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Life expectancy in Gaucher disease type 1
Author(s) -
Weinreb Neal J.,
Deegan Patrick,
Kacena Katherine A.,
Mistry Pramod,
Pastores Gregory M.,
Velentgas Priscilla,
vom Dahl Stephan
Publication year - 2008
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.21305
Subject(s) - life expectancy , medicine , population , disease , malignancy , pediatrics , environmental health
We estimated life expectancy at birth for Gaucher disease type 1 (GD1) patients by comparing survival data from GD1 patients enrolled in ICGG Gaucher Registry to the U.S. population using standard life table methods. 2,876 GD1 patients had 102 reported deaths in 13,509 person‐years of follow‐up. Estimated life expectancy at birth was 68 y, compared with 77 y in reference population; splenectomized patients, 64 y; nonsplenectomized, 72 y. Causes of death for 63/102 patients were malignancy (17/63), cardiovascular (11/63), and cerebrovascular (8/63). Estimated life expectancy at birth for GD1 patients was ∼9 y less than reference population. Malignancies did not contribute to shortened life expectancy. Am. J. Hematol., 2008. © 2008 Wiley‐Liss, Inc.