Inherited defective platelet aggregation with arachidonate as the main expression of a defective metabolism of arachidonic acid.
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Biomedical subjects
Publications and source records attributed to D Nyman.
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20 coagulation parameters were investigated in 144 patients with different liver diseases. The groups of acute hepatitis, chronic active hepatitis and liver cirrhosis were compared and the prognostic value of the coagulation analyses investigated. It is clear that the determination of the factor V activity is a good and easy test for detection of actual liver function. Repeated controls over several weeks revealed with a statistical significance (p less than 0.0005) that all patients with a factor XIII below 35% and a plasminogen below 19% will die in liver coma, if they have not died beforehand from acute gastrointestinal haemorrhage, acute infection or cardiac arrest. Plasminogen is also lower in the group of non-survivors but the values of the two groups are overlapping and of no prognostic help in a single case. The possible causes of the diminution of factor XIII activity are discussed.
It has been demonstrated that activated factor XIII may catalyze the formation of covalent cross-links between fibrin and collagen. This is shown by the disappearance of the gamma-gamma dimer band in PAA-SDS gel electrophoresis when fibrinogen is clotted in presence of collagen, factor XIII and Ca ions, and by the binding of labeled fibrinogen. This reaction may explain the outstanding physiological importance of factor XIII.
The snake venom enzymes Ancrod and Batroxobin marajoensis are able to activate human plasma factor XIII as shown by the formation of the gamma-dimers. The concentration of gamma-dimers increases with the concentration of the activating enzymes. Factor XIII activated by Ancrod or Batroxobin marajoensis is, however, unable to catalyse the incorporation of the amine dansyl-cadaverine into casein. The partially activated factor XIII is therefore not demonstrable by means of the artificial test system. This factor XIII loses little activity and remains activable by thrombin.
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A woman of 40 had cyclical thrombocytopenia occurring midway between menstruations, with bruising and platelet counts down to 2000/mm3. The thrombocytopenic episodes disappeared after treatment with lynestrenol was started. The patient's platelets had a functional defect of the "aspirin-like" type. The availability of platelet factor 3 increased when oestrogen was added to platelet-rich plasma from the patient.
Coagulation disorders in hemorrhagic shock need not represent an isolated intravascular coagulation. They may also occur as a complex of local disseminated intravascular consumption, extravascular consumption, dilution, and reduced synthesis of coagulation factors. In the severely bleeding patient with hemorrhagic diathesis heparin is contraindicated because it does not normalize coagulability. Therefore, it fails to stop hemorrhage and shock remains untreatable. Fresh frozen plasma, however, has proved to be suitable as simultaneous substitution therapy of coagulopathy and of hypovolemic shock. 11 patients suffering from traumatic-hemorrhagic shock associated with intravascular coagulation and hemorrhagic diathesis were successfully treated with fresh frozen plasma, after conventional shock therapy had failed over a period of hours.
Coagulation disorders in traumatic-haemorrhagic shock need not represent a simple coagulation problem. They may also occur as a complex of local and disseminated intravascular consumption, dilution, extravascular loss and depressed synthesis of coagulation factors. In the severely bleeding patient with a haemorrhagic diathesis heparin is contrainedicated because it does not normalize coagulability immediately. Therefore, it fails to stop haemorrhage and the shock becomes untreatable. Fresh frozen plasma, however, has proved to be suitable as a simultaneous substitution therapy for the coagulation disorder and the hypovolaemic shock. 25 patients suffering from severe traumatic-hemorrhagic shock associated with coagulation disorders and haemorrhagic diathesis were successfully treated with fresh frozen plasma, after conventional shock therapy had failed over a period of 2 hours. The success was documented clinically and by numerous laboratory tests. Thrombocytopenia has only a secondary responsibility for the haemorrhagic state.
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Brinase was administered intra-arterially in 16 patients with thrombotic or embolic arterial occlusions. Angiography could be performed before and after treatment in 13 patients. Thrombolysis was obtained in 3 of 9 patients with thrombotic and in 3 of 4 patients with embolic occlusions. In 3 patients severe local side effects occurred.
Factor IX concentrates are of paramount importance in the treatment of hemophilia B. Growing reports of thromboembolic complications and of disseminated intravascular coagulation, coupled with the danger of hepatitis transmission, suggest that the concentrates should be primarily reserved for the treatment of hemophilia B. Concise guidelines for treatment are presented.
In 15 patients on long-term oral anticoagulation the new antirheumatic drug tolmetin (Tolectin) was administered over a period of 10 days. Based on 11 parameters of coagulation, no interaction between the drug and phenprocoumon was found. A significant though clinically irrelevant prolongation of bleeding time was observed.
Using one-stage quantitative factor VIII assay and the quantitative immunoelectrophoretic method of LAURELL a good correlation has been found between factor VII activity and protein concentration. In 80% of the known hemophilia A carriers the protein concentration was about twice as high as in normals as compared to the factor VIII activity, whereas about 20% of the known carriers have the same ratio between factor VIII protein and factor VIII activity as normals. The reliability of the test and its usefulness for the detection of hemophilia A carriers are discussed.
Brinase infusions in man cause, without excessive lowering of the inhibitor capacity, a slowing of the fibrin polymerization. This is combined with a degradation of the A alpha chain into two major fragments. Both fragments carry crosslinking sites. Brinase infusion also causes positive ethanol gelation. Evidence for the formation of gamma dimers was found.
Two methods for the assay of brinase inhibitors are compared. Neither provided a reliable means of brinase dose prediction. Overdosage can, however, be avoided by preinfusion inhibitor assay.
From September 1962 to May 1972 145 patients with acute or subacute deep vein thrombosis confirmed by phlebography were treated with streptokinase. During the same period 42 patients considered unfit for thrombolytic therapy were treated with herapin and oral anticoagulants. The results, assessed by repeat phlebography, in 93 of the patients treated with streptokinase were compared with those in 42 patients treated with heparin. The age, sex, and severity of occlusion were roughly similar in both groups. Streptokinase treatment was successful in 42 per cent, partially successful in 25 per cent, and unsuccessful in 32 per cent of the 93 patients compared with none, 10 per cent, and 88 percent respectively in the 42 patients treated with heparin. Streptokinase was more effective when the thrombus was in proximal rather than calf veins. Thrombi of more than six days old were readily lysed. Plasma fibrinogen levels were below 0-8 g/1 (80 mg/100 ml) in nearly all patients successfully treated. The incidence of pulmonary embolism was no greater with streptokinase than with heparin treatment. Only prolonged follow-up would show whether thrombolytic treatment would be effective in preventing late complications of deep vein thrombosis such as chronic venous insufficiency.
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