先天性肾病综合征的治疗进展

Advances on Treatment of Congenital Nephrotic Syndrome

先天性肾病综合征(CNS)通常指出生3个月内发病,临床表现符合肾病综合征(NS)(大量蛋白尿、低清蛋白血症、严重水肿和高胆固醇血症)。根据病因可分为原发性(遗传性)和继发性(非遗传性),原发性因肾小球滤过屏障组成成分或足细胞发育所需调节因子相关基因突变所致;继发性因多种病原体宫内感染或母亲疾病等导致。原发性CNS的临床多表现为激素耐药性NS,激素或免疫抑制剂治疗无效,肾移植是唯一有效的治疗方式。

Congenital nephrotic syndrome（CNS） is defined as heavy proteinuria,hypoalbuminemia and edema starting within 3 months after birth.According to the etiological factors,CNS can be classified to primary（hereditary） CNS and secondary（nonhereditary） CNS.Primary CNS is caused by genetic defects in the components of the glomerular filtration barrier or in a more generalized syndrome.Secondary CNS is caused by perinatal infections or maternal systemic diseases.The clinical manifestations of primary CNS are mainly steroid-resistant,corticosteroids or immunosuppressive medication is usually not helpful and kidney transplantation is the only curative therapy.