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低密度脂蛋白分离术治疗局灶节段性肾小球硬化的长期疗效

Long-term efficacy of low-density lipoprotein apheresis for focal and segmental glomerulosclerosis.

作者信息

Kawasaki Yukihiko, Suzuki Sigeo, Matsumoto Ayumi, Takano Kei, Suyama Kazuhide, Hashimoto Koichi, Suzuki Junzo, Suzuki Hitoshi, Hosoya Mitsuaki

机构信息

Department of Pediatrics, Fukushima Medical University School of Medicine, 1 Hikariga-oka, Fukushima City, Fukushima 960-1295, Japan.

出版信息

Pediatr Nephrol. 2007 Jun;22(6):889-92. doi: 10.1007/s00467-006-0426-6. Epub 2007 Feb 3.

Abstract

Recently, there have been reports on the efficacy of low-density lipoprotein (LDL) apheresis (LDL-A) for focal and segmental glomerulosclerosis (FSGS) in pediatric patients. However, there have been few reports on the long-term efficacy of LDL-A for FSGS in such patients. We report here a case of long-term efficacy of LDL-A for FSGS. The patient was a 13-year-old boy with FSGS who presented with steroid-resistant and cyclosporine-resistant nephrotic syndrome and hyperlipidemia. LDL-A was performed 24 times on one year. Following LDL-A, serum concentrations of LDL, very low-density lipoprotein (VLDL), apoprotein B, and vascular endothelial growth factor significantly decreased, and urinary excretion of protein also decreased. In addition, 3 years after LDL-A, the pathology findings on a second renal biopsy had improved. The patient has been in remission from FSGS for 12 years since LDL-A. These findings suggest that LDL-A may be useful in maintaining long-term remission from pediatric FSGS.

摘要

最近,有关于低密度脂蛋白(LDL)单采术(LDL-A)对小儿局灶节段性肾小球硬化症(FSGS)疗效的报道。然而,关于LDL-A对此类患者FSGS长期疗效的报道却很少。我们在此报告1例LDL-A对FSGS长期疗效的病例。该患者是一名13岁患FSGS的男孩,表现为激素抵抗和环孢素抵抗的肾病综合征及高脂血症。一年内进行了24次LDL-A治疗。LDL-A治疗后,血清LDL、极低密度脂蛋白(VLDL)、载脂蛋白B和血管内皮生长因子浓度显著降低,尿蛋白排泄也减少。此外,LDL-A治疗3年后,第二次肾活检的病理结果有所改善。自LDL-A治疗后,该患者FSGS已缓解12年。这些发现表明,LDL-A可能有助于维持小儿FSGS的长期缓解。

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