Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2018 Jun;47(2):451-462.
doi: 10.1016/j.ecl.2018.02.008.

Cushing's Syndrome in Pediatrics: An Update

Maya B Lodish  1 , Margaret F Keil  2 , Constantine A Stratakis  1
Affiliations
Review

Cushing's Syndrome in Pediatrics: An Update

Maya B Lodish et al. Endocrinol Metab Clin North Am. 2018 Jun.

Abstract

Cushing syndrome (CS) is a multisystem disorder resulting from the prolonged exposure to excess glucocorticoids. In children, CS most commonly results from the exogenous administration of steroids and the typical presentation is height deceleration concomitant with weight gain. Endogenous and ectopic causes are rare. CS in children may be associated with distinct germline and somatic mutations. Clinical practice guidelines are available assist clinicians. Patients should be referred to multidisciplinary centers of excellence with experience in endocrinology and surgery. Early detection and treatment is essential to reduce associated acute and long-term morbidity and potential death.

Keywords: Adrenal cancer; Adrenal cortex; Adrenocortical hyperplasia; Carney complex; Cushing’s syndrome; Pituitary tumors.

PubMed Disclaimer

Figures

Fig 1
Fig 1
Typical growth chart for a child with Cushing syndrome Linear height deceleration with concomitant weight gain starting at age 8years. From Constantine A. Stratakis. Cushing Syndrome in Pediatrics. Endocrinology and Metabolism Clinics of North America. Volume 41, Issue 4, December 2012, Pages 793-803, with permission.
Fig 2
Fig 2
Diagnostic algorithm in Cushing syndrome Screening for hypercortsolemia includes 1mg overnight (ON) dexamethasone (DEX), 24hr urine free cortisol, or midnight salivary cortisol. ACTH level is used to differentiate ACTH-independent vs. ACTH-independent CS and appropriate testing to confirm etiology of CS. From Constantine A. Stratakis. Cushing Syndrome in Pediatrics. Endocrinology and Metabolism Clinics of North America. Volume 41, Issue 4, December 2012, Pages 793-803, with permission.
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

See all "Cited by" articles

References

    1. Sharma ST, Nieman LK, Feelders RA. Cushing’s syndrome: epidemiology and developments in disease management. Clin Epidemiol. 2015;7:281–293. - PMC - PubMed
    1. Magiakou MA, Mastorakos G, Oldfield EH, et al. Cushing’s syndrome in children and adolescents. Presentation, diagnosis, and therapy. N Engl J Med. 1994;331(10):629–636. - PubMed
    1. Orth DN. Cushing’s syndrome. N Engl J Med. 1995;332(12):791–803. - PubMed
    1. Batista DL, Riar J, Keil M, Stratakis CA. Diagnostic tests for children who are referred for the investigation of Cushing syndrome. Pediatrics. 2007;120(3):e575–586. - PubMed
    1. Nieman LK, Biller BM, Findling JW, et al. The diagnosis of Cushing’s syndrome: an Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2008;93(5):1526–1540. - PMC - PubMed