Τίτλος:
Survival in a large cohort of Greek patients with transfusion-dependent beta thalassaemia and mortality ratios compared to the general population
Γλώσσες Τεκμηρίου:
Αγγλικά
Περίληψη:
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. Design and 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. © 2011 John Wiley & Sons A/S.
Συγγραφείς:
Ladis, V.
Chouliaras, G.
Berdoukas, V.
Chatziliami, A.
Fragodimitri, C.
Karabatsos, F.
Youssef, J.
Kattamis, A.
Karagiorga-Lagana, M.
Περιοδικό:
European Journal of Haematology
Λέξεις-κλειδιά:
deferasirox; deferiprone; deferoxamine, accident; adult; age distribution; aged; article; beta thalassemia; birth; controlled study; disease free survival; female; Greece; heart death; heart disease; human; Human immunodeficiency virus infection; liver disease; major clinical study; male; mortality; overall survival; priority journal; prospective study; risk assessment; septicemia; sex difference; stem cell transplantation; survival; thromboembolism; transfusion dependent beta thalassemia, Adolescent; Adult; beta-Thalassemia; Blood Transfusion; Cause of Death; Child; Child, Preschool; Cohort Studies; Female; Greece; Humans; Infant; Infant, Newborn; Kaplan-Meier Estimate; Male; Middle Aged; Prospective Studies; Young Adult
DOI:
10.1111/j.1600-0609.2011.01582.x
