原著・報告・記録(44行)/P134~147_報告 肝移植症例登録

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1 134 Vol. 52, No. 23 Liver transplantation in Japan. Registry by the Japanese Liver Transplantation Society The Japanese Liver Transplantation Society Summary As of December 31, 2016, a total of 8,825 liver transplants have been performed in 67 institutions in Japan. There were 378 deceased donor transplants375 from heart-beating donor and 3 from non-heart-beating donorand 8,447 living-donor transplants. The annual total of liver transplants in 2016 was deceased donor transplants and 381 living-donor transplants. The most frequent indication was cholestatic disease, followed by neoplastic disease. As for the graft liver in living-donor cases, right lobe graft was the most popular35%. Patient survivals following transplantation from heart-beating donors1-year 88.1%, 3-year 84.8%, 5-year 82.3%, 10-year 76.7%, and 15-year 76.7%were similar to those from living-donors1-year 84.7%, 3-year 80.6%, 5-year 78.2%, 10-year 72.8%, 15-year 68.5%, 20-year 66.0%, and 25-year 65.2%. Graft survivals were very much the same as patient survivals. Survival data were provided according to age and sex of recipients, indication, age and sex of donors, ABO-compatibility, and other factors. Japanese Liver Transplantation Society, registry, deceased liver transplantation, living-donor liver transplantation, prognosis web web 2011 web web 2016 web Kaplan-Meier logrank test ,

2 Living-donor Transplantation 8,447 Deceased-donor Transplantation 378 Heart Beating Donor 375 Non-heart Beating Donor 3 Primary Transplantation 8,537 Retransplantation 274 Third Transplantation , ,447 8, , / 9 76 Adults: 18 years Year Living-donor Deceaseddonor Total Total , , , ,730

3 136 Vol. 52, No. 23 primary sclerosing cholangitispsc primary biliary cirrhosis PBC 7 6 / 11 7 / 4 B NASH 2016 C 2 cryptogenic cirrhosis PSC III 1 DNA 1Budd-Chiari solid pseudopapillary tumor 1 epithelioid hemangioendothelioma PSC 5Caroli 2 1 B 1cryptogenic cirrhosis NASH Ia 3Ib 14IV 5 Ia 2Ib 2III 5 DNA Dubin-Johnson Protein C GVHD 6 5 SCID monosegment graft graft graft graft graft graft graft graft graft graft 1 2 dual graft 2 split dual graft 2 8, ,449 split 95% 44%24%18% 10% dual graft 8, ,449 4 split 8,445 3 FAP ABO

4 137 Age Total Male Female Total Age Total Male 1, , ,031 Female 1, ,416 Total 2, ,150 1, ,447 Age of Recipient 18 y.o. 18 y.o. Total Cholestatic Diseases Biliary Atresia Primary Sclerosing Cholangitis Primary Biliary Cirrhosis Others Hepatocellular Diseases HCV HBV Alcoholic NASH Autoimmune Hepatitis Cryptogenic Cirrhosis Others Vascular Diseases Budd-Chiari Others Neoplastic Diseases Hepatocellular Carcinoma Others Acute Liver Failure HBV Drug-induced Autoimmune Hepatitis ViralHBV Unknown Others Metabolic Diseases Wilson Disease Citrullinemia OTC Deficiency Familial Amyloid Polyneuropathy Glycogen Storage Disease Others Others Polycystic Liver Total

5 138 Vol. 52, No. 23 Age of Recipient 18 y.o. 18 y.o. Total Cholestatic Diseases 2,145 1,186 3,331 Biliary Atresia 1, ,183 Primary Biliary Cirrhosis Primary Sclerosing Cholangitis Alagille Syndrome Byler s Disease Caroli Disease Congenital Bile Duct Dilatation Others Hepatocellular Diseases 49 1,621 1,670 HCV HBV Alcoholic Autoimmune Hepatitis NASH Cryptogenic Cirrhosis Others Vascular Diseases Budd-Chiari Syndrome Congenital Absence of Portal Vein Others Neoplastic Diseases 121 1,633 1,754 Hepatocellular Carcinoma 8 1,590 1,598 HCV HBV Alcoholic Primary Biliary Cirrhosis NASH Others Hepatoblastoma Liver Metastatis Hemangioma Cholangiocellular Carcinoma Others Acute Liver Failure HBV Drug-induced Autoimmune Hepatitis ViralHBV Neonatal Hemochromatosis Unknown Others Metabolic Diseases Wilson Disease Familial Amyloid Polyneuropathy OTC Deficiency Citrullinemia Glycogen Storage Disease Methylmalonic Acidemia Primary Hyperoxaluria CPS deficiency Tyrosinemia Propionic acidemia Others Others Total 2,935 5,298 8,233

6 139 Year Total HCV HBV Alcoholic Autoimmune NASH Cryptogenic Others Total ,670 Age of Recipient Total 18 y.o. 18 y.o. Monosegment Lateral Segment Left Lobe Left Lobe Caudate Lobe Right Lobe Right Trisegment Whole Liver Age of Recipient Total 18 y.o. 18 y.o. Monosegment Lateral Segment 2, ,103 Posterior Segment Left Lobe 579 1,096 1,675 Left Lobe Caudate Lobe 114 1,255 1,369 Right Lobe 91 2,893 2,984 Right Trisegment Whole Liver Dual GraftLeft Right Lobes ,042 5,405 8,447 Age Unknown Total Male Female Unknown Total Age Total Male ,295 1, ,548 Female , ,901 Total ,103 2,934 1,741 1, ,449 dual graft 1 ABO ABO 2 8, ,446 dual graft 1 ABO 2 ABO 11.0% 14.2% ABO

7 140 Vol. 52, No y.o. Age of Recipient 18 y.o. Mother 1, ,877 Father 1, ,555 Son 0 1,632 1,632 Daughter Brother Sister Nephew Grandmother Aunt Cousin 3 Male 2, Female 1 31 Male 26, Female 5 34 Uncle Grandfather Niece Father s cousin 2 Male 1, Female Mother s cousin 0 1 Female 1 Grandson Cousin s son Cousin s daughter Wife Husband Brother-in-law Son-in-law Sister-in-law Father-in-law Daughter-in-law Nephew-in-law Mother-in-law Grandfather-in-law Uncle-in-law Common-law husband Common-law wife Friend 0 1 Female 1 Domino 6 Male 3, Female 3 50 Male 25, Female ,042 5,407 8,449 Total Year Total 18 years years Total

8 141 Year Age of Recipient 18 y.o. 18 y.o Total 18 years years Total Total Identical 1,986 3,597 5,583 Compatible 624 1,212 1,836 Incompatible ,027 3,042 5,404 8,446 n Patient Survival% Graft Survival% n year year year year year year year year year year year year year year Deceased Donor Heart-beating Non-heart-beating Living-Donor 8, , n 1 year Cumulative Survival% year year year year Primary or Primary Retransplant Re- and Re-re-transplantation Recipient Age Cholestatic Disease Primary Sclerosing Cholangitis Biliary Atresia Primary Biliary Cirrhosis Hepatocellular Disease HCV HBV Indication Neoplastic Disease Primary HCC Acute Liver Failure HBV Drug-induced Autoimmune Hepatitis Unknown Metabolic Disease Donor Age

9 142 Vol. 52, No / p p n % 1/ p p

10 143 n 1 year 3 year Cumulative Survival% year year year Primary or Primary 8, Retransplant Re- and Re-re-transplantation Recipient Gender Male 4, Female 4, Recipient Age 18 3, , year 25 year 9 2, , , Cholestatic Disease 3, Biliary Atresia 2, Primary Biliary Cirrhosis Primary Sclerosing Cholangitis Alagille Syndrome Byler s Disease Caroli Disease Congenital Bile Duct Dilatation Hepatocellular Disease 1, HCV HBV Alcoholic Autoimmune Hepatitis NASH Cryptogenic Cirrhosis Vascular Disease Budd-Chiari Congenital Absence of Portal Vein Neoplastic Disease 1, HCC 1, Indication Hepatoblastoma Primary Liver Metastasis Hemangioma CCC Acute Liver Failure HBV Drug-induced Autoimmune Hepatitis ViralHBV Neonatal Hemochromatosis Unknown Metabolic Disease Wilson Disease Familial Amyloid Polyneuropathy OTC Deficiency Citrullinemia Glycogen Storage Diseases Methylmalonic Acidemia Primary Hyperoxaluria CPS Deficiency Tyrosinemia Propionic Acidemia Donor Age , , , , Domino Domino Non-Domino 8, ABO Identical 5, Compatibility Compatible 1, Incompatible 1,

11 144 Vol. 52, No. 23 p p data not shown 46 p p PSC PSC %3 75.8%5 70.2% % % PBC %3 79.9%5 78.4% % % % p p epithelioid hemangioendothelioman %3 43.8% % p % % %3 84.8%5 78.3% % n % % GVHDn %3 50.0% %

12 145 5 ABO data not shown 6 data not shown 710 p p ABO data not shown

13 146 Vol. 52, No ABO p %3 86.4%5 86.2% % % %3 73.9%5 71.8% % % %3 67.9% % % % p ABO rituximab %3 84.2%5 83.3%1 86.9% % %3 74.7%5 72.4%p0.0065

14 ; 39: ; 35: ; 37: ; 38: ; 39: ; 40: ; 41: ; 43: ; 43: ; 44: ; 45: ; 46: ; 47: ; 48: ; 49: ; 50: ; 51:

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