Document Detail


Comparison of homozygous sickle cell disease in northern Greece and Jamaica.
MedLine Citation:
PMID:  1690325     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
The clinical and haematological features of homozygous sickle cell (SS) disease were compared in 30 Greek and 310 Jamacian patients. Deletional alpha-thalassaemia, which modifies SS disease, is rare among Greek patients, so only Jamacian patients with four alpha-globin genes were included in the control group. Greek patients had higher total haemoglobin concentration and red cell counts, and lower mean cell haemoglobin concentration (MCHC) and reticulocyte counts. They also had a more normal body build and more adults had persistent splenomegaly. Fewer had a history of leg ulceration or priapism but more reported acute chest syndrome. The comparatively mild disease in Greek patients is consistent with less haemolysis and sickling and therefore less bone marrow expansion. In the absence of amelioriating factors such as high HbF concentration or alpha-thalassaemia, these findings may be explained by the low MCHC.
Authors:
J Christakis; N Vavatsi; H Hassapopoulou; M Papadopoulou; K Mandraveli; D Loukopoulos; J S Morris; B E Serjeant; G R Serjeant
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Publication Detail:
Type:  Comparative Study; Journal Article    
Journal Detail:
Title:  Lancet     Volume:  335     ISSN:  0140-6736     ISO Abbreviation:  Lancet     Publication Date:  1990 Mar 
Date Detail:
Created Date:  1990-04-16     Completed Date:  1990-04-16     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  2985213R     Medline TA:  Lancet     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  637-40     Citation Subset:  AIM; IM    
Affiliation:
Department of Haematology, AHEPA General Hospital, University of Thessalonika, Greece.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Anemia, Sickle Cell / blood,  complications,  genetics*
Child
Cross-Sectional Studies
Erythrocyte Count
Erythrocyte Indices / genetics
Female
Fetal Hemoglobin / analysis
Genotype
Greece
Hemoglobin A2 / analysis
Hemoglobin, Sickle / analysis
Homozygote*
Humans
Jamaica
Male
Phenotype
Reticulocytes
Retrospective Studies
Thalassemia / blood,  complications,  genetics
Chemical
Reg. No./Substance:
0/Hemoglobin, Sickle; 9034-53-1/Hemoglobin A2; 9034-63-3/Fetal Hemoglobin
Comments/Corrections
Comment In:
Lancet. 1990 Apr 28;335(8696):1035   [PMID:  1970084 ]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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