Partial External Biliary Diversion for Severe Diarrhea After Liver Transplant in Patients with Progressive Familial Intrahepatic Cholestasis Type 1

Exp Clin Transplant. 2022 May;20(Suppl 3):81-84. doi: 10.6002/ect.PediatricSymp2022.O27.


Progressive familial intrahepatic cholestasis is a heterogeneous group of autosomal recessive disorders, and liver transplant is the only curative treatment. A biliary diversion operation for disruption of enterohepatic circulation in patients with progressive familial intrahepatic cholestasis type 1 without cirrhosis is another option. We present a pediatric patient with progressive familial intrahepatic cholestasis type 1 who underwent liver transplant due to end-stage liver disease. After transplant, diarrhea and growth retardation complications resolved after partial external biliary diversion surgery.

MeSH terms

  • Child
  • Cholestasis, Intrahepatic* / diagnosis
  • Cholestasis, Intrahepatic* / etiology
  • Cholestasis, Intrahepatic* / surgery
  • Diarrhea / diagnosis
  • Diarrhea / etiology
  • Diarrhea / surgery
  • Humans
  • Liver Transplantation* / adverse effects
  • Treatment Outcome

Supplementary concepts

  • Cholestasis, progressive familial intrahepatic 1