Pediatric Nursing 5

- pediatric diseases: either unique to children or

taking distinctive forms in children

- diseases of infancy (first year of life): highest

risk of mortality

- neonatal period (first 4 weeks of life): most

hazardous time
 Congenital anomalies
 - structural defects present at birth
 - important cause of infant mortality

 Malformations
 - primary error of morphogenesis (abnormal development)
 - usually multifactorial
 - single or multiple organs may be involved

 Disruptions
 - secondary destruction of normally developed organ
 - not heritable (no risk of recurrence in subsequent
pregnancies)
- amputation of limbs by amniotic bands
 Deformations
 - compression of growing fetus (uterine constraint)
 - malformed uterus, leiomyoma, multiple fetuses
 Agenesis
 - complete absence of the organ
 Hypoplasia
 - incomplete development or underdevelopment of the
organ
 Atresia
 - absence of opening of the hollow organ (intestine, bile
ducts)
Etiology of congenital anomalies
 Genetic causes
 - chromosomal abnormalities, gene mutations
 Environmental influences
 Infections
 - rubella (rubella embryopathy - eliminated by vaccination),
toxoplasmosis, syphilis, CMV
 Maternal diseases
 - diabetes mellitus: diabetic embryopathy (macrosomia, cardiac
anomalies, neural tube defects)
 - drugs: thalidomide (limb malformations - phocomelia), warfarin
 - alcohol: fetal alcohol syndrome: growth retardation, facial
anomalies (microcephaly, maxillary hypoplasia)
 - cigarette smoking: spontanneous abortion, placental anomalies,
low birth weight, SIDS
 - irradiation
Perinatal infections
 Transplacental
 - mostly viral and parasitic, a few bacterial infections
 - most important infections: TORCH (Toxoplasma, Other, Rubella,
CMV, Herpes)
 - toxoplasma: hydrocephalus, brain calcifications, chorioretinitis
 - rubella: cataract (blindness), deafness, heart anomalies

 Transcervical (ascending)
 - during pregnancy (infected amniotic fluid) or delivery
 - Streptococci, Neisseria gonorrhoeae, herpes virus
 - usually associated with inflammation of placental membranes
(chorioamnionitis) and umbilical cord (funisitis)
Prematurity
 - gestational age less than 37 weeks
 - second most common cause of neonatal mortality
 - higher incidence of morbidity

 Risk factors:
 - premature rupture of membranes
 - intrauterine infections (chorioamnionitis)
 - anomalies of placenta, uterus and cervix
 - twin pregnancy

 Complications:
 - respiratory distress syndrome
 - necrotising enterocolitis
 - CNS bleeding
Fetal Growth Restriction
 - one third of infants born in term but undergrown
(weight less than 2.500 g): „small-for-gestational-
age“ (SGA)
 - not only increased morbidity and mortality in
perinatal period, but also problems in adult life
(cerebral dysfunction, learning disability)

 Causes:
 Fetal
 - chromosomal disorders, congenital anomalies,
congenital infections
 - symmetric growth restriction (all organ systems
affected equally)
Fetal Growth Restriction
 Placental
 - impaired uteroplacental supply
 - placenta previa, placental abruption,
placental infarction
 - asymmetric growth restriction (brain spared
relative to visceral organs)
 Maternal
 - preeclampsia, chronic hypertension, alcohol,
drugs, smoking, malnutrition
 Unknown
Respiratory Distress Syndrome
 Risk factors
 - prematurity (60% before 28th week, only 5% after 37th
week)
 - maternal diabetes, cesarean section, twins
 Pathogenesis
- immature lungs cannot synthesize surfactant (complex of
phospholipids reducing surface tension within alveoli) →
alveoli tend to collapse → greater inspiratory effort →
atelectasis (loss of lung volume) → hypoxia → epithelial and
endothelial damage → hyaline membranes
RDS
 Morphology
 - airless, heavy lungs, mottled color
- congestion, solid appearance with collapsed alveoli
- hyaline membranes (necrotic cellular debris, extravasated fibrin)

 Complications (administration of high concentration of

oxygen)
 Bronchopulmonary dysplasia
 - arrested development of alveolar septation → reduced number
of mature alveoli and interstitial fibrosis → honeycomb lung
 Retrolental fibroplasia
 - retinal vessels proliferation → blindness
Necrotizing enterocolitis (NEC)
 - premature infants
 - intestinal ischemia (hypoperfusion)
 - bacterial colonisation of the gut
 - administration of formula feeds

 Macro:
 - terminal ileum, cecum, right colon most commonly involved
 - distended, friable, congested or gangrenous gut segment
 - intestinal perforation → peritonitis

 Micro:
 - mucosal or transmural necrosis
 - ulceration
 - submucosal gas bubbles
 - reparative changes: granulation tissue, fibrosis → post-NEC
strictures
Perinatal brain injury
 - premature infants

 Intraparenchymal hemorrhage
 - within germinal matrix
 - subependymal location
 - primitive neural cells and thin-walled vessels
 - persists until 35th week
 - hypoxia → endothelial damage → hemorrhage
 - ventricular system (hematocephalus)
 - death, survivors: scarring → obstructive hydrocephalus

 Infarcts
 - supratentorial periventricular white matter
 - residual changes: chalky white plaques (mineralisation), large
cystic lesions (multicystic encephalopathy)
Sudden Infant Death Syndrome
 “sudden death of an infant under 1 year of age which remains
unexplained even after complete autopsy, examination of the
death scene and revision of clinical history“

 - infant usually dies while asleep („crib death“)

 - leading cause of death in infancy (USA: 3,000 cases annually)
 - in 90% of cases infants younger than 6 months
- unknown cause; hypothesis: delayed development of some
regions of brainstem (arcuate nucleus) → impaired arousal
response to noxious stimuli
SIDS
 Risk factors:
 Maternal
 - young age, smoking during pregnancy, drug abuse (either
parent), short intergestational intervals, low prenatal care,
low socioeconomic status
 Infant
 - brain stem abnormalities (defective arousal and
cardiorespiratory control), prematurity, male sex, multiple
birth, SIDS in earlier sibling, respiratory infections
 Environment
 - prone sleep position, sleeping on soft surface, hyperthermia,
passive smoking
Autopsy findings
- multiple petechiae on thymus, pleura and
epicardium
lung congestion, lung edema
- brain stem abnormalities (hypoplasia of arcuate
nucleus, decrease of neuronal population)

SIDS is diagnosis of exclusion; to be excluded:

- infections (viral myocarditis, bronchopneumonia)
- congenital anomalies
- fatty acid oxidative disorders (medium chain acyl-
coenzyme A dehydrogenase deficiency)
- arrythmia (prolonged QT interval)
- traumatic child abuse („shaken baby syndrome“)
Fetal Hydrops
 - generalized edema of fetus (hydrops fetalis) or
localized forms (pleural or peritoneal effusion)

 Causes:
 - fetal anemia
 - immune hydrops (Rh and AB0 incompatibility)
 - non-immune hydrops (α-thalassemia, parvovirus
B19)
 - chromosomal abnormalities (trisomies, Turner
syndrome)
 - cardiovascular abnormalities (heart defects)
 - infections (CMV, syphilis, toxoplasmosis)
 - „twin-twin transfusion“ syndrome
Immune hydrops
 Immune hydrops
 - hemolysis of fetal RBCs induced by maternal antibodies
 - Rh or AB0 blood group incompatibility between mother and fetus
 - fetus inherits blood group antigens from father, that are foreign to
mother (mother Rh-negative - fetus Rh-positive; mother 0 - fetus A
or B)

- fetal RBCs reach maternal circulation (during last trimester of

pregnancy or during delivery) → mother senzitized and develops
antibodies
- next pregnancy: antibodies traverse placenta → destruction of
fetal RBCs → fetal anemia → tissue ischemia → cardiac failure
→ edema
Morphology of immune hydrops
 Morphology
 - pale fetus and placenta
 - hepatosplenomegaly (congestion from cardiac failure)
 - hyperplasia of erythroid precursors within bone marrow
 extramedullary hematopoiesis (liver, spleen, kidney, lungs)
 - presence of immature red cells in peripheral blood
(erythroblastosis fetalis)
 - hemolysis → unconjugated hyperbilirubinemia → toxic
damage of brain (basal ganglia and brain stem) →yellow hue
due to deposition of bilirubin pigment („kernicterus“)
Tumors of Infancy and Childhood
 Tumor-like lesions

 Hamartoma = focal overgrowth of tissue in organ where it

normally occurs
 - linkage between malformations and true tumors (dividing
line often not clear and variously interpreted)
 - hemangioma, lymphangioma, heart rhabdomyoma

 Choristoma = presence of normal cells in abnormal location

 - little clinical significance, may be confused with true tumors
 - pancreatic tissue in stomach wall, adrenal cortex in kidney,
lung, ovary
Benign Tumors
 Hemangioma (cavernous or capillary)
 - skin (face, scalp)
 - flat to elevated red blue mass („port wine stain“)
 - enlargement or spontaneous regression
 - rarely component of hereditary disorders (von Hippel-Lindau sy,
Sturge-Weber sy)

 Lymphangioma
 - cystic and cavernous lymphatic spaces
 - skin or deep tissues (neck, axilla, mediastinum, retroperitoneum)
 - tend to enlarge after birth → compression of adjacent structures
 -
Sacrococcygeal teratoma
- most common germ cell tumor in
childhood
- 10% of cases associated with congenital
anomalies of cloacal region and neural tube
defects (spina bifida, meningocele)
- majority of cases mature (benign)
- rarely immature (malignant)
Malignant Tumors
 - hematopoietic system, CNS and soft tissues most
commonly involved

 - primitive („embryonal“) microscopic appearance: „small

round blue cell tumors“

 - tendency to differentiate into more mature elements

 - improved survival due to chemotherapy and

radiotherapy → effort paid to minimizing of delayed
adverse effects of treatment (secondary neoplasms)
Neuroblastoma
 - derived from primordial neural crest cells populating adrenal
medulla and sympathetic ganglia
 - second most common solid malignancy in childhood (7-10%
of all pediatric neoplasms; 50% of all pediatric malignancies)
 - most sporadic, a few autosomal dominant transmission
(often multiple)
- from minute nodules to large masses

 Macro
 - soft, gray-tan tissue, areas of necrosis, cystic change and
hemorrhage
Micro Neuroblastoma
- solid sheets of small primitive cells (neuroblasts)
with dark nuclei and scant cytoplasm
- faintly eosinophilic fibrillary background
(processes of neuroblasts)
- Homer-Wright rosettes
- signs of maturation (spontaneous or therapy-
induced):
- ganglion cells admixed with neuroblasts
(ganglioneuroblastoma)
- ganglion cells and Schwann cell stroma
(ganglioneuroma)
Micro neuroblastoma
 - metastatic spread to liver, lungs and bones
 - production of catecholamines →
metabolites vanillylmandelic acid (VMA)
and homovanillic acid (HVA) within urine
(screening markers)
Retinoblastoma
 - most common malignant eye tumor in childhood
 - may be congenital
 - sometimes spontaneous regression
 - high incidence of second primary tumors
(osteosarcoma, soft tissue tumors)
 - sporadic (always unifocal and unilateral) or familial
(often multiple and bilateral)
- from neuroepithelial cells of posterior retina
 Macro Retinoblastoma
 - nodular mass with satellite seedings

 Micro Retinoblastoma
 - small rounded cells with large dark nuclei
 - Flexner-Wintersteiner rossetes

 - metastatic spread to CNS, bones and lymph nodes

 - poor vision, strabismus, whitish hue to pupil (“cat’s eye
reflex”)
 - untreated fatal, early treatment (enucleation,
chemotherapy, radiotherapy) → survival
Nephroblastoma (Wilm’s Tumor)
 - most common primary renal tumor in children

 Macro
 - large well-circumscribed mass
- soft, homogeneous, tan to gray, areas of hemorrhage, necrosis
or cystic degeneration

 Micro
 - histology reveals some recapitulation of nefrogenesis
 - three components: blastema, stroma and epithelium
 - blastema: sheets of small blue cells
 - stroma: spindle cells (fibroblasts), heterologous elements
(skeletal or smooth muscle, cartilage, osteoid, neurogenic tissue)
 - epithelium: abortive tubules and glomeruli
Nephroblastoma (Wilm’s Tumor)
 Clinical presentation
 - palpable abdominal mass, fever, abdominal pain,
hematuria, intestinal obstruction

 - generally good prognosis with appropriate therapy

(nephrectomy and chemotherapy)