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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) usually refers to the onset within 3 months of onset and its clinical manifestation is consistent with nephrotic syndrome (NS) (mass proteinuria, hypoalbuminemia, severe edema and hypercholesterolemia). According to the etiology can be divided into primary (genetic) and secondary (non-genetic), primary due to glomerular filtration barrier component or podocyte development required for regulatory factor related mutations; secondary Sex due to a variety of pathogens intrauterine infection or maternal disease caused. Clinical manifestations of primary CNS hormone-resistant NS, hormone or immunosuppressive therapy ineffective, renal transplantation is the only effective treatment.