Search PubMed⌕ Search

Biomedical subjects

R Bierme

Publications and source records attributed to R Bierme.

At least 19 recordsLinked to original sources
PubMed1998

Erythrocyte hyperaggregation and thrombogenic dysfibrinogenemia.

Erythrocyte aggregation was measured in 12 patients with congenital dysfibrinogenemia. The results showed hyperaggregation in four patients who had presented a thrombotic disorder, while aggregation was entirely normal in patients with asymptomatic dysfibrinogenemia. None of the four symptomatic patients had any other anomaly of hemostasis, in particular no coagulation inhibitor deficit or anti-phospholipid antibodies. The possible involvement of erythrocyte hyperaggregation in the thrombotic process is discussed.

Adult↗
PubMed1991

[Influence of protein S deficiency on the arterial thrombosis risk].

Protein S is the cofactor of activated protein C which, together with the antithrombin system, is a major regulator of coagulation. Congenital protein S deficiency is an important risk factor for venous thrombosis. In this study of 105 patients with protein S deficiency, 64 had one of several thromboembolic accidents, including 14 arterial thrombotic accidents involving the central nervous system or the myocardium. These accidents occurred in young subjects (mean age: 25 +/- 13 years) only one-third of whom had another risk factor. This high frequency of arterial thrombosis associated with protein S deficiency calls for a prospective study in young subject with cerebral vascular accident or myocardial infarction.

Adolescent↗
PubMed1991

Difference in activity properties and subcellular distribution of neutrophil alkaline phosphatase between normal individuals and patients with trisomy 21.

Biochemical, cytochemical characteristics and electron microscopy subcellular distribution of neutrophil alkaline phosphatase (NAP) were analysed in blood and/or smear samples from 39 trisomy 21 patients (Down's syndrome) aged 11.5-18 years (mean 15.5 years) and 55 normal subjects aged 12-20.5 years (mean 17 years). All patients were karyotyped. NAP cytochemical procedures were carried out on all subjects; biochemical NAP determinations were made in 10 patients and 20 controls; ultrastructural electron microscopy of AP was performed in three patients and four normal subjects. Neutrophil alkaline phosphatase from patients with trisomy 21 displayed the following changes: (1) a significant increase of enzyme activity, (2) a high thermal lability of enzyme. Electron microscope morphology exhibited large deposits of NAP reaction product associated with the plasma membrane and intracellular main organelles, like phosphasomes. The NAP biochemical and cytochemical characteristics suggest that trisomy 21 neutrophils contain a non-specific AP isoenzyme, closely related to the early placental form.

Adolescent↗
PubMed1990

[Recurrent thromboembolism disclosing protein C deficiency. Apropos of a case with familial investigation].

Every thromboembolic manifestation, especially in young subjects, calls for an aetiological study in which haemostasis is evaluated primarily with assays of physiological coagulation inhibitors: protein C, protein S and antithrombin III. Protein C deficiency is found in 6 to 7% of thromboembolic manifestations. We report the case of a 21-year old man who had phlebitis followed by pulmonary embolism without facilitating factors. Protein C level was 50% of normal value (0.50 IU/ml). The patient received heparin, subsequently replaced by oral anticoagulants after a long period of overlap between the two treatments. The outcome was favourable. Family investigation yielded a history of thromboembolic accidents in several members of the family, some of whom were protein C deficient (50% of normal value). Protein C synthesis is vitamin K-dependent. Protein C deficiency is transmitted as an autosomal dominant trait. Normal values range from 0.65 to 1.35 IU/ml. Clinically, 25% of the patients are said to be asymptomatic. The first thrombotic accidents occur in young subjects (mean age 29 +/- 14 years). Several points emerge from this case: full evaluation must be performed, especially in young subjects; family investigation consolidates the diagnosis and enables symptomatic protein C deficient patients to be treated and thrombotic manifestations to be prevented by effective anticoagulant therapy in high-risk situations; a prolonged period of heparin-oral anticoagulant overlap is needed to avoid cutaneous necrosis.

Adult↗
PubMed1989

New antibody in severe rhesus incompatible pregnancies: IgG-kappa antiplacental alkaline phosphatase.

As already found in other various diseases, a macromolecular alkaline phosphatase complex (HMW-AP) was also found in sera of two severe Rhesus-incompatible pregnancies complicated by ascites and fetal hydrops at delivery. This atypical complex was detected and isolated by agarose gel electrophoresis. Immunoelectrophoresis and heat inactivation of this HMW-AP complex revealed that it consisted of IgG of the kappa type and placental AP isoenzyme. The transitory presence of this immuncomplex is discussed. However, in all women with Rh-immunized complicated pregnancies, significant variations of neutrophil and serum AP activities were observed. A fall in AP activity and the presence of an antiplacental AP antibody in serum of women with complicated Rh immunization should be of value in assessing the prognosis of the disease.

Adult↗
PubMed1988

Isoelectric focusing of human neutrophil alkaline phosphatase isoenzymes in agarose gel.

An isoelectric focusing technique for human neutrophil alkaline phosphatase isoenzymes is described. After sonication with Zwittergent 3-12, butanol extraction and ultracentrifugation, dialysis of cytosols precedes focusing. Focusing patterns show a heterogeneity with two enzymatic activity areas: a main component at pI 6.7-6.8 with a minor component at pI 4.8-5.0 which is difficult to visualize due to its sensitivity to experimental conditions. The addition of 3 mmol/l ZnCl2 to the agarose gel improved the staining of focused bands and in particular the anodic component.

Alkaline Phosphatase↗
PubMed1986

[Thrombosis of the superior vena cava disclosing Behçet's disease].

The authors report the case of a 29 year old North African patient with Behçet's disease presenting with sudden thrombosis of the superior vena cava. Venous disorders are the fourth major sign of this disease. Although superficial thrombophlebitis is a common presenting sign, caval thrombosis is rare and usually occurs after several years' evolution. Superior vena caval thrombosis may be life threatening due to complications such as pulmonary embolism and haemoptysis. The anatomical substrate of this form of vascular disease is the same as that of the other visceral lesions of Behçet's disease: predominantly venous vasculitis with perivascularitis and secondary thrombosis. The pathogenesis of this thrombotic diathesis is discussed.

Adult↗
PubMed1982

[Importance of synoviorthesis using osmic acid in the treatment of hemophilic arthropathy. Apropos of 19 cases].

While getting synovial sclerosis and articular drying, the synoviorthesis breaks the circulus vicious which comes from iterative hemarthrosis to articular destruction. With 25 cases and a follow up of 6 years, we have got 39% of good results, 35% of medium and 26% of bad ones. The therapeutic efficiency requires a certain latent time (0 to 15 days for isotopical synoviorthesis, 2 to 3 months for those ones with osmical acid.) A new synoviorthesis can be made if needed. Neither age nor radiological stage are of any influence. The radiological worsening uses to go on unchanged. The synoviorthesis should be performed at an early stage, previous to the cartilage and bone destructions.

Adolescent↗
PubMed1981

Factor VIII complex in normal pregnancy, pre-eclampsia and fetal growth retardation.

The levels of the three components of factor VIII complex (VIII R:AG, VIII R:WF, VIII:C) were measured during normal late pregnancy, in pre-eclampsia and in pregnancies complicated by fetal growth retardation. In late normal pregnancy, there was a steady increase in factor VIII complex and the highest level was reached at delivery; in primary fetal growth retardation, the values were similar. In pregnancies complicated by pre-eclampsia, with or without fetal growth retardation, there was a significant increase of VIII R:AG VIII R:WF; the more severe the course of the disease, the greater the increase. The level of VIII R:AG-VIII R:WF appears to be an indicator of vascular pathology and it is suggested that this assessment may be of value in monitoring high risk pregnancies.

Factor VIII↗
PubMed1980

[Limits of platelet aggregation tests for investigating thromboses (author's transl)].

Three methods--spontaneous aggregation, ADP-induced aggregation and levels of circulating platelet aggregates--were used to test for platelet hyperaggregation in 87 adult patients divided into three groups. Group A comprised 26 patients with severe arterial diseases, including cerebral vascular accidents (16) and peripheral thrombosis (10) ; group B consisted of 31 patients with venous conditions, including deep phlebitis (22) and recurrent thrombo-embolic disease (9) ; group C, which served as control, comprised 30 patients with various non-vascular disorders. Spontaneous aggregation and enhanced reactivity to ADP correlated well with each other and were more frequent in patients with vascular diseases. However, one-third of patients in group C had pathological results, while one-third of patients in groups A and B had normal results, independently of any clinical particularity or therapeutic regimen. There were no significant differences between the three groups with regard to circulating platelet aggregates. These tests, therefore, appear to be of statistical and epidemiological interest, but of questionable practical value.

Adenosine Diphosphate↗
PubMed1980

Platelet production time, uricemia, and some hemostasis tests in pre-eclampsia.

In order to choose the best adapted test for pre-eclampsia monitoring, platelet production time (PPT) was measured simultaneously with uricemia, factor VIII complex, beta-thrombogobulin, and other tests of platelet activation including platelet volume, density and platelet very dense body content. In the pre-eclamptic group (11 patients). In the PPT was significantly reduced in comparison with normal pregnancies (6 patients). In the pre-eclamptic group, there was good and significant correlation between PPT and the VIIIrAg/VIIIc ration (r = 0.87) and between PPT and uricemia (r = 0.79). The correlations between PPT and the other tests are poor and non-significant. Thus, for clinical purposes, the VIIIrAg/VIIIc ratio and uricemia are convenient parameters, and give very reliable information on the severity of the consumption coagulopathy which characterizes pre-eclamptic pregnancies.

Adult↗