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J Cyst Fibros. 2019 Oct;18:S3-S9. doi: 10.1016/j.jcf.2019.08.016.

Cystic fibrosis related diabetes: Pathophysiology, screening and diagnosis.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society

Andrea Granados, Christine L Chan, Katie Larson Ode, Amir Moheet, Antoinette Moran, Reinhard Holl

Affiliations

  1. Department of Pediatrics, Washington University School of Medicine in St. Louis, MO, USA. Electronic address: [email protected].
  2. Children's Hospital Colorado, University of Colorado, Barbara Davis Center for Childhood Diabetes, Aurora, CO, USA.
  3. Department of Pediatrics, University of Iowa Stead Family Children's Hospital, Fraternal Order of Eagles Diabetes Research Center, University of Iowa, Iowa City, IA, USA.
  4. Department of Medicine, University of Minnesota, Minneapolis, MN, USA.
  5. Division of Pediatric Endocrinology, University of Minnesota, Minneapolis, MN, USA.
  6. University of Ulm, Ulm, Germany.

PMID: 31679726 DOI: 10.1016/j.jcf.2019.08.016

Abstract

Cystic fibrosis related diabetes (CFRD) is a distinct form of diabetes that is associated with significantly increased morbidity and mortality in the CF population. The primary etiology is relative insulin insufficiency secondary to destruction of pancreatic islets, and to other factors that affect the function of the remaining beta cells. The prevalence of CFRD increases markedly with age and more than half of CF adults develop the disease. As the initial presentation of CFRD may be clinically silent, annual screening is recommended starting at least by the age of 10 with an oral glucose tolerance test (OGTT). The importance of an early diagnosis is based on the association of diabetes and abnormal glucose tolerance with worse health outcomes in patients with CF, and evidence that early treatment of diabetes improves prognosis. This review presents a concise overview of the current understanding of CFRD pathogenesis, natural history, screening and diagnosis.

Copyright © 2019. Published by Elsevier B.V.

Keywords: Cystic fibrosis; Cystic fibrosis related diabetes; Diagnosis; Pathophysiology; Screening

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