[Treatment of hemgrrhagic syndromes associated with blood coagulation disorders].
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Blood clotting tests in 55 polytraumatized patients showed that a lapse in the hemostatic potential may already occur at a very early stage of the traumatic-hemorrhagic shock. The extent of these changes which are complex in their pathogenesis, is closely related to the degree of injury. Coagulation-analysis control of the development shows clearly that shock treatment and maintenance of adequate circulation, starting at the earliest possible moment at the scene of the accident, are important for spontaneous recompensation of the hemostatic defect.
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Disorders of hemostasis are frequently encountered, and, if not recognised as early as possible, in particular prior to surgery, may lead to bleeding complications which are much more difficult to treat than those where exact diagnosis permits specific preventive therapy. A first case report on a woman with an acquired inhibitor against factor VIII illustrates that even minor injury may lead to hemorrhage necessitating blood transfusion. Furthermore, as long as the defect remains inaccessible to successful substitution therapy, wound healing will not take place. A second case report describes a patient with a congenital dysfunction of blood platelets, and serves as an example for similar disorders which, if unrecognised, lead to multiple, otherwise avoidable complications. Unfortunately there is no clearcut correlation between a given defect of hemostasis and bleeding manifestations, since psychological factors, among others, appear to influence the occurrence of "spontaneous" hemorrhage. Any unexpected and unexplained bleeding complication requires careful evaluation of the possible underlying causes, since such investigations often not only affect the future of the propositus himself but also that of his family members.
BACKGROUND: The aim of this study is to assess the prevalence of Parvovirus B19 infection in a group of patients affected by congenital coagulation disorders and its association with epidemiological aspects. PATIENTS AND METHODS: We have analyzed a group of 50 patients (median age 28) diagnosed with haemophilia or any other congenital coagulation disorder and 111 healthy non-transfused controls (median age 30) for IgG and IgM antibodies to Parvovirus B19 (Dako A/S, Glostrup, Dinamarca). Other issues analysed were HIV coinfection, the use of virally inactivated or non-inactivated plasma products and clinical symptoms of the infection. RESULTS: 84% of the patients (93.3% of those previously transfused) and 60.3% of the controls subjects showed IgG antibodies against Parvovirus B19. None of them had specific IgM antibodies. Five patients (all of them seronegative) had never been exposed to any plasma derivative and 11 were HIV-positive. The differences found between the prevalence of parvoviral infection in patients and controls are statistically significant, but those differences are only confirmed in younger patients (< 30) when age groups are compared. However, the severity of the haemostatic disorder, the type of plasma products infused or HIV coinfection had no influence on prevalence rates. The infection was clinically asymptomatic in all the cases. CONCLUSIONS: Haemophilic patients of any age are exposed to a higher risk of Parvovirus B19 infection than general population, although this infection had no clinical relevance in our study. The use of virally inactivated factor concentrates or the severity of the haemostatic disorder has no influence on this infectious risk.
Patients with hereditary hemostatic disorders, characterized by a tendency to bleeding or thrombosis, constitute a serious challenge in the dental practice. Advances in the medical diagnosis of hemostatic disorders have exposed dental professionals to new patients not amenable to the application of the management protocols associated with other, more well-known, disorders. It is the aim of this paper to review the evidence, to highlight the areas of major concern, and to suggest management regimens for patients with hereditary hemostatic disorders. An extensive review has been made (PubMed, Science Direct, Web of Knowledge, etc.) of literature pertaining to hereditary disorders affecting blood coagulation factors and how they affect the practice of dentistry. Several aspects relating to the care of such patients must be recognized and taken into consideration when dental treatment is planned. Replacement of deficient coagulation factors ensures that safe dental treatment will be carried out. However, the half-life of such coagulation factors requires that dental treatment be specifically planned and adapted to the type of pathology involved.
The aim of this study was to test the reliability and validity of the SF-36 questionnaire among the patients with hereditary blood coagulation disorders, to compare their quality of life (QoL) to that of healthy controls, and to identify the dimensions of life the patients consider most important. Results showed that the SF-36 questionnaire had good internal consistency reliability and construct and known group validity in individuals with hereditary blood coagulation disorders. Leisure activities/hobbies, availability of work/ study, followed by relationships with other people, own health and relationships with family/relatives appeared most frequently across the patients' and controls' priority ranks. The areas affected most by the disease were financial security, own health and relationships with family/relatives. A comparison of standardized scale scores suggests that blood coagulation disorders are diseases with a predominantly physical impact. Patients with blood coagulation disorders had health-related quality of life that was lower in most domains compared to healthy controls. However, when a wider concept of QoL was applied no differences between the patients' and controls' perceived QoL could be noted.
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