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Current and Novel Approaches to Children and Young People with Congenital Adrenal Hyperplasia and Adrenal Insufficiency Webb, Emma A.; Krone, Nils

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Current and Novel Approaches to Children and Young People with Congenital Adrenal Hyperplasia and Adrenal Insufficiency Webb, Emma A.; Krone, Nils University of Birmingham Current and novel approaches to children and young people with congenital adrenal hyperplasia and adrenal insufficiency Webb, Emma A.; Krone, Nils DOI: 10.1016/j.beem.2015.04.002 License: Other (please specify with Rights Statement) Document Version Peer reviewed version Citation for published version (Harvard): Webb, EA & Krone, N 2015, 'Current and novel approaches to children and young people with congenital adrenal hyperplasia and adrenal insufficiency', Best practice & research. Clinical endocrinology & metabolism. https://doi.org/10.1016/j.beem.2015.04.002 Link to publication on Research at Birmingham portal Publisher Rights Statement: NOTICE: this is the author’s version of a work that was accepted for publication. Changes resulting from the publishing process, such as peer review, editing, corrections, structural formatting, and other quality control mechanisms may not be reflected in this document. Changes may have been made to this work since it was submitted for publication. A definitive version was subsequently published as Webb EA, Krone N, Current and novel approaches to children and young people with congenital adrenal hyperplasia and adrenal insufficiency, Best Practice & Research Clinical Endocrinology & Metabolism (2015), doi: 10.1016/j.beem.2015.04.002. General rights Unless a licence is specified above, all rights (including copyright and moral rights) in this document are retained by the authors and/or the copyright holders. 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Take down policy While the University of Birmingham exercises care and attention in making items available there are rare occasions when an item has been uploaded in error or has been deemed to be commercially or otherwise sensitive. If you believe that this is the case for this document, please contact [email protected] providing details and we will remove access to the work immediately and investigate. Download date: 28. Sep. 2021 Accepted Manuscript Current and novel approaches to children and young people with congenital adrenal hyperplasia and adrenal insufficiency Dr. Emma A. Webb, PhD MRCPCH MBBS BSc, Dr. Nils Krone, MD FRCPCH PII: S1521-690X(15)00025-1 DOI: 10.1016/j.beem.2015.04.002 Reference: YBEEM 1023 To appear in: Best Practice & Research Clinical Endocrinology & Metabolism Please cite this article as: Webb EA, Krone N, Current and novel approaches to children and young people with congenital adrenal hyperplasia and adrenal insufficiency, Best Practice & Research Clinical Endocrinology & Metabolism (2015), doi: 10.1016/j.beem.2015.04.002. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. ACCEPTED MANUSCRIPT Current and novel approaches to children and young people with congenital adrenal hyperplasia and adrenal insufficiency Emma A. Webb, Nils Krone Dr Emma Webb PhD MRCPCH MBBS BSc School of Clinical & Experimental Medicine, University of Birmingham Institute of Biomedical Research, Room 225 Birmingham B15 2TT MANUSCRIPT Tel: 0121 415 8705 Email: [email protected] Corresponding author: Dr Nils Krone MD FRCPCH School of Clinical & Experimental Medicine, University of Birmingham Institute of Biomedical Research, Room 225 Birmingham B15 2TT Tel: 0121 415 8705 Email: [email protected] ACCEPTED Key words: congenital adrenal hyperplasia, adrenal insufficiency, children, adolescence, novel, therapy 1 ACCEPTED MANUSCRIPT Summary Congenital adrenal hyperplasia (CAH) represents a group of autosomal recessives conditions leading to glucocorticoid deficiency. CAH is the most common cause of adrenal insufficiency (AI) in the paediatric population. The majority of the other forms of primary and secondary adrenal insufficiency are rare conditions. It is critical to establish the underlying aetiology of each specific condition as a wide range of additional health problems specific to the underlying disorder can be found. Following the introduction of life-saving glucocorticoid replacement sixty years ago, steroid hormone replacement regimes have been refined leading to significant reductions in glucocorticoid doses over the last two decades. These adjustments are made with the aim both of improving the current management of children and young persons and of reducing future health problems in adult life. However despite optimisation of existing glucocorticoid replacement regimens fail to mimic the physiologic circadian rhythm of glucocorticoid secretion, current efforts therefMANUSCRIPTore focus on optimising replacement strategies. In addition, in recent years novel expe rimental therapies been developed which target adrenal sex steroid synthesis in patients with CAH aiming to reduce co-morbidities associated with sex steroid excess. These developments will hopefully improve the health status and long-term outcomes in patients with congenital adrenal hyperplasia and adrenal insufficiency. ACCEPTED 2 ACCEPTED MANUSCRIPT Adrenal steroidogenesis The cells forming the adrenal cortex originate from the intermediate mesoderm and differentiate under the influence of various transcription factors during pregnancy and postnatal life. During foetal life and up to 12 months of age, two distinct zones are evident, an inner prominent foetal zone and an outer definitive zone that differentiates into the adult adrenal gland. After birth, the fetal zone regresses and the definitive zone, which contains an inner zona fasciculata and an outer zona glomerulosa, proliferates. The innermost zone, the zona reticularis, becomes evident after 2 years of life. These form three major functionally distinct parts of the adrenal cortex: the outer zona glomerulosa synthesizes mineralocorticoids, the middle zona fasciculata produces glucocorticoids, and the inner zona reticularis synthesises the androgen precursors dehydroepiandrosterone (DHEA) and androstenedione. Glucocorticoid synthesis is negatively controlledMANUSCRIPT by a feedback loop via the hypothalamus- pituitary-adrenal axis. A variety of central stimul i lead to the circadian and stress related secretion of corticotropin-releasing hormone stimulating the cleavage of polypeptide proopiomelanocortin (POMC) by prohormone convertase. This results in adrenocorticotropic hormone (ACTH) release from corticotroph cells of the anterior pituitary. ACTH is the key regulator of cortisol synthesis and has additional short-term effects on mineralocorticoid and adrenal androgen synthesis (1). ACTH binds to its adrenal receptor (melanocortin receptor 2, MC2R) and stimulates the rapid import of cholesterolACCEPTED into the mitochondrion by steroidogenic acute regulatory protein (StAR). In parallel, the transcription of steroidogenic genes ( CYP11A1, HSD3B2, CYP17A1, CYP21A2, CYP11B1 ) and co-factors relevant to glucocorticoid synthesis increases. Corticotropin-releasing hormone and subsequently ACTH are released in a pulsatile fashion. Following the pattern of ACTH secretion, adrenal cortisol secretion exhibits a distinct 3 ACCEPTED MANUSCRIPT circadian rhythm, with peak concentrations in the morning and low concentrations in the late evening hours (2). Mineralocorticoid synthesis is mainly controlled by the renin-angiotensin-system and a potassium feedback loop. Renin secretion from the renal juxtaglomerular cells are stimulated by a variety of factors with renal arterial perfusion (closely correlating with renal arterial pressure) being the most important regulator. Non-endocrine conditions affecting renal blood flow have significant pathophysiologic consequences on the renin-angiotensin-system. The rate limiting step of the renin-angiotensin-system is the secretion of renin. Angiotensinogen is converted by renin to angiotensin I, which itself is converted by angiotensin converting enzyme to Angiotensin II, a potent stimulator of aldosterone synthesis and secretion (2). The distinct regulation of glucocorticoid and mineralocorticoid biosynthesis has important clinical consequences for the differential diagnosis and management of adrenal insufficiency (AI). Secondary AI manifests with isolated glucocorticoid deficiency, whereas most classic forms of primary adrenal insufficiency (PAI) MANUSCRIPThave signs and symptoms of combined glucocorticoid and mineralocorticoid deficiency. Classic familial glucocorticoid deficiency characterised by unresponsiveness of the adrenal to ACTH leading to isolated glucocorticoid deficiency represents an exception.
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