Hemostatic disorders: hemophilia and the hemophilioid diseases.
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OBJECTIVE: To assess a point-of-care instrument for identification of primary hemostatic disorders in dogs. ANIMALS: 29 healthy dogs and 23 nonanemic dogs with primary hemostatic disorders (thrombocytopenia, n = 6; thrombopathia, 6; von Willebrand disease [vWD], 11). PROCEDURE: Citrated blood was obtained and closure times (CT) were determined by measuring the time required for occlusion of an aperture by a platelet plug within the point-of-care instrument. Reference ranges for CT were established, and CT were determined for dogs with primary hemostatic disorders. RESULTS: CT measured with adenosine diphosphate as the platelet agonist (ADP-CT) ranged from 52 to 86 seconds for healthy dogs (mean +/- 2 SD, 67 +/- 7.8 seconds; median, 65 seconds), and CT measured with epinephrine as the agonist (EPI-CT), from 97 to 225 seconds (151 +/- 38 seconds; 148 seconds). In thrombocytopenic dogs, ADP- and EPI-CT were prolonged (> 165 and > 264 seconds, respectively). Five of 6 dogs with thrombopathia had prolonged ADP-CT, whereas EPI-CT was prolonged in all 6 dogs. In all dogs with vWD, ADP-CT was prolonged; EPI-CT was prolonged in 10 of these dogs. Sensitivity and specificity for ADP-CT were 95.7 and 100%, respectively, and positive and negative predictive values, 100 and 96.7%, respectively, whereas for EPI-CT, these values were 95.7 and 82.8%, respectively, and 81.5 and 96%, respectively. CONCLUSIONS AND CLINICAL RELEVANCE: The point-of-care instrument allowed quick assessment of primary hemostasis in nonanemic dogs. Use of this instrument may be helpful for making decisions regarding management of dogs with primary hemostatic disorders.
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Therapy with blood and blood products related to hemostatic disorders in small animal practice is reviewed. Administration of platelet rich plasma and platelet concentrates in thrombocytopenia or thrombopathia is discussed. Vascular purpuras, vasculitis, and vascular inherited defects are also considered. Inherited coagulation disorders are summarized and the therapeutic choices in treating these disorders are also proposed. In addition, acquired coagulation disorders are briefly reviewed.
This report deals with clinical experience of urologic surgery of patients with hemostatic disorder or hemolytic disease. In the past 5 years from May 1986, 14 operations were conducted in our clinic on 13 patients, consisting of 4 with von Willebrand disease (vWd), 1 with hemophilia B, 4 who had warfarin administration, 3 with essential thrombocythemia and 2 with spherocytosis. Almost all patients were treated hematologically before the urological operations. Except in 1 case, the post-operative course was favorable and under hematologic control. Massive bleeding in 1 case was obviously attributable to over-dosage of warfarin. It is difficult to determine the optimal dose of warfarin under an unstable hemostatic condition during the operation and recovery periods. However, it is possible to carry out urologic surgery for these patients under appropriate hematologic control, and ESWL was safely performed without medical treatment on 3 patients; 1 with vWd, 1 treated with warfarin and 1 with spherocytosis.
OBJECTIVE: To distinguish which nosebleeds in children hide an underlying coagulation disorder, characteristics of recurrent epistaxis were evaluated correlating the severity with a battery of specific tests on primary and secondary hemostasis. PATIENTS AND METHODS: Epistaxis of fifty-eight children were classified as mild or severe according to frequency, duration, amount of blood lost, proportion of life that nosebleeds have been recurrent and uni- or bilateral bleeding. Epidemiological characteristics were evaluated, as well as hemostatic tests including: platelet count, mean platelet volume, plasmatic fibrinogen level, prothrombine time, activated partial thromboplastin time, thrombin time, bleeding time, von Willebrand factor antigen and ristocetin cofactor, factor VIII coagulant, and platelet aggregation to different agents. The effect of drugs or the presence of tumors was discarded. RESULTS: In the group of children with mild epistaxis (n = 39) there were three cases with laboratory abnormalities (10.3%). In severe epistaxis (n = 19) abnormalities were found in eleven cases (57.9%) and specific entities were detected in six of them (three children with von Willebrand's disease, one Bernard-Soulier syndrome, one autoimmune thrombocytopenic purpura and one Rendü-Osler-Weber disease). Epistaxis labeled as mild needed less cauterizations and packings, were more related to seasonal prevalence and self-handling, and poorly influenced iron metabolism. CONCLUSIONS: The five qualities pointed out on recurrent epistaxis in children allow the identification of who must be thoroughly studied by means of specific tests to rule out any kind of hemostatic disorder.
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Eighty-three general surgical patients completed the standardized bleeding history questionnaire, and screening tests of platelet counts, prothrombin times, partial thromboplastin times, and Ivy bleeding times were done on these patients. Fifty-two per cent had undergone previous operation; 25% described symptoms of potential hemostatic disorders and seven per cent had positive family histories. Laboratory results indicated abnormalities in five patients (6%). The bleeding history is an important part of the preoperative evaluation of a patient, but it can have serious false-negative results. This history should guide the selection of laboratory tests. Such testing can yield an unexpectedly high rate of abnormalities. When identified, these abnormalities require further investigation.
We describe a microtechnology for the study of the coagulation system in newborn infants. Interpretation of results demands an understanding of the techniques used and the nature of the control population from which normal values are drawn. We have examined two syndromes which represent the majority of hemostatic disorders of sick newborn infants. The first is thrombocytopenia resulting from bacterial infections in which there are minimal changes in the levels of blood coagulation factors and little tendency to bleed. The second is a syndrome of disseminated intravascular coagulation in which there is a profound disturbance in the coagulation mechanism, relatively little change in platelet counts, a severe hemorrhagic diathesis, and widespread ischemic necrosis.
Hemorrhagic and thrombophilic diathesis are important symptoms in patients with neoplastic diseases. From that point of view it is not surprising that malignancy is often complicated by bleeding or thromboembolism. These clinical manifestations are due to the interaction of the tumor and components of the hemostatic system (platelets, coagulation, and fibrinolytic factors). Specific and adequate therapy of the neoplasm will result in the disappearance of the hemostatic disorder. If no effective antitumor therapy is available drugs may be helpful, which influence the enhanced turnover of clotting factors. Furthermore, platelet transfusions are highly effective in thrombocytopenic bleeding.
Arginine vasopressin preparations have been used in the treatment of diabetes insipidus for many years. Compared with older antidiuretic agents, the synthetic analog desmopressin is more potent, longer acting and easier to use. It is available for intravenous, subcutaneous and intranasal administration. Desmopressin may be useful in the treatment of hemostatic disorders such as von Willebrand's disease and hemophilia A. It has also been used for nocturnal enuresis. The vasopressor effects of arginine vasopressin preparations have been exploited for use as a temporizing measure in controlling acute gastrointestinal bleeding. Side effects such as hyponatremia and water intoxication are uncommon when these drugs are used with proper precautions.
Morphologic studies were made on the brain tissue and internal organs of those deceased (n = 75) who had died of leptospirosis icterohemorrhagica during different periods of the condition, with the results obtained being compared to clinical and laboratory findings. It has been shown that realization of the pathogenic potential of the disease-producing germ is exercised both through direct generalized injury to the endothelium of microvessels, parenchymal cells (predominantly those of the liver and kidneys) by toxic substances of Leptospira and (to a larger degree) through disorders of hemostasis presented as thrombosis of vessels of the microcirculatory bed with subsequent development of hemorrhagic manifestations and alterative changes of hypoxic genesis in vital organs. Early haemocoagulatory disorders are characterized by marked and stable hypocoagulation, with direction of the processes of fibrin formation and fibrinolysis being different, which fact is consistent with diversity of structural formations originating from fibrin in the microcirculatory bed of the deceased. Prophylaxis of grave pathologic changes in the organism leading to lethal outcome consists in correction of hemostatic disorders during early phases of the disease course, which is to be pathogenetically substantiated and situational one because of the problem posed by verification of stages of that syndrome of disseminated intravascular coagulation in patients with grave course of leptospirosis icterohemorrhagica.
Bleeding times are reported in many studies using canine models, with a variety of techniques employed to adapt these tests for dogs. We evaluated a canine model of template bleeding time, the buccal mucosa bleeding time (BMBT), by examining the test's sensitivity and specificity for defects of primary hemostasis. We examined thirty-five dogs having defined defects of either primary hemostasis (Types I, II, III von Willebrand's disease, thrombasthenia, thrombopathia) or secondary hemostasis (hemophilia A and B, Factor VII deficiency). Comparisons of BMBT and cuticle bleeding time were made in a subset of these dogs. All dogs having primary hemostatic disorders had long BMBT, and all factor deficient dogs had BMBT within normal range. The BMBT in canine models appears to be a specific and sensitive test of primary hemostasis; suitable for evaluating factors affecting template bleeding time and potential efficacy and thrombogenicity of treatment regimens.
One hundred and seventeen female subjects were studied: 23 patients with cervical carcinoma; 25 with endometrial carcinoma; 29 with benign uterine diseases; 14 with ovarian carcinoma; 26 patients with benign ovarian tumors. These patients, together with 25 healthy female control subjects, underwent several coagulation tests including Beta-Thromboglobulin (Beta-TG) and Platelet Factor 4 (PF4) plasma levels. Among all Beta-TG and PF4 exhibited the most interesting results. They were increased in four groups of patients: those with malignant 92.2% (13/14) and benign 50% (13/26) ovarian tumors and those with endometrial 64% (16/25) and cervical carcinoma 69.5% (16/23). Our study showed a high incidence of abnormalities of Beta-TG and PF4, early signs of hemostatic disorders, in gynecological malignancies especially in ovarian carcinoma. These data suggest a possible value of these tests as tumor-markers and in order to detect the patients who develop thrombo-embolic accidents.