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Related Experiment Videos

[Primary adrenocortical micronodular dysplasia].

M Dumić1, J Ille, S Batinica

  • 1Klinika za pedijatriju, KBC Rebro, Zagreb.

Lijecnicki Vjesnik
|June 23, 1999
PubMed
Summary

Primary adrenocortical micronodular dysplasia (PAMD) caused Cushing's syndrome in two girls. Bilateral adrenalectomy was performed, with successful hormone replacement therapy, and one patient met Carney complex criteria.

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Area of Science:

  • Endocrinology
  • Pediatric Endocrinology
  • Pathology

Context:

  • Cushing's syndrome in pediatric patients is rare.
  • Primary adrenocortical micronodular dysplasia (PAMD) is an uncommon cause of cortisol excess.
  • Carney complex is a rare genetic disorder with multiple endocrine and non-endocrine manifestations.

Purpose:

  • To present two pediatric cases of Cushing's syndrome secondary to PAMD.
  • To highlight the diagnostic and therapeutic challenges in managing PAMD.
  • To discuss the association of PAMD with Carney complex.

Summary:

  • Two girls, aged 11 and 13, presented with Cushing's syndrome due to PAMD, characterized by independent adrenal cortisol hypersecretion.
  • Diagnostic findings included high plasma cortisol, elevated urinary free cortisol and 17-ketogenic steroids, with low/normal ACTH.

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  • Both patients underwent bilateral adrenalectomy and subsequent hormone replacement therapy. Histopathology revealed characteristic small adrenal nodules.
  • The younger patient also met the criteria for Carney complex due to the presence of lentigines alongside PAMD.

    • Impact:

    • This case report contributes to the understanding of PAMD as a cause of pediatric Cushing's syndrome.
    • It emphasizes the importance of considering PAMD in the differential diagnosis of pediatric Cushing's syndrome.
    • The findings underscore the need for comprehensive evaluation, including genetic assessment for Carney complex in affected individuals.