Abstract
INTRODUCTION THE familial occurrence of diabetes insipidus was first reported over one hundred years ago by La Combe in 1841, as cited by Forssman (1). At that time Mendel's laws of inheritance had not yet been described. Since then, about 35 reports with case analyses and family trees have appeared in the literature, primarily from Europe. The studies carried out by the Weils, Sr. and Jr., and summarized by Camerer (2) in 1935, are a classic reference on the mode of transmission of this anomaly, stressing the presence of a dominant gene. More recent comprehensive surveys are those of Forssman in Sweden in 1945 (1) and Pender and Fraser in Canada in 1953 (3). There have been only a few reports from this country (4–6), and it is the purpose of this paper to present a family history in which individuals of seven generations were affected by the disease and to discuss the genetic aspects of inherited diabetes insipidus.
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