Recent advances in the differential diagnosis of chronic cholestasis in the pediatric age group have made us aware of a heterogeneous group of children who have progressive familial intrahepatic cholestasis (PFIC). This particular case illustrates the importance of early recognition and treatment, which is surgical (partial biliary diversion) since - with proper surgical intervention before the onset of cirrhosis, or bridging fibrosis - the pruritus disappears and the progression to end-stage liver disease is halted. This case elucidates some of the typical historical, clinical, and pathological characteristics of progressive familial intrahepatic cholestasis.
|Original language||English (US)|
|Number of pages||3|
|State||Published - Jan 1 1995|
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health