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Cystic Fibrosis-Related Diabetes

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Cystic Fibrosis

Part of the book series: Respiratory Medicine ((RM))

Abstract

Cystic fibrosis-related diabetes (CFRD) is a common complication of cystic fibrosis (CF) and is associated with increased morbidity and mortality in children and adults. While its pathophysiology is complex, the main feature of CFRD is inadequate insulin secretion due to functional and structural abnormalities in islet cells. Insulin remains the mainstay of glycemic therapy as it improves health outcomes in patients with CFRD. While research has focused on the basic pathogenesis of CFRD over the last several decades, numerous questions still remain.

This chapter reviews the natural history and pathophysiology of CFRD; its association with clinical outcomes in CF; screening, diagnosis, and treatment of CFRD; and future directions of research in this field.

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Abbreviations

ADA:

American Diabetes Association

BMI:

Body mass index

CF:

Cystic fibrosis

CFF:

Cystic Fibrosis Foundation

CFRD:

Cystic fibrosis-related diabetes

CFTR:

Cystic fibrosis transmembrane conductance regulator

CGM:

Continuous glucose monitoring

FA:

Fructosamine

GA:

Glycated albumin

HbA1c:

Glycated hemoglobin

IFG:

Impaired fasting glucose

IGT:

Impaired glucose tolerance

INDET:

Indeterminate glycemia

NGT:

Normal glucose tolerance

OGTT:

Oral glucose tolerance test

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Authors and Affiliations

  1. Washington University School of Medicine in St. Louis, St. Louis Children’s Hospital, Department of Pediatrics, Division of Pediatric Endocrinology and Diabetes, One Children’s Place, St. Louis, MO, USA

    Andrea Granados

  2. Department of Pediatrics, Pittsburgh Heart, Lung, Blood and Vascular Medicine Institute, University of Pittsburgh, UPMC Children’s Hospital of Pittsburgh, Division of Pediatric Endocrinology and Diabetes, Pittsburgh, PA, USA

    Kara S. Hughan

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  1. Andrea Granados
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  2. Kara S. Hughan
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Correspondence to Andrea Granados .

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Editors and Affiliations

  1. The University of North Carolina at Chapel Hill, Department of Pediatrics, UNC Children’s Hospital, Chapel Hill, NC, USA

    Stephanie Duggins Davis

  2. Department of Pediatrics, University of Washington School of Medicine, Division of Pulmonary and Sleep Medicine, Seattle Children’s Hospital, Seattle, WA, USA

    Margaret Rosenfeld

  3. Department of Pediatrics, Indiana University School of Medicine, Division of Pediatric Pulmonology, Allergy and Sleep Medicine, Riley Hospital for Children at IU Health, Indianapolis, IN, USA

    James Chmiel

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Granados, A., Hughan, K.S. (2020). Cystic Fibrosis-Related Diabetes. In: Davis, S., Rosenfeld, M., Chmiel, J. (eds) Cystic Fibrosis. Respiratory Medicine. Humana, Cham. https://doi.org/10.1007/978-3-030-42382-7_16

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