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A Novel Method: Super-selective Adrenal Venous Sampling
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Published on: September 15, 2017

Postoperative testing to predict recurrent Cushing disease in children.

Dalia L Batista1, Edward H Oldfield, Margaret F Keil

  • 1Section on Endocrinology and Genetics, Program in Developmental Endocrinology and Genetics, National Institute of Child Health and Human Development, National Institutes of Health, 10 Center Drive, Bethesda, MD 20892, USA.

The Journal of Clinical Endocrinology and Metabolism
|May 28, 2009
PubMed
Summary

Postoperative testing after transsphenoidal surgery for Cushing disease in children is crucial. Morning ACTH and cortisol levels, along with ovine CRH tests, effectively predict disease recurrence.

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Area of Science:

  • Pediatric Endocrinology
  • Neurosurgery
  • Endocrinology

Background:

  • Postoperative testing after transsphenoidal surgery (TSS) for Cushing disease (CD) in children is not well-studied.
  • The usefulness of these tests in predicting residual disease or recurrence needs further investigation.

Purpose of the Study:

  • To identify the most effective postoperative test for identifying non-cured patients or predicting relapse in pediatric CD.
  • To compare the efficacy of plasma ACTH, serum cortisol, urinary free cortisol (UFC), and ovine CRH (oCRH) stimulation tests.

Main Methods:

  • Retrospective review of clinical data from 72 children with CD who underwent TSS.
  • Postoperative testing included plasma ACTH, serum cortisol, UFC, and oCRH stimulation tests.
  • Patients were followed for 24-120 months to assess remission and recurrence.

Main Results:

  • 94% of children achieved sustained remission after TSS.
  • Low UFC levels were not reliable predictors of sustained remission.
  • Morning ACTH and cortisol levels, and oCRH stimulation tests, significantly predicted disease recurrence (P < 0.001).

Conclusions:

  • Morning ACTH and cortisol levels and oCRH stimulation tests are valuable predictors of recurrence in pediatric CD patients post-TSS.
  • Urinary free cortisol (UFC) levels are not effective in predicting sustained remission.
  • Factors associated with relapse include lack of histological confirmation, normal initial hormone levels, normal oCRH response, and short-term glucocorticoid replacement.