{"id":29928,"date":"2023-05-06T22:07:23","date_gmt":"2023-05-06T15:07:23","guid":{"rendered":"http:\/\/noithathoaphat.in\/ctv\/?p=29928"},"modified":"2023-05-06T22:07:23","modified_gmt":"2023-05-06T15:07:23","slug":"viem-da-re-va-day-than-kinh-mat-myelin-man-tinh-cidp","status":"publish","type":"post","link":"http:\/\/noithathoaphat.in\/ctv\/index.php\/2023\/05\/06\/viem-da-re-va-day-than-kinh-mat-myelin-man-tinh-cidp\/","title":{"rendered":"Vi\u00eam \u0111a r\u1ec5 v\u00e0 d\u00e2y th\u1ea7n kinh m\u1ea5t myelin m\u1ea1n t\u00ednh (CIDP) \u2013 Nh\u1eefng b\u1eaby trong ch\u1ea9n \u0111o\u00e1n v\u00e0 c\u00e1ch ti\u1ebfp c\u1eadn \u0111i\u1ec1u tr\u1ecb"},"content":{"rendered":"

Vi\u00eam \u0111a r\u1ec5 v\u00e0 d\u00e2y th\u1ea7n kinh m\u1ea5t myelin m\u1ea1n t\u00ednh (CIDP)<\/a> l\u00e0 b\u1ec7nh l\u00fd \u0111\u1eb7c tr\u01b0ng b\u1edfi m\u1ea5t c\u1ea3m gi\u00e1c v\u00e0 li\u1ec7t ti\u1ebfn tri\u1ec3n t\u0103ng d\u1ea7n, th\u01b0\u1eddng \u1ea3nh h\u01b0\u1edfng \u0111\u1ebfn kh\u1ea3 n\u0103ng v\u1eadn \u0111\u1ed9ng c\u1ee7a ng\u01b0\u1eddi b\u1ec7nh c\u0169ng nh\u01b0 trong vi\u1ec7c th\u1ef1c hi\u1ec7n c\u00e1c ch\u1ee9c n\u0103ng s\u1ed1ng h\u00e0ng ng\u00e0y m\u1ed9t c\u00e1ch \u0111\u1ed9c l\u1eadp. V\u00ec kh\u00f4ng c\u00f3 c\u00e1c ch\u1ea5t ch\u1ec9 \u0111i\u1ec3m sinh h\u1ecdc \u0111\u1eb7c hi\u1ec7u, vi\u1ec7c ch\u1ea9n \u0111o\u00e1n CIDP d\u1ef1a ho\u00e0n to\u00e0n v\u00e0o ph\u00e1n \u0111o\u00e1n c\u1ee7a c\u00e1c b\u00e1c s\u1ef9 l\u00e2m s\u00e0ng, tri\u1ec7u ch\u1ee9ng ph\u00e1t hi\u1ec7n khi th\u0103m kh\u00e1m c\u0169ng nh\u01b0 c\u00e1c bi\u1ebfn \u0111\u1ed5i tr\u00ean \u0111i\u1ec7n c\u01a1 v\u00e0 sinh thi\u1ebft g\u1ee3i \u00fd t\u00ecnh tr\u1ea1ng m\u1ea5t myelin c\u1ee7a d\u00e2y th\u1ea7n kinh. Do \u0111\u00f3, b\u1ec7nh nh\u00e2n g\u1eb7p c\u00e1c r\u1ed1i lo\u1ea1n th\u1ea7n kinh kh\u00e1c th\u01b0\u1eddng xuy\u00ean b\u1ecb ch\u1ea9n \u0111o\u00e1n nh\u1ea7m v\u1edbi CIDP v\u00e0 \u0111\u01b0\u1ee3c \u0111i\u1ec1u tr\u1ecb b\u1eb1ng li\u1ec7u ph\u00e1p mi\u1ec5n d\u1ecbch m\u1ed9t c\u00e1ch kh\u00f4ng c\u1ea7n thi\u1ebft.
\nB\u00e0i \u0111\u00e1nh gi\u00e1 n\u00e0y s\u1ebd m\u00f4 t\u1ea3 chi ti\u1ebft c\u00e1ch ti\u1ebfp c\u1eadn m\u1ed9t b\u1ec7nh nh\u00e2n c\u00f3 ch\u1ea9n \u0111o\u00e1n nghi ng\u1edd CIDP, t\u1eeb c\u00e1ch phi\u00ean gi\u1ea3i k\u1ebft qu\u1ea3 \u0111i\u1ec7n c\u01a1 v\u00e0 d\u1ecbch n\u00e3o t\u1ee7y tr\u00ean nh\u1eefng tr\u01b0\u1eddng h\u1ee3p c\u00f3 l\u00e2m s\u00e0ng g\u1ee3i \u00fd, ph\u00e2n bi\u1ec7t c\u00e1c bi\u1ebfn th\u1ec3 kh\u00f4ng \u0111i\u1ec3n h\u00ecnh cho \u0111\u1ebfn s\u00e0ng l\u1ecdc nh\u1eefng b\u1ec7nh l\u00fd c\u00f3 th\u1ec3 g\u00e2y nh\u1ea7m l\u1eabn v\u1edbi CIDP. B\u00ean c\u1ea1nh \u0111\u00f3, ch\u00fang t\u00f4i c\u0169ng \u0111\u00e1nh gi\u00e1 c\u00e1c ph\u01b0\u01a1ng ph\u00e1p \u0111i\u1ec1u tr\u1ecb hi\u1ec7n \u0111ang \u0111\u01b0\u1ee3c \u00e1p d\u1ee5ng bao g\u1ed3m immunoglobulin \u0111\u01b0\u1eddng t\u0129nh m\u1ea1ch (IVIg), corticosteroids (CCS) v\u00e0 trao \u0111\u1ed5i huy\u1ebft t\u01b0\u01a1ng (PE), \u0111\u1ed3ng th\u1eddi h\u01b0\u1edbng d\u1eabn c\u00e1ch ti\u1ebfp c\u1eadn c\u00e1c ca b\u1ec7nh kh\u00e1ng tr\u1ecb. Cu\u1ed1i c\u00f9ng, ch\u00fang t\u00f4i s\u1ebd t\u1eadp trung v\u00e0o vi\u1ec7c theo d\u00f5i \u0111\u00e1p \u1ee9ng \u0111i\u1ec1u tr\u1ecb b\u1eb1ng c\u00e1ch thang \u0111i\u1ec3m ti\u00ean l\u01b0\u1ee3ng kh\u00e1ch quan.<\/p>\n

Ba\u0300i vi\u00ea\u0301t tri\u0301ch ngu\u00f4\u0300n t\u01b0\u0300 H\u00f4\u0323i th\u00e2\u0300n kinh ho\u0323c Vi\u00ea\u0323t Nam<\/em><\/p>\n

Ta\u0301c gia\u0309:\u00a0<\/strong><\/em><\/p>\n

Amro M. Stino MD1<\/sup>, Elie Naddaf MD2<\/sup>, P. James B. Dyck MD2<\/sup>, Peter J. Dyck MD2<\/sup><\/strong><\/em>
\nPhi\u00ean d\u1ecbch: Nguy\u1ec5n H\u1ea3i Anh3<\/sup>, \u0110inh Th\u1ecb L\u1ee3i3<\/sup><\/strong><\/em>
\n\u0110\u01a1n v\u1ecb Th\u1ea7n kinh c\u01a1, Khoa Th\u1ea7n kinh, \u0110\u1ea1i h\u1ecdc Y khoa Michigan, Ann Arbor, Michigan1<\/sup><\/em>
\nKhoa Th\u1ea7n kinh, Mayo Clinic, Rochester, Minnesota2<\/sup><\/em>
\nTrung t\u00e2m Th\u1ea7n kinh, B\u1ec7nh vi\u1ec7n B\u1ea1ch Mai3<\/sup><\/em><\/p>\n

T\u1ed5ng quan<\/strong>
\nT\u1eeb kh\u00f3a: CIDP, ch\u1ea9n \u0111o\u00e1n, IVIg, trao \u0111\u1ed5i huy\u1ebft t\u01b0\u01a1ng, steroids, \u0111i\u1ec1u tr\u1ecb.
\nVi\u1ebft t\u1eaft: AL, chu\u1ed7i nh\u1eb9 m\u1eafc ph\u1ea3i; A-CIDP, CIDP kh\u1edfi ph\u00e1t c\u1ea5p t\u00ednh; CCS, corticosteroids; CIDP, vi\u00eam \u0111a r\u1ec5 v\u00e0 d\u00e2y th\u1ea7n kinh m\u1ea5t myelin m\u1ea1n t\u00ednh; CMT, Charcot-Marie- Tooth; DNT, d\u1ecbch n\u00e3o t\u1ee7y; DADS, b\u1ec7nh th\u1ea7n kinh ngo\u1ea1i bi\u00ean v\u1eadn \u0111\u1ed9ng c\u1ea3m gi\u00e1c m\u1ea5t myelin m\u1eafc ph\u1ea3i \u01b0u th\u1ebf ng\u1ecdn chi; EFNS\/PNS, Hi\u1ec7p h\u1ed9i th\u1ea7n kinh h\u1ecdc ch\u00e2u \u00c2u\/H\u1ed9i th\u1ea7n kinh ngo\u1ea1i bi\u00ean; IVIg, globulin mi\u1ec5n d\u1ecbch \u0111\u01b0\u1eddng t\u0129nh m\u1ea1ch; GBS, h\u1ed9i ch\u1ee9ng Guillain-Barr\u00e9; I-RODS, Inflammatory Rasch-built Overall Disability Scale Thang \u0111i\u1ec3m t\u00e0n ph\u1ebf to\u00e0n th\u1ec3 tr\u00ean b\u1ec7nh nh\u00e2n b\u1ec7nh th\u1ea7n kinh ngo\u1ea1i bi\u00ean qua trung gian mi\u1ec5n d\u1ecbch x\u00e2y d\u1ef1ng b\u1edfi Rarsch; Ig, globulin mi\u1ec5n d\u1ecbch; INCAT, Nguy\u00ean nh\u00e2n b\u1ec7nh th\u1ea7n kinh do vi\u00eam v\u00e0 \u0111i\u1ec1u tr\u1ecb; IVMP, methylprednisolone \u0111\u01b0\u1eddng t\u0129nh m\u1ea1ch; MAG, glycoprotein li\u00ean quan myelin; MCID, s\u1ef1 kh\u00e1c bi\u1ec7t trong c\u1ea3i thi\u1ec7n l\u00e2m s\u00e0ng t\u1ed5i thi\u1ec3u; CHT, c\u1ed9ng h\u01b0\u1edfng t\u1eeb; PE, trao \u0111\u1ed5i huy\u1ebft t\u01b0\u01a1ng; NIS, thang \u0111i\u1ec3m t\u1ed5n th\u01b0\u01a1ng th\u1ea7n kinh; POEMS, h\u1ed9i ch\u1ee9ng POEMS; SCIg, globulin mi\u1ec5n d\u1ecbch ti\u00eam d\u01b0\u1edbi da; SIDP, b\u1ec7nh vi\u00eam \u0111a r\u1ec5 v\u00e0 d\u00e2y th\u1ea7n kinh m\u1ea5t mylein do vi\u00eam b\u00e1n c\u1ea5p; TTR, transthyretin.
\nM\u1ee5c ti\u00eau c\u1ee7a b\u00e0i \u0111\u00e1nh gi\u00e1 n\u00e0y nh\u1eb1m h\u1ed7 tr\u1ee3 c\u00e1c b\u00e1c s\u0129 hi\u1ec3u r\u00f5 v\u1ec1 l\u00e2m s\u00e0ng, \u0111i\u1ec7n c\u01a1, \u0111\u1eb7c \u0111i\u1ec3m sinh b\u1ec7nh h\u1ecdc, qua \u0111\u00f3 gi\u00fap ch\u1ea9n \u0111o\u00e1n ch\u00ednh x\u00e1c CIDP v\u00e0 ph\u00e2n bi\u1ec7t ch\u00fang v\u1edbi nh\u1eefng t\u1ed5n th\u01b0\u01a1ng kh\u00e1c, c\u00f3 kh\u1ea3 n\u0103ng nh\u1eadn di\u1ec7n c\u00e1c bi\u1ebfn th\u1ec3 v\u00e0 l\u00ean k\u1ebf ho\u1ea1ch \u0111i\u1ec1u tr\u1ecb cho c\u00e1c b\u1ec7nh nh\u00e2n CIDP.<\/p>\n

1. Gi\u01a1\u0301i thi\u00ea\u0323u v\u00ea\u0300 vi\u00eam \u0111a r\u1ec5 v\u00e0 d\u00e2y th\u1ea7n kinh m\u1ea5t myelin m\u1ea1n t\u00ednh<\/strong><\/h2>\n

Vi\u00eam \u0111a r\u1ec5 v\u00e0 d\u00e2y th\u1ea7n kinh m\u1ea5t myelin m\u1ea1n t\u00ednh (CIDP) l\u00e0 m\u1ed9t r\u1ed1i lo\u1ea1n t\u1ef1 mi\u1ec5n m\u1eafc ph\u1ea3i g\u00e2y \u1ea3nh h\u01b0\u1edfng \u0111\u1ebfn ch\u1ee9c n\u0103ng c\u1ee7a r\u1ec5 v\u00e0 d\u00e2y th\u1ea7n kinh ngo\u1ea1i bi\u00ean, l\u00e2m s\u00e0ng \u0111i\u1ec3n h\u00ecnh v\u1edbi li\u1ec7t \u0111\u1ed1i x\u1ee9ng c\u1ea3 ng\u1ecdn v\u00e0 g\u1ed1c chi, m\u1ea5t ph\u1ea3n x\u1ea1 g\u00e2n x\u01b0\u01a1ng v\u00e0 ti\u1ebfn tri\u1ec3n b\u1ec7nh v\u01b0\u1ee3t qu\u00e1 8 tu\u1ea7n [1]. CIDP l\u1ea7n \u0111\u1ea7u \u0111\u01b0\u1ee3c m\u00f4 t\u1ea3 v\u00e0 \u0111\u1eb7t t\u00ean b\u1edfi Peter. J. Dyck v\u00e0o n\u0103m 1975 [2], t\u1ed5n th\u01b0\u01a1ng \u0111\u01b0\u1ee3c m\u00f4 t\u1ea3 ch\u1ee7 y\u1ebfu t\u1ea1i c\u00e1c s\u1ee3i th\u1ea7n kinh l\u1edbn c\u00f3 bao myelin d\u1eabn \u0111\u1ebfn tri\u1ec7u ch\u1ee9ng li\u1ec7t v\u00e0 th\u1ea5t \u0111i\u1ec1u c\u1ea3m gi\u00e1c. T\u1ef7 l\u1ec7 m\u1eafc m\u1edbi h\u00e0ng n\u0103m \u01b0\u1edbc ch\u1eebng kho\u1ea3ng 1 tr\u00ean 100000 ng\u01b0\u1eddi. T\u1ef7 l\u1ec7 nhi\u1ec5m b\u1ec7nh dao \u0111\u1ed9ng t\u01b0\u01a1ng \u0111\u1ed1i l\u1edbn, t\u1eeb 3 \u0111\u1ebfn 8 ca b\u1ec7nh tr\u00ean 100000 ng\u01b0\u1eddi do ph\u1ee5 thu\u1ed9c v\u00e0o ti\u00eau ch\u00ed ch\u1ea9n \u0111o\u00e1n v\u00e0 k\u1ef9 thu\u1eadt s\u1eed d\u1ee5ng trong \u0111\u00e1nh gi\u00e1 b\u1ec7nh nh\u00e2n [3-5].
\nCIDP l\u00e0 b\u1ec7nh l\u00fd vi\u00eam m\u1ea5t myelin do trung gian \u0111\u1ea1i th\u1ef1c b\u00e0o, th\u01b0\u1eddng li\u00ean quan \u0111\u1ebfn \u0111o\u1ea1n g\u1ea7n h\u01a1n l\u00e0 \u0111o\u1ea1n xa c\u1ee7a d\u00e2y th\u1ea7n kinh.<\/p>\n

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H\u00ecnh 1. H\u00ecnh \u1ea3nh vi\u00eam v\u00e0 \u201cc\u1ee7 h\u00e0nh\u201d quan s\u00e1t \u0111\u01b0\u1ee3c tr\u00ean l\u00e1t c\u1eaft ngang c\u1ed1 \u0111\u1ecbnh b\u1eb1ng parafin c\u1ee7a hai b\u1ec7nh nh\u00e2n \u0111\u01b0\u1ee3c ch\u1ea9n \u0111o\u00e1n CIDP. B\u1ec7nh ph\u1ea9m \u0111\u01b0\u1ee3c nhu\u1ed9m v\u1edbi hematoxylin-eosin (A) v\u00e0 CD45 (B). T\u1ed5n th\u01b0\u01a1ng l\u1edbp v\u1ecf s\u1ee3i th\u1ea7n kinh (endoneurial) quanh m\u1ea1ch v\u1edbi h\u00ecnh \u1ea3nh vi\u00eam tr\u00ean n\u1ec1n \u201cc\u1ee7 h\u00e0nh\u201d c\u1ee7a b\u1ec7nh nh\u00e2n CIDP. Ba l\u00e1t c\u1eaft li\u00ean ti\u1ebfp h\u00ecnh \u1ea3nh nhu\u1ed9m hematoxylin-eosin c\u1ee7a c\u00e1c s\u1ee3i th\u1ea7n kinh l\u1edbn, trong \u0111\u00f3 l\u1edbp v\u1ecf d\u00e2y (epineurium) v\u00e0 v\u1ecf b\u00f3 (perineurium) (C) th\u1ea7n kinh mang kh\u00e1ng th\u1ec3 c\u1ee7a t\u1ebf b\u00e0o T (CD3) (D). H\u00ecnh \u1ea3nh \u201cc\u1ee7 h\u00e0nh\u201d \u0111\u01b0\u1ee3c x\u00e1c nh\u1eadn b\u1edfi ph\u1ea3n \u1ee9ng c\u1ee7a n\u00f3 v\u1edbi t\u1ebf b\u00e0o Schwann (S-100) (E). Th\u00e2m nhi\u1ec5m vi\u00eam th\u01b0\u1eddng g\u1eb7p h\u01a1n \u1edf c\u00e1c b\u1ec7nh th\u1ea7n kinh ngo\u1ea1i bi\u00ean m\u1eafc ph\u1ea3i [6].<\/em><\/p>\n

Th\u00e2m nhi\u1ec5m vi\u00eam b\u00ean c\u1ea1nh c\u00e1c s\u1ee3i th\u1ea7n kinh c\u00f3 myelin ho\u1eb7c quanh m\u1ea1ch t\u1ea1i l\u1edbp v\u1ecf d\u00e2y th\u1ea7n kinh (epineurium). \u1ede giai \u0111o\u1ea1n s\u1edbm c\u1ee7a b\u1ec7nh, m\u1ea5t myelin theo \u0111o\u1ea1n chi\u1ebfm \u01b0u th\u1ebf (quan s\u00e1t t\u1ed1t nh\u1ea5t tr\u00ean c\u00e1c s\u1ee3i th\u1ea7n kinh b\u1ecb r\u00e1ch). Tuy v\u1eady theo th\u1eddi gian c\u00f9ng v\u1edbi vi\u1ec7c m\u1ea5t myelin ti\u1ebfp t\u1ee5c di\u1ec5n ra, t\u1ed5n th\u01b0\u01a1ng d\u1ea1ng \u201cc\u1ee7 h\u00e0nh\u201d (c\u00e1c t\u1ebf b\u00e0o Schwann x\u1ebfp ch\u1ed3ng l\u00ean nhau) s\u1ebd xu\u1ea5t hi\u1ec7n do t\u00edch l\u0169y s\u1ef1 t\u00e1i c\u1ea5u tr\u00fac s\u1ee3i myelin, t\u1eeb \u0111\u00f3 g\u00e2y ra hi\u1ec7n t\u01b0\u1ee3ng ph\u00ec \u0111\u1ea1i d\u00e2y th\u1ea7n kinh. H\u00ecnh \u1ea3nh \u201cc\u1ee7 h\u00e0nh\u201d tr\u00ean b\u1ec7nh nh\u00e2n CIDP th\u01b0\u1eddng xu\u1ea5t hi\u1ec7n v\u1edbi h\u00ecnh th\u00e1i \u0111a d\u1ea1ng (H\u00ecnh 2) do t\u1ed5n th\u01b0\u01a1ng vi\u00eam m\u1ea5t myelin t\u1eebng \u0111o\u1ea1n \u2013 n\u1eb1m k\u1ebf ti\u1ebfp c\u00e1c s\u1ee3i th\u1ea7n kinh c\u00f3 myelin bao quanh b\u1edfi h\u00ecnh \u1ea3nh \u201cc\u1ee7 h\u00e0nh\u201d \u2013 b\u00ean c\u1ea1nh c\u00e1c s\u1ee3i th\u1ea7n kinh c\u00f3 myelin b\u00ecnh th\u01b0\u1eddng [6]. S\u1ef1 m\u1ea5t myelin kh\u00f4ng \u0111\u1ed3ng \u0111\u1ec1u gi\u1ea3i th\u00edch l\u00fd do t\u1ea1i sao \u0111i\u1ec7n c\u01a1 \u0111i\u1ec3n h\u00ecnh c\u1ee7a CIDP c\u00f3 s\u1ef1 ph\u00e2n t\u00e1n theo th\u1eddi gian. C\u01a1 ch\u1ebf mi\u1ec5n d\u1ecbch li\u00ean quan \u0111\u1ebfn \u0111\u1ebfn con \u0111\u01b0\u1eddng trung gian \u0111\u1ea1i th\u1ef1c b\u00e0o cho th\u1ea5y \u0111\u1ed3ng k\u00edch th\u00edch c\u00e1c y\u1ebfu t\u1ed1 B7-1 v\u00e0 B70-2 tr\u1ef1c ti\u1ebfp t\u1ea5n c\u00f4ng c\u00e1c t\u1ebf b\u00e0o Schwann v\u00e0 epitope c\u1ee7a myelin, m\u1eb7c d\u00f9 \u0111\u00edch c\u1ee5 th\u1ec3 c\u1ee7a qu\u00e1 tr\u00ecnh n\u00e0y hi\u1ec7n c\u00f2n ch\u01b0a x\u00e1c \u0111\u1ecbnh [7]. S\u1ef1 xu\u1ea5t hi\u1ec7n c\u1ee7a c\u00e1c kh\u00e1ng th\u1ec3 \u0111\u1eb7c hi\u1ec7u neurofascin -155 v\u00e0 contactin-1 cho th\u1ea5y con \u0111\u01b0\u1eddng b\u1ec7nh h\u1ecdc li\u00ean quan \u0111\u1ebfn c\u00e1c kh\u00e1ng th\u1ec3 kh\u00e1ng n\u1ed1t v\u00e0 c\u1eadn n\u1ed1t (nodal v\u00e0 paranodal) tr\u00ean m\u1ed9t s\u1ed1 b\u1ec7nh nh\u00e2n. Tuy nhi\u00ean, nh\u1eefng tr\u01b0\u1eddng h\u1ee3p n\u00e0y c\u00f3 \u0111\u00e1p \u1ee9ng \u0111i\u1ec1u tr\u1ecb r\u1ea5t \u0111a d\u1ea1ng. \u0110i\u1ec1u tr\u1ecb r\u1ed9ng r\u00e3i, bao g\u1ed3m IVIg, immunoglobulin ti\u00eam d\u01b0\u1edbi da (SCIg), corticosteroids (CCS) v\u00e0 trao \u0111\u1ed5i huy\u1ebft t\u01b0\u01a1ng \u0111\u1ec1u cho th\u1ea5y hi\u1ec7u qu\u1ea3 tr\u00ean th\u1ec3 b\u1ec7nh CIDP \u0111i\u1ec3n h\u00ecnh, m\u1eb7c d\u00f9 m\u1ed7i ph\u01b0\u01a1ng ph\u00e1p \u0111\u1ec1u c\u00f3 nh\u1eefng h\u1ea1n ch\u1ebf ri\u00eang.
\nCh\u1ea9n \u0111o\u00e1n CIDP hi\u1ec7n t\u1ea1i v\u1eabn c\u00f2n d\u1ef1a v\u00e0o l\u00e2m s\u00e0ng v\u00e0 \u0111\u01b0\u1ee3c \u1ee7ng h\u1ed9 b\u1edfi \u0111i\u1ec7n c\u01a1. \u1ede b\u00e0i \u0111\u00e1nh gi\u00e1 n\u00e0y, ch\u00fang t\u00f4i s\u1ebd tr\u00ecnh b\u00e0y c\u00e1c b\u1eaby trong ch\u1ea9n \u0111o\u00e1n CIDP v\u00e0 cung c\u1ea5p c\u00e1ch ti\u1ebfp c\u1eadn trong th\u1ef1c h\u00e0nh. Ti\u1ebfp theo, ch\u00fang t\u00f4i s\u1ebd m\u00f4 t\u1ea3 c\u00e1c ph\u01b0\u01a1ng ph\u00e1p \u0111i\u1ec1u tr\u1ecb hi\u1ec7n t\u1ea1i v\u00e0 l\u1ef1a ch\u1ecdn \u0111i\u1ec1u tr\u1ecb ph\u00f9 h\u1ee3p.<\/p>\n

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H\u00ecnh 2. C\u00e1c h\u00ecnh \u1ea3nh \u201cc\u1ee7 h\u00e0nh\u201d \u0111\u01b0\u1ee3c quan s\u00e1t tr\u00ean l\u00e1t c\u1eaft sinh thi\u1ebft th\u1ea7n kinh c\u1ed1 \u0111\u1ecbnh b\u1edfi epoxy nhu\u1ed9m xanh methyl, cho th\u1ea5y m\u1ed9t v\u00e0i s\u1ee3i th\u1ea7n kinh c\u00f3 \u201cc\u1ee7 h\u00e0nh\u201d bao quanh trong khi m\u1ed9t s\u1ed1 s\u1ee3i th\u1ea7n kinh c\u00f3 myelin kh\u00f4ng c\u00f3 c\u1ea5u tr\u00fac n\u00e0y, c\u00f3 th\u1ec3 th\u1ea5y tr\u00ean b\u1ec7nh nh\u00e2n CIDP (m\u1eafc ph\u1ea3i) khi sinh thi\u1ebft r\u1ec5 th\u1ea7n kinh (A), CIDP (m\u1eafc ph\u1ea3i) (B) v\u00e0 b\u1ec7nh th\u1ea7n kinh ngo\u1ea1i bi\u00ean ph\u00ec \u0111\u1ea1i khu tr\u00fa c\u1ee7a d\u00e2y th\u1ea7n kinh gi\u1eefa (CIDP khu tr\u00fa, m\u1eafc ph\u1ea3i) (C). H\u00ecnh th\u00e1i \u0111a d\u1ea1ng th\u01b0\u1eddng ph\u1ed5 bi\u1ebfn h\u01a1n \u1edf c\u00e1c b\u1ec7nh th\u1ea7n kinh ngo\u1ea1i bi\u00ean m\u1eafc ph\u1ea3i.<\/em><\/p>\n

2. B\u00e2\u0303y trong ch\u00e2\u0309n \u0111oa\u0301n Vi\u00eam \u0111a r\u1ec5 v\u00e0 d\u00e2y th\u1ea7n kinh m\u1ea5t myelin m\u1ea1n t\u00ednh va\u0300 \u0111i\u00ea\u0300u tri\u0323 qua\u0301 tay<\/strong><\/h2>\n

M\u1eb7c d\u00f9 ti\u00eau chu\u1ea9n ch\u1ea9n \u0111o\u00e1n Vi\u00eam \u0111a r\u1ec5 v\u00e0 d\u00e2y th\u1ea7n kinh m\u1ea5t myelin m\u1ea1n t\u00ednh \u0111\u00e3 \u0111\u01b0\u1ee3c thi\u1ebft l\u1eadp, \u0111\u1eb7c bi\u1ec7t l\u00e0 ti\u00eau ch\u00ed c\u1ee7a Hi\u1ec7p h\u1ed9i th\u1ea7n kinh ch\u00e2u \u00c2u\/H\u1ed9i th\u1ea7n kinh ngo\u1ea1i bi\u00ean (EFNS\/PNS) c\u0169ng nh\u01b0 l\u00e2m s\u00e0ng t\u01b0\u01a1ng \u0111\u1ed1i d\u1ec5 nh\u1eadn bi\u1ebft \u0111\u1ed1i v\u1edbi th\u1ec3 \u0111i\u1ec3n h\u00ecnh, ch\u1ea9n \u0111o\u00e1n nh\u1ea7m CIDP v\u1eabn t\u01b0\u01a1ng \u0111\u1ed1i ph\u1ed5 bi\u1ebfn, t\u1eeb \u0111\u00f3 d\u1eabn \u0111\u1ebfn vi\u1ec7c ch\u1ec9 \u0111\u1ecbnh li\u1ec7u ph\u00e1p mi\u1ec5n d\u1ecbch kh\u00f4ng c\u1ea7n thi\u1ebft. Ch\u1ea9n \u0111o\u00e1n nh\u1ea7m b\u1ec7nh th\u1ea7n kinh ngo\u1ea1i bi\u00ean kh\u00e1c v\u1edbi CIDP \u0111\u01b0\u1ee3c mi\u00eau t\u1ea3 chi ti\u1ebft trong m\u1ed9t nghi\u00ean c\u1ee9u th\u1ef1c hi\u1ec7n b\u1edfi Allen v\u00e0 c\u1ed9ng s\u1ef1, trong \u0111\u00f3 28 tr\u00ean 59 (47%) b\u1ec7nh nh\u00e2n \u0111\u01b0\u1ee3c ch\u1ea9n \u0111o\u00e1n x\u00e1c \u0111\u1ecbnh CIDP kh\u00f4ng th\u1ecfa m\u00e3n c\u00e1c ti\u00eau ch\u00ed v\u1ec1 l\u00e2m s\u00e0ng v\u00e0 \u0111i\u1ec7n c\u01a1. T\u00e1c gi\u1ea3 Allan cho r\u1eb1ng l\u1ed7i ch\u1ea9n \u0111o\u00e1n ch\u1ee7 y\u1ebfu do qu\u00e1 ph\u1ee5 thu\u1ed9c v\u00e0o b\u1ea5t th\u01b0\u1eddng trong d\u1eabn truy\u1ec1n th\u1ea7n kinh m\u1ee9c \u0111\u1ed9 nh\u1eb9 nh\u01b0ng \u0111\u01b0\u1ee3c gi\u1ea3i th\u00edch sai l\u00e0 do m\u1ea5t myelin, do t\u0103ng kh\u00f4ng c\u00f3 \u00fd ngh\u0129a protein trong d\u1ecbch n\u00e3o t\u1ee7y (DNT) v\u00e0 do \u0111\u00e1nh gi\u00e1 m\u1ee9c \u0111\u1ed9 c\u1ea3i thi\u1ec7n v\u1edbi \u0111i\u1ec1u tr\u1ecb m\u1ed9t c\u00e1ch ch\u1ee7 quan thay v\u00ec c\u00e1c b\u1eb1ng ch\u1ee9ng kh\u00e1ch quan.
\nTr\u00ean \u0111i\u1ec7n c\u01a1, c\u00e1c t\u1ed5n th\u01b0\u01a1ng th\u01b0\u1eddng b\u1ecb k\u1ebft lu\u1eadn nh\u1ea7m v\u1edbi CIDP bao g\u1ed3m suy gi\u1ea3m (t\u1ed1c \u0111\u1ed9 d\u1eabn truy\u1ec1n v\u00e0 th\u1eddi gian ti\u1ec1m) ph\u1ee5 thu\u1ed9c bi\u00ean \u0111\u1ed9 \u1edf B\u1ec7nh d\u00e2y th\u1ea7n kinh ph\u1ee5 thu\u1ed9c \u0111\u1ed9 d\u00e0i, suy gi\u1ea3m (t\u1ed1c \u0111\u1ed9 d\u1eabn truy\u1ec1n v\u00e0 th\u1eddi gian ti\u1ec1m) kh\u00f4ng ph\u1ee5 thu\u1ed9c bi\u00ean \u0111\u1ed9 \u1edf B\u1ec7nh \u0111a d\u00e2y th\u1ea7n kinh do \u0111\u00e1i th\u00e1o \u0111\u01b0\u1eddng, thay \u0111\u1ed5i th\u1eddi gian ti\u1ec1m \u0111o\u1ea1n xa \u0111\u01a1n \u0111\u1ed9c t\u1ea1i d\u00e2y th\u1ea7n kinh m\u00e1c (ghi \u0111i\u1ec7n c\u01a1 t\u1ea1i c\u01a1 du\u1ed7i c\u00e1c ng\u00f3n), v\u00e0 suy gi\u1ea3m d\u1eabn truy\u1ec1n khu tr\u00fa t\u1ea1i c\u00e1c v\u1ecb tr\u00ed g\u00e2y ch\u00e8n \u00e9p d\u00e2y th\u1ea7n kinh th\u01b0\u1eddng g\u1eb7p [11]. Do \u0111\u00f3, c\u00f3 th\u1ec3 th\u1ea5y \u0111\u01b0\u1ee3c t\u1ea7m quan tr\u1ecdng c\u1ee7a ph\u00e2n t\u00edch \u0111i\u1ec7n c\u01a1 d\u1ef1a v\u00e0o b\u1ed1i c\u1ea3nh l\u00e2m s\u00e0ng tr\u00ean t\u1eebng b\u1ec7nh nh\u00e2n c\u1ee5 th\u1ec3. \u0110\u1ed3ng th\u1eddi, n\u00ean c\u00e2n nh\u1eafc nh\u1eefng ch\u1ea9n \u0111o\u00e1n thay th\u1ebf khi b\u1ec7nh nh\u00e2n c\u00f3 bi\u1ec3u hi\u1ec7n l\u00e2m s\u00e0ng kh\u00f4ng \u0111i\u1ec3n h\u00ecnh, c\u00f3 b\u1ea5t th\u01b0\u1eddng c\u1eadn l\u00e2m s\u00e0ng kh\u00f4ng \u0111\u1eb7c hi\u1ec7u v\u00e0 kh\u00f4ng c\u00f3 b\u1eb1ng ch\u1ee9ng kh\u00e1ch quan trong \u0111\u00e1nh gi\u00e1 \u0111\u00e1p \u1ee9ng \u0111i\u1ec1u tr\u1ecb.
\nNgay c\u1ea3 khi ch\u1ea9n \u0111o\u00e1n ch\u00ednh x\u00e1c, vi\u1ec7c \u0111i\u1ec1u tr\u1ecb qu\u00e1 m\u1ee9c c\u00e1c c\u0169ng l\u00e0 m\u1ed9t v\u1ea5n \u0111\u1ec1 quan tr\u1ecdng m\u00e0 nguy\u00ean nh\u00e2n ch\u1ee7 y\u1ebfu l\u00e0 do c\u00e1c b\u00e1c s\u0129 kh\u00f4ng ki\u00ean quy\u1ebft d\u1eebng \u0111i\u1ec1u tr\u1ecb \u0111\u00fang th\u1eddi \u0111i\u1ec3m, do b\u1ec7nh nh\u00e2n c\u00f3 s\u1ef1 ph\u1ee5 thu\u1ed9c v\u1ec1 m\u1eb7t t\u00e2m l\u00fd v\u1edbi \u0111i\u1ec1u tr\u1ecb (d\u00f9 kh\u00f4ng c\u00f3 ch\u1ec9 \u0111\u1ecbnh) v\u00e0 do trong c\u00e1c tr\u01b0\u1eddng h\u1ee3p Vi\u00eam \u0111a r\u1ec5 v\u00e0 d\u00e2y th\u1ea7n kinh m\u1ea5t myelin m\u1ea1n t\u00ednh v\u1edbi t\u1ed5n th\u01b0\u01a1ng s\u1ee3i tr\u1ee5c th\u1ee9 ph\u00e1t, \u0111i\u1ec1u tr\u1ecb b\u1eb1ng li\u1ec7u ph\u00e1p mi\u1ec5n d\u1ecbch th\u01b0\u1eddng kh\u00f4ng mang l\u1ea1i hi\u1ec7u qu\u1ea3 [12-13].<\/p>\n

3. \u0110\u0103\u0323c \u0111i\u00ea\u0309m l\u00e2m sa\u0300ng cu\u0309a Vi\u00eam \u0111a r\u1ec5 v\u00e0 d\u00e2y th\u1ea7n kinh m\u1ea5t myelin m\u1ea1n t\u00ednh \u0111i\u00ea\u0309n hi\u0300nh<\/strong><\/h2>\n

Ki\u1ec3u h\u00ecnh kinh \u0111i\u1ec3n c\u1ee7a Vi\u00eam \u0111a r\u1ec5 v\u00e0 d\u00e2y th\u1ea7n kinh m\u1ea5t myelin m\u1ea1n t\u00ednh l\u00e0 b\u1ec7nh l\u00fd \u0111a r\u1ec5 d\u00e2y th\u1ea7n kinh v\u1eadn \u0111\u1ed9ng v\u00e0 c\u1ea3m gi\u00e1c, c\u00f3 t\u00ednh \u0111\u1ed1i x\u1ee9ng, y\u1ebfu k\u1ebft h\u1ee3p gi\u1eefa g\u1ed1c chi v\u00e0 ng\u1ecdn chi, m\u1ea5t ph\u1ea3n x\u1ea1 g\u00e2n x\u01b0\u01a1ng v\u00e0 kh\u00f4ng c\u00f3 \u0111au \u0111\u00e1ng k\u1ec3. M\u1ea1n t\u00ednh \u0111\u01b0\u1ee3c d\u00f9ng \u0111\u1ec3 ch\u1ec9 ti\u1ebfn tri\u1ec3n c\u1ee7a b\u1ec7nh v\u01b0\u1ee3t qu\u00e1 8 tu\u1ea7n. Thi\u1ebfu h\u1ee5t v\u1eadn \u0111\u1ed9ng ng\u1ecdn chi th\u01b0\u1eddng chi\u1ebfm \u01b0u th\u1ebf, t\u1ed5n th\u01b0\u01a1ng c\u1ea3m gi\u00e1c ch\u1ee7 y\u1ebfu l\u00e0 do th\u1ea7n kinh s\u1ee3i l\u1edbn. Hi\u1ec7n t\u01b0\u1ee3ng n\u00e0y \u0111\u01b0\u1ee3c l\u00fd gi\u1ea3i do c\u00e1c s\u1ee3i th\u1ea7n kinh v\u1edbi nhi\u1ec1u bao myelin nh\u1ea5t s\u1ebd b\u1ecb t\u1ed5n th\u01b0\u01a1ng n\u1eb7ng n\u1ec1 nh\u1ea5t. B\u1ea5t k\u1ec3 bi\u1ec3u hi\u1ec7n l\u00e2m s\u00e0ng n\u00e0o mang m\u00e0u s\u1eafc kh\u00e1c v\u1edbi b\u1ee9c tranh \u0111i\u1ec3n h\u00ecnh tr\u00ean n\u00ean \u0111\u01b0\u1ee3c c\u00e2n nh\u1eafc b\u1eb1ng m\u1ed9t nguy\u00ean nh\u00e2n thay th\u1ebf ho\u1eb7c do th\u1ec3 CIDP kh\u00f4ng \u0111i\u1ec3n h\u00ecnh (B\u1ea3ng 1). Ch\u1ea9n \u0111o\u00e1n ph\u00e2n bi\u1ec7t \u0111\u1ea7u ti\u00ean c\u1ea7n ngh\u0129 t\u1edbi l\u00e0 B\u1ec7nh th\u1ea7n kinh c\u1ea3m gi\u00e1c s\u1ee3i l\u1edbn thu\u1ea7n t\u00fay v\u1edbi th\u1ea5t \u0111i\u1ec1u \u2013 m\u1ed9t ph\u1ed5 b\u1ec7nh kh\u00e1c v\u1edbi CIDP. B\u00ean c\u1ea1nh \u0111\u00f3, bi\u1ebfn th\u1ec3 CIDP mang t\u00ean B\u1ec7nh \u0111a d\u00e2y th\u1ea7n kinh c\u1ea3m gi\u00e1c mi\u1ec5n d\u1ecbch m\u1ea1n t\u00ednh (CISP) [14] c\u0169ng n\u00ean \u0111\u01b0\u1ee3c c\u00e2n nh\u1eafc. C\u00e1c b\u1ec7nh l\u00fd th\u1ea7n kinh ngo\u1ea1i bi\u00ean \u0111a \u1ed5, kh\u00f4ng \u0111\u1ed1i x\u1ee9ng v\u00e0 \u01b0u th\u1ebf chi tr\u00ean c\u1ea7n ngh\u0129 \u0111\u1ebfn ch\u1ea9n \u0111o\u00e1n CIDP \u0111a \u1ed5 [15]. Ngo\u00e0i ra, b\u1ec7nh nh\u00e2n CIDP \u0111i\u1ec3n h\u00ecnh th\u01b0\u1eddng kh\u00f4ng c\u00f3 c\u00e1c tri\u1ec7u ch\u1ee9ng b\u1ec7nh h\u1ec7 th\u1ed1ng k\u00e8m theo nh\u01b0 s\u1ed1t, \u0111au nhi\u1ec1u, r\u1ed1i lo\u1ea1n th\u1ea7n kinh th\u1ef1c v\u1eadt v\u00e0 m\u1ec7t m\u1ecfi [16].<\/p>\n

4. \u0110\u0103\u0323c \u0111i\u00ea\u0309m \u0111i\u00ea\u0323n c\u01a1 va\u0300 test \u0111i\u00ea\u0323n sinh ly\u0301<\/strong><\/h2>\n

Nh\u01b0 \u0111\u00e3 \u0111\u1ec1 c\u1eadp, m\u1ed9t b\u00e1c s\u0129 gi\u1ecfi n\u00ean xem x\u00e9t k\u1ef9 l\u01b0\u1ee1ng c\u00e1c d\u1ea5u hi\u1ec7u l\u00e2m s\u00e0ng tr\u01b0\u1edbc khi s\u1eed d\u1ee5ng \u0111i\u1ec7n sinh l\u00fd \u0111\u1ec3 phi\u00ean gi\u1ea3i. C\u00e1c c\u00f4ng c\u1ee5 ch\u1ea9n \u0111o\u00e1n d\u1ef1a tr\u00ean vi\u1ec7c m\u1ea5t myelin, v\u00ed d\u1ee5 nh\u01b0 b\u1ed9 ti\u00eau ch\u00ed c\u1ee7a EFNS\/PNS, ch\u1ec9 n\u00ean \u0111\u01b0\u1ee3c \u00e1p d\u1ee5ng khi t\u1ed5n th\u01b0\u01a1ng tr\u00ean \u0111i\u1ec7n c\u01a1 cho th\u1ea5y h\u00ecnh \u1ea3nh m\u1ea5t myelin th\u1ef1c s\u1ef1. Tuy nhi\u00ean, vi\u1ec7c x\u00e1c \u0111\u1ecbnh m\u1ea5t myelin b\u1eb1ng \u0111i\u1ec7n sinh l\u00fd c\u00f3 th\u1ec3 b\u1ecb h\u1ea1n ch\u1ebf b\u1edfi \u201chi\u1ec7u \u1ee9ng tr\u1ea7n nh\u00e0\u201d, khi \u0111\u00f3 \u0111\u00e1p \u1ee9ng v\u1eadn \u0111\u1ed9ng v\u00e0 c\u1ea3m gi\u00e1c c\u00f3 th\u1ec3 th\u1ea5p h\u01a1n ho\u1eb7c kh\u00f4ng xu\u1ea5t hi\u1ec7n trong nh\u1eefng ca b\u1ec7nh kh\u00f3 do m\u1ea5t s\u1ee3i tr\u1ee5c th\u1ee9 ph\u00e1t, ph\u00e1t t\u00e1n theo th\u1eddi gian ho\u1eb7c ch\u1eb9n d\u1eabn truy\u1ec1n. H\u01a1n n\u1eefa, khi t\u1ed5n th\u01b0\u01a1ng ch\u1ec9 xu\u1ea5t hi\u1ec7n \u1edf r\u1ec5 th\u1ea7n kinh, b\u1ec7nh nh\u00e2n c\u00f3 th\u1ec3 kh\u00f4ng c\u00f3 c\u00e1c bi\u1ec3u hi\u1ec7n c\u1ee7a m\u1ea5t myelin r\u00f5 r\u00e0ng tr\u00ean \u0111i\u1ec7n sinh l\u00fd, \u0111\u1eb7c bi\u1ec7t trong c\u00e1c tr\u01b0\u1eddng h\u1ee3p m\u1ea5t s\u00f3ng F. Khi \u0111\u00f3, \u0111i\u1ec7n c\u01a1 kim (EMG) l\u00e0 l\u1ef1a ch\u1ecdn c\u1ea7n thi\u1ebft \u0111\u1ec3 ph\u00e2n bi\u1ec7t. M\u1ed9t b\u1ec7nh l\u00fd v\u1edbi \u01b0u th\u1ebf m\u1ea5t myelin (ch\u1eb9n d\u1eabn truy\u1ec1n ho\u1eb7c ph\u00e1t t\u00e1n theo th\u1eddi gian) n\u00ean \u0111\u01b0\u1ee3c nghi ng\u1edd khi thay \u0111\u1ed5i tr\u00ean \u0111i\u1ec7n c\u01a1 kim l\u00e0 nh\u1eb9 h\u01a1n mong \u0111\u1ee3i (gi\u1ea3m k\u1ebft t\u1eadp v\u1edbi duy nh\u1ea5t \u0111i\u1ec7n th\u1ebf ti\u1ec1m \u0111\u01a1n v\u1ecb v\u1eadn \u0111\u1ed9ng k\u00e9o d\u00e0i nh\u1eb9 v\u00e0 duy nh\u1ea5t b\u1ea5t th\u01b0\u1eddng ho\u1ea1t \u0111\u1ed9ng t\u1ef1 ph\u00e1t r\u1ea3i r\u00e1c), k\u1ec3 c\u1ea3 v\u1edbi s\u1ef1 xu\u1ea5t hi\u1ec7n c\u1ee7a gi\u1ea3m bi\u00ean \u0111\u1ed9 \u0111\u00e1p \u1ee9ng v\u1eadn \u0111\u1ed9ng. Ngo\u00e0i ra, c\u0169ng n\u00ean ch\u00fa \u00fd r\u1eb1ng m\u1ed9t s\u1ed1 b\u1ec7nh th\u1ea7n kinh ngo\u1ea1i bi\u00ean m\u1ea5t myelin c\u00f3 \u0111\u1eb7c \u0111i\u1ec3m \u0111i\u1ec7n c\u01a1 th\u1ecfa ti\u00eau ch\u00ed c\u1ee7a CIDP nh\u01b0ng c\u00f3 \u0111\u1eb7c \u0111i\u1ec3m l\u00e2m s\u00e0ng c\u0169ng nh\u01b0 c\u1eadn l\u00e2m s\u00e0ng kh\u00e1c bi\u1ec7t.<\/p>\n

B\u1ea3ng 1. C\u1eadn l\u00e2m s\u00e0ng h\u1ed7 tr\u1ee3 trong ch\u1ea9n \u0111o\u00e1n bi\u1ebfn th\u1ec3 CIDP v\u00e0 c\u00e1c b\u1ec7nh l\u00fd gi\u1ed1ng CIDP<\/em><\/p>\n\n\n\n\n\n\n\n\n\n
\u0110\u1eb7<\/strong>c<\/strong>\u00a0\u0111<\/strong>i\u1ec3m l\u00e2m<\/strong>\u00a0s\u00e0ng c\u00e1c<\/strong>\u00a0bi\u1ebfn th\u1ec3<\/strong>\u00a0c\u1ee7a CIDP<\/strong>\u00a0ho\u1eb7c c\u00e1c<\/strong>\u00a0b\u1ec7nh l\u00fd<\/strong>\u00a0gi\u1ed1ng CIDP<\/strong><\/td>\nC\u1eadn l\u00e2m s\u00e0ng h\u1ed7 tr\u1ee3 ch\u1ea9n \u0111o\u00e1n<\/strong><\/td>\n<\/tr>\n
B\u1ec7nh th\u1ea7n kinh ph\u1ee5 thu\u1ed9c chi\u1ec1u d\u00e0i \u01b0u th\u1ebf c\u1ea3m gi\u00e1c h\u01a1n v\u1eadn \u0111\u1ed9ng, B\u1ec7nh th\u1ea7n kinh ngo\u1ea1i bi\u00ean \u01b0u th\u1ebf s\u1ee3i tr\u1ee5c.<\/td>\nHbA1c, acid methylmanoic, vitamin B12, TSH, k\u1ebdm, \u0111\u1ed3ng, ceruloplasmin.<\/td>\n<\/tr>\n
B\u1ec7nh h\u1ea1ch c\u1ea3m gi\u00e1c\/B\u1ec7nh th\u1ea7n kinh ngo\u1ea1i bi\u00ean do tho\u00e1i h\u00f3a (neuronopathy) c\u1ea3m gi\u00e1c kh\u00f4ng ph\u1ee5 thu\u1ed9c chi\u1ec1u d\u00e0i.<\/td>\nKh\u00e1ng th\u1ec3 SSA v\u00e0 SSB, sinh thi\u1ebft tuy\u1ebfn n\u01b0\u1edbc b\u1ecdt nh\u1ecf, kh\u00e1ng th\u1ec3 kh\u00e1ng Hu, kh\u00e1ng CMRP, \u0111i\u1ec7n th\u1ebf g\u1ee3i c\u1ea3m gi\u00e1c, d\u1ecbch n\u00e3o t\u1ee7y, CHT r\u1ec5 th\u1ea7n kinh.<\/td>\n<\/tr>\n
\u01afu th\u1ebf chi tr\u00ean<\/td>\nKh\u00e1ng th\u1ec3 kh\u00e1ng GM1 v\u00e0 NS6S (MMN), c\u00f4ng th\u1ee9c m\u00e1u, m\u00e1u l\u1eafng, CRP, b\u1ed9 x\u00e9t nghi\u1ec7m ANCA, ANA, b\u1ed9 kh\u00e1ng nguy\u00ean nh\u00e2n t\u00e1ch chi\u1ebft \u0111\u01b0\u1ee3c (ENA), s\u00e0ng l\u1ecdc vi\u00eam gan, sinh thi\u1ebft th\u1ea7n kinh, x\u00e9t nghi\u1ec7m gene (PMP22 n\u1ebfu nghi ng\u1edd HNPP)<\/td>\n<\/tr>\n
B\u1ec7nh th\u1ea7n kinh m\u1ea5t myelin v\u1eadn \u0111\u1ed9ng v\u00e0 c\u1ea3m gi\u00e1c<\/td>\nX\u00e9t nghi\u1ec7m gene (PMP22), c\u00f4ng th\u1ee9c m\u00e1u, s\u00e0ng l\u1ecdc protein \u0111\u01a1n d\u00f2ng, kh\u00e1ng th\u1ec3 glycoprotein li\u00ean quan v\u1edbi myelin, n\u1ed3ng \u0111\u1ed9 VEGF, kh\u1ea3o s\u00e1t h\u1ec7 x\u01b0\u01a1ng.<\/td>\n<\/tr>\n
B\u1ec7nh th\u1ea7n kinh \u0111a d\u00e2y v\u00e0 r\u1ec5 v\u1eadn \u0111\u1ed9ng v\u00e0 c\u1ea3m gi\u00e1c<\/td>\nS\u00e0ng l\u1ecdc protein \u0111\u01a1n d\u00f2ng, chu\u1ed7i nh\u1eb9 t\u1ef1 do huy\u1ebft thanh, NT-proBNP, aspirate b\u00e9o, x\u00e9t nghi\u1ec7m gene TTR v\u00e0\u00a099m<\/sup>Tc-PYP.<\/td>\n<\/tr>\n
T\u1eeb vi\u1ebft t\u1eaft: ANA: kh\u00e1ng th\u1ec3 kh\u00e1ng nh\u00e2n; ANCA: kh\u00e1ng th\u1ec3 kh\u00e1ng b\u00e0o t\u01b0\u01a1ng c\u1ee7a b\u1ea1ch c\u1ea7u \u0111o\u1ea1n trung t\u00ednh; CRMP: protein trung gian \u0111\u00e1p \u1ee9ng collapsin; HbA1C: hemoglobin A1C; HNPP: b\u1ec7nh th\u1ea7n kinh ngo\u1ea1i bi\u00ean di truy\u1ec1n v\u1edbi nguy c\u01a1 li\u1ec7t do ch\u00e8n \u00e9p; MMN: b\u1ec7nh d\u00e2y th\u1ea7n kinh v\u1eadn \u0111\u1ed9ng \u0111a \u1ed5; PMP22: protein myelin ngo\u1ea1i bi\u00ean 22; TTR: transthyretin; VEGF: y\u1ebfu t\u1ed1 t\u0103ng tr\u01b0\u1edfng n\u1ed9i m\u00f4 m\u1ea1ch m\u00e1u<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n

T\u0103ng protein d\u1ecbch n\u00e3o t\u1ee7y kh\u00f4ng ph\u1ea3i \u0111i\u1ec1u ki\u1ec7n b\u1eaft bu\u1ed9c v\u1edbi \u0111a s\u1ed1 chuy\u00ean gia CIDP. Tuy v\u1eady, do xu\u1ea5t hi\u1ec7n \u1edf h\u01a1n 90% b\u1ec7nh nh\u00e2n CIDP, b\u1ea5t th\u01b0\u1eddng n\u00e0y gi\u00fap c\u1ee7ng c\u1ed1 ch\u1ea9n \u0111o\u00e1n [17]. T\u0103ng protein d\u1ecbch n\u00e3o t\u1ee7y kh\u00f4ng c\u00f3 t\u00ednh \u0111\u1eb7c hi\u1ec7u, ch\u00fang c\u00f3 th\u1ec3 b\u1ecb \u1ea3nh h\u01b0\u1edfng b\u1edfi tu\u1ed5i v\u00e0 c\u00e1c b\u1ec7nh l\u00fd k\u00e8m theo nh\u01b0 \u0111\u00e1i th\u00e1o \u0111\u01b0\u1eddng v\u00e0 h\u1eb9p \u1ed1ng s\u1ed1ng do tho\u00e1i h\u00f3a [18]. T\u0103ng gi\u1edbi h\u1ea1n c\u1ee7a protein DNT l\u1edbn h\u01a1n ng\u01b0\u1ee1ng 45mg\/dL (th\u01b0\u1eddng s\u1eed d\u1ee5ng) s\u1ebd l\u00e0m t\u0103ng \u0111\u1ed9 \u0111\u1eb7c hi\u1ec7u trong ch\u1ea9n \u0111o\u00e1n CIDP m\u00e0 kh\u00f4ng \u1ea3nh h\u01b0\u1edfng \u0111\u1ebfn \u0111\u1ed9 nh\u1ea1y (s\u1eed d\u1ee5ng \u0111i\u1ec3m c\u1eaft 50mg\/dL cho b\u1ec7nh nh\u00e2n < 50 tu\u1ed5i v\u00e0 60mg\/dL cho b\u1ec7nh nh\u00e2n 50 tu\u1ed5i [19]). M\u1eb7c d\u00f9 CHT c\u00f3 th\u1ec3 ph\u00e1t hi\u1ec7n t\u0103ng k\u00edch r\u1ec5 th\u1ea7n kinh v\u00e0 ph\u00ecnh \u0111\u00e1m r\u1ed1i [20] c\u0169ng nh\u01b0 si\u00eau \u00e2m gi\u00fap ph\u00e1t hi\u1ec7n ph\u00ec \u0111\u1ea1i th\u1ea7n kinh gi\u1eefa \u0111o\u1ea1n g\u1ea7n v\u00e0 \u0111\u00e1m r\u1ed1i c\u1ed5 [21-22], qua \u0111\u00f3 gi\u00fap c\u1ee7ng c\u1ed1 ch\u1ea9n \u0111o\u00e1n, CHT v\u00e0 si\u00eau \u00e2m s\u1ebd kh\u00f4ng \u0111\u01b0\u1ee3c t\u1eadp trung trong b\u00e0i \u0111\u00e1nh gi\u00e1 n\u00e0y.
\nTrong tr\u01b0\u1eddng h\u1ee3p c\u00e1c d\u1ea5u hi\u1ec7u l\u00e2m s\u00e0ng cho th\u1ea5y m\u1ed9t b\u1ec7nh c\u1ea3nh CIDP \u0111i\u1ec3n h\u00ecnh, sinh thi\u1ebft th\u1ea7n kinh l\u00e0 kh\u00f4ng c\u1ea7n thi\u1ebft. Tuy nhi\u00ean, sinh thi\u1ebft th\u1ea7n kinh n\u00ean \u0111\u01b0\u1ee3c c\u00e2n nh\u1eafc trong c\u00e1c tr\u01b0\u1eddng h\u1ee3p b\u1ec7nh th\u1ea7n kinh ngo\u1ea1i bi\u00ean ti\u1ebfn tri\u1ec3n nhanh ho\u1eb7c kh\u00e1ng tr\u1ecb, trong nh\u1eefng tr\u01b0\u1eddng h\u1ee3p b\u1ec7nh \u0111a \u1ed5, ho\u1eb7c khi vi\u00eam m\u1ea1ch, b\u1ec7nh m\u1ea1ch m\u00e1u nhi\u1ec5m b\u1ed9t ho\u1eb7c khi nghi ng\u1edd t\u1ed5n th\u01b0\u01a1ng d\u1ea1ng u.<\/p>\n

5. Ca\u0301c \u0111\u0103\u0323c \u0111i\u00ea\u0309m g\u01a1\u0323i y\u0301 CIPD kh\u00f4ng \u0111i\u00ea\u0309n hi\u0300nh ho\u0103\u0323c b\u00ea\u0323nh ly\u0301 gi\u00f4\u0301ng CIPD<\/strong><\/h2>\n

Trong ph\u1ea7n ti\u1ebfp theo, ch\u00fang t\u00f4i s\u1ebd t\u1eadp trung v\u00e0o nh\u1eefng \u0111\u1eb7c \u0111i\u1ec3m l\u00e2m s\u00e0ng v\u00e0 x\u00e9t nghi\u1ec7m m\u00e0 b\u00e1c s\u0129 l\u00e2m s\u00e0ng n\u00ean c\u1ea3nh gi\u00e1c v\u00ec c\u00f3 th\u1ec3 l\u00e0 tri\u1ec7u ch\u1ee9ng c\u1ee7a CIDP kh\u00f4ng \u0111i\u1ec3n h\u00ecnh ho\u1eb7c b\u1ec7nh l\u00fd gi\u1ed1ng CIDP (B\u1ea3ng 1).<\/p>\n

5.1. L\u00e2m s\u00e0ng ti\u1ebfn tri\u1ec3n nhanh (d\u1ef1a v\u00e0o \u0111\u1ecbnh ngh\u0129a)<\/strong><\/em><\/h3>\n

Vi\u00eam \u0111a r\u1ec5 v\u00e0 d\u00e2y th\u1ea7n kinh m\u1ea5t myelin m\u1ea1n t\u00ednh\u00a0l\u00e0 m\u1ed9t b\u1ec7nh l\u00fd m\u1ea1n t\u00ednh c\u00f3 th\u1eddi gian ti\u1ebfn tri\u1ec3n v\u01b0\u1ee3t qu\u00e1 8 tu\u1ea7n. Th\u1ec3 b\u1ec7nh ti\u1ebfn tri\u1ec3n ch\u1eadm th\u01b0\u1eddng g\u1eb7p \u1edf ng\u01b0\u1eddi tr\u01b0\u1edfng th\u00e0nh, trong khi th\u1ec3 b\u1ec7nh v\u1edbi c\u00e1c \u0111\u1ee3t t\u00e1i ph\u00e1t \u2013 lui b\u1ec7nh th\u01b0\u1eddng g\u1eb7p h\u01a1n \u1edf tr\u1ebb nh\u1ecf [23]. H\u1ed9i ch\u1ee9ng Guillian \u2013 Barr\u00e9 n\u00ean \u0111\u01b0\u1ee3c ngh\u0129 t\u1edbi n\u1ebfu c\u00e1c tri\u1ec7u ch\u1ee9ng \u0111\u1ea1t \u0111\u1ec9nh trong v\u00f2ng 4 tu\u1ea7n t\u1eeb th\u1eddi \u0111i\u1ec3m kh\u1edfi ph\u00e1t. C\u00e1c b\u1ec7nh nh\u00e2n Guillian \u2013 Barr\u00e9 c\u00f3 bi\u1ec3u hi\u1ec7n l\u00e2m s\u00e0ng dao \u0111\u1ed9ng li\u00ean quan \u0111\u1ebfn \u0111i\u1ec1u tr\u1ecb c\u0169ng c\u1ea7n ph\u00e2n bi\u1ec7t v\u1edbi CIDP [24]. Ch\u1ea9n \u0111o\u00e1n CIDP c\u00f2n g\u1eb7p kh\u00f3 kh\u0103n b\u1edfi th\u1ec3 b\u1ec7nh kh\u1edfi ph\u00e1t c\u1ea5p t\u00ednh (A-CIDP) v\u1edbi tri\u1ec7u ch\u1ee9ng xu\u1ea5t hi\u1ec7n c\u1ea5p t\u00ednh nh\u01b0ng ti\u1ebfp t\u1ee5c ti\u1ebfn tri\u1ec3n qu\u00e1 4-8 tu\u1ea7n. Hi\u1ec7n t\u1ea1i, A-CIDP \u0111\u01b0\u1ee3c \u0111\u1ecbnh ngh\u0129a l\u00e0 khi c\u00f3 \u00edt nh\u1ea5t 3 \u0111\u1ee3t t\u00e1i ph\u00e1t sau 9 tu\u1ea7n [24]. A-CIDP th\u01b0\u1eddng kh\u00f4ng \u0111\u01b0\u1ee3c xem nh\u01b0 m\u1ed9t th\u1ec3 CIDP kh\u00f4ng \u0111i\u1ec3n h\u00ecnh (v\u1ed1n d\u1ef1a v\u00e0o \u0111\u1eb7c \u0111i\u1ec3m l\u00e2m s\u00e0ng v\u00e0 \u0111i\u1ec7n c\u01a1) cho d\u00f9 t\u00ednh ch\u1ea5t kh\u1edfi ph\u00e1t c\u1ea5p t\u00ednh c\u1ee7a n\u00f3 r\u1ea5t kh\u00f4ng \u0111i\u1ec3n h\u00ecnh. D\u00f9 kh\u00f3 kh\u0103n, n\u00ean ch\u1ea9n \u0111o\u00e1n s\u1edbm v\u00e0 ph\u00e2n bi\u1ec7t v\u1edbi h\u1ed9i ch\u1ee9ng Guillian-Barr\u00e9 v\u00ec A-CIDP c\u1ea7n \u0111i\u1ec1u tr\u1ecb b\u1eb1ng li\u1ec7u ph\u00e1p mi\u1ec5n d\u1ecbch. C\u00f3 m\u1ed9t v\u00e0i \u0111\u1eb7c \u0111i\u1ec3m l\u00e2m s\u00e0ng kh\u00e1 nh\u1ea1y trong vi\u1ec7c ch\u1ea9n \u0111o\u00e1n ph\u00e2n bi\u1ec7t A-CIDP v\u1edbi h\u1ed9i ch\u1ee9ng Guillian- Barr\u00e9. A-CIDP th\u01b0\u1eddng c\u00f3 bi\u1ec3u hi\u1ec7n \u00edt tr\u1ea7m tr\u1ecdng h\u01a1n, kh\u00f4ng c\u1ea7n th\u00f4ng kh\u00ed c\u01a1 h\u1ecdc, hi\u1ebfm khi \u1ea3nh h\u01b0\u1edfng \u0111\u1ebfn d\u00e2y th\u1ea7n kinh s\u1ecd v\u00e0 c\u00f3 \u0111i\u1ec7n c\u01a1 bi\u1ec3u hi\u1ec7n t\u00ecnh tr\u1ea1ng m\u1ea5t myelin \u0111i\u1ec3n h\u00ecnh h\u01a1n khi so v\u1edbi h\u1ed9i ch\u1ee9ng Guillian-Barr\u00e9.
\nC\u00e1c b\u1ec7nh nh\u00e2n tr\u1ea3i qua m\u1ed9t \u0111\u1ee3t b\u1ec7nh v\u00e0 c\u00f3 l\u00e2m s\u00e0ng \u0111\u1ea1t \u0111\u1ec9nh trong 4-8 tu\u1ea7n s\u1ebd \u0111\u01b0\u1ee3c ch\u1ea9n \u0111o\u00e1n v\u1edbi m\u1ed9t nh\u00f3m b\u1ec7nh ri\u00eang bi\u1ec7t l\u00e0 Vi\u00eam \u0111a r\u1ec5 v\u00e0 d\u00e2y th\u1ea7n kinh m\u1ea5t myelin b\u00e1n c\u1ea5p (SIDP) d\u00f9 t\u01b0\u01a1ng \u0111\u1ed1i \u00edt g\u1eb7p tr\u00ean l\u00e2m s\u00e0ng [25,26]. C\u00e1c b\u1ec7nh nh\u00e2n c\u00f3 b\u1ec7nh th\u1ea7n kinh ngo\u1ea1i bi\u00ean kh\u1edfi ph\u00e1t b\u00e1n c\u1ea5p v\u1edbi tri\u1ec7u ch\u1ee9ng run r\u00f5 r\u1ec7t, th\u1ea5t \u0111i\u1ec1u, y\u1ebfu ng\u1ecdn chi, c\u1ea7n ch\u00fa \u00fd ki\u1ec3m tra d\u01b0\u1edbi nh\u00f3m CIDP li\u00ean quan \u0111\u1ebfn eo Ranvier (nodopathy), \u0111\u1eb7c bi\u1ec7t l\u00e0 kh\u00e1ng th\u1ec3 neurofascin-155 v\u00e0\/ho\u1eb7c contactin-1 [27], khi \u0111\u00f3 c\u00e1c protein n\u1eb1m tr\u00ean ho\u1eb7c c\u1ea1nh eo Ranvier l\u00e0 m\u1ee5c ti\u00eau c\u1ee7a kh\u00e1ng th\u1ec3 IgG4, t\u1ea1o th\u00e0nh m\u1ed9t th\u1ec3 b\u1ec7nh CIDP \u00edt g\u1eb7p [28]. Cu\u1ed1i c\u00f9ng, hi\u1ec7u \u1ee9ng \u201cwears off\u201d l\u00e0m c\u00e1c tri\u1ec7u ch\u1ee9ng CIDP x\u1ea5u \u0111i c\u00f3 th\u1ec3 b\u1ecb nh\u1ea7m l\u1eabn v\u1edbi CIDP kh\u00e1ng tr\u1ecb [29].<\/p>\n

5.2. B\u1ec7nh th\u1ea7n kinh ph\u1ee5 thu\u1ed9c chi\u1ec1u d\u00e0i \u01b0u th\u1ebf c\u1ea3m gi\u00e1c h\u01a1n v\u1eadn \u0111\u1ed9ng, b\u1ec7nh th\u1ea7n kinh ngo\u1ea1i bi\u00ean \u01b0u th\u1ebf s\u1ee3i tr\u1ee5c.<\/strong><\/em><\/h3>\n

B\u1ec7nh th\u1ea7n kinh ngo\u1ea1i bi\u00ean ph\u1ee5 thu\u1ed9c chi\u1ec1u d\u00e0i \u01b0u th\u1ebf c\u1ea3m gi\u00e1c h\u01a1n v\u1eadn \u0111\u1ed9ng c\u00f3 th\u1ec3 m\u1eafc ph\u1ea3i do nhi\u1ec1u nguy\u00ean nh\u00e2n v\u00e0 th\u01b0\u1eddng xuy\u00ean b\u1ecb ch\u1ea9n \u0111o\u00e1n nh\u1ea7m v\u1edbi CIDP v\u00ec b\u1ea5t th\u01b0\u1eddng \u0111i\u1ec7n c\u01a1 kh\u00f4ng \u0111\u1eb7c hi\u1ec7u \u0111\u01b0\u1ee3c gi\u1ea3i th\u00edch nh\u01b0 m\u1ed9t t\u00ecnh tr\u1ea1ng m\u1ea5t myelin. \u0110\u1eb7c \u0111i\u1ec3m n\u00e0y t\u01b0\u01a1ng \u0111\u1ed3ng v\u1edbi suy gi\u1ea3m t\u1ed1c \u0111\u1ed9 d\u1eabn truy\u1ec1n m\u1ee9c \u0111\u1ed9 nh\u1eb9 th\u01b0\u1eddng g\u1eb7p tr\u00ean b\u1ec7nh \u0111a d\u00e2y th\u1ea7n kinh do \u0111\u00e1i th\u00e1o \u0111\u01b0\u1eddng ph\u1ee5 thu\u1ed9c chi\u1ec1u d\u00e0i.<\/p>\n

5.3. B\u1ec7nh th\u1ea7n kinh h\u1ea1ch c\u1ea3m gi\u00e1c\/b\u1ec7nh th\u1ea7n kinh ngo\u1ea1i bi\u00ean do tho\u00e1i h\u00f3a (neuronopathy) c\u1ea3m gi\u00e1c kh\u00f4ng ph\u1ee5 thu\u1ed9c chi\u1ec1u d\u00e0i.<\/strong><\/em><\/h3>\n

Ch\u1ea9n \u0111o\u00e1n b\u1ec7nh th\u1ea7n kinh h\u1ea1ch c\u1ea3m gi\u00e1c n\u00ean \u0111\u01b0\u1ee3c ngh\u0129 \u0111\u1ebfn khi b\u1ec7nh nh\u00e2n kh\u1edfi ph\u00e1t v\u1edbi bi\u1ec3u hi\u1ec7n th\u1ea5t \u0111i\u1ec1u r\u00f5 r\u1ec7t c\u00f9ng t\u1ed5n th\u01b0\u01a1ng th\u1ea7n kinh s\u1ee3i l\u1edbn chi\u1ebfm \u01b0u th\u1ebf. Hai nguy\u00ean nh\u00e2n ch\u00ednh c\u1ee7a b\u1ec7nh h\u1ea1ch th\u1ea7n kinh c\u1ea3m gi\u00e1c l\u00e0 b\u1ec7nh h\u1ea1ch th\u1ea7n kinh c\u1eadn u v\u00e0 h\u1ed9i ch\u1ee9ng Sjo\u0308gren. Ngo\u00e0i ra, bi\u1ebfn th\u1ec3 c\u1ea3m gi\u00e1c c\u1ee7a CIDP \u2013 CISP c\u0169ng n\u00ean \u0111\u01b0\u1ee3c l\u01b0u \u00fd [14, 30]. M\u1eb7c d\u00f9 \u0111i\u1ec7n c\u01a1 tr\u00ean c\u00e1c b\u1ec7nh nh\u00e2n CISP \u00edt bi\u1ebfn \u0111\u1ed5i, th\u1eddi gian ti\u1ec1m c\u1ea3m gi\u00e1c b\u1ea3n th\u1ec3 th\u01b0\u1eddng k\u00e9o d\u00e0i, protein d\u1ecbch n\u00e3o t\u1ee7y th\u01b0\u1eddng t\u0103ng v\u00e0 sinh thi\u1ebft r\u1ec5 th\u1ea7n kinh th\u1eaft l\u01b0ng cho th\u1ea5y h\u00ecnh \u1ea3nh m\u1ea5t c\u00e1c s\u1ee3i th\u1ea7n kinh c\u00f3 myelin k\u00edch th\u01b0\u1edbc l\u1edbn, h\u00ecnh \u1ea3nh \u201cc\u1ee7 h\u00e0nh\u201d (b\u1eb1ng ch\u1ee9ng c\u1ee7a t\u00ecnh tr\u1ea1ng m\u1ea5t myelin v\u00e0 t\u00e1i c\u1ea5u tr\u00fac myelin \u0111ang di\u1ec5n ra) v\u00e0 \u0111\u1ea1i th\u1ef1c b\u00e0o t\u1ea1i l\u1edbp v\u1ecf s\u1ee3i th\u1ea7n kinh [14].<\/p>\n

5.4. B\u1ec7nh d\u00e2y th\u1ea7n kinh ngo\u1ea1i bi\u00ean \u01b0u th\u1ebf chi tr\u00ean<\/strong><\/em><\/h3>\n

CIDP \u0111a \u1ed5 (b\u1ec7nh th\u1ea7n kinh v\u1eadn \u0111\u1ed9ng v\u00e0 c\u1ea3m gi\u00e1c m\u1ea5t myelin b\u1ea5t \u0111\u1ed1i x\u1ee9ng \u0111a \u1ed5 [MADSAM] hay h\u1ed9i ch\u1ee9ng Lewis-Sumner) v\u00e0 B\u1ec7nh d\u00e2y th\u1ea7n kinh v\u1eadn \u0111\u1ed9ng \u0111a \u1ed5 (MMN) n\u00ean \u0111\u01b0\u1ee3c c\u00e2n nh\u1eafc tr\u00ean nh\u1eefng b\u1ec7nh nh\u00e2n c\u00f3 b\u1ec7nh th\u1ea7n kinh chi tr\u00ean kh\u1edfi ph\u00e1t b\u1ea5t \u0111\u1ed1i x\u1ee9ng. B\u1ea5t \u0111\u1ed1i x\u1ee9ng \u1edf \u0111\u00e2y \u0111\u01b0\u1ee3c xem l\u00e0 t\u00ecnh tr\u1ea1ng y\u1ebfu c\u01a1 kh\u00f4ng c\u00e2n x\u1ee9ng, t\u1eebng \u1ed5 v\u00e0 t\u1ed5n th\u01b0\u01a1ng ng\u1ecdn chi kh\u00f4ng t\u01b0\u01a1ng x\u1ee9ng, ng\u01b0\u1ee3c l\u1ea1i v\u1edbi CIDP \u0111i\u1ec3n h\u00ecnh v\u1ed1n th\u01b0\u1eddng \u0111\u1ed1i x\u1ee9ng. \u0110\u1ed1i v\u1edbi c\u00e1c b\u1ec7nh th\u1ea7n kinh ngo\u1ea1i bi\u00ean \u01b0u th\u1ebf chi tr\u00ean th\u1ec3 s\u1ee3i tr\u1ee5c, b\u1ec7nh neuron v\u1eadn \u0111\u1ed9ng c\u0169ng l\u00e0 m\u1ed9t ch\u1ea9n \u0111o\u00e1n ph\u00e2n bi\u1ec7t, \u0111\u1eb7c bi\u1ec7t khi b\u1ec7nh nh\u00e2n c\u00f3 teo c\u01a1 r\u00f5 r\u1ec7t v\u00e0 b\u1ea5t \u0111\u1ed1i x\u1ee9ng. Khi c\u00e1c tri\u1ec7u ch\u1ee9ng k\u1ebft h\u1ee3p v\u1edbi \u0111au vai nhi\u1ec1u, n\u00ean ngh\u0129 \u0111\u1ebfn b\u1ec7nh vi\u00eam th\u1ea7n kinh \u0111\u00e1m r\u1ed1i c\u1ed5 (teo c\u01a1 ngu\u1ed3n g\u1ed1c th\u1ea7n kinh hay h\u1ed9i ch\u1ee9ng Parsonage \u2013 Turner) ho\u1eb7c vi\u00eam m\u1ea1ch.
\n\u0110\u1eb7c \u0111i\u1ec3m m\u1ea5t myelin \u1edf b\u1ec7nh nh\u00e2n CIDP \u0111a \u1ed5 bao g\u1ed3m gi\u1ea3m t\u1ed1c \u0111\u1ed9 d\u1eabn truy\u1ec1n, s\u00f3ng F v\u00e0 th\u1eddi gian ti\u1ec1m k\u00e9o d\u00e0i, ph\u00e2n t\u00e1n theo th\u1eddi gian v\u00e0 ch\u1eb9n d\u1eabn truy\u1ec1n. C\u00e1c \u0111\u1eb7c \u0111i\u1ec3m n\u00e0y ng\u01b0\u1ee3c h\u1eb3n so v\u1edbi MMN, khi m\u00e0 \u201cm\u1ea5t myelin\u201d ch\u1ec9 bi\u1ec3u hi\u1ec7n th\u00f4ng qua ch\u1eb9n d\u1eabn truy\u1ec1n v\u1eadn \u0111\u1ed9ng. T\u01b0\u01a1ng t\u1ef1 v\u1edbi CIDP \u0111i\u1ec3n h\u00ecnh, sinh l\u00fd b\u1ec7nh c\u1ee7a CIDP \u0111a \u1ed5 l\u00e0 t\u00ecnh tr\u1ea1ng vi\u00eam m\u1ea5t myelin, kh\u00e1c h\u1eb3n so v\u1edbi sinh l\u00fd b\u1ec7nh c\u1ee7a MMN v\u1ed1n li\u00ean quan \u0111\u1ebfn tho\u00e1i h\u00f3a s\u1ee3i tr\u1ee5c v\u00e0 m\u1ea5t kh\u00f4ng c\u00e2n \u0111\u1ed1i c\u00e1c s\u1ee3i th\u1ea7n kinh c\u00f3 bao myelin. \u0110\u1ed1i v\u1edbi b\u1ec7nh neuron v\u1eadn \u0111\u1ed9ng, \u0111i\u1ec7n c\u01a1 cho th\u1ea5y gi\u1ea3m d\u1eabn truy\u1ec1n v\u1eadn \u0111\u1ed9ng m\u1ee9c \u0111\u1ed9 nh\u1eb9 nh\u01b0ng suy gi\u1ea3m bi\u00ean \u0111\u1ed9 \u0111\u01a1n v\u1ecb v\u1eadn \u0111\u1ed9ng c\u01a1 r\u00f5 r\u1ec7t, c\u0169ng nh\u01b0 c\u00f3 h\u00ecnh \u1ea3nh gi\u1eadt s\u1ee3i c\u01a1 tr\u00ean \u0111i\u1ec7n c\u01a1 kim.<\/p>\n

5.5. B\u1ec7nh th\u1ea7n kinh ngo\u1ea1i bi\u00ean m\u1ea5t myelin v\u1eadn \u0111\u1ed9ng v\u00e0 c\u1ea3m gi\u00e1c<\/strong><\/em><\/h3>\n

Hai nh\u00f3m b\u1ec7nh ch\u00ednh bao g\u1ed3m b\u1ec7nh th\u1ea7n kinh ngo\u1ea1i bi\u00ean m\u1ea5t myelin v\u1eadn \u0111\u1ed9ng v\u00e0 c\u1ea3m gi\u00e1c di truy\u1ec1n hay Charcot-Marie-Tooth lo\u1ea1i 1 (CMT1) v\u00e0 b\u1ec7nh th\u1ea7n kinh ngo\u1ea1i bi\u00ean paraprotein huy\u1ebft (paraprotein l\u00e0 c\u00e1c immunoglobulins \u0111\u01b0\u1ee3c s\u1ea3n xu\u1ea5t d\u01b0 th\u1eeba b\u1edfi c\u00e1c t\u1ebf b\u00e0o huy\u1ebft t\u01b0\u01a1ng ho\u1eb7c lympho B). CMT1 th\u01b0\u1eddng c\u00f3 bi\u1ec3u hi\u1ec7n l\u00e2m s\u00e0ng ti\u1ebfn tri\u1ec3n ch\u1eadm h\u01a1n nhi\u1ec1u. Khi th\u0103m kh\u00e1m, c\u00e1c thi\u1ebfu s\u00f3t v\u1eadn \u0111\u1ed9ng \u2013 c\u1ea3m gi\u00e1c \u01b0u th\u1ebf \u1edf ng\u1ecdn chi, th\u01b0\u1eddng xuy\u00ean k\u1ebft h\u1ee3p v\u1edbi bi\u1ebfn d\u1ea1ng pes cavus (b\u00e0n ch\u00e2n h\u00ecnh v\u00f2m) v\u00e0 ng\u00f3n ch\u00e2n h\u00ecnh b\u00faa, khai th\u00e1c ti\u1ec1n s\u1eed gia \u0111\u00ecnh th\u01b0\u1eddng c\u00f3 ng\u01b0\u1eddi mang b\u1ec7nh. Tr\u00ean \u0111i\u1ec7n c\u01a1, s\u1ef1 m\u1ea5t myelin th\u01b0\u1eddng xuy\u00ean v\u00e0 \u0111\u1ed3ng b\u1ed9 (gi\u1ed1ng nhau gi\u1eefa c\u00e1c d\u00e2y th\u1ea7n kinh v\u00e0 gi\u1eefa c\u00e1c \u0111o\u1ea1n c\u1ee7a c\u00f9ng m\u1ed9t d\u00e2y th\u1ea7n kinh) v\u00e0 kh\u00f4ng c\u00f3 ch\u1eb9n d\u1eabn truy\u1ec1n c\u0169ng nh\u01b0 ph\u00e1t t\u00e1n theo th\u1eddi gian, \u0111\u1ed3ng th\u1eddi th\u1eddi gian ti\u1ec1m s\u00f3ng F kh\u00f4ng k\u00e9o d\u00e0i [32]. H\u01a1n n\u1eefa, sinh thi\u1ebft th\u1ea7n kinh cho th\u1ea5y h\u00ecnh \u1ea3nh \u201cc\u1ee7 h\u00e0nh\u201d \u1edf b\u1ec7nh nh\u00e2n CMT1 th\u01b0\u1eddng lan t\u1ecfa, kh\u00f4ng \u0111a d\u1ea1ng ho\u1eb7c \u0111a \u1ed5 nh\u01b0 tr\u00ean b\u1ec7nh nh\u00e2n CIDP [6].
\nB\u1ec7nh d\u00e2y th\u1ea7n kinh paraprotein huy\u1ebft l\u00e0 nh\u00f3m b\u1ec7nh kh\u00f4ng \u0111\u1ed3ng nh\u1ea5t v\u00e0 c\u00f3 l\u00e2m s\u00e0ng gi\u1ed1ng v\u1edbi CIDP \u0111i\u1ec3n h\u00ecnh (B\u1ea3ng 2). B\u1ec7nh d\u00e2y th\u1ea7n kinh paraprotein huy\u1ebft IgM v\u1edbi ki\u1ec3u h\u00ecnh m\u1ea5t myelin \u0111\u1ed1i x\u1ee9ng \u01b0u th\u1ebf ng\u1ecdn chi m\u1eafc ph\u1ea3i (DADS) l\u00e0 m\u1ed9t b\u1ec7nh th\u1ea7n kinh \u01b0u th\u1ebf c\u1ea3m gi\u00e1c c\u00f3 l\u00e2m s\u00e0ng th\u1ea5t \u0111i\u1ec1u v\u00e0 m\u1ea5t th\u0103ng b\u1eb1ng t\u01b0 th\u1ebf r\u00f5 r\u1ec7t. Kh\u00e1ng th\u1ec3 MAG (glycoprotein li\u00ean quan v\u1edbi myelin) xu\u1ea5t hi\u1ec7n tr\u00ean m\u1ed9t n\u1eeda b\u1ec7nh nh\u00e2n c\u00f3 b\u1ec7nh th\u1ea7n kinh IgM. B\u1ec7nh nh\u00e2n DADS d\u01b0\u01a1ng t\u00ednh v\u1edbi IgM th\u01b0\u1eddng kh\u00e1ng tr\u1ecb v\u1edbi li\u1ec7u ph\u00e1p mi\u1ec5n d\u1ecbch CIDP ti\u00eau chu\u1ea9n. M\u1eb7c d\u00f9 gammopathy \u0111\u01a1n d\u00f2ng tr\u00ean b\u1ec7nh d\u00e2y th\u1ea7n kinh IgM c\u00f3 \u00fd ngh\u0129a kh\u00f4ng r\u00f5 r\u00e0ng (MGUS), s\u1ef1 hi\u1ec7n di\u1ec7n c\u1ee7a gammopathy \u0111\u01a1n d\u00f2ng IgG ho\u1eb7c IgA (\u0111\u1eb7c bi\u1ec7t l\u00e0 lambda) v\u00e0 y\u1ebfu t\u1ed1 t\u0103ng tr\u01b0\u1edfng n\u1ed9i m\u00f4 m\u1ea1ch m\u00e1u (VEGF) c\u00f3 th\u1ec3 li\u00ean quan t\u1edbi kh\u1ed1i u myelin x\u01a1 x\u01b0\u01a1ng, \u0111\u00f4i khi quan s\u00e1t \u0111\u01b0\u1ee3c trong h\u1ed9i ch\u1ee9ng POEMS (polyneuropathy: b\u1ec7nh \u0111a d\u00e2y th\u1ea7n kinh, organomegaly: t\u0103ng k\u00edch th\u01b0\u1edbc t\u1ea1ng, endocrinopathy: b\u1ec7nh l\u00fd h\u1ec7 n\u1ed9i ti\u1ebft, M-protein, skin changes: thay \u0111\u1ed5i da).
\nV\u1ec1 l\u00e2m s\u00e0ng, h\u1ed9i ch\u1ee9ng POEMS bi\u1ec3u hi\u1ec7n v\u1edbi b\u1ec7nh l\u00fd \u0111a r\u1ec5 v\u00e0 d\u00e2y th\u1ea7n kinh m\u1ea5t myelin v\u1eadn \u0111\u1ed9ng v\u00e0 c\u1ea3m gi\u00e1c n\u1eb7ng n\u1ec1 t\u01b0\u01a1ng t\u1ef1 nh\u01b0 CIDP. B\u1ec7nh nh\u00e2n POEMS th\u01b0\u1eddng c\u00f3 c\u00e1c tri\u1ec7u ch\u1ee9ng c\u1ee7a m\u1ed9t b\u1ec7nh h\u1ec7 th\u1ed1ng, m\u1ec7t m\u1ecfi, ph\u00f9 r\u00f5 r\u1ec7t v\u00e0 thay \u0111\u1ed5i \u0111\u1eb7c t\u00ednh da. V\u1edbi l\u00e2m s\u00e0ng t\u0103ng ti\u1ec3u c\u1ea7u tr\u00ean c\u00e1c b\u1ec7nh nh\u00e2n nghi ng\u1edd CIDP, h\u1ed9i ch\u1ee9ng POEMS n\u00ean \u0111\u01b0\u1ee3c s\u00e0ng l\u1ecdc n\u1ebfu c\u00f3 s\u1ef1 hi\u1ec7n di\u1ec7n c\u1ee7a protein \u0111\u01a1n d\u00f2ng [37]. Tr\u00ean l\u00e2m s\u00e0ng, c\u00f3 th\u1ec3 nh\u1eadn di\u1ec7n h\u1ed9i ch\u1ee9ng POEMS d\u1ef1a v\u00e0o m\u1ed9t s\u1ed1 g\u1ee3i \u00fd quan tr\u1ecdng bao g\u1ed3m k\u00e9m \u0111\u00e1p \u1ee9ng v\u1edbi li\u1ec7u ph\u00e1p mi\u1ec5n d\u1ecbch ho\u1eb7c \u0111au nhi\u1ec1u g\u00e2y t\u00e0n ph\u1ebf, v\u1ed1n r\u1ea5t hi\u1ebfm g\u1eb7p v\u1edbi CIDP \u0111i\u1ec3n h\u00ecnh. Nhi\u1ec1u b\u1ec7nh nh\u00e2n POEMS \u0111\u01b0\u1ee3c ch\u1ea9n \u0111o\u00e1n ban \u0111\u1ea7u l\u00e0 CIDP. So v\u1edbi CIDP, t\u1ed5n th\u01b0\u01a1ng \u0111i\u1ec7n c\u01a1 c\u1ee7a b\u1ec7nh nh\u00e2n POEMS bi\u1ec3u hi\u1ec7n t\u00ecnh tr\u1ea1ng m\u1ea5t myelin v\u00e0 tho\u00e1i h\u00f3a s\u1ee3i tr\u1ee5c \u0111\u1ed3ng b\u1ed9 h\u01a1n [38]. Sinh thi\u1ebft th\u1ea7n kinh tr\u00ean b\u1ec7nh nh\u00e2n POEMS cho th\u1ea5y tho\u00e1i h\u00f3a s\u1ee3i tr\u1ee5c v\u00e0 t\u0103ng sinh m\u1ea1ch m\u1edbi v\u1edbi \u00edt h\u00ecnh \u1ea3nh \u201cc\u1ee7 h\u00e0nh\u201d, \u0111\u1ed3ng th\u1eddi m\u1ee9c \u0111\u1ed9 m\u1ea5t myelin c\u0169ng \u00edt h\u01a1n so v\u1edbi khi sinh thi\u1ebft c\u01a1 tr\u00ean b\u1ec7nh nh\u00e2n CIDP [39].<\/p>\n

5.6. B\u1ec7nh \u0111a r\u1ec5 v\u00e0 d\u00e2y th\u1ea7n kinh s\u1ee3i tr\u1ee5c v\u1eadn \u0111\u1ed9ng v\u00e0 c\u1ea3m gi\u00e1c<\/strong><\/em><\/h3>\n

B\u00ean c\u1ea1nh h\u1ed9i ch\u1ee9ng POEMS, b\u1ec7nh l\u00fd \u0111a d\u00e2y th\u1ea7n kinh nhi\u1ec5m b\u1ed9t mang t\u00ednh gia \u0111\u00ecnh (FAP) chu\u1ed7i nh\u1eb9 (AL) v\u00e0 transthyretin (TTR) c\u0169ng c\u00f3 th\u1ec3 bi\u1ec3u hi\u1ec7n v\u1edbi b\u1ec7nh c\u1ea3nh \u0111a r\u1ec5 v\u00e0 d\u00e2y th\u1ea7n kinh \u01b0u th\u1ebf s\u1ee3i tr\u1ee5c v\u1eadn \u0111\u1ed9ng v\u00e0 c\u1ea3m gi\u00e1c [40,41]. Kh\u00e1c v\u1edbi CIDP, b\u1ec7nh th\u1ea7n kinh nhi\u1ec5m b\u1ed9t th\u01b0\u1eddng c\u00f3 tri\u1ec7u ch\u1ee9ng c\u1ee7a th\u1ea7n kinh t\u1ef1 \u0111\u1ed9ng r\u00f5 r\u1ec7t (h\u1ea1 huy\u1ebft \u00e1p t\u01b0 th\u1ebf, r\u1ed1i lo\u1ea1n nhu \u0111\u1ed9ng ru\u1ed9t v\u00e0 r\u1ed1i lo\u1ea1n ch\u1ee9c n\u0103ng c\u01b0\u01a1ng d\u01b0\u01a1ng) [16], g\u00e2y \u0111au th\u1ea7n kinh ti\u1ebfn tri\u1ec3n nhanh v\u00e0 kh\u00e1ng tr\u1ecb v\u1edbi li\u1ec7u ph\u00e1p \u0111i\u1ec1u tr\u1ecb CIDP ti\u00eau chu\u1ea9n. Ngo\u00e0i ra, m\u1ed9t s\u1ed1 b\u1ec7nh l\u00fd kh\u00e1c c\u0169ng c\u00f3 l\u00e2m s\u00e0ng m\u00f4 ph\u1ecfng CIDP. Khi b\u1ec7nh nh\u00e2n c\u00f3 ti\u1ec1n s\u1eed u lympho (\u0111\u1eb7c bi\u1ec7t l\u00e0 u lympho kh\u00f4ng Hodgkin), s\u00fat c\u00e2n v\u00e0 tri\u1ec7u ch\u1ee9ng kh\u00f4ng \u0111\u1ed1i x\u1ee9ng, c\u1ea7n ph\u1ea3i ngh\u0129 \u0111\u1ebfn b\u1ec7nh u lympho t\u1ebf b\u00e0o th\u1ea7n kinh [42]. B\u1ec7nh th\u1ea7n kinh ngo\u1ea1i bi\u00ean r\u1ec5 v\u00e0 \u0111\u00e1m r\u1ed1i c\u1ed9t s\u1ed1ng th\u1eaft l\u01b0ng c\u00f3 ho\u1eb7c kh\u00f4ng li\u00ean quan t\u1edbi \u0111\u00e1i th\u00e1o \u0111\u01b0\u1eddng c\u0169ng c\u00f3 bi\u1ec3u hi\u1ec7n nh\u01b0 m\u1ed9t b\u1ec7nh l\u00fd \u0111a d\u00e2y v\u00e0 r\u1ec5 th\u1ea7n kinh ti\u1ebfn tri\u1ec3n, tuy nhi\u00ean kh\u00e1c v\u1edbi CIDP, b\u1ec7nh nh\u00e2n th\u01b0\u1eddng \u0111au d\u1eef d\u1ed9i t\u1ea1i th\u1eddi \u0111i\u1ec3m kh\u1edfi ph\u00e1t [43]. M\u1eb7c d\u00f9 v\u1eady, b\u1ec7nh d\u00e2y th\u1ea7n kinh r\u1ec5 v\u00e0 \u0111\u00e1m r\u1ed1i th\u1eaft l\u01b0ng do \u0111\u00e1i th\u00e1o \u0111\u01b0\u1eddng c\u0169ng c\u00f3 th\u1ec3 kh\u00f4ng \u0111au, \u0111\u1eb7c bi\u1ec7t khi tri\u1ec7u ch\u1ee9ng xu\u1ea5t hi\u1ec7n \u1edf c\u1ea3 t\u1ee9 chi, t\u1ed5n th\u01b0\u01a1ng s\u1ee3i tr\u1ee5c \u0111\u1ed1i x\u1ee9ng v\u00e0 sinh thi\u1ebft th\u1ea7n kinh c\u00f3 th\u1ec3 c\u00f3 h\u00ecnh \u1ea3nh nh\u1ed3i m\u00e1u v\u00e0 vi\u00eam vi m\u1ea1ch (b\u1ec7nh d\u00e2y th\u1ea7n kinh r\u1ec5 v\u00e0 \u0111\u00e1m r\u1ed1i th\u1eaft l\u01b0ng do \u0111\u00e1i th\u00e1o \u0111\u01b0\u1eddng g\u00e2y \u0111au c\u0169ng c\u00f3 th\u1ec3 c\u00f3 h\u00ecnh \u1ea3nh n\u00e0y) [44]. V\u1edbi nh\u1eefng b\u1ec7nh nh\u00e2n vi\u00eam \u0111a r\u1ec5 v\u00e0 d\u00e2y th\u1ea7n kinh \u01b0u th\u1ebf s\u1ee3i tr\u1ee5c v\u1eadn \u0111\u1ed9ng c\u00f3 bi\u1ec3u hi\u1ec7n l\u00e2m s\u00e0ng gi\u1ed1ng CIDP nh\u01b0ng kh\u00f4ng th\u1ecfa m\u00e3n ti\u00eau ch\u00ed tr\u00ean \u0111i\u1ec7n c\u01a1, n\u00ean \u0111i\u1ec1u tr\u1ecb b\u1eb1ng li\u1ec7u ph\u00e1p mi\u1ec5n d\u1ecbch v\u00e0 \u0111\u00e1nh gi\u00e1 \u0111\u00e1p \u1ee9ng m\u1ed9t c\u00e1ch kh\u00e1ch quan sau v\u00e0i th\u00e1ng, n\u1ebfu c\u00f3 ti\u1ebfn tri\u1ec3n s\u1ebd ti\u1ebfp t\u1ee5c li\u1ec7u tr\u00ecnh. Ng\u01b0\u1ee3c l\u1ea1i, d\u1eebng \u0111i\u1ec1u tr\u1ecb n\u1ebfu kh\u00f4ng c\u00f3 c\u1ea3i thi\u1ec7n.<\/p>\n

6. Ca\u0301c ph\u01b0\u01a1ng pha\u0301p \u0111i\u00ea\u0300u tri\u0323 \u0111ang s\u01b0\u0309 du\u0323ng: Ti\u00eau chu\u00e2\u0309n th\u01b0\u0323c ha\u0300nh va\u0300 so sa\u0301nh hi\u00ea\u0323u qua\u0309<\/strong><\/h2>\n

Nguy\u00ean t\u1eafc \u0111i\u1ec1u tr\u1ecb tr\u00ean b\u1ec7nh nh\u00e2n CIDP l\u00e0 \u0111\u1ea1t \u0111\u01b0\u1ee3c \u0111\u1ee3t lui b\u1ec7nh ho\u1eb7c gi\u00fap c\u1ea3i thi\u1ec7n ch\u1ee9c n\u0103ng v\u00e0 c\u01a1 l\u1ef1c m\u1ed9t c\u00e1ch c\u00f3 \u00fd ngh\u0129a. Nhi\u1ec1u b\u1ec7nh nh\u00e2n c\u1ea7n \u0111i\u1ec1u tr\u1ecb trong m\u1ed9t kho\u1ea3ng th\u1eddi gian d\u00e0i, trong khi m\u1ed9t s\u1ed1 kh\u00e1c l\u1ea1i nhanh ch\u00f3ng lui b\u1ec7nh. D\u00f9 \u00e1p d\u1ee5ng ph\u01b0\u01a1ng ph\u00e1p n\u00e0o, li\u1ec1u v\u00e0 s\u1ed1 l\u1ea7n \u0111i\u1ec1u tr\u1ecb n\u00ean \u0111\u01b0\u1ee3c \u0111i\u1ec1u ch\u1ec9nh cho ph\u00f9 h\u1ee3p v\u1edbi m\u1ee9c \u0111\u1ed9 n\u1eb7ng c\u1ee7a b\u1ec7nh nh\u00e2n tr\u00ean nguy\u00ean t\u1eafc \u0111em l\u1ea1i hi\u1ec7u qu\u1ea3 l\u1edbn nh\u1ea5t v\u1edbi li\u1ec1u \u0111i\u1ec1u tr\u1ecb th\u1ea5p nh\u1ea5t.<\/p>\n

6.1. IVIg<\/strong><\/em><\/h3>\n

C\u00e1c khuy\u1ebfn c\u00e1o hi\u1ec7n t\u1ea1i trong th\u1ef1c h\u00e0nh l\u00e2m s\u00e0ng t\u1eeb EFNS\/PNS \u0111\u1ec1u \u1ee7ng h\u1ed9 s\u1eed d\u1ee5ng li\u1ec1u IVIg th\u1ea5p nh\u1ea5t c\u00f3 hi\u1ec7u qu\u1ea3 k\u1ebft h\u1ee3p v\u1edbi gi\u1ea3m li\u1ec1u theo chu k\u1ef3 [9]. T\u1eeb n\u0103m 1994, hi\u1ec7u qu\u1ea3 c\u1ee7a IVIg tr\u00ean b\u1ec7nh nh\u00e2n CIDP \u0111\u00e3 \u0111\u01b0\u1ee3c m\u00f4 t\u1ea3 trong m\u1ed9t nghi\u00ean c\u1ee9u b\u1eaft ch\u00e9o so s\u00e1nh IVIg li\u1ec1u 0,4g\/kg m\u1ed7i tu\u1ea7n v\u1edbi trao \u0111\u1ed5i huy\u1ebft t\u01b0\u01a1ng v\u00e0 c\u1ea3 hai ph\u01b0\u01a1ng ph\u00e1p \u0111\u1ec1u cho th\u1ea5y c\u1ea3i thi\u1ec7n tr\u00ean l\u00e2m s\u00e0ng (\u0111\u00e1nh gi\u00e1 th\u00f4ng qua thang \u0111i\u1ec3m t\u1ed5n th\u01b0\u01a1ng th\u1ea7n kinh NIS) [45]. M\u1ed9t th\u1eed nghi\u1ec7m ch\u00e9o, nh\u00f3m ch\u1ee9ng \u2013 ki\u1ec3m so\u00e1t, \u0111a trung t\u00e2m quy m\u00f4 l\u1edbn ch\u1ee9ng minh hi\u1ec7u qu\u1ea3 c\u1ee7a IVIg li\u1ec1u 1g\/kg m\u1ed7i 3 tu\u1ea7n trong c\u1ea3 li\u1ec7u tr\u00ecnh ng\u1eafn v\u00e0 k\u00e9o d\u00e0i \u0111\u00e3 \u0111\u01b0\u1ee3c FDA ch\u1ea5p thu\u1eadn [46]. M\u1ed9t nghi\u00ean c\u1ee9u \u0111a trung t\u00e2m, ng\u1eabu nhi\u00ean gi\u1eefa hai nh\u00f3m IVIg v\u00e0 methylprednisolone \u0111\u01b0\u1eddng t\u0129nh m\u1ea1ch (IVMP) k\u1ebft lu\u1eadn hi\u1ec7u qu\u1ea3 c\u00f3 t\u00ednh c\u1ea1nh tranh khi s\u1eed d\u1ee5ng IVIg li\u1ec1u 0,5g\/kg m\u1ed7i ng\u00e0y trong 4 ng\u00e0y, h\u00e0ng th\u00e1ng trong 6 th\u00e1ng [47]. Qua \u0111\u00f3 c\u00f3 th\u1ec3 th\u1ea5y, hi\u1ec7u qu\u1ea3 c\u1ee7a IVIg ng\u00e0y c\u00e0ng \u0111\u01b0\u1ee3c ch\u1ee9ng minh. Theo m\u1ed9t \u0111\u00e1nh gi\u00e1 c\u1ee7a Cochrane n\u0103m 2013, m\u1ee9c \u0111\u1ed9 t\u00e0n t\u1eadt c\u1ee7a b\u1ec7nh nh\u00e2n CIDP s\u1ebd \u0111\u01b0\u1ee3c c\u1ea3i thi\u1ec7n trong \u00edt nh\u1ea5t 2 \u0111\u1ebfn 6 tu\u1ea7n v\u1edbi NNT l\u00e0 3 [48]. Tuy v\u1eady, kh\u00f4ng ph\u1ea3i m\u1ecdi b\u1ec7nh nh\u00e2n CIDP \u0111\u1ec1u \u0111\u01b0\u1ee3c h\u01b0\u1edfng l\u1ee3i t\u1eeb IVIg. Tr\u00ean nh\u00f3m b\u1ec7nh CIDP v\u1edbi kh\u00e1ng th\u1ec3 n\u1ed1t \u2013 c\u1eadn n\u1ed1t (nodopathy) neurofascin \u2013 155 v\u00e0 contactin \u2013 1 (thu\u1ed9c d\u01b0\u1edbi nh\u00f3m IgG4), IVIg kh\u00f4ng ch\u1ee9ng minh \u0111\u01b0\u1ee3c hi\u1ec7u qu\u1ea3 do IgG4 ho\u1ea1t h\u00f3a kh\u00f4ng ho\u00e0n to\u00e0n v\u00e0 c\u00f3 t\u00ednh h\u1ea5p d\u1eabn y\u1ebfu v\u1edbi th\u1ee5 th\u1ec3 Fc c\u1ee7a t\u1ebf b\u00e0o hi\u1ec7u l\u1ef1c [49,50].<\/p>\n

B\u1ea3ng 2. B\u1ec7nh th\u1ea7n kinh ngo\u1ea1i bi\u00ean paraprotein huy\u1ebft gi\u1ed1ng CIDP<\/em><\/p>\n\n\n\n\n\n\n\n\n
D\u01b0\u1edbi nh\u00f3m kh\u00e1ng th\u1ec3\u00a0<\/strong>\u0111\u01a1<\/strong>n d\u00f2ng<\/strong><\/td>\nR\u1ed1i lo\u1ea1n t\u1ebf b\u00e0o huy\u1ebft t\u01b0\u01a1ng<\/strong><\/td>\n\u0110\u1eb7<\/strong>c<\/strong>\u00a0\u0111<\/strong>i\u1ec3m t\u1ed5n th\u01b0\u01a1ng TK ngo\u1ea1i bi\u00ean<\/strong><\/td>\nR\u1ed1i lo\u1ea1n TK TV<\/strong><\/td>\nTri\u1ec7u ch\u1ee9ng b\u1ec7nh h\u1ec7 th\u1ed1ng<\/strong><\/td>\nCh\u1ea5t ch\u1ec9 \u0111i\u1ec3m<\/strong><\/p>\n

h\u1eefu \u00edch<\/strong><\/td>\n<\/tr>\n

IgM kappa ho\u1eb7c lambda<\/td>\nMGUS<\/td>\nM\u1ea5t myelin \u01b0u th\u1ebf c\u1ea3m gi\u00e1c, ph\u1ee5 thu\u1ed9c chi\u1ec1u d\u00e0i<\/td>\n\u2013<\/td>\n\u2013<\/td>\nKh\u00e1ng th\u1ec3 MAG<\/td>\n<\/tr>\n
<\/td>\nH\u1ed9i ch\u1ee9ng Wandenstrom macroglobulin ni\u1ec7u<\/td>\nB\u1ec7nh th\u1ea7n kinh ngo\u1ea1i bi\u00ean t\u01b0\u01a1ng t\u1ef1 MGUS \u2013 IgM li\u00ean quan s\u1ee3i tr\u1ee5c nhi\u1ec1u h\u01a1n<\/td>\n\u2013<\/td>\nC\u00f3<\/td>\nHemoglobin, ti\u1ec3u c\u1ea7u, n\u1ed3ng \u0111\u1ed9 IgM, beta 2 microglobulin<\/td>\n<\/tr>\n
IgA ho\u1eb7c IgG lambda<\/td>\nH\u1ed9i ch\u1ee9ng POEMS<\/td>\nB\u1ec7nh \u0111a r\u1ec5 v\u00e0 d\u00e2y th\u1ea7n kinh v\u1eadn \u0111\u1ed9ng v\u00e0 c\u1ea3m gi\u00e1c, t\u1ed5n th\u01b0\u01a1ng m\u1ea5t myelin nhi\u1ec1u h\u01a1n s\u1ee3i tr\u1ee5c.<\/td>\n\u2013<\/td>\nC\u00f3<\/td>\nTi\u1ec3u c\u1ea7u (gi\u1ea3m ti\u1ec3u c\u1ea7u, VEGF) x\u00e9t nghi\u1ec7m h\u1ec7 n\u1ed9i ti\u1ebft<\/p>\n

 <\/td>\n<\/tr>\n

B\u1ea5t k\u1ec3 d\u01b0\u1edbi nh\u00f3m n\u00e0o g\u1ed3m duy nh\u1ea5t chu\u1ed7i nh\u1eb9<\/td>\nB\u1ec7nh nhi\u1ec5m b\u1ed9t chu\u1ed7i nh\u1eb9 m\u1eafc ph\u1ea3i<\/td>\nB\u1ec7nh s\u1ee3i tr\u1ee5c v\u1eadn \u0111\u1ed9ng v\u00e0 c\u1ea3m gi\u00e1c (ho\u1eb7c b\u1ec7nh \u0111a r\u1ec5 v\u00e0 d\u00e2y th\u1ea7n kinh) ph\u1ee5 thu\u1ed9c chi\u1ec1u d\u00e0i<\/td>\n\u2013<\/td>\nC\u00f3 (Th\u1ec3 tr\u1ea1ng \u1ed1m y\u1ebfu)<\/td>\nProtein ni\u1ec7u 24h, c\u00f4ng th\u1ee9c m\u00e1u, creatinin, alkaline phosphate, troponin, peptid b\u00e0i ni\u1ec7u n\u00e3o, ho\u1eb7c n\u1ed3ng \u0111\u1ed9 proBNP<\/td>\n<\/tr>\n
AL: b\u1ec7nh nhi\u1ec5m b\u1ed9t chu\u1ed7i nh\u1eb9 m\u1eafc ph\u1ea3i, BNP: protein b\u00e0i ni\u1ec7u n\u00e3o; DADS: b\u1ec7nh th\u1ea7n kinh ngo\u1ea1i bi\u00ean c\u1ea3m gi\u00e1c v\u1eadn \u0111\u1ed9ng m\u1eafc ph\u1ea3i \u01b0u th\u1ebf ng\u1ecdn chi; glycoprotein li\u00ean quan myelin; MGUS, gammopathy \u0111\u01a1n d\u00f2ng kh\u00f4ng c\u00f3 \u00fd ngh\u0129a kh\u00f4ng x\u00e1c \u0111\u1ecbnh; NCS: kh\u1ea3o s\u00e1t d\u1eabn truy\u1ec1n th\u1ea7n kinh; NT proBNP, peptide b\u00e0i ni\u1ec7u n\u00e3o; POEMS, b\u1ec7nh \u0111a d\u00e2y th\u1ea7n kinh, ph\u00ec \u0111a t\u1eb7ng, b\u1ec7nh l\u00fd h\u1ec7 n\u1ed9i ti\u1ebft, protein \u2013 M v\u00e0 thay \u0111\u1ed5i \u0111\u1eb7c t\u00ednh da; VEGF, y\u1ebfu t\u1ed1 t\u0103ng tr\u01b0\u1eddng n\u1ed9i m\u1ea1c m\u1ea1ch m\u00e1u.<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n

Li\u1ec1u IVIg t\u1ed1i \u01b0u v\u1eabn \u0111ang \u0111\u01b0\u1ee3c ti\u1ebfp t\u1ee5c nghi\u00ean c\u1ee9u, ph\u1ed5 bi\u1ebfn nh\u1ea5t l\u00e0 \u0111i\u1ec1u tr\u1ecb m\u1ed7i 3 ho\u1eb7c 4 tu\u1ea7n (d\u1ef1a tr\u00ean th\u1eed nghi\u1ec7m ICE v\u00e0 IMC) v\u00e0 d\u01b0\u1eddng nh\u01b0 \u0111\u00e2y l\u00e0 li\u1ec1u trung b\u00ecnh c\u00f3 hi\u1ec7u qu\u1ea3 \u1edf \u0111a s\u1ed1 ng\u01b0\u1eddi b\u1ec7nh. B\u00ean c\u1ea1nh \u0111\u00f3, trong \u0111i\u1ec1u tr\u1ecb CIDP, nguy\u00ean t\u1eafc quan tr\u1ecdng nh\u1ea5t l\u00e0 kh\u00f4ng c\u00f3 m\u1ed9t li\u1ec1u ti\u00eau chu\u1ea9n tr\u00ean m\u1ecdi ng\u01b0\u1eddi b\u1ec7nh m\u00e0 c\u1ea7n ph\u1ea3i \u0111i\u1ec1u ch\u1ec9nh ph\u1ee5 thu\u1ed9c v\u00e0o nhu c\u1ea7u v\u00e0 \u0111\u00e1p \u1ee9ng c\u1ee7a t\u1eebng b\u1ec7nh nh\u00e2n. M\u1ed9t v\u00e0i nghi\u00ean c\u1ee9u \u1ee7ng h\u1ed9 quan \u0111i\u1ec3m d\u1eebng IVIg \u0111\u1ed9t ng\u1ed9t. Li\u1ec1u n\u1ea1p ti\u00eau chu\u1ea9n 2g\/kg \u0111\u01b0\u1ee3c s\u1eed d\u1ee5ng v\u00e0 nh\u1eafc l\u1ea1i sau 6 tu\u1ea7n n\u1ebfu l\u00e2m s\u00e0ng kh\u00f4ng \u1ed5n \u0111\u1ecbnh ho\u00e0n to\u00e0n. Sau \u0111\u00f3, b\u00e1c s\u1ef9 s\u1ebd \u0111\u00e1nh gi\u00e1 m\u1ee9c \u0111\u1ed9 ti\u1ebfn tri\u1ec3n v\u00e0 s\u1eed d\u1ee5ng kho\u1ea3ng th\u1eddi gian \u0111\u1ebfn l\u00fac c\u1ea3i thi\u1ec7n nh\u01b0 m\u1ed9t th\u00f4ng s\u1ed1 cho nh\u1eefng l\u1ea7n \u0111i\u1ec1u tr\u1ecb sau [51]. M\u1ed9t v\u00e0i nghi\u00ean c\u1ee9u kh\u00e1c \u1ee7ng h\u1ed9 quan \u0111i\u1ec3m t\u1ed1i \u01b0u h\u00f3a li\u1ec1u IVIg b\u1eb1ng vi\u1ec7c s\u1eed d\u1ee5ng n\u1ed3ng \u0111\u1ed9 IVIg nh\u01b0 m\u1ed9t ch\u1ea5t ch\u1ec9 \u0111i\u1ec3m sinh h\u1ecdc (ch\u00eanh l\u1ec7ch IgG tr\u01b0\u1edbc v\u00e0 sau \u0111i\u1ec1u tr\u1ecb), vi\u1ec7c l\u00e0m n\u00e0y s\u1ebd gi\u00fap c\u00e1 th\u1ec3 h\u00f3a \u0111i\u1ec1u tr\u1ecb m\u1eb7c d\u00f9 ch\u00fang kh\u00f4ng t\u01b0\u01a1ng quan v\u1edbi c\u1ea3i thi\u1ec7n l\u00e2m s\u00e0ng m\u1ed9t c\u00e1ch t\u1ed1t nh\u1ea5t [52-54]. \u0110\u00e1nh gi\u00e1 t\u00e1c \u0111\u1ed9ng kinh t\u1ebf v\u00e0 l\u00e2m s\u00e0ng gi\u1eefa c\u00e1c li\u1ec1u \u0111i\u1ec1u tr\u1ecb, Rajabally v\u00e0 Afzal cho r\u1eb1ng li\u1ec1u c\u00e1 th\u1ec3 h\u00f3a \u0111em l\u1ea1i hi\u1ec7u qu\u1ea3 \u0111i\u1ec1u tr\u1ecb kh\u00f4ng thua k\u00e9m, \u0111\u1ed3ng th\u1eddi ti\u1ebft ki\u1ec7m h\u01a1n so v\u1edbi li\u1ec1u ti\u00eau chu\u1ea9n [55]. C\u00e1c nghi\u00ean c\u1ee9u hi\u1ec7n \u0111ang \u0111\u01b0\u1ee3c th\u1ef1c hi\u1ec7n t\u1eadp trung v\u00e0o ba li\u1ec1u kh\u00e1c nhau, bao g\u1ed3m duy tr\u00ec t\u1ea7n s\u1ed1 m\u1ed7i 3 tu\u1ea7n, gi\u1ea3m li\u1ec1u v\u00e0 t\u0103ng t\u1ea7n s\u1ed1 (<14 ng\u00e0y) [54-57]. C\u01a1 s\u1edf cho vi\u1ec7c s\u1eed d\u1ee5ng IVIg d\u00e0i h\u1ea1n tr\u00ean b\u1ec7nh nh\u00e2n CIDP d\u1ef1a v\u00e0o d\u01b0\u1ee3c \u0111\u1ed9ng h\u1ecdc c\u1ee7a ch\u00fang [29-58]. Sau khi truy\u1ec1n t\u0129nh m\u1ea1ch, n\u1ed3ng \u0111\u1ed9 IgG \u0111\u1ea1t \u0111\u1ec9nh ngay l\u1eadp t\u1ee9c v\u00e0 gi\u1ea3m d\u1ea7n trong v\u00f2ng 2 \u0111\u1ebfn 4 ng\u00e0y khi IgG x\u00e2m nh\u1eadp v\u00e0o kho\u1ea3ng ngo\u1ea1i b\u00e0o. Th\u1eddi gian b\u00e1n th\u1ea3i c\u1ee7a IgG dao \u0111\u1ed9ng t\u1eeb 21 \u0111\u1ebfn 30 ng\u00e0y v\u00e0 ch\u00fang s\u1ebd nhanh ch\u00f3ng gi\u00e1ng h\u00f3a khi \u0111i v\u00e0o kho\u1ea3ng n\u1ed9i b\u00e0o m\u00e0 ch\u00eca kh\u00f3a cho qu\u00e1 tr\u00ecnh n\u00e0y l\u00e0 th\u1ee5 th\u1ec3 FcRn c\u1ee7a t\u1ebf b\u00e0o n\u1ed9i m\u1ea1ch b\u00e3o h\u00f2a, v\u1ed1n c\u00f3 ch\u1ee9c n\u0103ng b\u1ea3o v\u1ec7 IgG t\u1eeb qu\u00e1 tr\u00ecnh nh\u1eadp b\u00e0o v\u00e0 gi\u00e1ng h\u00f3a ti\u00eau th\u1ec3 [29]. H\u01a1n n\u1eefa, c\u00f3 s\u1ef1 kh\u00e1c bi\u1ec7t l\u1edbn gi\u1eefa ch\u00eanh l\u1ec7ch n\u1ed3ng \u0111\u1ed9 IgG t\u1eeb \u201c\u0111\u00e1y t\u1edbi \u0111\u1ec9nh\u201d khi so s\u00e1nh gi\u1eefa li\u1ec1u IVIg cao, th\u01b0a (2g\/kg m\u1ed7i th\u00e1ng) v\u1edbi li\u1ec1u IgG th\u1ea5p, mau ti\u00eam d\u01b0\u1edbi da (0,5g\/kg m\u1ed7i tu\u1ea7n) [59].
\nTrong qu\u00e1 tr\u00ecnh \u0111i\u1ec1u tr\u1ecb, b\u1ec7nh nh\u00e2n c\u00f3 th\u1ec3 xu\u1ea5t hi\u1ec7n c\u00e1c t\u00e1c d\u1ee5ng ph\u1ee5 nh\u01b0 \u0111au \u0111\u1ea7u, ban ngo\u00e0i da [60] v\u00e0 nghi\u00eam tr\u1ecdng h\u01a1n c\u1ea3 l\u00e0 c\u00e1c bi\u1ebfn c\u1ed1 g\u00e2y huy\u1ebft kh\u1ed1i. C\u1ea7n c\u1ea9n tr\u1ecdng khi s\u1eed d\u1ee5ng IVIg \u1edf c\u00e1c b\u1ec7nh nh\u00e2n c\u00f3 b\u1ec7nh m\u1ea1ch v\u00e0nh, ti\u1ec1n s\u1eed nh\u1ed3i m\u00e1u c\u01a1 tim g\u1ea7n \u0111\u00e2y, \u0111\u1ed9t qu\u1ef5 ho\u1eb7c c\u00e1c b\u1ec7nh l\u00fd huy\u1ebft kh\u1ed1i kh\u00e1c, t\u00ecnh tr\u1ea1ng t\u0103ng \u0111\u00f4ng (m\u1eafc ph\u1ea3i ho\u1eb7c c\u00f3 t\u00ednh gia \u0111\u00ecnh), s\u1eed d\u1ee5ng thu\u1ed1c tr\u00e1nh thai \u0111\u01b0\u1eddng u\u1ed1ng ho\u1eb7c c\u00f3 k\u1ebf ho\u1ea1ch \u0111i du l\u1ecbch. S\u1eed d\u1ee5ng IVIg s\u1ebd l\u00e0m t\u0103ng nguy c\u01a1 t\u1ed5n th\u01b0\u01a1ng th\u1eadn tr\u00ean b\u1ec7nh nh\u00e2n cao tu\u1ed5i, c\u00f3 b\u1ec7nh th\u1eadn k\u00e8m theo v\u00e0 ti\u1ec1n s\u1eed ti\u1ec3u \u0111\u01b0\u1eddng. C\u00f3 th\u1ec3 d\u1ef1 ph\u00f2ng ph\u1ea3n \u1ee9ng d\u1ecb \u1ee9ng v\u00e0 \u0111au \u0111\u1ea7u b\u1eb1ng vi\u1ec7c \u0111i\u1ec1u tr\u1ecb tr\u01b0\u1edbc \u0111\u00f3 v\u1edbi kh\u00e1ng histamine, corticosteroids ho\u1eb7c thu\u1ed1c kh\u00e1ng vi\u00eam kh\u00f4ng steroid. M\u1ed9t nghi\u00ean c\u1ee9u \u0111\u00e3 cho th\u1ea5y kh\u00f4ng c\u00f3 s\u1ef1 kh\u00e1c bi\u1ec7t trong vi\u1ec7c h\u00ecnh th\u00e0nh c\u00e1c bi\u1ebfn c\u1ed1 huy\u1ebft kh\u1ed1i gi\u1eefa nh\u00f3m \u0111i\u1ec1u tr\u1ecb IVIg li\u1ec1u h\u00e0ng ng\u00e0y ho\u1eb7c h\u00e0ng th\u00e1ng, tuy v\u1eady c\u00e1c y\u1ebfu t\u1ed1 nguy c\u01a1 b\u1ec7nh m\u1ea1ch m\u00e1u n\u00ean \u0111\u01b0\u1ee3c s\u00e0ng l\u1ecdc tr\u01b0\u1edbc khi \u0111i\u1ec1u tr\u1ecb v\u1edbi IVIg [61]. Ch\u1ee9c n\u0103ng th\u1eadn c\u0169ng c\u1ea7n \u0111\u01b0\u1ee3c theo d\u00f5i \u0111\u1ecbnh k\u1ef3. Do sucrose c\u00f3 th\u1ec3 l\u00e0m gia t\u0103ng t\u1ed5n th\u01b0\u01a1ng th\u1eadn v\u00e0 glucose g\u00e2y t\u0103ng \u0111\u01b0\u1eddng m\u00e1u, n\u00ean s\u1eed d\u1ee5ng dung m\u00f4i t\u1eeb c\u00e1c dung d\u1ecbch \u00e1p l\u1ef1c th\u1ea9m th\u1ea5u th\u1ea5p.<\/p>\n

6.2. Immunoglobulin d\u01b0\u1edbi da<\/strong><\/em><\/h3>\n

Trong qu\u00e1 tr\u00ecnh \u0111\u00e1nh gi\u00e1 hi\u1ec7u qu\u1ea3 c\u1ee7a IVIg [46,48], ch\u00fang t\u00f4i lu\u00f4n t\u1ef1 h\u1ecfi li\u1ec7u immunoglobulin c\u00f3 th\u1ec3 \u0111\u01b0\u1ee3c s\u1eed d\u1ee5ng m\u1ed9t c\u00e1ch thu\u1eadn ti\u1ec7n v\u00e0 \u00edt t\u00e1c d\u1ee5ng ph\u1ee5 h\u01a1n kh\u00f4ng? Immunoglobulin ti\u00eam d\u01b0\u1edbi da (SCIg) xu\u1ea5t hi\u1ec7n nh\u01b0 m\u1ed9t l\u1ef1a ch\u1ecdn thay th\u1ebf ti\u1ec1m n\u0103ng cho IVIg . T\u00ednh hi\u1ec7u qu\u1ea3 v\u00e0 d\u1ec5 dung n\u1ea1p c\u1ee7a SCIg \u0111\u00e3 \u0111\u01b0\u1ee3c x\u00e1c nh\u1eadn qua m\u1ed9t th\u1eed nghi\u1ec7m qu\u1ed1c t\u1ebf nh\u00f3m ch\u1ee9ng c\u00f3 ki\u1ec3m so\u00e1t, ng\u1eabu nhi\u00ean, quy m\u00f4 l\u1edbn [62]. Theo \u0111\u00f3, li\u1ec1u cao (0,4g\/kg) v\u00e0 li\u1ec1u th\u1ea5p (0,2g\/kg) \u0111\u01b0\u1ee3c s\u1eed d\u1ee5ng m\u1ed7i tu\u1ea7n tuy kh\u00f4ng cho th\u1ea5y s\u1ef1 kh\u00e1c bi\u1ec7t gi\u1eefa hai nh\u00f3m v\u1edbi nhau nh\u01b0ng \u0111\u1ec1u hi\u1ec7u qu\u1ea3 h\u01a1n nh\u00f3m ch\u1ee9ng. So v\u1edbi IVIg, t\u00e1c d\u1ee5ng ph\u1ee5 t\u1ed5ng th\u1ec3 c\u1ee7a SCIg l\u00e0 \u00edt h\u01a1n.
\nTr\u1edf ng\u1ea1i ch\u1ee7 y\u1ebfu trong \u0111i\u1ec1u tr\u1ecb b\u1eb1ng SCIg l\u00e0 g\u00e2y k\u00edch \u1ee9ng da ph\u1ee5 thu\u1ed9c li\u1ec1u nh\u01b0ng s\u1ebd gi\u1ea3m d\u1ea7n theo th\u1eddi gian [63]. V\u1edbi nh\u1eefng b\u1ec7nh nh\u00e2n kh\u00f3 ti\u1ebfp c\u1eadn ho\u1eb7c c\u00f3 t\u00e1c d\u1ee5ng ph\u1ee5 v\u1edbi \u0111\u01b0\u1eddng truy\u1ec1n t\u0129nh m\u1ea1ch, c\u00f3 hi\u1ec7u \u1ee9ng \u201ct\u1eaft\u201d cu\u1ed1i li\u1ec1u hay \u0111\u01a1n gi\u1ea3n v\u00ec t\u00ednh ti\u1ec7n l\u1ee3i, c\u00e2n nh\u1eafc l\u1ef1a ch\u1ecdn SCIg thay th\u1ebf IVIg v\u1ec1 c\u01a1 b\u1ea3n l\u00e0 an to\u00e0n v\u00e0 h\u1ee3p l\u00fd [64]. Tuy v\u1eady, do ch\u01b0a c\u00f3 \u0111\u1ea7y \u0111\u1ee7 b\u1eb1ng ch\u1ee9ng v\u1ec1 l\u1ee3i \u00edch c\u1ee7a SCIg tr\u00ean CIDP ch\u01b0a \u0111i\u1ec1u tr\u1ecb, b\u1ec7nh nh\u00e2n c\u1ea7n ph\u1ea3i \u1ed5n \u0111\u1ecbnh v\u1edbi IVIg tr\u01b0\u1edbc khi chuy\u1ec3n sang SCIg.<\/p>\n

6.3. CCS (Corticosteroid)<\/strong><\/em><\/h3>\n

M\u1eb7c d\u00f9 kh\u00f4ng th\u1ec3 ph\u1ee7 nh\u1eadn t\u00ednh hi\u1ec7u qu\u1ea3 c\u1ee7a corticosteroid, m\u1ed1i b\u1eadn t\u00e2m ch\u1ee7 y\u1ebfu v\u1edbi li\u1ec7u ph\u00e1p n\u00e0y xu\u1ea5t ph\u00e1t t\u1eeb c\u00e1c t\u00e1c d\u1ee5ng ph\u1ee5 m\u00e0 n\u00f3 g\u00e2y ra, \u0111\u1eb7c bi\u1ec7t khi d\u00f9ng k\u00e9o d\u00e0i. Theo m\u1ed9t trong nh\u1eefng th\u1eed nghi\u1ec7m \u0111\u1ea7u ti\u00ean ti\u1ebfn h\u00e0nh b\u1edfi Dyck v\u00e0 c\u1ed9ng s\u1ef1 n\u0103m 1982, hi\u1ec7u qu\u1ea3 c\u1ee7a prednisone \u0111\u00e3 \u0111\u01b0\u1ee3c ch\u1ee9ng minh v\u1edbi li\u1ec1u cao 120mg s\u1eed d\u1ee5ng c\u00e1ch ng\u00e0y trong v\u00f2ng 3 th\u00e1ng [65]. D\u00f9 c\u1ea3i thi\u1ec7n l\u00e2m s\u00e0ng \u0111\u01b0\u1ee3c quan s\u00e1t ngay sau khi b\u1eaft \u0111\u1ea7u \u0111i\u1ec1u tr\u1ecb, c\u1ea7n 3 \u0111\u1ebfn 6 th\u00e1ng \u0111\u1ec3 \u0111\u1ea1t \u0111\u01b0\u1ee3c m\u1ee9c \u0111\u00e1p \u1ee9ng t\u1ed1i \u0111a [66,67]. Khi \u0111i\u1ec1u tr\u1ecb v\u1edbi CCS, k\u1ebf ho\u1ea1ch gi\u1ea3m d\u1ea7n li\u1ec1u l\u00e0 v\u00f4 c\u00f9ng quan tr\u1ecdng. T\u01b0\u01a1ng t\u1ef1 nh\u01b0 IVIg, b\u1eaft \u0111\u1ea7u gi\u1ea3m li\u1ec1u CCS khi c\u00e1c tri\u1ec7u ch\u1ee9ng ng\u1eebng c\u1ea3i thi\u1ec7n v\u00e0 \u0111\u1ea1t \u0111\u01b0\u1ee3c tr\u1ea1ng th\u00e1i b\u00ecnh nguy\u00ean. C\u00f3 th\u1ec3 gi\u1ea3m li\u1ec1u CCS 5 \u0111\u1ebfn 10mg m\u1ed7i 1 \u0111\u1ebfn 4 tu\u1ea7n ho\u1eb7c chuy\u1ec3n sang s\u1eed d\u1ee5ng c\u00e1ch ng\u00e0y.
\nNgo\u00e0i prednisone d\u1ea1ng u\u1ed1ng, CCS c\u00f3 th\u1ec3 \u0111\u01b0\u1ee3c s\u1eed d\u1ee5ng d\u01b0\u1edbi c\u00e1c c\u00f4ng th\u1ee9c kh\u00e1c. C\u00e1c nghi\u00ean c\u1ee9u \u0111\u01b0\u1ee3c ti\u1ebfn h\u00e0nh so s\u00e1nh hi\u1ec7u qu\u1ea3 v\u00e0 t\u00ednh dung n\u1ea1p c\u1ee7a li\u1ec1u n\u1ea1p dexamethasone \u0111\u01b0\u1eddng u\u1ed1ng, 40mg\/ng\u00e0y, 4 ng\u00e0y m\u1ed7i th\u00e1ng v\u1edbi li\u1ec1u prednisolone ti\u00eau chu\u1ea9n. Theo \u0111\u00f3, nh\u00f3m b\u1ec7nh nh\u00e2n s\u1eed d\u1ee5ng dexamethasone cho th\u1ea5y th\u1eddi gian lui b\u1ec7nh trung b\u00ecnh l\u00e0 20 tu\u1ea7n (so v\u1edbi 39 tu\u1ea7n), c\u0169ng nh\u01b0 \u00edt chuy\u1ec3n n\u1eb7ng sau khi d\u1eebng \u0111i\u1ec1u tr\u1ecb, \u00edt m\u1ea5t ng\u1ee7 v\u00e0 h\u1ed9i ch\u1ee9ng cushing h\u01a1n [67]. M\u1ed9t nghi\u00ean c\u1ee9u h\u1ed3i c\u1ee9u \u0111\u01a1n trung t\u00e2m so s\u00e1nh methylprednisolone \u0111\u01b0\u1eddng t\u0129nh m\u1ea1ch (IVMP) li\u1ec1u n\u1ea1p v\u1edbi prednisone v\u00e0 IVIg \u0111\u01b0\u1eddng u\u1ed1ng h\u00e0ng ng\u00e0y [68]. IVMP th\u01b0\u1eddng \u0111\u01b0\u1ee3c d\u00f9ng v\u1edbi li\u1ec1u kh\u1edfi ph\u00e1t 1g\/ng\u00e0y trong 3 \u0111\u1ebfn 5 ng\u00e0y, sau \u0111\u00f3 l\u00e0 1g m\u1ed7i tu\u1ea7n v\u00e0 gi\u1ea3m d\u1ea7n t\u1ea7n s\u1ed1 c\u0169ng nh\u01b0 li\u1ec1u \u0111i\u1ec1u tr\u1ecb. Kh\u00f4ng nh\u1eefng \u0111em l\u1ea1i hi\u1ec7u qu\u1ea3 l\u00e2m s\u00e0ng cao h\u01a1n khi \u0111\u00e1nh gi\u00e1 \u1edf th\u00e1ng th\u1ee9 6, b\u1ec7nh nh\u00e2n \u0111i\u1ec1u tr\u1ecb v\u1edbi IVMP c\u0169ng c\u00f3 \u00edt t\u00e1c d\u1ee5ng ph\u1ee5 t\u0103ng c\u00e2n v\u00e0 h\u1ed9i ch\u1ee9ng cushing h\u01a1n so v\u1edbi nh\u00f3m prednisone \u0111\u01b0\u1eddng u\u1ed1ng h\u00e0ng ng\u00e0y.
\nT\u00e1c d\u1ee5ng gi\u1eefa IVIg v\u00e0 corticosteroid c\u0169ng \u0111\u01b0\u1ee3c \u0111\u01b0a ra so s\u00e1nh. Th\u1eed nghi\u1ec7m IMC trong 6 th\u00e1ng t\u1ea1i ch\u00e2u \u00c2u \u0111\u00e3 s\u1edbm m\u00f4 t\u1ea3 t\u00ednh hi\u1ec7u qu\u1ea3 v\u00e0 dung n\u1ea1p c\u1ee7a IVMP 0,5g\/ng\u00e0y, 4 ng\u00e0y\/th\u00e1ng m\u1ed7i th\u00e1ng so v\u1edbi IVIg 0,5g\/kg\/ng\u00e0y, 4 ng\u00e0y\/th\u00e1ng m\u1ed7i th\u00e1ng. Th\u1eddi gian \u0111\u1ebfn khi lui b\u1ec7nh c\u1ee7a nh\u00f3m IVIg nhanh h\u01a1n, tu\u00e2n th\u1ee7 t\u1ed1t h\u01a1n v\u00e0 \u00edt t\u00e1c d\u1ee5ng ph\u1ee5 h\u01a1n nh\u00f3m IVMP. Tuy nhi\u00ean, th\u1eddi gian lui b\u1ec7nh \u1edf nh\u00f3m IVMP d\u00e0i h\u01a1n (14 th\u00e1ng) so v\u1edbi 4,5 th\u00e1ng c\u1ee7a nh\u00f3m IVIg. Khi \u0111\u00e1nh gi\u00e1 \u1edf th\u00e1ng th\u1ee9 6 v\u00e0 sau \u0111\u00f3, t\u1ef7 l\u1ec7 b\u1ec7nh nh\u00e2n v\u1eabn ti\u1ebfp t\u1ee5c \u0111\u00e1p \u1ee9ng \u0111i\u1ec1u tr\u1ecb \u1edf nh\u00f3m IVMP c\u0169ng cao h\u01a1n. B\u00ean c\u1ea1nh \u0111\u00f3, m\u1ed9t nghi\u00ean c\u1ee9u h\u1ed3i c\u1ee9u so s\u00e1nh l\u00e2m s\u00e0ng gi\u1eefa c\u00e1c b\u1ec7nh nh\u00e2n c\u00f3 th\u1ec3 d\u1eebng \u0111i\u1ec1u tr\u1ecb v\u00e0 nh\u00f3m b\u1ec7nh nh\u00e2n ph\u1ee5 thu\u1ed9c \u0111i\u1ec1u tr\u1ecb cho th\u1ea5y nh\u00f3m ph\u1ee5 thu\u1ed9c \u0111i\u1ec1u tr\u1ecb \u0111\u00e1p \u1ee9ng t\u1ed1t h\u01a1n v\u1edbi IVIg, kh\u00e1ng CCS v\u00e0 th\u01b0\u1eddng c\u00f3 l\u00e2m s\u00e0ng bi\u1ec3u hi\u1ec7n t\u1ed5n th\u01b0\u01a1ng th\u1ea7n kinh \u0111a \u1ed5. Tuy nhi\u00ean, tr\u00ean nh\u1eefng b\u1ec7nh nh\u00e2n c\u00f3 th\u1ec3 d\u1eebng \u0111i\u1ec1u tr\u1ecb, t\u1ef7 l\u1ec7 s\u1eed d\u1ee5ng CCS l\u00e0 th\u01b0\u1eddng xuy\u00ean h\u01a1n [69].
\nQuan s\u00e1t c\u1ea3i thi\u1ec7n th\u1eddi gian lui b\u1ec7nh \u1edf nh\u00f3m IVMP, nhi\u1ec1u t\u00e1c gi\u1ea3 \u0111\u1eb7t c\u00e2u h\u1ecfi v\u1ec1 vai tr\u00f2 c\u1ee7a vi\u1ec7c k\u1ebft h\u1ee3p IVMP v\u00e0 IVIg k\u1ec3 t\u1eeb l\u00fac kh\u1edfi b\u1ec7nh. \u0110\u1ec3 tr\u1ea3 l\u1eddi c\u00e2u h\u1ecfi tr\u00ean, m\u1ed9t th\u1eed nghi\u1ec7m nhi\u1ec1u tri\u1ec3n v\u1ecdng (OPTIC) \u0111ang \u0111\u01b0\u1ee3c ti\u1ebfn h\u00e0nh, theo \u0111\u00f3 c\u00e1c b\u1ec7nh nh\u00e2n \u0111\u01b0\u1ee3c nh\u1eadn ng\u1eabu nhi\u00ean ho\u1eb7c IVIg 1g\/kg m\u1ed7i 3 tu\u1ea7n + 1g IVMP m\u1ed7i 3 tu\u1ea7n ho\u1eb7c IVIg 1g m\u1ed7i 3 tu\u1ea7n + gi\u1ea3 d\u01b0\u1ee3c v\u00e0 quan s\u00e1t trong 18 tu\u1ea7n [70].
\nB\u00ean c\u1ea1nh c\u00e1c t\u00e1c d\u1ee5ng ph\u1ee5 \u0111\u00e3 \u0111\u01b0\u1ee3c \u0111\u1ec1 c\u1eadp, s\u1eed d\u1ee5ng CCS c\u0169ng ti\u1ec1m \u1ea9n nhi\u1ec1u r\u1ee7i ro nh\u01b0 t\u0103ng \u0111\u01b0\u1eddng huy\u1ebft, \u0111\u00e1i th\u00e1o \u0111\u01b0\u1eddng do CCS, nhi\u1ec5m tr\u00f9ng, vi\u00eam \u0111\u01b0\u1eddng ru\u1ed9t, gi\u1ea3m m\u1eadt \u0111\u1ed9 x\u01b0\u01a1ng v\u00e0 nhi\u1ec1u b\u1ec7nh l\u00fd kh\u00e1c, \u0111\u00f2i h\u1ecfi ph\u1ea3i gi\u1ea3i th\u00edch c\u1eb7n k\u1ebd v\u1edbi b\u1ec7nh nh\u00e2n ngay t\u1eeb khi b\u1eaft \u0111\u1ea7u d\u00f9ng thu\u1ed1c. Ph\u1ea1m vi c\u1ee7a b\u00e0i \u0111\u00e1nh gi\u00e1 kh\u00f4ng cho ph\u00e9p ch\u00fang t\u00f4i t\u1eadp trung v\u00e0o nh\u1eefng nguy c\u01a1 n\u00e0y nh\u01b0ng \u0111\u00e3 \u0111\u01b0\u1ee3c t\u00f3m t\u1eaft \u0111\u1ea7y \u0111\u1ee7 trong c\u00e1c h\u01b0\u1edbng d\u1eabn [71].<\/p>\n

6.4. PE \u2013 Trao \u0111\u1ed5i huy\u1ebft t\u01b0\u01a1ng<\/strong><\/em><\/h3>\n

PE l\u00e0 m\u1ed9t ph\u01b0\u01a1ng ph\u00e1p h\u1eefu hi\u1ec7u trong \u0111i\u1ec1u tr\u1ecb c\u00e1c tr\u01b0\u1eddng h\u1ee3p CIDP kh\u00e1ng tr\u1ecb khi kh\u00f4ng \u0111\u00e1p \u1ee9ng v\u1edbi IVIg ho\u1eb7c CCS. H\u01a1n n\u1eefa, PE c\u00f2n l\u00e0 m\u1ed9t l\u1ef1a ch\u1ecdn thay th\u1ebf cho nh\u1eefng b\u1ec7nh nh\u00e2n kh\u00f4ng th\u1ec3 d\u00f9ng IVIg ho\u1eb7c CCSs do nguy c\u01a1 g\u00e2y t\u00e1c d\u1ee5ng ph\u1ee5 ho\u1eb7c c\u00f3 ch\u1ed1ng ch\u1ec9 \u0111\u1ecbnh. PE ho\u1ea1t \u0111\u1ed9ng d\u1ef1a tr\u00ean nguy\u00ean l\u00fd lo\u1ea1i b\u1ecf globulin mi\u1ec5n d\u1ecbch, b\u1ed5 th\u1ec3, kh\u00e1ng th\u1ec3 v\u00e0 cytokine trong h\u1ec7 tu\u1ea7n ho\u00e0n. Theo k\u1ebft qu\u1ea3 t\u1eeb th\u1eed nghi\u1ec7m gi\u1ea3 d\u01b0\u1ee3c c\u00f3 \u0111\u1ed1i ch\u1ee9ng (sham-controlled), m\u00f9 \u0111\u00f4i theo th\u1eddi gian n\u0103m 1986, so v\u1edbi nh\u00f3m ch\u1ee9ng, b\u1ec7nh nh\u00e2n PE c\u1ea3i thi\u1ec7n \u1edf c\u1ea3 \u0111\u00e1nh gi\u00e1 tr\u00ean d\u1eabn truy\u1ec1n th\u1ea7n kinh c\u0169ng nh\u01b0 tr\u00ean \u0111i\u1ec3m NIS t\u1ea1i tu\u1ea7n th\u1ee9 3 [72]. M\u1ed9t th\u1eed nghi\u1ec7m ch\u00e9o, gi\u1ea3 d\u01b0\u1ee3c c\u00f3 \u0111\u1ed1i ch\u1ee9ng theo th\u1eddi gian ti\u1ebfn h\u00e0nh 10 n\u0103m sau m\u1ed9t l\u1ea7n n\u1eefa cho th\u1ea5y l\u1ee3i \u00edch c\u1ee7a PE sau 10 l\u1ea7n trao \u0111\u1ed5i d\u1ef1a tr\u00ean \u0111i\u1ec3m NISs trung b\u00ecnh, s\u1ee9c c\u01a1 khi n\u1eafm tay, m\u1ee9c \u0111\u1ed9 t\u00e0n t\u1eadt tr\u00ean l\u00e2m s\u00e0ng v\u00e0 t\u1ed1c \u0111\u1ed9c d\u1eabn truy\u1ec1n c\u0169ng nh\u01b0 bi\u00ean \u0111\u1ed9 trung b\u00ecnh \u0111\u01a1n v\u1ecb v\u1eadn \u0111\u1ed9ng [45]. M\u1ed9t nghi\u00ean c\u1ee9u ch\u00e9o quan s\u00e1t c\u00f3 \u0111\u1ed1i ch\u1ee9ng theo th\u1eddi gian so s\u00e1nh PE v\u00e0 IVIg cho th\u1ea5y c\u1ea3 hai li\u1ec7u ph\u00e1p mi\u1ec5n d\u1ecbch \u0111\u1ec1u gi\u00fap c\u1ea3i thi\u1ec7n tri\u1ec7u ch\u1ee9ng th\u1ea7n kinh qua thang \u0111i\u1ec3m NIS v\u00e0 \u0111i\u1ec7n th\u1ebf \u0111\u01a1n v\u1ecb v\u1eadn \u0111\u1ed9ng. C\u00e1c t\u00e1c gi\u1ea3 k\u1ebft lu\u1eadn r\u1eb1ng, c\u1ea3 hai ph\u01b0\u01a1ng ph\u00e1p c\u00f3 hi\u1ec7u qu\u1ea3 t\u01b0\u01a1ng \u0111\u01b0\u01a1ng nh\u01b0ng IVIg \u0111\u01b0\u1ee3c \u01b0a chu\u1ed9ng h\u01a1n do d\u1ec5 ti\u1ebfp c\u1eadn.
\nPE th\u01b0\u1eddng \u0111\u01b0\u1ee3c s\u1eed d\u1ee5ng \u1edf nh\u1eefng b\u1ec7nh nh\u00e2n li\u1ec7t m\u1ee9c \u0111\u1ed9 n\u1eb7ng, nh\u1eefng b\u1ec7nh nh\u00e2n kh\u00e1ng tr\u1ecb v\u1edbi CCS\/IVIg ho\u1eb7c l\u00e0 m\u1ed9t bi\u1ec7n ph\u00e1p h\u1ed7 tr\u1ee3 khi CIDP chuy\u1ec3n bi\u1ebfn n\u1eb7ng. C\u00e1c d\u1eef li\u1ec7u v\u1ec1 t\u00ednh hi\u1ec7u qu\u1ea3 c\u1ee7a PE khi l\u1ef1a ch\u1ecdn \u0111i\u1ec1u tr\u1ecb d\u00e0i h\u1ea1n v\u1eabn c\u00f2n h\u1ea1n ch\u1ebf. \u0110\u00e1ng ch\u00fa \u00fd, trong v\u00f2ng v\u00e0i tu\u1ea7n ho\u1eb7c v\u00e0i th\u00e1ng sau khi ho\u00e0n t\u1ea5t \u0111i\u1ec1u tr\u1ecb, l\u00ean t\u1edbi 50 t\u1edbi 67% b\u1ec7nh nh\u00e2n c\u00f3 bi\u1ec3u hi\u1ec7n b\u1ec7nh tr\u1edf l\u1ea1i. Tuy nhi\u00ean, c\u1ea3 ba ph\u01b0\u01a1ng ph\u00e1p \u0111i\u1ec1u tr\u1ecb CIDP (IVIg, CCS, PE) \u0111\u1ec1u c\u00f3 ph\u00e1c \u0111\u1ed3 s\u1eed d\u1ee5ng k\u00e9o d\u00e0i do nguy c\u01a1 b\u1ec7nh tr\u1edf n\u1eb7ng n\u1ebfu \u0111i\u1ec1u tr\u1ecb m\u1ed9t th\u1eddi gian ng\u1eafn r\u1ed3i d\u1eebng \u0111\u1ed9t ng\u1ed9t. D\u00f9 v\u1eady, PE \u0111\u00f4i khi c\u00f3 th\u1ec3 s\u1eed d\u1ee5ng ng\u1eaft qu\u00e3ng \u0111\u01a1n \u0111\u1ed9c ho\u1eb7c nh\u01b0 m\u1ed9t li\u1ec7u ph\u00e1p ph\u1ed1i h\u1ee3p v\u1edbi CCS tr\u00ean nh\u1eefng b\u1ec7nh nh\u00e2n ngo\u1ea1i tr\u00fa.
\nC\u00e1c t\u00e1c d\u1ee5ng ph\u1ee5 \u0111\u00e1ng ch\u00fa \u00fd c\u1ee7a PE bao g\u1ed3m qu\u00e1 t\u1ea3i d\u1ecbch, \u0111\u1eb7c bi\u1ec7t \u1edf nh\u1eefng b\u1ec7nh nh\u00e2n suy tim sung huy\u1ebft, b\u1ec7nh gan, b\u1ec7nh th\u1eadn c\u0169ng nh\u01b0 l\u00e0m t\u0103ng nguy c\u01a1 nhi\u1ec5m khu\u1ea9n. C\u00e1c thu\u1ed1c \u1ee9c ch\u1ebf ACE n\u00ean \u0111\u01b0\u1ee3c d\u1eebng 24h tr\u01b0\u1edbc khi \u0111i\u1ec1u tr\u1ecb [74].<\/p>\n

7. Th\u00e2\u0301t ba\u0323i trong \u0111i\u00ea\u0300u tri\u0323 va\u0300 ca\u0301c th\u01b0\u0309 nghi\u00ea\u0323m \u0111ang th\u01b0\u0323c hi\u00ea\u0323n<\/strong><\/h2>\n

M\u1ed9t v\u00e0i b\u1ec7nh nh\u00e2n s\u1ebd kh\u00f4ng \u0111\u00e1p \u1ee9ng v\u1edbi b\u1ea5t c\u1ee9 ph\u01b0\u01a1ng ph\u00e1p n\u00e0o cho d\u00f9 IVIg, SCIg, CCS v\u00e0 PE \u0111\u1ec1u mang l\u1ea1i hi\u1ec7u qu\u1ea3 trong \u0111i\u1ec1u tr\u1ecb CIDP. Khi l\u00e2m s\u00e0ng kh\u00f4ng c\u1ea3i thi\u1ec7n, b\u01b0\u1edbc \u0111\u1ea7u ti\u00ean c\u1ea7n l\u00e0m l\u00e0 xem l\u1ea1i ch\u1ea9n \u0111o\u00e1n c\u1ee7a b\u1ec7nh nh\u00e2n. D\u1ef1a v\u00e0o kinh nghi\u1ec7m c\u1ee7a ch\u00fang t\u00f4i, tr\u00ean b\u1ec7nh \u0111a r\u1ec5 v\u00e0 d\u00e2y th\u1ea7n kinh m\u1ea5t myelin ti\u1ebfn tri\u1ec3n nhanh kh\u00f4ng \u0111\u00e1p \u1ee9ng v\u1edbi c\u00e1c li\u1ec7u ph\u00e1p mi\u1ec5n d\u1ecbch, c\u00e1c b\u1ec7nh l\u00fd gi\u1ed1ng CIDP n\u00ean \u0111\u01b0\u1ee3c c\u00e2n nh\u1eafc thay th\u1ebf m\u00e0 th\u01b0\u1eddng g\u1eb7p nh\u1ea5t l\u00e0 h\u1ed9i ch\u1ee9ng POEMS. Khi \u0111\u00f3, n\u1ed3ng \u0111\u1ed9 VEGF v\u00e0 ph\u01b0\u01a1ng ph\u00e1p \u0111i\u1ec7n di c\u1ed1 \u0111\u1ecbnh mi\u1ec5n d\u1ecbch (immunofixation electroporesis) c\u1ea7n \u0111\u01b0\u1ee3c th\u1ef1c hi\u1ec7n. C\u00e1c nguy\u00ean nh\u00e2n kh\u00e1c cho vi\u1ec7c k\u00e9m \u0111\u00e1p \u1ee9ng v\u1edbi \u0111i\u1ec1u tr\u1ecb c\u00f3 th\u1ec3 k\u1ec3 \u0111\u1ebfn bao g\u1ed3m bi\u1ebfn th\u1ec3 IgM DADS c\u1ee7a CIDP ho\u1eb7c nh\u00f3m b\u1ec7nh kh\u00e1ng th\u1ec3 kh\u00e1ng n\u1ed1t v\u00e0 c\u1eadn n\u1ed1t (neurofascin-155 v\u00e0 contactin-1). C\u00f3 th\u1ec3 k\u1ebft h\u1ee3p PE v\u1edbi CCS ho\u1eb7c IVIg v\u1edbi CCS n\u1ebfu b\u1ec7nh nh\u00e2n kh\u00e1ng tr\u1ecb thu\u1ed9c th\u1ec3 CIDP \u0111i\u1ec3n h\u00ecnh. Nh\u00f3m b\u1ec7nh nh\u00e2n c\u00f3 kh\u00e1ng th\u1ec3 kh\u00e1ng neurofascin-155 kh\u00f4ng \u0111\u00e1p \u1ee9ng v\u1edbi IVIg nh\u01b0ng t\u01b0\u01a1ng \u0111\u1ed1i nh\u1ea1y v\u1edbi rituximab v\u00e0 c\u00f3 th\u1ec3 l\u00e0 PE [75], trong khi nh\u00f3m c\u00f3 kh\u00e1ng th\u1ec3 kh\u00e1ng contactin-1 \u0111\u00e1p \u1ee9ng v\u1edbi CCS t\u1ed1t h\u01a1n so v\u1edbi IVIg [76]. Rituximab xu\u1ea5t hi\u1ec7n nh\u01b0 m\u1ed9t li\u1ec7u ph\u00e1p \u0111\u1ea7y h\u1ee9a h\u1eb9n cho nh\u00f3m b\u1ec7nh CIDP kh\u00e1ng tr\u1ecb ho\u1eb7c kh\u00f4ng x\u00e1c \u0111\u1ecbnh \u0111\u01b0\u1ee3c kh\u00e1ng th\u1ec3 m\u1eb7c d\u00f9 kh\u00f4ng ph\u1ea3i t\u1ea5t c\u1ea3 b\u1ec7nh nh\u00e2n \u0111\u1ec1u c\u00f3 l\u00e2m s\u00e0ng c\u1ea3i thi\u1ec7n sau khi s\u1eed d\u1ee5ng [75]. Tr\u00ean 28 b\u1ec7nh nh\u00e2n kh\u00f4ng x\u00e1c \u0111\u1ecbnh \u0111\u01b0\u1ee3c kh\u00e1ng th\u1ec3, Roux v\u00e0 c\u1ed9ng s\u1ef1 quan s\u00e1t th\u1ea5y 75% \u0111\u00e1p \u1ee9ng v\u1edbi rituximab d\u00f9 cho \u0111\u1ed3ng m\u1eafc v\u1edbi r\u1ed1i lo\u1ea1n huy\u1ebft h\u1ecdc ho\u1eb7c b\u1ec7nh t\u1ef1 mi\u1ec5n [77]. Th\u1eddi gian trung b\u00ecnh cho \u0111\u1ebfn khi c\u1ea3i thi\u1ec7n l\u00e0 6 th\u00e1ng v\u00e0 trong 2 n\u0103m theo d\u00f5i, ch\u1ec9 duy nh\u1ea5t 2 b\u1ec7nh nh\u00e2n y\u00eau c\u1ea7u t\u00e1i \u0111i\u1ec1u tr\u1ecb. Quan s\u00e1t c\u00e1c b\u1ec7nh nh\u00e2n CIDP kh\u00e1ng tr\u1ecb d\u01b0\u01a1ng t\u00ednh v\u1edbi contactin-1 v\u00e0 neurofascin-155 \u0111\u01b0\u1ee3c \u0111i\u1ec1u tr\u1ecb b\u1eb1ng rituximab, l\u00e2m s\u00e0ng v\u00e0 n\u1ed3ng \u0111\u1ed9 kh\u00e1ng th\u1ec3 \u0111\u1ec1u c\u00f3 c\u1ea3i thi\u1ec7n.
\nM\u1ed9t th\u1eed nghi\u1ec7m ng\u1eabu nhi\u00ean c\u00f3 \u0111\u1ed1i ch\u1ee9ng t\u1ea1i Nh\u1eadt B\u1ea3n \u0111ang ti\u1ebfn h\u00e0nh \u0111\u00e1nh gi\u00e1 hi\u1ec7u qu\u1ea3 v\u00e0 t\u00ednh an to\u00e0n c\u1ee7a rituximab tr\u00ean c\u00e1c b\u1ec7nh nh\u00e2n CIDP c\u00f3 ho\u1eb7c kh\u00f4ng c\u00f3 kh\u00e1ng th\u1ec3 kh\u00e1ng c\u1eadn n\u1ed1t. Ngo\u00e0i ra, li\u1ec1u cao cyclophosphamide c\u0169ng cho th\u1ea5y c\u1ea3i thi\u1ec7n s\u1ee9c c\u01a1, ch\u1ee9c n\u0103ng s\u1ed1ng v\u00e0 c\u00e1c ch\u1ec9 s\u1ed1 tr\u00ean \u0111i\u1ec7n c\u01a1 v\u1edbi b\u1ec7nh nh\u00e2n CIDP kh\u00e1ng tr\u1ecb, tuy nhi\u00ean c\u1ea7n gi\u1ea3i th\u00edch \u0111\u1ea7y \u0111\u1ee7 c\u00e1c t\u00e1c d\u1ee5ng ph\u1ee5 c\u00f3 th\u1ec3 x\u1ea3y ra v\u1edbi b\u1ec7nh nh\u00e2n [78-80].
\nD\u1eef li\u1ec7u \u0111\u00e1nh gi\u00e1 vai tr\u00f2 c\u1ee7a c\u00e1c thu\u1ed1c \u1ee9c ch\u1ebf mi\u1ec5n d\u1ecbch kh\u00e1c hi\u1ec7n c\u00f2n nhi\u1ec1u h\u1ea1n ch\u1ebf v\u00ec ch\u1ec9 gi\u1edbi h\u1ea1n \u1edf c\u00e1c chu\u1ed7i ca b\u1ec7nh, c\u00e1c th\u1eed nghi\u1ec7m ho\u1eb7c c\u00e1c b\u1eb1ng ch\u1ee9ng c\u00f2n tranh c\u00e3i [81]. V\u00ed d\u1ee5, azathioprine k\u1ebft h\u1ee3p c\u00f9ng prednisone kh\u00f4ng cho th\u1ea5y t\u00ednh \u01b0u vi\u1ec7t so v\u1edbi prednisone \u0111\u01a1n tr\u1ecb li\u1ec7u trong m\u1ed9t nghi\u00ean c\u1ee9u b\u1ecb gi\u1edbi h\u1ea1n trong th\u1eddi gian ng\u1eafn [82]. Hai th\u1eed nghi\u1ec7m ng\u1eabu nhi\u00ean, gi\u1ea3 d\u01b0\u1ee3c \u2013 c\u00f3 ki\u1ec3m so\u00e1t cho th\u1ea5y fingolimod [83] v\u00e0 methotrexate li\u1ec1u th\u1ea5p [84] kh\u00f4ng hi\u1ec7u qu\u1ea3 tr\u00ean b\u1ec7nh nh\u00e2n CIDP. C\u00e1c th\u1eed nghi\u1ec7m l\u00e2m s\u00e0ng \u0111ang di\u1ec5n ra \u0111ang \u0111\u00e1nh gi\u00e1 vai tr\u00f2 c\u1ee7a c\u00e1c li\u1ec7u ph\u00e1p \u0111\u00edch t\u1edbi th\u1ee5 th\u1ec3 Fc b\u00e0o thai (FcRn) (NCT04051944 v\u00e0 NCT04281472).<\/p>\n

8. \u0110a\u0301nh gia\u0301 ti\u00ean l\u01b0\u01a1\u0323ng Vi\u00eam \u0111a r\u1ec5 v\u00e0 d\u00e2y th\u1ea7n kinh m\u1ea5t myelin m\u1ea1n t\u00ednh<\/strong><\/h2>\n

CIDP l\u00e0 m\u1ed9t b\u1ec7nh l\u00fd kh\u00f4ng \u0111\u1ed3ng nh\u1ea5t v\u1edbi kh\u00e1c bi\u1ec7t r\u00f5 r\u1ec7t trong \u0111\u00e1p \u1ee9ng \u0111i\u1ec1u tr\u1ecb [29]. C\u00e1c t\u00e0n t\u1eadt k\u00e9o d\u00e0i th\u01b0\u1eddng \u00edt g\u1eb7p trong khi ti\u00ean l\u01b0\u1ee3ng x\u1ea5u s\u1ebd x\u1ea3y ra khi tr\u00ec ho\u00e3n \u0111i\u1ec1u tr\u1ecb [85]. Ti\u1ebfn tri\u1ec3n t\u1ef1 nhi\u00ean c\u1ee7a CIDP \u0111ang \u0111\u01b0\u1ee3c \u0111\u1ecbnh ngh\u0129a l\u1ea1i d\u1ef1a tr\u00ean m\u1ed9t nghi\u00ean c\u1ee9u \u0111a trung t\u00e2m [86]. Do hi\u1ec7n kh\u00f4ng c\u00f3 ch\u1ea5t ch\u1ec9 \u0111i\u1ec3m sinh h\u1ecdc n\u00e0o \u0111\u1eb7c tr\u01b0ng cho CIDP, c\u00e1c th\u00f4ng s\u1ed1 v\u1ec1 m\u1ee9c \u0111\u1ed9 n\u1eb7ng c\u1ee7a t\u1ed5n th\u01b0\u01a1ng th\u1ea7n kinh ngo\u1ea1i bi\u00ean l\u00e0 r\u1ea5t c\u1ea7n thi\u1ebft kh\u00f4ng ch\u1ec9 gi\u00fap ch\u1ea9n \u0111o\u00e1n m\u00e0 c\u00f2n \u0111\u1ec3 theo d\u00f5i \u0111\u00e1p \u1ee9ng \u0111i\u1ec1u tr\u1ecb.
\nNIS (tr\u01b0\u1edbc \u0111\u00e2y \u0111\u01b0\u1ee3c g\u1ecdi l\u00e0 Thang \u0111i\u1ec3m t\u00e0n ph\u1ebf do th\u1ea7n kinh) l\u00e0 thang \u0111i\u1ec3m \u0111\u1ea7u ti\u00ean \u0111\u01b0\u1ee3c s\u1eed d\u1ee5ng \u0111\u1ec3 \u0111\u00e1nh gi\u00e1 hi\u1ec7u qu\u1ea3 c\u1ee7a IVIg v\u00e0 PE trong \u0111i\u1ec1u tr\u1ecb CIDP [45, 72, 87]. Thang \u0111i\u1ec3m NIS bao g\u1ed3m c\u00e1c \u0111i\u1ec3m th\u00e0nh ph\u1ea7n li\u00ean quan \u0111\u1ebfn v\u1eadn \u0111\u1ed9ng, c\u1ea3m gi\u00e1c v\u00e0 ph\u1ea3n x\u1ea1 co c\u01a1 v\u1edbi m\u1ee5c \u0111\u00edch cung c\u1ea5p m\u1ed9t c\u00f4ng c\u1ee5 \u0111\u1ecbnh l\u01b0\u1ee3ng ti\u00eau chu\u1ea9n trong th\u0103m kh\u00e1m th\u1ea7n kinh ngo\u1ea1i bi\u00ean, cho ph\u00e9p c\u00f3 c\u00e1i nh\u00ecn kh\u00e1ch quan trong \u0111\u00e1nh gi\u00e1 \u0111\u00e1p \u1ee9ng \u0111i\u1ec1u tr\u1ecb. Trong m\u1ed9t nghi\u00ean c\u1ee9u b\u00e1n m\u00f9 theo th\u1eddi gian v\u00e0 \u0111\u00e1nh gi\u00e1 theo ti\u00eau chu\u1ea9n tr\u00ean b\u1ec7nh nh\u00e2n CIDP, thang \u0111i\u1ec3m NIS c\u0169ng nh\u01b0 NIS \u0111\u00e1nh gi\u00e1 \u0111\u1ed9 y\u1ebfu c\u01a1 \u0111\u01b0\u1ee3c s\u1eed d\u1ee5ng \u0111\u1ec3 ghi nh\u1eadn b\u1ea5t th\u01b0\u1eddng th\u1ea7n kinh ngo\u1ea1i bi\u00ean [88]. Ngo\u00e0i ra, \u0111i\u1ec3m CMAP t\u1ed5ng h\u1ee3p (t\u1ed5ng CMAP c\u1ee7a c\u00e1c d\u00e2y th\u1ea7n kinh v\u1eadn \u0111\u1ed9ng tr\u1ee5, ch\u00e0y v\u00e0 m\u00e1c) v\u1edbi b\u1ea5t th\u01b0\u1eddng th\u1ea7n kinh ngo\u1ea1i bi\u00ean c\u0169ng cho th\u1ea5y m\u1ed1i t\u01b0\u01a1ng quan v\u1edbi NIS. \u0110i\u1ec3m NIS \u0111\u01b0\u1ee3c ho\u00e0n thi\u1ec7n d\u1ea7n theo th\u1eddi gian \u0111\u1ec3 c\u00f3 th\u1ec3 \u00e1p d\u1ee5ng cho c\u00e1c d\u1ea1ng kh\u00e1c nhau c\u1ee7a b\u1ec7nh l\u00fd th\u1ea7n kinh ngo\u1ea1i bi\u00ean, bao g\u1ed3m b\u1ec7nh \u0111a d\u00e2y th\u1ea7n kinh do \u0111\u00e1i th\u00e1o \u0111\u01b0\u1eddng, CIDP v\u00e0 g\u1ea7n \u0111\u00e2y l\u00e0 b\u1ec7nh th\u1ea7n kinh ngo\u1ea1i bi\u00ean do l\u1eafng \u0111\u1ecdng tinh b\u1ed9t TTR [89].
\nTrong th\u1eadp k\u1ef7 qua, c\u00e1c th\u1eed nghi\u1ec7m l\u00e2m s\u00e0ng t\u1eadp trung v\u00e0o \u0111\u00e1nh gi\u00e1 ti\u00ean l\u01b0\u1ee3ng s\u1eed d\u1ee5ng \u0111a m\u00f4 th\u1ee9c. C\u00f4ng c\u1ee5 \u0111\u00e1nh gi\u00e1 ti\u00ean l\u01b0\u1ee3ng k\u1ebft h\u1ee3p bao g\u1ed3m: (a) t\u00e0n ph\u1ebf (I-RODS) v\u00e0 INCAT; (b) s\u1ee9c c\u01a1 (ki\u1ec3m tra s\u1ee9c c\u01a1 khi n\u1eafm, ki\u1ec3m tra s\u1ee9c c\u01a1 khi th\u1ef1c hi\u1ec7n \u0111\u1ed9ng t\u00e1c, thang \u0111i\u1ec3m t\u1ed5ng h\u1ee3p MRC); (c) \u0111\u00e1nh gi\u00e1 d\u00e1ng \u0111i (\u0111\u00e1nh gi\u00e1 th\u1eddi gian \u0111\u1ee9ng v\u00e0 \u0111i); v\u00e0 (d) \u0111\u00e1nh gi\u00e1 ch\u1ea5t l\u01b0\u1ee3ng cu\u1ed9c s\u1ed1ng (EuroQoL, PGIC) [90,91]. M\u1eb7c d\u00f9 h\u01b0\u1edbng ti\u1ebfp c\u1eadn n\u00e0y \u0111\u01b0\u1ee3c \u00e1p d\u1ee5ng trong nghi\u00ean c\u1ee9u th\u1eed nghi\u1ec7m, n\u00f3 c\u0169ng quan tr\u1ecdng trong l\u00e2m s\u00e0ng.
\nTrong c\u00e1c ph\u01b0\u01a1ng ph\u00e1p \u0111\u00e1nh gi\u00e1 tr\u00ean, ba ph\u01b0\u01a1ng ph\u00e1p y\u00eau c\u1ea7u s\u1ef1 ch\u00fa \u00fd \u0111\u1eb7c bi\u1ec7t bao g\u1ed3m \u0111o s\u1ee9c c\u01a1 khi n\u1eafm, I-RODS v\u00e0 INCAT. S\u1ee9c c\u01a1 khi n\u1eafm \u0111\u01b0\u1ee3c th\u1ef1c hi\u1ec7n b\u1eb1ng c\u00e1ch s\u1eed d\u1ee5ng ho\u1eb7c thi\u1ebft b\u1ecb Jamar ho\u1eb7c Vigorometer, c\u00f3 gi\u00e1 tr\u1ecb t\u1ed1t v\u00e0 nhanh ch\u00f3ng trong \u0111\u00e1nh gi\u00e1 t\u1ed5n th\u01b0\u01a1ng [90], gi\u00fap cung c\u1ea5p b\u1eb1ng ch\u1ee9ng kh\u00e1c quan t\u1ed5n th\u01b0\u01a1ng th\u1ea7n kinh v\u00e0 c\u00f3 m\u1ed1i t\u01b0\u01a1ng quan v\u1edbi thang \u0111i\u1ec3m I-RODS [92]. Trong m\u1ed9t nghi\u00ean c\u1ee9u t\u1ea1i H\u00e0 Lan tr\u00ean 14 b\u1ec7nh nh\u00e2n CIDP, s\u1ee9c n\u1eafm c\u01a1 c\u00f3 li\u00ean quan \u0111\u1ebfn n\u1ed3ng \u0111\u1ed9 IgG 1 tu\u1ea7n sau khi truy\u1ec1n IVIg, qua \u0111\u00f3 cho ph\u00e9p c\u00e1 th\u1ec3 h\u00f3a li\u1ec1u IVIg v\u00e0 c\u0169ng cho th\u1ea5y t\u00ednh \u0111a d\u1ea1ng trong d\u01b0\u1ee3c \u0111\u1ed9ng h\u1ecdc c\u1ee7a IVIg gi\u1eefa c\u00e1c b\u1ec7nh nh\u00e2n [93]. I-RODS l\u00e0 thang \u0111i\u1ec3m \u0111\u00e1nh gi\u00e1 ti\u00ean l\u01b0\u1ee3ng \u0111\u1eb7c hi\u1ec7u v\u1edbi CIDP v\u00e0 \u0111\u01b0\u1ee3c s\u1eed d\u1ee5ng r\u1ed9ng r\u00e3i [94]. B\u00ean c\u1ea1nh \u0111\u00f3, INCAT c\u0169ng l\u00e0 m\u1ed9t thang \u0111i\u1ec3m \u0111\u01b0\u1ee3c s\u1eed d\u1ee5ng nhi\u1ec1u trong ti\u00ean l\u01b0\u1ee3ng b\u1ec7nh nh\u00e2n CIDP m\u1eb7c d\u00f9 c\u00f2n t\u1ed3n t\u1ea1i nh\u1eefng h\u1ea1n ch\u1ebf, v\u00ed d\u1ee5 nh\u01b0 kh\u00f4ng nh\u1ea1y v\u1edbi c\u00e1c thay \u0111\u1ed5i nh\u1ecf, kh\u00f4ng c\u00f3 kh\u1ea3 n\u0103ng ghi l\u1ea1i c\u00e1c h\u1ea1n ch\u1ebf v\u1eadn \u0111\u1ed9ng do y\u1ebfu g\u1ed1c chi [95]. S\u1ef1 thay \u0111\u1ed5i tri\u1ec7u ch\u1ee9ng t\u1ed1i thi\u1ec3u (MCID) \u0111\u01b0\u1ee3c s\u1eed d\u1ee5ng \u0111\u1ec3 \u0111\u00e1nh gi\u00e1 hi\u1ec7u qu\u1ea3 trong th\u1eed nghi\u1ec7m ICE c\u0169ng v\u00f4 c\u00f9ng quan trong trong ti\u00ean l\u01b0\u1ee3ng b\u1ec7nh nh\u00e2n CIDP [96]. Theo \u0111\u00f3, MCID \u0111\u01b0\u1ee3c \u0111\u1ecbnh ngh\u0129a l\u00e0 \u201ckh\u00e1c bi\u1ec7t nh\u1ecf nh\u1ea5t v\u1ec1 m\u1eb7t \u0111i\u1ec3m s\u1ed1 \u1edf c\u00e1c kh\u00eda c\u1ea1nh m\u00e0 b\u1ec7nh nh\u00e2n cho r\u1eb1ng \u0111em l\u1ea1i hi\u1ec7u qu\u1ea3\u201d [97].<\/p>\n

9. Chu\u0301ng t\u00f4i \u0111i\u00ea\u0300u tri\u0323 Vi\u00eam \u0111a r\u1ec5 v\u00e0 d\u00e2y th\u1ea7n kinh m\u1ea5t myelin m\u1ea1n t\u00ednh nh\u01b0 th\u00ea\u0301 na\u0300o\u00a0<\/strong><\/h2>\n

M\u1eb7c d\u00f9 thi\u1ebfu s\u1ef1 \u0111\u1ed3ng thu\u1eadn v\u1ec1 \u0111i\u1ec1u tr\u1ecb d\u00e0i h\u1ea1n, ch\u00fang t\u00f4i v\u1eabn s\u1ebd chia s\u1ebb c\u00e1ch m\u00ecnh ti\u1ebfp c\u1eadn v\u1edbi m\u1ed9t b\u1ec7nh nh\u00e2n CIDP d\u1ef1a tr\u00ean t\u00ednh hi\u1ec7u qu\u1ea3 trong th\u1ef1c h\u00e0nh l\u00e2m s\u00e0ng qua nhi\u1ec1u n\u0103m. C\u00e1ch ti\u1ebfp c\u1eadn n\u00e0y kh\u00f4ng mang t\u00ednh m\u00f4 ph\u1ea1m v\u00e0 nh\u1eefng ph\u01b0\u01a1ng ph\u00e1p ti\u1ebfp c\u1eadn kh\u00e1c c\u0169ng n\u00ean \u0111\u01b0\u1ee3c nh\u00ecn nh\u1eadn m\u1ed9t c\u00e1ch ngang b\u1eb1ng.
\nTrong tr\u01b0\u1eddng h\u1ee3p kh\u00f4ng c\u00f3 ch\u1ed1ng ch\u1ec9 \u0111\u1ecbnh, thu\u1ed1c \u0111i\u1ec1u tr\u1ecb \u0111\u1ea7u tay c\u1ee7a ch\u00fang t\u00f4i l\u00e0 IVIg. Nh\u01b0 \u0111\u00e3 n\u00f3i, \u0111i\u1ec1u tr\u1ecb mang t\u00ednh c\u00e1 th\u1ec3 h\u00f3a ch\u1ee9 kh\u00f4ng c\u00f3 li\u1ec1u m\u1eb7c \u0111\u1ecbnh v\u00e0 t\u1ea7n s\u1ed1 s\u1eed d\u1ee5ng c\u1ed1 \u0111\u1ecbnh cho t\u1ea5t c\u1ea3 b\u1ec7nh nh\u00e2n CIDP. Nh\u1eefng b\u1ec7nh nh\u00e2n c\u00f3 l\u00e2m s\u00e0ng n\u1eb7ng n\u1ec1 v\u00e0 ti\u1ebfn tri\u1ec3n nhanh lu\u00f4n c\u1ea7n li\u1ec1u IVIg cao h\u01a1n so v\u1edbi th\u1ec3 nh\u1eb9 v\u00e0 ti\u1ebfn tri\u1ec3n ch\u1eadm. Nhi\u1ec1u chuy\u00ean gia \u1ee7ng h\u1ed9 t\u1ea7n s\u1ed1 3-4 tu\u1ea7n\/li\u1ec1u nh\u01b0ng t\u00f9y v\u00e0o t\u00ecnh tr\u1ea1ng b\u1ec7nh c\u00f3 th\u1ec3 d\u00f9ng mau ho\u1eb7c th\u01b0a h\u01a1n. Li\u1ec7u ph\u00e1p mi\u1ec5n d\u1ecbch d\u1ef1a v\u00e0o \u0111\u00e1p \u1ee9ng l\u00e2m s\u00e0ng n\u00ean \u0111\u01b0\u1ee3c s\u1eed d\u1ee5ng \u0111\u1ec3 \u0111i\u1ec1u ch\u1ec9nh li\u1ec1u sao cho ph\u00f9 h\u1ee3p v\u1edbi nhu c\u1ea7u c\u1ee7a m\u1ed7i b\u1ec7nh nh\u00e2n [88]. C\u00e1c chuy\u00ean gia c\u00f3 kinh nghi\u1ec7m v\u1ec1 \u0111i\u1ec1u tr\u1ecb b\u1ec7nh th\u1ea7n kinh c\u01a1 n\u00ean \u0111\u00e1nh gi\u00e1 b\u1ec7nh nh\u00e2n \u0111\u1ecbnh k\u1ef3 d\u1ef1a v\u00e0o c\u00e1c thang \u0111i\u1ec3m ti\u00ean l\u01b0\u1ee3ng c\u00f3 gi\u00e1 tr\u1ecb v\u1edbi m\u1ee5c \u0111\u00edch c\u00e1 th\u1ec3 h\u00f3a li\u1ec1u, \u0111\u1ea3m b\u1ea3o ph\u00f9 h\u1ee3p v\u00e0 k\u1ebft th\u00fac \u0111i\u1ec1u tr\u1ecb \u0111\u00fang th\u1eddi \u0111i\u1ec3m khi theo d\u00f5i d\u00e0i h\u1ea1n.
\nD\u1ef1a v\u00e0o c\u00e1c d\u1eef li\u1ec7u trong \u0111i\u1ec1u tr\u1ecb CIDP, t\u1eeb c\u00e1c nghi\u00ean c\u1ee9u ban \u0111\u1ea7u v\u1edbi IVIg cho \u0111\u1ebfn li\u1ec7u ph\u00e1p mi\u1ec5n d\u1ecbch v\u00e0 SCIg g\u1ea7n \u0111\u00e2y [45, 59, 62, 88], trong nh\u1eefng tr\u01b0\u1eddng h\u1ee3p n\u1eb7ng, ch\u00fang t\u00f4i th\u01b0\u1eddng \u0111i\u1ec1u tr\u1ecb v\u1edbi li\u1ec1u n\u1ea1p 2g\/kg trong 5 ng\u00e0y, sau \u0111\u00f3 d\u00f9ng 0,4g\/kg m\u1ed7i tu\u1ea7n. V\u1edbi nh\u1eefng ca b\u1ec7nh nh\u1eb9 h\u01a1n, c\u00f3 th\u1ec3 kh\u00f4ng c\u1ea7n d\u00f9ng li\u1ec1u n\u1ea1p m\u00e0 thay b\u1eb1ng li\u1ec1u 0,4g\/kg m\u1ed7i tu\u1ea7n trong 4 tu\u1ea7n, sau \u0111\u00f3 l\u00e0 m\u1ed7i 2 tu\u1ea7n ho\u1eb7c li\u1ec1u 0,4g\/kg 2 tu\u1ea7n m\u1ed7i l\u1ea7n c\u00f3 th\u1ec3 c\u00e2n nh\u1eafc khi l\u00e2m s\u00e0ng n\u1eb7ng h\u01a1n. Ch\u00fang t\u00f4i s\u1ebd duy tr\u00ec c\u00f9ng li\u1ec1u cho \u0111\u1ebfn khi l\u00e2m s\u00e0ng d\u1eebng c\u1ea3i thi\u1ec7n ho\u1eb7c b\u1ec7nh nh\u00e2n \u0111\u1ea1t \u0111\u01b0\u1ee3c tr\u1ea1ng th\u00e1i b\u00ecnh nguy\u00ean. Thay v\u00ec hi v\u1ecdng t\u00ecnh tr\u1ea1ng c\u1ee7a b\u1ec7nh nh\u00e2n tr\u1edf v\u1ec1 b\u00ecnh th\u01b0\u1eddng, m\u1ee5c \u0111\u00edch c\u1ee7a \u0111i\u1ec1u tr\u1ecb l\u00e0 gi\u00fap b\u1ec7nh nh\u00e2n c\u1ea3i thi\u1ec7n \u0111\u01b0\u1ee3c s\u1ee9c c\u01a1 v\u00e0 ch\u1ee9c n\u0103ng s\u1ed1ng m\u00e0 \u0111i\u1ec1u n\u00e0y c\u00f3 th\u1ec3 ph\u00e1t hi\u1ec7n \u0111\u01b0\u1ee3c qua th\u0103m kh\u00e1m l\u00e2m s\u00e0ng. C\u00e1c b\u1ec7nh nh\u00e2n \u0111\u01b0\u1ee3c theo d\u00f5i theo chu k\u1ef3 3 th\u00e1ng tr\u1eeb tr\u01b0\u1eddng h\u1ee3p ti\u1ebfn tri\u1ec3n nhanh s\u1ebd \u0111\u01b0\u1ee3c t\u00e1i kh\u00e1m th\u01b0\u1eddng xuy\u00ean h\u01a1n. Khi \u0111\u1ea1t \u0111\u01b0\u1ee3c tr\u1ea1ng th\u00e1i b\u00ecnh nguy\u00ean, c\u1ea7n b\u1eaft \u0111\u1ea7u l\u00ean k\u1ebf ho\u1ea1ch gi\u1ea3m li\u1ec1u t\u1eeb t\u1eeb, b\u1eaft \u0111\u1ea7u t\u1eeb vi\u1ec7c t\u0103ng kho\u1ea3ng th\u1eddi gian gi\u1eefa c\u00e1c li\u1ec1u IVIg, th\u01b0\u1eddng l\u00e0 m\u1ed7i 3 th\u00e1ng. Kh\u00f4ng c\u00f3 h\u01b0\u1edbng d\u1eabn r\u00f5 r\u00e0ng v\u1ec1 t\u1ed1c \u0111\u1ed9 gi\u1ea3m nh\u01b0ng v\u1edbi \u0111a s\u1ed1 b\u1ec7nh nh\u00e2n, ch\u00fang t\u00f4i th\u01b0\u1eddng k\u1ebft th\u00fac \u0111i\u1ec1u tr\u1ecb t\u1ea1i m\u1ed9t giai \u0111o\u1ea1n b\u1ec7nh n\u00e0o \u0111\u00f3. Tr\u1ea1ng th\u00e1i lui b\u1ec7nh c\u00f3 th\u1ec3 \u0111\u01b0\u1ee3c duy tr\u00ec sau khi ng\u1eebng thu\u1ed1c, nh\u01b0ng \u0111\u00f4i khi, b\u1ec7nh nh\u00e2n c\u1ea7n t\u00e1i s\u1eed d\u1ee5ng IVIg t\u1eeb h\u00e0ng tu\u1ea7n cho \u0111\u1ebfn m\u1ed7i 6 tu\u1ea7n d\u1ef1a tr\u00ean nguy\u00ean t\u1eafc \u201cc\u00e1 th\u1ec3 ca b\u1ec7nh\u201d.
\nC\u1ea7n nh\u1ea5n m\u1ea1nh r\u1eb1ng trong b\u00e0i \u0111\u00e1nh gi\u00e1 n\u00e0y, ch\u00fang t\u00f4i ch\u1ec9 t\u1eadp trung v\u00e0o c\u00e1ch ti\u1ebfp c\u1eadn \u0111\u00e3 mang l\u1ea1i hi\u1ec7u qu\u1ea3 t\u1ea1i c\u1edf s\u1edf c\u1ee7a m\u00ecnh. Ch\u00fang t\u00f4i khuy\u1ebfn kh\u00edch \u0111\u1ed9c gi\u1ea3 tham kh\u1ea3o li\u1ec1u IVIg kh\u00e1c nh\u01b0 \u0111\u00e3 li\u1ec7t k\u00ea ph\u00eda tr\u00ean.
\nPh\u1ee5 thu\u1ed9c v\u00e0o m\u1ee9c \u0111\u1ed9 n\u1eb7ng c\u1ee7a b\u1ec7nh t\u1ea1i th\u1eddi \u0111i\u1ec3m th\u0103m kh\u00e1m, ch\u00fang t\u00f4i c\u00f3 th\u1ec3 k\u1ebft h\u1ee3p IVMP v\u1edbi IVIg. C\u01a1 s\u1edf cho m\u00f3n cocktail n\u00e0y c\u00f2n thi\u1ebfu thuy\u1ebft ph\u1ee5c d\u1eabu r\u1eb1ng nghi\u00ean c\u1ee9u OPTIC \u0111\u00e3 s\u1edbm cho th\u1ea5y v\u00e0i tri\u1ec3n v\u1ecdng [70]. Ch\u00fang t\u00f4i duy tr\u00ec li\u1ec1u IVMP 1g\/tu\u1ea7n c\u00f9ng v\u1edbi IVIg 0,4g\/kg\/tu\u1ea7n. Tuy nhi\u00ean do ghi nh\u1eadn nhi\u1ec1u t\u00e1c d\u1ee5ng ph\u1ee5, ch\u00fang t\u00f4i ph\u1ea3i nhanh ch\u00f3ng d\u1eebng IVMP. T\u00e1c d\u1ee5ng ph\u1ee5 c\u0169ng l\u00e0 l\u00fd do khi\u1ebfn ch\u00fang t\u00f4i tr\u00e1nh s\u1eed d\u1ee5ng prednisone \u0111\u01b0\u1eddng u\u1ed1ng h\u00e0ng ng\u00e0y ho\u1eb7c c\u00e1ch ng\u00e0y, nh\u01b0ng n\u00f3 l\u1ea1i cho th\u1ea5y s\u1ef1 \u01b0u vi\u1ec7t tr\u00ean nh\u1eefng b\u1ec7nh nh\u00e2n kh\u00f3 l\u1ea5y \u0111\u01b0\u1eddng truy\u1ec1n t\u0129nh m\u1ea1ch ho\u1eb7c dung n\u1ea1p k\u00e9m v\u1edbi IVIg.
\nTrong tr\u01b0\u1eddng h\u1ee3p \u0111\u00e1p \u1ee9ng kh\u00f4ng th\u1ecfa \u0111\u00e1ng v\u1edbi IVIg v\u00e0\/ho\u1eb7c CCS, thay \u0111\u1ed5i sang PE c\u00f3 th\u1ec3 xem x\u00e9t. PE hi\u1ec7n v\u1eabn l\u00e0 ph\u01b0\u01a1ng ph\u00e1p \u0111i\u1ec1u tr\u1ecb \u0111\u1ea7u tay v\u1edbi nh\u1eefng b\u1ec7nh nh\u00e2n c\u00f3 l\u00e2m s\u00e0ng di\u1ec5n bi\u1ebfn x\u1ea5u nhanh. PE c\u00f3 th\u1ec3 \u0111i\u1ec1u tr\u1ecb t\u1ea1m th\u1eddi cho \u0111\u1ebfn khi m\u1ed9t ph\u01b0\u01a1ng ph\u00e1p \u0111i\u1ec1u tr\u1ecb h\u1eefu hi\u1ec7u kh\u00e1c \u0111\u01b0\u1ee3c t\u00ecm ra. N\u1ebfu c\u1ea7n thi\u1ebft, ch\u00fang t\u00f4i v\u1eabn k\u1ebft h\u1ee3p PE v\u1edbi IVIg tr\u00ean nh\u1eefng b\u1ec7nh nh\u00e2n kh\u00e1ng tr\u1ecb. \u1ede nh\u1eefng b\u1ec7nh nh\u00e2n n\u00e0y, PE d\u00e0i h\u1ea1n l\u00e0 r\u1ea5t hi\u1ec7u qu\u1ea3, m\u1eb7c d\u00f9 th\u00e1ch th\u1ee9c \u0111\u1eb7t ra khi truy\u1ec1n t\u0129nh m\u1ea1ch k\u00e9o d\u00e0i v\u00e0 t\u0103ng nguy c\u01a1 nhi\u1ec5m khu\u1ea9n.
\nV\u1edbi nh\u1eefng b\u1ec7nh nh\u00e2n CIDP kh\u00e1ng tr\u1ecb v\u1edbi ph\u01b0\u01a1ng ph\u00e1p \u0111i\u1ec1u tr\u1ecb ti\u00eau chu\u1ea9n, b\u01b0\u1edbc \u0111\u1ea7u ti\u00ean c\u1ea7n l\u00e0m l\u00e0 xem l\u1ea1i ch\u1ea9n \u0111o\u00e1n. Ph\u1ee5 thu\u1ed9c v\u00e0o tri\u1ec7u ch\u1ee9ng l\u00e2m s\u00e0ng, ch\u00fang t\u00f4i l\u1eb7p l\u1ea1i c\u00e1c b\u01b0\u1edbc th\u0103m kh\u00e1m nh\u01b0 \u0111\u00e3 li\u1ec7t k\u00ea \u1edf B\u1ea3ng 1. Sinh thi\u1ebft th\u1ea7n kinh c\u00f3 th\u1ec3 c\u1ea7n thi\u1ebft \u0111\u1ec3 ch\u1ea9n \u0111o\u00e1n x\u00e1ch \u0111\u1ecbnh. Tr\u00ean b\u1ec7nh nh\u00e2n ph\u00e1t hi\u1ec7n kh\u00e1ng th\u1ec3 kh\u00e1ng neurofascin-155 v\u00e0 contactin-1, rituximab n\u00ean \u0111\u01b0\u1ee3c l\u1ef1a ch\u1ecdn. Rituximab v\u00e0 cyclophosphamide c\u0169ng c\u1ea7n \u0111\u01b0\u1ee3c c\u00e2n nh\u1eafc tr\u00ean nh\u1eefng b\u1ec7nh nh\u00e2n kh\u00e1ng th\u1ec3 huy\u1ebft thanh \u00e2m t\u00ednh v\u00e0 kh\u00f4ng c\u00f3 nguy\u00ean nh\u00e2n thay th\u1ebf n\u00e0o ph\u00f9 h\u1ee3p, ho\u1eb7c tr\u00ean c\u00e1c b\u1ec7nh nh\u00e2n c\u00f3 \u0111\u00e1p \u1ee9ng v\u1edbi PEX m\u1eb7c d\u00f9 hi\u1ec7u qu\u1ea3 \u0111i\u1ec1u tr\u1ecb v\u1eabn c\u00f2n kh\u00e1 h\u1ea1n ch\u1ebf.<\/p>\n

10. K\u00ea\u0301t lu\u00e2\u0323n<\/strong><\/h2>\n

Ch\u1ea9n \u0111o\u00e1n CIDP v\u1ed1n d\u1ef1a v\u00e0o l\u00e2m s\u00e0ng, \u0111\u01b0\u1ee3c h\u1ed7 tr\u1ee3 b\u1edfi d\u1ea5u hi\u1ec7u m\u1ea5t myelin tr\u00ean \u0111i\u1ec7n c\u01a1 tu\u00e2n th\u1ee7 c\u00e1c ti\u00eau ch\u00ed chu\u1ea9n h\u00f3a nh\u01b0 c\u1ee7a EFNS\/PNS. \u1ede nhi\u1ec1u ca b\u1ec7nh, ph\u00e2n t\u00edch d\u1ecbch n\u00e3o t\u1ee7y, ch\u1ee5p MRI, si\u00eau \u00e2m ho\u1eb7c sinh thi\u1ebft th\u1ea7n kinh c\u00f3 th\u1ec3 h\u1ed7 tr\u1ee3 ch\u1ea9n \u0111o\u00e1n. \u0110\u00e1nh gi\u00e1 \u0111\u00e1p \u1ee9ng v\u1edbi c\u00e1c li\u1ec7u ph\u00e1p mi\u1ec5n d\u1ecbch c\u1ea7n ph\u1ea3i d\u1ef1a v\u00e0o c\u00e1c th\u00f4ng s\u1ed1 kh\u00e1ch quan. V\u1eabn c\u00f2n m\u1ed9t ch\u1eb7ng \u0111\u01b0\u1eddng d\u00e0i ph\u00eda tr\u01b0\u1edbc trong c\u00f4ng cu\u1ed9c t\u00ecm c\u00e1c ch\u1ea5t ch\u1ec9 \u0111i\u1ec3m sinh h\u1ecdc c\u1ea7n thi\u1ebft cho ch\u1ea9n \u0111o\u00e1n v\u00e0 di\u1ec1u tr\u1ecb, c\u0169ng nh\u01b0 ph\u00e1t hi\u1ec7n c\u00e1c ph\u01b0\u01a1ng \u00e1n \u0111i\u1ec1u tr\u1ecb thay th\u1ebf cho nh\u1eefng b\u1ec7nh nh\u00e2n kh\u00e1ng tr\u1ecb v\u1edbi ph\u01b0\u01a1ng ph\u00e1p c\u00f3 s\u1eb5n hi\u1ec7n t\u1ea1i.<\/p>\n","protected":false},"excerpt":{"rendered":"

Vi\u00eam \u0111a r\u1ec5 v\u00e0 d\u00e2y th\u1ea7n kinh m\u1ea5t myelin m\u1ea1n t\u00ednh (CIDP) l\u00e0 b\u1ec7nh l\u00fd \u0111\u1eb7c tr\u01b0ng b\u1edfi m\u1ea5t c\u1ea3m gi\u00e1c v\u00e0 li\u1ec7t ti\u1ebfn tri\u1ec3n t\u0103ng d\u1ea7n, th\u01b0\u1eddng \u1ea3nh h\u01b0\u1edfng \u0111\u1ebfn kh\u1ea3 n\u0103ng v\u1eadn \u0111\u1ed9ng c\u1ee7a ng\u01b0\u1eddi b\u1ec7nh c\u0169ng nh\u01b0 trong vi\u1ec7c th\u1ef1c hi\u1ec7n c\u00e1c ch\u1ee9c n\u0103ng s\u1ed1ng h\u00e0ng ng\u00e0y m\u1ed9t c\u00e1ch \u0111\u1ed9c l\u1eadp. V\u00ec […]<\/p>\n","protected":false},"author":42,"featured_media":0,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":[],"categories":[10,33],"tags":[],"yoast_head":"\nVi\u00eam \u0111a r\u1ec5 v\u00e0 d\u00e2y th\u1ea7n kinh m\u1ea5t myelin m\u1ea1n t\u00ednh (CIDP) \u2013 Nh\u1eefng b\u1eaby trong ch\u1ea9n \u0111o\u00e1n v\u00e0 c\u00e1ch ti\u1ebfp c\u1eadn \u0111i\u1ec1u tr\u1ecb<\/title>\n<meta name=\"description\" content=\"Vi\u00eam \u0111a r\u1ec5 v\u00e0 d\u00e2y th\u1ea7n kinh m\u1ea5t myelin m\u1ea1n t\u00ednh g\u00e2y m\u1ea5t c\u1ea3m gi\u00e1c, li\u1ec7t ti\u1ebfn tri\u1ec3n t\u0103ng d\u1ea7n, \u1ea3nh h\u01b0\u1edfng v\u1eadn \u0111\u1ed9ng, ch\u1ee9c n\u0103ng s\u1ed1ng h\u00e0ng ng\u00e0y.\" \/>\n<meta name=\"robots\" content=\"noindex, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<meta property=\"og:locale\" content=\"en_US\" \/>\n<meta property=\"og:type\" content=\"article\" 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