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Transcranial doppler and magnetic resonance in Tanzanian children with sickle cell disease

Transcranial doppler and magnetic resonance in Tanzanian children with sickle cell disease
Transcranial doppler and magnetic resonance in Tanzanian children with sickle cell disease
Background and Purpose: We determined prevalences of neurological complications, vascular abnormality and infarction in Tanzanian children with Sickle Cell Disease (SCD). Methods: Children with SCD were consecutively enrolled for Transcranial Doppler (TCD); those with slightly elevated (>150cm/s), low (<50cm/s) or absent cerebral blood flow velocity (CBFv) were invited for brain MRI and MRA. Results: Of 200 children (median age 9; range 6-13 years; 105 (52.5%) boys), 21 (11%) and 15 (8%) had previous seizures and unilateral weakness respectively. Twenty-eight (14%) had elevated and 39 (20%) had low/absent CBFv, all associated with lower haemoglobin level, but not higher indirect bilirubin level. On multivariable analysis CBFv>150cm/s was associated with frequent painful crises and low haemoglobin level. Absent/low CBFv was associated with low hemoglobin level and history of unilateral weakness. In 49/67 children with low/absent/elevated TCD undergoing MRI, 43% had infarction while 24/48 (50%) MRAs were abnormal. One had hemorrhagic infarction; none had microbleeds. Posterior circulation infarcts occurred in 14%. Of 11 children with previous seizure undergoing MRI, 10 (91%) had infarction (5 silent) compared with 11/38 (29%) of the remainder (p=0.003). Of 7 children with clinical stroke, 2 had recurrent stroke and 3 died; 4/5 had absent CBFv. Of 193 without stroke, one died and one had a stroke; both had absent CBFv.Conclusions: In one third of Tanzanian children with SCD, CBFv is outside the normal range, associated with frequent painful crises and low hemoglobin level but not hemolysis. Half have abnormal MRA. African children with SCD should be evaluated with TCD; those with low/absent/elevated CBFv should undergo MRI/MRA.
0039-2499
1719-1726
Kija, Edward N.
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Saunders, Dawn E.
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Munubhi, Emmanuel
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Darekar, Angela
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Barker, Simon
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Cox, Timothy C.S.
d20ae762-6fed-4c1d-ab79-db8145c29ade
Mango, Mechris
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Soka, Deogratias
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Komba, Joyce
794c2890-56f7-4fbb-b5d0-8cbe07bc07d9
Nkya, Deogratias A.
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Cox, Sharon E.
3eb867f2-b68d-40a5-b852-e01bd6e37829
Kirkham, Fenella J.
1dfbc0d5-aebe-4439-9fb2-dac6503bcd58
Newton, Charles R.J.C.
782d550e-56c1-4c8e-82d0-a0b7280b09ee
Kija, Edward N.
a6cacc30-76df-4d48-9d75-bcbf3d7c8ee5
Saunders, Dawn E.
0ceef8e6-dfd8-4c92-acf5-87543f449367
Munubhi, Emmanuel
63e78ebb-3e82-42ec-b003-b2f679258522
Darekar, Angela
62e6b511-a358-4e5d-a4ea-03890ba7c2c7
Barker, Simon
4ec1e930-ec52-4154-a7aa-537ff4785607
Cox, Timothy C.S.
d20ae762-6fed-4c1d-ab79-db8145c29ade
Mango, Mechris
ef7f097e-0a1a-4715-ab26-c424b2466af1
Soka, Deogratias
80b7ac9f-b909-48a3-b975-1afc7c739202
Komba, Joyce
794c2890-56f7-4fbb-b5d0-8cbe07bc07d9
Nkya, Deogratias A.
9ed557cf-189d-4425-b9fb-86daa91b6797
Cox, Sharon E.
3eb867f2-b68d-40a5-b852-e01bd6e37829
Kirkham, Fenella J.
1dfbc0d5-aebe-4439-9fb2-dac6503bcd58
Newton, Charles R.J.C.
782d550e-56c1-4c8e-82d0-a0b7280b09ee

Kija, Edward N., Saunders, Dawn E., Munubhi, Emmanuel, Darekar, Angela, Barker, Simon, Cox, Timothy C.S., Mango, Mechris, Soka, Deogratias, Komba, Joyce, Nkya, Deogratias A., Cox, Sharon E., Kirkham, Fenella J. and Newton, Charles R.J.C. (2019) Transcranial doppler and magnetic resonance in Tanzanian children with sickle cell disease. Stroke, 50 (7), 1719-1726. (doi:10.1161/STROKEAHA.118.018920).

Record type: Article

Abstract

Background and Purpose: We determined prevalences of neurological complications, vascular abnormality and infarction in Tanzanian children with Sickle Cell Disease (SCD). Methods: Children with SCD were consecutively enrolled for Transcranial Doppler (TCD); those with slightly elevated (>150cm/s), low (<50cm/s) or absent cerebral blood flow velocity (CBFv) were invited for brain MRI and MRA. Results: Of 200 children (median age 9; range 6-13 years; 105 (52.5%) boys), 21 (11%) and 15 (8%) had previous seizures and unilateral weakness respectively. Twenty-eight (14%) had elevated and 39 (20%) had low/absent CBFv, all associated with lower haemoglobin level, but not higher indirect bilirubin level. On multivariable analysis CBFv>150cm/s was associated with frequent painful crises and low haemoglobin level. Absent/low CBFv was associated with low hemoglobin level and history of unilateral weakness. In 49/67 children with low/absent/elevated TCD undergoing MRI, 43% had infarction while 24/48 (50%) MRAs were abnormal. One had hemorrhagic infarction; none had microbleeds. Posterior circulation infarcts occurred in 14%. Of 11 children with previous seizure undergoing MRI, 10 (91%) had infarction (5 silent) compared with 11/38 (29%) of the remainder (p=0.003). Of 7 children with clinical stroke, 2 had recurrent stroke and 3 died; 4/5 had absent CBFv. Of 193 without stroke, one died and one had a stroke; both had absent CBFv.Conclusions: In one third of Tanzanian children with SCD, CBFv is outside the normal range, associated with frequent painful crises and low hemoglobin level but not hemolysis. Half have abnormal MRA. African children with SCD should be evaluated with TCD; those with low/absent/elevated CBFv should undergo MRI/MRA.

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Accepted/In Press date: 4 April 2019
e-pub ahead of print date: 14 June 2019
Published date: July 2019

Identifiers

Local EPrints ID: 430259
URI: http://eprints.soton.ac.uk/id/eprint/430259
ISSN: 0039-2499
PURE UUID: 9236a63c-7afd-43d1-baa4-8930e696f8e9
ORCID for Fenella J. Kirkham: ORCID iD orcid.org/0000-0002-2443-7958

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Date deposited: 23 Apr 2019 16:30
Last modified: 16 Mar 2024 07:45

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Contributors

Author: Edward N. Kija
Author: Dawn E. Saunders
Author: Emmanuel Munubhi
Author: Angela Darekar
Author: Simon Barker
Author: Timothy C.S. Cox
Author: Mechris Mango
Author: Deogratias Soka
Author: Joyce Komba
Author: Deogratias A. Nkya
Author: Sharon E. Cox
Author: Charles R.J.C. Newton

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