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Ectodermal Dysplasia ȯÀÚ¿¡¼­ÀÇ º¸Ã¶ Ä¡Çè·¹

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Abstract

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Ectodermal Dysplasia(¿Ü¹è¿± Çü¼º ºÎÀüÁß)Àº ´ëº°ÇÏ¿© Hypo -ȤÀº Anhidrotic(Àú¹ßÇѼº
ȤÀº ¹«¹ßÇѼº) ectodermal dysplasia, Hidrotic(¹ßÇѼº) ectodermal dysplasia,
EEC(Ectrodactyly - ectodermal dysplasia - clefting) syndrome, Rapp - hodgkin
ectodermal dysplasia, Robinson type ectodermal dysplasia·Î ±¸ºÐµÈ´Ù. ÀÌ Áß Hypohidrotic
ectodermal dysplasia°¡ °¡Àå Àß ¾Ë·ÁÁ® ÀÖÀ¸¸ç, ÀÌ´Â X-linked recessive traitÀÇ À¯Àü¼ºÀ»
°¡Áø´Ù°í Çϸç, ³²¼º¿¡¼­ ÈÙ¾À ¸¹ÀÌ ³ªÅ¸³­´Ù°í ÇÑ´Ù. ÇÏÁö¸¸ ÀáÀ缺ÀÎ ¿©¼º¿¡¼­ ectodermal
dysplasiaÀÇ Á¦ Áõ»óÀÌ ³ªÅ¸³¯ ¼öµµ ÀÖ´Ù. µû¶ó¼­ X - linked semidominant inheritance¶ó°í
µµ ÇÑ´Ù.
Hypohidrotic ectodermal dysplasiaÀÇ ÀÓ»óÁõ»óÀ¸·Î´Â hypotrichosis(°¨¸ðÁõ),
hypohidrosis(°¨ÇÑÁõ), ±×¸®°í oligodontia(ÇÌÄ¡Áõ)ÀÌ °¡Àå Ư¡ÀûÀ̸ç, ÀÌ¿Ü¿¡ asteatosis(¹«
ÇÇÁöÁõ), temperature elevation, dry skin, depressed bridge of nose, protrusion of the lips,
defective mental development µîÀÌ ³ªÅ¸³¯ ¼ö ÀÖ´Ù.
O1igodontia´Â ectodermal dysplasiaÀÇ Æ¯Â¡ÀûÀÎ ÀÓ»óÁõ»óÀ¸·Î Ä¡¾Æ°á¼Õ ¹× Ä¡¾ÆÇüÅÂÀÇ ÀÌ
»ó, À¯Ä¡ÀÇ ¸¸±âÀÜÁ¸, Ä¡Á¶°ñ ¼ºÀåÀÇ °á¿©, free way spaceÀÇ Áõ°¡, ÇÏ¾Ç Àüµ¹, °úµµÇÑ ¼öÁ÷
ÇÇ°³ µîÀÌ ±¸°­³» ¼Ò°ßÀ¸·Î ³ªÅ¸³­´Ù.
º»°í¿¡¼­´Â º»°ú¿¡ ³»¿øÇÑ hypohidrotic ectoder-mal dysplasiaȯÀÚ¿¡¼­ oligodontia¿¡ ´ë
ÇÑ º¸Ã¶Ä¡·á Áõ·Ê¸¦ º¸°íÇÏ°íÀÚ ÇÑ´Ù.

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