title: Predictors of mortality in adults with cystic fibrosis.
abstract: Assessment of prognostic indicators in patients with cystic fibrosis (CF) is important. The study's aim was to assess the relative contribution of gender, genetics and microbiology on survival in adults with CF. Adult patients were studied from 1995 to 2005 and data collected included FEV(1) (%predicted), body mass index (BMI), genetics, and microbiology. Data was available on 183 patients in 1995. Forty-five patients died in the subsequent 10 years. Patients who died during the study had lower mean (SD) FEV(1) %predicted in 1995 when compared to those remaining alive, 41.5 (15.2)% versus 69.8 (23.2)% predicted, respectively, P<0.001 and they had lower mean (SD) BMI in 1995, 19.2 (3.3) kg/m(2) in comparison to those remaining alive, 20.7 (3.4) kg/m(2), P=0.008. The proportion of patients infected with Pseudomonas aeruginosa and Burkholderia cepacia complex was higher in the group who died during the study compared to those remaining alive, odds ratio 20.9 P<0.0001 and 7.1 P<0.0001, respectively. The presence of the Delta F508 homozygous mutation did not alter survival, P=0.3. Patients infected with either P.aeruginosa or B.cepacia complex had reduced survival compared to those without infection, P=0.01 and P<0.0001, respectively. FEV(1)% (P<0.0001), infection with P.aeruginosa (P=0.005) or B.cepacia complex (P=0.03) were the only significant predictors of mortality. This study demonstrates adults who died were more likely to have worse lung function and be infected with either P.aeruginosa or B.cepacia complex. FEV(1)% and infection with P.aeruginosa or B.cepacia complex were the most significant predictors of survival in adults with CF.
authors.affiliation: Adult Cystic Fibrosis Unit, Belfast City Hospital, Belfast, Northern Ireland, Ireland. courtneyjulia@hotmail.com
authors.forename: J M
authors.initials: JM
authors.lastname: Courtney
authors.forename: J
authors.initials: J
authors.lastname: Bradley
authors.forename: J
authors.initials: J
authors.lastname: Mccaughan
authors.forename: T M
authors.initials: TM
authors.lastname: O'Connor
authors.forename: C
authors.initials: C
authors.lastname: Shortt
authors.forename: C P
authors.initials: CP
authors.lastname: Bredin
authors.forename: I
authors.initials: I
authors.lastname: Bradbury
authors.forename: J S
authors.initials: JS
authors.lastname: Elborn
mesh_terms: D000293:Adolescent; D000328:Adult; D015992:Body Mass Index; D019121:Burkholderia Infections / Q000150:complications; D016956:Burkholderia cepacia / Q000302:isolation & purification / Q000472:pathogenicity; D003550:Cystic Fibrosis / Q000382:microbiology* / Q000401:mortality* / Q000503:physiopathology; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics / Q000502:physiology; D005260:Female; D005541:Forced Expiratory Volume; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D009154:Mutation / Q000235:genetics; D011237:Predictive Value of Tests; D011552:Pseudomonas Infections / Q000150:complications; D011550:Pseudomonas aeruginosa / Q000302:isolation & purification / Q000472:pathogenicity; D012044:Regression Analysis; D012189:Retrospective Studies; D012727:Sex Characteristics; D013183:Sputum / Q000382:microbiology*
chemical_list: C093349:cystic fibrosis transmembrane conductance regulator delta F508; D019005:Cystic Fibrosis Transmembrane Conductance Regulator