Perceived burden of respiratory physiotherapy in people with cystic fibrosis taking elexacaftor-tezacaftor-ivacaftor combination: a 1-year observational study.

Background: To limit the progression of disease, people with cystic fibrosis (pwCF) perform daily respiratory physiotherapy, which is perceived as the most burdensome routine in managing their condition. The elexacaftor-tezacaftor-ivacaftor (ETI) combination has changed respiratory management.

Objective: To investigate how the perceived treatment burden changed in 1 year of treatment with ETI.

Short-term effects of positive expiratory pressure mask on ventilation inhomogeneity in children with cystic fibrosis: A randomized, sham-controlled crossover study.

Background: Can physiotherapy with a positive expiratory pressure (PEP) mask improve peripheral ventilation inhomogeneity, a typical feature of children with cystic fibrosis (cwCF)? To answer this question, we used the nitrogen multiple-breath washout (NMBW) test to measure diffusion-convection-dependent inhomogeneity arising within the intracinar compartment (S*VT).

Methods: For this randomized, sham-controlled crossover trial, two NMBW tests were performed near the hospital discharge date: one before and the other after PEP mask therapy (1 min of breathing through a flow-dependent PEP device attached to a face mask, followed by three huffs and one cough repeated 10 times) by either a standard (10-15 cmH0) or a sham (<5 cmH0) procedure on two consecutive mornings. Deception entailed misinforming the subjects about the nature of the study; also the NMBW operators were blinded to treatment allocation.

Limitations of the dichotomized 6-minute walk distance when computing lung allocation score for cystic fibrosis: a 16-year retrospective cohort study.

Purpose: The 2010 Lung Allocation Score (LAS) version considers the estimated survival benefit offered by lung transplantation (LTx) and uses 6-minute Walk Test (6MWT) distance as a dichotomous covariate of whether an individual can walk more than 150 ft or 45.7 m in 6 min. This study aimed to provide evidence that 6MWT gives no clinically meaningful information to be used in the current LAS for candidates to LTx with cystic fibrosis (CF).

Time Free From Hospitalization in Children and Adolescents With Cystic Fibrosis: Findings From FEV, Lung Clearance Index and Peak Work Rate.

Background: An exercise test combined with a multiple breath washout nitrogen test (MBWN) may offer a comprehensive clinical evaluation of cystic fibrosis (CF) disease in children with normal spirometry. The purpose of the present study is to explore whether information derived from spirometry, MBWN, and exercise tests can help the CF multidisciplinary team to characterize time free from hospitalization due to pulmonary exacerbation (PE) in a cohort of pediatric patients with CF.

Methods: This prospective observational study was carried out at the Lombardia Region Reference Center for Cystic Fibrosis in Milano, Italy.

Lung clearance index to characterize clinical phenotypes of children and adolescents with cystic fibrosis.

Background: Lung clearance index (LCI) is accepted as an early marker of lung disease in cystic fibrosis (CF), however the utility of LCI to identify subgroups of CF disease in the paediatric age group has never been explored. The aim of the study was to characterize phenotypes of children with CF using LCI as a marker of ventilation inhomogeneity and to investigate whether these phenotypes distinguished patients based on time to pulmonary exacerbation (PE).

Methods: Data were collected on patients with CF aged < 18 years old, attending the CF Center of Milan during outpatient follow-up visits between October 2014 and September 2019.

Prevalence and factors associated with urinary incontinence in females with cystic fibrosis: An Italian single-center cross-sectional analysis.

Background: Individuals with cystic fibrosis (CF) are deemed to have a higher risk of developing urinary incontinence (UI), likely due to repeated increasing pressure on the pelvic floor. We aimed to determine the prevalence of female UI in a large CF referral center, and to assess the association between UI and severity of CF disease.

Methods: We consecutively recruited female patients regularly attending our CF center, aged ≥6 years and with a confirmed diagnosis of CF.

Home physiotherapists assisting follow-up treatment in cystic fibrosis: a multicenter observational study.

Inhaled therapies are relatively simple and easy to be managed however ineffective use of aerosols when self-administered may occur. We described variation of the number of clinic visits, lung function and number of antibiotic courses performed over 12 months in participants with cystic fibrosis (CF), when supervised or not by physiotherapists (PTs) at home. Participants in 8 Italian CF centers with a prescription of dry-powder antibiotic choose whether to be supervised at home (PT-FU) or not (non-PT-FU), in adjunct to routine clinic visits.

Remote support by multidisciplinary teams: A crucial means to cope with the psychological impact of the SARS-COV-2 pandemic on patients with cystic fibrosis and inflammatory bowel disease in Lombardia.

Background: During Coronavirus Disease 2019 (COVID-19) outbreak in Lombardia, people were recommended to avoid visiting emergency departments and attending routine clinic visits. In this context, it was necessary to understand the psychological reactions of patients with chronic diseases. We evaluated the psychological effects on patients with chronic respiratory conditions and inflammatory bowel disease (IBD) through the analysis of their spontaneous contacts with their referral centres.

Exercise capacity and ventilation inhomogeneity in cystic fibrosis: A cross-sectional study.

Background: Lung clearance index (LCI ) is a marker of overall lung ventilation inhomogeneity and has proven to be able to detect early peripheral damage in subjects with cystic fibrosis (CF), with greater sensitivity than conventional spirometry. Combining its sensitivity with the output of an incremental exercise testing, we hypothesized that any sign of ventilation inhomogeneity in subjects without severe airflow obstruction and with a normal exercise tolerance could be relevant for the CF team, tracking early lung disease and potential exercise limiting factors.

Methods: Patients with CF in clinical stable conditions were recruited between 2015 and 2017.

Delphi poll to assess consensus on issues influencing long-term adherence to treatments in cystic fibrosis among Italian health care professionals.

Purpose: The aim of this study was to determine the level of consensus among Italian health care professionals (HCPs) regarding factors that influence adherence to cystic fibrosis (CF) treatments.

Methods: A Delphi questionnaire with 94 statements of potential factors influencing adherence was developed based on a literature review and in consultation with a board of experts (n=4). This was distributed to a multidisciplinary expert panel of HCPs (n=110) from Italian CF centers.

Expiratory muscle strength and functional exercise tolerance in adults with cystic fibrosis: a cross-sectional study.

Objective: A 6-minute walk work (6MWW), that is the product of distance walked at the 6-minute walk test (6MWT) multiplied by body weight, has been suggested as an appropriate variable for estimating functional capacity. Under the hypothesis that 6MWW strongly correlates with expiratory muscle strength (P max), as found in Chronic Obstructive Pulmonary Disease (COPD), the aim of the study was to determine the degree of this correlation in adults with cystic fibrosis.

Methods: A cross-sectional study was carried out at the Regional Referral Centre for Cystic Fibrosis, where patients with cystic fibrosis attending their regular follow-up, aged 18 years or older with mild-to-normal lung disease, were asked to participate.

Ventilatory limitation and dynamic hyperinflation during exercise testing in Cystic Fibrosis.

Objective: To investigate the presence of dynamic hyperinflation after the Modified Shuttle Test (MST) and its relationship with lung function, exercise tolerance, and clinical symptoms in Cystic Fibrosis (CF).

Methods: Retrospective observational study. Subjects in clinically stable condition with a CF diagnosis based on a positive sweat test (chloride >60 mEq/L) and/or presence of two disease causing mutations, with available data on MST, spirometry, maximal voluntary ventilation, and inspiratory capacity manoeuvres were considered for the analysis.

Pilot Randomized Controlled Trial Evaluating the Effect of Hypertonic Saline With and Without Hyaluronic Acid in Reducing Inflammation in Cystic Fibrosis.

Background: Hypertonic saline (HS) has been established as a therapy aimed at restoring the surface liquid of airways liquid and enhancing mucociliary clearance in patients with cystic fibrosis (CF). A formula containing 7% HS and 0.1% hyaluronic acid (HA) is also available, basing its use on the protective effects of HA against elastin injury and on its greater ease of administration (i.

Aerosol delivery practice in Italian Cystic Fibrosis centres: a national survey.

Background: Physiotherapists (PTs) are ideally positioned to assist patients and families with inhalation therapies through monitoring, communication and education about available therapies and their proper use; indeed aerosoltherapy management is listed as part of Italian PTs' core competence and in the core syllabus for post-graduate training in respiratory physiotherapy. The aim of this study was to outline the involvement of Italian PTs working in Cystic Fibrosis (CF) centres in the aerosol delivery practice.

Methods: Physiotherapist coordinators ( = 29) of all Italian CF centres were invited to participate in a cross-sectional survey and a semi-structured questionnaire was developed and sent by e-mail.

Isocapnic hyperpnea with a portable device in Cystic Fibrosis: an agreement study between two different set-up modalities.

To evaluate the bias and precision of the respiratory muscle training device formulas to predict respiratory minute volume (RMV) and volume of the reservoir bag (BV) on a cohort of subjects with Cystic Fibrosis (CF). CF patients with available pulmonary function tests and maximal voluntary manoeuvres were included in the study. Vital capacity and maximal voluntary ventilation were extracted from subjects' records and then inserted to the manufacturer's formulas to obtain RMV and BV (measured setting).

Intradialytic cycling in children and young adults on chronic hemodialysis.

Background: Intradialytic exercise has been poorly investigated in pediatric patients on chronic hemodialysis (HD). The aim of this study was to assess the acceptability, safety and efficacy of intradialytic exercise in children and young adults on HD.

Methods: The intradialytic exercise program consisted of 30-min sessions of intra-HD exercise using a cycloergometer two to three times a week for 3 months.

Correlates of exercise capacity in pediatric patients on chronic hemodialysis.

Objective: Pediatric patients on chronic hemodialysis (HD) are at high risk of inactivity and poor physical fitness. The aim of this study was to assess the main correlates of exercise capacity in a cohort of children and young adults on chronic HD.

Methods: Twelve patients on chronic HD (median age 15.

Knowledge of oxygen administration, aerosol medicine, and chest physiotherapy among pediatric healthcare workers in Italy.

Background: Oxygen administration, aerosol devices and drugs, or the use of chest physiotherapy are common practices in pediatrics; however, little is known about the knowledge of pediatric healthcare workers concerning the right utilization of these tools. The aim of this study was to fill this gap as a preliminary step in the implementation of appropriate educational programs.

Methods: This cross-sectional survey of a nationally representative sample of Italian pediatricians and nurses was carried out between September 1 and October 8, 2008.