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Cardiovascular health, growth and gonadal function in children and adolescents with congenital adrenal hyperplasia
  1. Christiaan F Mooij1,2,
  2. Emma A Webb1,
  3. Hedi L Claahsen van der Grinten2,
  4. Nils Krone3
  1. 1Institute of Metabolism and Systems Research, Centre for Endocrinology, Diabetes and Metabolism, University of Birmingham, Birmingham, UK
  2. 2Department of Paediatric Endocrinology, Amalia Children's Hospital, Radboud University Medical Center, Nijmegen, The Netherlands
  3. 3Academic Unit of Child Health, Department of Oncology and Metabolism, University of Sheffield, Sheffield Children's Hospital, Sheffield, UK
  1. Correspondence to Dr Nils Krone, Academic Unit of Child Health, Department of Oncology and Metabolism, University of Sheffield, Sheffield Children's Hospital, Western Bank, Sheffield S10 2TH, UK; N.Krone{at}sheffield.ac.uk

Abstract

After the introduction of replacement therapy with glucocorticoids and mineralocorticoids in the 1950s, congenital adrenal hyperplasia (CAH) is no longer a life-limiting condition. However, due to the successful introduction of medical steroid hormone replacement, CAH has become a chronic condition, with associated comorbidities and long-term health implications. The aim of treatment is the replacement of mineralocorticoids and glucocorticoids and the normalisation of elevated androgen concentrations. Long-term consequences of the condition and current treatment regimens include unfavourable changes in the cardiovascular risk profile, impaired growth, testicular adrenal rest tumours (TART) in male and subfertility in both male and female patients with CAH. Optimising replacement therapy in patients with CAH remains challenging. On one hand, treatment with supraphysiological doses of glucocorticoids might be required to normalise androgen concentrations and decrease size or presence of TARTs. On the other hand, treatment with supraphysiological doses of glucocorticoids is associated with an increased prevalence of unfavourable cardiovascular and metabolic risk profiles as well as impaired longitudinal growth and gonadal function. Therefore, treatment of children and adults with CAH requires an individualised approach. Careful monitoring for early signs of complications is already warranted during paediatric healthcare provision to prevent and reduce the impact of comorbidities in later life.

  • endo
  • Growth
  • Vascular Disease

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Footnotes

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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