Neurocognitive impairment in Ugandan children with sickle cell anemia compared to sibling controls: a cross-sectional study
IntroductionThe neurocognitive functions in Ugandan children aged 1–12 years with sickle cell anemia (SCA) were compared to their non-SCA siblings to identify risk factors for disease-associated impairment.MethodsThis cross-sectional study of the neurocognitive functions in children with SCA (N = 24...
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Frontiers Media S.A.
2024-04-01
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| Series: | Frontiers in Stroke |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fstro.2024.1372949/full |
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| author | Paul Bangirana Paul Bangirana Amelia K. Boehme Annet Birabwa Robert O. Opoka Robert O. Opoka Deogratias Munube Deogratias Munube Ezekiel Mupere Phillip Kasirye Grace Muwanguzi Maxencia Musiimenta George Ru Nancy S. Green Richard Idro Richard Idro |
| author_facet | Paul Bangirana Paul Bangirana Amelia K. Boehme Annet Birabwa Robert O. Opoka Robert O. Opoka Deogratias Munube Deogratias Munube Ezekiel Mupere Phillip Kasirye Grace Muwanguzi Maxencia Musiimenta George Ru Nancy S. Green Richard Idro Richard Idro |
| author_sort | Paul Bangirana |
| collection | DOAJ |
| description | IntroductionThe neurocognitive functions in Ugandan children aged 1–12 years with sickle cell anemia (SCA) were compared to their non-SCA siblings to identify risk factors for disease-associated impairment.MethodsThis cross-sectional study of the neurocognitive functions in children with SCA (N = 242) and non-SCA siblings (N = 127) used age- and linguistically appropriate standardized tests of cognition, executive function, and attention for children ages 1–4 and 5–12. Test scores were converted to locally derived age-normalized z-scores. The SCA group underwent a standardized stroke examination for prior stroke and transcranial Doppler ultrasound to determine stroke risk by arterial flow velocity.ResultsThe SCA group was younger than their siblings (mean ages 5.46 ± 3.0 vs. 7.11 ± 3.51 years, respectively; p < 0.001), with a lower hemoglobin concentration (7.32 ± 1.02 vs. 12.06 ± 1.42, p < 0.001). The overall cognitive SCA z-scores were lower, −0.73 ± 0.98, vs. siblings, −0.25 ± 1.12 (p < 0.001), with comparable findings for executive function of −1.09 ± 0.94 vs. −0.84 ± 1.26 (p = 0.045), respectively. The attention z-scores for ages 5–12 for the SCA group and control group were similar: −0.37 ± 1.4 vs. −0.11 ± 0.17 (p = 0.09). The overall differences in SCA status were largely driven by the older age group, as the z-scores in the younger subsample did not differ from controls. Analyses revealed the strongest predictors of poor neurocognitive outcomes among the SCA sample to be the disease, age, and prior stroke (each p < 0.001). The impacts of anemia and SCA were indistinguishable.DiscussionNeurocognitive testing in children with SCA compared to non-SCA siblings revealed poorer SCA-associated functioning in children older than age 4. The results indicate the need for trials assessing the impact of disease modification on children with SCA. |
| first_indexed | 2024-04-24T09:44:55Z |
| format | Article |
| id | doaj.art-e6f37e23726544ada0e9855d71fbc086 |
| institution | Directory Open Access Journal |
| issn | 2813-3056 |
| language | English |
| last_indexed | 2024-04-24T09:44:55Z |
| publishDate | 2024-04-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Stroke |
| spelling | doaj.art-e6f37e23726544ada0e9855d71fbc0862024-04-15T04:24:31ZengFrontiers Media S.A.Frontiers in Stroke2813-30562024-04-01310.3389/fstro.2024.13729491372949Neurocognitive impairment in Ugandan children with sickle cell anemia compared to sibling controls: a cross-sectional studyPaul Bangirana0Paul Bangirana1Amelia K. Boehme2Annet Birabwa3Robert O. Opoka4Robert O. Opoka5Deogratias Munube6Deogratias Munube7Ezekiel Mupere8Phillip Kasirye9Grace Muwanguzi10Maxencia Musiimenta11George Ru12Nancy S. Green13Richard Idro14Richard Idro15Department of Psychiatry, Makerere University College of Health Sciences, Kampala, UgandaGlobal Health Uganda, Kampala, UgandaDepartment of Neurology, Columbia University Vagelos Medical Center, New York, NY, United StatesDepartment of Mental Health and Community Psychology, Makerere University College of Humanities and Social Sciences, Kampala, UgandaGlobal Health Uganda, Kampala, UgandaDepartment of Paediatrics and Child Health, Makerere University College of Health Sciences, Kampala, UgandaGlobal Health Uganda, Kampala, UgandaDepartment of Paediatrics and Child Health, Makerere University College of Health Sciences, Kampala, UgandaDepartment of Paediatrics and Child Health, Makerere University College of Health Sciences, Kampala, UgandaDirectorate of Paediatrics and Child Health, Mulago National Referral Hospital, Kampala, UgandaGlobal Health Uganda, Kampala, UgandaGlobal Health Uganda, Kampala, UgandaDepartment of Pediatrics, Columbia University Vagelos Medical Center, New York, NY, United StatesDepartment of Pediatrics, Columbia University Vagelos Medical Center, New York, NY, United StatesGlobal Health Uganda, Kampala, UgandaDepartment of Paediatrics and Child Health, Makerere University College of Health Sciences, Kampala, UgandaIntroductionThe neurocognitive functions in Ugandan children aged 1–12 years with sickle cell anemia (SCA) were compared to their non-SCA siblings to identify risk factors for disease-associated impairment.MethodsThis cross-sectional study of the neurocognitive functions in children with SCA (N = 242) and non-SCA siblings (N = 127) used age- and linguistically appropriate standardized tests of cognition, executive function, and attention for children ages 1–4 and 5–12. Test scores were converted to locally derived age-normalized z-scores. The SCA group underwent a standardized stroke examination for prior stroke and transcranial Doppler ultrasound to determine stroke risk by arterial flow velocity.ResultsThe SCA group was younger than their siblings (mean ages 5.46 ± 3.0 vs. 7.11 ± 3.51 years, respectively; p < 0.001), with a lower hemoglobin concentration (7.32 ± 1.02 vs. 12.06 ± 1.42, p < 0.001). The overall cognitive SCA z-scores were lower, −0.73 ± 0.98, vs. siblings, −0.25 ± 1.12 (p < 0.001), with comparable findings for executive function of −1.09 ± 0.94 vs. −0.84 ± 1.26 (p = 0.045), respectively. The attention z-scores for ages 5–12 for the SCA group and control group were similar: −0.37 ± 1.4 vs. −0.11 ± 0.17 (p = 0.09). The overall differences in SCA status were largely driven by the older age group, as the z-scores in the younger subsample did not differ from controls. Analyses revealed the strongest predictors of poor neurocognitive outcomes among the SCA sample to be the disease, age, and prior stroke (each p < 0.001). The impacts of anemia and SCA were indistinguishable.DiscussionNeurocognitive testing in children with SCA compared to non-SCA siblings revealed poorer SCA-associated functioning in children older than age 4. The results indicate the need for trials assessing the impact of disease modification on children with SCA.https://www.frontiersin.org/articles/10.3389/fstro.2024.1372949/fullsickle cell anemianeurocognitionneurocognitive impairmentpediatric sickle cellsub-Saharan Africa |
| spellingShingle | Paul Bangirana Paul Bangirana Amelia K. Boehme Annet Birabwa Robert O. Opoka Robert O. Opoka Deogratias Munube Deogratias Munube Ezekiel Mupere Phillip Kasirye Grace Muwanguzi Maxencia Musiimenta George Ru Nancy S. Green Richard Idro Richard Idro Neurocognitive impairment in Ugandan children with sickle cell anemia compared to sibling controls: a cross-sectional study Frontiers in Stroke sickle cell anemia neurocognition neurocognitive impairment pediatric sickle cell sub-Saharan Africa |
| title | Neurocognitive impairment in Ugandan children with sickle cell anemia compared to sibling controls: a cross-sectional study |
| title_full | Neurocognitive impairment in Ugandan children with sickle cell anemia compared to sibling controls: a cross-sectional study |
| title_fullStr | Neurocognitive impairment in Ugandan children with sickle cell anemia compared to sibling controls: a cross-sectional study |
| title_full_unstemmed | Neurocognitive impairment in Ugandan children with sickle cell anemia compared to sibling controls: a cross-sectional study |
| title_short | Neurocognitive impairment in Ugandan children with sickle cell anemia compared to sibling controls: a cross-sectional study |
| title_sort | neurocognitive impairment in ugandan children with sickle cell anemia compared to sibling controls a cross sectional study |
| topic | sickle cell anemia neurocognition neurocognitive impairment pediatric sickle cell sub-Saharan Africa |
| url | https://www.frontiersin.org/articles/10.3389/fstro.2024.1372949/full |
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