Burden of neurological and neurocognitive impairment in pediatric sickle cell anemia in Uganda (BRAIN SAFE): a cross-sectional study.

Autor: Green NS; Department of Pediatrics, Columbia University Vagelos Medical Center, 630 West 168 St., Black Building 2-241, Box 168, New York, NY, USA. NSG11@CUMC.Columbia.edu., Munube D; Department of Paediatrics and Child Health, Makerere University College of Health Sciences, Kampala, Uganda., Bangirana P; Department of Psychiatry, Makerere University College of Health Sciences, Kampala, Uganda., Buluma LR; Department of Paediatrics and Child Health, Makerere University College of Health Sciences, Kampala, Uganda., Kebirungi B; Department of Paediatrics and Child Health, Makerere University College of Health Sciences, Kampala, Uganda., Opoka R; Department of Paediatrics and Child Health, Makerere University College of Health Sciences, Kampala, Uganda., Mupere E; Department of Paediatrics and Child Health, Makerere University College of Health Sciences, Kampala, Uganda., Kasirye P; Department of Paediatrics and Child Health, Makerere University College of Health Sciences, Kampala, Uganda., Kiguli S; Department of Paediatrics and Child Health, Makerere University College of Health Sciences, Kampala, Uganda., Birabwa A; Department of Psychiatry, Makerere University College of Health Sciences, Kampala, Uganda., Kawooya MS; Department Radiology, Makerere University College of Health Sciences, Kampala, Uganda., Lubowa SK; Department Radiology, Makerere University College of Health Sciences, Kampala, Uganda., Sekibira R; Department of Paediatrics and Child Health, Makerere University College of Health Sciences, Kampala, Uganda., Kayongo E; Department of Paediatrics and Child Health, Makerere University College of Health Sciences, Kampala, Uganda., Hume H; Department of Paediatrics, CHU Sainte-Justine, University of Montreal, Montreal, Canada., Elkind M; Departments of Neurology, Epidemiology and Biostatistics, Columbia University Vagelos Medical Center, New York, NY, USA., Peng W; Department of Biostatistics, Mailman School of Public Health, Columbia University Vagelos Medical Center, New York, NY, USA., Li G; Department of Biostatistics, Mailman School of Public Health, Columbia University Vagelos Medical Center, New York, NY, USA., Rosano C; Epidemiology and of Clinical and Translation Science, University of Pittsburgh, Pittsburgh, PA, USA., LaRussa P; Department of Pediatrics, Columbia University Vagelos Medical Center, New York, NY, USA., Minja FJ; Department of Radiology, Yale University, New Haven, CT, USA., Boehme A; Department of Neurology, Columbia University Vagelos Medical Center, New York, NY, USA., Idro R; Department of Paediatrics and Child Health, Makerere University College of Health Sciences, Kampala, Uganda.
Jazyk: angličtina
Zdroj: BMC pediatrics [BMC Pediatr] 2019 Oct 25; Vol. 19 (1), pp. 381. Date of Electronic Publication: 2019 Oct 25.
DOI: 10.1186/s12887-019-1758-2
Abstrakt: Background: Children with sickle cell anemia (SCA) are highly susceptible to stroke and other manifestations of pediatric cerebral vasculopathy. Detailed evaluations in sub-Saharan Africa are limited.
Methods: We aimed to establish the frequency and types of pediatric brain injury in a cross-sectional study at a large SCA clinic in Kampala, Uganda in a randomly selected sample of 265 patients with HbSS ages 1-12 years. Brain injury was defined as one or more abnormality on standardized testing: neurocognitive impairment using an age-appropriate test battery, prior stroke by examination or transcranial Doppler (TCD) velocities associated with stroke risk in children with SCA (cerebral arterial time averaged mean maximum velocity ≥ 170 cm/second).
Results: Mean age was 5.5 ± 2.9 years; 52.3% were male. Mean hemoglobin was 7.3 ± 1.01 g/dl; 76.4% had hemoglobin < 8.0 g/dl. Using established international standards, 14.7% were malnourished, and was more common in children ages 5-12. Overall, 57 (21.5%) subjects had one to three abnormal primary testing. Neurocognitive dysfunction was found in 27, while prior stroke was detected in 15 (5.7%). The most frequent abnormality was elevated TCD velocity 43 (18.1%), of which five (2.1%) were in the highest velocity range of abnormal. Only impaired neurocognitive dysfunction increased with age (OR 1.44, 95%CI 1.23-1.68), p < 0.001). In univariate models, malnutrition defined as wasting (weight-for-height ≤ -2SD), but not sex or hemoglobin, was modestly related to elevated TCD (OR 1.37, 95%CI 1.01-1.86, p = 0.04). In adjusted models, neurocognitive dysfunction was strongly related to prior stroke (OR 6.88, 95%CI 1.95-24.3, p = .003) and to abnormal TCD (OR 4.37, 95%CI 1.30, p = 0.02). In a subset of 81 subjects who were enriched for other abnormal results, magnetic resonance imaging and angiography (MRI/MRA) detected infarcts and/or arterial stenosis in 52%. Thirteen subjects (25%) with abnormal imaging had no other abnormalities detected.
Conclusions: The high frequency of neurocognitive impairment or other abnormal results describes a large burden of pediatric SCA brain disease in Uganda. Evaluation by any single modality would have underestimated the impact of SCA. Testing the impact of hydroxyurea or other available disease-modifying interventions for reducing or preventing SCA brain effects is warranted.
Databáze: MEDLINE
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