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抄録集 - 筋ジストロフィーの治療と医学的管理に関する臨床研究班

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Contents

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6.
7.
8. 5 13
9. 23 9 11
10. a m 1
11. 11 10 NIPPV
12. QOL DMD BMD 4 4
13. Microsoft Office Excel Peak cough flow PCF QM I C
14. 4 Max QDA
15. 51 50 1
16. 4 E E 56 8 40
17. 4 73 26 3 6
18. ml dt il XY
19. 67 66 PT PT
20. Abdim
21. 2 4 7 8 4 5 8 6 EE
22. 20 15 IQ46
23. PDF PDF YAHOO0
24. Continuous Performance Test CPT X 9 LGMD 7 FSHD 3 SMA2 Miyoshi 1 4 6 CPT
25. 50 49 VTR
26. 1 gt
27. 2 4 45 2011 6 10 Ff
28. 1 44 SpO2 SpO2
29. E
30. QOL
31. 1 19 FFQg Ver 3 0 BMI18 5 18 5 11 18 5 8 2 21 3 6 4 1508 353kcal 17 0 7 5 1851 580kcal P F C P F C 13 32 55
32. E 2 1
33. uh k II 31 30 ME F 3
34. ADL 40 25 12
35. NST 17 23 NST 3 1 1 37
36. 10 2 I 1 7 E
37. 5 50
38. 32 Y x L 31 ff
39. RT 38 1 1
40. 1 B 20 E E 3 HH 19 11
41. 2 1 3 A 5 6 QOL 3 QOL
42. QOL IT 3 8 2 5 IT IT IT
43. t 60 59 OT JICA 0T 5
44. QOL 56 55
45. RY a 3 1 F 38 A XP CT MI E i 25psi 2 2
46. 1 57 32 42 25 24 23 9 26 1 E E 23 F 1
47. I B hb 3 12 12
48. 0 2010 10 9 1 2 7 1
49. 21 20 es h E Duchenne
50. NST PT 17 NST NST
51. 33 H 94 Sp02
52. 12 8 1 1 B 1 1
53. 2 2 C F 47 O
54. 819 43 42 Sp02
55. k 1 2 3
56. 3 86 3 47 47 88 E
57. 1 24 PE PT PT A
58. 2 2 4 1 5 5 A B A 14 16 B 1 4
59. PT ME 24 1 2 11 1 23 9 30
60. MAC Mechanically assisted Coughing CA 3000 MAC BacF BacF MAOC 23 1 18 23 7 25 16 6 1 3 2 2 2 16 BacF
61. x 33
62. 4 1 40 k ul p58 59 27 26 SW SW
63. 51 LP10 SIMV 12 21 320 329 2002 PowerLab8 AD Instruments AD Instruments ds32a PowerLab8 Thinklabs Medical 3 PowerLab8 4cc
64. 7 7 8 37 6 17 4 479 2 0 2 3 1 0 9 6 5 3 1
65. 2 19 28 17 5
66. 55 54 SW 5
67. QO L Mid 65 64 4
68. BS k ul k ig DX 28 27 O 1 6 1 3
69. 4 1 2 3 4 E E PC i 69
70. 1 1 1 5 2 H22 12 H23 10 1 2 1 30 80 3
71. 1 4 23 4 18 27 14 12 2 LAN 18
72. 3 a b 30 24 14 14 3 15 5 3 4 3 76
73. 30 ul 29 23 3
74. B B NR RN E E
75. OT OT OT OT 0T 0T OT OT 95 ADL QOL
76. 4 1 j 2 37 7 9 17 1 41 20 3 47 49 279 QOL
77. Abdimn 1981 Mayana Zatz NPO0 106 20 6 1 2 Abdim 2001 NPPV 2007 1992 2008
78. 71 H
79. x 1 10 j ME ME
80. 63 62 QOL 2008 2008 2008 2009 2010
81. 53 52
82. 41 59 87 55 45 2 27 77 3
83. E Il VT 24 RICU
84. PARS SRS 8 112 35 33 2 1 PARS 27 SRS 23 PARS 12 27 PDD PDD SRS 1 2 2 4 27 2
85. 6 2 81
86. 57 56 QOL 1981
87. 2 31 3 0mg ql 7 3 87 3kg HMB NST
88. 23 2 RICU Stiller 2000 ICU PT PT RICU PT RICU PT RICU 9 30 241 RICU PMD SMA RICU
89. MyD A A UE Rh MyD 1 RB o 6 2 PCR gt
90. 1j H 1 1
91. Sp0 Sp0 E 45 E MAC 1 PT PT PT PT PT PT ME
92. 18 21 12 80
93. 2 1 1 1 23 1 9 3 1 ME 5 23 10 19
94. 3 3 30 3 1 2
95. A AD 7 23 48 5 15 1 6 6 HH m N E 60
96. j H 3 46 32 1 3 6
97. 2 2 2 I 11 20 J
98. 12 26 27 23 10 1 22 10 2 23 10 1
99. MyD 10 F 50 66 FN 1 14 MyD EG FE 3 MyD MyD
100. 33 19 33 MyD DM1 NPPV DMD DMD
101. 68 1 A 18 2 JF 3 E 4A
102. Cue SEIQoL INDEX 70 4 Post test SEIQoL INDEX 83 4 TPN 3 TPN 2 Post test Cue Cue Cue Post test SEIQoL INDEX 86 4 Then test SEIQoL INDEX 76 5 Post test T 1 Post test SEIQoL INDEX TPN TPN
103. PGPB NGPB IRV ERV PGPB NGPB GPB PGPB NGPB ADL QOL 17 16
104. 2010 21 2010 10 2011 5 12 2010 8 1 2 34 Sp0 3 1 E Sp0 Sp0 2 Sp0
105. 19 6 2 4 1 4 0 QOL
106. Mechanical In Exsufator High frequency chest wall oscillation ECG PP RICU 26 25 1
107. RV ERV MIC 2 169 CPF 950 min 215 min MIC P02 81 8mmHg PC02 70 5mmHg 22 10 P02 104 0mmHg PCO2 50 4mmHg 23 1 BiPAP 21 5 VC 0 489 0 50 23 1 20 NGPB PGPB
108. ECS ECS 25 TV 67 62 57 PC 35 TV DVD TV PC 359 PC TV PC
109. 36 5 F B A 35 QOL
110. RNN OT OT ST 1 WM
111. ME Legendair Trilogy100 mobile1000 Legendair Legendair STP Standard Conditions USA Trilogy100 BTPS Body Temperature and Pressure Saturated mobile ATPS Ambient Temperature and Pressure Saturated with water vapor 3 Legendair Trilogy100 mobile1000 3 CV f1ow 12bpm
112. TT 1 2 3 4 12 38 A AMY
113. SEIQoL DW QOL A 25 DMD X X H23 8 1 H23 11 30 1 1
114. N 1 4 ADL 1 FIM ADL ADL 100 3 4 22 2 3 FIM 3 2 1 12 ADL
115. H23 3 4 43 1 95 4 67
116. 29 28 3
117. 4 1 709 100 809 1009 95
118. 4 7 27 23 2 PMDA PMDA
119. 1Q 1 1Q 1 5
120. 1 2 EN ET TT IED SC 1 5 0 2 2 1 1 B you you 23 you
121. NIPPV BiPAP 8 0 4 2 2 1 1 20
122. 63 MyD 3 1 10 5 10 IT HH 5 AMY 1 HI 1 5 i E AMY 3 R R HF 0 05 0 15Hz LF LF
123. EF 32 59 X 2 TIPPV 9 4 2 CT E
124. NO 4 4 E
125. 10 1 RCA 36 E 10 12 6 7 E 30 H21 8 830 17 15 2 17 15 8 80 8 2 5 RCA H21 2
126. Wii Wii IT IT IT 23 22 100 7 QOL 2006
127. 2010
128. Mi Chart5 0 16 13 De eae 0 9 Chart5 0 AD Instruments PC PC
129. 2008 4 2011 8 6 ME 4 E f j ME 2010 0
130. 23 10 11 A 1 8 ALS3 MD 2 2 F CMD1 1 1 MyD 1 1 PDM1 1 1 1 FSH 1 1 1 4 2 1
131. A 5 H17 11 H23 5 2 1 1 5 84 43 27 5
132. 32 60 38 14 14 4
133. 33 32 SEIQoL DW QOL 1 QOL SEIQoL DW DMD QOL SEIQoL DW
134. H SEIQoL INDEX Post test SEIQoL INDEX T Pre test Cue SEIQoL INDEX 81 4 Cue H20 FE Cue H23 1 Post test
135. 59 58
136. 4 23 6 1 90 11 E 41
137. 66 X 7 6 EE GE 0 28 8msec 1 4 6 DMD 1 4 MyD 1 0 BMD FSHD 2 SMA2 Miyoshi 9 4 65
138. PT o N 3 E 1 3 1 DMD 2 1 DMD DMD AU RST RST RTX
139. i 3 A k 1 6 ON MA a RE 8 8 2 5 7 10 4 5 A 80 C 409 A oe B C SS
140. TP Alb 1 23 8 3 DMD 19 NPPV 22 NPPV NPPV 39 3kg 1850kca1 9kca1 kg 2 28 30kca1 kg 39 5 40kg 6 34kg 1 9 1860kca1 47kcal kg 38Kg 3 NPPV 27kca1 kg 35 4Kg 3 9 NPPV 36kca1 kg 38 5kg 25 kca1 kg
141. 4 50 5 1 5 NN RE
142. 849 65 54 0
143. 2005 20 HI 1 LEF HE 5 AMY 5 LF y 0 15 0 4Hz HE 37 28 DMD I 5 BIMUTAS
144. MyD DMD 16 15 PT PT 1 PT PT PT 1 PT 0T OT OT ST Glosso Pharyngeal Breathing GPB GPB
145. 0 7 LG 1 DMD 3 FSH 2 MyD 1 1 160 100 130 23 9 12 7 NN LG DMD 70
146. 3 NIPPV 3 20 41 5 18 23 8 25 10 13 3 2
147. 61 60 2 3
148. 2 3 4 Burkholderia cep Serratia m A 2 3 4 37 4
149. f 30 4 1 3 VS 1 15 20 15 1 3 F 3 Sp02 7 X 2 CT 1 8 Ga 1 4 E 95
150. 2 PT OTT
151. R 45 30 0 2 H k 2 2 5 520
152. E 54 SRS PDD 2 PARS 53 2 DM
153. DMD CT B NPPV f 2 30 30 IPV RTX
154. T cho DMD ALB Hb TLC DXA 3 T ChE TG BMT DMD NIPPV 24 NIPPV BMI BMI ChE TG T cho DXA 1 DMD BMI
155. Cue SEIQoL INDEX 1 Then test 2 Post test SEIQoL DW Cue SEIQoL DW x
156. SpO2 Sp0 43 ME ME 110 Sp0 Sp0 Sp0
157. 1 2 3 7 2 1 42 2 3 1
158. positive pressure PGPB PGPB negative pressure NGPB NGPB 33 DMD stage WW 159cm 41kg BMI 16 2 61 3 1 2 2 PGPB 15 10 NGPB 23 1 NIPPV BiPAP NGPB PGPB NGPB PGPB 23 2 VC
159. 13 25 8 5 1 1 IC 5 104 39 12
160. 1 STP 2 BTPS 3 ATPS 300m1l 500m1 700m1 Legendair Trilogy100 1 Legendair STP 300ml Legendair 304m1 300m1 Trilogy100 244m1 304ml nmobile1000 252m1 500ml Legendair 512m1 500ml Trilogy100 419m1 500ml mobile1000 440ml 700ml Legendair 696m1 695m1 Trilogy100 600m1 701m1 mobile1000 631m1 2 Trilogy100 BTPS 300ml Legendair 349m1 295m1 Trilogy100 277m1 299m1l nmobile1000 283m1 500ml Legendair 570m1 495m Trilogy100 462m1 497ml nmobile1000 494m1 700ml Legendair 766m1 695m1 Trilogy100 661m1 703m1 mobile1000 689ml 3 mobile1000 ATPS
161. 10 1 2 62 61 verbal nonverbal 93 Merabian 1968
162. F 55 16 3 1
163. A B QOL B 3 QOL QOL B 24 6 H
164. 6 3 0 16 7 20 40 82 6 77 56 m IT IT 3 2
165. 10kg QOL 1 1 EE 7 7 se 2005 3 2011 8 6 5 2010 2 2011 4 2010 3 5
166. 4 6 3 9 MD 1 1 MyD8 BMD8 LGMD7 9 3 6 0 0 1 7 DMD 3 MyD 2 BMD1 FSHD 1 3 7 0 X 9 5 msec 8 7 9 7 5vs8 4 99 4 1 2 vs8 4 8 26 1 9 7 1 4 09 6vs7 5 4 89 4 84 9msec vs660 4 120 X ee ml
167. 90 85 100 80 3 H QOL gt 58 57
168. PCR plaque control record EL LE 6 5 F 33 6 MyD f
169. 52 3 51 70 ADL
170. NIPPV ALB Hb TLC 24 NIPPV NST ChE TG T cho 40 2 39 13 FIM FIM
171. lt lt gt 13 lt gt lt gt lt gt 1 3 0 6 1 6 0 5 lt gt 1 3 0 9 1 5 0 8 gt lt gt
172. SEIQoL DW QOL SEIQoL DW QO 34 33 SEIQoL DW H 1 9
173. ADL 3 ADL 4 4 2 1 0 E ADL 3 0 50kg
174. i IPV StageVMb DMD 1 11 oe IPV IPV 1C 240 5 200 15 10 RTCO 1 H24 10 160 3 4 11 5 2 2
175. 18 sj a 2 E
176. 5 7 5 f f 24 7 5kg
177. 2 1 6 6 QOL 2 7
178. 10 DMD DMD
179. 1 2 3 4 5 mR 0T
180. 2009 8 DXA gt Cr 2011 9 DM D 55 DXA 0 DMD f 2 DMD gt A Ww E DMD NIPPY 6 2 4
181. 1 2 PC 8 O R Mn HH 10 MyD 6 5
182. 80 2 1 ICU 2010 8 2011 5 9 48 2
183. 2 1 1 CD 15 13 6 6 26 Index67 2 15 8 8 9 9 Index50 1 Index 2
184. 27 E 21 12 A 15 4 3 22 E B 9 C 5 D 2 E 2 E PMDA 4
185. 4 Ea 25 DMD 21 4 3 NIPPV TTPPV 2011 5 6 gt 1 Qc ra
186. SpO2 SpO2 Sp02 30 SpO2 Sp02 SpO2 SpO2 Sp02 SpO2
187. mi 15 14 PT Duchenne MD TPV 1 1 1
188. 0 3 2011 10 12 1 40 Ns 1 1880 6619 2 1 Page115 116 2006 11 49 48
189. 4 160 110 17 1 2 2 3 4 12 19 12
190. a 1 et 37 3 36
191. 3 2 1 2 NPPV 5 3 8 ll CDR E QOL
192. 2011 10 19 40 36 5 22 11 10 11 24 12 2 0 1 23 1 20 10 19 i 4 RA
193. PDD ON NN MN TY 0 Hinton et a1 2006 QOL 2011 8
194. VF P F C
195. JF 6 69 H 1
196. 90 6 15 9 109 1 14 8 P lt 0 05 12 P lt 0 05 lt gt lt gt lt gt lt gt lt gt 19 2 3 3 8 6 2 0 lt gt 7 lt gt 6 lt gt 6 lt 7
197. MyD 38 2 3 3 gt gt a 1 EE gt DXA X DXA CT
198. pHT EHH T DMD NN DMD a NPPV DMD 2 NPPV NPPV
199. 1 SE i 2 MS 009900 C2700 0 6 8 A NE QOL
200. 1 rt eG 2013 8 FCMD FCMD
201. TPN Post test Then test H Pre test Cue PC TV CD 20 50 SEIQoL INDEX 15 H283 500 50 PC TV SEIQoL INDEX Post test 50 Then test 53 3 SEIQoL INDEX Post test 836 Then test 40 5 Cue TV PC
202. 4 j QO x x ee A 40 33 6
203. BacF 7 6acF 9 3 3 BacF CertifierPEA Plus SMS 5 cmHzO L sece 20mL cmHzO Peak flow 40cmHsO 5 2 BacF 3 2 BacF Staphylococcus Peak flow 40cmHzO 122 2 1 9L min 122 3 2 0L min
204. 23 10 31 23 2 066 2 193 Duchenne DWD 770 882 WD 327 411 11 29 22 132 0 11 37 9 22 62 9 DMD 11 58 7 22 85 1 11 36 6 22 45 3 DMD 10 95 16 22 70 6 12 22 1 307 DMD 409 MD 363 DMD 47 MD 64
205. ME F 23 3 11 gt
206. 1 RM 26 2 i 2 3 3 h 5 2
207. 2 1 0 1 5 12 1 Sp0 3 834 j 1 a Sp0 23 3 3 6 H
208. ADL ADL SERIQoL DW SEIQoL DW 1 Cue QOL SEIQoL DW QOL 2 QOL H 53 Post test Then test 3 Post test Then test 40 Duchenne 3
209. 1 2 4 NIPPV13 TIPPV12 1 2 1 1 1 1 48 BiPAP 11 12 3 TIPPV 2 3 16 15 3 TIPPV
210. 39 6Kg TP Alb NPPY NPPV 2 21 8 4 DMD 19 NPPV 21 NPPV NPPV 58 3kg 1600kca1 26kca1 kg 3 24 27kca1 kg 58 8 59 3kg 6 1 3 11 27kca1 kg 1 5 NPPV 8kca1 kg 39kg NPPY 40kca1 kg TP Alb NPPV
211. 1 2 a 26 WHO QOL26 2007 b WHO SUBT 1996 3 64 63
212. 3 1 2 F 41 CT VS FE X 1 60 1 45 3 5 20 cm H20 1 10 cm H20 FF 1
213. ME ME 14 L ME 4 E ME ME RI 13
214. P F C 15 28 57 11 8 4 6g 8 3 3 4g 28 14 9g 71 3 18 6g 83 4 41 9g 82 8 18 3g 1 0 3 0 1 0 1 2 VF 73 76 35 79 36 4
215. 3 VS VS 1 E E 4 68 40 QOL
216. 1 2 RST 1 2 4 DMD LGMD2B 63 3 IPV PPV E 2 lt 2 IPV
217. 3 6 2011 1 A 1 2011 1 13 2011 9 30 A 20 DMD
218. 15 Duchenne 32 71 o E 41 22 21 IT
219. a RT Tt Hy a 35 94 QOL O
220. PFC 5 72 LG DMD NR QOL
221. QOL AA 7 A ADL
222. PT PT retrospective RICU 92 51 31 10 11 5 DMD 2 SMA 2 BMD 1 2 DM myofibrillar myopathy CPA w 1 SMA 1 DMD 1 DMD 1 BMD 2 PT PT 9 23 6 10 3 ROM 7 ADL 2 8 2 7 2 SpO
223. lt BR RY 23 N H 3
224. you you you T 2 T A I eR 1 1 0 1 2 1 1 1 1 2 1 5 0
225. 3 0 1 1 1 3 ADL 4 0 9 1 5 ADL 5 2 2 ADL 2 2 0 1 1 2 2 4 3 4 4 3 150cm 172cm 27 6kg 63 8kg 1 1 2 ADL 2 2 4 3 2 0 ADL 4 4 2 1 0
226. 220 42 3 8 1 5 14 2 7 7d 39 8 1 29 111 21 3 9 1 7 61 29 4 0 8 30 5 8 67 12 9 10 1 9 34 6 5 686 300 43 7 26 3 8 13 1 9 9 1 3 149 21 79 18 2 6 6 0 9 39 5 7 90Q3 19 71 09 22 3 1 2 gt E 3 10 p 1
227. 3 1 AMY I HF LF AMY TI R R 3 H AMY 39
228. ChE DXA BMI 1 1 gt T cho F 12 15 5 3 8 8 21 4 3 8 24 NIPPV 23 25 1 5 1 TIPPV 12 30 4 TG Hb E Alb TLC E BMI 43 0 17 9kg m 23 3 13 1kg m
229. XP IPV 10 HR 3 E 12 E 2009 ME 2 2010 3 MR 2010 11 1
230. B 30 DMD C 30 DMD B A C C
231. BacF Peak flow BacF BacF BacF 3 BacF 3 CA 3000 BacF MAC 46
232. FIM 41 ADL 40
233. 11 65 73 23 3 11 116 31 73 24 54 6 3 1 lt gt 63 0 96 9 1 10 57 9 2 64
234. 47 46 CE en v 2 3
235. 1 8 3 E 10 1
236. 300ml Legendair 339m1 295m Trilogy100 272m 298m1 nmobile1000 275m1 500ml Legendair 551m1 500ml Trilogy100 458m1 498ml mobile1000 478m1 700ml Legendair 747m1 695m1 Trilogy100 646m1 700m1 mobile1000 680ml 10 Legendair STP Trilogy100 nmobile1000 10 Trilogy100 BTPS mobile1000 ATPS Legendair 10 760mmHg STP BTPS BTPS 9 Legendair 10 Trilogy100 10
237. 2 NPPY NPPV NPPV TP Alb 2
238. 3 6 E SpO HR PIP 10 IPV XP CT Sp0s TIPV i ET PIP 10 IPV 1 Dt IPV Sp0 gt IPV 10 10 WN
239. 1 1H v Y
240. HR 10 2 ot 5 4 6 5 1 IPV PIP ETCO2 ETCOs HR 13 1 TPV ETCOs DNS 3
241. 3 38 47 20 14 24 NIPPV3 5 20 13 28 3 1 4 1 2 1 2 2 2 1 2
242. 9 8 24 7 4 7 6 Becker 2 46 62 4 32 62 1 31 3 38 60 8 31 60 51 21 61 6 34 7 6 4 Becker 4 41 83 11 47 71 5 51 66 3 60 77 17 31 65 75 25 21 65
243. D DMD DMD 15 23 3 5 96 SD 2010 9 2011 10 WAIS WAIS 7 5 WAIS IO IQ VIQ 104 6 15 5
244. ul gt E 70

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