scholarly journals Coagulation Defects in Βeta-Thalassemia Major Patients at Haji Adam Malik Hospital Medan

2019 ◽  
Vol 8 (2) ◽  
pp. 592
Author(s):  
Eduward Situmorang ◽  
Adi Koesoema Aman ◽  
Bidasari Lubis
1964 ◽  
Vol 11 (02) ◽  
pp. 404-422 ◽  
Author(s):  
Annemarie Amris ◽  
C. J Amris

Summary14 patients (5 diabetics with arteriosclerotic complications, 4 patients with thrombo-embolic disease, 4 with cirrhosis, coagulation defects and increased fibrinolytic activity, and 1 cancer patient) and 3 control patients were subjected to turnover studies with 13iodine labelled human fibrinogen.Half-life times in the control patients were found to be 4 days, the fractional turnover rates 19–23 per cent, of intravascular fibrinogen per day, and the absolute turnover 0.02 to 0.06 gm per day per kg. body weight. The other patient’s half-life times and turnover rates varied considerably from 0.9–5.5 days, 13–160 per cent, per day of intravascular fibrinogen and 0.02–0.4 gm per day per kg. body weight respectively.As fibrinogen unlike other proteins subjected to turnover studies, is converted to fibrin, it is not possible to measure the true intra-extravascular distribution ratio of fibrinogen. But intravascular fibrinogen could be approximated to constitute 68–99 per cent, of the total fibrinogen. There is justification in believing that fibrinogen is degradated through a continuous coagulation in equilibrium with fibrinolysis, and that the organism contains a greater mass of fibrin, the “fibrin pool”. Considerations of the turnover mechanism can however only be hypothetical.


1961 ◽  
Vol 06 (01) ◽  
pp. 015-024 ◽  
Author(s):  
Sven Erik Bergentz ◽  
Oddvar Eiken ◽  
Inga Marie Nilsson

Summary1. Infusions of low molecular weight dextran (Mw = 42 000) to dogs in doses of 1—1.5 g per kg body weight did not produce any significant changes in the coagulation mechanism.2. Infusions of high molecular weight dextran (Mw = 1 000 000) to dogs in doses of 1—1.5 g per kg body weight produced severe defects in the coagulation mechanism, namely prolongation of bleeding time and coagulation time, thrombocytopenia, pathological prothrombin consumption, decrease of fibrinogen, prothrombin and factor VII, factor V and AHG.3. Heparin treatment of the dogs was found to prevent the decrease of fibrinogen, prothrombin and factor VII, and factor V otherwise occurring after injection of high molecular weight dextran. Thrombocytopenia was not prevented.4. In in vitro experiments an interaction between fibrinogen and dextran of high and low molecular weight was found to take place in systems comprising pure fibrinogen. No such interaction occurred in the presence of plasma.5. It is concluded that the coagulation defects induced by infusions of high molecular weight dextran are due to intravascular coagulation.


1982 ◽  
Vol 48 (01) ◽  
pp. 108-111 ◽  
Author(s):  
Elisabetta Dejana ◽  
Silvia Villa ◽  
Giovanni de Gaetano

SummaryThe tail bleeding time (BT) in rats definitely varies according to the method applied. Of the various variables that may influence BT, we have evaluated the position (horizontal or vertical) of the tail, the environment (air or saline), the temperature (4°, 23° or 37° C) and the type of anaesthesia. Transection of the tail tip cannot be used to screen drugs active on platelet function since it is sensitive to coagulation defects. Template BT in contrast is not modified by heparin and is sensitive to defects of platelet number and function (“storage pool disease”, dipyridamole-like drugs, exogenous prostacyclin). In contrast the test fails to detect aspirin-induced platelet dysfunction. The evidence reported indicates that thromboxane A2-prostacyclin balance is not a factor regulating BT. Aspirin treatment however may be a precipitating factor when associated with other abnormalities of platelet function.Template BT is a valid screening test for platelet disorders and for antiplatelet drugs.


2020 ◽  
Vol 18 (04) ◽  
pp. 214-216
Author(s):  
Soumya Roy

AbstractA 9-year-old girl patient presented with left-sided weakness and joint contractures developing over a period of 18 months. She was known to be suffering from β-thalassemia major and was on regular blood transfusions. Eighteen months ago, she had suffered from an episode of ischemic cerebrovascular accident affecting the right side of her brain. Magnetic resonance angiogram revealed vaso-occlusive disease affecting mainly the anterior cerebral circulation, resembling Moyamoya disease. She was advised to carry out regular physiotherapy but her parents discontinued it, which resulted in the gradual development of joint contractures and muscle wasting.


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