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Publication Title : A Continuing Quest for an Acceptable Alternative Sickle Cell Anaemia (SCA) Severity Scoring System in a Resource-Constrained Setting
Author(s) : Aliyu D Waziri, Sani Awwalu, Ismaila N Ibrahim Ibrahim U Kusfa, Aishatu M Suleiman, Abdulaziz Hassan
Abstract : Background: Sickle cell Anaemia (SCA) severity scoring has remained challenging. Scoring systems based on clinical and certain laboratory parameters are limited in simplicity and wide applicability. This is further worsened by dearth of exhaustive and accurate patient history. This study compared a simple, laboratory parameter-based severity scoring system to a pre-existing scoring system. Methodology: Derivation group (n = 45) and validation group (n = 300) were enrolled during steady state. Complications, haemoglobin (Hb) concentration and white blood cell counts (WBC) were documented. Severity scores were computed for each participant based on number of complications, Hb and WBC counts using a preexisting scoring system. Proposed scoring system (PropScore) was also computed based on Hb (¡Ü6, >6 to ¡Ü10 and >10 (g/dl) scored as 2, 1 and 0 respectively, and WBC (¡Ü10, >10 to ¡Ü20 and >20 (x 109 /L) scored as 0, 1 and 2 respectively. The aggregate scores of ¡Ü1, 2-3 and 4 were graded as mild, moderate and severe respectively. Severity scores were treated as ordinal variables and analysed using Spearman rank correlation, Kappa analyses and Kruskal Wallis H tests. Results: The median and interquartile range (IQR) age of the derivation group was 22 (7) years while mean and standard deviation (±SD) of the validation was 24.6±7.1 years. Among the derivation group, correlation between the pre-existing score and the PropScore was ñ = 0.844, p = <0.001 while the strength of agreement was; Kappa value = 0.745, p = <0.001. The values for the validation group were ñ = 0.601, p = <0.001 and Êappa value = 0.184, p = <0.001. Conclusion: There is poor agreement between the proposed scoring system and the pre-existing scoring system. However, we recommend that the impact of the proposed scoring system on quality of life as well as long-term morbidity be assessed.
DOI : 10.51658/ABMS.202011.5
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