Longitudinal impact of demographic and clinical variables on health-related quality of life in cystic fibrosis.

Objectives: The insights that people with cystic fibrosis have concerning their health are important given that aspects of health-related quality of life (HRQoL) are independent predictors of survival and a decrease in lung function is associated with a decrease in HRQoL over time. Cross-sectional data suggest that key variables, other than lung function, are also associated with HRQoL--although study results are equivocal. This work evaluates the relationship between these key demographic and clinical variables and HRQoL longitudinally.

Longitudinal association between lung function and health-related quality of life in cystic fibrosis.

Background: Lung function is an important indicator of cystic fibrosis disease status and those with better forced expiratory volume in 1 s (FEV(1))% predicted have tended to report a better health-related quality of life (HRQoL) in cross-sectional studies. The relationship between lung function and HRQoL over time is unknown. This work assesses the natural progression of HRQoL reporting over many years and compares assessments across a whole decade and evaluates the relationship between lung function and HRQoL longitudinally.

PmrB mutations promote polymyxin resistance of Pseudomonas aeruginosa isolated from colistin-treated cystic fibrosis patients.

Pseudomonas aeruginosa can develop resistance to polymyxin and other cationic antimicrobial peptides. Previous work has shown that mutations in the PmrAB and PhoPQ regulatory systems can confer low to moderate levels of colistin (polymyxin E) resistance in laboratory strains and clinical isolates of this organism (MICs of 8 to 64 mg/liter). To explore the role of PmrAB in high-level clinical polymyxin resistance, P.

Multicentre trial of weekly risedronate on bone density in adults with cystic fibrosis.

Background: The aim of this study was to assess the efficacy, tolerability and safety of risedronate in adults with CF.

Methods: Patients with a lumbar spine (LS), total hip (TH) or femoral neck (FN) bone mineral density (BMD) Z-score of -1 or less were randomised to receive risedronate 35 mg weekly or placebo, and calcium (1g)+vitamin D(3) (800IU).

Results: At baseline, BMD Z-scores in the risedronate (n=17) and placebo (n=19) groups were similar.

European cystic fibrosis bone mineralisation guidelines.

Patients with cystic fibrosis (CF) are at risk of developing low bone mineral density (BMD) and fragility fractures. This paper presents consensus statements that summarise current knowledge of the epidemiology and pathophysiology of CF-related skeletal deficits and provides guidance on its assessment, prevention and treatment. The statements were validated using a modified Delphi methodology.

Prevention of chronic Pseudomonas aeruginosa infection in people with cystic fibrosis.

Cystic fibrosis is the most common genetically inherited disease in the Caucasian population, with approximately 30,000 patients in the USA and more than 50,000 patients worldwide. The primary defect in the cystic fibrosis transmembrane regulator gene affects the production and/or function of the cystic fibrosis transmembrane regulator protein. Depending on the severity of the genetic defect, patients may have minimal disease expression (e.

Can health-related quality of life predict survival in adults with cystic fibrosis?

Rationale: Advances in the management of cystic fibrosis have led to a significant improvement in survival, although marked differences between individuals are still observed. The value of patient-reported health-related quality of life scores in predicting survival in adults with cystic fibrosis is unknown.

Objectives: To evaluate whether patient-reported health-related quality of life could predict survival in cystic fibrosis.

Regimens for eradicating early Pseudomonas aeruginosa infection in children do not promote antibiotic resistance in this organism.

Background: This study was designed to address the concern that intensive regimens to eradicate early Pseudomonas aeruginosa infection in patients with cystic fibrosis may lead to the emergence of antibiotic-resistant isolates.

Methods: Data was analysed retrospectively over a 10 year period at the Leeds Regional Paediatric Cystic Fibrosis Centre. All patients with first-ever isolation of P.

Decision-making about pregnancy for women with cystic fibrosis.

Objectives: This study aims to generate experiential data about the decision-making process encountered by 12 women with cystic fibrosis (CF) considering pregnancy.

Design: This study uses a qualitative grounded theory methodology to analyse the data and develop a conceptual hierarchical model of salient issues.

Methods: Twelve women completed a semi-structured interview accompanied by quantitative self-report measures of quality of life and psychological well-being, and indicators of health and treatment status were used.

Microvascular complications in patients with cystic fibrosis-related diabetes (CFRD).

Background: This study aims to quantitatively and qualitatively assess microvascular complications and their risk factors in patients with cystic fibrosis-related diabetes (CFRD) compared to those with type 1 diabetes mellitus (DM1).

Methods: 79 patients with CFRD were matched with 79 patients with DM1 according to sex, age and duration of insulin therapy. Retinopathy, peripheral neuropathy, nephropathy and microalbuminuria were the microvascular complications assessed.

A cross-sectional study of bone mineral density in children and adolescents attending a Cystic Fibrosis Centre.

Background: Low bone mineral density is common in adults with cystic fibrosis. Children with good lung function compared to controls matched for body size have normal bone mineralisation. There are few data in large unselected populations of children.

Nutritional intake and status in children with cystic fibrosis: does age matter?

Objectives: To determine whether nutritional intake and status vary with age in children with cystic fibrosis (CF).

Methods: Case-control study examining differences in nutritional parameters and intakes in 58 children with CF recruited from a regional centre (2000-2001) and 45 controls. Participants were divided into age groups of 5-8 years, 9-12 years and 13-16 years.

Nutritional status, perceived body image and eating behaviours in adults with cystic fibrosis.

Background & Aims: Achieving and maintaining an ideal nutritional status is the primary aim of the nutritional management of cystic fibrosis (CF). It is unclear how nutritional interventions impact on patients' perceptions and behaviours concerning body image and eating. This work aimed to provide a psychosocial profile and compare CF patients receiving (a) enteral tube feeding, (b) nutritional supplements, (c) no nutritional interventions, and (d) healthy controls.

Aspergillus bronchitis in cystic fibrosis.

Aspergillus fumigatus, a widely distributed spore-bearing fungus, is commonly grown in sputum cultures of patients with cystic fibrosis (CF). A fumigatus may cause allergic bronchopulmonary aspergillosis (ABPA), a complex condition that leads to worsening of airway inflammation and progressive damage and is diagnosed by specific criteria. In this report, we present six CF patients with respiratory deterioration that did not respond to appropriate antibiotic treatment.

Therapeutic drug monitoring of once daily tobramycin in cystic fibrosis--caution with trough concentrations.

Background: : Once daily intravenous aminoglycoside dosing (ODD) is widely used to treat acute Pseudomonas aeruginosa exacerbations in patients with cystic fibrosis. Controversy exists as to what is the most appropriate method of therapeutic drug monitoring (TDM) of such therapy with recommendations including trough plasma concentrations of <1 mg/L or <2 mg/L, area under curve (AUC) and various nomograms. This study aimed to compare the exposures to ODD of tobramycin in adults and children with cystic fibrosis using the AUC and trough TDM approaches.

Optimizing treatment policies and improving care: impact on outcome in patients with cystic fibrosis.

There is no cure for cystic fibrosis but recent advances in care have increased the average life expectancy to over 30 years. However, patients may find themselves prescribed over seven to eight different medications a day, some of which are laborious and time consuming to administer. The physician should balance potential benefits from treatments against quality-of-life requirements.

Diagnosis and treatment of intestinal malabsorption in cystic fibrosis.

Intestinal malabsorption is severe and of early onset in virtually all people who have cystic fibrosis. The main cause is deficiency of pancreatic enzymes. Bicarbonate deficiency, abnormal bile salts, mucosal transport problems, motility differences, and anatomical structural changes are other contributory factors.

Vitamin K status among children with cystic fibrosis and its relationship to bone mineral density and bone turnover.

Objective: The aim of this study was to assess vitamin K status in an unselected population of children with cystic fibrosis (CF) and to investigate any vitamin K effect on bone turnover and bone mineral status.

Methods: Children > or =5 years of age who were attending the CF unit were invited to enter the study. Fasting blood samples were analyzed for levels of vitamin K1 and prothrombin produced in vitamin K absence; total, undercarboxylated, and carboxylated osteocalcin (OC); and bone-specific alkaline phosphatase and procollagen I carboxy-terminal propeptide (bone formation markers).

Associations between clinical variables and quality of life in adults with cystic fibrosis.

Background: The disease progression of cystic fibrosis (CF) is marked by an increase in clinical conditions and therapeutic interventions, which have the potential to affect health-related quality of life (HRQoL). This cross-sectional study explored associations between clinical variables and HRQoL.

Methods: HRQoL was measured using the Cystic Fibrosis Quality of Life (CFQoL) questionnaire, which consists of nine domains: physical, social, treatment, chest symptoms, emotional functioning, concerns for the future, relationships, body image, and career concerns.

Risk factors for acquisition of methicillin-resistant Staphylococcus aureus (MRSA) by patients with cystic fibrosis.

Methicillin-resistant Staphylococcus aureus (MRSA) is an increasing problem for patients with cystic fibrosis (CF). It has been associated with clinical deterioration in some patients with CF, creates additional infection control problems, and may affect acceptance onto transplant waiting lists. Recent attempts to eradicate the organism have met with only moderate success.

Evaluation of a new definition for chronic Pseudomonas aeruginosa infection in cystic fibrosis patients.

Background: Patients were defined each successive month as either 'chronic' when more than 50% of the preceding 12 months were PA culture positive, 'intermittent' when < or =50% of the preceding 12 months were PA culture positive, 'free of PA', with no growth of PA for the previous 12 months, having previously been PA culture positive, or 'never infected', when PA had never been cultured.

Methods: Cross-sectional analysis of 146 children attending the Leeds Regional Cystic Fibrosis Centre was performed to assess relationship between the new definition and clinical scores and investigations. The response variable was regressed on age and sex and the residuals analysed using the Kruskal-Wallis test.

Pharmacokinetics and safety of itraconazole in patients with cystic fibrosis.

Objective: To assess the pharmacokinetics of itraconazole and hydroxy-itraconazole in patients with cystic fibrosis.

Methods: Patients were divided into those <16 and >/=16 years of age. All received itraconazole oral solution 2.

Reduction in prevalence of chronic Pseudomonas aeruginosa infection at a regional pediatric cystic fibrosis center.

Various management strategies were introduced at the Leeds Regional Cystic Fibrosis (CF) Unit in an attempt to reduce the prevalence of chronic Pseudomonas aeruginosa respiratory infection, previously thought to be inevitable in most children with CF. These included neonatal screening (1975), regular microbiological monitoring (1975), early antibiotic treatment of first isolations of P. aeruginosa (1985), intensive intravenous antibiotic treatment where nebulized antibiotics failed to eradicate P.