Background

Typical hemolytic uremic syndrome (HUS) causes acute kidney injury (AKI) and serious sequelae of chronic kidney disease (CKD) in some. Hyperuricemia is a common finding in typical HUS that may contribute to kidney damage. We explored whether aggressive management of hyperuricemia with rasburicase could improve outcomes in AKI patients with typical HUS.

Methods

We retrospectively analyzed medical records of children with typical HUS admitted to a tertiary center between 2005 and 2017. We compared clinical outcomes of hospitalization and 1-year post-discharge between those with rasburicase treatment (n = 13) and those without (controls, n = 29).

Results

With rasburicase treatment, hyperuricemia corrected more rapidly (median 36 vs. 120 h, p < 0.001), and hospital stays were shorter (median 9 vs. 12 days, p = 0.003) than in the controls. There was no difference in dialysis requirement. At 1-year post-discharge, the proportion of patients with impaired kidney function (estimated glomerular filtration rate < 90 mL/min/1.73 m²) was lower in the rasburicase group (7.7% vs. 41.4%, p = 0.036) than in the controls. Hypertension and proteinuria tended to be more common in the controls than in the rasburicase group. Collectively, long-term renal sequelae of impaired kidney function, proteinuria, or hypertension at a 1-year follow-up was less common in the rasburicase group than in the controls (7.7% vs. 62.1%; p = 0.001).

Conclusions

Children with typical HUS treated with rasburicase had shorter hospital stays and less long-term sequelae at 1-year post-discharge than those who were not treated with rasburicase. These results support the use of rasburicase to prevent CKD in pediatric patients with typical HUS-associated AKI.

中文翻译：

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Rasburicase可改善典型溶血性尿毒症综合征引起的急性肾损伤的预后。

背景

典型的溶血性尿毒症综合征（HUS）在某些情况下会导致急性肾损伤（AKI）和慢性肾脏病（CKD）的严重后遗症。高尿酸血症是典型HUS的常见发现，可能导致肾脏损害。我们探讨了使用rasburicase积极治疗高尿酸血症可改善患有典型HUS的AKI患者的预后。

方法

我们回顾性分析了2005年至2017年间收治于三级中心的典型HUS患儿的病历。我们比较了使用过糖酶治疗（n = 13）和未进行过（对照，n）的住院和出院后1年的临床结局。= 29）。

结果

葡萄干酶治疗后，高尿酸血症的纠正速度更快（中位36 vs. 120 h，p <0.001），住院时间短于对照组（中位9 vs. 12天，p = 0.003）。透析要求无差异。出院后1年，rasburicase组肾功能受损（肾小球滤过率<90 mL / min / 1.73 m ²）的患者比例较低（7.7％vs. 41.4％，p= 0.036）。对照组中的高血压和蛋白尿症比rasburicase组更常见。总的来说，在1年随访中，长期使用肾后遗症的肾脏功能受损，蛋白尿或高血压的发生率低于对照组（7.7％vs. 62.1％；p = 0.001）。

结论

与未接受过葡萄糖苷酶治疗的儿童相比，接受过葡萄糖苷酶治疗的典型HUS儿童出院后1年住院时间短，长期后遗症少。这些结果支持使用rasburicase预防典型HUS相关AKI患儿的CKD。