胆道閉鎖症 診療ガイドライン - JCQHC

1 1959 34 non-correctable type .. QOL.. 2013 25 .. Minds 2014 . CQ .. CQ .. QOL .. 296 .. iii . 1950 . 1989 2017. 29 3,200 . 1963 38 . Thomas E. Starzl .. 2013 25 . Minds . 2014 26 .. 2016 28 .. Minds .. CQ . delphi .. v .. CQ 2. 6. 8.. 12. 14. 17. 18. 20.. 24. 30.. 40. 40. 42. CQ . CQ1 44. CQ2 45. CQ3 47. CQ4 50. CQ5 51. CQ6 54.. 60. 60. 61. CQ . CQ7 K 65. CQ8 30 68. vi CQ9 71. CQ10 73. CQ11 UDCA 75. CQ12 77.. 82. 82. 83. 83. CQ . CQ13 86. CQ14 87. CQ15 . PTBD 89. CQ16 91. CQ17 SpO2 94. CQ18 96.. 100. 101. 102. 102. CQ . CQ19 103. CQ20 105. CQ21 107. CQ22 109. CQ23 112. CQ24 . 114. CQ25 PELD score 10 116. 120. vii CQ .. 1. CQ .. p. 39 .. CQ . C. 1 .. CQ.. D. 2 .. CQ.. B. 3 .. CQ .. C. 4 .. CQ . C. 5 .. CQ . C. 6 .. 2.. p. 59 .. CQ . D. 7 .. CQ . 30 30 C. 8 .. CQ . B. 9 .. CQ . D. 10 .. CQ . UDCA UDCA D. 11 .. CQ.. C. 12 .. 3.. p. 81 .. CQ .. TMP/SMZ C. 13 .. CQ . C. 14 .. CQ. percutaneous transhepatic biliary drainage PTBD D.

2 15 PTBD .. CQ . C. 16 .. CQ .. SpO2 D. 17 SpO2 . CQ .. B. 18 . 4.. p. 99 .. CQ.. C. 19 .. CQ . C. 20 .. CQ . as low as reasonably C. 21 achievable ALARA .. CQ .. C. 22 . CQ . D. 23 . CQ.. D. 24 .. CQ. PELD score 10 . D. 25 . 5.. 1 .. mm . I-cyst 1 1 2 . b mm . bile lake b1 b1 b2 . cyst .. c .. c1 c1 c2.. 1 . 1976 12(2) 327-331 . 1 .. QOL .. 6. 1 . / . 1+. 1+ 2+ 3+ . 1+ 2+ 3+ .. 1+ 2+ 3+.. 1+ 2+ 3+ . 1+ 2+ 3+ .. 2 2013 .. 2 .. CRP . ALP -GTP (GGT) AST ALT . ALP; alkaline phosphatase, -GTP (GGT); -glutamyltransferase AST; asparate aminotransferase, ALT; alanine aminotransferase BT>38 . WBC 1,000/ l <4, or >10. CRP mg/dl 1. T-Bil 2 mg/dl . ALP IU > STD. -GTP IU > STD. AST IU > STD. ALT IU > STD. STD standard .. 1 + . 2 + . 3 + .. 7.. BA .. CQ1, 2, 3. Yes . BA .. CQ4, 5.. No CQ7 .. BA .. CQ25 . BA .. CQ8.. CQ9, 11. CQ12 . CQ10. CQ6.. 8.. CQ4, 6, 17, 18, 20, 21.. CQ12 .. CQ13, 14, 15, 16, 22, 23.. CQ19, 24, 25. QOL . BA . Yes No.

3 9.. 11.. 2016 .. 2016 .. 12.. 2016 .. 13.. 2016 .. Minds 2014 .. COI . CQ .. evidence based consensus guideline .. CQ . 14.. 26 27 . 28 .. 15. 2013 11 15 . WG . 2013 11 22 WG .. 2014 .. 2014 11 .. CQ .. CQ patients interventions /. comparisons outcomes .. CQ 2014 10 2015 . 2015 . CQ CQ .. 2014 10 10 . 2014 12 27 . 2015 . CQ .. 2015 19 . 2015 22 .. 2015 10 12 . Delphi .. 2015 11 42 .. 16. 2016 12 10 12 .. 2015 10 12 10 17 .. Delphi . 36 32 . 33 . 2016 .. 2016 12 12 2017 31 .. Minds . Minds .. 2015 .. QOL . 17.. 2013 .. PBC 2012 .. QOL .. QOL .. p. 16 .. PubMed 2014 . 18. PubMed biliary atresia English . Japanese .. CQ .. SR . CQ .. CQ .. CQ .. CQ .. Minds . STEP 1 .. STEP 2 . Minds .. CQ .. 19.. CQ .. Delphi 70 .. Delphi 1-1, 1-2 . 70% .. 1-1 1-2 . 20. 1-1 1-2 . A . B .. C .. Delphi . D .. 2015 11 42 . 2016 12 .. p. 14 .. Minds .. CQ CQ .. 21.. 23.. Davenport 1) . biliary atresia splenic malformation BASM syndrome . 2) cytomegalovirus CMV 3).

4 4) . correctable non-correctable .. 5) 2-1 . 6) 7) 8) 9) . 10) . 2-1 2-3 . type 1 . type 2 . type 3 type 4 . type 1 type 2 type 3 4 subtype a subtype b 10) .. 2-2 .. 2-3 .. 11)12) 2-4 .. 24.. mm . I-cyst 1 1 2.. b mm . bile lake b1 b1 b2 . cyst .. c .. c1 c1 c2.. 2-1 . 1976 12(2) 327-331 . 2-1 .. -cyst . 2,900 8 2 6 . 244 7 . 424 2 8 . 214 9 . 2-2 . a b c d . a1 a2 b1 b2 c1 c2. 3 6 . 7 . 2-3 .. 3 6 . 0 7 7 . 25.. -cyst .. 0. 0 2,000 4,000 6,000 8,000 10,000. type 10 20 .. -cyst .. 2-2 2-3 .. 2014 2016 52(2) 291- . 297 . 2-4 .. epigenetics DNA .. ductal plate . ductal plate malformation DPM 13)14) 15) 19) . Sox1720) Hes121) HNF622) HNF-1 23) . Hhex24) . 26.. reovirus25)26) rotavirus27) cytomegalovirus28) Epstein-Barr virus29) papillomavirus30) . 31) . 32) rhesus rotavirus group A . 33) .. toll-like receptor TLR 34)35) . 36) . 37)38) Th1 . 37)39) Th 40) . 41) 46) HLA 47)48) Th17/Treg . 49) maternal microchimerism 50)51) .. genome imprinting DNA.

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