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61

Germinal Matrix

source of nerve cells in embryo and fetuses (up to 33 weeks of gestation.)

62

Neonatal Intraventricular hemorrhage

in long term survivors 

cavitations or pseudocysts surrounded by hemosiderin laden macrophages and gliosis.

 

 

63

Neonatal Intraventricular hemorrhage complication 

Hydrocephalus 

64

Physiological Jaundice

Elevated unconjugated bilirubin during first week of life.

 

65

Physiological Jaundice

2 functionally distinct periods

– Phase I – lasts 5 days in term infants and 7 days in preterm infants.

• Serum bilirubin level may reach 12-15 mg/dl

– Phase II – decline of serum bilirubin levels , lasts for 2 weeks.

66

Characterization of Pathological Jaundice

– Clinical jaundice appearing in first 24 hours

– Total bilirubin > 15 mg/dl ( Hyperbilirubinemia)

– Conjugated bilirubin > 2 mg/dl

67

Unconjugated Hyperbilirubinemia seen in

– Fetomaternal blood group incompatibility (Hemolytic Disease of the newborn)

– Crigler-Najjar syndrome type I & II

68

Conjugated Hyperbilirubinemia seen in

– Biliary atresia

– Neonatal hepatitis

69

Hemolytic disease of newborn

• Erythroblastosis Fetalis

• Antibody induced hemolytic disease in the newborn that is caused by blood group incompatibility between mother and fetus.

• Most common antigens – Rh (D) and ABO blood group antigens

70

Hemolytic disease of newborn

Pathogenesis

• Fetal RBCs reach maternal circulation in last trimester or during child birth

• Sensitization of mother to the foreign antigens – development of antibodies that can freely traverse the placenta to the fetus and cause hemolysis.

• Hemolysis leads to progressive anemia – intrauterine cardiac failure – Edema.

71

Hemolytic disease of newborn end results 

• Death in Utero – most extreme form of disease

• Hydrops Fetalis – most severe form in live born infants.

• Kernicturus – bilirubin encephalopathy

72

Findings in Hydrops Fetalis

• Atautopsy– hepatosplenomegaly and bile stained organs

• Microscopically–

– Erythroblastic hyperplasia of the bone marrow

– Extramedullary hematopoiesis in the liver, spleen.

73

Pathology – Kernicturus

disorder of newborns in which very high levels of unconjugated bilirubin bind to, and injure the immature neurons of the CNS

• Characterized by bile staining of the brain, particularly of the basal ganglia and brain stem.

74

Kernicturus dx

• Amniocentesis – high levels of bilirubin

• Human antiglobulin test (Coombs test) – Positive on fetal cord blood.

75

Kernicturus tx

– Exchange transfusion – Phototherapy

76

Prevention of Kernicturus

Human anti D globulin within 72 hours of delivery

77

Crigler-Najjar Disease

• Type I

recessively inherited disease

• Characterized by complete absence of UDP-glucuronyltransferase activity.

• Unremitting unconjugated hyperbilirubinemia leading to bilirubin encephalopathy.

• Most patients die within first year of life

78

Crigler-Najjar Disease

• Type II 

• Characterized by partial decrease in the activity of UDP-glucuronyltransferase activity.

• Hepatocytes have the capacity to synthesize this enzyme on treatment with Phenobarbital.

79

• Most frequent cause of chronic cholestasis in infants and children

• Most common indication for liver transplantation in this age group.

Biliary Atresia

80

Biliary Atresia

• Complete obstruction of the lumen of the extrahepatic biliary tree within the first three months of life.

• 1:10,000 live births

81

Biliary Atresia

Embryonic or fetal type – 20%

aberrant intrauterine development of the extrahepatic biliary tree

– early onset neonatal cholestasis

– No jaundice free period after physiological jaundice

– Associated congenital anomalies (malrotation of abdominal viscera, congenital heart disease)

82

Biliary atresia Perinatal type

– Normally developed biliary tree is destroyed following birth (virus induced injury to biliary epithelium)

– Late onset neonatal cholestasis (4-8 weeks)

– Jaundice free interval

– No associated congenital anomalies

83

Biliary Atresia

Morphology

• Inflammation and fibrosing stricture of the hepatic and common bile ducts

• Periductal inflammation of intrahepatic bile ducts – progressive destruction of intrahepatic biliary tree

• Features of extrahepatic biliary obstruction – Marked bile ductular proliferation

– Portal tract edema and fibrosis

– Parenchymal cholestasis

84

Biliary Atresia Prognosis

• 75% survival if operated in first 60 days of life

• 20-30% survival if operated after 90 days of life

• 80% survival with transplantation.

85

Idiopathic Neonatal Hepatitis

What is seen... excludes all other causes of hepatitis 

• Giantcell transformation. (diffuse prominent)

• Ballooning

• Acidophilic degeneration

• cholestasis

86

SIDS Risk factors 

75% of SIDS deaths have no associated risk factors.

 

87

Environmental risk factors for SIDS 

88

SIDS Pathogenesis

Multifactorial

• 1. Vulnerable infant

• 2. Critical developmental period in

homeostatic control. (arousal and cardiorespiratory)

• 3. One or more exogenous stressors (hypercarbia/hypoxia/thermal stress)

89

SIDS pathology

• Multiple petechiae (80%) in Thymus,

visceral/parietal pleura and epicardium.

• Lungs: Congestion +/- pulmonary edema

• Hypoplasia of arcuate nucleus in the brain stem.

• Decrease in brain stem neuronal populations in some cases.

90

SIDS Infectious differentials 

– Viral myocarditis

– Bronchopneumonia