Abstract
Genetically determined diabetes insipidus occurs in several different forms. One form is inherited as a simple dominant. Two other forms are caused by 2 different mutations in the X chromosome, one of which shows the usual susceptibility to antidiuretic hormone (ADH). In this paper the other form of sex-linked diabetes insipidus, which appeared to be an independent genotypic entity, was presented. A family containing 9 known cases in 4 generations was described. Four of the 9 were tested for their reaction to ADH, but there was a complete lack of response in each case.

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