NEONATAL HEPATITIS
 
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NEONATAL HEPATITIS

 

DEFINITION:

A disorder of unknown etiology resulting in intrahepatic cholestasis in the newborn period.

EPIDEMIOLOGY:

  • incidence: ?
  • age of onset:
    • newborn
  • risk factors:
    • familial (in 20% of cases) and sporadic
    • M > F (2:1)
    • premature, small for gestational age

PATHOGENESIS:

1. Background

  • a disorder causing direct hyperbilirubinemia and jaundice due to persistent intrahepatic cholestasis
  • likely represents a group of disorders presenting in the newborn period with idiopathic intrahepatic cholestasis (i.e., a diagnosis of exclusion)
  • the sporadic cases probably represent unidentified viral or metabolic etiolgies

CLINICAL FEATURES:

1. Gastrointestinal Manifestations

1. Cholestasis

  • poor feeding
  • lethargy
  • hepatomegaly +/- splenomegaly
  • vomiting
  • irritability (pruritis)
  • steatorrhea
  • jaundice
    • in 80% of cases, jaundice appears in the first week of life while in the other 20%, jaundice may not appear until between 1-3 months of life

2. Complications

  • malabsorption of fats and fat-soluble vitamins (D,E,A,K)
  • failure to thrive
  • hepatic fibrosis with portal hypertension
  • cirrhosis with liver failure
  • hepatocellular carcinoma

INVESTIGATIONS:

1. Serum

  • conjugated hyperbilirubinemia
  • elevated bile salts, cholesterol, AST, ALT, alkaline phosphatase
  • normal or slightly elevated PT
  • normal albumin and GGT
  • vitamin D,E,A,K deficiencies

2. For Malabsorption

  • elevated 72 hour fecal fat content

3. Biopsy

1. Liver

  • numerous giant cells, lobular disorganization, necrosis, inflammation without neoductular proliferation
  • progression may lead to loss of interlobular bile ducts

MANAGEMENT:

1. Medical

1. Symptomatic

  • cholestyramine to reduce bile salt concentration and pruritis
  • medium-chain triglyceride-containing formulas (Portagen, Pregestimil, Alimentum)
  • supplementation with fat-soluble vitamins (D,E,A,K)
  • promote bile flow with phenobarbital or rifampin

2. Surgical

  • liver transplanation for cirrhosis

3. Prognosis

  • complete recovery expected in 70-80% of cases within 6-8 months
  • risk factors for development of liver failure
    • familial hepatitis
    • failure of jaundice to resolve by 1 year of age
    • periportal inflammation and fibrosis

 

 

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