Predictors of mortality in adults with cystic fibrosis
- 27 April 2007
- journal article
- research article
- Published by Wiley in Pediatric Pulmonology
- Vol. 42 (6), 525-532
- https://doi.org/10.1002/ppul.20619
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 FEV1 (%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) FEV1 %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/m2 in comparison to those remaining alive, 20.7 (3.4) kg/m2, 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 ΔF508 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. FEV1% (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. FEV1% and infection with P.aeruginosa or B.cepacia complex were the most significant predictors of survival in adults with CF. Pediatr Pulmonol. 2007; 42:525–532.Keywords
This publication has 45 references indexed in Scilit:
- Pseudomonas aeruginosa and other predictors of mortality and morbidity in young children with cystic fibrosisPediatric Pulmonology, 2002
- Incidence, population, and survival of cystic fibrosis in the UK, 1968-95Archives of Disease in Childhood, 1997
- Nutrition and survival in cystic fibrosis.Thorax, 1996
- The cystic fibrosis gene and resting energy expenditureThe Journal of Pediatrics, 1991
- Heart-lung transplantation for cystic fibrosis. 1: Assessment.Archives of Disease in Childhood, 1991
- Severity of cystic fibrosis in patients homozygous and heterozygous for ΔF508 mutationThe Lancet, 1991
- Independent genetic determinants of pancreatic and pulmonary status in cystic fibrosisThe Lancet, 1990
- Effects of social class, sex, and region of residence on age at death from cystic fibrosis.BMJ, 1989
- Pseudomonas cepacia colonization in patients with cystic fibrosis: Risk factors and clinical outcomeThe Journal of Pediatrics, 1985
- Pseudomonas cepacia infection in cystic fibrosis: An emerging problemThe Journal of Pediatrics, 1984