Biliary atresia and congenital disorders of the extrahepatic bile ducts

İşlek, Ali; Tümgör, Gökhan

doi:10.4292/wjgpt.v13.i4.33

Cited by 2 publications

(2 citation statements)

References 76 publications

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“…This means that half of the children in this study had their first surgery after 8 weeks. The general recommendation is that surgery is performed before age 8 weeks for better long-term outcomes without liver transplantation [16,17]. The median length of stay of 8.0 days per year up to 10 years found in our study indicates continuous high morbidity for these children after the initial surgery.…”

Section: Discussionmentioning

confidence: 55%

Hospital Length of Stay and Surgery among European Children with Rare Structural Congenital Anomalies—A Population-Based Data Linkage Study

Garne

Tan

Damkjær

et al. 2023

IJERPH

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Little is known about morbidity for children with rare structural congenital anomalies. This European, population-based data-linkage cohort study analysed data on hospitalisations and surgical procedures for 5948 children born 1995–2014 with 18 rare structural congenital anomalies from nine EUROCAT registries in five countries. In the first year of life, the median length of stay (LOS) ranged from 3.5 days (anotia) to 53.8 days (atresia of bile ducts). Generally, children with gastrointestinal anomalies, bladder anomalies and Prune-Belly had the longest LOS. At ages 1–4, the median LOS per year was ≤3 days for most anomalies. The proportion of children having surgery before age 5 years ranged from 40% to 100%. The median number of surgical procedures for those under 5 years was two or more for 14 of the 18 anomalies and the highest for children with Prune-Belly at 7.4 (95% CI 2.5–12.3). The median age at first surgery for children with atresia of bile ducts was 8.4 weeks (95% CI 7.6–9.2) which is older than international recommendations. Results from the subset of registries with data up to 10 years of age showed that the need for hospitalisations and surgery continued. The burden of disease in early childhood is high for children with rare structural congenital anomalies.

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Section: Discussionmentioning

confidence: 55%

Hospital Length of Stay and Surgery among European Children with Rare Structural Congenital Anomalies—A Population-Based Data Linkage Study

Garne

Tan

Damkjær

et al. 2023

IJERPH

View full text Add to dashboard Cite

show abstract

“…While the terms "conjugated" and "direct" bilirubin are frequently used interchangeably, direct hyperbilirubinemia includes both conjugated and delta bilirubin, and an increase in either causes jaundice. 6 BA is a progressive fibrotic obstructive condition that affects the biliary system both intrahepatic and extrahepatic. This leads bile flow obstruction and the development of newborn jaundice.…”

Section: Discussionmentioning

confidence: 99%

Extrahepatic cholestasis arising from congenital biliary cistern adhesion: a case report

Hariyono,

Ibrohim,

Philo

2024

Int Surg J

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Biliary atresia (BA) is a progressive fibrotic obstructive condition that affects the biliary system, leads bile flow obstruction and the development of newborn jaundice. It is lethal if not treated, with a survival rate of less than 10% by the age of three. Bowel adhesions are anomalous bands of scar tissue that develop between bowel loops. The incidence rate for these bands remains uncertain. This study presents a 2-month-old infant with a progressive yellow discoloration dan diagnosed as BA. Intra operative colangiography was performed and congenital adhesions in the form of bands were discovered.

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Biliary atresia and congenital disorders of the extrahepatic bile ducts

Cited by 2 publications

References 76 publications

Hospital Length of Stay and Surgery among European Children with Rare Structural Congenital Anomalies—A Population-Based Data Linkage Study

Hospital Length of Stay and Surgery among European Children with Rare Structural Congenital Anomalies—A Population-Based Data Linkage Study

Extrahepatic cholestasis arising from congenital biliary cistern adhesion: a case report

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