An unusual type of hemoglobinopathy resembling sickle cell-thalassemia disease in a Jamaican family
Blood
; 13(6): 559-68, 1958.
Artigo
em Inglês
| MedCarib
| ID: med-9540
Biblioteca responsável:
JM3.1
Localização: JM3.1; RB145.A1B5
ABSTRACT
Three generations of a Jamaican family of African extraction are desribed, in several members of which an abnormal gene is carried. This gene produces high levels of fetal hemoglobin unassociated with the usual stigmata of thalassemia. It is found in all three generations of the family associated with hemoglobin A only and is also found in at least two members of the family interacting with hemoglobin S. In the latter combination little or no disability results. The mode of inheritance of this abnormal gene is discussed, and reasons are put forward for a possible protective effect of high fetal hemogobin levels due to inhibition of sickling. The findings in the cord blood of the youngest child, including an unusually high percentage of sickling, are discussed, together with follow-up studies to the age of 25 weeks.(AU)
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Coleções:
Bases de dados internacionais
Base de dados:
MedCarib
Assunto principal:
Talassemia
/
Hemoglobinopatias
Tipo de estudo:
Estudo observacional
/
Estudo prognóstico
/
Fatores de risco
Limite:
Adolescente
/
Adulto
/
Idoso
/
Criança
/
Criança, pré-escolar
/
Feminino
/
Humanos
/
Lactente
/
Masculino
/
Recém-Nascido
País/Região como assunto:
Caribe Inglês
/
Jamaica
Idioma:
Inglês
Revista:
Blood
Ano de publicação:
1958
Tipo de documento:
Artigo