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, 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.
18FDOPA PET Beckwith Wiedemann syndrome Congenital hyperinsulism Diazoxide-responsive hyperinsulinism Diazoxide-unresponsive hyperinsulinism Hypoglycemia Insulin secretion Kabuki syndrome Pancreatectomy Syndromic hyperinsulinism
Editors and affiliations
- 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
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