title: Survival in a large cohort of Greek patients with transfusion-dependent beta thalassaemia and mortality ratios compared to the general population.
abstract: BACKGROUND
  With transfusions and chelation therapy, the prognosis for transfusion-dependent beta thalassaemia has changed from being fatal in early childhood to a chronic disorder with prolonged survival.
  
  
  METHODS
  In this historical prospective study, we present survival, causes of death and mortality ratios compared to the general population in 1044 Greek patients with transfusion-dependent beta thalassaemia.
  
  
  RESULTS
  At the age of 50years, the overall survival was 65.0%, while the cardiac death-free survival was 77%. Birth cohort had a significant effect on survival (P<0.001) with a negative trend towards past decades. The standardised mortality ratio (standardised for sex and ages 20-40years) compared to the general population improved significantly from 28.9 in 1990-1999 to 13.5 in 2000-2008, while the standardised cardiac mortality ratio reduced from 322.9 to 106.6, respectively.
  
  
  CONCLUSIONS
  Survival in thalassaemia has dramatically improved over the last twenty years but mortality remains significantly increased, compared to the general population.
authors.affiliation: Thalassaemia Unit, 1st Department of Paediatrics, University of Athens, Aghia Sophia Children's Hospital, Athens, Greece.
authors.forename: Vassilis
authors.initials: V
authors.lastname: Ladis
authors.forename: Giorgos
authors.initials: G
authors.lastname: Chouliaras
authors.forename: Vasilios
authors.initials: V
authors.lastname: Berdoukas
authors.forename: Antonia
authors.initials: A
authors.lastname: Chatziliami
authors.forename: Christina
authors.initials: C
authors.lastname: Fragodimitri
authors.forename: Fotis
authors.initials: F
authors.lastname: Karabatsos
authors.forename: Jacqueline
authors.initials: J
authors.lastname: Youssef
authors.forename: Antonis
authors.initials: A
authors.lastname: Kattamis
authors.forename: Markissia
authors.initials: M
authors.lastname: Karagiorga-Lagana
mesh_terms: D000293:Adolescent; D000328:Adult; D001803:Blood Transfusion; D002423:Cause of Death / Q000639:trends; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D006115:Greece / Q000453:epidemiology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D055815:Young Adult; D017086:beta-Thalassemia / Q000401:mortality* / Q000628:therapy