Publications by authors named "B Hermeziu"

Background & Aims: Progressive familial intrahepatic cholestasis type 3 (PFIC3) is a rare liver disease caused by biallelic variations in . Data reporting on the impact of genotype and of response to ursodeoxycholic acid (UDCA) therapy on long-term outcomes are scarce.

Methods: We retrospectively describe a cohort of 38 patients with PFIC3 with a median age at last follow-up of 19.

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Article Synopsis
  • Chronic allograft dysfunction (CAD) is the leading cause of kidney transplant failure, characterized by the replacement of healthy kidney tissue with fibrosis.
  • The study investigated the role of cannabinoid receptor 1 (CB1) in CAD by analyzing its expression in kidney biopsies from 26 transplant patients, finding that CB1 was low in healthy grafts but increased significantly during CAD, particularly in tubular cells.
  • The research also demonstrated that blocking CB1 with rimonabant reduced collagen production in kidney cells, suggesting that targeting the cannabinoid system could be a potential therapeutic strategy for managing fibrosis in CAD.
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Objectives: This study analyses the prognosis of biliary atresia (BA) in France since 1986, when both Kasai operation (KOp) and liver transplantation (LT) became widely available.

Methods: The charts of all patients diagnosed with BA born between 1986 and 2015 and living in France were reviewed.

Results: A total of 1428 patients were included; 1340 (94%) underwent KOp.

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