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[ 57 2009 6 14 ] P.M. 100500

. 7 13001305 13051505

3

13051335 1. Fabry 2 3.

13351405 4 CPAP 1 5PD 2 microglobulin High flux HD 6

14051435 7 Cr 7mg/dl RPGN2

8 9 ABO 14351505 10 11(SPP) 12

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15201540

. 15401640

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1 Fabry 45 1991 Fabry 2003 2008 11 Cr 2.1 mg/dl, 0.22 g/ -galA 28.9 pmol/mg /934±197 13.2 pmol/ml/114±43 GL-3(globotriasylceramide) 4.94g/mgCr0.08±0.06 Fabry Hemi zebra body Fabry -galA IgG

2 83 H20.8 40mg 12 2 CT ABPC/SBT CRP MRI, CT nocardia facinica MEPM

3. 1990 450 60 aVL ST aVF ST NTG 4

4 CPAP 1 CPAP 68 1978 2005 2007 AF 2 ODI 20.5 PSG OSAS CSAS CPAP CPAP

5. PD 2 microglobulin High flux HD PD 2 microglobulin (2M)(Yoshida H. Kidney Int. 2007) 2M HD PD HD HD1 PD1 2M High flux HD 1 PD 2 PD HD 1 3.3 PD 8.0 L/ 125 mL/ HD PD 2M HD 42.8 mg/L 2M 1 35.8 mg/L 2M HD1 245 mgPD1 48.4 mg HD1 PD 5 2M PD 2M 2M 1 HD 2M

6 HFCKDCIN HF 9 6 3 2 70.5±16 CAG 3 PCI 4 TAE 2 139±133mleGFR 12.75±5.36ml/min HF 4 18 Qb 100ml/QF1000ml/ 2 Cr 3 1/Cr Cr 4.11±2.79mg/dl 4.22±2.58mg/dl Cr 10 1 3 1/Cr 1 CKD HF CIN

7. Cr 7mg/dl RPGN2 74 2009 3 4 6 4+ 3+Cr 3.3mg/dl13 Cr 6.8mg/dl16 Cr 8.1mg/dl RPGN 20 StageMPO-ANCA 485EUPSL 40mg/day 5 6 Cr 3.6mg/dl 2 76 2008 1 2 14 Cr 0.8mg/dl3 19 Cr 7.5mg/dl PSL 20mg/day 22 MPO-ANCA 27 Stage PSL 40mg/day 5 2 Cr 1.3mg/dl Cr 2

8. 1994 4 2008 12 20 346 5.8% 15 5 55.2 257 221ml 4 clear cell carcinoma 15 granular cell carcinoma 2 spindle cell carcinoma 1 papillary renal cell carcinoma 1 chromophobe cell carcinoma 1

9 ABO 24 49.5 16 rituximab rituximab 1 3 375mg/m2 14 200mg/body CD19 rituximab 1 rituximab 200mg/body

10. 2 1 3 66 11 2 15 50 HCO3 11 (SPP) SPP SPP 70.7±19.7mmHg SPP ABI R0.41 SPP ABI SPP R0.52 SPP /DW R-0.27 SPP ABI SPP SPP 12

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