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2007³â ¼±Ãµ¼º ¸é¿ª°áÇÌ °­ÀÇ·Ï

ÀÔ·ÂÀÏ:2007-11-23 / Á¶È¸¼ö:21745 / Ãßõ¼ö:311 / ÀÛ¼ºÀÚ:ÇѸ¸¿ë

¼±Ãµ¼º ¸é¿ª °áÇÌÁõÀº ³ªÀÇ Àü°ø°ú Á¶±Ý ¶³¾îÁø °ÍÀε¥ °­ÀÇÇÒ »ç¶÷ÀÌ ¾ø¾î ±×·¡µµ °¡Àå °¡±îÀÌ ºñ½ÁÇÑ ÀÏÀ» ÇÏ°í ÀÖ´Â ³»°¡ ÇÏ°Ô µÇ¾ú´Ù. ÀÏ´Ü °è¼Ó ¸Å³â °­ÀǸ¦ ÇÏ´Ùº¸´Ï ¸é¿ªÇп¡ °ü½ÉÀÌ °¡°Ô µÇ°í ¶Ç °­ÀǸ¦ ÇÏ´Â °ÍÀÌ Áñ°Ì±âµµ ÇÏ´Ù. À̹ø °Íµµ 2006³â ÆÄÀÏÀ» ÀÎÅÍ³Ý Çü½ÄÀ¸·Î ¹Ù²Ù¾î ¿Ã¸°´Ù. °­ÀÇ ¼ø¼­´Â ´ÙÀ½°ú °°´Ù.

 

¼±Ãµ¼º ¸é¿ª°áÇÌÀÇ ÇнÀ¸ñÇ¥´Â ´ÙÀ½°ú °°´Ù.

  • 2097. ¸é¿ª °áÇÌÁõÀ» ÀǽÉÇÒ ¼ö ÀÖ´Â ÀÓ»ó Áõ»óÀ» ¿­°ÅÇÑ´Ù.
  • 2098. °¡½¿»ù¹«Çü¼ºÁõÀÇ À¯Àü, º´¸®¼Ò°ß, ÀÓ»ó Áõ»ó, ¸é¿ª±â´É ÆľÇÀ» ¼³¸íÇÑ´Ù.
  • 2099. ¹üÀú°¨¸¶±Û·ÎºóÇ÷ÁõÀÇ À¯Àü, º´¸®¼Ò°ß, ÀÓ»ó Áõ»ó, ¸é¿ª±â´É ÆľÇÀ» ¼³¸íÇÑ´Ù.
  • 2100. ¼±ÅÃÀû IgA °áÇÌÁõÀÇ À¯Àü, º´¸®¼Ò°ß, ÀÓ»ó Áõ»ó, ¸é¿ª±â´É ÆľÇÀ» ¼³¸íÇÑ´Ù.

 

¸é¿ª°áÇÌÁúȯ ºÐ·ù

  • ¼±Ãµ¼º ¸é¿ª°áÇÌ(ÀÏÂ÷¼º ¸é¿ª°áÇÌ, ¿ø¹ß¼º ¸é¿ª°áÇÌ, Primary Immune deficiency ; PID)
  • ÈÄõ¼º ¸é¿ª°áÇÌ(ÀÌÂ÷¼º ¸é¿ª°áÇÌ, ¼Ó¹ß¼º ¸é¿ª°áÇÌ, Secondary Immune deficiency ; SID)
    • Áúº´ ; AIDS
    • ³ëÈ­
    • ¿µ¾ç°áÇÌ
    • Ä¡·áÀÇ ºÎÀÛ¿ë
    • ¸é¿ª¾ïÁ¦Á¦ »ç¿ë
    • À̽İü·Ã ¸é¿ª°áÇÌ

 

ÀÎü´Â ¿ÜºÎ Ç׿ø¿¡ ´ëÇØ ´Ù¾çÇÑ ¹æ¾îÀü¼±À» ¸¸µé¾î ³õ¾Ò´Ù.

    ¿ÜºÎ Ç׿ø¿¡ ´ëÇÑ ¹æ¾î´Â ´ÙÀ½°ú °°ÀÌ ºÐ·ùÇÑ´Ù.

      •   Anatomic/Physical barriers

      • ÇǺÎ
      • Á¡¸·
      • Mucous blanket
      • Ciliated epithelial cells¡¦

      •   Immune systems

      • T ¸²ÇÁ±¸ ; Cellular Immune deficiency
      • B ¸²ÇÁ±¸ ; Humolar Immune deficiency
      • Natural killer cells, Dendritic and phagocytic cells
      • º¸Ã¼°è

 

¼±Ãµ¼º ¸é¿ª °áÇÌÀº ÀǽÉÀ» ÇØ¾ß Áø´ÜÀÌ °¡´ÉÇÏ´Ù.

    ´ÙÀ½°ú °°Àº »çÇ×ÀÌ ÀÖÀ» ¶§ ¸é¿ª°áÇÌÀ» ÀǽÉÇÑ´Ù.

    • Two or more systemic or serious bacterial infections(e.g. sepsis, osteomyelitis, meningitis)
    • Three or more serious respiratory of documented bacterial soft tissue infections(e.g. cellulitis, draining otitis media, lymphadenitis)
    • Infections occurring at unusual sites(e.g. liver or brain abscess)
    • Infections with unusual pathogens(e.g. Aspergillus, Serratia marcescens, Nocardia, or Burkholderia cepacia)
    • Infections with common childhood pathogens but of unusual severity.

    À̸¦ ¿ì¸®¸»·Î ¹ø¿ªÇÏ¸é ´ÙÀ½°ú °°´Ù .

    • ù°. µÎ ¹ø ÀÌ»óÀÇ Àü½Å °¨¿°. (Àü½Å°¨¿°À̶õ ÆÐÇ÷Áõ(Sepsis), °ñ¼ö¿°(Osteomyelitis), ³ú¸·¿°(Meningitis)ÀÌ µÎ ¹ø ÀÌ»ó)
    • µÑ°. ¼¼ ¹ø ÀÌ»óÀÇ È£Èí±â °¨¿°À̳ª ±¹¼ÒºÎÀ§ °¨¿°. (¼¼ ¹ø ÀÌ»óÀÇ È£Èí±â °¨¿°À̶õ Æó·Å °°Àº °ÍÀ» ÀǹÌÇϸç, ºÎºÐ °¨¿°À̶õ, ¿¬Á¶Á÷¿°(Cellulitis, ºÀ¼Ò¿°), ÁßÀÌ¿°À¸·Î Æ©ºê¸¦ ¹Ýº¹ÀûÀ¸·Î ²È¾Æ¾ß ÇÒ ¶§, 1¼¼ ¹Ì¸¸¿¡¼­ ÀÓÆļ±¿°À» ¾ÎÀ» ¶§ÀÌ´Ù.)
    • ¼¼¹ø°. Àß ÀϾÁö ¾Ê´Â °÷¿¡¼­ÀÇ °¨¿°. (Àß ÀϾÁö ¾Ê´Â °÷ÀÇ °¨¿°À̶õ °£, ³ú °°Àº °÷¿¡¼­ ³ó¾çÀÌ »ý±â´Â °ÍÀ» ¸»ÇÑ´Ù.)
    • ³×¹ø°. ÈçÇÏÁö ¾ÊÀº ¿øÀαÕ. (ÈçÇÏÁö ¾ÊÀº ¿øÀαÕÀ¸·Î Aspergillus spp, Serratia marcescens, Nocardia Spp, Pseudomonas cepacia µî°ú °°Àº ±Õ¿¡ °¨¿°.)
    • ´Ù¼¸¹ø°. ÈçÈ÷ ¾Î´Â ±ÕÀε¥ ¸Å¿ì ½É°¢ÇÏ°Ô ÁøÇà ÇÒ ¶§.

 

¼±Ãµ¼º ¸é¿ª°áÇÌ ÁúȯÀº ´ÙÀ½°ú °°ÀÌ ºÐ·ùÇÑ´Ù.

  • ¸²ÇÁ°è Áúȯ(Lymphoid Lineage)
    • B-¼¼Æ÷ Áúȯ
    • T-¼¼Æ÷ Áúȯ
    • Combined B- and T- Cell Disease
  • °ñ¼ö°è Áúȯ(Myeloid lineage)
  • ´ë½Ä¼¼Æ÷°è Áúȯ
  • È¿¼Ò°è Áúȯ(Complement system)
  • ¼öÀÇ ¸¹°í ÀûÀ½ : Leukopenia : Neutropenia, Lymphopenia, Leukocytosis

    °¡Àå ÈçÇÑ ¸é¿ª°è ÁúȯÀº ¡®B ¼¼Æ÷ Áúȯ¡¯ÀÌ´Ù.

  ±×¸² µé¾î°¥ Àå¼Ò

ÀÇ½ÉµÉ ¶§ ¾î¶² °Ë»ç¸¦ ½ÃÇàÇØ¾ß ÇÏ´ÂÁö¸¦ ¾Ë¾ÆµÎ´Â °ÍÀº Áß¿äÇÏ´Ù.

  • ¹®Áø, ÁøÂû, °¡Á··Â, °ú°Å·Â
  • °Ë»ç½Ç ¼Ò°ß
    • CBC, PBS, ESR
    • Absolute lymphocyte count :R/O T-cell defect
    • Absolute neutrophil count : R/O Neutropenia, R/O LAD
    • Ç÷¼ÒÆÇ ¼öÄ¡ : R/O WAS
    • Howell-Jolly bodies : R/O Aspenia
    • ESR : R/O Chronic bacterial or fungal infection
  • Screening Test for B-cell defects
    • IgG, IgA, IgM, IgE
    • Isohemagglutinins
    • Tetanus, Diphtheria, Haemophilus influenzae, Pneumococci Ç×ü°¡
  • Screening Test for T-cell defects
    • Absolute lymphocyte count
    • Candida albicans ID Test
  • Screening Test for phagocytic cell defect
    • Absolute neutrophil count
    • Respiratory burst assay
  • Screening Test for Complement deficiency
    • CH50 

 

Ç×ü¸¦ ¸ø ¸¸µé¸é B ¼¼Æ÷ ±â´É Àå¾Ö¸¦ ÀǹÌÇÑ´Ù.

    B¼¼Æ÷ ±â´ÉÀÇ °¡Àå Áß¿äÇÑ °ÍÀº Ç×ü¸¦ ¸¸µå´Â °ÍÀÌ´Ù. ±×·¯¹Ç·Î Ç×ü°¡ ¾ø´Ù¸é B¼¼Æ÷¿¡ ¹®Á¦°¡ ÀÖ´Ù°í »ý°¢Çصµ µÈ´Ù. Áٱ⼼Æ÷¿¡¼­ ¹Ì¼º¼÷ B¼¼Æ÷, ¼º¼÷B ¼¼Æ÷·Î ºÐÈ­ÇÏ¿© Ç׿ø¿¡ ³ëÃâµÇ¸é ÇüÁú¼¼Æ÷·Î º¯È¯ÇÏ¿© ´Ù¾çÇÑ Ç×ü¸¦ ¸¸µé¾î ³½´Ù.

  • Ç׿ø°ú °ü·Ã¾øÀÌ ºÐÈ­½Ã±â(Antigen independent stage) ; 7 – 14 wk ;
    • immunoglobuline gene arrangement
    • Pro-B cell ; CD 34, CD 10
    • Pre-Pre B cell ; CD 34, CD 10, CD 19, CD 40
    • Pre B cell ; cytoplasmic u heavy chain
    • Immature B cell ; sIgM
    • Mature B cell ; sIgM, sIgD, CD 23
  • Antigen dependent stages
    • Five immunoglobulins isotypes
    • Immunoglobulin subclass

    ÀÌ ºÐÈ­°úÁ¤ Áß ¹®Á¦°¡ ÀÖ´Ù¸é B ¼¼Æ÷ ÁúȯÀÌ µÈ´Ù. Ç×ü¸¦ ¸¸µéÁö ¸øÇÏ´Â º´Àº ´ÙÀ½°ú °°´Ù. 

 

¼±Ãµ¼º ¸é¿ª°áÇÌÁõÀ¸·Î óÀ½ Bruton Áúº´ÀÌ ¹àÇôÁ³´Ù.

    ÀÌ´Â ¹üÀú°¨¸¶±Û·ÎºÒ¸°ÁõÀ̶ó°í ÇÑ´Ù. ¸» ±×´ë·Î °¨¸¶±Û·ÎºÒ¸°ÀÌ »ý»êµÇÁö ¾Ê´Â °ÍÀÌ´Ù. Brutons º´Àº 1952³â¿¡ ºê·çÅæÀÌ ¹ßÇ¥ÇÏ¿´´Ù.

    ¾î¸° ¼Ò³âÀ̾ú´Âµ¥ 3¹øÀÇ º¼°Å¸®¿Í 4³â µ¿¾È 19¹øÀÇ ¼¼±Õ¼º °¨¿°¿¡ °É¸®¾ú´Ù. Æó·Å¼º ±¸±ÕÀÌ Ç÷¾×¹è¾ç°Ë»ç¿¡¼­ 10¹øÀ̳ª ³ª¿Ô´Ù. Æó·Å¼º ±¸±Õ ¿¹¹æÁ¢Á¾À» ÇÏ¿´À½¿¡µµ ¶Ç´Ù½Ã Æó·Å¼º ±¸±Õ¿¡ °¨¿°µÇ¾î ¾ÆÀÌ°¡ ¿¹¹æÁ¢Á¾À» ÇÏ¿©µµ Æó·Å¼º ±¸±Õ Ç×ü¸¦ »ý»êÇÏÁö ¸øÇÑ´Ù°í ÀǽÉÇÏ¿´´Ù. ¾ÆÀÌ Ç÷¾×À» »Ì¾Æ¼­ Àü±â¿µµ¿À» ÇØ º¸¾Ò´õ´Ï ¾ËºÎ¹ÎÀº Á¦´ë·Î ³ª¿ÔÀ¸³ª °¨¸¶ ±Û·ÎºÒ¸°Àº ³ª¿ÀÁö ¾Ê¾Ò´Ù. À̸¦ È®ÀÎÇÑ ºê·çÅæÀº ÇÑ´Þ¿¡ Çѹø¾¿ Ç÷¾× ³» ±Û·ÎºÒ¸°À» Åõ¿©ÇÏ¿´°í ÀÌÈÄ ¾ÆÀÌ´Â 14°³¿ù µ¿¾È °¨¿°¿¡¼­ ÀÚÀ¯·Î¿ï ¼ö ÀÖ¾ú´Ù. ÀÌÈÄ ±×ÀÇ Ä¡·á°¡ Ç¥ÁØÄ¡·á°¡ µÇ¾ú´Ù.

    À¯ÀüÇÐÀÌ ¹ß´ÞÇϸ鼭 1990³â´ë¿¡ ¿°»öü X¿¡ ¹®Á¦°¡ ÀÖÀ½À» È®ÀÎÇÏ¿´´Ù. À̸¦ q22, Btk(Bruton tyrosine kinase)¶óÇÑ´Ù.

    À̸¦ Á¤¸®Çϸé

    1. X ¿°»öü ¹®Á¦À̹ǷΠ³²ÀÚ ¾ÆÀ̵鿡°Ô »ý±â¸ç
    2. ¾ö¸¶·ÎºÎÅÍ Ç×ü°¡ ¾ø¾îÁö±â ½ÃÀÛÇÏ´Â 6-9°³¿ù »çÀÌ¿¡ Áõ»óÀÌ ³ªÅ¸³ª±â ½ÃÀÛÇϸç
    3. encapsulated pathogen ¿¡ ÀÇÇØ °í»ýÇÑ´Ù.
    4. chronic entero virus meningitis °¨¿°Àº Ư¡ÀûÀÌ´Ù.
    5. À¯¼¼Æ÷ºÐ¼®±â(flow cytometry)·Î Mature B ¼¼Æ÷¸¦ È®ÀÎÇÒ ¼ö ¾ø´Ù.

                                                                      

ÀÏ°ú¼º Àú°¨¸¶±Û·ÎºÒ¸° Ç÷Áõ (Transient Hypogammaglobulinemia of infancy, THI)

    ¸é¿ª±Û·ÎºÒ¸° ±â´É °Ë»ç¸¦ ½ÃÇàÇÏ¿´À» ¶§ ¸ðµÎ Á¤»óÀÌ´Ù. ´ÜÁö ¾ÆÀÌÀÇ Ig »ý¼º¼Óµµ°¡ Á¤»óº¸´Ù ´À¸®±â¿¡ ³ªÅ¸³­ °ÍÀÌ´Ù. Á¤»ó º¯ÀÌ¶ó ¿©°ÜÁø´Ù. Ãà³óÁõÀ̳ª ÁßÀÌ¿°ÀÌ Áõ°¡µÇ¾î ³ªÅ¸³ªÁö¸¸ »ý¸í°ú´Â °ü°è¾ø´Ù. Ç×»ýÁ¦·Î Ä¡·á°¡ ÀßµÇ¸ç ³ªÀÌ°¡ µé¸é ȸº¹µÈ´Ù. ¸é¿ª±Û·ÎºÒ¸°À» Åõ¿©ÇÒ ÇÊ¿ä´Â ¾ø´Ù. Áø´ÜÀº ¸é¿ª±Û·ÎºÒ¸° ¼öÄ¡·Î È®ÀεȴÙ. 

    ±×¸² µé¾î°¥ Àå¼Ò

 

ºÐ·ùºÒ´ÉÇü ¸é¿ª°áÇÌÁõÀº 6-10¼¼, 26-30¼¼ »çÀÌ¿¡ °¡Àå ¸¹ÀÌ ¹ß»ýÇÑ´Ù.

    ºÐ·ùºÒ´ÉÇü ¸é¿ª°áÇÌÁõ(°øÅë °¡º¯Çü ¸é¿ª°áÇÌÁõ, Common Variable Immunodeficiency ; CVID)Àº ÈçÇÏ°í ÀÓ»óÀûÀ¸·Î ÀǹÌÀÖ´Â ¼±Ãµ¼º ¸é¿ª°áÇÌÁõÀÌ´Ù. XLA¿Í ´Þ¸® Mature B ¼¼Æ÷´Â º¸À̳ª Plasma ¼¼Æ÷·Î ºÐÈ­°úÁ¤ÀÇ ¹®Á¦°¡ ¹ß»ýÇÑ º´ÀÌ´Ù. Ç×ü¸¦ ¸¸µéÁö ¸øÇϱ⿡ »ó±âµµ, Çϱ⵵ È­³ó¼º °¨¿°À» Ư¡À¸·Î ÇÑ´Ù. XLA¿Í Â÷ÀÌÁ¡Àº ¼ºº°¿¡ °ü°è¾øÀÌ ³ªÅ¸³ª¸ç, Áõ»óÀÌ Á»´õ ´Ê°Ô ¹ßÇöµÇ°í Áõ»óÀÌ Á»´õ ¾àÇÏ´Ù.

    Áø´Ü : ¸é¿ª±Û·ÎºÒ¸°ÀÌ ³ªÀÌ¿¡ ºñÇØ ¶³¾îÁ® ÀÖ´Ù. B ¼¼Æ÷°¡ º¸ÀδÙ.

    Ä¡·á ; IVIG

    ÃßÀû : ÀÚ°¡¸é¿ªÁúȯ, Á¾¾ç ¹ß»ýÀ²ÀÌ ³ô´Ù. ÃßÀû°üÂûÀÌ ÇÊ¿äÇÏ´Ù.

 

¼±ÅÃÀû IgA ¸é¿ª °áÇÌÁõÀº IgA°¡ 5 mg/dL ÀÌÇÏÀ̸é Áø´ÜÇÑ´Ù.

    ¸é¿ª ±Û·ÎºÒ¸° Áß Æ¯¼öÇÑ °Í¸¸ °¨¼ÒÇÑ °æ¿ì°¡ ÀÖ´Ù. ÀÌÁß IgA¸¸ ¶³¾îÁø °æ¿ì¸¦ ¼±ÅÃÀû IgA ¸é¿ª°áÇÌÁõ(°í¸³¼º IgA °áÇÌÁõ, Selective IgA deficiencies)¶ó ÇÑ´Ù. 400¸í Áß 1¸í Á¤µµÀÇ ºñÀ²·Î °¡Àå ¸¹´Ù. ±×·¯³ª ±¹³» ¹ßº´À²Àº ¾ÆÁ÷ ¸ð¸¥´Ù. ´ëºÎºÐ ÀÓ»óÀûÀ¸·Î Á¤»óÀ̸ç ÀÓ»ó Áõ»óÀº ÁÖ·Î »ó±âµµ °¨¿°ÀÌ´Ù. ÁßÁõ ±âµµ°¨¿°À̳ª Çϱ⵵ °¨¿°È¯ÀÚ´Â ÀϹÝÀûÀ¸·Î IgG2 ¶Ç´Â IgG4 °áÇÌÀ̳ª CVID¸¦ ÀǽÉ

    Áø´Ü : B ¼¼Æ÷¸¦ °üÂû °¡´É, IgG, IgM Á¤»ó, Ç׿ø¿¡ ´ëÇÑ Ç×ü »ý¼º´É Á¤»ó, IgA 5 mg/dL ÀÌÇÏ

    Ä¡·á : ¸é¿ª±Û·ÎºÒ¸°(IgG)´Â µµ¿òÀÌ ¾ÈµÈ´Ù. ¿Ö³ÄÇϸé ÀÌ·± ȯÀÚµéÀº ´Ù¸¥ ¸é¿ª±Û·ÎºÒ¸°Àº »ý»êÇÒ ¼ö Àֱ⠶§¹®ÀÌ´Ù. ÀÚ°¡ Ç×ü¸¦ Àß ¸¸µç´Ù. IgA¿¡ ´ëÇÑ ´Ù¸¥ Ç×ü°¡ 44%³ª ¸¸µé¾îÁö±â¿¡ Ä¡·á¸¦ À§ÇØ ¸é¿ª±Û·ÎºÒ¸°À» ÁÖ¸é ¾Æ³ªÇʶô½Ã½º À§ÇèÀÌ ÀÖ´Ù. ¼öÇ÷ÀÌ ÇÊ¿äÇÑ °æ¿ì ¼¼Ã´ÇÑ ÀûÇ÷±¸¸¦ ÁÖ¾î¾ß ÇÑ´Ù.(Washed packed erythrocyte)

 

IgG ¾Æ±º °áÇÌÁõÀº IgG2 °áÇÌÀÌ °¡Àå ÈçÇÏ´Ù.

    IgG´Â IgG1, IgG2, IgG3 ¿Í IgG4 °¡ ÀÖ´Ù. IgG ¾ÆÇü(subclass)°¡ Çϳª ÀÌ»óÀÌ Á¤»ó ¼öÄ¡ÀÇ 2 SD ÀÌÇÏÀÎ °æ¿ì Áø´Ü °¡´ÉÇÏ´Ù. Àüü IgG ¼öÄ¡´Â Á¤»óÀ̰ųª Áõ°¡µÇ¾î ÀÖ´Ù. IgG2°¡ °áÇ̵Ǿî Àִ ȯÀÚÀÇ ´ëºÎºÐÀº IgA µµ °áÇ̵Ǿî ÀÖ´Ù. Ä¡·á´Â ¿¹¹æÀû Ç×»ýÁ¦, IVIG ÀÇ È¿°ú´Â ¾ÆÁ÷ È®½ÇÇÏÁö ¾Ê´Ù.

 

B¼¼Æ÷ ÁúȯÀÇ Áø´ÜÀº Ç×ü¸¦ ¸¸µé¾ú´ÂÁö¸¦ È®ÀÎÇÏ¸é µÈ´Ù.

    1. Ç÷¾×ÇüÀÇ Ç×ü ¼öÄ¡¸¦ È®ÀÎ. ´ëºÎºÐ IgM. À̸¦ Isohemagglutinin À̶ó ÇÑ´Ù.
    2. ½ÇÁ¦·Î Ç׿ø ÅõÀÔÇÏ¿© Ç×ü »ý¼º´É È®ÀÎ
    3. ¿¹¸¦µé¾î Diphtheria, Tetanus ¿¹¹æÁ¢Á¾À» ÁØ ÈÄ Ç×ü °¡°¡ º¯È­ÇÏ´Â °¡¸¦ È®ÀÎ. Á¢Á¾ Àü Ç×ü°¡¸¦ È®ÀÎÇÏ°í Á¢Á¾ 2ÁÖ ÈÄ ´Ù½Ã Ç×ü°¡¸¦ È®ÀÎÇÏ¿© Ç×ü¸¦ ¸¸µé¾ú´ÂÁö È®ÀÎÇÏ´Â ¹æ¹ý. ÀÌ´Â ´ëºÎºÐ IgG »ý¼º ´É·Â ÃøÁ¤. Polysaccharide »ý»ê ´É·ÂÀ» º¸±â À§ÇØ Pneuococcus ¿¹¹æÁ¢Á¾À» ½ÃÇàÇϱâ Àü°ú ½ÃÇà 3ÁÖ ÈÄ¿¡ Ç×ü°¡¸¦ ºñ±³ÇÏ¿© Ç×ü »ý»ê ´É·ÂÀ» È®ÀÎÇÕ´Ï´Ù. º»¿ø¿¡¼­´Â °£¿° ¿¹¹æÁ¢Á¾ ÈÄÀÇ Ç×ü°¡·Î IgG Ç×ü°¡ È®ÀÎ(ÀÌ·¸°Ô ÇßÀ» ¶§ÀÇ ¹®Á¦Á¡Àº ?)

    3. ÃÖÁ¾ »ý»ê¹°ÀÎ ¸é¿ª±Û·ÎºÒ¸°À» Á¤·®.

      IgG, IgA, IgM, IgE, IgD Á¤·®ºÐ¼®.  À§ °Ë»ç´Â IgG ³ª IgMÀÇ »ý»ê ´É·ÂÀ» º¸´Â °Í. ±×·¯¹Ç·Î IgA °áÇÌÁúȯÀÚ³ª THI, Protein losing state ´Â °¨º°Çϱ⠾î·Æ´Ù.

    4. IgG subclass ¼öÄ¡¸¦ È®ÀÎ

      ¸é¿ª±Û·ÎºÒ¸° ¼öÄ¡°¡ Á¤»óÀÌÁö¸¸ ¸é¿ª°áÇÌÀÌ ÀÖ´Â ¾ÆÀ̵éÀº ¼ö ÀÖ´Ù. IgG2´Â polysaccharide Ag ¿¡ ´ëÇÑ Ç×ü·Î Áß¿äÇÏ´Ù.

    5. B ¼¼Æ÷ ºÐÈ­°¡´É ¿©ºÎ¸¦ ÆľÇÇÑ´Ù.(Stimulation test)

      B ¼¼Æ÷¸¦ pokeweed mitogen(¶Ç´Â CD40) °ú »çÀÌÅäÄ«ÀÎÀ¸·Î ÀÚ±ØÀ» Çϸé ÇüÁú¼¼Æ÷·Î º¯È­Çϸ鼭 Ç×ü¸¦ »ý»êÇϴµ¥ ÀÌ·¯ÇÑ ´É·ÂÀÌ ÀÖ´ÂÁö È®ÀÎÇÏ´Â °ÍÀÔ´Ï´Ù.

    6. B¼¼Æ÷ Ç¥ÇöÇüÀ» È®ÀÎÇÑ´Ù.

      À¯¼¼Æ÷ ºÐ¼®±â È°¿ë  

    7. À§ °Ë»ç ÈÄ, ¸é¿ª±Û·ÎºÒ¸° ¼öÄ¡´Â ¶³¾îÁ® ÀÖÀ¸³ª ±â´É °Ë»ç°¡ Á¤»óÀ̸é ?

      ´Ü¹éÁúÀÌ ¸ö ¾È ¾îµð¿¡¼­ »õ°í ÀÖ´ÂÁö È®ÀÎ. Protein losing State.

                         

Èä¼± ¹«Çü¼ºÁõÀÇ Ã¹ Áõ»óÀº ÀúÄ®½·Ç÷ÁõÀÌ´Ù.

    ¼¼Æ÷¼º ¸é¿ª°áÇÌÁõÀº ü¾×¼º ¸é¿ª°áÇÌÁõº¸´Ù ÁßÁõÀ̸ç, Á¶±â¿¡ ³ªÅ¸³ª¸ç, ¹ÙÀÌ·¯½º, Áø±Õ, ¿øÃæ·ù, °áÇÙ±Õ, ±×¶÷ À½¼º±Õ¿¡ ÀÚÁÖ °¨¿°µÈ´Ù. ¾àµ¶È­ »ý¹é½ÅÀº ±Ý±âÀÌ´Ù. ¼¼Æ÷¼º ¸é¿ª °áÇÌÁõ¿¡´Â ´ÙÀ½°ú °°Àº Áúº´ÀÌ ÀÖ´Ù.

    • Thymic hypoplasia(DiGEORGE Syndrome)
    • Hyper IgM syndrome

    DiGeorge syndrome Àº ºÎ°©»ó¼± È£¸£¸ó °áÇÌ¿¡ ÀÇÇØ »ýÈÄ 24½Ã°£ À̳»¿¡ ÀúÄ®½·ÁõÀÌ ¿À°í °æ·ÃÀ̳ª Åן´Ï°¡ ¿Â´Ù. ÀÌ¿Ü¿¡µµ PDA, VSD, TOF ¿Í °°Àº ¼±Ãµ¼º ½ÉÀå Áúȯ, ÀÌ°³º¯Çü, ¾È±¸ °Ý¸®, ¾î±¸, ÇϺΠ¼Ò¾Ç µî ºñ Á¤»óÀûÀÎ ¾ó±¼ ¸ð¾çÀÌ º¸ÀδÙ. ¿ÏÀüÇü°ú ºÎºÐÇüÀÌ ÀÖ´Ù. 

    Hyper IgM syndrome Àº CD40L ÀÌ ¹ßÇö¾ÈµÇ¾î Ç×ü ÀüȯÀÌ ¾ÈµÇ´Â º´ÀÌ´Ù.

 

ÀӽŠ12ÁÖ¿¡ T¼¼Æ÷´Â ¹ÝÀÀÀ» ÇÑ´Ù.

  • ÀӽŠ10 ÁÖ ; Positive selection/Negative selection
  • ÀӽŠ12 ÁÖ ; Emigrate from thymus to the spleen, lymph nodes, appendix
    • Response to plant lectins(phytohemagglutinin[PHA] and concanavalin A
    • [Con A]) and to allogeneic cells
  • ÀӽŠ20 ÁÖ ; antigen – binding T cell 
  • CD ratio
    • Cord blood ; CD 4/CD 8 = 3.5 - 4/1
    • In children and adult ; 1.5 – 2/1
  • T cell subset
    • Th1 ; IL-2, IFN-gamma
      • Promoting cytotoxic T cell
      • Delayed hypersensitivity
    • Th2 ; IL-4, IL-5, IL-6 and IL-13
      • Promote B-cell responses
      • Allergic sensitization
  • T cell function
    • Cord blood T cell ; capacity to responsd two mitogen(PHA, Con A)
    • At birth ; capacity to antigen-specific T cell response(BCG)
    • First few months of life ; unrecognized severe T cell defect ; routinely irradiate all blood products given young infants

 

¼¼Æ÷¼º ¸é¿ª°áÇÌÁõ Áø´ÜÀÇ ÇÙ½ÉÀº Ç׿ø¿¡ ´ëÇÑ Áö¿¬¼º ÇǺιÝÀÀ °Ë»çÀÌ´Ù.

    1. °¡Àå ÁÁÀº °Ë»ç´Â ¸»ÃÊÇ÷¾×¿¡¼­ ¸²ÇÁ±¸ ¼ö¸¦ È®ÀÎ

      ¹éÇ÷±¸ ¸²ÇÁ±¸°¡ 1500/mm3 ÀÌ»óÀ̸é ÀÏ´Ü T ¼¼Æ÷ ÀÌ»óÀÌ ¾Æ´Ò °ÍÀ̶ó »ý°¢.

    1. Áö¿¬¼º ÇǺιÝÀÀ °Ë»ç¸¦ ½ÃÇà
    2. ¿ø¸®´Â ÇÇÇϳ»¿¡ Ç׿øÀ» Áý¾î³Ö°í Ç׿ø¿¡ ´ëÇØ ´ë½Ä¼¼Æ÷¿Í T¼¼Æ÷ÀÇ ¹ÝÀÀÀ¸·Î »çÀÌÅäÄ«ÀÎÀÌ ºÐºñµÇ°í ÇǺΰ¡ ¹ßÀûÀ» º¸ÀÌ´Â °ÍÀ» È®ÀÎÇÏ´Â °ÍÀÔ´Ï´Ù. Ç׿øÀ¸·Î »ç¿ëÇÒ ¼ö ÀÖ´Â °ÍÀ¸·Î´Â Candida, PPD, Tetanus toxoid µîÀÌ ÀÖ´Ù. ¾ç¼º ¹ÝÀÀÀ̶õ Ç׿øÀ» ÁÖÀÔÇÑ ÈÄ 48-72½Ã°£ ÈÄ 10mm ÀÌ»óÀÇ ¹ßÀû ¹× °æ°áÀÌ ÀÖ´Ù

    3. ÈäºÎ X¼± »çÁø¿¡¼­ Èä¼±ÀÇ À¯¹«, Å©±â ÃøÁ¤
    4.  

    5. Flow Cytometry·Î T ¼¼Æ÷ ¼ö¸¦ È®ÀÎ  
      1. CD3 55 – 80%  

        CD4/CD8 1.5 -2 : 1.  

    6. ½ÇÇè°ü³»¿¡¼­ T ¼¼Æ÷ÀÇ ¹ÝÀÀ È®ÀÎ
    7. T ¼¼Æ÷¿¡ mitogenÀ¸·Î ÀÚ±ØÀ» ÇÏ¸é ºÐ¿­À» ÇÑ´Ù. À̸¦ ÃøÁ¤.

    8. T ¼¼Æ÷¿¡¼­ ºÐºñÇÏ´Â »çÀÌÅäÄ«ÀÎÀ» ÃøÁ¤.

 

³ªÀÌ¿¡ µû¶ó ´ÜÇÙ¼¼Æ÷ÀÇ ºÐÆ÷°¡ ´Ù¸£´Ù.

 

º¹Çո鿪°áÇÌÁúȯÀº °ñ¼öÀ̽ÄÀ» ÇÏÁö ¾ÊÀ¸¸é Ãʱ⿡ »ç¸ÁÇÑ´Ù.

    º¹Çո鿪°áÇÌÁúȯ(combined Immune deficiency)Àº ´ÙÀ½°ú °°´Ù.

    • Severe combined immunodeficiency(SCID)
    • Combined immunodeficiency(CID)
    • Wiskott-Aldrich Syndrome
    • Ataxia-Telangiectasia
    • Hyper-IgE Syndrome

    Molecular defects

      Most autosomal recessive chromosome

      X linked immunodeficiency

      • X linked immunodeficiency with Hyper IgM
      • X linked lymphoproliferative syndrome
      • X linked agammaglobulinemia
      • X linked SCID
      • Wiskott Aldrich syndrome
      • nuclear factor kappa B essential modulator(NEMO)

      autosomal dominant

      • combined ID ; Adenosine deaminase(ADA)
      •               purine nucleside phosphorylase(PNP)
      • ZAP-70 mutation
      • Jak3 mutation

 

¹éÇ÷±¸°¡ ¿°ÁõºÎÀ§·Î À̵¿ÇÏ´Â µ¥¿¡´Â ¸¹Àº ¹°ÁúµéÀÌ °ü¿©ÇÑ´Ù.

    ¿°ÁõºÎÀ§¿¡¼­ ´Ù¾çÇÑ »çÀÌÅäÄ«Àΰú ÄɸðÄ«ÀÎÀº ÇÊ¿ä·Î ÇÏ´Â ¼¼Æ÷µéÀ» ºÒ·¯µéÀδÙ. ºÎ¸§À» ¹ÞÀº ¹éÇ÷±¸´Â ¿°ÁõºÎÀ§ Ç÷°ü¿¡¼­ Rolling, Activation, Adhesion, Migration °úÁ¤À» °ÅÃÄ Ç÷°ü³»ÇǸ¦ Åë°úÇÑ´Ù. ÀÌ À̵¿ Áß¿¡ ÇÑ°¡Áö ¹®Á¦°¡ ¹ß»ýÇÏ¸é °¨¿°¿¡ ÀûÀýÇÏ°Ô ´ëó¸¦ ÇÏÁö ¸øÇÑ´Ù.

    ±×¸²µé¾î°¥ Àå¼Ò

    •   ¹éÇ÷±¸ Á¢ÇÕ ÀÌ»ó (Adhesion Abnormalities)

    • ¹éÇ÷±¸ Á¢ÇÕ¹°Áú °áÇÌÁõ (Leukocyte adhesion deficiency 1:LAD-1)
    • ¹éÇ÷±¸ Á¢ÇÕ¹°Áú °áÇÌÁõ (Leukocyte adhesion deficiency 2: LAD-2)
    • Neutrophil actin dysfunction

    •   Disorders of Chemotaxis

    • Defects in the generation of chemotactic signals
    • Intrinsic defects of he neutrophil such as in the newborn
    • direct inhibition of neutrophil mobility by drugs
    • Immune complexes
    • Hyperimmunoglobulin E syndrome

     

½Ä¼¼Æ÷ ÀÛ¿ëÀº È£Áß±¸, È£»ê±¸, ´Ü±¸¼¼Æ÷(monocyte)¿Í ´ë½Ä¼¼Æ÷¿¡¼­ ÀϾ´Ù.

    À̶§ Ç׿øÀ» ó¸®ÇÏ´Â °úÁ¤Àº µÎ°¡Áö·Î ³ªÅ¸³­´Ù. Phagosome°ú Lysosome ÀÌ °áÇÕÇÏ¿© Phagolysosome ÀÌ µÇ¸ç À̶§ MHC II ·Î Ç׿øÀÇ ÀϺÎÀÎ Peptide°¡ TCR¿¡ Ç¥ÇöµÈ´Ù.

    •   Å»°ú¸³ ÀÛ¿ë Àå¾Ö(Degranulation Abnormalities)

    • Chediack-Higashi syndrome
    • Specific granule deficiency

    •   »ì±ÕÀÛ¿ë Àå¾Ö(Defects of Microbicidal Activity)

    • ¸¸¼º À°¾ÆÁ¾ Áúȯ : Chronic granulomatous disease(CGD)
    • G6PD deficiency
    • Myeloperoxidase deficiency
    • Deficiencies of glutathione reductase and glutathione synthetase

      ±×¸² µé¾î°¥ Àå¼Ò

»ì±ÕÀå¾ÖÀÇ Àå¾Ö´Â Respiratory burst assay·Î È®ÀÎÇÑ´Ù.

    Respiratory burst¶õ Ç׿øÀÌ phagocytosis µÇ¾î Oxidase¿¡ ³ëÃâµÇ¸é O2°¡ ¼ÒºñµÇ¾î Reactive Oxygen Intermediates¸¦ ¸¸µé¾î ³½´Ù. ÀÌ·¸°Ô O2°¡ ¼ÒºñµÇ´Â °ÍÀ» ¸»ÇÑ´Ù. À̸¦ ½ÄÀ¸·Î ³ªÅ¸³»¸é,

      NADPH + 2O2 + H+ -> NADP+ + 2O2- + 2H+

    ½Ä¼¼Æ÷ÀÇ »ì±ÕÀÛ¿ëÀº superoxide·Î ºÎÅÍ ½ÃÀ۵ȴÙ.

 

¸¸¼ºÀ°¾ÆÁ¾Àº Catalase¸¦ ¸¸µå´Â ¼¼±Õµé¿¡ Ãë¾àÇÏ´Ù.

    Catalase¶õ 2H2O2 -> 2H2O + O2¸¦ Ã˸ÅÇÏ´Â È¿¼ÒÀÌ´Ù. »óó°¡ »ý°åÀ» ¶§ ¼Òµ¶Á¦·Î ¾²´Â °ú»êÈ­¼ö¼Ò°¡ 3% H2O2ÀÌ´Ù. H2O2´Â »ì±ÕÁ¦ÀÌ´Ù. H2O2´Â Cytotoxic ÇÏ¿© Çø±â¼ºÀ» Á¦¿ÜÇÑ °ÅÀÇ ¸ðµç »ý¹°¿¡´Â Catalase°¡ Á¸ÀçÇÑ´Ù. ¹Ì»ý¹°ÀÌ Ä§ÀÔÇϸé Host´Â Phagosome ³»¿¡¼­ H2O2 ¸¦ ¸¸µé¾î ¹Ì»ý¹°À» Á×ÀÌ°íÀÚ ÇÑ´Ù. ¸¸¼ºÀ°¾ÆÁ¾(chronic granulomatosis disease)Àº NADPH oxidase ±â´ÉÀÇ Àå¾Ö·Î ȯ¿øÀÌ µÇÁö¾Ê¾Æ H2O2 ¸¦ ¸¸µéÁö ¸øÇÑ´Ù. ±×·¯¹Ç·Î Àû°Ô »ý»êµÈ H2O2´Â catalase ¾ç¼º±ÕÀ» Á×ÀÌÁö ¸øÇÏ°í º´ÀÌ »ý±ä´Ù.

    Catalase¸¦ ¸¸µå´Â ±Õ¿¡´Â ´ëºÎºÐÀÇ G(-) bacteria, S. aureus, Klebsiella, Pseudomonas, E. coli, Mycobacterium µîÀÌ ÀÖ´Ù. Áï, ÀÌ·¯ÇÑ ±Õ¿¡ ÀÚÁÖ °¨¿°µÈ´Ù. Catalase À½¼ºÀÎ ±ÕÀº Strepcocci, Pneumococci °¡ ÀÖ´Ù. ÀÌ·¯ÇÑ ±Õ¿¡´Â °¨¿°ÀÌ µÇÁö ¾Ê´Â´Ù.

    ±×¸² µé¾î°¡´Â Àå¼Ò

    Àá±ñ¸¸ ! CGD¸¦ °ª½Î°Ô Áø´ÜÇÏ´Â ¹æ¹ý(¹Ï°Å³ª ¸»°Å³ª)

    ȯÀÚ ÇǺο¡ »óó¸¦ ³½ ÈÄ °ú»êÈ­¼ö¼Ò¸¦ ¹Ù¸¥´Ù. ÀûÇ÷±¸°¡ Æı«µÇ¸é¼­ catalase°¡ ºÐºñµÇ¾î °ø±â¹æ¿ïÀÌ ¹ß»ýÇϸé catalase °¡ ȯÀÚ Ç÷¾×¿¡ ÀÖ´Â °ÍÀÌ°í »ê¼Ò°¡ ¹ß»ýÇÏÁö ¾ÊÀ¸¸é catalase¸¦ »ý»êÇÏÁö ¸øÇÏ´Â °ÍÀÌ´Ù.

 

CGD Áø´ÜÀº NBT(nitroblue tetrazolium) ȯ¿ø°Ë»ç ¶Ç´Â DHR test ·Î ÇÑ´Ù.

    NBT °Ë»ç´Â Park µîÀÌ 1968³â "Infection and nitroblue-tetrazolium reduction by neutrophils. A diagnostic acid." Lancet¿¡ ¹ßÇ¥ÇÏ¿© »ç¿ëµÇ°í ÀÖ´Ù.

    NBT ¹æ¹ý ; WBC¿¡ NBT ½Ã¾àÀ» ³Ö´Â´Ù. NBT ½Ã¾àÀº »ö±òÀÌ ¾ø´Ù. Áß¼º±¸(È£»ê±¸, ´Ü±¸¼¼Æ÷)´Â NBT ½Ã¾àÀ» Phagocytosis ÇÏ¿© Phagosome ³»·Î ²ø¾îµéÀδÙ. ¸¸ÀÏ H2O2 ¸¦ ¸¸µé¾î³½´Ù¸é NBT ½Ã¾àÀº ȯ¿øÇÏ¿© FormazanÀ» Çü¼ºÇϸé(Ǫ¸¥»ö) ¾ç¼ºÀÌ´Ù.  ¸¸ÀÏ H2O2 ¸¦ ¸¸µéÁö ¸øÇÑ´Ù¸é NBT ½Ã¾àÀº º¯È­°¡ ¾ø´Ù.(À½¼º)   

    NBT ȯ¿ø°Ë»ç´Â Á¤·®°Ë»ç°¡ ¾ÈµÇ°í Ç¥ÁØÈ­¸¦ ½ÃÅ°¸é False À½¼º¹ÝÀÀÀÌ ³ª¿Ã ¼ö ÀÖ´Ù. ¶ÇÇÑ ¸¹Àº Ç÷¾×ÀÌ ÇÊ¿äÇÏ´Ù. ÀÌ·¯ÇÑ ´ÜÁ¡À» º¸¿ÏÇÑ °Ë»ç°¡ À¯¼¼Æ÷ ºÐ¼®±â¸¦ È°¿ëÇÑ DHR(Dihydrorodamine 123) °Ë»ç°¡ ÀÖ´Ù.

    DHRÀº dyeÀÌ¸ç ¼¼Æ÷¸¦ ½±°Ô Åë°úÇÒ ¼ö ÀÖ°í H2O2 ¶Ç´Â O2-¸¦ ¸¸³ª¸é ¹ß±¤À» ÇÑ´Ù. À̸¦ À¯¼¼Æ÷ ºÐ¼®±â·Î ã¾Æ³»´Â °ÍÀÌ´Ù.

    ±×¸² µé¾î°¡´Â Àå¼Ò

    ±×¸²¼³¸í) À§ ±×¸²Àº Á¤»óÀÎÀÌ°í ¾Æ·¡ ±×¸²Àº CGD ȯÀÚÀÌ´Ù. ÁÂÃøÀº PMA·Î ÀÚ±Ø ÀüÀÌ°í ¿ìÃøÀº PMA·Î ÀÚ±Ø ÈÄÀÇ ¹ß»öÀ» º» °ÍÀÌ´Ù. Á¤»óÀο¡¼­´Â PMA·Î ÀÚ±ØÀ» ÁÖ¸é ¹ß»öÀÌ µÇÁö¸¸ CGD ȯÀÚ´Â H2O2 ¶Ç´Â O2-¸¦ ¸¸µéÁö ¸øÇϱ⿡ ¹ß»öÀÌ µÇÁö ¾Ê´Â´Ù.

     

    NADPH oxydase´Â ´Ù¼¸°¡Áö component·Î ÀÌ·ç¾îÁ® ÀÖ´Ù. Resting »óÅÂÀÇ È£Áß±¸´Â ¼¼Æ÷Áú³»¿¡ (p40phox,p47 phox °ú p67 phox)°¡ º¹ÇÕü¸¦ Çü¼ºÇÏ°í ÀÖ´Ù. ´Ù¸¥ µÎ°³ÀÇ component´Â p22phox ¿Í gp91phox ÀÌ´Ù. ÀÌ´Â ºÐºñÇü vesicleÀ̳ª granule ÀÇ ¸·¿¡ Á¸ÀçÇϸç cytochrome b558(cyt b558)·Î ¾Ë·ÁÁø º¹ÇÕü¸¦ Çü¼ºÇÏ°í ÀÖ´Ù.  ¼¼Æ÷°¡ Ç׿ø¿¡ ÀÇÇØ È°¼ºÈ­µÇ¸é ¼¼Æ÷Áú³»¿¡ ÀÖ´ø º¹ÇÕü°¡ ¼¼Æ÷¸·À¸·Î À̵¿ÇÏ°í cyt b558 °ú ÇÔ²² NADPH oxydase ¸¦ Çü¼ºÇÑ´Ù. ¸¸ÀÏ À§ ´Ù¼¸ ¿ä¼Ò Áß ÇÑ°³ÀÇ À߸øÀÌ ÀÖ´Ù¸é superoxide¸¦ ¸¸µé¾î ³»Áö ¸øÇÑ´Ù. CGD ȯÀÚÀÇ 60%´Â X-linked CGDÀÌ´Ù. ÀÌ´Â gp91phox ÀÇ µ¹¿¬º¯ÀÌ¿¡ ÀÇÇÑ´Ù. 40%´Â autosomal recessive ÀÌ´Ù. ÀÌ´Â ¿°»öü 7¹ø¿¡ ÀÖ´Â p47 phox ¿°»öü 1¹ø¿¡ ÀÖ´Â p67 phox , ¿°»öü 16¹ø¿¡ ÀÖ´Â p22phox ÀÇ µ¹¿¬º¯ÀÌ¿¡ ÀÇÇÑ´Ù. CGD ȯÀÚ´Â º¸Åë 5¼¼ Àü¿¡ Áø´ÜµÈ´Ù.

    °ñ¼öÀ̽ÄÀÌ Ä¡·áÀÌ´Ù. °ñ¼öÀÌ½Ä Àü¿¡´Â ¿¹¹æÇ×»ýÁ¦ Åõ¿©¿Í INF-g¸¦ Åõ¿©ÇÑ´Ù. Supportive care.

 

º¸Ã¼ °áÇÌÀº CH50·Î Screen ÇÑ´Ù.

    º¸Ã¼ °³°³ÀÇ ¼ººÐ¿¡ ´ëÇÑ À¯ÀüÀûÀÎ °áÇÌÀÌ º¸°íµÇÁö¸¸ µå¹°´Ù. ¼±º°°Ë»ç´Â CH50 ÀÌ´Ù. Ç÷ûÀÇ ÀûÇ÷±¸ ¿ëÇØ ´É·ÂÀ» ÃøÁ¤ÇÏ´Â °ÍÀÌ´Ù.

 

¸é¿ª°áÇÌÀº ¿ÜºÎ¿Í Â÷´ÜÀ» ÅëÇؼ­ Ä¡·áµÉ ¼ö ÀÖ´Ù.

    1971³â David Vetter III¶ó´Â ¾ÆÀÌ°¡ Severe combined Immunodeficiecy(SCID)¶ó´Â Áúº´À» °¡Áö°í ž´Ù. ¾ÆÀ̸¦ »ì¸®±â À§ÇÏ¿© ¹«±Õ Çöó½ºÆ½ »óÅ¿¡¼­ »ìµµ·Ï ÇÏ¿´°í ¸Åü´Â ÀÌ ¾ÆÀ̸¦ ¡®Çöó½ºÆ½ bubble ¼Ò³â¡¯À̶ó°í ĪÇÏ¿´°í 1976³â¿¡´Â ¹Ì±¹¿¡¼­ TV¿¡¼­ ¡®Bubble boy¡¯¶ó´Â ½Ãû¹°À» ³»³õ¾Ò´Ù. ¾ÆÀÌ´Â »îÀ» ÀÌ°÷¿¡¼­ À¯ÁöÇÏ¿´°í 1984³â °ñ¼öÀ̽ÄÀ» ½ÃÇàÇÏ¿´´Ù. ÀÌÈÄ Ã³À½À¸·Î ¿ÜºÎ·Î ³ª¿Ô´Ù. »õ·Ó°Ô °ñ¼öÀ̽ÄÀ» ½ÃÇàÇÑ ÈÄ Epstein-Barr ¹ÙÀÌ·¯½º¿¡ °¨¿°µÇ¾î Burkitt¡¯s Lymphoma ·Î »ç¸ÁÇÏ¿´´Ù.

 

IVIGÀº ÀÓ»ó¿¡¼­ ¸¹ÀÌ »ç¿ëµÇ°í ÀÖ´Ù.

    IV Immunoglobluin(IVIG, Á¤¸ÆÁÖ»ç¿ë ÀÎÇ÷û ¸é¿ª±Û·ÎºÒ¸°)

    Gene Therapy

    Bone marrow transplantation

 

 

 

 

 

 

 

 
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