Follow‐up in Children With Progressive Familial Intrahepatic Cholestasis After Partial External Biliary Diversion

Abstract

Biliary diversion should be regarded as the first choice of surgical treatment in noncirrhotic patients with severe ABCB11 disease and may also be efficacious in other forms of PFIC.

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

“…Partial biliary diversion (PBD) is currently regarded as the therapy of first choice for patients with progressive familial intrahepatic cholestasis (PFIC) associated with a low serum gammaglutamyl transpeptidase (GGT) [1][2][3][4][5]. This group comprises both PFIC type I (ATP8B1 deficiency) and PFIC type II (ABCB11 deficiency) [4].…”

Hepatobiliary scintigraphy during cholestatic and noncholestatic periods in patients with progressive familial intrahepatic cholestasis after partial external biliary diversion

“…We have recently described not only short-term relief of pruritus with decreasing biochemical cholestasis, but also catch-up in growth after PEBD in a majority of our patients with PFIC [7]. Further data suggest positive long-term effects including reversal of histologic cholestasis and fibrosis [8]. Still, the exact mechanisms explaining the positive effects of PEBD in most patients are still unknown.…”

“…Eight of the 9 included patients with mutations in ABCB11, thus suffering from PFIC type 2, whereas 1 patient showed no mutations in ATP8B1 or in ABCB11. They underwent PEBD at a median time of 10 (range, 4-13) years before inclusion in the study, and they all experienced shorter or longer periods (weeks to several months) of cholestasis after the surgical procedure as described elsewhere in further detail [8]. With few exceptions, these periods were preceded by a benign febrile illness, typically a viral upper respiratory tract infection, with a concomitant decrease in the volumes of bile excreted through the stoma.…”

Familial Intrahepatic Cholestasis

“…Partial external biliary diversion, ileal exclusion (IE) and LT are all recommended surgical treatment options [16,20,34,35]. Hepatocellular malignancy remains an indication for early LT in BSEP patients.…”