Kawasaki Yukihiko, Suzuki Sigeo, Matsumoto Ayumi, Takano Kei, Suyama Kazuhide, Hashimoto Koichi, Suzuki Junzo, Suzuki Hitoshi, Hosoya Mitsuaki
Department of Pediatrics, Fukushima Medical University School of Medicine, 1 Hikariga-oka, Fukushima City, Fukushima 960-1295, Japan.
Pediatr Nephrol. 2007 Jun;22(6):889-92. doi: 10.1007/s00467-006-0426-6. Epub 2007 Feb 3.
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.
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。