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- Jason Klein, MD
- Patricia Vuguin, MD, MSc
- Sharon Hyman, MD
- Department of Pediatric Endocrinology, Cohen Children’s Medical Center of New York, New Hyde Park, NY
AUTHOR DISCLOSURE
Drs Klein, Vuguin, and Hyman have disclosed no financial relationships relevant to this article. This commentary does not contain discussion of unapproved/investigative use of a commercial product/device.
Cushing syndrome (CS) is a state of glucocorticoid excess in which there is loss of the normal hypothalamic-pituitary-adrenal feedback axis. First described in 1912 by American neurosurgeon Dr Harvey Williams Cushing in reference to a 23-year-old patient with obesity, hirsutism, and amenorrhea, CS has since been recognized as a rare, yet serious and significant, condition in the pediatric population.
Under normal circumstances, hypothalamic corticotropin-releasing hormone (CRH) is delivered to the anterior pituitary via the portal circulation, resulting in adrenocorticotropin hormone (ACTH) release from the anterior pituitary into the systemic circulation. ACTH acts on the zona fasciculata of the adrenal cortex to stimulate release of cortisol. The secretion of cortisol results in negative feedback, downregulating production of both ACTH and CRH. Cortisol has metabolic and cardiovascular effects. It plays an important role in hepatic glucose metabolism by increasing gluconeogenesis and glycogenolysis, as well as affecting proteolysis and lipolysis. In addition, it influences myocardial contractility, cardiac output, and blood pressure. It also has a variety of effects on the immunologic and inflammatory systems, musculoskeletal and connective tissue, and fluid and electrolyte homeostasis, as well as neuropsychiatric, behavioral, gastrointestinal, and developmental effects. In the general population, there are approximately 2 to 5 new cases per million per year of CS; only approximately 10% of these cases occur in the pediatric population. Although adolescents with CS, similar to adults, have a slight female to male predominance, this predilection goes away in younger patients and may even be reversed in infants with CS.
Pediatric CS can be divided into 2 distinct categories: ACTH dependent and ACTH independent (Table 1). The most common cause of CS is …
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