Journal
JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION
Volume 51, Issue 4, Pages 494-499Publisher
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/MPG.0b013e3181df99d5
Keywords
chronic cholestasis; genotype; hepatic fibrosis; long-term follow-up; partial external biliary diversion; postoperative outcome; progressive familial intrahepatic cholestasis
Funding
- Samariten
- Jerring
- Frimurare Barnhuset
- Wera Ekstrom
- Kronprinsessan Lovisa Foundations for Pediatric Research
- Ronald McDonald Barnfond
- Sallskapet Barnavard
- Axel Werkells Minnesfond
- CLINTEC/Karolinska Institutet
- Swedish Society of Medicine
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Objectives: The aim of this study was to examine whether reversion of histological fibrosis followed partial external biliary diversion (PEBD) in patients with progressive familial intrahepatic cholestasis (PFIC); whether the duration of cholestatic episodes after PEBD influenced the evolution of fibrosis; and whether genotyping was helpful in predicting outcome of PEBD. Patients and Methods: Children with PFIC who underwent PEBD were investigated with genetic, biochemical, and anthropometric standard methods. Serial liver specimens were assessed histologically without knowledge of genotype and outcome. Findings were evaluated in the contexts of the total duration of cholestasis and the clinical outcome after PEBD. Results: From a total of 18 children with PFIC, 13 underwent PEBD, and 12 of these (among them 10 with identified ABCB11 mutations) were amenable for clinical and histological follow-up. When compared with baseline at PEBD, statistically significant reductions were found in histological cholestasis 1 and 3 years after PEBD, and in fibrosis 5 and >10 years after PEBD. The relative duration of cholestatic episodes after PEBD was positively correlated with the severity of fibrosis. Children homozygous for the missense mutation c.890A>G in ABCB11 responded well to PEBD. Conclusions: 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.
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