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Development of a Bedside Scoring Tool to Screen for Biliary Atresia in Infants with Neonatal and Infantile
Jainy Nellickal Jose1, Srinivas Rajkiran Raju2, Kaithon Jay Cheriyan1
1Department of Pediatrics, St John's National Academy of Health Sciences, Bengaluru, Karnataka, India.
Insights
A new bedside scoring system helps differentiate extrahepatic biliary atresia (EHBA) from other neonatal cholestasis causes. This tool aids early diagnosis, improving surgical outcomes for infants with biliary atresia.
Area of Science:
- Pediatric Gastroenterology
- Neonatal Surgery
- Diagnostic Tools
Background:
- Neonatal and infantile cholestasis (NIC) presents a diagnostic challenge.
- Extrahepatic biliary atresia (EHBA) requires timely surgical intervention for optimal outcomes.
- Differentiating EHBA from other NIC causes is crucial for appropriate management.
Purpose of the Study:
- To develop a simple, bedside scoring system for distinguishing EHBA from other NIC causes.
- To provide clinicians with an easy-to-use tool for early EHBA screening.
- To improve the diagnostic accuracy of EHBA in infants.
Main Methods:
- Retrospective review of 119 infants (0-12 months) diagnosed with NIC.
- Logistic regression analysis to identify predictors of EHBA.
- Development of a scoring tool based on identified independent variables.
Main Results:
- 36.2% of NIC cases were diagnosed with EHBA.
- Pale stools, splenomegaly, elevated GGT, and elevated ALP were significant predictors of EHBA.
- The derived scoring model demonstrated 80.56% sensitivity and 73.49% specificity.
Conclusions:
- A simple bedside scoring system effectively differentiates EHBA from other NIC causes.
- This tool can enhance early screening of infants with NIC.
- Optimized screening may lead to improved surgical outcomes for biliary atresia.
Objective:
To develop an easy-to-use bedside scoring system to help physicians distinguish extrahepatic biliary atresia (EHBA) from other causes of neonatal and infantile (NIC) cholestasis.
Methods:
Medical records of all infants between 0 and 12 months of age diagnosed as NIC between January 2016 and December 2024 were reviewed. A diagnosis of EHBA was made based on histopathology findings, per-operative cholangiogram, and/ or intraoperative findings. Using logistic regression analysis, candidate variables that predicted biliary atresia were identified, and a scoring tool was developed.
Results:
A total of 119 cases of NIC were analyzed; 36 (30.2%) were diagnosed with EHBA. The mean (SD) age was 82.05 (43.7) days in the biliary atresia group and 92.7 (74.1) days in the non-biliary atresia (non-BA group). 16/36 patients with EHBA were below 60 days of age. Presence of pale stools, splenomegaly, elevated gamma-glutamyl transferase (> 5 times the upper limit of normal), and elevated alkaline phosphatase (> 2 times the upper limit of normal for age) were independent variables strongly associated with biliary atresia based on multivariate logistic regression analysis. The receiver operating characteristic curve (ROC) for the derived model had a sensitivity of 80.56%, specificity of 73.49% with an overall diagnostic accuracy of 78.99%.
Conclusion:
This study provides a simple bedside scoring system that can be used to differentiate patients with EHBA from those with other causes of NIC. The derived score may enable clinicians to screen infants with NIC more effectively to optimize surgical outcomes in biliary atresia.
