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Related Experiment Videos

Primary cortisol resistance presenting as isosexual precocity.

C D Malchoff1, E C Javier, D M Malchoff

  • 1Department of Medicine, University of Connecticut Health Center, Farmington 06032.

The Journal of Clinical Endocrinology and Metabolism
|February 1, 1990
PubMed
Summary

Primary cortisol resistance (PCR) is a rare condition causing high cortisol levels without typical symptoms. This case study highlights PCR in a boy with early puberty due to abnormal glucocorticoid receptors.

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Area of Science:

  • Endocrinology
  • Genetics
  • Pediatrics

Background:

  • Primary cortisol resistance (PCR) is an uncommon endocrine disorder characterized by the body's reduced response to cortisol.
  • It typically presents with hypercortisolism but often lacks the overt clinical signs of Cushing's syndrome.

Observation:

  • A pediatric case presented with isosexual precocity, advanced bone age, and accelerated linear growth.
  • Despite markedly elevated serum cortisol levels, the patient exhibited no features of glucocorticoid excess.
  • Elevated adrenal androgens and testosterone were noted, alongside normal gonadotropin levels.

Findings:

  • The patient demonstrated resistance to dexamethasone suppression of both cortisol and adrenal androgens.
  • Analysis revealed functionally abnormal glucocorticoid receptors with increased dissociation constant (Kd) but normal binding capacity.

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  • These findings confirm primary cortisol resistance as the underlying cause of hypercortisolism and precocious puberty.

    • Implications:

    • This case underscores that primary cortisol resistance can manifest with isosexual precocity in males.
    • It suggests that increased adrenal androgen production, driven by ACTH stimulation in PCR, can cause precocious puberty.
    • Consideration of PCR is crucial in pediatric cases of precocious puberty, particularly in males with isosexual precocity and females with heterosexual precocity.