Pai ÁõÈıº 1·Ê
A Case of Pai Syndrome : First Reported Case in Korea
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ÀÌ¿¬°æ(Lee Yeon-Kyung) - °üµ¿´ëÇб³ ÀÇ°ú´ëÇÐ Á¦ÀϺ´¿ø ¼Ò¾Æû¼Ò³â°ú
°í¼±¿µ(Ko Sun-Young) - °üµ¿´ëÇб³ ÀÇ°ú´ëÇÐ Á¦ÀϺ´¿ø ¼Ò¾Æû¼Ò³â°ú
½Å¼Õ¹®(Shin Son-Moon) - °üµ¿´ëÇб³ ÀÇ°ú´ëÇÐ Á¦ÀϺ´¿ø ¼Ò¾Æû¼Ò³â°ú
Abstract
Pai ÁõÈıºÀº ºñ¿ëÁ¾°ú ÇÔ²² Á¤Áß±¸¼ø¿(median cleft of upper lip), ³ú·®ÁÖÀ§ Áö¹æÁ¾(pericallosal lipoma) ¶Ç´Â Áß°£¾Õ Ä¡Á¶µ¹±âÀÇ ¼±Ãµ¼º ¿ëÁ¾(congenital polyp of mid-anterior alveolar process) Áß ÇÑ°¡Áö ÀÌ»óÀÌ µ¿¹ÝµÇ´Â °ÍÀ¸·Î, ¾È±¸ÀÌ»óÀ» µ¿¹ÝÇÒ ¼ö ÀÖÀ¸¸ç, Á¤»ó ¹ß´ÞÀ» Ư¡À¸·Î ÇÏ´Â ¸Å¿ì µå¹® ÁúȯÀÌ´Ù. ÇöÀç±îÁö 36·Ê°¡ º¸°íµÇ¾úÀ¸¸ç ±¹³»¿¡¼´Â º¸°íµÈ ¹Ù°¡ ¾ø´Ù. ÀúÀÚµéÀº ¼±Ãµ¼º ºñ¿ëÁ¾°ú ³ú·® Áö¹æÁ¾°ú ÇÔ²² ³ú·®ÀÇ ºÎºÐ ¹«Çü¼ºÁõÀÌ µ¿¹ÝµÈ Pai ÁõÈıº ȯ¾Æ 1·Ê¸¦ °æÇèÇÏ¿´±â¿¡ º¸°íÇÏ´Â ¹ÙÀÌ´Ù.
Pai syndrome is a rare disorder, first described in 1987. Diagnostic criteria are the presence of the nasal polyp and one of the following: midline cleft lip, congenital polyp of mid-anterior alveolar process, and pericallosal lipoma. Thirty-six cases of Pai syndrome have been described so far. We report 1 case of Pai syndrome accompanied by congenital nasal polyp and callosal lipoma with partial agenesis of corpus callosum, the first time in Korea.
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Pai ÁõÈıº, ºñ¿ëÁ¾, ³ú·®ÁÖÀ§ Áö¹æÁ¾
Pai syndrome, Nasal polyp, Pericallosal lipoma
KMID :
0361720140250010017
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