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Risk factor analysis of cerebral white matter hyperintensities in children with sickle cell disease.

Abstract：

:Sickle cell disease (SCD) is complicated by silent cerebral infarcts, visible as white matter hyperintensities (WMHs) on magnetic resonance imaging (MRI). Both local vaso-occlusion, elicited by endothelial dysfunction, and insufficiency of cerebral blood flow (CBF) have been proposed to be involved in the aetiology. We performed an explorative study to investigate the associations between WMHs and markers of endothelial dysfunction and CBF by quantifying WMH volume on 3.0 Tesla MRI. We included 40 children with HbSS or HbSβ(0) thalassaemia, with a mean age of 12.1 ± 2.6 years. Boys demonstrated an increased risk for WMHs (odds ratio 4.5, 95% confidence interval 1.2-17.4), unrelated to glucose-6-phosphate dehydrogenase deficiency. In patients with WMHs, lower fetal haemoglobin (HbF) was associated with a larger WMH volume (regression coefficient = -0.62, R2 = 0.5, P = 0.04). Lower ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) levels were associated with lower CBF in the white matter (regression coefficient = 0.07, R2 = 0.15, P = 0.03), suggesting that endothelial dysfunction could potentially hamper CBF. The findings of our explorative study suggest that a high level of HbF may be protective for WMHs and that endothelial dysfunction may contribute to the development of WMHs by reducing CBF.

journal_name

Br J Haematol

journal_title

British journal of haematology

authors

van der Land V,Mutsaerts HJ,Engelen M,Heijboer H,Roest M,Hollestelle MJ,Kuijpers TW,Nederkoorn PJ,Cnossen MH,Majoie CB,Nederveen AJ,Fijnvandraat K

doi

10.1111/bjh.13819

subject

Has Abstract

pub_date

2016-01-01 00:00:00

pages

274-84

issue

2

eissn

0007-1048

issn

1365-2141

journal_volume

172

pub_type

杂志文章

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