Full Description
Written by experts and specialized investigators, this book presents a detailed overview of the recent progress in our understanding of the adrenal cortex and its pivotal roles in homeostasis. Genes, molecules and cell compartments directly or indirectly involved in the complex steroidogenesis pathway as well as the resulting end-hormones glucocorticoids, mineralocorticoids and androgens are analyzed. Furthermore, the defects of the genes responsible both for common and rare adrenal disorders are presented. The interactions of the adrenal cortices with the adrenal medulla and their importance in the integration of adrenocortical and adrenomedullary function are discussed. The complex molecular pathophysiology of congenital adrenal hyperplasia is presented; long-term effects of the disorder and the still controversial antenatal therapy are examined. Finally, expert review chapters discuss autoimmune Addison disease and the adrenoleukodystrophy/adrenomyeloneuropathy syndrome. Presenting novel research findings in adrenal gland physiology and pathophysiology, this book is a useful tool not only for pediatric endocrinologists, but also for clinicians and researchers studying human development, organogenesis, mitochondrial biology, nuclear receptors, stress biology and pharmacology.
Contents
Preface: Ghizzoni, L.; Cappa, M.; Chrousos, G.; Loche, S.; Maghnie, M.; Role of Mitochondria in Steroidogenesis: Miller, W.L.; The Physiology and Biochemistry of Adrenarche: Auchus, R.J.; Update on the Corticomedullary Interaction in the Adrenal Gland: Haase, M.; Willenberg, H.S.; Bornstein, S.R.; Role of DAX-1 (NROB1) and Steroidogenic Factor-1 (NR5A1) in Human Adrenal Function: El-Khairi, R.; Martinez-Aguayo, A.; Ferraz-de-Souza, B.; Lin, L.; Achermann, J.C.; Functional and Physiological Consequences of StAR Deficiency: Role in Lipoid Congenital Adrenal Hyperplasia: King, S.R.; Bhangoo, A.; Stocco, D.M.; P450 Side-Chain Cleavage Deficiency - A Rare Cause of Congenital Adrenal Hyperplasia: Hauffa, B.; Hiort, O.; Clinical and Biochemical Consequences of P450 Oxidoreductase Deficiency: Fluck, C.E.; Pandey, A.V.; Molecular Genetics of 21-Hydroxylase Deficiency: Wedell, A.; Brain Development and Cognitive, Psychosocial, and Psychiatric Functioning in Classical 21-Hydroxylase Deficiency: Meyer-Bahlburg, H.F.L.; Long-Term Outcome of Prenatal Dexamethasone Treatment of 21-Hydroxylase Deficiency: Lajic, S.; Nordenstrom, A.; Hirvikoski, T.; Role of Genetic Variation in Regulation of Aldosterone Biosynthesis: Alvarez-Madrazo, S.; Connell, J.M.; Freel, E.M.; Circadian CLOCK-Mediated Regulation of Target-Tissue Sensitivity to Glucocorticoids: Implications for Cardiometabolic Diseases: Kino, T.; Chrousos, G.P.; Glucocorticoid Resistance: van Rossum, E.F.C.; van den Akker, E.L.T.; Mineralocorticoid Receptor Gene Variants as Determinants of HPA Axis Regulation and Behavior: DeRijk, R.H.; de Kloet, E.R.; Zitman, F.G.; van Leeuwen, N.; Adrenoleukodystrophy: Cappa, M.; Bizzarri, C.; Vollono, C.; Petroni, A.; Banni, S.; Autoimmune Addison's Disease: Betterle, C.; Morlin, L.; Optimal Glucocorticoid Therapy: Debono, M.; Ross, R.J.; Modulation of Glucocorticoid Metabolism by the GH-IGF-I Axis: Neggers, S.J.C.M.M.; van der Lely, A.J.; Effects of Glucocorticoids on the Growth Plate: Lui, J.C.; Baron, J.; Growth Hormone Treatment in Children on Chronic Glucorticoid Therapy: Savage, M.O.; Simon, D.; Czernichow, P.C.; Ancient History of Congenital Adrenal Hyperplasia: New, M.I.
