Abstract

Aims: We evaluated the impact of cystic fibrosis-related diabetes (CFRD) on lung disease and nutritional status.Study Design: The retrospective cohort study evaluated the subjects' medical records from 2004 to 2019. All participants older than 10 years diagnosed by a 30-minutely sampled OGTT formed OGTT-CFRD subgroup. The participants diagnosed with continuous glucose monitoring (CGM) (at least two peaks above 11.1 mmol/l and more than 10% of recorded time above 7.8 mmol/l) formed a CFRD-CGM subgroup. The participants without CFRD formed a non-CFRD group. The longitudinal follow-up was made 2 years before and 3 years after insulin therapy initiation.Results: Of 144 participants included, aged 10–55 years (44% males), 28 (19.4%) had CFRD. The HbA1c was significantly lower in the CGM-CFRD in comparison to the OGTT-CFRD subgroup (5.9 ± 0.62 and 7.3 ± 1.7% respectfully; p = 0.04). Subjects with CFRD were malnourished in comparison to non-CFRD, with significant improvements with insulin replacement therapy in regard to BMI Z-score (−1.4 ± 1.3 vs. −0.5 ± 1.2%, p = 0.04) and pulmonary exacerbation score (p = 0.02). In OGTT-CFRD subgroup there is an increase in FEV1 (62.7 ± 26.3 to 65.1 ± 21.7%, p = 0.7) and decrease in FVC (from 76.4 ± 24.2 to 71.2 ± 20%, p = 0.003) from diagnosis to second year of follow-up. In CGM-CFRD subgroup there was a decrease in FEV1 (from 58.2 ± 28.2 to 52.8 ± 25.9%, p = 0.2) and FVC-values (from 72.4 ± 26.5 to 67.4 ± 29.1%, p = 0.08).Chronic Pseudomonas aeruginosa infection was more prevalent in the CFRD group (p = 0.003).Conclusion: Continuous glucose monitoring is a useful tool for insight of glucose impairment and diagnosis of CFRD. Early recognition of CFRD and therapeutic intervention has favorable effects on clinical course of the disease.

Highlights

  • METHODSCystic fibrosis (CF) is the most common lethal autosomal recessive disorder in non-Hispanic White with a prevalence of 1: 2,500–5,000 live births [1]

  • Disorders in glucose metabolism can occur in younger age, but the majority of patients are diagnosed with cystic fibrosis-related diabetes (CFRD) during adolescence or in adults [4]

  • In all patients in whom continuous glucose monitoring (CGM) measurement confirmed the diagnosis of CFRD, oral glucose tolerance test (OGTT) results were inconclusive for the diagnosis of CFRD

Read more

Summary

Introduction

Cystic fibrosis (CF) is the most common lethal autosomal recessive disorder in non-Hispanic White with a prevalence of 1: 2,500–5,000 live births [1]. Especially early diagnosis, hypercaloric diet, and aggressive treatment of exacerbations, have led to a shift in patients’ median life expectancy [2]. Prolongation of life led to increased prevalence of cystic fibrosis-related diabetes (CFRD) [1, 3]. The pathophysiological mechanism of CFRD is complex. It is primarily caused by insulin deficiency, but unlike diabetes mellitus type 1, β-cell damage in CF is not of autoimmune origin. Insulin resistance plays a role [6]. Insulin resistance can get worse with acute pulmonary exacerbation, severe chronic lung disease and systemic glucocorticoid therapy [5]

Methods
Results
Conclusion
Full Text
Published version (Free)

Talk to us

Join us for a 30 min session where you can share your feedback and ask us any queries you have

Schedule a call