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Pediatric emergency medicine trisk 563

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mutation, family history is often positive for bleeding symptoms; however, even within a family, the degree of symptomatology can be variable Type VWD is rare and inherited in an autosomal recessive fashion; clinical symptoms resemble severe hemophilia Diagnostic Testing Patients with VWD will typically have a normal PT/aPTT, except for those with more severe disease who have a prolonged aPTT The diagnosis of VWD requires a special coagulation study which examines VWF antigen level (VWF:Ag), its function, often ristocetin-induced platelet agglutination (VWF:RCo), and factor VIII activity (FVIII:C), the protein that it carries in plasma The normal level for each of these components is greater than 50% The lower limit of normal and how to define VWD has been controversial, but it is presently generally accepted that levels

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