AS heterozygote hemoglobinopathy and coronary risk

There have been several reports of vaso-occlusive events and sudden death in subjects with sickle cell trait. However, the precise mechanism underlying these episodes remains unclear. The clinical observations have been supported by in vitro studies in which haemoglobin AS (Hb AS) red cells showed a...

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Bibliographic Details
Published in:Archives des maladies du coeur et des vaisseaux Vol. 92; no. 12; p. 1727
Main Authors: Ould Amar, A K, Pi Gibert, A, Darmon, O, Besse, P, Cénac, A, Césaire, R
Format: Journal Article
Language:French
Published: France 01-12-1999
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Summary:There have been several reports of vaso-occlusive events and sudden death in subjects with sickle cell trait. However, the precise mechanism underlying these episodes remains unclear. The clinical observations have been supported by in vitro studies in which haemoglobin AS (Hb AS) red cells showed abnormalities of their filterability, probably related to gelling or polymerisation of the Hb AS. These in vitro studies and reports in the literature of sickle-cell hearts led the authors to investigate the possible association between AS subject and coronary risk. The results of coronary angiography in 9 patients with Hb AS, paired with respect to the usual cardiovascular risk factors, were compared with those of 18 AA subjects. The number of patients who underwent coronary bypass surgery for three-vessel disease was much greater in the AS subjects. However, the difference was not statistically significant. This tendency of AS subjects to develop thrombosis and coronary artery disease requires further study with larger numbers of patients.
ISSN:0003-9683