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Corticotropin Deficiency in Russell-Silver Syndrome-Reply

SUZANNE B. CASSIDY, MD; DENNIS E. CAREY, MD
Am J Dis Child. 1986;140(9):857. doi:10.1001/archpedi.1986.02140230026018.
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In Reply.—Drs Morris and Reiter have stated their concern that corticotropin deficiency may have been inadequately documented in a child with Russell-Silver syndrome and hypopituitarism.1 A prolonged metyrapone test was performed according to the method of Limal et al,2 in which metyrapone was administered orally every four hours for six doses; serum for cortisol and 11-desoxycortisol measurements was obtained at 8 AM, four hours after the last dose.2 The postmetyrapone serum 11-desoxycortisol level was 3.3 μg/dL (0.096 μmol/L) in our patient, compared with a range of 12.5 to 28.2 μg/dL (0.363 to 0.819 μmol/L) in control subjects, while the serum cortisol level was 9 μg/dL (250 nmol/L), compared with 1.9 to 6.0 μg/dL (50 to 160 nmol/L). In the face of well-documented growth hormone and thyrotropin deficiencies, the history of recurrent episodes of prostration, and the toleration of all doses of metyrapone, the test was interpreted

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