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May 1955

Congential Renal Tubular Dysfunction (nephrogenic Diabetes Insipidus): Report of a Case Complicated by Calcifications in the Renal Pedicle

AMA Am J Dis Child. 1955;89(5):602-605. doi:10.1001/archpedi.1955.02050110716014

In their discussion of diabetes insipidus, Holt and McIntosh1 consider the pathogenesis of this syndrome as being due to either intracranial pathology, a defect in renal function, or a spurious diabetes insipidus due to transient excessive fluid intake. In recent years several unusual cases of polyuria unaffected by vasopressin injection (Pitressin) have been described. They have been reported under various titles, such as "A Congenital Defect of Water Metabolism"2; "Nephrogenic Diabetes Insipidus"3; "Congenital Diabetes Insipidus Resistant to Treatment with Pitressin,"4 and "Diabetes Insipidus in Infancy Resistant to Pitressin."5 All of these cases represent instances of diabetes insipidus in which excessive volumes of urine of low specific gravity are excreted due to defective renal tubular physiology.

This report deals with a new member of the family discussed by Williams and Henry (Fig. 1) who had, in addition to the functional renal disability, peculiar calcific deposits in