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Histopathology of the Pancreas in Congenital Hyperinsulinism

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Congenital Hyperinsulinism

Part of the book series: Contemporary Endocrinology ((COE))

Abstract

The underlying genetic alterations in congenital hyperinsulinism (HI) are reflected in characteristic histopathologic changes in the pancreas of affected patients. The majority of cases with inactivating KATP mutations can be segregated into two morphologic forms, diffuse and focal HI, with corresponding alterations in islet cell nuclear size and growth patterns, respectively. Hyperinsulinism due to other gene defects and syndromes is less common, but these patients also demonstrate abnormalities in pancreatic endocrine tissue morphology. Correlation of the histologic and genetic findings remains a mainstay of both diagnosis and patient management and has especially important clinical implications at the time of surgery.

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Authors and Affiliations

  1. Department of Pathology and Laboratory Medicine, The Children’s Hospital of Philadelphia and Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA

    Tricia R. Bhatti & Eduardo D. Ruchelli

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  1. Tricia R. Bhatti
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  2. Eduardo D. Ruchelli
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Correspondence to Tricia R. Bhatti .

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Editors and Affiliations

  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, PA, USA

    Diva D. De León-Crutchlow

  2. 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, PA, USA

    Charles A. Stanley

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Bhatti, T.R., Ruchelli, E.D. (2019). Histopathology of the Pancreas in Congenital Hyperinsulinism. In: De León-Crutchlow, D., Stanley, C. (eds) Congenital Hyperinsulinism. Contemporary Endocrinology. Humana Press, Cham. https://doi.org/10.1007/978-3-030-02961-6_8

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  • DOI: https://doi.org/10.1007/978-3-030-02961-6_8

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  • Publisher Name: Humana Press, Cham

  • Print ISBN: 978-3-030-02960-9

  • Online ISBN: 978-3-030-02961-6

  • eBook Packages: MedicineMedicine (R0)

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