Gender Gap in Cystic Fibrosis Mortality

Abstract

The authors conducted the largest study to date of survival in cystic fibrosis. The study cohort consisted of all patients with cystic fibrosis seen at Cystic Fibrosis Foundation-accredited care centers in the United States between 1988 and 1992 (n = 21,047), or approximately 85% of all US patients diagnosed with cystic fibrosis. Cox proportional hazards regression analysis was used to compare the age-specific mortality rates of males and females and to identify risk factors serving as potential explanatory variables for the gender-related difference in survival. Among the subjects 1-20 years of age, females were 60% more likely to die than males (relative risk = 1.6, 95% confidence interval 1.4-1.8). Outside this age range, male and female survival rates were not significantly different. The median survival for females was 25.3 years and for males was 28.4 years. Nutritional status, pulmonary function, and airway microbiology at a given age were strong predictors of mortality at subsequent ages. Nonetheless, differences between the genders in these parameters, as well as pancreatic insufficiency, age at diagnosis, mode of presentation, and race, could not account for the poorer survival among females. Even after adjustment for all these potential risk factors, females in the age range 1-20 years remained at greater risk for death (relative risk = 1.6, 95% confidence interval 1.2-2.1). The authors concluded that in 1- to 20-year-old individuals with cystic fibrosis, survival in females was poorer than in males. This "gender gap" was not explained by a wide variety of potential risk factors.

Long noncoding RNA are aberrantly expressed in vivo in the cystic fibrosis bronchial epithelium

“…Females with CF have poorer lung function, higher mortality, earlier colonisation with Pseudomonas aeruginosa, and deteriorate with Burkholderia earlier than their male counterparts (Block et al, 2006, Corey et al, 1997, Rosenfeld et al, 1997, Demko et al, 1995, Jackson et al, 2011a, Jackson et al, 2011b. Recent work has focused on the role of steroid sex hormones such as estrogens in this "gender gap" (Chotirmall et al, 2010, Chotirmall et al, 2012a and has been reviewed elsewhere (Chotirmall et al, 2012b, Saint-Criq and Harvey, 2013, Sweezey and Ratjen, 2013.…”

Parallel evolution and local differentiation in quinolone resistance in Pseudomonas aeruginosa

“…In Canada, for example, it occurs in 60-70 % of adults with CF (Stephenson, 2008). Both population-based and case-control studies have demonstrated that infection with P. aeruginosa appears to be an independent prognostic factor that carries with it an increased risk of death in CF patients, irrespective of lung function (Corey & Farewell, 1996;Rosenfeld et al, 1997).…”

Resting energy expenditure in females with cystic fibrosis: Is it affected by puberty?

“…It is important to understand this mechanism despite overall improved nutrition, growth and survival for CF, as gender disparity still exists with poorer long-term survival in the female population (Rosenfeld et al, 1997). The results from this study suggest that REE is elevated in females with CF before the commencement of puberty and remains elevated postpuberty.…”

“…Our previous larger cross-sectional study of both prepubertal and pubertal children with CF reported a greater elevation of REE in the females compared with males (Allen et al, 2003). This gender difference may explain why females with CF have inferior growth and overall impaired survival compared with males (Rosenfeld et al, 1997;Stallings, 2003). An even more recent small study in females suggested that the difference in REE between girls with CF and controls was greater in postmenarcheal than premenarcheal group (Stallings et al, 2005); however, in our preliminary study, we were unable to confirm this finding (Allen et al, 2004).…”