Department of Pediatrics, Division of Pediatric Neurology, Antalya Training and Research Hospital, 07059, Antalya, Turkey.
Department of Pediatrics, Division of Pediatric Nephrology, Antalya Training and Research Hospital, 07059, Antalya, Turkey.
Pediatr Nephrol. 2020 Oct;35(10):1953-1958. doi: 10.1007/s00467-020-04587-3. Epub 2020 May 23.
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.
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.
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).
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.
肾功能障碍是杜兴氏肌营养不良症（DMD）成年患者的常见并发症；然而，儿科患者的肾功能很少受到关注。
回顾性分析了随访时间≥ 12 个月的 DMD 患者的病历。纳入标准为：（i）年龄 5-18 岁，（ii）肌营养不良蛋白基因突变证实，（iii）无肾脏和尿路结构异常。血清肌酸激酶（CK）用作肌肉破坏的间接标志物。
共纳入 44 例患者（平均年龄 10.9 ± 3.3 岁）。28 例患者行 24 小时动态血压监测评估血压。9 例（32.1%）发现高血压，其中 8 例正在使用类固醇。24 小时尿液采集发现 3 例（8.3%）存在轻度蛋白尿，5 例（13.9%）高钙尿症，7 例（19.7%）低钙尿症和 6 例（16.7%）高磷尿症。21 例（58.3%）存在高尿酸尿症，其中 4 例伴有高尿酸血症。对数胱抑素 C（CysC）与肌酐（Cr）（p = 0.001，r = 0.54）、CK（p = 0.048，r = 0.30）和甲状旁腺激素（PTH）（p = 0.001，r = 0.49）呈正相关。此外，根据 CysC 中位数将患者分为两组：组 1（n = 20，CysC ≤ 0.76 mg/L）和组 2（n = 24，CysC > 0.76 mg/L）。与组 1 相比，组 2 的平均 CK、PTH 和 Cr 水平显著升高（p = 0.010、0.033 和 0.023）。
长期暴露于受损肌肉释放的细胞内成分的过度负荷可能随着时间的推移增加儿科 DMD 患者慢性肾脏损伤的风险。
