Wright Jeremy F, Craig Wendy Y, Lucas F L, Goldfarb David S, Zuckerman Jonathan B, Taylor Eric N
Division of Nephrology and Transplantation, Maine Medical Center, Tufts University School of Medicine, c/o Karen Kinne, 22 Bramhall Street, Portland, ME, 04102, USA.
Center for Outcomes Research and Evaluation, Maine Medical Center Research Institute, Tufts University School of Medicine, 509 Forest Avenue Suite 200, Portland, ME, 04102, USA.
Urolithiasis. 2021 Oct;49(5):415-423. doi: 10.1007/s00240-021-01244-8. Epub 2021 Feb 6.
Cystic fibrosis (CF) may predispose patients to urinary stone disease (USD), but reported prevalence of USD in patients with CF in previous small studies is variable. To date, analysis of risk factors for USD within the CF population has been limited. We studied 29,396 patients in the Cystic Fibrosis Foundation Patient Registry to calculate age and sex-stratified prevalence of USD. For adult patients, we examined age and multivariable-adjusted cross-sectional associations between demographic and clinical factors, CFTR mutation class, and prevalent USD. Prevalence of USD was 0.4% (95% CI 0.3-0.5%) under age 18 years, 3.1% (2.7-3.6%) at 18-24 years, 6.4% (5.8-7.1%) at 25-34 years, 7.5% (6.5-8.5%) at 35-44 years, and 6.7% (5.8-7.8%) at 45 years and older. Prevalence for women was higher than men at younger (< 45 years) but not older ages (P value for interaction < 0.0005). Multivariable odds of prevalent USD were significantly increased for severe CFTR mutations, OR 1.53 (1.14-2.06), diabetes, OR 1.24 (1.03-1.50), hypertension, OR 1.58 (1.29-1.93), and chronic macrolide therapy, OR 1.27 (1.07-1.52). BMI was not associated with USD. USD prevalence in CF is similar to that in the general population. With the exception of BMI, known risk factors for USD in the general population also appear to be important for patients with CF. We identified several novel associations in CF patients, including greater prevalence of USD in individuals with severe CFTR mutations and among young women.
囊性纤维化（CF）可能使患者易患尿路结石病（USD），但以往小型研究报道的CF患者中USD的患病率各不相同。迄今为止，对CF人群中USD危险因素的分析有限。我们在囊性纤维化基金会患者登记处研究了29396名患者，以计算按年龄和性别分层的USD患病率。对于成年患者，我们检查了人口统计学和临床因素、CFTR突变类别与现患USD之间的年龄和多变量调整横断面关联。18岁以下人群中USD的患病率为0.4%（95%CI 0.3 - 0.5%），18 - 24岁为3.1%（2.7 - 3.6%），25 - 34岁为6.4%（5.8 - 7.1%），35 - 44岁为7.5%（6.5 - 8.5%），45岁及以上为6.7%（5.8 - 7.8%）。在较年轻（<45岁）时，女性的患病率高于男性，但在年龄较大时并非如此（交互作用P值<0.0005）。严重CFTR突变使现患USD的多变量比值显著增加，OR为1.53（1.14 - 2.06），糖尿病为OR 1.24（1.03 - 1.50），高血压为OR 1.58（1.29 - 1.93），长期大环内酯类药物治疗为OR 1.27（1.07 - 1.52）。BMI与USD无关。CF患者中USD的患病率与普通人群相似。除BMI外，普通人群中已知的USD危险因素似乎对CF患者也很重要。我们在CF患者中发现了几种新的关联，包括严重CFTR突变个体和年轻女性中USD患病率更高。
