ArticleBMJ case reports2020
Odevixibat and partial external biliary diversion showed equal improvement of cholestasis in a patient with progressive familial intrahepatic cholestasis.
Article in BMJ case reports, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers.
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Who cites it
13 citing papers in PubMed, 25 citations in OpenAlex.
- Case Report: Sustained biochemical remission following early initiation of odevixibat in an infant with monoallelic ABCB11 mutation and histologically confirmed PFIC2.Frontiers in pediatrics · 2025Article
- Practical Considerations for Odevixibat Treatment in Patients with Progressive Familial Intrahepatic Cholestasis: A Single-Center Case Series.Journal of clinical medicine · 2024Article
- Odevixibat treatment in an adult patient with advanced icteric progressive cholestatic liver disease.JHEP reports : innovation in hepatology · 2024Article
- Opinion paper on the diagnosis and treatment of progressive familial intrahepatic cholestasis.JHEP reports : innovation in hepatology · 2024Article
- Surgical versus Medical Management of Progressive Familial Intrahepatic Cholestasis-Case Compilation and Review of the Literature.Children (Basel, Switzerland) · 2023Review
- Case Report: Add-on treatment with odevixibat in a new subtype of progressive familial intrahepatic cholestasis broadens the therapeutic horizon of genetic cholestasis.Frontiers in pediatrics · 2023Article
- Progressive familial intrahepatic cholestasis-outcome and time to transplant after biliary diversion according to genetic subtypes.Frontiers in surgery · 2023Article
- Progressive Familial Intrahepatic Cholestasis: A Descriptive Study in a Tertiary Care Center.International journal of hepatology · 2023Article
- Gene Therapy for Acquired and Genetic Cholestasis.Biomedicines · 2022Review
- Ileal Bile Acid Transporter Inhibition Reduces Post-Transplant Diarrhea and Growth Failure in FIC1 Disease-A Case Report.Children (Basel, Switzerland) · 2022Article
- Recent updates on progressive familial intrahepatic cholestasis types 1, 2 and 3: Outcome and therapeutic strategies.World journal of hepatology · 2022Review
- MRGPRX4 in Cholestatic Pruritus.Seminars in liver disease · 2021Review
- [Partial External Biliary Diversion in Progressive Familial Intrahepatic Cholestasis and Alagille Syndrome: A Case Series].Acta gastroenterologica LatinoamericanaArticle
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2 authors at 1 institution in 1 country.
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No grant is acknowledged in the PubMed record.
Abstract
Untreated progressive familial intrahepatic cholestasis (PFIC) type 2, or bile salt exporter protein deficiency, frequently leads to severe pruritus, impaired growth and progressive liver fibrosis with risk of organ failure. We describe a 15-month-old male patient with severe pruritus diagnosed with PFIC type 2 enrolled in an open-label phase 2 study who received 4 weeks of treatment with odevixibat, an ileal bile acid transporter inhibitor under development for cholestatic liver disease treatment. The patient experienced reductions in serum bile acids and improvement in itching and sleep scores, and odevixibat was well tolerated. After the odevixibat study, symptoms returned and the patient underwent partial external biliary diversion (PEBD). Odevixibat treatment and PEBD produced similar normalisation of serum bile acid levels and improvements in pruritus and sleep disruptions. Thus, odevixibat appeared to be as effective as invasive PEBD in treating serum bile acids and cholestatic pruritus in this patient.
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