DDAVP (desmopressin; 1-deamino-cys-8-D-arginine-vasopressin) treatment in children with haemophilia B

被引：14

作者：

Ehl, S ^{[1
]}

Severin, T ^{[1
]}

Sutor, AH ^{[1
]}

机构：

[1] Univ Freiburg, Kinderklin, Dept Paediat, D-79106 Freiburg, Germany

来源：

BRITISH JOURNAL OF HAEMATOLOGY | 2000年 / 111卷 / 04期

关键词：

haemophilia B; DDAVP; children; aPTT; factor IX;

D O I：

10.1046/j.1365-2141.2000.02504.x

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

We tested the response to desmopressin (1-deamino-cys-8-D-arginine-vasopressin: DDAVP) in four patients with haemophilia B [factor IX (F IX) at diagnosis 1.4-5%]. The activated partial thromboplastin time (aPTT) was significantly shortened in all patients. Although there was an up to 1.4-fold increase in F IX levels in three patients, maximal F TX activity remained below 10%. Much more prominent were the increases in F VIII (three- to fourfold), in von Willebrand factor antigen (VWF:Ag; 2.5-fold) and particularly in VWF collagen-binding activity (VWF:CBA; fivefold). These changes were reflected by the prophylactic efficacy of DDAVP for dental surgery. After pretesting, DDAVP could be a useful drug for reducing the need for plasma products for prevention of minor surgical bleeding in patients with mild to moderate haemophilia B.

引用

页码：1260 / 1262

页数：3