1 B GIO DC V O TO TRNG I HC Y H NI B Y T BCH TH LY NA Nhận xét số biến chứng thờng gặp kết điều trị bệnh nhân teo mật bẩm sinh sau phẫu thuật Kasai bệnh viện Nhi Trung Ương LUN VN THC S Y HC H NI - 2016 B GIO DC V O TO TRNG I HC Y H NI B Y T BCH TH LY NA Nhận xét số biến chứng thờng gặp kết điều trị bệnh nhân teo mật bẩm sinh sau phẫu thuật Kasai bệnh viện Nhi Trung Ương Chuyờn ngnh: Nhi khoa Mó s: 60720135 LUN VN THC S Y HC Hng dn khoa hc: TS Nguyn Phm Anh Hoa PGS.TS Trn Ngc Sn H NI - 2016 LI CM N hon thnh lun vn, tụi xin by t lũng bit n chõn thnh v sõu sc nht ti: TS Nguyn Phm Anh Hoa, PGS.TS Trn Ngc Sn, hai ngi thy ó trc tip hng dn, dỡu dt v nhit tỡnh ch bo cng nh to iu kin thun li cho tụi quỏ trỡnh hc v nghiờn cu khoa hc Tụi cng xin by t lũng bit n chõn thnh ti: - Cỏc thy cụ giỏo B mụn Nhi i hc Y H Ni - Ban giỏm hiu, Phũng o to sau i hc, Trng i hc Y H Ni - Ban Giỏm c, cỏc khoa phũng Bnh vin Nhi Trung ng ó to iu kin thun li cho tụi quỏ trỡnh hc v hon thnh lun Tụi cng by t lũng bit n ti: - Tp th khoa Gan mt bnh vin Nhi Trung ng ni tụi ang cụng tỏc, ó ng viờn v khớch l tinh thn cng nh to mi iu kin thun li cho tụi quỏ trỡnh hc v cụng tỏc - Tp th khoa iu tr t nguyn C Bnh vin Nhi Trung ng, Phũng K hoch tng hp Bnh vin Nhi Trung ng ó giỳp tụi cú c kt qu bỏo cỏo ca lun Cui cựng tụi xin by t lũng bit n ti ngi thõn gia ỡnh, bn bố ng nghip ó quan tõm ng viờn giỳp tụi v mi mt tụi hon thnh lun ny H Ni, thỏng 10 nm 2016 Bch Th Ly Na LI CAM OAN Tụi l Bch Th Ly Na hc viờn lpcao hc khúa 23 chuyờn ngnh nhi khoa, Trng i hc Y H Ni xin cam oan õy l Lun bn thõn tụi trc tip thc hin di s hng dn ca TS Nguyn Phm Anh Hoa, PGS.TS Trn Ngc Sn Cụng trỡnh ny khụng trựng lp vi bt k nghiờn cu no khỏc ó c cụng b ti Vit Nam Cỏc s liu v thụng tin nghiờn cu l hon ton chớnh xỏc, trung thc v khỏch quan, ó c xỏc nhn v chp nhn ca c s ni nghiờn cu Tụi xin hon ton chu trỏch nhim trc phỏp lut v nhng cam kt ny H Ni, thỏng 10 nm 2016 Ngi vit cam oan Bch Th Ly Na CH VIT TT TMBS : Teo mt bm sinh ALP : Alkaline phosphatase ALT : Ananine Aminotranferase AST : Asparto-aminotranferase Bilirubin GT : Bilirubin giỏn tip Bilirubin TP : Bilirubin ton phn Bilirubin TT : Bilirubin trc tip Ch s APRI : Aspartate aminotransferase to- platelet ratio index CMV : Cytomegalovirus GGT : Gamma-glutamyl transferase GPB : Gii phu bnh NTM : Nhim trựng ng mt PT : Prothrombin SDD : Suy dinh dng TATMC : Tng ỏp tnh mch ca TMTQ : Tnh mch thc qun TC Sign : Triangular cord sign PELD : Pediatric End- stage Liver Disease INR : International Normalized ratio MCT : Median Chain Tryglycerit PT : Phu thut T VN Teo mt bm sinh (TMBS) l bnh lý c trng bi quỏ trỡnh viờm, x húa tin trin, t phỏt, phỏ hy ton b ng mt v ngoi gan dn n s cn tr lu thụng ca mt [1],[2] Mc dự l mt bnh him gp vi tn sut mc bnh thp t 1/8000 1/18000 tr sinh sng nhng TMBS l nguyờn nhõn gõy mt ph bin nht thi k s sinh c bit vi tr em Chõu [3] Nguyờn nhõn gõy TMBS cha c bit rừ, mt s gi thuyt v c ch bnh sinh ó c a nh nhim virus (nh cytomegalovirus, reovirus), ri lon iu hũa dch, c ch t hay di truyn nhng tt c u cha thc s rừ rng [2] Mc dự phu thut Kasai l phng phỏp sa cha tm thi nhng õy c xem nh iu tr ti u i vi bnh nhõn TMBS giai on u Nu TMBS khụng c chn oỏn v phu thut Kasai 50 80% bnh nhõn s t vong vỡ x gan mt tui v t l ny tng 90 100% lỳc tui [4],[5], [6],[7] Vỡ vy vic chn oỏn v phu thut kp thi s ngn nga quỏ trỡnh x gan mt Tuy nhiờn mc thnh cụng sau phu thut Kasai ph thuc vo rt nhiu yu t nh tui phu thut, kinh nghim ca phu thut viờn hay v trớ tc nghn ca ng mt [7-9] Thm phu thut Kasai thnh cụng quỏ trỡnh viờm, x húa ng mt tin trin dn ti x gan mt v 70 80% bnh nhõn cn ghộp gan [7],[10] Vic theo dừi v iu tr bnh nhõn sau phu thut rt quan trng giỳp phỏt hin v iu tr sm cỏc bin chng nh nhim trựng ng mt, tng ỏp lc tnh mch ca, suy dinh dng, thiu ht cỏc vitamin tan du nhm nõng cao cht lng cuc sng cng nh kộo di thi gian sng khụng ghộp gan cho bnh nhõn Bnh TMBS Vit Nam cha cú thng kờ chớnh xỏc v tn sut mc bnh, nhiờn TMBS l mt nhng nguyờn nhõn thng gp nht tr nh b vng da mt kộo di Ti Bnh viờn Nhi Trung ng t nm 1994 ó tin hnh phu thut Kasai cho nhng bnh nhõn b TMBS, cng ó cú mt s nghiờn cu c tin hnh v bnh nhng hiu qu iu tr, cỏc din bin sau phu thutl nhng cũn cha c nghiờn cu y Xut phỏt t nhng trờn chỳng tụi tin hnh nghiờn cu ti: Nhn xột mt s bin chng thng gp v kt qu iu tr bnh nhõn teo mt bm sinh sau phu thut Kasai ti bnh vin Nhi Trung ng vi cỏc mc tiờu nghiờn cu: Mụ t mt s bin chng thng gp ca bnh nhõn TMBS sau phu thut Kasai ti Bnh vin Nhi Trung ng t 6/2010 6/2016 Nhn xột kt qu iu tr bnh nhõn TMBS sau phu thut Kasai CHNG TNG QUAN TI LIU 1.1 Tng quan v teo mt bm sinh 1.1.1 Khỏi nim v TMBS Teo mt bm sinh l bnh lý c trng bi tỡnh trng viờm tin trin, t phỏt ca h thng ng mt, lm tc nghn v phỏ hy ton b ng mt ngoi gan cựng vi s thay i kớch thc ca h thng ng mt gan, dn ti s x húa ng mt v tin trin thnh x gan [1], [2], [6], [7] 1.1.2 Lch s nghiờn cu: Nm 350 trc cụng nguyờn Arstotle ó mụ t mt trng hp khụng cú tỳi mt Nm 1817 tỏc gi John Burns ó mụ t ca bnh TMBS ti khoa phu thut i hc Glasgow Tỏc gi Charles West (1816 1892) Anh (Hospital for Sick Children at Great Ormond Street), ó mụ t mt ca bnh TMBS mt tr gỏi 13 tun tun tui vi biu hin vng da, phõn bc mu v nc tiu sm mu Nm 1892 John Thomson t Scotland mụ t 50 ca bnh TMBS v cho rng ng mt b hp s thiu phỏt trin v b phỏ hy thm bin mt ca ng mt quỏ trỡnh viờm tin trin ton b ng mt Nm 1916, tỏc gi Holmes ó chia TMBS thnh loi: cha c v khụng cha c Nm 1940 William Ladd ca i hc Harvars ó bỏo cỏo trng hp TMBS ó phu thut thc hin ni ng mt ch vi tỏ trng thuc type teo mt cú kh nng cha c bnh nhõn sng t 13 nm sau phu thut Nhng bnh nhõn cũn li c xp vo nhúm teo mt khụng cú kh nng cha l nhng trng hp khụng cú vt tớch ca cỏc qun mt ca ng mt cú th ni vi rut non [10],[11] Nm 1959, ln u tiờn tỏc gi Morio Kasai ó cụng b k thut mi ni rn gan vi hng trng trờn bnh nhõn TMBS ti Nht Bn, sau ú vo nm 1963 ti c v nm 1968 ti Anh Sau phu thut Kasai khong 70% bnh nhõn ht vng da nhng ch khong 20% sng quỏ nm, cỏc trung tõm khỏc s ny thm cũn thp hn v mc dự phu thut cú thnh cụng quỏ trỡnh x gan din t t gõy nờn tht vng ln hu ht cỏc bnh nhõn Mt s nh phu thut cho rng thm phu thut thnh cụng thỡ cng ch cú tỏc dng kộo di s chu ng ca bnh nhõn TMBS Phn ln bnh nhõn TMBS phu thut hay khụng phu thut s t vong [10] n nhng nm gia thp niờn 80, ghộp gan tr thnh mt phng thc iu tr mi cho bnh nhõn TMBS, s i ca cyclosporine mt loi thuc c ch dch ó kộo di thi gian sng sau ghộp cho bnh nhõn v Vin nghiờn cu quc gia Hoa K ó khng nh ghộp gan l liu phỏp iu tr thớch hp cho bnh nhõn suy gan giai on cui bao gm TMBS [10] T nm 1994 phu thut Kasai ó c tin hnh ti Bnh vin Nhi Trung ng 1.1.3 Dch t hc: TMBS l mt bnh him vi tn sut mc bnh trờn ton th gii t 1/8000 1/18000 [1],[2],[3] Theo bỏo cỏo tn sut mc bnh Chõu u v Bc M t 1/14000 1/20000 tr sinh sng [12],[17] Ti Nht Bn tn sut mc bnh xp x 1:9000 tr sinh sng [13] Tn sut mc bnh cao nht khong 1: 3000 tr sinh sng c ghi nhn ti French Polynesia [14] v i Loan [15] Cỏc lớ 10 gii thớch ti t l mc bnh cao ti Chõu v Tõy Thỏi Bỡnh Dng cha rừ rng V gii mt s nghiờn cu cho thy t l n mc bnh cao hn nam (54 69%) [16] mt nghiờn cu Thy in li cho t l mc bnh n thp hn nam (40%) [17] Mựa: Mt s nghiờn cu Chõu u cho thy cú s gia tng tn sut tr b TMBS sinh vo thỏng 11, thỏng Thy in [17] v thỏng Anh v x Wales [18] Mt bỏo cỏo t bang M cho thy tr th thai vo xuõn cú tn sut b bnh cao so vi tr c th thai vo ụng (OR 2.33, 95% CI 1.05 5.16) [19] Tng t ti Nht Bn tn sut mc bnh cao nht vo lnh t thỏng 12 n thỏng [13] 1.1.4 Tn thng gii phu bnh ca TMBS 1.1.4.1 i th - Gan: mc tn thng nh mu sc, mt kớch thc ph thuc vo tng giai on tin trin ca bnh Nu giai on sm gan mt nh, gan to nhng mt mm thỡ giai on mun gan mt nhiu hn biu hin gan mu xanh thm, chc va hoc cng chc, mt gan cú th nhn búng hoc g gh, cú cỏc u cc nh, gan x u inh, b mt cú nhiu mch mỏu tng sinh v bch mch gión phự n [3], [20] - ng mt ngoi gan: cú th teo mt phn hoc ton b Tỳi mt kớch thc bỡnh thng hoc nh, ụi ch cũn l di x hoc vt tớch ca tỳi mt [3] 1.1.4.2 Vi th: - Nhng tn thng c trng ca TMBS l s tng sinh cỏc ng mt tõn to xung quanh cỏc khong ca v xut hin cỏc cc mt c lm tc nghn ng mt Cỏc tn thng khỏc cú th gp nh: Cỏc t bo khng l, t 31 Silveira TR, Salzano FM, Donaldson PT, et al (1993) Association between HLA and extrahepatic biliary atresia J Pediatr Gastroenterol Nutr; 16:114-7 32 A Kader HH, El- Ayyouti M, Hawas S, et al (2002) HLA in Egyptian children with biliary atresia J Pediatr; 141: 431-3 33 Vasiliauskas E, Targan S, Cobb L, et al (1995) Biliary atresia an autoimmune disorder? Hepatology; 22(4 Pt 2): 87 34 Petersen C, Davenport M (2013) Aetiology of biliary atresia: what is actually known? Orphaner Journal of Rare Diseases, 8:128 35 Harper P, Plant JW, Unger DB (1990) Congenital biliary atresia and jaundice in lambs and calves Australian veterinary journal 67(1):18-22 36 Schwarz KB, Haber BH, Rosenthal P, et al (2013) Extrahepatic anomalies in infants with biliary atresia: results of a large prospective North American multicenter study Hepatology 2013;58:1724 37 Davenport M, Tizzard SA, Underhill J, et al (2006) The Biliary atresia splenic malformation syndrome: 28 year single center retrospective study J Pediatr; 149: 393 38 Schneider B (2006) A Multicenter study of the outcome of biliary atresia in the US 1997 2000 The Journal of Pediatrics;148:467-74 39 Hay Dm, Kimura K (1981) Biliary atresia New concepts of management Curr Probl Surg; 18: 541-608 40 Kotb MA, Sheba MF, et al (2001) Evaluation of the triangular cord sign in the diagnosis of biliary atresia Pediatrics 108(2):416-20 41 Tan Kendrick AP, Phua KB, Ooi BC, et al (2000) Making the diagnosis of biliary atresia using the triangular cord sign and gallbladder length Pediatric radiology 30(2):69-73 42 Nadel HR (1996) hepatobiliary scintigraphy in children Semin Nucl Med; 26:25-42 [Pubmed:8623049] 43 Lai MW, Chang MH, Hsu SC, et al (1994) Differential diagnosis of extrahepatic biliary atresia from neonatal hepatitis: a prospective study J pediatr Gastroenterol Nutr; 18:121-127 44 B.A Haber, P Russo (2003) Biliary atresia Gastroenterol Clin N Am, 32: 891- 911 45 Ohnuma N, Takahashi T, Tanabe M, et al (1997) The role of ERCP in biliary atresia Gastrointest Endosc, 45:365-370 [Pubmed:9165316] 46 Barbara E.Wildhaber (2012) Biliary Atresia: 50 Years after the First Kasai ISRN Surgery, Article ID 132089 47 Nio M, Ohi R, Miyano T, et al (2003) Five- and 10-year survival rates after surgery for biliary atresia: a report from the Japanese biliary atresia registry J Ped Surg;38:997-1000 48 Barbara E Wildhaber The Kasai Portoenterostomy for Biliary Atresia: A Review of a 27-Year Experience With 81 Patients J Pediatr Surg 38:1480-1485 49 Rui Dong, et al (2013) Improved outcome of biliary atresia with postoperative high dose steroid Gastroenterology Research and Practice Article ID 902431 50 Benjamin L, et al (2012) Efficacy of Fat Soluble Vitamin Supplementation in infants with biliary atresia Pediatr Vol 130(3) 51 Mary Elizabeth M Tessier, et al (2014) Beyound the Pediatric end stage liver disease system: Solutions for infants with biliary atresia requiring liver transplant World Journal of Gastroenterology, 20(32): 11062-11068 52 Luo Y, Zheng S (2008) Current concept about postoperative cholangitis in biliary atresia World J Pediatr; 4:14 53 Miga D, Sokol RJ, Mackenzie T, et al (2001) Survival after first esophageal varices hemorrhage in patients with biliary atresia J Pediatr; 139:291-6 54 Ozsoylu S, Kocak N, Demir H, et al (2000) Propranolon for primary and secondary prophylasix of variceal bleeding in children with cirrhosis Turk J Pediatr; 42:31-3 55 Zhang HY, Han DW, Wang XG, et al (2005) Experimental study on the role of endotoxin in the development of hepatopulmonary syndrome World J Gastroenterol, 11: 567-72 56 Vincent Ho (2008) Current concepts in the management of hepatopulmonary syndrome Vascular Health and Risk Management, 4(5) 1035-1041 57 Rowen K Zetterman MD (2013) Pulmonary Complicatons of cirrhosis Medscape, vol 56 58 M Watanabe, T Hori, M Kaneko et al (2007) Intrahepatic biliary cysts in children with biliary atresia who have had a Kasai operation Journal of Pediatric Surgery, vol 42, no 7, pp.11851189 59 Bergasa NV (2011) The itch of liver disease Semin Cutan Med Surg.; 30(2):93-8 60 Davenport M (2011) Biliary atresia in England and Wales: results of centralization and new benchmark Journal of Pediatrics surgery; 46, 16891694 61 Davenport M, Ville de Goyet J, Stringer MD, et al (2004) Seamless management of biliary atresia in England and Wales (1999-2002) Lancet; 363:1354-1357 62 Serinet MO, P Broue (2006) Management of patients with biliary atresia in France: results of a decentralized policy 19862002 Hepatology, vol 44, no 1,pp 7584 63 B E Wildhaber, P Majno, et al (2008), Biliary atresia: Swiss national study, 19942004 Journal of Pediatric Gastroenterology and Nutrition, vol 46, no 3, pp 299307 64 HS Lai.(2006) Long term prognosis and factors affecting biliary atresia from experience over a 25 year period Chang Gung Med J ;29: 234-9 65 Trn Ngc Sn (2012) Kt qu phu thut Kasai iu tr teo mt tr em: kinh nghim mt trung tõm vi 139 trng hp Y hc Vit Nam 2012;399:77-81 66 Hong Th Xuyn (2013) Nghiờn cu c im lõm sng, cn lõm sng, phõn loi v t l nhim Cytomegalovirus bnh nhõn teo ng mt bm sinh Lun tt nghip thc s Y khoa i hc Y H Ni 67 Ohi R (2004) Biliary atresia A surgical perspective Clin Liver Dis; 4:779-804 68 Grieve A, Makin E, Davenport M (2013) Aspartart Aminotransferase to platelet ratio index in infants with biliary atresia: prognostic value at presentation J Pediatr Surg; 48:789-795 [PIMD:23583135] 69 Nguyn Cụng Khanh (2004) Huyt hc lõm sng nhi khoa Nh xut bn Y hc 70 NXB Y hc (2015) Bi ging Nhi khoa sỏch o to sau i hc Hi chng tang ỏp lc tnh mch ca tr em 379 -389 71 Way Seah Lee (2010) Clinical features differentiating biliary atresia from other causes of neonatal cholestasis Ann Acad Med Singapore; 39:648-54 72 Schwarz KB, Haber BH, Rosenthal P, et al (2013) Childhood Liver Disease Research and Education Network Extrahepatic anomalies in infants with biliary atresia: results of a large prospective North American multicenter study Hepatology;58(5): 17241731 73 Lien TH, Chang MH, Wu JF, et al (2011) Taiwan Infant Stool Color Card Study Group Effects of the infant stool color card screening program on 5-year outcome of biliary atresia in Taiwan Hepatology; 53(1): 202208 74 Tseng JJ, Lai MS Stool color card screening for biliary atresia Pediatrics 2011;128(5):209 -215 75 Liu CS, Chin TW, Wei CF (1998) Value of gamma-glutamyl transpeptidase for early diagnosis of biliary atresia Zhonghua yi xue za zhi = Chinese medical journal; Free China ed 61(12):716-20 76 Tang KS1, Huang LT (2007) Gamma-glutamyl transferase in the diagnosis of biliary atresia Acta Paediatr Taiwan; 48(4):196-200 77 Humphrey TM, Stringer MD (2007) Biliary atresia: US diagnosis Radiology 244(3): 845-51 78 Farrant P, Meire HB, Mieli-Vergani G (2001) Improved diagnosis of extrahepatic biliary atresia by high frequency ultrasound of the gallbladder Br J Radiol 74:952954 79 Kanegawa K, et al (2003) Sonographic Diagnosis of Biliary Atresia in Pediatric Patients Using the Triangular Cord Sign Versus Gallbladder Length and Contraction American Journal of Roentgenology.181: 1387-1390 80 Escobar MA (2006) Effect of corticosteroid therapy on outcomes in biliary atresia after Kasai portoenterostomy Journal of Pediatric Surgery 41(1): 99-103 81 Wu.ET, et al (2001) Bacterial cholangitis in patients with biliary atresia: impact on short-term outcome Pediatric Surgery International July ;17(5): 390395 82 Lunzmann K (1999) The influence of cholangitis on the prognosis of extrahepatic biliary atresia Eur J Pediatr Surg 9: 19 -23 83 M Duchộ, B Ducot, E Tournay et al (2010) Prognostic value of endoscopy in children with biliary atresia at risk for early development of varices and bleeding, Gastroenterology, vol 139, no 6, pp 1952 1960 84 EJ Bijl et al The long-term outcome of the Kasai operation in patients with biliary atresia: a systematic review The Netherland Journal of Medicine, vol 71, no 4: pp 170 -173 85 Shneider BL (2012) Portal hypertension in children and young adults with biliary atresia J Pediatr Gastroenterol Nutr Nov;55(5):567-73 86 Tsuchida Y, Honna T, Kawarasaki H (1994) Cystic dilatation of the intrahepatic biliary system in biliary atresia after hepatic portoenterostomy J Pediatr Surg.29: 630-634 87 McKiernan PJ, Baker AJ, Kelly DA (2000) The frequency and outcome of biliary atresia in the UK and Ireland Lancet, 355: 25-29 88 Serinet MO (2009) Impact of age at Kasai operation on its results in late childhood and adolescence: a rational basis for biliary atresia screening Pediatrics.123(5):1280-6 89 Altman RP, Lilly JR, Greenfeld J, et al (1997) A multivariable risk factor analysis of the portoenterostomy (Kasai) procedure for biliary atresia: Twenty-five years of experience from two centers Ann Surg.226:348-353; discussion 353-345 90 Yanchar NL, Shapiro AM, Sigalet DL (1996) Is early response to portoenterostomy predictive of long-term outcome for patients with biliary atresia J Pediatr Surg, 31:774-778 91 Davenport M, Kerkar N, Mieli-Vergani G, et al (1997) Biliary atresia: The Kings College Hospital experience (1974-1995) J Pediatr Surg, 32:479-485 92 Schoen BT, Lee H, Sullivan K, et al (2001) The Kasai portoenterostomy: When is it too late J Pediatr Surg, 36: 97-99 93 Bu LN, Chen HL, Chang CJ, et al (2003) Prophylactic oral antibiotics in prevention of recurrent cholangitis after the Kasai portoenterostomy J Ped Surg; 38: 590-593 94 van Heurn LW, Saing H, Tam PK (2003) Cholangitis after hepatic portoenterostomy for biliary atresia: a multivariate analysis of risk factors J Pediatr.142:566-571 95 Yang LY, Fu J, Peng SY, et al (2015) Validation of aspartarrt aminotransferase to platelet ratio for diagnosis of liver fibrosis and prediction of portoperative prognosis in infants with biliary atresia World J Gastroenterol; 21(19): 5893-5900 96 Lykavieris P, Charot C et at (2005) Outcome in adulthhood of biliary atresia: A study of 63 patients who survived for over 20 years with their native liver Hepatology; 41: 366 -371 MC LC T VN CHNG TNG QUAN TI LIU 1.1 Tng quan v teo mt bm sinh .8 1.1.1 Khỏi nim v TMBS 1.1.2 Lch s nghiờn cu: 1.1.3 Dch t hc: 1.1.4 Tn thng gii phu bnh ca TMBS 10 1.1.5 C ch bnh nguyờn bnh sinh: 11 1.1.6 Phõn loi teo mõt bm sinh: 16 1.2 Triu chng lõm sng, cn lõm sng: .17 1.2.1 Triu chng lõm sng: 17 1.2.2 Cn lõm sng: 19 1.2.3 Chn oỏn phõn bit .23 1.3 iu tr TMBS 23 1.3.1 Phu thut: 23 1.3.2 iu tr h tr: 24 1.3.3 Ghộp gan 26 1.3.4 Cỏc bin chng sau phu thut Kasai .27 1.3.5 Kt qu iu tr teo mt bm sinh: 32 1.3.6 Cỏc yu t tiờn lng sau phu thut: 33 CHNG 37 I TNG V PHNG PHP NGHIấN CU .37 2.1 i tng nghiờn cu: 37 2.1.1 Tiờu chun la chn bnh nhõn: .37 2.1.2 Tiờu chun loi tr: 37 2.2 Thi gian v a im nghiờn cu: 37 2.3 Phng phỏp nghiờn cu: 37 2.3.1 Thit k nghiờn cu: 37 2.3.2 C mu v chn mu: 38 2.3.3 Ni dung nghiờn cu .38 2.3.4 Cỏc bin s, ch s nghiờn cu: .39 2.4 Qun lý v phõn tớch s liu 45 2.5 o c nghiờn cu 46 CHNG 47 KT QU NGHIấN CU 47 3.1 c im chung ca nhúm nghiờn cu 47 3.1.1 c im nhõn khu hc 47 3.1.2 c im lõm sng, cn lõm sng ca nhúm nghiờn cu: .48 Cỏc du hiu, ch s 48 T l % 48 Trung bỡnh 48 Min max 48 Vng da .48 100% 48 Thay i mu sc phõn (vng nht hoc bc mu) 48 100% 48 Bil TP 48 179,353,7 48 74 392 48 AST 48 252,3 170,7 48 49,9 1128 48 ALT 48 150,7 106,2 48 20 600,3 48 GGT .48 658,6 508,04 48 43,6 2812,8 48 3.2 Mt s bin chng ca bnh nhõn TMBS sau phu thut Kasai 49 3.2.1 Bin chng sm sau m 49 3.2.2 Cỏc bin chng mun sau m 50 3.2.3 Mt s yu t liờn quan n cỏc bin chng mun sau phu thut: 53 3.3 Mc tiờu 2: Nhn xột kt qu iu tr bnh nhõn teo mt bm sinh sau phu thut Kasai 55 Trong nghiờn cu ca chỳng tụi cú 228 bnh nhõn iu kin tham gia nghiờn cu nhng quỏ trỡnh theo dừi cú bnh nhõn b theo dừi Tớnh n thi im nghiờn cu cú 60,2% bnh nhõn cũn sng nhúm nghiờn cu T l t vong thi im nghiờn cu l 39,8% 55 Trung v thi gian sng (Median survival time) chung hai nhúm l 13 thỏng nhúm t vong, thi gian sng t n 46 thỏng, trung v l thỏng Vi nhúm cũn sng, thi gian theo dừi t n 72 thỏng, trung v l 28 thỏng 55 3.3.1 T l ht vng da sau phu thut (Phu thut Kasai thnh cụng) 55 55 3.3.2 Kt qu phu thut v mt s yu t liờn quan: 55 3.3.3 T l sng sau phu thut: .57 3.3.4 Mi liờn quan gia ch s PELD v xỏc sut sng sau PT 64 CHNG 68 BN LUN 68 4.1 c im chung ca nhúm nghiờn cu 68 4.1.1 Gii 68 4.1.2 Tui phu thut: .68 4.2 c im lõm sng, cn lõm sng ca nhúm nghiờn cu: .69 4.3 Cỏc bin chng thng gp sau phu thut Kasai: .71 4.3.1 Cỏc bin chng sm: .72 4.3.2 Cỏc bin chng thng gp sau phu thut: 72 4.3.3 Mt s yu t liờn quan n cỏc bin chng sau phu thut: .76 4.4 Kt qu phu thut v cỏc yu t liờn quan: 77 KT LUN 85 KIN NGH 86 TI LIU THAM KHO 87 PH LC DANH MC BNG * Siờu õm bng 19 Bng 1.1: Kớch thc tỳi mt bỡnh thng 19 Bng 3.1 c im chung ca i tng nghiờn cu 47 Bng 3.2: Mt s ch s lõm sng v cn lõm sng ca nhúm NC .48 Bng 3.3 Cỏc du hiu trờn siờu õm gi ý teo mt bm sinh: 49 Bng 3.4: T l cỏc bin chng sm gp sau m 50 Cỏc bin chng sm 50 S bnh nhõn (n) 50 T l % 50 Nhim trựng ng mt sm sau m 50 29/228 50 12,7% 50 Xut huyt tiờu húa sm 50 9/228 50 3,9% 50 Suy gan, c chng .50 10/228 50 4,4% 50 Ri lon in gii 50 7/228 50 3,1% 50 51 Bng 3.5 Liờn quan gia tui phu thut (ngy) vi cỏc bin chng 53 Bng 3.6 Liờn quan gia nng bilirubin ton phn vi cỏc bin chng sau phu thut: 53 Bng 3.7 Thi gian theo dừi cỏc nhúm .55 Bng 3.8 ỏnh giỏ kt qu phu thut theo nhúm tui phu thut .56 Bng 3.9: Mi liờn quan gia ch s bilirubin ton phn trc PT vi kt qu PT 56 Bng 3.10 Liờn quan gia cỏc bin chng sau PT v t l thnh cụng sau PT .56 Bng 4.1: Kt qu ca phu thut Kasai theo mt s trung tõm 80 khỏc trờn th gii 80 DANH MC BIU Biu 3.1 Phõn b cỏc nhúm tui ti thi im phu thut 48 Biu 3.2 T l gp bin chng sm sau m 50 Nhn xột: 50 a s bnh nhõn (85,5%) khụng cú bin chng sm sau m T l bnh nhõn cú bin chng sau m ch chim 14,5% 50 Biu 3.3 Cỏc bin chng mun thng gp sau m teo mt bm sinh 50 Biu 3.4 S t nhim trựng ng mt sau phu thut 51 Biu 3.5: Phõn b tn sut NTM theo thi gian 52 Biu 3.6 ỏnh giỏ kt qu phu thut teo mt bm sinh 55 Biu 3.7 T l sng sau phu thut theo thi gian .58 Biu 3.8 T l sng sau phu thut theo thi gian hai nhúm thnh cụng v tht bi sau phu thut 58 Biu T l sng sút sau phu thut theo thi gian gia cỏc nhúm tui phu thut 59 Biu 10 T l sng sau phu thut gia cỏc nhúm Bilirubin ton phn trc phu thut 60 Biu 3.11 T l sng sau phu thut theo cỏc nhúm bin chng nhim trựng ng mt .61 Biu 12 T l sng sau phu thut theo cỏc nhúm bin chng x gan suy chc nng gan sau phu thut 62 Biu 3.13 T l sng sút sau phu thut theo thi gian cỏc nhúm bin chng TATMC sau phu thut 63 Biu 3.14: ng cong ROC, mi liờn quan ch s PELD v xỏc sut sng sau phu thut .64 Biu 3.15 T l sng sút sau phu thut theo thi gian gia cỏc nhúm im PELD thỏng sau phu thut 65 Biu 3.16: ng cong ROC, mi liờn quan gia ch s APRI .66 v xỏc sut t vong sau phu thut 66 Biu 17 T l sng sút sau phu thut theo thi gian 67 DANH MC HèNH Hỡnh 1.1: Phõn loi cỏc type teo mt bm sinh [15] 17 Hỡnh 1.2: Mu phõn bt thng (1-3) v mu phõn bỡnh thng (4-7) Ngun Pediatrics, Vol 117(4) Trang 1148 18 Hỡnh 1.3: Di x rn gan 20 (Ngun: Nelson Textbook of Pediatrics Edition 20) 20 ... HC Y H NI B Y T BCH TH LY NA Nhận xét số biến chứng thờng gặp kết điều trị bệnh nhân teo mật bẩm sinh sau phẫu thuật Kasai bệnh viện Nhi Trung Ương Chuyờn ngnh: Nhi khoa Mó s: 60720135 LUN VN... Nhi Trung ng vi cỏc mc tiờu nghiờn cu: Mụ t mt s bin chng thng gp ca bnh nhõn TMBS sau phu thut Kasai ti Bnh vin Nhi Trung ng t 6/2010 6/2016 Nhn xột kt qu iu tr bnh nhõn TMBS sau phu thut Kasai. .. [36] 1.1.6 Phõn loi teo mõt bm sinh: Cú rt nhiu cỏch phõn loi teo mt bm sinh c s dng nh phõn loi theo lõm sng, theo gii phu bnh 1.1.6.1 Phõn loi theo lõm sng: - Teo mt bm sinh khụng kốm cỏc d