Kaulitz R, Brodehl J
Medizinische Hochschule Hannover, Kinderklinik.
Klin Padiatr. 1989 Nov-Dec;201(6):425-30. doi: 10.1055/s-2008-1026741.
From 1975-1986 6 boys with congenital nephrogenic diabetes insipidus were diagnosed at the age of 3 months to 10 years. Symptoms appeared within the first few weeks of life. The diagnosis was confirmed by polyuria, low urinary osmolality (97-225 mosm/kg H20), hypernatraemia (max. 171 mmol/l) and the missing response to vasopressin. The treatment was variable; 4 boys received only hydrochlorothiazide (2-2.5 mg/kg/d) which lead to a reduction of the daily urinary volume of 26-44%. Hyperelectrolytaemia disappeared and a normal thriving could be achieved. Later an additional treatment with indomethacin (2 mg/kg/d) was necessary in 3 boys because of an increase of polyuria; there was a further reduction of the daily urinary volume of 50-60%. The combination of hydrochlorothiazide and indomethacin in the treatment of the congenital nephrogenic diabetes insipidus was well tolerated and seems to be--especially during the first few years of life--a necessary and effective treatment which allows a normal thriving and psychointellectual development.
1975年至1986年期间，6名先天性肾性尿崩症男孩在3个月至10岁时被确诊。症状在出生后的头几周内出现。通过多尿、低尿渗透压（97 - 225 mosm/kg H₂O）、高钠血症（最高171 mmol/l）以及对血管加压素无反应得以确诊。治疗方法各异；4名男孩仅接受氢氯噻嗪治疗（2 - 2.5 mg/kg/d），这使每日尿量减少了26% - 44%。高电解质血症消失，患儿得以正常成长。后来，3名男孩因多尿增加，需要加用吲哚美辛（2 mg/kg/d）治疗；每日尿量进一步减少了50% - 60%。氢氯噻嗪和吲哚美辛联合治疗先天性肾性尿崩症耐受性良好，而且似乎是——尤其是在生命的最初几年——一种必要且有效的治疗方法，可使患儿正常成长及心理智力发育。
