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CHAPTER 17  ■  Hemoglobinopathies and Thalassemias                                   323




                   Sickle Cell Trait

                                                                                                                                                m                             m
                   A       x m           %            k Am                            z                                                             .

                   H   S. T    ,                                    H   S        A. S   k                                           I                                    m                   m   ;

                                                                                                                -                        m         m                                   q

                   m        m                                       m         , P.  alciparum,                                          z                                     m     m      . G

                         m  . S   k       m              m        m                    . S   k                                                                      m     m                    . T    α

                                                                                    ,                    -                                 m                              ,       ζ         ,

                             m        k                                               . L       x                                    m     m       .                                   m                   m  -

                         m                                              k           (AS)       m                                  m  ,                                ,                   α-              q          .

                                       m                              H   A. AS                   k                            O                                   m     m  ,                               ,         -

                                                       SS              . A                , AS           -                          m                    -α                     m     m       . T    γ

                   z                             k                                                                                                                                         m     m  . I       m

                          x   .                                                                                                                               α        β             m   ,

                                            m                              m   ,                                                m                            .

                           m         ,                      k                    ,                                                  A               m

                   m                                         k                 . E                                                                                    m

                                                                 %         %        m                                                                       q                                 . O                     -

                                                  k                         H    S.  C

                             m     m                                  k                                                                        m   . T

                      m          ,                                                          (

                      ,    ),                        ,                     ,                                                    .  A                m                                          m

                                        .

                                                                                                                                .  A m                                                                         q

                                                                                                                                                                                      ,                           f

                     NOTE: This is a good time to complete Review Questions                                                                          mRNA

                     related to the preceding content.                                                                          .  A m                           m

                                                                                                                                              x

                                                                                                                                .  A m                          m


                    HALASSEMIA                                                                                                                                                                                   m
                                                                                                                                           ;       mRNA         m                                               -


                   Demographics
                                                                                                                                .  A                                                                  ,
                   T         m                                                                  m,                                                           q             m     m

                            m             ,                                                             -

                      m                      x                                             x                 .                 Pathophysiology

                   A                                                                            mm

                       m                                 . I                  , H   E–β          -                             T         m                           z

                      m           H   H                                m                      j

                                                  m   . W             , H   E–β             m                                         m       m             m            . I   α           m  ,

                                    m         q            m                         . T                                                         α-                                                                        -

                      H   E                        %                                 m                                                                            m                            H   H

                      S              A    . I                               N       Am        ,                                          m                           . T    m                 β             m

                                                                . α T          m                ,                                                  x    m                         α-          ,                    -

                                             ,                          z             m                                                  x                                                        β-

                                                        . H   H, H   H–                        (CS),                                        .  S                RNA  m            m

                             m  z          α             m                          m              -                                                         m                          β            m

                                      . C                             H   H                                                                     RNA                 . T                            f          RNA

                                      m                                                                  -                                                                                 .

                       m        H    H                              H    H–CS.  H  m  z

                   α             m   ,                    ,                     m          mm                                  b Thalassemia

                                .
                                                                                                                               β T          m                        m         mm              -             -


                   Etiology                                                                                                            . M     m                             β             m
                                                                                                                               m            ,                                     ,                          m     -

                   T         m                                               m                                                                                  β-                    .  M

                                                                . T                                                            m                                            β-                             k

                      m                         (  .  ., H   S        H   C)                   m                                                                             β-          ,
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