Bacterial Resistance Associated With Inhaled Tobramycin for Prevention of Ventilator-Associated Tracheitis in Mechanically Ventilated Neonates and Children Without Cystic Fibrosis: An Observational Pilot Study.

Early Aggressive Intervention in Cystic Fibrosis: Is It Time To Redefine Our “Best Practice” Strategies?

BackgroundThe effect of elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) on glucose tolerance and/or cystic-fibrosis-related diabetes (CFRD) is not well understood. We performed an observational study on the short-term effects of ELX/TEZ/IVA on glucose tolerance.MethodsSixteen adolescents with CF performed oral glucose tolerance tests (OGTT) before and 4–6 weeks after initiating ELX/TEZ/IVA therapy. A continuous glucose monitoring (CGM) system was used 3 days before until 7 days after starting ELX/TEZ/IVA treatment.ResultsOGTT categories improved after initiating ELX/TEZ/IVA therapy (p = 0.02). Glucose levels of OGTT improved at 60, 90, and 120 min (p < 0.05), whereas fasting glucose and CGM measures did not change.ConclusionShortly after initiating ELX/TEZ/IVA therapy, glucose tolerance measured by OGTT improved in people with CF. This pilot study indicates that ELX/TEZ/IVA treatment has beneficial effects on the endocrine pancreatic function and might prevent or at least postpone future CFRD.

In patients with cystic fibrosis (CF), pulmonary exacerbations (PEx) have an important influence on well-being, quality of life, and lung function decline. Early detection combined with early treatment may prevent severe PEx. To determine whether early detection of PEx is possible by non-invasive markers (volatile organic compounds) in exhaled breath. In a 1 year prospective observational pilot study, 49 children with CF were studied. At clinical visits with an interval of 2 months, lung function, volatile organic compounds (VOCs) in exhaled breath by means of gas chromatography-time-of-flight-mass spectrometry, and medication use were assessed. PEx were recorded. Random forest (RF) classification modelling was used to select discriminatory VOCs, followed by building of receiver operating characteristic curves. An inverse relation between the predictive power of a set of VOCs and time between exhaled breath sampling and the onset of PEx was found. When this time period was within 7 d, the RF model with the nine most discriminatory VOCs was able to correctly predict 79% of the children with an upcoming PEx or remaining stable (sensitivity 79% and specificity 78%). This result was validated by means of bootstrapping within the RF classification model. PEx in children with CF can be detected at an early stage by means of exhaled VOCs. The highest predictive value was reached if time between sampling and the onset of an exacerbation was no longer than 7 d.

BackgroundPhysical activity is a crucial demand on cystic fibrosis treatment management. The highest value of oxygen uptake (VO2peak) is an appropriate tool to evaluate the physical activity in these patients. However, there are several other valuable CPET parameters describing exercise tolerance (Wpeak, VO2VT1, VO2VT2, VO2/HRpeak, etc.), and helping to better understand the effect of specific treatment (VE, VT, VD/VT etc.). Limited data showed ambiguous results of this improvement after CFTR modulator treatment. Elexacaftor/tezacaftor/ivacaftor medication improves pulmonary function and quality of life, whereas its effect on CPET has yet to be sufficiently demonstrated.MethodsWe performed a single group prospective observational study of 10 adolescent patients with cystic fibrosis who completed two CPET measurements between January 2019 and February 2023. During this period, elexacaftor/tezacaftor/ivacaftor treatment was initiated in all of them. The first CPET at the baseline was followed by controlled CPET at least one year after medication commencement. We focused on interpreting the data on their influence by the novel therapy. We hypothesized improvements in cardiorespiratory fitness following treatment. We applied the Wilcoxon signed-rank test. The data were adjusted for age at the time of CPET to eliminate bias of aging in adolescent patients.ResultsWe observed significant improvement in peak workload, VO2 peak, VO2VT1, VO2VT2, VE/VCO2 slope, VE, VT, RQ, VO2/HR peak and RR peak. The mean change in VO2 peak was 5.7 mL/kg/min, or 15.9% of the reference value (SD ± 16.6; p= 0.014). VO2VT1 improved by 15% of the reference value (SD ± 0.1; p= 0.014), VO2VT2 improved by 0.5 (SD ± 0.4; p= 0.01). There were no differences in other parameters.ConclusionExercise tolerance improved after elexacaftor/tezacaftor/ivacaftor treatment initiation. We suggest that the CFTR modulator alone is not enough for recovering physical decondition, but should be supplemented with physical activity and respiratory physiotherapy. Further studies are needed to examine the effect of CFTR modulators and physical therapy on cardiopulmonary exercise tolerance.

<h3>Introduction</h3> Cardiopulmonary exercise testing (CPET) is the gold standard test of aerobic fitness in Cystic Fibrosis (CF). CPET is measured by peak oxygen uptake (VO2 peak) during maximal exercise. It can be used to identify the cause of exercise intolerance, prescribe exercise programmes, evaluate therapeutic interventions¹ and predict prognosis.² The American Thoracic Society defines a normal VO2 peak as &gt;84% predicted.³ In our centre, CPET was included in the CF annual reviews from 2013. Currently no guidelines exist as to the frequency of CPET in CF patients. <h3>Aims</h3> To determine whether CPET was a useful and feasible routine test in children with CF and to assess whether VO2 peak correlated with Forced Expiratory Volume in 1 s (FEV1), disease severity, gender, genotype or previous intravenous antibiotics. <h3>Method</h3> A pilot observational study was performed. Body mass index (BMI) was used as a marker of disease severity. Genotype was divided into 3 groups; DF508 homozygous, heterozygous and all other genotypes. Data was retrieved from case notes and our CF database. <h3>Results</h3> 38 patients (17 male, 21 female) underwent CPET. Age range 7–14 (mean 9.8) years. 36/38 had technically satisfactory CPET. Mean VO2 peak was 107% predicted, standard deviation 18%. Only 8% had an abnormal VO2 peak. Mean FEV1 was 91% predicted, standard deviation 15%. There was no relationship between VO2 peak and FEV1 [p = 0.297] or BMI [p = 0.382] (Pearson correlation). Additionally, no correlation was demonstrated between VO2 peak and genotype [p = 0.236] (one-way ANOVA), gender [p = 0.902] or patients who had received at least one course of intravenous antibiotics in the past year [p = 0.253] (two-sample T test). <h3>Conclusions</h3> CPET is a feasible test with 95% of our patients achieving technically satisfactory assessments and reassuringly, VO2 peak was largely normal. We could not demonstrate a relationship with FEV1 or disease severity although our study is limited by the small sample size. It is recognised that the CF annual review is already a long day for patients and we plan to reserve CPET for those showing exercise intolerance rather than performing it annually. <h3>References</h3> Rogers D, Prasad SA, Doull I. Exercise testing in children with cystic fibrosis. <i>J Royal Society Med</i> 2003;<b>96</b>:23–29 Nixon PA, Orenstein DM, Kelsey SF, <i>et al</i>. The prognostic value of exercise testing in patients with cystic fibrosis. <i>N Engl J Med</i> 1992;<b>327</b>:1785–88 American Thoracic Society/American College of Chest Physicians. ATS/ACCP statement on cardiopulmonauy exercise testing. <i>Am J Respir Crit Care Med</i> 2003;<b>167</b>:211–77

Background and Aims Maintenance hemodialysis (HD) and peritoneal dialysis (PD) patients have higher incidence of bone fractures and cardiovascular morbidity and mortality compared to the general population. We aimed to investigate the association between bone density and vascular calcification in HD and PD patients. Method In a pilot observational cohort study we included 56 patients (39 HD, 17 PD), 34 men with mean age 60 (±13) years and dialysis vintage 41 months (15–74 range). Vascular calcification was recorded as abdominal aortic calcification (AAC) by Leena Kauppila (LK) score (range 0–24) in a lateral lumbar spine x-ray. It was categorized as low score if AAC ≤4 or increased if &amp;gt;4. Bone density was recorded based on DEXA scan of right femoral bone as normal (NBD), osteopenia or osteoporosis. Laboratory parameters of Chronic kidney disease- Mineral Bone Disorders were recorded plus drug use and the presence or no of cardiac valve calcification. Results The prevalence of increased AAC in the three bone density groups (normal 29%, osteopenia 46%, osteoporosis 25%) was respectively 31, 46 and 50% (P = 0.050). Serum levels of Parathormone, calcium and calcium phosphate product were statistically increased in osteoporosis group compared to NBD and osteopenia. The same was found for use of calcimimetics, vitamin D agonists, aspirin and heparin (see Table 1). In patients with osteopenia and osteoporosis (n = 40) higher serum magnesium was associated with lower risk of AAC &amp;gt;4 [OR = 0.05 (95%CI: 0.0–0.067)]. On the other hand heparin use between hemodialysis sessions [OR = 13.48 (95%CI: 1.53–119.13)], arterial hypertension [OR=16.77 (95% CI: 1.27–221.27)], cardiac valve calcification [OR = 12.54 (95% CI: 1.48–106.58)], were associated with higher risk of AAC &amp;gt;4. Increase of LK score by 1 was associated with almost 5 times higher risk of coronary artery disease [OR = 5.40 (95% CI: 1.18–64.25)]. In patients with NBD, male sex, pulse pressure and aspirin use were positive predictive factors of AAC &amp;gt;4. Conclusion In this pilot observational study, abnormal bone density and increased vascular calcification (AAC &amp;gt; 4) are highly prevalent in hemodialysis patients (71% and 43%). Maybe their investigation by simple tools as DEXA and LK score could help in identifying patients with high risk of cardiovascular disease.

PURPOSE The implementation of Technology-Assisted Rehabilitation (TAR) introduces both challenges and opportunities to enhance patient-therapist interactions. However, the therapist’s perspective on TAR often remains understudied. This study aims to address this gap by investigating therapist perceptions of Robot-Assisted Gait Training (RAGT) by developing a specific questionnaire. METHODS The study is a real-life observational and multicentric pilot study. First, a multidisciplinary board was created to develop the questionnaire. Second, an observational pilot study was conducted, where the questionnaire was administered to therapists involved in RAGT working with a lower-limb robotic device for gait disorders. RESULTS Thirty-two questionnaires were collected. Results indicate favourable perceptions among therapists in terms of device’s usefulness, ease of use and safety. However, despite the overall positive response trend, therapist engagement showed more variability. CONCLUSIONS The findings underscore the need of reconceptualizing therapist contribution in TAR, for optimal integration into clinical practice and to improve patient-therapist-device interaction.

The 49th parallel: Does geographic position affect longevity of patients with cystic fibrosis?

Physical restraint (PR) is applied for patients' safety and to prevent the removal of inserted devices. No matter how well applied, PR causes undesired effects and discomfort to patients. Because PR-Guidelines are not yet implemented in Turkey, an observational study was performed to get baseline data on the type and number of PR-activities and on patients' complications in intensive care unit (ICU) patients. An observational pilot study was conducted in anesthesia and reanimation adult ICUs in a midsized general hospital in Turkey. Included were 31 patients and two data collection tools: a basic form (patient demographics, medical information, and complications) and a PR observation guide on nurses' PR-activities. Descriptive statistics (frequencies, percentages, and mean and standard deviation) were used for data evaluation. Most patients (61.3%) were male, and 74.2% were aged 60-79 years. Almost a third was unconscious (Glasgow Coma Scale <9) and at risk for falling. Of the total 33 activities of the Nursing Interventions Classification (NIC), 13 were never applied in 33% of patients. The most applied activity was "provide sufficient staff to assist with the safe application of physical restraining devices or manual restraints" (96.8%). Least applied were "explain inpatient and significant others the behaviors necessary for the termination of the intervention," "Provide the dependent patient with a means of summoning help" (6.5%), and "Teach family the risks and benefits of restraint reduction" (3.2%). Overall, 58.1% of patients had PR complications. For the first time, PR NIC activities were evaluated in a Turkish ICU. Findings show low performance of NIC activities and a high complication rate. The findings provide the basis to implement a PR-Guideline in Turkish ICUs to enhance patients' safety and comfort.

A Pilot Study of Mindfulness-Based Cognitive Therapy to Improve Well-Being for Health Professionals Providing Chronic Disease Care

Respiratory infections caused by respiratory viruses are common in paediatric cystic fibrosis (CF) patients and are associated with increased morbidity. There is only little data on the incidence of viral respiratory pathogens causing exacerbations in the adult CF patient population. In this observational pilot study we show, by using molecular as well as conventional techniques for viral isolation, that during 1 y a viral pathogen could be isolated in 8/24 (33%) adult CF patients who presented with a pulmonary exacerbation. This result shows that there is a considerable incidence of viral pathogens in pulmonary exacerbations in adult CF patients. Newly identified viruses such as pandemic influenza A/H1N1, human metapneumovirus, human bocavirus, and human coronavirus NL63 were not detected in our population, except for 1 human coronavirus NL63.

BackgroundThe early beginning of an adequate antibiotic therapy is crucial in hospital-acquired pneumonia (HAP), but depends on the results of conventional microbiological diagnostics (cMD). It was the aim of this study to evaluate the performance and turnaround times of a new point-of-care multiplex polymerase chain reaction (mPCR) system for rapid identification of pathogens and antibiotic resistance markers. We assessed the applicability of the system under real-life conditions in critical ill patients with HAP.MethodsWe enrolled forty critical ill patients with clinical signs for HAP into an observational study. Two samples of respiratory secretions were collected during one course of aspiration and cMD and mPCR testing (Unyvero, Curetis AG, Holzgerlingen, Germany) were performed immediately. The mPCR device was operated as a point-of-care system at the intensive care unit. We compared turnaround times, results of pathogen identification and results of antibiotic resistance testing of both methods.ResultsMean turnaround times (min-max) were 6.5 h (4.7–18.3 h) for multiplex PCR and 71 h (37.2–217.8 h) for conventional microbiology (final cMD results, incomplete results neglected). 60 % (n = 24) of the mPCR tests were completely valid. Complete test failure occurred in 10 % (n = 4) and partial test failure occurred in 30 % (n = 12). We found concordant results in 45 % (n = 18) and non-concordant results in 45 % (n = 18) of all patients. 55 % (n = 16) of the results were concordant in patients with a clinical pulmonary infection score (CPIS) > 5 (n = 29). Concordant results included three cases of multidrug resistant bacteria. MPCR frequently detected antibiotic resistance markers that were not found by cMD.ConclusionsUnyvero allowed point-of-care microbial testing with short turnaround times. The performance of the system was poor. However, an improved system with a more reliable performance and an extended microbial panel could be a useful addition to cMD in intensive care medicine.Trial registrationClinicalTrials.gov NCT01858974 (registered 16 May 2013)

The current literature inadequately addresses the extent to which remote monitoring should be integrated into care models for chronic respiratory diseases (CRDs). This study examined a remote monitoring program (RMP) in cystic fibrosis (CF) by exploring experiences, future perspectives, and use behavior over 3 years, with the aim of developing future directions for remote monitoring in CRDs. This was a mixed methods, multicenter, observational study in 5 Dutch CF centers following a sequential explanatory design. Self-designed questionnaires using the technology acceptance model were sent out to people with CF who had a minimum of 12 months of experience with the RMP and local health care professionals (HCPs). Questionnaire outcomes were used to inform semistructured interviews with HCPs and people with CF. Qualitative findings were reported following the COREQ (Consolidated Criteria for Reporting Qualitative Research) checklist. Anonymous data on use frequency of all people with CF were analyzed. Between the second quarter of 2020 and the end of 2022, a total of 608 people with CF were enrolled in the program, and a total of 9418 lung function tests and 2631 symptom surveys were conducted. In total, 65% (24/37) of HCPs and 89% (72/81) of people with CF responded to the questionnaire, and 7 HCPs and 12 people with CF participated in semistructured interviews. Both people with CF and HCPs were positive about remote monitoring in CF care and found the RMP a good addition to daily care (people with CF: 44/72, 61%; HCPs: 21/24, 88%). Benefits ranged from supporting individual patients to reducing health care consumption. The most valued monitoring tool was home spirometry by both people with CF (66/72, 92%) and HCPs (22/24, 92%). Downsides included the potential to lose sight of patients and negative psychosocial effects, as 17% (12/72) of people with CF experienced some form of stress due to the RMP. A large majority of people with CF (59/72, 82%) and HCPs (22/24, 92%) wanted to keep using the RMP in future, with 79% (19/24) of HCPs and 75% (54/72) of people with CF looking forward to more replacement of in-person care with digital care during periods of well-being. Future perspectives for the RMP were centered on creating hybrid care models, personalizing remote care, and balancing individual benefits with monitoring burden. Remote monitoring has considerable potential in supporting people with CF and HCPs within the CF care model. We identified 4 practice-based future directions for remote monitoring in CF and CRD care. The strategies, ranging from patient driven to prediction driven, can help clinicians, researchers, and policy makers navigate the rapidly changing digital health field, integrate remote monitoring into local care models, and align remote care with patient and clinician needs.

Primary Ciliary Dyskinesia and Cystic Fibrosis: Different Diseases Require Different Treatment

Background: Cricket is a widely played sport and has been evolved over the years. Agility being the key component of cricket is a collective coordinative ability that includes speed, balance and coordination and plays an important role in determining a player’s performance. The agility of players differs on the basis of their anthropometric measurements, position, training period and type of innings played. Improved agility enhances neuromuscular coordination thereby reducing the risk of injuries to the players. Cricketers require different types of agility training to meet their role in a match. Limited literature is available evaluating agility of cricket players. Hence, a need arises to conduct this study. The aim of this study is to evaluate and compare the agility of players depending on their positions. Objective: To evaluate and compare the agility of cricket players depending on their playing position using Agility T-test. Method: Institutional ethical clearance and consent from the players were sought. An observational pilot study was conducted on 30 players and were divided into 3 groups- batsmen, bowler and wicketkeepers Agility-T test was used to measure agility. Data was analysed using the software Graph Pad Prism, normality of the data was calculated using the Shapiro Wilk Test and comparison between the groups was done using One way ANOVA test. Results: The agility differed significantly (p value&lt;0.05). From the study it was revealed that the agility timing scores of cricket players varied according to their position. Batsmen recorded the lowest time followed by wicketkeeper whereas bowlers recorded the highest timings. Conclusions: A significant difference was found in the 3 groups of cricketers. Differences in current finding will help in formulating a structured agility training protocol depending on cricketers’ position. Keywords: [cricketers, cricket, agility, playing position, Agility T-test].