Cholestasis After Pediatric Liver Transplantation-Recurrence of a Progressive Familial Intrahepatic Cholestasis Phenotype as a Rare Differential Diagnosis: A Case Report.

摘要: Abstract Introduction Nonobstructive cholestasis after pediatric liver transplantation is a common diagnostic and therapeutic dilemma. We describe girl with neonatal because of progressive familial intrahepatic 2 (PFIC-2) presence homozygous splice site mutation in the ABCB11 gene. Liver was performed end-stage disease at age 6. Cholestasis normal gamma-glutamyl transferase (GGT) developed 8 years transplantation. A biopsy showed canalicular giant cell hepatitis without evidence rejection, mimicking PFIC-2. Immunofluorescence staining human sections patient's serum revealed reactivity toward epitope, which could be identified as bile salt export pump (BSEP) using BSEP–yellow fluorescent protein (YFP) transfected cells. Our patient recurrence PFIC-2 phenotype due to production antibodies against BSEP (alloimmune [AIBD]). Intensification immunosuppressive therapy well antibody treatment plasmapheresis Rituximab were initiated, leading stabilization clinical condition depletion anti-BSEP serum. However, 1 year necessary again insufficiency. Afterward, immunomodulatory consisted tacrolimus, mycophenolate mofetil, prednisone, immunoadsorption, high-dose immunoglobulin (1 g/kg/d). Conclusion may indicate an AIBD phenotype. Besides enhancement therapy, plasmapheresis, immunoglobulins, B-cell represents option.