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Background/aims: Biliary atresia (BA) is a progressive cholangiopathy of infancy that leads to cholestasis, bile duct fibrosis, and liver cirrhosis if untreated. This study aimed to evaluate the demographic, clinical, and laboratory characteristics of infants with BA and identify prognostic factors influencing treatment outcomes.
Methods: A retrospective observational design was used, analyzing medical records of 152 infants diagnosed with BA over a three-year period. Data included clinical manifestations, laboratory findings, diagnostic procedures, and treatment outcomes. Statistical analyses were conducted to assess factors affecting recovery and survival.
Results: The results indicated a mean patient age of 13.9 weeks, with jaundice onset at 3.3 weeks and an average duration of 10.7 weeks. The overall survival rate was 81.6%, while 48% experienced treatment failure. Recovery rates were significantly associated with younger age at diagnosis (p=0.000), shorter jaundice duration (p=0.002), and absence of portal hypertension (p<0.001).
Conclusions: These findings highlight the importance of early diagnosis and intervention in improving BA outcomes. Future research should focus on optimizing treatment strategies to enhance long-term survival.
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http://dx.doi.org/10.4166/kjg.2025.020 | DOI Listing |
Surg Case Rep
September 2025
Department of Surgery, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Nagasaki, Japan.
Introduction: Osler-Weber-Rendu syndrome, or hereditary hemorrhagic telangiectasia (HHT), is a rare autosomal dominant genetic vascular disease characterized by arteriovenous malformations, vascular wall fragility, dilatation, and rupture of the vessels with hepatic symptoms. As HHT with hepatic symptoms is recognized as the primary etiology for liver transplantation, liver transplantation with liver grafts from donors affected by HHT is extremely rare. Herein, we report a successful liver transplantation in a patient with biliary atresia who received a whole-liver graft from a young brain-dead donor with HHT.
View Article and Find Full Text PDFExp Clin Transplant
August 2025
>From the Department of Pediatric Hematology and Oncology, Baskent University Medical Faculty, Ankara, Turkey.
A 3-year-old female patient, who had received a liver transplant from her father 1 year previously to treat biliary atresia, was admitted with fever and pancytopenia. History showed Epstein-Barr virus polymerase chain reaction positivity detected in the patient 3 months earlier; the patient received reduced immunosuppression in doses of tacrolimus, and valganciclovir was administered. Physical exa-mination showed lymphadenopathies at the cervical, axillary and inguinal regions with 2 × 2 cm at diameter, splenomegaly of 5 cm, and fever of 39 °C.
View Article and Find Full Text PDFBMC Pediatr
September 2025
Pediatric Surgery Department, Faculty of Medicine, Minia University, Minia, Egypt.
Aim Of The Study: To present a case series of four pediatric patients with PDPV, each with a different clinical presentation and surgical management.
Methods: We retrospectively reviewed four cases of PDPV managed at our institution. Two cases were associated with extrahepatic biliary atresia (EHBA) and discovered incidentally during surgery.
J Pediatr Surg
September 2025
Dept. of Paediatric Surgery, Kings College Hospital, London, UK. Electronic address:
J Hepatol
September 2025
Department of Neonatal Surgery, Key Laboratory of Major Diseases in Children, Ministry of Education, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, 100045, China. Electronic address:
Background And Aims: Biliary atresia (BA) is a severe neonatal cholangiopathy characterized by progressive inflammation and fibrosis. We aimed to systematically investigate BA pathology using integrated multi-omics.
Methods: Multi-omics integration of BA and control livers revealed sphingolipid dysregulation.