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Biliary atresia (BA) is a neonatal progressive fibrosing cholan- giopathy and the most frequent indication for pediatric liver trans- plantation [1]. Surgical removal of biliary remnants and Roux-en-Y hepatoportoenterostomy (HPE) aims to restore biliary drainage and suppress progression to cirrhosis. Successful HPE, defined as a serum total bilirubin level <2 mg/dL at three months after surgery, occurs in ∼50% of patients in the United States [2]. Young age seems to be the best predictor of response to HPE, with limited data on the efficacy of adjuvant therapies such as corticosteroids, antibiotics, and choleretic agents [3,4]. Potential modes of action of these therapies are to increase bile flow as well as exert an anti- inflammatory effect [5].
In 2007, a double-blind randomized trial in the United Kingdom identified a beneficial effect on corticosteroid therapy on reduction of bilirubin level at one month post HPE without sig- nificant change in the need for liver transplantation [6]. Since then there have been multiple trial most prominent being, Kings hospital trial [7] and START trial [8] which demonstrated reduction in bilirubin levels; however both failed to demonstrate any effect on native liver survival.
However one study done by Bezerra et al [9] where they employed steroid in customised manner showed significant improvement in bile drainage in their subjects versus their historical cohort. Hence we propose to perform a prospective cohort study to assess the Efficacy of new post Kasai (steroid) ILBS protocol in Biliary Atresia.
Study population : Subject undergoing Kasai Sx at Institute of Liver and Biliary Sciences would be enrolled and will include retrospective historical cohort (Jan 2015 to Dec 2017) and retrospective + prospective cohort with new protocol (Jan 2018 till June 2024).
Study design: Cohort study with historical control ( Jan 2015- Dec 2017) Sample size: Time bound. All cases presenting during the study period will be included in the study.
Monitoring and assessment: Liver function test, Hemogram and International Normalised Ratio (INR) would be done weekly for one month, twice weekly for 2nd month and monthly thereafter till 1 year.
Statistical Analysis: Appropriate statistical test for correlation analysis will be applied.
Adverse effects: As per previous studies done , no serious adverse effect has been noted in treatment group vs control group.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Biliary Atresia | Subject undergoing Kasai Surgery at Institute of Liver and Biliary Sciences would be enrolled and will include retrospective historical cohort (Jan 2015 to Dec 2017) and retrospective + prospective cohort with new protocol (Jan 2018 till June 2024). |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Kasai Surgery | Other | As per institute treatment protocol |
|
| Measure | Description | Time Frame |
|---|---|---|
| We expect steroids used in customized manner to improve biliary drainage and thereby improve native liver survival in post kasai patients. | Within 6 months |
| Measure | Description | Time Frame |
|---|---|---|
| Improved survival with native liver at 12 months of age. | 12 months | |
| Reduced progression of portal Hypertension at 6 & 12 months | 6 & 12 months | |
| Improved Growth parameters at 3, 6, 12 months |
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Inclusion Criteria:
Exclusion Criteria:
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Subject undergoing Kasai Sx at Institute of Liver and Biliary Sciences would be enrolled and will include retrospective historical cohort (Jan 2015 to Dec 2017) and retrospective + prospective cohort with new protocol (Jan 2018 till June 2024).
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Dr Anmol Anmol, MD | Contact | 01146300000 | dranmol1991@gmail.com |
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Institute of Liver & Biliary Sciences | Recruiting | New Delhi | National Capital Territory of Delhi | 110070 | India |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 42165317 | Derived | Anmol, Alam S, Khanna R, Sood V, Lal BB, Kilambi R, Rastogi A. Efficacy and safety of post-Kasai steroid-based protocol in biliary atresia. J Pediatr Gastroenterol Nutr. 2026 May 21. doi: 10.1002/jpn3.70458. Online ahead of print. |
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| ID | Term |
|---|---|
| D001656 | Biliary Atresia |
| ID | Term |
|---|---|
| D001649 | Bile Duct Diseases |
| D001660 | Biliary Tract Diseases |
| D004066 | Digestive System Diseases |
| D004065 | Digestive System Abnormalities |
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| 3, 6, 12 months |
| Reduction in Bilirubin levels at 3, 6, 12 month | 3, 6, 12 months |
| Reduced PELD at 3, 6, 12 months | 3, 6, 12 months |
| D000013 | Congenital Abnormalities |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |