Endocrine Disorders in Cystic Fibrosis

• Type of Material: Article
• Authors: Scott M. Blackman, Johns Hopkins University; Vin Tangpricha, Emory University
• Language: English
• Date: 2016-08-01
• Publisher: WB Saunders
• Publication Version: Author Accepted Manuscript (After Peer Review)
• Copyright Statement: © 2016 Elsevier Inc.
• Final Published Version (URL): http://dx.doi.org/10.1016/j.pcl.2016.04.009
• Title of Journal or Parent Work: Pediatric Clinics of North America
• ISSN: 0031-3955
• Volume: 63
• Issue: 4
• Start Page: 699
• End Page: +
• Keywords: Life Sciences & Biomedicine; Hypoglycemia; Hypogonadism; Science & Technology; BONE-MINERAL DENSITY; MASS; YOUNG-ADULTS; INSULIN-SECRETION; Short stature; VITAMIN-D; Diabetes; BETA-CELLS; DISEASE; CHILDREN; Osteoporosis; Pediatrics; GROWTH-HORMONE; GENETIC MODIFIERS
• Research Categories: Health Sciences, General

Abstract

Cystic fibrosis can cause multiple endocrine disorders including diabetes, bone disease, short stature, and male hypogonadism. The etiologies are multifactorial but may be directly related in some cases to CFTR dysfunction. Diabetes is the most common endocrine complication and can be expected to affect in almost all people with pancreatic insufficient CF but varies widely in its age of onset. Early identification and treatment of diabetes improves morbidity and mortality in CF. Short stature during adolescence can be transient if puberty is later than average, but it can also be pathologic. Inhaled and systemic steroids can slow growth and should be used at the minimum effective doses. Bone disease in CF is a frequent cause of fragility fractures and associates with worse lung disease. Bone mineral density and vitamin D levels should be monitored. Hypogonadism has been reported in 25% of men with CF. Endocrine complications can cause morbidity and mortality in CF and need to be appropriately detected and managed as part of the medical care of individuals with CF. These complications tend to occur more frequently in older individuals and may be expected to become more common as the CF population grows older.