Monogenic Hyperinsulinemic Hypoglycemia Disorders (Frontiers in Diabetes Vol.21) （2012. 196 S. 54 fig., 10 in color, 20 tab.）

Herausgegeben von Stanley, C. A./ De Leó/n, D. D./ Porta, Massimo

KARGER（2012発売）

• 製本 Hardcover:ハードカバー版／ページ数 196 p.／サイズ 54 figs., 11 in color, 18 tbs.
• 商品コード 9783805599436

Full Description

In this volume of 'Frontiers in Diabetes' dealing with the molecular basis of monogenic disease of beta-cell insulin regulation, world-renowned experts provide in-depth descriptions of the many recent advances in genetic defects that cause hyperinsulinemic hypoglycemia. These disorders comprise the most important form of hypoglycemia in infants and children and are associated with a high risk of morbidity, including seizures and severe brain injury. The discovery of eight different genetic loci involved in congenital hyperinsulinism has led to greatly improved methods of diagnosis and treatment. New approaches to diagnosis are highlighted, such as 18F-DOPA PET scans for preoperative localization of focal hyperinsulinism, as well as potential new treatments, such as green tea polyphenols for GDH-HI and GLP-receptor antagonists for SUR1 and Kir6.2 hyperinsulinism. Practitioners, including pediatricians and specialists in endocrinology, surgery, genetics, pathology, and radiology, will find important up-to-date information for clinical diagnosis, management, and new treatments for infants and children with congenital hyperinsulinism. Researchers will discover how genetic hyperinsulinism disorders provide novel insights into the basic mechanisms regulating insulin secretion not only in diabetics, but also in healthy humans.

Contents

Preface: Stanley, C.A.; De Leon, D.D.; Historical Perspective on the Genetic Forms of Congenital Hyperinsulinism: Stanley, C.A.; Matschinsky, F.M.; Biochemistry and Physiology of the ATP-Sensitive Potassium Channel: Remedi, M.S.; Nichols, C.G.; Pathophysiology of Diffuse ATP-Sensitive Potassium Channel Hyperinsulinism: De Leon, D.D.; Stanley, C.A.; Molecular Defects of ATP-Sensitive Potassium Channels in Congenital Hyperinsulinism: Shyng, S.-L.; Bushman, J.D.; Pratt, E.B.; Zhou, Q.; Molecular Mechanisms and Clinical Pathophysiologies of Focal ATP-Sensitive Potassium Channel Hyperinsulinism and Beckwith-Wiedemann Syndrome: Arnoux, J.-B.; Verkarre, V.; Rossignol, S.; Aigrain, Y.; de Lonlay, P.; Pancreatic Histopathology of Hyperinsulinism: Suchi, M.; Bhatti, T.R.; Ruchelli, E.D.; Localization of a Focal Lesion of Congenital Hyperinsulinism: Imaging and Surgery: States, L.J.; Adzick, N.S.; Role of Incretin Hormones in Hyperinsulinemic Hypoglycemia: De Leon, D.D.; Glutamate Dehydrogenase: Structure, Regulation, and Its Role in Insulin Homeostasis: Smith, T.J.; Congenital Hyperinsulinism Due to Activating Mutations of Glutamate Dehydrogenase: The Hyperinsulinism/Hyperammonemia Syndrome: Kelly, A.; Palladino, A.; Stanley, C.A.; Amino Acid-Stimulated Insulin Secretion: The Role of the Glutamine-Glutamate-Alpha-Ketoglutarate Axis: Li, C.; Matschinsky, F.M.; Stanley, C.A.; Tissue Specificity of Glutamate Dehydrogenase as Illustrated in Pancreatic Beta-Cells and the Central Nervous System: Carobbio, S.; Vetterli, L.; Frigerio, F.; Karaca, M.; Maechler, P.; The Molecular Genetics and Pathophysiology of Congenital Hyperinsulinism Caused by Short-Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency: Molven, A.; Helgeland, G.; Sandal, T.; Njolstad, P.R.; Hyperinsulinism Due to Activating Mutations of Glucokinase: Sayed, S.; Matschinsky, F.M.; Stanley, C.A.; Hyperinsulinism Due to Mutations of Uncoupling Protein 2: del Mar Gonzalez-Barroso, M.; de Lonlay, P.; Ricquier, D.; Exercise-Induced Hyperinsulinism: A Failure of Monocarboxylate Transporter 1 Expression Silencing: Otonkoski, T.; Meissner, T.; HNF4A and Hyperinsulinemic Hypoglycemia: Kapoor, R.R.; James, C.T.; Hussain, K.; Author Index; Subject Index.