North American Indian childhood cirrhosis (NAIC)
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Epidemiology
- originally described in Ojibway-Cree children from northwestern Quebec
Pathology
- intrahepatic cholestasis
- biliary cirrhosis, fibrosis around bile ducts
- biochemical & histopathological features suggest involvement of bile ducts rather than of bile canaliculi
Genetics
- autosomal recessive
- associated with defects in CIRH1A
Clinical manifestations
- transient neonatal jaundice, in a child who is otherwise healthy
- progresses to biliary cirrhosis & portal hypertension
Laboratory
- elevated gamma glutamyltransferase & alkaline phosphatase levels