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Congenital Hyperinsulinism

A Practical Guide to Diagnosis and Management

  • Book
  • © 2019

Overview

Editors:
  1. Diva D. De León-Crutchlow

    1. Division of Endocrinology and Diabetes, Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania and The Children’s Hospital of Philadelphia, Philadelphia, USA

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  2. Charles A. Stanley

    1. Division of Endocrinology and Diabetes, Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania and The Children’s Hospital of Philadelphia, Philadelphia, USA

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  • A practical guide for diagnosing and managing neonates, infants and children with congenital hyperinsulinism (HI)
  • Discusses the diagnosis and medical and surgical management of diazoxide-responsive, diazoxide-unresponsive and syndromic HI
  • A valuable resource for pediatric endocrinologists, general pediatricians and neonatologists

Part of the book series: Contemporary Endocrinology (COE)

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Table of contents (14 chapters)

  1. Front Matter

    Pages i-xv
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  2. Approach to the Diagnosis of Neonates and Infants with Persistent Hypoglycemia

    • Paul S. Thornton, Charles A. Stanley
    Pages 1-13

  3. Diazoxide-Responsive Forms of Congenital Hyperinsulinism

    • Daphne Yau, Charles A. Stanley
    Pages 15-32

  4. Diazoxide-Unresponsive Forms of Congenital Hyperinsulinism

    • Arpana Rayannavar, Henrik Thybo Christesen, Diva D. De León-Crutchlow
    Pages 33-47

  5. Syndromic Causes of Congenital Hyperinsulinism

    • Jennifer M. Kalish, Jean-Baptiste Arnoux
    Pages 49-59

  6. Molecular Diagnosis of Congenital Hyperinsulinism

    • Sarah E. Flanagan, Kara E. Boodhansingh, Emilie Lalonde, Arupa Ganguly
    Pages 61-69

  7. Medical Management of Hyperinsulinism

    • Khalid Hussain, Thomas Meissner, Jean-Baptiste Arnoux
    Pages 71-83

  8. 18F-DOPA PET

    • Lisa J. States, Klaus Mohnike
    Pages 85-93

  9. Histopathology of the Pancreas in Congenital Hyperinsulinism

    • Tricia R. Bhatti, Eduardo D. Ruchelli
    Pages 95-104

  10. Surgery for Congenital Hyperinsulinism

    • N. Scott Adzick
    Pages 105-112

  11. Perioperative Management of Hyperinsulinism

    • Katherine Lord, Melissa Duran, Natalie Rintoul
    Pages 113-118

  12. Management of the Child with Persistent Hypoglycemia After Surgery

    • Amanda M. Ackermann, Diva D. De León-Crutchlow
    Pages 119-130

  13. Management of Diabetes and Pancreatic Insufficiency After Pancreatectomy

    • Jefferson N. Brownell, Heather McKnight-Menci, Asim Maqbool, Paul S. Thornton
    Pages 131-141

  14. Feeding Problems in Congenital Hyperinsulinism

    • Caroline Hall, Indraneel Banerjee
    Pages 143-154

  15. Neurodevelopmental Outcomes

    • Katherine Lord, Diva D. De León-Crutchlow
    Pages 155-160

  16. Back Matter

    Pages 161-165
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Keywords

About this book

This unique book is a practical guide for the clinician faced with the challenge of diagnosing and managing neonates, infants and children with congenital hyperinsulinism (HI), within the framework of pathophysiology and molecular genetics. Major advances have been made in HI research over the past two decades, and with this better understanding of the molecular genetics of HI, a “personalized” approach to management according to the type of hyperinsulinism, and particularly according to the likelihood of focal hyperinsulinism, is starting to emerge. The opening chapter discusses HI diagnosis using biochemical approaches and phenotype characterization. The various forms of HI are then presented in detail in three main categories: diazoxide-responsive, diazoxide-unresponsive and syndromic HI. Both medical and surgical management strategies are then discussed, covering imaging, histology, surgical approach, and post-operative management. Complications, such as feeding problems, andlong-term outcomes, such as neurodevelopmental issues, are carefully considered in the final chapter.


Practical and user-friendly, Congenital Hyperinsulinism is the go-to resource for pediatric endocrinologists, residents and fellows, general pediatricians and neonatologists.

Editors and Affiliations

  • Division of Endocrinology and Diabetes, Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania and The Children’s Hospital of Philadelphia, Philadelphia, USA

    Diva D. De León-Crutchlow, Charles A. Stanley

About the editors

Diva D. De León-Crutchlow, MD, MSCE, Associate Professor of Pediatrics, Chief, Division of Endocrinology and Diabetes, Director, Congenital Hyperinsulinism Center, The Children’s Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA




Charles A. Stanley, MD, Emeritus Professor of Pediatrics, The Children’s Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA

Bibliographic Information

  • Book Title: Congenital Hyperinsulinism

  • Book Subtitle: A Practical Guide to Diagnosis and Management

  • Editors: Diva D. De León-Crutchlow, Charles A. Stanley

  • Series Title: Contemporary Endocrinology

  • DOI: https://doi.org/10.1007/978-3-030-02961-6

  • Publisher: Humana Cham

  • eBook Packages: Medicine, Medicine (R0)

  • Copyright Information: Springer Nature Switzerland AG 2019

  • Hardcover ISBN: 978-3-030-02960-9Published: 17 January 2019

  • eBook ISBN: 978-3-030-02961-6Published: 04 January 2019

  • Series ISSN: 2523-3785

  • Series E-ISSN: 2523-3793

  • Edition Number: 1

  • Number of Pages: XV, 165

  • Number of Illustrations: 10 b/w illustrations, 12 illustrations in colour

  • Topics: Endocrinology, Pediatrics

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