Background: Bile atresia is children's leading cause of cirrhosis. Its high morbidity and mortality are explained by its complications, including gastrointestinal bleeding and portal hypertension. Umbilical hernia has been embryologically and clinically associated with portal hypertension and cirrhosis; however, the clinical evolution of patients with bile atresia and umbilical hernia is unknown, so the aim of the study is to describe this link.
Objective: To determine the prevalence of the coexistence of AVB and HU in patients in a tertiary hospital, and describe the clinical evolution of these patients with respect to the presence/absence of HU as a strategy in the search for prognostic factors of morbidity and mortality.
Material And Methods: This is an observational, descriptive, retrospective, and longitudinal study that included patients with bile atresia in a tertiary pediatric hospital. The variables related to bile atresia were analyzed, and the clinical evolution was described according to the presence or absence of UH.
Results: 56 patients with bile atresia were included, of which 69.6% were females. Portal hypertension occurred in 80.4%, with the presence of collateral venous network, gastrointestinal bleeding, ascites, and hepatomegaly being the indirect clinical data most often presented. The frequency of coexistence of umbilical hernia and bile atresia was 75%. Portal hypertension, collateral venous network, gastrointestinal bleeding, ascites, and small esophageal varices were the significantly more prevalent variables in patients with umbilical hernia.
Conclusions: Patients with bile atresia have a greater possibility of developing umbilical hernia and that it is associated with a more advanced evolution of portal hypertension and its associated signs and complications.
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http://dx.doi.org/10.5281/zenodo.10711842 | DOI Listing |
Preduodenal portal vein (PDPV) is a rare congenital vascular malformation, which was first described by Knight in 1921 as an anomalous vein that lies in front of the duodenum, common bile duct, and hepatic artery instead of beneath them. This abnormal position may result in congenital duodenal obstruction and puts it in danger during operations around this region. PDPV is typically associated with other congenital anomalies, mainly intraabdominal and cardiac ones.
View Article and Find Full Text PDFAbdom Radiol (NY)
December 2024
Mallinckrodt Institute of Radiology, Washington University School of Medicine, Saint Louis, MO, USA.
Biliary and peribiliary cystic lesions represent a diverse group of abnormalities, often discovered incidentally during imaging for unrelated conditions. These lesions, typically asymptomatic, necessitate precise imaging modalities to characterize their nature and determine subsequent clinical actions, such as follow-up imaging, biopsy, or surgical referral. The anatomic location of these cystic lesions, whether biliary or peribiliary, influences both diagnostic and prognostic outcomes.
View Article and Find Full Text PDFTransl Psychiatry
December 2024
Department of Psychiatry, The First Affiliated Hospital of Xi'an Jiaotong University, Xi'an, China.
Evidence suggests that complex interactions among the gut microbiome, metabolic abnormalities, and brain have important etiological and therapeutic implications in major depressive disorder (MDD). However, the influence of microbiome-gut-brain cross-talk on cognitive impairment in MDD remains poorly characterized. We performed serum metabolomic profiling on 104 patients with MDD and 77 healthy controls (HCs), and also performed fecal metagenomic sequencing on a subset of these individuals, including 79 MDD patients and 60 HCs.
View Article and Find Full Text PDFEur J Pediatr Surg
December 2024
Pediatric Surgery, UKE, Hamburg, Germany.
Background Kasai procedure (KPE) is a palliative intervention in infants with biliary atresia (BA) aiming to restore biliary drainage. While the measure of success in BA is the post-Kasai native liver survival, BA remains the most frequent indication for liver transplantation in children. While a considerable amount of children fail to clear their jaundice following KPE, resulting in early liver failure and transplantation, some children become jaundice-free after "successful" KPE.
View Article and Find Full Text PDFJHEP Rep
January 2025
Division of Gastroenterology and Hepatology, Department of Medicine, University of Pennsylvania, Philadelphia, PA, USA.
Background & Aims: EGF-containing fibulin extracellular matrix protein 1 (EFEMP1, also called fibulin-3) is an extracellular matrix protein linked in a genome-wide association study to biliary atresia, a fibrotic disease of the neonatal extrahepatic bile duct. Fibulin-3 is deposited in most tissues and null mice have decreased elastic fibers in visceral fascia; however, fibulin-3 does not have a role in the development of large elastic fibers and its overall function in the extrahepatic bile ducts remains unclear.
Methods: We used staining and histology to define the amount and organization of key extracellular matrix components in the extrahepatic bile ducts.
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