Long-term outcome after partial external biliary diversion for progressive familial intrahepatic cholestasis

Total biliary diversion as a treatment option for patients with progressive familial intrahepatic cholestasis and Alagille syndrome

“…As reported, TBD was performed directly or secondary to partial biliary diversion when results of this procedure were unsatisfactory or bile drainage was inadequate. Early postoperative (bleeding and biliary leakage) and long-term complications (stoma obstruction or prolapse and cholangitis) were similar to those complications encountered after PEBD [2,7,15,17].…”

“…Even better results were reported when PEBD was performed in non-cirrhotic patients [3,5]. In some individuals, PEBD was able to reverse hepatic fibrosis and reduce disease progression [1][2][3]5,7,15]. Therefore, PBD is now considered to be the first treatment in non-cirrhotic individuals with low-GGT PFIC (type I and II) [5].…”

Normalization of serum bile acids after partial external biliary diversion indicates an excellent long-term outcome in children with progressive familial intrahepatic cholestasis

“…Patients with PFIC type 2 manifested more severe hepatobiliary disease, whereas patients with PFIC type 1 showed greater evidence of extrahepatic disease. Progressive familial intrahepatic cholestasis type 3, which is caused by a deficiency in multidrug resistance protein 3, is associated with elevated serum GGT levels [7,8].…”

The histidin-loop is essential for transport activity of human MDR3. A novel mutation of MDR3 in a patient with progressive familial intrahepatic cholestasis type 3

“…It has been reported previously, that patients with MDR3 missense mutations, but not those with premature stop codons have a favorable response to UDCA and remain transplant free for a longer time . However, recurrence of symptoms is conceivable and a partial biliary diversion (Halaweish and Chwals, 2010) and/or a liver transplantation may be needed when end stage liver disease develops. We conclude from the observation of the novel mutation H1231Y that histidine 1231 of human MDR3 is essential for proper MDR3 function.…”