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Cushing Disease: Diagnosis and Treatment

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Pituitary Disorders of Childhood

Part of the book series: Contemporary Endocrinology ((COE))

Abstract

Pituitary corticotroph adenomas (causing Cushing disease, CD) are the most common cause of endogenous Cushing syndrome (CS) in children although their prevalence varies within age groups. The initial workup of a patient suspected to have CS involves the demonstration of hypercortisolemia and/or excess urinary free cortisol excretion, the loss of the normal circadian rhythm of cortisol production, and the lack of suppression of cortisol secretion after low-dose dexamethasone suppression test. The identification of the source of CS involves the measurement of the morning corticotropin (ACTH) levels, imaging studies (pituitary magnetic resonance imaging and adrenal gland computed tomography), and other tests, including high-dose dexamethasone suppression and corticotropin-releasing hormone (CRH) stimulation. The first-line management of CD is the transsphenoidal (TSS) resection of the pituitary adenoma. In cases where TSS fails, radiation and/or medical therapy and medical or surgical (bilateral) adrenalectomy may eventually lead to control of the hypercortisolemia.

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Acknowledgment

This work was supported by the intramural research program of the Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), NIH, Bethesda, MD20892, USA.

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  1. Eunice Kennedy Shriver, National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Bethesda, MD, USA

    Christina Tatsi & Constantine A. Stratakis

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  1. Christina Tatsi
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Correspondence to Constantine A. Stratakis .

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    Brenda Kohn

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Tatsi, C., Stratakis, C.A. (2019). Cushing Disease: Diagnosis and Treatment. In: Kohn, B. (eds) Pituitary Disorders of Childhood. Contemporary Endocrinology. Humana Press, Cham. https://doi.org/10.1007/978-3-030-11339-1_5

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