear a support bra that does not
cause painful pressure.
- Take Tylenol for fever or
discomfort.
Paediatrics
Cerebral Palsy
Source: Nelson’s (p. 2133), Toronto
September 2014
DEFINITION
Cerebral palsy (CP) – A term used to describe a GROUP of PERMANENT disorders of movement and posture, attributed
to NONPROGRESSIVE disturbance in the developing fetal or infant brain.
It is a symptom complex, NOT a disease
NOTE WELL:
• ** The motor disturbance is usually accompanied by sensory, perception, cognition, communication and
behaviour disturbances. (**Motor may sometimes actually be the least of their problems)
• *** CP is historically considered a “STATIC ENCEPHALOPATHY” BUT some of the neurologic features e.g.
movement disorders and orthopaedic complications e.g. scoliosis and hip dislocation can progress over time
INCIDENCE
• 1.5 -2.5/1000 live births
• M > F. 1.4:1 (**More common AND more severe in boys)
• Most common form of chronic motor disability that begins in childhood
AETIOLOGY/RISK FACTORS
CP caused by a broad group (below) of aetiologies that manifest as a common group of neurologic phenotypes:
(**Basically this means a lot of different kinds of insults to the developing brain can result in the damage that manifests itself
as CP. This is why CP itself is NOT a disease, but rather a manifestation of damage caused by other problems**)
• Developmental
• Genetic
• Metabolic
• Ischaemic
• Infectious (Intrauterine exposure to maternal infections)
• Thrombophilic disorders
• Kernicterus
• In utero/Neonatal stroke
***1/3 OF CASES HAVE NO DEFINITE AETIOLOGY!!***

***ALSO, the different types of cerebral palsy have different predominant aetiologies (see table below)
***NOTE: Most children with CP are actually born at term with uncomplicated labour and delivery (80% due to
antenatal factors causing abnormal brain development)
• 10% have evidence of intrapartum asphyxia
• 10% due to postnatal insult
Some important causes!!:

• ***Intrauterine exposure to maternal infection!! – Chorioamnionitis, inflammation of placental membranes,
maternal sepsis, umbilical cord inflammation, UTI associated with increased risk of CP
• Genetic factors: Abnormal IL-6 gene
• Multiple pregnancy (especially if one fetus dies in utero)
• Infertility treatments
• In *premature infants*, major factors are:

o Intraventricular haemorrhage
o Periventricular leukomalacia (PVL)
MUST HAVE A GOOD IDEA OF THIS TABLE
Table 591-1 CLASSIFICATION OF CEREBRAL PALSY AND MAJOR CAUSES
MOTOR SYNDROME (APPROX % OF CP) NEUROPATHOLOGY /MRl MAJOR CAUSES
Spastic diplegia (35%) Periventricular leukomaiacia Prematurity
Periventricular cysts or scars in Ischemia
White matter, enlargement of ventricles, squared of posterior ventricles
Infection
Endocrine/ metabolic (e.g. thyroid)
f
Spastic quadriplegia (20%) Periventricular leukomaiacia Ischemia, infection

Multicystic encephalomalacia Endocrine/metabolic, genetic/developmental
Cortical malformations
Hemiplegia (25%) Stroke: in utero or neonatal Thrombophilic disorders
Focal infarct or cortical, subcortical damage Infection
Cortical malformations
Genetic/developmental
Periventricular hemorrhagic infarction
Extrapyramidal (athetoid, dyskinetic) (15%) Asphyxia: symmetric scars in putamen and thalamus Asphyxia
Kemicterus: scars in globus pallidus, hippocampus Kemicterus
Mitochondrial: scaring globus pallidus, caudate, putamen, brainstem
Mitochondrial
No lesions: ? dopa-responsive dystonia
Genetic/metabolic
CLINICAL MANIFESTATIONS
Table 43. Types of Cerebral Palsy
Type % of Total CP Characteristics Area of Brain Involved
Spastic 70-80% Truncal hypotonia in yr1 st UMN of pyramidal tract
Increased tone, increased reflexes, clonus Diplegia associated with
Affects one limb ( monoplegia ), one side of body periventricular leukomaiacia in
( hemiplegia ), both legs ( diplegia), both arms and premature babies
legs ( quadriplegia) Quadriplegia associated with HIE
( asphyxia), associated with higher
incidence of MR
Athetoid/Dyskinetic 10-15% Athetosis ( involuntary writhing movements ) Basal ganglia ( may be associated
± chorea ( involuntary jerky movements) with kemicterus )
Can involve face, tongue ( results in dysarthria)
Ataxic < 5% Poor coordination, poor balance (wide based gait ) Cerebellum
Can have intention tremor
Mixed 10-15% More than one of the above motor patterns
***CP generally divided into several motor syndromes***:
1. Spastic (80%) – UMN of pyramidal tract involved

a. Monoplegia
b. Diplegia (Most common) - ***Know this one mainly associated with periventricular leukmal. + prematurity
c. Hemiplegia
d. Quadriplegia
2. Athetoid/Dyskinetic (10-15%) – basal ganglia/thalamus involved
3. Ataxic (<5%) – cerebellum
4. Mixed
** NOTE WELL: CP also commonly assc with many developmental disabilities incl. mental retardation, epilepsy,
visual, hearing, speech, cognitive, behavior (The motor problem may be the least of the child’s problems!!!)**
[**Remember it can affect eyes, ears, mouth, intelligence and behavior**]
SPASTIC HEMIPLEGIA (ONE SIDE OF BODY)
• ***Remember it is an UMN lesion!!!! – has inc. reflexes + Babinski + tone + clonus. Spastic in the name
gives it away as well
o *Spasticity refers to the quality of increased muscle tone which increases with the speed of passive
muscle stretching and is greatest in antigravity muscles
• Decreased spontaneous movements on affected side
Upper limbs:
• Arm more involved than leg
• **Hand preference at an early age (found in hemiplegia only: since one side is weaker, will start using other from
earlier because of this)
• Difficult in hand manipulation noted by 1 year
• Upper extremity assumes a flexed posture when child runs
Lower limbs:
• **Walking delayed usu until ~ 18-24 mo
• **Circumduction gait - Gait in which the leg is stiff, without flexion at knee and ankle, and with each step is rotated away from the
body, then towards it, forming a semicircle. Adapted swing phase of gait typical of cerebrovascular accident or any form of head injury
causing motor cortex or cerebellar damage; characteristic forward drag of affected limb (moving foot through an arc away from the
body, whilst toes remain in contact with the support surface), loss or marked reduction of arm swing, and leaning towards the
unaffected side to create sufficient hip height on the affected side to accommodate adapted leg
• Child often walks on tiptoe (on one side) – increased tone in gastrocnemius
• Extremities show growth arrest (especially hand and thumbnail)
o Most apparent in ankles – causes equinovarus of foot
• Increased deep tendon reflexes

• Ankle clonus
• Babinski sign
• About 1/3 of patients with spastic hemiplegia have a seizure disorder that develops in 1st 1-2 yrs
• MRI FAR MORE SENSITIVE THAN CT for most lesions seen in CP
SPASTIC DIPLEGIA (BOTH LEGS) - *MOST COMMON TYPE*
• Spasticity of the legs greater than in the arms

• Usually first indication is when infant begins to crawl – uses arms in the normal reciprocal fashion
BUT tends to drag the legs behind as a rudder – “commando crawl” – rather than using the normal 4-limb
crawling movement
• Spasticity (increased tone) in legs

• Brisk reflexes
• Ankle clonus
• Bilateral Babinski
}))
• If child is suspended by the axillae – scissoring posture of lower extremities
• Walking is significantly delayed legs crossed

like scissors
• Feet in equinovarus
• Walks on tiptoe
• Severe spastic diplegia à Disuse atrophy, impaired lower extremity growth, disproportionate growth
compared to upper torso
PriVntricular Lukomalacia
• ***Most common neurological finding in children with spastic diplegia is PVL – seen on MRI in > 70% of
cases***
o Scarring and shrinkage in the periventricular white matter
SPASTIC QUADRIPLEGIA (ALL LIMBS)
• MOST SEVERE FORM OF CP (Obviously..)

• Marked motor impairment in all extremities AND high association with mental retardation and seizures
• Swallowing difficulties common – due to bulbar palsies – often lead to aspiration pneumonia!!!
• MRI -> Shows severe PVL
• Decreased spontaneous movements

• Increased tone an spasticity in ALL extremities
• Brisk reflexes
• Babinski
ATHETOID/DYSKINETIC CP
• Less common than spastic

• ***Infants characteristically HYPOTONIC with POOR HEAD CONTROL and marked HEAD LAG
• Develop variably increased tone with rigidity and dystonia over years
• Athetosis = involuntary writhing movements. Chorea = involuntary jerking movements

• Dystonia -> abnormality in tone where muscles are rigid throughout their range of motion and involuntary
contractions can occur leading to fixed limb postures
• Unlike spastic, UPPER EXTREMITIES GENERALLY MORE AFFECTED THAN LOWER
• Speech is typically affected (*dysarthria) – because oropharyngeal muscles involved

• Tongue thrust and drooling may occur
• Mostly associated with BIRTH ASPHYXIA

• Also associated with KERNICTERUS
DIAGNOSIS
• Thorough history and examination to exclude progressive disorder – e.g. tumour, degenerative disease, muscular
dystrophy
• MRI of brain to assess location and extent of lesions
• Tests of hearing and vision
• Chromosome studies, serology, neuroimaging, EMG, EEG (if seizures)
TREATMENT
• Best treatment for CP is prevention before it occurs – difficult
• Maximize potential through multidisciplinary services such as primary care physician, OT, PT, SLP, school
supports, etc.
• Orthopedic management (e.g. dislocations, contractures, rhizotomy – divide roots of spinal nerves)’
• Management of symptoms:
o Spasticity (Dantrolene, Benzodiazepine, Baclofen, Botox.)
o Constipation (stool softeners)
o Wheelchairs in quadriplegics
Paediatrics September 2014 Wayne Robinson, MBBS Class of 2015
ANEUPLOIDIES: Change in the number of chromosomes that results from nondisjunction. Cell may have one (monosomy) or three (trisomy) copies of a particular chromosome
TRISOMY 21 TRISOMY 18 TRISOMY 13

Disease DOWN SYNDROME (47, XX, +21 OR 47 XY, +21) EDWARD’S SYNDROME (47, XX +18 (OR XY)) PATAU SYNDROME (47, XX, +13 (OR XY))
MUST SEE MY SEPARATE DOWN SYNDROME NOTES!!!!!!
nd
Incidence 1:600-800 - **Most common autosomal chromosomal abnrmlty 1:6000 live births (95% abort in T1) *2 most 1:10,000
- Rises w adv maternal age: 1:1500 @ 20 to 1:20 @ 45 common trisomy ~ 9% live past age 1
st nd
- *Most cases diagnosed in newborn period - Female > Male (3:1) (33% die in 1 mo, 50 by 2 , 90% by 1 yr)
- < 10% survive to age 1
Facial fts - Brachycephaly (short, broad head) - Microcephaly - Microcephaly, sloping forehead
- Microcephaly - Prominent occiput - Scalp lesion over occiput: “Aplasia cutis congenita” –
- Flattened occiput - Micrognathia (small jaw) Lesion above is PATHOGNOMONIC
- Hypoplastic midface **MIDLINE facial defects:
- Short neck - Cyclopia (single orbit), cebocephaly, cleft lip/palate
Eyes - Prominent medial epicanthic folds Microphthalmia Microphtalmia, or anopthlamia
- Upslanting eyes, speckled iris (Brushfield spots)
Disorders: Cataracts, myopia, strabismus
Ears Low-set, small (microtia) Low-set, malformed Low-set, small, malformed
Overfolding of superior helix
Disorders: Frequent AOM, hearing loss
Nose - Flattened nasal bridge, small nose Narrow nose, hypoplastic alae
Mouth - Open mouth with protruding tongue (not true macroglossia) Cleft lip or palate
Extremities - Brachydactyly: Short, broad hands - Hypoplastic nails - Polydactyly

- Clinodactyly: Short, incurved little fingers - Polydactyly, clinodactyly - Clubfeet or rocker-bottom feet
nd th
- Single palmar crease **Characteristic fist-clenching: 2 and 5 digits
st nd rd th
- Wide “sandal gap” between 1 & 2 toes overlap 3 and 4
- Rocker-bottom feet
Cardiac Defects in ~50% Defects in 60% Defects in 80%
** AVSD > VSD > ASD/PDA. Also mitral valve dx VSD, PDA, ASD VSD, PDA, ASD
GI Top 3: **Inguinal hernias
1. Duodenal atresia, 2. Annular pancreas 3. Imperforate anus
- Also Hirschsprung’s, oesoph. atresia
GU Cryptorchidism, infertility common Cryptorchidism *Hypospadias & *cryptorchidism in boys
PKD *Hypoplasia of labia minora in girls
PKD
CNS *HYPOTONIC & marked head lag at birth *HYPERTONIC **MIDLINE CNS defects: ***Alobar holoprosencephaly
Low IQ (25-70), developmental delay *Severe developmental delay Seizures
Deafness, severe developmental delay
OTHER **Normal birth wt & lgnth **Small and premature appearance ant birth Single umbilical artery
*Short stature (Usu small for gestational age (SGA)) Profound intellectual disability
*Dysplastic hips *Short stature
*Congenital/acquired hypothyroidism *Short sternum
* 1% risk of leukemia. < 2yo -> Acute megakaryoblastic leuk
> 2 yo -> ALL
*Polycythemia
*Atlantoaxial instability – increased distance btwn C1 & C2 -> incr
risk of spinal cord injury
*Inc risk of infection
* Alzheimer’s > 35 yo common
Prognosis/ Dx with karyotyping
Mgmt CBC, echo, yearly thyroid test, atlanto-occipital x-ray at 2 yr, sleep
study, hearing test, and ophthalmology assessment
SEX CHROMOSOME DISORDERS
KLINEFELTER SYNDROME TURNER SYNDROME FRAGILE X SYNDROME
GENOTYPE 47, XXY (most common), 48, XXXY 45, X most common (50%) X-linked
INCIDENCE 1:1000 live MALE births 1:4000 live FEMALE births, NOT assc with advanced maternal age 1:3600 males, 1:6000 females
Increased with advanced maternal age - Reason: The nondisjunction occurs after conception. Not in the ovum
- ONLY monosomy that can survive to term!! Most common heritable cause of intellectual
- 99% spontaneously aborted (most common chrm abn assc w disability in boys
spontaneous abortion!!)
PHENOTYPE TALL, long limbs, slim, underweight SHORT stature is a cardinal feature **Overgrowth**:
- Long and thin face
****Before puberty, pts phenotypically - Face: Triangular face - Prominent jaw, forehead, and nasal bridge
indistinguishable from normal population - Eyes: Epicanthal folds - Large protuberant ears
- Ears: Low-set, mildly malformed - High arched palate
Often dx at *age 15-16 when axillary/pubic hair - Nose: Flat nasal bridge - Macroorchidism
develop but testes remain infantile. - Hyperextensibility
- Neck: Short neck + webbing of neck +/- cystic hygroma
Adults: Gynaecomastia - Broad, shield-like chest w. wide internipple distance
- Extrm: Puffy hands and feet (lymphoedema)
IQ Mild intellectual disability, behavioural or psychiatric *Most have normal IQ and life expectancy Mild to moderate intellectual disability, 20% of
disorders affected males have normal IQ
Reproduction Failure of growth/maturation of testes lead to: ***Have streak gonads (Gonadal dysgenesis) -> oestrogen Premutation carrier females at risk of developing
o o
*** Low testosterone -> no male 2 sexual chars - deficiency -> do not develop 2 sexual characteristics + primary premature ovarian failure
o
> no facial hair, no deep voice, no libido. amenorrhoea (sometimes 2 )
- Later -> osteopenia and osteoporosis 10% normal pubertal development/fertile
- Most infertile -> few viable sperm

(hypo/azospermia)
** Can still father children with (will be normal):

- Testicular biopsy + IVF + ICSI
OTHER Notable for its mild physical and developmental findings Complications:
Seizures, scoliosis, mitral valve prolapse
Congenital cardiac defects in 45%:
***Coarctation most common!!!!!! > bicuspid aortic valve. Post stenotic
dilatation later
Renal (~50%): Horseshoe kidney

*Inc risk of HTN
*Acquired hypothyroidism
DX/MGMT ** Increased risk of germ cell tumours and ** 33% dx in neonate due to CHD, 33% childhood, 33% adolescence Dx: Molecular testing of FMR1 gene
breast cancer!!!! Management:
o
- Most need oestrogen replacement to develop 2 characteristics
Management: - BUT nfertility NOT corrected with oestrogen replacement
Testosterone supplementation is indicated in - Use ART with donor ova. Must monitor in pregnancy due to poststenotic
adolescence aortic dilation -> possible dissecting aneurysm
- ECHO, ECG to screen for cardiac malformation
- GH therapy for short stature
VERY BRIEF NOTES ON OTHER GENETIC DISORDERS
NOONAN SYNDROME
• 46, XX or 46, XY
• Autosomal Dominant!! (NOT a sex chromosome disorder) with variable expression
• Higher transmission of affected maternal gene
• 1:2000 male AND female live births
• ***Certain phenotypic features similar to females with Turner syndrome; therefore, sometimes called the “male Turner syndrome”, although it affects
both males and females (Unlike Turner’s which can only affect females)
o Short stature, webbed neck, triangular facies, hypertelorism, low set ears, epicanthic folds, ptosis (**basically same as Turner)
o Pectus excavatum
o Right-sided congenital heart disease: **Pulmonary stenosis = most common. (Turner’s syndrome has mainly left-sided CHD)
• Moderate intellectual disability in 25% of patients
• Delayed puberty
• Management:
o Affected males may require testosterone replacement therapy at puberty. ECHO, ECG
Disorders with dermatological features:

1. EHLERS-DANLOS SYNDROME
• Numerous types exist
• Classical EDS is Autosomal Dominant
• All forms:
o SKIN: Soft, fragile skin, easy ugly bruising and scarring, thin ‘cigarette paper’ scars
o Varicose veins
o Musculoskeletal discomfort, osteoarthritis
2. NEUROFIBROMATOSIS TYPE 1
• Autosomal Dominant – Mutation in NF1 gene on chromosome 17
• ***Clinical diagnosis requires 2 or more of the following:
1. 6 or more café-au-lait spots (means coffee with milk) (> 0.5 cm in children)
2. 2 or more neurofibromas of any type (dermal neurofibromata are small lumps in the skin that appear in adolescence) or 1 or more plexiform
neurofibromata;
3. 2 or more Lisch nodules (benign iris hamartomas)
4. Freckling in the axilla, neck, or groin
5. Optic glioma (tumour in the optic pathway)
6. A distinctive bony lesion, e.g. sphenoid wing dysplasia, or dysplasia or thinning of the long bone cortex, e.g. pseudoarthrosis
7. First-degree relative with NF1.
• Small risk of serious complications, e.g. scoliosis, pressure effects of tumours or malignant change, (e.g. neural crest tumours), hypertension
3. TUBEROUS SCLEROSIS
• Autosomal Dominant multisystem disorder
• Characterized by **hamartomas in the brain, skin, and other organs.
• Presents with INFANTILE SPASMS. **Seizures and mental retardation are often associated
• Hypomelanotic macules **(‘ash-leaf’ spots) occur in ~95% by age 5 yrs. A Wood’s light (UV) may be needed to visualize these
• **Angiofibromas occur in later childhood in a butterfly distribution over the nose and cheeks.
• Other cutaneous features include forehead fibrous plaque, shagreen patches, ungual fibromata, and dental pits.
• Thorough clinical evaluation, e.g. cranial MRI, eye exam, renal US, is indicated to make the diagnosis prior to genetic testing
Other chromosomal disorders:

PRADER-WILLI – Chromosome 15 affected. Associated with OBESITY and SHORT STATURE. Insatiable appetite. DM II. Hypotonia.
DIGEORGE SYNDROME
o Chromosome 22 affected.
o Second most common genetic diagnosis (next to Down syndrome)
o Clinical features: “CATCH 22”

- *Cyanotic CHD
- Anomalies: Micrognathia and low set ears
- *Thymic hypoplasia - “immunodeficiency” - recurrent infections
- Cognitive impairment
- Hypoparathyroidism, hypocalcaemia
- 22q11 microdeletions
DUCHENNE MUSCULAR DYSTROPHY

[Straight from Toronto Notes 2014]
Epidemiology
• 1:4000 males
Etiology
• X-linked recessive: 1/3 spontaneous mutations, 2/3 inherited mutations
• Missing structural protein (dystrophin) -> muscle fibre fragility -> fibre breakdown -> necrosis and regeneration
Clinical Presentation
• Proximal muscle weakness by age 3, positive Gower’s sign, waddling gait, toe walking
• Pseudohypertrophy of calf muscles (muscle replaced by fat) and wasting of thigh muscles
Gower 's Sign
• Decreased reflexes
• Non-progressive delayed motor and cognitive development (dysfunctional dystrophin in brain)
Child uses hands to "climb up" the legs
• Cardiomyopathy to move from a sitting to a standing
position.
Diagnosis
• Molecular genetic studies of dystrophin gene (DMD) (first line)
• Family history (pedigree analysis)
• Increased CK (50-100x normal) and LDH
• Elevated transaminases
• Muscle biopsy, electromyography (EMG)
Management
• Supportive (e.g. Physiotherapy, wheelchairs, braces), prevent obesity
• Cardiac health monitoring and early intervention
• Bone health monitoring and intervention (vit D, bisphosphonates)
• Steroids (e.g. Prednisone or deflazacort)
• Surgical (for scoliosis)
• Gene therapy trials underway
Complications
• Patient usually ***wheelchair-bound by 12 yr of age

• Early flexion contractures, scoliosis, osteopenia of immobility, increased risk of fracture
• *** Death due to pneumonia/respiratory failure or CHF in 2nd-3rd decade
SPINAL MUSCULAR ATROPHY

Paediatrics
Congenital Heart Disease
Source: Nelson’s (p. 1621 of 2682) (For pathophysiology), Toronto Notes (Summary)
September 2014
**DETAILS OF EACH SPECIFIC LESION ACTUALLY START AT THE END OF PAGE 4**
PRENATAL CIRCULATION
oxygenated blood deoxygenated blood

from placenta R atrium
returns via SVC to
Aorta
Superior Vena Cava Ductus Arteriosus
i
umbilical vein 1/3 of blood entering 1
R atrium does not flow
through foramen ovale and
ductus venosus flows to the R ventricle Foramen Ovale
IVC
i
pulmonary arteries !
Pulmonary Trunk
\1
I
R atrium
I
ductus arteriosus
Inferior Vena Cava
\
Descending Aorta
shunted through aorta

foramen ovale
i i
systemic circulation
Liver
Ductus Venosus
L atrium
i
placenta for reoxygenation
L ventricle
i
aorta
brairVmyocardium/
upper extremities Placenta Umbilical Arteries
© Bonnie Tang 2012
Fetal circulation is designed so that oxygenated blood is preferentially delivered to the brain and myocardium.
Embryologic Development
• Most critical period of fetal heart development is between 3-8 wks gestation
• Single heart tube grows rapidly forcing it to bend back upon itself and assume the shape of a four chambered
heart, insults at this time are most likely to lead to CHD
Characteristic Chest X-Ray Findings in
Before Birth Congenital Heart Disease
Fetal lungs are bypassed by flow through “fetal shunts”: 3 MAIN SHUNTS: • Boot- shaped heart: tetralogy of Fallot,
• Shunting deoxygenated blood tricuspid atresia
o Ductus arteriosus: Connection between pulmonary artery and aorta • Egg- shaped heart: transposition of
great arteries
• Shunting oxygenated blood • "Snowman" heart: total anomalous
o Foramen ovale: Connection between R and L atria pulmonary venous return
o Ductus venosus: Connection between umbilical vein and IVC
At Birth
With first breath, lungs open up and pulmonary resistance decreases allowing pulmonic blood flow
• Separation of low resistance placenta --> systemic circulation becomes a high resistance system --> ductus
venosus closure
• Increased pulmonic flow --> increased left atrial pressures --> foramen ovale closure
• Increased oxygen concentration in blood after first breath --> decreased prostaglandins --> ductus
arteriosus closure
• Closure of fetal shunts and changes in vascular resistance --> infant circulation assumes normal adult flow
COMMON CONGENITAL HEART DISEASES

Congenital Heart Disease
I
Acyanotic Cyanotic ( 5 T" lesions )
L -> R shunt Obstructive R Lshunt Other
ASD Coarctation
i
TOF
i
TGA
VSD Aortic stenosis Ebstein's anomaly Truncus arteriosus
PDA Pulmonic stenosis Total anomalous
Atrioventricular pulmonary venous
Septal defect drainage
( endocardial Tricuspid atresia
cushion defect ) Hypoplastic left
heart syndrome
EPIDEMIOLOGY
8/1000 (0.8%) live births have CHD, which may present as a heart murmur, heart failure, or cyanosis; ventricular
septal defect (VSD) is the most common lesion (by far – 30-35%)
INVESTIGATIONS
• Echocardiogram, ECG, CXR, CT, MRI, cardiac catheterization
CYANOTIC VS. ACYANOTIC CONGENITAL HEART DISEASE
• Cyanosis: blue mucous membranes, nail beds, and skin secondary to an absolute concentration of deoxygenated
haemoglobin of at least 5 g/dl
• **Anaemic patients may not become cyanotic even in the presence of marked arterial desaturation.
REASON:
o The presence of cyanosis is dependent upon there being an absolute quantity of deoxyhaemoglobin (>
5g/dl). In a patient with overall less haemoglobin who has a large portion of their haemoglobin being
deoxygenated, may still not have enough of deoxygenated haemoglobin to reach the minimum (5 g/dl) for
cyanosis. Example: A patient with a Hb of 8 g/dl, with 50% of that (ie. 4 g/dl) being deoxygenated Hb, is still less likely to
show cyanosis than a person with an Hb of 12 with 50% deoxygenated (ie. 6 g/dl), even though the anaemic patient still has less
oxygen available overall. In fact, the patient with Hb 12 would have already reached the 5 g requirement and be cyanosed while
still at a higher oxygen saturation that the anaemic patient, who is not yet cyanosed but has a lower oxygen sat
• Therefore, patients with polycythaemia develop also develop cyanosis at higher oxygen saturation levels
Applying this understanding:
• Acyanotic heart disease: (i.e. L to R shunt or obstruction occurring beyond lungs) blood passes through pulmonic
circulation -> oxygenation takes place -> low levels of deoxygenated blood in systemic circulation -> no cyanosis
• Cyanotic heart disease: (i.e. R to L shunt) blood bypasses the lungs -> no oxygenation occurs -> high levels of
deoxygenated hemoglobin enters the systemic circulation -> cyanosis
ACYANOTIC CONGENITAL HEART DISEASE
General Points to note:
Classified as in flow diagram above (L->R shunts = volume load lesions, obstructive = pressure load lesions)
1. Volume load/L->R shunt lesions: ASD, VSD, PDA, AVSD (AVSD is aka. AV canal, aka. Endocardial cushion
defect)
• All have communication between pulmonary and systemic circulation resulting in shunting of fully oxygenated
blood back to lungs.
• Shunt can be quantitated by calculating ratio of pulmonary to systemic blood flow (Qp:Qs). A 2:1 shunt implies
twice the normal pulmonary blood flow.
• Shunt direction and volume dependent upon three major factors: (1) size of defect (2) pressure gradient
between chambers or vessels (3) peripheral outflow resistance. Also compliance of the 2 connected chambers
• Chronic exposure of pulmonary circulation to high pressure and blood flow results in gradual increase in
pulmonary vascular resistance. Eventually may have a reversal of flow: Eisenmenger pathology
• Increased blood volume in lung (from the shunt) -> decreased lung compliance -> pulmonary oedema ->
symptoms of “heart failure”. However the heart itself is actually in a high output state, just that blood intended for
the systemic circulation is still being lost through a shunt.
• [Contrast with cardiomyopathies where heart muscle function actually decreases. Major causes of
cardiomyopathy in infants -> viral myocarditis, metabolic disorders, genetic defects]
• IMPORTANT: **HR and SV increase to maintain this high output. Mediated by sympathetic catecholamines.
This just worsens the problem as the sympathetic NS further increases body oxygen consumption.
o SNS activation also leads to symptoms incl. sweating etc. and the oxygen shortage leads to FAILURE
TO THRIVE.
• Heart remodeling occurs -> Mostly dilatation!! Some hypertrophy. (**LàR shunt lesions = eccentric
hypertrophy. Note: Compare with obstructive lesions, which cause more hypertrophy than dilatation and it is
concentric hypertrophy)
• Left untreated -> pressure builds up and eventually shunt reverses (Eisenmenger pathology)
2. Pressure load lesions (Obstructive) – (Coarctation of aorta, aortic stenosis, pulmonic stenosis)
• Obstruction to normal blood flow (Most commonly to ventricular outflow)
• Compensation predominantly involves hypertrophy!! But in later stages -> also dilatation
• Mild may be asymptomatic or minimal symptoms. Severe may lead to heart failure.
o Pulmonic stenosis -> Right heart failure incl. hepatomegaly, ascites, peripheral oedema
o Aortic stenosis -> Left heart failure incl. pulmonary oedema, poor perfusion AND right heart failure
o Coarctation -> Upper body HTN, diminished lower extremity pulses
***GENERAL INFORMATION***
• Chest X-ray good in determining if volume load problem (L->R shunt) OR pressure load problem (obstruction).
“Increased pulmonary vasculature”!!!! in shunts/volume load disease. Also cardiomegaly eg. in VSD
• ECG also useful to determine any hypertrophy of any chamber
A key point to note is that there are usually 3 factors that determine the extent/severity of a shunt:
1. The SIZE of the defect
2. The pressure gradient between chambers or vessels
3. Peripheral outflow resistance
• Symptoms of each abnormality depend upon the SEVERITY of the shunt or obstruction
• When thinking about signs/examination findings – think pulses (incl. volume, RF delay, collapsing/bounding,
comparison of upper and lower extremity pulses), apex beat (displaced eg. cardiomegaly? Character?), thrills?,
murmurs?
• Findings of most investigations ALSO really depend on the severity of the defect (size, etc). May be anywhere
from normal to very abnormal. Eg. Cardiomegaly or LVH in PDA. May be absent of severe depending on size of
defect.
• [TRY TO FIGURE OUT WHICH CHAMBERS/VESSELS ARE ENLARGED IN EACH CONDITION BASED
ON WHICH PART OF THE HEART/VESSELS IS UNDER MORE STRAIN/OVERWORKING WITHOUT
LOOKING]
• Just know that echo is diagnostic for each. It shows the exact structural defect and flow etc.
• So when thinking of investigations think: 1. ECG (where is enlarged or hypertrophied), 2. CXR, 3. Echo
• For each condition, know the ECG findings (Eg. LVH/RVH), X-ray findings (e.g. increased pulmonary vascular
markings) and auscultation findings (e.g. murmurs, splitting etc.)!!!
VERY IMPORTANT ASSOCIATIONS TO KNOW (FROM LECTURE)!!!!
Enviromental factors Syndromes with CHD
Disorder % Types Syndrome % with Types

CHD
Rubella 50 PDA. Pul Br Sten Trisomy 21 50 AVdef, VSD.TOF
Mat . diabetes 3-5 TGV. VSD. CoA Turner 30 Co Aorta
Mat . Phenylket 30 TOF. VSD. ASD Noonan 65 PS, ASD. ASH
Thalidomide 15 .
TOF, TGV DORV Tr 13 80 VSD. PDA
Foetal alcohol 35 VSD. ASD. TOF Tr 18 90 VSD. PDA
Marfan 60 MVP, AoA, AR
A. R -> L SHUNT/VOLUME OVERLOAD LESIONS
ATRIAL SEPTAL DEFECT (ASD)

3 MAIN TYPES
1. Ostium secundum – MOST COMMON – 50-70%
2. Ostium primum – common in Down syndrome (But Downs mainly associated with AVSD!)
3. Sinus venosus – defect at entry of SVC into RA
(Rarely, entire septum --> Single atrium)
INCIDENCE
• Majority sporadic. Some part of the AD syndrome “Holt-Oram” syndrome)
• Females > Males – 3:1
BRIEF PATHOPHYS
• See factors affecting degree of shunting above

• 80-100% spontaneous closure rate if ASD diameter < 8 mm
• BUT overall closure rate = 40%
• Large defects -> Qp:Qs usually between 2:1 and 4:1
• Minimal symptoms in newborn. [Because RV has thicker walls limiting L->R shunt. With age, pulm. resistance drops
so RV doesn’t need to be as thick -> increased shunt]
• With age -> increased L->R shunt -> RA and RV enlargement -> dilatation of pulmonary artery [ie. basically
whole right side enlarges]
o LA may also enlarge BUT LV and aorta are NORMAL size
• NOTE!!! Despite the large pulmonary blood flow, pulmonary arterial pressure is usually normal as atrium to
atrium is NOT a high-pressure communication UNLIKE in a VSD. However in adulthood it may start to increase
-> possible CHF in adulthood
CLINICAL FEATURES
• Often asymptomatic in childhood. Found on routine physical exam.
• May have subtle failure to thrive
• Majority have no symptoms until 20s – 30s when CCF, pulmonary HTN and AF develop
EXAMINATION FINDINGS INCL. AUSCULTATION

• Subtle but characteristic
• Mild left precordial bulge
• Sometimes pulmonic ejection click
nd
• Most patients: Characteristic finding is WIDE AND FIXED splitting of the 2 heart sound in ALL
phases of respiration
o [Because with an ASD, right ventricular diastolic volume is constantly increased, so the ejection time is
always prolonged in any part of respiration, unlike in normal where RV volume increases during
inspiration due to increased venous return]
• Ejection systolic murmur Grade 2-3/6. Best heard at ULSE.
o Due to increased flow across pulmonary artery valves
o (Remember what causes a murmur: Increased flow through a normal valve OR “normal” flow through an
abnormal valve. The former occurs in this case)
• +/- Mid-diastolic murmur heard best at LLSE with bell
o Due to increased flow across tricuspid. Same explanation as above.
NOTE WELL!!! Flow through the actual ASD itself has NO murmur!!!
INVESTIGATIONS
• ECG: Right axis deviation (RAD), mild RVH, right bundle branch block (RBBB)
• CXR: Increased pulmonary vasculature (remember this occurs in L->R shunts due to increased pulm. blood flow)
• ECHO diagnostic
MANAGEMENT
• Elective surgical or transcatheter closure between 2-5 yr of age
• Preferred procedure is percutaneous catheter closure with AS occlusion device using transvenous approach
in cardiac cath. lab
ATRIOVENTRICULAR SEPTAL DEFECTS (AVSD)

Aka. “AV canal defect” or “Endocardial cushion defect”
AV septal defect = Hole between the atria and between the ventricles
INCIDENCE
• Common in children with Down syndrome (This is the most common cardiac defect in Down’s!!!!)
BRIEF PATHOPHYS
• In complete AV septal defects, the L->R shunt occurs at both the atrial and ventricular levels.
• Pulmonary HTN and early increased pulmonary vascular resistance common (unlike ASD)
• With time --> Eisenmenger
CLINICAL FEATURES
[For complete AV septal defect]:
• Heart failure and intercurrent infections (why?) from infancy
EXAMINATION FINDINGS
• Signs of failure to thrive

• Precordial bulge
• Liver enlarged (due to heart failure)
• Moderate to marked cardiomegaly (laterally displaced apex beat)
• Systolic thrill at LLSE
• Widely split 2nd heart sound possible (same reason as ASD above)
• Mid-diastolic murmur at LLSE, low-pitched (bell) – Increased tricuspid flow
• Pulmonary ejection systolic murmur (ULSE)
• Possible holosystolic (pansystolic) murmur of mitral regurg.
INVESTIGATIONS
• ECG: See Nelson’s
• CXR: Mod-severe cardiomegaly, large pulmonary artery, increased pulmonary vascularity
• ECHO diagnostic
MANAGEMENT
• Surgical correction in infancy because of risk of pulmonary vascular disease.
VENTRICULAR SEPTAL DEFECT (VSD)

INCIDENCE
MOST COMMON CARDIAC MALFORMATION (Nelson’s says 25%, Toronto says 30-50%)
• Most are of the membranous portion of septum
Small VSD (majority)

• Clinical presentation:
o History: asymptomatic, normal growth, and development
o Physical exam: characteristic early systolic to holosystolic murmur, best heard at LLSE (VSD murmur), thrill
• Investigations: ECG and CXR are normal
• Management: Most close spontaneously (30-50%)
BASIC PATHOPHYS
• Size of defect is a major determinant of L->R shunt
• Also level of pulmonary vascular resistance
• 2 types:
o Restrictive (usu. < 5mm): RV pressure is normal
o Nonrestrictive (usu. > 10mm): RV and LV pressure EQUAL and magnitude of shunt determined by ratio
of pulmonary to systemic vascular resistance
• Pulmonary vascular resistance high at birth so L->R shunt low at birth -> normally decreases due to
involution of the media of the small pulm. arterioles -> falls after 1st few weeks of life -> L->R shunt increases ->
symptoms develop.
• Continued exposure of pulmonary vessels to high pressure from shunt -> “pulmonary vascular obstructive
disease” develops -> PVR:SVR reaches 1:1 -> bidirectional flow -> eventually reversal (Eisenmenger)
• Pulmonary HTN occurs in 10% OF LARGE DEFECTS
CLINICAL FEATURES
• Small VSDS (SEE ABOVE)
• LARGE VSD:
o History: (Because of pulmonary HTN): Dyspnoea, poor growth/failure to thrive, profuse sweating
(sympathetic NS – see explanation above), recurrent pulmonary infections (why?)
o CHF by 2 months
EXAMINATION
• Left precordial bulge
• Laterally displaced apex beat, thrusting
• Characteristic early systolic to holosystolic murmur, best heard at LLSE (VSD murmur), thrill (Due to
flow through VSD)
• NOTE: SIZE OF VSD IS INVERSELY RELATED TO INTENSITY OF MURMUR
• [POSSIBLE: Mid-diastolic, low-pitched rumble at the apex is caused by increased blood flow across the mitral valve and indicates a
Qp:Qs ratio of ≥ 2:1]
INVESTIGATIONS
• Small VSD (See above)
• Large VSD:
o ECG: LVH + RVH + LAH
o CXR: Gross cardiomegaly with both ventricles prominent, increased pulmonary vasculature, frank
pulmonary oedema + pleural effusion
o ECHO diagnostic
MANAGEMENT
• Small VSD (See above)

• Large: Treatment of CHF and surgical closure by 1 yr old
• Treat CCF, FTT -> increased calories (prior to correction)
PATENT DUCTUS ARTERIOSUS (PDA)

INCIDENCE
F > M, 2:1
BASIC PATHOPHYS
• Patent vessel between descending aorta and left pulmonary artery (normally, functional closure within first 15
hrs of life, anatomical closure within first days of life due to oxygen exposure inhibiting prostaglandins
which keep the ductus open in fetal life)
• The aortic end of the ductus is just distal to the origin of the left subclavian artery. Pulmonary end is
at the bifurcation of the pulmonary artery
• Association with maternal rubella infection!!! in early pregnancy
• PDA common in premature infants as smooth muscle of the ductus wall in preterm is less responsive to the high
pO2 after birth. BUT a PDA in term infants actually has defects of the media and endothelium compared to
normal PDA in preterm.
• As a result, PDA persisting in a term infant RARELY closes spontaneously or with pharmacologic intervention.
Whereas in preterm majority close spontaneously
• If small, pulmonary pressures, RV and RA pressures normal

• If large, pulmonary arterial pressures may be elevated to systolic levels. High risk of pulmonary
vascular disease if left unoperated
CLINICAL FEATURES
• Small usually asymptomatic. Large usually results in CHF (as seen in VSD)
History
• Growth retardation
EXAMINATION (For Large Defects)

• CCF in early infancy usually
• Hyperactive precordium
• Tachycardia
• Bounding peripheral pulses
• Wide pulse pressure. May be collapsing
• +/- Cardiomegaly if PDA large
• Thrill in 2nd LICS. Usu systolic
nd
• CLASSIC MURMUR: Continuous “machinery” murmur localized to 2 LICS or radiating to left
clavicle
INVESTIGATIONS
• ECG: May show LAE, LVH, RVH
• CXR: Normal to mildly enlarged heart, increased pulmonary vasculature, prominent pulmonary artery
• ECHO diagnostic
MANAGEMENT
• For preterm patients: Indomethacin (Indocid®): PGE2 antagonist (PGE2 maintains ductus arteriosus
patency)
• Term: Catheter or surgical closure if PDA causes respiratory compromise, FTT, or persists beyond 3rd month of
life
• So ALL PDA in TERM patients require surgical closure whether small or large!! Different rationale for small and
large but still every one should be surgically closed if it is still patent after the 3rd month (to 1 year?) of life
B. OBSTRUCTIVE LESIONS
COARCTATION OF THE AORTA

INCIDENCE
• M > F, 2:1
BASIC PATHOPHYS
• Can occur at any point from transverse arch to iliac bifurcation!!
• BUT 98% occur just below the origin of the left subclavian artery at the origin of the ductus
arteriosus (juxtaductal)
• Coarctation may be a feature of Turner syndrome (**This is the most common cardiac defect in Turner’s!!!!)
• Associated with a bicuspid aortic valve in 70% of patients
• In patients with discrete juxtaductal coarctation, ascending aortic blood flows through the narrowed segment to
reach the descending aorta although LV hypertension and hypertrophy result
• Blood pressure is elevated in the vessels that arise PROXIMAL to the coarctation. Blood pressure as well as pulse
pressure is lower below the constriction
CLINICAL FEATURES
Often asymptomatic.
Generally 2 groups: Those who present early with heart failure and those who present later with HTN
• Neonates: Differential cyanosis (Pink upper and cyanotic lower extremities) is seen in a neonate with severe
coarctation of the aorta and with a large PDA with a right-to-left shunt into the descending thoracic aorta. Heart
failure, shock when PDA closes.
• Infants and children: Most asymptomatic. May be incidental finding based on investigation for HTN or murmur.
4-limb BP important.
• Adolescents and adults: Medscape: In this age group, BEST diagnosed clinically based on simultaneous
palpation of femoral and brachial pulses (Radiofemoral or brachiofemoral delay). Also, BP in both arms and 1 leg
must be determined. Pressure difference > 20 mmHg in favour of arms may be evidence of coarctation of
aorta.
EXAMINATION
• Classic sign is a disparity in pulsation AND blood pressure in the arms and legs
• Radiofemoral delay – NOTE: Normally the femoral pulse occurs slightly BEFORE the radial pulse
• In normal persons (except neonates), systolic BP in the legs is 10-20 mmHg higher than in the
arms. In coarctation, BP in the legs is lower than that in the arms. Frequently it is difficult to obtain
• It is important to determine the BP in each arm. REASON: A pressure HIGHER IN THE RIGHT than the left arm
suggests involvement of the left subclavian in the area of coarctation. Less commonly, the right subclavian may
arise anomalously from below the area of coarctation and result in a left arm pressure that is higher than the right
--
• Upper extremity systolic pressures of 140-145 mm Hg
• Few have high BP in infancy (160-200 mm Hg systolic), but this decreases as collaterals develop
• Decreased blood pressure and weak/absent pulses in lower extremities
• Radial-femoral delay
• Absent or systolic murmur with late peak at apex or LSE, left axilla, and left back (Nelson’s says left
sternal border along 3rd and 4th ICS transmitted to left infrascapular region (on back auscultation))
• If severe, presents with heart failure/shock in the neonatal period when the ductus closes
INVESTIGATIONS
• ECG: Neonates - RVH. Older patients – LVH instead
• CXR: Cardiomegaly (usually after age 10). Pulmonary congestion. Descending aorta has a poststenotic
dilatation
o Notching of the inferior border of the ribs from pressure erosion by enlarged collateral vessels is
common by late childhood.
• ECHO diagnostic
MANAGEMENT
• Give prostaglandins to keep ductus arteriosus patent for stabilization and perform ->
• Surgical correction in neonates. For older infants and children balloon arterioplasty may be an alternative
to surgical correction.
AORTIC STENOSIS
INCIDENCE: M >F, 3:1
3 TYPES
• Valvular (75%), subvalvular (20%), supravalvular (least common)
• In valvular (most common), leaflets are thickened and the commissures are fused. LVH occurs in
compensation and as its compliance decreases, end diastolic pressure increases
CLINICAL FEATURES
• Depend on severity of obstruction
• Severe stenosis occurring early in infancy = “CRITICAL AORTIC STENOSIS” – associated with LVH and
signs of low cardiac output
HISTORY
• CHF features: Dyspnoea, orthopnoea, PND
• Exertional chest pain, syncope
• Sudden death
EXAMINATION
• Mild = Normal examination
• Weak pulses in ALL extremities
• CHF
• Displaced apex beat = Cardiomegaly
• Pulmonary oedema (crackles)
• Thrill assc with murmur below
• Ejection systolic murmur, URSE, radiates to neck with ejection systolic click at apex (only for
valvular!!)
• Possible 4th heart sound
INVESTIGATIONS
• ECG: Normal or LVH
• CXR: Prominent ascending aorta (post-stenotic dilation). Heart size usually normal
• ECHO diagnostic
MANAGEMENT
• Valvular stenosis is usually treated with balloon valvuloplasty if moderate to severe
• Patients with subvalvular or supravalvular stenosis require surgical repair, exercise restriction required
• IE prophylaxis?
Pulmonary Stenosis (Copy/Paste from Toronto Notes)

• 3 types: valvular (90%), subvalvular, or supravalvular
• definition of critical pulmonic stenosis:
. inadequate pulmonary blood flow, dependent on ductus for oxygenation, progressive
hypoxia and cyanosis
• natural history: may be part of other congenital heart lesions (e.g. Tetralogy of Fallot) or in association with syndromes (e.g. congenital rubella,
Noonan syndrome)
• clinical presentation
.history: spectrum from asymptomatic to CHF
.physical exam: wide split S2 on expiration, SEM at ULSE, pulmonary ejection click (for valvular lesions)
• investigations:
.ECG: RVH
.CXR: post-stenotic dilation of the main pulmonary artery. Decreased pulmonary vascular markings
• management: surgical repair if critically ill, or if symptomatic in older infants/children
CYANOTIC CONGENITAL HEART DISEASE

General points to note:
Classify based on if pulmonary blood flow is increased or decreased
DECREASED (lesions include an obstruction to pulmonary blood flow + a pathway for shunting systemic venous blood
from right to left eg. a patent ovale, ASD, VSD): TOF, Tricuspid atresia, TAPVR with obstruction
INCREASED (lesions have no obstruction to pulmonary blood flow): Transposition of the great vessels, Truncus
arteriosus, TAPVR without obstruction
LESIONS WITH DECREASED BLOOD FLOW
TETRALOGY OF FALLOT
The primary defect is an anterior deviation of the infundibular septum (the muscular septum that separates the aortic
and pulmonary outflows). The consequences are the 4 components:
1. Pulmonary stenosis (Right ventricular outflow tract obstruction - RVOTO)
2. Ventricular septal defect (VSD)
3. Aortic root “overriding the VSD”
4. Right ventricular hypertrophy
[Complete obstruction of right ventricular outflow (pulmonary atresia with VSD) is classified as an extreme form of TOF]
INCIDENCE
10% of all CHD, most common cyanotic heart defect diagnosed beyond infancy with peak incidence at 2-4 mo of age
BASIC PATHOPHYS
• The RVOTO is most commonly due to the subpulmonic/infundibular muscle known as the crista
superventricularis being hypertrophic. Contributes to a subvalvar stenosis.
• The aortic arch is right sided in 20% of cases. Aortic root is large and overrides the VSD
• ***Systemic venous return to the right atrium and ventricle is normal. When the right ventricle contracts in the
presence of marked pulmonary stenosis, blood is shunted across the VSD into the aorta. Persistent arterial
desaturation and CYANOSIS result, the DEGREE DEPENDENT UPON THE SEVERITY OF THE
PULMONARY OBSTRUCTION.
• ***NOTE: The degree of RVOTO also determines the 1) Timing of the onset of symptoms, 2) Direction of
shunt, 3) The severity of the cyanosis and 4) The degree of RVH!!
• Mild obstruction -> Patient may not be visibly cyanotic (Acyanotic or “pink” TOT). When severe, symptoms
begin from birth and worsen when ductus begins to close.
• [Often, cyanosis is NOT present at birth; but with increasing hypertrophy of the right ventricular infundibulum (which
worsens the RVOTO) as the patient grows, cyanosis occurs later in the 1st yr of life. In infants with severe degrees of right
ventricular outflow obstruction, neonatal cyanosis is noted immediately. In these infants, pulmonary blood flow may be
partially or nearly totally dependent on flow through the ductus arteriosus. When the ductus begins to close in the 1st few
hours or days of life, severe cyanosis and circulatory collapse may occur]
CLINICAL FEATURES
• History: Hypoxic “tet” spells (Nelson’s: aka. “Paroxysmal hypercyanotic attacks”/hypoxic/blue/tet spells) –
particular problem in first 2 years of life
o During exertional states (crying, exercise) the increasing pulmonary vascular resistance and decrease in
systemic resistance causes an increase in right-to-left shunting
o Clinical features include paroxysms of rapid and deep breathing, irritability and crying, increasing
cyanosis, gasping respirations, decreased intensity of murmur (decreased flow across RVOTO)
o If severe, can lead to decreased level of consciousness, seizures, hemiparesis, death
• Older children with long-standing cyanosis may have dusky blue skin, engorged blood vessels, marked
clubbing of fingers and toes. Dypnoea on exertion.
• ***Characteristically, children may assume a *squatting position for the relief of dyspnea
PHYSICAL EXAM
• Systolic thrill may be felt along the left sternal border in the 3rd and 4th parasternal spaces
• SINGLE, LOUD S2 due to severe pulmonary stenosis (i.e. RVOTO), (**Either the 2nd heart sound is single and
due to the aortic valve closure alone, or the pulmonic component is soft)
• Ejection systolic murmur (LSE) The murmur is caused by turbulence through the right ventricular outflow
tract. (Not from the VSD apparently) Tends to become louder, longer, and harsher as the severity of pulmonary
stenosis increases from mild to moderate; however, it can actually become less prominent with severe obstruction,
especially during a hypercyanotic spell due to shunting of blood away from the right ventricular outflow through
the aortic valve.
INVESTIGATIONS
• ECG: RAD, RVH

• CXR: Boot-shaped heart, decreased pulmonary vasculature, right aortic arch (in 20%)
• ECHO diagnostic
MANAGEMENT
***Management of spells (past paper question):
Toronto: O2, knee-chest position, fluid bolus, morphine sulfate, propranolol
According to Nelson’s:
Depending on the frequency and severity one or more of the following procedures should be instituted in sequence:
(1) Placement of the infant on the abdomen in the knee-chest position while making certain that the infant’s clothing is not
constrictive,
(2) Administration of oxygen (although increasing inspired oxygen will not reverse cyanosis caused by intracardiac shunting), and
(3) Injection of morphine subcutaneously in a dose not in excess of 0.2 mg/kg.
• Because metabolic acidosis develops when arterial PO2 is < 40 mm Hg, rapid correction (within several minutes) with
intravenous administration of sodium bicarbonate is necessary IF the spell is unusually severe and the child shows a lack of
response to the foregoing therapy
• Calming and holding the infant in a knee-chest position may abort progression of an early spell. Premature attempts to obtain
blood samples may cause further agitation and be counterproductive.
***HOW THE KNEE-CHEST POSITION WORKS: It simulates squatting. So in this position BOTH the femoral artery and the femoral vein are
kinked. Each of these has a significance. Kinking of the femoral vein reduces venous return to the right side of the heart, resulting in an overall
decrease in blood volume in the heart. Kinking of the femoral artery results in an increase in systemic vascular resistance in the upper limbs, which
increases the pressure that the left heart has to contract against (the afterload). Combining the decreased blood volume in the heart with the increased
systemic outflow pressure results in a decrease in the right to left shunting which is causing the cyanosis.
Treatment of blue spells

• Anticipate. Prevention - propanolol p.o.
• Knee chest position . Oxygen(not helpfiil)
• IV access - bolus - N saline / Hartmanns
- Propranolol / morphine
- Na HC03. phenylephrine
• If severe/persistent - anaesthetise/ventilate
- urgent Sx.
Definitive: Surgical repair at 4-6 months of age; earlier if marked cyanosis or “tet” spells
COMPLICATIONS
Nowadays rare. Were more common before the age of corrective surgery:
• Cerebral thrombosis
• Bran abscess
• Bacterial endocarditis in the right ventricular infundibulum mainly
LESIONS WITH INCREASED PULMONARY BLOOD FLOW
Transposition of the Great Arteries (TGA) – (Copy/paste from Toronto Notes)
EPIDEMIOLOGY
• 3-5% of all congenital cardiac lesions, most common cyanotic CHD in neonate
PATHOPHYSIOLOGY:
• Parallel pulmonary and systemic circulations (as opposed to in series, which is normal)
• Systemic: Body -‐> RA -‐> RV -‐> aorta -‐> body
• Pulmonary: Lungs -‐> LA -‐> LV -‐> pulmonary artery -‐> lungs
• **Survival is dependent on mixing through PDA and/or ASDs or VSDs
PHYSICAL EXAM:
• Neonates: ductus arteriosus closure causes rapidly progressive severe hypoxemia unresponsive to oxygen
therapy, acidosis, and death
• VSD present: Cyanosis is not prominent; CHF within first weeks of life
• VSD absent: No murmur
INVESTIGATIONS:
• ECG: RAD, RVH, or may be normal

• CXR: Egg-shaped heart with narrow mediastinum (“egg on a string”)
• ECHO diagnostic
MANAGEMENT:
• Symptomatic neonates: Prostaglandin E1 infusion to keep ductus open until balloon atrial
septostomy
• Surgical repair: “ARTERIAL SWITCH” performed in the FIRST TWO WEEKS in those without a
VSD while LV muscle is still strong
TOTAL ANOMALOUS PULMONARY VENOUS RETURN

BASIC PATHOPHYS
• Abnormal development of the pulmonary veins may result in either partial or complete anomalous drainage into
the systemic venous circulation
• TAPVR is associated with total mixing of systemic venous and pulmonary venous blood flow within the heart and
thus produces cyanosis
• In TAPVR, the heart has NO DIRECT pulmonary venous communication into the left atrium
• Instead, the pulmonary veins may drain ABOVE the diaphragm into the right atrium directly, into the coronary
sinus, or into the SVC via a “vertical vein”, or they may drain BELOW the diaphragm and join into a “descending
vein” that enters into the IVC or one of its major tributaries, often via the ductus venosus.
• All forms of TAPVR involve mixing of oxygenated and deoxygenated blood before or at the level of the right
atrium
POINT: A PATENT FORAMEN OVALE OR ASD MUST BE PRESENT FOR ANY BLOOD TO REACH INTO THE
LEFT ATRIUM AND INTO THE SYSTEMIC CIRCULATION OR ELSE THE CONDITION IS FATAL
TREATMENT
• Surgical repair in all cases during infancy

• Emergent repair for severe cyanosis/venous obstruction
TRUNCUS ARTERIOSUS
BASIC PATHOPHYS
• A single arterial trunk (truncus arteriosus) arises from the heart and supplies the systemic, pulmonary and
coronary circulations.
• A VSD is always present with the truncus receiving blood from both the right and left ventricles
• Both ventricles are at systemic pressure and both eject blood into the truncus
• When pulmonary vascular resistance is relatively high immediately after birth, pulmonary blood flow may be
normal. As pulmonary resistance drops in the first month of life, blood flow to the lungs is greatly increased and
heart failure ensues.
CLINICAL FEATURES
• Clinical picture dominated by features of heart failure with mild cyanosis in early life.
• Wide pulse pressure and bounding pulses
• Heart usually enlarged and precordium hyperdynamic
• 2nd heart sound usually loud and single. (Due to the single arterial trunk)
• Ejection systolic murmur +/- thrill usually audible along LSB
• Truncus arteriosus may be associated with DiGeorge syndrome
DIAGNOSIS
• ECG: RVH/LVH/combined VH
• CXR: Cardiomegaly, right sided aortic arch in 50%, prominent shadow produced by truncus, pulmonary
vascularity increased after first few weeks of life
TREATMENT
• Surgical repair within first 6 weeks of life
Paediatrics
Cough, Cold, SOB Jottings
**USE 3RD YEAR PAEDS DOCUMENT FOR THIS TOPIC**
RE: Differential Dx/Thought process for cough/cold/SOB
CLASSIFY CLASSIFY CLASSIFY!!
Think/Answer by system:
1. Resp.
2. Cardio
3. GI
4. Etc.
Re: Respiratory Disorders (List is NOT exhaustive)

Acute vs. Chronic
• ACUTE
o Upper RT (Think of all structures from outside in – Ears, nose, sinuses, pharynx etc.)
§ Infectious
• Viral
o Otitis media
o Common Cold (Rhinopharyngitis)
o Tonsillitis
o LTB
• Bacterial
o OM
o Tonsillitis
o Epiglottitis
o Bacterial tracheitis
• Other
§ Non- Infectious
o Lower RT
§ Infectious
• Viral
o Bronchiolitis
o Bronchopneumonia
• Bacterial
o Pneumonia
o Lung abscess
o Empyema
• Other
§ Non-infectious
• CHRONIC
o UPPER
§ Infectious
§ Noninfectious
o Lower
§ Infectious
• Viral
o HIV – Lymphocytic Interstitial Pneumonia
• Bacterial
o TB
• Protozoan
o PCP
§ Noninfectious
• Bronchiectasis
• Cystic Fibrosis
• Ciliary disorders
Acute
Upper RT Lower RT
Infectious Non-‐ Infectious Infectious Non-‐infectious
f f
Viral Bacterial Other Viral Bacterial Other
Otitis media OM Bronchiolitis Pneumonia
Common Cold
(Rhinopharyngitis) Tonsillitis Bronchopneumonia Lung abscess
Tonsillitis Epiglottitis Empyema
LTB Bacterial tracheitis
irrirant exposures I
NO
T
Remove imam Personal or family hi story Viral URI . LRI Whooping cough
of atopic disease Pneumonia syndromes
Pertussis URI
Sinusitis ( oougn Foreign txxly asplru’loo
may be subaculel
Uoktor.
Eczema
Mergic snu» tis
•awl salute
Coniunct val oottloolcring
Corwder :
Aiergc rhinitis
Asthma
' Cough vanant asthma
Allergic sinusitis Cystic ffcro& ie Recurrent URI
Immotlie dlia Whecprig cough syndromes
Immunodeficiency syndromes Foreign body aspiration
Neurclogcrswallowing Congenial anomaly airway or lung
abnormalities with aspiration Brcre xedaeis
Congeclive hoar failure Tumor ^
Chronic intecton histoplasmosis , cocadmKlomyosIs
AIDS
Tuberculosis
*®'DSl». Acouired irnmunodeltaerey syndrome;

Treel as Indented I
*®' respiratory
R ’ upper
tract Infection (e.g , bronchioirtis, pneumonia);
respiratory tract inlection ( e g . , coktsl.
Consider psychogenic
I
TABLE 56 - 1 . Causes of Cough
Congenital Anomalies
Tracheoesophageal fistula
Laryngeal cleft
Vocal cord paralysis
Mediastinal tumors
Pulmonary malformations
Tracheobronchomalacia
Congenital heart disease
Infections (e.g., upper respiratory infection, sinusitis, pneumonia)
Viral
Adenovirus
Influenza
Parainfluenza
Respiratory syncytial virus
Rhi novirus
Bacterial
Pertussis
Pneumococcal
Staphylococcal
Tuberculosis
Fungal
Coccidioidomycosis
Other
Chlamydial
Mycoplasmosis
Chronic Disease
Cystic fibrosis
Human immunodeficiency virus infection
Immunodeficiency syndrome
Dyskinetic cilia
Allergic Conditions
Allergic rhinitis
.Asthma
Serous otitis media
Foreign Body Aspiration
Gastroesophageal Reflux
Environmental Irritants
Psychogenic Cough
-
Drug Induced Conditions
T A B L E 381-1 Infectious Agents Associated with

the Common Cold
Category Agents
Common viruses that usually Rhinoviruses
cause common colds Parainfluenza viruses
Respiratory syncytial virus
Coronaviruses
Common agents that occasionally Adenoviruses
cause symptoms of the Enteroviruses
common cold Influenza viruses
Parainfluenza viruses
Reoviruses
Mycoplasma pneumoniae
Agents that rarely cause Coccidioides immitis
symptoms of the common cold Histoplasma capsulatum
Bordetella pertussis
Chlamydia psittari
Coxiella burnetii
Adapted from Cherry JD: The common cold . In: Feigin RD, Cherry JD (eds ): Textbook
of Pediatric Infectious Diseases . Philadelphia, WB Saunders , 1998.
UT
Paediatrics Croup, Epiglottitis, Laryngitis, Bacterial Tracheitis Sources: Nelson’s, Toronto Notes Wayne Robinson, Class of 2015

CROUP (LARYNGOTRACHEOBRONCHITIS) BACTERIAL TRACHEITIS EPIGLOTTITIS
Anatomy Subglottic Subglottic tracheitis Supraglottic
Epidemiology Most pts: 3 months – 5 years Rare VERY rare now due to Hib vaccine
nd
Peak in 2 year
M>F Prevaccine: 2-4 y.o
Most common form of acute upper resp. obstruction!! Now: Adults more common
Aetiology Parainfluenza virus – 75% STAPH AUREUS most common In the past: H. influenzae type B was most
common
Others: Also: Non-typable H. influenzae
- Influenza A (assc. with severe LTB) and B Moraxella catarrhalis Since vaccine:
- Adenovirus, RSV, Measles **Often follows a VIRAL infection Strep pyogenes, strep pneumoniae, staph aureus
Clinical Some use “LT” for common form and “LTB” for severe Potentially life-threatening DRAMATIC, POTENTIALLY LETHAL
Features CONDITION:
Common Prodrome: Rhinorrhoea, pharyngitis, mild Similar symptoms to croup BUT more rapid
cough, low-grade fever for 1-3 days before signs of upper deterioration with: - Acute, rapidly progressive
airway obstruction. Some children afebrile • High fever - TOXIC appearance
• Toxic appearance - High fever, sore throat, rapid obstruction
*** Then characteristic *barking cough, *hoarse voice, 4 Ds!!! (Must know):
*inspiratory stridor **Does NOT respond to croup treatments 1. Dysphagia
(because this is a bacterial infection) 2. Drooling
Symptoms characteristically worse at night. Usually 3. Distress – All the usual features
resolves within a week Also: NO drooling, NO dysphagia as in 4. Dysphonia
epiglottitis
Other family members may have a mild respiratory illness. - Stridor is a LATE finding
Older children not seriously ill due to larger airways - Neck hyperextended to maintain airway
Also:
Exam: Hoarseness, coryza, ENT: inflamed pharynx. Slight - Tripod position! – sit upright and lean forward
increased resp. rate. with chin up and mouth open while bracing on
RARE: Obstruction progresses -> Nasal flaring, SS, IS, IC, arms
SC recession, biphasic stridor - Cyanosis
- Coma
Rarely, severe LTB difficult to differentiate from epiglottitis Most patients have concomitant bacteraemia
Investigations CLINICAL DIAGNOSIS – Does NOT require CXR/Neck Diagnosis requires laryngoscopy IN OT OR
XR ICU!!
- Large, cherry red, swollen epiglottis
Neck XR: Typical subglottic narrowing – Steeple sign AVOID EXAMINING THROAT UNTIL AIRWAY
- Used in pts with atypical clinical course SECURE to prevent exacerbation
Classic XR: “Thumb sign”
Blood and epiglottic surface cultures!!
Management - MAIN: Airway mgmt. and treatment of hypoxia FIRST! Intubation may be necessary in 50-60%. O2 IMMEDIATE INTUBATION OR TRACHEOSTOMY
- Humidified O2 usu necessary IN ALL PATIENTS WITH EPIGLOTTITIS!!!!
Mortality rate now almost 0 due to this
- Oral corticosteroids – 1 PO dose dexamethasone – IV antibiotics!! in all patients incl anti Generally intubated for 2-3 days
Reduce oedema through anti-inflammatory action staphylococcal agents
- Nebulized epinephrine – reduces laryngeal edema Empiric antibiotics!!: Ceftriaxone, cefotaxime,
Intubation if unresponsive (RARELY NEEDED) Vancomycin & nafcillin or oxacillin meropenem then culture directed. 7-10 days
Paediatrics Croup, Epiglottitis, Laryngitis, Bacterial Tracheitis Sources: Nelson’s, Toronto Notes Wayne Robinson, Class of 2015

DEFINITIONS
Croup: A GROUP of acute or infectious processes characterized by a barking or brassy cough and may be associated with hoarseness, inspiratory stridor and respiratory
distress. Typically affects larynx, trachea and bronchi. Have spasmodic croup (may be allergic +/- viral) vs. LTB (almost always viral)
Stridor: A harsh, high-pitched respiratory sound, usually on inspiration but may be biphasic, produced by turbulent flow
Some general points:
• With the exceptions of diphtheria, bacterial tracheitis and epiglottitis, most acute infections of the upper airway are caused by viruses.
• Recall: Airway RESISTANCE is inversely proportional to radius of the airway to the 4th power. So two things: Smaller airway in child has exponentially greater
resistance compared to adult and any further slight decrease in lumen due to oedema or other inflammation causes further exponential increase in resistance
• Larynx: 4 major cartilages: Superior to inferior: Epiglottis, arytenoid, thyroid, cricoid
• ***Narrowest portion of upper airway in a child < 10 years old is cricoid cartilage
o Inflammation INFERIOR to the vocal cords: Laryngitis, laryngotracheitis or Laryngotracheobronchitis

o SUPERIOR: Eg. Epiglottitis = Supraglottitis
Not in table:
ACUTE INFECTIOUS LARYNGITIS
• Common illness. Viruses cause most cases. Diphtheria is exception but extremely rare.
• Physical exam unremarkable except for pharyngeal inflammation
• Subglottic area is main site of obstruction
SPASMODIC CROUP
• Most common in 1-3 y.o
• Similar clinically to acute LTB – except history of viral Prodrome usually absent
• Cause is viral in some cases. Allergy important in others
Differential diagnosis of all the above:

• Bacterial tracheitis is the most important differential
• Foreign body aspiration – sudden onset of resp obstruction
• Retropharyngeal abscess – CT helpful
• Peritonsillar abscess – clinical diagnosis
• Occasionally, upper airway obstruction associated with angioedema of the subglottic areas as part of anaphylaxis and generalized allergic reactions.
• Trauma
• Tumours of larynx
Paediatrics Development (Harriet Lane (Pg. 224 – Chapter 9)) September 2014 Wayne Robinson, MBBS Class of 2015
DEVELOPMENTAL MILESTONES
Age Gross Motor Fine-Motor/Problem-Solving Language Social/Adaptive
(Expressive vs. Receptive)
1 - Raises head from prone position - Visually fixes - *Alerts to sound (To test: clap from behind) - Regards face
mth - *Follows to midline
- *Tight grasp
2 - Holds head in midline - *Follows objects past midline - Smiles socially (ie. after being stroked or talked to) - Recognizes parent
- Lifts head and chest off table - *No longer clenches tightly
o
3 - Holds head up steadily - *Follows in circular fashion (180 ) - Coos (produces long vowel sounds in musical - Reaches for familiar people
- Supports on forearms in prone - Responds to visual threat fashion) or objects
position - *Hands open at rest - Anticipates feeding
4 - Rolls over - Reaches with arms in unison - Laughs - Enjoys looking around
- Supports on wrists, shifts weight - Brings hands to midline - Orients to voice
st
6 - Sits unsupported, puts feet in mouth - Unilateral reach - Babbles, ah-goos (1 consonants), razz? - Recognizes that someone
in supine position - Uses raking grasp - Lateral orientation to bell is a stranger
- **Transfers objects
st
9 - Pivots when sitting - Immature pincer grasp - Says “mama, dada” indiscriminately (1 words) - Starts exploring
- Pulls to stand, cruises - Probes with forefinger - Gestures, waves bye-bye environment
- Holds bottle, throws objects - Understands “no” - Plays gesture games
12 - Walks forward alone - Mature pincer grasp - Uses 2 words other than “mama, dada” or proper - Imitates actions
(1 yr) - Can make crayon mark nouns - Comes when called
- Releases object voluntarily - Jargoning (runs several unintelligible words - Cooperates with dressing
together with tone or inflection)
- One-step command with gesture
15 - Walks backward independently - Scribbles in imitation - Uses 4-6 words - Uses spoon and cup
- Creeps up stairs - Builds tower of 2 blocks in imitation - One-step command without gesture
18 - Runs - Scribbles spontaneously - 7-10 words - Copies parent in tasks
- Throws objects from standing w/o - Builds tower of 3 blocks - Mature jargoning (includes intelligible words) (sweeping, dusting)
falling - Turns 2 or 3 pages at a time - Knows **5 body parts - Plays in company of others
2 yrs - Walks up and down stairs without - Imitates stroke with pencil - 50 words - Parallel play
(24m) help - Builds tower of 7 blocks - (2) Two-word sentences
- Kicks ball (online source) - Turns 1 page at a time - Uses pronouns inappropriately
- *Removes shoes, pants, etc. - (2) Two-step commands
3 yrs - *Alternates feet going UP steps - Copies a circle - At least 250 words - Group play
- Pedals tricycle - *Undresses completely, *unbuttons - (3) Three-word sentences - Shares toys, takes turns
- Dresses partially - Uses plurals, knows all pronouns - Plays well with others
- Repeats 2 digits - Knows full name, age,
gndr
4 yrs - *Alternates feet going DOWN steps - Copies a square - **Knows colours - Tells “tall tales”
- Hops, skips - *Dresses self completely, *buttons - Song/poem from memory - Plays cooperatively with a
- *Catches ball - Asks questions group of children
5 yrs - Skips alternating feet - Copies triangle - Writes own first name - Plays competitive games,
- Jumps over low obstacles - **Ties shoes - Asks what a word means follows rules, likes to help in
- Spreads with knife household tasks
Paediatrics Development (Harriet Lane (Pg. 224 – Chapter 9)) September 2014 Wayne Robinson, MBBS Class of 2015
PRIMITIVE REFLEXES
PRIMITIVE REFLEXES
Primitive Reflexes Elicitation Response Timing
Moro reflex (“ embrace" response) of Supine: sudden neck extension; allow head to Extension , adduction, and then abduction of UEs, with Present at birth; disappears by 3-6 mo
fingers, wrists, and elbows fall back about 3 cm semiflexion
Galant reflex (GR ) Prone suspension: stroking paravertebral Produces truncal incurvature with concavity toward Present at birth; disappears by 2-6 mo
area from thoracic to sacral region stimulated side
Asymmetric tonic neck reflex (ATNR , Supine: rotate head laterally about 45-90 Relative extension of limbs on chin side and flexion Present at birth; disappears by 4-9 mo
“ fencer” response) degrees on occiput side
Symmetric tonic neck reflex (STNR , Sitting: head extension/flexion Extension of UEs and flexion of LEs/flexion of UEs and Appears at 5 mo; not present in most
“ cat” reflex ) LE extension normal children; disappears by 8-9 mo
Tonic labyrinthine supine (TLS ) Supine: neck extension alters relation of
( Tonic extension of trunk and LEs, shoulder retraction Present at birth; disappears by 6-9 mo
labyrinths) and adduction, usually with elbow flexion
Tonic labyrinthine prone (TLP) Prone: neck flexion Active flexion of trunk with protraction of shoulders Present at birth; disappears by 6-9 mo
Positive support reflex (PSR ) Vertical suspension; bouncing hallucal areas Neonatal: momentary LE extension followed by flexion Present at birth; disappears by 2-4 mo
on firm surface Mature: extension of LEs and support of body weight Appears by 6 mo
Stepping reflex (SR , walking reflex) Vertical suspension; hallucal stimulation Stepping gait Disappears by 2-3 mo
Crossed extension reflex (CER ) Prone; hallucal stimulation of LE in full Initial flexion, adduction, then extension of contra- Present at birth; disappears by 9 mo
extension lateral limb
Plantar grasp Stimulation of hallucal areas Plantar flexion grasp Present at birth; disappears by 9 mo
Palmar grasp Stimulation of palm Palmar grasp Present at birth; disappears by 9 mo
Lower extremity placing (LEP) Vertical suspension ; rubbing tibia or dorsum Initial flexion, then extension, then placing of LE on Appears at 1 day
of foot against edge of tabletop tabletop
Upper extremity placing (UEP ) Rubbing lateral surface of forearm along Flexion, extension , then placing of hand on tabletop Appears at 3 mo
edge of tabletop from elbow to wrist to
dorsal hand
Downward thrust (DT ) Vertical suspension; thrust LEs downward Full extension of LEs Appears at 3 mo
LE, Lower extremity; UE, upper extremity.
Playlist with primitive reflexes (Did not watch any – Not sure if good): https://www.youtube.com/playlist?list=PLz27Rlp3y6XsmcPui7cR2PfumjJwEZSjZ
Also in the chapter:

• Common age-specific problems: Tells the age for tantrums, nightmares etc
• INTELLECTUAL DISABILITY VS LEARNING DISABILITY
• ANOTHER CLASSIFICATION OF CEREBRAL PALSY
Paediatrics
Down Syndrome Notes
Sources: Dr. Pryce (May Pen) ward rounds, Random PDF
October 2014
DOWN SYNDROME (47, XX, +21 OR 47 XY, +21)
1:600-800 live births - **Most common autosomal chromosomal abnormality

- Rises with advanced maternal age: 1:1500 @ 20 to 1:20 @ 45 (although 80% are born to mothers < 35 years)
- 75% of fetuses with Trisomy 21 abort spontaneously
- *Most cases diagnosed in newborn period
GENETICS
3 types of mutation cause Downs (MUST KNOW!!!):
1. Nondisjunction: Most common (95%) Nondisjunction occurs when the sister chromosomes do not split. Increases greatly after 30
o 80% maternal, 20% paternal

o 1% recurrence risk
2. Translocation (3-4%) Movement of parts of one chromosome to another chromosome.

o Recurrence risk: 100% in a parent with 21:21 translocation (ALL offspring will have Downs)
3. Mosaicism (1-2%) More than one genotype in one individual
o Due to nondisjunction after meiosis
DYSMORPHISMS AND ABNORMALITIES IN DOWN’S SYNDROME

(See the PDF on Down’s syndrome. Some of the info edited into this)
HEAD
1. Brachymicrocephaly: Affects occiput -> Flat, short, broad
2. Microcephaly
3. Hypoplastic midface (lead to obstructive sleep apnoea, rhinosinusitis, nasolacrimal duct obstruction)
4. Low posterior whorl
5. Classically have a 3rd fontanelle (sagital fontanelle)
6. Delayed closure of fontanelles
7. Splayed (wide) sutures
EYES
1. Ocular hypertelorism: Hypertelorism refers to an abnormal increase in distance between any two organs
although some authors use the term synonymously with orbital hypertelorism. Wide inter-pupillary distance.
May be assessed at bedside - see if a third eye can fit between
2. Upslanting of eyes
3. Present medial epicanthic fold
4. Strabismus (60%)
5. Brushfield spots in iris (speckling of iris) - Almost pathognomonic
6. Pupil and iris: Coloboma -> Looks like a keyhole pupil
7. Cornea: Keratoconus
8. Visual disturbances: Congenital cataracts, congenital nystagmus, refractive errors (50%) myopia
9. Dacrocystitis: Important to know: Inflammation of the nasolacrimal sac, frequently caused by nasolacrimal duct
obstruction or infection
10. Blepharitis (eyelid inflammation)
EARS:
1. Microtia (small ears)
2. Low-set
3. Overfolding of the superior helix
4. Posteriorly rotated
Other problems:
5. Sensorineural hearing loss
• But also susceptible to conductive due to mid face abnormalities and the characteristic Eustachian tube
dysfunction
6. Recurrent otitis media
NOSE
1. Flat nasal bridge
MOUTH and TEETH
1. Small mouth with protruding tongue. So not true macroglossia (Relative macroglossia (?%))
2. Hypodontia (Missing teeth) (50%)
3. Delays and alterations in sequence of tooth eruption
FACE
1. Hypoplastic mid-face
SMALL CHIN (Micrognathia - and it recedes (displaced backward), so called:)
1. Microretrognathia
CHEST
1. Funnel or pigeon breast
HANDS
1. Clinodactyly: Due to hypoplasia of the middle phalanx (Short, incurved little fingers)
2. Brachydactyly (Short, broad hands)
3. Simian crease (single) or may have a transverse palmar crease
4. Tri-radius is distally placed

5. Have ulnar loops (normal people have upward or radial loops)
FEET
1. Sandal gap between 1st and 2nd toes and Sandal crease at same place
2. Open field hallux?
SYSTEM BY SYSTEM ABNORMALITIES
CNS/NEUROLOGIC:
1. HYPOTONIA in 100% - at birth, in infancy, and as a toddler (Improves with age) – may delay milestones
2. Low IQ (25-70)/Intellectual disability (Mental retardation)
3. Global developmental delay – Gross motor due to hypotonia, 75% have expressive language disability
4. Seizures in 10% = Generalized myoclonic most common
5. Behaviour problems: Aggression, depression
6. Alzheimer’s > 35 y.o
CVS (40-50%):
1. Structural
• AVSD (49%) (most common) > VSD (22%) > ASD
• Others: PDA, mitral valve prolapse, aberrant subclavian artery
2. Complications: Pulmonary HTN à cor pulmonale
GI:
Think all the atresias of the tract
Top 3:
1. Duodenal atresia (most common), 2. Annular pancreas 3. Imperforate anus
- Also Hirschsprung’s, oesophageal atresia, Meckel’s diverticulum
GU:
1. Kidney:
a. Ureteropelvic junction (UPJ) obstruction with hydronephrosis
2. External Genitalia:
a. Cryptorchidism
b. Hypospadias
c. Small penis and scrotum
d. Infertility
HAEMATOLOGIC:
1. 1% Risk of leukaemias:
o < 2 yrs AML > ALL
o > 2 yrs ALL > AML
o Also can have leukemoid reaction!!! Must test to differentiate leukaemia and leukaemoid reaction
2. Also possible “Transient (Congenital) Leukaemia” – Acute Megakaryoblastic Leukaemia (AMKL)

• Presence of a large number of megakaryoblasts in the peripheral blood
• 100% remission rate in 1st weeks of life
• Occurs only in Downs syndrome
3. Polycythaemias
ENDOCRINE:
1. Thyroid dysfunction (15%)

• Hypothyroidism – often due to lymphocytic thyroiditis
• Most present after 1st decade. But can be congenital
2. Growth disorders
a. Short stature – at or near 3rd % for population
• Decreased growth velocity
• There are specific growth charts for Down syndrome
b. Obesity
3. Sexual maturation:
• Males: No documented male has reproduced?? – Need newer info on this. Also see GU above
• Female: Normal sexual maturation and development. BUT fertility RARE but reported. Pregnancy possible
with a 50% chance of Trisomy 21 occuring??
OTHER MUSCULTOSKELETAL ABNORMALITIES
1. See hands and feet above
2. **Atlantoaxial instability (14%) important!! - Increased distance between C1 & C2 à increased risk of spinal
cord injury. So must avoid contact sports
3. *Spinal cord compression (1-2%)
4. *Dysplastic hips
**Know about prenatal diagnosis of Down’s
PRENATAL SCREENING/DIAGNOSIS
1. Nuchal translucency (look up)

2. Maternal serum tests (look up)
3. Chorionic Villus Sampling (at 9-12 weeks)
4. Amniocentesis (at 16-18 weeks)
Indications:
• Advanced maternal age > 35
• Previous child with Trisomy 21
• Balanced Translocation in parent
• Prenatal U/S findings suggesting Trisomy 21
• Abnormal triple screen suggesting Downs
INVESTIGATIONS
Need to karyotype baby AND both parents

• Chromosomal analysis to confirm diagnosis (can be performed from birth)
• REASON for karyotyping parents: 3 types of mutation cause Downs (MUST KNOW):
• Checking for genetic abnormality in parents to help predict recurrence risk
• ***If parent has 21-21 translocation, ALL the children will have Down’s
Imaging
• ALL patients must have Chest x-ray, ECHO regardless and cardio consult in first few days of life!!!
• GI: Abdominal x-ray, barium swallow/enema (Due to increased risk of atresias)
• Renal: Renal U/S
• Skeletal x-rays: C-spine: Atlanto-dens space > 5 mm suggests atlantoaxial instability (start at age ?)
(Think about this especially if child want to participate in contact sports) Children with Down syndrome are at increased risk of atlantoaxial
instability. However, not until age 3 years will they have adequate vertebral
mineralization and epiphyseal development for accurate radiographic
evaluation of the cervical spine. Plain radiographs do not predict well which
Serum children are at increased risk of developing spine problems. Therefore,
routine radiologic evaluation of the cervical spine in asymptomatic
children no longer is recommended.
• MUST have CBC with blasts!!!! – in neonatal period then annually AAP Updated guidelines - Anggelos
o Risk of leukemias: http://www.aappublications.org/content/early/2011/07/25/aapnews.201107
25-3
§ < 2 yrs is AML
§ > 2 yrs ALL
o Also can have leukemoid reaction!!! Must test to differentiate leukemia and leukemoid reaction (There is
a raise in Serum Leukocyte Alkaline Phosphatase in Leukamoid Reaction. It is depressed in Leukemia)
• TFTs (TSH, T3, T4) at birth, 6 and 12 months, then annually
Other investigations referrals at birth:
• Eye examination for red reflex from birth. Then refer all to ophthalmologist
• Referral to ENT and hearing screen from neonate. Audiology again at 6 monthly until age..?
**NEED TO KNOW HOW TO COUNSEL PARENT!!**

[Fairly common OSCE station]
General overview:
- Greet and ask parent if she knows why we are here
- Ask what she already knows about Down’s
- Tell parent what Down syndrome is AND how it was diagnosed (Clinically, NT, CVS)
- Be empathetic – say you know it must not be easy
- Discuss how the child got it (and that it is not the parents’ fault and was not preventable) – but mention maternal
age
- Point out the features on the child
- Discuss the other possible features/problems that may develop
o *Remember to address developmental delay expectation
- Discuss all investigations required – in newborn period and in future (including karyotyping of parents and child)
- Discuss risk of recurrence (based on the karyotyping)
- Discuss management of the issues
- Ask about parent coping/feelings! Recommend support groups as well
- Ask if any questions/if patient understands
- Ask for a summary
- Plan for follow-up
- Close interview
Point out all the features of Down’s on the child
Discuss what other problems to expect (Remember system by system)

Some pointers:
• Need to tell them patient won't develop at normal times (GDD)
• MUST!! reassure/advise to expect missing teeth/irregular eruption is common!!
• Also have dental enamel abnormalities - may mistake them for caries
• Important to mention risk of respiratory infection
Later on:
• Atlanto axial stability, testing for it and need to avoid all contact sports, trampolines, c-spine instability
• Behavioural problems, ADHD, OCD
Discuss other tests (Just think system by system of all the possible abnormalities and how you would test for them)
• The ones at birth, then repeat in 6 months, then yearly, then at 4 years etc.
• Karyotyping of child and parents from newborn
• Eye examination for red reflex from birth. Then refer all to ophthalmologist
• Referral to ENT and hearing screen from neonate. Audiology again at 6 monthly until age..?
• Regular health maintenance visits
• Physical, occupational and speech therapy as indicated
• CBC to rule out transient myeloproliferative disorder, polycythaemia – birth then annually
• TSH at birth, 6 and 12 months, then annually
• Pneumococcal vaccine (PPV-23) at 2-4 years if chronic cardiac or pulmonary disease
****Need to karyotype baby AND both parents!!! Tell them it is expensive in Jamaica
• Chromosomal analysis to confirm diagnosis (can be performed from birth)
• REASON for karyotyping parents: 3 types of mutation cause Downs (MUST KNOW):
o Checking for genetic abnormality in parents to help predict recurrence risk
• ***If parent has a 21-21 translocation, ALL his/her children will have Down’s
Discuss referrals
• Geneticist, paediatrician, Jamaica Down Syndrome Foundation
• Support groups with other parents of Down
• Books, pamphlets
Discuss follow-up
Discuss management

Paediatrics
Failure to Thrive: Short Pointers
Sources: Lecture, Toronto, Oxford
October 2014
**Use this with the lecture. Go through the lecture to review how to take the history and examination**
DEFINITIONS (Important: No consensus definition exists – but all consider age and sex)
Lecture & Toronto Notes use the same definitions. Dr. Olugbuyi: “Must know them ALL”:
1. Weight for age < 3rd percentile on the NCHS growth chart (National Centre for Health Statistics)
2. Weight for height < 5th percentile on the NCHS growth chart
3. Weight for age or height < 80% of ideal (ie. 50th percentile)
4. Weight: Downward crossing of 2 or more MAJOR percentile curves on NCHS chart (Fall off from previously
established growth curve)
5. 0 growth velocity for 6 months or more
6. Triceps skin fold thickness < 15 mm
*Oxford: Weight is most sensitive indicator in infants and young children, whilst height is better in the older child.
In infancy, birth weight reflects the intrauterine environment. It is a poor guide to the child’s correct ‘genetic
potential’and weight may naturally fall until the correct ‘level’ is attained. In a well, happy child consider
constitutional small stature (characterized by normal growth velocity in a healthy child of small stature parents).
PATHOGENESIS UNCLEAR:
1. Inadequate energy intake

2. Inadequate absorption/utilization/excess loss (Eg. diarrhoea)
3. Excess energy utilization
Or a combination
Oxford: 95% of true FTT is due to not enough food being offered or taken
CLASSIFICATION OF FTT
1. Psychosocial/Nonorganic (NOFTT)
2. Organic (OFTT)
3. Mixed (MOFTT)
*** Remember in family history: Parental height and weight including mid-parental height
*** Don’t forget psychosocial history!!
RE: MID-PARENTAL HEIGHT
Mid-Parental Height (MPH)

• Boys’ target height = (father ht + mother ht + 13)/2
• Girls’ target height = (father ht + mother ht -13)/2
Table 9 . Failure to Thrive Patterns

Healthy Medical Illness Non- Organic
Weight and height proportionally small Weight and height proportionally small Weight falls more than height
Familial ( BA = CA) Syndrome ( FTT)
Constitutional Growth Delay ( BA < CA) Chromosomal Multifactorial
Weight falls more than height ( FTT )
Chronic illness
Lack of intake
Height falls more than weight (short stature)
Endocrine
BA =bone age; CA =chronological age
ORGANIC CAUSES OF FTT (**ALSO SEE SLIDES 27-35 OF LECTURE**)
1. Inadequate consumption:
a. Decreased appetite, e.g. psychological or secondary to chronic illness.
b. Inability to ingest, e.g. GI structural or neurological problems.
2. Malabsorption – Coeliac disease etc.
3. Impaired utilization, e.g. Various syndromes, inborn errors of metabolism, endocrinopathies.
4. Increased energy demand/requirements, e.g. Congenital heart disease, cystic fibrosis, hyperthyroidism,
malignancy, infections/sepsis
5. Excessive food loss, e.g. Severe vomiting (gastro-oesophageal reflux disease (GERD), pyloric stenosis,
dysmotility), diabetes mellitus (urine).
Management: Thorough Hx, exam and treat the underlying cause
NON-ORGANIC FTT
Results from complex factors in parent-child relationship

• Dietary intake, knowledge about feeding
• Feeding environment
• Parent-child interaction, attachment
• Child behaviours, hunger/satiety cues
• Social factors – stress, poverty
Management
Most as outpatient using multidisciplinary approach: primary care physician, dietitian, psychologist, social work, child
protection services
• Medical: Oromotor problems, iron-deficient anemia, gastroesophageal reflux
• Nutritional: educate about age-appropriate foods, calorie boosting, mealtime schedules and environment to reach
goal of 90-110% IBW, correct nutritional deficiencies, and promote catch-up growth/development behavioural:
positive reinforcement, mealtime environment
Paediatrics
Febrile Seizures Notes
Source: Nelson’s (p. 2088 of 2682), Oxford
September 2014
DEFINITION
Febrile seizures: Seizures that occur:
1. Generally between age 6 months to 5 years with a

2. Temperature > 38oC
3. That are NOT due to CNS infection or any metabolic imbalance AND
4. Occur in the absence of a history of prior afebrile seizure
Types: SIMPLE and COMPLEX (different meanings from when used in general seizures classification)
1. Simple febrile seizure: Primary generalized seizure, usually tonic-clonic, that lasts < 15 minutes and does NOT
recur within a 24 hour period
2. Complex febrile seizure: More prolonged (> 15 minutes), +/- focal, and/or recurs within 24 hours
3. Febrile status epilepticus: lasts > 30 minutes (Oxford says 10 minutes)
• *Also “atypical febrile seizure”: Differs in some other way from the above, such as lower temperature than usual, unusual
age of the child, etc.
Important points:
• Simple febrile seizures do NOT confer increased risk of mortality

o ***Also, there are NO LONG-TERM ADVERSE EFFECTS of having simple febrile seizures. Ie.
***Recurrent simple febrile seizures do NOT damage the brain
• BUT Complex febrile seizures have a 2-fold increase in mortality over next 2 years
• Most febrile seizures last a minute or two, but it can be just a few seconds.
[NOTE: When referring to afebrile seizures, simple and complex refer to types of partial seizures. Simple = no LOC.
Complex = LOC]
INCIDENCE
• 2-5% of infants have at least one febrile seizure
Recurrence rate:
• ~30% after 1 episode
• 50% after 2 or more
• 50% if < 1 yr old at 1st febrile seizure
NOTE: Only 2-7% develop epilepsy later in life
AETIOLOGY
• Genetic factors supported by positive family history

o Many inherit it as an autosomal dominant trait
Other important points:
RE: EPILEPSY FOLLOWING FEBRILE SEIZURES
*** A few epilepsy syndromes start with febrile seizures:
1. Generalised Epilepsy with Febrile Seizures Plus (GEFS+)

• AD, presents in childhood, and stops in childhood
• Has multiple febrile and afebrile seizures of all types
2. Severe Myoclonic Epilepsy of Infancy (SMEI) aka Dravet syndrome

• Most severe of the spectrum of “febrile seizures plus”. One of the most severe epilepsy starting in
infancy
• Onset is in 1st yr
• Febrile + afebrile, unilateral clonic seizures recurring every month
• Longer and more frequent than usual febrile convulsions
• Assc with myoclonus, absences and developmental delay follows
• Good point: Many patients thought to have “vaccine encephalopathy” (seizures after a vaccine) actually
found to have Dravet mutations. This has raised doubt about the very existence of vaccine encephalopathy
3. Temporal lobe epilepsy (secondary to mesial temporal sclerosis)
*** MUST KNOW: Risk factors for development of subsequent epilepsy (Blue = ones from the lecture):
• **Neurodevelopmental delay/problems prior to 1st febrile seizure – 33%
• Focal complex febrile seizure – 29%
• **Family history of epilepsy – 18%
• **Complex febrile seizure, any type – 6%
• Recurrent febrile seizures – 4%
• Fever < 1 hr before febrile seizure – 11%
• Simple febrile seizures – 1%
WORKUP
1. Detailed history
2. Thorough general and neuro exam
3. Manage acute febrile seizure and acute illness (first aid, midazolam, diazepam, lorazepam)
4. Determine risk factors for recurrence
5. Determine risk factors for future epilepsy
6. Counsel
*** KNOW: Febrile seizures often occur in the context of:

• Otitis media
• Roseola & HHV-6 infection
• Shigella
• Similar infections
MUST KNOW RE: LUMBAR PUNCTURE
*** POINT: Lumbar puncture recommended in all children < 12 months old after their
first febrile seizure***
REASON: To rule out meningitis. Especially if the patient has received prior antibiotics that would mask the clinical
symptoms of meningitis. In general in this age group, signs of meningitis may not be present either way.
• Up to 18 months of age should be considered for LP!! Because the symptoms of meningitis may still be
subtle in this age group.
• **For children > 18 months an LP is indicated in the presence of clinical signs and symptoms of meningitis (eg.
neck stiffness, Kernig sign, Brudzinski sign)
RE: EEG
• NOT indicated IF the child is presenting with the 1st simple febrile seizure and is otherwise well
• EEGs performed within 2 weeks of a febrile seizure often have nonspecific slowing. If EEG indicated, should
defer 2 weeks. Restricted to cases in which epilepsy is highly suspected
RE: Blood studies
• According to Nelson’s, even CBC and U&Es are not routinely recommended with the 1st simple febrile seizure
RE: Neuroimaging:
• CT or MRI not recommended in child with 1st simple febrile seizure

• May be indicated in complex febrile seizure
MANAGEMENT
In general, antiepileptic therapy is NOT RECOMMENDED in children with one or more simple febrile
seizures.
• Parents should be counseled.
• If the seizure lasts for > 5 minutes, acute treatment with diazepam, lorazepam or midazolam is needed.
Often rectal diazepam prescribed at the time. Or buccal/intranasal midazolam which parents prefer.
• For febrile status epilepticus (> 10 (or 30?) minutes)

o IV benzodiazepenes, phenobarbital, phenytoin or valproate may be needed
• Medications to prevent future febrile seizures rarely ever justified even in future febrile illnesses. Little evidence
to support use
• NOTE WELL: Antipyretics can decrease discomfort BUT DO NOT REDUCE RISK of having a recurrent
seizure!! – Nelson’s: Probably because the seizure usually occurs while the temp is rising or falling
• Iron deficiency shown to be assc with an increased risk of febrile seizures
---
Oxford:
Safety
• Move any danger away from the child and consider their privacy
• Place the child on a protected surface on their side
• It is good practice to note the time
Assistance
• The family should call for help if unfamiliar with febrile seizures
• Then call ambulance
Treatment
• If the seizure lasts >10min, the child should be treated for status epilepticus
• Once the seizure has ended, the child should be assessed for the source of the fever, investigated, and treated appropriately
• Consider admission and observation, especially if this is the first episode
Wayne Robinson, Class of 2015
Paediatrics
Fever
Source: Nelson’s
October 2014
Fever without a
focus
1
I 1
Fever of
Fever without
unknown
localizing signs
origin
Wayne Robinson , MBBS Class of 2015
Paediatrics
Gastroenteritis
Sources: Nelson’ s (Chpt. 332), Toronto
September 2015
DEFINITION
• Inflammation (generally of infectious aetiology) of the stomach and intestines leading to illness characterized by
nausea, vomiting and diarrhea
o May have systemic features incl. abdominal pain and fever
INCIDENCE
• Second most common cause of child deaths worldwide!
• Viral gastroenteritis most commonly affects children aged 6 mo — 5 yr
RISK FACTORS
Environmental contamination (water, etc.)

Young age
Day care attendance
Infected household member
Immunocompromised
Malnutrition ( also increases risk of diarrhoea associated mortality ! Esp. Vitamin A and zinc deficiency)
Measles
Lack of exclusive/predominant breast-feeding ( Breastfeeding reduces incidence )
Antibiotic use (esp. clostridium difficile)
AETIOLOGY
[Remember to CLASSIFY: Infectious (Viral!Bacterial!Parasitic) vs. Noninfectious ]
May be due to infection via the faecal-oral route OR by ingestion of contaminated food or water
• VIRAL
o Most common viral agents: Rotavirus (-50% of all GE?) - primarily fecal-oral; 2 day incubation
period
o Adenovirus, Astrovirus, Norovirus (Norwalk-like)
• BACTERIAL
o E. coli, salmonella, shigella, Campylobacter, clostridium botulinum and perfringens
o Most common in developing countries: E. coli, salmonella, shigella
• Clostridium difficile linked to antibiotic-associated diarrhoea and pseudomembranous colitis

ALTHOUGH most antibiotic-associated diarrhoea in children actually NOT due to this
• Parasitic: Giardia lamblia, entamoeba histolytica
BASIC PATHOGENESIS
Depends on whether organisms have preformed toxins, produce secretory or cytotoxic toxins or are invasive
(See microbiology lecture for more detail if necessary - double check the timing with the microB lecture)
• Preformed toxin: Staph aureus, Bacillus cereus
• Secretory toxin: Cholera, shigella, salmonella, E. coli
• Cytotoxic toxin: Staph, shigella , C. diff, E. coli, C. jejuni
• Invasive
Pathogens can lead to either inflammatory or noninflammatory diarrhoea (**see notes on diarrhoea!!)
• Noninflammatory - through an enterotoxin production by some bacteria, or villus cell destruction by viruses
o ** Rotavirus has a viral enterotoxin. Watery , NO leucocytes.
• Inflammatory - usually caused by bacteria that invade the intestine or produce cytotoxins that enter intestinal
lumen. Bloody diarrhoea with leucocytes
Shigella spp. causes GE via superficial involvement of COLONIC mucosa
**Majority of cases of diarrhoea resolve within the 1st week of the illness
o Acute diarrhoea: Lasts < 2 weeks (Same timeline used for comiting)
o Persistent/Chronic diarrhoea: Episode that begins acutely but lasts >/= 2 weeks (Same timeline used for
vomiting)
CLINICAL FEATURES
• Related to the infecting pathogen and the dose/inoculum

• ALSO dependent on complications (dehydration, electrolyte imbalance)
• Preformed toxin: eg . Staph aureus -> N /V within 1-6 hrs +/- fever/abd cramps/diarrhoea in 8-72 hr
• Enterotoxin producing: eg. C perfringens and B cereus -> Watery diarrhoea + cramps in 8-16 hr
• Invasion: > 16 hrs
• Organisms that produce diarrhoea that contains blood and faecal leucocytes + abdominal pain + tenesmus + fever
o Salmonella, shigella, Campylobacter jejuni, EIEC/EHEC, Yersinia (DDx = IBD, Upper GI Bleed)
o Features suggest bacterial dysentery (bloody and mucous diarrhoea )
—
• Bloody diarrhoea with abdominal cramps after 72-120 hrs IP > Shigella, Shigatoxin producing E. coli Eg. E.
coli 0157:117
HISTORY
• Many manifestations non-specific: [ ALMOST ALL THE G . I SYMPTOMS] :
o Diarrhea, vomiting, nausea, fever, anorexia, headache, myalgias, abdominal cramps, weight loss
• Risk Factors. (Blood and/or mucus in stool is a presentation, not a risk factor)
ill contacts, recent travel, day-care attendance, consumption of unprocessed meats, recent antibiotic use or
hospitalization. Bacterial and parasitic agents more common in older children(2-4yr)
• MUST ask questions re the signs of dehydration ( ***Sunken eyes, decreased urination, altered LOC/lethargy ,
decreased or absent tears, dry mouth, sunken fontanelle (“ mole” ))
• MUST ASK: URTI symptoms!! Common with rotavirus so must ask (Dr. Gabay + Nelson’ s)
• Remember in history to ask if patient got a ROTAVIRUS VACCINE
Other points:
Severe abdominal pain and tenesmus indicate involvement of the large intestine and rectum
Fever suggests an inflammatory process (but may also be due to dehydration or co-infection Eg. UTI)
Viruses rarely produce bloody diarrhoea
Recent infectious contacts: symptoms usually begin 24-48 h after exposure
EXAMINATION
• Febrile
• Dehydrated: SEE THE FULL TABLE ON DEHYDRATION FEATURES FROM DEHYDRATION LECTURE
• URTI signs common with rotavirus
**SEE MICROB LECTURE FOR SLIDE WITH THE EXTRA-INTESTINAL MANIFESTATIONS OF SOME
INFECTIONS**
**EG . (KNOW THEM ):

• Bacteraemia
• Reiter’ s syndrome - (Shigella, Campylobacter ). [Triad of: Arthritis + conjunctivitis/ uveitis + urethritis/cervicitis ]
• Haemolytic-Uraemic syndrome (HUS) - (E. coli 0157:H 7, Shigella)
• Guillain-Barre syndrome - ( Campylobacter)
• Reactive arthropathy - (Yersinia)
• Haemorrhagic colitis
INVESTIGATIONS
**Diagnosis is based on clinical recognition
General:
• CBC - WBCs + differential
• U &Es - Hydration and electrolyte status
Specific investigations not usually necessary in young children
Stool analysis:
• Stool microscopy: Mucus, leukocytes, erythrocytes suggests bacterial invasion or parasitic etiology;
• pH < 6 and presence of reducing substances suggests viral etiology
• C difficile toxin
• Stool culture: * important in children with bloody diarrhoea
+/- Blood culture
TREATMENT
*3 BROAD PRINCIPLES*:
1. Correct dehydration: Oral rehydration therapy
2. Enteral feeding ( orally or NG tube) and diet selection
3. Possible antibiotic therapy (NOT routinely used. Depends on various factors)
1. Assess the degree of dehydration and acidosis and provide rapid resuscitation and rehydration with oral or IV
fluids
a. Replace deficits, ongoing losses and maintenance needs (Know dehydration calculations by heart)
b. Oral rehydration therapy (ORT) preferred for mild-moderate dehydration in acute gastroenteritis. IV if
in shock or unable to tolerate oral
2. Antiemetics (eg. phenothiazines) may reduce vomiting, but increase diarrhoea. NOT recommended . Potentially
serious side-effects. BUT ondansetron is effective and less toxic antiemetic
3. Anti-diarrhoeals NOT recommended either
4. Regular diet of small frequent feeds recommended in mild illness

a. May return to age-appropriate diet once re-hydrated and vomiting stops
5 . Antibiotics or antiparasitic agents usually NOT INDICATED.
• Duration of symptoms is not altered and may increase chronic carrier status, unless there is high risk of
disseminated disease , age < 6mths, enteric fever, cholera or E. coli 0157
• Sometimes indicated in bacterial or parasitic gastroenteritis. Antibiotics only used in select cases
6. Promote regular hand-washing and return to school 24 h after last diarrheal episode to prevent transmission
7. Additional therapies:
a. Zinc supplementation shown to reduce duration and severity of diarrhoea
b. Probiotics nonpathogenic bacteria for therapy of diarrhoea has been successful. Restores beneficial
intestinal flora
** Oxford handbook:
• Antibiotics are not indicated, as the duration of symptoms is not altered and may increase chronic carrier status, unless
there is high risk of disseminated disease, presence of artificial implants (e.g. V-P shunt), severe colitis, severe systemic
illness, age < 6mths, enteric fever, cholera or E. coli 0157. Most organisms are sensitive to ampicillin , co-trimoxazole, or
third generation cephalosporins.
Consider:
• Erythromycin if Campylobacter;
• Oral vancomycin or metronidazole if Clostridium difficile - this is a past paper question, (causes
pseudomembranous colitis).
Prevention
• Rotavirus vaccine is now available
Paediatrics
Child with Red Urine – Short Notes
Sources: Oxford, Nelson’s Essentials (Ch. 163), UHWI TICK SHEET (Last Page)
October 2014
Differential diagnosis of “RED URINE”

Table 163-1 Differential Diagnosis of Red Urine/
Hematuria
A. PATHOLOGIC:
FACTITIOUS HEMATURIA
1. Haematuria (See below) Nonpathologic (urate crystals in infants, ingested foods,
medications, dyes)
“Heme-positive urine” without RBCs is caused by the presence of either hemoglobin or Pathologic (hemoglobinuria from hemolytic anemia, myoglobinuria
from rhabdomyolysis)
myoglobin:
GLOMERULAR
2. Haemoglobinuria (Eg. Haemolytic anaemia, Intravascular?) Immunologic injury (GN— e.g., PSGN, IgA nephropathy, MPGN,
systemic diseases)
Structural disorder (Alport syndrome, thin basement membrane
3. Myoglobinuria (Eg. Rhabdomyolysis - secondary to viral myositis, disease)
Toxin-mediated injury (HUS)
crush injury, severe electrolyte abnormalities (hypernatremia,
hypophosphatemia), hypotension, DIC, toxins) TUBULOINTERSTITIAL/PARENCHYMAL
Inflammation (interstitial nephritis, pyelonephritis)
Vascular (sickle cell trait/disease, Nutcracker syndrome)
B. NON-PATHOLOGIC: Structural (cyst rupture, Wilms tumor, urinary tract obstruction,
renal trauma)
1. Foods – colouring (e.g. beetroot, food coloring) LOWER URINARY TRACT
2. Drugs (e.g. rifampicin, chloroquine, deferoxamine, Ibuprofen, Inflammation (cystitis, hemorrhagic cystitis, urethritis)
Metronidazole, Nitrofurantoin, Phenothiazines, Salicylates, Injury (trauma, kidney stone)
Hypercalciuria
Sulfasalazine) GN , Glomerulonephritis; HUS, hemolytic uremic syndrome; MPGN,
membranoproliferative glomerulonephritis; PSGN , poststreptococcal
glomerulonephritis.
3. Urate crystals (in young infants, usually ‘pink’ nappies)
4. External source (e.g. menstrual blood losses)
Gross
5. Fictitious – consider if no cause found. 1. Large amounts of RBC
2. Injury to glomeruli and interstitial architecture or bladder
wall
RE: HAEMATURIA Micro

1. Small amounts of RBC
2. Rupture of capillaries that supply the glomeruli, tubular
§ May be gross/macroscopic OR microscopic structures and bladder lining.
§ Microscopy: Nelson says > 3-5 RBCs per high-power field/microlitre is abnormal. Oxford says > 10 abnormal.
Benign in ~4% of healthy children.
§ Dipstick: Very sensitive and can be positive at < 5 RBCs per high-power field
§ Microscopic analysis of 10-15 mL of freshly centrifuged urine is essential in confirming the presence of RBCs suggested by a
positive dipstick.
§ Most common cause of gross hematuria is bacterial urinary tract infection
Causes: CLASSIFY: Upper urinary tract (kidney) vs. lower urinary tract vs. systemic
A. UPPER URINARY TRACT (Nephron (glomerulus, convoluted or collecting tubules, and interstitium))
a. Glomerular
• **Note: Hematuria from within the glomerulus is often associated with brown, cola or tea-colored, or burgundy urine,
proteinuria, urinary microscopic findings of RBC casts, and deformed urinary RBCs (particularly acanthocytes)
i. Immunologic: Glomerulonephritis –
- eg. **Post-streptococcal GN – Most common ACUTE GN [Read up this topic!]
- IGA nephropathy – Most common CHRONIC GN
- Membranoproliferative GN
- Systemic diseases eg. SLE – lupus nephritis
ii. Structural disorders (**Alport syndrome (also assc with deafness), ‘Thin basement membrane
disease – (aka. Benign Familial Haematuria)’)
iii. Toxin-mediated – **HUS
b. Tubulointerstitial/Parenchymal
i. Inflammation (Pyelonephritis)
ii. Vascular (Sickle cell trait/disease, renal vein thrombosis, arteritis, Nutcracker syndrome?)
iii. PKD and cyst rupture
iv. Tumour: Wilm’s tumour
v. Trauma
B. LOWER URINARY TRACT (Pelvocalycealsystem, ureter, bladder, or urethra)

• ** Note: may be associated with gross hematuria that is bright red or pink, terminal hematuria (gross hematuria
occurring at the end of the urine stream), blood clots, minimal proteinuria, normal urinary RBC morphology)
a. Inflammation – most common lower UT cause - (UTI incl. schistosomiasis and TB, haemorrhagic
cystitis)
b. Trauma
c. Urolithiasis/Kidney stones
d. Hypercalciuria
C. SYSTEMIC/GENERAL
a. Coagulopathy or platelet deficiency/disorders
b. Drugs - Cyclophosphamide
c. Exercise-induced
----
Figure 163 -1 Suggested algorithm for
evaluation of red urine/hematuria. GN ,
Glomerulonephritis; H& P, history and phys-
ical; Hgb, hemoglobin; RBCs, red blood
cells; 1/ 77, urinary tract infection.
Urinalysis to confirm RBCs

Cause apparent on H& P
History and Physical
Directed evaluation studies
e.g. UTI, trauma, based on suspected cause
kidney stone, GN
Cause not apparent
on H& P
r
'
Negative for blood Urate crystals in infants
Urinalysis
^ Medications, foods, dyes
Positive for blood
Minimal RBCs Hemolytic anemia

Urine microscopy
^ Rhabdomyolysis
RBCs confirmed RBCs confirmed
Isolated microscopic Symptomatic microscopic hematuria

hematuria or gross hematuria
' ' f
1
Repeat urinalysis x 2 at least one week apart Urine culture

Urine calcium to creatinine ratio
Tests to consider if hematuria persistent : Urine protein to creatinine ratio
Urine calcium to creatinine ratio Serum chemistries
Test first degree relatives for hematuria Serum albumin
Hgb electrophoresis to rule out sickle cell C 3 and C 4 complement
Serum chemistries Complete blood count
Renal ultrasound Renal ultrasound
Renal biopsy in selected cases
SPECIFIC TO PSGN - Anggelos
A. Differential Diagnosis (There are many but in OSCE at least say the following)
a. PSGN (Presentation is usually 2 weeks after Strep upper respiratory tract infection. Can
also occur from Strep infection)
b. MSGN
c. IgA Nephropathy
d. UTI (if fever is present unless it is atypical)
B. Investigations
a. Blood
i. CBC
1. Hb for anemia
2. WBC + differentials
3. Platelets
ii. Electrolytes and Urea
1. Done to identify electrolyte abnormalities and kidney function
iii. Creatinine
1. Done to identify proper kidney function
iv. Liver Function Tests
v. Inflammatory marker
1. ESR
vi. Anti-streptolysin O tire (ASOT)
1. Will be elevated
2. Anti-DNase B (Best/Most Specific Test for cutaneous infection)
3. May also do anti-hyaluronidase, anti-streptokinase to identify
Streptococcal infection
vii. C3 and C4
1. C3 will be reduced
b. Urine
i. Urine Dipstick
1. Done to identify
a. Blood (Hematuria)
b. Protein (Possible nephrotic)
c. Glucose
d. Leucocytes (Infection)
e. Nitrites (Infection)
ii. Urine Microscopy (of 5 – 15 mL of freshly centrifuged urine)
1. Done it identify RBC (intact or hemolyzed) and RBC casts
c. Throat swab
i. Taken for culture
d. Imaging
i. Renal Ultrasound
1. Done to rule out stones or other possible renal structural abnormalities
ii. Abdominal X-Ray
1. Done to rule out stones or other possible urinary tract abnormalities
C. Treatment
***TREATMENT IS SYMPTOMATIC/SUPPORTIVE***
a. Infection
i. 10 day course of oral penicillin (will not alter the natural history of GN)
1. Given to prevent spread to others
2. May prevent GN if given within 36 hrs
b. Edema
i. Fluid restriction to 1L/day and no added salt
ii. Furosemide to increase renal output
c. Hypertension
i. Furosemide to aid in renal output
ii. For persistent hypertension, use calcium-channel blockers such as amlodipine or
nifedipine
d. Renal Failure – Dialysis
e. Follow-up
i. Monitor blood pressure
ii. Urine dipstick (Hematuria may persist for 1 – 2 years)
iii. Renal function tests
D. Complications
a. Proteinuria/Nephrotic (rare)
b. Renal failure (rare)
c. Hypertension
i. If present, do a Chest X Ray as there can be possible Pulmonary edema in the
absence of CHF.
d. Encephalopathy
i. Secondary to hypertension
e. Congestive Heart Failure
i. Secondary to hypertension
E. Prognosis
a. Usually good, 95 resolves
b. Proteinuria and hypertension may resolve in 4 – 6 weeks
c. May have persistent hematuria for 1 – 2 years
Paediatrics
Immunization Notes – Anggelos (Updated Jan 2019)
IMMUNITY AND IMMUNIZATION OVERVIEW

Immunity refers to the defenses that are in place to prevent diseases
Types of immunity
ACTIVE PASSIVE
Always involves the ANTIGEN Always involves the ANTIBODIES
Natural Artificial Natural Artificial
Obtained through Obtained through Obtained from Obtained from
Infection Vaccine using antigen Mother (Breast milk, Administered antibodies
placenta)
Immunization refers to the method used in preventing diseases by use of vaccination

Vaccination is the main method of preventing many communicable diseases effectively
Vaccination is an artificial form of immunity and come in 2 forms:

1. Active immunization
a. Process by which the body’s immune system is stimulated to produce memory for a disease after
inoculation with parts or whole of the organism
b. Tetanus toxoid, Diphtheria toxin
c. Active immunization may be
i. Live (Live attenuated)
1. Organism is not at as strong as if it were in its full form
2. Usually provide lifelong immunity
ii. Dead
1. Require BOOSTERS
2. Passive immunization
a. Process by which the body’s immune system is strengthened by direct administration of preformed
antibodies or specific immune globulins
b. Given for
i. Immunocompromised patients
ii. Post-exposure prophylaxis (because the body may not have time to build up its own immunity via
antibodies but not strong enough to fight antigen even if given is weak amounts)
Each vaccine has information that must be read before administration:
- Names of vaccines
- Dose and Route of administration
- Storage/cold chain
- Side effects
- Contraindications
- Mandatory vaccines (Ministry of Health)
- Other vaccines
IMMUNIZATION SCHEDULE OF JAMAICA – Updated 2015
Immunization Goals - Jamaica

Vaccination of all children 0-11 months with BCG, DPT/HBV/HIB, MMR, Polio
Full immunization of all children less than 7 years before school entry (Public Health Law)
- Immunization of antenatal clients to prevent neonatal tetanus
IMMUNIZATION PRINCIPLES
1. VACCINES ARE GIVEN CLOSE TO EACH OTHER BECAUSE THEY ALL CAN CAUSE
FEBRILE STATES, HAVING 1 or CLOSE FEBRILE EPISODES DECREASES THE LENGTH
OF DISCOMFORT
2. ALL LIVE ATTENUATED VACCINES ARE CONTRA IN PREGNANCY AND MODERAT TO

SEVERE IMMUNOSUPPRESENT AND HOSPITALIZED PATIENTS
3. VACCINES ARE NOT CONTRAINDICATED FOR MILD ILLNESSES SUCH AS A COLD.

MANY NURSES WILL BE HASITANT TO GIVE VACCINES BECAUSE OF THIS…JUST
REMIND THEM OF THIS RULE
4. VACCINES ARE GIVEN AT CHRONOLOGICAL AGES NOT GESTAIONAL AGES
5. ROUTES – IF THE CHILD IS LESS THAN 2 OR BELOW NORMAL WEIGHT AND THE
VACCINE ROUTE IS INTRAMUSCULAR, CHOOSE THE THIGH (ANTEROLATERAL). IF
THE CHILD IS > 2, THE ARM (DELTOID) CAN BE USED.
6. COLD CHAIN
• To ensure the efficacy of the vaccines, certain guidelines that govern storage, handling and
transport of the vaccines must be adhered to
• Diluent and vaccine must be collected from the airport as soon as they arrive
- Transported at the correct temperature from one storage site to another
- Stored at the correct temperature at the central, parish and health centre levels
- Transported at the correct temperature to outreach sites
- Kept cold during immunization sessions
Mandatory Vaccines of Jamaica Other Vaccines
HPV-YMCHR
BCG (Bacille Calmette-Guerin) Varicella
Polio Meningococcus
Pentavalent (DPT/Hep B/Hib) HPV
MMR Yellow Fever
Cholera
Hep A
Pneumococcal
Rotavirus
From Anggelos: This is a LARGE topic and can be overwhelming, however I tried to focus on areas that you
must know by highlighting them as best as possible.
MANDATORY VACCINES OF JAMAICA
A. BCG (Bacille Calmette-Guerin vaccine)
Purpose: Prevent Tuberculosis
Content: Antigen (Mycobacterium bovis)
Type: Live attenuated
Dose: 0.1 mL INTRADERMALLY in right deltoid area
• Always use the 1mL syringe aka Insulin syringe, using the 3mL syringe will give incorrect
dosage
• This location is standard in Jamaica but maybe different in other countries
• Localized raise/swelling on the right-hand aids in easy identification in later years especially if
they have lost their health card and are below 7 years
• Raise may not be present due to administration technique
Storage Temp- 2.8 deg C
Age: 0 to 6 weeks routinely
• Sensitizes the vaccinated individual for 5-50 years
• Can be given vaccine until 7 years. The reason for the vaccine is the high risk of Tuberculosis
Meningitis below age 7. It is not effective against pulmonary tuberculosis. Child should have
annual tests for TB if over 7 years.
Mechanism: Stimulates both B and T-cell immune responses
• BCG reduces the risk of tuberculous meningitis and disseminated TB in paediatric populations
by 50-100% when given in the first month of life
Side Effects:
THERE IS NO FEVER IN BCG, AGAIN THERE IS NO FEVER WITH BCG
• Swelling at the injection site
• Local ulceration/abscesses and regional lymph node enlargement
o The local ulceration may look like PUSTULE. Remind parents NOT to rupture it.
• BCG adenitis (inflammation of local lymph nodes)
o This is a rare complication and requires surgical drainage if seen
• Lupus vulgaris- are painful cutaneous tuberculosis skin lesions with nodular appearance
o Most often on the face around nose, eyelids, lips, cheeks and ears
Contraindications:
• Pregnant women
o BCG can cross the placenta
• Immunocompromised individuals
o Can cause disseminated (as seen in a case with an HIV patient at May Pen Hospital)
or fatal infections
B. POLIO (Poliomyelitis)
Overview
• Paralytic disease that is caused by the poliovirus that attacks the anterior horn cells of the
spinal cord = LMN lesion
• Most are subclinical (90 – 95%)
• There are 3 serotypes = 1, 2 and 3
o The 3rd serotype gives the issues
• Presentation
o The initial symptoms are fever, myalgia, sore throat and headache for 2-6 days
- Mild cases resolve completely
- In only 1-2% of these children does high fever, severe myalgia and anxiety progression
to loss of reflexes and subsequent flaccid paralysis
o Pattern of presentation
▪ Asymmetric Flaccid paralysis + loss of reflexes
▪ Proximal limb > distal limb muscles
▪ Sensation remains intact
- However hyperaesthesia (excess sensitivity) of the skin overlying paralyzed
muscles is common and pathognomonic
▪ Bulbar involvement affects swallowing, speech and cardio respiratory function
- Bladder distention and marked constipation usually accompanies lower limb
paralysis
▪ Note: Aseptic meningitis due to poliovirus is indistinguishable from that due to
other viruses
- Paralytic disease in the USA is usually due to non-polio enteroviruses
▪ Polio DDX: Guillain-Barre syndrome, polyneuritis, tick paralysis
• Transmission
o Poliovirus only infects humans. It is very contagious and spreads through person-to-person
contact and aerosols.
o The virus lives in an infected person’s throat and intestines. It enters the body through
the mouth and spreads through contact with the feces (poop) of an infected person
and, though less common, through droplets from a sneeze or cough. You can get
infected with poliovirus if you have feces on your hands and you touch your mouth.
Also, you can get infected if you put in your mouth objects like toys that are
contaminated with feces (poop).
o An infected person may spread the virus to others immediately before and about 1 to 2 weeks
after symptoms appear. The virus can live in an infected person’s feces for many weeks. It
can contaminate food and water in unsanitary conditions.
o People who don’t have symptoms can still pass the virus to others and make them sick.
• Treatment
o Supportive
▪ Bed rest with fever and pain control
o Medical
▪ IM for acute phase
• Prevention
o Vaccination
• Other Notes
o In the USA there has been no wild-type polio for more than 20 years
- 1 in 2.4 million risk of vaccine associated polio with the use of OPV
- BUT the OPV is more effective in developing herd immunity than the IPV
Purpose: Prevent Polio disease
Content: Antigen
Type:
• OPV – Live attenuated
o Dose: 2 drops
• IPV – Killed virus
o Dose: O.5 mL IM
Age: 6 weeks, 3 months, 6 months with Booster at 18 months and 4 – 6 years routinely
Side Effects
• Fever, Redness, Swelling at injection site
• Loose stools
• Vaccine associate polio
o Rarely and with OPV only
• Allergic reactions
Contraindications
• Not given to the immunocompromised (HIV, steroid tx, chemo tx) IPV can be given however
• Live with immunocompromised patient
• Previous anaphylaxis to vaccine or components
C. PENTAVALENT
In the US the HEXAVALENT is given. There are some private practices in Jamaica do this as well. It
is the pentavalent + IPV. It is much more expensive but less injections. – Dr Pryce, May Pen Hospital
Overview:
• The pentavalent vaccine in is made of 5 vaccines hence the word PENTA. They are:
• DPT which contains 3 vaccines:
o Diphtheria
o Pertussis
o Tetanus
• HiB
• Hepatitis B
Route: IM
Age:
• ALL are given 6 weeks, 3 months, 6 months
• DPT Boosters 18 months and 4 – 6 years (like Polio)
THE SIDE EFFECTS IN THE PENTAVALENT IS RELATED MAINLY TO THE PERTUSIS

COMPONENT
REMEMBER THAT SOME OF THE VACCINES CAN BE GIVEN BY THEMSELVES AND

MAY HAVE INDIVIDUAL REGIMES ATTACHED WHEN NOT PART OF THE
PENTAVALENT
DPT
• DPT vaccine used in Jamaica
• DT does not protect against Pertussis
• DTaP vaccine is used in USA and other developed countries
▪ Uses the acellular comment for Pertussis
▪ Vaccine given for children younger than 7
▪ Precautions to DTaP vaccination include:
High fever (>40.5 degC)
Persistent inconsolable crying
Seizures within 3 days of a previous dose
Guillain Barre Syndrome less than 6 weeks after a previous tetanus like vaccine
• Tdap is a BOOSTER given at 11 – 12 years and upward. The amount of diphtheria is smaller
hence the small d.
• Diphtheria
• Overview
▪ Diphtheria is an acute infection of the upper respiratory tract or skin caused
by gram-positive bacilli toxin-producing Corynebacterium diphtheriae
▪ Pathophysiology
• The toxin is absorbed into the mucus membranes and causes destruction
of epithelium and a superficial inflammatory response
• The necrotic epithelium becomes embedded in exuding fibrin and red and
white blood cells → forms a greyish pseudomembrane over the tonsils,
pharynx or larynx
• Any attempt to remove the membrane exposes and tears the
capillaries resulting in bleeding
▪ Complications
• Toxic injury to heart muscle, liver, kidneys and adrenals
• Neuritis resulting in paralysis of the soft palate, eye muscles or
extremities
• Death due to respiratory obstruction or toxaemia and circulatory
collapse
▪ NB: Diphtheria can affect the immunized, partially immunized and un-
immunized persons
• Waning immunity in adolescents and adults hence it can be passed on to
others
• Purpose: Prevent diphtheria
• Content/Type: Diphtheria Toxoid
Dose:
• Storage Temp
• Mechanism: Kills susceptible cells by irreversible inhibition of protein synthesis
• Side Effects:
• Contraindications
• Pertussis
• Overview
• Bordetella pertussis is a highly communicable pathogen that causes whooping
cough.
• Incubation period of about 2 weeks
• Has 3 stages
▪ Catarrhal stage develops with mild coughing and sneezing
▪ Paroxysmal stage the cough develops its explosive character and the
characteristic whoop on inhalation
• This leads to rapid exhaustion and may be associated with vomiting,
cyanosis and convulsions
▪ Convalescent stage has a chronic cough that may last for weeks
• In Jamaica the WHOLE CELL Pertussis (wP) is given
▪ All the organism is present
▪ Side effects are more common
• In the US the ACELLULAR Pertussis (aP) is given. Also done privately.
▪ Contains components of the membrane
▪ Less side effects (reduces encephalitis)
• Complications
▪ Major complications occur mostly in infants
▪ Pneumonia, Seizures, Encephalopathy and Death
Increasing cases in adolescents and adults with mild disease with transmission to
new borns and infants with serious illness
• Purpose: Prevent Pertussis

• Content:
• Type:
• Mechanism:
• Side Effects: Format given by Dr. Pryce, May Pen Hospital
▪ HYPERPYREXIA within 48 hrs/ 2 days
• Fever >40.5 deg C
• FEVER NEVER PASTS 72 hrs/ 3 days
▪ HIGH PITCHED CRY within 48 hrs/ 2 days
• Prolonged hugh pitched crying that is inconsolable
• Crying for 3 hrs at a time
▪ SEIZURESs within 72 hrs/ 3 days
▪ HYPOTONIC HYPO-RESPONSIVE STATE within 72hrs/ 3 days
• Collapse/shock like state
▪ ENCAPHALOPATHY within 7 days
▪ Anaphylaxis
• Contraindications:
▪ Absolute
• Encephalopathy within 7 days
▪ Relative
• Can get the acellular alternative if all other side effects (except the
encephalopathy) have occurred
• Immediate anaphylactic reaction
▪ Individuals with progressive neurologic disorders until their neurologic status is
clarified and stabilized
• infantile spasms, uncontrolled epilepsy, progressive encephalopathy
▪ Moderate to severe acute illness w/ or w/o fever
• Other Notes:
▪ Note: Prophylactic administration of erythromycin for 5 days may also benefit
unimmunized infants or heavily exposed adults
• Tetanus
• Overview
• Purpose:
• Content/Type: Tetanus Toxoid
Dose:
• Storage Temp
• Mechanism:
• Side Effects:
• Hepatitis B
• Overview
• Purpose:
• Content/Type: Hepatitis B vaccine is a recombinant DNA vaccine
• Dose:
▪ Given with pentavalent timing
▪ Given by itself
• Minimum dosing interval between the 1st dose and 2nd dose is 4 weeks
• Minimum dosing interval between the 2nd and 3rd dose is 8 weeks
• There should be at least 16 weeks between the first and third doses
• Age
▪ Birth, then 1 month then at 16 weeks in the US
• Side Effects:
▪ Fever, redness and swelling at the injection site
▪ Previous anaphylaxis to this vaccine or any of its components
▪ Including a serious allergy to yeast
▪ Pregnancy is NOT a contraindication to vaccination
• Haemophilus Influenza B
• Overview
▪ 40% of cases occur in children younger than 6 months who are too young to have
completed a primary immunization series
• Eradicated in developed countries due to Hib vaccine implementation
• In KSA (Kingdome of Saudi Arabia) high morbidity associated with 26%
ampicillin resistance
▪ Hib vaccine is implemented in Jamaica
▪ Hib may cause the following:
• 1- Meningitis- infants usually present with fever, irritability, lethargy,
poor feeding with or without vomiting and a high-pitched cry
• 2- Bacteraemia/Septicaemia
• 3- Epiglottitis (supraglottic croup)- evidence of dysphagia, characterized
by a refusal to eat or swallow saliva and drooling.
o This finding along with a high fever in a toxic child should
strongly suggest the diagnosis and lead to prompt intubation
o The above signs are significant even without the classic cherry-
red epiglottis on direct examination
o Note: Stridor is a late sign
• 4- Septic arthritis- Hib is a common cause of septic arthritis in
unimmunized children younger than 4 years
o Child is febrile and refuses to move the involved joint and limb
o Examination reveals swelling, warmth, redness, tenderness on
palpation and severe pain when movement is attempted
• 5- Periorbital and facial cellulitis
• 6- Pneumonia
• 7- Sinusitis
• 8- Otitis media
▪ Four separate carbohydrate protein conjugate Hib vaccines are currently available
• HibTITER
• PedvaxHIB
• ActHIB
• ProHIBIT
▪ Treatment- All patients with bacteremic Hib disease require hospitalization for
treatment
• Use third generation cephalosporins to treat (cefotaxime or ceftriaxone)
• Meropenem is an alternative
• Note: In addition to antibiotics children with Hib meningitis should be
given dexamethasone immediately after diagnosis
o The steroid continued for 4 days may reduce the incidence of
hearing loss in children with Hib meningitis
• Purpose:
• Content/Type: Killed Vaccine
• Dose:
▪ 0.6 mg/kg/day in 4 divided doses for 4 days
▪ Given at 2, 4, 6 months and a booster at 15 months
▪ In Jamaica, Hib is given at 2,4, 6 months because the government does not
purchase the one for 15months. But 2,4,6 months is the recommendation. If the
patient has the money UWHI offers the 15months.
• Storage Temp: 2-8 deg C

• Side Effects:
▪ Local pain, redness and swelling in 25% of cases
▪ Systemic reactions such as fever and irritability are rare
▪ Should not be given to anyone who has had a severe allergic reaction to a prior
vaccine dose
▪ Should NOT be given to infants before 6 weeks of age
D. MMR
Overview
• Vaccination prevents measles in susceptible exposed individuals if given within 72 hrs
Purpose: Prevent Measles Mumps and Rubella
Content:
Type: Life Attenuated
Dose:
• 0.5 ML IT IS THE ONLY SUBCUTANEOUS VACCINE
• Given in the anterolateral thigh because the deltoid muscle is not well developed in children
Storage Temp
Age: 12 months and 18 months
Mechanism:
Side Effects:
• OCCURES IN 6 – 12 days after immunization
• Fever, redness and swelling at the site
o Fever can last up to 14 days after immunization
• Transient rash
• Encephalitis (rarely)
• Transient thrombocytopenia
Contraindications:
• Pregnancy
o Those currently or intending to become pregnant in the next 28 days
• Previous anaphylaxis to this vaccine, neomycin, which is one of its components or gelatin
• Severe immunodeficiency
o Children receiving high dose corticosteroid therapy (>2mg.kg/day or 20mg/daytotal, for
longer than 14 days
o Give MMTR to HIV/AIDS patients because that have a high risk of mortality if they
contract wild type measles
NB: EGG ALLERGY IS NOT LONGER A PRECAUTION

Measles and mumps vaccine viruses are grown in chick-embryo
cell culture. Even after extensive purifications final vaccine may
contain traces of avian proteins.
MMR AND AUTISM

• Flaws to that article
• Sample size was too small and the way the analysed it was flawed
• Paper was withdrawn but it was already printed
• Because there is still no cause of autism, people who are anti vaccine will still link MMR
to it
• Time that you give MMR is usually the time people notices signs of autism (illness
showing difficulty of communication/ social interaction)
• If parents who already has a child with autism and worried about their second child would
ask to delay MMR. That can be done until the parent notices she has normal communication
skills and then MMR can be given
OTHER VACCINES OF JAMAICA
A. HPV (Human Papilloma Virus)
Overview
• Approximately 70% of cervical cancers are caused by the high cancer risk types 16 and 18
• Over 90% of genital warts are caused by the low cancer risk types 6 and 11
• If the virus is obtained, the body normally clears it from the body in the youngers years. Older
person has an issue clearing the virus. If the virus is obtained, it is usually one serotype.
• Manifestations of HPV
o Cervical cancer precursors
o Vaginal and Vulval cancer precursors
o Anogenital warts
o HPV-related cervical cancer
• As of 2018 the HPV vaccine is being offered freely to girls in grade 7. It is not known how
long this initiative will continue by the government as there has not been good reception.
• Vaccines available
o The Divalent vaccine (Cervarix) covers HPV 16 and 18 only
▪ Given to females only (9 – 25)
o The Quadri-valent vaccine (Gardasil) covers HPV types 6,11,16, and 18
▪
Females
• Ages 9 – 45 (Max age changed in 2018 from 26 to 45)
• Given at this age as this is most likely before their 1st sexual contact
▪ Males
• Ages 9 - 45 (Max age changed in 2018 from 26 to 45)
▪ It being available for males allows for heard immunity
o Nano-valent vaccine (Gardasil 9) covers HPV types 6,11,16, 18, 31, 33, 45, 52 and 58
o A young age is chosen so as to get patients before sexual activity starts
Purpose: Prevent HPV manifestations

Dose:
• Divalent vaccine (Cervarix)
o Vaccine is given in 3 doses
o 0 months, 1 months, 6 months
o i.e. 1st dose given, 2nd dose is 1 months after, 3rd dose is 6 months after second dose
• Quadri-valent vaccine (Gardasil)
o Vaccine is given in 3 doses, 0.5mL each via IM route
o 0 months, 2 months, 6 months
o i.e. 1st dose given, second dose is 2 months after, 3rd dose is 6 months after second dose
Storage Temp: 2-8 deg C
Mechanism: Contains immuno-stimulant component that aid in protecting against other strains
Side Effects:
• Fever, redness and swelling at the injection site

• Myalgia, dizziness and headaches
• Post marketing reports of syncope have been reported after vaccination, so vaccine recipients
should be observed for 15 mins after vaccination
Contraindications:
• Previous anaphylaxis to the vaccine
• History of anaphylaxis to yeast
• Pregnancy
Other notes
• Vaccine can be given to those with the virus
B. Pneumococcus
Overview:
• Prevnar came out with the first conjugate pneumococcal vaccine
• Conjugate vaccines can be used in younger persons as they can respond to it better
• Polysaccharide pneumococcal vaccine is only given 2yrs and up (up until age 3)
o Where they are protected and that’s their highest risk period of pneumococcal
o immune response to a polysaccharide vaccine is significantly less
• Currently there are 10 and 13 valent on the market. 13 valent is the better
o Years ago Prevnar came out with a 7 valent pneumococcal vaccine
o Synflorix came out with 10 valent
o Prevnar then followed up with a 13 valent
▪ Protection against step. Pneumoniae but it goes with a higher cost.
Other Notes
• Risk factors means we must give this to them at all cost. This include:
o Sickle cell pts,
o Chronic renal pts,
o Pt with a non-functional spleen,
o Diabetic,
o Immunocompromised,
o Congenital heart disease pts
• Government will supply Prevnar free at cost for those pts but for everybody else would
have to pay
Read Sickle Cell Notes for more info

C. Varicella
Overview:
• Varicella- consists of mild systemic symptoms followed by groups of red macules that itch
o The incubation period for is 14 to 16 days after exposure to a varicella or a herpes
zoster rash, with a range of 10 to 21 days. A mild prodrome of fever and malaise may
occur 1 to 2 days before rash onset
o Macules becomes small vesicles with surrounding erythema
o Form pustules become crusted and then scab over
o The rash appears mainly on the trunk and face
o Lesions can occur in the scalp, nose, mouth, conjunctiva, and vagina
o Once crusting/scabbing begins the patient is no longer contagious. They are also
contagious a few days before the spots
o If varicella occurs in the first few months of life, it is often mild due to persisting
maternal antibody
• Varicella is responsible for:
o Chicken pox
o Bacterial super infection
o Thrombocytopenia
o Arthritis
o Hepatitis
o Cerebellar ataxia
o Encephalopathy
o Meningitis
o Glomerulonephritis
• Complications
o Secondary bacterial infection with staphylococci or group A streptococci is most common
o Protracted vomiting or a change in sensorium suggests Reye syndrome or encephalitis
▪ Reye syndrome would be seen in patients who were using salicylates
▪ Encephalitis usually involves cerebellitis with ataxia
o Varicella pneumonia usually affects immunocompromised children
▪ Especially those with leukemia or lymphoma
▪ Those receiving high doses of corticosteroids or chemotherapeutics
o Cough, dyspnoea, tachypnoea, rales and cyanosis occur several days after onset of rash
o Death to Neonates born to mothers who develop varicella from 5 days before to 2 days
after delivery are at high risk for severe or fatal disease
o These neonates should be given varicella-zoster immune globulin
o Varicella during the first 20 weeks of pregnancy may cause congenital infections
o Associated with cicatricial skin lesions, limb anomalies and cortical atrophy
o Note: Unusual complications of varicella include optic neuritis, myocarditis, transverse
myelitis, Orchitis, arthritis
DDx = Why does your skin have so many CHIPS
Coxsackievirus Infection – Seen on hand and foot areas, fewer lesions, lack of
crusting
Herpes zoster- eruption usually involves a single dermatome usually truncal or

cranial. The rash does not cross the midline
Impetigo- Honey crusting in nature, fewer lesions, smaller area, no classic

vesicles, positive Gram stain, peripheral lesions
Papular urticaria- insect bite history that show where clothing does not cover,
non-vesicular rash
Scabies- burrows in web spacies, no typical vesicles, failure to resolve
Purpose:
Prevent Chicken Pox
Chicken pox is not an awful disease but an expensive disease as it causes one to miss out on
school (for paediatric population) and work (guardian) for 2 weeks)
Content:
Type: Live Attenuated
Dose:
• 0.5 mL subcutaneous in the deltoid with Booster given 1 month or great after 1st dose
• Also for adolescents and adults without evidence of immunity
• Can be given simultaneously with MMR at 1year BUT at separate sites
• Vaccine is 85-90% effective in preventing varicella. Seroconversion in >95%
• Immunity for 11-20 years
• There is also a combination form of the drug that is not always in the island of Jamaica.
Storage Temp; 2-8 deg C

Age: >/= 1 year
Mechanism:
Side Effects:
• Minor injection site reactions
o Mild reactions in 5-35%- pain, redness, swelling, rash, low grade fever
o Mild varicella-like syndrome in 1-5%
• Severe allergic reaction after a previous vaccine dose or to a vaccine component
• May develop a varicelliform rash outside of the injection site
o Herpes zoster infection has occurred in recipients of VAR in immunocompetent and
immunocompromised persons within ~ 1 month to 2 years after immunization
Contraindications:
• Children receiving immunosuppressive therapy including high-dose steroids
• Children with cellular immunodeficiencies
• VAR and MMRV are live-virus vaccines therefore they are contraindicated in
o EX: leukemia, lymphoma, congenital T-cell abnormalities
o EXCEPTION: Can be given to HIV-infected children who are not severely
immunosuppressed
• Should NOT be given to pregnant women
o BUT can be given to children living with pregnant women in their household
D. Yellow Fever
Overview
• Flavivirus, causing febrile illness with hepatitis and haemorrhagic fever
• Found in Tropical areas of South America and Africa
• Transmission
o Yellow fever virus is transmitted to people primarily through the bite of infected Aedes or
Haemagogus species mosquitoes.
• Treatment
o There is no medicine to treat or cure infection from yellow fever.
o Rest, drink fluids, and use pain relievers and medication to reduce fever and relieve
aching.
o Avoid certain medications, such as aspirin or other nonsteroidal anti-inflammatory drugs,
for example ibuprofen (Advil, Motrin), or naproxen (Aleve), which may increase the risk
of bleeding.
o People with severe symptoms of yellow fever infection should be hospitalized for close
observation and supportive care.
Purpose: Protect against Yellow Fever

Content: 17D yellow fever attenuated virus strain grown in chick embryos
Type: Live Attenuated
Dose:
• Given as a single subcutaneous injection of 0.5 mL
• Protects for 10 years
Age:
• Indicated for children as young as 6 months traveling to endemic areas or to countries that
require it for entry
• Otherwise immunization should be delayed until 9 months or older
Side Effects:
• Mild usually low grade fever, mild headache and myalgia
• Serious adverse reaction syndrome is vaccine associated viscerotropic disease, consists of
severe multiple organ system failure and death within 1-2 weeks post vaccination
Contraindications:
• Infants younger than 6 months due to increased susceptibility to vaccine associated encephalitis
• Anaphylactic egg allergy
• Immunocompromised individuals
• Persons with a history of thymus disease
E. Meningococcus
• This is not given routinely because it is not endemic to Jamaica
• It is endemic to many states in the US and Europe
• There are 2 types:
a. Conjugate vaccine as it has the best protection and recommended
i. Always get the quadrivalent (A, C W, Y)
b. Polysaccharide vaccine
F. Cholera
• New oral vaccines are highly effective against Vibrio cholerae
• Does not prevent unapparent infection or introduction of organism into the country
• Do not give vaccine to close contacts
• Vaccine cannot control spread of cholera
• Given as 2 doses one week apart. Has an 85% vaccine efficacy against V. cholerae in the first 6
months provides protection for 6 months in children
• Greater than 50% cross protection against enterotoxigenic E. coli diarrhoea
G. Hep A
Overview
• Epidemiology
o Most hepatitis A infections occur in individuals without known risk factors for the
disease
o More than 50% of all infections are thought to occur in children
o Children are more likely than adults to be asymptomatic while infected. Therefore they
are often the mechanism by which hepatitis A is spread through households and
o Accounts for:
▪ Symptomatic hepatitis in 30% of children
▪ Symptomatic in most older children and adults with jaundice in 70%
• Presentation
o Prolonged, relapsing for > six months
o Fulminant hepatitis, underlying liver disease
• Transmission
o Fecal-oral
• Vaccine is recommended for the following:

1- Travelers to countries with high rates of hepatitis A
2- Children with chronic hepatitis B or Hepatitis C
3- Children with clotting factor disorders
4- Adolescent/adult males who have sex with men
5- Occupational exposure to hepatitis A
6- Illegal drug users
• Two inactivated hepatitis A vaccines

o Havrix and Vaqta the main ones used
o There is also a live attenuated version that is used mainly in China and India
Purpose: Prevent hepatitis A infection
Type: Inactivated
Dose:
• 2 doses (recommended)
• 0.5 mL for children dose, 1 mL for adult dose
• Given IM at 1 year and then 6 - 12 months later.
• Immunogeniticy- 88-95%
• Efficacy 94-100% and protects for 8 years
• The vaccines are interchangeable
Age: 1 – 15 years
Side Effects:
• Mild pain and induration at injection site
H. Rota Virus
Overview
• Jamaica was one of the sites for global clinical trial of this vaccine
• Rotavirus accounts for 45% of severe diarrhea in infants and children worldwide
o Oral tetravalent rotavirus vaccine (RRV-TV) was recommended

o July 1999 was associated with increased risk of intussusception
o The new Human Bovine Reassortant Pentavalent Rotavirus Vaccine (HBRP)
▪ Safe, immunogenic and efficacious
▪ No increased risk of fever, irritability, vomiting or serious adverse events
▪ Minimal replication of vaccine virus in GIT and low incidence of viral shedding
in stool
▪ NO increased risk of intussusception
▪ Prevents 88% of all rotavirus disease
• There are two brands:

o Rotarix (monovalent) Europe
o Rotateq (pentavalent) US
o There both equally effective monovalent
o Not interchangeable
Purpose: Prevent Rota Virus Infection
Type: Live attenuated vaccine
Dose:
• 2mL Oral
• Rotarix - give 2 doses
o 2 months and 4 months.
o 2nd dose can be given later than 4 months but not pass 6 months due to increased risk of
intussusception
• Rotateq - give 3 doses
o 2 moths, 4 months and 6 months.
o 3rd dose must pass 8 months due to increased risk of intussusception
Storage Temp: 0 deg C
Side Effects:
• Diarrhoea and vomiting
• Irritability
• Nasopharyngitis
• Bronchiolitis
• RARE: intussusception
Contraindications:
• Previous anaphylaxis to this vaccine or any of its components
• Precaution in immunocompromised host or children with chronic GI illnesses
• Infants transfused with blood products or immunoglobulins within 6 weeks
I. Influenza
Overview
• Flu vaccines are not given routinely in Jamaica, but it is available for people who can afford it
• Each year an active surveillance of what strains of influenza virus is there. They place the top 3 into
the vaccine where they distribute sept to oct
• Symptomatic infections of influenza are common in children because they lack immunologic
experience with influenza viruses
o Infection rates in children are greater than in adults

o Epidemics occur in the fall and winter
o Three main types of influenza viruses (A/H1N1, A/H3N2, B) cause most human
epidemics
o Spread of influenza occurs via airborne respiratory secretions.
o Incubation periods is 2-7 days
o Attack rates of 10-40% in healthy children
o Hospitalization rates of 1%
o Pneumonia, croup, bronchiolitis- 0.20-0.25%
• Increased morbidity and mortality with:
o Sickle cell anaemia
o Asthma
o Neonates with neoplasms, diabetes, chronic renal disease, heart disease with L-R shunts
• The following persons should receive an influenza vaccine:

• Asthma
• Chronic lung disease
• Cardiac disease
• Immunosuppression
• HIV
• Sickle cell anaemia
• Elderly
• Chronic renal disease
• Long term aspirin use
• Diabetes
• Late pregnancy
• Health care personnel
• Persons less than 6 months
• There are 2 types of influenza vaccine: injection or nasal spray
o Activated injectable type is only available in Jamaica but overseas there are nasal,
intranasal, etc
Purpose: Prevent annual flu infection
Content/Type/Dose/Age:
• Administer vaccine annually, 1-2 months before influenza season
• 2 doses for 1st time you are getting the
• ½ dose up until age 3 you get half a dose
• 1 dose >3
• Flu shot- killed vaccine given from age 6 months and older
• Flu nasal spray- live attenuated- recommended from 2 years onward
Storage Temp: Stored at 2-8 deg C
Side Effects:
• Local reactions
• Mild fever
• Guillain Barre syndrome
• Anaphylaxis- due to allergy to egg or chicken protein
Contraindications:
• Allergy to egg or neomycin
• Prior history of Guillen Barre Syndrome
Paediatrics
Infective Endocarditis Notes
Source: Nelson’s, Toronto Notes
September 2014
DEFINITIONS
(Just think about the name and it literally gives the definition)
• IE: Infection of the cardiac endothelium (endocarditis), most commonly the valves
• Leaflet “vegetation”: Thrombus + Bacteria + WBCs
Classifications:
• TIMING - Acute vs. Subacute endocarditis
• CAUSE - Bacterial vs. Nonbacterial endocarditis (viruses, fungi, other)
• VALVE TYPE - Native valve vs. Prosthetic valve
• SIDE OF HEART - Right-sided vs. Left-sided
Significant cause of morbidity and mortality in children despite advances in antimicrobial management
INCIDENCE
[Some of the aetiology/pathophysiology is here!]
Endocarditis is RARE IN INFANCY
***IMPORTANT: IE is often a complication of congenital or rheumatic heart disease***

BUT can also occur in children without any abnormal valves or cardiac malformations
Know this: Explanation of pathophysiology
1. Patients with congenital heart disease where there is TURBULENT BLOOD FLOW due to a hole or stenosis,
especially if there is a high-pressure gradient across the defect, are more susceptible to IE.
2. The turbulent blood flow traumatizes the vascular endothelium, which creates a substrate for DEPOSITION OF
FIBRIN AND PLATELETS leading to the formation of “nonbacterial thrombotic embolus” (NBTE) –
NBTE is initiating lesion for IE
Also BIOFILMS on mechanical devices such as valves, catheters or pacemaker wires may also act as a nidus for
infection
3. The development of TRANSIENT BACTEREMIA then colonizes the NBTE or biofilm & leads to proliferation of
bacteria within the lesion
*RE: Source of the bacteraemia: Mucosal surfaces (oropharynx, GI, vaginal or urinary tracts) heavily colonized with
potentially pathogenic bacteria. These surfaces are thought to be the origin of the TRANSIENT BACTEREMIA. Extent is
controversial.
[Interesting: Transient bacteremia reported in 20-68% of patients after tooth brushing and even in 7-51% after chewing
food!!]
Maintenance of good oral hygiene may be important in decreasing frequency of bacteraemia
RISK FACTORS
• High risk: Prosthetic cardiac valve, previous IE, congenital heart disease (unrepaired, repaired within 6 mo, repaired
with defects), cardiac transplant with valve disease (surgically constructed systemic-to-pulmonary shunts or conduits)
• Moderate risk: Other congenital cardiac defects, acquired valvular dysfunction, hypertrophic cardiomyopathy
• *Low/no risk: Secundum ASD or surgically repaired ASD (remember flow through an ASD is NOT high pressure) < VSD,
PDA, MV prolapse, IHD, previous CABG
-
Etiology of Culture negative
Endocarditis
• Opportunity for bacteremia: Intravenous drug users • HACEK (fastidious Gram-negative
bacilli)
Haemophilus parainfluenzae
Acgrecatibacter aphrophilus /
In 30% of patients with IE, a predisposing factor is recognized Acgrecatibacter
actinomycetemcomitans
Cardiobacterium hominis
Preceding dental procedure is controversial as a risk factor Eikenella corrodens
Kincella kingae
• Coxiella burnetii
Primary bacteremia with staph aureus is thought to be another risk factor • Bartonella species
• Tropheryma whipplei
• Fungi
• Mycobateria
AETIOLOGY
***Frequency of valve involvement: MV far > AV > TV > PV***

BUT in 50% of IVDU-related IE the tricuspid valve is involved
Streptococcus viridans (alpha haemolytic strep) and Staph. Aureus are the leading causative agents in
paediatric patients. [Note: NOT group A beta-haemolytic strep, which causes rheumatic fever]
Note well: Staphylococcal endocarditis is the most common in patients with no underlying heart disease!
~6% of cases = Culture negative for any organisms (May be due to the HACEK organisms in these cases)
Pseudomonas aeruginosa and serrate marcescens seen more frequently in IV drug users
Toronto Notes Table

Table 16. Microbial Etiology of Infective Endocarditis Based on Risk Factors
Native Valve IVDU Prosthetic Valve Prosthetic Valve
(recent surgery < 2 months) ( remote surgery > 2 months)
Streptococcus 1 (36% ) S. aureus ( 68 %) S. aureus ( 36% ) Streptococcus ( 20% )
S. aureus4 ( 28% ) Streptococcus ( 13% ) S. epidermidis (17%) S. aureus ( 20%)
Enterococcus ( 11%) Enterococcus Other S. epidermidis ( 20%)
S. epidermidis GNB Enterococcus Enterococcus (13%)
GNB Candida GNB Other2
Other 2 Other3 Other2
Organisms in bold are the most common isolates.
1. Streptococcus includes mainly Viridans group streptococci
2. Other includes less common organisms such as:
• Streptococcus bovis (usually associated with underlying Gl malignancy, cirrhosis)
• Culture-negative organisms including nutritionally- deficient streptococci, HACEK, Bartonella, Coxiella, Chlamydia, Legionella, Brucella
• Candida
3. IVDU endocarditis pathogens depend on substance used to dilute the drugs (i.e. tap water = Pseudomonas , saliva = oral flora, toilet water = Gl flora )
Strep. viridans is LOW VIRULENCE and only affects valves that have been damaged before
Staph. aureus is HIGH VIRULENCE and may affet valves that have never been damaged before
CLINICAL FEATURES of bacterial endocarditis = FROM MS JAN. PS ♥ (Rearranged by Anggelos)
FROM
Fever
Due to bacteremia
Seen in 80 - 90% of cases
Fever may be
Prolonged without any other manifestation
Acute onset, severe and intermittent with prostration
Roth Spots
Retinal hemorrhage with pale center
Osler's Nodes
TENDER/PAINFUL lesions on fingers pulps or toes
A complication due to embolization of septic vegetations
OUCH OUCH OSLER- To remember that this is the painful lesions
THIS DEVELOPS LATES
Murmur
Due to vegetations on heart valve
Can be NEW or CHANGING
These are common
Associated with heart failure
Symptoms: Dyspnea, orthopnea, PND.
Signs: Resp. distress, Creps, hepatomegaly, ascites, distended neck veins, peripheral oedema
MS
Myocardial abscesses
Usually seen in staph disease
May cause
Heart block
Purulent pericarditis (due to rupture of pericardium)
Signs: Arrhythmias
Splenomegaly
Relatively common
JAN
Janeway lesions
Erythematous NONTENDER/PAINLESS lesions on palms and soles
THIS DEVELOPS LATE
Anemia of chronic disease
Nail-bed splinter hemorrhages

THIS DEVELOPS LATE
PS♥
Petechiae
Relatively common
Serious neurological complications
All the following are usually associated with staph etiology
Embolic stroke
Due to vegetations breaking off from the valve
Cerebral abscesses
Mycotic aneurysms
Hemorrhage
Signs:
Meningismus
Increased ICP
Altered sensorium
Seizures
Focal deficits
Other symptoms often nonspecific: Low-grade fever, chills, night sweats, fatigue/malaise, myalgia, arthralgia, weight
loss, headache, chills, Nausea, Vomiting
HISTORY SYMPTOMS EXAMINATION

1. Prior congenital or Nonspecific/constitutional: 1. Elevated temperature
rheumatic heart disease - Fever 2. Tachycardia
- Chills
2. Prosthetic heart valve - Malaise, weakness 3. Embolic phenomena
- Night sweats • Eyes - Roth spot
- Weight loss • Skin - Petechiae
3. Preceding dental, urinary - Arthralgia, myalgia • Splinter
tract, or intestinal procedure - Chest and abdominal pain haemorrhages
• Osler nodes
4. Intravenous drug use Heart failure: • CNS – focal
- Dyspnoea, orthopnoea, PND neurological signs, ocular lesions)
4. Janeway lesions
5. Central venous catheter CNS manifestations: 5. New or changing murmur
(Stroke, seizures, headache) 6. Splenomegaly
7. Arthritis
8. Signs of heart failure
9. Arrhythmias
10. Metastatic infection (arthritis,
meningitis, mycotic
arterial aneurysm, pericarditis,
abscesses, septic
pulmonary emboli)
11. Clubbing
INVESTIGATIONS
THE CRITICAL INFORMATION FOR THE APPROPRIATE TREATMENT OF IE COMES FROM BLOOD
CULTURES. ALL OTHER LAB DATA ARE SECONDARY IN IMPORTANCE
Cultures
• 3 sets (each containing one aerobic and one anaerobic sample) collected from different sites >1 h apart
• Persistent bacteraemia is the hallmark of endovascular infection (such as IE)
• Repeat blood cultures (at least 2 sets) after 48 to 72 h of appropriate antibiotics to confirm clearance
• Timing of collections is NOT important because bacteraemia is relatively constant
Other specimens for culture: Urine, CSF, Synovial fluid, abscesses and if features of meningitis
3 different sets @ 3 different times @ 3 different times

Bloodwork
1. CBC and differential (normochromic normocytic anaemia, neutophillia)
2. U&E
3. ESR (increased)
4. RF (+)
Urine
1. Urinalysis (proteinuria, haematuria, red cell casts)
2. Urine C&S
ECG - Prolonged PR interval may indicate perivalvular abscess. Also might see AV heart block and LV Hypertrophy
Imaging
• ECHO findings (Transthroacic Esophageal Echo [TTE] AND Transesophageal Echo [TEE]) Chest X-Ray
- **Lesions > 1 cm = greatest risk for embolization, vegetations, regurgitation, abscess - Cardiomegaly
o TTE (poor sensitivity) inadequate in 20% (obesity, COPD, chest wall deformities)
o TEE/TOE indicated if TTE is non-diagnostic
DIAGNOSIS
• Duke criteria help in diagnosis
Modified Duke Criteria [2 major and 5 minor]

[Established 1994. Modified in 2000]
(Pay attention to the details of the table)
• Definitive diagnosis if: 2 (all) major, or 1 major + 3 minor, or 5 (all) minor

• Possible diagnosis if: 1 major + 1 minor, or 3 minor

Table 17. Modified Duke Criteria
Major Criteria ( 2 )
Positive culture 1-2-3 1. Positive blood cultures for IE
RULE • Typical microorganisms for IE from 2 separate blood cultures (Streprococcus viridans, HACEK group ( see ID 17 ),
by Anggelos for
MAJOR CRITERIA 1 Streptococcus bovis, Staphylococcus aureus, community-acquired enterococci ) OR
• Persistently positive blood culture, defined as recovery of a microorganism consistent with IE from blood drawn > 12 h apart
1 +ve culture of
Coxiella burnetii or all of 3 or a majority of 4 or more separate blood cultures, with first and last drawn > 1 h apart OR
• Single positive blood culture for Coxiella burnetii or antiphase I IgG antibody titer > 1 :800
2 separate +ve cultures
of typical organism 2. Evidence of endocardial involvement
• Positive echocardiogram for IE (oscillating intracardiac mass on valve or supporting structures, or in the path of regurgitant
3 persistent +ve cultures
jets, or on implanted material in the absence of an alternative anatomic explanation OR abscess OR new partial dehiscence
of prosthetic valve )
• OR New valvular regurgitation (insufficient if increase or change in preexisting murmur )
Minor Criteria ( 5 )
MINOR Criteria = 1. Predisposing condition (abnormal heart valve, IVDU)
VIPP+ by Anggelos
2. Fever 38.0°C/100.4°F )
Vascular phenom 3. Vascular phenomena: major arterial emboli, septic pulmonary infarcts, mycotic aneurysms, ICH, conjunctival hemonhages,
Immune phenom Janeway lesions
Pyrexia
Predisposing condition 4. Immunologic phenomena: glomerulonephritis, rheumatoid factor, Osier 's nodes, Roth's spots
+ve bld culture not 5. Positive blood culture but not meeting major criteria OR serologic evidence of active infection with organism consistent with IE
in major crit
Other minor criteria (Nelson’s): New clubbing, splenomegaly, splinter haemorrhages, petechiae, high ESR or CRP,
microscopic haematuria
MANAGEMENT
Medical
• Usually non-urgent and can wait for confirmation of aetiology before initiating treatment
• Empiric antibiotic therapy (i.e. before the agent is cultured) may be indicated if patient is unstable
o First-line: vancomycin + gentamicin or ceftriaxone
o High bactericidal levels must be maintained to eradicate the organisms that are growing in relatively
inaccessible, avascular vegetations!!! Several weeks are required for a vegetation to organize completely
so therapy must be continued throughout this period
• Targeted antibiotic therapy: antibiotic and duration (usually 4-6 wks) adjusted based on valve, organism
and sensitivities
• Monitor for complications of IE (e.g. CHF, conduction block, new emboli) and complications for antibiotics (e.g.
interstitial nephritis)
Streptococcus viridans: Aqueous penicillin G, ceftriaxone, + gentamicin/vancomycin

Staph aureus in the absence of prosthetic valves: Nafcillin/oxacillin + gentamicin/cefazolin. Vancomycin if oxacillin
resistant
Surgical intervention
• Indicated for severe aortic or mitral valve involvement with intractable heart failure
• Other indications, include valve ring abscess, fungal etiology, valve perforation, unstable prosthesis, ≥2 major
emboli, antimicrobial failure (persistently positive blood cultures), mycotic aneurysm, Staphylococci on a
prosthetic valve
FUNGAL IE: More difficult to treat

• Drugs of choice: Amphotericin B and 5-fluorocytosine. Surgery may be indicated
PROGNOSIS
Adverse prognostic factors: CHF, prosthetic valve infection, valvular/myocardial abscess
Mortality: prosthetic valve IE (25-50%), non-IVDU S. aureus IE (30-45%), IVDU S. aureus or streptococcal IE (10-15%)
Paediatrics
Kawasaki Disease
Source: Kaplan Paeds 2013 September 2014 (Nelson’s Updated December 2017 – Anggelos)
Severe acute vasculitis of ALL blood vessels but mostly affecting medium-sized arteries, **especially coronary;
worldwide (higher in Asians) According to the American Heart Association a
diagnosis of Incomplete Kawasaki disease is
possible in cases with FEVER and 2 principal
• Now the leading cause of acquired heart disease in US/UK/Japan features.
• **(without treatment, 20% develop coronary artery abnormalities)
• Usually children under 5 years old (80%) with peak @ 2 – 3 years; occasionally teenagers and younger adults
**Diagnostic criteria**:
- Fever for > 5 days not improved with ibuprofen or acetaminophen, plus 4 of the following 5 criteria:
1. Conjunctivitis (bilateral, without exudate (may have early anterior uveitis))
2. Changes of lips and oral cavity: Intraoral erythema, strawberry tongue, dry and cracked lips
3. Cervical lymphadenitis (Nonsuppurative) at least 1 node > 1.5 cm
4. Changes of extremities: Erythema and swelling of hands and feet; desquamation of fingertips 1-3 weeks
after onset; may involve entire hand or foot
5. Various forms of rash (but not vesicular); diapered children may have perineal desquamation
Other findings, also = Complications:

• Extreme irritability
• CNS: Aseptic meningitis
• ENT: Otitis media
• GI: 1. Diarrhoea + Vomiting, 2. Hepatitis, 3. Gallbladder Mucocele aka Hydrops
• GU: Urethritis with sterile pyuria
• MSK: Arthritis
• ***Cardiac findings:
 Early myocarditis (50%) with tachycardia and decreased ventricular function
 Pericarditis
 Coronary artery aneurysms in the second to third week. THIS IS THE MOST IMPORTANT
MANIFESTION
#Myocardial infarction has been documented as well due to Coronary artery thrombosis
Presentation
1. There are 3 phases
a. Acute (1-2 weeks)
i. Sudden onset of fever
ii. Eyes signs
iii. Oral signs
iv. Lymph signs
v. Skin signs (Rash esp. on the chest and inguinal area)
#Coronary aneurysms may appear in this phase but is rare
b. Subacute (2-4)
i. Resolution of fever
ii. Desquamation of skin (fingers and toes)
iii. Coronary artery aneurysm (most common phase of appearance)
c. Convalescent (4-6 or 4-8 weeks)

i. Resolution of clinical symptoms
ii. Beau lines may appear (deep grooved lines that run from side to side on the fingernail or the toenail.)
Lab abnormalities
1. CBC:
a. Hb - Normocytic anemia of chronic disease
b. WBC - normal to increased; neutrophils and bands. T cell lymphopaenia
c. Plts - Platelets high/normal in week 1, then significant increase in weeks 2-3 (often more than a
million)
2. Acute Phase Reactants: Extremely elevated ESR +/- CRP (non-specific markers of inflammation)
3. Urine: Sterile pyuria
4. LFTs: Increased hepatic transaminases
5. CSF: Cerebrospinal fluid (CSF) pleocytosis (abnormally large amounts of lymphocytes present)
Imaging:
2D echocardiogram Most important test; repeat at 2-3 weeks and, if normal, at 6-8 weeks. Also get ECG, follow
platelets.
Treatment:
1. Intravenous immunoglobulin (IVIg) = TREATMENT OF CHOICE
2. Aspirin
a. Anti-inflammatory dose (High-dose 80 mg/kg/day) - Acute phase
b. Antithrombotic dose – (Low-dose 3-5 mg/kg/day) - Subacute until the Convalescent phase
NB: One of the few times aspirin is used in pediatrics. Usually avoided due to risk of Reye Syndrome
3. Influenza vaccines if in winter (Reye syndrome) Rheumatic Heart Disease is the other condition in which Aspirin
can be used
Differential Diagnosis
Infectious Inflammatory Hypersensitivity
Scarlet fever Juvenile idiopathic arthritis Drug reaction
Measles Polyarteritis nodosa Steven-Johnson Syndrome
Rubella
Scald Skin Syndrome
Staphylococcus Toxic Shock Syndrome
Leptospirosis
Paediatrics
Malnutrition
Sources: Lecture [MUST still use lecture]
September 2014
BLUE = Comments from Prof. Thame in the actual class
*Prof. Thame recommends Oxford Textbook of Medicine 2nd Ed, Ed Weatherall et al, pp 8.12-8.21, 1986. Says you don't need to
go anywhere else. I didn’t use it.
Primary malnutrition:
Secondary malnutrition:
Classification of Malnutrition:
1. Gomez (weight for age only)
2. Wellcome (weight for age AND oedema)
3. Waterlow (weight for height AND height for age)
4. WHO
GOMEZ CLASSIFICATION
• Uses weight-for-age only. Expressed as a percentage
• Used in public health screening
• Used to evaluate the impact of public health interventions
• ***Limited clinical use. Reasons:
o *By using wt. for age, it fails to differentiate between longstanding growth failure and acute weight loss
o *Doesn’t take into consideration oedema - so oedematous children may be misclassified into less severe
grades (Oedema causes a higher weight even though the child may be very malnourished)
Wt. for age (%) Degree
90-110 Normal Normal

75-89 Grade I Mild
60-74 Grade II Moderate
<60 Grade III Severe
(Increments of 15%)
WELLCOME CLASSIFICATION
2 criteria: Weight for age AND Oedema

***Limitations:
• Restriction of the definition of kwashiorkor to patients with nutritional oedema only
• “Marasmic” children includes both stunted and wasted children
• (Like Gomez – by using weight for age only – fails to differentiate between longstanding growth failure and acute
weight loss)
Wt. for age (%) Oedema absent Oedema present
>80 Normal Oedematous Malnutrition

60-80 Undernutrition Kwashiorkor
<60 Marasmus Marasmic-kwashiorkor
• Know the term “oedematous malnutrition”: Look up

• Currently speak of “Severe Acute Malnutrition” at WHO (SAM)
o Intended to replace terms like kwashiorkor but even recent papers published still use the old terms
• Kwashiorkor: Means “moved away from the breast”.
• Look up a bit about kwashiorkor: Protein deficiency. Aflatoxin?
WATERLOW CLASSIFICATION
- Height-for-age is for stunting. Worried about < 85% (severe)

- Weight-for-height is for wasting. Worried about < 70% (severe)
- John Waterlow was the first director of TMRU. He created it.
Waterlow classification divides underweight for age children into 2 groups:

1. Stunted but normal proportion
2. Normal height but thin and wasted
[Simple way to remember: 1. Stunted (abnormal low height) but not wasted. 2. Wasted but not stunted (normal height)]
1. Stunted (short for age) but normally proportioned causes:

1. *Familial short stature
2. *Premature birth - “Premies”
3. Nutritional dwarfism: Reflects a MILD INSULT over a LONG PERIOD resulting in slowing or cessation of
growth (but the patient not wasted because the insult is mild)
• High energy feeding NOT appropriate: If you try to manage with dietary therapy for severe malnutrition (high
energy feeding), the patient will just become obese. *The body has basically adapted to the chronic malnutrition and trying to
correct it as if it is acute will just make the patient obese.
2. Normal height but thin and wasted:

• Caused by acute episode of malnutrition – will stop growing in height acutely and also lose weight
• Will become underweight for age more rapidly
• Not normally proportioned (thin and wasted)
• **High energy feeding IS appropriate
- Stunting = Height-for-age. Height of a child relative to the height of a normal child of the same age
- Wasting = Weight-for-height (NOT weight-for-age). *Weight for height = Weight of the child relative to what would be
normal weight for his height. Need to assess if the child’s weight is normal for the height that he is at. Not for his age. If weight-for-age
were used, that would not take into account the possibility of being very short (stunting) or being very tall affecting weight, and thus could
not determine wasting. Ie. A very short person having a low weight for his age would not be wasting, it would be expected. Need to
compare the weight with his height to be sure.
Wt. for Ht. deficit (%) Ht. for age deficit (%)
Normal 90-120 95-110
Mild 80-89 90-94
Moderate 70-79 85-89
Severe < 70 < 85
• Wasted child – Presents an immediate clinical problem where rehabilitation can lead to restoration of the lost
tissue
• Stunted child – Likely to depend upon public health measures aimed at environmental improvement
WHO Classification
Criteria Moderate Severe
Oedema No Yes
Wt/Ht -3 <SD Score <-2 < -3 SD Score
(70-79%) (<70%)
Ht/age -3 <SD Score <-2 < -3 SD Score
(85-89%) (<85%)
RESEARCH STUDIES – TMRU
• Glutathione (GSH) concentration and synthesis found to be significantly decreased in children with
oedematous malnutrition
o GSH deficiency important in pathophysiology of oedematous malnutrition
**CRITERIA FOR ADMISSION**
1. < 80% wt-for-ht WITH < 60% wt-for-age

2. < 70% wt-for-ht ALONE
3. Have nutritional oedema (***but she said ANY OEDEMA AT ALL IN CLASS)
4. A child who does not precisely meet these criteria (ie. Undernutrition by the Wellcome Classification) BUT who
is obviously clinically sick, or, who will obviously get worse quickly if sent home
5. Anorexia > a few days
6. Failure of immediate outpatient management
..
Management of malnutrition basically is the same for the 3 types:
1. Inadequate intake
2. Decreased absorption
3. Increased metabolism
HISTORY TAKING – MUST SEE THE POWERPOINT FOR THE FULL HISTORY (from slide 39)
In history - Work the symptoms!!! = Eg. If vomiting --> Ask every single thing about vomiting etc.
The typical child with severe malnutrition was usually just malnourished and just at the brink, and it is an additional insult
(usually an infection) that pushes them over. So MUST ask infection questions on history incl. RTI
Malnutrition history is very long!!!

• Very much detail required in basically every aspect of history, including in the dietary history. Family history
must ask about caregiver’s/parents’ age, education level, occupation, average weekly income, details of housing,
siblings. Remember perinatal history, esp. birth weight.
• Get the detailed history of breastfeeding (how often/day, for how long) and formula feeding (which, dilution, how
she prepares AND how much is actually eaten by the baby!!) as well as detailed history of other feeding.
Specifically ask.
Need to delve into the history of how they feed. Example, do they dilute the formula inappropriately just to stretch it.
EXAMINATION (IMPORTANT – POSSIBLE OSCE EXAMINATION STATION)

MARASMUS KWASHIORKOR MARASMIC-KWASHIORKOR
Apathetic Apathetic & irritable Combines clinical characteristics of kwashiorkor

*Generalized muscular wasting *Pitting, painless oedema and marasmus
*Absence of subcutaneous fat *Dermatitis
‘Skin and bones’ appearance Hair changes *Muscle wasting & *decreased SC fat (marasmus)
Hair – sparse, thin, dry, loss of sheen ± abdominal distension
‘Old person’s face’ – sunken cheeks due *Hepatomegaly *Oedema ± *skin lesions (kwashiorkor)
to disappearance of fat pads
± abdominal distension
Skin – dry, thin, wrinkles easily
*Full anthropometry: Weight, height, head circumference, % wt/age, % wt/ht, % ht/age! Plot it all on a growth chart!!
INSPECTION
(TRY TO THINK GENERAL APPEARANCE FROM THE HEAD TO TOE!!)
Assess GENERAL mood and behavior: *Look at entire body* - Important features are on front AND back,
head to toe. Good to make a running commentary of EVERYTHING you’re looking for in exam so the examiner
knows what you’re doing
• Altered affect, apathetic, irritable

• Stereotyped self-stimulating behavior - body rocking, rumination
• Cry – monotonous, loud groan
• Wasting: *Severity. Evidence of wasting: *Prominent ribs & limb joints. *Redundant skin folds - axillary,
gluteal. **Winging of scapula.
o ***Other sites for wasting (from top to bottom):

§ Temporalis
§ Shoulder girdle, axillary folds, inter and infra-scapular wasting (back), and wasting of deltoids
§ Prominent ribs and other joints
§ Winging of scapula (back)
§ Then pelvic girdle
§ Buttocks
§ Thighs: Wasting of quadriceps and guttering
• Pitting oedema (This is palpation, not inspection): severity – NOT JUST FEET!!: feet, legs, thighs, sacrum,
hands, periorbital. *Dependent and periorbital oedema. *In severe cases, entire body and internal organs may be
oedematous (anasarca)
SKIN
***Sequential changes (1 -> 2 -> 3):

1. First becomes darker esp. over pressure areas & places exposed to minor trauma
2. Drying & cracking of superficial skin revealing pale areas between the cracks (crazy pavement dermatosis)
3. Dry cracked layer then peels off leaving thin hypopigmented skin (flaky paint dermatosis)
• Xerosis (Dry skin)

• Skin friable
• Ulceration/maceration – perineum, flexures, behind ears
• Petechiae
• Follicular hyperkeratosis??
• Face – depigmentation/hyperpigmentation, scaling of skin around nostrils
HAIR (*Not just head hair. Also actual scalp, eyebrows, eyelashes, nails)
• Scalp hair – Describe: dull, dry, thin, fine, sparse, bald

o Atrophy of hair roots – easily pluckable
o *Hair loss – may see hair in bed
o *Forest sign – Straightening of hair at the bottom and curling on the top giving an impression of a forest
o *Flag sign – Alternating bands of discoloration of hair (reddish, blond, or gray, depending on original color) resulting from
fluctuations in nutrition/intermittent malnutrition characteristic of kwashiorkor and in diseases with protein depletion such
as ulcerative colitis.
• Eyebrows – lost, colour change
• Eyelashes – long & luxuriant, colour change
• Excess growth of lanugo hair
• Nails – rate of growth impaired ± colour change
EYES
• Conjunctival Pallor – Anaemia from malnutrition

• Angular palpebritis
• Corneal & conjunctival xerosis (Dry eyes) – Vitamin A deficiency
• Bitot’s spots – Vitamin A deficiency
• Jaundice – Liver impairment
CHEEKS
• Jowls – fullness of cheeks associated with oedematous malnutrition – ALSO may be seen in marasmus
o Cause of jowls unknown
• OR Sunken cheeks due to disappearance of fat pads - marasmus
MOUTH
• Angular stomatitis, angular cheilitis (Cheilitis = inflammation of lips)

• Oral candidiasis – Candida opportunistic in malnourished, also may have HIV
• Tongue – hyperaemic, swollen, smooth, sores
• Teeth – missing or erupting abnormally, cavities
• Gums – bleeds easily, recession of gums
Gynaecomastia – Liver impairment. Associated finding is gross hepatomegaly.
ABDOMEN
• Distended – usually gaseous distension secondary to bacterial overgrowth

• Peristalsis may be seen if abdominal wall is sufficiently thin
• Hepatomegaly – smooth, firm, non-tender due to triglyceride accumulation
• Petechiae & hyperbilirubinemia (liver dysfunction) – poor prognostic sign
BONE
• Enlargement of costochondral junction – Rachitic/rickety rosary (Vit D, Vit C, copper, phosphate

deficiency)
• Craniotabes?
• Frontal & parietal bossing
• Persistently open anterior fontanelle
• Knock-knees, bow-legs
• X-ray – marked osteopaenia
NEUROLOGIC
• Variety of abnormal neurologic signs – Don’t forget to test: **Hyper/hypotonia, **Hypo/hyper-reflexia,

abnormal movements (uncommon – may be caused by specific limiting nutrient deficiencies or underlying
disease process
• NB: Peripheral nervous system usually well preserved
• ‘Kwashi shakes’- Parkinsonian-like. In recovery phase (transient)
NOTE: “Apparent iron deficiency anaemia”

• Inability to utilize Fe rather than dietary unavailability
• Storage Fe may be increased, circulating - ferritin high/normal
• Levels of Fe binding protein reduced
• Free transferrin exerts bacteriostatic effects which are lost when the molecule is bound with Fe
Investigations (**Explanations below)

1. CBC, diff, film, Hb electrophoresis
2. Urea & electrolytes, LFTs, cholesterol, TG
3. Mg, Ca, Phos
4. VDRL, HIV
5. Blood culture, urine culture
6. CXR
7. Stool – oc&p, culture
8. Lumbar puncture / skin swab if indicated
9. Other tests as indicated
---
Clinical manifestations
• ***Hypothermic infant is more ill than a hyperthermic. Means they cannot mount an appropriate response
• Jaundice = Very bad sign. Suggests liver failure --> Only 1% of cases with liver failure no matter what u do will
survive??
Vitamin A deficiency manifestations

• Early signs:
• “Bitot spot”
• Wiki: Bitot's spots are the buildup of keratin debris located superficially in the conjunctiva, which are oval, triangular or
irregular in shape. These spots are a sign of vitamin A deficiency and are associated with conjunctival xerosis
Vitamin A deficiency is a medical emergency!!

• Can cause blindness if not promptly managed
---
• Child with severe anaemia from malnutrition e.g. Hb of 4 --> DO NOT want to replace rapidly. May cause many
problems
• Feed slowly.
• Replace their haemoglobin slowly etc. It did not get there overnight so do not try to replace it overnight
---
• Jowls --> Fat pads on the cheek in malnutrition --> Cause is unknown
• Oxford online dictionary Jowl: the lower part of a person's or animal's cheek, especially when it is fleshy or drooping
---
• Mouth -> See lecture

• Also angular chelosis
• **Smooth, pale tongue à IDA

• **Large, beefy red à B12 deficiency
---
2 very classical signs in malnutrition:
1. Crazy-pavement dermatosis
2. Flaky-paint dermatosis
---
Satellite lesions from candida (Classic lesion)
Usually if you get candida in the mouth, check the groin and vice versa.
---
Distended abdomen is usually due to a hepatomegaly!! This is why on examination, MUST start from iliac fossa
in palpation
***Primary malnutrition does NOT present with splenomegaly. Look for other pathology!
---
Rachitic/rickety rosary: Comes down side of sternum
---
Gynaecomastia due to liver impairment
Neurological signs may vary. Hypo or hypertonic etc.
Kwashi shakes: See it in the recovery phase then gets worse and then disappears. Rare. She has only seem it once
---
Investigations: Many to do
1. CBC: Invariably anaemic. May be macro or microcytic

2. U&Es: Esp. if vomiting or diarrhea. Electrolyte disturbances. Elevated urea and creatinine
3. Albumin and globulins in impaired liver function
Must investigate to detect/exclude infections!!:

4. X rays: May have a pneumonia going on but they cannot manifest the clinical features
5. Then cultures: Stool, urine etc.
6. Used to do routine LP routinely. NOT ROUTINE ANYMORE
7. Also VDRL and HIV for ALL patients.

a. Both may present like that
Treatment divided
See the lecture for the order
1. Resuscitation (1-2 weeks)
2. Maintenance (included in the above)
3. Rapid catch-up/Rehabilitation (4-6 weeks)
4. Prepare for discharge (1-2 weeks)
5. Follow-up
…
RESUSCITATION:
Aim:
Resuscitate patient – treat infections **(ALWAYS assume they have infection on board), restore electrolyte balance:
• **Broad spectrum antibiotics - 10 days
• First line treatment – Amoxil, gentamicin, Flagyl
• If fail to improve within 48 hrs / deteriorates - switch to second line therapy – cephalosporin & amikacin
Other infections:
• *Staphylococcus skin infection – Cloxacillin
• *Oral candidiasis – Nystatin suspension
• *Groin candidiasis – Antifungal cream
Treat or prevent dehydration (± vomiting & diarrhea)
Dietary management:
*Give enough to prevent hypoglycemia and hypothermia, to prevent further tissue catabolism and allow for
reversal of physiological changes without overloading the limited capacity of the heart, kidney, intestine or liver
• Oral route preferred
• IV fluids only if there are definite signs of shock
• Diet solution used: **ReSoMal solution (less sodium and more potassium than WHO soln. = better)
• Hypoglycemia can precipitate seizures

• Don't rush to correct hypernatraemia!! They didn't get there overnight
MAINTENANCE
• Recovery syndrome may be iatrogenic in maintenance and can kill child.
• Start to feed the child to: Reverse physiological changes, prevent further tissue catabolism
• No commercial formula to give what we want. TMRU prepares own milk feeds
• Energy 80-100 kcal/kg/d. Protein: 0.7 – 1.2 g/kg/day
• So must only give minimal feeding during the maintenance phase!!!

• That is, give exactly what is needed and no more!!
• May worsen oedema if too much
• See the slide for the values
• Give the feeds in divided doses

o Small volumes
o Eg. **Give every 3 hrs. Or even every 2 with even smaller volumes
• See everything given on slide
• Note that you give them **NO IRON in early phases!!!

o The iron if given may be feeding the microorganisms AND also may be toxic
• Also always assume they have infection on board. Broad spectrum + metronidazole!!!
• Maintenance usually 10-14 days
• Oral foods preferred

• Try best not to give fluid IV may worsen edema. Only if cannot tolerate oral
• Weigh every day
• See the mineral mix on slide

End of resuscitative phase:

• Treatment of infection
• Loss of oedema
• Return of appetite
• Return of affect
REHABILITATION
• Usually takes about 6-8 weeks
• Gradual increase in volume of feeds

• This leads to increase in caloric intake and weight gain
• Stomach capacity approx. 30% of body weight
• The energy density of the feed is increased by adding a concentrated source of calories to the diet
• Oil is particularly effective as it has over twice the energy density of CHO
• Protein:energy ratio at least 7 (<7 – more fat than lean tissue deposited)
RAPID CATCH-UP
• FeSO4 added
• Progress assessed by daily weights plotted on a graph (gain 5-20 g/kg/day)
• Encouraged to complete feeds
• Takes between 150-220 kcal /kg/day
• Feeds increased daily at TMRU until child fails to complete feeds

• Can be allowed out of crib for playtime – risk of cross infection is less, minimize delay in mental development
• End of RCUP marked by plateau –usually between 90-110% of expected weight for height
• DO NOT DISCHARGE BELOW 90%
PREPARE FOR DISCHARGE
FOLLOW-UP
Paediatrics
Meningitis
Sources: Nelson’s Essentials, Toronto Notes, Path and Microb. notes
September 2014
DEFINITION
• Inflammation of the leptomeninges (arachnoid and pia mater) surrounding the brain and spinal cord
Aseptic meningitis: Meningitis with negative bacterial cultures. Principally refers to viral meningitis BUT there are
many other causes of meningitis with negative CSF bacterial cultures:
a. Other non-bacterial organisms: Lyme disease (Borrelia burgdorferi), syphilis, TB, cat-scratch disease
(Bartonella henselae)
b. Parameningeal infections: Brain abscess, epidural abscess
c. Chemicals: NSAIDs, IVIG, Betadine
d. Autoimmune disorders
Partially treated meningitis: Bacterial meningitis complicated by antibiotic treatment before lumbar puncture
resulting in negative CSF cultures although other CSF findings of bacterial meningitis persist.
• *Can sometimes be confirmed with PCR of the CSF
EPIDEMIOLOGY
• Peak age: 6-12 months

• 90% of cases occur in children < 5 yr old
AETIOLOGY
[CLASSIFY: Infectious (Viral, bacterial, fungal, parasitic) vs. Non-infectious (Chemical, autoimmune)]
• Viral: Most common overall

o Most common viruses are enteroviruses (ECHOVIRUS most common) and parechoviruses. Also herpes
simplex virus (HSV), EBV, CMV, HIV, mumps.
• Bacterial: Age-related variation in specific pathogens (see Table below)
• Fungal (cryptococcal, candida) and parasitic (toxoplasma) meningitis also possible
• **Most often due to **haematogenous spread**!!!!!!

o or direct extension from a contiguous site (eg. ear infection, skull fracture)
MUST KNOW THIS TABLE FOR BACTERIAL MENINGITIS

NEONATE (*UP TO 28 DAYS – see below) > 3 months old – Pre-school OLDER CHILD+ADULT
Common 1. Gram negative bacilli – 50% 1. H. influenzae – 50% 1. S. pneumoniae – 30%

- (E. coli, klebsiella, proteus)
2. N. meningitides – 25% 2. N. meningitides – 15%
2. Group B strep (GBS) – 20%
3. S. pneumoniae
Uncommon Listeria (usu. immunosuppressed) M. tuberculosis Staph, M. TB, Listeria, H flu
***NOTE WELL: For age group for meningitis

• Have an “overlap group”
o Neonate organisms occur up to 28 days
o Age > 3 months for the other organisms
o But between this period is the “overlap group”
o (Usually don't mention pneumococcus in the overlap group)
RISK FACTORS
• Unvaccinated
• Immunocompromised: Asplenia, diabetes mellitus, HIV, prematurity
• Haemoglobinopathies: Sickle cell
• Recent or current infections: AOM, sinusitis, orbital cellulitis,

• Neuroanatomical: congenital defects, dermal sinus, neurosurgery, cochlear implants, recent head trauma (basal
skull fracture)
• Exposures: day care centres, household contact, recent travel
CLINICAL FEATURES
NOTE: Signs and symptoms variable and dependent on age, duration of illness and host response to infection
General points:
• Preceding upper respiratory tract symptoms are common
• Fever is usually present
• Indications of meningeal inflammation: Headache, nuchal rigidity, photophobia, lethargy, irritability,

nausea, vomiting
There are 3 Brudzinski signs
1. Cheek sign - Pressure of on the cheek cause
• Triad of meningism: flexion of the forearm
2. Neck sign - The one most people know
1. Neck stiffness 3, Symphyseal sign - Pressure on the pubic
2. Kernig’s sign – With the hip joint flexed, knee extension causes hamstring spasm symphysis causes flexion of the hip and knee and
adduction of the leg
3. Brudzinski’s neck sign – Passive neck flexion causes flexion of the thighs and knees
• Kernig and Brudzinski signs usually present in children older than 12 months
o Question: Can you assess Kernig’s and Brudzinski’s sign in less than 2 years old?
o Answer: YES. BUT absence of these signs does not exclude meningitis in this age group
• Focal neurologic signs: Seizures, arthralgia, myalgia, petechial/purpura, sepsis, shock, coma
• Symptoms of increased ICP: Headache, diplopia, vomiting, bulging anterior fontanelle (bulging “mole”)
• Signs of increased ICP with brain herniation: Ptosis, 6th nerve palsy, anisocoria, bradycardia with
hypertension, apnoea
HISTORY
• Infants: Fever, lethargy, irritability, poor feeding, vomiting, diarrhea, respiratory distress, seizures
• Children: Fever, headache, photophobia, N/V, confusion, back/neck pain/stiffness, lethargy, irritability
NB: MENINGOCOCCAL MENINGITIS ASSOCIATED WITH RASH IN 70% OF CASES
EXAMINATION
• Infants: Toxic appearance, hypothermia, bulging anterior fontanelle (due to increased ICP), respiratory
distress, apnea, petechial/purpuric rash, jaundice, omphalitis
• Children: Toxic, decreased LOC, nuchal rigidity, Kernig’s and Bruzinski’s signs, focal neurologic findings,
petechial/purpuric rash
Signs of Meningismus
BONK on the head
Brudzinski's sign
Opisthotonos *
Nuchal rigidity
Kernig' s sign
•Opisthotonos: rigid spasm of the body,

with the back fully arched and the heels
and head bent back
INVESTIGATIONS
A. Blood work
• CBC – Increased WBC common
• Sepsis screen: Urine, Blood, CSF examination (can do Sputum culture/sensitivity, but is not routine)
• Blood cultures positive in 90% of bacterial meningitis
B. Lumbar puncture mandatory for definitive diagnosis:

• (***Do not perform if evidence of cardiovascular instability or increased ICP other than a bulging fontanelle –
because of risk of herniation)
• Ensure no antibiotics given prior to LP
***CSF assessment:
1. Don’t forget: Macroscopic inspection: CSF cloudy in bacterial meningitis
2. Haematology: WBC count and differential, RBC

3. Chem. path: Protein and glucose concentration
4. Microbiology:
a. Gram stain (India Ink stain for cryptococcus)
b. Culture and sensitivity/resistance: Bacteria, and if appropriate fungi, virus and mycobacteria
c. Serology
d. PCR: To diagnose bacteria in partially treated meningitis. Also for enteroviruses, HSV – more sensitive
and rapid than viral culture
***Diagnosis of meningitis using CSF. Normal values:
1. Normal WBC count:

a. Infants and children 5-10 lymphocytes/mononuclear cells per ml
b. Neonates up to 15-20 or 30 is normal (for memory, just add 10 to both the ULN and LLN for infants/children
2. Normal glucose: 45-80 mg/dl or 2/3 serum values

3. Normal CSF:Serum glucose ratio: is 50-60% (low in bacterial meningitis)
4. Normal Protein: is 0.15 – 0.45 g/L (same as 150 – 450 mg/L) (just remember platelets values – very similar)
***(From Dr. Reem ward rounds) RE: CSF WBC count:

• ***WBC needs to be corrected if RBC also present in CSF!!!
• Correct using 500. Ie. For every 500 RBC in CSF, subtract 1 WBC!!
NOTE:
***Question: Can 3 cells in the CSF be significant in bacterial meningitis?
• Answer: YES, if they are neutrophils!!
***Reason for low CSF glucose in bacterial meningitis: GLUT1 transporter affected. The bacterial agents prevent
glucose from being transported across the GLUT1 transport by interfering with the transporter mechanism.
C. Urinalysis and urine C&S in infants
D. EEG (Electroencephalogram): May confirm an encephalitis component
E. Neuroimaging: CT or MRI
**Table with CSF findings in meningitis from path and microb lecture**
MANAGEMENT
A. General supportive Care

• Treat dehydration and shock
• Preservation of adequate cerebral perfusion by maintaining normal BP and managing increased ICP
• Close monitoring of fluids, electrolytes, glucose, acid-base disturbances, coagulopathies
B. Specific
Bacterial Meningitis
**If suspected or cannot be excluded, commence empiric antibiotic therapy immediately while awaiting
cultures or if LP contraindicated or delayed. Regime is AGE dependent**
• Aim: Sterilization of the CSF using antibiotics AND maintenance of cerebral and systemic perfusion
• Due to increasing resistance of S. pneumonia to penicillins and cephalosporins, empirical cefotaxime or

ceftriaxone PLUS vancomycin should be administered until antibiotic susceptibility testing is available.
• Cefotaxime or ceftriaxone are also adequate to treat N. meningitides and H. influenzae
• *Infants younger than 2 months - Add ampicillin to cover possibility of listeria!!
• ***KNOW THIS: **Adjuvant dexamethasone** BEFORE antibiotics for Hib meningitis. REASON:
Significantly diminishes the incidence of hearing loss and neurologic deficits associated with Hib meningitis;
also consider for those > 6 wk with pneumococcal meningitis
***MENINGOCOCCAL MENINGITIS NEEDS TO BE ISOLATED***
Duration of treatment: 5-7 days for N. meningitides, 7-10 days for H. influenzae and 10-14 days for S. pneumonia.
Viral Meningitis
• Usually benign and self-limiting
• Mainly supportive (except for HSV)
• Acyclovir for HSV meningitis
PREVENTION
Vaccination against pneumococcus (see sickle cell notes for the available vaccines) and Hib
Chemoprophylaxis with refampicin, ciprofloxacin or ceftrixone
COMPLICATIONS
[CLASSIFY: ACUTE vs. LONG-TERM]
• Mortality: Neonate 15-20%, children 4-8%; pneumococcus (25% mortality) > meningococcus (15%) > HiB
(8%)
[Note: Pneumococcus has highest mortality and highest risk of long-term complications]
• Acute:
o SIADH
o Subdural effusion/empyema (seen on CT/MRI – most do not need drainage unless assc with neurologic
signs),
o Brain abscess
o Disseminated infection (osteomyelitis, septic arthritis, abscess)
o Shock/DIC
• Long term sequelae (present in 35% survivors – esp after pneumococcal infection): deafness, blindness,
neuromotor/cognitive delay, learning disabilities, neurological deficit, seizure disorder, hydrocephalus
• ***ALL patients with meningitis should have a hearing evaluation before discharge and at follow-up
1. Meningitis
2. Encephalitis
3. Hemorrhage
4. Rheumatic disease
5. Malignancy
Paediatrics
Neonatal Jaundice and Hyperbilirubinaemia
Source: Nelson’s (Ch. 96.3)
October 2014
General points:
• Hyperbilirubinaemia is common and in most cases benign in neonates.

• Observed in first week of life in ~60% of term infants and 80% of preterm infants
• Bilirubin has a physiologic role as an antioxidant

• Elevations of INDIRECT/UNCONJUGATED bilirubin is potentially NEUROTOXIC
• DIRECT/CONJUGATED NOT neurotoxic, BUT may indicate a potentially serious hepatic or posthepatic illness
AETIOLOGY
Unconjugated bilirubin may be increased by any factor that does any of the following 4 things:
1. Increases the load of bilirubin to be metabolized by the liver:

a. Haemolytic anaemias
b. Polycythaemia
c. Bruising or internal haemorrhage (e.g. cephalhaematoma)
d. Increased enterohepatic circulation of bilirubin (e.g. due to intestinal obstruction à more bili reabsorbed)
e. Infection
2. Damages or reduces the ACTIVITY of the transferase enzyme
a. Genetic, hypoxia, infection, hypothyroidism
3. Competes for or blocks the transferase enzyme
a. Drugs
4. Leads to absence or reduced AMOUNTS of the enzyme/reduction of bilirubin uptake by liver
a. Genetic, prematurity
***The toxic effects of high serum unconjugated bilirubin are increased by factors that reduce the retention of bilirubin
within the circulation (ie. increase ability to leave the circulation and enter unwanted sites)
• Include: Hypoproteinaemia, displacement of bilirubin from its binding site on albumin, competitive binding of
drugs to albumin e.g. sulfamethoxazole, ceftriaxone, acidosis
Neurotoxic effects also related to the permeability of the blood-brain barrier and nerve cell membranes and neuronal
susceptibility to injury – all of which are worsened by asphyxia, prematurity, infection
- Breast-feeding and dehydration increase serum bilirubin

- Delay in passage of meconium increases serum bilirubin – due to enterohepatic recirculation after deconjugation
by intestinal glucuronidase
- Diabetic mother is also a risk factor
Online source: It is important to note that only conjugated bilirubin appears in urine (unconjugated bilirubin is albumin
bound and water insoluble). The presence of bilirubin in urine almost always implies liver disease.
CLINICAL FEATURES
Jaundice usually becomes apparent in a cephalocaudal progression

• General estimation: Face ~5 mg/dl, Mid-abdomen ~15 mg/dl, Soles ~20 mg/dl – But clinical exam CANNOT be
used to estimate serum levels reliably!!
Infants with severe hyperbilirubinaemia may present with lethargy and poor feeding and without treatment may progress
to BILIRUBIN ENCEPHALOPATHY (kernicterus)
• ** Jaundice, consisting of either indirect or direct bilirubin that is PRESENT AT BIRTH OR APPEARS WITHIN
THE FIRST 24 HOURS OF LIFE requires IMMEDIATE ATTENTION
o May be due to erythroblastosis fetalis, concealed haemorrhage, sepsis, congenital infections incl.
syphilis, CMV, rubella, toxoplasmosis (TORCH infections – a group of congenitally acquired infections
that cause significant morbidity and mortality)
• Jaundice appearing on 2nd or 3rd day is usually PHYSIOLOGIC – but may be due to Familial nonhaemolytic
icterus (Crigler-Najjar syndrome) or early-onset breastfeeding jaundice
• Jaundice appearing after the 3rd day but within 1st week suggests bacterial sepsis or UTI – or syphilis, toxo,
CMV, enterovirus
Polycythaemia may also lead to early jaundice
Long differential diagnosis for starting AFTER the 1st week of life. Includes:
• Breast milk jaundice
• Septicaemia
• Congenital atresia of bile ducts
• Hepatitis
• Galactosaemia
• Hypothyroidism
• Cystic fibrosis
• Congenital haemolytic anaemia – membranopathies (e.g. sphero- & elliptocytosis), enzymopathies (e.g. G6PD,
PK deficiency)
Increased direct bilirubin Increased indirect
bilirubin
Persistent jaundice after 1st
I ’
1
month:
• Cholestasis
Sepsis
Intrauterine infection
Toxoplasmosis
Cytomegalovirus
Positive Coombs test
r Negative Coombs test
• Hepatitis
• CMV
Rubella
Herpes
Syphilis
Paucity of bile ducts
Isoimmunization
Rh
ABO
Other blood group
r
Normal or low
Hemoglobin
High (polycythemia)
• Syphilis Disorders of bile acid metabolism
Severe hemolytic disease I
• Toxoplasmosis Biliary atresia Reticulocyte count [ Twin transfusion
• Crigler-Najjar
Giant cell hepatitis
Choledochal cyst £ Maternal-fetal
transfusion
r
Cystic fibrosis Increased Delayed cord
• Congenital atresia of bile- Galactosemia
Alpha1-antitrypsin deficiency
clamping
Small for gestational
ducts Tyrosinemia
\ Red cell morphology \
age infant
Hyperalimentation cholestasis
• Galactosaemia i
Characteristic Nonspecific Normal
Complete diagnostic evaluation in Spherocytosis G6PD deficiency Enclosed hemorrhage

any patient with significant Elliptocytosis
Stomatocytosis
PK deficiency
Other enzyme
Increased enterohepatic circulation,
delayed or infrequent stooling.
hyperbilirubinaemia: Pyknocytosis
Fragmented cells
deficiency
Disseminated
bowel obstruction
Inadequate caloric intake
intravascular Neonatal asphyxia
coagulation
1. Measure bilirubin i
(transcutaneous or serum) Prolonged hyperbilirubinemia
in babies with jaundice.

2. Direct + Indirect bilirubin
*
Gilbert syndrome
Down syndrome
3. Hb Hypothyroidism
Breast -feeding
Cngler-Najjar syndrome
4. Ret count
5. Direct Coomb’s test
6. Blood smear
Also a good image to look through. Source: Toronto Notes

**This table is important to go through as it shows which causes conjugated and which causes unconjugated**
Hyperbilirubinema
i
Unconjugated
l
Conjugated
Pathologic Physiologic
i
Always pathologic
i
Hemolytic
i
Non-Hemolytic Hepatic Post -Hepatic
Hematoma Infectious Biliary atresia
( cephalohematoma) Sepsis Choledochal cyst
*
Intrinsic Extrinsic
Polycythemia
Sepsis
Hep B, TORCH
Metabolic
Membrane Immune
Hypothyroidism Galactosemia
Spherocytosis ABO incompajtability
Elliptocytosis Rh incompatability Gilbert syndrome Tyrosinemia
Enzyme Kell, Duffy, etc. Crigler-Najjar a- 1 -antitrypsin deficiency
G6PD deficiency Non-immune Hypothyroidism
PK deficiency Splenomegaly CF
Hemoglobin Sepsis Drugs
a thalassemia AV malformation TPN
Idiopathic neonatal hepatitis
Figure 13. Approach to neonatal hyperbilirubinema
PHYSIOLOGIC JAUNDICE (ICTERUS NEONATORUM)
Becomes visible on the 2nd or 3rd day, peaks 3rd to 4th day, and decreases between 5th and 7th (So 2-3, 3-4, 5-7 10-14)
**Indirect bilirubin levels in term infants decline to adult levels by 10-14 days of life
• CAUSES INDIRECT HYPERBILIRUBINAEMIA
Believed to be due to:

1. Increased bilirubin production from the breakdown of fetal RBCs and
2. Immaturity of hepatic bilirubin conjugation system (transient)
3. Increased enterohepatic circulation
***Persistent indirect hyperbilirubinaemia (> 14 days) suggests haemolysis, hereditary glucuronyl transferase
deficiency (Gilbert syndrome), breast milk jaundice, hypothyroidism, intestinal obstruction
PATHOLOGIC HYPERBILIRUBINAEMIA
Assume any jaundice in first 24 hours is pathological

Toronto: Jaundice in the first 24 h and conjugated hyperbilirubinemia are always pathological.
Risk factors incl: Asian, prematurity, breast-feeding, weight loss
Some causes: Gilbert syndrome, G6PD deficiency, mutations in glucuronyl transferase gene
Greatest risk associated with indirect hyperbili: BILIRUBIN ENCEPHALOPATHY (KERNICTERUS)

• Development depends on the level of indirect bili, duration of exposure, infant’s wellbeing
• May occur at lower bilirubin levels in preterm infants
BREAST-MILK VS BREAST-FEEDING JAUNDICE
Breast-milk jaundice develops in ~ 2% of breastfed term infants after the 1st week of life. Peaks in 2nd – 3rd week
• If breastfeeding is continued, may persist for up to 3-10 weeks but gradually decreases
• INDIRECT HYPERBILIRUBINAEMIA
• Cause unclear but related to presence of glucuronidase in milk
• Phototherapy may benefit. Kernicterus may occur but uncommon.
• Should be distinguished from:
Breast-feeding jaundice: early-onset breast-feeding jaundice which occurs in 1st week of life (usu in 1st 3 days) in
breastfed infants. Hyperbilirubinaemia develops in 13% of breastfed infants in 1st week of life and may be due to
decreased milk intake/production with dehydration and reduced caloric intake --> Exaggerated physiologic
jaundice. Worsened if given glucose water as this is even less calorie dense
Frequent breastfeeding and ongoing lactation may reduce the incidence. Should continue breastfeeding still
KERNICTERUS AKA. BILIRUBIN ENCEPHALOPATHY

A neurologic syndrome resulting from the deposition of unconjugated bilirubin in the BASAL GANGLIA AND
BRAINSTEM NUCLEI
Multifactorial pathogenesis:
• Involves unconjugated bili, albumin binding and unbound bilirubin, passage across BBB and neuronal
susceptibility to injury
• Disruption of BBB by asphyxia, disease increase risk
• Precise blood level of indirect bilirubin that causes kernicterus in an individual infant is unpredictable – large
study showed all cases > 20 mg/dl
• 90% developed in previously healthy, mainly breastfed, near-term infants
• More immature the infant = more susceptibility to kernicterus
CLINICAL FEATURES
Signs and symptoms usually appear 2-5 DAYS AFTER BIRTH IN TERM INFANTS (EARLIER)
o AS LATE AS 7TH DAY IN PREMATURE
o But may occur at any time in neonatal period
• Initial signs: Lethargy, poor feeding and loss of Moro reflex are common
• After this, infant may appear seriously ill and prostrate

o DECREASED tendon reflexes
o Respiratory distress
o Opisthotonos with a bulging fontanelle + twitching of face/limbs may follow
• Advanced cases:
o Convulsions and spasms
o Stiff extension of arms with inward rotation and clenched fists
• *Many infants who progress to this severity die (> 75%)
Survivors usually seriously damaged:

• First 2-3 months: APPEAR to recover and show few abnormalities
• Later in 1st year: Opisthotonus, muscle rigidity, irregular movements, convulsions recur
• 2nd year: Opisthotonus and seizures abate – Involuntary muscle movements, rigidity or hypotonia increase
• 3rd year: Complete neurologic syndrome apparent. Bilateral choreoathetosis, involuntary spasms, seizures, mental
deficiencies, dysarthric speech, hearing loss, squinting, defective upward eye movement
o Hypotonia + ataxia in some
Some cases milder: Partial deafness, minimal brain dysfunction
TREATMENT OF HYPERBILIRUBINAEMIA
o Regardless of cause, goal is to prevent neurotoxicity while not causing undue harm
o Phototherapy and if unsuccessful, exchange transfusion are the primary treatment modalities used to keep
serum bilirubin low
o Treat underlying cause when identified
PHOTOTHERAPY:
• FOR UNCONJUGATED HYERBILIRUBINAEMIA ONLY
• Indirect hyperbilirubinaemia reduced by exposure to high intensity of light. Bilirubin absorbs light maximally
in the blue range.
• May use age/gestation specific charts to determine level to start phototherapy
• Mode of action: Bilirubin in the skin absorbs light energy causing several photochemical reactions. One major
product of phototherapy is due to photoisomerization reaction converting toxic native unconjugated bilirubin
into another unconjugated isomer which can be excreted in bile without conjugation
• Other major product of phototherapy is lumirubin which can be excreted by kidneys in unconjugated state
• Dark skin does NOT reduce the efficacy of phototherapy
• Use of phototherapy has decreased the need for exchange transfusion. BUT when indications for exchange
transfusion are present, phototherapy should not be used as a substitute
• Must cover eyes
• Should be discontinued as soon as bilirubin is in safe range
• Measure serum bilirubin 4-24 hrly during therapy. Continue monitoring serum bilirubin for at least 24 hrs
Complications
• Loose stools
• Macular rash
• Purpuric rash
• Overheating
• Dehydration
• Hypothermia
• Bronze baby syndrome – benign condition – dark brown discolouration of infant
**Contraindicated in the presence of porphyria AND CONJUGATED HYPERBILIRUBINAEMIA
**There are no known long-term effects of phototherapy

IVIG – useful in hyperbilirubinaemia due to IMMUNE HAEMOLYTIC ANAEMIAS
EXCHANGE TRANSFUSION:
Double-volume exchange transfusion is performed if intensive phototherapy has failed and if risk of kernicterus exceeds
risk of procedure
Complications are not trivial:

1. Metabolic acidosis
2. Electrolyte abnormalities
3. Hypoglycaemia
4. Hypocalcaemia
5. Thrombocytopaenia
6. Volume overload
7. Arrhythmias
8. Infection
9. GVHD
10. Death
Repeated if necessary to keep bilirubin in safe range
SEE INDICATIONS:
1. Appearance of clinical signs suggesting kernicterus
2. Most commonly performed for haemolytic disease and G6PD deficiency
3. …
Straight from Toronto notes:
BILIARY ATRESIA
Definition
• atresia of the extrahepatic bile ducts which leads to cholestasis and increased conjugated
bilirubin after the first week of life
Epidemiology
• incidence: 1:10,000-15,000 live births
Clinical Presentation
• dark urine, pale stool, jaundice (persisting for >2 wk), abdominal distension, hepatomegaly
Diagnosis
• conjugated hyperbilirubinemia, abdominal ultrasound
• HIDA scan
• liver biopsy
Treatment
• surgical drainage procedure
• hepatoportoenterostomy (Kasai procedure; most successful if <8 wk of age)
• usually requires liver transplantation later in life
• vitamins A, D, E, and K; diet should be enriched with medium-chain triglycerides to ensure
adequate fat ingestion
Paediatrics
Nephritic Syndrome, Poststreptococcal GN, Haemolytic Uraemic Syndrome, Other causes
Sources: Toronto, Oxford, Nelson’s for PSGN
October 2014
**Use this along with notes on haematuria**
**Notes addresses nephritic syndrome first, then goes into some of the common causes e.g. PSGN**
DEFINITION
• Acute or chronic syndrome affecting the kidney, characterized by glomerular injury and inflammation, and
defined by haematuria (> 5 RBCs per high-powered microscope field – Tea or cola/pepsi-colored urine since
problem is at the glomerular level) and the presence of dysmorphic RBCs (eg. acanthocytes) and RBC casts on
urinalysis
• Often accompanied by at least one of = HOOPA

o Hypertension
o Proteinuria (< 50 mg/kg/d) – (Ie. NOT nephrotic-range proteinuria. LESS.)
o Facial or body oedema
o Azotemia and oliguria
EPIDEMIOLOGY
• Highest incidence in children aged 5-15 yr old (Coincides with age for strep pharyngitis)
AETIOLOGY
• Humoral immune response to a variety of aetiologic agents --> immunoglobin deposition --> complement
activation, leukocyte recruitment, release of growth factors/cytokines --> glomerular inflammation and injury -
-> porous podocytes -> haematuria + RBC casts ± proteinuria
• **Hypertension secondary to fluid retention and increased renin secretion by ischemic kidneys
• Primary (idiopathic) vs. secondary (to a systemic disease), low complement levels vs. normal complement levels
• Majority of cases post-infectious

• Usually presents 1–2 wks after a URTI and sore throat.
Table 39. Major Causes of Nephritic Syndrome

Decreased C3 Normal C 3
Primary Post-infectious GN ( most common cause of acute IgA Nephropathy
GN in pediatrics) Idiopathic rapidly progressive GN
Membranoproliferative Anti- GBM disease
• Type I ( 50- 80% )
• Type II ( > 80%)
Secondary SLE Henoch-Schonlein purpura ( very common )
Bacterial endocarditis Polyarteritis nodosa
Abscess or shunt nephritis Granulomatosis with polyangiitis (GPA )
Cryoglobulinemia Goodpasture's syndrome
RISK FACTORS
• Recent streptococcal pharyngitis or skin infection, systemic illnesses
HISTORY AND PHYSICAL

• Often asymptomatic; some overlap in clinical findings for nephritic and nephrotic syndrome
• Gross haematuria (Tea- or cola-colored urine), mild-moderate oedema, oliguria
• Signs and symptoms suggestive of underlying systemic causes (e.g. fever, arthralgias, rash, dyspnea,
pulmonary hemorrhage)
INVESTIGATIONS
***(So want to look at BLOOD and URINE and consider biopsy)***
Urine
• Dipstick (haematuria, 0 to 2+ proteinuria)
• Urine microscopy (>5 RBCs per high-powered microscope field, acanthocytes, RBC casts)
• “First morning” urine protein:creatinine ratio (<200 mg/mmol)
Blood work
• CBC: Mild anaemia on CBC (secondary to haematuria)
• Albumin: Hypoalbuminaemia (secondary to proteinuria)
• U&Es: Impaired renal function (Cr and BUN) resulting in pH and electrolyte abnormalities (hyperkalemia,
hyperphosphataemia, hypocalcaemia)
• Appropriate investigations to determine aetiology: *C3/C4 levels, serologic testing for *recent streptococcal
infection (ASOT, anti-hyaluronidase, anti-streptokinase, anti-NAD, anti-DNase B), *ANA, *anti-DNA
antibodies, ANCA, serum IgA levels, anti-GBM antibodies
Renal biopsy
• ***NOTE WELL: Should be considered only in presence of: acute renal failure, no evidence of streptococcal
infection, normal C3/C4, low C3 (hypocomplementaemia) persisting for > 2 months
MANAGEMENT
• Treat underlying cause

• Symptomatic
o Renal insufficiency: Supportive (dialysis if necessary), proper hydration
o Hypertension: Salt and fluid restriction (but not at expense of renal function), ACE inhibitors or ARBs
for chronic persistent HTN (not acute cases since ACE inhibitors or ARBs may decrease GFR further)
o Oedema: Salt and fluid restriction, possibly diuretics (avoid if significant intravascular depletion)
• Corticosteroids if indicated: IgA nephropathy, lupus nephritis, etc.
PROGNOSIS
• Dependent on underlying aetiology
• Complications include hypertension, heart failure, pulmonary oedema, chronic kidney injury (requiring renal
transplant)
ACUTE POSTSTREPTOCOCCAL GLOMERULONEPHRITIS (APSGN)

(Big Nelson’s Ch. 505)
Group A Strep (GAS) infections common in children and can lead to the postinfectious complication of GN.
“APSGN is a classic example of acute nephritic syndrome”!!! Characterized by the sudden onset of gross haematuria,
oedema, hypertension and renal insufficiency
ONE OF the most common causes of gross haematuria in children (UTI is the most common)
INCIDENCE
• Most common in children aged 5-12
• Uncommon before age 3
• M>F
AETIOLOGY
PSGN follows throat OR skin infection by “NEPHRITOGENIC” STRAINS of GAS
NOTE WELL: PSGN commonly follows *streptococcal pharyngitis in cold months and *streptococcal skin
infections or pyoderma in warm months (So don’t forget that it may be pharyngitis or skin infection!! Illicit in history!!)
PATHOLOGY
• Kidneys appear SYMMETRICALLY enlarged
• Glomeruli appear enlarged and show diffuse mesangial cell proliferation
• Neutrophil infiltration common in glomeruli in the early stage
• Immunofluorescence microscopy reveals a pattern of “lumpy-bumpy” deposits of immunoglobulin and

complement on the glomerular basement membrane
PATHOGENESIS
Morphology studies + depression in serum complement (C3) levels provide strong evidence that PSGN is mediated by
IMMUNE COMPLEXES
Exact mechanisms not fully known. ***Some proven ones include:

1. Circulating IMMUNE COMPLEX formation with streptococcal antigens followed by glomerular deposition of
these complexes
2. MOLECULAR MIMICRY whereby circulating antibodies formed against streptococcal antigens react with
normal glomerular antigens
3. Complement activation by directly deposited streptococcal antigens
Group A strep possess M proteins and nephritogenic strains are related to the M-protein serotype. The exact nephritogenic
antigens on or produced by the GAS are not fully known.
**Typical patient develops acute nephritic syndrome 1-2 weeks after a *streptococcal pharyngitis OR 3-6 weeks after
a *streptococcal pyoderma
**History of a specific infection may be absent because symptoms may have been mild or gone unnoticed
See all the features of nephritic syndrome on page 1
Also non-specific symptoms common: Malaise, lethargy, abdominal pain, flank pain
ALSO NOTE:!!!
• The severity of kidney involvement varies!!:
o From asymptomatic microscopic haematuria to gross haematuria with acute renal failure
NOTE WELL: Patients are at risk for developing ENCEPHALOPATHY and/or HEART FAILURE, both
secondary to hypertension. The encephalopathy may also result from toxins produced by GAS
***MUST KNOW: HYPERTENSIVE ENCEPHALOPATHY must be considered in patients with blurred vision, severe
headaches, altered mental status, new seizures. MUST ASK IN HISTORY!
Heart failure and pulmonary oedema: Respiratory distress, orthopnoea, cough. MUST ASK!
Peripheral oedema: Due to salt and water retention!! Very uncommonly (< 5%) due to nephrotic syndrome in childhood
cases. ASK ABOUT EYE/LEG SWELLING!
***ACUTE PHASE USUALLY RESOLVES IN 6-8 WEEKS**

Proteinuria and HTN usually resolve by 4-6 weeks after onset BUT persistent microscopic haematuria can persist for 1-2
years
DIAGNOSIS
(See nephritic syndrome section above for excluding differentials)
• URINALYSIS: RBCs, often with RBC casts, proteinuria, neutrophils

• CBC: Mild normochromic, normocytic anaemia due to haemodilution
• *SERUM C3 LEVEL! – Significantly reduced in > 90% of patients in the acute phase. Returns to normal 6-8
weeks after onset!!
o NOTE: C4 is often normal or only mildly depressed
- NB: C3-C9 may be elevated. Normal C1 and C4
NOTE VERY WELL: CONFIRMATION OF THE DIAGNOSIS REQUIRES CLEAR EVIDENCE OF

PRIOR STREPTOCOCCAL INFECTION!!!
***Positive throat culture may support the diagnosis or may simply suggest the carrier state BUT RISING
ANTIBODY TITRE (serology) to streptococcal antigens CONFIRMS a recent strep infection
• *SEROLOGY: Antibodies titres assessed:
1. ASO titre – commonly elevated after pharyngeal infection but rarely increases after streptococcal skin
infections
2. ***The single best antibody titer to document cutaneous streptococcal infection is the anti-
deoxyribonuclease B (anti-DNase B) level
3. Anti-hyaluronidase
**NB: Serologic evidence of strep infection more sensitive than history of recent infection and far more sensitive than
positive bacterial cultures obtained at the onset of acute nephritis
• MRI OF BRAIN indicated in patients with severe neurological symptoms and can demonstrate reversible
posterior leukoencephalopathy
• Chest X-ray in patients with signs of heart failure
The clinical diagnosis of PSGN is likely in a child presenting with acute nephritic syndrome, evidence of recent strep
infection and a low C3 level. But must consider differentials such as SLE.
Differential diagnosis includes many of the causes of haematuria – See notes on haematuria
See indications for renal biopsy in nephritic syndrome above
**ACUTE POST-INFECTIOUS GN CAN ALSO FOLLOW OTHER INFECTIONS INCL COAGULASE POSITIVE
AND COAGULASE NEGATIVE STAPH, STREP PNEUMONIAE, GRAM-NEGATIVE BACTERIA
COMPLICATIONS
*Acute complications result from hypertension and acute renal dysfunction
• HYPERTENSION IS SEEN IN 60% OF PATIENTS AND IS ASSOCIATED WITH HYPERTENSIVE

ENCEPHALOPATHY IN 10% OF CASES
• Severe prolonged HTN can lead to intracranial bleeding
• Other complications: Heart failure, hyperkalaemia, hyperphosphataemia, hypocalcaemia, acidosis, seizures,

uraemia
• Acute renal failure may require dialysis
TREATMENT
• Mainly supportive
• Directed at treating the acute effects of renal insufficiency and HTN
• Penicillin for 10 days
• NOTE WELL: Although a 10 day course of systemic antibiotic therapy with PENICILLIN is recommended to
limit the spread of nephritogenic organisms, ANTIBIOTIC THERAPY DOES NOT AFFECT THE NATURAL
HISTORY OF GN
• For HTN: Standard therapies used are sodium restriction, diuresis with IV furosemide, drug therapy with calcium
channel blockers, vasodilators, ACEis
PROGNOSIS
• Complete recovery occurs in > 95% of patients with PSGN

• Recurrences are extremely rare
• Mortality can be avoided by appropriate management of ARF, CCF and HTN
Other causes of GN to know something about:
1. IgA Nephropathy: More variable and may take the form of acute GN, asymptomatic microscopic hematuria, or recurrent
gross hematuria concurrent with an upper respiratory infection as opposed to several days later, as with PSGN. Lab findings:
↑ Serum IgA (50%). Treatment: Uncertain (options include steroids, fish oil, and ACE inhibitors)
2. Membranoproliferative GN
3. Haemolytic Uraemic Syndrome (See below)
4. Henoch-Schonlein purpura: Most common small vessel vasculitis in childhood. Characterized by a purpuric rash and
arthritis and abdominal pain. ~50% of patients with HSP develop renal manifestations, mediated by the deposition of IgA
in glomeruli. Glomerular findings can be indistinguishable from those of IgA nephropathy. Nephritis that can accompany
HSP usually follows onset of the rash, often weeks or even months after the initial presentation of the disease. Treatment:
Spontaneous and complete resolution of the nephritis typically occurs in those with mild initial manifestations. Studies have
reported benefit from aggressive immunosuppression (high-dose and extended courses of corticosteroids with
cyclophosphamide or azathioprine) in patients with poor prognostic features.
5. Alport syndrome: Sensorineural deafness with progressive nephritis. Caused by X-chromosome mutations in type IV
collagen leading to an abnormal glomerular basement membrane (GBM) and may present with either asymptomatic
microscopic or gross hematuria. Males typically develop progressive renal failure and sensorineural hearing loss during
adolescence and young adulthood. Females typically have a more benign course but usually have at least microscopic
hematuria.
6. Goodpasture syndrome: Goodpasture disease is characterized by pulmonary hemorrhage and glomerulonephritis.

Results from the attack of these normal organs by antibodies directed against specific epitopes of type IV collagen within
the alveolar basement membrane in the lung and glomerular basement membrane (GBM) in the kidney. The etiology
of these antibodies is unknown. Rare in childhood. Patients usually present with haemoptysis from pulmonary
hemorrhage that can be life threatening. Concomitant renal manifestations include acute nephritic syndrome.
Diagnosis is made by a combination of the clinical presentation of pulmonary hemorrhage with acute
glomerulonephritis, the presence of serum antibodies directed against GBM. Untreated, the prognosis of Goodpasture
disease is poor. The combination of high-dose intravenous methylprednisolone, cyclophosphamide, and
plasmapheresis appears to improve the possibility of survival
6. SLE associated GN
HAEMOLYTIC URAEMIC SYNDROME (TORONTO NOTES)

DEFINITION
Simultaneous occurrence of the triad of:
1) Non-immune microangiopathic haemolytic anemia
2) Thrombocytopenia and
3) Acute renal injury
***Most common cause of acute renal failure in children
(Note: Name gives away 2 of the 3: Haemolytic → Haemolytic anaemia, Uraemic → Acute renal injury)
AETIOLOGY
• Diarrhea positive HUS: 90% of pediatric HUS from E. coli O157:H7, shiga toxin aka verotoxin
• Diarrhea negative HUS: other bacteria, viruses, familial, drugs
PATHOPHYSIOLOGY
• Toxin binds, invades and destroys colonic epithelial cells, causing bloody diarrhea
• Toxin enters the systemic circulation, attaches and injures endothelial cells (especially in kidney) causing a
release of endothelial products (e.g. von Willebrand factor, platelet aggregating factor)
• Form platelet/fibrin thrombi in multiple organ systems (e.g. kidney, pancreas, brain, etc.) resulting in
thrombocytopenia
• RBCs are forced through occluded vessels resulting in fragmented RBCs (schistocytes) that are removed by the
reticuloendothelial system (haemolytic anaemia)
HISTORY AND PHYSICAL

• Initial presentation of abdominal pain and diarrhea, followed by bloody diarrhea + ABSENCE OF FEVER
• Within 5-7 d begins to show signs of anaemia, thrombocytopenia and renal insufficiency
• Other findings in history: weakness, lethargy, oliguria
• Physical exam: Pallor, jaundice (hemolysis), oedema, petechiae, hypertension
INVESTIGATIONS
• Blood
• CBC (anemia, thrombocytopenia),
• Electrolytes,
• Renal function
• Blood smear (schistocytes)
• Urine
• Urinalysis (microscopic hematuria)
• Stool
• Stool cultures
• Verotoxin/shigella toxin assay
MANAGEMENT (Mainly Supportive)
• Nutrition
• Hydration
• Ventilation (if necessary)
• Blood transfusion for symptomatic anemia
• Monitor
• Electrolytes and renal function: dialysis if electrolyte abnormality cannot be corrected,
• Fluid overload
• Uremia
• Blood pressure
• Contraindicated
• Antibiotics because death of bacteria leads to increased toxin release and worse clinical course
• Anti-diarrheal
• nsAids
• Steroids are NOT helpful
PREVENTION
• Stay away from unpasteurized milk
• Wash hands after using bathroom
• Do no used contaminated foods and water
PROGNOSIS
5-10% mortality, 10-30% renal damage
Wayne Robinson, MBBS Class of 2015 1
Paediatrics
Nephrotic Syndrome Notes
Source: Nelson’s (Ch. 521), Toronto
September 2014
DEFINITION
Clinical syndrome affecting the kidney, characterized by nephrotic-range proteinuria, peripheral oedema,
hypoalbuminaemia, and hyperlipidemia
• **Nephrotic-range proteinuria definitions (Lecture):

1. > 50 mg/kg/d in 24 hour urine collection
2. > 40 mg/m2/hr in 12-24 hour urine collection
3. First morning URINE (total) protein:creatinine ratio > 2
4. 4+ proteinuria on at least 5 consecutive urines
• *Hypoalbuminaemia (< 20-25 g/L)

A manifestation of glomerular disease
INCIDENCE
• Highest incidence in children of 2 - 6 yr old,
• M>F (2:1) in childhood. M=F in adolescence
AETIOLOGY
[CLASSIFY: Primary vs. Secondary]
[Primary can be further classified by histological types]
1. Primary/Idiopathic nephrotic syndrome (>90%); Nephrotic syndrome (NS) in the absence of systemic disease
(most common cause in pediatrics)
• Glomerular inflammation ABSENT on renal biopsy:
i. Minimal change disease (>90% of all NS)
ii. Focal segmental glomerular sclerosis (FSGS)
• Glomerular inflammation PRESENT on renal biopsy:

i. Membranoproliferative glomerulonephritis,
ii. Mesangial proliferative GN,
iii. membranous nephropathy,
iv. IgA nephropathy,
v. other minor causes
2. Secondary nephrotic syndrome: NS associated with systemic disease or due to another process causing
glomerular injury (very rare in pediatrics)
• Infections: Post-streptococcal, HBV/HCV, infective endocarditis, HUS, HIV, HTLV-1 etc.
• Autoimmune: SLE, Henoch-Schonlein (HSP), diabetes mellitus, rheumatoid arthritis (JRA), etc.
• Genetic: Sickle cell disease, Alport syndrome, etc.
• Malignancies: Leukemia, Hodgkin’s lymphoma, etc.
• Medications: Captopril, penicillamine, NSAIDs, anticonvulsants, etc.
3. Congenital nephrotic syndrome: Congenital nephropathy of the Finnish type, Denys-Drash syndrome, etc.
SEE NELSON’S TABLE

PATHOPHYSIOLOGY
The protein loss causes a lot of the problems. Many significant important molecules in the blood are proteins incl. albumin,
enzymes, immunoglobulins etc. So, many problems associated.
1. PROTEINURIA and 2. HYPOALBUMINAEMIA: ***Underlying abnormality is increased permeability of the

glomerular capillary wall --> Massive proteinuria and hypoalbuminaemia
• On biopsy, extensive effacement of podocyte foot processes (the HALLMARK OF IDIOPATHIC NEPHROTIC
SYNDROME)
3. OEDEMA: Massive protein loss --> Hypoalbuminaemia --> disequilibrium of Starling’s forces --> decreased plasma
oncotic pressure --> transudation of fluid from intravascular to interstitial compartment --> oedema (when serum albumin
< 25 g/l)
ALSO:
• Decreased IV volume —> Decreased renal perfusion pressure —> activates RAAS —> stimulates Na and H2O
reabsorption
• Decreased IV volume --> also stimulates ADH --> stimulates water reabsorption in collecting duct
4. HYPERCHOLESTEROLAEMIA AND HYPERTRIGLYCERIDAEMIA:
Serum lipid levels elevated for 3 reasons:

1. Hypoalbuminaemia -> stimulates hepatic protein synthesis with increased alpha-2 macroglobulins AND
LIPOPROTEINS
2. Loss of enzyme lipoprotein lipase in urine
3. Decreased transport of lipids to adipose tissue
5. HYPERCOAGULABLE STATE
Hypercoagulable state -> Thromboembolic events

1. Increased hepatic production of clotting factors and fibrinogen
2. Decreased serum anticoagulation factors
6. INCREASED INFECTIONS
Increased risk of infections (sepsis, peritonitis, pyelonephritis) - especially with encapsulated organisms incl s. pneumonia
and H influenza
1. Urinary loss of complement factor C3b
2. Loss of opsonins
3. Loss of immunoglobulins
4. Immunosuppresive medications (eg. steroids) used to treat nephrotic
IDIOPATHIC NEPHROTIC SYNDROME

Primary glomerular disease without evidence of a systemic cause
Accounts for 90% of nephrotic syndrome in children
Multiple histologic types: Minimal change nephrotic syndrome (MCNS), mesangial proliferation, FSGS, membranous
nephropathy, membranoproliferative glomerulonephritis
• Mediated by immune system modulation. Shown by - use of immunosuppressive drugs controls some causes, MCNS
assc with T-lymphocyte disorders
M>F, 2:1
PATHOLOGY
MCNS - Cause of 85-90% of nephrotic syndrome in children < 6 years (in adolescents accounts for only 20-30% pts
presenting for the 1st time. FSGS more common in this age group)
• Glomeruli appear normal or show a minimal increase in mesangial cells with retraction of podocytes on renal
biopsy
• Present with the features of nephrotic syndrome (above). Also, anorexia, abdominal pain, diarrhoea common
• May have preceding URTI
• ***Very important in MCNS: Renal vein thrombosis is increased in MCNS (60%)
 NO HTN
 NO CCF
 NO GROSS HAEMATURIA
• *** > 95% respond to corticosteroid therapy
FSGS:
• Glomeruli show lesions that are both focal (present only in a proportion of glomeruli) and segmental (localized to
>/= to 1 intraglomerular tuft)
• Segmental scarring
• Only 20% respond to prednisone
• NOTE: Often progressive ultimately involving all glomeruli and ultimately leads to end-stage renal disease in
most patients
HISTORY AND EXAM
***Initial episode usually follows minor infections
Oedema:
• Often first sign; detectable when fluid retention exceeds 3 to 5 percent of body weight
• Starts periorbital and often pretibial -> oedematous areas are white, soft, and pitting
• Gravity dependent: periorbital oedema decreases and pretibial oedema increases over the day
• Anasarca may develop (i.e. marked periorbital and peripheral oedema, ascites, pleural effusions, scrotal/labial
oedema)
Non-specific features common: (e.g. irritability, malaise, fatigue, anorexia, diarrhea)
***Important in MCNS: NO HTN, NO CCF , NO GROSS HAEMATURIA**
DDx of marked oedema: (think all the possible ways to lose protein or decreased protein intake/absorption/production,
then kidney pathology, CCF) Protein-losing enteropathy, hepatic failure (decreased production), heart failure, acute
or chronic glomerulonephritis, protein malnutrition
INVESTIGATIONS
1. Urine
• Urine dipstick (3 to 4+ proteinuria, microscopic hematuria (only 20%))
• Spot protein:creatinine ratio > 2
• Urinary protein:
• > 50 mg/kg/d in 24 hour urine collection
• > 40 mg/m2/hr in 12-24 hour urine collection
2. Bloodwork
 Diagnostic:
 Hypoalbuminaemia (< 20 - 25 g/L)
 Hyperlipidemia/hypercholesterolemia (total cholesterol > 5 mmol/L)
• Secondary:
 Electrolytes (hypocalcaemia, hyperkalemia, hyponatraemia)
 Renal function (BUN and Cr),
 Coagulation profile (PTT)
Appropriate investigations to rule out secondary causes of NS (think of all the differentials in order to remember):
• CBC, blood smear, C3/C4, ANA, HBV/HCV titers, ASOT, HIV serology, VDRL etc.
3. Imaging
 Renal ultrasound
4. Biopsy
ALL ADULTS MUST GET BIOPSY - Dr. Soyibo
BIOPSY CHILDREN IN THE FOLLOWING SETTINGS:
 Renal biopsy in those presenting with atypical features and steroid resistant NS 1. Steroid dependent
2. Relapse after steroid usage
3. HTN present
4. CCF present
TREATMENT
NB: MCGN IS STEROID SENSITIVE...SO IF STEROIDS DO
Establish diagnosis of cause and severity of the NS NT WORK THEN THE PATHOLOGY IS NOT MCGN
A. GENERAL/SYMPTOMATIC
Oedema control:
• If Mild: Salt and fluid restriction, possibly diuretic (avoid if significant intravascular depletion); spironolactone;
may add chlorothiazide.
• If Severe/Anasarca: Loop diuretics (Furosemide) + albumin (25%?) for anasarca. EXTREME CAUTION The Lasix Problem
1. Needs to be filtered
WITH DIURETIC THERAPY. MAY SIGNIFICANTLY INCREASE RISK FOR THROMBOSIS by the body before it
can be effective
2. Bound to alb
Hyperlipidemia: Generally, resolves with remission; limit dietary fat intake; consider Statins therapy if persistently
FIX BY GIVING
nephrotic LASIX-ALB
INFUSION. BEST TO
GO IV ROUTE GIVEN
THAT ORAL ROUTE
Hypoalbuminaemia: IV albumin and Lasix® NOT routinely given; consider if refractory oedema IS LESS EFFECTIVE
DUE TO 1st PASS
EFFECT.
Abnormal BP: Control BP; fluid resuscitation if severe intravascular depletion;
 Acute: Beta or calcium channel blockers
 Chronic: ACE inhibitors or ARBs (not acute cases since ACE inhibitors or ARBs may decrease GFR further)
ACE inhibitors or ARBs for persistent HTN
Diet: NAS (no added salt) diet; monitor caloric intake and supplement with Ca2+ and Vit D if on corticosteroids
ACEi is given to all patients b/c
**Daily weights and blood pressure 4 hrly to assess therapeutic progress it decreases the pressure
gradient across the glomeruli
--> decrease protein
filtration/loss b/c of vasodilation
Secondary infections: of the efferent renal arteriole.
THIS IS DIFFERENT FROM
• Treat with appropriate antimicrobials; antibiotic prophylaxis not recommended BLOOD PRESSURE
MANAGEMENT.
• Pneumococcal vaccine AT diagnosis and
In adults with DM, oral meds
• Varicella vaccine AFTER remission; varicella Ig + will need a switch to insulin as
• Acyclovir if exposed to varicella while on corticosteroids steroid admin will cause
problems with glycemic control.
• NO live vaccines while on immune-modulating agents
Secondary hypercoagulability:
 Prevention of thrombi: mobilize, avoid haemoconcentration due to hypovolaemia, prompt sepsis treatment;
 If thrombi: Give heparin If albumin < 20: Give Heparin
THE 2 MOST COMMON COMPLICATIONS ARE INFECTIONS AND HYPERLIPIDEMIA
B. SPECIFIC (LECTURE)
Minimal change disease:

Induction of remission:
• Oral prednisone 2 mg/kg/d (or equivalent) for 6 weeks as a single dose or divided dose(or until remission for relapses) -
varicella status should be known before starting
Maintenance of remission:
• Prednisone 2 mg/kg/d as single dose, alternate days for 6 weeks; then taper over 2-3 months
NB: Tapering off steroids is due to the duration. This is done when steroids have been given over a 2-week period, as in the
case of NS treatment. Always give Vit D and calcium to
those on longterm steroid usage.
Longterm steroids can cause bone
If no remission has occurred after 4 weeks the patient is steroid resistant and should have renal biopsy loss as a S/E --> osteoperosis.
NB: Patients can be: Steroid Sensitive, Steroid Dependent, Steroid Resistant
If there is Steroid resistance = No remission with high-dose daily prednisone after 28 days: Alternatives can be used
STEROID RESISTANCE AND
• Consider cytotoxic agents, immunomodulators or high-dose pulse corticosteroid if steroid resistant STEROID SPEARING regimes.
• Cyclophosphamide This is given in the morning. The break down products that are inactive can cause hemorrhagic cystitis. These products build up if not
voided. Stasis occurs in the night --> build up. Steroid spearing allows less use
of steroids and decrease the S/E
• Cyclosporine This is nephrotoxic of steroids.
• Tacrolimus Dopamine is also used to
• Mycophenolate mofetil increase flow to the kidney
Monitoring MCNS progress (Lecture)

MUST KNOW:
• Remission = trace/negative proteinuria for 3-5 consecutive days
• Relapse = proteinuria > 2+ (cloudy urine on SSA) for 3-5 consecutive days or > 2+ proteinuria with oedema
COMPLICATIONS
• Increased risk of infections (spontaneous peritonitis (strep. pneumoniae most common), cellulitis, sepsis) due to
loss of immunoglobins and C3b
• Acute renal failure

• Hypovolemia due to diarrhea and diuretic use
• Anemia due to loss of transferrin. This is seen in chronic cases.
• Hypercoagulability due to decreased intravascular volume, increased coagulation entities [fibrinogen,

factor V and factor VIII] and antithrombin III depletion  (pulmonary embolism, renal vein thrombosis;
intravascular depletion-hypotension, shock, renal failure; side effects of drugs)
• Complications of steroid therapy – Put in place by Anggelos

1. CNS – Sleep disturbances, Mood swings, Behavioral changes Anxiety, Depression, Euphoria, Psychosis
2. EYES – Blurred vision, Retinopathy, Glaucoma, Cataract
3. ORAL - Thrush
4. RESPI - Hoarseness
5. CARDIO – HTN (due to Na retention), Edema
6. GASTRO – Decrease appetite, Vomiting, Peptic ulcers, Hematemesis, Colitis
7. MSK – Muscle weakness
8. BONES – Osteopenia, Osteoporosis,
9. SKIN – Hyper/Hypo-pigmentation, Rash, Acne, Easy bruising, Increase hair
10. IMMUN – Decrease immunity
11. METABOLIC – Muscle wasting, Moon faces and Buffalo hump (due to movement of fat from body to
torso and face), Weight gain
12. ENDOCRINE - Hyperglycemia, Insulin resistance, DM
13. PREGNANCY – Teratogenic effect
Paediatrics
Pneumonia Notes
Source: Nelson’s (Ch 392), Oxford
September 2014
DEFINITION
• Inflammation of the lower respiratory tract and lung parenchyma
INCIDENCE
• WHO 2004: Pneumonia is the leading killer of children < 5 yrs worldwide
NOTE:
o Bronchiolitis peaks in first year of life
o Viral pneumonia peaks between age 2-3
AETIOLOGY
[CLASSIFY: Infectious (Viral/Bacterial/TB) vs. Noninfectious (Aspiration/Drugs/Radiation))
MUST KNOW: VERY IMPORTANT TABLE (FROM NELSON’S)
Table 392- 3 ETIOLOGIC AGENTS GROUPED BY AGE OF THE PATIENT

AGE GROUP FREQUENT PATHOGENS (IN ORDER OF FREQUENCY)
Neonates (<3 wk) Group B streptococcus, Escherichia coli, other gram-

negative bacilli, Streptococcus pneumoniae, Haemophilus
influenzae (type b * nontypable)
3 wk-3 mo Respiratory syncytial virus, other respiratory viruses
(parainfluenza viruses, influenza viruses, adenovirus),
S. pneumoniae, H. influenzae (type b * nontypable); if
patient is afebrile, consider Chlamydia trachomatis
4 mo-4 yr Respiratory syncytial virus, other respiratory viruses
(parainfluenza viruses, influenza viruses, adenovirus),
S. pneumoniae, H. influenzae (type b,* nontypable),
Mycoplasma pneumoniae, group A streptococcus
>5 yr M. pneumoniae, S. pneumoniae, Chlamydophila
pneumoniae, H. influenzae (type b,* nontypable), influenza
viruses, adenovirus, other respiratory viruses, Legionella
pneumophila
* H. influenzae type b is uncommon with routine H. influenzae type b immunization.
Oxford:
• Neonates: Group B streptococcus, Escherichia coli, Klebsiella, Staphylococcus aureus
• Infants: Streptococcus pneumoniae, Chlamydia trachomatis
• School age: Streptococcus pneumoniae, Staphylococcus aureus, group A streptococcus, Bordetella pertussis,
Mycoplasma pneumoniae.
Noninfectious causes:
• Aspiration of food, gastric acid, foreign bodies
• Hypersensitivity reactions
• Drug or radiation induced pneumonitis
• Children with HIV: M Tuberculosis, pneumocystis jiroveci (PCP/PJP), atypical mycobacterium, Salmonella,
E coli
• Cystic fibrosis: Pseudomonas
Viral pathogens are a common cause of LRTIs in infants and children < 5 yrs old. Influenza virus and RSV most
common. (To remember: So basically the same viral organisms that cause bronchiolitis)
• Others: Parainfluenza virus, adenovirus, rhinovirus, human metapneumovirus
PATHOGENESIS
Viral pneumonia:
• Direct injury of resp epithelium -> airway obstruction from swelling, abnormal secretions, cellular debris.
• Small caliber of airways in young infants makes them susceptible to severe infection
• Atelectasis, interstitial oedema, ventilation-perfusion mismatch causing significant hypoxemia often accompany
airway obstruction.
VIRAL INFECTION OF THE LRT CAN PREDISPOSE TO SECONDARY BACTERIAL INFECTION
Bacterial pneumonia:
• Occurs when respiratory tract organisms colonize the trachea and subsequently gain access to the lungs. May also
result from direct seeding of lung tissue after bacteremia
Recurrent pneumonia: is defined as 2 or more episodes in a single year OR 3 or more episodes ever. Must consider an
underlying disorder
NOTE WELL: PNEUMONIAS OFTEN PRECEDED BY SYMPTOMS OF A URTI

NOTE WELL: CLINICAL MANIFESTATION IS AGE DEPENDENT
Viral pneumonia:
• Fever usually present
o LOWER TEMPERATURES than bacterial pneumonia
• RESPIRATORY DISTRESS manifested by:

o SOB
o Tachypnoea is the most consistent clinical manifestation of pneumonia
o Grunting
o Nasal flaring
o IC, SC, suprasternal recession
o Accessory muscle use – look up accessory muscles
• Severe infection -> Cyanosis

• Pulse oximetry should be performed on every child admitted to hospital with pneumonia. SpO2 ≤92% in
room air indicates severe illness.
• Auscultation: +/- crackles and wheeze
Bacterial pneumonia:
• Typically begins suddenly with shaking chills and then high fever, cough (> 7 y.o produce sputum), chest pain
• Other symptoms: Tiredness, restlessness, tachypnoea, anxiety, circumoral cyanosis

o +/- splinting on affected side to minimize pleuritic chest pain and improve ventilation
• Examination:
o Diminished breath sounds
o Crackles
o Wheeze
• Increasing consolidation OR complication such as effusion, empyema, pyothorax ->

o Dull percussion, increased TVF and VR
ALSO:
• Abdominal distension -> swallowed air OR ileus
• **Liver may seem enlarged -> Reason: downward displacement of the diaphragm secondary to hyperinflation
of lungs
INFANTS:
• URTI prodrome symptoms, diminished appetite, abrupt onset of fever, restlessness, respiratory distress
• Appear ill
• Tachypnoea, resting respiratory rate of 70 breaths/min in infants or > 50 breaths/min in children indicates
severe illness
• Tachycardia
• Nasal flaring
• IC, SC, suprasternal recession
• *** Infants may have associated GI disturbances!!: Vomiting, anorexia, diarrhoea, and abdominal distension
2nd to ileus
DIAGNOSIS
Oxford
1. CBC: WBC count WITH differential: May be useful in differentiating viral from bacterial pneumonia (See below)
2. Sputum: Culture may be of limited value depending on the age of the child.
3. Nasopharyngeal aspirate: Viral immunofluorescence in infants.
4. Blood: Culture should be done in all children with severe bacterial pneumonia (not necessary in community-acquired pneumonia).
5. CXR: Not routine
6. Pleural fluid: When there is a significant pleural effusion, an aspirated sample should be sent for culture and antigen testing once a drain is
inserted.
Chest X-ray:
• In general, routine CXR is NOT needed in children with mild uncomplicated LRTI
• An infiltrate on CXR supports the diagnosis of pneumonia

• CXR may also indicate a complication: Eg. Pleural effusion, empyema
o **Viral pneumonia: Usually characterized by hyperinflation with BILATERAL INTERSTITIAL infiltrates

and peribronchial cuffing
o **Bacterial pneumonia: Confluent LOBAR consolidation is typically seen with pneumococcal/bacterial
pneumonia
• CXR alone is NOT diagnostic – must consider other clinical features
WBC count AND differential:

May be useful in differentiating viral from bacterial pneumonia
• Viral: WBC may be normal or elevated but usually not over 20, 000/mm3 with LYMPHOCYTE predominance
• Bacterial: Often has elevated WBC between 15 – 40,000 and GRANULOCYTE predominance
NOTE: Large pleural effusion, lobar consolidation and high fever at onset *SUGGESTS* bacterial (not confirms)
Atypical pneumonia: Due to chlamydia pneumonia or mycoplasma pneumoniae

• Pattern on x-ray cannot confirm whether viral or bacterial
• **Definitive diagnosis of viral infection requires isolation of the virus (viral cultures) or detection of the viral
genome (DNA or RNA PCR) or antigen (Serology e.g. ELISA) in respiratory tract secretions
• Definitive diagnosis of bacterial infection requires isolation of organism in blood, pleural fluid or lung.
• Sputum culture of little value in dx in young children
• Blood culture positive in only 10% of children with pneumococcal pneumonia
TREATMENT
[Classify: Supportive and Specific]
Supportive: (From Oxford)

Consider whether any of the following are needed:
• Antipyretics for fever.
• IV fluids: Consider if dehydrated or not drinking.
• Supplemental oxygen: Administer oxygen via headbox or nasal cannulae so that Sp02 is maintained >92%
• Chest drain: for fluid or pus collections in the chest, as in empyema.
Specific: [Classify: (Suspected) Bacterial vs. Viral]
A. BACTERIAL PNEUMONIA
Treatment of suspected bacterial pneumonia based on presumptive cause, AGE and CLINICAL APPEARANCE of the
child
• Mildly ill who do not require hospitalization :

• Amoxicillin recommended (First-line)
o Alternatives include cefuroxime and Augmentin
• ***School-aged children suspected to have mycoplasma or chlamydial -> Macrolide e.g. Azithromycin!!!
• Adolescents -> May consider a fluoroquinolone instead
• Empiric treatment in a patient who requires hospitalization:
• IV cefotaxime or ceftriaxone is the MAINSTAY of therapy when bacterial pneumonia is suggested

• IF clinical features suggest staph pneumonia (Eg. pneumatoceles, empyema) -> Include vancomycin or
clindamycin in initial therapy
• ***For pneumococcal pneumonia, antibiotics should be continued until patient is afebrile for 72 hours and the
total duration should not be less than 10-14 days (5 if azithromycin used)
• By age (From Oxford)

i. Under 5 yrs
Streptococcus pneumoniae is the most likely pathogen. The causes of atypical pneumonia are Mycoplasma
pneumoniae and Chlamydia trachomatis
• First-line treatment: Amoxicillin
• Alternatives: Co-amoxiclav or cefaclor for typical pneumonia; erythromycin, clarithromycin, or
azithromycin for atypical
Pneumonia
ii. Over 5 yrs

Mycoplasma pneumoniae is more common in this age group
• First-line treatment: Amoxicillin is effective against the majority of pathogens, but consider macrolide
antibiotics if mycoplasma or
chlamydia is suspected
• Alternatives: If Staphylococcus aureus is suspected consider using a macrolide, or a combination of flucloxacillin
with amoxicillin
B. VIRAL PNEUMONIA suspected
• May withhold antibiotic therapy, esp. in those who are mildly ill and in no resp distress
• Up to 30% with known viral infection may have coexisting bacterial pathogens. Clinical deterioration should
signal the possibility of a bacterial infection and appropriate antibiotics started
KNOW THESE: INDICATIONS FOR ADMISSION:
• Age < 6 months

• Sickle cell anaemia with acute chest syndrome
• Multiple lobe involvement
• Immunocompromised
• Toxic appearance
• Moderate to severe respiratory distress
• Requirement for supplemental oxygen
• Dehydration
• Vomiting or inability to tolerate oral fluids or meds

• No response to oral antibiotic therapy
11 . Social factors (Inability of caregiver to administer medication at home or follow up appropriately)
PROGNOSIS
• Radiographic evidence of improvement lags substantially behind clinical improvement
• In general, a repeat CXR is the 1st step in determining the reason for a delay in response to treatment
COMPLICATIONS DIFFERENTIAL DIAGNOSIS

1.Acute pulmonary diseases
a.Allergic pneumonitis
Usually the result of direct spread of bacterial infection within the thoracic cavity: b.Asthma
c.Cystic fibrosis
• Pleural effusion d.Acute chest (Sickle cell)
• Empyema
2.Cardiac disease
• Pericarditis a.Pulmonary edema secondary to heart failure
3.Autoimmune diseases
OR bacteraemia and haematogenous spread a.SLE
b.Vasculitides
Rare complications of pneumococcal or HiB infection:
• Meningitis
• Suppurative arthritis
• Osteomyelitis
**S. aureus, S pneumoniae and S pyogenes are the most common causes of parapneumonic effusions and empyema
Treatment of EMPYEMA - Mainstays include antibiotic therapy and drainage with a chest tube
• Urokinase for empyema
• In empyema, as opposed to simple pleural effusion, instillation of urokinase via the chest drain is recommended.
Surgical referral
• If the effusion or empyema fails to resolve over a period of 7 days then a surgical opinion may be sought. Sometimes a chest
CT scan is needed
Paediatrics
Proteinuria Short Notes
Source: Lecture
October 2014
CLASSIFICATIONS
***Emphasis placed on “Non-pathologic (Non-nephrotic) vs. Pathologic (Nephrotic)”
Non-pathologic (Non-nephrotic) Pathologic (Nephrotic)

• Transient/Intermittent • 1o or 2o Nephrotic syndrome
• False +ve • Glomerular nephritides
• Contamination eg. Vaginal secretions • HUS
• Exercise • Failed tubular reabsorption
• Fever • Inflammation of interstitium (interstitial
• Orthostatic proteinuria (postural proteinuria) nephritis)
• UTI • HTN
• CCF • DM
**Associated haematuria = always pathological

**Nephrotic-range proteinuria = always
pathological
Orthostatic proteinuria (postural proteinuria): This is a common cause of referral in older children. There is usually no history of
significance and a normal examination. Investigations reveal a normal UP:UCr ratio in early morning urine with elevated level
in afternoon specimen (may require two 12hr collections). This is regarded as a benign finding and requires no treatment.
One useful classification:

• Pre-glomerular – Increased filtration
• Glomerular – Increased glomerular permeability
• Post-glomerular – Tubular and interstitial
Others: Transient vs. Persistent vs. Intermittent (orthostatic)
IMPORTANT INVESTIGATIONS
Qualitative vs. Semi-quantitative vs. Quantitative
QUALITATIVE
1. Urine dipstick:
• False +ve: Concentrated urine, alkaline urine, contamination with chlorhexidine
• False –ve: Dilute urine
2. 3% Sulphosalicylic acid (SSA)
• False +ve: Conc. Urine, penicillin, cephalosporins
• False –ve: Dilute urine
SEMI-QUANTITATIVE
• Spot urine protein:creatinine ratio

o Just remember 0.2 and 2
o =/< 0.2 = Normal
o 0.2 – 2 = Minimal-mod proteinuria
o > 2 = Nephrotic-range proteinuria
QUANTITATIVE
• 12-24 hour urine collection (for urinary protein excretion)

• This is the gold standard test and requires a 24hr collection of urine to estimate urinary protein excretion.
**Interpretation of results. Nephrotic-range proteinuria =

1. >50 mg/kg/d in 24 hour urine collection
2. > 40 mg/m2/hr in 12-24 hour urine collection
3. First morning protein:creatinine ratio > 2
4. 4+ proteinuria on at least 5 consecutive urines
Look up additional investigations:

• They are in the lecture. Same as those for investigating NEPHRITIC and NEPHROTIC syndrome
**Get a differential diagnosis for proteinuria from a summary source and put it here**
(All the causes of nephritic and nephrotic syndrome and some of the causes of haematuria)
**MAY USE THE TABLE ON PAGE 1 ABOVE!!**
Paediatrics
Rashes and Paediatric Exanthems
Source: Nelson’s Essentials, Toronto 2014, Images from Paediatrics at a Glance
September 2014
NOTE: EXANTHEMS COVERED FIRST. GOOD IMAGES AND OTHER RASHES START AT PAGE 9!!!!
• Exanthem: An eruption on the skin occurring as a symptom of a systemic disease typically with a fever
o An exanthem is a rash that ‘bursts forth or blooms’ towards the end of incubating an infection. The 6
classic exanthemata are characteristically:
§ Widespread, symmetrically distributed on the body;
§ Red, discrete, or confluent macules or papules.
• Enanthem: An eruption on a mucous membrane occurring in the context of an exanthem
TORONTO NOTES (CAN SKIP THIS AND GO STRAIGHT TO PAGE 3)

Table 25. Common Pediatric Exanthems
Exanthem Etiology Clinical Description Important Complications Management
Chicken Pox HHV3 Diffuse itchy vesicular pustular eruption Secondary infection, necrotizing fasciitis, Supportive treatment, acyclovir or
Incubation 10-21 d, beginning on thorax spreading to extremities meningitis, encephalitis, cerebellar valacyclovir, IV if severe; if severe,
Communicable 1-2 d New lesions every 2-3 d ( multiple stage ataxia, pneumonitis, disseminated Varicella Zoster immunoglobulin
pre-rash to 5 d post-rash eruption: macule-papule-vesicle-cmst ) intravascular coagulation ( DIC), hepatitis ( within 96 h of contact ), Varicella
Enanthems vaccine
Enteroviral Enteroviruses Polymorphous rash (macules, papules, None Supportive treatment for majority
Most common exanthem in vesicles, petechiae, urticaria) Serious cases ( immunosuppressed)
summer and fall can be treated with pleconaril
Erythema Parvovirus B19 Slapped cheeks ( red, flushed cheeks) then STAR complex (Sore Throat, Arthritis, No treatment: children often feel well
Infectiosum Incubation 4-14 d 1-4 d later lacy/reticular maculo -papular rash Rash) NSAIDs for symptomatic arthropathy
Peaks in winter and spring of trunk/extremities Fetal infection ( anemia, fetal hydrops
Glove and stocking purpura or death)
Aplastic crisis in sickle cell patients
Exanthem Etiology Clinical Description Important Complications Management

Gianotti-Crosti Epstein-Barr virus most Symmetric papular eruption of face, None Supportive treatment
Syndrome common, hepatitis B, buttocks, and extremities
Coxsackie, Parvovirus Sparing of trunk
Spring and early summer Preceded by viral prodrome
Hand, Foot and Coxsackie A and B viruses Vesicular eruption of palms and soles with Pulmonary, dehydration, neurological Supportive treatment
Mouth Disease Highly contagious vims an erosive stomatitis death
Enanthem: vesicles involving tongue and
posterior pharynx
Kawasaki Disease Unknown etiology - Fever >5 d and 4/5: Most common cause of vasculitis ASA, IVIG, baseline echo and repeat
infectious etiology unilateral lymphadenopathy; puffy/red palms and acquired heart disease in children in 6 wk
suggested and soles; red, cracked lips/strawberry (coronary artery aneurysm)
tongue; skin rash; non-purulent bilateral
Late winter to early spring CNS, Gl tract, kidney, eyes
conjunctivitis
Measles Paramyxovirus Morbiliform rash starts at hairline and Otitis media, pneumonia, Vitamin A, immunoglobulin, MMR
Incubation 8-13 d spreads down to face/neck/trunk, encephalitis, SJS, glomerular nephritis, vaccine
Communicable 4 d pre-rash desquamates (no palm or myocarditis/pericarditis
and post-rash sole involvement )
Prodrome: cough, coryza, conjunctivitis
( 3 Cs)
Enanthem: Koplik spots ( grey/white papules
on buccal mucosa)
Meningococcemia Neisseria meningitidis Purpuric and petechial rash ("stellate purpura Hearing loss, intellectual disability, 5-7 d course of 3rd generation
with a central gunmetal- grey hue") necrosis and loss of digits and/or limbs, cephalosporin
septic shock, death
Roseola HHV 6, HHV 7 Pink macules and papules Febrile seizures, neurological Supportive treatment
Incubation 5- 15 d on neck/arms/trunk ± face involvement Antipyretics during the
Eruption after high fever ends Viral reactivation in febrile period
Posterior cervical nodes immunosuppressed
Enanthem: Nagayama sign ( red papules on patients
soft palate)
Rubella RNA virus of the 1 - 5 d following mild prodrome (fever, STAR complex Supportive treatment
Togaviridae family headache, respiratory symptoms ), a pink Congenital rubella (cataract, glaucoma, MMR vaccine
Incubation 14-21 d maculopapular rash erupts on face spreading thrombocytopenia, hepatitis, deafness, Serologic testing in rubella- exposed
Communicable 7 d pre-rash down to neck/trunk congenital heart disease) pregnant women
and post-rash Occipital and retroauricular nodes
Scarlet Fever GAS toxin types Generalized rash, red papules, "sand-paper " Mastoiditis, otitis, sinusitis, pneumonia, 10-14 d course of penicillin
A, B, and C texture, desquamation ( palms and soles), meningitis, myocarditis, arthritis,
Late fall, winter, and flexural accentuation (Pastia's lines ) hepatitis, rheumatic fever, and
early spring Enanthem: strawberry tongue, petechiae glomerulonephritis
on palate
OXFORD HANDBOOK OF PAEDIATRICS

Box 19.7 The six exanthemata
First associated disease: measles virus
• Incubation: 8 12 days
-
• Duration: 6 8 days
-
• Infectivity: from 4 days before to 4 days after the rash appears

• Rash: maculopapular, starts on the face and spreads
• Features: prodromal upper respiratory symptoms and cough, Koplik
spots on the oral mucosa rarely seen
•Treatment supportive, contact public health
Second associated disease: group A streptococcus
• Incubation: 2-5 days
• Duration: 7 days
• Infectivity: can become colonized
• Rash: fine papular rash on flushed skin, sandpaper texture
• Features: sore throat, strawberry tongue, rash, lymphadenopathy
•Treatment penicillin V , consider need for prophylaxis only in family
where infant less than 1mth of age or multiple family members
affected
Third associated disease: rubella
• Incubation: 14-21 days
• Duration: 2-3 days
• Infectivity: from 7 days before to 7 days after the rash
• Rash: maculopapular, rapidly spreads and fades
• Features: enlarged lymph nodes at the back of the neck and ears
•Treatment supportive
Fourth associated disease: possible coxsackie virus
No longer recognized as a disease entity
Fifth associated disease: parvovirus
• Incubation: 4 14 days
-
• Duration: 3 7 days prodrome then rash for 1

- 4 days; then
-
evanescent rash over 1 3wks followed by arthropathy

-
• Infectivity: no isolation needed for children

• Rash: slapped cheek appearance, erythema infectiosum
Sixth associated disease: human herpes virus 6
• Incubation: 10 days
• Duration: 3 7 days
-
• Infectivity: unclear
• Rash: maculopapular
• Features: temperature for 3 days then, as the rash appears, the
temperature falls rapidly
EXANTHEM 1: MEASLES (RUBEOLA)
CAUSATIVE AGENT: Paramyxovirus (ssRNA)
IP: 8-12 days
INFECTIVITY: 4 days pre-rash - 4 days post-rash (or from 1-2 days before onset of any symptoms).
DROPLETS OR AIRBORNE ROUTE. Highly contagious
ONSET OF RASH: 14 days after infection (Range 7-21)
CLINICAL FEATURES
4 PHASES
[Virus infects the upper respiratory tract and is spread first in a brief low-titre primary viraemia]
1. Incubation: 8-12 days (from EXPOSURE to symptom onset)
2. Prodromal (catarrhal): Lasts 3 days The conjunctivitis is PURULENT vs that in Kawaski which is not
1. Consists of: Cough, Coryza, Conjunctivitis (The 3 C’s)
2. AND the pathognomonic Koplik spots (gray-white, sand-grain sized dots on the buccal mucosa
opposite the lower molars – lasts only 12-24 hours!!)
3. Conjunctiva – May reveal Stimson’s line – characteristic transverse line of inflammation along eyelid
margin
3. Exanthematous phase (rash): The classic symptoms of cough, coryza and conjunctivitis occur in the secondary
viraemia (5-7 days after first infection). Often also HIGH fever (peaks when rash appears - 40-40.5 or 104-105)
above/along hair line and behind ears
• *Maculopapular rash – begins on head – above hairline & behind ears, spreads over body in a
cephalad to caudal pattern over 24 hours (face -> neck -> trunk). NO palm or sole involvement!
• Starts 14 days after exposure
• Lasts 6-8 days
• Areas of rash become confluent/coalesce
• Rash fades in the same pattern. i.e. from cepahlad to caudal
• May be petechial or haemorrhagic (black measles)
• As it fades -> brownish discolouration and desquamation
Other features: Cervical lymphadenitis, mesenteric lymphadenopathy, anorexia, diarrhoea, abdominal

•
Cervical lymphadenitis is mostly in the posterior cervical region
pain.
• Otitis media, pneumonia and diarrhoea more common in infants
• Liver involvement in adults
4. Recovery
INVESTIGATIONS
• Clinical: Koplik spots are pathognomonic, but not always seen. (only last for 12-24 hours)
• CBC + Differential: Leucopaenia and lymphopaenia are characteristic!!
• LFTs: Raised transaminases.
• PCR of Oral fluid test: measles RNA on oral fluid specimen confirms the diagnosis.
• Confirmation: Serum serology of ACUTE AND CONVALESCENT samples may also be used.
• In encephalitis, CSF: Increased protein, normal glucose, lymphocytic pleocytosis
MANAGEMENT:
• GENERALLY SUPPORTIVE – Adequate hydration and antipyretics
• WHO recommends high-dose Vitamin A supplementation for 2 days in developing countries vitamin A deficiency and
malnutrition lead to a protracted course of illness with severe complications
• Prevention: MMR vaccine at 12–18 mths and preschool booster to all children and at 4-6 years. (MMRV also has varicella)
COMPLICATIONS
• Otitis media = most common complication (10%)
• Interstitial measles pneumonia from secondary bacterial infection – S. pneum, S. aureus or GAS
• Encephalitis (1 in 5000): occurs ~8 days after the onset of illness and starts with headache, lethargy, irritability,
followed by seizures and coma. Mortality is high and there are neurological sequelae in survivors.
• PAST QUESTION!: Subacute Sclerosing Panencephalitis (SSPE, 1/10 000). A rare and fatal neurological
disease with progressive intellectual deterioration, ataxia, and seizures about 7-10 years after measles infection.
• Others: Myocarditis
• Death most frequently from bronchopneumonia or encephalitis
EXANTHEM 2: SCARLET FEVER

GROUP A STREPTOCOCCUS
Scarlet fever is an erythematous rash that may occur with streptococcal pharyngitis. Other patterns of infection caused by
Group A streptococcus (GAS) include toxic shock syndrome and necrotizing fasciitis.
IP: 2 – 5 days
INFECTIVITY: Spread by respiratory secretions and droplets or by self-infection from nasal carriage.
ONSET OF RASH:
CLINICAL FEATURES
• Prodrome: During the incubation period the child may have fever, vomiting, and abdominal pain.
• Exanthematous phase: ‘sandpaper-like’ diffuse rash in the neck and chest area (with perioral pallor)
spreading to the flexor creases (Pastia’s lines).
o The pharynx is erythematous and there may be exudative tonsillitis, uvular oedema, and
o ENANTHEM: Strawberry tongue, palatal petechiae
• Other features: Tender anterior cervical lymphadenopathy.
INVESTIGATIONS
• Throat swab: Culture and growth of the organism in a symptomatic individual (note also asymptomatic carriage
common)
• Serum: Antistreptolysin O (ASO) and anti-DNase B titres – one or both may rise in acute infection.
MANAGEMENT
• Antibiotics: Penicillin V for 10-14 days. This will **prevent the development of rheumatic fever (BUT
not glomerulonephritis) and may reduce the length of illness. Antibiotics should be started within 9 days of
acute illness.
• Isolation: Children should be isolated until 24hr after the start of antibiotics!!!.
Complications
• Peritonsillar abscess, retropharyngeal abscess

• Post-streptococcal glomerulonephritis (PSGN)
• Rheumatic fever
EXANTHEM 3: RUBELLA
Also known as German measles or 3-day measles
CAUSATIVE AGENT: ssRNA virus of the Togavirus family

IP: 14-21 days
INFECTIVITY: From 7 days pre-rash to 7-14 days post-rash (still found in NP secretions in that
period). Most contagious through direct or droplet contact with nasopharyngeal secretions in that period.
• **Infants with congenital rubella syndrome (CRS) may shed the virus in NP secretions for > 12 months after
birth and transmit in this time
ONSET OF RASH:
CLINICAL FEATURES
Has a primary viraemia after invading respiratory epithelium -> replicates in reticuloendothelial system -> secondary
viraemia
NB: Infection in utero results in significant morbidity from congenital rubella syndrome.
Maternal infection in the first trimester results in fetal infection with generalized vasculitis in > 90% of cases
• Rubella is a MILD disease (But congenital rubella syndrome may be severe)

• Transplacental antibody is protective in the first 6 months of life
--
• Prodrome: During IP, the child may have a mild illness with low-grade fever + occular pain + sore throat.
• Exanthematous phase: Maculopapular rash starting on the face, then spreading to cover the whole body
• Lasting up to 3 days (Oxford says 5)
• Other characteristic features: Suboccipital, post-auricular/retroauricular and posterior cervical

lymphadenopathy.
• Forchheimer spots: Rose-coloured spots on the soft palate – 20% of patients. May develop before
the rash
• General: +/- Mild pharyngitis, conjunctivitis, anorexia, headache, malaise, low-grade fever, polyarthritis of hands
INVESTIGATIONS
• Routine labs generally do not help with diagnosis

o WBC count: Usually normal or low
o Thrombocytopaenia rare
• Confirm diagnosis with serology: for IgM antibodies (typically positive 5 days after symptom onset)
• OR 4-fold or greater increase in IgG antibodies in ACUTE and CONVALESCENT samples
TREATMENT
• SUPPORTIVE – Adequate hydration and antipyretics

• THERE IS NO SPECIFIC THERAPY FOR RUBELLA
COMPLICATIONS
• Other than with CRS, complications from rubella are very rare
EXANTHEM 4: ENTEROVIRUSES
The majority of infections due to human enteroviruses (coxsackie viruses, echoviruses, and polio viruses) produce non-
specific illness. They are the most common cause of exanthems in the summer months. Over 68 types identified.
EXANTHEM 5: ERYTHEMA INFECTIOSUM

Aka. “Fifth disease”
CAUSATIVE AGENT: Parvovirus B19 (ssDNA)

IP: 4-14 days (rarely up to 21)
INFECTIVITY: Transmitted by resp secretions and droplets and blood product transfusions.
ONSET OF RASH: Sometimes 7-10 days after prodrome onset
GENERAL INFO:
NOTE: The viral affinity for red blood cell progenitor cells makes it an important cause of aplastic crisis in patients with haemolytic anaemias (SCD,
spherocytosis, thalassaemias). Also causes fetal anaemia and hydrops fetalis after primary infection during pregnancy. Cell receptor for
Parvovirus B19 is the erythrocyte P antigen. Virus replicates rapidly in actively dividing erythroid stem cells (erythroblastoid precursors) ->
cell death -> erythroid aplasia and anaemia.
• Usually causes pure red-cell aplasia.
• Erythema infectiosum is common
CLINICAL FEATURES
• Prodrome: INFECTIOUS PHASE Absent/mild. Low-grade fever, headache, & coryza 7 days after exposure. .
• Exanthematous phase: NON-INFECTIOUS PHASE
• ***The rash appears in 3 stages***:
1. “Slapped cheek” rash of face with circumoral pallor
2. Erythematous, symmetric, Maculopapular, truncal rash appears 1-4 days later then fades as central
clearing takes place giving àà
3. A distinctive lacy, reticulated rash that lasts 2-40 days! (usually 11 days)
o So it overall lasts at least 15 days

o May be pruritic
o Spares palms and soles
o Does NOT desquamate
o Worsen with exercise, heat, stress
• Other features: other patterns of illness include asymptomatic infection, aplastic crisis, +/- myalgias, significant
arthralgias/arthritis, GI upset
o Fetal hydrops (from maternal infection).
INVESTIGATIONS
• Clinical diagnosis from characteristic rash

• CBC: +/- Reticulocytopaenia lasting 7-10 days, mild anaemia, thrombocytopaenia, lymphopaenia,
neutropaenia (According to Nelson’s)
• PCR of erythroid precursors in bone-marrow
• Serology: IgM antibody to parvovirus
MANAGEMENT
• SUPPORTIVE – Hydration and antipyretics
• NO SPECIFIC THERAPY!
• Transfusions may be required for transient aplastic crisis
EXANTHEM 6: ROSEOLA INFANTUM
Aka. “Sixth disease”, “Exanthem subitum”
CAUSATIVE AGENT: Human Herpes Virus 6 (HHV-6) mainly. Also HHV-7 in 10-30% (both dsDNA)
IP: 4-14 days (rarely up to 21)
INFECTIVITY:
ONSET OF RASH:
GENERAL INFO:
Can be detected in saliva of HEALTHY adults which suggests as with other herpesviruses, LIFELONG LATENT INFECTION
By 12 months of age, approximately 60-90% of children have antibodies to HHV-6 and essentially ALL CHILDREN ARE SEROPOSITIVE BY AGE 2-3
YEARS
HHV-6 is a major cause of acute febrile illnesses in infants and may be responsible for 20% of visits to emergency dept in children 6-18 months
CLINICAL FEATURES
• Prodrome: High-spiking fever (> 40oC), with abrupt onset lasting 3-5 days.
*** The fever classically stops once the rash appears!!!!!! ***
• Exanthematous phase: Rose-coloured maculopapular rash erupts with defervescence (abatement of a

fever), beginning on the trunk and spreading peripherally.
o It lasts for 1–3 days. BUT may fade rapidly and is NOT ALWAYS present.
• Other features: Upper respiratory symptoms, nasal congestion, erythematous tympanic membranes, cough.
o Also vomiting, diarrhoea, pharyngeal injection without exudates, cervical lymphadenopathy, and febrile
convulsions prior to rash (5–10% of cases).
o ***Roseola is associated with ~1/3 of febrile seizures (From Nelson’s, so American number)
INVESTIGATIONS
Routine labs DO NOT help diagnosis

Diagnosis is clinical. Typical history.
• CBC: May have neutropaenia

• Serology: 4-fold or greater increase in ACUTE and CONVALESCENT sera
• PCR of CSF for HHV-6 DNA is diagnostic
MANAGEMENT
• SUPPORTIVE – Adequate hydration and antipyretics

• NO SPECIFIC THERAPY (Don’t just jump and put acyclovir. Wrong)
• Gancyclovir considered in immunocompromised
COMPLICATIONS
Most common complication is febrile convulsion

• Roseola is associated with ~1/3 of febrile seizures
• Rare: Few deaths with HHV-6. Attributed to encephalitis or haemophagocytosis syndrome
VARICELLA-ZOSTER VIRUS INFECTION
CHICKENPOX AND HERPES ZOSTER
CAUSATIVE AGENT: Varicella-zoster virus (VZV) aka. HHV-3 (dsDNA – member of herpesvirus family)
IP: 14-16 days (Range: 10-21)
INFECTIVITY: 2 days pre-rash to 7 days after the last vesicle crusts off
Transmission via direct contact with lesions, droplet and air
ONSET OF RASH:
GENERAL INFO:
• Chickenpox is the manifestation of PRIMARY infection
• Infects via conjunctivae or respiratory tract and replicates in URT (nostril, nasal cavity, nsoharynx, phrynx, larynx).
• Disseminated via a primary viraemia to LNs, liver, spleen and other organs
• Secondary viraemia follows resulting in the cutaneous infection with the typical vesicular rash
• After resolution of chickenpox, the VIRUS PERSISTS AS LATENT INFECTION IN THE DORSAL ROOT GANGLIA
CELLS
• Zoster (Shingles) is the manifestation of reactivated latent infection of VZV
• 75% of zoster occurs after age 45 years. But can occur in childhood (eg. Immunocompromised). Incidence increased in
immunocompromised especially in childhood
CLINICAL FEATURES
• Prodrome: Fever, malaise, anorexia. May precede rash by 1 day.

• Rash: Characteristic rash appears initially as small red papules that rapidly progress to NONUMBILICATED,
OVAL, “TEARDROP” VESICLES on an erythematous base
o STARTS ON HEAD AND TRUNK THEN REST OF BODY
o ***Stages: Macule à Papule à Vesicles à Pustule à Ulcerate à Crust and heal
o New crops appear for 3-4 days usually starting on trunk, followed by head, then face, then less
commonly extremities
o May be a total of 100-500 lesions
o Pruritus is universal and marked
o ***Lesions may be present on mucous membranes! (Enanthem)
• Other: Signs of URTI

• May have generalized lymphadenopathy
• Systemic signs and fever generally abate after 3-4 days
RE: ZOSTER:
• Pre-eruption phase has intense, localized and constant pain and tenderness (acute neuritis) ALONG A
DERMATOME
• + malaise and fever
• After days: Eruption of papules which become vesicles occurs IN THE DERMATOME OR IN TWO
ADJACENT DERMATOMES
• Groups of lesions occur for 1-7 days then crust and heal
• Thoracic and lumbar regions typically involved
• Generally unilateral + regional lymphadenopathy
• Any branch of cranial nerve V may be involved which may also cause corneal and intraoral lesions
• Involvement of CN VII -> facial paralysis and ear canal vesicles (Ramsay Hunt syndrome)
• Ophthalmic zoster may be associated with ipsilateral cerebral angiitis and stroke
INVESTIGATIONS
• Lab testing is usually unnecessary
• PCR is the current diagnostic method of choice
• Serology: Acute and convalescent
• Diagnosis based on the distinctive characteristics of the rash (see stages)
MANAGEMENT
• Isolation (School exclusion, house room exlcusion)

• Symptomatic therapy:
o Nonaspirin antipyretics
o Cool baths
o Careful hygiene!!
• Routine acyclovir is NOT RECOMMENDED in otherwise healthy children. Useful in preventing severe
complications (Eg. pneumonia, encephalitis) in immunocompromised (Eg. HIV, steroids, oncology). Use
acyclovir or Valacyclovir.
• Zoster: Antiviral treatment. Oral Famicyclovir and Valacyclovir recommended > Acyclovir in adults
o BUT Acyclovir recommended in children
COMPLICATIONS
• Secondary bacterial infection may occur with invasive group A streptococcus leading to necrotizing fasciitis or
toxic shock syndrome.
• Other rare complications:
o Purpura fulminans, cerebrovascular stroke, and encephalitis.
• Life-threatening pneumonitis may occur in the young infant and immunosuppressed child.
OTHER COMMON PAEDIATRIC RASHES (PEADIATRICS AT A GLANCE)

Can use 3rd year document or derm handout or another source
NEWBORN, INFANCY AND CONGENITAL SKIN DISORDERS

Vascular birthmarks
* y-
mm
Capillary haemangioma Capillary malformation Mongolian blue spot

•Very common, especially in preterm infants •Sharply circumscribed, pink to purple lesion •Blue/grey lesions in the sacral
•bright red lumpy lesion due to proliferation •Present from birth (3 in 1000 births) area
of blood vessels •Abnormal dilatation of normal dermal •More common in racial groups with
pigmented skin
•Enlarges until age 2-4 years then regresses capillaries
•Usually resolves spontaneously with no •May be a sign of Sturge-Weber syndrome •Fade during the early years
treatment with an underlying meningeal haemangioma, •Can be confused with bruises
•If near important structures (airway, eyes), intracranial calcification and fits
injection with steroid may speed regression •Do not resolve but some lesions may be
•Treatment - propanolol improved with laser therapy
Pigmentation disorders
Pigmented naevus Cafe au lait* Depigmentation
•Can be present from •May develop •Depigmented skin
birth (congenital increasing size and patches seen in
naevus) or appear number through genetic disorder
during childhood childhood tuberous sclerosis
(moles) •5een in genetic •May develop brain
•Contains conditions abnormalities,
melanocytes •Neurofibromatosis epilepsy, learning
•May require surgical •McCune-Albright difficulties
excision if large syndrome
•If large, at risk •Links with neurological
of malignant change and skeletal problems
Common transient neonatal rashes
• I. .-
'
-
X,- :
Erythema toxicum neonatorum Milia Miliaria (heat rash)

•Commonest rash in newborns •Very common •Occlusion of sweat ducts
•Small erythematous macules •Tiny epidermal cysts •More common in hot humid environment
•+/- central small pustules •1-2mm white/yellow popular spots •Miliaria rubra usually at 10-15 days of age
•Resolves over a few days •Usually on nose, cheeks, chin, forehead •Resolves with reduced temperature
•Resolve
Nappy rash
Ammoniacal dermatitis Candidal nappy rash Seborrhoeic nappy rash
•Erythematous or papulovesicular •Bright red rash with clearly •Pink, greasy lesions with yellow scale
lesions, fissures and erosions demarcated edge •Often in skin folds
•Skin folds spared •Satellite lesions beyond border •Cradle cap may be present
•Caused by irritation from excretions •Inguinal folds usually involved •Treat with mild topical
and chemicals •May have oral thrush corticosteriods
•Unusual with modern disposable nappies ( white plaques in mouth)
•Secondary bacterial and candidal •Treatment with nystatin
infection common , and limited use of cream, and orally if
hydrocortisone and nystatin cream. necessary
Treat by regular washing and changing,
exposure to air, and use of protective
creame
Psoriatic nappy rash
•Appearance similar to seborrhoeic
dermatitis
•Family history of psoriasis
INFECTIONS AND INFESTATIONS
Meningococcal septicaemia
^•Rapid onsetsepticaemia +/ - meningitis
•Commonly due to meningococcus B, or C (other forms
also seen)
• Vaccination for meningococcus C has reduced rate of
infection
•Evolves with purple (purpuric) rash that does not
blanch with pressure
•Severe septicaemic shock, coma and death within hours
•Immediate treament with antibiotic and fluid
resuscitation
Chickenpox
•Very common childhood infection
•Onset 14-17 days after exposure
•Fever then rash (macule, vesicle, crusting)
•Sometimes see mucosal involvement (mouth, genitalia)
•Complications of pneumonia, eecondaiy infection, encephalitis
•Treat symptomatically in healthy children without complications
•Immunosuppressed children at risk of severe complications
•Treat with zoster immune globulin after exposure, and aciclovir
if signs of infection develop
Measles
•Rare in immunized population ( MMR vaccine protects)
•Onset 10-14 days post exposure
•Morbilliform rash
•Cough, fever, conjunctivitis, irritability
•Koplick’s spots (white spots in mouth)
•Rare complication of encephalitis
r
*».
Rubella Fifth disease
•Rare in immunized population (MMR •Mild illness with low-grade fever
vaccine protects) •Slapped cheek appearance
•Onset 14-21 days after exposure •Lace-like rash on body
•Pale morbilliform rash moves down •Lasts up to 6 weeks
body •Parvovirus S19 infection
•Severe fetal anomalies if mother
develops rubella in first trimester
/
/
-•tK '
" «*
5 -
Y
Scarlet fever
•Group A streptococcus tonsillitis
•Erythematous rash, sandpaper-like skin
Staphylococcal scalded skin syndrome*
•Usually triggered by staphylococcal infection
•Pale around lips
•Can cause systemic illness of shock symptoms •Inflamed tongue, strawberry appearance
•Swab skin to confirm infection and sensitivity •Risk of sequelae of glomerulonephritis and
•Treat with intravenous antibiotics and rheumatic fever
systemic support measures Treat with penicillin
Molluscum contagiosum Tinea corporis (ringworm)
•Pearly dome-shaped papules with •Dry , scaly papule which spreads
central umbilicus centrifugally with central clearing
•Particularly on face, axillae, neck •Diagnosis confirmed microscopically
and thighs by scrapings in a potassium
•Self -limited disease hydroxide wet mount
•Due to molluscipox virus infection •Treat with topical antifungal agents
•'Kissing lesions' occur on opposing for 2-4 weeks
skin surfaces, e.g. under arms and
on chest
Impetigo Cold sore

•Sticky, heaped-up, honey-coloured crusts •Single or grouped vesicles or
•Group A haemolytic streptococci or staphylococci pustules sited periorally
•Highly infectious •Recurrent herpes simplex infection
•Treat with antibiotics (flucloxacillin or •Recur with colds and stress
erythromycin orally, or antibiotic cream if •May be treated with aciclovir
<5 lesions)
Scabies Common warts

•Wheals, papules and vesicles with superimposed eczema •Roughened keratotic lesions with
•Intensely itchy an irregular surface
•Characteristic lesion is the mite burrow between the fingers •Occur on hands, face, knees and
•Head, neck, palms and soles are spared in children but not babies elbows
•Mites can be seen on scrapings •Called verrucas if present on feet
•Treat all the household with scabicides and launder bedding •Transferred by direct contact
•Disappear spontaneously, but can
be treated with salicylic acid or
liquid nitrogen >
Head lice (pediculosis capitis)

•Very common in schools— affects clean hair as well as dirty
•Itchy scalp
•Nits (the eggs) are visible as white specks on hair shafts
•Transmitted on clothing, combs or by direct contact
•treated by regularly combing out the eggs using an extra fine comb
or the use of anti-pediculosis shampoos. Resistance to these agents
is increasing
COMMON INFLAMMATORY DISORDERS
Atopic dermatitis (eczema)

•Erythema, wet ' weeping areas, dry scaly, thickened skin
1
•Intensely itchy
•Risk of secondary bacterial (staphylococcal) and viral (herpes zoster) infection
•Often linked with other atopic problems, e.g. asthma and hay fever
•Some cases linked with food and environmental allergens
•Sreast-feeding may reduce risk of eczema
•Treat with moisturizing creams to prevent skin drying
•Cream ( water based) to wet areas
•Ointment (oil based) to dry areas
•Wet wraps to prevent drying and reduce scratching
•Topical steroids to persistent inflamed areas
•Topical (tacrolimus) and oral (ciclosporin) immunomodulators if severe
•Family support and follow-up important for chronic condition
Seborrhoeic dermatitis Contact dermatitis

•Pry, scaly and erythematous •Erythema and weeping
•Cradle cap in infancy •Itching
•Affects face, neck, axillae •Caused by irritants such as
and nappy area . .. saliva, detergents and L -
k>
•May look like psoriasis Isa V . H synthetic shoes SM&S xf. . Mad
'
•Looks like atopic dermatitis

V
Psoriasis Henoch-Schonlein purpura
•Erythematous plaques •Vasculitic illness of uncertain
•Silver/white scales aetiology, often follows viral illness
•Extensor surfaces— scalp, knees, •Purpuric rash to buttocks and legs
elbows •+/- Arthritis
•Guttate psoriasis— linked to •+/- Abdominal pain with
streptococcal tonsillitis gastrointestinal vasculitis, risk
(antibiotic may improve skin) of intussusception
•Pitting of nail bed •+/- Nephritis (haematuria, proteinuria,
•Guttate psoriasis— multiple hypertension) rarely renal failure
tiny psoriatic plaques over large area of body •5ome evidence steroid helpful if
•Treat with topical vitamin P analogues (calcipotriol), coal tar abdominal pain severe
Acne*
•Very common at puberty
•Linked to androgen hormones
•Pustular erythema to face,
scalp and trunk
•Treat with antibiotic
erythromycin or tetracyclines
( over age 12)
•Hormonal treatment with
antiandrogen sometimes used
•Isotretinoin for severe cases under dermatology
Kawasaki disease*
•Acute inflammatory systemic disorder
•Many features of infectious illness
•Fever > 5 days
•Macular erythematous rash
•Peeling skin typically at fingers and
toes
•Lymphadenopathy
•Mucosal changes (cracked lips,
strawberry tongue)
•Conjunctivitis
•Risk of coronary artery aneurysms
•Treat with immunoglobulin and aspirin
s
Paediatrics
UHWI Class – Resuscitation of the Newborn
Dr. Trotman (Very good class)
November 2014
Have primary apnoea and secondary apnoea
1. Primary apnoea:
2. Secondary apnoea:
• Going on for so long that myocardium is affected à Bradycardic or arrest
***Treat every baby who is not breathing as being in secondary apnoea***
Most important thing about resuscitation is equipment

---
Latest literature says you can still resuscitate with room air if oxygen not available. Some recommend room air.
EQUIPMENT DESCRIPTION:
1. Self-inflating bag. (Ie. When you squeeze out the air, it reinflates itself)
NOTE:
• Bag used in the neonatal period should never be more than 250 mls. May blow a pneumothorax if more. Do
NOT use paediatric size in neonate!!
2. Reservoir: Self-inflating bags must always be used with a reservoir (tube)

• If you do not have one, every time it reinflates, it will pull in room air and mix it with the oxygen and will
only deliver about 40% oxygen
• The reservoir (tube) stores some oxygen from what is pumped directly into the bag from the actual oxygen
tube. (How: Some oxygen overflows into the attached end of the reservoir tube so when the bag reinflates
itself, it pulls in oxygen first rather than room air)
3. Oxygen tube: Also connects to the bag and supplies it with 100% oxygen
4. Pop-off valve: Pops off when the pressure is too high. Regulates the pressure
5. Manometer: Should always have a manometer! To monitor how much pressure being given
NOTE:
• Pressure should be between 15-20 mmHg of pressure. Should not exceed this
6. Mask:
• Important!!: Mask goes over the nose, around the mouth and just above the chin BUT should NOT go over
eyes. Must say all this in exam (while demonstrating)!
**Ensure you have different size masks, different size ET tubes.
7. ET tubes: Usually use size range from 2-4

• Remember to use uncuffed tubes in neonate. Easy to cause necrosis with cuffed
8. Resuscitaire®: Also need to check the resuscitaire

• Cannot allow babies who are not breathing to become cold. This increases the oxygen demand. Why?
• **Oxygen needed to produce heat/energy via aerobic respiration equation. Decreased oxygen = decreased heat
---
RESUSCITATION
Point: In real life, usually try to have more than one person so some of the steps can run simultaneously
Resuscitation is a CYCLE!
1. Evaluate
2. Make a decision
3. Action
4. Then reevaluate and repeat as necessary
***Each ACTION broken down into 30-second portions
INITIAL RESUSCITATION
***FOR ALL INFANTS FROM BIRTH, EVEN BEFORE ABC:
**Always receive the baby head first in a warm towel. This allows for a smooth movement to the resuscitaire in one
motion, without having to be turning and positioning the baby etc. which wastes time.
• So baby lies on back with scalp toward you and feet away from you
SCENARIO 1: Term baby in clear liquor (not meconium stained etc.)

• Receive and move to baby to the resuscitaire as above
• Dry entire body
o Head in neonate is 1/3 of body. Must dry it properly as due to its surface area it causes heat loss easily
o Drying also acts as stimulation NOTE: 2 accepted ways of stimulation
1. The drying motion
• Throw away this wet towel and replace with a new one 2. Flicking bottom of foot
(Toronto notes says can also rub the lower back
gently instead)
Now resuscitate:
• Position in sniffing position. Can manually do it or just put a warm towel rolled behind shoulder and baby will
automatically go in sniffing position
• Suction quickly. Suction the mouth first. Then nose. M BEFORE N Reason:…
• Assess heart rate: Place hand on umbilical cord: Best place to time heart rate is umbilical artery
• Count rate for 6 seconds and multiply by 10
Need to know 3 things!!!

1. Is baby pink?
2. Is baby breathing?
3. Is heart rate over 100?
90% of time this is all that needs to be done. No further interventions required.
Note: The 2 values used for heart rate are 60 and 100.
• **Less than 100 needs oxygen
• **Less than 60 needs external cardiac massage (cardiac compressions)
SCENARIO 2: Clear liquor, but baby apnoeic (not breathing) or heart rate < 100 on initial assessment
***In a baby who is not breathing the most important thing is to get oxygen to the lungs**
• Start same as above with warm and dry + stimulate etc. But intermittent positive pressure ventilation (IPPV) is
required
• Need to place baby in sniffing position.

• Place mask properly on face as described above
• Bag at 40-60/min as this is normal heart rate of neonate AND observe for GENTLE RISE AND FALL OF
CHEST
o So a good way to time: “Pump 2, 3, pump 2, 3, pump 2, 3” – Can say this in exam when demonstrating
• Do this for 30 seconds of adequate bag and mask
• Reevaluate
1. Breathing?
2. Pink enough?
3. Heart rate?
• If these are now ok: No more need for IPPV. STILL NEED OXYGEN, but not IPPV. Still using 100% oxygen.
Then slowly remove the oxygen. Not abruptly.
Consider if after the first 30 seconds heart rate good (>100), but inadequate respiration and baby just starting to pink up.
Decision: Continue IPPV
• Suction again
• Reassess technique and mask seal (readjust mask and reposition airway if necessary)
• Continue 30 more seconds IPPV
• Reassess:
A. If respiration now adequate, can stop IPPV. Still need oxygen.
B. If after second assessment, not improved: By this time heart rate is almost always falling as well. ***If
below 60, need external cardiac massage. If you reach this point, you NEED A SECOND PERSON.
Cannot do both ventilation and external cardiac massage as a single person. See below.
***Note that if after 2 attempts the patient is not fully improved, but heart rate is still over 60, move on to intubation
instead as there is no indication for external cardiac massage. This is unlikely to happen though***
2 ways to find the site for external cardiac massage (Lower 1/3 of sternum)
1. Can use inter-nipple line and go just below it OR

2. Find edge of costal margin and follow it to xiphisternum and go just above it
Both take you to the lower third of sternum
2 ways to perform external cardiac massage

1. Can Use 2-thumb method. Use TIPS of thumbs with other fingers on either side of baby holding baby trunk.
• Disadvantages: Hands block the umbilicus so the person ventilating and assessing heart rate has a harder
time. Person compressing tires more easily. Just generally this method is a more difficult method overall
2. 2-finger method preferred by most. Use index and third finger

• Better access for resuscitation.
--
• Find landmark for compression (lower third of sternum). One you reach the landmark your fingers never
leave the chest!!!
• The depth of compression should be about 1/4-1/3 of the AP height of the chest. (At a rate of 120 events
per minute according to Toronto: Ie. 3 compressions:1 ventilation = 90 compressions/min:30 breaths/min)
• Do 3 compressions to 1 ventilation in neonate
• Note: When you do this must note that the ventilation rate now falls from 40 to 30 based on the above
explanation
• Good pattern for timing is: “And-one-and-two-and-three-and-breathe”. When you say breathe, other
person gives the squeeze. (Can say this in exam when demonstrating)
• Reassess after 30 seconds
• Once the heart rate passes 60, no more need for external cardiac massage. BUT IPPV continues
• If baby did not improve, try it one more time. Remember each thing you do you try twice
Note: Timing of intubation could be 2 points:
1. From the point of deciding that external cardiac massage was necessary
OR
2. If after 2 rounds of the external cardiac massage failed, intubate and CONTINUE COMPRESSIONS
If after intubation, 2 sets of compressions with ET tube in place not working: Need to move to drugs
• Start with adrenaline

o 2 possible routes of administration:
1. Endotracheally (Through ET tube) OR
2. IV through umbilical vein
• Use 1:10 000 solution for neonate: 0.1 to 0.3 ml/kg/dose (Usually don't give more than 3 rounds of adrenaline)
• Usually administer through ET tube

• Usually get an immediate response
• If not working, consider there may be acidosis on board:
***Managing the acidosis***
• Give a bolus of LR or NS. Trying to increase peripheral perfusion. Can bolus twice (10 mL/kg each)
• If no response, may consider sodium bicarbonate. 2 reasons we are hesitant to give this.
o Reason 1: End product is CO2. And baby is already not breathing.
o Reason 2: And in preterm, cerebral autoregulation has not developed so rapid changes in systemic pressure
translate to rapid change in cerebral perfusion pressure and can cause hemorrhage. (Remember water
follows sodium resulting in expanding the intravascular volume)
• Note: Give it over 20 minutes. Never give bolus of bicarb to anybody any age.
---
FOR MECONIUM-STAINED LIQUOR:
For baby born through meconium only the 1st part is different
3 possible scenarios
*CLEAR SCENARIO #1
• If baby crying and good apgars à meconium gone. Still need to keep in hospital for 48 hours
*CLEAR SCENARIO #2
• Flat baby
- Start resuscitation with bag and mask immediately

- If versed in intubation, may go straight to intubation. So, going to suction baby quickly and intubate. This is
not any thorough suction trying to retrieve meconium
The most important thing here is NOT clearing the meconium, it is resuscitation!! GET OXYGEN IN THE LUNGS
*Now the in between ones

• Not totally limp, not breathing but has a good heart rate
Now this one you DO NOT stimulate him. Do NOT want this one to cry à Theoretical risk of aspiration. He has not
started to breathe yet
In this one you need to find and suction meconium from beneath the cords and try to clear it. Retrieving
meconium is only done in this case. Then intubate (Even though the most recent studies say this may not help even in
this case as most of the aspiration probably already occurred in utero)
Then everything is the same after
TORONTO NOTES SUMMARY
Initial Resuscitation
• anticipation: know maternal history, history of pregnancy, labour, and delivery
• steps to take tor all infants ( before ABCs )
warm ( radiant heater, warm towels ) and dry the newborn ( remove wet towels)
position and clear airway ( “ sniffing" position )
stimulate infant: rub lower back gently or flick soles of feet EXCEPT if meconium present ( in
which case tracheal suction first )
assess breathing and heart rate
• Airway
if meconium is present and
baby is vigorous ( strong respiratory effort , good muscle tone, HR > 100 ): no further
resuscitative interventions required
baby is not vigorous: intubate and suction trachea while monitoring vital signs. If
prolonged or unsuccessful intubation , attempt bag mask ventilation
if no meconium and suction required, suction mouth first and then nose
• Breathing
if HR < 100 or apnoeic, apply positive pressure ventilation ( PPV)
PPV at rate of 40 - 60 / min with enough pressure to see visible chest expansion and note
increase in HR
if PPV not effective ( no increase in HR , no chest rise), incorporate MRSOPA corrective actions
• Circulation
if HR <60 after 30 s of effective ventilation , start chest compressions ( “ 60 or less, compress” )
should provide 100% oxygen as soon as chest compressions are required
chest compressions at lower 1 /3 of the sternum and 1 /3 of the AP depth at a rate of 120
events per min ( 3 compressions:! ventilation = 90 compressions/ min:30 breaths/ min )
Paediatrics
Rheumatic Fever Notes
Source: Nelson’s (p. 992 of 2692)
September 2014 | 2017 | 2018
DEFINITION OF RHEUMATIC FEVER: An autoimmune inflammatory process that develops as a sequela of streptococcal infection
DEFINITION OF RHEUMATIC HEART DISEASE: Cardiac inflammation and scarring triggered by an autoimmune reaction to infection with GAS
INCIDENCE/EPIDEMIOLOGY
• Incidence of both initial attacks and recurrence of acute rheumatic fever peaks in children **5-15 years**(the
age of greatest risk for Group A Streptococcus (GAS) pharyngitis [Streptococcus pyogenes specifically]
• NOTE: Worldwide, rheumatic heart disease remains the MOST COMMON FORM OF ACQUIRED HEART
DISEASE IN ALL AGE GROUPS accounting for as much as 50% of all cardiovascular disease and as much as
50% of all cardiac admissions in many developing countries.
o MUST KNOW THIS TOPIC WELL. VERY COMMON AND IMPORTANT IN OUR SETTING.
• Historically, acute rheumatic fever has been associated with poverty, particularly in urban areas.
o Crowding is the most significant factor which contributes to the spread of GAS infections and the
incidence of acute rheumatic fever
• *In addition to the specific characteristics of the GAS organism, the risk of a person developing acute rheumatic
fever is also dependent on various host factors
o Association with specific HLA markers and a specific B-cell alloantigen (D8/17)
AETIOLOGY
There is considerable evidence to support the link between Group A streptococcus (GAS) upper pharyngitis tract
infections and acute rheumatic fever and acute rheumatic heart disease
• ***2/3 of patients with an acute episode of rheumatic fever have a history of an upper respiratory tract
infection several weeks before. [So 1/3 do not have this history]
• Their antibody titres on serology are considerably higher than those patients with GAS infections without acute
rheumatic fever
Certain serotypes (M types 1, 3, 5, 6, 18, 24) are more frequently isolated from patients with acute rheumatic fever than
are other serotypes.
PATHOGENESIS
Several theories have been proposed for acute RF and acute RHD. Only 2 are seriously considered
1. Cytotoxicity theory
2. Immunologic theory
• Cytotoxicity theory: GAS produces streptolysin O (+several other enzymes) that are cytotoxic for mammalian
cardiac cells. Problem with this theory is that it doesn’t explain the latent period between pharyngitis and onset of
acute RF
• Immunologic theory: Suggested by the latent period between GAS infection and acute RF. Also, the
immunologic cross reactivity (MOLECULAR MIMICRY) between GAS components and mammalian
tissues also supports this.
o Bacterial M protein resembles proteins in cardiac self-antigens. There is an immune response that target
theM proteins of the bacteria which also attack the cardiac tissue. Common antigenic determinants shared
between GAS and specific mammalian tissues (eg. heart, brain, joints). Eg. Certain M proteins of GAS
with human tropomyosin and myosin.
CLINICAL FEATURES
•  H/O- sore throat – 70% adults, children 20%

- fever - most cases
•  CARDITIS
symptoms – chest pain, breathlessness, edema, cough, orthopnoea, palpitations signs of valvulitis, Murmurs (apical Pan
systolic Murmur, apical Mid Diastolic Murmur, basal End Diastolic Murmur)
•  pericarditis – friction rub, distant heart sounds, increased cardiac dullness, ECG/Cxray and 2Decho features
•  tachycardia – due to valvulitis, fever, CCF
•  cardiac failure secondary to MI and AI
•  ARTHRITIS
migratory, large joints, much pain and tenderness, rapid response to ASA and NSAIDS, no permanent effects.
•  CHOREA - emotionally labile, clumsy, difficulty writing & speaking, grimacing, choreiform movements of arms &
legs, trombone tongue, milkmaid grip.
LABORATORY INVESTIGATIONS
•  CBC, ESR, ASTO, CRP, Sickle test (This is a differential dx for presentation)
•  Blood culture, Throat culture
•  ECG
•  CXR
•  2D Echo
DIAGNOSIS
No clinical or lab finding is pathognomonic for acute rheumatic fever.
JONES’ CRITERIA
T. Duckett Jones in 1944 proposed guidelines - Jones’ criteria

• Revised in 1992 by AHA
***Intended only for the diagnosis of the INITIAL ATTACK. NOT for recurrences!
5 major and 4 minor criteria AND AN ABSOLUTE REQUIREMENT FOR EVIDENCE (MICROBIOLOGIC OR
SEROLOGIC) OF RECENT GAS INFECTION
Diagnosis is based on Jones criteria.

1 required criteria + 2 major + 0 minor OR 1 required criteria + 1 major + 1 minor
Required criteria
Evidence of prior group A Beta-hemolytic streptococcal infection by the use of markers with the presence of major
and minor criteria. The markers are:
ANO titers (Anti Streptolysin O titers) AND ADB titers (Anti DNase B titers)
Minor criteria are nonspecific and include = FRAPP:

Fever (Clinical)
Raised ESR or CRP (Laboratory)
Arthralgia (Clinical)
Prolonged PR (Laboratory)
Previous rheumatic fever
Major criteria = JONES
* The major issue is with the heart problems as all the others are transient
J - Joint problems
Migratory polyarthritis
Swelling and pain in a large joint (e.g., wrist, knees, ankles) that resolves within days and
"migrates" to involve another large joint
O - Heart problems (O around the shape of a ♥ :))

Carditis
Endocarditis
Mitral valve is involved more commonly than the aortic valve. Characterized by
small vegetations along lines of closure that lead to regurgitation
Myocarditis
Associated with Aschoff bodies (microscopic finding) that is a foci of chronic
inflammation characterized by HALLMARK FINDINGS
Giant cells
Fibrinoid material
Anitschkow cells (catapillar nuclei)
reactive histocytes with slender, wavy nuclei
It is the most common cause of death during the acute phase.
Pericarditis
Leads to friction rub and chest pain
N - Nodules (Subcutaneous)
E - Erythema marginosum
Annular, nonpruritic rash with erythematous borders, commonly involving trunk and limbs
S - Sydenham Chorea
Rapid, involuntary muscle movements
Modified Jones
Criteria
Recurrent w/o Rheumatic

Primary RF
CHF Recurrent w/ Chorea Insideous onset
CHF
Manifestations:
Manifestations: Manifestations: Manifestations: Chronic RHD
2 Major or
2 major or Manifestations: Other Other
1 Major & 2
1 major & 2 2 minor + manifestation or manifestation or Manifestations:
minor
minor + evidence of evidence of evidence of NOT REQUIRED
+ evidence of
evidence of recent GABHS recent GABHS recent GABHS
preceding
recent GABHS infection infection NOT infection NOT
GABHS
infection REQUIRED REQUIRED
MAJOR MANIFESTATIONS
1. Migratory polyarthritis: In about 75% of patients with acute rheumatic fever
• Frequently the earliest manifestation of acute rheumatic fever
• Typically involves LARGER joints (esp. elbows, wrists, knees, ankles)

• Joints generally HOT, RED, SWOLLEN, TENDER (Ie. All the features of acute inflammation - calor, rubor,
tumor, dolor)
• Characteristic feature: Migratory
• Characteristic feature: Dramatic response to even small doses of salicylates

• Typically nondeforming
• Monoarticular arthritis is unusual
2. Carditis: occurs in about 50-60% of all cases of rheumatic fever
NOTE: Carditis and resultant chronic rheumatic heart disease are the most serious manifestations of acute
rheumatic fever and account for essentially all of the associated morbidity and mortality!!!!
***Rheumatic carditis characterized by PANCARDITIS – active inflammation of endocardium, myocardium and pericardium.
• BUT! Endocarditis (valvulitis) is a UNIVERSAL FINDING IN RHEUMATIC CARDITIS
• Pericarditis or myocarditis is variable

• (So always have endocarditis if carditis is present, but may or may not have myo- or pericarditis)
Most cases consist of either isolated mitral valve disease OR combined aortic and mitral valve disease
aortic or right-sidedvalvular involvement is uncommon
• Valvular insufficiency is characteristic of both the acute and convalescent stages of acute RF!!
• Valvular stenosis usually appears years later. BUT in developing countries, mitral stenosis and aortic stenosis
may develop earlier
o ***NOTE: Rheumatic carditis usually presents as tachycardia + cardiac murmurs

o ***NOTE: Moderate to severe -> cardiomegaly, CCF with hepatomegaly and pulmonary oedema
Clinically, rheumatic carditis is almost always associated with a murmur of valvulitis.
Echo findings: Pericardial effusion, decreased contractility, mitral and or aortic regurgitation. Subclinical valvular
regurgitation detected on echo is not currently accepted as either a major or minor Jones criterion by the AHA
3. Sydenham Chorea: Occurs in about 10-15% of patients
o Incoordination, poor school performance, uncontrollable movements, facial grimacing disappearing with sleep are
characteristic
o See the clinical maneuvers to elicit features of chorea
4. Erythema marginatum: Rare (< 3%) of patients

o Consists of erythematous, macular lesions with pale centers. Not pruritic
o Primarily on trunk and extremities but NOT on the face
5. Subcutaneous nodules: Rare (< 1%) of patients

o On extensor surfaces of tendons near bony prominences
RECENT GAS INFECTION

IS AN ABSOLUTE REQUIREMENT FOR DIAGNOSIS OF ACUTE RF
Acute RF typically develops 2-4 weeks after an acute episode of GAS pharyngitis when clinical findings
are no longer present
1/3 of patients with acute RF have no history of an antecedent pharyngitis
95-100% have an elevation among 3 antibodies: Antistreptolysin O, anti-DNase B, antihyaluronidase. Not

necessarily all will be elevated. Most commonly antistreptolysin O used (detects only 80-85% used alone)
Except for chorea, clinical findings of acute RF generally coincide with peak antistreptococcal antibody responses
NB: Do not make diagnosis based on antibody titres alone if Jones criteria not fulfilled
***Depends on which feature of the RF is predominant***:
Arthritis
• Must consider a collagen vascular disease. Rheumatoid arthritis in particular
o RA= Usually younger age, usually has spiking fevers/lymphadenopathy/splenomegaly, much less dramatic
response to salicylates
o SLE = use antinuclear antibodies (ANA) to distinguish

• Other causes: Gonococcal arthritis, malignancies, serum sickness, Lyme disease, sickle cell, reactive arthritis
related to GI infections e.g. shigella, salmonella, Yersinia
When carditis is the sole major manifestation:

• Viral myocarditis
• Viral pericarditis
• Kawasaki disease
• Infective endocarditis (may also have joint manifestations) – can usu distinguish IE from acute RF by blood
cultures and presence of associated findings (Eg. haematuria, splenomegaly, splinter haemorrhages)
When chorea is the sole manifestation:

• Huntington chorea
• Wilson disease
• SLE
• Other encephalitides
MANAGEMENT
ALL PATIENTS THIS IS A MUST THIS IS A MUST !!! -> Bed rest and monitoring for carditis
• Allowed to ambulate when signs of acute inflammation subside
• BUT patients with carditis require longer periods of bed rest
Antibiotic therapy:
• 10 days of oral penicillin OR erythromycin
OR
• Single IM injection of Benzathine penicillin
**After this initial course of antibiotic therapy, the patient should be started on long-term antibiotic prophylaxis:
• Benzathine penicillin G EVERY 28 DAYS for the next 5 years or until age 18 - 21 whichever is longer
Anti-inflammatory therapy
• Anti-inflammatory agents (e.g. salicylates, corticosteroids) should be WITHHELD IF ARTHRALGIA OR

ATYPICAL ARTHRITIS IS THE ONLY CLINICAL MANIFESTATION OF SUSPECTED ACUTE
RHEUMATIC FEVER
• **Reason: Premature treatment with one of these agents may interfere with the development of the characteristic
migratory polyarthritis and thus obscure the diagnosis of acute rheumatic fever
• Acetaminophen may be used to control pain while patient being observed
NOTE VERY WELL:

o If carditis + typical migratory polyarthritis WITHOUT cardiomegaly or CCF ->
PO salicylates (aspirin) for ~ 4 weeks
o If carditis WITH cardiomegaly or CCF ->

Corticosteroids (prednisone) ~ 3 wks then taper and switch to aspirin for ~ 6 weeks
Supportive therapies for mod – severe carditis: Digoxin, fluid and salt restriction, diuretics, oxygen
Sydenham chorea: anti-inflammatory agents usually not indicated

• Sedatives may be useful: Drug of choice = Phenobarbital
• Others: Haloperidol or chlorpromazine
COMPLICATIONS
• Long-term sequelae of rheumatic fever are usually limited to the heart
PREVENTION
Prevention of both initial and recurrent episodes of acute rheumatic fever depends on controlling GAS infections of the
upper respiratory tract.
Prevention of initial attacks (primary prevention) depends on identification and eradication of the GAS that produces
episodes of acute pharyngitis.
Individuals who have already suffered an attack of acute rheumatic fever are particularly susceptible to recurrences of
rheumatic fever with any subsequent GAS upper respiratory tract infection, whether or not they are symptomatic.
Therefore, these patients should receive continuous antibiotic prophylaxis to prevent recurrences (secondary prevention).
PRIMARY PREVENTION
Appropriate antibiotic therapy instituted before the 9th day of symptoms of acute GAS pharyngitis is highly effective in
preventing 1st attacks of acute rheumatic fever from that episode.
• However, about 30% of patients with acute rheumatic fever do not recall a preceding episode of pharyngitis.
SECONDARY PREVENTION
Preventing acute GAS pharyngitis in patients at substantial risk of recurrent acute rheumatic fever.
Requires continuous antibiotic prophylaxis à Should begin as soon as the diagnosis of acute rheumatic fever has been
made and immediately after a full course of antibiotic therapy has been completed.
• Because patients who have had carditis with a valvular lesion their initial episode of acute rheumatic fever are at a
relatively high risk for having carditis with recurrences and for sustaining additional cardiac damage, they should
receive long-term antibiotic prophylaxis well into adulthood and perhaps for life.
• Patients who did not have carditis their initial episode of acute rheumatic fever have a relatively low risk for
carditis with recurrences. Antibiotic prophylaxis should continue in these patients until the patient reaches 21 yr
of age or until 5 yr have elapsed since the last rheumatic fever attack, whichever is longer.
The decision to discontinue prophylactic antibiotics should be made only after careful consideration of potential risks
and benefits and of epidemiologic factors such as the risk for exposure to GAS infections.
The regimen of choice for secondary prevention is a single IM injection of benzathine penicillin G (Penadur®) every 4
wk (1 every 28 days!).
• In certain high-risk patients, and in certain areas of the world where the incidence of rheumatic fever is particularly high, use
of benzathine penicillin G every 3 wk may be necessary because levels of penicillin may decrease to marginally effective
amounts after 3 wk.
**In compliant patients, continuous oral antimicrobial prophylaxis can be used:
• Penicillin V given twice daily OR sulfadiazine given once daily are equally effective when used in such
patients.
• If allergic to both penicillin and sulfonamides, a macrolide (erythromycin or clarithromycin) or azalide
(azithromycin) may be used.
Table 176- 4 CHEMOPROPHYLAXIS FOR RECURRENCES OF ACUTE
RHEUMATIC FEVER
DRUG DOSE ROUTE Table 176-5 DURATION OF PROPHYLAXIS FOR PEOPLE WHO HAVE
Penicillin G benzathine 600,000 U for children, <60 lb Intramuscular HAD ACUTE RHEUMATIC FEVER: RECOMMENDATIONS OF THE
1.2 million U for children >60 lb, AMERICAN HEART ASSOCIATION
every 4 wk*
CATEGORY DURATION
OR
Penicillin V 250 mg, twice a day Oral Rheumatic fever without carditis 5 yr or until 21 yr of age, whichever is
OR longer
Sulfadiazine or sulfisoxazole .
0.5 g once a day for patients Oral Rheumatic fever with carditis but 10 yr or until 21 yr of age, whichever is
<60 lb without residual heart disease (no longer
1.0 g, once a day for patients valvular disease*)
>60 lb Rheumatic fever with carditis and 10 yr or until 40 yr of age, whichever is
FOR PEOPLE WHO ARE ALLERGIC TO PENICILLIN AND SULFONAMIDE DRUGS residual heart disease (persistent longer, sometimes lifelong prophylaxis
Macrolide or azalide Variable Oral valvular disease*)
*ln high-risk situations, administration every 3 weeks is recommended. •Clinical or echocardiographic evidence.
1. Sickle cell disease

2. Arthritis
--- Rheumatoid arthritis
--- Juvenile Idiopathic arthritis
--- Reactive arthritis
--- Septic arthritis
--- Gonococal arthritis
3. Mixed Connective Tissue Disease
4. Lyme Disease
Paediatrics
Seizures Notes
Sources: Nelson’s, Lecture from OurVLE
September 2014
DEFINITIONS
Seizure: Disturbance in motor, sensation or consciousness caused by paroxysmal discharge of electrical activity in the
cerebral cortex
Epilepsy: Tendency for having recurrent, unprovoked seizures
TYPES
1. Focal seizures
a. Simple focal: No impaired consciousness
b. Complex focal: Consciousness affected
• One can lead to the other (mostly simple to complex)

• Or either can progress to secondarily generalized (Most often tonic-clonic. Also tonic, clonic, atonic)
2. Generalized
a. Primary generalized
b. Secondarily generalized
Status epilepticus (According to lecture): Tonic-clonic status epilepticus defined as a continuous convulsion lasting
30 minutes or more, or repeated convulsions without complete recovery of consciousness between attacks
Re: Generalized Seizures:

• May be convulsive or non-convulsive
Generalized MOTOR seizures can be:
• Tonic: Sustained contraction

• Clonic: Rhythmic contraction
• Tonic-Clonic Sustained muscular contraction followed by rhythmic movements (Most common)
• Absence:
o Usually < 10-20 seconds
o Have no aura and no post-ictal phase. Automatisms common
o Typical absences associated with 3 Hz spike-and-slow wave discharges on EEG
• Myoclonic: Rapid, muscular contraction. Usually not rhythmic
• Atonic
May be difficult to differentiate among the above when family only reports a fall. In such cases, may
describe the seizure as a “drop attack”
Tonic, clonic, myoclonic or atonic can be focal with secondary generalization or primary generalized
Other important definitions:

• Aura: SENSORY experiences reported by the patient, not observed externally.
o May be visual (eg. flashing lights, colours), somatosensory (eg. tingling), olfactory, auditory,
vestibular, experiential (déjà vu, deja vecu) depending on site of origin of seizure
• Automatism: Automatic, semipurposeful movements of the mouth (“oral” eg. chewing) or extremities
(“manual” eg. fixing sheets, “leg” eg. walking)
Epilepsy is a symptom not a..
APPROACH TO UNPROVOKED SEIZURES
Acute setting:
• Evaluate vital signs and resp. + cardiac functions
• Measures to normalize and stabilize the above
History (Important):
• Details of seizure manifestation – esp. those at initial onset – may suggest type and brain localization
• Specifically question for all the symptoms/signs: (Duration, jerking of limbs, eye rolling, frothing at mouth,
tongue biting, urinary and stool incontinence, cyanosis, LOSS OF CONSCIOUSNESS)
• Possible causes: Fever (Only if 6 months – 5 years)? Head injury? Fall? Hypoglycaemic agent ingestion? Lead
exposure (paint, eating dirt)? Ackee ingestion? Medications? Family history?
• Ask about patterns – e.g. clustering
• Ask about precipitating conditions – e.g. Sleep, sleep deprivation, TV, visual patterns, mental activity, stress
• Exacerbating conditions (e.g. menstrual cycle, medications)
• Frequency
• Duration
• Time of occurrence and other characteristics
• Commonly overlooked/underreported: Absence, complex partial, myoclonic
• Personality change
• Cognitive regression
• Developmental history
• Medication history
• Pre/perinatal distress
• Family history of epilepsy
Guidelines for evaluation/treatment of “FIRST, unprovoked, nonfebrile seizures”:

• Careful history and examination
• Brain imaging with CT or MRI (note that in first FEBRILE seizures, these are NOT indicated)
• Emergency head CT often useful in acute management
• EEG
• Lab studies are recommended. Choices depend on the clinical history and top differentials
• Spinal tap/LP if suspected meningitis or encephalitis
• Many others depending on suspected cause
INCIDENCE
-
AETIOLOGY
-
BASIC PATHOPHYS
-
MORE ON TYPES OF SEIZURES
PARTIAL SEIZURES (AND EPILEPSY SYNDROMES ASSOCIATED WITH THEM)
Simple Partial seizures:
Can take the form of:

1. SENSORY SEIZURES (AURAS) or
2. Brief MOTOR seizures – most common (incl focal tonic, clonic or atonic)
3. Autonomic
4. Psychological
Often have a Jacksonian march or postictal (Todd’s) paralysis. (Can look these up)
Complex Partial seizures:
• Usually last 1-2 minutes

• Often preceded by an aura (see auras on 1st page)
• Children < 7 less likely to report auras. Parents may report unusual preictal behaviours
• After aura: May have decreased responsiveness, blank staring, automatisms
• Patient does not recall the epileptic event
• Patient may have postictal automatisms, sleepiness and/or focal deficits e.g. weakness
Secondary Generalized seizures:
• Can start after obvious simple/complex partial seizure with subsequent clinical generalization
• OR can start with generalized clinical phenomena (due to rapid spread from partial to generalized)
• Often generalized tonic-clonic activity

• Most last 1-2 minutes
• Tongue biting, urinary and stool incontinence, vomiting with aspiration risk and cyanosis common
• Rare complications incl fractures of vertebrae or humerus
• EEG in patients with partial seizures usually shows focal spikes or sharp waves in the lobe it originates in
• ~15% have normal EEGs
• Brain imaging is CRITICAL in focal seizures
• MRI preferred to CT in these cases
SOME BENIGN EPILEPSY SYNDROMES WITH PARTIAL SEIZURES:
***Most common:
1. Important: **Benign Childhood Epilepsy with Centrotemporal spikes (BECTS) (aka. Benign Rolandic
Epilepsy of Childhood)
• Starts in childhood (peak 5-10 years) and **outgrown in adolescence (spontaneous remission)
• **Precipitated by sleep!! May **wake child at night
• No neurological or intellectual deficit
• Autosomal dominant/Multifactorial
• EEG: Broad-based centrotemporal spikes
• MRI is normal
• Drug therapy indicated in 30%. Respond well to carbamazepine
Others
2. Benign Epilepsy with Occipital Spikes
3. Benign Infantile Familial Convulsion Syndromes
SOME SEVERE EPILEPSY SYNDROMES WITH PARTIAL SEIZURES:
Epilepsy secondary to focal brain lesions has a higher chance of being severe and refractory to therapy than idiopathic
epilepsy
Often due to severe metabolic problems, hypoxic-ischaemic injury, congenital malformations
Migrating Partial Epilepsy of Infancy:

• Multifocal, severe, partial seizures
• Mental regression and cerebral atrophy
Others:
Temporal Lobe Epilepsy (usually caused by mesial/medial temporal sclerosis) – often preceded by febrile seizures
GENERALIZED SEIZURES (AND EPILEPSY SYNDROMES ASSOCIATED WITH THEM)
ABSENCE SEIZURES
• Usually start at 5-8 years

• Girls > Boys
• Often missed/overlooked by parents due to how short they are even though they may occur up to hundreds of
times per day
• **DO NOT have an aura, unlike complex partial
• **DO NOT have a postictal period – patient immediately resumes what he/she was doing before
• May have simple automatisms: Eg. lip-smacking, picking at clothes
• Last a few seconds (10-20 s)
• Hyperventilation for 3-5 minutes can precipitate them
• 3 Hz spike-and-slow wave discharge
• First line drugs: Ethosuximide, Valproic acid, lamotrigine
• Second line: Clonazepam, clobazam, acetazolamide
GENERALIZED MOTOR SEIZURES
• MOST COMMON = TONIC-CLONIC – Can either be primarily or secondarily generalized

• If primary, then usually seizure starts with loss of consciousness +/- sudden cry, eye-rolling, generalized tonic
contraction with falling, apnoea and cyanosis
• Tonic phase followed by a clonic phase
• Lasts 1-2 mins
• Incontinence and post-ictal period often follow
• Post-ictal period usually lasts ~ 30 minutes to hours – sleepiness, ataxia, hypo/hyper-reflexia, headaches
• ***First-aid measures incl positioning patient on side, clearing mouth if open, loosen tight clothes, +/-
insertion of airway
BENIGN GENERALIZED EPILEPSY SYNDROMES
1. Petit-mal epilepsy – most outgrow before adulthood

2. Benign Myoclonic Epilepsy of Infancy
3. Febrile Seizures Plus Syndrome – febrile seizures and other seizures in multiple family members
4. Juvenile Myoclonic Epilepsy (Janz syndrome) – Most common generalized epilepsy in young adults
o Starts in early adolescence with any of:
§ Myoclonic jerks in the morning
§ Generalized tonic-clonic seizure on waking
§ Juvenile absences
SEVERE GENERALIZED EPILEPSY SYNDROMES

**These are associated with intractable seizures and developmental delay**
• Early Myoclonic Infantile Encephalopathy (EMIE) – starts during first 2 months of life – myoclonic seizures
• Early Epileptic Infantile Encephalopathy (EEIE) – Ohtahara syndrome – tonic seizures
• Severe Myoclonic Epilepsy of Infancy (Dravet syndrome)
• **West syndrome – (Infantile Spasms) – MUST KNOW:

o Triad of:
1. Myoclonic spasms in clusters
2. Psychomotor developmental arrest and regression
3. Hypsarrhythmia
o **Begins between 4-6 months of life. Insidious onset

o Commonest of the epileptic encephalopathies
o Symmetrical, bilateral, brief and sudden contraction of axial muscle groups
o Flexor (“Salaam attacks”), extensor and mixed
o Occur in clusters on awakening or falling asleep
o Followed by cry and brief period of attenuated responses
o Aetiology: Symptomatic vs. Cryptogenic/Idiopathic (9-15%)
§ Symptomatic aetiologies may be prenatal, perinatal or postnatal
o MRI finding: Lissencephaly (Wiki: Literally means smooth brain)
o MANAGEMENT:
1. ACTH or Prednisone (According to Medscape and lecture)
2. Nitrazepam
3. Valproic acid
4. Vigabatrin
o PROGNOSIS:
• Spasms and hypsarrhythmia disappear before 3 years
• 55-60% develop other types of seizures
• Prognosis re: normal development is poor – 70-78% are mentally retarded. Cryptogenic group
does better
• Lennox-Gestaut syndrome – Triad of 1) Multiple generalized seizure types incl absence, and myoclonic,
atonic and tonic, 2) Developmental delay + mental retardation 3) Diffuse slow spike-and-waves on EEG
o Onset 2-8 years
o Boys > Girls
o Low incidence
o Prevalence: 5% of all epileptic patients
o Aetiology: Symptomatic (70% - 1/3 evolve from infantile spasms) vs. Cryptogenic (30%)
o Management: Valproic acid, felbamate, nitrazepam, lamotrigine, topiramate, vigabatrin, ketogenic diet,
immunoglobulin, corpus callostomy, vagus nerve stimulation
TREATMENT OF SEIZURES AND EPILEPSY

**EDIT NOTES FROM MY NOTEBOOK IN HERE**
After a first seizure, IF the risk of recurrence is low and pt has normal development, EEG and MRI, then
treatment is NOT STARTED
If any of these abnormal, treatment often started
Mechanism of action of antiepileptic drugs (AEDs). Either:

• Reduce excitability by interfering with sodium and/or calcium ion channels
• Reducing glutamate induced excitatory function or
• Enhancing GABAergic inhibition
• (Many meds combine some of the above)
In general, drug of 1st choice in

• Partial or secondary generalized seizures and epilepsies: are oxcarbazepine and carbamazepine (Others
valproate, phenobaribital)
• For absence: Ethosuximide (others incl valproate and lamotrigine)
• Juvenile Myoclonic Epilepsy: Valproate and Lamotrigine
• Lennox-Gestaut: Valproate, topiramate
• Dravet syndrome: Valproate and benzodiazepine
• Valproate effective for generalized and unclassified seizures/idiopathic epilepsy
Control with 1 drug (monotherapy) should be the goal
How long to treat?:
Discontinuation usually indicated when children are free of seizures for at least 2 years
Factors associated with a low recurrence rate following discontinuation:

• Onset of seizures at an early age
• Prompt seizure control
• Seizure type: Tonic-clonic, absence
• EEG normal or normalizes
Indications for surgery

• If patient has failed 3 drugs, chance of achieving seizure freedom using AEDs is < 10%
• Usually considered if 3 failed AEDs in 2 years of onset of epilepsy
• Focal resection of the epileptogenic zone is most common

• Hemispherectomy for diffuse hemispheric lesions
GET SIDE EFFECTS OF THE COMMON DRUGS!!

Wayne Robinson, MRBS Class of 2015
Paediatrics
Sickle Cell Disease - Notes
Sources: Lecture on SCD, Nelsons P. 1735 of 2682 September 2014 (Heavily Modified June 4, 2018 - Anggelos)
Hb S is the result of a single base-pair change, thymine for adenine at the 6th codon of the B globin gene (on
chromosome 11). Result is valine (amino acid) replaces glutamic acid at position 6 of the beta subunit of the haemoglobin
(protein). Homozygous Hb S occurs when both B globin genes have the sickle mutation. Remember a codon is 3 nucleotides
and it codes for a specific amino acid. So if one base changes in the codon, it will cause a different amino acid to be added to the
protein being made, in this case the beta chain of haemoglobin. NB: Change from glutamine to lysine = Hb C
***NOTE: From May Pen Ward Rounds: Definition of sickle cell disease: Combination of haemoglobin S and another
abnormal haemoglobin
SCD is a haemoglobinopathy with the following consequences:

* Increased RBC fragility → Haemolytic anaemia
• Anaemia
• Jaundice
• Gallbladder disease
• Vasculopathy → Vaso-occlusion
• Pain
• Acute Chest Syndrome
• Stroke
DIFFERENCES BETWEEN NORMAL RBC AND SICKLED RBC

ATTRIBUTES NORMAL RBC SICKLED RBC
SHAPE Oval and Biconcave Sickled
LIFESPAN 120 days < 20 days
O2 CARRYING CAPACITY Normal Decreased
FLEXIBILITY Normal Decreased
RIGIDITY Normal Increased
Inheritance
• 10% of Jamaican population has sickle cell trait
• Some form of SCD affects 1:150 births in Jamaica
• Autosomal recessive
• Passing on the gene
a. Both parents with the trait = ¼ or 25% chance of a child with SCD
b. One parent with trait and other with disease = ½ or 50% chance of child with SCD
c. Both parents with disease = 100% chance of child with SCD
Genotype Incidence Genotype

prevalence (%)
SS 1:300 50
Sickle-Hb C 1:500 30
SB+ thalassemia 1:3000 8
SBU thalassemia 1:7000 2
Others - SD Punjab
SO Arab
NB: SS most common

SB+ Thal produces some amount of HbA. HbS > HbA
SB0 Thal produces NO HbA
Diagnosis
1. HB electrophoresis - Most
common
• Hb separation at varying pH
• Hb A2, F, A, S, C
#When reading the gel, focus on the adult forms of Hb and not HbF for results (Dr L King)
• Uses 2 types of medium for testing
a. Screening: ACID MEDIUM = Agar gel - pH 6.
b. Confirmatory: ALKALINE MEDIUM = CAM (Cellulose Acetate Membrane) - pH 8.4
Others
2. High Pressure Liquid Chromatography (HPLC)
3. Iso-electric focusing
4. Sickle Solubility Test IS NOT USED FOR DIAGNOSIS
Sodium dithionite or a similar chemical is then added to the blood sample. The sodium dithionite makes the red
blood cells “lyse” or break open, releasing the hemoglobin from inside the red blood cells into the blood plasma.
Normal hemoglobin, dissolves easily in the blood plasma, and the plasma will remain clear, though it will take on
a red color. SCD patient, hemoglobin S does not dissolve easily in blood plasma, and after the sodium dithionite
is added the plasma will become cloudy because the hemoglobin S is actually forming small crystals.
PRO CONS
Good for mass screening to determine if there is sickle Cannot differentiate between sickle cell trait or disease
or not
Cannot tell if trait is of a thalassemia nature
• NOTE WELL: ALSO CANNOT be used to reassure anyone that they will not have a child with sickle cell
disease. ***REASON: Because it doesn't detect the C gene or thalassemia! Therefore if one parent has, for
example Hb AC, it would report him as normal, while reporting his Hb AS partner as having an S gene. By these
results, it would falsely suggest that any offspring could only possibly inherit the trait. It would have completely
missed the possibility of an offspring with Hb SC, which is sickle cell disease, not the trait.
•
NOTE: HB SF is sickle cell disease until proven otherwise!! Must be treated as such
***NOTE: Symptoms unlikely before age 4 months. REASON: Protection due to presence of fetal haemoglobin until
about age 4-6 months
• NOTE: Dactylitis is the most frequent initial complication!!

• NOTE: Peak incidence of death from SCD is first three years!!!
• NOTE: Acute splenic sequestration and sepsis - highest mortality!!!
Sickle Cell is a highly variable disease:

Variability between genotypes:
• Increasing severity:
SS > SBeta0 > SC > SBeta+
Variability within genotype:

(Spectrum of benign to severe)
• Benign: Incidental finding in 60-70 year old
• Severe: Characterized by:
• Increased susceptibility to infection
• Tendency to acute splenic sequestration
• Vaso-occlusive crises
• Death in early childhood
Management
PRINCIPLES OF MANAGEMENT
1. NEWBORN SCREENING
a. Card is used to collect blood from cord
a. CARD MUST BE SOAKED FROM FRON TO BACK
b. Must be filled out with relevant detail
c. Wipe the cord to prevent maternal blood going on the card
b. Want to screen early as the peak for incidence of death is 0 – 3years of life
2. UNDER 5 YEARS
a. Health visits every 3 months, childhood immunizations
b. Train caregivers on splenic palpation
c. Pneumococcal prophylaxis
3. OVER 5 YEARS
a. Health visits every 6 months and immunizations
b. Social Issues
c. Disease counselling
4. Screen for complications:

a. BRAIN - For risk of stroke - Transcranial Doppler (TCD)
b. EYES – Proliferative Sickle Cell Retinopathy (Fundoscopy) -
c. KIDNEY – Renal Disease – (Kidney Function Tests)
d. Other methods of disease monitoring:
a. Steady state, Hb, splenic size, 02 sat
b. Leg ulcers
ALL POSSIBLE COMPLICATIONS IN SCD BY SYSTEM (Cephalic to Caudal) - Anggelos

FEVER
NEUROLOGICAL Stroke (Ischemic, Hemorrhagic), TIA
OCULAR Sickle Cell Proliferative Retinopathy
ENT Adenotonsillar Hyperplasia
PULMONARY Acute Chest Syndrome, Pulmonary HTN
ABDOMINAL Hepatic Sequestration, Cholelithiasis, Cholecystic, Splenic
Sequestration, Splenic Infarcts, Splenic Abscesses
CARDIO Left Ventricular Diastolic Dysfunction
RENAL Enuresis, Acute Papillary Necrosis, Renal Disease
GU Priapism
BONE Avascular Necrosis, [Aplastic Anemia*], Osteomyelitis, Dactylitis
Stunted Growth
VASCULATURE Vaso-occlusion (Painful Crisis, Dactylitis)
BLOOD Haemolytic Crisis, [Aplastic Anemia*]
IMMUNOLOGY Infection [Aplastic Anemia*], Sepsis
HORMONAL Delayed Puberty
SKIN Leg Ulcers
PREGNANCY Spontaneous Abortions
*Aplastic Anaemia can be placed in 3 areas. Explanation is the same.
A. FEVER IS BEING HIGHLIGHTED HERE

a. This occurs because patients with SCD have a defective immune system. It maybe the only presenting feature
and so history is very important.
b. Classified as:
i. Fever with a focus
ii. Fever without a focus
1. Can be challenging to treat
c. Usually associated with the following
i. Dactylitis
ii. ACS
iii. Walking Bateremina/Septicaemia
iv. Meningitis
v. UTI
vi. Other (Osteomyelitis)
B. Neurological
a. Due to the increase viscosity of blood and increase vaso-occlusive probability, patients with sickle have an
increased possibility of CVA
b. CVA can occur due to: Stroke, Aneurysm, Moyamoya, Silent Infarcts
i. Moyamoya Syndrome is Japanese for “puff of smoke”. It refers to a radiographical pattern seen
on angiography that is due to occlusion of the major intracerebral vessels with the development
of telangiectatic collateral vessels.
1. MRI investigation of choice
c. Strokes that occur are either ischemic or hemorrhagic
i. Ischemic strokes usually seen before age 10.
1. It has a DOUBLE PEAK time of ages 2-5 and ages 40.
ii. Hemorrhagic strokes are seen 20s and above
iii. CVAs (~11% have overt and ~20% have silent strokes before 18)
d. Area that is most affect is the Circle of Willis (Anterior, Middle and Posterior Cerebral Arteries)
e. Other pathologies
i. TIA
ii. Seizures
iii. Headaches
f. John is 6 years old with acute weakness of the left side of the body for past 3 hrs. Child has SCD.
i. Investigation
1. Best 1st step is to get a CT
2. This is needed to image the brain and determine if the stroke is ischemic or hemorrhagic
3. CT will show pathology for hemorrhagic stroke BUT maybe normal in first 48 – 72
hours for ischemic strokes
*Most likely this child has an ischemic rather than a hemorrhagic given the incidence of
each type
ii. Management of Ischemic Stroke

1. Exchange Transfusion
a. REASON: Provides all cells with oxygen and limits the damage to the brain
b. MUST BE DONE within 72 hrs of onset of symptoms
2. Patient Education
a. May reoccur
b. Locally patients are given Hydroxyurea
i. Hydroxyurea increases HbF, which has a higher oxygen capacity than
normal hemoglobin and decreases HbS polymerization
c. 1st world countries a CHRONIC EXCHANGE TRANFUSION PROGRAM.
i. Complications
1. Iron overload
2. Infection
3. Alloimmunization
g. John has a twin sister, Sarah. Can Sarah have a stroke too? If so, what is her management?
i. YES she can
ii. Management
1. Patients with increased chances of having a stoke are investigated by Transcranial Doppler
(TCD)
a. Why is it done = Used to assesses risk of stroke in children with SCD
b. Pros = Simple non-invasive test
c. How is it done = Uses the trans-temporal window to assesses the velocities in the
anterior, middle and posterior cerebral arteries
d. Readings can be:
TCD category TCD velocity (cm/s) Stroke per

Estimated
prevalence
(%)
Normal <170 74 1-2
Conditional >170 - 199 17 3-8
Abnormal >200 9 9
e. Recommended in ages 2-16. This is so because the risk of ischemic stroke falls
after this age.
2. Place on Chronic Exchange Transfusion Program (1st world) or Hydroxyurea locally
C. Ocular
a. Sickle Cell Retinopathy
i. Condition characterized by vaso-occlusion of micro-vessels of the eye under certain conditions
1. Stress
2. Hypoxia
3. Cold Temperatures
4. Acidosis
5. Dehydration
ii. Occurs mostly in HbSC (33% vs 3% in HbSS)

iii. Annual screening is recommended for ALL patients from age 10-11 with SCD.
iv. Management with laser photocoagulation
D. Pulmonary
a. Acute Chest Syndrome
i. Clinically: Including the following; chest pain, fever +/- associated respiratory symptoms
(cough, SOB)
ii. Radiographically: NEW infiltrate on CXR
1. (Predominantly involves the LEFT LOWER LOBE)
2. May find multiple infiltrates + pleural effusions on CXR
iii. Nelson’s:
1. Even in the absence of respiratory symptoms, ALL SCD PATIENTS WITH FEVER
should receive a chest radiograph to identify ACS!!!! because clinical examination alone
is insufficient to identify patients with a new radiographic density, and early detection of
acute chest syndrome will alter clinical management.
2. Given that pneumonia and sickling in the lung can both produce these symptoms, the
patient is treated for both conditions.
iv. Pathophysiology:
1. Not fully known. Infection is best known cause/embolism/fat
emobolism/sequestration/infarction
v. Aetiology for infection:

1. Atypicals such as: Pneumococcus, mycoplasma, chlamydia most common (So, infection or
anything causing vaso-occlusion in lung may cause ACS)
#Infiltrates are seen in both pneumonia and ACS BUT Pneumonia is seen in a non SCD
patient and infiltrates seen in an ACS patient because of the pathology. The pathology for
ACS is multifactorial – Dr King Dec 2017
vi. Treatment: = Triple A, Double T and a HOe - Anggelos

1. ALWAYS REMEMBER ABC
2. Oxygen monitoring +/- administration (Keep SpO2 > 95%)
3. Hydration
4. Analgesics
5. Antibiotics - Penicillin based AND macrolide - for ALL EPISODES - because of clinical
overlap with pneumonia
a. Nelson’s: As a result of the clinical overlap between pneumonia and ACS, ALL
episodes should be treated promptly with antimicrobial therapy!!, including at
least a macrolide AND a third-generation cephalosporin to treat the most
common pathogens associated with ACS, namely Streptococcus pneumoniae,
Mycoplasma pneumoniae, and Chlamydia spp.
6. Transfusion (Simple transfusion or exchange)
a. Maybe needed - Reason: Need to correct the hypoxia.
b. Nelson’s: Some indications for transfusion: = RaPID - Anggelos
i. Rapid change in respiratory effort either with or without a worsening chest
radiograph
ii. Previous history of severe ACS requiring admission to the intensive care
unit.
iii. Increase work of breathing
iv. Decreasing oxygen saturation
7. Treatment of comorbidities e.g. Asthma
E. Abdominal
a. Splenic Sequestration
i. *** An acute enlargement of the spleen associated with a fall in Hb of 2 g/dl or more below
steady state due to pooling of blood.
ii. Issues related to pathology
1. Reticulocytosis and a decrease platelets may be present
2. Recurrence is likely (~ 50% and usually within 6 months of previous episode)
3. Following episodes usually worse than the previous. Significant percentage fatal
4. Autosplenectomy - Because of its narrow vessels and function in clearing defective red
blood cells, the spleen is frequently affected. It is usually infarcted before the end of
childhood. This autosplenectomy increases the risk of infection from encapsulated
organisms
iii. Management
1. Principle is supportive with a simple transfusion as this usually resolves itself
2. If 2 episodes occur, recommendation for prophylactic splenectomy is given
3. Caregiver education
a. Teach them how to palpate the spleen (Do at least once a day)
b. Those with chronic splenomegaly, always assess the spleen as follows
i. Given a spleen stick (a tongue depressor that has on the genotype and
name of patient) to assess the size of the spleen
ii. Stick is placed at the costal margin vertically, in line with the
1. NIPPLE LINE for PREPUBERTAL patients
2. MID CLAVICULAR LINE for PUBERTAL patients
iii. A horizontal mark is made at the lowest edge of the spleen.
iv. The vertical distance from the costal margin to the lowest edge of the
spleen is the size of the spleen
v. Size is compared to previous measurements
4. If splenectomy done:
a. Need to ensure vaccines up to date
b. Continuous penicillin prophylaxis for 3 more years
5. If autosplenectomy
a. Preventive antibiotics and vaccinations are recommended for those with such
asplenia.
b. Splenic Infarction
i. More common in the milder genotypes (SC & SB+ > SS & SB0)
ii. Seen in older patients with chronic splenomegaly as well
iii. This is associated with pain
c. Cholelithiasis (Gall Stones)

i. 50% of patients with SCD have this
ii. Occurs due to increase haemolysis of RBC
iii. Types of stones formed
1. Pigment stone – Black in colour
iv. Presentations and Management
1. Asymptomatic
a. Leave alone
2. Symptomatic
a. Elective or Prophylactic cholecystectomy
F. Renal
a. Issues can start very early in patient with SCD
b. Renal complications
i. Enuresis
ii. Hyposthenuria – Inability to concentrate urine = low specific gravity.
1. Early sign of renal failure
iii. Haematuria/Papillary necrosis
iv. Proteinuria
1. Early sign of renal failure
v. Hyperkalaemia
1. Decreased potassium excretion
vi. Increased chance of UTI
vii. Risk factors for progression: Anaemia, proteinuria, haematuria Etc. Chronic renal failure
G. Genitourinary
a. Priapism
i. PAINFUL, PROLONGED, PURPOSLESS PENILE erection
1. Involuntary penile erection lasting > 30 minutes - (20% have at least 1 episode before 20)
ii. Mechanism
1. Vaso-oclusive crisis causes accumulation of blood in the corpora cavernosa from being
drained
iii. Types
1. Classified based on
a. Mechanism
i. Ischemic – Low flow (seen in SCD)
1. Most common type, results from failure of venous outflow.
Prolonged low-flow priapism leads to a painful ischemic state,
which can cause fibrosis of the corporeal smooth muscle and
cavernosal artery thrombosis. The degree of ischemia is a function
of the number of emissary veins involved and the duration of
occlusion
ii. Non ischemic - High flow
b. Timing
i. Stuttering: Last 10-15 mins - repeated over several hours
ii. Prolonged: Several hours
iii. Persistent: Last weeks to years
# Minor is < 4 hours. Major is > 4 hours. 4 hours is the cutoff for
permanent erectile dysfunction.
iv. Management:
1. Conservative (Trying to abort the episode)
a. Warm showers
b. Pass urine
c. Exercise or light activities
d. Increased fluid intake
e. Strong analgesia
NB: It is important to note how far one lives from the hospital. If priapism
continues after above mechanisms have failed, make sure enough time is left so the
hospital can be reached before 4 hours. If there is resolution on the way to the
hospital, then the patient can turn back. – Dr. L King
2. Surgical
a. Urologist attends to these cases
b. Aspiration (of blood from corpora cavernosa) and Shunting
3. Prevention: Very difficult

a. Phenyl ephedrine
i. Alpha adrenergic agonists
b. Refer to urologist if there is recurrence and medication does not work
4. In the case of erectile dysfunction

a. Use penile prosthesis
i. Balloon/Inflatable
1. Inflate for erection
ii. Rigid
1. Unbend/Unkink for erection, opposite action when done
H. Bone
a. Dactylitis (Hand-Foot Syndrome)
i. ***Avascular necrosis of the bone marrow of the proximal phalanges
ii. *** Usually first sign of SCD in infants over the age of 6 months
iii. Treatment
1. Symptomatic
a. Analgesics
b. Hydration/Fluid replacement
c. Keep warm
2. NOTE WELL: If unilateral, MUST differentiate from osteomyelitis as treatment
significantly differs. Osteomyelitis requires at least 4-6 weeks of IV antibiotics
b. Avascular Necrosis vs Osteomyelitis

i. Difficult to tell the difference between the 2
1. They may present the same way with pain, fever, erythema,
2. X-ray is not useful
a. Osteomyelitis presents after 2 weeks has passed
ii. AVN
1. Suspect in patient with SCD and has a limb
2. Manage by giving weight baring education e.g. use crotches
iii. OSTEO
1. Presence of positive aspirate and blood culture
iv. Investigations
1. CBC
2. WBC with Differentials
3. CRP
4. Blood Cultures
5. Admission
c. Aplastic Anaemia
i. APLASTIC CRISIS
1. Usually Pure red cell aplasia --> Parvovirus B19 (Also assc. with ASS, ACS, stroke,
glomerulonephritis)
2. May also have Pancytopaenia (thrombocytopaenia, lymphopaenia and neutropaenia)
ii. Pathophysiology
1. Triggered by parvovirus B19, which directly affects erythropoiesis (production of red
blood cells). Parvovirus infection nearly completely prevents red blood cell production
for two to three days. In normal individuals, this is of little consequence, but the
shortened red cell life of sickle-cell patients results in an abrupt, life-threatening
situation.
iii. Diagnosis
1. History and examination
2. CBC
a. Hb levels
b. WBC and Differentials
3. Reticulocyte count
a. Very low reticulocyte count. May be 0. The reticulocytopaenia lasts 7-10 days
4. Blood Film
a. Normal/Normochromic RBC (maybe Macrocytic)
b. Pancytopenia present
c. Abnormal cells are not usually present
5. Bone Marrow Aspirate (if done)
a. Hypocellular bone marrow showing
i. Increased fat spaces
ii. Lymph, plasma, macrophages and mast cells
iv. Management
1. SUPPORTIVE/Symptomatic management. May require transfusion if Hb very low or if
there is bleeding due to low platelets. It is self-limiting
2. Must ask if anyone else in family has SCD. If another family member has SCD, ask them to
come in as well for management. The condition is VERY contagious.
v. Additional information
1. These patients are at increased risk of acquiring nephrotic syndrome
I. Vasculature
a. Pain
i. Pathophysiology
1. Pain is the clinical hallmark of SCD and can be experienced in every part of the body
2. Vaso-occlusion leads to hypoxia, ischemia, → tissue damage followed by chronic vascular
inflammation, underlying many features of sickle cell pain. Inflammatory mediators released
from injured cells, macrophages, mast cells, and platelets activate nociceptors on the
peripheral afferent, thus initiating the nociceptive insult. It is the combination of
hypoxia/reperfusion injury, ischemic tissue damage, and inflammation that makes the
pain of SCD unique
ii. Types
1. Acute – The most common cause of hospitalization
a. Acute painful crises - Risk factors
i. Gender - Males > Females
ii. Age - 15-29 years
iii. Genotype – SS and SBeta° Thal
iv. Haematology
1. High Hb
2. Low fetal Hb concentration
v. Other
1. Last trimester of pregnancy
NB: Crises are more painful in the presence of malignancies
2. Chronic -
3. Neuropathic – Associate with leg ulcers. Normal pain killers will not work
ACUTE PAIN CHRONIC PAIN OTHER CAUSES

• Dactylitis • Osteomyelitis • Cholecystitis
• Painful crisis • Avascular necrosis of the femoral and humeral • Peptic ulcer disease
• Acute chest syndrome • Acute surgical
• Hepatic sequestration • Leg ulcers • Withdrawal
abdomen from
• Hepatic crisis • Arthropathy • Loose
opiateship or shoulder
• Splenic sequestration • Trauma
prosthesis
iii. Syndromes of pain??
iv. Precipitators of pain

1. Dehydration
2. Infection
3. Acidosis
4. Change in weather (Cold)
5. Hypoxia
6. Prolonged swimming
7. Psychological stressors
v. Management of pain
1. Approach = ABCDE
a. Access the pain (Use a pain chart)
b. Believe the patient
c. Choose correct medication
d. Deliver/Dispense medication in timely fashion
i. By MOUTH = Best route
ii. By LADDER = Best approach
iii. By CLOCK = Keeps therapeutic levels appropriate
e. Empower/Educate patient and family
2. WHO 3-Step ladder is implemented
a. STEP 1 = MILD
i. Non-opioids: Paracetamol (Acetaminophen), NSAIDs
b. STEP 2 = MILD TO MODERATE
i. Opioids for mild-moderate pain: Codeine +/- paracetamol, Tramadol
(atypical opioid analgesic), Dextropropoxyphene + Paracetamol
c. STEP 3 = MODERATE TO SEVERE
i. Opioids for moderate to severe pain: Morphine, Oxycodone, Fentanyl
Diamorphine, Hydromorphone, Methadone
#ALWAYS STEP 1 UP AT A TIME THEN STEP BACK DOWN
3. Adjuvant therapy
Adjuvant tx such as antihistamines,
a. Anti-histamines antidepressants, benzodiazepines and
i. Decreases itching caused by opioids anticonvulsants are heterogenous
ii. Makes opioids more effective compunds that potentiate the analgesic
effect of opioids
b. Anti-emetics
i. Decreases N/V caused by opioids
c. Anti-depressants
i. Relieves neuropathic pain e.g. Amitriptyline
d. Anticonvulsants (all seizure medications can be used for neuropathic pain except Ethuxomide)
i. Relieves neuropathic pain e.g Gabapentin, Lamotrigine
e. Laxatives
i. Relieves constipation caused by chronic opioid usage
J. Immunology
a. Infections
i. Usually encapsulated organisms - REASON: Due to absence of a functional spleen - functional
hyposplenia or asplenia
ii. Pathophysiology
1. Patients lose the ability to get rid of organisms by splenic macrophages. Normally, C3b or
IgG would opsonize these organisms and send them to the spleen for destruction, but because
the spleen is nonfunctional, they are unable to deal with organisms.
iii. The encapsulated organisms

1. NOTE: Online Source: One of the most high yield mnemonics in microbiology is the one for
encapsulated organisms: "Some Nasty Killers Have Some Capsule Protection"
2. Streptococcus pneumoniae
3. Neisseria meningitides
4. Klebsiella pneumoniae
5. Haemophilus influenzae
6. Salmonella typhi
7. Cryptococcus neoformans
8. Pseudomonas aeruginosa
iv. Management
1. Important Organisms
a. Pneumococcus
i. Prophylaxis FOR THOSE WITH SEVER GENOTYPE
1. Prophylactic therapy with penicillin has been advocated in
recognition of the fact that a majority of the causative organisms
are sensitive to penicillin.
2. Age: 4 months to 4 years
3. Options: (One or the other, NOT BOTH)
a. IM: Penadur® -> Benzathine Penicillin G -> Given
every 28 days NOT every month
b. ORAL: Penicillin B. Given every day, twice a day (PO
bd)
i. Morning and evening
ii. Offered to the severe genotypes
iii. If allergic to penicillin give erythromycin
ii. Vaccination FOR EVERYONE
1. Always say "prophylaxis is given as a conjugate or
polysaccharide" and then use brands as examples. This is so
because other countries do not necessarily use these brands. -
May Pen Dr Pryce and Dr Griffiths – Anggelos
2. Types
a. CONJUGATE such as PCV - Pneumococcal
Conjugate Vaccine (Prevnar®)
i. Covalently bonded and gives T-cell mediated
response → Offers memory. Therefore, it is
given to the younger child.
ii. Given before 4 years old
iii. Given with the pentavalent schedule (6-3-6)
iv. Given 2-4-6 in US
v. Prevnar 13® is a 13-valent vaccine
b. POLYSACCHARIDE such as- Pneumococcal

Polysaccharide Vaccine 23
i. B-cell mediated response → Doesn't confer
memory
ii. Given at 4 and repeated every 7 years.
#In the US it is given at 2, but Sickle Cell
Unit states better effect seen at 4 years old
3. NB: Pneumococcal vaccine is provided for SCD, HIV and
Down’s by MOH. But not provided routinely. Cost about $8000
4. Online source: Immunization with broadly polyvalent vaccines
against Streptococcus pneumoniae, Haemophilus influenzae type
b, and Neisseria meningitidis may ultimately represent the most
effective way to reduce the incidence of catastrophic infections.
iii. Eventually health visits every 6 months

b. H. influenzae
i. This is given in the normal schedule for vaccination in the 6 week, 3
moths, 6 months pattern.
c. Salmonella
i. GENERAL
1. Hygiene: Esp. hand washing after handling food esp. chicken and
eggs.
2. Cooking: Must thoroughly cook eggs as well. No licking cake
batter. No runny eggs no sunny side up eggs. Must cook
thoroughly.
3. Pets/Animals: Can also get salmonella from pet lizards or reptiles
ii. MEDICAL
1. Amoxicillin, Arimethoprim-sulfamethoxazole (TMP-
SMZ)/Bactrim.
2. In areas with multidrug resistance, Cefotaxime or Ceftriaxone
d. E.coli – UTI
IMMUNIZATIONS
Additional immunizations for sticklers:
PCV Schedule (Prevnar 13®): 2, 4 and 6 mo OR 6 wks, 3 mo, and 4 mo, then 15-18 months
Age Standard Recommended immunizations

Existing Required
BCG Hib/Hep MMR DPT/DT Hib PCV PPV 23 Varicella Meningococcal
& & OPV booster MCV
OPV or IPV
6/52 X
2/12 X X X
4/12 X X X
6/12 X X X
. year X X
“15- X X X X
18/12
4-5 X X X X
years
Hib & DT booster every 10 years
PPV 23 booster every 7 years
Flu vaccine recommended annually
K. Skin
a. Leg Ulcers
i. They are neither absolute venous or absolute arterial. They are a mixture
ii. Usually seen in young adult patients (30%)
iii. Difficult to treat once it becomes chronic
iv. Management
NO ULCER
1. Prevention – THE BEST STEP
a. Good skin care
b. Moisturize
#MILD TRAUMA MAY CAUSE ULCERS
IN CASE OF AN ULCER
2. Conservative
a. Clean
b. Daily dressing and elastic bandaging
c. Elevation of leg
d. Bed rest
e. Wear proper shoes
3. Pharmaceutical
a. Possible zinc supplement may increase healing rate
4. Surgical
a. Skin grafting – This has not proven to work locally. The grafts are put in place in
hospital. While the patient is in bed rest, the grafts are efficient. Once the patient is
discharged and active, the grafts deteriorate. – Dr L King Dec 2017
v. Depression due to stigma may occur. Be mindful of this. This a possible complication of any ulcer.
MODIFYING/CURING SCD
Options for CURE:
1. Hb F therapy: See moa
2. BM transplant: See moa
3. Gene therapy in future
Hb F
• High Hb F concentration is associated with a milder course
• High Hb F levels inhibits deoxygenated Hb S polymer formation.
• Hydroxyurea
o The only drug FDA approved for use in SCD at this time
o Ribonucleotide reductase inhibitor which alters the maturation of erythroid precursors and promotes Hb F
production indirectly
o Indications in Jamaica = PASS
Hb SS or Hb SBthal AND one or more of the following:
▪ Painful crises (recurrent)
▪ Acute chest syndrome (recurrent)
▪ Severe symptomatic anaemia
▪ Stroke: 2 indications reasons:
i. Abnormal TCD — to prevent the FIRST stroke
ii. Prevent recurrence
NOTE - KNOW THESE WELL!!!!:

1. Gold standard for treatment for persons who have had a stroke. Once haemorrhagic stroke ruled out:
a. EXCHANGE TRANSFUSION WITHIN 48 HOURS FROM ONSET OF SYMPTOMS IDEALLY
2. Gold standard for child with first stroke to minimize risk of recurrent stroke:
a. LIFELONG CHRONIC EXCHANGE TRANSFUSION. Every 2-3 months. To keep Hb S below 30%!!!
In mostly 1st world. Has the problems of iron overload
b. But in Jamaica can't sustain this so use HYDROXYUREA. Exchange transfusion NOT offered in Ja.
3. Gold standard for abnormal TCD is the same as trying to prevent the first stroke:
a. CHRONIC EXCHANGE TRANSFUSION
Others: Inc. serum Creatinine, hepatitis
Decreases the following by 50%:

1. Painful crisis episodes
2. ACS episodes
3. Blood transfusions
Bone marrow and stem cell transplant

1. In first world
2. Reserved for severe cases
3. Best done in children
4. Significant risk of mortality: 1 in 10 will die
Paediatrics Topic: Paediatric Surgery Source: Toronto Notes 2014 (Copy) November 2014
Table 22. Pediatric Surgery

Condition Epidemiology and Pathophysiology Clinical Features and Physical Investigations Treatment Prognosis
Risk Factors History
Hydrocele ( see 1-2% of live births Communicating Painless scrotal mass Transillumination U/S if suspect Most resolve < 2% recurrence
Urolony. U28) Present at birth, hydroceles: processus Communicating suggests hydrocele pathology spontaneously by 1 yr
majority close vaginalis fails to dose hydroceles increase Silk glove sign: gently Surgical repair if:
spontaneous by 1 yr with small opening for in size with standing palpating hydrocele - Persistence > 2 yr
M:F = 6:1 fluid to move freely or Valsalva, may be sac over pubic - Pain
Prematurity between peritoneal cavity absent in the morning tubercle feels like - Fluctuating in size
through patent processus and large in the mbbing silk on silk which suggests
(if opening progresses evening communication
to allow passage of - Cosmetic reasons
intestine, it is a hernia ) - Infection
Noncommunicating
hydroceles: fluid trapped
in tunica vaginalis; in
older children, may be
secondary to testicular
pathology (reactive
hydrocele )
Hypertrophic 0.03- 1.0% of live births Acquired pyloric circular Projectile non-bilious Smooth oblong Electrolytes ( assess Fluid resuscitate with Pyloromyotomy
Pylo ric Stenosis Can present at muscle hypertrophy vomiting 1-2 cm mass palpable hypochloremia, normal saline, correct curative
1-20 wk, most results in gastric outlet Vomiting above umbilicus, dehydration) electrolyte and acid/
commonly at 6-8 wk obstruction 30-60 min after feeds "olive'' U/S shows pyloric base abnormalities with
M:F = 4:1 Hypovolemia caused by Hungry after vomiting Visible left-to-right length > 14 mm, D5, 1/2NS + 20 mEq/L
Early erythromycin emesis of gastric content Dehydration ( variable gastric contraction muscle thickness KCI at maintenance rate.
exposure ( < 13 d old ) causes hypochloremic severity ) "waves" after feeding > 4 mm NG tube decompression
hypokalemic metabolic Upper Gl series unnecessary
alkalosis. Electrolyte necessary only when Pyloromyotomy,
exchange based volume U/S unavailable or non- open (Ramstedt vs.
retention in kidneys diagnostic will show transumbilical or
results in paradoxical "string sign’ laparoscopic approach)
aciduria Alternative therapies such
as TPN/wait or atropine
impractical due to long
time course of effect
Congenital 1 in 2000 to 5000 live Left-sided: small bowel, Early respiratory Decreased air entry Prenatal US/MRI Intubate Later presentations
Diaphragmatic births large bowel, stomach distress ± bowel sounds in ABG Orogastric suction have better outcomes
Hernias Presents within hours and solid viscera Cyanosis the chest CXR ( bowel loops in Period of respiratory Hearing deficit (40%)
3 types: of life although some (spleen, left lobe of liver) Scaphoid abdomen Displaced heart hemithorax, shifted stabilization due to Associated GERD
- Posterolateral cases of delayed herniate into thorax Prenatal diagnosis sounds heart) associated pulmonary MSK defects - chest
(Bochdalek) presentation Echocardiography hypoplasia (may require wall and scoliotic
- Left-sided, 85% M = F Right- sided: liver, large Genetic consultation if extracorporeal membrane defects a potential
- Right- sided, > 10% are associated bowel herniate into warranted oxygenation). complication of
13% with other congenital thorax Surgical repair after thoracotomy.
- Bilateral, rare, anomalies Pulmonary hypoplasia stable by hernia
often fatal Prenatal diagnosis Pulmonary hypertension reduction and closure of Need for long term
- Anterior common diaphragmatic defect - surveillance for
(Morgagni) open vs. thoracoscopic potential recurrence
- Hiatus vs. laparoscopic with Failure to thrive
or without prosthetic or Chronic lung disease
muscular patch if severe hypoplasia
depending on size of
defect
Meckel's 1 -3% of population Failure of vitelline duct Bright red blood per Tenderness (lower AXR Stabilize, resection Resection curative
Diverticulum M:F = 3:1 to regress 5- 7 wk in rectum ( heterotopic abdomen) near Meckel scan: scan for by laparotomy or
Most common Present most frequently utero; 50% contain gastric mucosa in umbilicus ectopic gastric mucosa laparoscopy ± incidental
remnant of vitelline first 5 yr of life heterotopic tissue Meckel's causing with technetium appendectomy
duct that connects Symptomatic in 2% (e.g. gastric mucosa, mucosal ulceration and Tc99m pertechnetate
yolk sac with of cases ectopic pancreas); bleeding in adjacent IV ( sensitivity 85%,
primitive midgut other associated small bowel mucosa) specificity 95%)
anomalies include Abdominal sepsis
omphalomesenteric (Meckel's diverticulitis
fistula, umbilical sinus, ± perforation)
umbilical cyst, fibrous Small bowel volvulus
band around fibrous band
Malrotation 1:500 live births Failure of gut to normally Bilious emesis is THE Bilious drainage from AXR : obstruction of IV antibiotics Mortality related
1/3 present by 1 wk of rotate around superior cardinal sign, NG tube proximal small bowel, Fluid resuscitation to length of bowel
age, #4 by 1 mo of age, mesenteric artery with especially if abdomen Tachycardic, pale double-bubble sign, EMERGENT LAPAROTOMY loss: 10% necrosis
90% by 1 yr of age associated abnormal nondistended. If Diaphoretic intestinal wall thickened Ladd procedure: - 100% survival
M:F = 1:1; higher intestinal attachments bilious emesis in ill Flat abdomen Immediate UGI: counterclockwise reduction rate, 75% necrosis
incidence among and anatomic positions child with distend Tenderness dilated duodenum, of midgut volvulus, - 35% survival rate
patients with cardiac Represent a spectrum of abdomen, consider duodenojejunal division of Ladd's bands, Recurrence 2-6 %
anomalies, heterotaxy rotational abnormalities surgical exploration segment (Ligament division of peritoneal
syndromes including complete non- to rule out volvulus. of Treitz) right of attachments between
rotation which is not at Rectal bleed (late/o midline and not fixed cecum and abdominal
high risk for volvulus) minous signs) posteriorly over spinal wall that obstruct
Intermittent column, “ corkscrew" duodenum, broadening
symptoms sign indicating volvulus of the mesentery (open
U/S: “ whirlpool" sign, folded mesentery like
abnormal SMA/SMV a book and divide
relationship indicates congenital adhesions), ±
UGI to rule out appendectomy
rotational anomalies Positioning the bowel into
non-rotation ( small bowel
in right abdomen, large
bowel in left abdomen)
Gastroschisis 1:2000 live births Defect of abdominal wall, Not associated with Hollow viscera Prenatal ultrasound, NG decompression > 90% survival rate
Antenatal diagnosis with free extrusion of genetic syndromes stomach, small and elevated MS- AFP IV fluids
common intestine into amniotic 10% with intestinal arge bowels) IV antibiotics
Increases with younger cavity atresia Defect lateral to cord Keep viscera moist and
maternal age and No specific environmental Some cases usually right ) protected until surgical
associated with IUGR factor identified associated with short Bowel may be reduction with primary
M:F = 1:1 Defect in embryogenesis bowel syndrome due inflamed, thickened, abdominal closure or
unclear to antenatal volvulus matted, foreshortened staged closure with silo
and necrosis of Defect size variable May have bowel
herniated bowel dysmotility requiring
motility medications
Omphalocele 1:5000 live birth Defect of abdominal Associated with Hollow viscera Prenatal ultrasound NG decompression 40-70% survival
Antenatal diagnosis wall, with extrusion of genetic syndromes stomach, small and Elevated MS- AFP IV fluids rate
common sac covered viscera 30-70% ( e.g. arge bowels, often IV antibiotics Higher survival
Lower gestational age (amnion, Wharton's jelly, Pentalogy of liver) Small defect ( <2 cm): rates most likely
Increased maternal age peritoneum) Cantrell, congenital Cord on the sac Primary closure related to antenatal
M:F = 1.5:1 Duhamel' s theory - heart disease, Medium (2-4 cm) and mortality of
failure of body wall Beckwith-Wiedemann large ( > 4 cm) defects fetuses with giant
morphogenesis syndrome) best treated with silver omphaloceles
Associated pulmonary sulfadiazine to promote
hypoplasia epithelialization coupled
with compression dressing
to allow gradual reduction,
followed by future repair
with or without mesh
Umbilical Hernias Incidence 2-14% Incomplete closure of Majority Protrusion from None if uncomplicated Repair if not Low risk of
Increases with peritoneal and fascial aspptomatic umbilicus Important to spontaneously closed recurrence
prematurity Decreases layers within umbilicus Majority differentiate from less by age 5
with increasing age by 5 yr spontaneously resolve common abdominal Earlier repair of large
by age 5 wall hernias that don't “ proboscoid" hernias with
Incarceration prior to spontaneously resolve extensive skin stretching
age 5 very rare (e.g. epigastric may be warranted for
Most symptoms occur hernias) cosmetic reasons
in late adolescence or Most umbilical fascial Simple primary closure of
adulthood defects > 1.5 cm in fascial defect
infancy will not close
spontaneously
Intestinal Atresia Incidence 2-14% Duodenal - failure of Gastric distension Complete physical Contrast enema ± UGI NPO Long term survival
May be antenatally bowel to recanalize after and vomiting (usually Special notice to with small bowel follow NG tube decompression Duodenal - 86%
diagnosed by dilated endodermal epithelium bilious) abdominal exam through (SBFT) Fluid rescuscitate Jejunal/ileal - 84%
bowel loops or "double- proliferation (wk 8- 10) Duodenal - may Perineum and anus Group and screen TPN Colonic - 100%
bubble" sign on x-ray for JejunaMeal - acquired be associated with Include evaluation of INR and PTT if for Broad spectrum
duodenal atresia as result of vascular other anomalies respiratory distress surgery antibiotics
Decreasing with disruption -» ischemic (tracheoesophageal and signs of volume Duodenal -
increasing age necrosis -» resorption of fistula, cardiac, depletion duodenoduodenostomy or
necrotic tissue -* blind renal and vertebral Congenital anomalies duodenojejunostomy
distal and proximal ends anomalies ), 24-28% Jaundice Jejunal/ileal - primary
Colon mechanism
-
have Down syndrome anastomosis; or if
unknown, thought to be Jejunal/ileal - within atresia associated with
similar to small bowel 2 d of birth, may be short bowel then may
atresia associated with cystic create end stoma or
fibrosis defer surgery for bowel
Colonic within 3 d
- lengthening procedures
of birth Colonic - primary
anastomosis
Hirschsprung's 1 :5000 births Defect in migration Failure to pass ± abdominal Rectal biopsy ( gold Surgical resection Most have normal
Disease M:F = 3:14:1, of neurocrest cells to meconium distension standard) - look for of aganglionic near-normal
approaches 1:1 when intestine resulting in spontaneously within Squirt/blast sign aganglionosis and intestinal segment and anorectal function
whole colon involved aganglionic bowel that 48 h of life is the neural hypertrophy anastomosis of remaining Complications:
Can have aganglionosis fails to peristalse and classic history (95% of AXR intestine to anus Fecal incontinence
of small bowel as well internal sphincter that normal children should Contrast enema to find Either in newborn period and constipation,
Familial Hirschsprung's fails to relax (internal pass meconium narrow rectum and or staged if extensive post- operative
in < 5% of cases anal sphincter achalasia) within 24 h, and the transition zone. Anal aganglionosis enterocolitis
causing functional and remaining 5% within manometry unreliable (medical
partial mechanical 48 h). in infants - classic emergency if
obstruction, respectively; Symptoms of finding is absence of progresses to
always starts in the bowel obstruction: rectoanal inhibitory sepsis)
rectum and variable abdominal distension, reflex
involvement proximally; constipation, bilious
RET mutation emesis
Enterocolitis/sepsis
Failure to thrive
Cryptorchidism 2-5% of term males - Idiopathic Palpable testicle Bi-annual testicular Depends on age of hCG to stimulate Orchidopexy
most of these descend Descent is mediated within inguinal canal exam with palpation presentation testosterone production Decreased risk of
spontaneously by 6 mo by descendin which is or testicle which Distinguish truly U/S or MRI exam if no and descent torsion and blunt
of age created in response to can be milked down undescended testis palpable testis Orchidopexy - especially trauma to testicle
1% of males do not testosterone into scrotum ( called from retractile testis Older child: LH, FSH, if undescended by age No effect on
spontaneously descend Descent usually begins retractile testis) ( which is "high" testis MIS, hCG stimulation 6 mo-2 yr malignant potential
at 28 wk Occasionally no due to hyperactive test for gonadotropin of testicle
palpable testis as it is cremasteric muscles) production Descent can
intra-abdominal nfant: U/S, FSH, preserve
Consider other LH, karyotype, spermatogenesis if
congenital MIS, 17-Hydroxy- performed by 1 yr
abnormalities pfogesterone of age
1/1000 risk for
testicular cancer
(population risk is
1/ 4000 )
Inguinal Hernias 5% of all term newborns All infant hernias are Most common Palpate for 'bag of Physical exam is gold Manual reduction - to Risk of recurrence
2x risk and more likely indirect: Descent of presentation: painless worms" suggests standard relieve acute symptoms after surgical
bilateral if pre -term intra-abdominal contents intermittent mass possible testicular U/S only if physical Hemiorraphy - definitive reduction <3%
More common in males through the internal in groin, may also varicocele exam uncertain (e.g. treatment by reduction of but higher if repair
(4:1) inguinal ring through a note extension into Biannual testicular in small infants where herniated contents and done in premature
Low birth weight patent tunica vaginalis scrotum ( scrotal mass exam + palpation exam can be difficult) high ligation of sac for infants or if hernia
increases risk in absence of inguinal along inguinal canal indirect hernias was incarcerated/
1/5 inguinal hernias will mass is a hydrocele) to evaluate for any Laparoscopic or open strangulated at
become incarcerated if If incarcerated: masses techniques repair
patient is < 1 yrold tender, vomiting, firm “Silk sign' - palpable
Incarceration is more mass, erythema then thickening of cord
common in females cyanosis of mass may Mass palpated at
Associated with other be noted external inguinal ring
conditions: androgen and reducible through
insensitivity, connective inguinal canal into
tissue diseases abdomen
Must always try
reduction to confirm
that hernia is not
incarcerated
Rule of 2s for Meckel' s Diverticulum

• 2 % of the population
• 2: 1 male- to-female ratio
• Symptomatic in 2 % of cases
• Found within 2 feet ( 10- 90 cm ) of the
ileocecal ( 1C ) valve
Hypertrophic Pyloric Stenosis • 2 inches in length
Non-bilious emesis in infant is the
Bilious vomiting in infant is a life-
• 2 inches in diameter
classic presentation. threatening emergency secondary to • 2 types of tissue ( gastric, pancreatic )
midgut volvulus until proven otherwise. • Often present by 2 yr of age
Paediatrics
UTI Notes – VERY important topic to know completely!
Source: Nelson’s (Ch. 532), Toronto, UHWI UTI management guidelines
September 2014
INCIDENCE
• 1-3% of girls – 1st UTI usually by age 5

• 1% of boys – Most UTIs occur in 1st year of life
• 7% of infants and young children who present with fever
• During 1st year of life: M > F – 3-5:1

• Beyond 1-2 years: F > M – 1:10
[Only 2% of children with renal insufficiency report a history of UTI]
AETIOLOGY
**LOOK UP ORGANISMS BY AGE GROUP (PROBABLY FROM A UHWI LECTURE)**
Remember: Viruses, esp. Adenovirus, can also cause UTI
• **Majority (>95%) have a mono-microbic cause with E. coli identified as the causative agent most of the
time (~70%)
o Polymicrobial infections common in those with structural abnormalities
• Mainly caused by COLONIC bacteria

o Girls -> 75-90% due to E. coli. Then klebsiella (2nd) and Proteus spp.
• Gram-negative bacilli: E. coli, Klebsiella, Proteus, Enterobacter, Pseudomonas

• Gram-positive cocci: S. saprophyticus, Enterococcus
• VIRAL aetiologies also possible, esp. with cystitis: Adenovirus
CLASSIFICATION
3 basic forms of UTI (Based on UHWI document – so this classification is more from a management perspective):
1. Pyelonephritis – Febrile bacteriuria with systemic symptoms (vomiting, renal angle tenderness etc.)
2. Cystitis – UTI without systemic symptoms but with lower tract symptoms and signs (dysuria, frequency, etc.)
3. Asymptomatic bacteriuria – Significant bacteriuria without any symptoms
Pyelonephritis and Cystitis:

• Pyelonephritis: Inflammation of renal pelvis and parenchyma
• Cystitis: Inflammation of the bladder AND urethra
Asymptomatic bacteriuria:
• Positive urine culture without any manifestations of infection
• More common in girls
• RARE in boys
• Benign and causes no renal injury. EXCEPT in pregnancy where it may progress to a symptomatic UTI
PATHOGENESIS
• Ascending infections = Most UTIs

o FROM ANUS - Bacteria arise from faecal flora -> colonize perineum -> enter urethra -> enter bladder ->
some cases ascend to kidney
o FROM PENIS - In uncircumcised boys, bacterial pathogens arise from the flora beneath the prepuce•
• Haematogenous spread - Rarely causes renal infection
NB: Pyelonephritis can result in renal injury and scarring. Risk highest in age < 2
RISK FACTORS
VACTERL stands for vertebral defects, anal atresia,
cardiac defects, tracheo-esophageal fistula, renal
A. HOST FACTORS anomalies, and limb abnormalities. People diagnosed with
VACTERL association typically have at least 3 of these
characteristic features. They are increased risk pf UTI.
• Non-modifiable: Female gender, previous UTIs, family history
• Modifiable:
o Urinary tract abnormalities (vesicoureteral reflux*, neurogenic bladder, obstructive uropathy -> stasis,
posterior urethral valves*)
o Uncircumcised males
o Labial adhesions
o Urethral catheterization/instrumentation Eg. for voiding cystourethrogram
o Dysfunctional voiding
o Sexually active
o Constipation -> voiding dysfunction
o Toilet training
o Wiping from back to front
NOTE WELL:
• ** Vesicoureteral reflux is a risk factor for pyelonephritis NOT cystitis
• ** Breast-fed infants have less UTIs than formula fed
• ** Grade III, IV or V VUR + Febrile UTI = 90% have acute pyelonephritis Put grades here
B. BACTERIAL FACTORS
• Bacteria with P fimbriae
[***Unlikely that we would ever need to know this: Basically have an idea that there are 2 types of fimbriae. Type 1 and
type 2. Most E coli strains have type 1, few have type 2. Type 1 has no role in pyelonephritis. Type 2 aka. P fimbriae
attach to receptors on uroepithelium and RBCs. Up to 96% of pyelonephritogenic strains have P fimbriae]
CLINICAL FEATURES
Say this: Clinical features depend on **AGE GROUP of patient and **TYPE of UTI
GENERAL FOR UTIs

HISTORY
• Infants and young child: Often just fever or non-specific symptoms (poor feeding, irritability, FTT
(Failure to Trive)***jaundice if < 28 d old, vomiting)
• Older child: Fever, urinary symptoms (dysuria, urgency, frequency, incontinence, hematuria), abdominal
and/or flank pain. I.E. THEY HAVE SYMPTOMS SIMILAR TO THAT OF ADULTS
UHWI document:
• Straining + constipation + urge incontinence + secondary diurnal enuresis + encopresis -> suggest dysfunctional
elimination syndrome
• Poor urinary stream – MUST ASK IN HISTORY!!! – May indicate posterior urethral valves
• Poor stream + gait disturbance + spinal cord problems -> suggest possible neurogenic bladder
EXAMINATION
• Infants and young child: Toxic vs. non-toxic, febrile, **FTT, jaundice; look for external genitalia
abnormalities (phimosis, labial adhesions) and lower back signs of occult myelodysplasia (e.g. hair tufts),
which may be associated with neurogenic bladder
• Older child: Febrile, suprapubic and/or CVA tenderness, abdominal mass (enlarged bladder or kidney); may
present with short stature, FTT (**remember to assess growth parameters) or **hypertension secondary to renal
scarring from previously unrecognized or recurrent UTIs
SPECIFIC:
• PYELONEPHRITIS:
Characterized by any or all of:
o Fever [FEVER MAY BE THE ONLY MANIFESTATION!!!]
o Abdominal, back or flank pain
o Malaise
o Nausea
o Vomiting
o +/- diarrhoea
o NEWBORNS: Non-specific symptoms – poor feeding, irritability, jaundice and weight loss
o These symptoms indicate bacterial involvement of the UPPER URINARY TRACT

o Involvement of the renal parenchyma is termed acute pyelonephritis
o No parenchymal involvement (ie. pelvis only) = pyelitis
o ***Acute pyelonephritis can result in renal injury -> PYELONEPHRITIC SCARRING
o Renal abscess can also occur following pyelonephritis OR may be secondary to a primary bacteraemia (S.
aureus)
o Perinephric abscess can occur secondary direct spread from infection in the perirenal area incl. vertebral
osteomyelitis, psoas abscess OR from pyelonephritis that dissects into renal capsule
• CYTITIS:
o Dysuria, urgency, frequency, suprapubic pain, incontinence, malodorous urine

o IMPORTANT: CYSTITIS DOES NOT CAUSE FEVER and does NOT RESULT IN RENAL INJURY
o Acute haemorrhagic cystitis --> often due to E. coli. Also ADENOVIRUS TYPES 11 and 21
o Adenovirus more common in boys. Self-limiting with haematuria < 4 days
INVESTIGATIONS AND DIAGNOSIS

[Nelsons, Toronto an UHWI document]
(Must know this perfectly. See details in the UHWI document)
GOLD STANDARD FOR DIAGNOSIS: URINE CULTURE NECESSARY FOR CONFIRMATION AND
APPROPRIATE THERAPY
1. Urine C&S/R – Gold standard – See interpretation below

2. Urine microscopy: Bacteria and leucocytes, microscopic haematuria. White blood cell casts suggests renal
involvement BUT RARELY SEEN
3. Urinalysis/Dipstick: Leukocyte esterase, nitrites, erythrocytes
4. CBC
5. U&Es
6. +/- Blood culture, septic screen if sepsis suspected
** 3 MAIN WAYS OF URINE COLLECTION
1. Bladder tap/Suprapubic aspirate (SPA)

• Percuss first! Do not perform if not percussable. Apply Betadine + alcohol
• Can use UP TO AGE 2. Reason: After this, bladder descends into pelvis!!
2. Voided urine (MSU, clean catch urine)

• In toilet-trained
• Clean external genitalia first
• In uncircumsized boys -> MUST retract prepuce!
3. Catheter Sample of Urine (CSU)

• If not toilet trained
• Can use for any age (?)
• Clean with betadine (see details in UHWI document)
CULTURE-based diagnosis (not microscopy):
Significant bacteriuria:
1. SPA = Any growth
2. CSU = ≥ 104 organisms/ml
3. MSU = > 105 organisms/ml. (Between 104 – 105 = suspicious) – (***2 samples with same organism, same sensitivity)
Sterile pyuria = Positive leucocytes, negative culture (ie. Pus/WBCs in urine but no infection)
TIMING FOR ASSESSMENT: Assess urine promptly OR refrigerate specimen as minor contamination can overgrow
Acute renal infection:

• Increased WBC, neutrophilia, increased ESR and CRP
• Renal abscess: WCC markedly elevated
• Sepsis is common in pyelonephritis so blood cultures & sepsis screen should be considered
UHWI DOCUMENT:
MUST KNOW WELL: FURTHER INVESTIGATIONS
ALL CHILDREN IRRESPECTIVE OF AGE SHOULD BE INVESTIGATED AFTER THE FIRST UTI
**NOTE: This is separate from (ie. in addition to) the investigations to diagnose the UTI. These investigations are
to detect abnormalities of the urinary tract that may be predisposing the patient to UTI**
These further investigations mainly depend on whether the child is < 5 years old or ≥ 5 years old
P.T.O
AGE < 5 at first UTI
1. Renal U/S – FOR ALL. Renal length and looking for anatomical abnormalities
2. MCUG – FOR ALL. Look for dilatation of posterior urethra in boys (suggesting PUV), VUR, “spinning top
urethra” -> unstable bladder, “Christmas tree” appearance -> neurogenic bladder
3. +/- Nuclear scan – evaluate scarring
AGE ≥ 5 at first UTI
1. Renal U/S – FOR ALL

2. MCUG – FOR ALL MALES. Or if female with abnormal history, exam or U/S
3. +/- Nuclear scan – evaluate scarring
Nuclear scan options:

Indications: Febrile UTI, recurrent febrile UTI, abnormal MCUG or U/S, follow-up of VUR, **repeat in 6 months if
initial scan abnormal!
Scarring only:
1. Gold standard – 99 Tc DMSA (dimercaptosuccinic acid) – NOT AVAILABLE LOCALLY
Scarring and function:

2. 99Tc GC scan (Glucoheptonate) – 1st choice locally
3. 99Tc MAG 3 scan – 2nd choice locally
MANAGEMENT
READ THIS ALL THE WAY TO THE END!! MUST KNOW THE FOLLOW UP AND PROPHYLAXIS!!
***Remember NEVER to give antibiotics in UTIs for less than 10 days in a paediatric patient***
3 main groups:
1. Afebrile, asymptomatic
2. Afebrile, symptomatic
3. Febrile – Outpatient vs. Inpatient
AFEBRILE ASYMPTOMATIC UTI:

All properly diagnosed UTI should be treated even if asymptomatic , and detected during the
monitoring of a child on UTI prophylaxis for urological pathology.
If UTI is asymptomatic and not based on a bladder sample (CSU or bladder tap ) the diagnosis
should be ideally confirmed by a second voided sample showing the same organism and
same sensitivity before treatment is started. _
• If parenteral antibiotics are the only treatment option the diagnosis should be confirmed
with a bladder sample of urine.
• Treatment 10 days
AFEBRILE SYMPTOMATIC UTI - OFFENSIVE URINE, HAEMATURIA, STRAINING - DRUGS (TABLE 1)

• Oral broad spectrum antibiotics initially
In infants > 3 months old - < 1 year - Amoxicillin / Clavulanic acid is a good first choice
single agent
Infants 1 - < 12 years Trimethoprim / sulfamethoxasole could be used interchangeably with
Amoxicillin / Clavulanic acid for the first UTI
• Infants with previous UTI - treatment should ideally be based on the sensitivity pattern of
the most recent uropathogen
• Duration 10 days
FEBRILE UTI - PRESUMED ACUTE PYELONEPHRITIS ( APN) DRUGS (TABLE 2)
OUT-PATIENT MANAGEMENT!
• Non-toxic children and infants > 3 months old
• If compliance expected
• Drugs / route of administration
Initial 2 days of long acting 3rd generation Cephalosporin ( IM Ceftriaxone) or od
Aminoglycoside, then oral antibiotics based on sensitivity of organism to complete 10
days of treatment, OR
Infants and children age > 3 months who do not appear septic and are not vomiting may
be treated orally for 10 days
IN-PATIENT MANAGEMENT :
All infants < 3 months old and children systemically ill or immunocompromised
Children unable to tolerate oral medication
• Concern re: compliance
DRUGS:
Amoxicillin / Clavulanic acid combined with an Aminoglycoside ( Aminoglycoside may be
given o.d).
• Or 3rd generation Cephalosporin e.g. Cefotaxime, Ceftriaxone
• Antibiotic adjusted according to sensitivity of organism and age of patient
ROUTE:
• Parenteral 10 days - neonate ( up to 14 days if severely ill) 4 NOT CEFTRIAXONE )
• Parenteral until afebrile for 2 days and culture sterile, then oral to complete 10 days or
parenteral for a total of 10-14 days depending on severity of illness and clinical response
DURATION OF TREATMENT :
• Duration - 10 days ( usual) - up to 14 days depending on the severity of the illness
• Shorter courses - unreliable results
TABLE 1: ORAL ANTIBIOTICS FOR THE TREATMENT OF CHILDHOOD URINARY TRACT INFECTION
MEDICATION DOSE
Amoxicillin / Clavulanic acid 45 mg/ kg/ day ( Amoxil component ) - ql2 h
Trimethoprim ( TMP ) sulfamethoxazole ( SMX ) 8 mg/ kg/ day TMP- ql2 h ( not neonate )
Cefalexin ( Ospexin ) 30-50 mg/ kg/day- ql2 or 8h
Cefaclor ( Ceclor ) 40 mg/ kg/ day -ql2h
Cefuroxime ( Zinnat ) 20mg/ kg/ day - ql2h - max 250mg - infants
30mg/ kg/ day - ql2h - max 500mg- > 2 yrs age
Cefprozil 30mg/ kg/ day - ql2h
RESISTANT ORGANISMS RESISTANT ORGANISMS
Ciprofloxacin 30mg/ kg / day - ql2 h (max 1.5 g/ day )
Norfloxacin 10 - 15 mg/ kg/day - ql2h ( max 800 mg/ day )
Cefixime (Suprax )( Denvar ) 8 mg/ kg/ day - ql2 h ( max 400 mg/ day )
FLUOROQUINOLONES - NOT FIRST LINE
• Have been used to treat UTI with highly resistant organisms in children
• Side effect profile similar to adults
NOT FDA approved for children but has been widely used in children with specific
indications. Cartilage toxicity observed in immature mice not seen in children
• USE CAREFULLY and only if there are no other oral options
VERY VERY IMPORTANT

FOLLOW-UP AND MONITORING (UHWI DOCUMENT + CONSULTANT)
Note well:
• ***MUST repeat urine cultures 48 hours after starting AND 48 hours after finishing!!! the course.
Must be clear at BOTH.
• Adjust antibiotic therapy according to sensitivity and response
• At day 10, start prophylaxis!!!!! in cases where indicated (see below)
RE: PROPHYLAXIS AGAINST RECURRENT UTI
• Prophylaxis is STARTED for ALL children with UTI. Continued until investigations normal.
o ***Prophylaxis dose is 25-30% of treatment dose!!
• < 1 year old:

o Start and keep them on prophylaxis until 1 year of age AND in addition the further investigations must be
completed negative. Must meet other criteria
• For age > 1: Keep them on prophylaxis until investigations are done completed and are normal. Must do repeat
urine cultures every 2-3 months to pick up recurrence
Other indications to continue prophylaxis!!!

• VUR – all grades
• Hydronephrosis – until resolved
• Obstructive uropathy
• Recurrent symptomatic UTI
OTHER NOTES:
• Aminoglycosides (eg. gentamicin) --> Potential nephrotoxicity and ototoxicity
• Aminoglycosides particularly effective against pseudomonas
• Nitrofurantoin NOT good for renal involvement as it does not reach significant tissue levels
• Renal or peri-renal abscess:

o May require surgical or percutaneous drainage + antibiotic therapy
COMPLICATIONS
• RENAL SCARRING
• Chronic renal damage --> ARTERIAL HYPERTENSION AND END STAGE RENAL INSUFFICIENCY
• HYDRONEPHROSIS
o SEE UHWI DOCUMENT
Paediatrics
Vesicoureteric reflux
Source: Oxford
October 2014
DEFINITION AND INCIDENCE
• VUR: The retrograde flow of urine from the bladder into the upper urinary tract.
• Usually congenital, but may be acquired (e.g. post-surgery)
• VUR combined with UTI leads to progressive renal scarring.
• Such reflux nephropathy may progress to end-stage renal failure if untreated
• Incidence of VUR is ~1% in newborn infants. Observed in 30–45% of young children (< 5yrs) presenting with
UTI. There is often a strong family history with a 35% incidence rate among siblings of affected children.
GRADE OF VUR
International Reflux Study grading system:
Table 9: Grading of Vesico-ureteral reflux (MR ) •International Classification
GRADE DEGREE OF REFLUX

I Into distal ureter
n Up ureter into pelvis and calyces. No dilatation,nonnal calyceal Cornices
III Same as II, btf »nh mild dilatation of pelvis and calyces
FV Same as III, but with moderate dilatation and or tortuosity of the ureter and moderate dilatation of the renal pehrit
V Gross dilatation and tortuosity of ureter, pelvis and calyces wth significant blurting of the majority of the fomiccs
DIAGNOSIS
The diagnosis of VUR is established by radiological techniques.
1. Micturating Cystourethrogram (MCUG)
This technique involves urinary catheterization and the administration of radiocontrast medium into the bladder.
Reflux is detected on voiding.
• Advantages: GRADE of reflux seen
• Disadvantages: Requires bladder catheterization, radiation dose.
2. Indirect Cystogram
A radionucleotide method. Includes mercaptoacetyltriglycine (MAG-3) and diethylenetriamine pentaacetic acid
(DTPA) scans.
• Advantages: No catheterization required; lower radiation dose.
• Disadvantages: False negatives found; co-operation of child to void is needed
FOLLOW-UP AND TREATMENT

• The aims are to prevent progressive renal scarring.
• Prophylactic antibiotics may be used to prevent this
• Imaging by indirect cystogram (e.g. MAG-3) and DMSA are sometimes used for follow-up
• ***Randomized controlled trials of medical versus surgical treatment show surgery can reduce the incidence of
pyelonephritis, but there is no difference in scarring compared with medical treatment.
Medical therapy
• Prophylactic antibiotic therapy (See UTI notes)
Paediatrics
Wheezing, Bronchiolitis Notes
Source: Nelson’s (Ch 383), Toronto, Lecture, WHEEZE HISTORY OSCE TICK SHEET (Last Page)
September 2014
DEFINITIONS
• Wheeze: A musical and continuous sound that originates from oscillations in narrowed airways
• Obstruction/narrowing of lower airways produces wheeze (heard mostly on expiration. Airways are narrower in
expiration)
• Obstruction of extrathoracic airways produces stridor (heard mostly on inspiration)
AETIOLOGY OF WHEEZE
• Most wheezing in infants caused by inflammation. Generally bronchiolitis (SEE PAGE 4)
Other causes of wheezing:
NOTE FROM LECTURE: The most likely diagnosis in children with recurrent wheezing is asthma, regardless of
the age of onset, evidence of atopic disease, precipitating causes, or frequency of wheezing
• Chronic infectious wheezing --> Cystic fibrosis – suspect in pt with persistent respiratory symptoms, clubbing,
malabsorption, failure to thrive, electrolyte abnormalities, resistance to bronchodilators
• Allergy and asthma
• Congenital malformations of the respiratory tract cause wheezing in early infancy
o Can be due to external compression or intrinsic abnormality

o External vascular compression: Vascular ring vs. vascular sling. Ring encircles trachea and oesophagus
completely. Sling incomplete
• Cardiovascular causes of wheezing:

o ***Enlarged chambers: Cardiomegaly, LAH/LAD, dilated pulmonary arteries -> All cause extrinsic
compression
o ***Also CCF --> Pulmonary oedema --> wheezing due to bronchial vessel engorgement
• Foreign body aspiration -> Can cause acute or chronic wheezing
• GER(D) – WITH OR WITHOUT direct aspiration. Without aspiration --> causes wheeze by triggering a vagal
(parasympathetic) or neural reflex -> Increased airway resistance and reactivity.
• Trauma/tumors – RARE causes in infancy

Differential diagnosis
Extrinsic lower airway compression
• cyst.
Lung parenchyma: e.g. pneumonia, pulmonary oedema, bronchogenic
• Vascular: e.g. enlarged left atrium compressing left mainstem bronchus,

pulmonary artery vascular ring.
Lymphatic e.g. enlarged hilar lymph nodes (O pp.290-291, 654).
• Chest
• deformity: e.g. scoliosis (£Q p.746).
Intrinsic change in lower airway dimension
• Asthma (Cl p.262).
• Bronchiolitis (Cl p.288).
• Bronchitis and bronchiectasis (Cl pp.257, 291).
• Cystic fibrosis (Cl pp.270-273).
• Ciliary disease.
• Haemangioma.
Polyps.
•
• Tracheobronchomalacia.
Intraluminal lower airway obstruction
• 295, 326).
Aspiration of food or milk from gastro -oesophageal reflux (ffi pp.276,
• Foreign body inhalation (Cl p.51).

• Mucus, pus, and blood.
FROM LECTURE:
(Consider AGE of patient when thinking about top differentials - < 5 or > 5)
• Other causes of ACUTE: Acute asthma, bronchitis, LTB, bacterial tracheitis, foreign body aspiration,
oesophageal foreign body
• CHRONIC wheezing:
o Asthma o Vocal cord dysfunction
o Gastroesophageal reflux o Interstitial lung disease
o Recurrent aspiration o Tuberculosis
o Obstructive Sleep Apnea o Lymphoma
o Cystic fibrosis o Worms
o Immunodeficiency (Congenital and o Sickle cell disease
Acquired)
o HIV: Lymphocytic interstitial pneumonia Structural abnormalities:
o Primary ciliary dyskinesia o Tracheo-bronchomalacia
o Bronchopulmonary dysplasia o Vascular compression/rings
o Retained foreign body (trachea or o Tracheal stenosis/webs
oesophagus) o Cystic lesions/masses
o Bronchiolitis obliterans o Tumors/lymphadenopathy
o CHD: L -> R shunts o Cardiomegaly
o LV failure and Pulmonary edema
CLINICAL MANIFESTATIONS OF WHEEZING

**HISTORY IN ANY CHILD WITH WHEEZE**
Must include history of the current complaint including onset, duration
ALSO
• Birth history:
o Weeks of gestation
o NICU admission
o History of intubation/oxygen requirement
o Maternal complications including infection with HSV or HIV
o Prenatal smoking
• Past medical history – any comorbid conditions
• Family history:
o Asthma in a 1st degree relative

o Immunodeficiencies
o Cystic fibrosis
• Social history:
o Include environmental history

o Smokers at home (inside and outside)
o Daycare exposure (all exposures, even church in a 2 week old. Got grinded for this on rounds)
o Number of siblings
o Occupation of inhabitants at home! (again related to exposure risk. Got grinded on rounds)
o Pets
o TB exposure
o General home environment: Dust mites, construction work, AC, mold, cockroaches
PHYSICAL EXAMINATION
***Respiratory rate
***Pulse ox!!
• Expiratory time may be prolonged

• Biphasic wheezing may occur if there is central, large airway obstruction!!
***Lack of audible wheezing is NOT REASSURING if the infant shows other signs of respiratory distress because
complete obstruction to airflow can eliminate the turbulence
• Must note air entry
***Listening to breath sounds over the neck helps differentiate upper airway sounds from lower airway sounds
Note the PRESENCE OR ABSENCE of stridor
Signs of respiratory distress:

• Nasal flaring
• Tracheal tugging
• Infraclavicular, intercostal, subcostal recession
• Excess use of accessory muscles (incl. sternocleidomastoid, scalene muscles (3))
Upper airway signs of atopy: Boggy turbinates, posterior oropharynx cobblestoning
*** Evaluate the skin of the patient for eczema and any significant haemangiomas
From Lecture:
HISTORY FOR A WHEEZING PATIENT (SEE LAST PAGE OF DOC FOR TICK SHEET):
o Age of onset o Greasy stool (cystic o Number of siblings

o Course of onset (Acute vs. fibrosis?) o Occupation of inhabitants
chronic) o Eczema at home
o Cough o Choking o Pets
o Shortness of breath o Triggers o TB exposure
o Chest pain o Cold air o Worms
o Cyanosis o Allergic rhinitis o Family history of
o Exercise-induced o Weight loss Atopy/Asthma
symptoms o Recurrent infections o Past use and response to
o Postnasal drip o Birth history bronchodilators
o Snoring o Environmental history o Food allergies
o Spitting up o Smokers at home o Co-morbid conditions
Lecture: EXAMINATION
• Anthropometry tracheal deviation

• Vital signs, SPo2 • Chest wall asymmetry Crackles can be present in
• Cyanosis or clubbing with expansion conjunction with wheezing
• Allergic shiners • Asthma
• Lymph nodes Percussion • Bronchiectasis
• Position of the diaphragm
Chest examination & detect differences in Examination should also focus
Inspection resonance on cardiac findings
• Respiratory distress, • Murmurs
tachypnea, retractions Auscultation • Signs of heart failure
• Increased anteroposterior • Location of wheezing
(AP) diameter • Character Skin for eczema
• Harrison sulci • Prolonged expiratory
• Scoliosis complicated by phase suggests airway ENT examination
airway compression narrowing • Allergic rhinitis
• Sinusitis
Palpation Focal wheezing suggest localized • Nasal polyps
• Supratracheal & structural airway abnormality • Tonsils
lymphadenopathy or • Airway evaluation by
imaging or bronchoscopy
Investigations – See “Wheezing Child” lecture
---
ACUTE BRONCHIOLITIS
DEFINITION
• LRTI that has wheezing and signs of respiratory distress
• Medscape: An acute inflammatory injury of the bronchioles (hence bronchiolitis) that is usually caused by a viral
infection (most commonly respiratory syncytial virus and human metapneumovirus). This condition may
occur in persons of any age, but severe symptoms are usually evident only in young infants.
EPIDEMIOLOGY
• The most common LRTI in infants, affects 50% of children in first 2 yr of life; peak incidence at 6 mo, winter or
early spring
• Increased incidence of asthma in later life!
AETIOLOGY
Most wheezing in infants caused by inflammation. Generally bronchiolitis:
• Acute bronchiolitis and inflammation of the airway

• Infection can cause obstruction to flow by internal narrowing of the airway
• ***RSV invades the nasopharyngeal epithelium and spreads to the lower airways where it causes increased
mucus production, desquamation, and then bronchiolar obstruction
• Acute bronchiolitis is predominantly a viral disease

o RSV in > 50% of cases
• Also parainfluenza, influenza, rhinovirus, adenovirus. M. pneumoniae (rare)

• Emerging pathogen: Human metapneumovirus
• **THERE IS NO EVIDENCE OF A BACTERIAL CAUSE FR BRONCHIOLOITIS although bacterial

pneumonia is sometimes confused clinically with bronchiolitis
• Bronchiolitis is RARELY followed by bacterial superinfection
RISK FACTORS
1. Boys > girls

2. Non-breastfed
3. Crowded conditions
4. Mother smoked during pregnancy
5. See others throughout
**Older family members are a COMMON SOURCE OF INFECTION – they may only experience minor URT
symptoms. Older people tolerate bronchial oedema better
** There is an increased risk of severe infection in infants with CHD, CLD of prematurity, immunodeficiency, and other
lung disease.
* Coinfection with > 1 virus can also alter severity
**Airway resistance is inversely proportional to radius of the bronchioles to the 4th power. Children have smaller
airways so resistance is exponentially higher in both inspiration and expiration. Because airways smaller in expiration,
respiratory obstruction leads to AIR TRAPPING AND HYPERINFLATION
** If obstruction complete --> ATELECTASIS
• Hypoxaemia is a consequence of ventilation-perfusion mismatch resulting from the air trapping early in the
course!
• Severe obstructive disease and tiring of respiratory effort --> Hypercapnia later!
History
***Usually preceded by exposure to an older contact with a minor respiratory syndrome the previous week
• Infant 1st develops prodrome of a mild URTI with sneezing and clear rhinorrhea
• +/- Decreased appetite and
• Feeding difficulties caused by tachypnoea
• Fever – ALTHOUGH the temperature can range from SUBNORMAL to MARKEDLY ELEVATED
• Irritability
• Dyspnoea
• Cough
• Wheezing
• Cyanosis
• Apnoea
Physical Examination:
• Tachypnoea
• Cyanosis
• Prolonged expiration
• Wheezing and crackles
• Barely audible breath sounds suggest very severe disease with nearly complete bronchiolar obstruction
• ***Hyperinflation of the lungs can permit palpation of the liver and spleen below the costal margin
INVESTIGATIONS
• ***Diagnosis of acute bronchiolitis IS CLINICAL
KEY INVESTIGATIONS
1. PULSE OX AND NONINVASIVE CO2 essential as tachypnoea does not always correlate with hypoxemia or
hypercarbia
2. CXR (usually in severe disease, poor response to therapy, chronic episode)

a. Air trapping, hyperinflation, peribronchial thickening, patchy atelectasis, increased linear markings,
retrosternal air
3. Nasopharyngeal swab
a. Direct detection of viral antigen (immunofluorescence)
Others
• WBC can be normal
• “Trial of bronchodilator” – diagnostic and therapeutic can reverse conditions incl bronchiolitis and asthma but
will not affect a fixed obstruction
• Sweat test – evaluate for cystic fibrosis
TREATMENT
MAINSTAY OF TREATMENT OF BRONCHIOLITIS IS SUPPORTIVE
Self-limiting disease with symptoms usually lasting 2-3 wks
Mild distress:
• Supportive: Oral or IV hydration, O2 (nasal cannula/facemask) antipyretics for fever
• Treat usu with patients sitting with head and chest elevated at a 30o angle with neck extended
• SUCTIONING OF SECRETIONS IS AN ESSENTIAL PART OF MANAGEMENT
• Feed via NGT if risk of severe. Due to risk of aspiration
• If very severe and intubation may be requires -> IV fluids only
• Bronchodilators for wheeze: nebulized salbutamol, ipratropium, and adrenaline have all been used in studies.
The best evidence is for nebulized adrenaline.
• Mechanical ventilation for severe respiratory distress or apnoea.
Moderate to severe distress

• As above ± intubation and ventilation as needed
• Consider rebetol (Ribavirin®) in high risk groups: bronchopulmonary dysplasia, CHD, congenital lung disease,
immunodeficient
Monthly RSV-Ig or palivizumab (monoclonal antibody against the F-glycoprotein of RSV) is protective against severe
disease in high risk groups; case fatality rate <1%
Antibiotics have no therapeutic value unless there is secondary bacterial pneumonia
INDICATIONS FOR HOSPITALIZATION:
• Hypoxia: O2 saturation <92% on initial presentation

• Persistent resting tachypnea > 60/min and retractions after several salbutamol masks
• Past history of chronic lung disease, haemodynamically significant cardiac disease, neuromuscular problem,
immunocompromised
• Young infants < 6 months old (unless extremely mild)
• Significant feeding problems
• Social problem (e.g. Inadequate care at home)
Prophylaxis
• IM Palivizumab is a monoclonal antibody to RSV and can be used as prophylaxis.
Wayne Robinson, Cl
ass of 2015
Medicine and Therapeatia EtMmlaatioaj : OSCE PrLay 24 A 2.' , > OM3 . SUfioD 2 a , 2 b [Moot ]
History taking: Long Gu« Part !
Student no: Examiner Name:
•• This station is 15 minutes. You are asked to Ukc a focused history from a mother who has taken her child to the
Accident and Emergency Unit because of worsening shortness of breath and wheezing. You have 10 minutes to do
this followed by 1 minute to collect your thoughts. Then, in the last 4 minutes, present your findings to the exam iner
and discuss the management of the patient ’ s problems.
2 1 0
Good Adequate Not done
Inadcqualc
Appropriate personal iatroductioo j
)
r
( )
J
Elicits patient ’* namc/parcnl’s name(s)
Elicits patient’s age/dale of birth, sex. ] ( )
Elicits the following aspects of tie pi dent ’s key history

Relevant FMH: known diagnosis/duration, drugs used [ ] ( J [ )
Seven tyffrcqucncyof episodes,
ability to participate in activities . ( ]
( )
( )
[ )
( )
( ]
Elicits all of patient 's complain ts/duration
Characteristics of cough, timing, nature, ( ) [ J
Post tussive vomiting, chest pain
Severity of SOB, at rest, cyanosis, hypoxic symptoms ,
activities affected
Definition of historian’ s understanding of wheezing [ ] ( ]
Treatment given at home for this acute illness/ response ]
Use of AeroChsmbers ]
Features of acute infection J
Environmental triggers ( J ]
Pcrsoaal / family history of atopy ]
Features suggesting wheezing due to non -asthmatic causes J (
Aaks routine screening questions unrelated to key complaints

PMHfchCdbood Illnesses f J )
Vutritioo/ currcnt diet ]
immvnlzatlcxis ]
Vcaatal/occcuifal history ( 1
Developmental mffestoaes/schooling, age appropriate ( ) [ J
ntcrricnlng a kills (»ce comm onica (ions checklist)
Establishes and maintain * rapport with patient throughout ( J
>anoostralej appropriate Listening skills )
ieneral fluency Including non -use or jargon and repetition )
hecks and summarizes data and concludes interview ) 1
lobsl rating 5 4 3 2 I
an didst e’ s overall attifudioal ipjx-oaah to patient ( J [ ] ( 1 1 1 ( )
otal marfci out of 32

Page 1 of 2
Paediatrics Respiratory Distress in the Newborn Source: Toronto Notes Wayne Robinson, MBBS Class of 2015

Respiratory Distress Syndrome Transient Tachypnoea of the Meconium Aspiration
(RDS) Newborn (TTN) Syndrome (MAS)
(“Hyaline Membrane Disease”) (“Wet Lung Syndrome”)
Aetiology Surfactant deficiency à poor lung Delayed resorption of fetal lung fluid à Meconium is sterile but
compliance due to high alveolar accumulation of fluid in peribronchial causes
surface tension à atelectasis à ↓ lymphatics and vascular spaces à airway obstruction, chemical
surface area for gas exchange à tachypnoea inflammation, and surfactant
hypoxia + acidosis à respiratory inactivation
distress
Gestational Preterm More commonly term and late preterm Term and post-term
Age
Risk Factors Maternal diabetes Maternal diabetes Meconium-stained amniotic
Preterm delivery Maternal asthma fluid
Male sex Male sex Post-term delivery
Low birth weight Macrosomia (> 4500 g)
Acidosis, sepsis Elective caesarean section or short
Hypothermia labour
Second born twin Late preterm delivery
Clinical Onset within first few hours of life, Tachypnoea within the first few hours Respiratory distress within
Presentation worsens over next 24-72 h of life ± retractions, grunting, nasal hours of birth
Respiratory distress (tachypnoea, flaring Small airway obstruction,
tachycardia, grunting, intercostal Often NO hypoxia or cyanosis chemical pneumonitis à
indrawing, nasal flaring, cyanosis, lung tachypnea, barrel chest with
crackles) audible crackles
Hypoxia Hypoxia
Cyanosis
CXR Findings Homogenous infiltrates Perihilar infiltrates Hyperinflation
Reticulogranular pattern “wet silhouette”; fluid in fissures Patchy atelectasis
Air bronchograms Patchy and coarse infiltrates
Decreased lung volumes 10-20% have pneumothorax
May resemble pneumonia (GBS)
If severe, “white-out” with no
differentiation of cardiac border
Prevention Prenatal corticosteroids (e.g. Where possible, avoidance of elective If infant is depressed at birth,
Celestone® 12 mg q24h x 2 doses) if caesarean delivery, particularly before intubate and suction below
risk of preterm delivery < 34 wks 38 wks gestation vocal cords
Avoidance of factor associated
Monitor lecithin:sphingomyelin (L/S) with in utero passage of
ratio with amniocentesis, L/S >2:1 meconium, e.g. post term
indicates lung maturity delivery
Treatment Resuscitation Supportive Resuscitation

Oxygen Oxygen if hypoxic Oxygen
Ventilation Ventilator support (e.g. CPAP) Ventilatory support
Surfactant (decreases alveolar IV fluids and lavage feeds Surfactant
surface tension, improves lung Inhaled nitric oxide,
compliance and maintains functional extracorporeal membrane
residual capacity) – only useful in the oxygenation at some centres
first 72 hrs for PPHN
Complications In severe prematurity and/or Hypoxaemia Hypoxaemia
prolonged ventilation, increased Hypercapnoea Hypercapnoea
risk of bronchopulmonary dysplasia Acidosis Acidosis
(BPD) PPHN PPHN
Pneumothorax
Pneumomediastinum
Chemical pneumonitis
Secondary surfactant inhibition
Respiratory failure
Prognosis Dependent on gestation at birth and Recovery usually expected in Dependent on severity,
severity of underlying lung disease; 2-5 d mortality up to 20%
long term risks of CLD

Paediatrics
Diarrhoea
Source: Ward rounds (May Pen) – Dr. Reem, Toronto Notes
October 2014
Topic: Diarrhoea
Remember when thinking of causes of diarrhea, classify into infectious and non-infectious
• Then under infectious, viral, bacterial and parasitic. (STUDY ALL THE CAUSES IN THE TABLE BELOW)
Causes of dysentery = Shigella, EHEC, EIEC, Entamoeba histolytica
Re: Acute diarrhoea:

• Normal stool frequency and consistency vary, e.g. breastfed infants may pass 10–12 stools per day, primary
school children may pass stool from three times a day to once every three days. Diarrhoea is a change in
consistency and frequency of stools with enough loss of fluid and electrolytes to cause illness.
• In older children, usually > 3 loose/watery stools/day
In diarrhoea history: DON’T FORGET:

• Blood or mucus in the stool?
• Ill contacts/recent travel
• Ask about signs/symptoms of dehydration!!
• Ask how/with what exactly were the losses being replaced with
• Antibiotic use!!! (antibiotic-associated diarrhoea) - C. Deficle. This is treated Metranidazole
• Ask about rotavirus vaccine!!
TREATMENT OF DIARRHOEA
MANAGEMENT OF DEHYDRATION IS MOST IMPORTANT!!! (See dehydration lecture)
Anti-motility drug treatment is NOT recommended; it can be harmful, particularly in acute infection/inflammation.
Antibiotics are NOT indicated unless cause is proven, e.g. Yersinia or Campylobacter infection, parasitic infection, NEC,
or proven bacteraemia/systemic infection.
Table 14. Differential Diagnosis of Diarrhea

Infectious Non-infectious
Acute Viral Bacterial Parasitic Antibiotic-induced
Rotavirus Salmonella Giardia lamblia Non- specific: associated with
Norwalk Campylobacter Entamoeba histolytica systemic infection
Enteric Adenovirus Shigella Hirschsprung' s disease
Pathogenic £ coli Toxin ingestion
Yersinia Primary disaccharide
C. difficile deficiency
Chronic 0 - 3 months 3 months - 3 years 3 - 1 8 years Uncommon
0FFT Gl infection Gl infection Gl infection Drug- induced
Toddler ' s diarrhea Lactase deficiency Chronic constipation
Irritable bowel syndrome UTI
FFT Disaccharidase Celiac disease IBD Short bowel syndrome
deficiency Endocrine (thyrotoxicosis, Schwachman- Diamond
Cow 's milk protein Addison's) syndrome
intolerance Neoplastic
Cystic fibrosis ( pheochromocytoma,
lymphoma )
Table 15. Infective Diarrhea

Viral Infection Bacterial Infection
Etiology Most common cause of gastroenteritis
Commonly: rotaviruses, astrovimses, Norwalk vims
(typically older children)
Presentation Clinical Associated with URTIs Severe abdominal pain

Resolves in 3- 7 d High fever
Slight fever, malaise, vomiting, vague abdominal Bloody diarrhea
pain
Risk Factors Daycare Travel

Poorly cooked meat
Poorly refrigerated foods
Prolonged antibiotics
Management Prevention and treatment of dehydration most important ( see Dehydration, P79 )
Early refeeding advisable, start with small amounts of easily digested carbohydrates, postpone dairy
and fibrous vegetables
Antibiotic therapy when indicated, antidiarrheal medications not indicated
Notify Public Health authorities if appropriate
Toddler 's Diarrhea Lactase Deficiency (Lactose Intolerance)

Epidemiology Clinical Presentation
• most common cause of chronic diarrhea during infancy • chronic, watery diarrhea and abdominal pain, bloating associated with dairy intake
• onset between 6-36 mo of age, ceases spontaneously between 2-4 yr • primary lactose intolerance: crampy abdominal pain with loose stool (older children, usually of
East Asian and African descent)
Clinical Presentation • secondary' lactose intolerance: older infant, persistent diarrhea ( post viral /bacterial infection,
• diagnosis of exclusion in thriving child Celiac disease, or IBD)
• 4-6 bowel movements per day
• diet history ( e.g. excess juice intake overwhelms small bowel resulting in disaccharide Diagnosis
malabsorption ) • trial of lactose-free diet
• stool may contain undigested food particles • watery stool, add pH , positive reducing sugars
• excoriated diaper rash • positive breath hydrogen test if >6 yr
Management Management
• reassurance that it is self -limiting • lactose-free diet, soy formula
• 4Fs ( adequate Fibre, normal Fluid intake, 35-40% Fat, discourage excess Fruit juice) • lactase-containing tablets/capsules/drops (e.g. Lacteeze* Lactaid*)

Paediatrics
Diphtheria Summary Notes
Source: Toronto, Oxford
October 2014
DEFINITION
Upper respiratory bacterial illness caused by Corynebacterium diphtheriae (Gram positive bacilli, aerobic)
• Characterized by pharyngitis, low-grade fever, and nasopharyngeal pseudomembranes released by bacteria
(with possible dermatologic, cardiac and/or nervous system involvement)
EPIDEMIOLOGY
• Routine immunization has significantly reduced morbidity and mortality
• Diphtheria now very rare
AETIOLOGY
• Caused by lysogenized phage
• Transmitted by direct contact or droplet spread; incubation period is 2-5 d
RISK FACTORS
• Unvaccinated, immunocompromised, travel to or inhabitants of endemic countries
HISTORY
• Early symptoms similar to a common cold: low-grade fever, sore throat, anorexia, malaise
• Later symptoms (due to Diphtheria toxin): Pallor, diaphoresis, stupor, coma
PHYSICAL
• Grey membranes may cover tonsils and soft palate (at day 2-3); becomes greenish or black with
haemorrhage
• Cervical lymphadenopathy
• “Bull neck” secondary to submandibular oedema in severe disease
INVESTIGATIONS
*THROAT SWAB:
1. Microscopy: Albert’s stain: 1. Metachromatic granules, 2. Chinese letter configuration
2. Culture (specifically state that diphtheria is suspected as some labs only look for group A Streptococcus on
routine throat cultures)
a. Loeffler’s serum (If growth, then do Albert’s stain)
b. Tinsdale agar (Chocolate tellurite)
3. Toxin detection: Elek test
MANAGEMENT
Treat based on clinical SUSPICION!!; awaiting culture results will postpone treatment and worsen prognosis
• Diphtheria antitoxin
• **Penicillin G or **Erythromycin (halts furthers toxin production and prevents carrier state)
PROGNOSIS
• 5-10 % mortality for respiratory diphtheria

• Complications: airway obstruction, recurrent laryngeal nerve palsy
• Associated conditions: Diphtheritic peripheral neuritis, myocarditis
Paediatrics
Pertussis Summary Notes
October 2014
DEFINITION
• Prolonged respiratory illness characterized by paroxysmal coughing and inspiratory “whoop” (Hence known
as “Whooping cough”). Caused by Bordetella pertussis (Gram-negative coccobacilli, aerobic)
**A whooping cough-like syndrome may be caused by Bordetella PARApertussis, Mycoplasma pneumoniae,
Chlamydia, or adenovirus.
EPIDEMIOLOGY
~10 million children < 1 yr old affected worldwide, causes up to 400,000 deaths per year
• Greatest incidence among children < 1 yr (not fully immunized) AND adolescents (waning immunity)
AETIOLOGY
• Bordetella pertussis: Gram-negative pleomorphic rod

• Highly contagious; transmitted via respiratory droplets released during intense coughing
• Incubation period: 6-20 d; ***most contagious during catarrhal phase but may remain contagious for weeks
after
HISTORY
May be a typical history. Typically induces three stages of illness:
1. Catarrhal stage
• Lasts 1-7 d; URTI symptoms (coryza, mild cough, sneezing) with NO or LOW-GRADE fever
• Most contagious during catarrhal stage
2. Paroxysmal stage
• Lasts 4-6 wks; characterized by severe paroxysms of cough (“100 day cough”), sometimes followed by:
• Inspiratory whoop (“whooping cough”) and post-tussive emesis, may become cyanotic before whoop
• ***Infants < 6 mo: whoop is often absent and post-tussive apnea is more common!
3. Convalescent stage
• Lasts 2-4 wks; characterized by occasional paroxysms of cough, but decreased frequency and severity
• Non-infectious but cough may last up to 6 mo
INVESTIGATIONS
1. Nasopharyngeal (NP) specimen using aspirate OR NP swab
• Gold standard: Culture using special media (Regan-Lowe agar) (previously Bordet-Gengou was
preferred)
• PCR to detect pertussis antigens
2. Blood work: CBC (lymphocytosis) and serology (IgG antibodies against B. pertussis)
In infants you will need to make sure that the problem is not pneumonia. Also, check the following:
• Eyes: Subconjunctival haemorrhages are common.
• CXR.
MANAGEMENT
• Hospital care
o Infants: Admission is required for those with a history of apnoea, cyanosis, or significant paroxysms. Close
monitoring is required particularly in infants since there is a risk of seizures, encephalopathy, and death.
o Isolation: Patients should be isolated for 5 days after starting treatment with antibiotics!!!. Report to
public health
• Supportive care
• Antimicrobial therapy indicated if B. pertussis isolated, or symptoms present for <21 d

o Use macrolide antibiotics (azithromycin, erythromycin or clarithromycin)
o Erythromycin for 14 days (or clarithromycin for 7 days) to reduce infectivity but this may have
minimal effect on the cough
.
PROPHYLAXIS
• CONTACTS: Macrolide antibiotics for all household contacts
• Prevention with vaccination in infants and children < 7 (Pentacel), and booster in adolescents (Adacel)
COMPLICATIONS
• Pressure related from paroxysms: Subconjunctival hemorrhage, rectal prolapse, hernias, epistaxis
• Respiratory: Sinusitis, pneumonia, aspiration, atelectasis, pneumomediastinum, pneumothorax, alveolar rupture
• ***Neurological: Seizures (~3%), encephalopathy, intracranial haemorrhage
• Mortality: ~0.3%; highest risk in infants < 6 mo old
Paediatrics
Vomiting Summary Notes
October 2014
HISTORY
• Characteristics of emesis (e.g. projectile?, bilious?, bloody?)
• Pattern of emesis (e.g. association with feeds, cyclic, morning e.g. early morning vomiting with CNS tumour)
• Associated symptoms (e.g. anorexia, diarrhoea, etc.)
• Red flags: Bilious or bloody emesis, projectile vomit (pyloric stenosis), abdominal distension and tenderness,
high fever, signs of dehydration
Vomiting: forceful expulsion of stomach
PHYSICAL FINDINGS contents through the mouth.
• Vital signs to determine clinical status and hydration state
• Dictated by suspected differential Regurgitation: the return of partially
digested food from the stomach to the
mouth.
INVESTIGATIONS
• CBC, electrolytes, BUN, Cr, amylase, lipase done routinely
• In sick child, add: ESR, venous blood gases, culture and sensitivity (blood, stool), imaging dictated by suspected
differential (see Table 13)
TREATMENT
• Supportive treatment as needed: e.g. oral or IV REHYDRATION (SEE DEHYDRATION LECTURE)
• Treat UNDERLYING CAUSE: e.g. pyloromyotomy for hypertrophic pyloric stenosis.
Pharmacological:
• Antihistamines; phenothiazines (side-effects: extrapyramidal reactions);
• Prokinetic drugs, e.g. domperidone.
• 5-HT3 antagonists, e.g. ondansetron, are increasingly being used for treating post-operative or chemotherapy
induced vomiting.
• 5-HT1D agonists, e.g. pizotifen, are useful as prophylaxis and treatment for cyclic vomiting syndrome
COMPLICATIONS
• Dehydration
• Electrolyte disturbance (e.g. decreased K+, decreased Cl–, alkalosis with pyloric stenosis)
• Acute or chronic GI bleeding (e.g. Mallory–Weiss tear)
• Oesophageal stricture
• Barrett’s metaplasia
• Broncho-pulmonary aspiration
• Faltering growth (FTT)
• Iron deficiency anaemia (HCl required for iron absorption. HCl lost in vomitus (also causing alkalosis))

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Wayne Robinson, MBBS Class of 2015

Notes Prepared and Created by:

Compiled and Bookmarked by:

• Cause not fully determined

• More than 100 gene loci linked to asthma.

RISK FACTORS (Must Know)

2 main types of childhood asthma: 1. Recurrent wheezing 2. Chronic asthma

Airway obstruction in asthma results from numerous pathologic processes:

CLINICAL MANIFESTATIONS AND DIAGNOSIS

MOST COMMON CHRONIC SYMPTOMS:

Other symptoms in older children/adults:

**NB**: The respiratory symptoms are WORSE AT NIGHT!!

• ***MUST ASK ABOUT PREVIOUS BRONCHODILATOR USE IN HISTORY!!:

From lecture: HISTORY FOR A WHEEZING PATIENT:

History suggestive of asthma

Examination (See Notes on wheezing for general wheezing examination)

Inspection: - BARREL-SHAPED chest, possible signs of resp. distress if severe exacerbation

In extremis -> Airflow may be so limited that wheezing cannot be heard

Other common causes of intermittent chronic coughing --> GER, rhinosinusitis

DIFFERENTIAL DIAGNOSIS OF CHILDHOOD ASTHMA

UPPER RT CONDITIONS MIDDLE RT CONDITIONS LOWER RT CONDITIONS

- Allergic rhinitis * - Laryngotracheobronchomalacia * Viral bronchiolitis *

1. PULMONARY FUNCTION TESTS (Spirometry and Peak flow)

A. Spirometry is helpful as an objective measure of airflow limitation

SEE MY FULL NOTES ON PEFR

TREATMENT **MUST SEE ENTIRE UHWI OFFICIAL EMED DOCUMENT**

GINA (GLOBAL INITIATIVE FOR ASTHMA) GUIDELINE

• Short-acting beta 2 agonists: Salbutamol (aka albuterol), terbutaline

RE: TREATMENT OF INFLAMMATION AND HYPERREEACTIVITY

• ICS: Budesonide, beclomethasone, fluticasone

Immune Functions of Milk

-Ig-specific, antigen binding

CONTRAINDICATIONS = VACSS - Anggelos

NB: MASTITIS IS NOT A CONTRAINDICATION FOR BREASTFEEDING

OTHER IMPORTANT POINTS

COMPARISON OF BREAST MILK TO COW MILK.

COMPONENT HUMAN MILK COW MILK

Water/solids Same Same

BREAST ANATOMY AND FUNCTION

- Alveoli are small sacs made of milk-secreting cells

Prolactin Milk secreting cells

- Prolactin is secreted after the feed to Ducts

produce milk for the next feed Larger ducts

- Oxytocin works before or during feed to make milk flow

Inhibition of breast milk

- Taken much areola and underlying tissue into the mouth

A. **HOW TO ACHIEVE THE ATTACHMENT**

B. POSITIONING THE BABY

NAME DISCRIPTION NOTES

Rest your arm on a pillow in your lap or right

With your baby facing you, draw him close and

• Encourage breastfeeding on demand

OTHER IMPORTANT POINTS

• Foods better saved for year 2:

Storage of milk to the left

Problem Prevention Treatment

Oral thrush - Washing hands carefully Seek advice from doctor

It is a symptom complex, NOT a disease

***1/3 OF CASES HAVE NO DEFINITE AETIOLOGY!!***

Some important causes!!:

• In *premature infants*, major factors are:

Spastic quadriplegia (20%) Periventricular leukomaiacia Ischemia, infection

***CP generally divided into several motor syndromes***:

1. Spastic (80%) – UMN of pyramidal tract involved

NB: The respiratory symptoms are WORSE AT NIGHT!!

TREATMENT MUST SEE ENTIRE UHWI OFFICIAL EMED DOCUMENT

A. HOW TO ACHIEVE THE ATTACHMENT

1/3 OF CASES HAVE NO DEFINITE AETIOLOGY!!

• In premature infants, major factors are:

CP generally divided into several motor syndromes:

SPASTIC DIPLEGIA (BOTH LEGS) - MOST COMMON TYPE