1型戈谢病患者的预期寿命。
Life expectancy in Gaucher disease type 1.
作者信息
Weinreb Neal J, Deegan Patrick, Kacena Katherine A, Mistry Pramod, Pastores Gregory M, Velentgas Priscilla, vom Dahl Stephan
机构信息
University Research Foundation for Lysosomal Storage Diseases, Northwest Oncology Hematology Associates PA, 8170 Royal Palm Boulevard, Coral Springs, Florida 33065, USA.
出版信息
Am J Hematol. 2008 Dec;83(12):896-900. doi: 10.1002/ajh.21305.
Abstract
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 approximately 9 y less than reference population. Malignancies did not contribute to shortened life expectancy.
摘要
我们采用标准生命表方法，将国际合作Gaucher病注册研究中纳入的1型Gaucher病（GD1）患者的生存数据与美国人群进行比较，从而估算了GD1患者的出生时预期寿命。2876例GD1患者在13509人年的随访中有102例报告死亡。出生时预期寿命估计为68岁，而参考人群为77岁；脾切除患者为64岁；未行脾切除患者为72岁。102例患者中有63例的死亡原因是恶性肿瘤（17/63）、心血管疾病（11/63）和脑血管疾病（8/63）。GD1患者出生时预期寿命比参考人群约少9岁。恶性肿瘤并非导致预期寿命缩短的原因。
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