DiSn San
B$NH KAWASAKI
Nguyen Thi Thoi Loan*
T h u a t nguTbenh
Hpi chdng da niem mac vd hach lympho (mucocutaneous lymph node syndrome) Mot so ddc diem chia khoa:
- Bieu hien benh: sot, viem ki't mgc mat, thay doi niem mac mieng, phu dau chi vd benh lyhgeh lympho deo.
- Thuang ton la phdt ban da dgng (polymorphous exanthem), vi tri thddng gap nhat Id vung day chdu.
- Benh gap d cdc quoc gia phdt trien, bien chdng thddng gap nhat la benh tim mdc phai d tre em.
I. Gidll THIEU VA LfCH S\f B | N H
Benh Kawasaki la benh sot cap co thUOng t o n nhieu CO quan bo p h i n t r o n g co the. ThUdng gap d tre dudi 5 tuoi.
Benh dugc m d t i lan dau tien bdi Kawasaki la tre em ngudi Nhat, va sau d d ddge b i o eao tren toan the gidi. Cie bao eao d My cho r i n g benh la nguyen nhan gay benh t i m m i c p h i i d tre em. Gian hoac phinh d d n g mach v i n h p h i t trien khoang 20-25% d n h d n g benh n h i n khdng dugc dieu tri, la nguyen n h i n g i y ra t d vong d t r e .
Bieu hien lam sang c d t h e nham lan vdi nhieu benh, d i e biet la p h i t ban do virus va vi khuan.
Benh g i y sdt va viem khdp cap t i i phat, l i nguyen nhan pho bien nhi't gay benh t i m m i c phai d t r ^ e m
Nguyen n h i n ciia benh edn chda dugc biet nhung d i e trUng dich te goi y can nguyen do nhiem triing.
* Benh vien Phong Da Lieu TW Quy Hoa
I I . D j C H T ^ H p C
Tuoi mac b e n h d d i n h t u o i la 2 t u d i nhUng co the nhd b a n , ngudi ta cho r i n g d o thieu khing t h e b i o ve d Ida t u o i nay.
Khoang 85% tre e m m i c benh nay d t u o i nho ban 5 tudi, tre trai g a p 1,5 lan tre g i i .
d Bac My, b e n h xuat hien t h e o miia, cuoi mua d d n g v i dau mua xuan.
Benh p h o bien h o n d tre e m c h i u A, dieu do ggi y yeu t d gen cd vai t r d t r o n g c i m nhiem benh nay. Tr^ e m nam n h d hon m g t tudi mac benh, it cd n g u y co bien chdng t i m mach hon tre Idn hon 6 t u o i .
III. SINH BENH HOC
Can nguyen g i y b e n h t h i chda dugc biet nhUng nhieu q u a n s i t g g i y can n g u y e n d o nhiem t r u n g dUa tren n h d n g d i e diem lim s i n g , t i n h chat tai p h i t , t u o i mac benh, djch te theo m u a . . . Tuy n h i e n , nuoi ci'y benh pham da va m i u k h d n g t i m t h a y vi khuan g i y benh. Vi khuan sinh dgc t o la y e u t o can nguyen dUdc
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D A L l £ U H O C So 10 (Thang 03/2013)nghi den nhung cung ehua ed bang chdng de chdng minh.
Benh dUge d i e t r u n g bdi kich boat c i c marker cua he t h d n g mien djch, tang so lugng te bao lympho B va T duge hoat hda., te b i o don nhan eung nhU cac cytokine tien viem.
6 nhdng t r u d n g h g p benh Kawasaki t d v o n g , ngddi ta tim thi'y trong d g n g mach v i n h , tuy, than v i h d hap tren cd n h d n g te bao plasma tiet IgA, chat nay dugc t i m thi'y d n h d n g tr6 em bj tCf vong vi viem d u d n g hd hap tren do virus.
Cac nghien cdu ve mien djch thay rang:
khing the Oligoelone duge t d n g hop trong phong t h i nghiem chi ra cd gan ket vdi k h i n g nguyen b i o tUOng (cytoplasmic) ngay tren Idp te bao bieu m d ciia phe q u i n gde, te bao ngi mo ciia dong mach v i n h , m d t sd m d khae v i dai thUe bao d benh nhan Kawasaki. Nhdng p h i t hien nay khang dinh c i c t i c n h i n g i y nhiem triing dddng ho hap cd xu hUdng lam t h u o n g ton mach mau.
IV. Ti£u CHUAN CHAN DOAN
sdt keo dai hon 5 ngay kem theo 4 tren 5 tieu chuan sau:
I.Viem ket mac mat hai ben khdng kem theo d d m i t
2. Nhdng thay doi d mieng hgng g o m : viem do hau hgng lan tda, nut mdi, ludi do n h u q u i d i u tiy.
3. Benh ly hach lympho d cd
4. Thay ddi d tay, chan nhU: d d da, phu ne, bong vay d tay va c h i n .
5. Phat ban da dang
Chan doan benh Kawasaki ddge nghT den; d tr§ em sdt keo d i i k h o n g g i i i thich 6dac kem theo eic trieu chdng da. Dau tien sdt cao dot ngot, sot keo d i i v i khdng dap dng vdi thuoc ha nhiet.
Han 8 0 % b e n h nhan Kawasaki ed phat ban tren da, ban bieu hien da dang nhU ban d a n g sdi, ban dang dat do, ban dang san do n h u n g ed khi la ban d a n g t i n h h d n g nhiet, ed khi g i d n g h o n g ban da d a n g v i cd khi la ban m u n m u .
Thuong tdn it gap la mun nUde, bgng nUde, v i y tiet va xuat huyet.
d mdt s d b e n h nhan, loet d nhdng v i t r i tiem vaccine BCG cung ed the xay ra trong qua trinh benh.
Bieu hien da d i e trdng l i do da vung day chau va tien trien den d d da trong vdng 48 gid.
Phil edng tay, c h i n l i bieu hien sdm va thudng gap nha't eua benh, tien trien den bong v i y da va ehii yeu b o n g vay d viing quanh mdng. Hoai t d quanh cac dau chi ddi khi cd the x i y ra.
Viem ket mac mat nhung khong co tang tiet nudc mat, khdng cd d d mat, cd quang do d b d ngoai giac mae, Viem giac mac va sg i n h s i n g it gap nhdng neu cd ggi y chan doan phan biet vdi benh k h i c .
Nhdng thay doi d mieng hgng bao gom: khd moi, nut mdi, lUdi do nhUqua dau tay, d mgt so benh nhan tang xui't huyet d niem mac mieng.
Bieu hien t i m mach g d m : viem cO t i m , suy tim sung huyet v i phinh dgng mach v i n h la nguyen n h i n g i y t d vong nhieu nha't d tre em mac benh nay. Benh van t i m cd the xua't hien d benh nhan n i y nhUng hay gap la van hai l i va dgng mach chii. Tim nhanh, cd tieng ngUa phi, tieng t i m xa x i m . . . l a nhdng trieu chdng can duoc chu y khi kham t i m .
C i c bieu hien k h i e kem theo cung can ddpc tham kham nhU: he than kinh trung uong (viem mang nao vd trung, liet day than kinh sg n i o , diec tai); he tieu hda (dau bung, tieu chay, roi loan chdc nang gan, vang da, tac mat); he ca xuong (viem khdp, dau khdp); he nieu due
So 10 (Thing 03/2013) D A L l £ U H O C I 6 3
(viem d u d n g tiet ni^u, cd bach cau t r o n g nUdc tieu n h U n g v d khuan)
C i c bleu hien khdng dien hinh cda b e n h l i sdt keo d i i kem vdi b e n h d g n g mach vanh (xac dinh b i n g sieu am t i m v i chup mach vanh), trong trUdng hgp nay kho de eh^n doan xac d j n h benh Kawasaki.
V. CAN LAM SANG
Cong thdc m i u : bach c i u m a u t i n g chii y ^ u l i bach cau da n h i n trung t i n h .
Mau l i n g : t i n g va tang protein C phan d n g . Men gan: tang
Cd bach eau trong nddc tieu nhUng v d t r u n g . Thieu mau va giam so lugng tieu cau.
Tuan t h d h a i den t u a n t h d b a , thrombocytosis t h u d n g hien dien.
Dien t i m do: sdng R g i i m dien the, ST chenh, T det hoae dao ngugc
Md benh hgc: khdng d i e hieu
Phu trung bi v i x i m n h i p viem bach cau dan nhan quanh cac mach mau.
VI. CHAN D O A N P H A N BIET
Sdt p h i t ban d o virus (sdi, adenovirus) hay vi khuan.
Sot tinh hdng nhiet.
Hgi chdng sdc nhiem dgc Hgi chdng b o n g v i y da d o t u eau Hong ban da dang
Di dng thudc
P h i n d n g g i o n g h g i c h d n g huyet t h a n h Viem n u t q u a n h d g n g mach d tre e m Hgi c h d n g toan t h a n cua b e n h viem khdp 6 t r ^ e m
V I I . D I ^ U T R )
1. Giai dogn cap (10 ngdy dau cua benh) - Chgn lUa d a u tien la: I m m u n o g l o b u l i n (Ig) t i n h mach 2 g / k g m o i 12 g i d .
Tiem eho d e n khi b e n h c i i t h i e n va phdng bien c h d n g p h i n h d g n g mach vanh.
Co che t h u d e Ig t r o n g d i e u trj benh n i y eon chua ro.
Nghien c d u cho thay, t i e m Ig lam g i i m tl le bien c h d n g p h i n h d g n g mach vanh t d 20-25%
x u o n g 2-4%, ha t i le chet t i r 2 % x u d n g 0,3%
- Aspirin la t h u o c c u n g duge de cap trong dieu tri benh nay d giai d o a n cap.
Lieu 80-1 OOmg/kg/ngay chia 4 lan, diing den khi eat sdt ( t h u d n g ia 14 ngay) thi ha lieu xuong cdn 3-Smg/kg ( t h u d n g 6-8tuan) va eat aspirin khi dien t a m d o k h d n g cd bieu hien benh ly gi.
Neu dien t a m d o co bieu hien benh ly thi van p h i i d u y tri aspirin lieu t h a p Hen tuc.
C i c loai vaccine t i e m p h o n g cho tre can dugc hoan tai sau khi t i e m Ig 6-11 t h i n g .
2. Sau giai dogn cap
Theo d d i dien t i m d o ; Cdng thdc mau; Toe do i i n g m i u sau giai d o a n cap 2-3 tuan. Kiem tra lai sau 6-8 t u a n . N g d n g d u n g aspirin khi dien t i m d d va toe d o lang m i u b i n h t h u d n g .
TAILIIUTHAMKHAO
Jaen L Bolognia (2008), Kawasaki disease.
Dermatology, p l 2 3 2 - 1 2 3 3
Thomas R Habif (2004), Kawasaki syndrome,.
Clinical dermatology, p475-456
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(Thang 03/2013)HiNH A N H M I N H HOA B E N H KAWASAKI IGIAI O O A N M A N )
Benh nhi 36 thdng tuoi, dieu tri tgi khoa Hoi site cap cifu Benh vien Phong Da Lieu TVJ Quy Hoa
So 10 (Thang 03/2013) DA Ll£U HOC I 6 5
