PEDIATRICS Vol. 86 No. 2 August 1990, pp. 238-243
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Intravenous Desmopressin Acetate in Children With Sickle Trait and Persistent Macroscopic Hematuria

Lou Anne Baldree MD1, Bettina H. Ault MD1, Carolyn M. Chesney MD1, and F. Bruder Stapleton MD1

1 From the Departments of Pediatrics and Medicine, University of Tennessee, and LeBonheur Children's Medical Center, Memphis, Tennessee

Persistent gross hematuria associated with sickle hemoglobinopathy that fails to respond to conventional supportive therapy represents a difficult management dilemma. Two such patients with protracted, often painful, sickle trait macrohematuria are described. Both patients had normal renal anatomy and vasculature and had failed to respond to bed rest, intravenous hydration, and a trial of oral jukcy-aminocaproic acid. Patient 1 had normal coagulation function. Patient 2 had von Willebrand disease (decreased factor VIII antigen and quantitative ristocetin cofactor activity). Patient 1 responded to intravenous desmopressin acetate at a dose of 0.3 µg/kg with a 155% increase in factor VIII clotting activity and a 135% increase in ristocetin cofactor and cessation of her macrohematuria within 18 hours after completion of the desmopressin infusion. She remained free of gross hematuria for 5 months with the exception of short-lived trauma-induced hematuria (in three voids) 6 weeks after desmopressin therapy. Patient 2 did not respond to intravenous desmopressin infusion despite a 234% and a 360% increase in factor VIII clotting activity and ristocetin cofactor, respectively. Intravenous desmopressin acetate may be helpful in halting protracted significant macrohematuria associated with sickle trait hemoglobinopathy in some patients when conventional management fails.

Key Words: hematuria • sickle hemoglobinopathy • desmopressin acetate • aminocaproic acid

Submitted on December 27, 1988
Accepted on May 17, 1989