Congenital Hyperinsulinism -

Congenital Hyperinsulinism

A Practical Guide to Diagnosis and Management
Buch | Hardcover
XV, 165 Seiten
2019 | 1st ed. 2019
Springer International Publishing (Verlag)
978-3-030-02960-9 (ISBN)
96,29 inkl. MwSt
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.

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

Approach to the Diagnosis of Neonates and Infants with Persistent Hypoglycemia.- Diazoxide-Responsive Forms of Congenital Hyperinsulinism.- Diazoxide-Unresponsive Forms of Congenital Hyperinsulinism.- Syndromic Causes of Congenital Hyperinsulinism.- Molecular Diagnosis of Congenital Hyperinsulinism.- Medical Management of Hyperinsulinism.- 18F-DOPA PET.- Histopathology of the Pancreas in Congenital Hyperinsulinism.- Surgery for Congenital Hyperinsulinism.- Perioperative Management of Hyperinsulinism.- Management of the Child with Persistent Hypoglycemia After Surgery.- Management of Diabetes and Pancreatic Insufficiency after Pancreatectomy.- Feeding Problems in Congenital Hyperinsulinism.- Neurodevelopmental Outcomes.

Erscheinungsdatum
Reihe/Serie Contemporary Endocrinology
Zusatzinfo XV, 165 p. 22 illus., 12 illus. in color.
Verlagsort Cham
Sprache englisch
Maße 155 x 235 mm
Gewicht 432 g
Themenwelt Medizinische Fachgebiete Innere Medizin Endokrinologie
Medizin / Pharmazie Medizinische Fachgebiete Pädiatrie
Schlagworte 18FDOPA PET • Beckwith Wiedemann syndrome • Congenital hyperinsulism • Diazoxide-responsive hyperinsulinism • Diazoxide-unresponsive hyperinsulinism • hypoglycemia • Insulin Secretion • Kabuki syndrome • Pancreatectomy • Syndromic hyperinsulinism
ISBN-10 3-030-02960-3 / 3030029603
ISBN-13 978-3-030-02960-9 / 9783030029609
Zustand Neuware
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