Hepatocellular and Familial Cholestasis

摘要: Progressive familial intrahepatic cholestasis (PFIC), as a diagnosis, was for years an omnium-gatherum. The qualifiers of in PFIC mean that the liver disease does not resolve, but instead persists, and progresses to fibrosis or cirrhosis; within family siblings are at risk developing same proband (although, course, this actuality may never occur); biliary tract is abnormal, inference —not necessarily correct—from demonstration extrahepatic frankly obstructed. One can see misdiagnosed tyrosinemia, example, lies these bounds. Clinical data must be reviewed ensure usual causes pediatric have been adequately assessed before invoked, care taken used too loosely, “wastebasket” diagnosis.