ESPE Yearbook of Paediatric Endocrinology

Brief summary: This review covers the recent advances in the understanding of fetal adrenal development and the interplay with the feto-placental unit. A short overview of related adrenal disorders is presented. The effect of other hormone systems is also discussed and the reliability of using rodent models to study adrenal pathophysiology.Structural development of the fetal adrenal and its transition to the adult organ: There are differ...

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: This narrative review summarizes the important progress made in the past 10 years in our understanding of the genetics of primary bilateral macronodular adrenal hyperplasia (PBMAH).Primary bilateral macronodular adrenal hyperplasia (PBMAH) is an adrenal cause of Cushing syndrome, attributed to the disrupted integrity of the adrenal cortex zonation that is important for steroidogenesis (1). Nowadays, the diagnosis of PBMAH ...