Hartenberg M A, Cory M, Chan J C
Int J Pediatr Nephrol. 1985 Oct-Dec;6(4):281-6.
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.
本文报告了两例患有肾性尿崩症的兄弟。强调了早期识别和治疗以逆转相应并发症的重要性。该疾病具有X连锁隐性遗传，患病母亲的所有男性后代均表现出该病极度口渴的特征；这些男性尽管输注了精氨酸加压素，仍存在低渗尿，且其血清精氨酸加压素浓度与血清渗透压相关，这有助于鉴别肾性尿崩症和垂体性尿崩症。文中还讨论了“非梗阻性”肾积水和输尿管积水的放射学特征，以及氢氯噻嗪和保钾利尿剂阿米洛利联合使用与前列腺素抑制剂相比的情况。
