Preoperative observations and short-term outcome after partial external biliary diversion in 13 patients with progressive familial intrahepatic cholestasis

Arnell, Henrik; Bergdahl, Sven; Papadogiannakis, Nikos; Németh, Antal; Fischler, Björn

doi:10.1016/j.jpedsurg.2007.10.055

Cited by 40 publications

(71 citation statements)

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“…Rifampicin may alleviate pruritus. Partial biliary diversion and ileal exclusion have been reported in case series to improve signs and symptoms of particularly PFIC1 and also PFIC2 [173,174]. Liver transplantation is the recommended treatment of end-stage disease in PFIC.…”

Section: Therapymentioning

confidence: 99%

EASL Clinical Practice Guidelines: Management of cholestatic liver diseases

2009

Journal of Hepatology

1,496

529

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Section: Therapymentioning

confidence: 99%

EASL Clinical Practice Guidelines: Management of cholestatic liver diseases

2009

Journal of Hepatology

1,496

529

View full text Add to dashboard Cite

“…Symptomatic treatment with ursodeoxycholic acid (UCDA) has shown clinical efficacy in only 60% of patients [2]. Liver transplantation, which is the only definitive treatment of this condition, is limited by organ availability and may be associated with significant morbidity and mortality [3,4]. Alternative surgical treatments include partial biliary diversion (PBD) via a hepaticojejunal or a cholecystojejunal Roux-en-Y with a cutaneous loop stoma, a cutaneous end cholecystojejunostomy or cholecystoappendicostomy, a partial internal biliary diversion via a jejunal conduit connecting the gallbladder with the colon, and an ileal bypass [5][6][7].…”

mentioning

confidence: 99%

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

Halaweish

Chwals

2010

Journal of Pediatric Surgery

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“…Previous reports [3,[5][6][7][9][10][11][12][13] have noted therapeutic effect from PEBD in patients with PFIC1. However, such benefits are not seen in all PFIC1 patients.…”

Section: Discussionmentioning

confidence: 88%

Clinical Course Following Partial External Biliary Diversion for Progressive Familial Intrahepatic Cholestasis 1: A Report of Two Patients

Kimura

Abukawa²,

Takei³

et al. 2016

Journal of Gastroenterology and Hepatology Research

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AIM:To better understand the effect of partial external biliary diversion (PEBD) in patients with progressive familial intrahepatic cholestasis 1 (PFIC1). MATERIALS AND METHODS:We analyzed quantitative changes in the concentrations of individual bile acids in serum, urine, and biliary bile, and concomitant changes in symptoms such as pruritus, in 2 patients whose PFIC1 clinically evolved to resemble the course of benign recurrent intrahepatic cholestasis (BRIC) upon treatment with PEBD. RESULTS: Patient 1 showed good therapeutic effect after PEBD including improved liver function test results and decreased total bile acids in serum, as well as decreased pruritus. The cholic acid (CA)/chenodeoxycholic acid (CDCA) ratio in biliary bile also decreased. Patient 2, however, experienced little decrease in pruritus after PEBD, and showed no clear change in the CA/CDCA ratio. Nonetheless, this patient's course did not rapidly worsen. CONCLUSION: In PFIC1, increased bile secretion and a decreased CA/CDCA ratio in biliary bile may be the most important responses after PEBD. Relief of pruritus is also an important sign of therapeutic effect. Moreover, progression of symptoms in PFIC1 sometimes can be delayed by PEBD, resulting in a relatively mild BRIC-like course, even when the CA/CDCA ratio and pruritus show little change. However, mechanisms underlying clinical benefits remain obscure. Abbreviations PFIC1: progressive familial intrahepatic cholestasis 1; GGT: γ-glutamyltransferase;

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Preoperative observations and short-term outcome after partial external biliary diversion in 13 patients with progressive familial intrahepatic cholestasis

Cited by 40 publications

References 30 publications

EASL Clinical Practice Guidelines: Management of cholestatic liver diseases

EASL Clinical Practice Guidelines: Management of cholestatic liver diseases

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

Clinical Course Following Partial External Biliary Diversion for Progressive Familial Intrahepatic Cholestasis 1: A Report of Two Patients

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