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ObjectiveTo obtain multicentre data on the prevalence of normal, high or conditional (intermediate) blood velocity in the cerebral arteries among children with sickle cell disease (SCD) in Nigeria.DesignA prospective observational study in five tertiary healthcare institutions. By transcranial Doppler (TCD) ultrasonography, cerebral artery peak systolic blood velocity (PSV) was determined in 193 children with SCD and time averaged mean of the maximum blood velocity (TAMMV) in a different cohort of 115 children. This design was to make the findings relevant to hospitals with TCD equipment that measure either PSV or TAMMV.SettingNigeria.Participants308 children (126 girls, 182 boys; age 2–16 years).Main outcome measuresPercentage of children with SCD who have normal, high or intermediate (often termed conditional) PSV or TAMMV.ResultsIn the cohort of 193 children, PSV was normal in 150 (77.7%), high in 7 (3.6%) and conditional in 36 (18.7%). In the cohort of 115 children, TAMMV was normal in 96 (84%), high in 7 (6%) and conditional in 12 (10%). There were no significant differences in gender or age distribution between the PSV and TAMMV cohorts. Altogether, cerebral artery blood velocity was normal in 246/308 children (80%), high in 14 (4.5%) and conditional in 48 (15.5%).ConclusionSince conditional blood velocity in cerebral arteries can progress to high values and predispose to stroke, the proportion of children with SCD who are affected (15.5%) raises the question of whether regular monitoring and proactive intervention ought to be the standard of care.

Sickle leg ulcer (SLU) occurs as a result chronic occlusion of the vasculature with consequent necrosis of the skin and subcutaneous tissue usually in proximity of the malleoli. The description of clinical associations and the simultaneous occurrence of SLU and other complications of SCD compared to the non‐SLU patients was the aim of this work. A total of 272 (60.8% males and 39.2% females) patients were captured during this time period out of which 68 (51 males and 17 females) had SLU out of whom 20 patients had bilateral leg ulcers. Prevalence of SLU was 25% and the median age of patients was 25 years, frequency of crisis 2 per annum and 44 (74.6%) had been transfused in the past. Median Hb of the group was 7.6 g/dl and 25% had values lower than 6.5. The occurrence of other complications in SLU patients was as follows; 10 had AVN, 9 priapism, 8 had osteomyelitis, 6 nephropathy, stroke 2, osteoarthritis 4 and cholelithiasis 4. There was a significant relationship between the occurrence of SLU and gender of the patient being more in 67males—Likelihood ratio 4.610 (p = 0.032) and the occurrence of pulmonary hypertension—Likelihood ratio 4.762 (p = 0.029). There was no significant association between the occurrence of SLU and other complications of SCD. Leg ulcer patients have a median age of 25 years with a prevalence of 25% but do not necessarily show other features of severe disease phenotypes. SLU patients were more likely to develop pulmonary hypertension. Further studies on impact of environmental factors on the occurrence of SLU are needed to further evaluate its aetiology.

Background: Sickle cell disease (SCD) is the commonest inherited qualitative blood disorder worldwide. Aims: To guide haematologists and other clinicians in making appropriate clinical judgement in the management of SCD patients in resource poor countries. Objective: To evaluate the demographic characteristics, clinical phenotype, laboratory parameters and complications of the enrolled patients during the period of the study. Study Design: A retrospective study. Place and Duration of the Study: Haematology and Immunology department, UNTH over 8 years period. Methodology: We enrolled 135 patients grouped into mild or severe SCD. The objective parameters were extracted from their case notes. Data analyzed using GraphPad Prism version 8. Results: Of the135 subjects, 39 (29%) had mild disease while 96 (71%) had severe disease. The mean ages in the groups were 27 and 26 years respectively (age ranged from 10 to 55years). Mean weight and frequency of crises per year between the groups were 59 and 58kg, 1.0 and 5.0 crises, respectively. Significant difference existed in the mean blood counts, F = 323.3. P < 0.0001. Renal and liver functions showed no significant derangement. Vaso-occlusive crisis (VOC) was predominant crisis. Leg ulcer (20%) was commonest complication. Significant relationship existed between frequency of crisis and number of complications, r = 0.17, P = .04, [CI = 0.003 – 0.332]. No relationship was observed between the number of blood units and frequency of crisis, r = 0.05, P = .61, [CI = -0.12 - 0.21]. Our patients were counselled at every clinic visit with proper guidance. Significant difference existed between blood pressure and proteinuria, P < .0001, [CI = 4.73 – 23.2]. Conclusion: Multidisciplinary approach improved their clinical outcome. Component blood transfusion practice is strongly advocated. We recommend further studies on the psycho-socio-economic, and environmental factors that impact on clinical phenotypes of this medical condition.