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Cushing disease in pediatrics: an update.

Marcio José Concepción-Zavaleta1, Cristian David Armas2, Juan Eduardo Quiroz-Aldave3

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Summary

Cushing disease (CD) in children, caused by pituitary adenomas, hinders growth and development. Early diagnosis and treatment are crucial for managing hypercortisolism and improving outcomes in pediatric patients.

Keywords:
Cushing diseaseCushing syndromeDiagnosisNeuroendocrinologyTreatment

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Area of Science:

  • Pediatric Endocrinology
  • Neuroendocrinology
  • Oncology

Background:

  • Cushing disease (CD) is a primary cause of endogenous Cushing syndrome (CS), driven by ACTH-producing pituitary adenomas.
  • In pediatric patients, CD significantly impacts growth and development due to hypercortisolism.
  • Key childhood CS symptoms include weight gain, facial changes, hirsutism, virilization, and acne.

Purpose of the Study:

  • To provide a comprehensive overview of Cushing disease in the pediatric population.
  • To summarize current knowledge on the pathophysiology, diagnosis, and treatment of pediatric CD.
  • To highlight the challenges and importance of early management in children.

Main Methods:

  • This narrative review synthesizes existing literature on pediatric Cushing disease.
  • It covers diagnostic criteria, including biochemical tests and pathological confirmation.
  • Treatment modalities such as surgery, medication, and radiotherapy are discussed.

Main Results:

  • Diagnostic confirmation relies on establishing endogenous hypercortisolism and ACTH dependence, followed by pathology.
  • Treatment aims to normalize cortisol levels and alleviate clinical signs and symptoms.
  • Management strategies encompass surgical, medical, radiotherapeutic, or combined approaches.

Conclusions:

  • Cushing disease in children presents unique challenges due to its effects on growth and pubertal development.
  • Early diagnosis and intervention are essential for controlling hypercortisolism and optimizing prognosis.
  • The rarity of pediatric CD necessitates increased physician experience and awareness for effective management.