The role of inspiratory capacity and tidal flow in diagnosing exercise ventilatory limitation in Cystic Fibrosis.

Background: Exercise ventilatory limitation conventionally defined by reduced breathing reserve (BR) may underestimate the effect of lung disease on exercise capacity in patients with mild to moderate obstructive lung diseases.

Objective: To investigate whether ventilatory limitation may be present despite a normal BR in Cystic Fibrosis (CF).

Methods: Twenty adult CF patients (age 16-58y) with a wide range of pulmonary obstruction severity completed a symptom-limited incremental exercise test on a cycle ergometer.

Spontaneous pneumothorax-When do we need to intervene?

Background: Pneumothorax can be classified as traumatic, iatrogenic or spontaneous (SP), which can be subdivided into primary spontaneous pneumothorax (PSP), a condition without preexisting lung disease, or secondary spontaneous pneumothorax (SSP) a complication of a preexisting lung disease. Recurrence rate of PSP is 30% whereas for SSP rate is unknown. This article explores the experience of a tertiary center over 20 years.

Advanced Lung Disease in Patients with Cystic Fibrosis Is Associated with Low Diffusion capacity.

Background: The single-breath diffusing capacity of the lungs (DLCOSB) test measures the extent to which carbon monoxide (CO) passes from the lung air sacs into the blood. The accessible alveolar volume (VASB) is measured by inert gas during a 10-second period. The single-breath transfer coefficient of the lung for carbon monoxide (KCOSB) is the DLCOSB divided by VASB.

The benefits of mechanical insufflator-exsufflator compared to autogenic drainage in adults with cystic fibrosis.

Background: Autogenic drainage (AD) is used for airway clearance in individuals with cystic fibrosis (CF). The mechanical insufflator-exsufflator (MI-E) device is commonly used in patients with neuromuscular diseases for airway clearance by increasing inspiratory volume and expiratory flow. MI-E use has not been studied extensively in CF patients.

Resting Energy Expenditure in Patients With Familial Dysautonomia: A Preliminary Study.

Objectives: Familial dysautonomia (FD) is a rare hereditary sensory and autonomic neuropathy characterized by chronic lung disease and cyclic vomiting due to hyper-adrenergic crises. Most FD patients are in a depleted nutritional state; however, the phenotype of the disease is quite different between patients, as for the severity of lung disease and the intensity and frequency of these pathognomonic crises. In this study we wanted to investigate whether resting energy expenditure (REE) levels are increased in this population, and if correlations exist between REE levels and phenotype severity.

Ivacaftor for the p.Ser549Arg (S549R) gating mutation - The Israeli experience.

Background: Ivacaftor is a drug that increases the probability of the cystic fibrosis transmembrane conductance regulator (CFTR) chloride channel remaining open. Information about the efficacy of ivacaftor in patients carrying the rare p.Ser549Arg (S549R) CFTR mutation is sparse.

High Mid-Flow to Vital Capacity Ratio and the Response to Exercise in Children With Congenital Heart Disease.

Background: Pulmonary mechanics may play a role in exercise intolerance in patients with congenital heart disease (CHD). A reduced FVC volume could increase the ratio between mid-flow (FEF) and FVC, which is termed high dysanapsis. The relationship between high dysanapsis and the response to maximum-intensity exercise in children with CHD had not yet been studied.

Assessment of Airway Bronchodilation by Spirometry Compared to Airway Obstruction in Young Children with Asthma.

A reversibility test by an increase of greater than 12% in FEV1 can support a diagnosis of asthma and alter a patient's treatment plan but may not be applicable to the young ages. We retrospectively gathered spirometric data from 85/271 asthmatic children having mild obstruction (FEV1 > 80% predicted), age 2.6-6.

Consequences of Expiratory Flow Limitation at Rest in Subjects with Cystic Fibrosis.

Rationale: Expiratory flow limitation at resting tidal volume (EFLTV) presents a severe mechanical constraint in chronic lung diseases and has not yet been studied longitudinally in cystic fibrosis.

Objectives: To study the effect of EFLTV as it emerged from simple spirometry on lung function and clinical status in cystic fibrosis.

Methods: Best year spirometry that included tidal flow/volume curves and the related clinical data were retrospectively collected over 12 ± 3.

Glucose intolerance in cystic fibrosis as a determinant of pulmonary function and clinical status.

Background: Cystic fibrosis related diabetes (CFRD) is associated with a decrease in pulmonary function and nutritional status. We investigated the clinical significance of impaired glucose tolerance (IGT) in cystic fibrosis (CF) patients.

Methods: Fifty-five CF patients (aged 22.

The Short-Term Effect of Breathing Tasks Via an Incentive Spirometer on Lung Function Compared With Autogenic Drainage in Subjects With Cystic Fibrosis.

Background: Forced expiration may assist secretion movement by manipulating airway dynamics in patients with cystic fibrosis (CF). Expiratory resistive breathing via a handheld incentive spirometer has the potential to control the expiratory flow via chosen resistances (1-8 mm) and thereby mobilize secretions and improve lung function. Our objective was to explore the short-term effect of using a resistive-breathing incentive spirometer on lung function in subjects with CF compared with the autogenic drainage technique.

Cough ability measurements and recurrent respiratory symptoms in individuals with Ataxia Telangiectasia.

Objectives: Ataxia-Telangiectasia (A-T) individuals often present with respiratory muscle weakness, causing recurrent respiratory system infections, asthma-like symptoms, and chronic cough life-threatening events. The cough flow volume maneuver may reveal powerless airflow needed for efficient cough. The study aims to explore cough ability in relation to the flow/volume maneuver.

Nocardia Colonization: A Risk Factor for Lung Deterioration in Cystic Fibrosis Patients?

Background: Cystic fibrosis (CF) patients are predisposed to infection and colonization with different microbes. Some cause deterioration of lung functions, while others are colonizers without clear pathogenic effects. Our aim was to understand the effects of Nocardia species in sputum cultures on the course of lung disease in CF patients.

FVC deterioration, airway obstruction determination, and life span in Ataxia telangiectasia.

Rationale: Forced vital capacity (FVC) values decrease with progress of the disease in Ataxia telangiectasia (AT).

Objective: To study the effect of this process on airway obstruction determination and life span in AT.

Methods: Clinical data and yearly best spirometry maneuvers were collected retrospectively from 37 AT patients (196 spirometry tests) during 5.

Maximal flow at functional residual capacity in asthmatic preschool children.

Background: Many preschool children will perform correct peak-flow but will not exhale to residual volume, thus limiting the determination of airways obstruction. The maximal flow measured at function residual capacity (V'maxFRC) is independent of lung empting and could potentially serve as a parameter for describing flow at low lung volumes. The study determines the detection of airway obstruction/dilation in asthmatic preschool children by V'maxFRC, compared to FEV1 and FEF25-75.

Long-term follow-up of distal intestinal obstruction syndrome in cystic fibrosis.

Aim: To investigate the long-term follow-up of distal intestinal obstruction syndrome (DIOS) in Israeli cystic fibrosis (CF) patients.

Methods: This is a multi-center, comparative, retrospective study in which we reviewed the medical records of all CF patients from three major CF centers in Israel who were treated in the period from 1980 to 2012. Patients diagnosed with DIOS were defined as the study group.

Several siblings with Cystic Fibrosis as a risk factor for poor outcome.

Background: Occurrence of Cystic Fibrosis (CF) in more than one member in a family is not uncommon. The aim of our study was to assess the influence of multiple siblings with CF on disease expression and outcome.

Methods: Study group consisted of 2-siblings (2-sibs, n = 42) or 3/4 siblings (3/4-sibs, n = 22) with CF in one family.

Cough characteristics and FVC maneuver in cystic fibrosis.

Background: Cough is part of life in patients with cystic fibrosis (CF). Weak coughing may add to increased respiratory disease that affects the mechanical properties of cough in these patients. In this study, we investigated cough characteristics in relation to forced expiratory flow/volume indices in CF.

Forced expiratory decay in asthmatic preschool children--is it adult type?

Background: The forced expiratory decay in healthy preschool children portrays a convex shape that differs from the linear decay in the older healthy population. The "adult-type" expiratory decay during airway obstruction is concave. The study objective was to determine if the expiratory decay in young asthmatic children is "adult-type".

An official American Thoracic Society workshop report: optimal lung function tests for monitoring cystic fibrosis, bronchopulmonary dysplasia, and recurrent wheezing in children less than 6 years of age.

Although pulmonary function testing plays a key role in the diagnosis and management of chronic pulmonary conditions in children under 6 years of age, objective physiologic assessment is limited in the clinical care of infants and children less than 6 years old, due to the challenges of measuring lung function in this age range. Ongoing research in lung function testing in infants, toddlers, and preschoolers has resulted in techniques that show promise as safe, feasible, and potentially clinically useful tests. Official American Thoracic Society workshops were convened in 2009 and 2010 to review six lung function tests based on a comprehensive review of the literature (infant raised-volume rapid thoracic compression and plethysmography, preschool spirometry, specific airway resistance, forced oscillation, the interrupter technique, and multiple-breath washout).

Progressive flow-to-volume dysanapsis in cystic fibrosis: a predictor for lung transplantation?

Rationale: Airways obstruction and lung volume restriction, major features of lung disease in cystic fibrosis (CF), may regress independently, causing dysanapsis between these parameters.

Objectives: To explore the significance of dysanapsis (FEF(25-75)/FVC) ratio in CF.

Methods: Yearly best spirometry data, collected during 8.

The effect of beclomethasone dipropionate in ultrafine particles on bronchial hyper-reactivity in young children.

Aim: Bronchial hyper-reactivity (BHR) provides a tool for asthma diagnosis, assessment of severity and response to treatment. The effect of beclomethasone dipropionate in ultrafine particles (BDP-HFA) on BHR as measured by the adenosine challenge test in young children has not yet been determined. Our aim was to determine the effect of BDP-HFA (100 μg twice daily) on BHR as evaluated by a reduction of 20% from baseline FEV1 (PC20-FEV1) values in young asthmatic children.

Hospital versus home treatment of respiratory exacerbations in cystic fibrosis.

Background: Treatment of respiratory exacerbations in Cystic Fibrosis (CF) is important in slowing disease progression. The treatment may be given either at home or at the hospital. The aim of our study was to compare both treatment settings.