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Recovery of hypothalamic-pituitary-adrenal axis in paediatric Cushing disease

Christina Tatsi1, Megan Neely2, Chelsi Flippo1

  • 1Section on Endocrinology and Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health, Bethesda, MD, USA.

Clinical Endocrinology
|July 30, 2020
PubMed

Insights

Postoperative adrenal insufficiency in children with Cushing disease (CD) is common. Earlier recovery of adrenal function after surgery is linked to a higher risk of CD recurrence, impacting patient follow-up.

Area of Science:

  • Pediatric Endocrinology
  • Neurosurgery
  • Endocrinology

Background:

  • Cushing disease (CD) in children often leads to temporary adrenal insufficiency (AI) post-surgery.
  • Understanding factors influencing AI duration and its link to CD recurrence is crucial for pediatric patient management.

Purpose of the Study:

  • To investigate clinical and biochemical factors affecting AI duration in pediatric CD patients post-transsphenoidal surgery (TSS).
  • To determine the correlation between AI recovery time and the risk of CD recurrence.

Main Methods:

  • Retrospective study of 130 pediatric CD patients followed for at least 3 months post-TSS.
  • Multivariable Cox proportional hazards analysis to assess factors influencing AI duration and recurrence risk.

Main Results:

  • 102 patients (78.5%) recovered adrenal function, with a median recovery time of 12.7 months.
  • Preoperative clinical and biochemical factors, except urinary free cortisol (UFC), did not significantly correlate with AI recovery time.
  • Longer time to adrenal recovery was associated with a decreased risk of CD recurrence (HR: 0.86).

Conclusions:

  • AI recovery in pediatric CD patients post-TSS is minimally influenced by preoperative factors, except for UFC.
  • Earlier recovery of adrenal function is paradoxically associated with an increased risk of CD recurrence.
  • These findings necessitate adjusted follow-up strategies and patient counseling for pediatric CD survivors.
Abstract

Keywords:
CushingHPA axisadrenal functionpituitaryrecoverytranssphenoidal surgery

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