Y HQC VigT NAM THANG 9 • S6 BAC BlgT/2012 4. John H.Menkes, Harvey B.Samat (2006)
Child Neurology, Heredodegenerative '!, Diseases; Lippincott Williams & Wilkins,
seven edition,p. 163-179.
5. Jean Alcardl (2009). Diseases of the
„ nervous system in childhood, movement , A. disorders (predominant involvement ofthe basal ganglia). 3^^ edition, Mac Keith , Press,p 350-351.
Segawa M, Hosaka A, MIyagawa F, Nomura V, Imal H. (1976). Herediatary progressive dystonia with marked diumal fluctuation. Adv. Neurol.; 14: 215-33.
. Segawa M. (2011) Hereditary progressive dystonia with marked diumal fluctuation.
Brain Dev. 2011 Mar; 33(3) : 195-201.
Epub 2010 Nov.
NHAN M O T TRUdNG HOP HOI CHliNG RO RI MAO MACH XAY RA SAU DUNG YEU T 6 KiCH THJCH T A N G SINH BACH CAU HAT DUUG DIEU TRj
THANH C O N G D & N G LOC MAU LIEN TUC VA CORTICOSTEROIDS BfngAnh Buong*, Trin Minh Blin*, Dfng Vdn Thi-c*, Phgm Hong San*
T 6 M T A T :
Hil ching r i ri mao mach (capillary leak syndrome - CLS) Id mdt tinh tr^ng binh hiim gjp. Binh xdy ra do tinh trang tdng tinh thdm thinh mgch. CLS Id mdt trong nhO'ng blin ching sau ghdp t i bdo gdc tuj xuong, Ca binh: Binh nhdn nam, 32 thdng tuii, 10 kg, dupc chlin dodn binh iy thupng bl bpng nude bdm sinh. Binh nhdn dupc ghdp t i bdo gdc tuy xuong. Sau ghip 57 ngdy binh nhdn c6 tinh trgng gidm bach ciu hgt trong mdu vd dupc ding yiu to kich thich tdng sinh bgch clu hgt (granulocyte colony stimulating factor - G- CSF). Sau 2 ngdy su dgng G- CSF binh nhdn xudt hiin CLS. Bliu trj CLS ngodi hi trp hd hdp, tudn hodn, binh nhdn dupc dting melhylprednlsolone 2 mg/kg/ngdy ffnh mgch vd lpc mdu liin lyc vdl kiiu lpc CWHDF.
KSt qud diiu trj, sau 48 gid lpc mdu binh nhdn cal dupc mdy thd vdl cdc chi s6 hd hdp, huyit ding in djnh, hit phi vd ngdng lpc sau 80 gid
* i Idm sdng dn d[nh, bdl nliu tdt Kit lujn: CLS li mit blin chirng sau ghip t i bdo gic tu9 'Binh vifn Nhi tmng uang
xuong, binh cd thi xay ra sau dung G- CSF. Su dgng methylprednisoion vd Ioc mdu lidn tgc cd thi dliu tri CLS vd cCru sing binh nhdn.
ru" khda: h& chdng rd ri mao mach, iQC m&u liin tyc, ghip ti btio gdc tdy xuong, thuic tSng sinh bach ciu hat
I. OifkT V A N oi:
Hdi ching rd ri mao mach (capillary leak syndrome - CLS) la mot tinh ttang b$nh hiim gdp. Bgnh xdy ra do tinh tt^g tang tinh thim thdnh mgch, do do gdy ra thodt djch vd protein t i Idng mgch vdo khodng gian bdo, phi todn than, sic va suy thjn. Tren binh nhan sau ghip ti bdo gic tuy xuong, CLS Id mpt trong nhttng biin chimg sdm cd thi gdp sau ghep do tin thuong cac te bdo ndi md mao mgch gjy ra thodt dich vao khoang ki.
Chan doan chu yiu dya vdo cdc trigu chimg Iam sdng bao gim: binh thudng xdy ra ttong khoang 15 ngay sau ghep, tdng cdn > 3%
C H U Y E N og: HQI NGHI K H O A H Q C N G A N H NHI KHOA T O A N QUO'C L A N T H O IX ttong vdng 24 gid, phi todn thdn (ttdn djch i
byng, ming phii, ming tim), tinh trgng phi khdng ddp ting vdi dliu ttj Ipi tiiu, nhjp tim nhanh, hg huyit dp, suy thjn cd nguin gic trudc thjn vd gidm albumin mdu[l]. CLS khdng cd diiu ttj djc hiju, ty Ii t i vong cao niu cd suy da tgng. Binh vijn Nhi Trang uong dd tiin hinh nhiiu ca ghip ti bdo gic tuy xuong thdnh cdng, nhung chua timg gjp biin ching CLS. Trong qud trinh chdm sdc theo ddi bjnh nhdn sau ghip ti bdo gic ttiy xuong cho binh nhdn mdc ching bgnh ly thupng bl bpng nude bim sinh, chung tdi dd gjp hpi ching ndy vd dd diiu trj thdnh cdng bdng phucmg phdp diiu ttj thay the thjn liin tyc. Do vjy ching tdl muin thdng bdo h$i chung hiim gjp ndy sau ghip ti bdo gic tuy xuong de chia si vd giup cdc ding nghiip phdt hiin sdm vd diiu trj hpp ly cd thi ciru sing benh nhan.
II. THONG TIN BENH NHAN:
Bgnh nhan Id tti trai, 32 thdng tuii, 10 kg, dupc chin dodn bjnh ly thupng bl bpng nude bim smb. Bgnh nhdn dupc ghip ti bdo gic tuy xuong t i ngudi cho Id chj radt vdi sy hod hpp md Id 7/ 8 allele.
Sau ghip bgnh nhdn dupc diiu trj bdng thuic ching thdi ghip cyclosporine, duy tri ning dp TO 200 - 300 ng/ml, cellcept t i ngdy -I cho den ngdy thi 35 sau ghip, phdng ching nhiem b ^ g bdng khdng sinh meropenem, phdng ching nam (nystatin uing), phdng nhiem virus (acyclovh uing), phdng pneumocitis carini (bactrim uing), G - CSF di kich thich tgo bgch cau hgt t i ngdy thi 15 sau ghip, ttuyin khii hing ciu lpc bgch cdu chiiu tia xg de duy tri hemoglobin 7- 10 g/dl, khii tiiu cdu lpc bgch cau chiiu tia xg di duy hi tiiu ciu > 30000/mm' vd chdm sdc da, dam bdo dinh dudng cho bgnh nhan.
Diin blin sau ghip 3 ngdy txi sit cao liin tyc vd ciy mdu cd nhilm khuin mdu Burkhoderia cepacia vd bgnh nhdn dupc diiu trj theo khdng sinh di (meropenem), sau 4 ngdy kiim sodt dvpc nhilm tring mdu, tri hit sit vd cdc xit nghiim khdng cdn tinh trgng nhilm tring vd ciy mdu Igi khdng thay vi khuin mpc. Sau 21 ngdy, bgnh nhdn dupc chpc tu^ vd ciy nhilm sdc thi thi cho thiy tuy dd mpc tit, nhilm sdc thi Id 46, XX.
Nhvng tiiu ciu mdu ludn thip vd phdi tniyin khii tiiu ciu mdy lpc bgch ciu chiiu tia xg hdng ngdy. Sau ghip 40 ngiy tti bj nhiim CMV (cytomegalo viras) 200 000 copy/ml vd dupc dieu trj bdng ganciclovir 5mg/kg, 2 lin/ngdy trong 2 tuin, sau dd ngdy 1 lin tiip tyc ttong 2 tuin.
Ngdy thi 57 sau ghip, bgch ciu hgt bjnh nhdn thip, vd dupc ding G-CSF 10 pg/kg ttnh mgch ngdy mpt Idn. Sau tiim G - CSF 2 ngdy, bgnh nhan phi tdng dan, tdng cin tu 10 kg lin 11,5 kg trong 48 gid, ddi it din, khong ddp ing vdi lasix tinh mgch (1 mg/kg x 3 lin/ngdy), nong dd cyclosporine TO tdng din t i 240 -> 326 ng/ml mjc d i lliu lupng thuic cyclosporine khdng thay dii. Din ngdy 59 sau ghip, bgnh nhdn phu to toan thdn, xuit hign khd thd, nhjp thd 60 lin/ phit, SpOi gidm < 85%, hai phii cd nhiiu ran im, mgch 170 lin/phut, huyit dp gidm 81/32 mmHg, vi ddi rit il, CVP 5 mmHg. Sieu am cd djch mdng phii, mdng tun, mdng byng, chic ning tim binh thudng. Chyp X quang phoi md khdng ding diu lan tod khdp hai phii. Binh nhdn dvpc djt ngi khi qudn thd mdy hoin todn, hit n$i khi qudn cd nliiiu djch tiet ttong qua npi klii qtidn, chpc hit djch mdng phii moi bin 70 ml djch ving trong, proteui ttong djch mdng phii < 30g/I, ding thuic ttp tim dopamine 5 - 7.5 )ig/kg/phit, lasix 20 mg tinh mgch sau dd truyin vdi lieu lupng 0.8
386
Y HQC VI$T NAM T H A N G » • s g OAC BieTf2012
ttg/kg/gid. Sau thd may 24 gid, tinh ttgng hd hip cdi thign din, SpOa tdng dan t i 40 90%, chyp X quang phii cd cdi thiin hon tinh tttmg thdng khi nhung cdn md nhiiu hai phi ttudng, huyit ddng in djnh vdi liiu dopamine 7.5 pg/kg/phit, nhung bgnh nhdn c6 ttah tt^gng qud tdi djch rat rd, phi to hon, thiiu niju (nude tiiu 0.6 ml/kg/gid) vdi liiu lasix 0.8 mg/kg/gid. Binh nhdn dupc tiin hinh lpc mdu liin tyc vdi mode CWHDF
Diin biin lam sdng
(Continuous Veno Venous Hemodiafilttation), ttong thdi gian lpc mdu binh nhdn khdng ding thuic Ipi tiiu. Sau lpc mdu 54 gid, tinh ttang ldm sdng cdi thiin rd rit, hai phii thdng khi tit khdng cd ran im, huyit ddng in djnh, binh nhdn hit phi, binh nhdn dupc ding Igi lasix vd cd nude tiiu trd Igi 3 ml/kg/gid vd bgnh nhdn dupc ding lpc mdu liin tuc.
Hi hdp SPO,(%) Mgch (iin/phiit) Huyitdp (mmHg) CVP (cmHjO) Nude tiiu (ml/kg/gld) Lasix (mg/kq/qld) (^n ndnq (kq)
B i t ddu IQC mdu TM 90 92 112/42
5 0.97
0.8 12
Sau 12 g i d TIVI 100 93 122/55
6 0.12
0 11.5
Sau 24 g i d TM 99 77 106/39
6 0.13
• 0
11 Sau 36
gid TM 100 62 130/69
5 0 0 10.5
Sau 48 g i d TM 100 127 136/73
8 0 0 10.2
Sau 60 g i d T u t h d
100 132 132/73
5 1 0.4 9.8
Sau 72 g i d T u l h d
100 120 137/87
6 3 0.4 9.5
Sau 80 gid T u t h d
99 117 138/73
5 2 0.4 10 TM: tha may
f. Thoi gian loc mau lien tiic 80 gicr, thcri gian tha may 58 gifir t Cdcxdtttghifm
Hemoqiobin Hematocrite (%) ilgch cdu (1000) .Tliu odg (1000) .Ure (mmol/l) Creafnin (umol/i) Froleln (a/I) Albumin (q/l)
Bit diu lpc mdu 68 18.3 2.7
62 24.8 135.5 64.8 37
Sau 12 gid
99 29.1 1.98 57 6.2 64.6 69.1 39.2
Sau 24 gid 101 29 1.63 126 3.1 46.4 68.1 39.9
Sau 36 gid 99 29.1 2,21 120 3.3 59.4 75.6 43.5
Sau 48 gid
93 26.2 2.75 70 3.2 46.7 64.1 38.1
Sau 60 gid
90 25.2 2.25 41 2.9 51 66.4 38.4
Sau 72 gid
84 23.6 2.22 210 3.8 40.9 63.2 42.3
C H U Y S N Bi: HQI N G H | K H O A HQC N G A N H NHI KHOA T O A N Q U O C LAN THCT IX
III. BAN L U A N :
Hpi ching rd ri mao mgch hi thing Id mit hdi chimg hiim gjp, khd chin dodn giai dogn sdm, tiiudng dupc phdt hiin khi cdc ttiiu ching diy di. Triiu ching bao gim hg huyet dp, cd djc mdu vd gidm albumin mdu, phi todn thdn tiin triin nhanh, phi mjt, thdn, chi vd kim theo ttdn djch da mdng, kim ddp ing vdi thuic Ipi tiiu, khdng nguyin nhdn rd rdng [1,2]. Ddy Id lin dau tiin chung tdi gjp hpi ching ndy ttin binh nhdn sau ghip ti bdo gic tuy xuong, do vjy cing chi chin dodn dupc CLS khi cdc diu hiju Idm sdng tuong dii diy di, bgnh nhdn phi todn thdn, trdn djch da mdng, tdng can 5% trong vdng 48 gid, CVP thap, huyit dp thap hon nhiiu (81/32 mmHg) so vdi nin huyit dp trade khi bgnh nhdn cd dau higu phi (120-130/70-80 nmiHg), ndng dp albumin ttong mdu gidm 32-33 g/l.
Nguyin nhdn ciia CLS chua rd rdng, nhung theo bdo cao cia tdc gia I. Rechner vd cdng sy cho thdy CLS xdy ra sau khi binh nhan dupc dung G - CSF trin bgnh nhdn ung thu mdu cd gidm bgch ciu hgt[3]. Naoki Nakagawa vd cpng sy cung dd thdng bdo mpt tradng hpp CLS tien phdt sau khi s i dyng G - CSF, tdc gid dd tim thiy mii liin hg gitta ndng dp G - CSF cao ttong mdu vd tien trien lam sing cia CLS vd G - CSF cd thi ddng vai ttd quan ttpng khdi phdt bgnh vd dupc s i dyng nhu mpt marker sinh hpc cho chin dodn njng CLS[4]. Theo A. Dagdemir vd cpng sy cho rdng dnh hudng tryc tiip cia G - CSF len te bdo npi md thdnh mgch cd thi ddng vai ttd chi yiu trong co chi binh, G- CSF cd the gay ra CLS thjm tti khi si Ivpng bgch ciu thip. G- CSF cd thi kich thich hogt hod ti bdo bgch ciu ttimg tinh gidi phdng ra cdc chdt trang gian hod hpc, t i dd gay ra pha hu^ ti chic vd xuit hijn ttigu chimg todn
thdn do tdng tinh thim thdnh mgch.'Mgh nhdn cia ching tdl sau ghip ti bdo ^^aj xuong ngdy thi 57, si lupng bgch ciu trang tinh cdn lit thip < 500 bgch ciu/mm', ain binh nhdn dupc dung G- CSF vdi liiu 10
^g/kg tiim tinh mgch, ngdy m$t lin. Sau 2 ngdy bjnh nhdn diln biin rit nhanh vd biiu hiin diy di cdc triiu ching cia CLS mi trade khi ding thuic bjnh nhdn khing ci.
Hon nia bgnh nhan cia ching tdi dvpc ^ i p te bdo gic tuy xuong ttin binh nhdn mdc binh ly thupng bl bpng nude bim suih nin bdn thdn cdc t i chic da, niim mgc, npi mgc mgch mdu cflng rit de bj tin thuong. Do vjy mjc d i ching tdi khdng djnh Iirpng dupc ning dd G- CSF, nhung theo cdc bdo cdo cua cdc tdc gid nude ngodl[l, 3, 5], thi ching tii nghl nhiiu din CLS gay ra do ding G- CSF.
Diiu trj CLS cdn gjp nhiiu khd khdn, vi tiin lupng xiu. Theo Naoki, b#nh nhdn dupc diiu trj thdnh cdng vdl corticosteroids, khdng beta - 2 terbutaline, vd theophylline. Nhung theo tdc gid M. Kirk, steroid khdng ngdn cin dupc tien triin cia bgnh vd cd thi Idm cho bgnh trim ttpng hon. Khi cd tinh trgng giim huyit dp, ding cdc thuic vjn mgch, vi ttuyin nhanh cdc dung djch cao phdn tu (albumin 25%) dya vdo gidm sdt dp lyc flnh mgch ttimg tdm di duy tri huyit ding in djnh. Bgnh nhdn cia chiing tdi khi cd tinh trgng suy hd hip, khd thd, phii nhiiu ian im, ttdn djch mdng phii, X quang phii tin md Ian tod 2 phii (hinh 1), binh nhan dvpc hi tip thd mdy vdl cdc thdng si cdi djt cao (ip lyc mmg binh dudng thd 19 cmHzO, FiOi 100%, PEEP 9 cmHjO), nhung tinh ttgng oxy mdu rat thip, SpOj 45- 50%, PvO: 15%, duy tti tinh trgng huyit dpng i n djnh vdi lieu Dopamm 7.5 pg/kg/phit. Ching tdi dung thuic Ipi tiiu trayin tinh mgch lien tyc 0.8 mg/kg/gid nhtmg nude tieu khdng cd.
Y HQC V I $ T NAM T H A N G 9 • s 6 B A C BlgT/2012
Hlnh 2: Sau lpc mdu 48 gid
Mjc du diiu tti tich eye nhung tinh trgng binh nhan khdng cdi thign, phi toan thdn ting ddn, nude tiiu khong cdi thign, ching til quyet djnh lpc mdu liin tyc vdi kiiu lpc CWHDF. Vdi kiiu lpc mdu nay ngodi vigc lit nude ra khdi bgnh nhdn, cdn giip lpc cdc chit trung gian hod hpc gjy viim ttpng lupng phan hi vira vd nhd. Chinh vi vgy, sau 48 gid Igc miu mdu, binh nhan dupc thoat may vdi tinh ttgng oxy mdu tit, X quang phdi hai
^ i thing khi tit (hinh 2) vd sau 80 gid lpc liiiu bjnh nhdn dupc ngimg lpc vdi cac dau liigu sing in dinh, nude tiiu tit, hit phi.
Theo bdo cdo mdi nhdt 2011 vi kinh nghijm 1 ^ ttj CLS 28 bgnh nhdn CLS d chau Au,
M. Gousseff vd cpng sy cho rdng diiu trj phdng khdng beta 2 hojc immunoglobulin tinh mgch cd thi ldm gidm nguy co mdc bjnh vd mic dp njng vd cd thi cdi thiin t^ li sing [6]. Nhung vdi bjnh nhdn ndy ching tdi chi dung corticosteroids phii hpp vdi lpc mdu liin lyc cho kit qud tit, ciru sing binh nhan.
IV. KET LU$N:
Hpi chimg rd ri mao mach Id mdt ttong nhttng biin ching cua bgnh nhdn sau ghip te bdo gic tuy xuong. G- CSF la mot yiu to thic day CLS. Chan dodn sdm thudng khd, chi phdt hiin khi bieu hijn cdc Idm sdng diy di. Diiu ttj, ngodi cdc bign phip hii sic ho ttp hi hip, tuan hoin, duy tri cac chic ndng sing, ding methylprednisolone vd chi djnh Igc mdu sdm khi cd tinh trang tdng can > 3%
ttong lupng CO thi ttong 24 gid giup phdng ching dupc suy hd hip, cim sdng bgnh nhdn.
TAI UEU THAM K H A O :
1. J. Apperley, E.C., E. Gluckman, A.
Gratwohl, T. Masszi, Haemalopoielic Stem Cell Transplanlalion. 5 * ed. 2008: European School of haematology. 188 -189.
2. Druey, K.]VI. and F.R. Greipp, Narrative review: the systemic capillary leak syndrome.
Ann Intern Med. 153(2): p. 90-8.
3. Rechner, 1., F. Brito-Babapulle, and J.
Fielden, Systemic capillary leak syndrome after granulocyte colony-stimulating factor (G-CSF). Hematol J, 2003.4(1): p. 54-6.
4. Nakagawa, N., et al., A case of idiopathic systemic capillary leak syndrome with high serum levels of G-CSF on exacerbation.
Intern Med. 50(6): p. 597-600.
5. Dagdemir, A., et al., G-CSF related capillary leak syndrome in a child with leukemia. Leuk Lymphoma, 2001. 42(6): p. 1445-7.
6. Gousseff, IM., et al., The systemic capillary leak syndrome: a case series of 28 patients from a European registry. Ann Intern Med.
154(7): p. 464-71.
