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首页> 外文期刊>Clinics >Familial steroid-sensitive idiopathic nephrotic syndrome: seven cases from three families in China
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Familial steroid-sensitive idiopathic nephrotic syndrome: seven cases from three families in China

机译：家族性类固醇敏感性特发性肾病综合征：来自中国三个家庭的七例

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OBJECTIVES: Familial steroid-sensitive idiopathic nephrotic syndrome is rare, and only approximately 3% of patients have affected siblings. METHODS: Herein, we report seven cases of patients with steroid-sensitive idiopathic nephrotic syndrome from three Chinese families. Mutational screening of the Nphs2 gene was performed in all the patients. RESULTS: All seven of the familial steroid-sensitive idiopathic nephrotic syndrome cases in our sample exhibited minimal change disease, and one case also presented with mesangial proliferative glomerulonephritis, according to the renal pathology. No significant was associations were found between Nphs2 gene mutations and the onset of proteinuria and nephrotic syndrome in these familial cases. CONCLUSIONS: The presence of minimal change disease is important, but it is not an unusual finding in patients with familial steroid-sensitive idiopathic nephrotic syndrome, which appears to be clinically benign and genetically distinct from other types of nephrosis.
机译：目的：家族性类固醇敏感性特发性肾病综合征很少见，只有大约3％的患者患有兄弟姐妹。方法：在本文中，我们报道了来自三个中国家庭的7例类固醇敏感性特发性肾病综合征患者。在所有患者中进行了Nphs2基因的突变筛选。结果：根据肾脏病理，我们样本中的所有7例家族性类固醇敏感性特发性肾病综合征病例均表现出微弱的病变，其中1例还伴有系膜增生性肾小球肾炎。在这些家族性病例中，Nphs2基因突变与蛋白尿和肾病综合征的发作之间没有相关性。结论：最小变化疾病的存在很重要，但对于家族性类固醇敏感的特发性肾病综合征患者而言，这并不是一个不寻常的发现，该患者在临床上似乎是良性的，并且在遗传上与其他类型的肾病有所不同。

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