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Background: Primary stroke prevention programmes for children with sickle cell disease (SCD) have been shown to be feasible interventions in resource-poor countries. Different hydroxyurea (HU) regimens have been utilised in ameliorating the severity of SCD., Objective: To determine the long-term outcomes of the stroke prevention programme for children with SCD in Ibadan (SPPIBA), Nigeria., Methods: A longitudinal study of 396 children with haemoglobin SS disease who had been on the stroke prevention programme for a minimum period of 5 years. All enrollees had nonimaging TCD performed at baseline and thereafter 3-monthly or annually. Children with TCD velocities ≥170 cm/s were treated with HU by dose-escalation regimen., Results: The mean age at first TCD examination was 102 ± 46.7 months and the period of follow-up ranged from 5 to 10 years (mean = 7.2 ± 1.7). Time to significant decline in TCD velocities ranged from 5 to 35 months, (median = 10.0 months). The minimum dose of HU required to achieve significant decline in TCD velocities ranged from 15 to 31 mg/kg/day, mean 23.7 (±3.9). HU dose escalation beyond 20 mg/kg/day was required to attain significant reductions in the time-averaged mean of maximal velocities (TAMMV) in 69.1% of the cases. Two stroke events occurred giving a stroke incidence of 0.08 per 100 patient-years., Conclusion: The majority of Nigerian children with SCD and elevated TCD velocities achieved significant decline in TAMMV within the first year of HU therapy but on higher doses of HU. It might be important to individualise HU doses for optimal outcomes in primary stroke prevention., (© 2021 Wiley Periodicals LLC.)

Background: Elevated transcranial Doppler (TCD) velocities accurately predict stroke risk in children with sickle cell disease (SCD). Chronic blood transfusion, the gold standard for primary stroke prevention, is faced with numerous challenges in Africa. Hydroxyurea (HU) has been shown to reduce elevated TCD velocities in children with SCD., Aim: To determine the effectiveness of HU in reducing the risk of primary stroke in a cohort of Nigerian children with SCD and elevated velocities treated with HU., Methods: Children with SCD and TCD velocities ≥170 cm/sec treated with HU were prospectively followed with 3-monthly TCD and neurological evaluations for ≥12 months to determine the incidence of primary stroke., Results: One hundred and four children, 53 males, and 51 females were enrolled into the study. Their ages ranged from 2 to 16 years with a mean of 6 years. At first TCD examination, velocities ranged from 173 to 260 cm/sec with conditional and abnormal risk velocities in 60 (57.7%) and 44 (42.3%) children, respectively. Follow up ranged from 1 to 8 years with a mean of 3.6 years. Mean TCD velocities showed a significant decline from 198.2 (standard deviation [SD] = 15.6) cm/sec to 169.3 (SD = 21.4) cm/sec (P < 0.001). One stroke event occurred in the cohort, giving a stroke incidence of 0.27/100 person years., Conclusion: HU significantly reduces TCD velocities in Nigerian children with SCD and elevated TCD velocities, with a corresponding reduction in the incidence of primary stroke. HU may represent a potential alternative for primary stroke prevention in low and middle income countries where the burden of SCD resides., (© 2018 Wiley Periodicals, Inc.)

Background: The World Health Organization (WHO) considers early and rapid diagnosis as one of the strategies to control malaria. This study compared the performance of Quantitative Buffy Coat (QBC) test and the Plasmodium lactate dehydrogenase (pLDH) rapid diagnostic test (RDT) with microscopy as the gold standard., Materials and Methods: The study involved children ages 0-5 years who presented with a history of fever at the University College Hospital, Ibadan, Nigeria. Blood was collected from each patient and used for RDT, QBC and Giemsa-stained blood films for malaria parasites (MP). Results of QBC and RDT were compared with microscopy results for the diagnosis of malaria., Results: A total of 370 cases (194 boys and 176 girls) were studied giving a male: female ratio of 1.1:1. Of the 370 cases tested using Giemsa-stained thick blood films for MP, 78 (21 %) were positive. For the QBC test, 78 (21%) of the cases were positive with sensitivity, specificity, positive and negative predictive values of 70.5 %, 92.1%, 70.5 % and 92.1 % respectively. Seventy-six (20%) of the cases were positive by RDT with sensitivity, specificity, positive and negative predictive values of 84.2 %, 95.2 %, 82.1 %, and 95.9 % respectively. There was no significant difference in the sensitivity of QBC compared with the RDT., Conclusion: Both the QBC and the pfLDH (RDT) performed reasonably well in this study Malaria rapid diagnostic tests are recommended in malaria endemic clinical settings to avoid unnecessary antimalarial treatment. List of Abbreviations: AO: Acridine orange, AIDS: Acquired immunodeficiency syndrome, ACT: Artemisinin-based combination therapy, CM:Cerebral malaria, BCP:Benzothiocarboxypurine, DDT:Dichloro-diphenyl-trichloroethane, DNA:DeoxyriboNucleic Acid, ELAM-1: Endothelial leukocyte adhesion molecule, G6PD: Glucose-6-Phosphate Dehydrogenase, HIV: Human immuno deficiency virus, HRP 2: Histidine Rich Protein 2, ICAM -1: Inter cellular adhesion molecule1, ICER: Incremental cost effectiveness ratio, IL-1: Interleukin -1, IFN-g: Interferon-gamma, IgG: Immunoglobulin G, MP: Malaria parasite, NADP: Oxidised Nicotinamide Adenine Dinucleotide Phosphate, NADPH: Reduced Nicotinamide Adenine Dinucleotide Phosphate, PCV: Packed Cell Volume (haematocrit), P. falciparum: Plasmodium falciparum , PLDH: Plasmodium lactate dehydrogenase, PCR: Polymerase Chain Reaction, PPV: Positive predictive value, QBC: Quantitative Buffy Coat examination, TNF: Tumour necrosis factor, NPV: Negative predictive value, RDT: Rapid diagnostic test, SP: Sulphadoxine -Pyrimethamine, SMA: Severe malarial anaemia, UM: Uncomplicated malaria, USA:United States of America, VCAM-1: Vascular cell adhesion molecule , WBC: White Blood Cell, WHO: World Health Organization.

Background: Chronic blood transfusion (CBT) diminishes the risk of primary and secondary stroke in sickle cell disease (SCD). We appraised CBT and assessed its feasibility as an option for stroke prevention in a setting of limited resources., Methods: All new cases of SCD seen in the Paediatric Hematology/Neurology units of the University College Hospital, Ibadan, Nigeria over a 5-year period were screened and followed up to identify those who had an indication for CBT for stroke prevention. Caregivers were counseled and offered CBT when indicated. Children of caregivers who accepted chronic transfusion were carefully followed up and outcomes documented., Results: Five (10%) of the caregivers of the 50 children who had an indication for CBT for stroke prevention consented to the treatment. They all had homozygous sickle cell anemia and had suffered a stroke. None of the children with abnormal TCD velocities consented to CBT. Two children experienced transfusion reactions, fatal in one. The mean annual cost of chronic transfusion (without chelation) was $3,276 (SD = 1,168). Major reasons given for declining CBT were high costs of blood transfusion, unavailability of blood, the need to regularly seek for blood donors, and the indefinite duration of blood transfusions., Conclusion: High economic costs, unavailability of blood, need to regularly seek for blood donors, cultural beliefs, and high frequency of transfusion reactions are major challenges to a successful CBT program in Nigeria. There is a need for government subsidy on blood transfusions and improved efforts towards provision of safe and affordable blood., (© 2013 Wiley Periodicals, Inc.)

A prospective study of 104 consecutive cases of patients with sickle-cell anaemia (SCA) presenting with severe anaemia (packed cell volume < or = 15%) was carried out in the Children's Emergency Ward of the University College Hospital, Ibadan, in 1991. The patients were classified according to the type of anaemic crisis, by physical findings, serum bilirubin and reticulocyte counts. Other investigations included a blood film for malaria parasites, blood culture, radiological investigation and lumbar puncture when necessary. The most common problems associated with SCA patients in anaemic crisis were malaria and bacterial infections--68 (66%) and 18 (17.3%) of cases, respectively. Acute chest syndrome was significantly more frequent in patients with hyperhaemolytic and acute splenic sequestration crisis compared with aplastic crisis (P < 0.05). Conjugated hyperbilirubinaemia was also significantly more frequent among patients with hyperhaemolytic crisis compared with all other anaemic crises (chi2 = 13.18, P = 0.001). The overall case fatality was 86.5/1,000 SCAs, with no fatalities in those with aplastic crisis. There were complications in six of the nine mortalities. Co-existing bacterial infections and conjugated hyperbilirubinaemia were associated with increased morbidity and mortality in patients with anaemic crisis. Patients with SCA crisis should have early evaluation and prompt treatment for associated infections.