Oranan Siwamogsatham

The aim of this review is to provide an update on various relevant endocrine aspects of care in adolescents and adults with cystic fibrosis. As life expectancy in cystic fibrosis has continuously improved, endocrine complications have become more apparent. The common endocrine complications include cystic fibrosis related diabetes, cystic fibrosis related bone disease, vitamin D deficiency and poor growth and pubertal development. Thyroid and adrenal disorders have also been reported, although the prevalence appears to be less common. Endocrine diseases are an increasingly recognized complication that has a significant impact on the overall health of individuals with cystic fibrosis. This review summarizes the updated screening and management of endocrine diseases in the cystic fibrosis population.

Background: Pancreatic insufficiency is common in patients with cystic fibrosis (CF) and leads to malabsorption of fat‐soluble vitamins. Multivitamins, including vitamins A, D, E, and K, are routinely prescribed to patients with CF to prevent vitamin deficiencies. Our objective was to examine the relationship between fat‐soluble vitamin supplements and their impact on blood concentrations. Methods: This was a retrospective chart review of patients with CF who were treated at Emory Clinic and Emory University Hospital during 2008–2012. The amount of fat‐soluble vitamin supplementation, serum markers of fat‐soluble vitamin concentrations, CF transmembrane conductance regulator genotype, and other demographic information were recorded from electronic medical records. Mixed‐effects models were used to investigate the trends over time of fat‐soluble vitamin supplements and serum vitamin concentrations. Results: In total, 177 charts were eligible. Mean (SD) age was 26.1 (10.2) years. Ninety‐two percent of patients had pancreatic insufficiency and 52% had the homozygous ΔF508 mutation. Recorded fat‐soluble vitamin supplementation increased in the past 5 years (P < .001 for all). Serum 25‐hydroxyvitamin D increased slightly (3% increase; P < .01); however, there were no changes in the blood concentrations of vitamins A, E, and K (P = .26–.96). Conclusions: Despite a near doubling of recorded fat‐soluble vitamin supplementation over the past 5 years, there was no parallel increase in blood concentrations of these vitamins. Potential reasons include suboptimal dosages, low adherence, or ongoing issues with malabsorption.

Introduction.Bone disease is a common complication of cystic fibrosis (CF). To date, there have been no reports on the effectiveness of teriparatide, recombinant human parathyroid hormone, to treat CF-related bone disease.Case Presentation.We report on four patients with CF-related bone disease who were treated with teriparatide. Three patients completed two years of therapy with teriparatide, and all had significant improvements in their bone mineral density (BMD). One patient was unable to tolerate teriparatide and discontinued treatment 1 week into therapy.Conclusion.Teriparatide may be a potential treatment option for CF-related bone disease. This report highlights the need for further investigation into the use of teripartide in the CF population.