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PubMed2002

[Rhinitis in patients with cystic fibrosis].

The author presents material of 110 cystic fibrosis patients, 81 with typical clinical features and 29 with atypical phenotype. Based on clinical examination and nasal cytological smears infective chronic nonspecific rhinitis (n = 80), infective acute rhinitis (n = 16), perennial allergic rhinitis (n = 4), nonalergic rhinitis with eosinophilia (n = 2), and moreover 19 patients with nasal polyposis, and 8 patients without nasal symptoms were distinguished. Infective chronic nonspecific rhinitis and infective acute rhinitis were present in typical and atypical cystic fibrosis patients with similar frequency but in patients with atypical phenotype perennial allergic rhinitis and nonalergic rhinitis with eosinophilia were observed more frequently. Nasal polyposis was associated with typical cystic fibrosis phenotype, and with two "severe" mutations.

Cystic Fibrosis↗

PubMed2003

Improved glutathione status in young adult patients with cystic fibrosis supplemented with whey protein.

BACKGROUND: The lung disease of cystic fibrosis is associated with a chronic inflammatory reaction and an over abundance of oxidants relative to antioxidants. Glutathione functions as a major frontline defense against the build-up of oxidants in the lung. This increased demand for glutathione (GSH) in cystic fibrosis may be limiting if nutritional status is compromised. We sought to increase glutathione levels in stable patients with cystic fibrosis by supplementation with a whey-based protein. METHODS: Twenty-one patients who were in stable condition were randomly assigned to take a whey protein isolate (Immunocal, 10 g twice a day) or casein placebo for 3 months. Peripheral lymphocyte GSH was used as a marker of lung GSH. Values were compared with nutritional status and lung parameters. RESULTS: At baseline there were no significant differences in age, height, weight, percent ideal body weight or percent body fat. Lymphocyte GSH was similar in the two groups. After supplementation, we observed a 46.6% increase from baseline (P < 0.05) in the lymphocyte GSH levels in the supplemented group. No other changes were observed. CONCLUSION: The results show that dietary supplementation with a whey-based product can increase glutathione levels in cystic fibrosis. This nutritional approach may be useful in maintaining optimal levels of GSH and counteract the deleterious effects of oxidative stress in the lung in cystic fibrosis.

Adolescent↗

PubMed1998

Cystic fibrosis genotypes and alcoholic pancreatitis.

Pancreatitis and pancreatic insufficiency are associated with both cystic fibrosis and alcoholism. The pathogenesis of alcoholic pancreatitis is unknown, but only a minority of alcoholics develop pancreatitis, and it has been suggested that a genetic predisposition may play a role in this disease. Two observations led to the hypothesis that this genetic predisposition could result from mutations in the cystic fibrosis gene. First, the prevalence of cystic fibrosis mutations in the Caucasian population (approximately 5%) is similar to the prevalence of pancreatitis among heavy drinkers. Second, in both diseases, pancreatic duct damage is a prominent feature and has been postulated to be the initial site of injury. Therefore, the aim of this study was to determine whether an increased frequency of mutations in the cystic fibrosis gene occurs in alcoholic pancreatitis. The 15 most common cystic fibrosis mutations in a Caucasian community were sought in 24 subjects with alcoholic pancreatitis. None were homozygous or heterozygous for these mutations. These findings suggest that cystic fibrosis mutations are not a major genetic factor predisposing to pancreatic injury in alcoholics.

Cystic Fibrosis↗

PubMed1994

Rise in total IgE as an indicator of allergic bronchopulmonary aspergillosis in cystic fibrosis.

BACKGROUND: Allergic bronchopulmonary aspergillosis is a serious complication of cystic fibrosis and may be difficult to diagnose. The aim of this study was to define the usefulness of measuring total IgE compared with other major criteria in the diagnosis of allergic bronchopulmonary aspergillosis in children with cystic fibrosis. METHODS: A retrospective analysis was carried out of the case records of 160 children attending a tertiary referral paediatric cystic fibrosis clinic. RESULTS: Sixteen children had a total IgE level above 500 IU/ml. Eleven children had six or more other major criteria and were considered to have allergic bronchopulmonary aspergillosis. These 11 children had a fourfold rise in IgE in association with clinical deterioration. A further child had a fourfold rise in IgE to 341 IU/l, and was also thought to have allergic bronchopulmonary aspergillosis. Eleven had a fall in IgE with successful treatment; one patient died with uncontrolled disease. Only one of these 12 children had negative precipitins to Aspergillus fumigatus. The five children with a raised IgE not thought to have bronchopulmonary aspergillosis had four or fewer major criteria and were not treated; none had positive precipitins. CONCLUSIONS: A fourfold rise in total IgE, particularly to above 500 IU/ml, is strongly suggestive of the diagnosis of allergic bronchopulmonary aspergillosis in children with cystic fibrosis. The measurement of total IgE has the merit of being simple to perform and objective. Positive aspergillus precipitins provide useful confirmatory evidence. These two criteria, taken in conjunction with clinical deterioration and new radiological shadowing, allow simplification of the diagnosis of allergic bronchopulmonary aspergillosis in cystic fibrosis.

Adolescent↗

PubMed2001

Nocturnal oximetry in infants with cystic fibrosis.

AIM: To investigate whether children with cystic fibrosis under 3 years of age have disordered breathing and episodes of oxygen desaturation during sleep. METHODS: We studied 19 infants (9 boys and 10 girls) with cystic fibrosis, mean age 13.1 months (range 3-36 months) and 20 age and sex matched healthy subjects. Patients and controls underwent an overnight polysomnographic study and respiratory function testing on the following morning. RESULTS: Seven patients with ongoing respiratory tract inflammation had disordered breathing and episodes of oxygen desaturation during sleep. Pulse oximetry showed a significantly lower mean oxygen saturation (SaO(2)) and a higher percentage of total sleep time spent with SaO(2) less than 93% in symptomatic children than in controls. CONCLUSION: Results suggest that infants and young children with cystic fibrosis and mild airways inflammation (rhinitis, cough, red throat) have episodes of oxygen desaturation during sleep.

Acute Disease↗

PubMed1993

Relationship of genotype to early pulmonary function in infants with cystic fibrosis identified through neonatal screening.

To determine whether pulmonary function in infants with asymptomatic cystic fibrosis is related to genotype, we studied 18 infants with cystic fibrosis identified through neonatal screening and 18 healthy control infants. Infants with cystic fibrosis (mean age, 2.0 months; range, 1.0 to 4.6 months) were identified from June 1990 to September 1991 through a statewide screening program based on elevated immunoreactive trypsinogen concentrations. Pulmonary function testing was done before and after inhalation of albuterol. There were no differences in pulmonary function between the cystic fibrosis group and the control infants (mean age, 2.7 months; range, 0.9 to 4.5 months). However, infants homozygous for the delta F508 deletion (n = 10) differed from infants with other genotypic variants of cystic fibrosis and control infants with respect to respiratory system resistance (79.4 +/- 11.5 vs 52.0 +/- 3.8 vs 55.5 +/- 5.0 cm H2O/L per second, respectively; p = 0.04) and specific conductance (0.15 +/- 0.02 vs 0.21 +/- 0.02 vs 0.21 +/- 0.02 cm H2O-1 sec-1, respectively; p = 0.02). Infants homozygous for the delta F508 deletion, but not other infants, responded to albuterol with a decrease in respiratory system resistance. We conclude that infants with asymptomatic cystic fibrosis homozygous for the delta F508 deletion have early evidence of airways obstruction and may need early respiratory treatment.

Cystic Fibrosis↗

PubMed1996

Correlates of osteopenia in patients with cystic fibrosis.

OBJECTIVE: As the expected survival improves for individuals with cystic fibrosis, these individuals face myriad medical complications. The goals of this study were to examine the prevalence of osteopenia in children and adults with cystic fibrosis and to elucidate the risk factors associated with deficits in bone mineral. METHODOLOGY: We compared bone mineral levels in 49 patients (30 female and 19 male) ages 8 through 48 years with those of age- and sex-matched control subjects. Lumbar spine, femoral neck, and whole-body bone mineral were measured by dual-energy radiographic absorptiometry and expressed in terms of bone mineral content, areal bone density (BMD), and bone mineral apparent density (BMAD), which corrects for differences in bone size. Clinical variables were evaluated as potential correlates of bone mineral. RESULTS: Patients with cystic fibrosis had significantly less bone mineral than did control subjects at all sites using all expressions of bone mass. Mean BMD z scores were -1.7 (lumbar spine), -1.9 (femoral neck), and -1.2 (whole body). BMAD z scores also were significantly low for age and gender. Twenty-six of the 49 patients (8 adolescents) had significant osteopenia according to their BMD z scores; 14 of the 45 patients (5 adolescents) with available BMAD z scores had significantly low values at one or more sites. Age, pubertal stage, body mass, caloric expenditure, illness severity, glucocorticoid therapy, and gonadal function predicted bone mineral status. Serum parathyroid hormone and calcium, carbohydrate intake, and weight-bearing activity had limited predictive value. Daily calcium intake and cystic fibrosis transmembrane regulator genotype did not predict bone mineral status. CONCLUSIONS: Osteopenia is common at all ages in cystic fibrosis, suggesting that inadequate bone mineral accretion as well as increased bone loss contribute to the deficits in bone mineral observed. Several clinical factors seem to contribute to these deficits.

Absorptiometry, Photon↗

PubMed1991

Activation of chloride channels in normal and cystic fibrosis airway epithelial cells by multifunctional calcium/calmodulin-dependent protein kinase.

Cystic fibrosis is associated with defective regulation of apical membrane chloride channels in airway epithelial cells. These channels in normal cells are activated by cyclic AMP-dependent protein kinase and protein kinase C. In cystic fibrosis these kinases fail to activate otherwise normal Cl- channels. But Cl- flux in cystic fibrosis cells, as in normal cells, can be activated by raising intracellular Ca2+ (refs 5-10). We report here whole-cell patch clamp studies of normal and cystic fibrosis-derived airway epithelial cells showing that Cl- channel activation by Ca2+ is mediated by multifunctional Ca2+/calmodulin-dependent protein kinase. We find that intracellular application of activated kinase and ATP activates a Cl- current similar to that activated by a Ca2+ ionophore, that peptide inhibitors of either the kinase or calmodulin block Ca2(+)-dependent activation of Cl- channels, and that a peptide inhibitor of protein kinase C does not block Ca2(+)-dependent activation. Ca2+/calmodulin activation of Cl- channels presents a pathway with therapeutic potential for circumventing defective regulation of Cl- channels in cystic fibrosis.

Calcium-Calmodulin-Dependent Protein Kinases↗

PubMed2000

Oral non-steroidal anti-inflammatory drug therapy for cystic fibrosis.

BACKGROUND: Maintenance of optimal lung function is an important therapeutic goal in cystic fibrosis as it is lung damage that, in the long term, is responsible for most premature death among affected people. It has been hypothesised that lung damage results from inflammation and that prolonged use of non-steroidal anti-inflammatory drugs may prevent progressive pulmonary deterioration and respiratory morbidity in cystic fibrosis. It is thus important to establish the current level of evidence about the potential benefits and harms of treatment with non-steroidal anti-inflammatory drugs. OBJECTIVES: The aim of this systematic review is to assess the effectiveness of treatment with non-steroidal anti-inflammatory agents in cystic fibrosis. SEARCH STRATEGY: Trials were ascertained from the Cochrane Cystic Fibrosis and Genetic Disorders Specialised Register of Controlled Trials which includes published and unpublished trials identified through electronic databases such as Medline and Embase as well as those identified from handsearching of journals and conference proceedings. Pharmaceutical companies manufacturing non-steroidal anti-inflammatory drugs were also contacted to identify any trials of non-steroidal anti-inflammatory drugs in cystic fibrosis. Date of the most recent search of the Group's specialised register: November 1999. SELECTION CRITERIA: All randomised or pseudorandomised controlled trials, published and unpublished, comparing non-steroidal anti-inflammatory drugs, administered orally at any dose for a period of at least two months, to placebo in patients with cystic fibrosis. DATA COLLECTION AND ANALYSIS: The following outcomes were assessed: objective measures of lung function, nutritional status, radiological assessment of pulmonary involvement, use of intravenous antibiotics, hospital admissions, survival, frequency of major and minor adverse effects and compliance with therapy. MAIN RESULTS: Three trials involving 145 patients aged from five to 39 years with a maximum follow up of four years met the inclusion criteria. Methodological quality was deemed good or adequate in two. Two trials, both reporting effectiveness of ibuprofen in subjects with mild lung disease, were from the same centre and included some patients in common, while the third assessed piroxicam in subjects with more severe impairment of respiratory function. Variation in outcomes reported and their summary measures precluded calculation of pooled treatment estimates. Only one trial reported within-subject changes in pulmonary function and the findings of this trial suggested that there was a greater absolute annual decline in percentage predicted forced expiratory volume in one second among controls than among those treated with ibuprofen. In a post-hoc sub-group analysis this effect was confined to children aged five to 13 years. In addition, in this one trial long term use of high dose ibuprofen was associated with reduced intravenous antibiotic usage, improved nutritional and radiological pulmonary status. No major adverse effects were reported but the power of the trials to identify clinically important differences in the incidence of adverse effects was low. REVIEWER'S CONCLUSIONS: While there is preliminary evidence to suggest that non-steroidal anti-inflammatory drugs may prevent pulmonary deterioration in subjects with mild lung disease due to cystic fibrosis, currently their routine use cannot be recommended. Further trials are required to confirm that their use prevents pulmonary deterioration and is associated with improved nutritional status. Such trials should also address the age group of subjects most likely to benefit, the prevalence of important adverse effects and the optimal dosage schedule as well as any reduction in concomitant therapy. Multi-centre trials will add to the validity of findings by enhancing their generalisability. The question of whether anti-inflammatory treatment prevents lung damage in pre-symptomatic

Anti-Inflammatory Agents, Non-Steroidal↗

PubMed1980

Allergic bronchopulmonary aspergillosis complicating cystic fibrosis in childhood.

Allergic bronchopulmonary aspergillosis, known to be associated with cystic fibrosis in older patients, occurred in 7 young atopic children with cystic fibrosis. The diagnosis was suggested by the onset of, or the increase in, asthmatic symptoms accompanied by major chest x-ray changes ranging from total collapse of a lung or lobe to extensive but changing areas of consolidation. Each of the children had a blood eosinophilia, positive type I skin tests to Aspergillus fumigatus, and reversible airways obstruction. Most had a positive type III skin test and circulating precipitins to A. fumigatus, with raised IgE levels which contained specific antibodies to the fungus on radioallergosorbent (RAST) test. None had advanced suppurative chest disease of cystic fibrosis. None was given specific antifungal agents; two received systemic treatment with corticosteroids, the other received additional drugs for their asthma. Two developed total collapse of one lung, one child being only 2 years old. Five have had recurrences of pulmonary shadowing typical of allergic aspergillosis but are not showing significant progression of their cystic fibrosis lung disease. Our experience suggests that there should be an increased awareness of this condition, particularly its association with extensive pulmonary collapse or consolidation in children with cystic fibrosis who are atopic.

Aspergillosis, Allergic Bronchopulmonary↗

PubMed1991

The basic defect in cystic fibrosis.

Recent evidence strongly suggests that the cystic fibrosis gene product (CFTR) is a Cl- channel. Its properties, however, differ from those of a 30-50 pS outwardly rectifying channel previously implicated as defective in cystic fibrosis. It is still uncertain whether the pleiotropic effects of the CF defect, such as increased airway Na+ absorption and mucus sulfation, are secondary to reduced Cl- conductance, or reflect additional functions of CFTR.

Chloride Channels↗

PubMed1991

Neonatal screening strategy for cystic fibrosis using immunoreactive trypsinogen and direct gene analysis.

OBJECTIVE: To assess the effectiveness of a two tier neonatal screening strategy for cystic fibrosis, which combines estimation of immunoreactive trypsinogen followed by direct gene analysis in dried blood spot samples collected at age 5 days. DESIGN: Prospective study of two tier screening strategy. The first tier of testing immunoreactive trypsinogen concentration was measured in dried blood spot samples from neonates aged 4-5 days. In the second tier direct gene analysis to detect cystic fibrosis mutations deltaF508 and deltaI506 was performed in those blood spot samples which produced the highest 1% of immunoreactive trypsinogen values. Direct gene analysis was also performed on blood spot samples from infants with suspected or confirmed meconium ileus, regardless of the immunoreactive trypsinogen value. SETTING: The South Australian Neonatal Screening Programme, operating from the department of chemical pathology at Adelaide Children's Hospital. Subjects--All 12,056 neonates born in South Australia between December 1989 and June 1990. No selection criteria were applied. INTERVENTIONS: All infants found to have two recognised cystic fibrosis mutations on direct gene analysis were referred directly for clinical management, and those with one recognised cystic fibrosis mutation were recalled for a sweat test; their families were given genetic counselling. MAIN OUTCOME MEASURES: Direct or exclusion of cystic fibrosis by sweat testing of neonates identified as being at high risk of cystic fibrosis on screening and of those at minimum risk but whose subsequent clinical history raised suspicion about the disease. RESULTS: Of the 12,056 infants screened, 11,907 (98.8%) were reported as "cystic fibrosis not indicated" on the basis of low immunoreactive trypsinogen values. Of the 148 (1.23%) infants with raised immunoreactive trypsinogen values and one (0.008%) with meconium ileus, 132 (1.09%) were reported as cystic fibrosis not indicated, four (0.033%) were identified as having cystic fibrosis, and 13 (0.108%) were recalled for sweat testing after direct gene analysis for the presence of the deltaF508 and deltaI506 cystic fibrosis mutations. No cases of affected infants are known to have been missed to date. CONCLUSION: The strategy of measurement of immunoreactive trypsinogen followed by direct gene analysis is a highly specific neonatal screen for cystic fibrosis, requiring only 2.8 families to be contacted for every case of cystic fibrosis diagnosed.

Cystic Fibrosis↗

PubMed2006

Long-term docosahexaenoic acid therapy in a congenic murine model of cystic fibrosis.

We used a congenic C57Bl/6J cystic fibrosis transmembrane conductance regulator (Cftr)(-/-) mouse model, which develops cystic fibrosis (CF)-like pathology in all organs, to evaluate the short- and long-term therapeutic effects of dietary docosahexaenoic acid (DHA). Thirty-day-old Cftr(-/-) mice and wild-type littermates were randomized to receive a liquid diet with or without DHA (40 mg/day). Animals were killed for histological and lipid analysis after 7, 30, and 60 days of therapy. DHA had no significant therapeutic or harmful effect on the lung, pancreas, or ileum of the Cftr(-/-) mice or their wild-type littermates. In contrast, dietary DHA resulted in highly significant amelioration of the severity of liver disease in the Cftr(-/-) mice, primarily a reduction in the degree of peri-portal inflammation. Additionally, these detailed measurements confirm our previous findings that Cftr(-/-) mice have significant alterations in the pancreas (except external acinar diameter), ileum, liver, lung, and salivary (except sublingual) glands at all ages compared with their age-matched wild-type littermates. In conclusion, inhibition of cytokines and/or eicosanoid metabolism and release of endogenous inhibitors of inflammation by DHA may account for the anti-inflammatory effects in the liver of this congenic murine model of CF. The potential therapeutic benefits of DHA in severe CF-associated liver disease remain to be explored.

Administration, Oral↗

PubMed2006

Respiratory epithelial gene expression in patients with mild and severe cystic fibrosis lung disease.

Despite having identical cystic fibrosis transmembrane conductance regulator genotypes, individuals with DeltaF508 homozygous cystic fibrosis (CF) demonstrate significant variability in severity of pulmonary disease. This investigation used high-density oligonucleotide microarray analysis of nasal respiratory epithelium to investigate the molecular basis of phenotypic differences in CF by (1) identifying differences in gene expression between DeltaF508 homozygotes in the most severe 20th percentile of lung disease by forced expiratory volume in 1 s and those in the most mild 20th percentile of lung disease and (2) identifying differences in gene expression between DeltaF508 homozygotes and age-matched non-CF control subjects. Microarray results from 23 participants (12 CF, 11 non-CF) met the strict quality control guidelines and were used for final data analysis. A total of 652 of the 11,867 genes identified as present in 75% of the samples were significantly differentially expressed in one of the three disease phenotypes: 30 in non-CF, 53 in mild CF, and 569 in severe CF. An analysis of genes differentially expressed by severity of CF lung disease demonstrated significant upregulation in severe CF of genes involved in protein ubiquination (P < 0.04), mitochondrial oxidoreductase activity (P < 0.01), and lipid metabolism (P < 0.03). Analysis of genes with decreased expression in patients with CF compared with control subjects demonstrated significant downregulation of genes involved in airway defense (P < 0.047) and protein metabolism (P < 0.048). This study suggests that differences in CF lung phenotype are associated with differences in expression of genes involving airway defense, protein ubiquination, and mitochondrial oxidoreductase activity and identifies specific new candidate modifiers of the CF phenotype.

Adolescent↗

PubMed1999

Lung resection for the treatment of severe localised bronchiectasis in cystic fibrosis patients.

BACKGROUND: A small proportion of cystic fibrosis patients develop severe localised bronchiectasis. When this persists despite maximal medical therapy it presents a difficult management problem. Lung transplantation cannot be justified. We report encouraging results in six patients with severe localised bronchiectasis and cystic fibrosis who underwent pulmonary resection. METHODS: Each child had severe localised bronchiectasis despite maximal medical therapy. Intensive preoperative toilet was instituted and pulmonary resection undertaken when lung function was optimal. RESULTS: There was a marked improvement in symptoms in every case. No significant long-standing morbidity was associated with the resection. There was no significant decrease in pulmonary function following resection. CONCLUSION: Pulmonary resection should be considered in the management of severe localised bronchiectasis unresponsive to maximal medical therapy in cystic fibrosis patients.

Bronchiectasis↗

PubMed2003

Evaluation of MRP1-5 gene expression in cystic fibrosis patients homozygous for the delta F508 mutation.

Cystic fibrosis (CF), due to mutations of the cystic fibrosis transmembrane conductance regulator (CFTR), exhibits a wide range of disease severity, even among deltaF508 homozygous patients, and the mechanisms of this variability have yet to be elucidated. In view of the close structural homology and possible functional overlap between CFTR and Multidrug Resistance-associated Proteins (MRPs), MRPs were investigated as potentially relevant factors in CF pathophysiology. MRP1-5 gene expression was analyzed in nasal respiratory epithelial cells from deltaF508 homozygous patients (n = 19) and control subjects (n = 20) using semiquantitative RT-PCR. Significantly lower MRP1 and MRP5 transcript levels were found in CF patients than in control subjects. MRP1 and MRP5 transcript levels were strongly correlated (r = 0.71). In CF patients, low MRP1 transcript levels were associated with more severe disease as assessed by the Shwachman score. A relation was also observed between MRP1 levels and presence of a cAMP-independent chloride conductive pathway, as determined by a halide-sensitive fluorescent assay. These results suggest that MRPs, especially MRP1, might play a role in CF phenotype and might therefore constitute a target for a novel pharmacotherapy of CF.

Adolescent↗

PubMed1976

Abnormal levels of 3':5'-cyclic AMP in isoproterenol-stimulated fibroblasts from patients with cystic fibrosis.

To determine if the abnormalities of exocrine secretion characteristic of cystic fibrosis could be investigated in vitro, I studied the synthesis of 3':5'-cyclic AMP after isoproterenol stimulation in skin fibroblasts derived from patients with cystic fibrosis and from normal individuals. Comparison of normal and cystic fibrosis cells showed that the latter had 2- to 5-fold greater levels of intracellular cyclic AMP after stimulation with isoproterenol. The difference between the strains was observed at every stage of the culture cycle and was specific to stimulation by beta-adrenergic agonists. It could not be accounted for by different dose- or time-response curves nor by leakage of cyclic AMP into the medium. The increased sensitivity to catecholamines may reflect an intrinsic genetic property of cystic fibrosis cells, and it may be feasible to use this system to study the biochemical basis of the genetic defect in cystic fibrosis.

Age Factors↗

PubMed1993

Case-control and vector studies of nosocomial acquisition of Pseudomonas cepacia in adult patients with cystic fibrosis.

OBJECTIVE: To examine factors associated with nosocomial acquisition of Pseudomonas cepacia in adult patients with cystic fibrosis. DESIGN: A retrospective case-control study of 5 patients with nosocomial acquisition of P cepacia versus 20 matched controls who failed to develop P cepacia infection. Selective handwashing, air sampling, and respiratory equipment sampling also were performed. SETTING: A university hospital providing tertiary care to 95 adult cystic fibrosis patients. PATIENTS: All patients are adults with known cystic fibrosis. Case definition required multiple negative sputum cultures for P cepacia prior to and during admission, with a positive sputum culture prior to discharge. Controls had negative sputum cultures for P cepacia prior to and throughout hospitalization. Controls were matched for age, gender, disease severity, and frequency of hospitalizations. RESULTS: Factors associated with increased risk of nosocomial acquisition of P cepacia included receiving humidifier or nebulized treatments (60% versus 5%, p = .016, odds ratio = 28.5, 95% confidence interval = 1.93 to 420.58). Factors without significance included ward, room, teaching versus nonteaching status, use of steroids, sharing a hospital room with another cystic fibrosis patient, antibiotic use, presence of portocath in situ, or socializing with another individual with cystic fibrosis known to be P cepacia-positive. Air sampling studies failed to demonstrate aerosolization of P cepacia by coughing cystic fibrosis patients over a 1-hour sampling time. Handwashing studies failed to demonstrate P cepacia on hands of cystic fibrosis patients, nurses, or physiotherapists (before or after physiotherapy). Reservoirs from nebulizers consistently grew P cepacia following therapy. CONCLUSIONS: Respiratory equipment may be an important source of nosocomial acquisition of P cepacia in adult cystic fibrosis patients.

Adult↗