Burden and risk of neurological and cognitive impairment in pediatric sickle cell anemia in Uganda (BRAIN SAFE): final results of the cross-sectional analysis

Nancy Green; Deogratias Munube; Paul Bangirana; Linda Buluma; Bridget Kebirungi; Robert Opoka; Ezekiel Mupere; Philip Kasirye; Sarah Kiguli; Annet Birabwa

doi:10.1186/s12887-019-1758-2

Burden and risk of neurological and cognitive impairment in pediatric sickle cell anemia in Uganda (BRAIN SAFE): final results of the cross-sectional analysis

Nancy Green, Deogratias Munube, Paul Bangirana, Linda Buluma, Bridget Kebirungi, Robert Opoka, Ezekiel Mupere, Philip Kasirye, Sarah Kiguli, Annet Birabwa

Medical College, East Africa

Research output: Contribution to journal › Article

9 Citations (Scopus)

9 Downloads (Pure)

Abstract

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

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.

Original language	Undefined/Unknown
Journal	Paediatrics and Child Health, East Africa
DOIs	https://doi.org/10.1186/s12887-019-1758-2
Publication status	Published - 1 Oct 2019

Access to Document

10.1186/s12887-019-1758-2Licence: Unspecified

Burden and risk of neurological and cognitive impairment in pedia

http://ecommons.aku.edu/eastafrica_fhs_mc_paediatr_child_health/475

Cite this

Green, N., Munube, D., Bangirana, P., Buluma, L., Kebirungi, B., Opoka, R., Mupere, E., Kasirye, P., Kiguli, S., & Birabwa, A. (2019). Burden and risk of neurological and cognitive impairment in pediatric sickle cell anemia in Uganda (BRAIN SAFE): final results of the cross-sectional analysis. Paediatrics and Child Health, East Africa. https://doi.org/10.1186/s12887-019-1758-2

@article{d1fafd74a68b4cf5b750bb482185014b,

title = "Burden and risk of neurological and cognitive impairment in pediatric sickle cell anemia in Uganda (BRAIN SAFE): final results of the cross-sectional analysis",

abstract = "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 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.",

author = "Nancy Green and Deogratias Munube and Paul Bangirana and Linda Buluma and Bridget Kebirungi and Robert Opoka and Ezekiel Mupere and Philip Kasirye and Sarah Kiguli and Annet Birabwa",

year = "2019",

month = oct,

day = "1",

doi = "10.1186/s12887-019-1758-2",

language = "Undefined/Unknown",

journal = "Paediatrics and Child Health, East Africa",

}

TY - JOUR

T1 - Burden and risk of neurological and cognitive impairment in pediatric sickle cell anemia in Uganda (BRAIN SAFE): final results of the cross-sectional analysis

AU - Green, Nancy

AU - Munube, Deogratias

AU - Bangirana, Paul

AU - Buluma, Linda

AU - Kebirungi, Bridget

AU - Opoka, Robert

AU - Mupere, Ezekiel

AU - Kasirye, Philip

AU - Kiguli, Sarah

AU - Birabwa, Annet

PY - 2019/10/1

Y1 - 2019/10/1

N2 - 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 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.

AB - 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 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.

U2 - 10.1186/s12887-019-1758-2

DO - 10.1186/s12887-019-1758-2

M3 - Article

JO - Paediatrics and Child Health, East Africa

JF - Paediatrics and Child Health, East Africa

ER -