Management aspects of congenital adrenal hyperplasia during adolescence and transition to adult care

Chamila Balagamage, Amynta Arshad, Yasir S. Elhassan, Wogud Ben Said, Ruth E. Krone, Helena Gleeson, Jan Idkowiak*

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

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Abstract

The adolescent period is characterised by fundamental hormonal changes, which affect sex steroid production, cortisol metabolism and insulin sensitivity. These physiological changes have a significant impact on patients with congenital adrenal hyperplasia (CAH). An essential treatment aim across the lifespan in patients with CAH is to replace glucocorticoids sufficiently to avoid excess adrenal androgen production but equally to avoid cardiometabolic risks associated with excess glucocorticoid intake. The changes to the hormonal milieu at puberty, combined with poor adherence to medical therapy, often result in unsatisfactory control exacerbating androgen excess and increasing the risk of metabolic complications due to steroid over‐replacement. With the physical and cognitive maturation of the adolescent with CAH, fertility issues and sexual function become a new focus of patient care in the paediatric clinic. This requires close surveillance for gonadal dysfunction, such as irregular periods/hirsutism or genital surgery‐associated symptoms in girls and central hypogonadism or testicular adrenal rest tumours in boys. To ensure good health outcomes across the lifespan, the transition process from paediatric to adult care of patients with CAH must be planned carefully and early from the beginning of adolescence, spanning over many years into young adulthood. Its key aims are to empower the young person through education with full disclosure of their medical history, to ensure appropriate follow‐up with experienced physicians and facilitate access to multispecialist teams addressing the complex needs of patients with CAH.
Original languageEnglish
Article number14992
Number of pages14
JournalClinical Endocrinology
Early online date14 Nov 2023
DOIs
Publication statusE-pub ahead of print - 14 Nov 2023

Bibliographical note

Research Funding:
Academy of Medical Sciences. Grant Number: Starter Grant for Clinical Lecturers SGL020\1013 t.

Keywords

  • androgens
  • 21‐hydroxylase deficiency
  • cortisol
  • glucocorticoid
  • puberty

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