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Published on: September 15, 2017
Cushing's disease in childhood
1Department of Endocrinology, St Bartholomew's Hospital, London EC1A 7BE, UK.
Trends in Endocrinology and Metabolism: TEM
|August 1, 1996
Summary
Pediatric Cushing's disease, a rare condition causing excess cortisol, presents with weight gain and growth issues. Diagnosis involves specific tests, with transsphenoidal surgery being the primary treatment for effective outcomes.
Area of Science:
- Pediatric Endocrinology
- Neuroendocrinology
Background:
- Cushing's disease is rare in children, affecting males and females equally.
- It represents approximately 75% of pediatric Cushing's syndrome cases.
- Key symptoms include weight gain, growth failure, virilization, and headaches.
Purpose of the Study:
- To outline the diagnostic approach for pediatric Cushing's disease.
- To describe the primary therapeutic strategies for this condition.
Main Methods:
- Confirmation of inappropriate hypercortisolemia.
- Differential diagnosis using high-dose dexamethasone suppression test (>50% cortisol suppression) and corticotropin-releasing hormone (CRH) stimulation test.
- Pituitary imaging and inferior petrosal sinus sampling for ACTH to confirm pituitary source and lateralization.
Main Results:
- Diagnostic criteria include specific responses to dexamethasone and CRH tests.
- Pituitary microadenomas are found in a minority of cases.
- Inferior petrosal sinus sampling aids in tumor localization.
Conclusions:
- Transsphenoidal surgery is the primary treatment for pediatric Cushing's disease.
- Pituitary irradiation can be an adjunct therapy if hypercortisolemia persists.
- Effective treatment yields good therapeutic outcomes in experienced centers.
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