Diagn sis and treatment of familial neurohypophyseal diabetes insipidus with hydronephrosis / 中华泌尿外科杂志

ABSTRACT

Objective To investigate the causes and treatment of familial neurohypophyseal diabetes insipidus with hydronephrosis.Methods A retrospective analysis was conducted in 6 cases (5 males and 1 female,aged 11 to 53 years) of familial neurohypophyseal diabetes insipidus with hydronephrosis treated in our institute from June 2009 to December 2010.All cases had polydipsia and polyuria since their childhood.The daily output of urine ranged from 5,290 to 15,040 ml.The urine specific gravity was less than 1.005.The water deprivation and vasopressin injection test showed positive results,and MRI showed that the shape and size of pituitary gland were in normal range.Ultrasound and IVU showed that all cases had hydronephrosis.Five adult cases were administered with Desmopressin 0.2 mg three times a day,and 1 juvenile patient given half dosage of Desmopressin as in adult.The case No.1 underwent percutaneous nephrostomy and bilateral ureteral reimplantation.Case No.2 received urethral catheterization for 5 days and Tamsulosin.Three cases with urinary tract infection were given antibiotics on the base of urine culture and antibiotic sensitivity test results.Follow-up was undertaken every 3 mon for the duration of 18-36 mon.Results In 6 cases,polydipsia and polyuria were significantly improved after the treatment.Daily urine output dropped to 6000 ml in 5 adult cases and decreased to 2000 ml in the juvenile case.The flank sore of case No.1 was relieved after percutaneous nephrostomy,and hydronephrosis improved 6 mon after bilateral ureteral reimplantation.The residual urine volume of case No.2 was reduced to 40 ml,and no recurrence was observed after anti-infection therapy.During the follow-up,6 cases showed relieved hydronephrosis and no recurrent infection.Conclusions It is of important to reduce the urine volume for the treatment of familial neurohypophyseal diabetes insipidus with hydronephrosis.Early diagnosis and treatment of the diseases is crucial for the improvement of renal function.