RESUMO
Hematologic and globin chain synthesis studies have been made in 21 children, aged 2 to 6 years, many of their parents, and several normal adults and alpha-thalassemia heterozygotes. At birth, 11 children had about 5% hemoglobin (Hb) Bart's, 5 had about 2% Hb Bart's, and 5 had no trace of Hb Bart's. A significant decrease in mean corpuscular volume. (MCV) and mean corpuscular hemoglobin (MCH) values and an increase in the beta/alpha ratio was observed in the first group; microcytosis and hypochromia were absent in the children of the second group although the beta/alpha ratio was significantly increased. The alpha chain deficiency is familial. Increased alpha/alpha ratios were present in many parents although only two parents of children with 5% Hb Bart's at birth had hematologic findings suggestive of the presence of the same type of defect as observed in the children with the larger amount of Hb Bart's at birth.
Assuntos
Hemoglobinopatias/genética , Adulto , População Negra , Criança , Pré-Escolar , Eritrócitos/crescimento & desenvolvimento , Feminino , Genótipo , Georgia , Hematócrito , Hemoglobinometria , Hemoglobinopatias/sangue , Hemoglobinas Anormais , Humanos , Recém-Nascido , Estudos Longitudinais , Masculino , Linhagem , Talassemia/genéticaRESUMO
Haemoglobin A(2) in 11 normal and 42 B thalassaemia minor subjects was estimated by simple visual assessment of the electrophoretogram. The results obtained give rise to doubts as to the reliability of this technique when used to estimate haemoglobin A(2) under normal laboratory conditions.
Assuntos
Hemoglobinas/análise , Eletroforese das Proteínas Sanguíneas , Cromatografia , Hemoglobinometria , Humanos , Métodos , Talassemia/sangue , Visão OcularRESUMO
An automated technique is described which is capable of detecting sickle-cell haemoglobin and differentiating the sickle-cell trait from sickle-cell anaemia. The method is based upon the Itano solubility test and utilizes Technicon equipment.
Assuntos
Anemia Falciforme/diagnóstico , Autoanálise , Hemoglobinas Anormais/análise , Anemia Falciforme/sangue , Soluções Tampão , Diagnóstico Diferencial , Doença da Hemoglobina C/sangue , Hemoglobinometria/instrumentação , HumanosAssuntos
Anemia Falciforme/diagnóstico , Doenças do Recém-Nascido/diagnóstico , Análise Química do Sangue , Eletroforese das Proteínas Sanguíneas , Eletroforese em Papel , Feminino , Hemoglobina Fetal/análise , Doença da Hemoglobina C/diagnóstico , Hemoglobinas/análise , Humanos , Recém-Nascido , Doenças do Prematuro/diagnóstico , Métodos , Gravidez , Talassemia/diagnóstico , Cordão UmbilicalAssuntos
Mioglobina/análise , Acrilatos , Idoso , Sequência de Aminoácidos , Aneurisma Aórtico , Arginina , Eletroforese , Feminino , Código Genético , Glutamina , Humanos , Proteínas Musculares/análise , Peptídeos/análise , TripsinaRESUMO
A simple and rapid screening test which differentiates sickle-cell trait and sickle-cell anaemia is described. The test utilizes 0.1 ml of whole blood and is based on the low solubility of reduced sickle haemoglobin. Results intermediate between the sickle-cell trait and sickle-cell anaemia are obtained in unusual cases of sickle-cell anaemia with high foetal haemoglobin. The need to supplement the results with haematological and electrophoretic techniques is discusses.
