Definition, diagnosis and clinical management of non-obstructive kidney dysplasia: a consensus statement by the ERKNet Working Group on Kidney Malformations.
Definition, diagnosis and clinical management of non-obstructive kidney dysplasia: a consensus statement by the ERKNet Working Group on Kidney Malformations.
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DOI：
10.1093/ndt/gfac207
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发表时间：
2022-11-23
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--
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肾发育不良是儿童慢性肾衰竭的最常见原因之一。虽然发育不良是一种组织学诊断，但“肾发育不良”一词在日常临床生活中经常使用，但未经组织病理学证实。肾发育不良的临床参数尚未明确定义，导致医疗保健专业人员和患者之间的沟通不准确。这种缺乏共识阻碍了对疾病的精确理解和特定疗法的开发。基于结构化文献检索，我们在此提出与功能性肾损伤相关的非梗阻性肾发育不良的临床、影像、遗传、病理和基础科学方面的共同基础。我们建议接受标志性超声检查结果作为定义发育不良肾脏临床诊断的替代参数。我们建议对肾发育不良儿童采取差异化的临床随访计划，并总结非梗阻性肾发育不良的已确定的单基因原因。最后，我们指出并讨论了该领域的研究空白。
Kidney dysplasia is one of the most frequent causes of chronic kidney failure in children. While dysplasia is a histological diagnosis, the term ‘kidney dysplasia’ is frequently used in daily clinical life without histopathological confirmation. Clinical parameters of kidney dysplasia have not been clearly defined, leading to imprecise communication amongst healthcare professionals and patients. This lack of consensus hampers precise disease understanding and the development of specific therapies. Based on a structured literature search, we here suggest a common basis for clinical, imaging, genetic, pathological and basic science aspects of non-obstructive kidney dysplasia associated with functional kidney impairment. We propose to accept hallmark sonographic findings as surrogate parameters defining a clinical diagnosis of dysplastic kidneys. We suggest differentiated clinical follow-up plans for children with kidney dysplasia and summarize established monogenic causes for non-obstructive kidney dysplasia. Finally, we point out and discuss research gaps in the field.
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