Unexpected Infant Death Due to Undiagnosed Biliary Atresia: A Case of Fatal Neglect

Biliary atresia (BA) is a fatal condition resulting in the lack of effective biliary drainage leading invariably to liver failure and cirrhosis within a year, and it is often lethal within a few months in the absence of corrective surgery or liver transplantation. In fact, BA is the most common indication for pediatric liver transplantation. Herein, we present a rare case of unexpected infant death due to BA diagnosed only postmortem in a context of child neglect and carelessness on the part of the parents. It emerged from the clinical history that after a few months, the parents no longer took their daughter to any medical checkups despite the indications and express recommendations for follow-up. The autopsy revealed agenesis of the gallbladder with BA and complete disruption of the hepatic architecture and parenchyma from biliary cirrhosis. Histological examinations documented severe biliary cirrhosis from hypoplasia of the biliary ducts. The child neglect in this case proved fatal inasmuch as an early diagnosis by a pediatrician would have likely allowed appropriate surgical treatment, thus avoiding the untimely death of the child. We highlight the importance of educating and informing parents (especially the disadvantaged) in matters of health. At the same time, primary care physicians should closely monitor the conditions and development of infants so as to recognize the early warning signs and symptoms of BA, bearing in mind that a timely diagnosis and proper surgica...
Source: The American Journal of Forensic Medicine and Pathology - Category: Forensic Medicine Tags: Original Articles Source Type: research

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Abstract The disease phenotype in biliary atresia (BA) is caused by a fibro-inflammatory process leading to destruction of cholangiocytes, obstruction of ductular pathways and eventual progression to liver cirrhosis. The first line of management is a Kasai portoenterostomy (KPE) followed by liver transplantation (LT) in some children. Several factors have been postulated to affect the outcome of KPE and/or the subsequent progression of liver disease. However, no biomarkers have been identified in the liver for BA. We aimed to address this deficit. Whole transcriptome mRNA sequencing was performed for 29 samples (2...
Source: Molecular Biology Reports - Category: Molecular Biology Authors: Tags: Mol Biol Rep Source Type: research
ConclusionThe prevalence of HEV infection in our cohort is low and similar to other pediatric reports. We saw no cases of chronic hepatitis or fibrosis attributable to HEV. The lower immunosuppressive regimen used in PLT children compared to other solid organ transplant recipients may account for this good outcome.
Source: Clinics and Research in Hepatology and Gastroenterology - Category: Gastroenterology Source Type: research
ConclusionAlthough LT is feasible for patients with HPS, early transplantation and avoiding hypo ‐oxygenemia immediately after transplantation are important.
Source: Journal of Hepato-Biliary-Pancreatic Sciences - Category: Gastroenterology Authors: Tags: Original Article Source Type: research
ABSTRACT BACKGROUND: Biliary atresia represents the most common surgically treatable cause of cholestasis in newborns. If not corrected, secondary biliary cirrhosis invariably results. OBJECTIVE: To evaluate, through multivariate analysis, the prognostic factors associated with the presence of biliary flow and survival with the native liver following Kasai portoenterostomy. METHODS: The study analyzed data from 117 biliary atresia patients who underwent portoenterostomy and had suitable histological material for evaluation. A logistic regression model was used to assess the presence of biliary flow. Survival was investigat...
Source: Arquivos de Gastroenterologia - Category: Gastroenterology Source Type: research
ConclusionAlthough LT is feasible for patients with HPS, early transplantation and avoiding hypo ‐oxygenemia immediately after transplantation are important.This article is protected by copyright. All rights reserved.
Source: Journal of Hepato-Biliary-Pancreatic Surgery - Category: Surgery Authors: Tags: Original Article Source Type: research
CONCLUSION: A younger age at diagnosis is strongly associated with surgical success in BA. Surgical success, the prothrombin time (INR) at presentation, and liver pathology are independent prognostic factors affecting the long-term outcomes in patients with BA. Therefore, timely diagnosis and early referral to experienced surgical centers are crucial for optimal management and favorable long-term results in BA. PMID: 31061002 [PubMed - in process]
Source: The Turkish Journal of Gastroenterology - Category: Gastroenterology Authors: Tags: Turk J Gastroenterol Source Type: research
Authors: Okochi M, Okochi H, Sakaba T, Ueda K Abstract In living donor liver transplantation (LDLT) patients, the reconstruction of insufficiently long hepatic artery (HA) is difficult. However, no report has described the relationship between the length of HA and its reconstructive procedure. Herein, we aimed to identify the risk factors for the requirement of additional reconstructive procedures of direct anastomosis. Sixty-eight HA reconstructions in LDLT were conducted (37, men; 31, women). The causes of LDLT were hepatitis (n = 36), biliary atresia (n = 13), and primary biliary cirr...
Source: Journal of Plastic Surgery and Hand Surgery - Category: Surgery Tags: J Plast Surg Hand Surg Source Type: research
Discussion The liver is one of the largest organs in the body, weighing just over 3 pounds in an adult. It is found in the upper right abdomen, under the right dome of the diaphragm. Grossly, it has asymmetric lobes with the right being larger than the left. The lobes are separated by a fibrous connective tissue band that also anchors the liver in the abdominal cavity. The gallbladder is located on the inferior surface of the liver and stores bile, which is then released into the duodenum. Microscopically, the liver cells are arranged in lobules with canals carrying blood vessels and bile ducts. At any moment about 10-13% ...
Source: PediatricEducation.org - Category: Pediatrics Authors: Tags: Uncategorized Source Type: news
Abstract OBJECTIVES: Liver transplant has been used as a curative approach for children with end-stage liver diseases. Here, we describe the underlying causes for pediatric liver transplant performed at the Shiraz Organ Transplantation Center, Nemazee Hospital, Shiraz, Iran. MATERIALS AND METHODS: In this cross-sectional des-criptive study, children
Source: Experimental and Clinical Transplantation : official journal of the Middle East Society for Organ Transplantation - Category: Transplant Surgery Authors: Tags: Exp Clin Transplant Source Type: research
CONCLUSIONS: Understanding the mechanisms of the liver fibrosis in BA may pave the way to improved survival after the Kasai procedure. PMID: 30465125 [PubMed - as supplied by publisher]
Source: World Journal of Pediatrics : WJP - Category: Pediatrics Authors: Tags: World J Pediatr Source Type: research
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