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Congenital Hyperinsulinism

A Practical Guide to Diagnosis and Management

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

Part of the Contemporary Endocrinology book series (COE)

Table of contents

  1. Front Matter
    Pages i-xv
  2. Daphne Yau, Charles A. Stanley
    Pages 15-32
  3. Arpana Rayannavar, Henrik Thybo Christesen, Diva D. De León-Crutchlow
    Pages 33-47
  4. Jennifer M. Kalish, Jean-Baptiste Arnoux
    Pages 49-59
  5. Sarah E. Flanagan, Kara E. Boodhansingh, Emilie Lalonde, Arupa Ganguly
    Pages 61-69
  6. Khalid Hussain, Thomas Meissner, Jean-Baptiste Arnoux
    Pages 71-83
  7. Lisa J. States, Klaus Mohnike
    Pages 85-93
  8. Tricia R. Bhatti, Eduardo D. Ruchelli
    Pages 95-104
  9. N. Scott Adzick
    Pages 105-112
  10. Katherine Lord, Melissa Duran, Natalie Rintoul
    Pages 113-118
  11. Amanda M. Ackermann, Diva D. De León-Crutchlow
    Pages 119-130
  12. Jefferson N. Brownell, Heather McKnight-Menci, Asim Maqbool, Paul S. Thornton
    Pages 131-141
  13. Caroline Hall, Indraneel Banerjee
    Pages 143-154
  14. Katherine Lord, Diva D. De León-Crutchlow
    Pages 155-160
  15. Back Matter
    Pages 161-165

About this book

Introduction

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, and long-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.

Keywords

18FDOPA PET Beckwith Wiedemann syndrome Congenital hyperinsulism Diazoxide-responsive hyperinsulinism Diazoxide-unresponsive hyperinsulinism Hypoglycemia Insulin secretion Kabuki syndrome Pancreatectomy Syndromic hyperinsulinism

Editors and affiliations

  • Diva D. De León-Crutchlow
    • 1
  • Charles A. Stanley
    • 2
  1. 1.Division of Endocrinology and Diabetes, Department of PediatricsPerelman School of Medicine at the University of Pennsylvania and The Children’s Hospital of PhiladelphiaPhiladelphiaUSA
  2. 2.Division of Endocrinology and Diabetes, Department of PediatricsPerelman School of Medicine at the University of Pennsylvania and The Children’s Hospital of PhiladelphiaPhiladelphiaUSA

Bibliographic information

  • DOI https://doi.org/10.1007/978-3-030-02961-6
  • Copyright Information Springer Nature Switzerland AG 2019
  • Publisher Name Humana Press, Cham
  • eBook Packages Medicine
  • Print ISBN 978-3-030-02960-9
  • Online ISBN 978-3-030-02961-6
  • Series Print ISSN 2523-3785
  • Series Online ISSN 2523-3793
  • Buy this book on publisher's site
Industry Sectors
Pharma
Biotechnology