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

Nancy S. Green; Deogratias Munube; Paul Bangirana; Linda Rosset Buluma; Bridget Kebirungi; Robert Opoka; Ezekiel Mupere; Philip Kasirye; Sarah Kiguli; Annet Birabwa; Michael S. Kawooya; Samson K. Lubowa; Rogers Sekibira; Edwards Kayongo; Heather Hume; Mitchell Elkind; Weixin Peng; Gen Li; Caterina Rosano; Philip LaRussa; Frank J. Minja; Amelia Boehme; Richard Idro

doi:10.1186/s12887-019-1758-2

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

Nancy S. Green
, Deogratias Munube
, Paul Bangirana
, Linda Rosset Buluma
, Bridget Kebirungi
, Robert Opoka
, Ezekiel Mupere
, Philip Kasirye
, Sarah Kiguli
, Annet Birabwa
, Michael S. Kawooya
, Samson K. Lubowa
, Rogers Sekibira
, Edwards Kayongo
, Heather Hume
, Mitchell Elkind
, Weixin Peng
, Gen Li
, Caterina Rosano
, Philip LaRussa

Frank J. Minja, Amelia Boehme, Richard Idro

Medical College, East Africa

Research output: Contribution to journal › Article

17 Citations (Scopus)

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

http://ecommons.aku.edu/eastafrica_fhs_mc_paediatr_child_health/357

Cite this

Green, N. S., Munube, D., Bangirana, P., Buluma, L. R., Kebirungi, B., Opoka, R., Mupere, E., Kasirye, P., Kiguli, S., Birabwa, A., Kawooya, M. S., Lubowa, S. K., Sekibira, R., Kayongo, E., Hume, H., Elkind, M., Peng, W., Li, G., Rosano, C., ... Idro, R. (2019). Burden of neurological and neurocognitive impairment in pediatric sickle cell anemia in Uganda (BRAIN SAFE): a cross-sectional study. Paediatrics and Child Health, East Africa. https://doi.org/10.1186/s12887-019-1758-2

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

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 = "Green, \{Nancy S.\} and Deogratias Munube and Paul Bangirana and Buluma, \{Linda Rosset\} and Bridget Kebirungi and Robert Opoka and Ezekiel Mupere and Philip Kasirye and Sarah Kiguli and Annet Birabwa and Kawooya, \{Michael S.\} and Lubowa, \{Samson K.\} and Rogers Sekibira and Edwards Kayongo and Heather Hume and Mitchell Elkind and Weixin Peng and Gen Li and Caterina Rosano and Philip LaRussa and Minja, \{Frank J.\} and Amelia Boehme and Richard Idro",

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

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

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Green, NS, Munube, D, Bangirana, P, Buluma, LR, Kebirungi, B, Opoka, R, Mupere, E, Kasirye, P, Kiguli, S, Birabwa, A, Kawooya, MS, Lubowa, SK, Sekibira, R, Kayongo, E, Hume, H, Elkind, M, Peng, W, Li, G, Rosano, C, LaRussa, P, Minja, FJ, Boehme, A & Idro, R 2019, 'Burden of neurological and neurocognitive impairment in pediatric sickle cell anemia in Uganda (BRAIN SAFE): a cross-sectional study', Paediatrics and Child Health, East Africa. https://doi.org/10.1186/s12887-019-1758-2

TY - JOUR

T1 - Burden of neurological and neurocognitive impairment in pediatric sickle cell anemia in Uganda (BRAIN SAFE): a cross-sectional study

AU - Green, Nancy S.

AU - Munube, Deogratias

AU - Bangirana, Paul

AU - Buluma, Linda Rosset

AU - Kebirungi, Bridget

AU - Opoka, Robert

AU - Mupere, Ezekiel

AU - Kasirye, Philip

AU - Kiguli, Sarah

AU - Birabwa, Annet

AU - Kawooya, Michael S.

AU - Lubowa, Samson K.

AU - Sekibira, Rogers

AU - Kayongo, Edwards

AU - Hume, Heather

AU - Elkind, Mitchell

AU - Peng, Weixin

AU - Li, Gen

AU - Rosano, Caterina

AU - LaRussa, Philip

AU - Minja, Frank J.

AU - Boehme, Amelia

AU - Idro, Richard

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 -