Gender differences in the Scandinavian cystic fibrosis population

Abstract

AimsTo explore whether gender differences in the Scandinavian Cystic Fibrosis (CF) patients exist in the areas of key clinical parameters, complications, and medication.MethodsCross‐sectional data on 890 (416 female) pancreatic insufficient CF patients were evaluated regarding chronic infection, body mass index, lung function, medication, and diabetes, as well as data of Pseudomonas infection status, antibiotic treatment and hospitalization from 1‐year follow‐up.ResultsWe found no differences in lung function, body mass index, or frequency of diabetes. The adult group consisted of more males than females (208:168). We found no significant difference in prevalence of chronic Pseudomonas aeruginosa infection, but during the follow‐up the incidence of new chronic infection was higher in adult females (10/33 vs. 4/56). Females had higher prevalence of Burkholderia infection (21/416 vs. 11/474). Adult females had more days on intravenous antibiotics (median 39 vs. 26 days/year), and days in hospital (median 2 vs. 0 days/year). More adult females received inhaled and oral steroids. In the pediatric cohort, females were treated more often with macrolides as an anti‐inflammatory agent.ConclusionWe found no gender difference in key clinical parameters in our CF population. However, our study showed a higher risk of Pseudomonas and Burkholderia infection among the female patients. Additionally, we found that female patients require more intensified treatment regarding antibiotics, macrolides, steroids and days of hospitalization, indicating a true female disadvantage even with modern aggressive treatment. The finding of more males than females in the adult population suggesting a male advantage, warrants a mortality study. Pediatr Pulmonol. 2010; 45:959–965. © 2010 Wiley‐Liss, Inc.

Effect of Estrogen on Pseudomonas Mucoidy and Exacerbations in Cystic Fibrosis

“…P seudomonas aeruginosa is a gram-negative opportunistic pathogen associated with cystic fibrosis, 1 a multisystem genetic disease characterized by defects in the cystic fibrosis transmembrane conductance regulator (CFTR) protein, which results in recurrent infective exacerbations. Median overall survival among patients with cystic fibrosis in the United States is 39 years, although a sexual dichotomy persists in disease severity, [2][3][4] with women having a survival disadvantage and poorer lung function. [5][6][7] The Republic of Ireland has the highest incidence and carrier rate of cystic fibrosis worldwide 8 and a survival disadvantage for women.…”

Pulmonary Inflammation in Cystic Fibrosis: Impact of Innate Immunity and Estrogen

“…However, it is clear that female gender is a negative prognostic factor in CF, a finding that has been demonstrated in several countries, registries, and CF care centers. Two recent studies found no difference in survival between genders [19,20]; however, Olsen et al did acknowledge that female patients with CF are at a higher risk of P aeruginosa and Burkholderia spp. colonization, require more intensive treatment with antibiotics, and have a greater rate of hospitalization compared with male patients [19].…”

“…Two recent studies found no difference in survival between genders [19,20]; however, Olsen et al did acknowledge that female patients with CF are at a higher risk of P aeruginosa and Burkholderia spp. colonization, require more intensive treatment with antibiotics, and have a greater rate of hospitalization compared with male patients [19]. It was noted in that study that a gender gap may appear after adolescence, which is the timeframe within which other published studies have shown such differences to exist [20].…”

The Prognosis of Cystic Fibrosis - A Clinician's Perspective

“…One example of 'the clinician's choice' in the discussion is not to dwell on the often reported issue of a 'female disadvantage' (Rosenfeld et al, 1997;Mehta et al, 2010;Olesen et al, 2010), which may not concern adult diagnosed CF patients (Nick et al, 2010). Numerous hypotheses have been brought forward to account for it, without any practical impact to date.…”

“…Numerous hypotheses have been brought forward to account for it, without any practical impact to date. Furthermore, though this might have been achieved at the price of a higher burden of treatment in females (Olesen et al, 2010), there is evidence that modern intensive treatment may result in similar key clinical parameters for the two genders (Verma et al, 2008;Olesen et al, 2010).…”