| Summary: | Progressive familial intrahepatic cholestasis type 2 (PFIC-2), formerly known as Byler’s syndrome, is an autosomal recessive disorder. In infancy or early childhood, this disease leads to end-stage hepatic disease, in which liver transplantation is the only radical treatment. In general, liver transplantation outcomes are good, but in the long term, PFIC-2 may reoccur. We present a case where a girl, aged 28 months, who suffered from cirrhosis resulting from PFIC-2, underwent a related transplantation of the left lateral sector of the liver (her grandmother as the donor). Punch biopsy was performed 8 years after the liver transplant due to graft dysfunction. Histopathology revealed a recurrent PFIC-2. F4. Increased liver failure was the reason for retransplantation of the left lobe of the liver also from a related donor (mother). Pathological pictures in the biopsy specimen and in the liver removed during retransplantation were identical, which once again confirmed PFIC-2 recurrence.
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