Determinants of mortality from cystic fibrosis in Canada, 1970-1989
Determinants of mortality from cystic fibrosis in Canada, 1970-1989
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DOI：
10.1093/oxfordjournals.aje.a008664
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发表时间：
1996-05-15
影响因子：
5
通讯作者：
Farewell, V
中科院分区：
文献类型：
--
作者：
Corey, M;Farewell, V
对1970年-在加拿大患者数据登记处记录的3,795名患者的囊性纤维化的频率、患病率和死亡模式进行了分析。1970-1979年出生队列中囊性纤维化的频率几乎与通常引用的2,500人中有1人相同。1985年-，男性和女性的中位生存年龄分别为36.7岁和27.8岁，而1970-1974年分别为26.6岁和19.7岁。然而，当加拿大被划分为魁北克东部、魁北克、安大略省和西部四个地区时，存在显著的地区差异。患者确诊时较年轻，直到最近死亡率高于其他地区的患者，这意味着更严重的疾病；20世纪80年代存活率的显著提高恰逢从限制脂肪饮食转变为高脂肪饮食，20世纪70年代安大略省存活率的提高伴随着饮食疗法的变化，这也可能解释了整个研究期间东部地区的良好存活率。西方国家的存活率逐渐提高，与世界其他地区的报道相似，比例风险分析显示肺功能是最好的生存预测因子，女性生存不良与体重下降有关，但肺功能下降与体重维持、性别和死亡率的关系有待进一步研究，铜绿假单胞菌对肺定植的影响与肺功能障碍的程度相混淆，但洋葱伯克霍尔德氏菌(以前称为洋葱假单胞菌)定植与各级肺功能死亡率的增加有关。
The frequency, prevalence, and mortality patterns of cystic fibrosis were analyzed in 3,795 patients documented in the Canadian Patient Data Registry in 1970-1989, Cystic fibrosis frequency in the 1970-1979 birth cohort was virtually identical to the commonly quoted 1 in 2,500, In 1985-1989, median survival age was 36.7 years for males and 27.8 years for females, compared with 26.6 and 19.7, respectively, in 1970-1974, However, there were significant regional differences when Canada was divided into the four regions, East, Quebec, Ontario, and West, In Quebec, patients were younger at diagnosis and until recently had greater mortality than patients in other regions, which suggests more severe disease; dramatically improved survival in the 1980s coincided with a change from a restricted fat diet to a high fat diet, Improved survival in Ontario in the 1970s accompanied this change in dietary therapy, which may also account for good survival throughout the study period in the East. The West showed gradually improving survival, similar to that reported in other parts of the world, Proportional hazards analysis showed pulmonary function to be the best predictor of survival, Poorer survival in females was associated with poorer weight, but the interrelation of declining pulmonary function, weight maintenance, sex, and mortality requires further study, The effect of pulmonary colonization with Pseudomonas aeruginosa was confounded with degree of pulmonary dysfunction, but colonization with Burkholderia cepacia (previously Pseudomonas cepacia) was associated with increased mortality at all levels of pulmonary function.