Nephrogenic diabetes insipidus. Radiological and clinical features

Two brothers with nephrogenic diabetes insipidus are presented. The importance of early recognition and treatment to reverse the consequential complications are underscored. The disorder has a sex-linked recessive transmission, where all male offspring of an affected mother show the extreme thirst characteristic of the disease; in whom hyposthenuria persisted despite arginine vasopressin infusion, and in whom the serum arginine vasopressin concentrations correlate with the serum osmolality which serves to discriminate nephrogenic diabetes insipidus from the pituitary diabetes insipidus. The radiological features of "non-obstructive" hydronephrosis and hydroureters, the combined use of hydrochlorothiazide and the potassium-sparing diuretic amiloride in comparison to the prostaglandin inhibitor are discussed.