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Biomedical subjects

J Goudemand

Publications and source records attributed to J Goudemand.

At least 91 records · Page 5Linked to original sources
PubMed1988

Investigation of a large kindred with type IIB von Willebrand's disease, dominant inheritance and age-dependent thrombocytopenia.

Twenty-six members of four generations of one family in which a man was diagnosed in 1961 as having von Willebrand's disease (vWD) have been studied. Subtype IIB vWD and autosomal dominant inheritance was identified in 19 individuals with bleeding signs varying in severity and frequency. The absence of high molecular weight multimers of plasma von Willebrand factor (vWf) and the heightened interaction of plasma vWf with platelets in the presence of low ristocetin concentrations were consistent in all affected subjects. Nevertheless the degree of these abnormalities was variable without clearcut linkage to the severity of clinical symptoms. Thrombocytopenia was present only in the adult affected family members and the platelet count appeared to be age-dependent. The investigation of this family provides further evidence of the phenotypic variability in the vWf-platelet interactions within this vWD subtype.

Adolescent↗
PubMed1988

Acquired type II von Willebrand's disease: demonstration of a complexed inhibitor of the von Willebrand factor-platelet interaction and response to treatment.

An acquired von Willebrand's disease developed in two patients in association with a monoclonal gammopathy plus a Sjögren's syndrome and a chronic lymphocytic leukaemia (CLL). In both cases a plasma inhibitor to von Willebrand factor (vWf) was suspected and characterized after plasma gel filtration. The inhibitor was shown to be entirely complexed with vWf and was only demonstrated after complex dissociation by heating. The inhibitor was able to inhibit the binding of 125I-vWf to platelets in the presence of ristocetin in both cases and to thrombin-stimulated platelets in one case. In the two patients, the highest molecular weight multimers (HMWM) of vWf were absent when assessed by sodium dodecyl-sulphate agarose plasma electrophoresis. Intravenous infusion of 1-deamino-(8-D-arginine) vasopressin (DDAVP) resulted in the appearance of the HMWM in both cases and of the satellite bands of each multimer subunit which were lacking prior to the infusion in one patient. After transfusion of a VIII/vWf concentrate containing a significant amount of HMWM, there was a rapid plasma clearance of the vWf-related activities and of the HMWM when compared to that seen in a patient with type III constitutional vWD. We conclude that in the two patients studied the coagulation defect was related to the presence of a circulating inhibitor to vWf which could be responsible for the disappearance of the HMWM from plasma.

Aged↗
PubMed1988

Lupus anticoagulant: a clinical and laboratory study of 100 cases.

The clinical and laboratory features of 100 patients with lupus anticoagulant (LA) are reviewed. Subjects were divided into three groups according to their age (1-5, 15-35, 45-89 years). Female prevalence was observed in each group and overall F/M ratio was 3/1. An underlying autoimmune disease (principally lupus erythematosus) was found in 47 cases (10% of the children, 80% of the 15-35-year-old patients and 37% of the elderly patients). Biological criteria for the LA diagnosis were prolonged activated partial thromboplastin time and diluted thromboplastin time (1.3 x control), not corrected after addition of control to patient's plasma. Thromboplastin time was normal in 77 patients. Other types of coagulation inhibitors were eliminated by specific factor assays (with a 10-fold increase of cephalin concentration when necessary). Twenty-three thrombotic episodes were observed. No significant difference was found in the incidence of thrombosis between the autoimmune and non-autoimmune disease group, but the age when first thrombosis occurred was clearly lower in the former. Fourteen obstetrical accidents were noted in eight women but 13 pregnancies terminated without accident. Four patients experienced haemorrhagic complications; they all presented with a severe thrombocytopenia associated with the LA. In our experience, LA is a frequent coagulation abnormality, associated in about half of the cases with a clearly defined autoimmune disease. Clinical presentation appears as notably different according to the patient's age; it is particularly noteworthy that in nine out of 10 children, LA disappeared spontaneously within 6 months.

Adolescent↗
PubMed1986

[Inhibitors of factor VIII in non-hemophilic patients. Biological and therapeutic aspects. Apropos of 3 cases].

A coagulation inhibitor of the anti-factor VIII: C type was detected in three non-haemophilic male patients aged 75, 70 and 52 respectively. In all three patients antibody titres were low (less than 12.5 Bethesda units initially, less than 20 units subsequently), and a low but detectable level of factor VIII: C persisted (7 to 12 p. 100 in two patients who had severe haemorrhages and 2.100 in the third one). The 3 inhibitors inactivated factor VIII: C with a complex, type II kinetics (Biggs et al.). Strong doses of anti-haemophilic A fractions were biologically effective in one patient but could not stop severe bleeding. Activated plasma fractions were used successfully on several occasions. Once, moderate and repeated doses of anti-haemophilic A fractions resulted in satisfactory correction of factor VIII: C level, and a minor surgical operation could be performed. An immunosuppressive treatment was administered for 3 weeks to one patient and for 3 months to the other two patients. In all three cases the inhibitor disappeared after 5 to 8 months. In non-haemophilic patients with factor VII: C inhibitor the treatment of haemorrhagic episodes must take into account the severity of bleeding, then the usually complex kinetics of the inhibitor; thus it cannot be a direct copy of the treatment used in haemophiliacs with type I inhibitors.

Aged↗
PubMed1985

Effect of carbohydrate modifications of factor VIII/von Willebrand factor on binding to platelets.

This study compares the ability of unmodified and carbohydrate-modified forms of factor VIII/von Willebrand factor (FVIII/vWF) protein to bind to platelets in the presence of ristocetin or thrombin. Treatment of intact FVIII/vWF with alpha-D-neuraminidase results in more than 95% desialylation. Asialo FVIII/vWF retains total activity in ristocetin- and thrombin-mediated binding to platelets as demonstrated by direct and competitive binding assays. Examination of its multimeric pattern by sodium dodecyl sulfate-agarose electrophoresis reveals a normal multimeric structure. Treatment of intact FVIII/vWF with beta-D-galactosidase results in the removal of 20% of galactose (agalacto FVIII/vWF) whereas 55% of galactose is released from asialo FVIII/vWF (asialo agalacto FVIII/vWF). Agalacto and asialo-agalacto FVIII/vWF are both unable to bind to platelets in the presence of ristocetin. In contrast, they still bind to thrombin-stimulated human (except thrombasthenic) platelets. Removal of either ultimate (agalacto FVIII/vWF) or ultimate and penultimate (asialo-agalacto FVIII/vWF) galactose results in the same loss of the larger molecular weight multimers and in an increase of smaller multimers. These results suggest (1) that sialic acid does not play a significant role in ristocetin- or thrombin-mediated FVIII/vWF-platelets interactions and multimeric structure of FVIII/vWF (2) that ultimate beta-linked galactose residues are essential for the maintenance of a normal multimer organization (3) that ristocetin- and thrombin-mediated binding of FVIII/vWF to platelets differ in FVIII/vWF galactose requirement.

Aspergillus niger↗
PubMed1985

Comparison between the effect of pentosan polysulphate heparin and antithrombin III injections in antithrombin III deficient patients.

Pentosan polysulphate is an heparin analogue which acts via an antithrombin III (AT III) independent pathway. We compared the effect of this drug to that of heparin and AT III infusions in AT III deficient patients. Four patients with AT III congenital deficiency received on three different occasions: (i) an infusion of human AT III concentrate (20 U/kg or 40 U/kg), (ii) an intramuscular injection of pentosan polysulphate (2 mg/kg), (iii) a subcutaneous calcium heparin injection (100 U/kg). AT III infusion inhibits the excessive thrombin generation (46% of inhibition) observed in the plasma of AT III deficient patients during at least 12 hours, but does not modify the factor Xa formation. On the contrary, pentosan polysulphate has a marked effect on both thrombin (62% of inhibition) and factor Xa generation (57% of inhibition) still present 8 hours after injection. Heparin injection has the same effect, more prolonged, as pentosan polysulphate on thrombin generation but is not so effective on impairing factor Xa generation (27% of inhibition). The marked effect of pentosan polysulphate on thrombin and factor Xa generation in these patients is due to its AT III independent mechanism of action.

Antithrombin III↗
PubMed1984

[Idiopathic thrombopenic purpura. Diagnostic and prognostic value of Dixon's test].

The levels of immunoglobulin G (PIgG) bound to platelet membrane can be measured by complement lysis inhibition test (Dixon's assay). In normal subjects, this level is usually below 15 X 10(-15)g = IgG/platelet. Similar values have been obtained with different techniques. PIgG values found on normal platelets might be related to clearance of the oldest platelets. PIgG levels are greatly increased in 85 - 100% of patients with idiopathic thrombocytopenic purpura. Purpura patients with normal PIgG levels might have only C3, IgM or IgA bound to their platelets. The Dixon's assay is not specific to idiopathic thrombocytopenic purpura, since it is positive in other types of thrombocytopenia. PIgG's may be related to platelet size, serum hypergammaglobulinaemia or circulating immune complexes. In the case of idiopathic thrombocytopenic purpura, they probably represent antiplatelet antibodies. The results of Dixon's assay in idiopathic thrombocytopenic purpura must be interpreted with caution. They may offer new possible explanations for the thrombocytopenia associated with other diseases.

Antigen-Antibody Complex↗
PubMed1983

[Behçet's disease and vascular malformations: double etiology of recurrent thrombophlebitis].

Repeated thrombophlebitis with pulmonary embolism in a 28-year-old female led to the discovery of a complex malformation of the inferior vena cava which required the insertion of a clip. Three years later, recurrence of phlebitis with appearance of bipolar aphthae, arthritis and a meningeal syndrome led to the diagnosis of Behçet disease. Despite various medications for this disorder and heparin or oral anticoagulant therapy, the thromboembolic disease remained active, accompany with new exacerbations of Behçet disease. Diminished plasma fibrinolytic activity was demonstrated, probably resulting from venous endothelial vascularitis, which aggravates the consequences of the preexisting vascular malformation.

Adult↗
PubMed1983

[Circulating anticoagulant, recurrent abortions and venous thrombosis: a new entity or a pre-lupus syndrome? 2 cases].

The authors report the presence of a circulating anticoagulant in two patients who had suffered multiple abortions. The circulating anticoagulant was discovered during evaluation of repeated phlebothromboses in the first case and of a chronic inflammatory syndrome in the second. While the apparently paradoxical relationship between the circulating anticoagulant and venous thrombosis is relatively well-known, that between the circulating anticoagulant and repetitive abortions is more difficult to establish. The rarely reported association of these three events leads us to a discussion of the nosologic place of this pathology and of the common ground it shares with S. L. E., as the circulating anticoagulant is of the antiprothrombinase variety, the antibody commonly called the lupus anticoagulant.

Abortion, Habitual↗
PubMed1982

[Acute arsenious hydride intoxication. Four cases (author's transl)].

While engaged in the repair of a zinc furnace, 4 workers were accidentally exposed to arsenious hydride (AsH3) fumes. Acute intravascular haemolysis developed within a few hours. On admission, the patients immediately underwent exsanguino-transfusion; 8.2 to 10.2 l of blood were exchanged through a continuous perfusion pump at the rate of 1 l/hour. Two patients resumed diuresis during transfusion, but the other two required repeated haemodialysis. Between the 10th and 30th days, while renal function was gradually returning to normal, mildly megaloblastic anaemia developed. This was followed during the 3rd month by clinical and electric signs of polyneuritis of the lower and upper limbs, which subsequently regressed. Regular measurements of arsenic levels in the blood and urine were performed between and during exsanguino-transfusion and haemodialysis.

Acute Disease↗
PubMed1982

[Treatment of idiopathic thrombopenic purpura. Results obtained in a series of 219 cases].

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.

Adolescent↗
PubMed1981

Red cell filterability and chronic renal failure.

Filterability has been studied during chronic renal failure (with creatinin level above 350 mumol/l); red cells were washed thrice with 9 g/l NaCl or 40 g/l human serum albumin), adjusted to give a PCV of 0.3 and filtered on polycarbonate sieves with pore sizes of 5 micrometer. In the uremic patients groups, the results are heterogeneous and show a significant reduction of the red cell filterability. The filtration time is not well correlated with the degree of uraemia but with the haemoglobin level.

Erythrocyte Membrane↗
PubMed1981

[Schizophrenic psychoses and HLA antigens. Personal data and review of the literature].

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.

Adult↗