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Assessment and Evaluation of the High Risk Neonate: The NICU Network Neurobehavioral Scale
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Published on: August 25, 2014

Nelson's syndrome.

T M Barber1, E Adams, O Ansorge

  • 1Department of Endocrinology, Oxford Centre for Diabetes, Endocrinology and Metabolism, Churchill Hospital, University of Oxford, Headington, Oxford, UK.

European Journal of Endocrinology
|July 30, 2010
PubMed
Summary
This summary is machine-generated.

Nelson's syndrome, a serious condition post-adrenalectomy for Cushing's disease, needs clearer diagnostic criteria. Predictive factors include pituitary tumors and rising ACTH levels, with temozolomide showing therapeutic promise.

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Area of Science:

  • Endocrinology
  • Oncology
  • Neurosurgery

Background:

  • Nelson's syndrome is a serious complication after total bilateral adrenalectomy (TBA) for Cushing's disease.
  • Current diagnostic criteria for Nelson's syndrome are insufficient.
  • Management strategies require further investigation and standardization.

Purpose of the Study:

  • To review controversial aspects of Nelson's syndrome, including diagnosis, predictive factors, and management.
  • To propose new diagnostic criteria for Nelson's syndrome.
  • To highlight potential therapeutic agents like temozolomide.

Main Methods:

  • Literature review of existing data.
  • Analysis of tertiary center experience.
  • Proposal of new diagnostic criteria based on pituitary mass and ACTH levels.

Main Results:

  • Proposed diagnostic criteria include an expanding pituitary mass or elevated ACTH levels (>500 ng/l) with progressive elevations.
  • Predictive factors for Nelson's syndrome include residual pituitary tumors, aggressive tumors, lack of radiotherapy, and rapid ACTH rise.
  • Temozolomide shows promise for treating aggressive corticotroph tumors associated with Nelson's syndrome.

Conclusions:

  • Clearer diagnostic criteria for Nelson's syndrome are urgently needed.
  • Identifying predictive factors can aid in early detection and intervention.
  • Further research into therapeutic strategies, including temozolomide, is essential for effective management.