Kavanagh Catherine, Uy Natalie S
Department of Pediatric Nephrology, Columbia University Medical Center, 3959 Broadway, CHN 1115, New York, NY 10032, USA.
Department of Pediatric Nephrology, Columbia University Medical Center, 3959 Broadway, CHN 1115, New York, NY 10032, USA.
Pediatr Clin North Am. 2019 Feb;66(1):227-234. doi: 10.1016/j.pcl.2018.09.006.
Nephrogenic diabetes insipidus (NDI) results from the inability of the late distal tubules and collecting ducts to respond to vasopressin. The lack of ability to concentrate urine results in polyuria and polydipsia. Primary and acquired forms of NDI exist in children. Congenital NDI is a result of mutation in AVPR2 or AQP2 genes. Secondary NDI is associated with electrolyte abnormalities, obstructive uropathy, or certain medications. Management of NDI can be difficult with only symptomatic treatment available, using low-solute diet, diuretics, and prostaglandin inhibitors.
肾性尿崩症(NDI)是由于远端肾小管和集合管对血管加压素无反应所致。不能浓缩尿液导致多尿和烦渴。儿童存在原发性和获得性肾性尿崩症。先天性肾性尿崩症是AVPR2或AQP2基因突变的结果。继发性肾性尿崩症与电解质异常、梗阻性尿路病或某些药物有关。由于只有对症治疗可用,采用低溶质饮食、利尿剂和前列腺素抑制剂,肾性尿崩症的治疗可能很困难。