Hemoglobin O Arab in four negro families and its interaction with Hemoglobin S and Hemoglobin C

Hemoglobin O Arab was found in 25 members of four apparently unrelated negro families in the West Indies of Jamaica. In each family the propositus had Hb SO disease. Two cases had been mistakenly diagnosed as Hb SC disease. Two persons heterozygous for both Hb C and Hb O Arab were found in these families, and Hb O Arab áthalassemia in one other relative. The clinical course and symptomatology of Hb SO disease is incomparable to that in Hb SD (O2á2121 Glu-> GluNH2) disease and more severe than Hb S2C2 disease. In vitro mixtures of Hb O Arab and Hb S change from a liquid to a gel phase at total hemoglobin concentrations weaker than those required to gel pure Hb S, whereas mixtures of Hb S with Hb A or Hb C require a stronger total hemoglobin concentration before gelling will occur. Oxygen dissociation studies on red cells containing Hb SO show a lowered oxygen affinity comparable to that found in homozygous sickle-cell anemia and outside the range for subjects with sickle-cell Hb C disease.(AU)

Serum gamma globulins and infection in Jamaica

It is widely claimed that people living in the tropics, particularly negroes, have higher gamma globulin levels than Europeans. No such difference was found in a series of healthy Jamaicans and of healthy Europeans recently arrived in Jamaica. The zinc sulphate turbidity test does not measure gamma globulins alone (AU)

The quantitation of globulins by paper electrophoresis

A method is proposed for the quantitation of globulin sub-fractions by paper electrophoresis which overcomes certain difficulties, mainly albumin trailing and the differential dye uptake between albumin and globulins. By this method the globulin sub-fractions can be quantitated with good reproducibility and the results obtained compare favourably with those obtained by the use of Cellulose acetate strips. The error involved in the quantitation of the Alpha globulins is so great that one should be very cautious in interpreting quantitative results of the Alpha globulins. On the other hand the Beta and Gamma can be quantitated with reasonable accuracy (AU)

Serum and urinary electrophoretic studies in proteinuria of pregnancy

Six patients with pre-eclamptic toxaemia were studied by paper elctrophoresis of serum and urine. There was an increase of the O2 Globulin in the sera while in the urine the main proteins found were albumin and B Globulin. In one case there was a significant increase of O1 Globulin in the urine, greater than B. Globulin (A)

Investigation of abnormal haemoglobins in Jamaica: a preliminary study

The literature on abnormal haemoglobins is briefly reviewed. The laboratory findings on 39 cases of sickle cell anaemia (genotype S-S), 14 of whom were adults, and on 13 cases of sickle cell-haemoglobin C disease (genotype S-C), are presented and discussed. Two family studies are included, in one of which three cases of sickle cell-haemoglobin C disease were found. (AU)

The hereditary haemolytic syndromes

A review is made of recent developments in the study of the hereditary haemolytic syndromes. Current concepts in the pathogenesis of haemolysis are briefly discussed and the various intrinsic haemolytic disorders classified, with particular reference to anaemias due to red cell defects. The hereditary haemolytic syndromes are discussed in detail, as regards to both their genetic interrelationship and the recent demonstration of abnormal haemoglobin moieties in sickle cell trait and anaemia, and in haemoglobin C. disease. Reference is made to the recent application of paper electrophoretic analysis to the identification of these abnormal haemoglobins. Whilst hereditary sphercytosis and the thalassaemia syndromes are but rarely seen in the Caribbean area, the incidence of sickle cell trait is noted to vary between 5-12 percent of the mixed populations. Haemoglobin C. trait has been found to occur in 2 per cent of North American Negroes, so that these two anomalies alone or together may be present in a significant proportion of persons. Emphasis is placed on the potentiating effect which results from the linking of the dissimilar genes responsible for the hereditary haemolytic syndromes. The simultaneous occurrence of any one of these together with that determining the sickle cell trait is the probable cause of the so-called "mild" anaemia found to occur in those heterozygous children of whom one parent fails to show the sickling trait. It is stressed that while the hereditary haemolytic syndromes have many feature in common, the actual cause of red cell destruction varies with the shape to which the erythrocyte is ultimately changed (AU)