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

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 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.

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.

The Relationship Between Sleep Disturbance and Pulmonary Function in Stable Pediatric Cystic Fibrosis Patients

CHEST Journal ◽  
2005 ◽  
Vol 128 (3) ◽  
pp. 1357-1363 ◽  
Author(s):  
Raouf Amin ◽  
Judy Bean ◽  
Kathleen Burklow ◽  
Jennifer Jeffries
Keyword(s):  
Cystic Fibrosis ◽  
Pulmonary Function ◽  
Sleep Disturbance ◽  
The Relationship

scholarly journals Health Disparities among adults cared for at an urban cystic fibrosis program

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Emily DiMango ◽  
Kaitlyn Simpson ◽  
Elizabeth Menten ◽  
Claire Keating ◽  
Weijia Fan ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
New York ◽  
New York City ◽  
Health Disparities ◽  
Pulmonary Function ◽  
Health Outcomes ◽  
York City ◽  
Median Income ◽  
Cross Sectional ◽  
Caucasian Patients

Abstract Background Evidence is conflicting regarding differential health outcomes in racial and ethnic minorities with cystic fibrosis (CF), a rare genetic disease affecting approximately 28,000 Americans. We performed a cross-sectional analysis of health outcomes in Black/Latinx patients compared with non-Hispanic Caucasian patients cared for in a CF center in New York City. Adult patients enrolled in the CF Foundation Patient Registry at the Columbia University Adult CF Program and seen at least once during 2019 were included. Health metrics were compared between Black/Latinx and non-Hispanic Caucasian patients. Results 262 patients were eligible. 39 patients (15%) identified as Black/Latinx or non-Hispanic Caucasian. Descriptive statistics are reported with mean (standard deviation). Current age was 35.9 (13.3) years for non-Hispanic Caucasian and 32.0 (9.3) years for Black/Latinx patients (p = 0.087). Age of diagnosis did not differ between groups; 9.56 (15.96) years versus 11.59 (15.8) years for non-Hispanic Caucasian versus Black/Latinx respectively (p = 0.464). Pulmonary function, measured as mean forced expiratory volume in one second (FEV1) was 70.6 (22.5) percent predicted in non-Hispanic Caucasian versus 59.50 (27.9) percent predicted in Black/Latinx patients (p = 0.010). Number of visits to the CF clinic were similar between groups. When controlled for age, gender, co-morbidities, median income, and insurance status, there was a continued association between minority status and lower FEV1. Conclusions Minorities with CF have significantly lower pulmonary function, the major marker of survival, than non-Hispanic Caucasians, even when controlled for a variety of demographic and socioeconomic factors that are known to affect health status in CF. Significant health disparities based on race and ethnicity exist at a single CF center in New York City, despite apparent similarities in access to guideline based care at an accredited CF Center. This data confirms the importance of design of culturally appropriate preventative and management strategies to better understand how to direct interventions to this vulnerable population with a rare disease.

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