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Haptoglobin, Alpha-thalassaemia and Glucose-6-phosphate Dehydrogenase Polymorphisms and Risk of Abnormal Transcranial Doppler among Patients with Sickle Cell anaemia in Tanzania.

Cox, S. E., Makani, J., Soka, D., L'Esperence, V. S., Kija, E., Dominguez-Salas, P., Newton, C. R. J., Birch, A. A., Prentice, A. M. and Kirkham, F. J. (2014) Haptoglobin, Alpha-thalassaemia and Glucose-6-phosphate Dehydrogenase Polymorphisms and Risk of Abnormal Transcranial Doppler among Patients with Sickle Cell anaemia in Tanzania. British journal of haematology, 165 (5). pp. 699-706. ISSN 1365-2141

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Abstract

Transcranial Doppler ultrasonography measures cerebral blood flow velocity (CBFv) of basal intracranial vessels and is used clinically to detect stroke risk in children with sickle cell anaemia (SCA). Co-inheritance in SCA of alpha-thalassaemia and glucose-6-phosphate dehydrogenase (G6PD) polymorphisms is reported to associate with high CBFv and/or risk of stroke. The effect of a common functional polymorphism of haptoglobin (HP) is unknown. We investigated the effect of co-inheritance of these polymorphisms on CBFv in 601 stroke-free Tanzanian SCA patients aged <24 years. Homozygosity for alpha-thalassaemia 3•7 deletion was significantly associated with reduced mean CBFv compared to wild-type (β-coefficient -16•1 cm/s, P = 0•002) adjusted for age and survey year. Inheritance of 1 or 2 alpha-thalassaemia deletions was associated with decreased risk of abnormally high CBFv, compared to published data from Kenyan healthy control children (Relative risk ratio [RRR] = 0•53 [95% confidence interval (CI):0•35-0•8] & RRR = 0•43 [95% CI:0•23-0•78]), and reduced risk of abnormally low CBFv for 1 deletion only (RRR = 0•38 [95% CI:0•17-0•83]). No effects were observed for G6PD or HP polymorphisms. This is the first report of the effects of co-inheritance of common polymorphisms, including the HP polymorphism, on CBFv in SCA patients resident in Africa and confirms the importance of alpha-thalassaemia in reducing risk of abnormal CBFv.

Item Type: Article
Keywords: Sickle cell disease, Africa, children, Cerebral blood flow velocity
Subjects: Health Systems > Surveillance, monitoring & evaluation
Divisions: Muhimbili University of Health and Allied Sciences (MUHAS)
Depositing User: Mr Joseph Madata
Date Deposited: 27 Jun 2016 05:36
Last Modified: 27 Jun 2016 05:36
URI: http://ihi.eprints.org/id/eprint/3782

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