[Soluble transferrin receptors: significance and diagnostic value in anemia]. / Rastvorimye retseptory transferrina: znachenie v diagnostike anemii.

The study was undertaken for the purpose of demonstrating a diagnostic importance of determining the level of soluble transferrin receptors not only in the diagnosis of iron-deficiency conditions (IDC) or in the differential diagnosis of anemia in a chronic disease (ACD) and of iron-deficiency anemia (IDA) but also in making a diagnosis in case of transfusion-dependent patients with Cooley's anemia. The iron metabolism (including determination of the serum iron (SI), of the total iron-binding ability (TIBA), of saturation of transferrin with iron (STFI), of serum transferrin (ST), and of soluble transferrin receptors (s-STR) was examined in 31 patients with different-genesis anemias, aged 3 to 7. Diagnoses were made for all children on the basis of the standard clinical-and-laboratory examinations and tests. A pattern of iron-metabolism parameters, typical of this pathology, was detected in all IDC patients; it is noteworthy, that the s-STR concentration amounted in this category to 9.4 +/- 1.35 mg/l, which essentially topped the normal values. It can be concluded on the basis of the obtained results that s-STR can be regarded as a key IDC marker. There was an increased SF (up to 324 +/- 64.5 mkg/L) alongside with hypoferremia in the ACD patients; the s-STR content was found to be within the reference values (3.21 +/- 0.55 mg/L), the determination of s-STR can be regarded as a test in the differential diagnosis of IDC and ACD. The results of s-STR research ranged, in patients with Cooley's anemia, drastically from 0.8 mg/L to 17 mg/L; it is noteworthy, that there was a reliable dependence on an adequacy of the conducted therapy in case of such patients. The highest sSTR values were found in children with the hereditary spherocytic hemolytic anemia (11.85 +/- 2.5 mg/L), which is, apparently, explained by a proliferative super-activity of the bone marrow observed in this pathology.

[Pathogenetic mechanisms of the so-called astronauts' anemia]. / Mekhanizmy patogeneza tak nazyvaemoi anemii astronavtov.

The authors analyze the literature data published in the last 20 years on clinical, laboratory, physiological, hematological and biochemical evidence obtained at preflight and postflight examinations of astronauts and experimental animals who spent 5-12 months in space. After space flights the astronauts exhibited a noticeable decrease in red cell mass, in reticulocyte count, hemoglobin and erythropoietin levels (associated with hypochromia and hyposideremia). This condition of erythron in microgravity is termed astronauts' anemia. The authors consider such anemia as an adaptive erythrocytopenic microgravity syndrome with complicated pathogenesis and suggest its model in the form of acquired transitory thalassemia.

[Dynamics of iron metabolism in patients with homozygotic beta-thalassemia after splenectomy]. / Dinamika metabolizma zheleza u bol'nykh gomozigotnoi beta-talassemiei posle splenéktomii.

Iron metabolism was followed up in patients with homozygotic beta-thalassemia after 6 to 18 months, 2-3 years, and 4 to 12 years after splenectomy. Iron reserves of the body were found gradually increasing with time, this being expressed in very high values of serum and red cell ferritins and serum iron. Noteworthy is also an increase of circulating immune complex levels and appearance of a "free" pool of iron. This necessitates regular check-ups of iron metabolism parameters and timely correction of desferal dose.

[Lymphocyte chemiluminescent activity under an iron overload of the body]. / Khemiliuminestsentnaia aktivnost' limfotsitov pri peregruzke organizma zhelezom.

Hyperferremia is shown to affect antioxidant system of the body, oxidation-reduction reactions in the cells seen as shifts in lymphocyte chemiluminescence. Dynamic changes in lymphocyte chemiluminescence reflect the level of hyperferremia.

[Köln hemoglobinopathy: 3 patients in one Russian family]. / Gemoglobinopatiia Köln: troe bol'nykh v odnoi russkoi sem'e.

A rare disease with autosomal dominant inheritance was diagnosed in mother, her son and daughter. The condition was due to unstable hemoglobin Köln in 10% quantity. Clinical symptoms manifested most dramatically in a son, moderately in a daughter and slightly in a mother who had undergone splenectomy. All the hemolytic rises were provoked by antipyretics or, in occasional cases, by respiratory viral infections running with hyperthermia. To alleviate the symptoms, the son is scheduled for splenectomy.

[Indicators of iron metabolism in pregnant women and infants]. / Pokazateli metabolizma zheleza u beremennykh zhenshchin i detei rannego vozrasta.

Iron metabolism parameters were studied in pregnant women in time course of trimesters depending on the pregnancy character and in newborns and infants in relation to the antenatal period. The dynamic study of serum and erythrocytic ferritins has confirmed the expediency of iron therapy during pregnancy for prevention of iron deficiency in infants. Differences detected in the rate of using iron reserves by infants of the first year of life depending on the antenatal period character have necessitated an individual approach to preventive iron therapy.

[Transferrin iron-binding capacity in hypersideremia]. / K voprosu ob obshchei zhelezosviazyvaiushchei sposobnosti transferrina pri gipersideremiiakh.

Three methods for evaluation of serum iron-binding capacity have been described: biochemical, immunological based on transferrin assay, and biophysical based on electron-paramagnetic resonance (EPR) spectroscopy of transferrin. Interrelation has been shown between transferrin and general serum iron-binding capacity. Basing on the data presented it is suggested that in secondary hemochromatosis plasma contains an iron pool that is not specifically bound with transferrin, while in primary hemochromatosis such pool was not detected.

[Erythrocytic ferritin in pregnant women and infants in the first year of life]. / Eritrotsitarnyi ferritin u beremennykh zhenshchin i detei rannego vozrasta.

Erythrocytic ferritin levels were followed up in pregnant women with reference to its course, in newborns, and in infants during the first year of life. It is shown that the level of erythrocytic ferritin remains high enough in women with complicated pregnancy attended by a sharp reduction of iron reserves. No clear dependence of erythrocytic ferritin concentration on the character of the antenatal period course was observed in infants. The highest values of erythrocytic ferritin parameters were recorded by the first month and they decreased constantly by the first year of life.

[Antioxidant activity of blood serum amd erythrocytes in patients with refractory anemia]. / Antioksidantnaia aktivnost' syvorotkii éritrotsitov u bol'nykh refrakternymi anemiiami.

Iron metabolism was studied in patients with refractory anemia. It is shown that continuous transfusions of erythrocytes result in a sharp rise of iron reserves that plays an important role in the development of hemosiderosis. To prevent iron oxidative action it is recommended that the patients should be subjected to therapy with chelating agents and antioxidants simultaneously with transfusions of erythrocytes. A relationship has been noted between blood antioxidant capacity decrease and iron-overloading.

[Regulation of erythropoiesis in patients with iron deficiency anemia]. / Reguliatsiia éritropoéza u bol'nykh zhelezodefitsitnoi anemiei.

Erythropoietin level in the blood plasma, iron metabolism, and some erythron parameters characterizing anemia expression, erythropoiesis effectiveness and processes of hemoglobin synthesis were studied in patients with iron-deficiency anemia (IDA). The results of the investigation helped the authors to establish that hormone production in IDA patients is under the control of a feedback mechanism that is functioning at a lower level as compared to other non-renal anemias. It has been suggested that optimum possible erythron functioning in IDA patients is achieved under these conditions.

[Relation between circulating immune complexes and serum ferritin in hemosiderosis of different etiologies]. / Vzaimosviaz' mezhdu tsirkuliruiushchimi immunnymi kompleksami i ferritinom syvorotki pri gipersiderozakh razlichnoi étiologii.

Parameters of iron metabolism and humoral immunity were studied in patients with chronic diffuse diseases of the liver (cirrhosis, chronic hepatitis), beta-thalassemia major, dyserythropoiesis, hereditary hemochromatosis. High ferritin content has been recorded in the plasma of these patients, that leads to the formation of antibodies to this protein followed by the production of circulating immune complexes inducing metabolic disorders that aggravate the pathologic process. Plasmapheresis and deferoxamine therapy result in a decrease of ferritin and circulating immune complex content in the plasma, that produces a favourable effect on the patients' condition.

[Study of iron metabolism in chronic diffuse diseases of the liver]. / Izuchenie metabolizma zheleza pri khronicheskikh diffuznykh zabolevaniiakh pecheni.

Iron metabolism was studied in patients with chronic diffuse diseases of the liver. A sharp increase of ferritin content at the expense of its release from affected hepatocytes, and formation of antibodies to ferritin of the splenic type have been recorded in patients with cirrhosis of the liver. The observed growth of ferritin content in red blood cells can be explained by functional deficiency of red blood cells and by the presence of hemolytic process (twofold decrease of haptoglobin level). A conclusion has been made that ferritin content in the blood serum does not correlate with the total iron reserves, and serum ferritin level should be considered not as a sign of the pathologic process, but as an index of iron reserves capable of participating in metabolic processes.

[The first case of prenatal diagnosis of sickle cell anemia in the USSR]. / Pervyi sluchai prenatal'noi diagnostiki serpovidnokletochnoi anemii v SSSR.

A married couple with a risk to have a child with sickle-cell anemia has been detected as a result of mass screening of pregnant women in Baku. The prenatal diagnosis was made on the 26th week of pregnancy by means of chordocentesis and biosynthesis of globin chains that revealed the absence of normal beta-globin chain synthesis in the presence of beta S. The diagnosis of sickle-cell anemia was confirmed after abortion in the fetal blood material obtained from the heart by isoelectro-focussing. The family has received a recommendation on the prenatal DNA-diagnosis in the first trimester on the next pregnancy.

[Hereditary cyanosis caused by the presence of abnormal hemoglobin M in the blood: its detection, identification and properties]. / Nasledstvennye ysianozy, obuslovlennye prisutstviem v krovi anomal'nykh Hb gruppy M: vyiavlenie, identifikatsiia, svoistva.

A total of 17 cases of anomalous hemoglobin M (Hb M) were detected among subjects of varying nationalities in different regions of the USSR. The methods used for identification of Hb M Saskatoon, Hb M Boston, Hb M Iwate, Hb M Hyde Park have been described, among them--electron paramagnetic resonance. Spectral characteristics, electrophoretic mobility of these Hb in pH gradient, reaction with cyanides, thermal stability, in vitro reduction with methemoglobin reductase, isolated from donor's red blood cells, have been investigated. The functional parameters (log P50 and n) have been determined for hemolysates containing anomalous hemoglobin, as well as for chromatographically pure fractions of anomalous hemoglobins. The importance of the proper diagnosis of hemoglobinosis M has been stressed.

[Myocardial function in patients with hereditary hemochromatosis based on echocardiographic data]. / O sostoianii miokarda u bol'nykh nasledstvennym gemokhromatozom po dannym ékhokardiografii.

In order to reveal changes in the myocardium at the preclinical stage of heart failure, 34 patients with hereditary hemochromatosis (HHC) underwent echocardiography in the M-mode. There were 26 men and 8 women aged 24 to 59 years. The control group was made up of 20 healthy persons. The HHC patients over 35 years of age (49.5 years on the average) manifested enlargement of the left ventricular cavity, an increase of the thickness of the interventricular septum and of the posterior heart wall, and reduction of pump function of the left ventricle.

[Indicators of nonspecific immunity in patients with hereditary hemochromatosis]. / Pokazateli nespetsificheskoi zashchity u bo'lnykh nasledstvennym gemokhromatozom.

Parameters of nonspecific defence--complement protein function, circulating immune complex level, neutrophil phagocytic activity, completeness of the phagocytic reaction, C3c level, were evaluated in 162 carriers of hemochromatosis gene (114 homozygotes and 48 heterozygotes with respect to hereditary hemochromatosis) aged from 7 to 64 years. The analysis of the results obtained has permitted a conclusion that the suppression of the complement system function, according to the classic or alternative type, is associated with the disease pathogenesis--hyperferremia, and is caused by the damage of the immunocompetent organs and cells synthesizing individual proteins of the complement, as well as by infectious complications leading to hypocomplementemia with respect to separate components.