Dataset Information

Obesity in Cystic fibrosis: prevalence, trends and associated factors data from the US cystic fibrosis foundation patient registry.

ABSTRACT: Strong emphasis has been placed historically on increasing weight and improving nutritional status in cystic fibrosis patients. Due to correlation between nutritional indices (e.g. BMI) and lung function, CF Nutrition Guidelines have recommended BMI percentile goals at the 50th percentile or higher. Trends in increasing BMI across CF programs suggest significantly increasing proportions of overweight and obese status in recent years. We identify that between 2000 and 2019 there has been a relative decrease in underweight status by ∼40%, simultaneously with a > 300% increase in overweight status, and >400% increase in obesity. Patient specific factors associated with higher prevalence of obesity included age ≥46, living in a zip code where the median income was < $20,000, having at least one allele with a class IV or V mutation, a ppFEV₁ >90 prescribed ivacaftor, and not prescribed pancreatic enzymes. Program specific factors were not identified.

SUBMITTER: Szentpetery S

PROVIDER: S-EPMC9509402 | biostudies-literature | 2022 Sep

REPOSITORIES: biostudies-literature

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Obesity in Cystic fibrosis: prevalence, trends and associated factors data from the US cystic fibrosis foundation patient registry.

Szentpetery Sylvia S Fernandez Gabriela S GS Schechter Michael S MS Jain Raksha R Flume Patrick A PA Fink Aliza K AK

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 20220405 5

Strong emphasis has been placed historically on increasing weight and improving nutritional status in cystic fibrosis patients. Due to correlation between nutritional indices (e.g. BMI) and lung function, CF Nutrition Guidelines have recommended BMI percentile goals at the 50th percentile or higher. Trends in increasing BMI across CF programs suggest significantly increasing proportions of overweight and obese status in recent years. We identify that between 2000 and 2019 there has been a relati ...[more]

PMID: 35396178

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Project description:BACKGROUND: over the last 25 years several national registries of CF have been set up. Such systems can be very useful in providing an integrated resource for improving patient care and conducting research on the disease. Minimum Data Set is a common set of data items that should be used to collect and report data in the registry. The principal aim of this research was to determine minimum data set for the CF registry in north-west of Iran. METHODS: data items collected by several selected registries of cystic fibrosis were studied and an initial set of data was selected by the researchers. A group of experts including epidemiologists, pediatricians, and CF specialists were asked to review the proposed data elements and score them based on their importance by using a nine-point Likert scale. The items scored as important or highly important by more than 50 % of the experts, were included in final list of minimum data set. Availability of data was evaluated through reviewing medical records of 144 patients hospitalized in Children Hospital located in Tabriz. RESULTS: overall six classes of data (46 items) were identified in the selected registry systems for cystic fibrosis: patient demographics, administrative data, survival status, diagnostic procedures, genetic and clinical manifestations, and therapeutics. Thirty two data elements from all six categories of data were approved by the experts as the minimum data set for cystic fibrosis registry system. Availability of data in administrative category and survival class was 100 percent. Collecting data on medications was feasible in 100% of the cases as well. In class of demographic data, accessibility of patient name, age, gender, place of birth, and date of birth was 100 percent. In group of diagnostic procedures, partial availability of data was found for sweat test and genetic test. No data was found on the antenatal screening, exercise tolerance test, and glucose tolerance test. CONCLUSION: this work can be considered as a first step toward establishing CF registry system in Iran. Minimum data set can be also useful in designing electronic registry or electronic patient records for those suffering from CF toward integration of their fragmented records across continuum of the health care system in order to improve quality of shared patient care.

Project description:BackgroundCough is a common symptom of interstitial lung disease (ILD) and negatively impacts health-related quality of life (QOL). Previous studies have shown that among patients with idiopathic pulmonary fibrosis, cough may predict progression of lung disease and perhaps even respiratory hospitalizations and mortality.Research questionDoes cough-specific QOL predict disease progression, respiratory hospitalization, lung transplantation, and death among patients with ILD?Study design and methodsWe analyzed data from the Pulmonary Fibrosis Foundation Registry, which comprises a multicenter population of well-characterized patients with ILD. We first examined associations between patient factors and baseline scores on the Leicester Cough Questionnaire (LCQ), a cough-specific QOL tool, using a proportional odds model. Next, we examined associations between baseline LCQ scores and patient-centered clinical outcomes, as well as pulmonary function parameters, using a univariable and multivariable proportional hazards model that was adjusted for clinically relevant variables, including measures of disease severity.ResultsOne thousand four hundred forty-seven patients with ILD were included in our study. In the multivariable proportional odds model, we found that the following patient factors were associated with worse cough-specific QOL: younger age, diagnosis of "other ILD," gastroesophageal reflux disease, and lower FVC % predicted. Multivariable Cox regression models, adjusting for several variables including baseline disease severity, showed that a 1-point decrease in LCQ score (indicating lower cough-specific QOL) was associated with a 6.5% higher risk of respiratory-related hospitalization (hazard ratio [HR], 1.065; 95% CI, 1.025-1.107), a 7.4% higher risk of death (HR, 1.074; 95% CI, 1.020-1.130), and an 8.7% higher risk of lung transplantation (HR, 1.087; 95% CI, 1.022-1.156).InterpretationAmong a large population of well-characterized patients with ILD, cough-specific QOL was associated independently with respiratory hospitalization, death, and lung transplantation.

Dataset Information

Obesity in Cystic fibrosis: prevalence, trends and associated factors data from the US cystic fibrosis foundation patient registry.

Publications

Obesity in Cystic fibrosis: prevalence, trends and associated factors data from the US cystic fibrosis foundation patient registry.

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