Sickle cell disease: acute clinical manifestations in early childhood and molecular characteristics in a group of children in Rio de Janeiro
Rev. bras. hematol. hemoter
;
34(3): 196-201, 2012. tab
Article
in English
| LILACS
| ID: lil-640867
ABSTRACT
OBJECTIVE:
To describe clinical events of sickle cell disease and the correlation with β-globin haplotypes and α-thalassemia in under 6-year-old children.METHODS:
A retrospective study was conducted of under 6-year-old children from the neonatal screening program in Rio de Janeiro. Forty-eight male and 48 female children were enrolled in this study, 79 with sickle cell anemia and 17 with hemoglobin SC. The mean age was 29.9 (standard deviation = 20.9) months, 62 (16.2 ± 8.6) were aged between 0-3 years old and 34 (54.9 ± 11.3) were from 3-6 years old. Painful events, acute splenic sequestration, hemolytic crises, hand-foot and acute chest syndromes and infections were evaluated.RESULTS:
The events were more frequent in under 3-year-old children, 94% of children had at least one episode. Infection was the most common event affecting 88.5% of children. Acute splenic sequestration took place earlier, while painful crises and acute chest syndromes in under 6-year-old children. Thal-α 3.7 was observed in 20.9% of cases. Bantu was the most frequent haplotype found, followed by Benin. No correlation was observed between clinical events and β-globin haplotypes. Children with sickle cell anemia and α-thalassemia have less infectious events. No correlation was found among these polymorphisms and clinical events, however, the majority of children with Bantu/Bantu and without α-thalassemia had more clinical events.
Full text:
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Index:
LILACS (Americas)
Main subject:
Haplotypes
/
Clinical Evolution
/
Neonatal Screening
/
Alpha-Thalassemia
/
Anemia, Sickle Cell
Type of study:
Observational study
Limits:
Child
/
Child, preschool
/
Female
/
Humans
/
Male
Language:
English
Journal:
Rev. bras. hematol. hemoter
Journal subject:
Hematology
Year:
2012
Type:
Article
Affiliation country:
Brazil
Institution/Affiliation country:
Fundação Oswaldo Cruz/BR
/
Instituto Estadual de Hematologia Arthur de Siqueira Cavalcanti/BR
/
Universidade Federal Fluminense/BR
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