ISSN:1424-8832    

DOI:10.1159/000215149

出版商:S. Karger AG    

年代:1985

数据来源: Karger

ISSN:1424-8832    

DOI:10.1159/000215150

出版商:S. Karger AG    

年代:1985

数据来源: Karger

ISSN:1424-8832    

DOI:10.1159/000215151

出版商:S. Karger AG    

年代:1985

数据来源: Karger

ISSN:1424-8832    

DOI:10.1159/000215152

出版商:S. Karger AG    

年代:1985

数据来源: Karger

摘要:

The syndrome of the hereditary tendency to venous thrombosis or thrombophilia has been recognized only after the discovery of regulatory mechanisms of the haemostatic system. At present, several distinct defects are known as causes of this syndrome: anti-thrombin III deficiency, protein C deficiency, protein S deficiency, dysfibrinogenaemia and dysplasminogenaemia. It is likely that several additional defects will be found in the near future. Consequently, it is important that a family history be taken in all cases of ‘spontaneous’ venous thrombosis and that laboratory studies be done to identify any underlying defect in the regulation of the blood coagulation system. We present preliminary data, which suggest that the incidence of the thrombophilia syndrome is higher than that of the haemophil

ISSN:1424-8832    

DOI:10.1159/000215153

出版商:S. Karger AG    

年代:1985

数据来源: Karger

摘要:

Hereditary protein C deficiency, which is inherited as an autosomal-dominant trait, predisposes to venous thrombotic disease. Heterozygotes are at risk for superficial thrombophlebitis, deep venous thrombosis and/or pulmonary embolism, which may occur without apparent cause at a young age. Other manifestations are cerebral venous thrombosis and mesenteric vein thrombosis. In severe, often homozygous, protein C deficiency, a purpura fulminans syndrome may occur shortly after birth, resulting in death due to extensive thrombosis, if it is not adequately treated. The thrombotic manifestations in heterzygotes are effectively prevented by coumarin therapy. However, in the initial phase of oral anticoagulant therapy, the patients have an increased risk for the development of coumarin-induced haemorrhagic skin necrosis. The purpura fulminans syndrome can be treated with either replacement therapy or with coumarin therapy. Heparin appears to be ineffective in the prevention of both the purpura fulminans syndrome and the coumarin-induced skin necrosis.

ISSN:1424-8832    

DOI:10.1159/000215154

出版商:S. Karger AG    

年代:1985

数据来源: Karger

摘要:

Protein S is a vitamin K-dependent plasma protein that serves as a cofactor of activated protein C (APC) in its inhibitory action on activated factor V and factor VIII and in its stimulation of fibrinolytic activity. In plasma, part of the protein S is complexed with the C4b-binding protein. Only the free protein S has APC cofactor activity. In our laboratory, 30 patients from 8 nonrelated families were detected that fulfilled the criteria of an isolated protein S deficiency. All patients were heterozygotes for the defect that is inherited as an autosomal-dominant disorder. Patients with a protein S deficiency were found to be at risk for the development of venous thrombotic disease at a relatively young age.

ISSN:1424-8832    

DOI:10.1159/000215155

出版商:S. Karger AG    

年代:1985

数据来源: Karger

摘要:

On a theoretical basis, delayed fibrinolysis has always been assumed to allow a harmless, or rather, useful clotting process to develop into manifest thrombosis. Since the early sixties, when most modern concepts on fibrinolysis were postulated, the evidence for a causal relationship between impaired fibrinolysis and venous thrombosis has not become much stronger. On the other hand, several observations indicate an association between impaired fibrinolysis and arterial thrombosis. Recent years have witnessed a flare-up of interest in fibrinolytic therapy, especially of acute coronary artery thrombosis, and thanks to the availability of human tissue-type plasminogen activator, expansion of its field of application is to be expected. However, with drugs for maintenance enhancement of fibrinolysis still being out of reach, we rely on the well-established oral anticoagulant regimen for the prophylaxis of thrombosis in patients with an obvious thrombotic disposition.

ISSN:1424-8832    

DOI:10.1159/000215156

出版商:S. Karger AG    

年代:1985

数据来源: Karger

摘要:

25 patients with lupus anticoagulant (LA) and a history of thrombosis are described and the cases reported in the literature with this association are reviewed. From the combined data it is concluded that the prevalence of thrombosis in patients with LA is about 30%, the thrombosis sites are the leg veins in about 66%, the cerebral arteries in 25% and the peripheral arteries in 10% of the patients. High anticardiolipin levels are associated with a higher risk, while age of less than 10 years, low prothrombin activity and a platelet count of less than 50,000/μl is associated with a lower risk of thrombosis. Heparin and oral anticoagulants are effective in the treatment and prevention of thrombosis without untoward risk of bleeding

ISSN:1424-8832    

DOI:10.1159/000215157

出版商:S. Karger AG    

年代:1985

数据来源: Karger

摘要:

A short review on the discovery of the relation between vitamin K and the synthesis of clotting factors and on the development of anticoagulant therapy is given. The pro-coagulant activity induced by vitamin K resides in the postribosomal modification of N-terminal glutamic acids in γ-COOH glutamic acids. The mode of action of oral anticoagulants is explained. The concentration-effect relations and the choice of an oral anticoagulant is discussed

ISSN:1424-8832    

DOI:10.1159/000215158

出版商:S. Karger AG    

年代:1985

数据来源: Karger