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.
The role of endotoxin aggression in the development of hemostatic disorders was analyzed in 62 children who had undergone urgent and elective surgeries. It is demonstrated that indices of endotoxin-antiendotoxin system and hemostasis before surgery may be regarded as prognostic criteria for development of complications during and after surgery.
OBJECTIVE: The study was undertaken to evaluate the levonorgestrel-releasing intrauterine system (LNG-IUS) in managing menorrhagia associated with hemostatic disorders. STUDY DESIGN: Retrospective notes review. RESULTS: Nineteen of 28 (68%) patients experienced improvement with the LNG-IUS. There was nonacceptance by 9 of 28 (32%) who refused the LNG-IUS or had it definitively removed. CONCLUSION: The LNG-IUS should be considered when managing menorrhagia because surgery carries additional risks in this subset of patients.
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.
The responses of blood coagulation and fibrinolysis show a clear phase course in patients with pancreonecrosis in the dynamics of the postresuscitation period. There were different changes in the phasic pattern of hemostatic disorders in survivors as compared with a group of deceased patients. With a good outcome, there was a twofold reduction in the concentration of fibrinogen as compared with the normal values and an increase in whole blood fibrinolytic activity on days 21-23. The increased whole blood fibrinolytic activity in the group of survivors in the presence of invariably decreased Hageman-dependent plasma fibrinolytic activity in both groups of the patients suggests that formed blood cells contributes to the process of recovery.
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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.