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Cushing syndrome.

Lynnette K Nieman1, Frederic Castinetti2,3, John Newell-Price4

  • 1Section on Translational Endocrinology, Diabetes, Endocrine and Obesity Branch, National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK), National Institutes of Health, Bethesda, MD, USA. NiemanL@nih.gov.

Nature Reviews. Disease Primers
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Summary
This summary is machine-generated.

Cushing syndrome (CS) is a hormonal disorder from excess cortisol. Diagnosis involves specific tests, and treatment focuses on surgery or medication to manage this serious condition.

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Area of Science:

  • Endocrinology
  • Internal Medicine

Background:

  • Cushing syndrome (CS) results from prolonged exposure to excess glucocorticoids, either endogenous or exogenous.
  • It causes significant multisystem morbidity, impacting cardiovascular, metabolic, infectious, and neuropsychiatric health, and increases mortality.
  • Variable clinical presentation can delay diagnosis until numerous signs and symptoms accumulate.

Purpose of the Study:

  • To outline the diagnostic approaches and therapeutic strategies for Cushing syndrome.
  • To emphasize the importance of identifying the underlying cause of endogenous CS for effective management.

Main Methods:

  • Screening for suspected CS includes 24-hour urine cortisol, late-night salivary cortisol, and low-dose dexamethasone suppression tests.
  • Diagnostic workup aims to determine the etiology: adrenal CS, pituitary Cushing's disease (CD), or ectopic ACTH secretion.

Main Results:

  • First-line treatment for endogenous CS is surgical resection of the causative tissue (pituitary adenoma, ectopic tumor, or adrenal tumor).
  • Second-line options include steroidogenesis inhibitors, pituitary radiation for CD, and adrenalectomy for persistent ACTH-dependent CS.

Conclusions:

  • Prompt diagnosis and etiological determination are crucial for managing Cushing syndrome.
  • Treatment strategies are tailored to the cause, with surgery being the primary approach, followed by medical or radiation therapies.