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Semin Respir Crit Care Med 2019; 40(06): 810-824
DOI: 10.1055/s-0039-1697917
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Endocrine Complications of Cystic Fibrosis: A Multisystem Disease of the Endocrine Organs

Katie Larson Ode
1   Division of Pediatric Endocrinology and Diabetes, Department of Pediatrics, University of Iowa Stead Family Children's Hospital, Iowa City, Iowa
,
Christine L. Chan
2   Division of Pediatric Endocrinology, Department of Pediatrics, Colorado Children's Hospital, University of Colorado School of Medicine, Aurora, Colorado
,
Andrea Granandos
3   Division of Endocrinology and Metabolism, Department of Pediatrics, Children's Washington University School of Medicine in St. Louis, St. Louis, Missouri
,
Melissa Putman
4   Division of Endocrinology, Boston Children's Hospital, Boston, Massachusetts
5   Division of Endocrinology, Massachusetts General Hospital, Boston, Massachusetts
,
Amir Moheet
6   Department of Medicine, University of Minnesota, Minneapolis, Minnesota
› Author Affiliations
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Publication History

Publication Date:
03 November 2019 (online)

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Abstract

Cystic fibrosis (CF) is one of the most common life-limiting genetic disorders. Although CF is typically considered primarily as a pulmonary disease, the CF conductance transmembrane regulator is present throughout the body. From an endocrine perspective, this multisystem disease manifests primarily in the pancreas as a unique form of diabetes (CF-related diabetes mellitus), as bone disease, and as reproductive health issues in people with CF. These complications have become ever more concerning to people with CF as treatment for pulmonary disease improves and lifespans lengthen, increasing the impact of nonpulmonary complications. Our understanding of the management of these concerns continues to evolve, and, although there are some effective treatments, there is great opportunity for continued investigation into the pathophysiology of the endocrine complications of CF and their treatment.

Keywords

cystic fibrosis-related diabetes - cystic fibrosis - insulin - cystic fibrosis bone disease - hypogonadism - infertility - continuous glucose monitoring - bone mineral density