What is the relationship between Allgrove syndrome and chronic Addison disease?

Updated: Mar 11, 2020
  • Author: George T Griffing, MD; Chief Editor: Romesh Khardori, MD, PhD, FACP  more...
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Answer

Allgrove syndrome [31, 32]

  • Although patients with congenital adrenocortical unresponsiveness to ACTH (Allgrove syndrome) may present with features of glucocorticoid deficiency and skin hyperpigmentation, the aldosterone production and function in these patients is normal and responds appropriately to low sodium intake.

  • This typically presents in childhood with failure to thrive, features of adrenocortical insufficiency, and hypoglycemia.

  • Some patients may have components of alacrima and achalasia. [33] It is also sometimes called triple A syndrome.


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