scholarly journals Growth Deficiency in Cystic Fibrosis Is Observable at Birth and Predictive of Early Pulmonary Function

2016 ◽  
Vol 18 (5) ◽  
pp. 498-504 ◽  
Author(s):  
Rebecca Darrah ◽  
Rebecca Nelson ◽  
Elizabeth G. Damato ◽  
Michael Decker ◽  
Anne Matthews ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Birth Weight ◽  
Pulmonary Function ◽  
Pulmonary Disease ◽  
Complex Disease ◽  
Nutritional Intervention ◽  
Early Age ◽  
Growth Deficiency ◽  
The Relationship ◽  
Linear Regressions

Introduction: Cystic fibrosis (CF) is a complex disease that includes both pulmonary and gastrointestinal challenges, resulting in decreased weight. Pulmonary symptoms of CF are extremely variable. Greater body mass at an early age is associated with improved pulmonary function, but it is unknown at what age weight becomes predictive of pulmonary disease severity. The purpose of this study was to investigate the relationship between birth weight and pulmonary function in CF. Methods: Birth weight and pulmonary data were obtained. Linear regressions were used to examine the relationship between these two variables. A one-tailed t-test was used to compare birth weights between CF patients and the national average. Results: Birth weight was significantly lower in babies with CF and correlated with pulmonary disease at ages 6 and 10 years but not with age at which Pseudomonas aeruginosa colonization was observed. Discussion: These data suggest that CF growth deficiency has prenatal origins. Early nutritional intervention for babies with CF and a low birth weight is warranted to maximize pulmonary potential.

The relationship between body growth and pulmonary function in children with cystic fibrosis

2013 ◽  
Vol 103 (2) ◽  
pp. 162-167 ◽  
Author(s):  
Janna W Woestenenk ◽  
Rebecca K Stellato ◽  
Suzanne W Terheggen−Lagro ◽  
Cornelis K van der Ent ◽  
Roderick HJ Houwen
Keyword(s):  
Cystic Fibrosis ◽  
Pulmonary Function ◽  
Body Growth ◽  
The Relationship

scholarly journals EDNRA variants associate with smooth muscle mRNA levels, cell proliferation rates, and cystic fibrosis pulmonary disease severity

2010 ◽  
Vol 41 (1) ◽  
pp. 71-77 ◽  
Author(s):  
Rebecca Darrah ◽  
Edward McKone ◽  
Clare O'Connor ◽  
Christine Rodgers ◽  
Alan Genatossio ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Cell Proliferation ◽  
Smooth Muscle ◽  
Pulmonary Function ◽  
Smooth Muscle Cells ◽  
Pulmonary Disease ◽  
Muscle Cells ◽  
Airway Smooth Muscle Cells ◽  
Mrna Levels ◽  
Protective Allele

Airway inflammation and pulmonary disease are heterogeneous phenotypes in cystic fibrosis (CF) patients, even among patients with the same cystic fibrosis transmembrane conductance regulator (CFTR) genotype. Endothelin, a proinflammatory peptide and smooth muscle agonist, is increased in CF airways, potentially contributing to the pulmonary phenotype. Four cohorts of CF patients were screened for variants in endothelin pathway genes to determine whether any of these variants associated with pulmonary function. An initial cohort of 808 CF patients homozygous for the common CF mutation, ΔF508, showed significant association for polymorphisms in the endothelin receptor A gene, EDNRA ( P = 0.04), but not in the related endothelin genes ( EDN1, EDN2, EDN3, or EDNRB) or NOS1, NOS2A, or NOS3. Variants within EDNRA were examined in three additional cohorts of CF patients, 238 patients from Seattle, WA, 303 from Ireland and the U.K., and 228 from Cleveland, OH, for a total of 1,577 CF patients. The three additional groups each demonstrated a significant association between EDNRA 3′-untranslated region (UTR) variant rs5335 and pulmonary function ( P = 0.002). At the molecular level, single nucleotide primer extension assays suggest that the effect of the variants is quantitative. EDNRA mRNA levels from cultured primary tracheal smooth muscle cells are greater for the allele that appears to be deleterious to lung function than for the protective allele, suggesting a mechanism by which increased receptor function is harmful to the CF airway. Finally, cell proliferation studies using human airway smooth muscle cells demonstrated that cells homozygous for the deleterious allele proliferate at a faster rate than those homozygous for the protective allele.

Jointly modelling the relationship between survival and pulmonary function in cystic fibrosis patients

2002 ◽  
Vol 21 (9) ◽  
pp. 1271-1287 ◽  
Author(s):  
Mark D. Schluchter ◽  
Michael W. Konstan ◽  
Pamela B. Davis
Keyword(s):  
Cystic Fibrosis ◽  
Pulmonary Function ◽  
The Relationship

scholarly journals Relationships between body composition and pulmonary function in a community-dwelling population in Japan

PLoS ONE ◽  
2020 ◽  
Vol 15 (11) ◽  
pp. e0242308
Author(s):  
Ryosuke Kawabata ◽  
Yuki Soma ◽  
Yutaro Kudo ◽  
Junichi Yokoyama ◽  
Hiroyasu Shimizu ◽  
...  
Keyword(s):  
Body Composition ◽  
Pulmonary Function ◽  
Pulmonary Disease ◽  
Visceral Fat ◽  
Community Dwelling ◽  
The Body ◽  
Whole Body ◽  
Obstructive Pulmonary Disease ◽  
Skeletal Muscle Index ◽  
The Relationship

Pulmonary diseases, including chronic obstructive pulmonary disease (COPD), are major chronic diseases that result in decreased pulmonary function. Relationships between body composition and pulmonary function have been reported. However, few epidemiological studies have used the visceral fat area (VFA) to measure body composition. This study aimed to examine the relationship between body composition and pulmonary function. A cross-sectional study was conducted between 2015 and 2016, using data obtained from 1,287 residents aged between 19 and 91 years living in the Iwaki area of Hirosaki City, a rural region in Aomori Prefecture, Japan. Pulmonary function was evaluated using the forced vital capacity (FVC) as a percentage of the predicted value (predicted FVC%) and the ratio of forced expiratory volume in one second (FEV1) to FVC. The measurements for evaluating body composition included the body fat percentage (BFP) of the whole body and trunk, skeletal muscle index (SMI), body mass index (BMI), VFA, waist circumference (WC) at the navel level, and waist-to-hip ratio (WHR). To adjust for potential confounders, Spearman’s partial correlation analysis was used to examine the relationship between the measurements of body composition and pulmonary function. There were significant correlations between the predicted FVC% and the following parameters: BFP (whole body and trunk) in younger males; SMI in older males; WC, VFA, BMI, and SMI in younger females; and BFP (whole body and trunk) and VFA in older females. Contrastingly, WC and VFA in younger males and WC in younger females were correlated with the FEV1/FVC ratio. VFA was correlated with the FEV1/FVC ratio in younger males and predicted FVC% in older females. These findings suggest that visceral fat accumulation may increase the development of obstructive pulmonary disease in young males and accelerate the decline of pulmonary function (predicted FVC%) in older females.

THE SITE OF AIRWAY OBSTRUCTION IN CYSTIC FIBROSIS

PEDIATRICS ◽  
1969 ◽  
Vol 44 (3) ◽  
pp. 315-318
Author(s):  
Robert B. Mellins
Keyword(s):  
Cystic Fibrosis ◽  
Natural History ◽  
Pulmonary Function ◽  
Airway Obstruction ◽  
Pulmonary Disease ◽  
Airway Resistance ◽  
Large Airway ◽  
History Of

Evidence has been presented that some patients with cystic fibrosis have significant amounts of obstruction to the ventilation of portions of lung, as indicated by tests of the distribution of ventilation, but they have little or no evidence of obstruction by conventional measurements of airway resistance.1-5 This discrepancy may be attributed to the fact that various tests of pulmonary function differ in sensitivity with respect to small and large airway obstruction. It is the purpose of the present Commentary to summarize the evidence for this hypothesis and to suggest that differences in the extent to which measurements of airway resistance and distribution of ventilation are altered in patients with cystic fibrosis may be used to localize the site of obstruction and to follow the different stages in the natural history of the pulmonary disease.

scholarly journals What Is Most Suitable for Children With Cystic Fibrosis—The Relationship Between Spirometry, Oscillometry, and Multiple Breath Nitrogen Washout

2022 ◽  
Vol 9 ◽  
Author(s):  
Magdalena Postek ◽  
Katarzyna Walicka-Serzysko ◽  
Justyna Milczewska ◽  
Dorota Sands
Keyword(s):  
Cystic Fibrosis ◽  
Pulmonary Function ◽  
Study Group ◽  
Z Score ◽  
Nitrogen Washout ◽  
Significant Difference ◽  
Function Test ◽  
Test Sets ◽  
The Relationship ◽  
Function Testing

IntroductionIn cystic fibrosis (CF), pathological lung changes begin early in life. The technological progress currently gives many diagnostic possibilities. However, pulmonary function testing in children remains problematic.ObjectivesOur study aimed to correlate the results of impulse oscillometry (IOS) with those of multiple breath nitrogen washout (MBNW) in our pediatric CF population. We also compared those parameters between the groups with and without spirometric features of obturation.MethodsWe collected 150 pulmonary function test sets, including spirometry, IOS, and MBNW in patients with CF aged 12.08 ± 3.85 years [6–18]. The study group was divided into two subgroups: IA (without obturation) and IB (with obturation). We also compared Sacin, Scond, and oscillometry parameters of 20 patients aged 14–18 years who reached the appropriate tidal volume (VT) during MBNW.ResultsStatistical analysis showed a negative correlation between lung clearance index (LCI) and spimoetric parameters. Comparison of subgroups IA (n = 102) and IB (n = 48) indicated a statistically significant difference in LCI (p < 0.001) and FEV1z-score (p < 0.001), FEV1% pred (p < 0.001), MEF25z-score (p < 0.001), MEF50 z-score (p < 0.001), MEF75 z-score (p < 0.001), R5% pred (p < 0.05), and R20% pred (p < 0.01). LCI higher than 7.91 was found in 75.33% of the study group, in subgroup IB—91.67%, and IA−67.6%.ConclusionsLCI derived from MBNW may be a better tool than IOS for assessing pulmonary function in patients with CF, particularly those who cannot perform spirometry.

scholarly journals Percutaneous endoscopic gastrostomy feeding in patients with cystic fibrosis

Gut ◽  
1999 ◽  
Vol 44 (1) ◽  
pp. 87-90 ◽  
Author(s):  
S G J Williams ◽  
F Ashworth ◽  
A McAlweenie ◽  
S Poole ◽  
M E Hodson ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Nutritional Status ◽  
Pulmonary Function ◽  
Pulmonary Disease ◽  
Percutaneous Endoscopic Gastrostomy ◽  
Tube Feeding ◽  
Late Complications ◽  
Albumin Concentration ◽  
Management Problem ◽  
Endoscopic Gastrostomy

BackgroundMalnutrition is a common management problem in patients with cystic fibrosis (CF). Various approaches to supplemental nutrition by both parenteral and enteral routes have been used.AimTo analyse the efficacy and acceptability of supplemental overnight feeding using a percutaneous endoscopic gastrostomy (PEG) in patients with CF.Patients53 patients with CF (43 adults; age >17 years) with severe pulmonary disease.MethodsThe technical success and complications of PEG insertion were documented together with changes in nutritional and pulmonary status of the cohort.ResultsPEG tubes were successfully inserted in all patients, with immediate complications (respiratory depression) in two (4%) and late complications in 13 (25%). Feeding was well tolerated by 50/51 (98%) of the cohort during a mean (SEM) follow up of 14.5 (2.1) months. The adult cohort had a significant increase in weight and body mass index at six months which was maintained at 12 months. Serum albumin concentration remained stable at six months but had fallen by 12 months, although the differences were not statistically significant. These results were reflected in the paediatric cohort. Pulmonary function in those followed up for one year had apparently stabilised, but the number of admissions to hospital over the year before and the year after PEG did not change. Half of the cohort were accepted for heart-lung/lung transplantation, the improvement in nutritional status being a prerequisite for this.ConclusionSupplemental PEG tube feeding is well tolerated and results in a significant improvement in nutritional status and an apparent stabilisation of pulmonary function in severely malnourished CF patients with advanced pulmonary disease.

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