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.

Seizure-related injuries in children and adolescents with epilepsy.

BACKGROUND: Children with epilepsy are reported to be at a greater risk of injuries compared with their peers who do not have epilepsy. OBJECTIVES: We set out to determine the frequency and pattern of seizure-related injuries in children with epilepsy seen at the University College Hospital (UCH), Ibadan, Nigeria. METHODS: Consecutive cases of epilepsy seen at the pediatric neurology clinic of the UCH, Ibadan over a period of 6months were evaluated for injuries in the preceding 12months using a structured questionnaire. These were compared with age- and sex-matched controls. RESULTS: A total of 125 children with epilepsy and 125 age- and sex-matched controls were studied. Injuries occurred more frequently in children with epilepsy than in their peers (p=0.01, OR 1.935, 95% CI 1.142-3.280). Epilepsy was generalized in 80 (64.0%), and localization-related in 45 (36.0%). Idiopathic epilepsy accounted for 74 (59.2%), and the remaining 51 (40.8%) had remote symptomatic epilepsy. Fifty-seven (45.6%) children had suffered seizure-related injuries with multiple injuries in 31 (24.8%). The most frequent were skin/soft tissue lacerations (26.4%), injuries to the tongue and soft tissues of the mouth (19.2%), minor head injuries (15.2%), and dental injuries with tooth loss (8.0%). There was a statistically significant association between seizure frequency and seizure-related injuries (p=0.002). Children on polytherapy had a significantly higher frequency of seizure-related injuries (p<0.001). CONCLUSION: Epilepsy is a major risk factor for injuries in childhood. High seizure frequency increases the risk of multiple injuries in children with epilepsy.

Epidemiology of Stroke in Sickle Cell Disease.

Sickle cell disease is the most common cause of stroke in childhood, both ischaemic and haemorrhagic, and it also affects adults with the condition. Without any screening or preventative treatment, the incidence appears to fall within the range 0.5 to 0.9 per 100 patient years of observation. Newborn screening with Penicillin prophylaxis and vaccination leading to reduced bacterial infection may have reduced the incidence, alongside increasing hydroxyurea prescription. Transcranial Doppler screening and prophylactic chronic transfusion for at least an initial year has reduced the incidence of stroke by up to 10-fold in children with time averaged mean of the maximum velocity >200 cm/s. Hydroxyurea also appears to reduce the incidence of first stroke to a similar extent in the same group but the optimal dose remains controversial. The prevention of haemorrhagic stroke at all ages and ischaemic stroke in adults has not yet received the same degree of attention. Although there are fewer studies, silent cerebral infarction on magnetic resonance imaging (MRI), and other neurological conditions, including headache, epilepsy and cognitive dysfunction, are also more prevalent in sickle cell disease compared with age matched controls. Clinical, neuropsychological and quantitative MRI screening may prove useful for understanding epidemiology and aetiology.

Pediatric Hearing Thresholds Post-bacterial Meningitis.

Introduction: Disabling hearing loss as a sequela of bacterial meningitis results from damage to the auditory system. This study was designed to ascertain the hearing thresholds in survivors of bacterial meningitis and the risk factors of hearing loss in childhood bacterial meningitis. Methodology: One hundred and two children admitted and treated for bacterial meningitis were recruited prospectively along with 102 age- and sex-matched controls who had auditory evaluation using otoacoustic emission and auditory brain stem response tests 48 h prior to hospital discharge. This was also repeated at the follow-up clinic at 1 month after hospital discharge, irrespective of the initial hearing assessment results. Result: There were 57 (55.9%) males and 45 (44.1%) females among the cases (mean age, 5.34 ± 4.40 years) and 55 (53.9%) males and 47 (46.1%) females among the controls (mean age, 5.31 ± 3.15 years). The prevalence of hearing loss was 30.4% among the cases, while it was 6.9% among the controls. The risk factors of hearing impairment in this study were the presence of anemia, leukocytosis, and hypoglycorrhachia. Conclusion: Hearing impairment with varying degrees of severity is a frequent complication of bacterial meningitis in children.