Caletti María Gracia, Balestracci Alejandro, Di Pinto Diana
Department of Nephrology, Hospital de Pediatría Prof. Dr. Juan P. Garrahan, Buenos Aires, Argentina,
Pediatr Nephrol. 2014 Mar;29(3):487-90. doi: 10.1007/s00467-013-2689-z. Epub 2013 Dec 14.
Nephrogenic diabetes insipidus (NDI) is characterized by the kidney's inability to concentrate urine, which causes intense polyuria that may lead to urinary tract dilation. We report the morphological findings of the urinary tract in ten boys with NDI specifically addressing the presence and changes of urinary tract dilation during treatment.
DIAGNOSIS/TREATMENT: Patients were diagnosed at a median age of 1.6 years (range, 0.16-6.33 years) and treated with a low osmotic diet, hydrochlorothiazide-amiloride and indomethacin, which decreased the diuresis from a median of 10.5 ml/kg/h to 4.4 ml/kg/h (p < 0.001). Three patients showed normal renal ultrasound before treatment until last control, while the remaining seven showed urinary tract dilation. In this second group, dilation was reduced with treatment in four patients and disappeared in the remaining three. Children without dilation or in whom the dilation disappeared were diagnosed and treated earlier than those with persistent dilation (median 1.66 versus 4.45 years, respectively). After a median of 10.4 (range, 2.3-20.3) years of follow-up, no patients showed urological complications.
Medical treatment of the disease improved the dilation in all cases, preventing its potential complications. Regardless of the good outcome of our patients, periodic urologic follow-up is recommended in NDI patients.
肾性尿崩症（NDI）的特征是肾脏无法浓缩尿液，这会导致强烈的多尿，进而可能导致尿路扩张。我们报告了10例NDI男孩尿路的形态学结果，特别关注治疗期间尿路扩张的存在及变化。
诊断/治疗：患者诊断时的中位年龄为1.6岁（范围0.16 - 6.33岁），接受低渗饮食、氢氯噻嗪 - 阿米洛利和吲哚美辛治疗，这使尿量从平均10.5 ml/kg/h降至4.4 ml/kg/h（p < 0.００１）。3例患者治疗前直至最后一次检查时肾脏超声均正常，而其余7例显示尿路扩张。在第二组中，4例患者的扩张经治疗后减轻，其余3例消失。无扩张或扩张消失的儿童比持续存在扩张的儿童诊断和治疗更早（中位年龄分别为1.66岁和4.45岁）。中位随访10.4年（范围2.3 - 20.3年）后，无患者出现泌尿系统并发症。
该疾病的药物治疗改善了所有病例的扩张情况，预防了其潜在并发症。尽管我们的患者预后良好，但建议对NDI患者进行定期的泌尿外科随访。
