[Sickle cell anemia (HBSS) in an adult patient from Tanzania]. / Serpovidnaia kletochnaia anemiia (HBSS) u vzroslogo bol'nogo iz Tanzanii.

There is a description of an observation of patient N., aged 29, (a student of Russian University for Peoples' Friendship and a citizen of Tanzania) suffering from sickle-cell anemia--SCA--(HbSS), homozygous type, which was detected at screening of abnormal Hb by electrophoresis on the cellulose acetate strips. The results of clinical-and-biological examinations of the patients are described. The patient had anemia (Hb 98-83 g/L); thrombotic (pain) crises with dark urination were periodically observed. HbS accounted for the bulwark (98%) of Hb, while small fractions of Hb (HbF and HbA2) were within the normal limits. This was a comparatively mild form of the disease; the patient had minor changes in the heart and lungs with other internal bodies being intact. Patient N. received, from childhood, an appropriate therapy including antibiotics, folic acid, analgesics etc. Obviously, this provides for an explanation to that he lived up to 29 years, withstood the acclimatization in Russia and could get education. A comparison of the clinical-and-laboratory indices of patient N. with the data of patients with SCA of other types (HbS-thalassemia, HbSC and HbSK) is presented.

[Limits of chromatographic determination of glycated hemoglobin (Hb A1c) in diabetes mellitus in presence of abnormal hemoglobins]. / Ogranichenie vozmozhnostei khromatograficheskogo opredeleniia glikirovannogo gemoglobina (Hb A1c) pri sakharnom diabete v prisutstvii anomal'nykh gemoglobinov.

The content of glycated hemoglobin (Hb A1c) evaluated by high pressure chromatography on a VARIANT analyzer using Hb A1c software correlated with the mean daily blood glucose level in the majority of diabetics with types 1 and 2 disease and helped evaluate the compensation of diabetes mellitus during the latest 2-3 months of observation. Low Hb A1c values in combination with an extra hemoglobin fraction, unidentified by the software we used, were detected in 3 Russian women suffering from type 2 diabetes mellitus, with high blood glucose levels. Application of Hemoglobinopathy software showed an abnormal spectrum of hemoglobin fractions in the blood of all 3 patients: appearance of hemoglobin D paralleled by decrease of Hb A0. The presence of abnormal hemoglobin D in these patients was confirmed by the results of electrophoresis on cellulose acetate films and a negative test for sickle erythrocytes. Abnormal hemoglobins are responsible for discoordination between glucose content and Hb A1c in the blood of diabetics. Measurement of serum fructosamine is recommended for evaluation of diabetes compensation in patients with hemoglobinopathies.

[Hemoglobinopathies in students at the Russian University of the Friendship of Peoples]. / Gemoglobinopatii u studentov Rossiuskogo universiteta druzhby narodov.

Screening of the university students for hemoglobinopathies detected 153 patients with various hemoglobin abnormalities; 69 with Hb AS, 3 with Hb S-beta-thalassemia, 4 with Hb S-alpha-thalassemia, 3 with hb SC, 1 with Hb SK, 1 with Hb CC, 38 with Hb AC, 1 with Hb A O Arab, 4 with Hb EE, 25 with Hb AE, 1 with Hb AD, 1 with Hb AI, 1 with beta-thalassemia major, and 1 with beta-thalassemia minor. The most grave disease was observed in a child with Hb S-beta-thalassemia and in a youth with beta-thalassemia major. The patterns of Hb SC disease varied. The majority of heterozygote carriers of Hb S, Hb C, Hb E, etc. were healthy. A total of 7000 students were screened by express electrophoresis on cellulose acetate films. more than 400 subjects with abnormal hemoglobins were detected, the most numerous of which were heterozygote carriers of Hb S (Hb AS).

[Beta-thalassemia and Hb D in patients with anemia]. / Beta-talassemiia i Hb D u bol'nykh s anemiei.

Twelve patients with anemias and their close relatives were examined: 8 adults (3 men and 5 women) and 4 children (3 boys and 1 girl). Six of them were Armenians, 1 woman was Russian, and the rest were of mixed origin: 3 Russian-Azerbaijan-Ukrainian, 1 child Russian-Ukrainian-African, and 1 woman Russian-Ukrainian. Hemoglobinopathies were detected in 10 subjects from 4 families (3 families from Donetsk and 1 from Moscow). Homozygotic beta-thalassemia major (Hb F 98.9%) was diagnosed in a 2-year-old Armenian girl from Donetsk. The girl lags behind in development and suffers from anemia with hepatosplenomegaly and jaundice. Heterozygotic beta-thalassemia minor with increased levels of Hb A2 and Hb F was diagnosed in her parents (Armenians from Azerbaijan). A 15-year-old Russian-Azerbaijan-Ukrainian boy from another family in Donetsk had beta-thalassemia with HbD (94%). The boy suffers from anemia with hepatosplenomegaly, jaundice, and chronic hepatitis. Heterozygotic beta-thalassemia with increased levels of Hb A2 and Hb F was revealed in proband's mother and brother; the father was not examined. alpha-Thalassemia is suspected in a 3-year-old boy from a Russian-Ukrainian-African family in Donetsk; he presented with a very small "fast" abnormal hemoglobin fraction. The boy suffers from anemia with splenomegaly and systolic murmur. Blurred form of thalassemia minor is diagnosed in the mother. The father, an African from Zaire, was not examined. Heterozygotic beta-thalassemia with increased Hb A2 level was revealed in a 20-year-old Armenian boy from Moscow. He presented with manifest splenomegaly, chronic gastritis, and mitral valve prolapse. His mother suffers from thalassemia minor, was anemic during pregnancy, and there are cases of anemia in the family. No hematologic disorders were found in the father. No hemoglobinopathies were detected in a 59-year-old Russian women from the town of Tver with very grave anemia; apparently, her condition was acquired, but not hereditary. Data on patients in the city of Donetsk are of special interest, for there are virtually no reports about hemoglobinopathies in the Ukraine.

[Beta-thalassemia and glucose-6-phosphate dehydrogenase deficiency]. / Beta-talassemiia i defitsit gliukozo-6-fosfatdegidrogenazy.

Twenty subjects--patients with anemia and their close relatives representing 7 families, were tested for thalassemia. Heterozygotic beta-thalassemia was detected in 17 cases. The national composition of this patient population was as follows: 7 Pathans (Afghanistan), 1 family; 2 Armenians (Georgia), mother and son; 2 Tajiks, sibs; 2 patients of mixed Russian-Ukrainian-Polish-Azerbaijan origin, mother and son; 1 Russian-Arab child and his Arab father (Syria); 1 Uzbek woman and 1 Russian child. The mean content of Hb in the blood for patients with heterozygotic beta-thalassemia is 110 +/- 3.8 g/liter, that of HbA2 fraction 4.8 +/- 0.26% and of HbF fraction 2.6 +/- 0.39%. Clinical manifestations of the disease varied, being more grave in children than in adults. In an Armenian family from Batumi thalassemia minor was diagnosed in the mother and son, whereas in the father and other son a periodic disease was revealed. In a family of 7 members from Afghanistan thalassemia minor was found in 4 representing the paternal line, minimal thalassemia was suspected in 3, and a deficiency of glucose-6-phosphate dehydrogenase in red blood cells was revealed in 5: in the mother and 4 children. No increase of methemoglobin level was revealed in either of the examinees.

[Apparatus for electrophoresis on cellulose acetate films]. / Pribor dlia élektroforeza na plenkakh iz atsetata tselliulozy.

Type EFPA-30 apparatus for electrophoresis on cellulose acetate films was developed at the Specialized Design Office for Biophysical Equipment of the Moscow Research and Production Association BIOFIZPRIBOR. Three autonomic separation cameras are united in one block, that permits separation of 12, 24, and 36 samples; the apparatus is supplied with time transducer, acoustic and visual indication. Clinical trials of the apparatus were carried out at the Institute of Biological and Medical Chemistry of the USSR Academy of Medical Sciences, its commercial production is to be started. The apparatus is intended for protein separation and may be used for studies of hemoglobins, serum proteins, lipoproteins, enzymes, and other biologic compounds. Methods for hemoglobin and serum protein separation are presented.