Nasal spray desmopressin (DDAVP) for mild hemophilia A and von Willebrand disease

Article Abstract:

Bleeding disorders, such as hemophilia A and von Willebrand disease, arise from the patient's lack of certain components of serum necessary for blood clotting. Administration of the drug desmopressin (DDAVP) causes a rise in these components, known as factor VIII activity, von Willebrand antigen, and ristocetin cofactor activity. The use of desmopressin in patients for whom it is effective can eliminate the need for transfusions of blood factors during a bleeding episode. DDAVP was given intravenously until the development of a subcutaneous form, which patients could self-administer at home. A method of intranasal delivery of desmopressin developed in Sweden was assessed in 22 patients who were known to respond to intravenous DDAVP; 11 with hemophilia A or carriers of it, and 11 with von Willebrand disease. The levels of their coagulation factors and bleeding times (the period necessary for a tiny cut to stop bleeding) were measured with both intravenous and intranasal DDAVP. The hemophilia patients had a median rise of 30 percent over their baseline factor VIII levels with intranasal desmopressin, and a 62 percent rise with the intravenous form. The von Willebrand patients had significant increases in the various factors and decreases in bleeding times with either route of administration. One of the more common bleeding complications these patients suffer is bleeding into joints, or hemarthrosis. A factor VIII level of 20 percent is necessary to stop hemarthrosis. All patients obtained levels of at least 20 percent with the intravenous form of the drug; 82 percent did so with intranasal DDAVP. Sixty-four percent achieved factor VIII levels of 30 percent after the intranasal dose, which is the level required to safely perform a surgical procedure. The side effects of intranasal DDAVP included facial flushing and headache, which occur with the intravenous form as well. A low level of sodium can result with intravenous DDAVP, but were not observed with the intranasal form. These results suggest that intranasal DDAVP is convenient and effective for many patients with hemophilia A and von Willebrand disease, and offers the convenience of being easily used at home, where so many bleeding episodes occur. (Consumer Summary produced by Reliance Medical Information, Inc.)

Blood clotting disorders, Blood coagulation disorders, Dosage and administration, Desmopressin, Von Willebrand's disease

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Mortality in patients with hemophilia: changes in a Dutch population from 1986 to 1992 and 1973 to 1986

Article Abstract:

Viral infections, especially HIV and hepatitis, may be responsible for the decreases in the life expectancy of men with hemophilia occurring since the mid-19702. Researchers in the Netherlands compared survival rates of two large groups of hemophiliacs, the first group consisting of patients tracked from 1973 to 1986, and the second group tracked from 1986 to 1992. The average life expectancy increased from 66 years in the first group to 68 years in the second group. However, twice as many patients died (45) in the second group as was predicted (22.6). HIV infection was primarily responsible for the increase in deaths, followed by hepatitis. Hemophiliacs who are not affected by viral infection may have the same life expectancy as the rest of the male population.

Health aspects, Patient outcomes, Mortality, Risk factors, Virus diseases

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Formation of antibodies to factor VIII in patients with hemophilia A who are treated with interferon for chronic hepatitis C

Article Abstract:

Long-term interferon therapy for hepatitis C in hemophilia patients does not appear to produce factor VIII inhibitors. A deficiency of factor VIII is the cause of hemophilia A and is a serious side effect of interferon therapy. Over an average of 19.5 months, only two out of 21 hemophilia patients receiving interferon treatment for hepatitis C developed factor VIII inhibitors. Three of 14 untreated patients developed factor VIII inhibitors on one occasion.

Care and treatment, Physiological aspects, Hepatitis C, Interferon, Blood coagulation factor VIII, Factor VIII (Blood coagulation)

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