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Frontiers in Diabetes
Monogenic Hyperinsulinemic Hypoglycemia Disorders
S.Karger AG
Volume
21
Status:
Available
ISBN electronic:
978-3-8055-9944-3
ISBN print:
978-3-8055-9943-6
Book Series:
Frontiers in Diabetes
Publication date:
2012
Book Details
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.
Monogenic Hyperinsulinemic Hypoglycemia Disorders
Edited by: D.D. De León, C.A. Stanley
https://doi.org/10.1159/isbn.978-3-8055-9944-3
ISBN (print): 978-3-8055-9943-6
ISBN (electronic): 978-3-8055-9944-3
Publisher: S.Karger AG
Published: 2012
Download citation file:
Digital Version
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Print Version
Monogenic Hyperinsulinemic Hypoglycemia Disorders
$209.00
Table of Contents
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1 - 6: Historical Perspective on the Genetic Forms of Congenital HyperinsulinismByCharles A. Stanley;Charles A. StanleyaDivision of Endocrinology/Diabetes, The Children's Hospital of Philadelphia andSearch for other works by this author on:Franz M. MatschinskyFranz M. MatschinskybInstitute for Diabetes, Obesity and Metabolism, University of Pennsylvania School of Medicine, Philadelphia, Pa., USASearch for other works by this author on:
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7 - 17: Biochemistry and Physiology of the ATP-Sensitive Potassium ChannelByMaria S. Remedi;Maria S. RemediDepartment of Cell Biology and Physiology, Washington University School of Medicine, St. Louis, Mo., USASearch for other works by this author on:Colin G. NicholsColin G. NicholsDepartment of Cell Biology and Physiology, Washington University School of Medicine, St. Louis, Mo., USASearch for other works by this author on:
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18 - 29: Pathophysiology of Diffuse ATP-Sensitive Potassium Channel HyperinsulinismByDiva D. De León;Diva D. De LeónDivision of Endocrinology/Diabetes, The Children's Hospital of Philadelphia, Philadelphia, Pa., USASearch for other works by this author on:Charles A. StanleyCharles A. StanleyDivision of Endocrinology/Diabetes, The Children's Hospital of Philadelphia, Philadelphia, Pa., USASearch for other works by this author on:
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30 - 42: Molecular Defects of ATP-Sensitive Potassium Channels in Congenital HyperinsulinismByShow-Ling Shyng;Show-Ling ShyngDepartment of Biochemistry and Molecular Biology, Oregon Health and Science University, Portland, Oreg., USASearch for other works by this author on:Jeremy·D. Bushman;Jeremy·D. BushmanDepartment of Biochemistry and Molecular Biology, Oregon Health and Science University, Portland, Oreg., USASearch for other works by this author on:Emily B. Pratt;Emily B. PrattDepartment of Biochemistry and Molecular Biology, Oregon Health and Science University, Portland, Oreg., USASearch for other works by this author on:Qing ZhouQing ZhouDepartment of Biochemistry and Molecular Biology, Oregon Health and Science University, Portland, Oreg., USASearch for other works by this author on:
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43 - 56: Molecular Mechanisms and Clinical Pathophysiologies of Focal ATP-Sensitive Potassium Channel Hyperinsulinism and Beckwith-Wiedemann SyndromeByJean-Baptiste Arnoux;Jean-Baptiste ArnouxaCentre de Référence des Maladies Héréditaires du Métabolisme de l'Enfant et l'Adulte,Search for other works by this author on:Virginie Verkarre;Virginie VerkarrebDépartement d'Anatomopathologie,Search for other works by this author on:Sylvie Rossignol;Sylvie RossignoldLaboratoire d'Explorations Fonctionnelles, Hôpital Trousseau, AP-HP, Université Pierre et Marie Curie, Paris, FranceSearch for other works by this author on:Yves Aigrain;Yves AigraincService de Chirurgie Viscérale Pédiatrique, Hôpital Necker-Enfants Malades, AP-HP, Université Paris Descartes, andSearch for other works by this author on:Pascale de LonlayPascale de LonlayaCentre de Référence des Maladies Héréditaires du Métabolisme de l'Enfant et l'Adulte,Search for other works by this author on:
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57 - 70: Pancreatic Histopathology of HyperinsulinismByMariko Suchi;Mariko SuchiaDepartment of Pathology, Medical College of Wisconsin,bDepartment of Pathology and Laboratory Medicine, Children's Hospital of Wisconsin, Milwaukee, Wisc.,Search for other works by this author on:Tricia R. Bhatti;Tricia R. BhatticDepartment of Pathology and Laboratory Medicine, The Children's Hospital of Philadelphia, andSearch for other works by this author on:Eduardo·D. RuchelliEduardo·D. RuchellicDepartment of Pathology and Laboratory Medicine, The Children's Hospital of Philadelphia, anddDepartment of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, Pa., USASearch for other works by this author on:
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71 - 78: Localization of a Focal Lesion of Congenital Hyperinsulinism: Imaging and SurgeryByLisa J. States;Lisa J. StatesDepartment of Radiology, The Children's Hospital of Philadelphia, Philadelphia, Pa., USASearch for other works by this author on:N. Scott AdzickN. Scott AdzickDepartment of Radiology, The Children's Hospital of Philadelphia, Philadelphia, Pa., USASearch for other works by this author on:
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79 - 86: Role of Incretin Hormones in Hyperinsulinemic HypoglycemiaByDiva D. De LeónDiva D. De LeónDivision of Endocrinology/Diabetes, The Children's Hospital of Philadelphia, Philadelphia, Pa., USASearch for other works by this author on:
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87 - 99: Glutamate Dehydrogenase: Structure, Regulation, and Its Role in Insulin HomeostasisByThomas James SmithThomas James SmithDonald Danforth Plant Science Center, Saint Louis, Mo., USASearch for other works by this author on:
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100 - 111: Congenital Hyperinsulinism Due to Activating Mutations of Glutamate Dehydrogenase: The Hyperinsulinism/Hyperammonemia SyndromeByAndrea Kelly;Andrea KellyDivision of Endocrinology/Diabetes, The Children's Hospital of Philadelphia, Philadelphia, Pa., USASearch for other works by this author on:Andrew Palladino;Andrew PalladinoDivision of Endocrinology/Diabetes, The Children's Hospital of Philadelphia, Philadelphia, Pa., USASearch for other works by this author on:Charles A. StanleyCharles A. StanleyDivision of Endocrinology/Diabetes, The Children's Hospital of Philadelphia, Philadelphia, Pa., USASearch for other works by this author on:
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112 - 124: Amino Acid-Stimulated Insulin Secretion: The Role of the Glutamine-Glutamate-Alpha-Ketoglutarate AxisByChanghong Li;Changhong LiaDivision of Endocrinology/Diabetes, The Children's Hospital of Philadelphia, andSearch for other works by this author on:Franz M. Matschinsky;Franz M. MatschinskybInstitute for Diabetes, Obesity and Metabolism, University of Pennsylvania School of Medicine, Philadelphia, Pa., USASearch for other works by this author on:Charles A. StanleyCharles A. StanleyaDivision of Endocrinology/Diabetes, The Children's Hospital of Philadelphia, andSearch for other works by this author on:
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125 - 136: Tissue Specificity of Glutamate Dehydrogenase as Illustrated in Pancreatic Beta-Cells and the Central Nervous SystemByStefania Carobbio;Stefania CarobbioDepartment of Cell Physiology and Metabolism, Geneva University Medical Centre, Geneva, SwitzerlandSearch for other works by this author on:Laurène Vetterli;Laurène VetterliDepartment of Cell Physiology and Metabolism, Geneva University Medical Centre, Geneva, SwitzerlandSearch for other works by this author on:Francesca Frigerio;Francesca FrigerioDepartment of Cell Physiology and Metabolism, Geneva University Medical Centre, Geneva, SwitzerlandSearch for other works by this author on:Melis Karaca;Melis KaracaDepartment of Cell Physiology and Metabolism, Geneva University Medical Centre, Geneva, SwitzerlandSearch for other works by this author on:Pierre MaechlerPierre MaechlerDepartment of Cell Physiology and Metabolism, Geneva University Medical Centre, Geneva, SwitzerlandSearch for other works by this author on:
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137 - 145: The Molecular Genetics and Pathophysiology of Congenital Hyperinsulinism Caused by Short-Chain 3-Hydroxyacyl-CoA Dehydrogenase DeficiencyByAnders Molven;Anders MolvenaThe Gade Institute and Departments ofbPathology,Search for other works by this author on:Geir Helgeland;Geir HelgelandaThe Gade Institute and Departments ofcClinical Medicine andSearch for other works by this author on:Tone Sandal;Tone SandalaThe Gade Institute and Departments ofbPathology,Search for other works by this author on:Pål R. NjølstadPål R. NjølstadcClinical Medicine anddPediactrics, Haukeland University Hospital, University of Bergen, Bergen, NorwaySearch for other works by this author on:
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146 - 157: Hyperinsulinism Due to Activating Mutations of GlucokinaseBySamir Sayed;Samir SayedaDivision of Endocrinology/Diabetes, The Children's Hospital of Philadelphia, andSearch for other works by this author on:Franz M. Matschinsky;Franz M. MatschinskybInstitute for Diabetes, Obesity and Metabolism, University of Pennsylvania School of Medicine, Philadelphia, Pa., USASearch for other works by this author on:Charles A. StanleyCharles A. StanleyaDivision of Endocrinology/Diabetes, The Children's Hospital of Philadelphia, andSearch for other works by this author on:
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158 - 171: Hyperinsulinism Due to Mutations of Uncoupling Protein 2ByMaria del Mar Gonzalez-Barroso;Maria del Mar Gonzalez-BarrosoaLaboratory of Metabolic Biochemistry, Hôpital Necker-Enfants Malades, Assistance Publique Hôpitaux de Paris,eCentro de Investigaciones Biologicas, CSIC, Madrid, SpainSearch for other works by this author on:Pascale de Lonlay;Pascale de LonlaybUniversité Paris Descartes,cUnit of Metabolism, Department of Pediatrics, Hôpital Necker-Enfants Malades, andSearch for other works by this author on:Daniel RicquierDaniel RicquieraLaboratory of Metabolic Biochemistry, Hôpital Necker-Enfants Malades, Assistance Publique Hôpitaux de Paris,bUniversité Paris Descartes,dInstitut Cochin, CNRS and INSERM, Paris, France;Search for other works by this author on:
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172 - 181: Exercise-Induced Hyperinsulinism: A Failure of Monocarboxylate Transporter 1 Expression SilencingByTimo Otonkoski;Timo OtonkoskiaChildren's Hospital, University of Helsinki and Helsinki University Central Hospital, andbResearch Programs Unit, Molecular Neurology, Biomedicum Stem Cell Centre, University of Helsinki, Helsinki, Finland;Search for other works by this author on:Thomas MeissnerThomas MeissnercDepartment of General Pediatrics, University Children's Hospital Düsseldorf, Düsseldorf, GermanySearch for other works by this author on:
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182 - 190: HNF4A and Hyperinsulinemic HypoglycemiaByRitika R. Kapoor;Ritika R. KapoorClinical and Molecular Genetics Unit, The Developmental Endocrinology Research Group, Institute of Child Health, University College London and Great Ormond Street Hospital for Children NHS Trust London, London, UKSearch for other works by this author on:Chela T. James;Chela T. JamesClinical and Molecular Genetics Unit, The Developmental Endocrinology Research Group, Institute of Child Health, University College London and Great Ormond Street Hospital for Children NHS Trust London, London, UKSearch for other works by this author on:Khalid HussainKhalid HussainClinical and Molecular Genetics Unit, The Developmental Endocrinology Research Group, Institute of Child Health, University College London and Great Ormond Street Hospital for Children NHS Trust London, London, UKSearch for other works by this author on:
