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...

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