Hemoglobinopatias / Hemoglobinopathies

The hereditary hemoglobinopathies are frequent disease among the Brazilian population, consisting of a public health problem. The two hemoglobinopathies mostly found our environment are: S hemoglobinopathies and the thalassemia B. While the S hemoglobin is a national problem, the thalassemia of B type represents only a regional problem, being observed, with greater incidence, in some Brazilian states where thre were a large numbre og immigrants coming from the Mediterranean. The hemoglobinopathies are mutations that affect the regulators genes of the human hemoglobin synthesis,...

Specific health problems of Southeast Asian refugees in middle Tennessee.

Three diseases representative of specific health conditions affecting the Southeast Asian refugees living in middle Tennessee are leprosy (chronic bacterial infections), liver fluke infection (parasitic diseases), and hemoglobin E-beta-thalassemia (hematologic disorders). In this paper we discuss incidence, causative agent, mode of transmission, metabolic abnormalities, and management of these conditions.

[Thalassemia and Hansen's disease]. / Talassemia e hanseníase.

The beta-thalassemia trait was investigated among 165 Brazilians who were unmixed Italian descendants (80 Virchowian patients and 85 normal controls, composed of universitary students). The frequency of the beta-thalassemia trait was 6.25% among the Virchowian patients and 5.88% in the control group. In spite of the similar geographical distribution of both hanseniasis and the gene for beta-thalassemia in Asia, the present data does not support the hypothesis that hanseniasis might have contributed to maintain high prevalence of this allele by selection favouring beta-thalassemia trait.

Talassemia e hanseniase / Thalassemia and leprosy

A talassemia B foi investigada em 165 brasileiros descendentes nao miscigenados, 80 dos quais eram doentes de hanseniase, da forma virchowiana, e 85 estudantes universitarios (grupo controle). A frequencia da talassemia foi de 6,25 por cento entre hansenianos e de 5,88 por cento entre os estudantes. Apesar da similaridade da distribui;ao geografica tanto da hanseniase quanto do gene da talassemia B eram algumas regioes do globo, sobretudo da Asia, a frequencia de individuos, com o estigma talassemico observada nas amostras analisadas nao apoia a hipotese de que a hanseniase possa ter contribuido para manter as altas frequencias desse alelo, por intermedio da sele;ao a favor dos talassemicos heterozigotos.

[Frequency of hemoglobin abnormalities and hemoglobinopathies in Mozambique peoples (comparison between leprosy and non-leprosy subjects]. / Uber die Häufigkeit von Hämoglobinanomalien und Hämoglobinopathien bei moçambiquanischen Völkerschaften. (Mit Vergleich zwischen Leprösen und Nicht-Leprösen)

In 588 bloodsamples of negride natives from Moçambique, preferably Chuabo and Macua, haemoglobin analyses were performed. In 21 cases an increase of Hb A2 was found, indicating the presence of heterozygous beta-thalassaemia, in one case the changes in Hb-analysis were typical for beta-delta-thalassaemia, 18 samples could be shown to contain Hb S, typical for the heterozygous sickle cell trait. Futhermore in 7 cases Hb A2' was found. In two bloodsamples haemoglobin variants were observed, which according to their electrophoretical mobility were assumed to represent Hb D in one case, and Hb G in the other. In the Chuabo population the frequency of the thalassaemia gene was found to be more than twice as high as in the Macua population. In non-lepers Hb S was observed with a remarkable higher incidence than in lepers.