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Copious Podocyturia without Proteinuria and with Normal Renal Function in a Young Adult with Fabry Disease

机译:年轻人患有法布里病的无蛋白尿且肾功能正常的足足足

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摘要

The time for starting a patient with Fabry disease on enzyme replacement therapy is still a matter of debate, particularly when no overt classical clinical signs or symptoms are present. With respect to Fabry nephropathy, a dual problem coexists: the reluctance of many nephrologists to start enzyme replacement infusion until signs of renal disease appear as the appearance of proteinuria or an elevation in serum creatinine and the lack of validated biomarkers of early renal damage. In this regard, proteinuria is nowadays considered as an early and appropriate marker of kidney disease and of cardiovascular morbidity and mortality. However, in this report we demonstrate that podocyturia antedates the classical appearance of proteinuria and could be considered as an even earlier biomarker of kidney damage. Podocyturia may be a novel indication for the initiation of therapy in Fabry disease.
机译:用酶替代疗法治疗法布里病患者的时间仍然是个争论的话题,尤其是在没有明显的经典临床症状或体征的情况下。对于法布里(Fabry)肾病,存在一个双重问题:许多肾脏科医生不愿开始酶替代输注,直到出现肾脏疾病的迹象出现为蛋白尿或血清肌酐升高以及缺乏经过验证的早期肾脏损害的生物标志物。在这方面,如今蛋白尿被认为是肾脏疾病以及心血管疾病发病率和死亡率的早期和适当的标志物。但是,在本报告中,我们证明了足癣早于蛋白尿的经典出现,并且可以被认为是肾脏损害的更早生物标记。足癣可能是法布里病开始治疗的新适应症。

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