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Cushing Syndrome, Pediatric

Maya B. Lodish, MD, MHSCR and Constantine A. Stratakis, MD, D(med)Sci Reviewed 10/2018
 


BASICS

DESCRIPTION

Cushing syndrome is a multisystem disorder resulting from prolonged exposure to excess glucocorticoid. 
  • Characterized by growth deceleration, truncal obesity, characteristic skin chan...

DIAGNOSIS

HISTORY

  • The most common presenting symptom of the syndrome in childhood is weight gain accompanied by poor linear growth.

  • Additional pertinent features in the history of presenting illness oft...

TREATMENT

  • The treatment of choice for Cushing disease is transsphenoidal surgery (TSS).

  • In most specialized centers with experienced neurosurgeons, the success rate of the first TSS is close to, or eve...

ONGOING CARE

  • Following TSS in Cushing disease, patients will be transiently adrenally insufficient while the hypothalamic-pituitary-adrenal axis is recovering.

  • Stress doses of cortisol are necessary in...

ADDITIONAL READING

  • Batista DL, Riar J, Keil M, et al. Diagnostic tests for children who are referred for the investigation of Cushing syndrome. Pediatrics.  2007;120(3):e575–e586. [View Abstract ...

CODES

ICD9

255.0 Cushing’s syndrome 

ICD10

  • E24.9 Cushing’s syndrome, unspecified

  • E24.0 Pituitary-dependent Cushing’s disease

  • E24.2 Drug-induced Cushing’s syndrome

  • E24.3 Ectopic ACTH syndrome

  • E24.8 Other Cush...

FAQ

  • Q: What clinical clue is most useful to determine which children have obesity alone versus those with Cushing syndrome?

  • A: Cushing syndrome is associated with growth failure, whereas obesity is ass...

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