Search PubMed⌕ Search

Biomedical subjects

J Y Muller

Publications and source records attributed to J Y Muller.

At least 145 records · Page 8Linked to original sources
PubMed1979

The evolution of asymptomatic monoclonal gammopathies. A follow-up of 20 cases over periods of 3-14 years.

Twenty cases of "asymptomatic" monoclonal gammopathies were detected by routine electrophoresis in patient's sera or in blood donors and were followed over 3-14 years. Four cases have shown a malignant evolution-two evolved toward Waldenström's macroglobulinemia after 3 years and two could be classified as myeloma 3 and 7 years, respectively, after detection of the monoclonal protein. The remaining cases were still "asymptomatic" 4 years later (7 cases), 7-9 years later (8 cases) and 14 years later (1 case). A malignant evolution occurred in approximately 20% of cases.

Adult↗
PubMed1978

[Hereditary haemorrhagic telanglectasia in a large Saharan familly. 87 cases in the same family (author's transl)].

Study of a large family in the Sahara led to the discovery of 87 cases of hereditary haemorrhagic telangiectasia in six generations. This disease had not previously been described in Arab families. The particular structure of this family and the high incidence of consanguinity made it possible to study one individual who was homozygous for hereditary haemorrhagic telangiectasia gene. Examination of the family tree confirms the autosomal dominant transmission of the disorder.

Algeria↗
PubMed1978

[Glanzmann thrombasthenia, PLA1 antigen and anti-Glanzmann antibody].

Six cases of Glanzmann's thrombasthenia were studied using a platelet indirect radioactive Coombs (PIRC). In serum of two among six patients, an antibody was found, which reacted positively with all platelets except those of thrombasthenic patients. Such "anti-public" antibody which shortens the life of transfused platelets is a very serious complication of Glanzmann's thrombasthenia. Attempts to define the PLA group of the six Glanzmann patients, with human allo antisera recognizing PLA1 antigen, gave negative results. Three hypothesis were discussed: (1) Interference in the test of the aggregation defect of thrombasthenic platelets. However, anti-HLA antibodies were normally fixed in the PIRC test. (2) PLA2 gene and Glanzmann gene have a strong gametic association and all Glanzmann's patients are PLA1 negative. (3) Glanzmann gene is coding for the PLA gene substrate. In such case, thrombasthenic patients might be genetically PLA1 positive and phenotypically PLA1 negative. Moreover they would also be PLA2 negative, this could not be tested due to the lack of anti-PLA2 antiserum.

Antibodies↗
PubMed1976

[Substitutive treatment of hemopjolia A using a new factor VIII concentrate].

Thirty children with classical hemophilia were treated with an intermediate-purity factor VIII concentrate prepared according to Newman. The average factor VIII concentration was 21 U/ml. Thirty five injections ranging from 3 to 55 U/kg of body weight were given. The clinical efficacy was satisfactory, no side effects were observed. In vivo factor VIII recovery was initially very high, decreased rapidly until the 4th hour post infusion and then slowed according to a biological half-life of 11.3 +/- 3.1 hours. A satisfactory correlation between the dose injected and the factor VIII in vivo recovery was observed only after four hours post injection. The commercially available concentrate contains in average 25 U of factor VIII per ml, 13 U/mg of fibrinogen and 2.03 U/mg of proteins. It can be injected with a seringe and is therefore adapted to home care treatment. In addition, it is convenient for prolonged treatment with high doses of factor VIII as required by surgical procedure of treatment of patients with an inhibitor, avoiding limitations caused by hypervolemia or hyperfibrinogenemia.

Adolescent↗
PubMed1975

[Cold lymphocytotoxins in various pathological situations].

Cold lymphocytotoxins have been described extensively in many situations. In the present work, this kind of antibody has been found less frequently than previously in some diseases like infectious mononucleosis, rheumatoïd arthritis, or kidney transplanted patients. These discrepancies may be due to technical considerations. In contrast, cold lymphocytotoxins, in the present work, have been frequently detected in auto-immune hemolytic anemias with cold agglutinins, and in patients having immuno-deficiencies.

Anemia, Hemolytic, Autoimmune↗