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Gender differences in cystic fibrosis: Pseudomonas aeruginosa infection
C A Demko1, P J Byard, P B Davis
1Department of Pediatrics, Case Western Reserve University, Rainbow Babies and Children's Hospital, Cleveland, OH 44106, USA.
Insights
Females with cystic fibrosis (CF) acquire Pseudomonas aeruginosa (PA) earlier, but this doesn't fully explain their poorer survival. Other factors likely contribute to survival differences in CF patients with PA infection.
Area of Science:
- Medical Research
- Pulmonology
- Infectious Disease
Background:
- Females with cystic fibrosis (CF) have a median survival age approximately 3 years younger than males.
- Pseudomonas aeruginosa (PA) infection is a common complication in CF, impacting lung disease severity and survival.
Purpose of the Study:
- To investigate whether earlier acquisition of PA or its greater impact on lung disease contributes to poorer female CF survival.
- To analyze the association between PA infection phenotypes (mucoid vs. nonmucoid) and patient outcomes.
Main Methods:
- Analysis of data from 848 CF patients followed for at least 2 years.
- Inclusion of PA infection status, survival data, pulmonary function tests, and chest X-ray scores.
- Comparison of outcomes based on PA infection status and phenotype (mucoid vs. nonmucoid).
Main Results:
- Females with CF acquired chronic mucoid PA infection at a median age 1.7 years earlier than males.
- Mucoid PA infection was linked to poorer survival, worse chest X-ray scores, and reduced pulmonary function.
- The rate of pulmonary function decline after mucoid PA infection was similar in males and females.
- Males demonstrated better survival and pulmonary function (percent predicted FEV1) even when stratified by PA infection phenotype.
Conclusions:
- Earlier acquisition of mucoid PA in females does not fully account for their reduced survival in cystic fibrosis.
- Additional factors beyond PA infection timing and impact likely influence the survival disparity between male and female CF patients.
Abstract:
The median survival age for females with cystic fibrosis (CF) is approximately 3 years younger than for males. We tested whether earlier acquisition of Pseudomonas aeruginosa (PA) by female CF patients or the greater impact of this organism on their lung disease, or both, contribute to their poorer survival. PA infection status, survival, pulmonary function tests, and chest X-ray scores from patients who were followed at our center for at least 2 years with a minimum of three respiratory cultures per year were analyzed (n = 848). The median age of chronic infection with mucoid PA was 1.7 years earlier in females than in males. Patients infected with mucoid PA had poorer survival, chest X-ray scores, and pulmonary function tests than patients who had either no Pseudomonas species or only the nonmucoid phenotype. Acquisition of mucoid PA was associated with an accelerated rate of decline in pulmonary function. However, the rate of change of pulmonary function after mucoid PA infection was similar for males and females. Moreover, even among patients who had only the mucoid form or only the nonmucoid form, males had better percent predicted forced expiratory volume in 1 sec and better survival. Therefore, factors in addition to earlier acquisition of mucoid PA may contribute to the poorer survival of female CF patients.