Phenotypes for disease #02418 (PFIC3 (cholestasis, intrahepatic, familial, progressive, type 3 (PFIC-3)), OMIM:602347)

3 entries on 1 page. Showing entries 1 - 3.
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AscendingPhenotype ID     

Phenotype details     

Diagnosis/Initial     

Diagnosis/Definite     

Inheritance     

Age/Examination     

Age/Diagnosis     

Age/Onset     

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Owner     

Individual ID     
0000041472 3m-recurrent bouts of jaundice, presented with severe icterus, diarrhea, fever, pruritus; 3y-hepatosplenomegaly, elevated serum liver enzymes, increased γ-GT activity (6x), high serum bile acid concentration (50 x)., prothrombin time 0.50 (normal >0.70); liver biopsy revealed nonspecific portal inflammation, extensive portal fibrosis, cirrhosis, no response to UDCA treatment; 3.5y orthotopic liver transplantation for complications liver cirrhosis, extrahepatic bile ducts patent at time liver transplantation; 6y good clinical condition - - Isolated (sporadic) - - 00y03m - - Johan den Dunnen 00054818
0000041473 8m recurrent episodes severe pruritus; 3y hepatosplenomegaly, strongly increased serum γ-GT activity (38x), increased serum bile acids (16x), serum aspartate and alanine aminotransferase activities mildly elevated, prothrombin time 0.10 (normalized after vitamin K1 injection); liver histology ductular proliferation, extensive portal fibrosis; cholangiography normal intra-/extrahepatic bile ducts; 4y-UDCA treatment ineffective, liver enzymes and liver histology further deteriorated toward end-stage liver disease with ascites and severe jaundice; 9y-liver transplantation, now good clinical condition - - Isolated (sporadic) - - 00y08m - - Johan den Dunnen 00054819
0000041516 PFIC; 6y received liver graft - - Isolated (sporadic) - - - - - Johan den Dunnen 00054853
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