[Short- and long-term benefits of smoking cessation]. / Bénéfices à court et à long termes de l'arrêt de la consommation de tabac.

The beneficial effects of withdrawal smoking on the disrupted physiological functions bound to tobacco spread differently: some weeks for smell, taste and breathing, to the less six months to recover tolerance to the effort without dyspnea. For the cardiac attack the risk bound to tobacco disappears after five years, and the risk for cancers after ten years. The expectation of life of the ex-smoker joined the one of non-smokers 15 years after the stop of smoking. The tobacco use by the women raise some particular problems. To convince the smoker to persevere in the decision of withdrawal smoking and to be free of dependences (pharmacological and psychological) it could be necessary to use the nicotinic substitutes and/or a psychological support. Stop smoking is the better and the most economic therapeutic to avoid recurrence of cardiac attack for a lot of authors.

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.

[Influence of protein S deficiency on the arterial thrombosis risk]. / Influence du déficit constitutionnel en protéine S sur le risque thrombotique artériel.

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.

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.

[Recurrent thromboembolism disclosing protein C deficiency. Apropos of a case with familial investigation]. / Maladie thrombo-embolique récidivante révélant un déficit en protéine C. A propos d'une observation avec enquête familiale.

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.

[Constitutional abnormalities of hemostasis: diagnostic strategy]. / Anomalies constitutionnelles de l'hémostase: stratégie du diagnostic.

The most common clinical and biological expressions of constitutional abnormalities of haemostasis on which rests the diagnostic approach are influenced by the level where deficiency occurs as well as by the degree of this deficiency and its mode of transmission. Four situations encountered in practice are described: a severe form with loud and early clinical expression, a moderate form with subdued clinical expression but potential risk of haemorrhage, a genetic enquiry and the discovery of a biological abnormality. Concerning the first two situations, the stress is placed on their clinical signs, since peculiarities in a routine laboratory examination lead to a more appropriate exploration performed by a specialized laboratory.

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.

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.

Thin-layer agarose isoelectric focusing of alkaline phosphatase isoenzymes from human neutrophils.

An improved method for thin-layer agarose isoelectric focusing of alkaline phosphatases (AP) from human neutrophils is described. The solubilization of AP isoenzymes was studied with four detergents. The best results were obtained after sonication with Zwittergent 3-12 (1% final concentration), followed by butanol extraction and ultracentrifugation 105,000 x g for 1 h. The cytosol can be stored at 0 degrees C or -80 degrees C, but not at -20 degrees C. Dialysis of the cytosol against a Tris buffer, pH 7.5, was imperative prior to focusing for removal of the detergent. Enzyme visualization is enhanced by incorporation of ZnCl2 (3 mM) into the agarose gel. The focusing patterns consist of two sets of isoenzymes: two main zones with pI 6.4 and 6.8 and minor components with pI 4.2, 4.8 and 5.2.

IgG/placental alkaline phosphatase complexes in Rh-incompatible pregnancy with fetal hydrops.

In 11 cases with anti-Rh-complicated pregnancies, i.e. ascites at 21/25 weeks and fetal hydrops at delivery, we detected the presence of IgG/placental alkaline phosphatase complexes in the maternal serum.

[Thrombosis of the superior vena cava disclosing Behçet's disease]. / Thrombose cave supérieure révélatrice d'une maladie de Behçet.

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.

[Evaluation of the performance of an automatic coagulation tester: the Electra 700]. / Evaluation des performances d'un automate en coagulation: l'Electra 700.

The Electra 700, an automatic coagulation tester recently available in France, was investigated for its performance on routine tests: Quick's test, APTT, thrombin clotting time test, assay of fibrinogen and prothrombin complex factors. Reproducibility was exceptionally good for Quick's test (CV less than 1%), excellent for the other tests (CV less than 3%), acceptability of the various commercial reagents very good, and standard linearity excellent. There was close correlation with manually obtained (electromagnetic B.M.) or semiautomated (KC 10) results (0.9948 to 0.9345). The apparatus is entirely automatic once specimens have been loaded, and it is particularly adapted to dealing with long series of specimens (60 samples). Its original loading system makes it possible to carry out two types of test concurrently, as well as to insert emergency tests. Economically, the cost of disposables and "reagent loss" is satisfactory; however, all tests are carried out relatively slowly, though this is compensated for by the fact that the reactions are completely automatic.

[Monitoring anticoagulant treatment with the KEM-O-MAT 2HP autoanalyzer]. / Surveillance des traitements anticoagulants par automate KEM-O-MAT 2 HP.

Synthetic chromogenic substrates were adapted to KEM-O-MAT 2HP for an automated monitoring of anticoagulant treatments, determination of factor X (S 2337) in oral anticoagulation, anti-Xa (Hepachrom) and anti-lla (S 2238) activities of heparin in prophylactic and curative heparin therapies. In all cases, these methods revealed high sensitivity, reproducibility, and a good correlation of their results with those of classical clotting assays. The present cost of synthetic substrates seriously limits the potential value of chromogenic assays for routine controls. However, the development of automated amidolytic techniques should provide an easier standardization in the laboratory control of anticoagulant treatments.

[Evaluation of Owren's thrombotest in the monitoring of treatment with antivitamin K]. / Evaluation du thrombotest d'Owren dans la surveillance des traitements par antivitamine K.

The need to standardize the monitoring of oral anticoagulant treatments has led some countries to adopt, generally, Owren's Thrombotest. Apart from needing only one reagent and being perfectly adapted to this type of monitoring, the thrombotest has the advantage of being able to be used on capillary blood and venous blood and of not being influenced by the storage of the blood samples. Its disadvantages are that it is slow to perform, difficult to automate and that it has a therapeutic range which is narrow and close to the limits of precision of the technique. The results of the Thrombotest correlate well with those of the Quick test, the activated cephalin time and the X level measured by the amidolytic technique. Until the chromogenic assays are developed, the Owren's Thrombotest provides a better homogeneity and standardization of the results coming from different laboratories.

[Importance of synoviorthesis using osmic acid in the treatment of hemophilic arthropathy. Apropos of 19 cases]. / Intérêt de la synoviorthèse à l'acide osmique dans le traitement de l'arthropathie hémophilique.

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.