Document Detail


New results in clinical severity of homozygous sickle cell anemia, in Dakar, Senegal.
MedLine Citation:
PMID:  10651122     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Despite the unicity of its genetic mutation, Sickle cell homozygosity presents different clinical features. Our objectives were to evaluate disease severity in Senegalese patients. Sixty (60) homozygous sickle cell patients were followed up monthly during one year and disease severity was assessed using the "severity index" (SI) which is resulting from epidemiologic, clinic and biological data. Mean age was 20.13, sex ratio was 0.87 and mean age of diagnosis was 9.8 years. 90% of patients presented vaso-occlusive crisis (2.53 per patient), 73.3% had infectious episodes (1.9 per patient), 69.3% had never been transfused and 25% of patients had presented chronic complications linked to anemia or ischemia. Mean hemoglobin value was 8.1 g/dl and mean Hb F was 8.2%. Low seric ferritin was found in 1.7% of patients. Benign form of homozygous sickle cell anemia (SI< or =6) was found in 48.3% of patients. Our data confirm the relative good tolerance of homozygous sickle cell disease in Senegal. The haplotype Senegal may play an important role but others host and environmental factors operate certainly because some severe cases were identified in our patients. The identification of all these factors might contribute to a better follow up of sickle cell disease.
Authors:
S Diop; D Thiam; M Cisse; A O Toure-Fall; K Fall; L Diakhate
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Hematology and cell therapy     Volume:  41     ISSN:  1269-3286     ISO Abbreviation:  Hematol Cell Ther     Publication Date:  1999 Nov 
Date Detail:
Created Date:  2000-02-23     Completed Date:  2000-02-23     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  9613253     Medline TA:  Hematol Cell Ther     Country:  FRANCE    
Other Details:
Languages:  eng     Pagination:  217-21     Citation Subset:  IM    
Affiliation:
Service d'Hématologie et d'Immunologie, CNTS, Dakar-Fann Senegal.
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MeSH Terms
Descriptor/Qualifier:
Activities of Daily Living
Adolescent
Adult
Anemia, Sickle Cell / complications,  epidemiology*,  physiopathology*
Arterial Occlusive Diseases / etiology
Blood Cell Count
Blood Transfusion
Child
Child, Preschool
Family Health
Female
Fetal Hemoglobin / analysis
Hemoglobins / analysis
Homozygote
Humans
Infection / etiology
Iron / blood
Longitudinal Studies
Male
Middle Aged
Prospective Studies
Senegal / epidemiology
Severity of Illness Index
Chemical
Reg. No./Substance:
0/Hemoglobins; 7439-89-6/Iron; 9034-63-3/Fetal Hemoglobin

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


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