Effect of alpha-globin genotype on the pathophysiology of sickle cell disease.
Pediatr Pathol Mol Med
; 20(2): 107-21, 2001.
Article
en En
| MEDLINE
| ID: mdl-12673836
ABSTRACT
The clinical picture of sickle cell disease is heterogeneous and varies tremendously among patients and in the same patient from time to time. The level of HbF, alpha-genotype, beta-haplotype, age, sex, and the environment are important factors that modify the clinical picture of sickle cell disease. My paper focuses on the effect of alpha-globin genotype on the pathophysiology of sickle cell anemia, HbSC disease, and sickle beta-thalassemia. The data indicate that the coinheritance of alpha-thalassemia results in some beneficial effects and in some harmful effects. Thus, there are trade-offs involved in this interaction in which the salutary effects are undermined by harmful ones.
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Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Globinas
/
Anemia de Células Falciformes
Límite:
Adult
/
Female
/
Humans
/
Male
/
Middle aged
Idioma:
En
Revista:
Pediatr Pathol Mol Med
Asunto de la revista:
BIOLOGIA MOLECULAR
/
PATOLOGIA
/
PEDIATRIA
Año:
2001
Tipo del documento:
Article
País de afiliación:
Estados Unidos