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Endocrine Abstracts (2019) 63 S19.3 | DOI: 10.1530/endoabs.63.S19.3

ECE2019 Symposia Adrenal insufficiency (3 abstracts)

Optimising care delivery in congenital adrenal hyperplasia

Nils Krone


UK.


Congenital adrenal hyperplasia represents a group of autosomal recessive disorders in steroidogenesis causing deficient cortisol biosynthesis. The incidence of congenital adrenal hyperplasia (CAH) in the general population of Western countries is approximately 1 in 10,000 to 1 in 15,000 life births with about 95% of cases caused by 21-hydroxylase deficiency. After the introduction of replacement therapy with glucocorticoids and mineralocorticoids in the 1950s, congenital adrenal hyperplasia is no longer a life-limiting condition. However, CAH has become a chronic life-long condition, with associated comorbidities and long-term health implications. Long-term consequences of CAH and current treatment regimens include unfavourable outcomes involving all organ systems. Comprehensive long-term data on mortality are not available. An important emerging task for paediatric health care provision in CAH is the prevention of long-term health problems. Pharmacotherapy with current approaches remains challenging, with keeping the balance between glucocorticoid overexposure and androgen excess. Over the life-span type and dose of glucocorticoid can change. The relative mineralocorticoid dose per body surface area declines with increasing age. Replacement doses are monitored by clinical and biochemical parameters, with suppression of steroid hormones commonly indicating overtreatment. Several novel experimental pharmacotherapies with the aim to reduce glucocorticoid exposure and to mimic physiological glucocorticoid secretion are under development. The approach to treatment and monitoring remains highly variable and the effects on long-term outcomes remain elusive. This presentation will provide an update on current developments covering care provision, novel adrenal-specific biomarkers with the potential to improve monitoring and experimental approaches. All of these ongoing approaches have will in theory improve CAH management; however, the evidence for improved long-term outcomes is lacking.

Volume 63

21st European Congress of Endocrinology

Lyon, France
18 May 2019 - 21 May 2019

European Society of Endocrinology 

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