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Papers of the Week


2022 May


Exp Clin Transplant


20


Suppl 3

Long-Term Outcomes of Patients With Progressive Familial Intrahepatic Cholestasis After Biliary Diversion.

Authors

Öztürk H, Sarı S, Sözen H, Eğritaş Gürkan Ödül, Dalgıç B, Dalgıç A
Exp Clin Transplant. 2022 May; 20(Suppl 3):76-80.
PMID: 35570606.

Abstract

Progressive familial intrahepatic cholestasis is a heterogeneous group of genetic disorders characterized by disrupted bile homeostasis. Patients with this disease typically present with cholestasis and pruritus early in life and often progress to end-stage liver disease. The clinical symptoms that patients with progressive familial intrahepatic cholestasis encounter are usually refractory to medical treatment. Although the effects of biliary diversion surgery on native liver survival are not exactly known, this procedure may provide a positive impact on pruritus and laboratory parameters in these patients.