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        マイクロプレー ト法によるメイプルシロップ尿症のマス ・ スク リーニング
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1.                                                                                                                                                                                                                                                                           11                   9                                                                2                                                                    1                MSUD              BCA                                                                                                                                                       2                                                                                                                             233                                                  BCA   NAD    HO                          NADH  NH     N 36                                   HPLC                     BCA                                         NADH Co 5BrPAPS                   660480m      3 94mg dl                                            NA GE NADH 2                          BCA                                        1 MPMS            1 MPMS                                                             Co lD Co l  5BrPAPS     Val                 131 2 117 2 1 12           SM                                                     590nm                                          2SD          1 MPMS  1 methoxy 5 methylphenazinium methylsulfate     7mg dl                                   
2.                                                                                                                                                          Phe  Gal                   BCA  mg dD                                                                4            BCA                                                                                                        Freduencu                235            2 MSUD                               BCA                                                      ILS  MSUD                 Leu ile Val BCA  78 017 10 23 8 6 1 10 9 44 2  80 024 5 43 7 11 9 11 4 68 5  81 016 6 36 4 8 5 8 6 54 6  81 017 5 29 2 11 2 9 4 51 0  82 019 8 69 7 15 5 16 4 103 7  82 021 10 35 8 5 6 8 7 51 2  84 022 9 28 9 7 3 11 8 49 5  84 023 10 45 5 9 6 13 7 70 6  87 203 10 23 2 8 2 8 5 41 0  88 201 10 8 3 3 5 7 3 20 0  88 202 10 75 8 11 2 12 4 101 0  80 023 15 5 3 2 4 2 5 10 5  82 018  1          4 0 1 5 5   11 7  83 022 12 3 4 1 3 2 8 7 9  91 204 16 5 5 2 2 4 5 12 8                     Mean 1 26 0 70 1 77 3 94    N 36  SD 0 28 0 23 0 44 1 00                                                       HPLC                         mg dI blood                                                   BCA                                                                                                                                            10 000                              BCA                       4        MSUD                                 BCA           2                 
3.                                                                                                                  BCA                                                                                                               a 0 1 rk A g c H                                                                                                 2 BCA                                                                                                                                    _                                                                         2  3                                         Phe   Gal                                                                                                                                                                                                                                                         BCA                                              MSUD                               3                   BCA                        Leu                                                                                                                                                                                                                                                                                                                                                                                                                         96                                                                                                     
4.                                                                                       3                                                                                                                                                                                                                                                                                         BCA                                                                                                                                                                                      4                                      Leu             3 4 5 5mgdl                                        BCA                                           Leu                                                                                                                                                                                                         BIA         Leu 4 6mgdl                         7 9   9 0mg dl                            BCA                                                             MSUD                                                             Leu                                                                      BIA                                                 234                                  MSUD                                                                                                                                                                                                              
5.                                                       5 4        1                               7 0                                           5                                                              20 PV                                                                                                                                                                                                                                            1 036 0 A EA  TA 2    PS     wh 2                                   1332                           2 i                                                            1 076 922                                           MSUD              TA           a        y     x                                      1 186 1 186                                                                                                             279 482                                              10                                                                     1 600 560                               BCA                                   1 104 ny                                                            1 073 957             20 104mg d1                                                                   2 516 2517                                                                   12 HG 9807                                                                                                                                                   2                             
6.                        8 10mgl                                BIA                                 Leu 4mg dl                                                                                                         BCA                                                     MSUD                                                                                                   2SD             7mg dl                                                                                               HPLC                                                                                                                                                            Leu                    BIA                                                                                    Leu                                                                                                                                                                                                                                       MSUD                                                                                                                                                                                                                                                                                                                                                                                                                  1 Naruse H  et al  Neonatal screening in the  ninties  59  The Kevin Place  Manly Vale  1991  2 Yamag
7.        5                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                 MSUD                                                                                                                                                                                                                                            11                                                                                                                                                                                          BCA           Leu  Ile  Val                              SD  mg d                           4 52     1 20 In 12 933                 4 19     1 51  n 9 807                                                                                                                                                                                                                                                                                                                                         10                  
8.    11                     BCA                                                                                                                       BIA                                                                                                                                                                                                  MSUD                                                                                                                                                                                                                                      Phe  Gal                                                                                                                                    LeuDH      Leu  Ile            Val                                                                                                           NADH                   BCA                                                      NADH                                                                                   NADH                                                                                                                       1      BCA                 Val                     117 2      Leu lle     131 2                                                                                                                                                                     Leu                    BCA                                                            IDL                 
9. uchi A  et al  Screening  1  49  1992   3                           3                                                                                                                                                                                                                 101  1992        236     9    
    
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