難治性ネフローゼ症候群の治療アフェレシス療法
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概要
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We investigated the safety and efficacy of low-density lipoprotein adsorption (LDL-A) therapy alone and in combination with double-filtration plasmapheresis (DFPP) therapy in 13 patients with focal glomerular sclerosis (FGS) who were resistant to conventional therapy. Transdermal needle biopsies of the kidney were obtained from 6 men and 7 women with focal glomerular sclerosis. Patients then received LDL-A therapy alone (n=7) or in combination with DFPP (n=6). Levels of total cholesterol, triglycerides and low-density lipoprotein decreased significantly after treatment by approximately 50% in all patients. The glomerular filtration rate (GFR) increased from 56.5 ml/min to 69.8 ml/min, and the daily urinary protein excretion (Up/ day) decreased from 6.1 g/day to 3.8g/day. Significantly more patients treated with the DFPP combined therapy showed an improvement in GFR of greater than 30% (p<0.05). In the subgroup of patients in whom Up/day fell below 3.5 g/day (n=6), the mean age (28.7 years) was significantly lower than that of those patients whose Up/day did not fall below 3.5 g/day (n=7,46.2 years), but there were no other significant differences in patient characteristics. Light microscopy of histological specimens showed no changes after LDL-A therapy, but electron microscopy showed the disappearance of severe fusion of foot processes and improvement in subendothelial sprending. Our results suggest that LDL-A therapy is recommended for patients with FGS who have an obstinate steroid-resistant nephrotic syndrome, particularly those with early onset disease. In cases with elevated urinary protein excretion, combination therapy with DFPP and LDL-A should be considered.
- 日本アフェレシス学会の論文
- 1998-05-31
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- 難治性ネフローゼ症候群の治療アフェレシス療法