von Willebrand's Disease and Its Response to DDAVP

Abstract

Abstract: Objectives: 1. The descriptive analysis of the cal manilestations and the evaluation of the dagnosite laboratlon testings 2. The comparison between the efficiency of intravenous and intranasal 1-deamino 8D-arginine vassin (DDA/DDAVP) among Thai patients with vWD. Study design: The clinical manifestations and the laboratory testings among Thai patients with VWD were analyzed. Then, 0.3 ug/kg and/or 150-300 ug of DDAVP were subsequently given. Blood was drawn for the measurement of factor VII cotting actvity (F VII), von Willebrand factor antigen (WII-Ag) and ristopein cofactor (RCoF) activivite and and at 1,6 and/ or 24 hours after the administion of DDAVP. In addition, the bleeding fime and ristocalinduced plalet aggregation (R) was checked before and 1 hour after the medication. Result: Twenty four cases with WWD were included. The most common bleeding symptoms were ecchymosis (41.7%) and epistaxistaxis (37.5%) were manliested as early as 2 years Although vWD is mosty inherted by autosomal dominance, only 17 cases(70.8%) had a family history of bleeding disorder. The patients be olassifed as type 1, 22 cases; type II and type II, one each. Consequently, the percent of posive laboratory testing for the dis of oi vW was as followed : F VIl:C 41.5%, APTT = 44.7%, bleeding = 67.6%, VWF:Ag = 73.2%, RIPA = 78.6% and RCoF activity = 94.1%. Fifteen patients received intavenous DDAVP, the levels of VIII:C, vWF.Ag and RCoF activity reache maximum inorement of 3.63, 2.79 and 2,60 times, respectively one hour after the medcation. Subsequenty, 8 out 15 cases also recelved intranasal DDAVP, the levels of F VIIIAg and ROoF activity reached the maxim ingrement of 2.74, 2.19 and 1.35 times, respectively one hour affer the medication. These levels were significantly higher than the pretreatment level (p:05) except RCooC activity via intranasal route.However the levels of intravenous route were not significantly higher than those of the intranasal route. Conclusion: The diagnosis of vWD depends primarly upon the manifestation of the bleeding disorder and laboratory testings. The vWF:Ag and RCoF activity.