一例杜兴氏肌肉萎缩症患者从长期缺血性肾损伤中恢复,肌酐水平正常。

IF 1 Q4 UROLOGY & NEPHROLOGY
CEN Case Reports Pub Date : 2024-10-01 Epub Date: 2024-03-04 DOI:10.1007/s13730-024-00858-2
Kensuke Daikoku, Hinako Kondo, Masataka Kudo, Akira Sugiura
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引用次数: 0

摘要

杜兴氏肌营养不良症(DMD)是一种遗传性疾病,其特点是骨骼肌进行性退化。随着呼吸设备和心力衰竭疗法的发展,患者的预期寿命延长,DMD 患者的肾功能障碍近来变得越来越明显。一名需要鼻饲管喂养的 23 岁 DMD 男性患者因 4 个月的肾功能障碍和贫血病史转诊至我院。患者的血清肌酸酐(sCr)水平在正常范围内(0.84 mg/dL),但其血清胱抑素 C 水平和按胱抑素 C 计算的估计肾小球滤过率(分别为 5.90 mg/L 和 7.5 mL/min/1.73 m2)显示其肾功能严重受损。尿检显示蛋白质和肾小管标志物水平升高。患者的血红蛋白和促红细胞生成素水平表明存在肾性贫血。低血压、下腔静脉塌陷和插管喂养不良表明,肾损伤是由肾缺血引起的,进而发展为肾小管间质肾损伤,这是一种内在性肾损伤。患者停用了血管紧张素转换酶抑制剂和β-受体阻滞剂,并输注了细胞外液。此后,患者的肾功能得到恢复。随后,患者的泌尿系统检查结果和贫血也得到了改善。虽然心脏保护药物的进步有望改善 DMD 患者的预后,但重要的是要考虑到因肾缺血导致肾损伤的患者人数可能会增加,而且由于骨骼肌质量下降,很难通过 sCr 水平评估 DMD 患者的肾功能。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
A case of Duchenne muscular dystrophy recovered from prolonged ischemic kidney injury which emerged with a normal creatinine level.

Duchenne muscular dystrophy (DMD) is an inherited disease characterized by progressive degeneration of the skeletal muscles. Renal dysfunction in patients with DMD has recently become more apparent as life expectancy has increased owing to advances in respiratory devices and heart failure therapies. A 23-year-old man with DMD who required nasal tube feeding was referred to our hospital with a 4-month history of renal dysfunction and anemia. The patient's serum creatinine (sCr) level was within the normal range (0.84 mg/dL), but his serum cystatin C level and estimated glomerular filtration rate calculated by cystatin C (5.90 mg/L and 7.5 mL/min/1.73 m2, respectively) indicated severe renal impairment. A urinalysis revealed elevated levels of protein and tubular markers. The patient's hemoglobin and erythropoietin levels indicated renal anemia. Hypotension, a collapsed inferior vena cava, and a poor tube feeding episode suggested that the kidney injury was due to renal ischemia, which progressed to tubulointerstitial kidney injury, an intrinsic kidney injury. The angiotensin-converting enzyme inhibitors and beta-blockers were discontinued, and extracellular fluid was infused. Thereafter, the patient's renal function recovered. Subsequently, the patient's urinary findings and anemia improved. Although advances in cardioprotective agents are expected to improve the prognosis of patients with DMD, it is important to consider that the number of patients with kidney injury due to renal ischemia may increase and that it is difficult to evaluate renal function using sCr level in patients with DMD because of decreased skeletal muscle mass.

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来源期刊
CEN Case Reports
CEN Case Reports UROLOGY & NEPHROLOGY-
CiteScore
1.90
自引率
0.00%
发文量
80
期刊介绍: Clinical and Experimental Nephrology (CEN) Case Reports is a peer-reviewed online-only journal, officially published biannually by the Japanese Society of Nephrology (JSN).  The journal publishes original case reports in nephrology and related areas.  The purpose of CEN Case Reports is to provide clinicians and researchers with a forum in which to disseminate their personal experience to a wide readership and to review interesting cases encountered by colleagues all over the world, from whom contributions are welcomed.
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