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Cushing Syndrome

Abstract

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NORD is very grateful to Lynnette Nieman, MD, FACP, Senior Investigator, Intramural Research Program on Reproductive and Adult Endocrinology, The Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD); Chief, Endocrinology Consultation Service, National Institutes of Health and Constantine A. Stratakis, MD, D(med)Sci, Scientific Director, The Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health, for assistance in the preparation of this report.

Synonyms of Cushing Syndrome

  • hypercortisolism

Disorder Subdivisions

  • endogenous Cushing syndrome
  • exogenous Cushing syndrome

General Discussion

Summary
Cushing syndrome is a rare endocrine disorder characterized by a variety of symptoms and physical abnormalities that occur due to excessive amounts of the hormone cortisol, a type of glucocorticoid. Glucocorticoids are a class of steroid hormones that are important in the regulation of the metabolism of glucose and also modulate the response to stress. Cushing syndrome most commonly affects adults between the ages of 25 to 40. It can be caused by prolonged exposure to elevated levels of glucocorticoids produced within the body (endogenous) or introduced from outside the body (exogenous). Symptoms can include upper body obesity, a rounded face, thin purple streaks (purple striae) which occur on the skin, increased fat around the neck, and slender arms and legs. Children with Cushing syndrome are typically obese with slowed growth rates.

Introduction
In 1912, Harvey Cushing described a patient with hypercorticism but assumed it to be a polyglandular disorder. The cause was disputed for almost 40 years. Cushing disease, which is pituitary adrenocorticotropin hormone (ACTH) dependent Cushing syndrome, was first described by Dr. Cushing in 1932. Though pituitary surgery was introduced in the early 20th century, it was not until 1933 that neurosurgery was performed on the first patient with Cushing disease.

Cushing Syndrome Resources

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