特集・総説・報告(44行)/P045-055_報告 日本肝移植研究会

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45 Liver Transplantation in Japan in 2006 Part 2 Registry by the The Summary Four thousand three hundred thirty liver transplants have been performed as of December 31, 2006 in 59 institutions in Japan. There were 4,292 living-donor transplants and 38 cadaveric transplants 35 from heart-beating donor and 3from non-heart-beating donor. Although the number of living-donor transplants has increased progressively every year since inception in 1989, reaching 566 in 2005, the total decreased for the first time, to 505, in 2006. The most popular frequent indication was cholestatic disease, followed by neoplastic disease. As for the graft liver in living-donor cases, the proportion of right lobe graft has been increasing. Patient survival following transplantation from heart-beating donor 1 year, 80.0%; 3 year, 77.0%; 5 year, 73.2% was similar to that from living donor 1 year, 82.2%; 3 year, 78.2%; 5 year, 76.0%; 10 year, 72.4%; 15 year, 68.8%., registry, cadaveric liver transplantation, living-donor liver transplantation, prognosis 1992 1998 2000 2002 1-7 2006 2002 3-7 25 1 2001 9 FAX 1 2006 1998 3 2007 12 31 2006 Kaplan-Meier logrank test 59 1 2326 15 109 159 1 46 26 43 15 7 246 1,235 33 157

46 Vol. 43, No. 1 52123 17 35 21 6 2 97 1 23 1 255 19 27 6 52 101 391 105 816 1 4 62 54 95 Living-donor Transplantation 4,292 Cadaveric Transplantation 38 Heart Beating Donor 35 Non-heart Beating Donor 3 Primary Transplantation 4,215 Retransplantation 110 Third Transplantation 5 9 6713 2117 22 91 3 1 25 25 16424 96 1 431 2006 4,330 38 35 3 4,292 4,215 110 5 32 5 1 4,183 105 4 1989 2005 2005 566 2006 505 18 18 1991 Year 1964 1968 1989 1990 1991 1992 1993 1994 1995 1996 1997 1998 1999 2000 2001 2002 2003 2004 2005 2006 Total Living-donor 0 0 1 10 30 31 51 82 111 120 157 208 251 327 417 434 440 551 566 505 4,292 2 2 6 10 22 48 90 142 188 264 292 330 426 446 383 2,621 Cadaveric 1 1 0 0 0 0 1 0 0 0 0 0 2 6 6 7 2 3 4 5 38 1 1 4 3 4 1 3 4 5 26 Total 1 1 1 10 30 31 52 82 111 120 157 208 253 333 423 441 442 554 570 510 4,330 2 3 6 10 22 48 90 143 192 267 296 331 429 450 388 2,647 Adults: 18 years Age 0 9 10 19 20 29 30 39 40 49 50 59 60 69 70 79 Total Male 3 1 2 3 7 1 1 0 18 Female 4 4 2 5 0 4 1 0 20 Total 7 5 4 8 7 5 2 0 38 Age 0 9 10 19 20 29 30 39 40 49 50 59 60 69 70 79 Total Male 554 184 115 139 262 589 213 1 2,057 Female 796 206 136 150 264 474 205 4 2,235 Total 1,350 390 251 289 526 1,063 418 5 4,292

47 Cholestatic Diseases 14 Biliary Atresia 9 Primary Biliary Cirrhosis 3 Primary Sclerosing Cholangitis 2 Hepatocellular Diseases 7 HCV 3 Cryptogenic Cirrhosis 2 HBV 1 Alcoholic 1 Vascular Diseases 0 Neoplastic Diseases 2 Hepatocellular Carcinoma 2 Acute Liver Failure 5 HBV 4 Unknown 1 Metabolic Diseases 4 Wilson Disease 2 Familial Amyloid Polyneuropathy 2 Total 32 Age of Recipient 18 y.o. 18 y.o. Total Whole Liver 6 24 30 Lateral Segment 5 0 5 Right Lobe 1 2 3 12 26 38 Age of The Recipient Total 18 y.o. 18 y.o. Monosegment 49 0 49 Lateral Segment 1,165 6 1,171 Posterior Segment 2 46 48 Left Lobe 342 469 811 Left Lobe Caudate Lobe 53 517 570 Right Lobe 60 1,568 1,628 Whole Liver Domino 0 13 13 Dual Graft Left Right Lobes 0 2 2 1,671 2,621 4,292 Age of The Recipient Total 18 y.o. 18 y.o. Cholestatic Diseases 1,238 618 1,856 Biliary Atresia 1,141 101 1,242 Primary Biliary Cirrhosis 0 388 388 Primary Sclerosing Cholangitis 13 99 112 Alagille Syndrome 49 2 51 Byler s Disease 27 2 29 Others 8 26 34 Hepatocellular Diseases 42 635 677 HCV 1 292 293 HBV 0 175 175 Alcoholic 0 60 60 Autoimmune hepatitis 3 36 39 Cryptogenic cirrhosis 30 64 94 Others 8 8 16 Vascular Diseases 18 15 33 Budd-Chiari Syndrome 5 13 18 Others 13 2 15 Neoplastic Diseases 40 829 869 Hepatocellular Carcinoma 3 802 805 HCV 0 479 479 HBV 0 263 263 Alcoholic 0 22 22 Others 3 38 41 Hepatoblastoma 31 0 31 Liver Metastasis 1 15 16 Others 5 12 17 Acute Liver Failure 138 313 451 HBV 6 95 101 Drug-induced 1 22 23 Autoimmune hepatitis 2 17 19 Viral HBV 6 10 16 Unknown 121 168 289 Others 2 1 3 Metabolic Diseases 128 142 270 Wilson Disease 53 42 95 Familial Amyloid Polyneuropathy 0 53 53 Citrullinemia 4 33 37 OTC Deficiency 20 1 21 Glycogen Storage Disease 8 6 14 Tyrosinemia 12 0 12 Primary hyperoxaluria 7 3 10 Others 24 4 28 Others 8 19 27 Total 1,612 2,571 4,183

48 Vol. 43, No. 1 Age 0 9 10 19 20 29 30 39 40 49 50 59 60 69 Unknown Total Male 0 0 3 4 4 4 1 2 18 Female 0 2 4 1 4 2 1 4 18 Unknown 1 0 0 0 0 0 0 1 2 Total 1 2 7 5 8 6 2 7 38 Age 0 9 10 19 20 29 30 39 40 49 50 59 60 69 70 79 Total Male 0 33 682 769 456 300 94 1 2,335 Female 0 17 442 709 433 300 58 0 1,959 Total 0 50 1,124 1,478 889 600 152 1 4,294 2004 400 2006 300 / 1999 1 2002 2 2004 3 1999 2 7 1964 1968 1993 27 70 11 Caroli 8 4 HCV HBV NASH 6 cryptogenic cirrhosis 20 7 3 2 3 16 13 11 2 solid pseudopapillary tumor 1 8 4, epithelioid hemangioendothelioma 3 1 1 1 9 5 3 2 2 2 2 Dubin-Johnson 1 10 10 graft split 3 reduced 2 graft split graft graft 28 87 split 3 1 2 dual graft 2 3 20 30 40 split 6 6 20 30 40

49 Age of The Recipient Total 18 y.o. 18 y.o. Age of The Recipient Total Mother 877 158 1,035 18 y.o. 18 y.o. Father 720 156 876 Identical 1,137 1,859 2,996 Son 0 769 769 Compatible 330 577 907 Daughter 0 305 305 Incompatible 204 184 388 Brother 8 296 304 1,671 2,620 4,291 Sister 4 207 211 Nephew 0 34 34 Grandmother 25 0 25 Cousin 1 Male 16 Male 15, Female 1 17 1 35 Grandfather 14 0 14 30 Aunt 9 4 13 20 17 Uncle 8 5 13 3 1 1 70 1 Niece 0 8 8 Father s cousin 2 Male 1, Female 1 0 2 dual graft 2 Grandson 0 1 1 4,292 2 4,294 Cousin s son 0 1 1 3 20 Wife 0 340 340 50 60 split Husband 0 263 263 20 Brother-in-law 0 11 11 50 60 1 Son-in-law 0 7 7 4,291 Father-in-law 1 2 3 Sister-in-law 0 3 3 Nephew-in-law 0 3 3 96% Daughter-in-law 0 2 2 41%23% 19% Mother-in-law 0 1 1 12% dual graft Uncle-in-law 0 1 1 4,292 2 4,294 Grandfather-in-law 1 0 1 3 split Common-law wife 0 1 1 Common-law husband 0 1 1 4,291 split Friend 0 1 Female 1 6 1 Domino 1 Male 27 Male 15, Female 12 28 5 3 2 1,671 2,623 4,294 Year 1989 1990 1991 1992 1993 1994 1995 1996 1997 1998 1999 2000 2001 2002 2003 2004 2005 2006 Total 18 years 0 0 0 0 0 0 0 0 0 0 3 5 4 1 7 4 2 1 27 18 years 0 0 0 0 0 0 0 0 0 0 0 0 0 0 1 0 0 0 1 Total 0 0 0 0 0 0 0 0 0 0 3 5 4 1 8 4 2 1 28

50 Vol. 43, No. 1 Year 1989 1990 1991 1992 1993 1994 1995 1996 1997 1998 1999 2000 2001 2002 2003 2004 2005 2006 Total 18 years 0 0 1 0 0 1 1 0 5 3 5 5 17 13 22 33 47 31 184 18 years 0 0 4 4 11 12 9 11 14 9 13 8 13 21 13 20 24 18 204 Total 0 0 5 4 11 13 10 11 19 12 18 13 30 34 35 53 71 49 388 Patient Survival % Graft Survival % n 1 3 5 10 15 n 1 3 5 10 15 year year year year year year year year year year Cadaveric Donor 38 73.7 71.0 67.4 38 73.7 71.0 67.4 Heart-beating 35 80.0 77.0 73.2 35 80.0 77.0 73.2 Non-heart-beating 3 0.0 3 0.0 Living Donor 4,292 82.2 78.2 76.0 72.4 68.8 4,292 81.7 77.5 75.1 70.6 67.0 FAP ABO dual graft 1 ABO ABO 2 dual graft 1 ABO 2 ABO 7% 12% 204 0 86 1 39 2 16 ABO 1 p 0.0001 2

51 Primary or Retransplant Recipient Gender Recipient Age Indication Donor Age Graft Gender Combination ABO Compatibility n Cumulative Survival % 1year 3year 5 year 10 year 15 year Primary 4,183 83.1 79.1 76.8 73.2 69.5 Re-transplantation 105 46.7 44.5 44.5 39.0 Re-re-transplantation 4 75.0 75.0 75.0 Male 2,057 81.6 76.6 74.2 69.4 68.9 Female 2,235 82.8 79.7 77.7 74.8 69.6 18 1,671 86.6 84.9 83.8 80.3 77.1 18 2,621 79.4 73.8 70.4 66.3 Cholestatic Disease 1,856 86.6 84.9 83.7 79.8 76.1 Biliary Atresia 1,242 89.4 88.2 87.3 83.5 80.2 Primary Biliary Cirrhosis 388 80.9 77.7 76.2 73.8 Primary Sclerosing Cholangitis 112 77.7 74.7 70.1 54.9 Alagille Syndrome 51 92.1 90.0 90.0 86.9 86.9 Byler s Disease 29 89.7 86.1 86.1 86.1 86.1 Hepatocellular Disease 677 78.4 73.4 71.1 63.8 63.8 HCV 293 75.0 69.4 65.8 HBV 175 82.8 80.1 79.1 73.1 Alcoholic 60 83.1 81.3 76.8 Autoimmune hepatitis 39 79.3 79.3 79.3 Cryptogenic Cirrhosis 94 77.6 70.6 66.7 66.7 66.7 Vascular Disease 33 93.9 83.3 83.3 83.3 83.3 Budd-Chiari Syndrome 18 88.9 76.2 76.2 76.2 76.2 Congenital Absence of Portal Vein 13 100.0 90.9 90.9 90.9 Neoplastic Disease 869 82.6 72.8 65.9 62.0 62.0 HCC 805 82.9 72.3 65.8 62.4 62.4 Hepatoblastoma 31 83.7 83.7 71.7 71.7 Liver Metastasis 16 75.0 75.0 64.3 Acute Liver Failure 451 72.7 69.0 67.0 66.6 55.5 HBV 101 79.9 75.5 75.5 75.5 Drug-induced 23 78.3 78.3 78.3 Autoimmune hepatitis 19 63.2 63.2 63.2 Viral HBV 16 75.0 75.0 75.0 Unknown 289 70.0 65.8 62.8 62.1 49.7 Metabolic Disease 270 88.8 84.7 82.4 81.7 79.2 Wilson Disease 95 90.5 89.2 85.9 83.5 83.5 Familial Amyloid Polyneuropathy 53 94.3 84.5 78.9 78.9 Citrullinemia 37 94.6 94.6 94.6 94.6 OTC Deficiency 21 95.2 95.2 95.2 95.2 Glycogen Storage Diseases 14 84.6 66.6 66.6 66.6 Tyrosinemia 12 83.3 75.0 75.0 75.0 Primary Hyperoxaluria 10 50.0 50.0 50.0 50.0 29 1,174 83.8 80.7 78.8 77.6 71.5 30 39 1,478 85.5 81.5 79.9 75.8 75.8 40 49 889 81.5 78.0 76.0 70.3 62.7 50 59 600 76.3 70.3 66.1 63.3 29.9 60 153 66.1 60.2 54.5 50.6 Monosegment 49 73.5 70.8 64.9 Lateral Segment 1,171 87.9 86.3 85.8 82.2 79.4 Posterior Segment 48 81.2 75.8 72.2 Left Lobe 811 78.5 74.8 71.6 68.5 62.8 Left Lobe Caudate Lobe 570 79.4 75.3 71.2 66.5 Right Lobe 1,628 81.2 75.3 72.3 69.8 69.8 Whole Liver 13 84.6 76.2 76.2 Male Female 1,276 81.8 78.5 76.2 72.8 70.3 Male Male 1,058 83.5 78.5 76.4 72.6 71.5 Female Male 998 79.5 74.5 71.8 66.1 66.1 Female Female 959 84.1 81.4 79.6 77.2 69.3 Identical 2,996 83.3 79.5 77.1 73.4 71.2 Compatible 907 82.4 78.2 76.7 73.0 70.0 Incompatible 388 73.1 68.8 66.3 63.1 51.8

52 Vol. 43, No. 1 p 0.0114 3 p 0.000110 p 0.0001 2 0 10 p 0.013 20 60 p 0.0001 10 20 p 0.0121 30 p 0.002 40 p 0.0193 50 p 0.0001 60 p 0.0014 20 60 4 6 p 0.00012 p 0.0001 p 0.0001 p 0.0001 p 0.0001

53 p 0.0463 p 0.0001 p 0.0001 p 0.0001 2 p 0.0001 p 0.0001 Alagille p 0.0346 Alagille p 0.0042 Byler p 0.045 n 8 1 87.5% 3 72.9% 5 72.9% HCV 1 82.4% 3 71.1% 5 63.8% HBV 1 84.7% 3 76.1% 5 69.3% 1 77.0% 3 67.4% 5 67.4%

54 Vol. 43, No. 1 p 0.00762 Wilson p 0.0016 FAP p 0.0074 p 0.0024 OTC p 0.0373 OTC p 0.0024 1 90.0% 3 90.0% 5 77.1% 1 90.0% 3 90.0% 5 77.1% 1 3 5 70.0% 5 7 graft p 0.0001 6 ABO data not shown 7 data not shown 830 30 40 50 60 5 p 0.0001HCV 5 p 0.00160 HCV n 21 1 47.6% 3 37.5% 5 28.1% 9 1 82.1% 3 70.9 5 65.0% 10

55 p 0.0009 2 3 11 ABO 2 1 84.4% 3 83.6% 5 82.4% 10 81.0% 15 81.0% 3 18 1 68.3% 3 63.1% 5 60.9% 10 55.0% 15 32.1% 18 1 66.2% 3 58.6% 5 54.6% 2 ABO 2001 2002 4 p 0.0001 1 39.5% 3 34.2% 5 34.2% 1 73.1% 3 65.9% 5 59.5% p 0.0001 p 0.0056 1 75.0% 3 73.1% 5 71.3% 10 68.1% 15 55.9% 1 84.4% 3 82.0% 5 82.0% p 0.1024 1992 8 2006 1 1 2006 2005 2 2006 505 1 1.. 1998; 39: 5-12. 2.. 2000; 35: 133-144. 3.. 2002; 37: 245-251. 4.. 2003; 38: 401-408. 5.. 2004; 39: 634-642. 6.. 2005; 40: 518-526. 7.. 2006; 41: 599-608.