Clinical Outcomes for Cystinuria Patients with Unilateral Versus Bilateral Cystine Stone Disease.
Clinical Outcomes for Cystinuria Patients with Unilateral Versus Bilateral Cystine Stone Disease.
复制标题
患有单侧与双侧胱氨酸结石病的胱氨酸尿症患者的临床结果。
DOI：
10.1089/end.2017.0335
复制
发表时间：
2018
影响因子：
2.7
通讯作者：
Chi,Thomas
中科院分区：
文献类型：
--
作者：
Usawachintachit,Manint;Sherer,Benjamin;Hudnall,Matthew;Tzou,DavidT;Taguchi,Kazumi;Hsi,RyanS;Stoller,Marshall;Chi,Thomas
介绍：胱氨酸尿症是一种遗传性疾病，以尿胱氨酸排泄量增加和复发性胱氨酸肾结石为特征。有趣的是，尽管对侧肾脏解剖结构看似相似，但一部分胱氨酸尿患者始终仅在一个肾脏中形成结石。本研究的目的是评估单侧vsbilateral胱氨酸结石former.Patients和方法的临床结果：我们进行了一项回顾性病例对照研究，在加州大学旧金山弗朗西斯科1994年和2015年之间评估和治疗的胱氨酸尿患者，并将患者分类为单侧或双侧结石形成者。临床表现，基线患者人口统计学，结石程序，药物治疗方案，和长期肾功能进行了比较between two group.Results：共42胱氨酸结石患者（22名女性，20名男性）被纳入分析。首次就诊时的中位年龄为18.5岁，研究结束时的中位年龄为45.5岁。三分之二的患者（n= 28）有双侧结石病史，而三分之一（n= 14）有单侧结石。两组的药物治疗方案相似。尽管一生中平均手术次数增加（双侧7.5次vs单侧3.7次，P< 0.05），与单侧结石患者相比，最近肾小球滤过率中位数无显著差异（分别为81.5vs95 mL/min;p= 0.28）。我们队列中的大多数胱氨酸尿患者在其一生中形成双侧结石，并且需要比单侧结石形成者更多的外科手术干预。尽管如此，在接受微创取石术治疗的单侧和双侧胱氨酸尿性结石患者中，总体肾功能保持良好。
Introduction:Cystinuria is a genetic disorder marked by elevated urinary cystine excretion and recurrent cystine nephrolithiasis. Interestingly, despite seemingly similar contralateral renal anatomy, a subset of cystinuric patients consistently form stones in only one kidney. The aim of this study is to evaluate clinical outcomes in unilateralvsbilateral cystine stone formers.Patients and Methods:We performed a retrospective case–control study of cystinuric patients evaluated and treated at the University of California, San Francisco between 1994 and 2015 and categorized patients as either unilateral or bilateral stone formers. Clinical presentation, baseline patient demographics, stone procedures, medical therapy regimens, and long-term renal function were compared between the two groups.Results:A total of 42 cystine stone patients (22 female, 20 male) were included in the analysis. The median age at first presentation was 18.5 years and median age at study conclusion was 45.5 years. Two-thirds of patients (n= 28) had a history of bilateral stones, whereas one-third (n= 14) had unilateral stones. Medical therapy regimens were similar between groups. Despite an increased average number of lifetime surgeries (7.5 sessions for bilateralvs3.7 sessions for unilateral,p< 0.05), there was no significant difference in medians of the most recent glomerular filtration rate when compared with unilateral stone formers (81.5vs95 mL/min, respectively;p= 0.28).Conclusions:The majority of cystinuric patients within our cohort form stones bilaterally during their lifetime, and require more surgical interventions than unilateral stone formers. Despite this, overall renal function is well preserved in unilateral and bilateral cystinuric stone formers treated with minimally invasive stone extraction procedures.