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Clinical and genetic characteristics of patients with Alagille syndrome in China: identification of six novel and mutations.
Transl Pediatr
December 2024
Department of Gastroenterology, Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou, China.
Background: Alagille syndrome (ALGS) is a rare disease. The variable clinical manifestations make the diagnosis of ALGS difficult. This study aimed to provide a basis for the early diagnosis of ALGS patients whose clinical identification is difficult and to enrich the spectrum of genetic variants implicated in Chinese children with ALGS.
View Article and Find Full Text PDFAnnual cholangitis more than twice predicts liver transplant in biliary atresia patients who achieve jaundice-free after Kasai portoenterostomy.
J Formos Med Assoc
January 2025
Division of Gastroenterology, Department of Pediatrics, Linkou Chang Gung Memorial Hospital, Taoyuan City 33305, Taiwan; Liver Research Center, Linkou Chang Gung Memorial Hospital, No.5, Fuxing St., Guishan Dist., Taoyuan City, 33305, Taiwan; Chang Gung University College of Medicine, No. 259, Wenhua 1st Rd., Guishan Dist., Taoyuan City, 33302, Taiwan. Electronic address:
Background: Biliary atresia (BA) is a progressive liver disease even after Kasai portoenterostomy (KPE), and the most common cause of liver transplant (LT) in the pediatric population. This study aimed to unveil the risk factors for LT in BA patients post-KPE.
Methods: We conducted a retrospective study of BA patients in a northern Taiwan Children's Medical Center from Jan 2000 to Oct 2020.
"Post-Kasai Portoenterostomy Cholangitis: What Have We Learnt So Far?".
J Clin Exp Hepatol
December 2024
Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow 226014, India.
Post-Kasai portoenterostomy (KPE) cholangitis is one of the most common complications that has a negative impact on liver function and native liver survival. Early diagnosis and judicious empiric antimicrobial management are, therefore, important to prevent further liver damage and decompensation. However, there is no consensus regarding the standard definition of post-KPE cholangitis, and established guidelines on evaluation and management are also lacking.
View Article and Find Full Text PDFBackground: Vaginal atresia (VA) is a rare female genital tract malformation characterized by the absence or incomplete development of the vagina, often leading to cyclic abdominal pain and menstrual blood retention in adolescent patients. Vaginal atresia is often accompanied by multiple organ malformations. The condition poses significant challenges in diagnosis and management, requiring a multidisciplinary approach.
View Article and Find Full Text PDFDNAJB1-PKAc Kinase Is Expressed in Young Patients with Pediatric Liver Cancers and Enhances Carcinogenic Pathways.
Cancers (Basel)
December 2024
Division of General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH 45229, USA.
Hepatoblastoma (HBL) and fibrolamellar hepatocellular carcinoma (FLC) are the most common liver malignancies in children and young adults. FLC oncogenesis is associated with the generation of the fusion kinase, DNAJB1-PKAc (J-PKAc). J-PKAc has been found in 90% of FLC patients' tumors but not in other liver cancers.
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