Sections View Full Chapter Figures Tables Videos Full Chapter Figures Tables Videos Supplementary Content ++ Key Features ++ Easy bruising and epistaxis from early childhood Menorrhagia Prolonged platelet function analyzer (PFA)-100 (or bleeding time), normal platelet count, absence of acquired platelet dysfunction Reduced amount or abnormal activity of von Willebrand factor (vWF) ++ Clinical Findings ++ A history of increased bruising and excessive epistaxis is often present Prolonged bleeding also occurs with trauma or at surgery Can develop in association with hypothyroidism, Wilms tumor, cardiac disease, kidney disease, or systemic lupus erythematosus, and in individuals receiving valproic acid ++ Diagnosis ++ Prothrombin time is normal Activated partial thromboplastin time is sometimes prolonged Prolongation of the PFA-100 or bleeding time is usually present ++ Treatment ++ Desmopressin May be administered intravenously or subcutaneously Typically elicits a three- to fivefold rise in plasma vWF A high-concentration desmopressin nasal spray (150 μg/spray), different from the preparation used for enuresis, may be used May cause fluid shifts, hyponatremia, and seizures If further therapy is indicated, vWF-replacement therapy (eg, plasma-derived concentrate) is recommended Antifibrinolytic agents (eg, ε-aminocaproic acid) may be useful for control of mucosal bleeding GET ACCESS TO THIS RESOURCE Sign In Username Error: Please enter User Name Password Error: Please enter Password Forgot Username? Forgot Password? Sign in via OpenAthens Sign in via Shibboleth Get Free Access Through Your Institution Contact your institution's library to ask if they subscribe to McGraw-Hill Medical Products. Access My Subscription GET ACCESS TO THIS RESOURCE Subscription Options Pay Per View Timed Access to all of AccessPediatrics 24 Hour $34.95 (USD) Buy Now 48 Hour $54.95 (USD) Buy Now Best Value AccessPediatrics Full Site: One-Year Individual Subscription $595 USD Buy Now View All Subscription Options