Quantitative evaluation of mepacrine labelled human platelet dense bodies in normals and in cases of peripheral thrombocytopenia.
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Biomedical subjects
Publications and source records attributed to R Bierme.
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Factor VIII complex was studied in patients presenting arterial hypertension. Visceral involvement was quantified using a clinical index calculated from ocular fundus, renal function and left ventricular hypertrophy data. A significant correlation was found between the mean arterial pressure, the visceral involvement and the level of complex VIII. Nevertheless, other data obtained in different patients (Conn's disease) suggest that the visceral involvement (and not the mean arterial pressure) is the main determining factor in the increase of factor VIII complex.
In several patients suffering from congenital non-spherocytic hemolytic anemia or from malignant hemotological disorder associated with erythrocyte pyruvate kinase (PK) deficiency, a metabolic study has been carried out involving the following biochemical determinations: assay of red cell enzyme activities; estimation of glucose consumption; measurement of the rate of glycolytic intermediates; and, in some cases, enzyme purification and characterization of the PK variant. Metabolic equilibrium most probably does not depend on kinetic characteristics of PK molecules. Furthermore, the data obtained allow separation of cases with congenital non-spherocytic hemolytic anemia (hereditary defect) and acquired PK deficiencies.
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Glucose-6-phosphate dehydrogenase was purified from blood samples originating from 33 subjects living in the 'Midi-Pyrenees' region (Southwest France). Biochemical and electrophoretic characteristics of several enzymatic variants were revealed. The distribution of the mutations in the subjects studied gave the following results: (1) Meditteranean Gd(-) and Gd(-)A identified variants; they constitute the main part of our observations. (2) Two original variants confirmed by the WHO International Reference Laboratory, that is: Gd(-) Toulouse and Gd(+) Luz-Saint-Sauveur; the latter mutation did not produce any haematological disorder. Absence of chronic haemolytic anaemia as well as a high rate of favism could be found in the families studied. As it is generally observed in Mediterranean regions, Southwest France population is characterized by a certain heterogeneity in biochemical and genetic mutations.
After a brief survey of some physiologic properties of Von Willebrand factor activity (VIII r WF) the numerous clinical states with an increase of this activity are described. The authors report their own results concerning sepsis, chronic arteritis of lower limbs, diabetes, toxemia of pregnancy. In most cases the increase of VIII r WF activity appears as a sign of vascular injury and/or the consequence of tissular damage.
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