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Desmopressin increases endogenous factor VIII levels in hemophilia A. Large inter-individual variation in the response to desmopressin is observed. Patients with a lower baseline factor VIII activity tend to show a reduced response. Therefore, desmopressin is less frequently used in moderate hemophilia A patients (baseline factor VIII activity 1-5 international units/deciliter), even though factor VIII levels may rise substantially in some of them. We aim to describe the response to desmopressin in moderate hemophilia A patients and to identify predictors. We selected data on 169 patients with moderate hemophilia from the multicenter RISE cohort study. Adequate response to desmopressin was defined as a peak factor VIII level ≥ 30 and excellent response as ≥ 50 international units/deciliter after desmopressin administration. We used univariate and multiple linear regression techniques to analyze predictors of the peak factor VIII level. Response was considered adequate in 68 patients (40%), of whom 25 showed excellent response (15%). Intravenous administration, age, pre-desmopressin factor VIII activity and Von Willebrand factor antigen, peak Von Willebrand factor activity and desmopressin induced rise in Von Willebrand factor antigen were significant predictors of peak factor VIII level and explained 65% of the inter-individual variation. In 40% of moderate hemophilia A patients, desmopressin response was adequate. Therefore, it is important not to refrain this group of patients from desmopressin responsiveness. Among the six predictors that we identified for desmopressin induced FVIII rise, factor VIII activity and desmopressin induced rise in Von Willebrand factor antigen had the strongest effect.
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To collect and analyze data on females with hemophilia so as to better define the difference between the study population and the male population with hemophilia.
A deficiency of blood coagulation factor IX inherited as an X-linked disorder. (Also known as Christmas Disease, after the first patient studied in detail, not the holy day.) Historical and clinical features resemble those in classic hemophilia (HEMOPHILIA A), but patients present with fewer symptoms. Severity of bleeding is usually similar in members of a single family. Many patients are asymptomatic until the hemostatic system is stressed by surgery or trauma. Treatment is similar to that for hemophilia A. (From Cecil Textbook of Medicine, 19th ed, p1008)
Method of psychotherapeutic treatment based on assumption of patients' personal responsibility for their own behavior. The therapist actively guides patients to accurate self-perception for fulfillment of needs of self-worth and respect for others. (From APA, Thesaurus of Psychological Index Terms, 8th ed.)
The classic hemophilia resulting from a deficiency of factor VIII. It is an inherited disorder of blood coagulation characterized by a permanent tendency to hemorrhage.
Bleeding into the joints. It may arise from trauma or spontaneously in patients with hemophilia.
A hereditary deficiency of blood coagulation factor XI (also known as plasma thromboplastin antecedent or PTA or antihemophilic factor C) resulting in a systemic blood-clotting defect called hemophilia C or Rosenthal's syndrome, that may resemble classical hemophilia.