Nephrogenic Diabetes Insipidus in North America — TheHopewellHypothesis

Nephrogenic diabetes insipidus is prevalent among the descendants of the so-called Ulster Scot who settled in Nova Scotia during the eighteenth century. The folklore description of its mode of inheritance implies X-linked transmission. This is in conformity with analyses of previously published data and five new pedigrees. In the latter all 12 sons of eight affected males were healthy, but each of their 21 examined daughters were heterozygotes. Among the 221 offspring of 53 heterozygous females close to the expected half (121) were gene carriers. Historical data suggest that female carriers were among the 1761 settlers arriving at Halifax aboard the ship Hopewell. Since it has been possible to link both the new and several previously published pedigrees from other parts of the Continent to these immigrants, it is tempting to propose that most North American patients suffering from this disorder are among the progeny of the Ulster Scot.