Update on cystic fibrosis epidemiology

被引:31
作者
Goss, CH
Rosenfeld, M
机构
[1] Univ Washington, Med Ctr, Dept Med, Seattle, WA 98195 USA
[2] Univ Washington, Sch Med, Dept Pediat, Seattle, WA 98195 USA
关键词
cystic fibrosis; epidemiology; survival; outcome;
D O I
10.1097/01.mcp.0000138994.46519.72
中图分类号
R56 [呼吸系及胸部疾病];
学科分类号
摘要
Purpose of review With the improving survival of cystic fibrosis (CF) patients, the clinical spectrum of this complex multisystem disease continues to evolve. Epidemiologic studies have provided important insight into the disease course, prognosis, and complications. This review summarizes recent advances in our understanding of predictors of survival and outcome and modifiers of disease in CF. This review is riot meant to be comprehensive, but highlights selected studies, many of which have particular relevance to the growing number of older CF patients. Recent findings Survival rates of US CF patients improved remarkably over the past 15 years, but most of the improvement was limited to patients 2 to 15 years of age. Both median household income and ambient air pollutants were found to be important modifiers of disease, echoing research reported in other chronic lung diseases. Genotype classified according to functional mutation class was highly associated with outcome (class I, II, and III mutations were associated with the highest mortality). Of the emerging pathogens, B. cepacia complex and B. gladioli are the most prominent. A small but significant percentage of patients have been shown to acquire new B. cepacia complex or B. gladioli strains with time. Summary Epiclemiologic research in cystic fibrosis continues to inform patient care and clinical research, and to generate new hypotheses regarding pathophysiology. Survival and outcomes continue to improve in this multisystem disease. With continued improving survival, epidemiologic studies will be critical to tracking changes in prognosis and outcome.
引用
收藏
页码:510 / 514
页数:5
相关论文
共 36 条
[1]  
ALOUL AM, 2004, THORAX, V59, P334
[2]   Evidence for spread of a clonal strain of Pseudomonas aeruginosa among cystic fibrosis clinics [J].
Armstrong, D ;
Bell, S ;
Robinson, M ;
Bye, P ;
Rose, B ;
Harbour, C ;
Lee, C ;
Service, H ;
Nissen, M ;
Syrmis, M ;
Wainwright, C .
JOURNAL OF CLINICAL MICROBIOLOGY, 2003, 41 (05) :2266-2267
[3]   Detection of a widespread clone of Pseudomonas aeruginosa in a pediatric cystic fibrosis clinic [J].
Armstrong, DS ;
Nixon, GM ;
Carzino, R ;
Bigham, A ;
Carlin, JB ;
Robins-Browne, RM ;
Grimwood, K .
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE, 2002, 166 (07) :983-987
[4]   Burkholderia cepacia complex in cystic fibrosis:: Frequency of strain replacement during chronic infection [J].
Bernhardt, SA ;
Spilker, T ;
Coffey, T ;
LiPuma, JJ .
CLINICAL INFECTIOUS DISEASES, 2003, 37 (06) :780-785
[5]   Liver disease in cystic fibrosis: A prospective study on incidence, risk factors, and outcome [J].
Colombo, C ;
Battezzati, PM ;
Crosignani, A ;
Morabito, A ;
Costantini, D ;
Padoan, R ;
Giunta, A .
HEPATOLOGY, 2002, 36 (06) :1374-1382
[6]  
Corey M, 1996, AM J EPIDEMIOL, V143, P1007, DOI 10.1093/oxfordjournals.aje.a008664
[7]   OUTCOME OF PREGNANCY IN WOMEN WITH CYSTIC-FIBROSIS [J].
EDENBOROUGH, FP ;
STABLEFORTH, DE ;
WEBB, AK ;
MACKENZIE, WE ;
SMITH, DL .
THORAX, 1995, 50 (02) :170-174
[8]   International comparison of median age at death from cystic fibrosis [J].
Fogarty, A ;
Hubbard, R ;
Britton, J .
CHEST, 2000, 117 (06) :1656-1660
[9]   Cystic fibrosis and pregnancy. Report from French data (1980-1999) [J].
Gillet, D ;
de Braekeleer, M ;
Bellis, G ;
Durieu, I .
BJOG-AN INTERNATIONAL JOURNAL OF OBSTETRICS AND GYNAECOLOGY, 2002, 109 (08) :912-918
[10]   Pregnancy in cystic fibrosis - Fetal and maternal outcome [J].
Gilljam, M ;
Antoniou, M ;
Shin, J ;
Dupuis, A ;
Corey, M ;
Tullis, DE .
CHEST, 2000, 118 (01) :85-91