์˜์•ฝํ’ˆ๊ณผ ์งˆ๋ณ‘์ •๋ณด/์งˆ๋ณ‘๏ฝœํฌ๊ท€์งˆํ™˜
[์งˆ๋ณ‘์ •๋ณด] ๋นˆํ˜ˆ์˜ ์ฆ์ƒ, ์›์ธ, ์ง„๋‹จ๊ณผ ์น˜๋ฃŒ
2019.08.30
๋นˆํ˜ˆ์ด๋ž€? ํ˜ˆ์•ก๋‚ด์˜ ์ ํ˜ˆ๊ตฌ ์ˆ˜์˜ ๊ฐ์†Œ๋ฅผ ์˜๋ฏธํ•œ๋‹ค. ์ด ๋•Œ ํ˜ˆ์ƒ‰์†Œ(ํ—ค๋ชจ๊ธ€๋กœ๋นˆ) ์ˆ˜์น˜๋กœ ํŒ๋‹จํ•จ. ๋‚จ์ž ์„ฑ์ธ์€ ํ—ค๋ชจ๊ธ€๋กœ๋นˆ ๋†๋„๊ฐ€ 13g/dl์ดํ•˜, ์—ฌ์ž ์„ฑ์ธ์˜ ๊ฒฝ์šฐ 12g/dl ์ดํ•˜์ผ ๋•Œ ๋นˆํ˜ˆ๋กœ ์ง„๋‹จํ•œ๋‹ค. ๋นˆํ˜ˆ์˜ ์ฆ์ƒ ๊ฒฝ๋ฏธํ•œ ๊ฒฝ์šฐ์—๋Š” ๋ณ„๋‹ค๋ฅธ ์ฆ์ƒ์ด ์—†๋Š” ๊ฒฝ์šฐ๊ฐ€ ๋งŽ์Œ. ๊ธ‰์„ฑ ๋นˆํ˜ˆ์€ ๋Œ€๊ฐœ ์‹ฌํ•œ ์ถœํ˜ˆ๋กœ ๋ฐœ์ƒํ•˜๊ณ , ์ด ๊ฒฝ์šฐ ์‡ผํฌ์— ์˜ํ•œ ์ฆ์ƒ์ด ์ฃผ๋กœ ๋‚˜ํƒ€๋‚จ. ๋งŒ์„ฑ๋นˆํ˜ˆ์˜ ๊ฒฝ์šฐ ํ˜ˆ์ƒ‰์†Œ ์ˆ˜์น˜๊ฐ€ ์ƒ๋‹นํžˆ ๋–จ์–ด์ง€๊ธฐ์ „์—๋Š” ์ฆ์ƒ์ด ์—†๋Š” ๊ฒฝ์šฐ๊ฐ€ ๋งŽ์Œ. (ํ˜ˆ์ƒ‰์†Œ ์ˆ˜์น˜๊ฐ€ 7-8g/dl ๊นŒ์ง€๋Š” ์ฆ์ƒ์ด ์—†์„ ์ˆ˜ ์žˆ๊ณ , ๋นˆํ˜ˆ์ด ๋งŽ์ด ์ง„ํ–‰๋˜์—ˆ์Œ์—๋„ ์ง„๋‹จ๋˜์ง€ ์•Š๋Š” ๊ฒฝ์šฐ๋„ ์žˆ์Œ) ๋งŒ์„ฑ์ธ ๊ฒฝ์šฐ ๋Œ€ํ‘œ์ ์œผ๋กœ๋Š” ์šด๋™์‹œ ํ˜ธํก๊ณค๋ž€, ์–ด์ง€๋Ÿฌ์›€์ฆ, ๋‘ํ†ต, ์‹ฌ๊ณ„ํ•ญ์ง„์ฆ, ํ”ผ๊ณคํ•จ, ์ˆ˜๋ฉด์žฅ์• , ์„ฑ์š• ๊ฐํ‡ด, ๊ธฐ๋ถ„ ์žฅ์• , ์ง‘์ค‘๋ ฅ ๊ฐํ‡ด ๋“ฑ์ด ๋‚˜ํƒ€๋‚  ์ˆ˜ ์žˆ์Œ. ํ˜„๊ธฐ์ฆ์ด ๋‚˜ํƒ€๋‚œ..
์˜์•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..