[Epidural localization disclosing an acute lymphoblastic leukemia. Apropos of 2 cases].
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Biomedical subjects
Publications and source records attributed to M Goudemand.
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From 1971 to 1979, 219 patients with idiopathic thrombocytopenic purpura (ITP) were seen and all subjected to the same scheme of treatment. there were 150 adults and 69 children ( less than 15 years old), 140 were females and 79 males. At presentation, 152 had had symptoms for less than 3 months ("recent" ITP) and 67 could be immediately considered as chronic ITP (since they had had symptoms for more than 3 months). Whatever the duration of symptoms, corticosteroid therapy (1.5 to 2 mg/kg/day during 3 weeks) was first administered. At the end of the treatment, normal platelet count was achieved in 92.5% (136/147) of the "recent" ITP and 77% (51/66) of the chronic ITP. One year later, a complete remission was still evident in 65% (96/147) of the "recent" ITP but in only 14% (9/66) of the chronic ITP 3 of whom had died of hemorrhage. So the total number of patients with chronic ITP, non responders to prednisone therapy was 110. In 19 cases, the further progression was unknown and 16 patients were in partial remission. Splenectomy, indicated in 75 patients, was performed in only 64. After 2 weeks the platelet count was normal in 94% (60/64) of the patients. The success (platelets greater than 100 000/microliter) was pronounced, one year after splenectomy, in 86% (55/64) of them. A splenic sequestration of the radiolabelled platelets appeared to be the only positive prognostic factor. Of 15 patients treated with azathioprine (2.5 mg/kg/day) over a 1 to 5 year period, 8 have gone into remission and 7 failed to respond (2 died from hemorrhage). Excluding the 19 patients with an unknown progression, the results are finally as follows: remissions (or success): 84.5% (169/200), partial remission: 8% (16/200), complete failure: 7.5% (15/200) with 2.5% (5/200) of deaths directly related to the disease. A total of 75 patients have been followed for more than 5 years and 8 of them (11%) relapsed following 4 to 6 years of remission.
We observed for a two years period 157 hemophiliacs (138 with hemophilia A whose 13 were severe and 19 with hemophilia B whose 13 were severe) and we studied the incidence of liver dysfunction and the role played by HB and non-A, non-B, viruses. Whereas 32 patients not related had no evidence of serological HB virus markers (by radioimmunoassay), 88 (70,4 %) among the 135 hemophiliacs with large or small exposure to blood products were "HB positive". 90,9 % were positive for anti-HBs and anti-HBc antibodies and only two patients had persistent antigenemia. These results appeared independent of the kind of treatment (factor VIII or factor IX concentrates). Six among 17 children born since 1974, when the antigen was detected by RIA, had the serological HB virus markers, showing that this method is not sufficient to completely eliminate the HB virus. However the amount of viruses injected is too small to induce an acute hepatitis and rather produces specific antibodies which protect hemophiliacs against reinfection. An elevated level of serum transaminases (SGPT) was observed in 9,4 % of non treated hemophiliacs, 15,1 % of treated hemophiliacs with no serological markers of HB virus and 27,7 % of treated hemophiliacs "HB positive". This shows that the use of concentrates and the occurring of HB virus in the patients are not the only factors producing liver dysfunction. The role of non-A, non-B viruses has been recognized in 7 patients out of 9 with transient elevation of serum transaminase levels, by Trepo with an immunodiffusion technique.
This report presents further progress on the characterization of blood group activities associated with F VIII/vWf. Evidence is provided that a relationship exists between the nature of the soluble blood group substance of plasma and of F VIII/vWf isolated from this plasma. A mixture of F VIII/vWf from O blood group plasma with A and B substances leads to a purified F VIII/vWf with AB blood group activity. Furthermore, chemical analysis of glycans released from F VIII/vWf by alkaline-borohydride treatment or hydrazinolysis shows the absence of N-acetylgalactosamine residues in non-reducing terminal positions. These results suggest that the blood group activity of F VIII/vWf preparations may be related to minor contamination of this molecule by glycolipidic or glycoproteinic plasma components with A or B oligosaccharide structures.
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Genetic factors play probably an important part in the development of schizophrenic psychoses. As a consequence the use of a genetic marker as the HLA system appears to be interesting in determining the disease susceptibility gene of these psychoses. Methods and results of an investigation about the frequencies of 33 HLA alleles observed in 51 patients considered as paranoid schizophrenics are presented. The frequency of HLA-A 29 was diminished while the one of HLA-B 15 was increased but the differences were no longer significant when p was corrected. However when all the results published from 1974 to 1980 were pooled in a combined statistical analysis, some associations became significant. It seems that schizophrenia as a whole, once paranoid and hebephrenic sub-types have to be distinguished. It may be concluded from these data that correlations between schizophrenia and HLA antigens which remain doubtful could be explained with a biological genetic heterogeneity of schizophrenic disorders. Review of literature concerning the identification of HLA haplotypes in schizophrenics pedigrees and about the HLA system as a genetic marker for the clinical response to neuroleptics in schizophrenic patients or in vitro, is also discussed.
Childhood psychoses could have a genetic etiology, but early and late onset psychoses have to be separated. Genetic factors are certainly implicated in the development of late onset psychoses and appear to overlap with those of adult schizophrenia. Evidence implicating genetics in early onset psychoses is likely, as twin studies have supported this hypothesis. HLA system is a particularly suitable genetic marker. We report the results of an investigation on HLA A and B antigens we have performed on 57 psychotics (30 early onset psychotics, 27 late onset psychotics). No genetic association appears in the whole population, as well as in the early and late onset psychosis series. These results have to be compared with Golse's et al. (1977, 1980), Stebbs and Magenis' (1980) and adult schizophrenia data. Further researches with population and family pedigree are required with other major histocompatibility complex markers.
Acquired von Willebrand's syndrome with a regressive evolution is described in a 66 year old man with Waldenström's disease. An inhibitor electively directed against Ristocetin cofactor activity has been demonstrated, active in vitro after incubation at 37 degrees C. Serum fractionation showed that the inhibitor was independent of the monoclonal IgM and subsequent purification that it was IgG in nature. The results permit its classification as an auto-antibody.
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A Bradley's modified procedure is described herein. It allows the diagnosis of acute viral hepatitis A by evidence of IgM anti HAV: after IgG anti-HAV have been preferentially absorbed with Staphylococcus aureus cells positive for protein A, we test for residual IgM anti-HAV in the supernatant (absorbed serum) by radioimmunoassay. We have documented the ability of this procedure to differentially detect IgM or IgG anti-HAV by analyzing 308 sera. This simple method clearly distinguishes between recent (28 cases) and past HAV infection which is very frequent in France: 80% in adults.
We report here a sandwich immunoenzymatic assay to detect and determine the amount of antibodies against tetanus toxoid. The procedure used for coating the polystyrene microplates allows the measurement of antibodies in undiluted serum, in one hour. 1100 sera and 80 plasmas were tested. These results show the reproductibility of this simple and automated method. An objective quantification and a rapid screening of sera containing 4 IU/ml or more of antitoxin by direct observation or by measuring the optical density are possible.
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