AUSTRALIA & 27TH. Melbourne

Transcription

1 AUSTRALIA & NEW ZEALAND Brisbane Perth Adelaide Sydney Melbourne Auckland N e w Z e a l a n d F r o m t h e C o m b i n e d R e g i s t r i e s o f t h e A u s t r a l i a n a n d N e w Z e a l a n d L i v e r Tr a n s p l a n t C e n t r e s CD Included INSIDE BACK COVER copyright ANZLTR Data to.. D A T A T O Report PowerPoint SLIDES

2 COORDINATING CENTRE ANZLT Registry Princess Alexandra Hospital Ipswich Rd WOOLLOONGABBA, QLD, Professor Stephen Lynch Ms Glenda Balderson Ms Debra Cormack Editor Editor/Liaison Officer Graphics Phone (67) 76 8 G.Balderson Fax (67) G.Balderson Glenda.Balderson@health.qld.gov.au MANAGEMENT COMMITTEE Professor L. Delreviere Sir Charles Gairdner Hospital, WA Professor R.M. Jones Austin Hospital, VIC Professor S.V. Lynch Princess Alexandra Hospital, QLD Professor G.W. McCaughan Royal Prince Alfred Hospital, NSW Professor S. Munn Auckland Hospital, NEW ZEALAND Dr. R. Padbury Flinders Medical Centre, SA Ms G.A. Balderson Princess Alexandra Hospital, QLD FUNDING ANZLTR is funded by the Australian Organ and Tissue Authority. CITATION The suggested citation for this report is as follows: ANZLT Registry Report Australia and New Zealand Liver Transplant Registry Brisbane, QLD, AUSTRALIA Editors: S.V. Lynch, G.A. Balderson STATISTICAL METHODS KaplanMeier survival curves have been produced using IBM SPSS for Windows Release.. ACKNOWLEDGMENT The Cancer Registry is maintained at Transplantation Services, Royal Prince Alfred Hospital, Sydney. Report prepared by Pamela Dilworth, Marie Mulhearn and Dr Deborah Verran. Director: Professor G.W McCaughan All queries to: Dr Deborah Verran verran@ausdoctors.net copyright ANZLTR Data to..

3 Preface Summary Section Summary Statistics Number of New Patients Age of Recipients Contents Number of Transplants and Type of Graft by Year Section Primary Diseases of Recipients Primary Diagnosis by Era Chronic Viral Hepatitis Adults Recipients Section Patient Survival Patient Survival by Age at Primary Transplant and Era of Transplant Patient Survival by Type of Primary Graft Patient Survival by Weight at Transplant Children Patient Survival by Primary Disease Section Graft Survival Indication for Retransplantation Section Causes of Patient Death Section 6 Deceased Donors by Year Donor Age and Graft Outcome Section 7 Living Donor Transplantation Section 8 Waiting List Activity and Outcome Waiting Time by Blood Group and Outcome Section 9 Summary and Type of Cancer Liver Malignancy as Primary or Secondary Diagnosis Type, Incidence, Survival & Mortality De Novo Non Skin Cancers Skin Cancers Post Transplant Cumulative Rate of Cancer Development Appendix I Transplant Units Australia and New Zealand Appendix II Metabolic Disorders Appendix III Other Diseases Appendix IV Fulminant Hepatic Failure Appendix V Causes of Patient Death Demographic Data Primary Diagnosis Patient Survival 6 79 Graft Outcome 8 9 Causes of Death Deceased Donor Information 6 Living Donor Transplantation 7 Waiting List 8 9 Liver Transplantation and Cancer DATA TO // copyright ANZLTR Data to.. CONTENTS

4 Preface We are pleased to present the 7th Report of the Australia and New Zealand Liver Transplant Registry (ANZLTR). This report contains data to the st December and analyses the cumulative data since the establishment of the first liver transplantation unit in Australia or New Zealand in 98. The Australia and New Zealand Liver Transplant Registry (ANZLTR) is a collaborative effort of the liver transplantation centres in Australia (Adelaide, Brisbane, Melbourne, Perth, Sydney) and New Zealand (Auckland). The Registry is supervised by the Management Committee which is involved in the ongoing supervision of the development of the Registry. The members of the Management Committee are listed on the front page. Donor data have been supplied by the Australia and New Zealand Organ Donor Registry and we thank them for their collaboration. The Editors would also like to thank the staff of all the Liver Transplant Units who contribute their data by direct entry into the ANZLTR database. A full list of the Units and their contact information can be found in Appendix I. In particular we are grateful to the efforts of Pamela Dilworth, Program Manager and Marie Mulhearn for their continuing contribution to the maintenance of the Cancer Registry which is based at the Royal Prince Alfred Hospital, Sydney and who, together with Dr Deborah Verran, prepare the Cancer Report. We are grateful to the Australian Government, through the Australian Organ and Tissue Authority, for their ongoing financial support. Comments are always welcome and should be forwarded to the Coordinating Centre at the contact information listed on the front page as should requests for further copies of this Report. The report is now also available on the ANZLTR public web site from where the report can be downloaded. Slides are available on request from the Coordinating Centre. Stephen Lynch Glenda Balderson. DATA TO // copyright ANZLTR Data to.. PREFACE

5 Page Summary. Between January 98 and st December, 8 orthotopic liver transplants (OLT) were performed in Australia and New Zealand on 8 patients, 966 adult patients [8%] and 8 children ( < 6 years) [7%]. The median age of all recipients was 8.6 years. The ages ranged from days to 7. years. There is a significant difference in gender distribution between children (M=8%) and adults (M=66%). 6. Two hundred and ninetyfive new patients were transplanted in compared with 6 in. 7. The trend to increasing age of adult recipients in recent years continued and the overall adult median age is now. years. The median age of new adult recipients in was 6. years. 89. In, there was an increase in the number of transplants with 8 more performed [6 vs 78]. Split grafts continue to make a significant contribution to the total number of paediatric transplants performed providing of 9 [6%] of deceased donor grafts in and 6 of 9 [7%] overall. In children, other reduced size grafts have been used in [%] cases including 78 living donor grafts. One child has been treated with liver cell implantation. Of adult patients, have received reduced size grafts 6 split liver grafts (including as auxiliary graft), other reduced size grafts ( as auxiliary graft) and living donor grafts. Three domino transplants of a whole liver have been performed.. Overall, chronic viral hepatitis (CVH) is the most common primary indication for liver transplantation. In children biliary atresia (BA) is the most common primary disease. In adults chronic hepatitis C [CVH : HCV] is the primary disease in.6% of recipients and chronic hepatitis B [CVH : HBV] in.9 %. Full details of specific diagnoses categories by age group are listed in the Appendices for Metabolic disorders (Appendix II), Other diseases (Appendix III) and Fulminant Hepatic Failure (Appendix IV).. The number of patients transplanted with nonalcoholic fatty liver disease [ NAFLD/NASH] as the primary diagnosis continued to increase with 8 [7%] of new patients transplanted in bringing the total to 9. The proportion of adult patients transplanted with a primary diagnosis of chronic viral Hepatitis B, C or B/C/D fell in compared with the previous eras but the number of patients with a primary diagnosis of hepatocellular carcinoma [HCC] increased and accounted for % in. The majority of these patients have a secondary diagnosis of CVH: HCV or HBV. When patients with either primary or secondary diagnosis of Hepatitis B,C or both are included, the overall incidence of CVH in new adult patients in was %. 6. Overall year patient survival of all patients is 9% at year, 8% at years and 7% at years. Children have a significantly better survival rate than adults with an actuarial survival of 7% at years posttransplant. 7. Whilst older children had superior early survival than infants and babies, long term survival is similar. Older adult recipients had poorer longer term outcomes. 89. Patient survival in later cohorts show continued improvement in outcome for the first years compared with earlier cohorts. This is seen in both children and adults. One year patient survival in cohort was 96% for all patients [% for children, 9% for adults].. In both children and adults, there are worse early outcomes in patients receiving a deceased donor reduced size graft as their primary graft compared with split liver graft or whole liver grafts. Split liver grafts and whole livers have similar early outcomes in both children and adults.. DATA TO // copyright ANZLTR Data to.. SUMMARY

6 Page Summary. Smaller children and babies weighing < 8 kg at the time of transplant had inferior early survival compared to heavier children but similar long term results.. Adult patients transplanted for biliary atresia or hepatitis virus coinfections had the best longer term survival while those whose primary disease was malignancy or Hepatitis C have significantly lower survival rates.. In children, patient survival was similar for all disease groups though lower in patients whose primary disease was malignancy. There were no differences in survival between adults and children transplanted for fulminant hepatic failure [acute and subacute] with overall year survival of 77%.. Recent cohorts of adult patients with a primary diagnosis of hepatitis B continue to show a significantly improved survival which is not seen in adult patients with hepatitis C as primary disease. Patients transplanted for malignancy continue to have a poor outcome but some improvement in longer term outcome is seen in patients transplanted since. 6. Overall graft survival was 86% at year and 77% at years with significantly better graft survival longer term in children. Graft survival was significantly worse in second grafts in both children and adults. Third grafts in adults have better outcomes than in children. 7. Overall split liver grafts have similar graft survival to whole liver grafts. Reduced grafts have lower graft survival in the early posttransplant years in both children and adults. 8. Graft survival has increased significantly over time, both for all deceased donor grafts and split liver grafts by era of transplantation. 9. Vascular complications and rejection were the commonest indications for retransplantation. Thirteen percent of retransplants were due to poor early graft function. Retransplantation for recurrent disease was most prevalent in adults [% PSC, PBC, AIH and 9% HBV, HCV].. Sepsis is the most frequent cause of death in both adults and children. Full details of Miscellaneous and Other Graft Failure deaths are listed in Appendix V. Twenty nine percent of all deaths occurred within 6 months of transplant. Deaths from early graft failure were due to poor or no early graft function. By year malignancy and graft failure from recurrent disease or chronic rejection cause most deaths. Deaths due to de novo malignancy and chronic rejection are increasing with longer survival time particularly in children surviving years or longer.. There was an increase in the number of cadaveric donors in to 88 with grafts transplanted from deceased donors. The number of livers split to produce two transplantable grafts was in. Sixteen liver grafts donated after cardiac death were transplanted. The number of people on the waiting list at December was higher than the number on the waiting list at December. 6. Donor age has increased significantly in recent years. Long term graft survival trends lower in several older donor age groups. 7. Ninetyfour patients [78 children, 6 adults] have now received a living donor graft with 6 performed in. In 88 patients the living donor graft was a primary graft, in as a second and as a third graft. The median age of the donors was. years with a range of 9. to. years. Three adult grafts were domino whole liver graft.. DATA TO // copyright ANZLTR Data to.. SUMMARY

7 Page Summary 8. Waiting list activity for shows the number of patients listed for transplantation continued to increase with 7 remaining on the waiting list at December. Patient delistings due to death, becoming too ill or tumour [HCC] progression accounted for 7.% of all delistings. Only child was delisted in these categories for tumour progression. Three hundred and sixteen patients were transplanted [%]. Forty seven patients were listed as urgent in [ with initial listing as Category and Category ]. Twenty two [88%] of Category and [9%] of Category patients had a positive outcome. 9. Median waiting times tended to be higher in in some blood groups. Blood group B patients had the longest waiting times to transplant but blood group O longest waiting time overall.. Cancer in liver transplant recipients are analysed from two perspectives. Firstly, those who had a liver cancer diagnosis at the time of transplantation (as primary, secondary or incidental) and secondly those who developed a cancer post transplantation (de novo skin and de novo non skin cancer). Overall 9* patients (%) had a liver cancer at the time of transplantation with HCC being the most common (9%). 97 patients (8%) were transplanted for liver cancer, 69 patients (%) had liver cancer as a secondary or incidental diagnosis, of which (%) were undiagnosed prior to transplantation. Three patients had both primary and secondary liver cancers and had multiple secondary or incidental liver cancers. Post transplant (%) of pretransplant cancers recurred and (.% of those with cancer at transplantation) died as a result of recurrence.. Actuarial patient survival was 9% at years in patients with primary liver cancer. Patients with a diagnosis of HCC or hepatoblastoma had the best survival rate [8% and 6%]. Those with Cholangiocarinoma had significantly poorer survival. 6. In patients with liver cancer as a secondary diagnosis, year patient survival was %. Sixtysix [% of patients] died from recurrence of their cancer. Overall patients with a diagnosis of pretransplant malignancy had worse survival than patients with benign diseases. 7. Incidence of liver cancer at time of transplantation continues to increase, climbing from 7 to 6 over the last decade. 7. Three hundred and eighty two de novo nonskin types of cancer developed in (7%) of patients. Thirty patients developed more than one de novo nonskin cancer. The three most common categories of de novo nonskin cancer were cancers of the alimentary tract (7), lymphoma (9) and genitourinary (7). Patients who develop a de novo nonskin cancer post transplant have significantly worse survival than other patients. Incidence of de novo nonskin malignancy is greatest in those with underlying hepatitis C, primary sclerosing cholangitis and alcoholic cirrhosis (p<.).. Six hundred and seventy three (%) developed a first skin cancer, with a peak of years after transplantation, with going on to develop multiple types of skin cancer. Thirty seven patients developed melanoma. The cumulative risk of diagnosis of any cancer post transplant is approximately % at years.. DATA TO // copyright ANZLTR Data to.. SUMMARY

8 Section D e m o g r a p h i c D a t a DATA TO // copyright ANZLTR Data to.. SECTION : DEMOGRAPHIC DATA

9 Cumulative Number of Patients & Transplants,, Number,,,,, Transplants (n=8) Patients (n=8),, Year of Transplant Summary Statistics Age and Gender ALL PATIENTS TRANSPLANTED Children [<6y] Adults Total Patients Age Mean ± SD. ±.y 9. ±.7y. ±.y Median.y.y 8.6y Range d.9y 6. 7.y d 7.y Gender Female (%) (%) 78 (7%) Male (8%) 6 (66%) (6%) Surviving 676 (8%) 777 (7%) (7%). DATA TO // copyright ANZLTR Data to.. SECTION : DEMOGRAPHIC DATA

10 Number of New Patients Transplanted by Year Number of Patients Cumulative Number of New Patients Transplanted Age Group 988 Children (n=8) Adult (n=96) ,,8 6. Number of Patients,6,,,,8,6,,,,8,6,,, Age Group 988 Children (n=8) Adult (n=96) Year of Transplant DATA TO // copyright ANZLTR Data to SECTION : DEMOGRAPHIC DATA

11 Number of Recipients By Age at Primary Transplant N=798,6,,,,, Median = 8.6y Range d 7.y Count, 9 8 7,6 6 9 Age at Primary Transplant by Era < y y 9y y 6 9y 9y 9y 6 6y >=6y Age Strata, Age Group Children (n=8) Median =.y [d.9y] Adult (n=96) Median =.y [6. 7.y] P<. Age at transplant 6.9y.9y 8.y.y.y.y 6.y.y.9y.y.y.y.9y.y P= Era DATA TO // copyright ANZLTR Data to.. SECTION : DEMOGRAPHIC DATA

12 Number of Transplants by Year Number of Transplants Age Group 988 Children (n=9) Adult (n=6) Cumulative Number of Transplants,,,7,, Age Group Children (n=9) Adult (n=6) Number of Transplants,,7,,,,7,,, Year of Transplant DATA TO // copyright ANZLTR Data to SECTION : DEMOGRAPHIC DATA

13 Type of Graft by Year Split vs Reduced vs Whole Number of Grafts Type of Graft Liver cells Split Split LRD Reduced Whole n= n= 6 (7%) n= 78 (8%) n= (%) n= 79 (%) Children (n = 9) Type of Graft Adults (n = 6) Number of Grafts Split Split LRD Reduced Whole * domino liver 9 n= 6 (6%) n= (.%) n= (.7 %) n= 9 (9%)* Year of Transplant DATA TO // copyright ANZLTR Data to.. SECTION : DEMOGRAPHIC DATA

14 Section P r i m a r y D i a g n o s i s DATA TO // copyright ANZLTR Data to.. SECTION : PRIMARY DIAGNOSIS

15 Primary Diseases of All Recipients CVH : HCV 8.7% CVH : HBV.8% CAH : AI.% OTH 6.% NAFLD.7% FHF 9.6% MAL 9.% CVH : B/C/D.% BA.% MET 6.% ALD.% CC.% PBC.7% PSC 8.8% Diagnosis Group BA MET ALD CC PBC PSC MAL FHF NAFLD OTH CAH : AI CVH : HBV CVH : HCV CVH : B/C/D Biliary atresia Metabolic diseases Alcoholic cirrhosis Cryptogenic cirrhosis Primary biliary cirrhosis Primary sclerosing cholangitis Malignancy Fulminant hepatic failure Nonalcoholic fatty liver disease Other diseases * Chronic active hepatitis [autoimmune] Chronic viral hepatitis B Chronic viral hepatitis C Chronic viral hepatitis B / C / D * * * See Appendices for details. DATA TO // copyright ANZLTR Data to.. SECTION : PRIMARY DIAGNOSIS

16 Primary Diseases of Children n =8 OTH.9% FHF.8% CAH:AI.% MAL.% PSC.% CC.% MET.% Primary Diseases of Adult Recipients n = 966 CVH : HCV.6% CVH : HBV.9% CAH:AI.8% OTH.% NAFLD.% CVH : B/C/D.% FHF 9.% MAL.% Diagnosis Group BA.% BA % MET.% ALD.% CC.% PBC.7% PSC.% BA MET ALD CC PBC PSC MAL Biliary atresia Metabolic diseases Alcoholic cirrhosis Cryptogenic cirrhosis Primary biliary cirrhosis Primary sclerosing cholangitis Malignancy FHF NAFLD OTH CAH : AI CVH : HBV CVH : HCV CVH : B/C/D Fulminant hepatic failure Nonalcoholic fatty liver disease Other diseases Chronic active hepatitis [autoimmune] Chronic viral hepatitis B Chronic viral hepatitis C Chronic viral hepatitis B / C / D. DATA TO // copyright ANZLTR Data to.. SECTION : PRIMARY DIAGNOSIS

17 Primary Diseases Percent of New Patients 7% 6% % % % % Children (n=8) Era 9889 (n=7) 999 (n=) 9999 (n=7) (n=) 9 (n=) (n=97) (n=9) % % Biliary Atresia Metabolic diseases Cryptogenic cirrhosis PSC Malignancy FHF Other diseases CAH : AI Percent of New Patients % % % % Adults (n = 966) Era 9889 (n=) 999 (n=) 9999 (n=8) (n=78) 9 (n=8) (n=) (n=) % %. % BA MET ALD CC PBC PSC MAL FHF NAFLD OTH CAH: CAH: CAH: CVH: AI HBV HCV Mixed Primary Diagnosis Group DATA TO // copyright ANZLTR Data to.. SECTION : PRIMARY DIAGNOSIS

18 Adult Primary Diagnosis by Era (n=) 99 9 (n) (n=8) % 7% % 6% % 9% 7% 8% % 7% 8% % % 9% % % (n=78) 9 (n=8) (n=) % 8% % % % % % % 7% 8% % 8% % % 8% % % % 6% % % (n=) % % Adult Diagnosis 7% 9% Other diseases ALD Hep B Hep B/C/D % HCC NAFLD/NASH % Hep C 7%. DATA TO // copyright ANZLTR Data to.. SECTION : PRIMARY DIAGNOSIS

19 Adult Primary Diagnosis by Year Count Primary Diagnosis Hep B/C/D Hep B Hep C HCC ALD NAFLD/NASH 6 67 Other diseases Year of Transplant. DATA TO // copyright ANZLTR Data to.. SECTION : PRIMARY DIAGNOSIS

20 Chronic Viral Hepatitis as Primary or Secondary Diagnosis in Adult Patients Primary Diagnosis Hepatitis C Hepatitis B 9 6 Hepatitis 7 BD/BC/BCD n = Hepatitis C Hepatitis B Secondary / Tertiary diagnosis Hepatitis B,C HCC + cirrhosis ALD 9 6 HCC NAFLD ALD NAFLD 9 9 Other TOTAL 966 Type of Chronic Viral Hepatitis in Adult Patients % 6% 6% % % 6% % 6% % % % 68% 7% 8% 76% 9999 [n=9] [n=] 9 [n=68] Era [n=6] [n=6] Hepatitis diagnosis Count Chronic viral hepatitis Hepatitis B Hepatitis C Mixed B/C/D Hepatitis Negative Year of Transplant DATA TO // copyright ANZLTR Data to.. SECTION : PRIMARY DIAGNOSIS

21 Section P a t i e n t S u r v i v a l DATA TO // copyright ANZLTR Data to.. SECTION : PATIENT SURVIVAL

22 Patient Survival Post Transplant 9 N = 798 Median = 6.7y Range (.8y) 8 Patient Survival (%) 7 6 y y y y y y y No. at risk Actuarial Survival (%) 9% 8% 8% 7% 6% % 9% y 8% % P< % 87% 8% 8% 8% 8% 8% 77% 7% 7 7% Patient Survival (%) 6 Age group 6% 9% % Children (n=8) Median 8.y (.8y) Adult (n=966) Median 6.y (9.y) Time Posttransplant (years) DATA TO // copyright ANZLTR Data to.. SECTION : PATIENT SURVIVAL

23 Patient Survival by Age at Primary Transplant 9 Children n= 8 p =ns 8 Patient Survival (%) 7 6 Age Strata y y y y y y y <y (n=9) y (n=7) 9y (n=9) 87% 88% 9% 8% 8% 9% 8% 8% 88% 8% 78% 8% 8% 76% 8% 78% 7% 8% y (n=7) 9% 9% 9% 87% 8% 77% 7% 7% 78% 7% Adults n = 966 p =. 8 Patient Survival (%) 7 6 Age Strata y y y y y y y 69y (n=79) 9y (n=) 9y (n=6) 66y (n=89) >=6y (n=6) 89% 87% 8% 77% 69% 9% 9% 8% 8% 7% 6% % 9% 8% 8% 7% 7% % 89% 8% 79% 68% % % 86% 8% 7% 6% % 7% % 6% % Time Posttransplant (years) DATA TO // copyright ANZLTR Data to.. SECTION : PATIENT SURVIVAL

24 All Patient Survival by Year of Transplant Patient Survival (%) % 9% 9% 9% 86% 8% 7% 87% 8% 8% 79% 77% 6% Era 9889 (n=) 999 (n=) 9999 (n=697) (n=86) 9 (n=96) (n=8) (n=9) 78% 76% 7% 67% % 6% 6% 6% 7% % % % p <. 6% 6% % Time Posttransplant (years) 8. DATA TO // copyright ANZLTR Data to.. SECTION : PATIENT SURVIVAL

25 Patient Survival Children 9 % 9% 9% 9% Children n = 8 9% 9% 9% 86% 8% 8% p <. Patient Survival (%) % 86% 69% 8% 8% 6% Era 8% 78% 78% 77% 6% 6% 7% 7% % 7% 8% 9889 (n=7) 999 (n=) 9999 (n=7) (n=) 9 (n=) (n=97) (n=9) Patient Survival Adults 9 9% 9% 9% 9% 86% 8% 8% Adults n = 966 p <. 8 87% 8% 7% 7% Patient Survival (%) 7 6 7% 78% 7% 9% Era 9889 (n=) 999 (n=) 9999 (n=8) (n=78) 9 (n=8) (n=) (n= ) 69% 6% 9% 6% 9% % % % % % 7% 9% Time Posttransplant (years) DATA TO // copyright ANZLTR Data to.. SECTION : PATIENT SURVIVAL

26 Patient Survival by Type of Primary Graft [Deceased donors] Patient Survival (%) % 9% 8% 9% 88% 78% Children n = 79 88% 8% 8% 8% 76% 7% 8% 78% 7% p =. 76% 6% Type of Graft Reduced (n=8) Split (n=) Whole (n= ) % Adults n = 9 P = NS % 8% 76% Patient Survival (%) % 8% 68% 7% 6% 6% 6% % % % 8% 6% % Type of Graft Reduced (n=8) Split (n=) Whole (669) Time Posttransplant (years) DATA TO // copyright ANZLTR Data to.. SECTION : PATIENT SURVIVAL

27 Patient Survival by Weight Children Patient Survival (%) 9% 9 87% 8% 79% 8 78% 78% 7 6 8% 78% 8% 78% 78% 77% 76% 77% 76% P = NS 7% 7% Weight Group > 8 kg (n=6) 8 kg (n=8) < kg (n=9) Time Posttransplant (years). DATA TO // copyright ANZLTR Data to.. SECTION : PATIENT SURVIVAL

28 Patient Survival by Primary Disease 9 () Adults [excluding FHF] n = 8 p = NS 8 Patient Survival (%) 7 6 Diagnosis Group n = y y y y Biliary atresia 86% 8% 8% 78% Alcholic cirrhosis 9 8% 7% 8% 7% Metabolic diseases 7 8% 76% 6% % CAH: AI 8% 7% 9% 7% Cryptogenic cirrhosis 78 8% 69% 7% 6% Other 8% 7% 7% % y 78% 9% % 9% 6% 6% 9 () Adults [excluding FHF] n = 8 p =. 8 Patient Survival (%) 7 6 Diagnosis Group n = y CVH : B,C,D 9% NAFLD 9 8% CVH : Hep B 8% CVH : Hep C 898 8% PBC 7 8% PSC 8% Malignancy 7 7% y y 9% 87% 7% 7% 79% 67% 6% % 7% 66% 7% 6% 6% 6% y 87% 8% % % 8% % y 8% 7% % % % Time Posttransplant (years) DATA TO // copyright ANZLTR Data to.. SECTION : PATIENT SURVIVAL

29 Patient Survival by Primary Disease () Paediatric recipients [excluding FHF] n = 7 p = NS 9 8 Patient Survival (%) 7 6 Diagnosis Group n = y y y y Biliary atresia 86% 8% 8% 77% Autoimmune hepatitis 9 % % 7% 7% Cryptogenic cirrhosis 8 88% 8% 8% 6% Malignancy 9 7% 9% % % Metabolic diseases 89% 8% 8% 79% Other 7 89% 88% 8% 8% PSC 8 88% 88% 88% 88% y 76% 6% 67% 8% 88% () Fulminant hepatic failure n = 9 Patient Survival (%) % 8% 79% 77% 77% 76% 7% 7% 7% 7% 67% 66% 6% 8% 6% p = NS 6% % 7% Age group Adult (n=69) Child (n=9) All Patients (n=9) Time Posttransplant (years) DATA TO // copyright ANZLTR Data to.. SECTION : PATIENT SURVIVAL

30 Patient Survival by Primary Disease Patient Survival (%) % 9% 8% 8% 6% % Adults CVH: Hepatitis B n = 9% 9889 (n=9) 999 (n= 6) 8% 9999 (n= ) 67% (n= 9) 79% 9 (n= 8) 6% (n= ) (n= ) 6% % % Era 9 % % Adults CVH: Hepatitis C n = 898 p < P = NS Patient Survival (%) Era n = y 7% 8% 7% 8% 77% 8% y y % % 9% 8% 6% 8% 7% % 68% y % 9% % Patient Survival (%) Malignancy Adults and Children n=6 Adults (n = 7) Children (n=9) Era p < (n=) 999 (n=) 9999 (n=) (n=6) 9 (n=) (n=6) (n=6) Time Posttransplant (years) DATA TO // copyright ANZLTR Data to.. SECTION : PATIENT SURVIVAL

31 Section G r a f t O u t c o m e DATA TO // copyright ANZLTR Data to.. SECTION : GRAFT OUTCOME

32 Graft Survival All Grafts 9 N = Graft Survival (%) Graft Survival (%) 6 Graft Survival by Age Group % 8% y y y y y y y No. at risk Actuarial Survival (%) 77% 76% 86% 8% 77% 68% 9% 9% % Time Posttransplant (years) 7% 67% 69% 7% 6% % 8% P<. 7% Age group Children (n=9) Adult (n=6) Time Posttransplant (years) DATA TO // copyright ANZLTR Data to.. SECTION : GRAFT OUTCOME

33 Graft Survival by Graft Number All Grafts (n= 79) Children (n= 9) Graft Survival (%) Graft Survival (%) % 77% 7% 8% 67% 66% 78% 67% 6% Graft Number 69% 6% % st Grafts (n=797) [9.6%] nd Grafts (n=6) [6.7%] rd Grafts (n=6) [.7%] 78% 9% 7% Graft Number 7% 9% 7% st Grafts (n=8) [88%] nd Grafts (n=96) [.%] rd Grafts (n=) [.6%] 6% 6% % 7% % % % 66% 8% % 6% p < p < % 9% % Adult (n= 6) % 8% 78% 7% 7% 6. Graft Survival (%) % 7% 66% Graft Number 68% % st Grafts (n=96) [9.6%] nd Grafts (n=) [6%] rd Grafts (n=) [.%] 7% 7% Time Posttransplant (years) DATA TO // copyright ANZLTR Data to.. % 7% 7% 7% p < SECTION : GRAFT OUTCOME

34 Graft Survival by Type of Graft [Deceased Donors] All Grafts (n= 8) Children (n= 86) Graft Survival (%) Graft Survival (%) % 86% 7% 88% 88% 7% 78% 78% 66% Type of Graft Split (n=) Reduced (n=) Whole (n=8) 8% 8% 67% Type of Graft Split (n=6) Reduced (n=) Whole (n=79) 68% 68% 6% 78% 7% 6% 6% 6% 9% 77% 66% 6% 7% % 7% 68% 9% 6% % % 6% % P = NS P= % Adult (n= ) Graft Survival (%) % 8% 6% 77% 76% 6% Type of Graft Split (n=6) Reduced (n=) Whole (n=99) 67% 6% 8% 7% % % % % % 7% P = NS Time Posttransplant (years) DATA TO // copyright ANZLTR Data to.. SECTION : GRAFT OUTCOME

35 All Deceased Donor Grafts Survival by Year of Transplant 9 9% 9% 9% 88% 8% 8% 79% p <. Graft Survival (%) Graft Survival (%) Deceased Donor Split Liver Grafts by Era % 78% 6% Era 9889 (n=) 999 (n=97) 9999 (n=78) (n=99) 9 (n=99) (n=9) (n=) % 9% 89% 78% 7% 7% 7% % Era 8% 8% 7% 7% 9999 (n=6) (n=8) 9 (n=) (n=8) (n=) 7% 7% 6% 6% 6% 9% 7% % % 6% % % Time Posttransplant (years) 7% 6% 6% 6% % % 9% p =.8 % Time Posttransplant (years) DATA TO // copyright ANZLTR Data to.. SECTION : GRAFT OUTCOME

36 Indication for Retransplantation n = 8 ( nd grafts, 6 rd grafts) % 8% 8% 6% % 9% % Recurrent PBC/PSC/CAH:AI Recurrent HBV /HCV Rejection PNF/poor graft function Biliary Other Vascular Age Group Children (n= ) % Adults (n= 9) 6% % % 8% % % 9% 8% 6% % % % 9. DATA TO // copyright ANZLTR Data to.. SECTION : GRAFT OUTCOME

37 Indication for Retransplantation n = 8 ( nd grafts, 6 rd grafts) Number of Grafts d 8d m m 6m 6m y y y yy >y N = (%) (%) 6 (7%) (9%) 8(%) (%) (%) Recurrent PBC/PSC/CAH:AI Rejection PNF/poor graft function Other Recurrent HBV /HCV Vascular Biliary Children (n=) Adults (n=9) 7d 8d m m 6m 6m y y y y y >y 7d 8d m m 6m 6m y y y y y >y N = (%) (%) (%) (8%) (6%) (%) (%) N = (%) (%) (%) (%) (9%) (%) (9%). DATA TO // copyright ANZLTR Data to.. SECTION : GRAFT OUTCOME

38 Section Cause of Patient Death DATA TO // copyright ANZLTR Data to.. SECTION : CAUSE OF PATIENT DEATH

39 Causes of Death All Patients n = 7 Operative Respiratory Cerebrovascular Cardiovascular Gastrointestinal Sepsis Malignancy recurrent Malignancy de novo Graft failure Recurrent HBV / HCV Miscellaneous * Rejection Other * * See Appendix V for details 6% % 6% 7% 9% 8% % % 8% % % %. DATA TO // copyright ANZLTR Data to.. SECTION : CAUSE OF PATIENT DEATH

40 Causes of Death in Children n =6 % % % % 9% 7% % 9% 6% % 9% Causes of Death in Adult n = 9 % 7% 6% 6% 8% 8% % % 9% % % % Operative Gastrointestinal Graft failure Recurrent HBV / HCV Respiratory Cerebrovascular Cardiovascular Sepsis Malignancy recurrent Malignancy de novo Miscellaneous * Rejection Other *. DATA TO // copyright ANZLTR Data to.. * See Appendix V for details SECTION : CAUSE OF PATIENT DEATH

41 Cause of Death by Time Post Transplant % 9% 8% 7% 6% % % % % % % 7d 8 d m m6m 6m y yy yy y y >y N = 9 (%) (8%) 8 (%) (7%) 7 (%) 8 (8%) 7 (%) (%) Operative Respiratory Cerebrovascular Cardiovascular Gastrointestinal Sepsis Malignancy de novo Malignancy recurrent Recurrent HBV / HCV Rejection Other [graft failure] Miscellaneous * * * See Appendix V for details. DATA TO // copyright ANZLTR Data to.. SECTION : CAUSE OF PATIENT DEATH

42 Cause of Death by Time Post Transplant % 9% Children (n=6) 8% 7% 6% % % % % % % 7d 8 d m m6m 6m y yy >yy y y >y N = (6%) 6 (7%) 8 (8%) 9 (6%) 9 (9%) 6 (%) 6 (%) 7 (%) Cause of Death by Time Post Transplant Adult (n=9) % 8% 6% % % % 7d 8 d m m6m 6m y yy >yy y y >y N = (9%) 8 (7%) (%) 9 (8%) 78 (%) (9%) 66 (%) 6 (%) Operative Respiratory Cerebrovascular Cardiovascular Gastrointestinal Sepsis Malignancy de novo Malignancy recurrent Recurrent HBV / HCV Rejection Other [graft failure] Miscellaneous. DATA TO // copyright ANZLTR Data to.. SECTION : CAUSE OF PATIENT DEATH

43 Section 6 Deceased Donor Information DATA TO // copyright ANZLTR Data to.. SECTION 6 : DECEASED DONOR INFORMATION

44 Deceased Donation by Year QLD NSW/ACT VIC/TAS SA/NT WA NZ TOTAL 6 8 / 9/ / 6 9/ 66 8 / / / 9 6/ 6/ 8/ /8 /6 8/ 7 /7 9/ / 9 6 /7 / /6 8 66/ /7 / 8 /7 6/8 7/ 8 7/8 /7 6/ 88 Grafts from deceased donors Number Year Cadaveric donors Grafts transplanted Waiting list Dec DCD. DATA TO // copyright ANZLTR Data to.. SECTION 6 : DECEASED DONOR INFORMATION

45 Donor Age by Era N = N = 698 Median Age:.y Range: d 8.y P <. 7 Graft Survival (%) Donor Age (years) 6.y Graft Survival by Donor Age N = y n = y Donor age group.y y y 6y 66y >6y n= 8 n= 7 n= 68 n= 78 n= 7 n=.y Era.9y 9 6.y 7.y 88 P < Time Posttransplant (years) DATA TO // copyright ANZLTR Data to SECTION 6 : DECEASED DONOR INFORMATION

46 Section 7 L i v i n g D o n o r T r a n s p l a n t a t i o n DATA TO // copyright ANZLTR Data to.. SECTION 7 : LIVING DONOR TRANSPLANTATION

47 Living Donor Transplantation N = 9 Donor gender Male Female Donor age Median Range Donor relationship Mother Father Son Daughter Grandmother Grandfather Sister Brother Aunt Uncle Family friend Cousin Spouse Child [n=78].y 9..y * x whole liver domino transplant Recipient Age Group 8 6 Adult [n=6] 6.y.8.y * All [n=9].8y 9.y Survival (%) 6 All Patients (n= 88) (88 patients received LD graft as first graft) All Grafts (n= 9) o nd rd [88 x, x, x, ] Paediatric Patient Survival (n= 7) Adult Patient Survival (n= ) Time Posttransplant (years) DATA TO // copyright ANZLTR Data to.. 7 SECTION 7 : LIVING DONOR TRANSPLANTATION

48 Section 8 W a i t i n g L i s t DATA TO // copyright ANZLTR Data to.. SECTION 8 : WAITING LIST

49 Waiting List Activity [ Data // //] Activity Listed at January Activated TOTAL Adult Paediatric TOTAL OUTCOME OUTCOME Transplant [9%] 68 [%] 8 [%] 78 [9%] 6 [%] 7 [%] 6 [6%] Delisted Died on list Too sick Tumour progression Improved Other 8 [%] 7} 8% 9 * 89 [%] 9 6} 8% 7 7 * 98 [8%] 6 } % 6 * 89 [6%] 8 } 8% 8 8* [%] } [ 7.% ] 7 * } 9% }.% Active at Dec 9 [6%] 86 [%] 6 [%] [6%] 7 [%] 86 [* Patient declined, malignancy, drug use, infection, temporary delist for further investigations, medical] Outcome of Initial Urgent Listing CATEGORY OUTCOME (n=) (n=6) (n=9) (n=8) N= Adult n= Paediatric n= TRANSPLANTED IMPROVED } 8% } 8% } 7% 6 } 88% }88% 9 DIED / TOO SICK OTHER TREATMENT CATEGORY OUTCOME (n=8) (n=9) (n=9) (n=) N= Adult n=9 Paediatric n= TRANSPLANTED IMPROVED } } 86% } 89% 8 } 9% }9% 8 DIED / TOO SICK OTHER TREATMENT active // active // active // active // active // active // 8. DATA TO // copyright ANZLTR Data to.. SECTION 8 : WAITING LIST

50 Outcome by Blood Group Blood Group A O B AB TOTAL n= Not transplanted (7%) * 7 (%) 9 (%) 6 (%) Transplanted 9 (8%)** (%) (%) (7%) 6 (%) * % of total number listed ** % of blood group Waiting Time to Transplant 8 6 P= NS 8 6 Waiting Time (Months) Median 69d d 8d d n= 9 (%) (%) (6%) (9%) A AB B O Blood Type 9. DATA TO // copyright ANZLTR Data to.. SECTION 8 : WAITING LIST

51 Waiting Time by Outcome Waiting Time (Months) Median d Listed pre ' Transplant Waiting Time by Outcome & Blood Group d Listed pre ' Delisted Listed pre ' Waiting 6d Listed Transplant Patient Outcome Listed Delisted Median waiting time to transplant = 8d (7m) 8d Median waiting time listed patients // = d (dm) 6d Listed Waiting 98d. Waiting Time (Months) Patient Outcome Transplanted Not transplanted Median= 8d d 6d d A Blood Type DATA TO // copyright ANZLTR Data to.. AB B O SECTION 8 : WAITING LIST

52 Section 9 L i v e r T r a n s p l a n t a t i o n a n d C a n c e r DATA TO // copyright ANZLTR Data to.. SECTION 9 : LIVER TRANSPLANTATION AND CANCER

53 Cancer in Liver Transplant Recipients n = 8 At Tx Liver Cancer as indication for Transplant 97 (8%) Ca Total number pts. transplanted = 8 Liver Ca as a Secondary Diagnosis 69 (%) 66 Ca Total 9* (%) Post Tx Recurrent Liver Ca (% of all pts, % pts with Ca at Tx) De Novo Ca (7%) 8 Ca Skin Ca 677 (%) Total 9 (%) Multiple Cancer types (non skin and skin) (7% of all pts) Multiple non skin cancers 87 (% of all pts) Transferred from Donor Developed non skin Ca < 9days 8 * pts had primary and a secondary liver cancer; pts had multiple secondary liver cancers Liver Cancer as Primary Diagnosis n = 97/8 (8%) TYPE OF CA No DIED DIED OF THIS CA HEPATOCELLULAR CA 78 (%) HEPATOBLASTOMA (6%) FIBROLAMELLAR 7 (9%) CARCINOID (%) EPITHELOID HAEMANGIOENDOTHELIOMA HEPATOCELLULAR MALIGNANT NEOPLASM CHOLANGIOCARCINOMA (%) ANGIOSARCOMA (%) GASTRINOMA (%) PANCREATIC ISLET CELL (%) ERYTHROID LEUKAEMIA (%) TOTALS * (8% of pts) 98 (% of those with PCa) 6 (% of those with PCa) * pts had two primary liver cancers. DATA TO // copyright ANZLTR Data to.. SECTION 9 : LIVER TRANSPLANTATION AND CANCER

54 Overall Survival Primary Liver Cancer n =97/8 (8% of pts transplanted) 8 % Surviving 6 y y y y No at risk 9 6 Actuarial Survival % Primary Liver Cancer Incidence n=97/8 Years Post Tx DATA TO // copyright ANZLTR Data to.. SECTION 9 : LIVER TRANSPLANTATION AND CANCER

55 Overall Survival Primary Liver Cancer n=97/8 (8%) % Surviving 8 6 p<. HCC (n=) Hepatoblastoma (n=) Fibrolamellar (n=7) CC (n=) Other (n=) Years Post Tx Primary Liver Cancer Actuarial Survival Summary n = 97/8 HCC (n=) Hepatoblastoma (n=) Other (n=) Fibrolamellar (n=7) CC (n=) yr yr yr yr yr yr n % n % n % 8 n 7 6 % n %. DATA TO // copyright ANZLTR Data to.. SECTION 9 : LIVER TRANSPLANTATION AND CANCER

56 Liver Cancer as a Secondary Diagnosis n = 69/8 (% pts) No Died Died of This Cancer HEPATOCELLULAR CA* 6 9 (7%) CHOLANGIO CA 9 9 (8%) OTHER 8 (%) FIBROLAMELLAR HEPATOBLASTOMA* Total 66* in 69 pts (%) 96 (% of pts with SCa) 66 (% of pts with SCa) * patients had secondary cancers Overall Survival Liver Cancer as a Secondary Diagnosis n=69/8 pts (%) 8 % Surviving 6 y y y y No at risk Actuarial Survival % Years Post Tx. DATA TO // copyright ANZLTR Data to.. SECTION 9 : LIVER TRANSPLANTATION AND CANCER

57 Liver Cancer as a Secondary Diagnosis n =69/ 8 (%) p<. % Surviving 8 6 Hepatoblastoma (n=) HCC (n=6) Fibrolamellar (n=) Other (n=8) CC (n=9) Years Post Tx Secondary Liver Cancer Actuarial Survival Summary n =69/ 8 (%) HCC (n=6) CC (n=9) Other (n=8) Fibrolamellar (n=) Hepatoblastoma (n=) yr yr yr yr yr n % n 8 % n 8 6 % 88 6 n % n % DATA TO // copyright ANZLTR Data to.. SECTION 9 : LIVER TRANSPLANTATION AND CANCER

58 Liver Cancer (Primary or Secondary Diagnosis) n = 9/8 (%) n = 9/8 (%) TYPE OF CA No. DIED DIED OF THIS CA HEPATOCELLULAR CA* (9%) CHOLANGIOCARCINOMA* (9%) HEPATOBLASTOMA* 8 6 (%) FIBROLAMELLAR (%) CARCINOID (%) ADENOCARCINOMA (%) EPITHELOID HAEMANGIOENDOTHELIOMA 6 ANGIOSARCOMA (%) GASTRINOMA (%) PANCREATIC ISLET CELL (%) ERYTHROID LEUKAEMIA (%) HEPATOCELLULAR MALIGNANT NEOPLASM (NOS*) TOTALS 9* Ca in 6 pts (% of pts) 9 (8%of those with Ca) (% of those with Ca at Tx) * patients had secondary cancers; patients had a primary and secondary cancer Patient Actuarial Survival Benign Disease vs Pre Transplant Liver Malignancy n = 8 8 % Surviving Benign disease (n=7) Malignant disease (n=9) 6 Benign Disease Malignant Disease n % Surviving n % Surviving y y y y y y Years Post Tx DATA TO // copyright ANZLTR Data to.. SECTION 9 : LIVER TRANSPLANTATION AND CANCER

59 Liver Cancer at Transplantation n = 9/8 (%) HCC (n=96) 67 Other (n=*) * patients had secondary cancers; patients had a primary and secondary cancers De Novo Non Skin Cancer n = /8 (7%) No Male Female Age of pts (yrs) Time to diagnosis (mths) Died of This Cancer Alimentary* (m 9) 77 (m 77) 6 (%) Lymphoma* 9 7 (m 9) 8 (m 6) (7%) Genitourinary* (m 6) (m 7) (9%) Breast (m ) 8 (m 98) (7%) Respiratory (m 6) 7 (m 9) 6 (7%) Endocrine 6 7 (m 6) (m 8) (%) CNS (m 66) (m 9) 6 (86%) Kaposi s 6 (m 8) 9 (m 7) Leukaemia 66 (m ) 6 7 (m 7) Miscellaneous 6 7 (m 68) 6 (m ) (%) Total *8 ca in pts 7 8 (m 8) (m 7) 7 (% of pts with Ca) * 9 patients had more than de novo cancer m=median 7. DATA TO // copyright ANZLTR Data to.. SECTION 9 : LIVER TRANSPLANTATION AND CANCER

60 Time to De Novo Non Skin Cancer n = 8 8 cancers in pts (7% of all pts) Alimentary tract Breast CNS Endocrine Genitourinary Kaposi's sarcoma Leukaemia Lymphoma Miscellaneous Respiratory m m y y y y >y De Novo Non Skin Cancer vs All Patients n =8 8 % Surviving Other Pts (n=8) De Novo (n=) p<. 6 All Pts De Novo y y y y n % Surviving n 78 % Surviving Years Post Tx 8. DATA TO // copyright ANZLTR Data to.. SECTION 9 : LIVER TRANSPLANTATION AND CANCER

61 De Novo Non Skin Cancer n = /8 (7%) Miscellaneous % Respiratory % Lymphoma % Upper GI % Alimentary, 9% Leukaemia % Kaposi's sarcoma % Genitourinary % Breast 8% Lower GI % Pancreas % Endocrine % CNS % Pre Transplant Liver Disease and De Novo Non Skin Cancer n = /8 pts (7%) HCV Alcohol PSC HBV PBC Other Autoimmune Metabolic Alimentary Tract Breast CNS Endocrine Genitourinary Kaposi's sarcoma Leukaemia Lymphoma Other Respiratory Biliary Atresia Cryptogenic DATA TO // copyright ANZLTR Data to.. SECTION 9 : LIVER TRANSPLANTATION AND CANCER

62 Pre Transplant Primary Disease and De Novo Non Skin Cancer n = ( Ca)/6 pts (7%) 9 HCV 87/8 % of de novo Ca Alcohol 8/8 % of de novo CA 7 6 Leukaemia Other CNS Breast Lymphoma Genitourinary Respiratory Alimentary Tract Merkel Cell CNS Leukaemia Kaposi's Breast Genitourinary Lymphoma Respiratory Alimentary Pre Transplant Primary Liver Disease and De Novo Non Skin Cancer n = (8 Ca)/8 pts (7%) 6 Genitourinary Cancers (7 Ca)/ (% de novo Ca) Genitourinary Cancers Time to Diagnosis (7ca)/ (% de novo pts) >yrs yrs yrs yrs mths <mths HBV AI CC Fulminant Malignancy Other PBC Metabolic PSC HCV Alcohol. DATA TO // copyright ANZLTR Data to.. SECTION 9 : LIVER TRANSPLANTATION AND CANCER

63 De Novo Non Skin Cancer Genitourinary Tract Incidence n = 7/8 cancers (%) 7 Prostate Kidney Cervix Bladder Testis Fallopian Uterus Tube Urethra Vagina Pre Transplant Primary Disease and De Novo Non Skin Cancer n = (8 Ca)/8 pts (7%) Alimentary Cancers 7/8 (6% de novo ca) 6 Alimentary Cancers Time to Diagnosis 7/8 (6% de novo ca) >yrs yrs yrs yrs mths <mths Other CC Fulminant Metabolic AI PBC Malignancy HBV Alcohol HCV PSC. DATA TO // copyright ANZLTR Data to.. SECTION 9 : LIVER TRANSPLANTATION AND CANCER

64 De Novo Non Skin Cancer Alimentary Tract Incidence n = 7/8 cancers (6%) Colon Head and neck Stomach Liver Pancreas Rectum Appendix De Novo Non Skin Cancer Respiratory Cancer Incidence Respiratory cancers /8 cancers (9%) Respiratory cancers /8 cancers (9%) 7 >yrs yrs yrs yrs mths PBC HBV AI Fulminant Other Metabolic Malignancy Alcohol HCV. DATA TO // copyright ANZLTR Data to.. SECTION 9 : LIVER TRANSPLANTATION AND CANCER

65 De Novo Non Skin Cancer Respiratory Cancer Incidence Respiratory cancers /8 cancers (9%) 6TH Lung Larynx Glottis Time to Melanoma Skin Cancer Development Post Tx. n =8 7 (.8% of all pts) Melanoma 8 6 m Yrs yrs yrs >yrs. DATA TO // copyright ANZLTR Data to.. SECTION 9 : LIVER TRANSPLANTATION AND CANCER

66 Time to st Skin Cancer Development 67/8 9 (% of all pts) BCC Melanoma Other SCC 6TH Time to Multiple Skin Cancer Development /677*/8 7 (7% of all pts) 6 m m yr yr y >y BCC Melanoma Other SCC m m y y y >y 677 pts developed skin cancer post Tx. have multiple skin cancer types. Cumulative Risk of Diagnosis of Cancer Following Liver Tx. 98 Patients at Risk (8) 8 6 Any Ca Skin Non skin (includes recurrent primary and secondary liver cancer) De Novo Non skin AgeMatched Gen. Pop. Years Post Tx DATA TO // copyright ANZLTR Data to.. SECTION 9 : LIVER TRANSPLANTATION AND CANCER

67 Appendix I Liver Transplant Units of Australia and New Zealand Australian National Liver Transplant Unit Royal Prince Alfred Hospital Missenden Road CAMPERDOWN NSW pamela.dilworth@sswahs.nsw.gov.au and The Children's Hospital at Westmead Hawkesbury Road WESTMEAD NSW Victorian Liver Transplantation Unit The Austin Hospital Studley Road HEIDELBERG VIC 8 and The Royal Children's Hospital Flemington Road PARKVILLE VIC Queensland Liver Transplant Service Princess Alexandra Hospital Ipswich Road WOOLLOONGABBA QLD and The Lady Cilento Children's Hospital Stanley Street SOUTH BRISBANE QLD South Australian Liver Transplant Unit Flinders Medical Centre Flinders Drive BEDFORD PARK SA WA Liver Transplantation Service Sir Charles Gairdner Hospital Verdun Street NEDLANDS WA 69 New Zealand Liver Transplant Unit Auckland City Hospital Park Road Auckland New Zealand and Starship Children s Hospital Park Road AUCKLAND New Zealand. DATA TO // copyright ANZLTR Data to.. APPENDIX

68 Appendix II ANZLTR PRIMARY Diagnosis Metabolic disorders by Age Group Primary Diagnosis Child Age group Adult Total Antitrypsin deficiency 9 9 CriglerNajjar Familial amyloid polyneuropathy Glycogen storage disease 6 Haemochromatosis 9 Homozygous Hypercholesterolemia 7 9 Idiopathic copper toxicosis Indian childhood cirrhosis Other * 7 Primary hyperoxaluria Tyrosinemia Urea cycle disorders** Wilsons disease 8 8 Total 7 9 * ** Maple syrup urine disease Amyloidosis Bile acid Transport disorder Protein C deficiency Propionic acidemia Methylmalonic acidemia Familial immunodeficiency Mitochondrial disease Porphyria OTC deficiency Citrullinemia Argininosuccinic aciduria Carbamyl phosphate synthetase deficiency 6. DATA TO // copyright ANZLTR Data to.. APPENDIX

69 Appendix III ANZLTR PRIMARY Diagnosis Other by Age Group Primary Diagnosis Child Age group Adult Total Alagille syndrome 7 8 Alagille nonsyndromic Benign liver tumour Adenomatosis Benign liver tumour Hemangioma Caroli's disease / congenital hepatic fibrosis Choledocal cyst Cholestatic diseaseother Chronic Budd Chiari Congenital biliary fibrosis Ductopenia Granulomatous hepatitis / sarcoidosis Histiocytosis X 6 Liver Trauma Neonatal hepatitis Nodular regenerative hyperplasia 6 6 Polycystic Liver disease Polycystic liver and kidney disease 7 Progressive familial intrahepatic cholestasis(pfic) 7 Secondary biliary cirrhosis 6 9 Secondary biliary cirrhosis Hepatolithiasis Secondary biliary cirrhosis Cystic fibrosis # Other specify 6 Total 7 7. # Vanishing bile duct syndrome Haemangiotelangiectasia Venoocclusive disease Chronic Active Hepatitis A Noncirrhotic portal hypertension KassabachMerritt syndrome Arterialvenous malformation Hereditary haemorrhagic telengectasia / OWRD Oriental cholangio hepatitis DATA TO // copyright ANZLTR Data to.. COACH syndrome Biliary sclerosis Cornelia De Lange Syndrome Hepatic Lymphangiomatosis APPENDIX

70 Appendix IV ANZLTR PRIMARY Diagnosis Fulminant Hepatic Failure by Age Group Primary Diagnosis Children Age group Adult Total Acute Budd Chiari Acute Wilson's 8 7 Acute AAT Acute Autoimmune hepatitis 8 8 Acute Unknown / unspecified 7 9 Acute Paracetamol 8 Acute Other drugs 6 9 Acute Herbs / mushrooms Acute Hepatitis A Acute Hepatitis B Acute Non AG Acute Hepatitis E Acute Post liver resection/trauma Subacute Budd Chiari Subacute Wilson's 7 Subacute Autoimmune hepatitis 8 Subacute Drug / Herbs 6 Subacute Unknown / unspecified 7 Subacute Hepatitis A Subacute Hepatitis B Subacute Non AG Total DATA TO // copyright ANZLTR Data to.. APPENDIX

71 Appendix V ANZLTR Causes of Patient death Graft failure other Children Age group Adult Total Vascular thrombosis 8 6 Hepatic artery Portal vein Hepatic vein Non thrombotic infarction Primary non function 8 Massive haemorrhagic necrosis Recurrent disease (ALD, PSC, CAH:AI) De novo Hep C Biliary Complications Other (PNC, immune hepatitis, outflow obstruction) 8 7 TOTAL 87 7 Miscellaneous Children Adult Multiorgan failure Renal Failure Graft vs Host disease Social (accident, suicide,noncompliance, Rx withdrawn) 8 9 Sudden death (cause unknown) Other (Hyperkalaemia,motor neurone disease diabetes complications, drug reaction, progression FAP essential thrombocythemia) TOTAL DATA TO // copyright ANZLTR Data to.. APPENDIX