{"id":1494,"date":"2023-04-08T12:54:07","date_gmt":"2023-04-08T05:54:07","guid":{"rendered":"http:\/\/noithathoaphat.in\/ctv\/?p=1494"},"modified":"2023-04-17T10:18:49","modified_gmt":"2023-04-17T03:18:49","slug":"xo-nang-phoi","status":"publish","type":"post","link":"http:\/\/noithathoaphat.in\/ctv\/index.php\/2023\/04\/08\/xo-nang-phoi\/","title":{"rendered":"T\u1eeb c\u01a1 ch\u1ebf cho \u0111\u1ebfn ch\u1ea9n \u0111o\u00e1n b\u1ec7nh x\u01a1 nang ph\u1ed5i"},"content":{"rendered":"

X\u01a1 nang ph\u1ed5i l\u00e0 m\u1ed9t b\u1ec7nh l\u00fd m\u1ea1n t\u00ednh c\u1ee7a h\u1ec7 th\u1ed1ng h\u00f4 h\u1ea5p, g\u00e2y ra s\u1ef1 x\u00e2m nh\u1eadp v\u00e0 t\u1ed5n th\u01b0\u01a1ng c\u00e1c m\u00f4 m\u1ec1m c\u1ee7a ph\u1ed5i. B\u1ec7nh n\u00e0y l\u00e0 k\u1ebft qu\u1ea3 c\u1ee7a m\u1ed9t \u0111\u1ed9t bi\u1ebfn gen v\u00e0 s\u1ebd d\u1ea7n d\u1ea7n l\u00e0m thay \u0111\u1ed5i c\u1ea5u tr\u00fac t\u1ed5 ch\u1ee9c c\u1ee7a ph\u1ed5i c\u0169ng nh\u01b0 c\u00e1c c\u01a1 quan ngo\u00e0i ph\u1ed5i.<\/strong><\/p>\n

X\u01a1 nang ph\u1ed5i l\u00e0 do r\u1ed1i lo\u1ea1n nhi\u1ec5m s\u1eafc th\u1ec3 l\u1eb7n, g\u00e2y ra b\u1edfi \u0111\u1ed9t bi\u1ebfn gen \u0111i\u1ec1u h\u00f2a d\u1eabn ch\u1ea5t xuy\u00ean m\u00e0ng x\u01a1 h\u00f3a nang (Cystic fibrosis transmembrane conductance regulator gene \u2013 CFTR), n\u1eb1m tr\u00ean nhi\u1ec5m s\u1eafc th\u1ec3 s\u1ed1 7, m\u00e0 h\u1eadu qu\u1ea3 l\u00e0 g\u00e2y r\u1ed1i lo\u1ea1n c\u01a1 quan ngo\u1ea1i ti\u1ebft, ch\u1ee7 y\u1ebfu l\u00e0 h\u1ec7 h\u00f4 h\u1ea5p v\u00e0 h\u1ec7 ti\u00eau h\u00f3a.<\/p>\n

 <\/p>\n

\"\"
Minh h\u1ecda t\u1ed5n th\u01b0\u01a1ng x\u01a1 nang ph\u1ed5i<\/figcaption><\/figure>\n

1. NGUY\u00caN NH\u00c2N C\u1ee6A X\u01a0 NANG PH\u1ed4I<\/strong><\/h2>\n

X\u01a1 nang ph\u1ed5i l\u00e0 do \u0111\u1ed9t bi\u1ebfn gen CFTR, m\u1ed9t chu k\u1ef3 AMP \u0111i\u1ec1u h\u00f2a Clorua. Th\u00f4ng th\u01b0\u1eddng, CFTR duy tr\u00ec \u0111\u1ed9 \u1ea9m d\u1ecbch ti\u1ebft c\u1ee7a c\u01a1 quan ngo\u1ea1i ti\u1ebft. Khi CFTR b\u1ea5t th\u01b0\u1eddng l\u00e0m gi\u1ea3m ti\u1ebft Clorua v\u00e0 t\u0103ng h\u1ea5p thu Natri tr\u00ean b\u1ec1 m\u1eb7t c\u1ee7a t\u1ebf b\u00e0o bi\u1ec3u m\u00f4, h\u1eadu qu\u1ea3 t\u0103ng ti\u1ebft d\u1ecbch d\u00e0y trong \u0111\u01b0\u1eddng h\u00f4 h\u1ea5p, xoang, \u1ed1ng t\u1ee5y, \u0111\u01b0\u1eddng m\u1eadt, ru\u1ed9t, tuy\u1ebfn m\u1ed3 h\u00f4i v\u00e0 \u0111\u01b0\u1eddng sinh d\u1ee5c.<\/p>\n

\"\"
Minh h\u1ecda v\u1ec1 \u0111\u1ed9t bi\u1ebfn gen CFRT<\/figcaption><\/figure>\n

2. Bi\u1ec2U HI\u1ec6N L\u00c2M S\u00c0NG<\/strong><\/h2>\n

2.1. T\u1ea1i ph\u1ed5i<\/strong><\/p>\n

    \n
  • Ho kh\u1ea1c \u0111\u1eddm m\u1ee7.<\/li>\n
  • Nhi\u1ec5m tr\u00f9ng xoang \u2013 ph\u1ebf qu\u1ea3n v\u1edbi t\u00e1c nh\u00e2n g\u00e2y b\u1ec7nh \u0111i\u1ec3n h\u00ecnh c\u1ee7a x\u01a1 h\u00f3a nang ph\u1ed5i.<\/li>\n
  • Kh\u00f3 th\u1edf ti\u1ebfn tri\u1ec3n.<\/li>\n
  • \u0110\u1ee3t c\u1ea5p b\u1ec7nh ph\u1ed5i c\u1ea5p t\u00ednh.<\/li>\n
  • Nhi\u1ec5m tr\u00f9ng v\u00e0 vi\u00eam t\u00e1i ph\u00e1t.<\/li>\n
  • Gi\u00e3n ph\u1ebf qu\u1ea3n.<\/li>\n
  • T\u1eafc ngh\u1ebdn \u0111\u01b0\u1eddng d\u1eabn kh\u00ed.<\/li>\n<\/ul>\n

    2.2. Ngo\u00e0i ph\u1ed5i.<\/strong><\/p>\n

      \n
    • Vi\u00eam xoang m\u00e3n t\u00ednh.<\/li>\n
    • Polyp m\u0169i.<\/li>\n
    • Suy dinh d\u01b0\u1ee1ng.<\/li>\n
    • Suy tuy\u1ebfn t\u1ee5y \u2013 thi\u1ebfu vitamin A,D,E,K.<\/li>\n
    • Ph\u00e2n su ru\u1ed9t.<\/li>\n
    • H\u1ed9i ch\u1ee9ng t\u1eafc \u0111\u1ea7u xa ru\u1ed9t.<\/li>\n
    • Xo\u1eafn, l\u1ed3ng ru\u1ed9t v\u00e0 sa tr\u1ef1c tr\u00e0ng.<\/li>\n
    • \u0110\u00e1i th\u00e1o \u0111\u01b0\u1eddng.<\/li>\n
    • X\u01a1 gan v\u00e0 t\u0103ng \u00e1p t\u0129nh m\u1ea1ch c\u1eeda.<\/li>\n
    • S\u1ecfi m\u1eadt v\u00e0 vi\u00eam t\u00fai m\u1eadt.<\/li>\n
    • S\u1ecfi th\u1eadn.<\/li>\n
    • V\u00f4 sinh \u1edf nam (kh\u00f4ng c\u00f3 \u1ed1ng d\u1eabn tinh 2 b\u00ean) v\u00e0 vi\u00eam m\u00e0o tinh ho\u00e0n.<\/li>\n
    • Ch\u1eadm t\u0103ng tr\u01b0\u1edfng, b\u1ec7nh x\u01b0\u01a1ng kh\u1edbp v\u00e0 lo\u00e3ng x\u01b0\u01a1ng.<\/li>\n<\/ul>\n

      3. CH\u1ea8N \u0110O\u00c1N X\u01a0 NANG PH\u1ed4I<\/strong><\/h2>\n

      Ch\u1ea9n \u0111o\u00e1n x\u01a1 ph\u1ed5i khi c\u00f3 \u00edt nh\u1ea5t m\u1ed9t ti\u00eau ch\u00ed c\u1ee7a m\u1ed7i nh\u00f3m \u0111\u1eb7c \u0111i\u1ec3m l\u00e2m s\u00e0ng sau:<\/p>\n

        \n
      • L\u00e2m s\u00e0ng bi\u1ec3u hi\u1ec7n b\u1ec7nh x\u01a1 ph\u1ed5i, ho\u1eb7c<\/li>\n
      • Ti\u1ec1n s\u1eed gia \u0111\u00ecnh c\u00f3 ng\u01b0\u1eddi b\u1ecb b\u1ec7nh, ho\u1eb7c<\/li>\n
      • X\u00e9t nghi\u1ec7m s\u00e0ng l\u1ecdc s\u01a1 sinh d\u01b0\u01a1ng t\u00ednh, v\u00e0<\/li>\n
      • T\u0103ng cao Clorua trong m\u1ed3 h\u00f4i > 60 mmol\/L tr\u00ean hai l\u1ea7n, ho\u1eb7c<\/li>\n
      • S\u1ef1 hi\u1ec7n di\u1ec7n c\u1ee7a hai \u0111\u1ed9t bi\u1ebfn gen g\u00e2y b\u1ec7nh \u1edf CFTR, ho\u1eb7c<\/li>\n
      • X\u00e9t nghi\u1ec7m b\u1ea5t th\u01b0\u1eddng \u0111i\u1ec7n th\u1ebf qua bi\u1ec3u m\u00f4 m\u0169i.<\/li>\n<\/ul>\n

        X\u01a1 ph\u1ed5i th\u01b0\u1eddng \u0111\u01b0\u1ee3c ch\u1ea9n \u0111o\u00e1n trong th\u1eddi th\u01a1 \u1ea5u, nh\u01b0ng c\u00f3 kho\u1ea3ng 10% b\u1ec7nh nh\u00e2n \u0111\u01b0\u1ee3c s\u00e0ng l\u1ecdc sau khi 10 tu\u1ed5i.<\/p>\n

        S\u00e0ng l\u1ecdc s\u01a1 sinh t\u0103ng ch\u1ea9n \u0111o\u00e1n s\u1edbm .<\/p>\n

        B\u1ec7nh nh\u00e2n kh\u00f4ng \u0111i\u1ec3n h\u00ecnh c\u00f3 th\u1ec3 thi\u1ebfu c\u00e1c tri\u1ec7u ch\u1ee9ng v\u00e0 d\u1ea5u hi\u1ec7u c\u1ed5 \u0111i\u1ec3n ho\u1eb7c l\u00e0m x\u00e9t nghi\u1ec7m m\u1ed3 h\u00f4i b\u00ecnh th\u01b0\u1eddng.<\/p>\n

        M\u1eb7c d\u00f9 ki\u1ec3u gen c\u00f3 th\u1ec3 c\u00f3 th\u1ec3 h\u1ed7 tr\u1ee3 trong vi\u1ec7c ch\u1ea9n \u0111o\u00e1n, tuy nhi\u00ean m\u1ed9t m\u00ecnh bi\u1ebft n\u00f3 kh\u00f4ng th\u1ec3 kh\u1eb3ng \u0111\u1ecbnh ho\u1eb7c lo\u1ea1i tr\u1eeb ch\u1ea9n \u0111o\u00e1n x\u01a1 h\u00f3a nang ph\u1ed5i v\u00e0 l\u1ef1a ch\u1ecdn ban \u0111\u1ea7u v\u1eabn l\u00e0 x\u00e9t nghi\u1ec7m m\u1ed3 h\u00f4i.<\/p>\n

        4. CH\u1ea8N \u0110O\u00c1N PH\u00c2N BI\u1ec6T<\/h2>\n
          \n
        • R\u1ed1i lo\u1ea1n v\u1eadn \u0111\u1ed9ng th\u1ec3 mi ti\u00ean ph\u00e1t.<\/li>\n
        • H\u1ed9i ch\u1ee9ng Shwachman \u2013 Diamond.<\/li>\n
        • H\u1ed9i ch\u1ee9ng Young.<\/li>\n
        • Thi\u1ebfu Immunoglobulin.<\/li>\n
        • Gi\u00e3n ph\u1ebf qu\u1ea3n v\u00f4 c\u0103n.<\/li>\n
        • Vi\u00eam m\u0169i xoang m\u00e3i t\u00ednh.<\/li>\n
        • Vi\u00eam t\u1ee5y m\u00e3n t\u00ednh v\u00f4 c\u0103n.<\/li>\n<\/ul>\n

          5. C\u1eacN L\u00c2M S\u00c0NG.<\/h2>\n

          X\u00e9t nghi\u1ec7m m\u1ed3 h\u00f4i da v\u1edbi ph\u01b0\u01a1ng ph\u00e1o \u0111\u1ecbnh l\u01b0\u1ee3ng chu\u1ea9n b\u1eb1ng ph\u01b0\u01a1ng ph\u00e1p ion Pilocarpine v\u1eabn l\u00e0 ti\u00eau chu\u1ea9n v\u00e0ng ch\u1ea9n \u0111o\u00e1n b\u1ec7nh x\u01a1 h\u00f3a nang.<\/p>\n

            \n
          • N\u1ed3ng \u0111\u1ed9 clorua m\u00f4i h\u00f4i \u226560 mmol\/L trong hai l\u1ea7n ri\u00eang bi\u1ec7t \u0111\u01b0\u1ee3c ph\u00e1t hi\u1ec7n \u1edf b\u1ec7nh nh\u00e2n c\u00f3 x\u00fac ho\u1ea1 n\u1eb7ng.<\/li>\n
          • K\u1ebft qu\u1ea3 x\u00e9t nghi\u1ec7m m\u1ed3i h\u00f4i ch\u1ec9 ranh gi\u1edbi (n\u1ed3ng \u0111\u1ed9 clorua m\u1ed3i h\u00f4i 40 \u0111\u1ebfn 59 mmol\/L) ho\u1eb7c k\u1ebft qu\u1ea3 kh\u00f4ng r\u00f5 ch\u1ea9n \u0111o\u00e1n trong tr\u01b0\u1eddng h\u1ee3p l\u1ea7n l\u1eb7p l\u1ea1i x\u00e9t nghi\u1ec7m m\u1ed3i h\u00f4i, x\u00e9t nghi\u1ec7m mu\u1ed1i kh\u00e1c bi\u1ec7t \u00e1i \u0111o\u1ea1n th\u1eeba m\u00e3n, x\u00e9t nghi\u1ec7m di truy\u1ec1n, ho\u1eb7c \u00e1p l\u1ef1c m\u00f4i tr\u01b0\u1eddng t\u0103ng cao th\u00ec c\u1ea7n \u0111\u01b0\u1ee3c xem x\u00e9t l\u1ea1i.<\/li>\n
          • X\u00e9t nghi\u1ec7m m\u1ed3i h\u00f4i n\u00ean \u0111\u01b0\u1ee3c th\u1ef1c hi\u1ec7n t\u1ea1i m\u1ed9t trung t\u00e2m chuy\u00ean tr\u1ecb b\u1ec7nh x\u00fac ho\u1ea1 n\u1eb7ng v\u00e0 \u0111\u01b0\u1ee3c \u0111\u00e1nh gi\u00e1 k\u1ebft h\u1ee3p v\u1edbi c\u00e1c th\u00f4ng tin kh\u00e1c c\u1ee7a k\u1ebft qu\u1ea3 x\u00e9t nghi\u1ec7m. N\u1ed3ng \u0111\u1ed9 clorua m\u1ed3i h\u00f4i cao l\u00e0 hi\u1ebfm khi \u0111\u01b0\u1ee3c ph\u00e1t hi\u1ec7n \u1edf nh\u1eefng ng\u01b0\u1eddi kh\u00f4ng m\u1eafc b\u1ec7nh x\u00fac ho\u1ea1 n\u1eb7ng.<\/li>\n<\/ul>\n

            X\u00e9t nghi\u1ec7m di truy\u1ec1n<\/p>\n

              \n
            • Ph\u1ea3i c\u00f3 m\u1eb7t c\u1ee7a hai b\u1ed9 gen l\u1eb7n tr\u00ean alen kh\u00e1c nhau \u0111\u1ec3 ch\u1ea9n \u0111o\u00e1n b\u1ec7nh x\u01a1 h\u00f3a nang.<\/li>\n
            • C\u00e1c bi\u1ebfn th\u1ec3 gen ph\u1ed5 bi\u1ebfn nh\u1ea5t trong b\u1ec7nh x\u01a1 ho\u00e1 nang l\u00e0 x\u00f3a m\u1ea5t m\u1ed9t trong ba nucleotide m\u00e3 h\u00f3a cho phenylalanine (F) t\u1ea1i v\u1ecb tr\u00ed amin 508 (F508del ho\u1eb7c \u0394F508) c\u1ee7a protein CFTR, \u0111\u01b0\u1ee3c t\u00ecm th\u1ea5y trong kho\u1ea3ng 70% c\u00e1c b\u1ec7nh nh\u00e2n.<\/li>\n<\/ul>\n

              Nu\u00f4i c\u1ea5y \u0111\u1eddm<\/p>\n

              X\u00e9t nghi\u1ec7m \u0111\u00e1nh gi\u00e1 k\u00e9m h\u1ea5p thu.<\/p>\n

              C\u00e1c x\u00e9t nghi\u1ec7m nh\u1eb1m \u0111\u00e1nh gi\u00e1 vi\u00eam xoang m\u00e3n t\u00ednh v\u00e0 v\u00f4 sinh.<\/p>\n

              Theo d\u00f5i \u0111i\u1ec7n gi\u1ea3i \u1edf nh\u1eefng ng\u01b0\u1eddi c\u00f3 ti\u1ec1n s\u1eed r\u1ed1i lo\u1ea1n \u0111i\u1ec7n gi\u1ea3i hay suy th\u1eadn.<\/p>\n

              Ch\u1ee5p X-quang ng\u1ef1c cho th\u1ea5y t\u1ed5n th\u01b0\u01a1ng ph\u1ed5i n\u1eb7ng, gi\u00e3n ph\u1ed5i qua\u0303n v\u00e0 l\u1ea5p \u0111\u1ea7y ch\u1ea5t nh\u1ea7y, \u0111\u1eb7c bi\u1ec7t l\u00e0 th\u00f9y tr\u00ean.<\/p>\n

              Ch\u1ee5p CT ng\u1ef1c c\u00f3 \u0111\u1ed9 ph\u00e2n gi\u1ea3i cao c\u00f3 th\u1ec3 h\u1ed7 tr\u1ee3 \u0111\u00e1nh gi\u00e1 b\u1ec7nh nh\u00e2n b\u1ecb b\u1ec7nh s\u1edbm ho\u1eb7c nh\u1eb9 b\u1edfi nh\u1eefng thay \u0111\u1ed5i \u0111\u01b0\u1eddng th\u1edf s\u1edbm.<\/p>\n

              Th\u0103m d\u00f2 ch\u1ee9c n\u0103ng h\u00f4 h\u1ea5p cho th\u1ea5y r\u1ed1i lo\u1ea1n th\u00f4ng kh\u00ed t\u1eafc ngh\u1ebdn v\u1edbi t\u0103ng th\u1ec3 t\u00edch kh\u00ed c\u1eb7n v\u00e0 dung t\u00edch ph\u1ed5i to\u00e0n ph\u1ea7n.<\/p>\n

              Gi\u1ea3m trao \u0111\u1ed5i kh\u00ed ph\u1ebf nang c\u00f9ng v\u1edbi s\u1ef1 thi\u1ebfu oxy v\u00e0 t\u0103ng carbonic.<\/p>\n

              X\u01a1 nang ph\u1ed5i l\u00e0 m\u1ed9t b\u1ec7nh l\u00fd kh\u00e1 ph\u1ed5 bi\u1ebfn, nh\u01b0ng v\u1eabn c\u1ea7n s\u1ef1 \u0111\u00e1nh gi\u00e1 v\u00e0 ch\u1eefa tr\u1ecb k\u1ecbp th\u1eddi \u0111\u1ec3 ng\u0103n ng\u1eeba c\u00e1c bi\u1ebfn ch\u1ee9ng nghi\u00eam tr\u1ecdng v\u00e0 c\u1ea3i thi\u1ec7n ch\u1ea5t l\u01b0\u1ee3ng cu\u1ed9c s\u1ed1ng c\u1ee7a ng\u01b0\u1eddi b\u1ec7nh.<\/p>\n

              B\u1ec7nh nh\u00e2n x\u01a1 nang ph\u1ed5i ho\u1eb7c b\u1ec7nh nh\u00e2n nghi ng\u1edd x\u01a1 nang ph\u1ed5i c\u1ea7n \u0111\u01b0\u1ee3c chuy\u1ec3n \u0111i\u1ec1u tr\u1ecb t\u1ea1i Trung t\u00e2m x\u01a1 nang ph\u1ed5i c\u1ee7a Qu\u1ed1c gia.C\u00e1c x\u00e9t nghi\u1ec7m nh\u01b0 x\u00e9t nghi\u1ec7m clorua m\u1ed3i h\u00f4i v\u00e0 c\u00e1c x\u00e9t nghi\u1ec7m kh\u00e1c \u0111\u01b0\u1ee3c th\u1ef1c hi\u1ec7n t\u1ed1t nh\u1ea5t t\u1ea1i Trung t\u00e2m x\u01a1 nang ph\u1ed5i. M\u1ed9t nh\u00f3m chuy\u00ean gia v\u1ec1 x\u01a1 nang ph\u1ed5i, bao g\u1ed3m b\u00e1c s\u0129, chuy\u00ean gia dinh d\u01b0\u1ee1ng, tr\u1ee3 l\u00fd h\u00f4 h\u1ea5p v\u00e0 ng\u01b0\u1eddi b\u1ec7nh \u0111\u00e3 \u0111\u01b0\u1ee3c th\u00e0nh l\u1eadp \u0111\u1ec3 ch\u0103m s\u00f3c th\u01b0\u1eddng xuy\u00ean cho c\u00e1c b\u1ec7nh nh\u00e2n n\u00e0y.<\/p>\n","protected":false},"excerpt":{"rendered":"

              X\u01a1 nang ph\u1ed5i l\u00e0 m\u1ed9t b\u1ec7nh l\u00fd m\u1ea1n t\u00ednh c\u1ee7a h\u1ec7 th\u1ed1ng h\u00f4 h\u1ea5p, g\u00e2y ra s\u1ef1 x\u00e2m nh\u1eadp v\u00e0 t\u1ed5n th\u01b0\u01a1ng c\u00e1c m\u00f4 m\u1ec1m c\u1ee7a ph\u1ed5i. B\u1ec7nh n\u00e0y l\u00e0 k\u1ebft qu\u1ea3 c\u1ee7a m\u1ed9t \u0111\u1ed9t bi\u1ebfn gen v\u00e0 s\u1ebd d\u1ea7n d\u1ea7n l\u00e0m thay \u0111\u1ed5i c\u1ea5u tr\u00fac t\u1ed5 ch\u1ee9c c\u1ee7a ph\u1ed5i c\u0169ng nh\u01b0 c\u00e1c c\u01a1 quan […]<\/p>\n","protected":false},"author":4,"featured_media":0,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":[],"categories":[20,33],"tags":[3],"yoast_head":"\nT\u1eeb c\u01a1 ch\u1ebf cho \u0111\u1ebfn ch\u1ea9n \u0111o\u00e1n b\u1ec7nh x\u01a1 nang ph\u1ed5i<\/title>\n<meta name=\"description\" content=\"X\u01a1 nang ph\u1ed5i l\u00e0 do r\u1ed1i lo\u1ea1n nhi\u1ec5m s\u1eafc th\u1ec3 l\u1eb7n, g\u00e2y ra b\u1edfi \u0111\u1ed9t bi\u1ebfn gen \u0111i\u1ec1u h\u00f2a d\u1eabn ch\u1ea5t xuy\u00ean m\u00e0ng x\u01a1 h\u00f3a nang\" \/>\n<meta name=\"robots\" content=\"noindex, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<meta property=\"og:locale\" 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