Management of adolescents with congenital adrenal hyperplasia. Review uri icon

Overview

abstract

  • The management of congenital adrenal hyperplasia involves suppression of adrenal androgen production, in addition to treatment of adrenal insufficiency. Management of adolescents with congenital adrenal hyperplasia is especially challenging because changes in the hormonal milieu during puberty can lead to inadequate suppression of adrenal androgens, psychosocial issues often affect adherence to medical therapy, and sexual function plays a major part in adolescence and young adulthood. For these reasons, treatment regimen reassessment is indicated during adolescence. Patients with non-classic congenital adrenal hyperplasia require reassessment regarding the need for glucocorticoid drug treatment. No clinical trials have compared various regimens for classic congenital adrenal hyperplasia in adults, thus therapy is individualised and based on the prevention of adverse outcomes. Extensive patient education is key during transition from paediatric care to adult care and should include education of females with classic congenital adrenal hyperplasia regarding their genital anatomy and surgical history. Common issues for these patients include urinary incontinence, vaginal stenosis, clitoral pain, and cosmetic concerns; for males with classic congenital adrenal hyperplasia, common issues include testicular adrenal rest tumours. Transition from paediatric to adult care is most successful when phased over many years. Education of health-care providers on how to successfully transition patients is greatly needed.

publication date

  • November 15, 2013

Research

keywords

  • Adrenal Hyperplasia, Congenital
  • Disease Management

Identity

PubMed Central ID

  • PMC4163910

Scopus Document Identifier

  • 84887568540

Digital Object Identifier (DOI)

  • 10.1016/j.jpurol.2013.03.013

PubMed ID

  • 24622419

Additional Document Info

volume

  • 1

issue

  • 4