Familienuntersuchungen bei Patienten mit von Willebrand-Jürgens-Syndrom

Wendisch, J.; Thomas, Kathy B.; Siegert, G.; Sutor, A. H.; Weissbach, G

doi:10.1007/978-3-642-60418-8_38

Cited by 1 publication

(1 citation statement)

References 2 publications

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“…18,19,50 In a series of 20 patients with vWD, the vWF:CBA was below the normal range only in those patients who had a history of clini cally relevant bleeding and whose bleeding time was prolonged. 51 Whereas vWF:Ag and vWF:Ristocetin Cofactor (RiCof) increased to a similar extent after DDAVP, 52,53 the vWF:CBA increased to a much greater extent. The dissociation was unexpected, because a good correlation between the levels of vWF:Ag, vWF:RiCof, and vWF:CBA was found previously in the plasma of normal donors and patients with vWD type 1 under rest ing conditions.…”

Section: Monitoringmentioning

confidence: 96%

Desmopressin (DDAVP) in Bleeding Disorders of Childhood

Sutor

1998

Semin Thromb Hemost

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As in adults, desmopressin (DDAVP) can be used in children for prophylaxis of bleeding and to stop bleeding in many hereditary and acquired bleeding disorders. DDAVP is the treatment of choice in children with mild hemophilia and type 1 von Willebrand's disease (vWD). It is effective in some variants of vWD and in many patients with platelet function defects. It reduces the bleeding diathesis of children with uremia and drug-induced bleeding complications. In any case, a test dose of DDAVP has to be given to the patient to predict the hemostatic effect before relying on this drug for treatment. The response can be measured by shortening of the bleeding time (BT) and of partial thromboplastin time (PTT), indicating a rise of Factor (F) VIII or von Willebrand factor (vWF). Side effects such as facial flushing, transient headache, increased pulse rate, and drop in systolic blood pressure are mild and transient. They can be minimized when the dose is not exceeding 0.3 microg/kg body weight, and the infusion lasts at least 20 to 30 minutes. The strong antidiuretic action of DDAVP has some potential problems that are negligible in adults and older children when water intake is restricted. In infants and small children under the age of 18 months, however, DDAVP should be used with caution and with close surveillance in order to prevent water intoxication and electrolyte imbalance. The danger is increased when the patients receive parenteral fluid substitution. The advantages of DDAVP include the reduction in the use of plasma factor concentrates, thereby minimizing the danger of immunological or infectious complications, as well as the considerable reduction of costs realized by treatment with this form of medication. Fortunately, it can be applied successfully in the most frequent hereditary bleeding disorder, namely vWD type 1.

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Section: Monitoringmentioning

confidence: 96%

Desmopressin (DDAVP) in Bleeding Disorders of Childhood

Sutor

1998

Semin Thromb Hemost

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Familienuntersuchungen bei Patienten mit von Willebrand-Jürgens-Syndrom

Cited by 1 publication

References 2 publications

Desmopressin (DDAVP) in Bleeding Disorders of Childhood

Desmopressin (DDAVP) in Bleeding Disorders of Childhood

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