Histological evolution of fibrosis in patients with biliary atresia

Conclusion: Most infants with BA have liver fibrosis at diagnosis and it progresses rapidly. The presence of cirrhosis is correlated with the age at surgery, which suggests the importance of this clinical variable in the evolution of fibrosis.RESUMEN Objetivo: Analizar la evoluci ón de los hallazgos histológicos de pacientes con atresia de vías biliares (AVB), resaltando la progresión de la fibrosis y comparando la biopsia hepática diagnóstica (BHD) con la biopsia hepática quirúrgica (BHQ) mediante la hepatoportoenterostomía de Kasai. Método: Estudio retrospectivo con 51 pacientes con AB sometidos a hepatoportoenterostomía, BHD y BHQ. Resultados: La edad mediana para BHD y BHQ fue 69 días y 77 días, respectivamente. El tiempo mediano entre las biopsias fue ocho días. La cirrosis fue más frecuente en la BHQ de lo que en la BHD, tanto de acuerdo con el e score de Metavir (p = 0,006) como con el escore de Ishak (p = 0,016). El escore de Metavir aumentó un punto o más en 29/51 (59,9%) pacientes, con evidencias de progresión hacia cirrosis hepática en 11/29 (37,9%), en los pacientes con progresión de la fibrosis. La edad mediana para cirugía de l os que tuvieron progresión de la fibrosis fue 77 días; en los 11 que evolucionaron hacia cirrosis, esa mediana fue 92 días. La variable clínica de edad en el momento...
Source: Jornal Brasileiro de Patologia e Medicina Laboratorial - Category: Pathology Source Type: research

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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 medic...
Source: The American Journal of Forensic Medicine and Pathology - Category: Forensic Medicine Tags: Original Articles Source Type: research
ConclusionWe present the first study of aberrantAPTR expressions in the liver of BA infants which might contribute to liver fibrogenesis in BA infants.
Source: Pediatric Surgery International - Category: Surgery Source Type: research
We examined the chronological changes in M2BPGi levels in BA patients with cirrhosis.MethodsPatients with cirrhosis were selected from among pediatric BA patients who had their native livers. Serum M2BPGi levels and Child –Pugh classification were evaluated. A total of 11 pediatric BA patients with cirrhosis were recruited.ResultsInitial M2BPGi level after diagnosis of liver cirrhosis based on liver biopsy was on average 3.4, and the most recent M2BPGi level under observation was on average 4.3. The follow-up period from the initial M2BPGi measurement averaged 22.6  months. The ratio of the initial and most rece...
Source: Pediatric Surgery International - Category: Surgery Source Type: research
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
CONCLUSION: APRI is not an effective tool to measure fibrosis or cirrhosis in patients with non-BA neonatal cholestasis in Indian children. PMID: 31208747 [PubMed - in process]
Source: Annals of Hepatology - Category: Gastroenterology Tags: Ann Hepatol 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
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