リンパ脈管筋腫症に対するシロリムスの 安全性確立の
Contents
1. CYP3A4 E 63 LAM2. 5 1 5 2 0mg d1 eGFR 30 50ml min 1 73m 1lmg 1mg 2 1mg d1 eGFR 29m1 min 1 73m CT
3. 2 B 10 i i iv
4. _ 24 PH X 25 2013 3 YRR EIEE LAM LAM 90 LAM LAM
5. Bh2e Erde C 2013 2 15 180 8 4
6. 7 2 CT CT active CYP3A4 CYP3A4
7. Acti 54812145 5 P3315 TREE X7 iX0BUD SEO FER DU HE BRAE D ffl poao 12 Visit 0 2 gt MI 4 2 15 Visit4 11 Visit0 Visit 1 58 PFT D
8. Multicenter Lymphangioleiomyomatosis Sirolimus Trial For Safety MLSTS MLSTS 1 5hR 24 9 19H 51 1 PFT FEM FVC 2004 MILES Pulmonary Function Test PFT AS 407
9. 4 12 8 12 12 3 Visit 4 Visit 11 2 iv 1 v 4 v FVC
10. MILES B SERE 1 2 3 4
11. vi 1 830 2240 d 5v 255839 110 6 1 1 57 3 PFT v 2 gt 200 240 gt 100 110 6 1 3 1 1 PFT PFT
12. lmg 3 Grade4 00 EM 2 8 2 4
13. FVC 61 PFT FVC 30 FVC 4 1 FEV1 FVC 3 4 3 3
14. 5 6 AS 407 2012 7 14 10 8 MLSTS 8 11 49 2012 1
15. PI3K Akt mTOR PI3K Akt mTOR LAM LAM
16. I II MILES LAM L AM LAM MILES A LAM mTOR
17. i ii 93 PFT i in i iv 10 4 LRM 1010 hPa sr UST E 1 ss 2 083 L RUSM Etro BMA v
18. 7 8 2 4 4 1 3 2 4 6 4 2 8 qe i 63 E 4 3 8 ii l 12 G ii 4 5 6 PFT FAF 2 4 8 2 8 2 A 64
19. d v 2 ID i 2L i 2L jii 94 PFT v 1 L AT H P PC a me Mar Ra wt 7 dn ytorna e am O gt M yA o a E P M 1 x at i ya do Pap 1 Am s wew gt r o
20. LCD BET 2 6 I 10 7 52 PFT DID i i p 24 iii o 47 OTTEY KE i
21. LCD 2 3 3 3 i 60 PFT TX i FVC 5 0 15L i
22. 46 6 AST ALT 100IU L 2mg lmg AST ALT 100IU L Img AST X ALT 1001U L 7
23. 7 4 5 USB 6 EDC 5 2 10 35 2 0 2 7 30 75 700 1060hPa 62 PFT 6 Visit
24. AGE 8 fij 1978 8 31 19780831 i XE 59 PFT FER 1 25 1 25 50 5 50 2 1 iv Kg i
25. 2 lmg 3 2007 8 amp http jas umin ac jp publications guideli ne html A K ORFA E
26. 2 6 LAM 6 i 3 FVC 2 FVC 0 2L 2 FEV1 0 2L i 8 FVC FVC FVC
27. Visit0 2 m 1 X 2 O 3 AC1 0 0V 50 60Hz 4 5
28. E 3 4 99 PFT FIRE 2 FVC FVC FVC START FVC RV OTLC RV RV 6 STOP 6 3 84 3 FVC
29. C 1 Kawamura K Shimohata T Nakayama H Tomita M et al Factors influencing the cognitive function in patients with multiple system atrophy Mov Disord 25 2891 2892 2010 2 Ishikawa M Shimohata T Ishihara T Nakayama H et al Sleep apnea associated with floppy epiglottis in adult onset Alexander disease a case report Mov Disord 25 1098 1100 2010 3 Ozawa T Shinoda H Tomita M Shimohata T et al Tremulous arytenoid movements predict severity of glottic stenosis in multiple system atrophy Mov Disord 25 1418 1423 2010 4 Shimohata T Tomita M Nakayama H Aizawa N et al Floppy epiglottis as a contraindication of CPAP in patients with multiple system atrophy Neurology 76 1841 1842 2011 5 Shimohata T Nakayama H Tomita M Ozawa T et al Daytime sleepiness in Japanese patients with multiple system atrophy prevalence and determinants BMC Neurol 12 130 2012 6 Ozawa T Sekiya K Sekine Y Shimohata T et al Maintaining glottic opening in multiple system atrophy efficacy of serotonergic therapy Mov Disord 27 919 921 2012 7 Furushima H Shimohata T H Ozawa T et al Significance and usefulness of heart rate Nakayama variability in patients with multiple system atrophy Mov Disord 27 570 574 2012 90 PFT PFT Sirolimus
30. 2 II 8 F H 48 A Sirolimus MLSTS 9 3 11 MILES
31. JRS i i 2 96 PFT 2 i 72 i 1 Visit 0
32. LAM wuyAA B 3d gmmj3 c opi A LAM MILES B 1 1 45 HRCT CT 2 DA DRA Zib n meo SEES CREE A
33. x RR 23 0 20X E 101205 0 57 anra AE AEMT p BUS EW UCET PRAL 1 48 iv v 3 2L 0 5 1 4 vi vi 8 i ID Visit ID ID 001 001 Visit 0 001001 0 ID 001 001 Visit 4 001001 4 i El BIRTHDAY
34. 47 2013 2 15 8 2 2 CHR m 2 18 10 7 29 50 48 Do D wnuDyAApm REMA c L TREBE A mammalian target of rapamycin mTOR
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