ArticleWorld journal of surgery2011
Living-donor liver transplantation for progressive familial intrahepatic cholestasis.
Article in World journal of surgery, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 10 papers.
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Who cites it
10 citing papers in PubMed, 19 citations in OpenAlex.
- Odevixibat after liver transplant in patients with progressive familial intrahepatic cholestasis type 1: A case series.Journal of pediatric gastroenterology and nutrition · 2025Article
- Progressive Familial Intrahepatic Cholestasis: A Descriptive Study in a Tertiary Care Center.International journal of hepatology · 2023Article
- Long-Term Results of Pediatric Liver Transplantation for Progressive Familial Intrahepatic Cholestasis.Journal of clinical medicine · 2022Article
- Living donor liver transplantation in an infant patient with progressive familial intrahepatic cholestasis along with hepatocellular carcinoma: a case report.Korean journal of transplantation · 2022Article
- Liver transplantation in pediatric patients with progressive familial intrahepatic cholestasis: Single center experience of seven cases.Annals of hepato-biliary-pancreatic surgery · 2022Article
- Clinical outcomes of surgical management for rare types of progressive familial intrahepatic cholestasis: a case series.Surgical case reports · 2022Article
- Long-Term Outcome after Liver Transplantation for Progressive Familial Intrahepatic Cholestasis.Medicina (Kaunas, Lithuania) · 2021Article
- Liver Transplantation for Progressive Familial Intrahepatic Cholestasis.Annals of transplantation · 2018Article
- Liver transplantation and the management of progressive familial intrahepatic cholestasis in children.World journal of transplantation · 2016Review
- Mutations in the nuclear bile acid receptor FXR cause progressive familial intrahepatic cholestasis.Nature communications · 2016Article
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Authors and funding
7 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundProgressive familial intrahepatic cholestasis (PFIC) results in liver cirrhosis during the disease course, although the etiology includes unknown mechanisms. Some PFIC patients require liver transplantation (LT).
methodsIn this study, 11 patients with PFIC type 1 (PFIC1) and 3 patients with PFIC type 2 (PFIC2) who underwent living-donor LT (LDLT) were evaluated.
resultsDigestive symptoms after LDLT were confirmed in 10 PFIC1 recipients (90.9%); 8 PFIC1 recipients showed steatosis after LDLT (72.7%), which began during the early postoperative period (71.5±55.1 days). Seven of the eight steatosis-positive PFIC1 recipients (87.5%) showed a steatosis degree of ≥80%, which was complicated with steatohepatitis and resulted in fibrosis. Cirrhotic findings persisted in six PFIC1 recipients even after LDLT (54.5%), and three PFIC1 recipients finally died. The survival rates of the PFIC1 recipients at 5, 10, and 15 years were 90.9%, 72.7%, and 54.5%, respectively. In contrast, the PFIC2 recipients showed good courses and outcomes without any steatosis after LDLT.
conclusionsThe clinical courses and outcomes after LDLT are still not sufficient in PFIC1 recipients owing to steatosis/steatohepatitis and subsequent fibrosis, in contrast to PFIC2 recipients. PFIC2 is good indication for LDLT. PFIC1 patients require LT during the disease course; therefore, we suggest that the therapeutic strategies for PFIC1 patients, including the timing of LDLT, under the donor limitation should be reconsidered. The establishment of more advanced treatments for PFIC1 patients is required to improve the long-term prognosis of these patients.
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