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DDAVP (desmopressin; 1‐deamino‐cys‐8‐ d ‐arginine‐vasopressin) treatment in children with haemophilia B
Author(s) -
Ehl S.,
Severin T.,
Sutor A. H.
Publication year - 2000
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.2000.02504.x
Subject(s) - desmopressin , vasopressin , medicine , haemophilia b , partial thromboplastin time , haemophilia , factor ix , arginine , coagulopathy , von willebrand factor , endocrinology , coagulation , haemophilia a , gastroenterology , surgery , chemistry , biochemistry , platelet , amino acid
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 IX 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.