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Title Monogenic hyperinsulinemic hypoglycemia disorders / volume editors, Charles A. Stanley, Diva D. De León
Published Basel : Karger, 2012

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Description 1 online resource (205 pages) : illustrations
Series Frontiers in diabetes, 0251-5342 ; vol. 21
Frontiers in diabetes ; v. 21. 0251-5342
Contents 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 hyperinsulinism / De León, D.D., Stanley, C.A. -- Molecular defects of ATP-sensitive potassium channels in congenital hyperinsulinism / Shyng, S.-L. [and others] -- Molecular mechanisms and clinical pathophysiologies of focal ATP-sensitive potassium hyperinsulinism and Beckwith-Wiedemann Syndrome / Arnoux, J.-B. [and others] -- 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 León, 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. [and others] -- The molecular genetics and pathophysiology of congenital hyperinsulinism caused by short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency / Molven, A. [and others] -- Hyperinsulinism due to activating mutations of glucokinase / Sayed, S., Matschinsky, F., 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
Summary 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 <sup>18</sup>F-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
Bibliography Includes bibliographical references and indexes
Notes Online resource; title from electronic title page (ebrary, viewed on October 18, 2013)
Subject Hypoglycemia.
Insulin -- Metabolism
Pancreas -- Diseases.
Insulin shock.
Hyperinsulinism
Hypoglycemia
Insulin -- metabolism
Pancreatic Diseases
MEDICAL -- Endocrinology & Metabolism.
Hypoglycemia
Insulin -- Metabolism
Insulin shock
Pancreas -- Diseases
Hyperinsulinämie
Hypoglykämie
Hypoglykemi.
Insulin.
Bukspottkörteln.
Form Electronic book
Author Stanley, Charles A., editor
De León, Diva D., editor
LC no. 2011048792
ISBN 9783805599443
3805599447