Low-density lipoprotein apheresis in a pediatric patient with refractory nephrotic syndrome due to focal segmental glomerulosclerosis
Low-density lipoprotein apheresis in a pediatric patient with refractory nephrotic syndrome due to focal segmental glomerulosclerosis
复制标题
局灶节段性肾小球硬化所致难治性肾病综合征儿科患者的低密度脂蛋白单采术
DOI：
10.1007/s00540-008-0726-z
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发表时间：
2009
影响因子：
2.8
通讯作者：
M. Nishimura
中科院分区：
文献类型：
--
作者：
J. Oto;K. Suga;S. Matsuura;Shuji Kondo;Y. Ohnishi;D. Inui;H. Imanaka;S. Kagami;M. Nishimura
局灶节段性肾小球硬化（FSGS）常导致难治性肾病综合征（NS）。高水平的低密度脂蛋白（LDL）是NS进展的危险因素。一个8岁的女孩提出了严重的蛋白尿难治性类固醇治疗。她被诊断为非IgA弥漫性系膜增生性肾小球肾炎。口服强的松龙、甲基强的松龙（mPL）冲击治疗、环孢霉素和环磷酰胺治疗均未能达到缓解。随访肾活检显示FSGS。她的血清LDL水平很高，进行了5次LDL单采术（LDL-A），随后进行了mPL脉冲治疗。尿蛋白从2-4 g·d-降至0.5-1.0 g·d-。LDL-A在治疗多药耐药FSGS中可能是有益的。
Focal segmental glomerulosclerosis (FSGS) often leads to refractory nephrotic syndrome (NS). A high level of low-density lipoprotein (LDL) is a risk factor for the progression of NS. An 8-year-old girl presented with severe proteinuria refractory to steroid therapy. She was diagnosed with non-IgA diffuse mesangial proliferative glomerulonephritis. Oral prednisolone, methylprednisolone (mPL) pulse therapy, and cyclosporine and cyclophosphamide therapy failed to achieve remission. Follow-up renal biopsy revealed FSGS. Her serum level of LDL was high, and LDL-apheresis (LDL-A) was performed five times, followed by mPL pulse therapy. Urinary protein decreased from 2–4 g·day−to 0.5–1.0 g·day−. LDL-A may be beneficial in the treatment of multidrug-resistant FSGS.