Ouyang Lijing, Bolen Julie, Valdez Rodolfo, Joseph David, Baum Michelle A, Thibadeau Judy
Rare Disorders and Health Outcomes Team, National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, Bethesda, Maryland.
Rare Disorders and Health Outcomes Team, National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, Bethesda, Maryland.
J Urol. 2015 Feb;193(2):558-64. doi: 10.1016/j.juro.2014.08.092. Epub 2014 Aug 25.
We describe the characteristics, treatments and survival of patients with spina bifida in whom end stage renal disease developed from 2004 through 2008 in the United States Renal Data System.
We used ICD-9-CM code 741.* to identify individuals with spina bifida using hospital inpatient data from 1977 to 2010, and physician and facility claims from 2004 to 2008. We constructed a 5:1 comparison group of patients with end stage renal disease without spina bifida matched by age at first end stage renal disease service, gender and race/ethnicity. We assessed the risk of mortality and of renal transplantation while on dialysis using multivariate cause specific proportional hazards survival analysis. We also compared survival after the first renal transplant from the first end stage renal disease service to August 2011.
We identified 439 patients with end stage renal disease and spina bifida in whom end stage renal disease developed at an average younger age than in patients without spina bifida (41 vs 62 years, p <0.001) and in whom urological issues were the most common primary cause of end stage renal disease. Compared to patients with end stage renal disease without spina bifida those who had spina bifida showed a similar mortality hazard on dialysis and after transplantation. However, patients with end stage renal disease without spina bifida were more likely to undergo renal transplantation than patients with spina bifida (HR 1.51, 95% CI 1.13-2.03). Hospitalizations related to urinary tract infections were positively associated with the risk of death on dialysis in patients with end stage renal disease and spina bifida (HR 1.42, 95% CI 1.33-1.53).
Spina bifida was not associated with increased mortality in patients with end stage renal disease on dialysis or after renal transplantation. Proper urological and bladder management is imperative in patients with spina bifida, particularly in adults.
我们描述了2004年至2008年在美国肾脏数据系统中发展为终末期肾病的脊柱裂患者的特征、治疗方法和生存率。
我们使用ICD-9-CM编码741.*，利用1977年至2010年的医院住院数据以及2004年至2008年的医生和医疗机构索赔数据来识别脊柱裂患者。我们构建了一个5:1的对照组，即没有脊柱裂的终末期肾病患者，根据首次进入终末期肾病服务时的年龄、性别和种族/民族进行匹配。我们使用多变量特定病因比例风险生存分析评估透析期间的死亡风险和肾移植风险。我们还比较了从首次进入终末期肾病服务到2011年8月首次肾移植后的生存率。
我们识别出439例患有终末期肾病和脊柱裂的患者，这些患者发展为终末期肾病的平均年龄比没有脊柱裂的患者年轻（41岁对62岁，p<0.001），并且泌尿系统问题是终末期肾病最常见的主要原因。与没有脊柱裂的终末期肾病患者相比，患有脊柱裂的患者在透析和移植后的死亡风险相似。然而，没有脊柱裂的终末期肾病患者比患有脊柱裂的患者更有可能接受肾移植（风险比1.51，95%置信区间1.13 - 2.03）。与尿路感染相关的住院与患有终末期肾病和脊柱裂的患者透析期间的死亡风险呈正相关（风险比1.42，95%置信区间1.33 - 1.53）。
脊柱裂与透析或肾移植后的终末期肾病患者死亡率增加无关。对于脊柱裂患者，尤其是成年人，适当的泌尿系统和膀胱管理至关重要。
