Hemoglobine C (α2β2 6Glu→Lys) Etude De 19 Porteurs Heterozygotes Ac Et De 5 Cas De Double Hemoglobinopathy Sc
作者:
CaluwéJ.P. De,
AlexanderM.,
BondueH.,
期刊:
Acta Clinica Belgica
(Taylor Available online 1993)
卷期:
Volume 48,
issue 5
页码: 297-306
ISSN:1784-3286
年代: 1993
DOI:10.1080/17843286.1993.11718322
出版商: Taylor&Francis
数据来源: Taylor
摘要:
SummaryThis paper describes clinical findings in subjects heterozygous for hemoglobin C and in compounds heterozygotes SC. Most of our patients were coming from lands hording the Guinea Gulf, on the inside of the loop made by the river Niger and from Haiti, which populated by African slaves coming mainly from the Golden Coast (actually Ghana). Heterozygous are Symptomatic and do not suffer from anemia. Age at diagnosis is higher than for SCA, although one of our cases was diagnosed at age one. Overwhelming infections or major anemia were not observed. Basal hemolysis remained moderate. HbF levels were not increased as they may be in sickle cell anemia. Splenomegaly was observed in 2/5 patients. Among severe complications we encountered, we must mention one case of foetal death, one tibial infarct, one case of pure red cell aplasia caused by parvovirus B19 infection, and one case of proliferative retinopathy.
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