Glycogen storage Diseases(±Û¸®ÄÚ°Õ Ä§ÂøÁõ)

1.Áúº´ÀÇ ÀϹÝÀûÀÎ °³¿ä
- ´ç¿øÀÌ Ã¼³»¿¡ ÃàÀûµÊÀ¸·Î½á ³ªÅ¸³ª´Â ´ë»çÀÌ»ó À¯ÀüÁúȯÀ¸·Î¼­, ±× Á¾·ù¸¦ ¿©·¯ °¡Áö·Î ³ª´©¾î º¼ ¼ö ÀÖ´Â µ¥, °¢°¢ ±× ¹ß°ßÀÚÀÇ À̸§À» µû¼­ Von Gierkeº´, Pompeº´, Coriº´, Andersonº´, McArdleº´, Hersº´ µîÀ¸·Î ¸í¸íÇÑ´Ù. ¸ðµÎ »ýü³» ź¼öÈ­¹°´ë»çÀÇ Áß¿äÇÑ È¸·Î ±âÀüÀÎ ´ç¿ø´ë»ç¿¡ ÀÌ»óÀÌ »ý°Ü¼­ ¹ßº´ÇÏ´Â ÁúȯÀ¸·Î, ±× °á¼ÕÈ¿¼Ò¿¡ µû¶ó¼­ ´Ù¾çÇÑ Áõ»óÀ» ³ªÅ¸³»°Ô µÈ´Ù.
- ´ë°³ Autosomal recessive ¹æ½ÄÀ¸·Î À¯ÀüµÈ´Ù.
- °£Á¾´ë°¡ ¸¹Àº °æ¿ì¿¡ ³ªÅ¸³ª´Â °ÍÀ¸·Î ¾Ë·ÁÁ® ÀÖ´Ù.
- °á¼ÕÈ¿¼Ò¿¡ µû¶ó ¿©·¯ Á¾·ùÀÇ ÇüÀÌ ÀÖÀ¸³ª Å©°Ô °£Çü°ú ±ÙÇüÀ¸·Î ºÐ·ùÇÒ ¼ö ÀÖ´Ù.

      °£Çü : °£Á¾´ë, ÀúÇ÷´ç ¹ßÀÛ, Ȳ´Þ, º¹¼öÁõ»óÀÌ ³ªÅ¸³­´Ù.
      ±ÙÇü : ±Ù·ÂÀúÇÏ, ½±°Ô ÇǷΰ¨À» ´À³¤´Ù
¡ß ´ç¿øº´ÀÇ °¢Çü
ºÐ·ù Çü °á¼ÕÈ¿¼Ò ´ç¿ø ÃàôÀå±â º°¸í
°£Çü
±ÙÇü
°£Çü, ±ÙÇü °£
Çü
±ÙÇü
°£Çü
±ÙÇü
°£Çü
¥°Çü
¥±Çü
¥²Çü
¥³Çü
¥´Çü
¥µÇü
¥¶Çü
¥·Çü

glucose-6-phosphatase ¥á-glucosidase
Å»ºÐÁöÈ¿¼Ò
ºÐÁöÈ¿¼Ò
±Ù phosporylase
°£ phosporylase
±Ù phosphofructokinase °£
phosphorylase
°£, ½ÅÀå
°£, ±ÙÀ°, ½ÉÀå
°£, ±ÙÀ°
°£, ºñ
°ñ°Ý±Ù
°£
°ñ°Ý±Ù
°£, ±ÙÀ°
Von Gierke º´
Pompe º´
Cori º´ Anderson º´
McArdle º´ Hers º´
Taruiº´
Lewis º´

2. ÀÓ»óÀû ÁõÈÄ
- Von Gierkeº´ÀÇ °æ¿ì : °øº¹½Ã ÀúÇ÷´ç, »êÇ÷Áõ, °íÀ¯»ê Ç÷Áõ µîÀÌ ³ªÅ¸³­´Ù.
- Pompeº´ÀÇ °æ¿ì : ½ÉÀåÀÇ Á¾´ë·Î ÀÎÇÑ ½ÉÀüµµ ÀÌ»ó, ±Ù·ÂÀúÇÏ, °Ç¹Ý»çÀúÇÏ, cyanosis µîÀÌ ³ªÅ¸³­´Ù.

3.Áø´Ü
- °á¼ÕÈ¿¼Ò¿¡ ´ëÇÑ È¿¼ÒÈ°¼ºÀúÇϸ¦ È®ÀÎÇÑ´Ù.


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