Cystic fibrosis (CF)-related diabetes (CFRD) is a specific type of diabetes associated with an elevated risk of nutritional (weight loss) and pulmonary complications, and early mortality. A recent Canadian survey of health-care professionals (HCPs) shows great heterogeneity in screening and treatment practices across the country. In addition, North American recommendations are more than 10 years old and do not reflect new knowledge about the increase in life expectancy and weight, especially with the arrival of the genetic therapy era (modulators). The objective of these new guidelines written by the Canadian CFRD Working Group including persons living with CF (pwCF) is to provide an update of the scientific evidence regarding the screening, diagnosis, and care of CFRD.

The classical glycosylated hemoglobin (A1C) threshold of 6.5% is an insensitive screening test for cystic fibrosis (CF)—related diabetes (CFRD). We sought to identify CF-specific A1C thresholds associated with 1) risk of progression to CFRD, and 2) changes in body mass index (BMI) and forced expiratory volume (FEV1).

mathematical algorithms.These systems have primarily been developed for, and studied in, patients with type 1 diabetes.There are limited prospective clinical trials investigating these devices in CFRD.The iLet ® bionic pancreas (BP) uses the Dexcom G6 CGM and automates all insulin delivery after being initialized with only the user's weight, without prescribed insulin settings, and without a run-in period, and adapts autonomously and continually to each user's changing insulin needs.The system eliminates the need for quantitative carbohydrate counting; users enter meal announcements based on meal type ("breakfast," "lunch," or "dinner") and relative size for the user ("usual for me," "less" or "more").Methods: We tested the feasibility of using the BP for the management of CFRD in an open-label, randomized, cross-over trial compared to usual diabetes care (UC).Each study arm was 2 weeks in duration.Participants were instructed to maintain similar dietary habits and exercise habits during both study arms.During the UC arm, prior CGM users wore an unblinded Dexcom G6; otherwise, they wore the blinded Dexcom G6 Pro.Patient selection criteria: Major inclusion criteria were confirmed cystic fibrosis, age ≥10 years, and history of diabetes managed with either an insulin pump or multiple daily insulin injections.Participants were required to have a baseline A1c ≥6% or mean CGM mean glucose ≥125 mg/dL.Exclusion criteria included severe liver disease, end-stage renal disease requiring dialysis, a recent pulmonary exacerbation or current treatment with IV antibiotics, or change in CFTR modulator therapy within the prior 4 weeks.Results: The primary outcome was the percentage of time with CGM glucose in the target range of 70-180 mg/dl.Key secondary endpoints included mean CGM glucose and the percentage of time with CGM glucose <54 mg/dl, <70 mg/dl, >180 mg/dl, and >250 mg/dL.Conclusions: The percentage of time in the target glucose range of 70-180 mg/dl was 74.6% ± 11.3% in the BP arm versus 62.3 ± 22.2% in the usual care arm; p = 0.001.Mean glucose and time spent in hyperglycemic ranges were lower in the BP arm ( p < 0.05 for all), but there were no significant differences in time spent in hypoglycemic ranges between arms ( p > 0.05 for all).No episodes of severe hypoglycemia occurred in either arm.In conclusion, participants using the iLet bionic pancreas for glucose management had improved time in the target glucose range, a lower mean glucose, and less hyperglycemia without increases in hypoglycemia.Larger and longer studies are needed to test the effects of automated glycemic control in patients with CFRD.

variation (Table 1).Those who did not currently have set targets for CGM were asked what targets they would like patients to aim for, again producing varied suggestions (Table 2)

Methods: All U.S. CF care centres were invited to participate in PEP through a call for applications (21 programs applied; 20 participated).The pilot consisted of an 8-hour workshop and follow-up session (3-months post) held at participating CF centres and led by certified CF clinician trainers.Participants completed a series of surveys throughout their participation (before and after the workshop and post follow-up).Patient/family impact was assessed by comparing the CF centre's IntegRATE and CollaboRATE scores from the CFF's Patient and Family Experience of Care (PFEC

Background: For patients with cystic fibrosis (CF), maintaining a normal BMI is associated with better pulmonary function (FEV1) and survival. Given therapy improvements, some patients are now overweight, obese or present rapid weight gain. However, the impact of being overweight on clinical outcomes (e.g. FEV1 & metabolic complications) remains unknown. Methods: Baseline data from 290 adult CF patients and observational follow-up (3.5 years; n = 158) were collected. BMI categories: underweight (UW < 18.5 kg/m(2)), normal (NW 18.5-26.9 kg/m(2)), and overweight/obese (OW >= 27 kg/m(2)). Follow-up data (weight change over time): weight loss (WL>10%), stable (WS), and weight gain (WG>10%). BMI categories and follow-up data were compared to FEV1 and cardiometabolic parameters: glucose tolerance, estimated insulin resistance (IR), blood pressure (BP), and lipid profile. Results: For BMI categories, 35 patients (12.1%) were UW, 235 (81.0%) NW, and 20 (6.9%) OW. Compared to UW and NW patients, OW patients are older (p < 0.001), had less pancreatic insufficiency (p = 0.009), a higher systolic BP (p = 0.004), higher LDL (p < 0.001), and higher IR (p < 0.001). Compared to UW patients, OW patients had a better FEV1 (p < 0.001). For weight change, WL was observed in 7 patients (4.4%), WS in 134 (84.8%) and WG in 17 patients (10.8%). Compared to WL and WS patients, WG patients had a 5% increase in FEV1 accompanied by higher IR (p = 0.017) and triglycerides (p < 0.001). No differences were observed for glucose tolerance for neither BMI nor weight change. Conclusion: A higher weight or weight gain over time are associated with a better FEV1 but also some unfavorable cardiometabolic trends. (C)2020 Elsevier Ltd and European Society for Clinical Nutrition and Metabolism. All rights reserved.

homozygous for F508 del that were seen in our Cystic Fibrosis Centre in year 2017, in order to see whether the sweat chloride concentration available at diagnosis might be prognostic of later growth.Results: This study included 20 patients (11 female), aged 6.45-13.5years(average 9.18).The best body mass index percentile value (BMI%) in year 2017 was analysed.We found a moderate negative correlation between the sweat chloride level at diagnosis and the BMI% (r=-0.492;p < 0.001; y = -0.3907x+ 128.53;R² = 0.2424).Conclusion: These results support the hypothesis that sweat chloride concentration could be predictive of the clinical course of growth in homozygous F508del patients.However, they should be interpreted with caution, since it is a very small study with limitations.A larger and welldesigned longitudinal study that would take in consideration the age and methodology of sweat-test and the mean annual change of BMI could better elucidate this matter.

As high plasma glucose was associated with worse CF outcome. Our objective was to evaluate the association between the 1-hour plasma glucose (PG1) during oral glucose tolerance testing (OGTT) with pulmonary function and nutritional status. Data from Glyc-one database regrouping two prospective observational cohorts of adult CF patients from France (Lyon) and Canada (Montreal). Inclusion criteria were patients with forced expiratory volume in one second (FEV1) ≥ 30% and OGTT PG1 value available both at inclusion (2003-2016). Linear mixed models were used to assess the association between annual changes of FEV1 or BMI and PG1 at baseline (< 11 mmol/L versus ≥ 11 mmol/L) over 4-year follow-up period. Models were adjusted for age, BMI and pseudomonas colonization at baseline, gender, CFTR mutation, the year of study entry and the group of patients. The cohort included 119 French patients [mean ± standard deviation (SD): 24.9 ± 6.8 years] and 210 Canadian patients (24.5 ± 6 years) with a mean baseline FEV1 of 63.9 ± 20.8% and 71.4 ± 18.9% and mean baseline BMI of 20.3 ± 2.2 kg/m2 and 21.4 ± 2.8 kg/m2, respectively. The average follow-up time ± SD was 3.5 years ± 3.0. FEV1 at baseline was significantly greater for Canadian patients than French ones [+5.3%, 95% confidence interval (CI): 1.1 to 9.4; P = 0.013]. The annual rate of decline in the FEV1 was not significantly different between groups and was estimated at −1.0% (95% CI: −1.4 to −0.5). PG1 greater than 11 mmol/L at baseline was associated with a lower mean inclusion FEV1 of −3.5% (95% CI: −1.4 to −0.5, P = 0.082) whereas no significant difference was shown in rate of change of FEV1 over time. BMI at baseline was greater for Canadians than for French patients (+1.9 kg/m2, 95% CI: 1.1 to 2.6) but the change over time (+0.2 kg/m2, 95% CI: 0.1 to 0.3) was similar in both cohorts. No association between PG1 and BMI was shown. Canadian CF patients had a better pulmonary and nutritional status than French patients at their entry in the cohort, but changes over time were comparable between the groups. PG1 values were associated with the mean baseline FEV1 but not with clinical degradation over time. These data do not support a direct role of high PG1 values in clinical evaluation of CF adult patients.

Background: Due to lack of vitamin D absorption in patients with cystic fibrosis (CF), vitamin D supplementation becomes necessary. Our aim was to study the association between serum vitamin D levels and key clinical factors, such as nutritional status, pulmonary function and pulmonary exacerbations (PEx) frequency, in an adult CF population. Methods: Prospective analysis of a published vitamin D (VitD(3)) supplementation protocol (N = 200 adult patients) over a follow-up period of 5 years. Data were collected from the medical files before (baseline) and after (follow-up) the implementation of the VitD(3) supplementation protocol, between 2009 and 2014. Serum samples to measure vitamin D were also collected at baseline and follow-up. Results: A positive relationship between serum vitamin D and lung function was observed at baseline (R = 0.158, P = 0.027), but it disappeared at follow-up (P = 0.454). There was no association between serum vitamin D levels and body mass index. At follow-up, patients with significantly higher serum vitamin D levels were women, older in age, had CF-related diabetes or had a history of recurring PEx. Conclusion: No direct link was observed between heightened serum vitamin D and lung function or BMI in an adult CF population. We suggest that better compliance to treatments and closer follow-up from health professionals could partially explain why such patients reached higher vitamin D serum levels. (C) 2018 Elsevier Ltd and European Society for Clinical Nutrition and Metabolism. All rights reserved.