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Cerebral infarcts and vasculopathy in Tanzanian children with sickle cell anemia

Cerebral infarcts and vasculopathy in Tanzanian children with sickle cell anemia
Cerebral infarcts and vasculopathy in Tanzanian children with sickle cell anemia

Background: Cerebral infarcts and vasculopathy in neurologically asymptomatic children with sickle cell anemia (SCA) have received little attention in African settings. This study aimed to establish the prevalence of silent cerebral infarcts (SCI) and vasculopathy and determine associations with exposure to chronic hemolysis, anemia, and hypoxia.

Methods: We prospectively studied 224 children with SCA with transcranial Doppler (TCD), and magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA). Regressions were undertaken with contemporaneous hemoglobin, reticulocyte count, mean prior hemoglobin, oxygen content, reticulocyte count, and indirect bilirubin.

Results: Prevalence of SCI was 27% (61 of 224); cerebral blood flow velocity was abnormal (>200 cm/s) in three and conditional (>170<200 cm/s) in one. Vasculopathy grades 2 (stenosis) and 3 (occlusion) occurred in 16 (7%) and two (1%), respectively; none had grade 4 (moyamoya). SCI was associated with vasculopathy on MRA (odds ratio 2.68; 95% confidence intervals [95% CI] 1.32 to 5.46; P = 0.007) and mean prior indirect bilirubin (odds ratio 1.02, 95% CI 1.00 to 1.03, P = 0.024; n = 83) but not age, sex, non-normal TCD, or contemporaneous hemoglobin. Vasculopathy was associated with mean prior values for hemoglobin (odds ratio 0.33, 95% CI 0.16 to 0.69, P = 0.003; n = 87), oxygen content (odds ratio 0.43, 95% CI 0.25 to 0.74, P = 0.003), reticulocytes (odds ratio 1.20, 95% CI 1.01-1.42, P = 0.041; n = 77), and indirect bilirubin (odds ratio 1.02, 95% CI 1.01 to 1.04, P = 0.009).

Conclusions: SCI and vasculopathy on MRA are common in neurologically asymptomatic children with SCA living in Africa, even when TCD is normal. Children with vasculopathy on MRA are at increased risk of SCI. Longitudinal exposure to anemia, hypoxia, and hemolysis appear to be risk factors for vasculopathy.

MRA, MRI, Silent cerebral infarction, Transcranial Doppler, Vasculopathy
0887-8994
Jacob, Mboka
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Saunders, Dawn E.
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Sangeda, Raphael Z.
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Ahmed, Magda
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Tutuba, Hilda
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Kussaga, Frank
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Musa, Balowa
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Mmbando, Bruno
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Slee, April E.
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Kawadler, Jamie M.
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Makani, Julie
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Kirkham, Fenella J.
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Jacob, Mboka
21ec01f2-e57b-487c-b282-a93e57e59078
Saunders, Dawn E.
21dab713-1e6a-486f-8bfa-eea35b382b02
Sangeda, Raphael Z.
3ad535ce-45d1-410f-9ddf-068ad7dfb129
Ahmed, Magda
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Tutuba, Hilda
f57728b3-55e1-4791-bbd3-a0985d43da21
Kussaga, Frank
dea166f7-0dbc-495d-8c68-80a66c1b36ed
Musa, Balowa
e08fc8ca-b571-4028-ab04-4d6b1c13dc65
Mmbando, Bruno
204e8234-af85-45e7-842c-9184f50a0122
Slee, April E.
993ac4ad-4ee4-437a-9f42-e27f8a7046d0
Kawadler, Jamie M.
7d035760-69ea-4b6c-8a7a-771b73453db8
Makani, Julie
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Kirkham, Fenella J.
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Jacob, Mboka, Saunders, Dawn E., Sangeda, Raphael Z., Ahmed, Magda, Tutuba, Hilda, Kussaga, Frank, Musa, Balowa, Mmbando, Bruno, Slee, April E., Kawadler, Jamie M., Makani, Julie and Kirkham, Fenella J. (2019) Cerebral infarcts and vasculopathy in Tanzanian children with sickle cell anemia. Pediatric Neurology. (doi:10.1016/j.pediatrneurol.2019.12.008).

Record type: Article

Abstract

Background: Cerebral infarcts and vasculopathy in neurologically asymptomatic children with sickle cell anemia (SCA) have received little attention in African settings. This study aimed to establish the prevalence of silent cerebral infarcts (SCI) and vasculopathy and determine associations with exposure to chronic hemolysis, anemia, and hypoxia.

Methods: We prospectively studied 224 children with SCA with transcranial Doppler (TCD), and magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA). Regressions were undertaken with contemporaneous hemoglobin, reticulocyte count, mean prior hemoglobin, oxygen content, reticulocyte count, and indirect bilirubin.

Results: Prevalence of SCI was 27% (61 of 224); cerebral blood flow velocity was abnormal (>200 cm/s) in three and conditional (>170<200 cm/s) in one. Vasculopathy grades 2 (stenosis) and 3 (occlusion) occurred in 16 (7%) and two (1%), respectively; none had grade 4 (moyamoya). SCI was associated with vasculopathy on MRA (odds ratio 2.68; 95% confidence intervals [95% CI] 1.32 to 5.46; P = 0.007) and mean prior indirect bilirubin (odds ratio 1.02, 95% CI 1.00 to 1.03, P = 0.024; n = 83) but not age, sex, non-normal TCD, or contemporaneous hemoglobin. Vasculopathy was associated with mean prior values for hemoglobin (odds ratio 0.33, 95% CI 0.16 to 0.69, P = 0.003; n = 87), oxygen content (odds ratio 0.43, 95% CI 0.25 to 0.74, P = 0.003), reticulocytes (odds ratio 1.20, 95% CI 1.01-1.42, P = 0.041; n = 77), and indirect bilirubin (odds ratio 1.02, 95% CI 1.01 to 1.04, P = 0.009).

Conclusions: SCI and vasculopathy on MRA are common in neurologically asymptomatic children with SCA living in Africa, even when TCD is normal. Children with vasculopathy on MRA are at increased risk of SCI. Longitudinal exposure to anemia, hypoxia, and hemolysis appear to be risk factors for vasculopathy.

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Accepted/In Press date: 14 December 2019
e-pub ahead of print date: 27 December 2019
Keywords: MRA, MRI, Silent cerebral infarction, Transcranial Doppler, Vasculopathy

Identifiers

Local EPrints ID: 440617
URI: http://eprints.soton.ac.uk/id/eprint/440617
ISSN: 0887-8994
PURE UUID: 9e8a8e79-3b36-4b03-9c40-ea8940456095
ORCID for Fenella J. Kirkham: ORCID iD orcid.org/0000-0002-2443-7958

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Date deposited: 12 May 2020 16:35
Last modified: 13 Aug 2022 01:39

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Contributors

Author: Mboka Jacob
Author: Dawn E. Saunders
Author: Raphael Z. Sangeda
Author: Magda Ahmed
Author: Hilda Tutuba
Author: Frank Kussaga
Author: Balowa Musa
Author: Bruno Mmbando
Author: April E. Slee
Author: Jamie M. Kawadler
Author: Julie Makani

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