Abstract
DDAVP has been described as a stimulator of plasma F-VIII and VWF. The present report describes a study of this response in 4 patients with end-organ resistance to DDAVP, X-linked NDI. DDAVP, 0.3 ugr/kg b.w. was infused IV over 20 min. At 0 time, 1 and 3 hrs urine was measured for osmolality (osm) and plasma for osm, F-VIII and VWF. In one patient this protocol was preceded by a 20 mg/kg water-load, to suppress endogenous vasopressin. Baseline F-VIII and VWF were 144-212 u/dl and 186-362 u/dl resp. in the 3 unsuppressed patients and 104 u/dl and 89 u/dl resp. in the water-loaded patient (normal 63-139 u/dl and 53-177 u/dl resp.). Following DDAVP plasma osm increased slightly, from 300 to 309 mOsm/kg to 305-313 mOsm/kg in the unsuppressed patients, while urine osm remained below 100 mOsm/kg. In the water-loaded patient plasma osm was 281 mOsm/kg. F-VIII increased to 272-760 u/dl and VWF to 213-632 u/dl. This increments were significant (p < 0.01) and similar to those observed in normal controls. It is concluded that in patients with NDI F-VIII and VWF are responsive to DDAVP and that end-organ resistance in NDI is confined to the kidneys.
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Brenner, B., Seligshon, U. & Hochberg, Z. NORMAL RESPONSE OF FACTOR (F)-VIII AND VONWILLEBRAND FACTOR (VWF) TO DDAVP IN NEPHRDGENIC DIABETES INSIPIDUS (NDI)). Pediatr Res 21 (Suppl 4), 339 (1987). https://doi.org/10.1203/00006450-198704010-01033
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DOI: https://doi.org/10.1203/00006450-198704010-01033