A study of 23 patients with Hb H disease and their 82 relatives in 17 families showed that 2 types of this condition exist. One is associated with the presence of a small slow-moving component, which we tentatively called the X component and which was invariably present in one parent. Some siblings also had it. The other type was not associated with this component. Two patients without X component had a newborn with Bart’s haemoglobin without X component. None of the parents of 20 newborns with Hb Bart’s without the X component had the X component. It was present in only one parent of each of 2 newborns with Hb Bart’s and the X component. They are thought to represent Hb H disease in the newborn period. We suggest that at least 3 abnormal genes may lead to Hb H disease, which results when 2 of the 3 combine. Severity of clinical and haematological symptoms depends upon which abnormal gene is present and which 2 are involved in any particular combination.
Key Words: a-Thalassaemia; Haemoglobin Bart’s; Haemoglobin H disease; Haemoglobinopathies
MeSH terms: Adolescent; Adult; Anemia, Hemolytic/complications; Blood Protein Electrophoresis; Inclusion Bodies; Child; Chromatography, DEAE-Cellulose; Female; Genes; Hemoglobinopathies/complications; Hemoglobinopathies/genetics*; Hemoglobins, Abnormal/analysis*; Humans; Infant; Iron/blood; Malaysia; Male; Molecular Biology; Protein Denaturation; Thalassemia/epidemiology; Umbilical Cord; Asian Continental Ancestry Group
Serological surveys have been widely used in South-East Asia to determine the presence and activity of arboviruses. The haemagglutination-inhibition test has been most frequently employed but complement-fixation and neutralization tests have also been used in some investigations.Although virus isolations provide the most conclusive evidence, they can be carried out in a few specialized centres only, and serological surveys are very important for studying the distribution of arboviruses.The surveys have shown that group B arboviruses (principally all four types of dengue, Japanese encephalitis, and West Nile) are widely prevalent. Dengue and Japanese encephalitis viruses are more widespread than West Nile virus, which was not known previously to extend east of India although recent survyes have shown that its range extends to Burma. Japanese encephalitis is frequent in most of South-East Asia but in India is found mainly in eastern and south-eastern parts of the country. Kyasanur Forest disease (KFD) and Langat viruses are the only tick-borne group B arboviruses definitely known to occur in the region, the former in India, the latter in Malaysia. KFD virus has been isolated only from a small focus in Mysore, although human and animal sera containing neutralizing antibodies to this virus have been found sporadically in widely scattered areas. Among the group A arboviruses, chikungunya and Sindbis have been detected in serological surveys, but the former has not yet been found in Malaysia.
Measurements of child growth have become the most accepted means of assessing the protein-calorie nutritional status of economically developing populations. International reference standards have been suggested for a number of body measurements, but there has been little evaluation of their applicability in South-East Asia.In order to delineate the present range of nutritional standards in West Malaysia, and to aid in the development of guidelines for further nutritional work in this region, anthropometric tests were made on 3 groups of children below school age belonging to the Malay ethnic group and living under diverse conditions. Children of Malaysian Army personnel differed significantly in most measurements from rural village children in Trengganu and children from a small urban elite in Kuala Lumpur. Measurements for the latter group approximated to internationally used standards of height and weight. Parental height data suggest that these differences are not primarily genetic. Anaemia, malaria, and ascariasis were common among the Trengganu children.Weight and height emerge as the measurements that are most sensitive in differentiating between the groups; arm circumferences and skinfold measurements are also useful because they are relatively independent of age. Measurement of chest and head circumferences and of crown-rump lengths appeared to be of little value.