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CHAPTER 17  ■  Hemoglobinopathies and Thalassemias                                   317
















































                           A                                                                   B                                                                    C



                                     FIGURE 17.4  C                       . A. “S       ”                                             m                k                 m   . B. B
                                                                        k                 m           m      m      . C. L        m                                                                   k

                                                m   . (F   m N          DG, H         MA: Rogers’ Handbook o  Pediatric Intensive Care,           , P                  , PA:

                                     L               W      m  & W  k    ,         .)







                        m                 m                                                  ,                                                                                                            m

                                  m                                     ,      k  ,            ,

                      m      ,                    m               . T                                    -                     j       ,                m                                                    .

                                                                  q

                                               k                                                     .                         Sym ptom s Associated w ith Pregnancy

                        T                                                   SCD. M                                             I                   ,                                                m

                                                                                        m               .                                                              m            m                    %

                   H           ,   %         %                                         %         %                                     m                    %.

                                      .
                                                                                                                               Clinical Manifestations in Adults

                   Sym ptom s in Children                                                                                      SS      m  z                                          m                 m   ,


                   I   SCD,                                                             -                                         m                                                     %            %. R

                      m                                                        . T                                                                        H   F                   k                  m       k

                          m                                    ,                                                                           ,              ,                               . I

                          k                           m                                    ,                                                                                  ,      m            m             -

                   m  m                 Streptococcus pneumoniae        Haemophilus                                                               . T          m                                                        -

                   in  uenzae             .  I                                        m          q                                                                            m                                           .

                                                                                          (             .  ).                  A                                 m               ,                                -

                        S  m     m                                                                                                       ,                m                                                          -

                     m        . V

                           m                           . C           m                                                                                      q      m                                     m        .

                                                                                                                               T       m                       mm                                        m

                                                                                                                                m      m                                                                  m        .

                                                Causes of Death Among Children                                                      A                           m
                       TABLE         17.1
                                                with SCD                                                                                                                    SCD. H

                                                                                                                                                               m               ,                       m
                       Cause                                        Percentage of Total Deaths                                                                   , m                                 ,               -


                                                                                                                                      m                                                             . T
                       Infection                                                           44
                                                                                                                                                                         m                                  %         %
                       Splenic sequestration                                               16                                              ,           m     m          %         %. R        m            -


                       Sudden, unexpected death                                            14                                                                                  . L               m              .


                       Cerebrovascular accident                                            12                                       F                                                SCD
                                                                                                                                                . A
                       Congestive heart failure                                              7

                       Miscellaneous                                                         7                                       ,                    m  ,          k  .
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