[Long-term course of 6 boys with congenital nephrogenic diabetes insipidus].

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.
AuthorsR Kaulitz, J Brodehl
JournalKlinische Pàˆdiatrie (Klin Padiatr) 1989 Nov-Dec Vol. 201 Issue 6 Pg. 425-30 ISSN: 0300-8630 [Print] GERMANY, WEST
Vernacular TitleLangfristige Verläufe von 6 Jungen mit kongenitalem nephrogenem Diabetes insipidus.
PMID2601280 (Publication Type: Case Reports, English Abstract, Journal Article)
Chemical References
  • Hydrochlorothiazide
  • Adolescent
  • Child
  • Child, Preschool
  • Diabetes Insipidus (drug therapy, genetics)
  • Follow-Up Studies
  • Humans
  • Hydrochlorothiazide (therapeutic use)
  • Infant
  • Kidney Concentrating Ability (drug effects)
  • Male
  • Pedigree
  • Water-Electrolyte Balance (drug effects)

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