Transcranial Doppler screening in Nigerian children with sickle cell disease: A 10-year longitudinal study on the SPPIBA cohort.

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.

Prevalence of Hearing Impairment Among High-Risk Newborns in Ibadan, Nigeria.

The burden of severe hearing impairment is increasing with two-thirds of these hearing impaired people residing in developing countries. Newborn hearing screening helps to identify early, babies who need intervention in order to prevent future disability. Neither universal nor targeted hearing screening programme is available in Nigeria. Objectives: This study was carried out to assess the prevalence of hearing impairment among high-risk newborns in UCH and the associated risk factors. Materials and Methods: Two hundred one newborns in the neonatal unit of UCH with risk factors for hearing impairment had hearing screening done using automated auditory brainstem response (AABR) at 30, 45, and 70 dB at admission and discharge, and those that failed screening at discharge were rescreened at 6 weeks post-discharge. Results: Eighty-three (41.3%) and 32 (15.9%) high-risk newborns failed at admission and discharge screening respectively, and 19 (9.5%) still failed at follow up screening. The majority of hearing loss at follow up was bilateral (94.7%) and severe (52.6%). The risk factors associated with persistent hearing loss at follow up were acute bilirubin encephalopathy (RR = 11.2, CI: 1.4-90.6), IVH (RR = 8.8, CI: 1.1-71.8), meningitis (RR = 4.8, CI: 1.01-29), recurrent apnoea (RR = 2.7, CI: 1.01-7.3), severe perinatal asphyxia NNE III (RR = 7, CI: 2.4-20.2). Conclusion: Severe and bilateral hearing impairment is a common complication among high risk newborns in UCH persisting till 6 weeks post-neonatal care. Severe perinatal asphyxia with NNE III, ABE, IVH, meningitis and administration of amikacin for more than 5 days were significant risk factors. We recommend that SCBU graduates with these risk factors should have mandatory audiologic evaluation at discharge.