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Introduction Stenotrophomonas maltophilia is common in the sputum of people with cystic fibrosis related diabetes (CFRD), raising the question as to whether this is a risk factor for its acquisition. We investigated this at a population level. Methods We analysed national Cystic Fibrosis Registry data 2011–2015 for 8047 people with CF > age 6 years, looking at demographics, diagnosis of CFRD, lung function and sputum microbiology; using descriptive and multivariate strategies to establish independent predictors for S. maltophilia culture and associated outcomes. Results S. maltophilia was present in 1148 (14.1%). Although univariate analysis confirmed it was more prevalent in those with CFRD, when adjusted for other clinical parameters there was no longer a relationship. Markers of more severe lung disease were independent risk-factors for S. maltophilia . Conclusion Although S. maltophilia is more common in people with CFRD, it is not an independent risk-factor for S. maltophilia acquisition.

Introduction Continuous glucose monitoring (CGM) allows assessment of day to day glycaemic excursions and detects early glucose handling abnormalities that may not be apparent on oral glucose tolerance testing (OGTT). However, there is little published evidence as to whether these early dysglycaemic changes are amenable to treatment. We present outcomes following CGM guided insulin initiation at our centre. Methods Adults without a prior diagnosis of cystic fibrosis related diabetes (CFRD) whom underwent >72 h CGM at our adult CF centre were included in the study. Clinical outcomes including weight and pulmonary function changes over the next 12 months were compared between groups based on CGM results and subsequent management. Results CGM profiles for 59 patients were analysed. Insulin was commenced in 37 patients who had evidence of hyperglycaemia on CGM. Significant improvements in mean [95% confidence intervals] forced expiratory volume in 1 s (FEV1) (+4.3% predicted [1.06–7.48], p = 0.01) and weight (+1.2 kg [0.32–2.15], p = 0.01) were observed at 3 months in the insulin group. Annual rate of pulmonary function decline was also improved following insulin initiation. Conclusion Insulin treatment targeted towards glycaemic excursions seen on CGM is associated with improvements in lung function and weight with subsequent reduced pulmonary function decline.

Renal disease is a well-recognized manifestation of cystic fibrosis (CF) and people with CF are at increased risk of nephrolithiasis. Lithotripsy is the preferred treatment but has occasionally been associated with pulmonary complications. Here we report the case of a person with CF who developed a pneumothorax soon after lithotripsy and discuss the potential mechanism of injury. We hope this case highlights some of the additional considerations clinicians should take into account when managing patients with advanced pulmonary disease in CF.

Cystic fibrosis is primarily considered a respiratory disease with superadded pancreatic dysfunction; however the underlying genetic mutation results in the abnormal function of cells throughout the body - including tissues involved in reproduction. Practically all males and up to 50% of females with CF will suffer fertility issues - the causes of which are multifactorial and could be improved by treatments that target the underlying physiological deficit, such as ivacaftor. We consider the mechanisms by which the rapidly developing field of systemic treatments for CF could impact on the fertility of this group and review the available real world evidence.

Malignant large airway obstruction is life threatening and may not be amenable to urgent radiotherapy. Palliative airway stenting is difficult and traditionally carried out under general anaesthesia and fluoroscopy. We have shown that self expanding Gianturco metal stents can be placed under local anaesthesia using fibreoptic bronchoscopy and direct vision for the treatment of malignant airway tumours, and report our 10 year experience. All referrals for stenting referred to our unit between 1990 and 1999 were included, looking for histological type, number and site of stents, complications of the procedure, other interventions, and survival. One hundred and sixty two patients (average age 64 years, (range 21–89)) had 307 stents inserted during 167 procedures (144 primary lung tumours, 18 secondary malignancy). There were no operative deaths, but three patients developed a pneumothorax, one requiring intercostal drain insertion. Average survival following stent insertion was less for primary lung cancer than for secondary disease (103 vs. 431 days, P

Telomerase is a ribonucleoprotein DNA polymerase that maintains the telomeric region of chromosomes lost during successive rounds of cell division. We used the telomeric repeat amplification protocol (TRAP) assay to examine telomerase activity in bronchial lavage (BL) samples from individuals undergoing diagnosis of lung cancer. Telomerase activity was detected in 17 (47%) of 36 samples examined. In particular, 16 (70%) of 23 BL specimens obtained from lung cancer patients showed detectable telomerase activity, while only 1 of 13 (8%) specimens obtained from patients without lung cancer demonstrated activity (P=0.00038). Moreover, 9 (90%) of 10 BL specimens, which were cytologically positive for lung cancer, were also positive for telomerase activity, while 7 (54%) of 13 cytologically negative BL specimens for lung cancer showed detectable telomerase activity. Detection of telomerase activity combined with cytology were able to identify 17 (74%) of 23 lung cancer cases whereas cytology alone identified 10 (43%) of 23 such cases (P=0.035). Our findings indicate that telomerase is a specific marker for malignant lung disease and a potential complementary tool to cytology in the diagnosis of certain lung cancer cases.

Background Cystic fibrosis (CF) affects over 9,000 people in the UK and limits life expectancy. CF patients are susceptible to lung infections, most commonly Pseudomonas aeruginosa. Once infection is established, patients require lifetime treatment using nebulised antibiotics. Newer dry powder formulations of antibiotics may reduce treatment burden and improve compliance.

This is a personal selection of papers that were presented at the 27th North American Cystic Fibrosis Conference held in Salt Lake City in October 2013. The papers discussed in this review are thought to be of particular interest to CF caregivers in the UK.

Summary Infection with transmissible strains of Pseudomonas aeruginosa can occur in uncolonised patients, but cross infection (superinfection) of patients already colonised with P aeruginosa has not been reported. With genotypic identification, we found superinfection by a multiresistant transmissible strain of P aeruginosa in four patients with cystic fibrosis (CF) who were already colonised by unique strains of P aeruginosa . No evidence of environmental contamination was found, but all patients became superinfected after contact with colonised individuals during inpatient stays. Inpatients with CF who are colonised with P aeruginosa should be separated by strain type. Such strain typing can only be reliably done by genomic methods, but this has resource implications.

Objectives GLP1, an incretin hormone important in post-prandial glucose regulation, is inactivated by enzymes which may be up-regulated in inflammatory conditions such as CF. We looked for evidence of incretin inactivation in individuals with and without CF-related diabetes (CFRD). Methods GLP1 levels (total & active) were measured during stimulation (oral glucose tolerance test) in 10 individuals with CF (5 CFRD) whilst clinically stable and compared to a matched control group. Samples were processed with DPP4 inhibitor using an ELISA assay (Millipore, Germany). Results Baseline circulating levels of both active and total GLP1 were similar in all groups. Total GLP1 responses were most pronounced in the CFRD group whose levels rose most sharply; peaked highest and then returned to baseline quicker than the remaining CF patients who in turn had a higher and more sustained rise than controls. Both CF groups therefore significantly hypersecreted total GLP1 (p CFRD (n = 5) Nondiabetics (n = 5) Controls (n = 6) Baseline GLP1 levels 12.9 (2.5) 11.4 (3.0) 10.0 (2.5) Peak GLP1 27.0 (7.9) 22.8 (7.2) 13.1 (6.3) Time to peak GLP1 (min) 30 (15) 45 (45) 30 (30) % total secretion 0–30 min 78% (65%) 51% (47%) 61% (49%) % total secretion 0–60 min 100% (92%) 91% (91%) 95% (97%) Total incremental rise in GLP1 448.6 (203.0) 736.9 (272.4) 178.1 (167.1) Values in parentheses are active levels. Conclusion Individuals with CF, irrespective of glycemic status, hypersecrete total GLP1 to achieve similar levels of active hormone seen in matched controls. This suggests that CF is associated with higher levels of incretin degradation even in stable non-diabetic patients. Delayed insulin release – common in CFRD – may contribute to the high peaks of GLP1 seen in this group, as normal physiological negative feedback will be lost.

Objectives The development of complex self-management skills for successful CFRD outcomes requires education and supervision from the CF team. To aid this, we explored patients’ views on what is important in CFRD education. Methods When in a stable state, 60 of our 133 CFRD patients (45%) completed a 4-point scale of importance questionnaire covering the sources of CFRD information and areas of education (biology, glycaemic control, complications, effects on daily life). Results 58 (97%) felt reasonably/ very well informed. The most common sources of information (each could endorse multiple sources) were the CFRD team (80%), UK CF Trust (18%) and paediatric CF team (13%). 54 (90%) said they had received the right amount of information. 56 (93%) would go to the CFRD practitioner with questions. Glycaemic control skills were rated as quite or very important by all, with the exception of one person who rated insulin storage “a little important”. All thought information on complications quite/ very important. A small minority thought information on screening a little important (1, 3 and 2 respondents for eye, foot and renal screening). Biology of CFRD was the least valued information (up to 55% of items rated very important). Lifestyle and CFRD ratings varied (up to 70% rated “very important”). Conclusion The CFRD service is vital in providing education to patients. Patients feel well informed, and value a wide range of information. The challenge is to deliver this in a useable way. CFRD biology is important but undervalued by some patients. Education on lifestyle and CFRD should begin with assessment of areas pertinent to the individual to target information.