[Adult-onset mucoviscidosis: longer survival of patients in Moscow and Moscow Region].
OBJECTIVE
To estimate survival median and its changes, number of patients over 18 years of age for 1991-2000 and 2001-2010 for 20-year period (1991-2010), to elucidate factors affecting survival for 2001-2010 in mucoviscidosis children living in Moscow and Moscow Region and treated outpatiently in specialized medical centers.


METHODS
Case records were analysed for mucoviscidosis patients registered in specialized clinics of Moscow on 01.01.01 and 01.01.11. Survival was assessed with Kaplan-Meier curve.


RESULTS
Survival medians for 1991-2000 and 2001-2010 was 25.7 and 35.1 years, respectively In the group of mucoviscidosis patients with Staphylococcus aureus infection survival was significantly higher than in those infected with gram-negative microflora. Longer survival was reported in patients with "soft" mutation (p = 0.06927).


CONCLUSIONS
The survival median for mucoviscidosis patients for 2001-2010 was 35.1 years. The percentage of adult patients in the last decade significantly rose from 19.5 to 32%. Gram-negative microflora significantly reduces survival, while 'soft" mutation prolongs survival. Creation of National Register will specify survival of mucoviscidosis patients in all regions of the Russian Federation.
D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D015331:Cohort Studies; D003550:Cystic Fibrosis / Q000235:genetics / Q000382:microbiology / Q000401:mortality; D005260:Female; D005820:Genetic Testing; D016678:Genome; D006090:Gram-Negative Bacteria / Q000302:isolation & purification*; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D009031:Moscow / Q000453:epidemiology; D009154:Mutation / Q000235:genetics; D013211:Staphylococcus aureus / Q000302:isolation & purification*; D016019:Survival Analysis; D055815:Young Adult