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Liver and intrahepatic bile ducts-nontumor

Hepatitis - noninfectious

Neonatal hepatitis


Reviewers: Komal Arora, M.D. (see Reviewers page)
Revised: 5 May 2012, last major update May 2012
Copyright: (c) 2004-2012, PathologyOutlines.com, Inc.

General
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● Neonatal cholestasis, usually due to prolonged conjugated hyperbilirubinemia
● Use of terminology has declined as specific causes are identified (Clin Liver Dis 2006;10:27)
● Affects 1/2500 live births
● Due to extrahepatic biliary atresia and neonatal hepatitis disorders (non-infectious and infectious)
● Some use terms neonatal hepatitis and giant cell hepatitis in neonates interchangeably
● Hypopituitarism is common recognizable clinical association (Am J Surg Pathol 2010;34:1498)
● Death in a few months or evolution to cirrhosis in 30% in some studies
● May be associated with mitochondrial DNA depletion
Symptoms: jaundice, dark urine, light or acholic stools, hepatomegaly; may have hypoprothrombinemia

Causes
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● Idiopathic (50%), bile duct obstruction (20%)
● Neonatal infection: CMV, congenital rubella, Coxsackievirus, echovirus, Hepatitis B, herpes simplex, rubella, sepsis, syphilis, Toxoplasmosis, urinary tract infection, varicella
● Metabolic disease: alpha-1-antitrypsin disease [15%], cystic fibrosis, defective bile acid synthesis disorders, galactosemia, Gaucher’s disease, Neimann-Pick disease, tyrosinemia), other genetic disorders (Alagille’s syndrome, Down’s syndrome, trisomy 17/18, Turner’s syndrome, Zellweger’s syndrome)
● Toxins: drugs, intravenous nutrition
● Other: Indian childhood cirrhosis, myeloproliferative disorder, shock/hypoperfusion

Case reports
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● Newborn boy with lactic acidosis, hepatomegaly, hypoglycemia, icterus, muscle hypotonia and abnormal mitochondria (Hum Pathol 2002;33:247)

Treatment
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● Palliative surgery for extrahepatic biliary atresia; definitive treatment for other causes
● Also supportive case, possibly liver transplant (Semin Neonatol 2003;8:357)

Micro description
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● Lobular disarray (evident at low power) with mildly inflamed portal tracts and giant cell transformation of hepatocytes (particularly around central vein), focal hepatocyte necrosis, prominent hepatocellular and canalicular cholestasis, extramedullary hematopoiesis, reactive changes in Kupffer cells
● No portal fibrosis, no/patchy ductular proliferation
● Note: in neonates, giant cell transformation is a nonspecific response to any hepatocyte injury

Micro images
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Alpha-1-antitrypsin related neonatal hepatitis: left - giant cell transformation and focal cholangiopathy; right - storage globules are readily demonstrated in this case (PAS-diastase)


CMV neonatal hepatitis

Negative stains
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● CD56

End of Liver and intrahepatic bile ducts-nontumor > Hepatitis - noninfectious > Neonatal hepatitis


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