MEQ PJP

1. Kawasaki disease (R319, R318, R418, R618, R516)

a) Provisional dx
Kawasaki disease
b) Pathophysiology
Autoimmune dissease where there is inflammation of the medium sized vessels
c) Management
IV immunoglobulin for 10 hours
Aspirin

 2y/0, fever for 5 days, conjunctivitis, red cracked lips, strawberry tongue.
a. provisional dx and justify
 Kawasaki
b. ddx
 measles
 dengue
 scarlet fever
c. outline tx
 iv immune protein (gamma globulin) infusion over 10 hrs (reduce risk coronary artery
aneurysm)
 aspirin (High doses aspirin treat inflammation.)

 Kawasaki with signs.

a) Dx with justify
b) Ddx n complication
Measles
Dengue fever
Scarlet fever
Hsp
Erythema marginatum in rheumatic fever
Complication-coronary artery aneurysm
c) Mx

 2 years old boy, having fever for more than 5 days with non-purulent conjunctivitis, one sided
cervical lymphadenopathy, edema of the palms with peeling of skins.
a) Provisional diagnosis.
Kawasaki disease
b) State the criteria (I think so, not really sure).
Fever for at least 5 days, plus 4 out of 5 criteria below:
- Bilateral non-purulent conjunctivitis
- Mucosal changes in the oropharynx (strawberry tongue, red and dry
fissured lips, injected pharynx)
- Cervical lymphadenopathy
- Changes in extremities (edema and/or erythema of the hands or
feet, desquamation, beginning periungually
- Rash, usually truncal, polymorphous but not vesicular
 Illness not explained by other disease process
c) Management.
IV immunoglobulins
Oral aspirin
2. HSP (R119, R319, R118, R216, R416, R616)
A 6 years old boy presented with purpuric skin rash
a) Give 4 ddx
 Henoch schonlein purpura (HSP)
 Immune thrombocytopenic purpura (ITP)
 Leukaemia
 Scurvy (defect in vascular and perivascular collagen tissue synthesis)
 Hereditary connect tissue disorder such as ehlers danlos syndrome
b) What is the most common purpuric skin rash: HSP
There is deposition of immunoglobulin A into the small vessels of the joints,skin,GIT and
kidneys causing arthritis,palpable purpura at lower extremities,buttock and extensor
aspect of upper limb
c) Give 4 clinical features of the above condition
 palpable purpura symmetrical, over pressure dependent area buttock and lower limbs
 Abdominal pain
 Arthralgia affecting large joints of lower lib, no effusion or warmth
 haematuria
d) Treatment/Managemet
● Symptomatic management, usually lasts 4-6 week
● NSAID for arthralgia
● Monitoring

 Henoch Scholein purpura

a) Provisional dx]
Henoch scholein prupura
b) Pathophysiology
There is deposition of immunoglobulin A into the small vessels of the joints,skin,GIT and
kidneys causing arthritis,palpable purpura at lower extremities,buttock and extensor
aspect of upper limb
d. Management
Self-limiting disease
Can give analgesia such as NSAIDS for the pain
Corticosteroids for significant GIT involvement

 Boy with non-blanching maculopapular rash over buttock, trunk and limbs. He also has fever,
abdominal pain and swelling at both knee and ankles.
a. Diagnosis: HSP
b. Investigation
Full blood count-platelets normal
Bleeding time-normal
Renal profile-normal
PT and APTT-normal
Urinalysis-hematuria
c. Management
 symptomatic: pain give analgesia, steroid for renal involvement or significant GIT
involvement
d. 1 complication of renal involvement: Esrd, nephritis, , nephrotic
 Boy with non-blanching maculopapular rash and fever. Abdominal pain, joint pain.
a) Diagnosis (1M)
HSP
b) Investigation (2M)
FBC – platelets will be normal
ESR CRP – elevated
Urinalysis – hematuria
Stool culture
c) Management (2M)
Supportive
Joint pain – NSAIDs
GI symptoms – corticosteroids

 6 year old boy presented with coryzal and fever with non-blanching purpuric rashes on the
extensor surface of lower limb including buttock, abdominal pain and joint pain. On
examination, abdomen is slightly tender and bilateral swelling of elbow and knee joints.
a) State your provisional diagnosis [1m]
Henoch-Scholein Purpura
b) Two Complications [2m]
Acute kidney injury
Intussusception
c) Follow up plan for this child [2m]
Follow up for one year if renal involvement. If persisting urinary abnormalities, long term
follow up.
3. VSD (Why arrive at 2 months? – due to left shunt arises after peripheral resistance increases)
R319, R219, R118, R218, R318, R516
A 2-months-old child, breastfeeding difficulty, episode of cyanosis during crying and feeding grade 3
systolic murmur at left sternal edge, hepatomegaly, lung clear.
a) Provisional dx (large VSD)
Right sided heart failure secondary to large VSD
b) Why does the patient present at 2 months of age?
There is increase pulmary vasculature resistance leading to reversal of shunt to right-to-left
shunt and thus causing deoxygenated blood to enter in the systemic circulation
c) Management
Oxygen
Anti-failure medications-diuretics,digoxin,ACEI
Surgical closure of the VSD
 VSD (Acyanotic heart disease: tachypnea, tachycardia, acyanosed)
A. Types of acyanotic congenital heart disease
SHUNT-VSD,ASD and PDA
Obstructive-pulmonary stenosis,coarctation of aorta
B. Murmur grading
Grade 1-very soft murmur which can only be heard by expert
Grade 2-soft murmur with no thrills
Grade 3-easily heard murmur with no thrills
Grade 4-loud murmur with palpable thrills
Grade 5-very loud murmur with easily palpable murmur
Grade 6-extremely loud murmur which can be heard even without stethoscope not
touching chest
C. Management
Small VSD can close spontaneously
Large VSD-can have heart failure so anti-failure medications can be given

 4-month old boy, bronchopneumonia at 2 month old, Not gaining weight. Profuse sweating
when breastfeeding, reduce effort tolerance.
Grade 3 pansystolic mm LLSE, loud p2 at pulmonary area.
a) provisional dx + justification
 heart failure secondary to ventricular septal defect
 reason: failure to thrive, reduced effort tolerance, sweat when feeding
b) investigation + finding
 chest xray: cardiomegaly, enlargement of left or biventricular, prominent pulmonary vascular
marking, pulmonary edema (kerley b lnes)
 ecg: left or biventricular hypertrophy, left axis deviation/ noral
 echocardiography
c) 3 complication
 Eisenmenger syndrome
 Infective endocarditis
 Secondary aortic insufficiency
 Rvoto
 Pulmonary hypertension
  A child diagnosed with Down syndrome presented with murmur at left sternal edge.
a) Possible diagnosis of the heart lesion
 vsd
b) List 3 investigation that can be done at antenatal to diagnose Down syndrome
 chorionic villus sampling (CVS) 10-13
 amniocentesis 15-20
 nuchal thickness on ultrasound 10-13
c) List 3 systemic complication
 Duodenal atresia
 Cardio-AVSD,PDA
 Congenital hypothyroidism
 Vision-cataract,glaucoma,squint
 Hearing-SNHL
 Hypotoina and joint laxity
 Seizure
 Delayed development
 Alzhemier if goes past 40 years old
 Atlantoaxial laxity
 Recurrent chest infections
 Hirschsprung disease
Congenital heart diseases
e. common CHD
 vsd
f. Pathophysiology of cyanosis
 Cyanosis is due to increase in deoxygenated blood at 5g/dl
 Reduction of blood return to the lung for proper gaseous exchange to take place
g. What CHD for this following CXR features
i. egg on side
tga
ii. boot shaped heart
tof

 6 weeks of life, boy, with pansystolic murmur heard on LLSE, associated with bibasal crepitus
and hepatomegaly.
a) Provisional diagnosis, justify.
Congestive cardiac failure secondary to large VSD
b) Investigations.
Chest X-ray – cardiomegaly, enlarged pulmonary arteries, increased pulmonary vascular
markings, pulmonary edema
ECG – biventricular hypertrophy
Echocardiogram – anatomy of defect, hemodynamic effect, pulmonary hypertension
c) Why does surgery need to be done early in this case? (3-6 months)
Manage heart failure and failure to thrive.
Prevent permanent lung damage from pulmonary hypertension and high blood flow
that lead to Eisenmenger syndrome.
4. Severe AEBA (features, allergic march?, medicine & its pharmacological & mode of action)
R119, R319, R318, R418, R518, R618
AEBA
a) Give 4 common triggering factors
 Allergen such as food, dust, mites
 Exercise induced
 Cold weather
 infection
b) Define FEV1
 Amount of expired air that can be expelled forcefully in 1 second
c) Give 2 components of Intermittent asthma
 Daytime symptoms less than one a week
 Night time symptoms less than once a month
 no exercise induced
 normal lung function
 brief exacerbation not affecting sleep or activity
d) Outline the 3 treatment modalities acute attack of asthma
 Oxygen therapy
 Nebulised salbutamol
 Systemic steroid
 Nebulised ipratropium romid

 Asthma
a) State 4 clinical features of acute severe asthma
Talk in words
Tachycardia more than 120bpm
Severe usage of accessory muscle
Pefr less than 60
Oxygen saturation less than 92%
Tachypnea
Very loud wheezing
Dyspnea when resting
b) 3 Risk factors for a child to get the disease
Family history of atopy
History of atopy
Exposure of allergens

c) Name the 4 front-line therapy for acute asthmatic attack and their mode of action
Salbutamol-beta2 receptor agonists which can cause bronchodilatation
Ipratropium bromide-anticholinergics which cause sympathetic activity to be dominant
Prednisolone-anti-inflammatory
Theophylline-inhibit phosphodiesterase enzyme which cause breaking of cAMP(important
for bronchodilatation)-this one not sure whether can or not
Oxygen-maybe
 8 y/o girl, diagnosed bronchial asthma since age 2, cannot join in sports because sob,
disturbed sleep every night because of sob.
h. Asthma category and justify
 Severe persistent asthma
 daily night symptoms
 daily daytime
 daily exercise induced
 exervation affecting sleep anda activity
 asthma control: uncontrolled
i. Clinical signs
 Wheezing, brethlesnowss, chest pain, rapid breat
 Harrison sulcus
 Prolonged expiration
 Generalized rhonchi
 Reduced air entry
j. Management
 Asses control
 Compliance
 Asthma education
 Step up asthma treatment
5. Leukemia (R319, R416)
Leukemia
a) Dx
Acute lymphoblastic leukemia
b) 2 relevant lab ix
Fbc-pancytopenia
Peripheral blood film-increase blastocyst more than 20%
Bone marrow aspiration and trephine biopsy-increase blastocyst more than 20%,erythroid
hyperplasia
immunophenotyping-find out the lineage of the leukemia
c) Give 4 phases of treatment for this condition.
Induction
CNS prophylaxis
Consolidation
maintenance

 3 years old child with fever and epitaxis, pallor, generalised lymphadenopathy, purpuric rash
and hepatosplenomegaly.
a) 3 differential diagnosis [1.5m]
ALL, AML, Lymphoma
b) Provisional diagnosis and justify [1.5m]
ALL since ALL is very common in children
There is generalised lymphadenopathy and hepatosplenomegaly
There is also pancytopenia features evident by pallor,epistaxis and purpuric rash
c) 2 investigation to confirm diagnosis [2m]
FBC – pancytopenia
Bone marrow aspiration and trephine biopsy– increase blastocyst more than 20%
Peripheral blood film-blastocyst more than 20%
6. Viral croup/epiglottis (not common nowadays)
R219, R218, R418, R116, R616
13 months girl came to aed with respiratory distress, has history of coryza for 2-3 days, rapid
breathing since evening, there is mild recessions of subcostal, and wheezing. (Acute bronchiolitis)
k. gives 2 important diff diag.
Acute bronchiolitis
Viral croup
l. how to clinically differentiate between the 2, and lab test to differentiate the 2.
Clinical
Viral croup-triad of hoarseness of voice,inspiratory stridor and barking cough
Acute bronchiolitis-wheezing,reduced air entry and fine inspiratory crackles

Lab test
Macam no lab investigation

3) outline mx

 Acute stridor, drooling saliva, sit in tripod position

a) Differential diagnosis
 Epiglotitis
 Viral croup
 Bacterial tracheitis
 Retrophayngeal abscess
 Foreign body obs
b) Investigations with findings to confirm diagnosis
 Lateral chest xray showing thumb sign
 Full blood count-increase leucocytosis
 Blood culture-to find out the etiological agent
c) Illustrate diagram why tripod position
Sitting upright with arms at back and neck extended to maximize the airway calibers

Sniffing-immobile,sitting straight,head held forward,mouth open and jaw thrust forward

 A child presents with noisy breathing and cough. Father has a history of URTI the previous
week. On examination, there is stridor, no wheezing and crackles.
a) Provisional dx (1M)
Croup
b) 3 differential dx (1.5M)
Bacterial tracheitis
Acute epiglottitis
Foreign body
c) Management (2.5M)
this is mild viral croup
can start oral dexamethasone or IV prednisolone or nebulised budesonide

if moderate-stridor with intercostal,subcostal or sternal recession

start dexamethasone,with ot without nebulised budesonide and oxygen
if no improvement-start nebulised adrenaline 1:1000 0.5mls/kg with maximum dose of 5mls

if severe-stridor,recession,decrease air entry and altered level of consciousness

start nebulised adrenaline conc of 1:1000 0.5mls/kg with maximum 5mls with
oxygen,dexamethasone and nebulised budesonide
if no improvement,intubation
7. SEPTIC ARTHRITIS R319
5 years old child fever with right knee joint pain, refuse to move the joint and stay in fix position
a) differential diagnosis
septic arthritis
HSP
Acute rheumatic fever
Reactive arthritis
b) Investigations
Full blood count-leucocytosis
Esr and crp-elevated
Joint aspiration-cloudy,WBC more than 50000cells/mm3
c) if the joint infected, what is commonest organism
Staph aureus

8. 2 years child with abdominal pain,...having stool mixed with blood sometimes

a) possible organisms
Shigella
Enterohemorrhagic E.coli
Salmonella
Campylobacter jejuni
Enteroinvasive E.coli
Clostridium difficile

b) what are 3 findings in stool microscopic examination

Ova
The parasites
RBC
Leukocytes
bacteria

c) Outline management
Admit and monitor vital signs
Analgesia for the pain
Maintain hydration
Assess the hemoglobin status.If symptomatic anemia,blood transfusion should be done
Find the cause and treat the cause.Antibiotics such as ciprofloxacin for the bacterial cause
of dysentery
9. Breastfeeding (how to give) R319, R218, R518, R116, R316, R516, R616
A mother come to you, ask for advices regarding breastfeeding
a) Is there any situation that the mother cannot give breastfeeding?
If mother is taking anti-thyroid or radioisotopes
If baby has phenylketonuria or galactosemia
b) Suggestions for breastfeeding in working mother
Can use the breast pump and store the milk in the refrigerator
c) What is calorie intake for her?

 Breastfeeding
a) List 4 advantages of breastfeeding
Readily available
Free and at suitable temperature
Create bonding between mother and infant
Can prevent obesity in baby
Give passive immunity since it contain immunoglobulin
b) List 2 contraindication
Refer above
c) List 3 Difference between breast milk and cow milk
 Cow contain more iron than breast milk
 Cow contain more protein than breast milk
 Iron in breast is readily absorbable compared to cow

 Breastfeeding
a) Benefit to infants (2M)
Ideal nutrition
Protective against diseases and infections
Improve cognitive development
Enhance mother baby bonding
b) 2 contraindications for breast feed (1M)
Mother has breast cancer.
Ongoing chemotherapy.
c) Differences between colostrum n mature milk (2M)
Colostrum has higher protein, IgA, lactoferrin, lactoalbumin and cholesterol; but lower
energy, total fat, casein and lactose content than mature milk.

 Breast milk
a) 4 advantages of breast feeding to baby [2m]
Ideal nutrition
Protective against infection and diseases
Improve cognitive development
Enhance mother-child bonding
b) 3 Contraindications of breast feeding [1.5m]
Mother has HIV, malignancy, on certain drugs, on chemotherapy or undergoing radiation
therapy.
c) 3 Differences between cow milk and breast milk [1.5m]
Breast milk has lower energy, protein, fat, sodium, calcium, phosphorus and casein:whey
ratio; but has higher carbodydrates and iron content than cow’s milk.
10. Nutritional Anemia: 3types (pyq: definition, ix, blood film features) R119, R319
A 8 months old Sabahan girl come to hospital with pallor for 2 weeks
 Give 2 ddx
 Thalassemia
 Iron deficiency anaemia
 Give 4 ix with relevant findings
 Fbc: low hb level
 Rbc indices: low mcv and mch indicates mchc anemia
 Reticulocyte count: elevated to exclude bone marrow involvement such as aplastic anemia
or infiltrative disease
 Iron study: low serum ferritin, high tibc, low serum iron
 Mentzer index:
 Hb electrophoresis: Hba decreased or absent for major, elevated for carrier Hba2
 Pathophysiology of common genetic inherited anaemia
 Thalassemia is an autosomal recessive disorder where genetic mutation leads to
decreased or absent production of alpha or beta globin chain of haemoglobin
 Severity depends on extent of deficiency of globin chain synthesis
 In beta thalassemia major, beta chain synthesis may be absent b0 or variably reduced b+
 Normally, alpha and beta chain are synthesised in equal amount, for necessary normal
function of hb
 Thalassemia major path roles are ineffective erythropoiesis and extravascular haemolysis
 In b thalassemia, synthesis of beta chain is deficient, imbalance of alpha and beta chain
 Excess alpha chain will precipitate inside rbc and form inclusion, these inclusions cause a
variety of untoward effects, such as membrane damage. Many rbc succumbs to membrane
damage and undergo apoptosis, 70-85% rbc undergoes this mechanism, ineffective
erythro,
 The remaining bear inclusion and membrane damage are prone to splenic sequestration
and extravascular haemolysis
 Ineffective erythro also lead to increased erythropoitetic drive. Erythroid hyperplasia in the
marrow and extramedullary erythropoiesis. Erythroid hyperplasia leads to increase in rbc
precursor in marrow which erodes bony cortex, impairs bone growth and cause skeletal
defects. Extramedullary happens in spleen liver and lymph node
 Excessive absorption of iron from gut due to ineffective erythro. Cause low hepcidin level
which is a negative regulator for iron absorption, leading to iron overload

Anemia
a) Define nutritional anemia and what causes it.
Reduced hemoglobin for age and sex which is due to reduction of certain nutrients intake
b) Name 2 causes of microcytic, hypochromic anemia.
IDA
Thalassemia
c) What is the most common of nutritional anemia and the management?
IDA
Increase iron rich food intake
Can give hematinics to increase hemoglobin level for 3-4 months
If symptomatic or Hb is severely low can transfuse packed red cells
Find the cause and treat the cause
11. Bilirubin metabolism (causes of acute hepatitis children) R319
(a) Briefly explain bilirubin metabolism.
Hemoglobin will be apoptosis into heme and globin where globin will be reused for new HB
Heme will be converted to biliverdin and then to bilirubin
Bilirubin will be brought to liver by albumin
Hepatocytes uptake of bilirubin and conjugation of bilirubin by UDP glucuronyl transferase
to form conjugated bilirubin
The conjugated bilirubin is then passed through the extrahepatic tract into the duodenum
and is then excreted through the urine and stool
Some of the conjugated bilirubin leaked into the systemic circulation
(b) What are the investigations to be done when there is liver damage?
LFT
Infective screening
Imaging
(c) What are the type of viral hepatitis that are transmitted by parenteral route?
Viral hepatitis B,C,D

12. AGN R119, R219

The 9 years old boy presented with Haematuria, edema, hypertension, no skin rash other PE were
normal
a) Give the pdx and ddx
Acute glomerulonephritis
HSP
Nephrotic-nephritis syndrome
b) Give 4 ix
Full blood count-maybe decrease hemoglobin
Urinalysis-red cell casts,leukocytes,nitrites and protein 2+
RP-increase creatinine,hyperkalemia
Complement levels-C3 will be low at early onset but normalized after 6 weeks and C4 is
normal
ASOT-raised in PSAGN more than 200IU/ml
Throat swab-for evidence of GABHS infection

c) What is the Pathophysiology

d) There is formation of immune complexes which is deposited into the glomerular basement
membrane causing decrease to the GFR.This lead to decrease urine output and can cause
fluid overload thus leading to hypertension and also electrolyte imbalance.This is known as
AKI.There is also inflammation and injury to the basement membrane which can lead to
hematuria and proteinuria
 e) Outline management
Penicillin V given to eradicate the residual infection
Strict input output chart
Fluid and salt restriction with normal protein
Diuretics such as furosemide can be given
Management of complication such as hypertensive encephalopathy,hyperkalemia and acute
pulmonary edema
Hyperkalemia
 IV 10% calcium gluconate
 IV insulin with IV dextrose 10%
 IV 8.4% sodium bicarbonate for associated metabolic acidosis
 salbutamol
 Ion-exchange resins
 Dialysis
Hemodialysis only indicated in:
 Failure of conservative management
 Severe derangement such as metabolic syndrome,hyperkalemia
 APO or hypertension due to fluid overload
 Multisystem failure