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Biliary atresia Registry for 10 years (2001-2010)

Biliary atresia Registry for 10 years (2001-2010). Primary Kasai operation (435 명 ). Demographic result. Sex distribution M:F:1:1.46 (177 명 :258 명 ) Gestational age and birth weight Mean : 38.8 ± 1.7 주 Mean : 3.08 ± 0.47kg. 수술일 기준 연도별 환자수. Mean 43 명. 병원별 Case no. 병원별 환자수.

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Biliary atresia Registry for 10 years (2001-2010)

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  1. Biliaryatresia Registry for 10 years (2001-2010) PrimaryKasai operation (435명)

  2. Demographic result • Sex distribution • 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 Mean 43명

  4. 병원별Case no.

  5. 병원별환자수 Mean 23.8명

  6. 입원 당시 결과 • Age • Mean age : 60.1±31.4days(1-231), • median : 58days • 증상 - 기타 : Fever(5), 산전검사에서choledochal cyst 발견되어 내원(4). vomiting(2), prematurity, 산전 진단에서 물혹 발견, failure to thrive, Ilealatresia, Inguinal hernia, splenomegaly, skin infection (N : 404)

  7. 입원시laboratory finding (N : 404)

  8. Diagnostic tools

  9. Ultrasonogram

  10. DISIDA

  11. CT

  12. MRCP

  13. Liver biopsy

  14. Fibroscan

  15. Other studies • 기타 : duodenalintubation/analysis(10), • 그 외 복강경(3)

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

  17. Opcholangiogram

  18. Extrahepatic bile duct occlusion type • N : 350 • 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 • N: 402 • Roux-en-Y portojejunostomy: 401/402 : 99.8% • Others : 1/402 : 0.2%  Roun-en-Y hepaticojejunostomy(type I, 아산)

  22. Intraoperativecomplication • N: 378 • 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

  24. Antibiotics Treatment

  25. Antibiotics duration

  26. Oral antibiotics use (N:404)

  27. Oral antibiotics use

  28. Duration of oral antibiotics use

  29. Steroid use (N:404)

  30. Steroid use start day

  31. Steroid use duration

  32. UDCA 사용

  33. Postoperative minimal bilirubinbefore discharge When, POD mean 15.8 ± 8.4 day (0-31)

  34. Postoperative minimal bilirubin before discharge

  35. Jaundice free rate (bilirubin<2) (N : 404) When, POD, Mean 17.1 ±8.9 day (1-31)

  36. Postoperative status 435 kasai op. 363 DC (31)2nd 36 LT5 death (53)loss 207(7) alive 157(24)LT18(5) death (1)loss 105s 40c11 death (54) loss 352 alive29 death

  37. Discharge date (POD)

  38. Discharge medication (N:404) •  Others : Phenobarbital 78명(전체 중 19.2%)로 m/c • Tocopherol (Vt E)70명 (전체 17.2%) • Albityl41명 (10.1%) • Multivitamin 종류(3), 그외calico(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, intrahepaticbiliary cyst… (N : 435)

  40. Late complication (N : 435) •  Others : Hyperbilirubinemia4(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 (N : 435)

  42. Last follow up • Mean POD : 3.4±2.8 years(0-10.0) • Mean total bilirubin : 2.7±5.2(0.1-35.8)

  43. Last f/u total bilirubin 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), biliarycirrhosis(4). Hepatosplenomegaly(4), abdominal distension(3), hypoalbuminemia, liver cirrhosis(4), hepatic richets(4), viral infection(1), anuria(1), acholic stool, thrombocytopenia (N : 435)

  46. Overall survival (전체 )

  47. Overall survival (두 그룹 )

  48. Overall survival (6그룹)

  49. Nativeliver survival (전체 )

  50. Nativeliver survival (두 그룹 )

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