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International Journal of Pediatrics
Volume 2011, Article ID 784867, 3 pages
Case Report

Severe Hypoglycemia due to Isolated ACTH Deficiency in Children: A New Case Report and Review of the Literature

Department of Pediatrics (M.Y.T., G.W.M.), and Department of Radiology (R.W.), University of Mississippi Medical Center, Jackson, MS 39216, USA

Received 10 December 2010; Accepted 31 January 2011

Academic Editor: Sunit C. Singhi

Copyright © 2011 Michael Y. Torchinsky et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Isolated ACTH deficiency causes life-threatening severe hypoglycemia. A 7-year-old girl with hypoglycemia due to this rare disorder is described. Our patient had undetectable plasma ACTH repeatedly and cortisol 0 mcg/dl before and after ACTH 1-24 stimulation. There was no evidence of other pituitary hormone deficiency. Glucocorticoid replacement therapy resulted in resolution of all symptoms and normalization of blood glucose. Previously published data on isolated ACTH deficiency in children is summarized. Review of the literature showed that the prevalence of this condition could be underestimated in the neonatal period and in Prader-Willi syndrome. Isolated ACTH deficiency occurs in older children as well as in neonates.