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Functional studies of abnormal hemoglobins in Japan.

Oxygen binding properties of fifteen kinds of abnormal hemoglobins discovered in Japan were elucidated with the automatic oxygenation apparatus which was developed by Imai et al. This paper reviews the functional characteristics of those hemoglobins previously reported, presents functional data of some newly studied hemoglobins, and gives a discussion on structure-function relationships in those hemoglobins. This paper also describes a simple procedure for generating a predicted oxygen equilibrium curve of non-isolatable hemoglobin from equilibrium curves of normal hemolysate and hemolysate containing that hemoglobin.

Hemoglobins, Abnormal↗

Quantitative analysis of abnormal hemoglobins by agar gel electrophoresis.

Measurement of the proportion of abnormal hemoglobin in a hemolysate is essential for differentiation of simple and compound heterozygotes (e.g., sickle cell trait vs. sickle thalassemia), for differential of alpha- and beta-thalassemia in compound heterozygotes, and for differentiation of various types of beta-thalassemia in such persons. Utilization of such measurements is hampered by the imprecision and inconvenience of current methods. We have adapted a readily available agar electrophoresis method for this purpose, scanning unstained gels at 420 nm. The new method is particularly valuable for rapid estimation of percent of HbS after partial exchange transfusion in patients with sickle cell anemia. It cannot be used for quantitation of HbF or for quantitation of hemoglobins that comigrate with HbA; contrariwise, it can be used for hemoglobins that only separate from HbA on agar (e.g., HbBethesda).

Anemia, Sickle Cell↗

[Acquired hemoglobin abnormalities (author's transl)].

Along with the hereditary forms of pathological hemoglobin, there are a certain number of acquired hemoglobin abnormalities, in particular hemoglobin H and reappearance of foetal hemoglobin. Reactivation of foetal hemoglobin synthesis is seen in a wide variety of physiopathological states and may be accompanied by other changes in erythrocytic properties. On the other hand, hemoglobin H is far more more rare and is always associated with leukemia. Study of family history and the hematologic content generally allow differentiation between an acquired abnormality and hereditary forms of pathological hemoglobin.

Anemia, Aplastic↗

Abnormal hemoglobins, glucose-6-phosphate dehydrogenase deficiency and hereditary ovalocytosis in the Dayaks of Sarawak.

A survey of abnormal hemoglobins, G6PD deficiency and hereditary ovalocytosis was carried out among the Dayaks of Sarawak. The only abnormal hemoglobin found was Hb Co Sp, which occurred in 0.35% of the Land Dayaks and 0.83% of the Sea Dayaks. G6PD deficiency occurred in 5.3% of the male Land Dayaks and 5.0% of the male Sea Dayaks; no electrophoretic variant of G6PD was found in any of the 285 Land Dayaks and 240 Sea Dayaks examined. Hereditary ovalocytosis was found in 12.7% of the Land Dayaks and 9.0% of the Sea Dayaks.

Elliptocytosis, Hereditary↗

A simple finger-prick method of screening for abnormal hemoglobins.

Thambipillai, Shanthi, and Senewiratne, B.: A simple finger-prick method of screening for abnormal hemoglobins; Am J Clin Pathol 63: 836-840, 1975. Two simple methods of hemoglobin electrophoresis of samples obtained by finger prick are described. The methods do not involve venipuncutre, centrifugation, or the preparation of a hemolysate, and can be carried out on starch gel or cellulose acetate membranes. The specimen can be collected even by non-medical personnel and sent by post to the laboratory. Alternatively, electrophoresis can be carried out in the field and the results obtained within 20 minutes. Both methods are suitable for population screening and pediatric studies. The method will detect structurally abnormal hemoglobins only.

Blood Protein Electrophoresis↗