改革开放30余年来，经几代肾脏病学者的艰苦努力和攀登，我国肾脏病事业取得了飞速发展，对常见的肾脏疾病、特别是肾小球疾病的诊治取得了长足的进步，某些研究领域己逐步与国际接轨。但基于我国肾脏病研究基础薄弱、地广人多，发展很不平衡，因此提升我国少见肾脏病的诊治水平尚有很大空间，加强少见肾脏病的临床和基础研究仍任重而道远。本文就如何提高我国少见肾脏病诊治水平提出几点看法：(1)认真做好肾活检病理检查，重视免疫病理和电镜在病理诊断中的作用。强调制片、染色质量，光镜、免疫荧光和电镜检查不应缺失任何一项，互为补充进行综合判断。提倡临床病理讨论会，病理学家与临床专家互动，是提高少见肾脏病诊断率和正确性的有效方法。(2)重视临床资料收集和分析，结合基因检测，提高少见肾脏病的诊断率，防止误诊、漏诊。重视病史(包括家族史)和临床资料(查体、实验室及相关医学影像检查结果)收集，并加以认真分析；在此基础上应努力开展与疾病相关的特殊检查，对于疑似遗传性肾病患者应做遗传基因检测，避免把少见的继发性肾脏病和遗传性疾病漏诊或误诊为原发性肾脏病。(3)对危害性大的少见肾脏病积极开展流行病学调查和发病机制的研究，以期深入阐明其发病机制。(4)组织好高水平临床治疗研究，以期获得高质量循证医学证据，指导和提高我国整体肾脏病的临床治疗水平。(5)强调依据患者具体病情制定个体化治疗方案和充分发挥中医中药优势，努力提高我国少见肾脏病的疗效。

Since the reform and opening up 3 decades ago, China′s nephrology has made great achivements after hard work of several generations of Chinese nephrologists; in the diagnosis and treatment for common kidney diseases, especially glomerular diseases, striking progress has been made; and China′s nephrology in some research fields has geared to the international conventions. But as the study foundation of kidney diseases in China is weak, with widespread population and very uneven development, there is still much space for improving China′s diagnosis and treatment of rare kidney diseases, and there is still a long way to go to enhance clinical and basic researches on rare kidney diseases. To achieve this goal, we put forward the following opinions: 1. Standardization of renal biopsy pathologic examination, paying attention to the roles of the immune pathology, and electron microscopy in pathological diagnosis. We emphasize on high quality production and dyeing of pathological specimen; and light microscopy, immunofluorescence, and electron microscopy are all indispensable and mutually complementary. We advocate clinical pathological conference to promote interactions between pathologists and clinical experts. 2. Attaching great importance to the collection and analysis of clinical data, combining genetic screening to effectively improve the diagnosis of rare kidney diseases, and prevent misdiagnosis and failure to diagnose. We emphasize on complete collection of illness history (including familial history) and clinical data (including physical, laboratory, and related medical image examinations) for serious analysis; on the basis of the above, disease-relevant special examination and gene detection for patients with suspected inherited kidney diseases should be performed, in order to avoid failure to diagnose rare secondary renal diseases and genetic diseases or misdiagnosing them as primary kidney diseases. 3. We also advocate active epidemiological investigation and the pathogenesis researches on very harmful rare kidney diseases so as to elucidate the pathogenesis. 4. We should organize high quality clinical therapeutic trials to obtain high quality evidence-based medical data for the purpose of providing guidance and improving the treatment of rare kidney diseases in China. 5. We emphasize individualized treatment and giving full play to the advantages of traditional Chinese medicine for improving the efficacy of rare kidney diseases.