Cushing's Syndrome in Pediatrics: An Update

Maya B Lodish; Margaret F Keil; Constantine A Stratakis

doi:10.1016/j.ecl.2018.02.008

Cushing's Syndrome in Pediatrics: An Update

Endocrinol Metab Clin North Am. 2018 Jun;47(2):451-462. doi: 10.1016/j.ecl.2018.02.008.

Authors

Maya B Lodish¹, Margaret F Keil², Constantine A Stratakis¹

Affiliations

¹ Section on Endocrinology and Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), NIH-Clinical Research Center, 10 Center Drive, Building 10, Room 1E-3330, MSC1103, Bethesda, MD 20892, USA.
² Section on Endocrinology and Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), NIH-Clinical Research Center, 10 Center Drive, Building 10, Room 1E-3330, MSC1103, Bethesda, MD 20892, USA. Electronic address: keilm@mail.nih.gov.

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.

Publication types

Review

MeSH terms

Child
Cushing Syndrome / complications
Cushing Syndrome / diagnosis*
Cushing Syndrome / etiology
Cushing Syndrome / therapy*
Humans

Grants and funding

ZIA HD008920-01/Intramural NIH HHS/United States