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[Angiodysplasia Ç÷°üÀÌÇü¼ºÁõ°ú hereditary hemorrhagic telangiectasia] - ðû


1. Introduction to angiodysplasia

Angiodysplasia´Â ¸Ö¸®¼­ º¸¸é ºÓÀº Á¡ÀÌÁö¸¸, Á¢±ÙÇϸé Ç÷°üÀÌ È®ÀåµÈ ÇüÅ°¡ ¸íÈ®È÷ °üÂûµÈ´Ù. ´ëºÎºÐ ÇÏ¾á ¶ì(halo)¸¦ °¡Áö°í ÀÖ´Ù. Çϳª¸¸ º¸ÀÌ´Â °æ¿ì°¡ ¸¹Áö¸¸ ´Ù¹ß¼ºµµ ÀûÁö ¾Ê´Ù. ¹ß»ý±âÀüÀº ±ÙÀ°ÃþÀ» Åë°úÇÏ´Â ºÎÀ§ÀÇ ¾Ð·ÂÀÌ ³ô¾ÆÁ® Á¡¸·ÇÏ Ç÷°üÀÌ È®ÀåµÈ °ÍÀ¸·Î ¼³¸íµÇ°í ÀÖ´Ù.

Pathogenesis of angiodysplasia (Boley et el. Am J Gastroenterol 1977;72:650)


2. Gastric angiodysplasia

°ËÁø ³»½Ã°æ µµÁß ÃâÇ÷À» º¸¿´À¸³ª irrigation Çϸ鼭 °üÂûÇÏ¿´´õ´Ï ÀúÀý·Î ¸Ü¾úÀ½. ¸î ³â ÈÄ °üÂû¿¡¼­µµ º¯È­°¡ ¾ø¾úÀ½.

Hemoglobin 3.7 ±îÁö ³»·Á°¬´ø ¸¸¼º ºóÇ÷ ȯÀÚ

¿ì¿¬È÷ ¹ß°ßµÈ angiodysplasia

NBI·Î °üÂûÇϸé Ç÷°üÀº °ËÀº »öÀ¸·Î º¸ÀδÙ. Halo´Â ´õ ¸í·áÇØÁø´Ù.

ÀÓ»óÀûÀ¸·Î ÃâÇ÷À» º¸¿´´ø Áõ·Ê. ¸Å¿ì ºÓÀº »öÀÌ°í halo°¡ ¶Ñ·ÇÇÏÁö ¾Ê¾Ò´Ù.

¸Å¿ì ³ÐÀº angiodysplasia. Á¶Á÷°Ë»ç: submucosal tortuous blood vessels and mucosal congestion strongly suggestive of angiodysplasia

(2015) ºóÇ÷·Î ³»¿øÇÑ ¿©¼º. È®´ë³»½Ã°æ ¼Ò°ß

Multiple small bowel andiodysplasia. ½ÊÀÌÁöÀåºÎÀ§°¡ ½ÉÇÏ¿´±â ¶§¹®¿¡ »óºÎ³»½Ã°æÀ¸·Î argon plasma coagulation ½ÃÇàÇÔ

Halo°¡ ¶Ñ·ÇÇÏÁö ¾ÊÀº ÀÛÀº angiodysplasia Near Focus ¿µ»ó


3. Small bowel angiodysplasia

Small bowel angiodysplasia (capsule endoscopy)

Small bowel angiodysplasia (capsule endoscopy)


4. Colonic angiodysplasia

Edoxaban µå½Ã´Â hematochezia ȯÀÚ¿¡¼­ angiodyplasia ¹ß°ßÇÏ¿© Áï½Ã APC ¼ÒÀÛ¼ú


5. Hereditary hemorrhagic telangiectasia (HHT, Osler-Weber-Rendu disease)

¸Å¿ì º¸±â Èûµç ÁúȯÀÔ´Ï´Ù. Autosomal dominant·Î ALK-1 À¯ÀüÀÚÀÇ º¯ÀÌ°¡ ÁÖ¿øÀÎÀÔ´Ï´Ù. ¾ÆÁÖ ¾î¸± ¶§ºÎÅÍ ÄÚÇÇ°¡ Àæ´Ù°¡ 10¼¼Âë µÇ¸é Àý¹ÝÀÌ À§Àå°ü ÃâÇ÷À» º¸ÀÔ´Ï´Ù. ÇϺÎÀ§Àå°üº¸´Ù´Â »óºÎÀ§Àå°ü¿¡ º´¼Ò°¡ ½ÉÇϹǷΠhematocheziaº¸´Ù´Â melena°¡ ÈçÇÕ´Ï´Ù. À§Àå°ü Á¡¸· »Ó¸¸ ¾Æ´Ï¶ó ÀÔ¼ú, ±¸°­Á¡¸·, ºñ°­Á¡¸· µî¿¡¼­µµ telangiectasia°¡ µ¿¹ÝµË´Ï´Ù. 80%ÀÇ È¯ÀÚ°¡ °¡Á··ÂÀÌ ÀÖ½À´Ï´Ù.

- Autosomal dominant, ALK-1 mutation
- Recurrent epistaxia in childhood
- By age 10, GI bleeding in 50 %
- Severe hemorrhage is unusual before the fourth decade.
- Peak incidence of severe hemorrhage in the sixth decase.
- Melena >> hematochezia: telengiectasia in stomach and small intestine >> colon
- Telangiectasias in lips, oral, nasopharingeal membranes, tongue, and periungal areas, and liver
- Family history in 80 %
- Gross appearance: millet seed-size, cherry-red, smooth hillocks
- Mainly involves capillaries and venules >> arterioles: irregular, ectatic, tortuous blood spaces lined by a delicate single layer of endothelial cells and supported by a fine layer of fibrous connective tissue. No elastic laminar or muscular tissue in the vessels, so they cannot contract -- tend to bleed.
- In contrast to the thinned venules of angioectasias, venules are abnormally thick in HHT
- Tx. Estrogen, aminocaproic acid, endoscopic thermal ablation (APC etc), surgery

À§Àå°ü ÃâÇ÷·Î ³»¿øÇÑ 63¼¼ ³²¾Æ¿¡¼­ hereditary hemorrhagic telangiectasia (HHT, Osler-Weber-Rendu disease)¸¦ Áø´ÜÇÏ¿© º¸°íÇÑ °¡ÃµÀÇ´ë Áõ·Ê¸¦ ¼Ò°³ÇÕ´Ï´Ù (World J Gastroenterol 2012).

»ï¼º¼­¿ïº´¿ø ¼øȯ±â³»°ú¿¡¼­ Áø´ÜÇÏ°í Ä¡·áÇÑ hereditary hemorrhagic telangiectasia (HHT, Osler-Weber-Rendu disease) Áõ·Ê series¸¦ ¼Ò°³ÇÕ´Ï´Ù (J Korean Med Sci 2009).

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© ÀÏ¿ø³»½Ã°æ±³½Ç ¹Ù¸¥³»½Ã°æ¿¬±¸¼Ò ÀÌÁØÇà. EndoTODAY Endoscopy Learning Center. Lee Jun Haeng