An overview of international literature from cystic fibrosis registries. Part 4: Update 2011

Abstract

A total of 53 national cystic fibrosis (CF) patient registry studies published between July 2008 and November 2011 have been reviewed, focusing on the following topics: CF epidemiology, nutrition, microbiology, clinical complications, factors influencing diagnosis and lung disease, effects of socioeconomic status, therapeutic strategy evaluation, clinical trial methodology. The studies describe the clinical characteristics of CF patients, the incidence and prevalence of disease and role of gender gap, as well as the influence of socioeconomic status and environmental factors on clinical outcomes, covering a variety of countries and ethnic groups. Original observations describe patients as they get older, with special reference to the adult presentation of CF and long-term survival. Methodological aspects are discussed, covering the design of clinical trials, survival analysis, auxometry, measures of quality of life, follow up of lung disease, predictability of disease progression and life expectancy. Microbiology studies have investigated the role of selected pathogens, such as Burkholderia species and MRSA. Pulmonary exacerbations are discussed both as a factor influencing morbidity and an endpoint in clinical trials. Finally, some studies give insights on complications, such as CF-related diabetes and hemoptysis, and emerging problems, such as chronic nephropathy.

Dental utilization for Medicaid‐enrolled children with cystic fibrosis

“…This historical trend was attributed to earlier screening for males and reaffirmed recommendations that strongly support universal newborn screening programs so life--prolonging treatment may be initiated as soon as possible (14). In the US, the percentage of individuals with CF who are male has steadily declined from 53 (7,19).…”

Detection of Achromobacter xylosoxidans in Hospital, Domestic, and Outdoor Environmental Samples and Comparison with Human Clinical Isolates

“…Over the last 2 decades, new emerging bacteria have been found to invade the airways of CF patients, including nontuberculous mycobacteria, Burkholderia cepacia complex, Stenotrophomonas maltophilia, and Achromobacter xylosoxidans. Most of them are multidrug-resistant organisms thought to have been selected by intense antibiotic use over many years (1)(2)(3)(4).…”

Investigating the host and bacterial factors in cystic fibrosis that promote persistence of infection in the lung

“…The expansion of health information systems has allowed data to be collected and collated on large populations of patients and CF Data Registries are now available in many parts of the world (1,3,203,204). Utilisation of data collected through registries has enabled betweencentre comparisons to identify variations in clinical outcomes between CF centres and has led to the establishment of quality improvement programs, including benchmarking of outcomes with "high-performing" centres (203)(204)(205).…”

“…Utilisation of data collected through registries has enabled betweencentre comparisons to identify variations in clinical outcomes between CF centres and has led to the establishment of quality improvement programs, including benchmarking of outcomes with "high-performing" centres (203)(204)(205). However, to date, few studies have used national datasets to assess the variability and impact of antibiotic treatment strategies for P. aeruginosa in CF patients.…”

“…(223) Utilising data collected by national CF data registries allows between-centre comparisons and quality improvement programs to benchmark against "high-performing" centres. (203)(204)(205) However, few studies have assessed P. aeruginosa treatment strategies using national datasets. Here, data from the Australian Clonal Pseudomonas aeruginosa in CF (ACPinCF) study (107) and the Australian CF Data Registry (ACFDR) were linked to examine differences in managing P. aeruginosa infection.…”