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July 1970

Nephrogenic Diabetes Insipidus in a Negro Kindred

Author Affiliations

St. Louis
From the departments of pediatrics (Drs. Feigin, Rimoin, and Kaufman) and medicine (Drs. Rimoin and Kaufman), Washington University School of Medicine, and the divisions of infectious diseases (Dr. Feigin) and medical genetics (Drs. Rimoin and Kaufman), St. Louis Children's Hospital, St. Louis.

Am J Dis Child. 1970;120(1):64-68. doi:10.1001/archpedi.1970.02100060098016

Nephrogenic diabetes insipidus was first called to medical attention in 1945, by Waring et al1 who described six patients with this disorder. In 1947, Williams and Henry2 noted that the infusion of antidiuretic hormone in doses sufficient to produce systemic side effects failed to correct the defect and proposed the theory of end-organ resistance. Bode and Crawford3 recently reported the results of a careful geneological survey and hypothesized that most, "if not all, of the persons with nephrogenic diabetes insipidus are among the posterity of the original settlers in Nova Scotia." The descendents of these Ulster Scotsmen are now scattered throughout the United States. On the basis of their study, Bode and Crawford3 concluded that new mutations giving rise to the disorder appear to be exceedingly rare.

The purpose of this paper is to report four cases of nephrogenic diabetes insipidus in a Negro kindred. We