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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.

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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.
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