Biliary atresia Registry for 10 years ( )

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1 Biliary atresia Registry for 10 years (2001-2010)
Primary Kasai operation (435명)

2 Demographic result Sex distribution Gestational age and birth weight
M:F:1:1.46 (177명:258명) Gestational age and birth weight Mean : 38.8±1.7주 Mean : 3.08±0.47kg

3 수술일 기준 연도별 환자수 Year N Percent missing 6 1.4 2001 42 9.7 2002 31 7.1
2003 46 10.6 2004 52 12.0 2005 39 9.0 2006 45 10.3 2007 36 8.3 2008 44 10.1 2009 51 11.7 2010 43 9.9 Total 435 100.0 Mean 43명

4 병원별 Case no. Frequency 전원(2nd surgeon보고) 1차 수술병원 Total 경북대 4 계명대 36
계명대 36 부산대학 12 삼성서울 64 18 46 서울대학 1 서울성모 2 6 서울아산 80 5 75 아주대병 7 여의도성 10 11 연세의대 137 8 138 영남대학 3 이대목동 전남대병 충남대 단국대 인하대 원광대 고신대 중국 Missing 435 31

5 병원별 환자수 Mean 23.8명

6 입원 당시 결과 Age 증상 Mean age : 60.1±31.4days(1-231), median : 58days
- 기타 : Fever(5), 산전검사에서 choledochal cyst 발견되어 내원(4). vomiting(2), prematurity, 산전 진단에서 물혹 발견, failure to thrive, Ileal atresia, Inguinal hernia, splenomegaly, skin infection Jaundice Acholic stool Hepato-megaly Abdominal distension 기타 있음 (n) 381 251 31 26 20 Percent (%) 94.3 62.1 7.7 6.4 5.0 (N : 404)

7 입원시 laboratory finding
Mean ± SD (Min-Max) Totalbil 396 10.1 ±3.6 ( ) Directbil 380 6.7±2.4( ) AST 394 246.8±204.9( ) ALT 165.7±131.0(7-1110) GGT 342 553.6±521.9( ) (N : 404)

8 Diagnostic tools hospital Valid USG DISIDA CT MRCP Liver biopsy
Fibroscan others 연세의대 Count 130 128 95 4 118 6 58 1 % within hospital 98.5% 73.1% 3.1% 90.8% 4.6% 44.6% 0.8% 서울아산 75 73 69 8 49 97.3% 92.0% 10.7% 5.3% 65.3% .0% 1.3% 서울대학 63 27 3 9 10 92.1% 42.9% 4.8% 14.3% 15.9% 삼성서울 46 45 20 22 2 97.8% 43.5% 6.5% 47.8% 4.3% 2.2% 계명대 36 16 35 100.0% 44.4% 0% 16.7% 97.2% 5.6% 그외병원 54 51 48 23 15 94.4% 88.9% 3.7% 42.6% 27.8% Total 404 391 275 182 110 18 96.8% 68.1 4.9% 45.0% 27.1% 14.4% 4.5%

9 Ultrasonogram hospital Valid USG 연세의대 Count 130 128 % within hospital
98.5% 서울아산 75 73 97.3% 서울대학 63 58 92.1% 삼성서울 46 45 97.8% 계명대 36 100.0% 그외병원 54 51 94.4% Total 404 391 96.8%

10 DISIDA hospital Valid DISIDA 연세의대 Count 130 95 % within hospital 73.1%
서울아산 75 69 92.0% 서울대학 63 27 42.9% 삼성서울 46 20 43.5% 계명대 36 16 44.4% 그외병원 54 48 88.9% Total 404 275 68.1

11 CT hospital Valid CT 연세의대 Count 130 4 % within hospital 3.1% 서울아산 75 8
10.7% 서울대학 63 3 4.8% 삼성서울 46 6.5% 계명대 36 0% 그외병원 54 2 3.7% Total 404 20 4.9%

12 MRCP hospital Valid MRCP 연세의대 Count 130 118 % within hospital 90.8%
서울아산 75 4 5.3% 서울대학 63 9 14.3% 삼성서울 46 22 47.8% 계명대 36 6 16.7% 그외병원 54 23 42.6% Total 404 182 45.0%

13 Liver biopsy hospital Valid Liver biopsy 연세의대 Count 130 6
% within hospital 4.6% 서울아산 75 49 65.3% 서울대학 63 3 4.8% 삼성서울 46 2 4.3% 계명대 36 35 97.2% 그외병원 54 15 27.8% Total 404 110 27.1%

14 Fibroscan hospital Valid Fibroscan 연세의대 Count 130 58 % within hospital
44.6% 서울아산 75 .0% 서울대학 63 삼성서울 46 계명대 36 그외병원 54 Total 404 14.4%

15 Other studies hospital Valid Other studies 연세의대 Count 130 1
기타 : duodenal intubation/analysis(10), 그 외 복강경(3) hospital Valid Other studies 연세의대 Count 130 1 % within hospital 0.8% 서울아산 75 1.3% 서울대학 63 10 15.9% 삼성서울 46 2.2% 계명대 36 2 5.6% 그외병원 54 3 Total 404 18 4.5%

16 Kasai 수술 N : 404 수술 시 나이 Mean : 66.2±28.7days (10-235) Median : 64days

17 Op cholangiogram hospital valid Op cholangiogram 연세의대 Count 130 92
70.8% 아산병원 75 23 30.7% 서울대학교 61 9 14.8% 삼성서울병원 41 21 51.2% 계명대 동산병원 36 100.0% 그외 병원 54 29 53.7% Total 397 210 52.9%

18 Extrahepatic bile duct occlusion type
Type I 7명 : 2.0% II 29명 : 8.3% III 314명 : 89.7%

19 Liver fibrosis stage N : 321 Stage I (minimal fibrosis) 89/321 : 27.7%
II (moderate fibrosis) 135/321 : 42.1% III (severe fibrosis/cirrhosis) 97/321 : 30.2%

20 Portal fibrotic tissue pathology
N : 319 Maximal size of microscopic Duct : Absent duct : 73/319 : 22.9% <150µm : 133/319 : 41.7% >150µm : 113/319 : 35.4%

21 Type of reconstruction
Roux-en-Y portojejunostomy : 401/402 : 99.8% Others : 1/402 : 0.2%  Roun-en-Y hepaticojejunostomy(type I, 아산)

22 Intraoperative complication
없음:373/378 : 98.7% 있음 : 5/378 : 1.3% Postop bleeding(서울대2명) Hepatic a. injury로 repair(서울대1명, 세브란스 최승훈 1명) 아주대1명 – 이유 안적힘.

23 동반기형 종류 N % (n:404) % (n:435) Gastrointestinal 24 5.9% 5.5%
malrotation 7 1.7% 1.6% 기타 17 4.2% 3.9% Vascular 6 1.5% 1.4% Preduodenal vein 2 0.5% 4 1.0% 0.9% Splenic polysplenia Cardiac 28 6.9% 6.3% ASD 15 3.7% 3.4% VSD PDA(op needed) 3 0.7% 1.3% Genitourinary 20 5.0% 4.6% cystic kidney 1 0.2% 19 4.7% 4.4% 그외 동반기형 25 6.2% 6.7% 전체 66 16.3% 15.2%

24 Antibiotics Treatment
IVantitype Total cefa only cefa + AG cefa + AG + MN 기타 연세의대 3 14 110 130 2.3% 10.8% 84.6% 100.0% 아산병원 39 34 2 75 52.0% 45.3% .0% 2.7% 서울대학교 9 43 4 59 5.1% 15.3% 72.9% 6.8% 삼성서울병원 35 8 81.4% 18.6% 계명대 동산병원 36 그외 병원 6 21 13 54 11.1% 38.9% 24.1% 25.9% 51 149 174 23 397 12.8% 37.5% 43.8% 5.8%

25 Antibiotics duration N Mean Median 전체 397 28.0±17.4 (4-91) 24 연세의대 130
32.0±11.2 (9-91) 29 아산병원 75 23.7±16.1 (5-90) 18 서울대학교 60 54.1±10.7 (13-75) 56 삼성서울병원 43 8.5±3.3 (4-22) 8 계명대 동산병원 36 21.0±0 (21-21) 21 그외병원 53 15.1±11.0 (6-74) 13

26 Oral antibiotics use (N:404) USE Percent Antibiotics 1위 2위 3위 전체 use
303 75.0% 116 (38.2%) Bactrim 73 (24.0%) Sulfamet 69(22.7%) Septrin 연세의대 44 33.8% 20 (14.5%) 8 (5.8%) Cefaclor 4 (2.9%) Klaricid 아산병원 73 97.3% 40 (54.8%) 32 (43.8%) 1(1.4%) Augmentin 서울대학교 56 88.8% 35 (62.5%) 21 (37.5) - 삼성서울병원 43 93.5% 40 ( 93.0%) 1 (2.3%) amoxicillin 계명대 동산병원 36 100.0% 36 (100.0% 그외병원 51 94.4% 17 (32.7%) 12 (23.1%) Slfamet 5 (9.6%) bactrim (N:404)

27 Oral antibiotics use

28 Duration of oral antibiotics use
Mean Median 전체 298 134.7±155.3 (1-730) 연세의대 44 11.3±8.8 (2-35) 아산병원 74 151.6±147.8 (5-730) 서울대학교 55 208.6±143.8 (30-665) 삼성서울병원 42 7.8±2.7 (1-15) 계명대 동산병원 36 365.0±0 ( ) 그외병원 47 71.4±121.2 (2-365)

29 Steroid use (N:404) TRUE Percent 전체 use 305 75.5% 연세의대 81 62.3% 아산병원
67 89.3% 서울대학교 43 68.3% 삼성서울병원 41 93.5% 계명대 동산병원 36 100% 그외병원 37 68.5% (N:404)

30 Steroid use start day N Mean Median 전체 309 7.6±8.4 (0-124) 연세의대 83
12.1±14.8 아산병원 69 4.4±3.7 (0-20) 서울대학교 42 6.5±2.2 (2-18) 삼성서울병원 43 6.9±1.1 (2-9) 계명대 동산병원 36 7.0±0 (7-7) 그외병원 6.1±3.1 (1-16)

31 Steroid use duration N Mean Median 전체 302 29.0±26.3 (3-97) 연세의대 83
25.9±18.5 아산병원 69 20.8±6.0 (8-49) 서울대학교 40 19.8±4.8 (6-31) 삼성서울병원 43 8.4±1.7 (5-13) 계명대 동산병원 36 90.0±0 (90-90) 그외병원 31 25.3±22.6 (7-90)

32 UDCA 사용 TRUE Percent 전체 use 383 94.8% 연세의대 127 97.7% 아산병원 72 96.6%
서울대학교 58 92.1% 삼성서울병원 42 91.3% 계명대 동산병원 36 100% 그외병원 48 88.9%

33 Postoperative minimal bilirubin before discharge
Mean Median 전체 387 4.8±2.8 ( ) 연세의대 127 4.8±2.7 ( ) 아산병원 75 4.7±2.1 ( ) 서울대학교 52 4.1±3.0 ( ) 삼성서울병원 45 5.0±3.0 ( ) 계명대 동산병원 36 4.2±3.4 ( ) 그외병원 53 6.3±3.0 ( ) When, POD mean 15.8 ± 8.4 day (0-31)

34 Postoperative minimal bilirubin before discharge

35 Jaundice free rate (bilirubin<2)
TRUE Percent 전체 use 263 65.1% 연세의대 103 79.2% 아산병원 53 70.7% 서울대학교 32 50.8% 삼성서울병원 22 47.8% 계명대 동산병원 28 77.8% 그외병원 25 46.3% (N : 404) When, POD, Mean 17.1 ±8.9 day (1-31)

36 Postoperative status 435 kasai op. 363 DC (31)2nd 36 LT 5 death
(53)loss 207(7) alive (24)LT (5) death (1)loss s c death (54) loss alive death

37 Discharge date (POD) N Mean 전체 395 32.3±26.1 (8-415) 연세의대 130
32.9±16.2 (8-173) 아산병원 75 28.0±15.6 (12-69) 서울대학교 58 60.4±48.0 (12-415) 삼성서울병원 45 18.6±17.2 (12-128) 계명대 동산병원 36 25.1±7.0 (16-49) 그외병원 51 22.1±11.6 (9-71)

38 Discharge medication No. Percent Oral antibiotics 292 72.3%
Oral steroid 165 40.8% UDCA 376 93.1% Other medication 202 50.0% (N:404)  Others : Phenobarbital 78명(전체 중 19.2%)로 m/c Tocopherol (Vt E)70명 (전체 17.2%) Albityl 41명 (10.1%) Multivitamin 종류(3), 그외 calico(4), lasix(3), 등…

39 Readmission Rate : 269명/435명 (61.8%) Mean no. : 2.7 ± 2.7 회(0-21)
Cause of readmission  Others : Splenomegaly, liver failure, intrahepatic biliary cyst… Cause No. Percent Cholangitis 215 49.4% Varices 55 12.6% Ascites 31 7.1% Others 43 9.9% (N : 435)

40 Late complication 종류 No. Percent None 91 20.9%
Cholangitis episode during follow up 171 39.3% Nutritional problem 6 1.4% Ascites 29 6.7% Growth retardation 4 0.9% Varices bleeding 26 5.9% Liver cirrhosis/failure 9 2.1% Others 14 3.2% (N : 435)  Others : Hyperbilirubinemia 4(0.9%), Hepatopulmonary syndrome 3(0.7%), Splenomegaly, pancytopenia, intestinal obstruction, Delayed bile secretion, pancreatic pseudocyst

41 Reoperation Rate : 14명/435명 : 3.2% Reoperation cause 종류 No. Percent
Redo-operation (Failed Kasai op.) 5 1.1% Anastomosis leakage 3 0.6% Intestinal obstruction Postoperative bleeding 1 0.2% Liver biopsy/splenectomy Biliary cyst (N : 435)

42 Last follow up Mean POD : 3.4±2.8 years(0-10.0)
Mean total bilirubin : 2.7±5.2( )

43 Last f/u total bilirubin
Mean Median 전체 378 2.7±5.2 ( ) 연세의대 124 (0.2-32) 아산병원 74 1.8±3.5 ( ) 서울대학교 55 3.5±6.3 ( ) 삼성서울병원 47 2.5±6.6 계명대 동산병원 36 2.9±5.0 ( ) 그외병원 42 3.0±4.4 ( ) When, mean POD 3.4±2.8 years(0-10.7)

44 Last f/u total bilirubin

45 Liver transplantation
LT rate : 157명/435 : 36.1% Interval from Kasai op. : 1.1±1.3 year(0-7.1) LT 후 사망 11명/157 : 7.0% LT 후 사망일 : Mean 123.2day(median 114일) Cause of LT  others :intrahepatic cyst, liver failure(7), HPS(2), melena/hematochezia(5), biliary cirrhosis(4). Hepatosplenomegaly(4), abdominal distension(3), hypoalbuminemia, liver cirrhosis(4), hepatic richets(4), viral infection(1), anuria(1), acholic stool, thrombocytopenia Frequency Percent Hypehypehypehyp 130 29.9% Uncontrolled ascites 31 7.1% Growth retardation 5 1.1% Coagulation defect varices 44 10.1% Recurrent cholangitis 60 13.8% 기타 33 7.6% (N : 435)

46 Overall survival (전체 ) Total N N of Events Censored N Percent 422 29
N Percent 422 29 393 93.1%

47 Overall survival (두 그룹 )
hospitalgroup Total N N of Events Censored N Percent .00 352 21 331 94.0% 1.00 69 8 61 88.4% Overall 421 29 392 93.1% Chi-Square df Sig. Log Rank (Mantel-Cox) 3.208 1 .073

48 Overall survival (6그룹) Chi-Square df Sig. Log Rank (Mantel-Cox) 9.289
5 .098 여섯group Total N N of Events Censored N Percent 1.00 135 9 126 93.3% 2.00 74 2 72 97.3% 3.00 64 4 60 93.8% 4.00 44 1 43 97.7% 5.00 35 5 30 85.7% 6.00 69 8 61 88.4% Overall 421 29 392 93.1%

49 Native liver survival (전체 )
Total N N of Events Censored N Percent 423 170 253 59.8

50 Native liver survival (두 그룹 )
hospitalgroup Total N N of Events Censored N Percent .00 353 138 215 60.9% 1.00 69 32 37 53.6% Overall 422 170 252 59.7% Chi-Square df Sig. Log Rank (Mantel-Cox) .737 1 .391

51 Native liver survival (6그룹 )
Chi-Square df Sig. Log Rank (Mantel-Cox) 37.958 5 .000 여섯group Total N N of Events Censored N Percent 1.00 136 41 95 69.9% 2.00 74 26 48 64.9% 3.00 64 23 35.9% 4.00 44 21 47.7% 5.00 35 7 28 80.0% 6.00 69 32 37 53.6% Overall 422 170 252 59.7%

52 Native liver survival & bil<2 (전체 )
Total N N of Events Censored N Percent 422 204 218 51.7%

53 Native liver survival & bil<2 (두 그룹)
hospitalgroup Total N N of Events Censored N Percent .00 353 163 190 53.8% 1.00 68 41 27 39.7% Overall 421 204 217 51.5% Chi-Square df Sig. Log Rank (Mantel-Cox) 2.072 1 .150

54 Native liver survival & bil<2 (6그룹 )
Chi-Square df Sig. Log Rank (Mantel-Cox) 45.598 5 .000 여섯group Total N N of Events Censored N Percent 1.00 136 48 88 64.7% 2.00 74 29 45 60.8% 3.00 64 47 17 26.6% 4.00 44 15 34.1% 5.00 35 10 25 71.4% 6.00 68 41 27 39.7% Overall 421 204 217 51.5%