Glucocorticosteroids for infants with biliary atresia following Kasai portoenterostomy

Tyraskis, Athanasios; Parsons, Christopher; Davenport, Mark

doi:10.1002/14651858.cd008735.pub2

Cited by 6 publications

(7 citation statements)

References 31 publications

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“…Consistently, they conclude that no benefit has been shown so far in terms of mid-or long-term improved survival with native liver. In this context, a recently published Cochrane review also stated that "further randomized, placebo-controlled trials are required to be able to determine if glucocorticosteroids may be of benefit in the postoperative management of infants with biliary atresia treated with Kasai portoenterostomy" [21,22].…”

Section: Discussionmentioning

confidence: 99%

Adjuvant Therapy with Budesonide Post-Kasai Reduces the Need for Liver Transplantation in Biliary Atresia

Kuebler

Madadi-Sanjani

Pfister

et al. 2021

JCM

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Based on the hypothesis that autoimmunological factors coregulate the pathomechanism in biliary atresia (BA), adjuvant therapy with steroids has become routine, although its efficacy has never been proven. In 2010, a study on the advantages of budesonide compared to prednisolone in autoimmune hepatitis gave rise to experimental therapy using budesonide as an adjuvant BA treatment. Ninety-five BA patients prospectively received a budesonide 2 mg/dose rectal foam daily for three months (SG). A case-matched control group (CG: 81) was retrospectively recruited. The outcome measures were survival with native liver (SNL), determined at six months and two years after the Kasai procedure. The follow-up rate was 100%. At six months, SNL was statistically not different but became so after two years (SG: 54%; CG: 32%; p < 0.001). No steroid-related side effects were observed, except for eight patients with finally caught-up growth retardation. This study demonstrates for the first time a significantly longer survival with native liver in patients with BA after adjuvant therapy. However, indication, dosage, and duration of any budesonide application is not given in neonates with BA. Hence, we suggest extending the postoperative use of budesonide in a multicenter observational study with a clearly defined follow-up protocol, particularly in terms of potentially underestimated side effects.

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Section: Discussionmentioning

confidence: 99%

Adjuvant Therapy with Budesonide Post-Kasai Reduces the Need for Liver Transplantation in Biliary Atresia

Kuebler

Madadi-Sanjani

Pfister

et al. 2021

JCM

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“…Previous reports suggest various prognostic factors such as age at operation, disease classification, cholangitis, 6 adjuvant therapy, including ursodeoxycholic acid 7 and corticosteroid use, 8 and the association of congenital anomalies (especially splenic malformations). 5,9 The age at KP has been considered to be a prognostic factor following KP by many researchers.…”

Section: Discussionmentioning

confidence: 99%

Anatomical patterns of biliary atresia including hepatic radicles at the porta hepatis influence short‐ and long‐term prognoses

Sasaki

Nio

Ando

et al. 2021

J Hepato Biliary Pancreat

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Background/Purpose: The biliary atresia (BA) inflammatory process leads to various obstructive patterns of extrahepatic biliary trees. The significance of the various BA obstructive patterns is unclear. This study aimed to determine the relationship between the anatomical patterns of the biliary tract and short-and long-term prognoses in BA. Methods: Between 1989 and 2018, 3483 patients were registered in the Japanese Biliary Atresia Registry. For this study, we selected 2649 patients who underwent Kasai portoenterostomy (KP) between the ages of 31 and 90 days to eliminate the influence of age at KP as much as possible. Results: Regarding the main type, there were significant differences in the jaundice clearance rate (JCR; Type I: 67.9%, Type I-cyst: 79.4%, Type II: 74.5%, Type III: 60.9%; P < .0001) and the native liver survival rate (NLSR; P < .0001). In subgroups with hepatic radicles in Types I, II, and I-cyst, there was a significant difference in JCR (P = .0004) and NLSR (P = .0026). In subgroups with hepatic radicles in Type III, there was a significant difference in JCR (P = .0148) and NLSR (P = .0421). Conclusions: Anatomical patterns of obstruction influenced short-and long-term prognoses in BA. These patterns were suggested to be prognostic factors following KP.

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“…32 Also, the lack of unanimous response to steroids in controlling BA provides compelling evidence that the immune pathogenesis is not the sole determinant of outcome. 24 Neutrophil elastase is a serine proteinase, that hydrolyzes proteins and destroys the outer membrane of E. coli, Shigella, Salmonella, and Yersinia. 70 Moreover elastase hydrolyzes extracellular matrix and causes proteolysis of collagen-IV and elastin of the extracellular matrix, 71 and the aflatoxin B1 bound DNA, parenchymal and nonparenchymal cells.…”

Section: Neutrophil Pivotal Role In Kotb Diseasementioning

confidence: 99%

Congenital aflatoxicosis, mal-detoxification genomics & ontogeny trigger immune-mediated Kotb disease biliary atresia variant: SANRA compliant review

Kotb

Talaat

et al. 2022

Medicine

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Biliary atresia (BA) is the most common indication for pediatric liver transplantation. We describe The BA variant: Kotb disease. Liver tissue in the Kotb disease BA is massively damaged by congenital aflatoxicosis resulting in inflammation, adhesions, fibrosis, bile duct proliferation, scarring, cholestasis, focal syncytial giant cell transformation, and typical immune response involving infiltration by CD4+, CD8+, CD68+, CD14+, neutrophil infiltration, neutrophil elastase spill, heavy loads of aflatoxin B1, accelerated cirrhosis, disruption of p53 and GSTPi, and have null glutathione S transferase M1 (GSTM1). All their mothers are heterozygous for GSTM1. This inability to detoxify aflatoxicosis results in progressive inflammatory adhesions and obliterative cholangiopathy early in life. The typical disruption of both p53 and GSTPi causes loss of fidelity of hepatic regeneration. Hence, regeneration in Kotb disease BA typically promotes accelerated cirrhosis. The immune response in Kotb disease BA is for damage control and initiation of regeneration, yet, this friendly fire incurs massive structural collateral damage. The Kotb disease BA is about actual ongoing hepatic entrapment of aflatoxins with lack of ability of safe disposal due to child detoxification-genomics disarray. The Kotb disease BA is a product of the interaction of persistent congenital aflatoxicosis, genetic lack of GSTM1 detoxification, ontogenically impaired activity of other hepatic detoxification, massive neutrophil-elastase, immune-induced damage, and disturbed regeneration. Ante-natal and neonatal screening for aflatoxicosis, avoiding cord milking, and stringent control of aflatoxicosis content of human, poultry and live-stock feeds might prove effective for prevention, prompt diagnosis and management based on our recent understanding of its patho-genomics.

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Glucocorticosteroids for infants with biliary atresia following Kasai portoenterostomy

Cited by 6 publications

References 31 publications

Adjuvant Therapy with Budesonide Post-Kasai Reduces the Need for Liver Transplantation in Biliary Atresia

Adjuvant Therapy with Budesonide Post-Kasai Reduces the Need for Liver Transplantation in Biliary Atresia

Anatomical patterns of biliary atresia including hepatic radicles at the porta hepatis influence short‐ and long‐term prognoses

Congenital aflatoxicosis, mal-detoxification genomics & ontogeny trigger immune-mediated Kotb disease biliary atresia variant: SANRA compliant review

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