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Primary empty sella syndrome, ACTH hypersecretion, and normal adrenocortical function. Report of two cases
The American Journal of Medicine
|September 1, 1982
Summary
This study reports two unique cases of ACTH hypersecretion with primary empty sella syndrome and normal cortisol levels. This suggests a potential link between pituitary conditions and hormonal imbalances, even with normal adrenal function.
Area of Science:
- Endocrinology
- Neuroendocrinology
- Pituitary Disorders
Background:
- Primary empty sella syndrome (PESS) typically presents with normal endocrine function or mild pituitary insufficiency.
- Previous reports link ACTH hypersecretion and PESS to Cushing's or Addison's disease.
- Pituitary hormone hypersecretion in PESS is a recognized, though less common, clinical presentation.
Observation:
- Two unique patients presented with ACTH hypersecretion and PESS.
- Both patients exhibited normal cortisol dynamics, indicating normal adrenocortical function.
- This presentation differs from previously reported cases associated with overt adrenal dysfunction.
Findings:
- The study identified a novel association between ACTH hypersecretion, PESS, and normal adrenocortical function.
- Investigators hypothesize that infarction of hyperplastic corticotrophs, due to reduced biologic activity of ACTH peptides, may underlie this association.
- These findings highlight a specific subtype of PESS with unique hormonal characteristics.
Implications:
- These cases expand the understanding of potential endocrine dysfunctions associated with PESS.
- The findings suggest the need for careful hormonal evaluation in PESS patients, even with seemingly normal cortisol levels.
- Further research into the pathophysiology of ACTH hypersecretion in PESS is warranted to elucidate the role of ACTH peptide activity.