title: Cystic fibrosis mortality trend in Italy from 1970 to 2011.
abstract: BACKGROUND
  Survival in cystic fibrosis (CF) has progressively improved and the female-gender disadvantage first described many years ago remains controversial.
  
  
  OBJECTIVE
  To describe the mortality trend due to CF in Italy over the last decades; to verify the female-mortality disadvantage; to compare the comorbidities reported in death certificates of CF patients with those of the general population.
  
  
  METHODS
  Mortality data were extracted from the database of underlying cause of death (1970-2011) and multiple causes of death (2003-2011) of the Italian National Institute of Statistics. Age-standardized mortality ratio (SMR) was calculated to compare the mortality between genders. The association between CF and other contributing causes of death was verified by calculating the age- and gender-adjusted proportional mortality ratio (PMR).
  
  
  RESULTS
  During the study period, 1947 death certificates reported CF as the underlying cause of death. Mortality rate due to CF decreased in newborns and children and by the end of the 1990s also in adolescents and young adults. Adult mortality started to increase in the early 1990s. Over the whole period an excess in mortality was observed in young CF females (1-29 years). The multiple causes of death database included 531 certificates with CF listed as cause of death. Pneumonia, chronic lower respiratory diseases, pulmonary heart disease and diseases of pulmonary circulation, aspergillosis, sepsis, renal failure, diabetes, malnutrition and amyloidosis were more frequently reported in CF death certificates compared to those of the general population (PMR>1).
  
  
  CONCLUSIONS
  This mortality trend provides evidence of a consistent improvement in survival, although the excess female-mortality persists despite aggressive treatment of CF lung disease. Several extra-pulmonary conditions associated with CF contributed to the morbidity leading to death.
authors.affiliation: National Institute of Statistics (ISTAT), Rome, Italy; Cystic Fibrosis Center, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, University of Milan, Italy.
authors.forename: Gianfranco
authors.initials: G
authors.lastname: Alicandro
authors.affiliation: National Institute of Statistics (ISTAT), Rome, Italy.
authors.forename: Luisa
authors.initials: L
authors.lastname: Frova
authors.affiliation: National Institute of Statistics (ISTAT), Rome, Italy.
authors.forename: Gennaro
authors.initials: G
authors.lastname: Di Fraia
authors.affiliation: Cystic Fibrosis Center, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, University of Milan, Italy. Electronic address: carla.colombo@unimi.it.
authors.forename: Carla
authors.initials: C
authors.lastname: Colombo
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D002423:Cause of Death / Q000639:trends*; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000401:mortality*; D005260:Female; D006801:Humans; D007223:Infant; D007558:Italy / Q000453:epidemiology; D008297:Male; D009026:Mortality / Q000639:trends; D017678:Sex Distribution; D016019:Survival Analysis
keywords: Causes of deaths; Cystic fibrosis; Death certificates; Gender gap; Mortality