The biliary atresia splenic malformation syndrome: A 28-year single-center retrospective study

Davenport, Mark; Tizzard, Sarah; Underhill, James A.; Mieli‐Vergani, Giorgina; Portmann, B; Hadžić, Nedim

doi:10.1016/j.jpeds.2006.05.030

Cited by 224 publications

(193 citation statements)

References 57 publications

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“…Postoperative median (interquartile) bilirubin in the steroid group (n ϭ 34) versus the placebo group (n ϭ 37). *P ϭ 0.06. syndromic features, 7,8 and the expertise of the surgical team. 1,11 The importance of adjunctive medical therapy in achieving the clearance of jaundice is unclear.…”

Section: Discussionmentioning

confidence: 99%

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Randomized, double-blind, placebo-controlled trial of corticosteroids after Kasai portoenterostomy for biliary atresia

et al. 2007

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The objective of this study was to evaluate adjuvant corticosteroids after Kasai portoenterostomy for biliary atresia. The study consisted of a prospective, 2-center, double-blind, randomized, placebo-controlled trial of post-Kasai portoenterostomy corticosteroids (oral prednisolone: 2 mg/kg/day from day 7 to day 21 and 1 mg/kg/day from day 22 to day 28). The data were compared with 2 or Mann-Whitney tests, as appropriate. Seventy-one postoperative infants with type 3 biliary atresia were randomized to receive either oral prednisolone (n ‫؍‬ 36) or a placebo (n ‫؍‬ 37). At 1 month, the median bilirubin level was lower in the steroid group (66 versus 92 mol/L, P ‫؍‬ 0.06), but no difference was evident at 6 (P ‫؍‬ 0.56) or 12 (P ‫؍‬ 0.3) months. The proportion of infants with a normal bilirubin level (<20 mol/L) at 6 (47% versus 49%, P ‫؍‬ 0.89) and 12 months (50% versus 40%, P ‫؍‬ 0.35) was not significantly different. The need for transplantation by 6 (12% versus 13%, P ‫؍‬ 0.99) and 12 months (26% versus 35%, P ‫؍‬ 0.47) was not significantly different. The steroid effect was more pronounced in younger infants (less than 70 days at Kasai portoenterostomy, n ‫؍‬ 51), with a reduced bilirubin level at 1 month (64 versus 117 mol/L, P ‫؍‬ 0.01) and with a greater proportion with a normal bilirubin level at 12 months (54% versus 37%, P ‫؍‬ 0.22). Conclusion: There was a beneficial effect on the rate of reduction of bilirubin in the early postoperative period (specifically in infants less than 70 days old at surgery), but this steroid regimen did not reduce the need for liver transplantation.

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Section: Discussionmentioning

confidence: 99%

“…We excluded all infants with biliary atresia splenic malformation, 7,8 severe cardiac anomalies, close contact with or clinical evidence of an ongoing infection, definite macroscopic cirrhosis, and type 1 or 2 (correctable) biliary atresia.…”

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confidence: 99%

Randomized, double-blind, placebo-controlled trial of corticosteroids after Kasai portoenterostomy for biliary atresia

et al. 2007

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“…2) [40]. Twenty percent of patients with BA demonstrate anatomical variants including polysplenia and asplenia (also known as biliary atresia splenic malformation syndrome (BASM), gastrointestinal, venous and cardiac malformations [41]. Untreated, it progresses rapidly to biliary cirrhosis and death within two years.…”

Section: Biliary Atresiamentioning

confidence: 99%

The management of childhood liver diseases in adulthood

Joshi

Gupta

Samyn

et al. 2017

Journal of Hepatology

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SummaryAn increasing number of patients with childhood liver disease survive into adulthood. These young adults are now entering adult services and require ongoing management. Aetiologies can be divided into liver diseases that develop in young adults which present to adult hepatologists i.e., biliary atresia and Alagille syndrome or liver diseases that occur in children/adolescents and adults i.e., autoimmune hepatitis or Wilson's disease. To successfully manage these young adults, a dynamic and responsive transition service is essential. In this review, we aim to describe the successful components of a transition service highlighting the importance of self-management support and a multi-disciplinary approach. We will also review some of the liver specific aetiologies which are unique to young adults, offering an update on pathogenesis, management and outcomes. Ó

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“…Intraepithelial lymphocytes, as well as signs of epithelial injury, including nuclear enlargement, stratification, overlapping, and loss of cytoplasm, are often seen in these cases, followed by destruction of ducts. Interlobular bile duct injury or inflammation has been reported in 31% to 94% of cases 77,87,88 and bile duct loss, in approximately 8% of cases, on initial biopsy samples. 80,86 Alternatively, bile duct abnormalities may be seen in the form of a duct plate malformation (DPM)-like lesion in otherwise typical cases of biliary atresia.…”

Section: Pathologic Evaluation Liver Biopsy Findingsmentioning

confidence: 99%

Biliary Atresia: A Multidisciplinary Approach to Diagnosis and Management

Moreira¹,

Cabral²,

Cowles³

et al. 2012

Archives of Pathology &Amp; Laboratory Medicine

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Context.—Biliary atresia is an inflammatory cholangiopathy of infancy that results in progressive fibrosis and obliteration of bile ducts and represents the main indication for liver transplant in young children. In spite of extensive investigation, its etiology has remained poorly understood. Timely surgical intervention (Kasai procedure) may result in significant benefit to these patients and represents the final goal of an accurate diagnostic evaluation. Objective.—To present an overview of biliary atresia, including clinical and surgical approaches to this disease, with emphasis on the histopathologic evaluation. Data Sources.—Review of relevant literature indexed in PubMed (US National Library of Medicine). Conclusion.—A well-coordinated multidisciplinary approach is required in the assessment of suspected cases of biliary atresia. Pathologic examination of biopsy specimens is an integral part of the diagnostic algorithm and, therefore, plays a pivotal role in the diagnostic evaluation of this disease.

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The biliary atresia splenic malformation syndrome: A 28-year single-center retrospective study

Cited by 224 publications

References 57 publications

Randomized, double-blind, placebo-controlled trial of corticosteroids after Kasai portoenterostomy for biliary atresia

Randomized, double-blind, placebo-controlled trial of corticosteroids after Kasai portoenterostomy for biliary atresia

The management of childhood liver diseases in adulthood

Biliary Atresia: A Multidisciplinary Approach to Diagnosis and Management

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