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A 21-year-old female underwent a Kasai hepatoportoenterostomy with Roux-en-Y reconstruction for typeIII biliary atresia at age 63 days. At the age of 19 years, she developed cholangitis and CT scan revealed hepatolithiasis. She presented for treatment of the intrahepatic stone and the hepatportoenterostomy was directly visualized with double-balloon endoscopy (DBE). Endoscopic findings showed multiple intrahepatic bile ducts open to the jejunum through multiple orifices. Cholangiography showed narrowing of intrahepatic bile duct branches with a "fire-like" appearance. These findings have not been previously reported, since endoscopic approaches to patients with a hepaticojejunostomy were limited. DBE was useful to directly visualize the anastomosis in a patient status-post the Kasai operation for biliary atresia with a Rouxen-Y reconstruction.
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http://dx.doi.org/10.1016/j.jpedsurg.2016.06.019 | 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.