Risk factors for mortality before age 18 years in cystic fibrosis
Risk factors for mortality before age 18 years in cystic fibrosis
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DOI：
10.1002/ppul.23715
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发表时间：
2017-07-01
影响因子：
3.1
通讯作者：
Konstan, Michael W.
中科院分区：
文献类型：
--
作者：
McColley, Susanna A.;Schechter, Michael S.;Konstan, Michael W.
BackgroundUnderstanding early-life risk factors for children death in cystic fibrosis（CF）is important for clinical care，including identifying of effective interventions.MethodsData from the Epidemiologic Study of Cystic Fibrosis（ESCF）collected 1994-2005 were linked with the Cystic Fibrosis Foundation Patient Registry（CFFPR）demographic and mortality data from 2013.入选标准为3-5岁时每年1次访视和6-8岁时1次FEV 1测量。人口统计学数据、营养参数、肺部体征和症状、微生物学和FEV 1被评估为18岁之前死亡的风险因素。多变量考克斯比例风险回归模型的同时影响的危险因素与死亡年龄18 years.ResultsAmong 5365例患者参加ESCF谁符合纳入标准，3880（72%）与CFFPR。其中，191例（5.7%）在18岁之前死亡;死亡时的中位年龄为13.43.1岁。多变量回归显示杵状、爆裂音、女性、未知CFTR基因型、少数民族或种族、医疗补助保险（低社会经济地位的代表）、2种或2种以上培养物中的铜绿假单胞菌和年龄别体重
BackgroundUnderstanding early-life risk factors for childhood death in cystic fibrosis (CF) is important for clinical care, including the identification of effective interventions.MethodsData from the Epidemiologic Study of Cystic Fibrosis (ESCF) collected 1994-2005 were linked with the Cystic Fibrosis Foundation Patient Registry (CFFPR) demographic and mortality data from 2013. Inclusion criteria were 1 visit annually at age 3-5 years and 1 FEV1 measurement at age 6-8 years. Demographic data, nutritional parameters, pulmonary signs and symptoms, microbiology, and FEV1 were evaluated as risk factors for death before age 18 years. Multivariable Cox proportional hazards regression was used to model the simultaneous effects of risk factors associated with death before age 18 years.ResultsAmong 5365 patients enrolled in ESCF who met inclusion criteria, 3880 (72%) were linked to the CFFPR. Among these, 191 (5.7%) died before age 18 years; median age at death was 13.43.1 years. Multivariable regression showed clubbing, crackles, female sex, unknown CFTR genotype, minority race or ethnicity, Medicaid insurance (a proxy of low socioeconomic status), Pseudomonas aeruginosa on 2 or more cultures, and weight-for-age