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Coagulation and thrombosis disorders

1. Factor VIII deficiency: hemophilia A

1) º´ÀÎ ¹× ÀÓ»ó¹ßÇö

2) Ä¡·á

Á¶±âÄ¡·á°¡ ´õ È¿°úÀûÀÌ¸ç ºñ¿ëÀÌ Àû°Ô µé°í lifesavingÇÒ¼ö ÀÖ´Ù.

aspirin or aspirin-containing drugsÀº Ç÷¼ÒÆÇ ±â´ÉÀ» ¼Õ»ó½ÃŰ°í ½ÉÇÑ ÃâÇ÷À» ¾ß±âÇÒ¼ö ÀÖ±â

¶§¹®¿¡ ÇÇÇØ¾ß ÇÑ´Ù. COX-2 inhibitors´Â Ç÷¼ÒÆÇ ±â´ÉÀ» ¼Õ»ó½ÃŰÁö ¾Ê±â ¶§¹®¿¡ »ç¿ëÇÒ ¼ö

ÀÖ´Ù.

factor VIII concentratesÀÇ ±¤¹üÀ§ÇÑ »ç¿ëÀº ¶ÇÇÑ ½ÉÇÑ ÇÕº´ÁõÀ» ¾ß±âÇϴµ¥ ÀÌ¿¡´Â viral

hepatitis,chronic liver disease, and AIDS¿Í °°Àº °ÍµéÀÌ ÀÖ´Ù. cryoprecipitate´Â original

volumeÀÇ 1/10¿¡

FFPÀÇ factor VIII acitivity 1/2À» Æ÷ÇÔÇÑ´Ù. ÀÌ´Â º´¿ø ¹× Ç÷¾×ÀºÇà¿¡¼­ °£´ÜÇÏ°Ô ÁغñÇÒ ¼ö

ÀÖ´Ù.

¼ö¼úÀü ¸ðµç Ç÷¿ìº´ ȯÀÚ´Â factor VIII inhibitorÀÇ Á¸Àç¿¡ ´ëÇØ¼­ screeningÇØ¾ß ÇÑ´Ù.

inhibitor°¡ ¾ø´Â Ç÷¿ìº´È¯ÀÚ´Â ¼ö¼úÁ÷Àü¿¡ factor VIII infusionÀ» ¹Þ¾Æ¾ß ÇÏ¸ç ¼ö¼úÈÄ 10-14

Àϰ£ factor VIII levelÀÌ 50%ÀÌ»óµÇ°Ô ¸ÅÀÏ monitorÇØ¾ß ÇÑ´Ù. ȯÀÚ°¡ joint replacement or

major orthopedic surgery¸¦ ½ÃÇàÇÒ¶§´Â wound healing ¹× PT¿¡ ÇÊ¿äÇÑ 3ÁÖ°£ Ä¡·á¸¦ °è¼Ó

ÇÏ¿©¾ß ÇÑ´Ù.

dental procedureÀü¿¡µµ Ä¡·á°¡ ÇÊ¿äÇÏ´Ù. ÃæÄ¡Ä¡·á¶§´Â factor VIII concentrateÀÇ single

infusion°ú EACA¸¦ ÇÏ·ç 4ȸ ¡¿ 3-4Àϰ£ Åõ¿©ÇÑ´Ù. EACA(¥å-aminocaproic acid)´Â oral

secretion¿¡ Á¸ÀçÇÏ´Â plasminogen activators¸¦ ¾ïÁ¦ÇÏ´Â °­·ÂÇÑ antifibrinolytic agentÀ̸ç

oral tissueÀÇ clot formationÀ» ¾ÈÁ¤È­½ÃŲ´Ù. ´ë¿ëÀ¸·Î tranexamic acid(=longer-acting

antifibrinolytic)°¡ ÀÖ´Ù.

2. Fac»ö IX deficiency - Hemophilia B

3. factor XI deficiency

4. other factor deficiencies

contact activation pathway¿¡ °ü¿©ÇÏ´Â Hageman factor(factor XII),

HMWK(high-molecular-weight kininogen), and prekallikreinÀÇ °áÇÌÀº °Ë»ç»ó ÀÌ»óÀ» ³ªÅ¸

³»Áö¸¸ ÀÓ»óÀû ÃâÇ÷Àº ¾ß±âÇÏÁö ¾Ê´Â´Ù. 100ÃÊÀÌ»ó PTTÀÇ ½ÉÇÑ ¿¬Àå¿¡µµ ºÒ±¸Çϰí ÀÌ·¯ÇÑ

ÀÎÀÚÀÇ °áÇÌȯÀÚ´Â Á¤»ó hemostasis¸¦ º¸À̰í

5. Afibrinogenemia and dysfibrinogenemia

6. Factor XIII deficiency and defective fibrin cross-linking

7. vitamin K deficiency