Predictors of mortality in adults with cystic fibrosis

Pediatric Pulmonology - Tập 42 Số 6 - Trang 525-532 - 2007
Jo‐Maree Courtney1, Judy Bradley1,2, John McCaughan3, Terence M. O’Connor4, C. Shortt4, C. P. Bredin4, Ian Bradbury5, J.S. Elborn1
1Adult Cystic Fibrosis Unit, Belfast City Hospital, Belfast, Northern Ireland, Ireland.
2Health and Rehabilitation Sciences Research Institute, University of Ulster, Belfast, Ireland
3Department of Microbiology, Queens University, Belfast, Ireland
4Adult Cystic Fibrosis Unit, Cork University Hospital, Cork, Ireland
5Department of Medical Statistics, University of Ulster, Belfast, Ireland

Tóm tắt

AbstractAssessment 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. © 2007 Wiley‐Liss, Inc.

Từ khóa


Tài liệu tham khảo

10.1136/adc.77.6.493

10.1136/thx.46.12.881

10.5694/j.1326-5377.1979.tb112071.x

Anonymous, 1984, Survival in cystic fibrosis [editorial], Lancet, 2, 663

10.1016/0895-4356(88)90063-7

10.1111/j.1651-2227.1982.tb09645.x

10.1136/bmj.316.7147.1771

10.1136/adc.66.9.1018

10.1056/NEJM199204303261804

10.1016/S0022-3476(97)70025-8

10.1016/0002-9343(87)90147-1

10.1111/j.1651-2227.1978.tb16273.x

10.1093/oxfordjournals.bmb.a072580

10.1136/thx.51.10.971

10.1136/bmj.298.6672.483

10.1378/chest.106.3.800

10.1016/S0031-3955(16)33752-X

10.1164/ajrccm.160.5.9805046

10.1046/j.1440-1754.2001.00629.x

10.1016/S0022-3476(84)80993-2

Speert DP and the Clinical Subcommittee, Canadian Cystic Fibrosis Foundation, 1993, Epidemiology of Pseudomonas cepacia in cystic fibrosis, Can J Infect Dis, 4, 163

10.1007/BF03167716

10.1016/j.jcf.2004.01.005

10.1016/S0022-3476(85)80511-4

10.1164/ajrccm.154.5.8912731

Clode FE, 2000, Distribution of genes encoding putative transmissibility factors among epidemic and nonepidemic strains of Burkholderia cepacia from cystic fibrosis patients in the United Kingdom, J Clin Microbiol, 38, 1763, 10.1128/JCM.38.5.1763-1766.2000

10.1002/ppul.1950120306

10.1002/ppul.10100

10.1002/ppul.10042

10.1016/0140-6736(91)92449-C

10.1093/oxfordjournals.aje.a009172

10.1016/0140-6736(90)92571-X

10.1164/ajrccm.163.1.9811076

10.1136/thx.51.4.374

10.1136/thx.53.5.432

10.1002/ppul.10127

10.1164/ajrccm.157.6.9704086

10.1016/0895-4356(94)00230-N

10.1001/archpedi.1985.02140090031019

10.1056/NEJM199011293232203

10.1016/0140-6736(90)92566-Z

10.1056/NEJM199310283291804

10.1136/thx.51.2.126

10.1016/S0022-3476(05)83042-2

10.1042/cs0850563