BACKGROUND Kidney dysfunction is a common complication in adults with Duchenne muscular dystrophy (DMD); however, little attention has been paid to kidney function in pediatric patients. METHODS Medical records of patients with DMD who were followed up for ≥ 12 months were retrospectively reviewed. Inclusion criteria were (i) aged 5-18 years, (ii) proven mutations in the dystrophin gene, and (iii) absence of structural anomalies of the kidney and urinary tract. Serum creatine kinase (CK) was used as an indirect marker of muscle destruction. RESULTS Forty-four patients (mean age, 10.9 ± 3.3 years) were included. Blood pressure was evaluated by 24-h ambulatory blood pressure monitoring in 28 patients. Hypertension was found in 9 (32.1%), eight of whom were using steroids. Mild proteinuria, hypercalciuria, hypocalciuria, and hyperphosphaturia in 24-h urine collection (n = 36) were detected in 3 (8.3%), 5 (13.9%), 7 (19.7%), and 6 (16.7%) patients, respectively. Twenty-one (58.3%) demonstrated hyperuricosuria, associated with hyperuricemia in 4. Logarithmic cystatin C (CysC) had a positive correlation to creatinine (Cr) (p = 0.001, r = 0.54), CK (p = 0.048, r = 0.30), and parathormone (PTH) (p = 0.001, r = 0.49). Moreover, the patients were divided into two groups according to median CysC value: group 1 (n = 20, CysC ≤ 0.76 mg/l) and group 2 (n = 24, CysC > 0.76 mg/l). Mean CK, PTH, and Cr levels were significantly elevated in group 2 compared with group 1 (p = 0.010, 0.033, and 0.023, respectively). CONCLUSIONS Long-term exposure to the excessive burden of intracellular components released from damaged muscles may be associated with an increased risk over time of chronic kidney impairment in pediatric DMD patients. Graphical abstract.

中文翻译：

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杜氏肌营养不良症儿童的肾脏受累和相关危险因素。

背景肾功能障碍是成人杜氏肌营养不良症 (DMD) 的常见并发症。然而，很少有人关注儿科患者的肾功能。方法回顾性回顾随访≥12个月的DMD患者的病历。入选标准是 (i) 5-18 岁，(ii) 经证实的抗肌萎缩蛋白基因突变，以及 (iii) 没有肾脏和泌尿道结构异常。血清肌酸激酶 (CK) 被用作肌肉破坏的间接标志物。结果 包括 44 名患者（平均年龄，10.9 ± 3.3 岁）。28 名患者通过 24 小时动态血压监测评估血压。9 人 (32.1%) 发现高血压，其中 8 人使用类固醇。轻度蛋白尿、高钙尿、低钙尿、和 24 小时尿液收集中的高磷酸盐尿 (n = 36) 分别在 3 (8.3%)、5 (13.9%)、7 (19.7%) 和 6 (16.7%) 名患者中检测到。21 人 (58.3%) 表现出高尿酸尿症，4 人与高尿酸血症相关。对数胱抑素 C (CysC) 与肌酐 (Cr) (p = 0.001, r = 0.54)、CK (p = 0.048, r = 0.30) 呈正相关) 和甲状旁腺激素 (PTH)（p = 0.001，r = 0.49）。此外，根据 CysC 中值将患者分为两组：第 1 组（n = 20，CysC ≤ 0.76 mg/l）和第 2 组（n = 24，CysC > 0.76 mg/l）。与第 1 组相比，第 2 组的平均 CK、PTH 和 Cr 水平显着升高（分别为 p = 0.010、0.033 和 0.023）。结论 长期暴露于受损肌肉释放的细胞内成分的过度负担可能与儿科 DMD 患者慢性肾功能损害的风险随时间增加有关。图形概要。