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http://dx.doi.org/10.1055/s-0043-119345 | DOI Listing |
Z Geburtshilfe Neonatol
January 2025
Department of Obstetrics and Gynecology, Sichuan University West China Second University Hospital, Chengdu, China.
Intrahepatic cholestasis of pregnancy (ICP) is a pregnancy-specific liver disease characterized by pruritus and elevated total bile acid (TBA) levels. The most serious impact of ICP is sudden unexplained intrauterine fetal death, especially when an associated TBA ≥ 100 µmol/L is confirmed.We report a case of a 27-year-old female patient with early-onset severe refractory ICP.
View Article and Find Full Text PDFCurr Opin Gastroenterol
January 2025
Reference Center for Inflammatory Biliary Diseases and Autoimmune Hepatitis, European Reference Network on Hepatological Diseases (ERN Rare-Liver), Saint-Antoine Hospital, Assistance Publique - Hôpitaux de Paris; Sorbonne University, INSERM, Saint-Antoine Research Center (CRSA).
Purpose Of Review: Low phospholipid-associated cholelithiasis (LPAC) syndrome is a rare genetic form of intrahepatic cholesterol lithiasis, affecting mainly young adults. This review describes the recent advances in genetic and clinical characterization, diagnosis and management of LPAC syndrome.
Recent Findings: Recent publications report data from several retrospective cohorts.
J Clin Med
December 2024
Department of Pediatric Surgery, Tokyo Women's Medical University, Tokyo 162-8666, Japan.
Pancreaticobiliary maljunction (PBMJ) has a long common channel (CC) that causes pancreaticobiliary reflux (PBR), which has been implicated in gallstones, cholangiocarcinoma, and pancreatitis. By contrast, PBR has occurred in cases with normal and longer CCs than normal but shorter than PBMJ. This pathophysiology has been primarily reported in adults and rarely in children.
View Article and Find Full Text PDFJ Clin Med
December 2024
Pediatric Gastroenterology and Hepatology, University Children's Hospital Tübingen, Hoppe-Seyler Str. 1, 72076 Tübingen, Germany.
: Patients with progressive familial intrahepatic cholestasis (PFIC) experience cholestasis-associated symptoms, including severe pruritus. Odevixibat is an ileal bile acid transporter inhibitor indicated for treatment of PFIC in the European Union and for the treatment of pruritus in PFIC in the United States. The aim of the current study was to characterize the real-world effectiveness and safety of odevixibat in patients with PFIC.
View Article and Find Full Text PDFAliment Pharmacol Ther
December 2024
Division of Gastroenterology and Hepatology, University of Kansas School of Medicine, and Kansas City VA Medical Center, Kansas City, Missouri, USA.
Background: Many patients diagnosed with gastro-oesophageal reflux disease (GERD) have persistent symptoms despite proton pump inhibitor (PPI) therapy.
Aims: The aim of this consensus is to provide evidence-based statements to guide clinicians caring for patients with refractory reflux-like symptoms (rRLS) or refractory GERD.
Methods: This consensus was developed by the International Working Group for the Classification of Oesophagitis.
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