Outcomes following partial external biliary diversion in patients with progressive familial intrahepatic cholestasis

Liver-directed gene therapy results in long-term correction of progressive familial intrahepatic cholestasis type 3 in mice

“…Partial external biliary diversion (PEBD) is the primary surgical intervention in the management of patients with PFIC and can postpone the need for eventual liver transplantation, but only if attempted before major liver damage has occurred. 10,11 We have to note that the reported number of PEBD performed in patients with PFIC3, as opposed to those with PFIC1 and PFIC2, is extremely low and that only a marginal therapeutic effect can be expected based on the pathophysiology. Liver transplantation is the only curative treatment for PFIC3.…”

Genotype correlates with the natural history of severe bile salt export pump deficiency

“…6,7 It has been observed that patients may benefit from surgical biliary diversion (SBD) procedures, such as partial external biliary diversion (PEBD) or ileal exclusion. [8][9][10][11][12][13][14][15][16][17] SBD aims to decrease the size of the bile acid pool by interrupting the enterohepatic circulation. Unfortunately, not all patients benefit from SBD and, at some point, many require a liver transplantation (LT) for refractory pruritus or end-stage liver disease.…”

Long‐term outcomes after cholecystocolostomy for progressive familial intrahepatic cholestasis

“…found that a significant reduction in TBIL was observed following PEBD in FIC1 patients but not in BSEP patients. Lemoine et al . reported that BA levels decreased in PFIC1 patients and in the single PFIC3 patient, but not significantly in PFIC2 patients following PEBD procedures.…”