์˜์•ฝํ’ˆ๊ณผ ์งˆ๋ณ‘์ •๋ณด/์งˆ๋ณ‘๏ฝœํฌ๊ท€์งˆํ™˜
[ํฌ๊ท€์งˆํ™˜] ์œ ์ „์„ฑ ๋น„๊ตฌ์ƒ์ ํ˜ˆ๊ตฌ์„ฑ ์šฉํ˜ˆ์„ฑ ๋นˆํ˜ˆ/ Hereditary Nonspherocytic Hemolytic Anemia ์ฆ์ƒ,์ง„๋‹จ,์น˜๋ฃŒ
2019.08.30
ํŠน์ง• ์œ ์ „์„ฑ์šฉํ˜ˆ๋นˆํ˜ˆ์˜ ์›์ธ์€ ํฌ๊ฒŒ 3๊ฐ€์ง€๋กœ ๋‚˜๋‰จ. 1. ์ ํ˜ˆ๊ตฌ ๋ง‰์˜ ์ด์ƒ (์ ํ˜ˆ๊ตฌ ๋ชจ์–‘ ์ด์ƒ์œผ๋กœ ํ˜ˆ๊ด€์„ ์ง€๋‚˜๊ฐ€๋‹ค๊ฐ€ ๊นจ์ง. ๋ถˆ๊ทœ์น™ํ•˜๊ณ  ๋น„๊ตฌํ˜•์˜ ๋ชจ์–‘. ์‰ฝ๊ฒŒ ํ„ฐ์ง) 2.ํ—ค๋ชจ๊ธ€๋กœ๋นˆ์˜ ์ƒ์„ฑ์žฅ์•  3. ์ ํ˜ˆ๊ตฌ ๋‚ด ํšจ์†Œ ์ด์ƒ (๋Œ€ํ‘œ์ ์œผ๋กœ G6PD ๋ถ€์กฑ, Pyruvate kinase ๋ถ€์กฑ์ด ์žˆ์Œ) ==> ํšจ์†Œ ์ด์ƒ์ด ์žˆ์œผ๋ฉด ์ ํ˜ˆ๊ตฌ๊ฐ€ ์ƒ์กด๊ธฐ๊ฐ„์„ ๋ชป์ฑ„์šฐ๊ณ  ํ„ฐ์ง. ๊ตญ๋‚ด 1997๋…„~2006๋…„ ์กฐ์‚ฌ ๊ฒฐ๊ณผ G6PD ๊ฒฐํ• ์‚ฌ๋ก€ 5๊ฑด, Pyruvate kinase ๊ฒฐํ• 1๊ฑด, Enolase ๊ฒฐํ• 1๊ฑด ๋ณด๊ณ ๋จ. ์˜ํ–ฅ๋ถ€์œ„: ์ฒด๋‚ด๋กœ๋Š” ํ˜ˆ๊ด€(ํ˜ˆ์•ก), ์ฒด์™ธ๋กœ๋Š” ํ”ผ๋ถ€์— ์˜ํ–ฅ์„ ์คŒ. ๊ด€๋ จ์งˆํ™˜๋ช…: ์„ ์ฒœ์„ฑ ๋น„๊ตฌํ˜•์ ํ˜ˆ๊ตฌ์„ฑ ์šฉํ˜ˆ์„ฑ๋นˆํ˜ˆ (Congenital Nonspherocytic Hemolytic Anemia) ์œ ์ „์„ฑ ๋น„๊ตฌ์ƒํ˜•์ ํ˜ˆ๊ตฌ์„ฑ ์šฉํ˜ˆ์„ฑ๋นˆํ˜ˆ (H..