ArticleEJHaem2026
Development of a Clinical Severity Score for Indian Sickle Cell Anaemia Patients.
Article in EJHaem, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.
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7 authors.
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Abstract
Background: Sickle cell anaemia (SCA) is a monogenic disorder but shows variable disease severity. We evaluated the utility of paediatric severity scores (PSS) in Indian patients and developed two modified scores: Indian Severity Score 1 (ISS1) by adding four clinical parameters to PSS, and ISS2 by excluding four biochemical measures from ISS1. Method: Clinical details of 171 SCA patients were collected from hospital records and categorized into mild, moderate, and severe groups by two clinical experts. Same patients were randomly assigned to three training sets-R1, R2 and R3 ( Results: The concordance of PSS was 66.7%, while ISS1 and ISS2 were 82.8% and 85.1%, respectively. In all three severity scoring models, the highest concordance was observed in mild (81%-100%) followed by severe (57%-85%), and the lowest in the moderate groups (10%-48%). Conclusion: Overall, we have developed a robust, population-specific severity score for Indian SCA patients, highlighting the importance of including population-specific clinical features.
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