20 July 2021
: Case report
A Report of 2 Infant Siblings with Progressive Intrahepatic Familial Cholestasis Type 1 and a Novel Homozygous Mutation in the ATP8B1 Gene Treated with Partial External Biliary Diversion and Liver Transplant
Unusual clinical course, Unusual or unexpected effect of treatment, Rare disease, Congenital defects / diseases
Irena Jankowska1ABCDEF, Joanna Pawłowska1ACDE, Marek Szymczak2BDE, Hor Ismail2BDE, Dorota Broniszczak2BDE, Joanna Cielecka-Kuszyk3BCE, Piotr Socha1ACDE, Dorota Jarzębicka1BDEF*, Piotr Czubkowski1ABCDEFDOI: 10.12659/AJCR.932374
Am J Case Rep 2021; 22:e932374
Figure 1. Liver biopsies after LTx – Case 1. (A) Mixed macro- and microvesicular steatosis (95%) and portal tract without fibrosis, without ductular proliferation (H&E staining). (B) Mixed macro- and microvesicular steatosis (70%), portal and periportal fibrosis without ductular proliferation (Azan staining). (C) Mixed macro- and microvesicular steatosis (40%), portal tract without fibrosis, without ductular proliferation, mild inflammatory infiltrates (H&E staining). (D) Mixed macro-and microvesicular steatosis (30%), fibrous septa, and mild inflammatory filtrates (H&E staining).






