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Persistent Hyperinsulinemic Hypoglycemia

  • Chapter
Inborn Metabolic Diseases

Abstract

Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) is the most important cause of hypoglycemia in early infancy. The inappropriate oversecretion of insulin is responsible for profound hypoglycemias, which require aggressive treatment to prevent severe and irreversible brain damage. A combination of glucose and glucagon is started as an emergency treatment as soon as a tentative diagnosis of PHHI is made. It is followed by treatment with diazoxide and other drugs and, finally, by pancreatectomy if the patient is drug resistant. PHHI is a heterogeneous disorder with two histopathological lesions, diffuse (DiPHHI) and focal (FoPHHI), which are clinically indistinguishable. FoPHHI is characterized by a somatic islet cell hyperplasia. DiPHHI corresponds to a functional abnormality of insulin secretion in the whole pancreas and involves several genes with different transmissions. The therapeutic outcome differs for both histological entities, as does genetic counseling.

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Authors
  1. Pascale de Lonlay
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  2. Jean-Marie Saudubray
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Editor information

Editors and Affiliations

  1. Veldweg 87, 8051 NP, Hattem, The Netherlands

    John Fernandes (Professor Emeritus of Pediatrics) (Professor Emeritus of Pediatrics)

  2. Hospital Necker-Enfants Malades, 149 Rue de Sevres, 75743, Paris Cedex 15, France

    Jean-Marie Saudubray

  3. Institute of Cellular Pathology, Université Catholique de Louvain, Avenue Hippocrate 75, 1200, Brussels, Belgium

    Georges Van den Berghe (Christian de Duve) (Christian de Duve)

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© 2000 Springer-Verlag Berlin Heidelberg

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de Lonlay, P., Saudubray, JM. (2000). Persistent Hyperinsulinemic Hypoglycemia. In: Fernandes, J., Saudubray, JM., Van den Berghe, G. (eds) Inborn Metabolic Diseases. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-04285-4_9

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  • DOI: https://doi.org/10.1007/978-3-662-04285-4_9

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-662-04287-8

  • Online ISBN: 978-3-662-04285-4

  • eBook Packages: Springer Book Archive

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