July 1965

Corticosteroid Metabolism in Cystic Fibrosis

Author Affiliations

From the Department of Pediatrics, University of Utah, Salt Lake City (Drs. Chodos and Ely), and University of Washington, Seattle (Drs. Limbeck and Kelley). Dr. Chodos is now with the Division of Clinical Research, The Upjohn Company, Kalamazoo, Mich.

Am J Dis Child. 1965;110(1):76-80. doi:10.1001/archpedi.1965.02090030082011

FEW STUDIES concerning adrenocortical function in children with cystic fibrosis of the pancreas have been reported. In two patients, di Sant'Agnese et al1 noted an increased excretion of urinary 17-ketosteroids (17-KS) in response to corticotropin, a normal reduction of urinary electrolyte excretion during salt restriction, and little response of sweat electrolytes to desoxycorticosterone administration. Siegenthaler et al2 have demonstrated normally elevated urinary aldosterone levels, but persistently pathological sweat electrolyte concentrations during salt restriction in one child, and normal control urinary aldosterone levels in four children with this disease. Recently, Lietman and di Sant'Agnese3 reported studies on ten patients with cystic fibrosis of the pancreas. These studies yielded the following observations: (1) normal urinary aldosterone levels during control and salt-restriction periods in eight patients, (2) the lack of a decrease in sweat electrolyte concentrations during salt restriction in all patients, and (3) normal oral glucose response tests, urinary

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