[Biochemical and functional characteristics of erythrocytes in paroxysmal nocturnal hemoglobinuria]. / Biokhimicheskie i funktsional'nye osobennosti éritrotsitov pri paroksizmal'noi nochnoi gemoglobinurii.

Hb composition in varying populations of red blood cells as well as the oxygen-transport function of hemolysates and blood were studied in patients with paroxysmal nocturnal hemoglobinurea (PNH). The dissociation curves obtained for oxyhemoglobin of blood and hemolysates were diphase, that denoted heterogeneity of Hb content and appearance of Hb with high O2 affinity in the blood of PNH patients. Isoelectrofocusing permitted detection of two additional fractions X1 and X2. The immunochemical method was used to show that X1 fraction was semi-Hb, and X2 fraction, by its physicochemical properties, was affined to Hb conformer--Hb precursor during its biosynthesis. The data obtained have shown that in PNH patients a pathologic population of red blood cells with incomplete process of hemoglobin formation appears in the blood flow.

[Differential diagnosis of unconjugated hyperbilirubinemia]. / Differentsial'naia diagnostika nekon''iugirovannykh giperbilirubinemii.

Eighty-four patients with unconjugated hyperbilirubinemia were examined for erythron. The authors managed to reveal a group of patients with evident signs of the derangement of the nucleus-containing cells of erythropoiesis which was manifested in the accumulation of SIA-positive material in the cytoplasm and by the presence of morphological abnormalities in the peripheral red blood cells.

[Pulmonary arterial hypertension in patients with hereditary hemorrhagic telangiectasia (Osler-Rendu disease)]. / Legochnaia arterial'naia gipertenziia u bol'nykh nasledstvennoi gemorragicheskoi teleangiektaziei (bolezn'iu Randiu-Oslera).

Pulmonary arterial pressure was studied using a noninvasive method in 23 patients with hereditary hemorrhagic telangiectasia (HHT) without clinical and x-ray signs of arterial-venous shunting in the lungs. It was established that pulmonary arterial hypertension was typical of HHT patients. A significant decrease in the volume and frequency of hemorrhages in 12 of 17 HHT patients was combined with a statistically significant decrease in pulmonary arterial pressure as a result of HBO therapy. It was assumed that pulmonary microcirculatory disorders played an important role in HHT pathogenesis and were expressed in metabolic derangement of a number of biologically active substances in the lungs resulting in the development of telangiectasia.