Sani Awwalu

Background: Sickle cell disease (SCD) is an autosomal recessive haemoglobin disorder, affecting about 7.74 million individuals worldwide, but it is more prevalent among Africans and Asians. SCD is characterised by many complications, and it is a major health issue in Nigeria, the country with the largest burden of the disease globally. This work aims to present the design and implementation of electronic registries (ER) for SCD in a tertiary hospital in Nigeria. Methods: Registry design was initiated during a staff exchange programme within the ARISE initiative (EU grant agreement no. 824021). Ethical approval was obtained, and paper records were retrieved and transferred into one adult and one paediatric database, developed with Microsoft Access. Results: Data from 2659 SCD patients were entered in the ERs, including 698 (26.3%) adults and 1961 (73.7%) children. There were 287 (41%) male adults, 404 (58%) female and 7 (1%) patients whose gender was missing. There were 1041 (53.1%) male children, 906 (46.2%) female and 14 (0.7%) whose gender was missing. Information on phenotype was available for 2385 subjects, and most of them (2082, 87.3%) were SS. The most prevalent SCD-related complication was painful events (26.6% in adults and 68.7% in children, considering valid cases). Conclusions: About 60% of SCD patients in the centre were included in the ERs providing useful, hands-on recommendations for future ER design in SCD. These ERs might be an appropriate tool for collecting and analysing SCD patients’ data.

BACKGROUND: Vascular E- and P-selectins play important roles in sickle cell anemia (SCA), vaso-occlusive crises (VOC); however, the extent to which they determine VOC has not been studied. OBJECTIVES: The aims of the study were to assess vascular selectin levels and the extent to which they determine VOC among SCA patients. SUBJECTS AND METHODS: This was a cross-sectional comparative study conducted among patients with SCA in a tertiary hospital, Northwestern Nigeria. Eighty-eight participants were enrolled (44 each in VOC and steady state). Soluble E (sE) and P (sP) selectin levels were assessed using enzyme-linked immunosorbent assay technique. The frequency of blood transfusion and bone pains was collated. RESULTS: Patients with VOC compared to those in steady state had higher levels of sP-selectin (5.5 ± 4.6 ng/ml vs. 3.2 ± 0.8 ng/ml, P = 0.001) and mean rank sE-selectin (53.2 vs. 35.8, P = 0.001). The odds ratio (OR) for sE-selectin levels and VOC was 1.135 (P = 0.009), while that of sP-selectin was 2.693 (P = 0.002). The adjusted ORs for sE-selectin and sP-selectin were 1.184 (95% confidence interval [CI]: 1.021, 1.373) and 3.748 (95% CI: 1.475, 9.524), respectively. CONCLUSIONS: Patients in VOC have elevated sP-and E-selectin levels. sP-selectin level is a better predictor of VOC compared to sE-selectin.