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314            PART 4  ■  Erythrocyte Disorders




                                                   β 2                      β 1

                                                                                                                                BOX  17.1






                                                                                                                              Percentage of Eastern Mediterranean Region

                                                                                                                              with an Abnormal Hemoglobin                                     *




                                                                                                                                 Proportion                 Country of Population



                                                                                                                                 >10%                       Cyprus (17%), Bahrain (13%)


                                                                                                                                 6%–10%                     Iraq, Morocco, Oman, Qatar, Saudi

                                                                                                                                                            Arabia, Sudan, Syria, Yemen

                                                                                                                                 4%–6%                      Iran, Kuwait, Lebanon, Libya, Pakistan,

                                                                                                                                                            Tunisia, United Arab Emirates



                                                      α 2                     α 1                                                <4%                        Afghanistan, Ethiopia, Egypt, Jordan


               FIGURE  17.1  S                              m            .  (R                   m                            *
               P       CM. Pathophysiology Concepts o  Altered Health States,                                                  T         m                m                                                                  z  -

                  , P                 , PA: L              W      m  & W  k    ,         ,                                    m             ,        x  m     , G  PD,                  m  m                     . D
                                                                                                                                              m A                         M        .
                   m        .)







               HEMOGLOBIN DEFEC  S                                                                                         Demographics

                                                                                                                           SCD        β thalassemias             m         mm     m

               A       m        m             m                                             sickle                                         m    . T                             “m                ”
               cell  disease  (SCD)              m m        ,         m                  .                                  x             m        M                     (     B  x      .  )               -

               P                       m                  m              m  z          (S/S)                               S            A                     S              A                          C       .

                             z          (S/A)              trait.  T         k               m                             T           m              m

                                   m             C, E,     D,                      SC, SE,                                                                              z                                                -

                  SD            . I                        m             S,                                                                                          Plasmodium  alciparum.

               m                            m            m              m                                                       T         k     m                                         q                          -

                          x                                                                                                              C          A       . T          m    m  j

                             m                        . T      m                                                                 k              M                    .  T          m                     mm

                                                                   k                     x                                     N       Am                                                                     ,

                  m              S        m    z                 m                                -                                              m                                       . T    M         E    ,

                                      m                     m                      m                                       S        A    ,              O                 α             m

                       m                               x                . A                                                   m                      .

                         m             S m                                                                                      SCD                           mm       m                                      -

                                  m                                                                                                  m       m S    -S            A       , S        Am        , C      ,

                                              . I            m        m             C,            m                        C           Am        , S        A        ,  I       ,         M

                      m              m                                      x                                                                            k   , G      ,        I     . I        U

                                                                              m                         .                  S       ,                               ,                  , m                            -

               A     m                                                                           m                                   m       m A       . T

                  q                 m                       m             A                                                     A          Am                                            ,          ,

               F             .  .                                                                                          H            Am                   . A           m         Am           ,

                               m                                   m                                                                A          Am           ,                    k                    .

                                                                               m                                                B                                              A              H                 -

                              -                         -                    .                                             m                 U.S.                  ,


                                                                                                                              m                              x                       m                           -

                                                                                                                                                        .


               Normal hemoglobin                                      Glu                            Beta chain                 T               m                                                                 -
                                                                                                                               k           m

                                                                                                                                                         m ,                                             .





                       Hemoglobin S                                   Val                            Beta chain            Etiology


               FIGURE 17.2  C  m                     m               k        m             m   -                          H  m                         ,        x  m     , SCD,                               -

                       . (G   ,        m         ; V   ,           .)                                                                                                      m                              q
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