Cushing Syndrome in Childhood

  • Maya Lodish
  • Margaret F. Keil
  • Constantine A. Stratakis
Chapter

Abstract

Cushing syndrome is a multisystem disorder resulting from the body’s prolonged exposure to excess glucocorticoids. It is characterized by truncal obesity, growth deceleration, striae, muscle weakness, and hypertension. Most commonly, Cushing syndrome in childhood results from the exogenous administration of glucocorticoids. In this chapter, we present the causes and discuss the treatment of endogenous Cushing syndrome. The most common cause of endogenous Cushing syndrome in children is adrenocorticotropin overproduction from a pituitary adenoma, known as Cushing disease, accounts for approximately 75% of all cases of Cushing syndrome in children older than 7 years. Cushing syndrome may also be due to adrenal hypersecretion of cortisol secondary to carcinoma, adenoma, or hyperplasia, especially in younger children. Finally, the most infrequent form of Cushing syndrome is due to ectopic secretion of adrenocorticotropin and/or corticotropin releasing factor. Genetic causes of Cushing syndrome are increasingly identified, including somatic and germline mutations. Early identification and treatment of Cushing syndrome is imperative for successful treatment and optimization of growth and cardiovascular health.

Keywords

Cushing syndrome Adrenal Pituitary ACTH dependent ACTH independent Adenoma Tumor Hyperplasia Cancer Corticotropinoma 

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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Maya Lodish
    • 1
  • Margaret F. Keil
    • 2
  • Constantine A. Stratakis
    • 3
  1. 1.Pediatric EndocrinologySection on Endocrinology and Genetics, National Institute of Child Health and Human Development, National Institutes of HealthBethesdaUSA
  2. 2.Clinical Center, National Institutes of HealthBethesdaUSA
  3. 3.National Institute of Child Health and Human Development, Section on Genetics and Endocrinology (SEGEN), National Institutes of HealthBethesdaUSA

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