ESPE Yearbook of Paediatric Endocrinology

To read the full abstract: J Clin Endocrinol Metab 2017;102:1797-1806We know that conventional glucocorticoid therapies fail to mimic the normal diurnal profile of cortisol secretion, which should show an early morning surge in circulating levels, followed by a gradual daytime reduction, and night-time suppression. The early morning surge in ACTH also drives adrenal androgen production, which is e...

J Clin Endocrinol Metab 2021; 106(5): e2063–e2077.https://pubmed.ncbi.nlm.nih.gov/33527139/The authors report the findings of a 6-month, randomized, phase 3 trial, with a single arm extension, to investigate the efficacy, safety and tolerability of modified release hydrocortisone (MC-HC) replacement therapy versus standard glucocorticoid replacement therapy in 122 adult patients with c...

Brief summary: This comprehensive narrative review provides a “Seminar” on congenital adrenal hyperplasia (CAH). The authors performed a thorough comprehensive qualitative summary of articles published mainly in Cochrane Library, MEDLINE, and Embase in English between Jan 1, 2005, and June 30, 2021, while not excluding commonly referenced and highly regarded older publications.Congenital adrenal hyperplasia (CAH) is an ‘umbrella’ clin...

Brief summary: In this Minireview, one pediatric and one adult case with 21-hydroxylase deficiency (21OHD) are discussed with respect to different clinical questions and steroid biomarkers reflecting their diagnosis, treatment and disease control. Basics of the disease mechanisms with different aspects throughout life (childhood, adulthood, sex, fertility and pregnancy) are discussed to lay grounds for the interpretation and use of laboratory data, including the newer 11-oxyge...

To read the full abstract: Proc Natl Acad Sci U S A. 2019; 116(44): 22294-22299. PMID: 31611378.Androgen biosynthesis in the human fetus proceeds through the adrenal sex steroid precursor dehydroepiandrosterone (DHEA), which is converted to testosterone in the gonads, followed by further activation to 5α-dihydrotestosterone (DHT) in genital skin, thereby facilitating male external genit...