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Female survival worse than male between 2-20 years of age 1
35% of patients are older than 18 years of age 2
Median survival 36.8 years 3 compared to 1930s when life expectancy was about 6 months 2
1.Goss, CH and Rosenfeld, M. Update on cystic fibrosis epidemiology. Current Opinion in Pulmonary Medicine. 10:510-514; 2004. 2. Davis, P. Cystic Fibrosis Since 1938. AJRCCM Articles in Press. Doi: 10.1164/rccm.200505-840OE; 2005. 3.www.cff.org/news/general_news
B. cepacia syndrome: fevers, rapidly progressive necrotizing pneumonia, death
Chronic cepacia infection—decreased lung function and increased mortality
Several closely related species termed genomovars 1
III has been associated with more severe disease
Holmes, A, Govan, J, and Goldstein, R. Agricultural Use of Burkholderia (Pseudomonas) cepacia: A Threat to Human Health? Emerging Infectious Diseases. 4(2):221-227; 1998 1. Gibson, RL, Burns, JL, and Ramsey, BW. AJRCCM 168 (918-951); 2003.
Viscid mucoid cervical secretions of low volume in women with CF 1
Pregnancy and CF:
Goss et al, 2003---no significant difference in survival in women who became pregnant with CF compared to women who did not become pregnant (after adjusting for disease severity) 2
1.Quinton, P. Physiologic Basis of Cystic Fibrosis. Physiol Rev 79:3-22, 1999 2.Goss, CH, Rubenfeld, GD, Otto, K and Aitken, ML. The effect of pregnancy on survival in women with cystic fibrosis. Chest 124(4):1460-68; 2003.