DDAVP challenge tests in XXXX with mild/moderate @entity447 .

@entity2801 @entity1 @entity7062 @entity447
@entity7062 (DDAVP) increases plasma factor VIII coagulant activity ( @entity2801 :C) levels in @entity1 with mild/moderate @entity447 . In some subjects, @entity2801 can be increased to haemostatic levels, thereby avoiding use of factor VIII concentrates. We reviewed our hospital's experience with 62 @entity1 with @entity2801 :C levels 0.01-0.3 IU/ml who had a DDAVP challenge test (i.v. 0.3 microg/kg) following diagnosis. A therapeutic response was defined as a 1 h post- @entity2801 :C increase at least twofold over baseline and > 0.3 IU/ml. Of the total group, 29 (47%) @entity1 responded to DDAVP, all of them with mild @entity447 (baseline @entity2801 :C > or = 0.05 IU/ml), yielding a response rate of 57% in this subgroup. @entity1 who responded to @entity7062 had higher baseline @entity2801 :C levels (mean +/- SEM, 0.17 +/- 0.01 vs 0.10 +/- 0.01 IU/ml, P < 0.01) and were older (5.2 +/- 0.8 vs 3 +/- 0.4 years, P = 0.02) than those who failed to do so. The association between DDAVP response and age, however, remains unclear: seven @entity1 who failed the initial challenge test responded to re-challenge after a mean of 6.3 years (median 4.9, range 0.5-12.5), increasing the response rate in @entity1 with mild @entity447 to 71%. Age and @entity2801 :C association with DDAVP response are both important in @entity1 with mild/moderate @entity447 . Absence of response to DDAVP should therefore be confirmed by later re-challenge.
