原著・報告・記録(44行)/P261~274_報告 肝移植症例登録

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1 261 Liver Transplantation in Japan Registry by the Japanese Liver Transplantation Society The Japanese Liver Transplantation Society Summary As of December 31, 2013, a total of 7,474 liver transplants have been performed at 66 institutions in Japan. This total included 7,255 living-donor transplants and 219 cadaveric-donor transplants216 from heart-beating donors and 3 from non-heart-beating donors. The annual total of liver transplants in 2013 decreased to 408, from 422, in The number of liver transplants from living donors decreased to 369, from 381, whereas the number of liver transplants from heart-beating cadaveric donors did not change significantly. The most frequent indication was cholestatic disease, followed by neoplastic disease. As for the graft liver in living-donor cases, a right-lobe graft was the most popular36%. Patient survival following transplantations from heart-beating donors1, 85.9%; 3 s, 82.6%; 5 s, 81.3%; 10 s, 73.8%was similar to those from living donors1, 83.8%; 3 s, 79.6%; 5 s, 77.1%; 10 s, 71.9%; 15 s, 67.8%; 20 s, 66.1%. Graft survival was very much the same as patient survival. As for ABO-incompatible transplantation, new strategies, including portal vein infusion and rituximab prophylaxis, have been improving prognoses in adults as well as in children older than 3 s. Japanese Liver Transplantation Society, registry, cadaveric liver transplantation, living-donor liver transplantation, prognosis FAX 1 / web

2 262 Vol. 49, No Kaplan-Meier logrank test , Living-donor Transplantation 7,255 Cadaveric Transplantation 219 Heart Beating Donor 216 Non-heart Beating Donor 3 Primary Transplantation 7,239 Retransplantation 223 Third Transplantation , ,255 7, , Adults: 18 s Year Living-donor Cadaveric Total Total , , , ,835

3 /C / 10 6 / 4 B NASH cryptogenic cirrhosis solid pseudopapillary tumor 1 9epithelioid hemangioendothelioma Caroli 2 1 B 1cryptogenic cirrhosis NASH DNA Dubin- Johnson GVHD monosegment graft graft graft graft graft graft graft graft split dual graft 2 split dual graft 2 7, , split ,254 95% 43%23%18% 10% dual graft 7, ,257 3 split 7,254 split

4 264 Vol. 49, No. 23 Age Total Male Female Total Age Total Male , ,462 Female 1, ,793 Total 2, , ,255 Age of Recipient 18 y.o. 18 y.o. Total Cholestatic Diseases Biliary Atresia Primary Biliary Cirrhosis Primary Sclerosing Cholangitis Alagille Syndrome Caroli Disease Hepatocellular Diseases HCV HBV Alcoholic NASH AIH Cryptogenic Cirrhosis Vascular Diseases Budd-Chiari Neoplastic Diseases Hepatocellular Carcinoma Hemangioma Acute Liver Failure HBV Drug-induced Autoimmune Hepatitis ViralHBV Hemochromatosis Unknown Metabolic Diseases Wilson Disease Citrullinemia Familial Amyloid Polyneuropathy Glycogen Storage Disease OTC Deficiency Others Polycystic Liver Total

5 y.o. Age of Recipient 18 y.o. Total Cholestatic Diseases 1,869 1,008 2,877 Biliary Atresia 1, ,897 Primary Biliary Cirrhosis Primary Sclerosing Cholangitis Alagille Syndrome Byler s Disease Caroli Disease Congenital Bile Duct Dilatation Others Hepatocellular Diseases 43 1,323 1,366 HCV HBV Alcoholic Autoimmune Hepatitis NASH Cryptogenic Cirrhosis Others Vascular Diseases Budd-Chiari Syndrome Congenital Absence of Portal Vein Others Neoplastic Diseases 88 1,462 1,550 Hepatocellular Carcinoma 8 1,423 1,431 HCV HBV Alcoholic Primary Biliary Cirrhosis NASH Others Hepatoblastoma Liver Metastatis Hemangioma Others Acute Liver Failure HBV Drug-induced Autoimmune Hepatitis ViralHBV Unknown Others Metabolic Diseases Wilson Disease Familial Amyloid Polyneuropathy OTC Deficiency Citrullinemia Glycogen Storage Disease Methylmalonic Acidemia Primary Hyperoxaluria CPS deficiency Tyrosinemia Others Others Total 2,515 4,551 7,066

6 266 Vol. 49, No. 23 Year Total HCV HBV Alcohol AIH NASH Cryptogenic Others Total ,366 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 1, ,814 Posterior Segment Left Lobe ,416 Left Lobe + Caudate Lobe 100 1,080 1,180 Right Lobe 84 2,509 2,593 Whole LiverDomino Dual GraftLeft + Right Lobes ,609 4,646 7,255 Age Unknown Total Male Female Unknown Total Age Total Male ,139 1, ,928 Female , ,329 Total ,838 2,519 1,481 1, , FAP ABO dual graft 1 ABO ABO 2 7, ,254 dual graft 1 ABO 2

7 y.o. Age of Recipient 18 y.o. Mother 1, ,622 Father 1, ,335 Son 0 1,405 1,405 Daughter Brother Sister Nephew Grandmother Aunt Cousin 2 Male 2 28 Male 23, Female 5 30 Uncle Grandfather Niece Father s cousin 2 Male 1, Female Grandson Cousin s son Wife Husband Brother-in-law Son-in-law Sister-in-law Father-in-law Nephew-in-law Mother-in-law Daughter-in-law Grandfather-in-law Uncle-in-law Common-law husband Common-law wife Friend 0 1 Female 1 Domino 1 Male 40 Male 20, Female ,609 4,648 7,257 Total Year Total 18 s s Total

8 268 Vol. 49, No. 23 Age of Recipient 18 y.o. 18 y.o. Total Identical 1,717 3,148 4,865 Compatible 535 1,040 1,575 Incompatible ,609 4,645 7,254 Year Total 18 s s Total n 1 Patient Survival% n 1 Graft Survival% Cadaveric Donor Heart-beating Non-heart-beating Living Donor 7, , n Cumulative Survival% Primary or Primary Retransplant Re- and Re-re-transplantation Recipient Age Indication Primary Cholestatic Disease Biliary Atresia Primary Biliary Cirrhosis Primary Sclerosing Cholangitis Hepatocellular Disease HCV HBV Neoplastic Disease HCC Acute Liver Failure HBV Unknown Metabolic Disease

9 269 ABO 9.8% 13.7% ABO 10 1 / p / p p p p p p p0.0226hcv HBV p p n= %3 5

10 270 Vol. 49, No. 23 n 1 Cumulative Survival% Primary or Primary 7, Retransplant Re- and Re-re-transplantation Recipient Gender Male 3, Female 3, Recipient Age 18 2, , Indication Primary , , Cholestatic Disease 2, Biliary Atresia 1, 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, Hepatoblastoma Liver Metastasis Hemangioma Acute Liver Failure HBV Drug-induced Autoimmune Hepatitis ViralHBV Unknown Metabolic Disease Wilson Disease Familial Amyloid Polyneuropathy OTC Deficiency Citrullinemia Glycogen Storage Diseases Methylmalonic Acidemia Primary Hyperoxaluria CPS Deficiency Tyrosinemia

11 271 n 1 Cumulative Survival% Donor Age , , , , ABO Identical 4, Compatibility Compatible 1, Incompatible

12 272 Vol. 49, No. 23

13 %epithelioid hemangioendothelioman= % % p= n= % % % %3 75.5% % % n= %GVHDn= %3 50.0% % 5 ABO data not shown 6 data not shown 7 10 p0.0001hcv 60 HCV n= %3 47.2%5 38.6% 66 8 ABO data not shown 9 ABO p %3 85.5%5 84.8% % % %3 72.0%5 69.1% % % %3 64.8% % % p ABO rituximab

14 274 Vol. 49, No % % %3 56.5%5 52.9% % %3 69.0%5 66.7% p % p= ; 39: ; 35: ; 37: ; 38: ; 39: ; 40: ; 41: ; 43: ; 43: ; 44: ; 45: ; 46: ; 47: ; 48:

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