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lung function tests in cystic fibrosis

Valérie Boudreau, Quitterie Reynaud, Catherine Lehoux Dubois, Adèle Coriati, Katherine Desjardins, Isabelle Durieu, Rémi Rabasa-Lhoret
Nearly 50% of adult patients with cystic fibrosis (CF) have diabetes. The occurrence of CF-related diabetes (CFRD) is preceded and is associated with deterioration of lung function and nutritional status. Microvascular complications can occur, but the main cause of death is respiratory failure rather than cardiovascular causes as in type 1 or type 2 diabetes. Because other methods such as glycated hemoglobin (A1C) levels are less sensitive in patients with CF, the recommended screening test is the oral glucose tolerance test (OGTT) with a 75 g glucose dose...
October 2016: Canadian Journal of Diabetes
Solon Karapanagiotis, Simone Gambazza, Anna Brivio, Francesco D'Abrosca, Carla Colombo
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...
October 13, 2016: Pediatric Pulmonology
Jonathan Grigg, Jürg Barben, Kajsa Bohlin, Mark L Everard, Graham Hall, Mariëlle Pijnenburg, Kostas N Priftis, Franca Rusconi, Fabio Midulla
The Paediatric Assembly of the European Respiratory Society (ERS) maintained its high profile at the 2015 ERS International Congress in Amsterdam. There were symposia on preschool wheeze, respiratory sounds and cystic fibrosis; an educational skills workshop on paediatric respiratory resuscitation; a hot topic session on risk factors and early origins of respiratory diseases; a meet the expert session on paediatric lung function test reference values; and the annual paediatric grand round. In this report the Chairs of the Paediatric Assembly's Groups highlight the key messages from the abstracts presented at the Congress...
April 2016: ERJ Open Research
Valerie Waters, Sanja Stanojevic, Felix Ratjen
Introduction Cystic fibrosis (CF) is a disease characterized by recurrent flares of respiratory symptoms, known as pulmonary exacerbations (PExs), which have a cumulative, detrimental effect on lung function decline and overall mortality. Although much research has been done on the effects of PExs in adults with CF, considerably less is known about these events in young children with CF. Areas covered This review describes the typical presentation of PExs in children and their impact on long-term clinical outcomes...
October 8, 2016: Expert Review of Respiratory Medicine
Simon Veldhoen, Andreas M Weng, Janine Knapp, Andreas S Kunz, Daniel Stäb, Clemens Wirth, Florian Segerer, Helge Hebestreit, Uwe Malzahn, Herbert Köstler, Thorsten A Bley
Purpose To assess the clinical feasibility of self-gated non-contrast-enhanced functional lung (SENCEFUL) magnetic resonance (MR) imaging for quantitative ventilation (QV) imaging in patients with cystic fibrosis (CF). Materials and Methods Twenty patients with CF and 20 matched healthy volunteers underwent functional 1.5-T lung MR imaging with the SENCEFUL imaging approach, in which a two-dimensional fast low-angle shot sequence is used with quasi-random sampling. The lungs were manually segmented on the ventilation-weighted images to obtain QV measurements, which were compared between groups...
October 6, 2016: Radiology
Gregg A Duncan, James Jung, Justin Hanes, Jung Soo Suk
Recent evidence suggests that the airway mucus gel layer may be impermeable to the viral and synthetic gene vectors used in past inhaled gene therapy clinical trials for diseases like cystic fibrosis. These findings support the logic that inhaled gene vectors that are incapable of penetrating the mucus barrier are unlikely to provide meaningful benefit to patients. In this review, we discuss the biochemical and biophysical features of mucus that contribute its barrier function, and how these barrier properties may be reinforced in patients with lung disease...
September 20, 2016: Molecular Therapy: the Journal of the American Society of Gene Therapy
Richard W Bohannon, Rebecca Crouch
RATIONALE, AIMS, AND OBJECTIVES: The 6-minute walk test (6MWT) is widely used as a test of functional exercise capacity. Several studies have reported the minimal clinically important difference (MCID) for the 6MWT; however, the findings of the studies have not been examined in the context of one another. In this review, we aimed to summarize available information on the MCID for the 6MWT performed by patients with pathology. METHODS: Relevant literature was identified by searches of 3 electronic databases (PubMed, Scopus, and Cumulative Index of Nursing and Allied Health), examination of article reference lists, and consultation with an expert...
September 4, 2016: Journal of Evaluation in Clinical Practice
Lyle L Pritchard
Cystic fibrosis (CF) is an autosomal recessive genetic disease that occurs in approximately 1 in 2,500 white live births. It is less common in nonwhite individuals. A dysfunctional epithelial chloride channel leads to excessively thick mucus affecting multiple organ systems. Common issues include mucous plugging of the airway, lung inflammation, chronic pulmonary infections, intestinal malabsorption, and malnutrition. Universal screening of newborns for CF is recommended in many countries. CF can be diagnosed based on clinical evidence of disease along with genetic testing or other laboratory evidence of chloride channel dysfunction...
September 2016: FP Essentials
Lacrecia J Britton, Gabriela R Oates, Robert A Oster, Staci T Self, Robert B Troxler, Wynton C Hoover, Hector H Gutierrez, William T Harris
OBJECTIVE: The clinical benefit of newborn screening (NBS) for cystic fibrosis (CF) has been primarily nutritional, with less overt respiratory impact. Identification of risk factors for infant CF lung disease could facilitate targeted interventions to improve pulmonary outcomes. METHODS: This retrospective study evaluated socioeconomic information, clinical data, and results from routine infant pulmonary function testing (iPFT) of infants diagnosed with CF through NBS (N = 43) at a single CF center over a 4-year period (2008-2012)...
November 2016: Pediatric Pulmonology
B Schullcke, S Krueger-Ziolek, B Gong, U Mueller-Lisse, K Moeller
Besides the application of EIT in the intensive care unit it has recently also been used in spontaneously breathing patients suffering from asthma bronchiole, cystic fibrosis (CF) or chronic obstructive pulmonary disease (COPD). In these cases large thorax excursions during deep inspiration, e.g. during lung function testing, lead to artifacts in the reconstructed images. In this paper we introduce a new approach to compensate for image artifacts resulting from excursion induced changes in boundary voltages...
September 2016: Physiological Measurement
Charlene S Stahr, Chaminda R Samarage, Martin Donnelley, Nigel Farrow, Kaye S Morgan, Graeme Zosky, Richard C Boucher, Karen K W Siu, Marcus A Mall, David W Parsons, Stephen Dubsky, Andreas Fouras
Computed tomography (CT) and spirometry are the mainstays of clinical pulmonary assessment. Spirometry is effort dependent and only provides a single global measure that is insensitive for regional disease, and as such, poor for capturing the early onset of lung disease, especially patchy disease such as cystic fibrosis lung disease. CT sensitively measures change in structure associated with advanced lung disease. However, obstructions in the peripheral airways and early onset of lung stiffening are often difficult to detect...
2016: Scientific Reports
Saira Ahmad, Jean Tyrrell, William G Walton, Ashutosh Tripathy, Matthew R Redinbo, Robert Tarran
The opportunistic bacteria of the Burkholderia cepacia complex (Bcc) are extremely pathogenic to cystic fibrosis (CF) patients, and acquisition of Bcc bacteria is associated with a significant increase in mortality. Treatment of Bcc infections is difficult because the bacteria are multidrug resistant and able to survive in biofilms. Short palate, lung, and nasal epithelial clone 1 (SPLUNC1) is an innate defense protein that is secreted by the upper airways and pharynx. While SPLUNC1 is known to have antimicrobial functions, its effects on Bcc strains are unclear...
October 2016: Antimicrobial Agents and Chemotherapy
Yaling Yi, Andrew W Norris, Kai Wang, Xingshen Sun, Aliye Uc, Antoinette Moran, John F Engelhardt, Katie Larson Ode
RATIONALE: In cystic fibrosis, abnormal glucose tolerance is associated with decreased lung function and worsened outcomes. Translational evidence indicates that abnormal glucose tolerance may begin in early life. OBJECTIVES: To determine whether very young children with cystic fibrosis have increased abnormal glucose tolerance prevalence compared with control subjects. The secondary objective was to compare area under the curve for glucose and insulin in children with cystic fibrosis with control subjects...
October 15, 2016: American Journal of Respiratory and Critical Care Medicine
Kate Cumming, Lisa O'Brien, Jane Harris
BACKGROUND/AIM: Limited information about return to productive activities after lung transplantation has been published. The purpose of our study was to identify issues relating to occupational engagement in employment after surgery. METHOD: We conducted a cross-sectional study of surviving lung transplant recipients from one transplant service in Australia. We used descriptive statistics, chi-square tests and Cox regression to analyse the data. RESULTS: A total of 100 lung transplant recipients completed the assessment (83...
October 2016: Australian Occupational Therapy Journal
Constantin Adams, Vanya Icheva, Caroline Deppisch, Josefine Lauer, Gloria Herrmann, Ute Graepler-Mainka, Susanne Heyder, Erich Gulbins, Joachim Riethmueller
BACKGROUND/AIMS: Several recent clinical studies revealed an accumulation of ceramide in bronchial epithelial cells of patients with cystic fibrosis (CF). Degradation of ceramide concentrations in lungs of CF patients employing the functional acid sphingomyelinase inhibitor amitriptyline revealed a benefit in lung function, weight and exacerbation rates. METHODS: To test for a beneficial effect of amitriptyline in vivo, we performed two phase II randomised, double-blind, placebo-controlled studies...
2016: Cellular Physiology and Biochemistry
G Belle-van Meerkerk, H W de Valk, M C Stam-Slob, F Teding van Berkhout, P Zanen, E A van de Graaf
AIMS: Pulmonary infections are more frequent in and associated with higher mortality in Cystic Fibrosis-Related Diabetes (CFRD) patients compared to CF patients without CFRD. Hyperglycaemia can lead to a higher vulnerability for infections. Aim of the study was to test whether the infection rate in well-controlled CFRD patients was similar to that in CF patients without CFRD. METHODS: This is a retrospective six-year cohort analysis on a consecutive series of 138 CF patients...
June 2016: Diabetes Research and Clinical Practice
N Decorte, M Gruet, B Camara, S Quetant, L Mely, J M Vallier, S Verges, B Wuyam
BACKGROUND: Specific alterations in skeletal muscle related to genetic defects may be present in adults with cystic fibrosis (CF). Limb muscle dysfunction may contribute to physical impairment in CF. AIMS AND OBJECTIVES: We hypothesized that adults with CF would have altered calf muscle metabolism during exercise. METHODS: Fifteen adults with CF and fifteen healthy controls matched for age, gender and physical activity performed a maximal cycling test and an evaluation of calf muscle energetics by (31)P magnetic resonance spectroscopy before, during and after plantar flexions to exhaustion...
June 14, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
Tim W R Lee, Kevin W Southern, Luke A Perry, Jahan C Penny-Dimri, Aisha A Aslam
BACKGROUND: Cystic fibrosis is caused by a defective gene encoding a protein called the cystic fibrosis transmembrane conductance regulator (CFTR), and is characterised by chronic lung infection resulting in inflammation and progressive lung damage that results in a reduced life expectancy. OBJECTIVES: To determine whether topical CFTR gene replacement therapy to the lungs in people with cystic fibrosis is associated with improvements in clinical outcomes, and to assess any adverse effects...
2016: Cochrane Database of Systematic Reviews
Bernadette Prentice, Shihab Hameed, Charles F Verge, Chee Y Ooi, Adam Jaffe, John Widger
INTRODUCTION: Cystic fibrosis-related diabetes (CFRD) is the end-point of a spectrum of glucose abnormalities in cystic fibrosis that begins with early insulin deficiency and ultimately results in accelerated nutritional decline and loss of lung function. Current diagnostic and management regimens are unable to entirely reverse this clinical decline. AREAS COVERED: This review summarises the current understanding of the pathophysiology of CFRD, the issues associated with using oral glucose tolerance tests in CF and the challenges faced in making the diagnosis of CFRD...
July 2016: Expert Review of Respiratory Medicine
Barbara Egger, Kerstin Jost, Pinelopi Anagnostopoulou, Sophie Yammine, Florian Singer, Carmen Casaulta, Urs Frey, Philipp Latzin
BACKGROUND: Multiple-breath washout (MBW) is increasingly used for infant lung function testing. Current guidelines recommend calculating lung clearance index (LCI) and functional residual capacity (FRC) at 2.5% of normalized tracer gas concentration, without clear recommendation for moment ratios (MR). Whether the 2.5% cut-off has the highest discriminative power to detect ventilation inhomogeneity in infants with lung diseases is unknown. METHODS: We used sulfur-hexafluoride MBW measurements from 32 infants with cystic fibrosis, 32 preterm infants, and 32 healthy controls at postmenstrual age of 41-54 weeks...
May 23, 2016: Pediatric Pulmonology
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