Biliary atresia: Clinical advances and perspectives

Nizery, Laure; Chardot, Christophe; Sissaoui, Samira; Capito, Carmen; Henrion‐Caude, Alexandra; Debray, Dominique; Girard, M

doi:10.1016/j.clinre.2015.11.010

Cited by 54 publications

(40 citation statements)

References 73 publications

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“…In this study, a rate of 72.2% at 5 years and 64.2% at 10 years of follow-up was recorded. Global survival rate was 88.9%, in line with other numbers documented in the literature [4,10,12,15,16], even though one of the registered deaths was due to post-transplant complications and is not directly related to the primary disease.…”

Section: Discussionsupporting

confidence: 71%

“…Multiple studies document a native liver survival rate of approximately 30% after 20 years of follow-up [4,10,15]. Two studies conducted in France and the United Kingdom registered a survival rate without liver transplant of 43 and 51%, respectively, at 4 years of follow-up [16].…”

Section: Discussionmentioning

confidence: 99%

“…All the patients were given UDCA in the postoperative period, even though its beneficial effects, related to the increase in biliary acid drainage, hence preventing fibrosis and consequent progression of hepatic injury and a possible immunomodulator effect [4,15], are still unclear [9,12]. On the other hand, corticoids were not systematically prescribed, having only been given to those patients in whom early biliary drainage was not satisfactory.…”

Section: Discussionmentioning

confidence: 99%

“…It represents the main cause of death by hepatic failure and the main indication for hepatic transplant in children [2][3][4].…”

Section: Introductionmentioning

confidence: 99%

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Biliary Atresia - Clinical Series

Neto¹,

Borges-Dias²,

Trindade

et al. 2017

GE Port J Gastroenterol

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Introduction: Biliary atresia is the main cause of death by hepatic failure and the main indication for liver transplant in children. This study aims to analyze the population with this diagnosis, treated between 2000 and 2015 at Hospital de São João. Material and Methods: Descriptive, observational, and retrospective study, including the patients with biliary atresia, diagnosed and treated between January 1, 2000 and December 31, 2015. We analyzed epidemiologic, clinical, biochemical, and image data, as well as registered complications and present status. Results: Eighteen patients were evaluated. The median age at time of Kasai portoenterostomy was 63 days of life, with better prognosis for those patients who had surgery before 72 days. The procedure was successful in 2/3 of cases. There was a significant association between recurrent cholangitis and survival. Five cases of transplant and 2 deaths, one of them after transplant, were registered. Survival with native liver was 77.8%, 72.2%, and 64.2% at 1, 5, and 10 years of follow-up, respectively. Discussion: The presentation and evolution of patients was similar to other studies. However, there was a higher surgical success and survival rates at 5 and 10 years of follow-up than most series. Age at surgery and recurrence of cholangitis were the only factors significantly related to prognosis. Conclusion: In spite of the low number of patients (1,125/year), our results were similar to those of other reference centers.

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Section: Discussionsupporting

confidence: 71%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

“…It represents the main cause of death by hepatic failure and the main indication for hepatic transplant in children [2][3][4].…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Biliary Atresia - Clinical Series

Neto¹,

Borges-Dias²,

Trindade

et al. 2017

GE Port J Gastroenterol

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“…6 The standard treatment strategy for BA is the Kasai portoenterostomy. [8][9][10][11] Recently, great efforts have been made to search for new biomarkers for BA. In addition to this, liver transplantation may be a possibility if a suitable donor is available.…”

Section: Introductionmentioning

confidence: 99%

Gut microbial profile in biliary atresia: a case‐control study

Wang

Tian

Jiang

et al. 2019

J of Gastro and Hepatol

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Background and Aim Biliary atresia (BA) is a progressive fibro‐inflammatory cholangiopathy with an unclear etiology. Various liver disorders are associated with an altered microbiome. However, gut microbiome in BA remains unknown. Here, we performed a case‐control study to investigate the gut microbiota in BA. Methods A cross‐sectional analysis was first conducted for 34 BA patients and 34 healthy controls. Then we investigated the shift in gut microbiota 2 weeks after the Kasai procedure in 16 BA patients. Gut microbiome was initially analyzed using 16S ribosome RNA gene sequencing and further validated by metagenomic sequencing. Fecal bile acids were determined using ultra‐high performance liquid chromatography. Results Compared with healthy controls, BA showed lower diversity and significant structural segregation in the microbiome. At phylum level, Proteobacteria numbers increased, whereas those of Bacteroidetes decreased in BA. At genus level, several potential pathogens such as Streptococcus and Klebsiella thrived in BA, while numbers for Bifidobacterium and several butyrate‐producing bacteria declined. The microbiome was also disturbed after the Kasai procedure. Operational taxonomic units responding to BA showed significant correlation with liver function. Furthermore, the abundance ratio of Streptococcus/Bacteroides showed great promise in distinguishing BA from healthy controls. Intestinal bile acids were dramatically decreased in BA, and Clostridium XIVa positively correlated with the ratio of primary/secondary bile acids. Conclusions Gut microbial dysbiosis, may be caused by decreased bile acids, was associated with liver function and had a good diagnostic potential for BA. Therefore, further exploration of gut microbiota may provide important insights into their potential diagnostic and therapeutic benefits.

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High Mobility Group Box 1 Release by Cholangiocytes Governs Biliary Atresia Pathogenesis and Correlates With Increases in Afflicted Infants

et al. 2021

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These results suggest that HMGB1 plays a role in virus induced BA pathogenesis and could be a target for therapeutic interventions in a subset of patients with BA and high HMGB1. (Hepatology 2021;74:864-878). B iliary atresia (BA) is a disease of infancy in which a devastating fibroinflammatory cholangiopathy occurs, leading to obstructive jaundice. BA leads to end-stage liver disease (ESLD). (1)(2)(3)(4) In the United States, the incidence of BA is 1 in 15,000 births, (5) and it is the most common indication for pediatric liver transplantation. (6,7) In an effort to restore bile flow, a Kasai hepatoportoenterostomy (HPE) is typically performed soon after diagnosis. (8) However, even if an HPE is performed and cholestasis resolves, bile duct proliferation and fibrosis may progress, (7) resulting in the development of portal hypertension and the complications of ESLD. (9) It is estimated that 60% of the patients who overcome perinatal cholestasis will still need a liver transplantation before the age

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Biliary atresia: Clinical advances and perspectives

Cited by 54 publications

References 73 publications

Biliary Atresia - Clinical Series

Biliary Atresia - Clinical Series

Gut microbial profile in biliary atresia: a case‐control study

High Mobility Group Box 1 Release by Cholangiocytes Governs Biliary Atresia Pathogenesis and Correlates With Increases in Afflicted Infants

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