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Risk factor analysis of cerebral white matter hyperintensities in children with sickle cell disease
Author(s) -
Land Veronica,
Mutsaerts Henri J. M. M.,
Engelen Marc,
Heijboer Harriët,
Roest Mark,
Hollestelle Martine J.,
Kuijpers Taco W.,
Nederkoorn Paul. J.,
Cnossen Marjon H.,
Majoie Charles B. L. M.,
Nederveen Aart J.,
Fijnvandraat Karin
Publication year - 2016
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/bjh.13819
Subject(s) - hyperintensity , medicine , cardiology , cerebral blood flow , endothelial dysfunction , magnetic resonance imaging , odds ratio , white matter , leukoaraiosis , risk factor , confidence interval , pathology , radiology
Summary Sickle cell disease ( SCD ) is complicated by silent cerebral infarcts, visible as white matter hyperintensities ( WMH s) 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 WMH s and markers of endothelial dysfunction and CBF by quantifying WMH volume on 3·0 Tesla MRI . We included 40 children with Hb SS or HbSβ 0 thalassaemia, with a mean age of 12·1 ± 2·6 years. Boys demonstrated an increased risk for WMH s (odds ratio 4·5, 95% confidence interval 1·2–17·4), unrelated to glucose‐6‐phosphate dehydrogenase deficiency. In patients with WMH s, lower fetal haemoglobin (HbF) was associated with a larger WMH volume (regression coefficient = −0·62, R 2 = 0·25, P = 0·04). Lower ADAMTS 13 (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, R 2 = 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 WMH s and that endothelial dysfunction may contribute to the development of WMH s by reducing CBF .