For a long time, small children could not undergo either a heart transplantation (HTx) or heart-lung transplantation (HLTx) in Japan and many Japanese children were compelled to go abroad to undergo an HTx. After revision of the Organ Transplant Act, a small child could donate organs if the family allowed it, and in fact, eventually six children (one less than 6 years old, three from 10 to 14, and two 15 and 17) had donated his or her heart by the end of 2013. In this review, the current status of pediatric HTx and HLTx in Japan and an indication of pediatric HTx and HLTx and management before and after HTx were described. Briefly, most indications of HTx were dilated cardiomyopathy (DCM) and restrictive cardiomyopathy in Japan and many candidates with DCM required a left-ventricular assist device (LVAS) for a bridge to HTx in Japan. Patient survival at 10 years after HTx was 100% in children transplanted in Japan and 87.6% in children transplanted abroad. No child has undergone an HLTx yet. Post transplant lymphoproliferative disorder and various infections were major morbidity and mortality. Non-adherence should be paid attention, especially during the care of adolescent patients.

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In 2010, the Japanese transplant law was revised so that a pediatric brain-dead donor could be accepted for transplantations. However, until now a pediatric lung transplantation has never been performed from brain dead pediatric donor in Japan.
As of April 2014, lung transplantations have been performed in 367 patients in Japan. Among them, 42 recipients (11.4%) were children under the age of 16 years. Thirty-nine of them (92.9%) received a living-donor lobar lung transplantation.
The author's personal experience included 22 pediatric patients who underwent single (n=12) or bilateral (n=10) living-donor lobar lung transplantation. The 5-year survival rate was 80%, much better than the rate reported by ISHLT.
Living-donor lobar lung transplantation is a viable option for pediatric patients. Efforts should be directed to increase brain-dead donors for pediatric patients who do not find suitable living-donors.

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Pediatric liver transplantation is an established treatment. The 1- and 10-year survival rates for pediatric liver-transplanted patients are 88.5% and 83.2% in the Japanese registry. Recently, there have been several evolutions and challenges in the specialty of pediatric liver transplantation, such as overcoming the graft-size matching and posttransplantation lymphoproliferative disorder. Because most donor sources are living donors in Japan, their safety would be imperative. An urgent need exists to expand the donor pool, including split-liver transplantations, because of the shortage of deceased donor liver transplantations in Japan.

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The current status of pediatric kidney transplantation in Japan is briefly reviewed. Recent advances in medical treatment, including dialysis and kidney transplantation (KTx), have made it possible for pediatric patients with end-stage kidney disease (ESKD) to survive to adulthood. Since KTx is the best option for children with ESKD, the number of children receiving a preemptive KTx is increasing. ABO-incompatible KTx and sequential combined liver-kidney transplantation have been successfully performed. At present, the goal of therapy for pediatric patients with ESKD is to attain good psychosocial outcomes as adults. Therapy for pediatric patients with ESKD should be multidisciplinary, including not only the pediatric nephrologists, but also surgeons, nurses, social workers, dieticians, and psychologists.

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Although the development of parenteral nutrition has improved outcomes of intestinal failure, parenteral nutrition still often leads to numerous life-threatening complications. Intestinal failure-associated liver disease (IFALD), which can be progressive and fatal in children, is one of the most serious complications. Intestinal transplantation (Itx) represents a definitive treatment for patients with irreversible intestinal failure. By 2013, a total of 2887 intestinal transplants had been performed worldwide, 55% of which were in patients under 18 years old. In Japan, 60% of all recipients were under 18 years old. Because of recent advances in the management of Itx, short-term outcomes following transplantation have improved; however, the long-term outcomes remain insufficient. Furthermore, some patients with severe IFALD require liver-intestinal transplantation, a procedure that remains practically unavailable in Japan. In this article, we provide an overview of the process and the problems of pediatric Itx, with special focus on the situation in Japan.

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Over the past decade, important progress has been made in the use of chemotherapy for pediatric leukemia, both lymphoblastic and myeloid leukemia by sophisticated methods. As a result, the frequency of patients who should receive a stem cell transplantation (SCT) has decreased. However, for high-risk patients of relapse, SCT remains a curative strategy. Recent progress in SCT provides various transplantation strategies. SCT donor selection is very important for the effectiveness and safety of SCT. We find that an SCT from HLA haploidentical and/or a mismatch of killer cell immunoglobulin-like receptor (KIR) phenotype donor is an effective strategy for high-risk patients. Late complications for pediatric SCT is a serious issue to be resolved. The prevention of growth retardation, endocrine complications and infertility should be considered for long term cancer survivors.

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After the enforcement in Japan of the amended Act on Organ Transplantation in July 2010, the number of brain-dead donors vastly increased. But the total numbers of the deceased donors for organ transplantation were almost the same in 2010, 2011 and 2012. The percentage of the brain-dead donors increased, but the total numbers of cardiac-arrested donors decreased. Unexpectedly, in 2013, the total number of the deceased donors decreased to 84. The numbers of brain-dead and cardiac-arrested donors were 47 and 37. The organ transplantations from the deceased donors are realized with the efforts of the procurement teams. This is a report on organ procurement from the deceased donors for organ transplantations in Japan in 2013.

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A number of 1,586 kidney transplants including 1,431 from living donors, 67 from non-heart-beating donors and 88 from heart-beating donors were performed in 2013 in Japan.
The data obtained from the Japanese Renal Transplant Registry are shown and analyzed in this annual report. The characteristics of recipients and donors such as relationships, original diseases, duration of dialysis therapy, blood transfusion, the status of viral antigens and antibodies, pretransplant complications, the causes of death of deceased donors, ischemic time and the histocompatibilities are described. In addition, immunosuppressants used initially and other treatments are analyzed.
We also reported the results of follow-up survey for all recipients and donors of the transplants from living donors.

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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 transplants (216 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 2012. 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 popular (36%). Patient survival following transplantations from heart-beating donors (1 year, 85.9%; 3 years, 82.6%; 5 years, 81.3%; 10 years, 73.8%) was similar to those from living donors (1 year, 83.8%; 3 years, 79.6%; 5 years, 77.1%; 10 years, 71.9%; 15 years, 67.8%; 20 years, 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 years.

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Since the Organ Transplantation Law was passed in October 1997, a total of 185 heart transplantations (HTx) have been performed in Japan as of December, 2013. Of those, 116 HTx was performed after activation of modified transplant law. Most recipients had dilated cardiomyopathy; waiting condition of all patients, except for one pediatric patient, was status 1 at HTx. The mean waiting time as status 1 was 864 days. Ninety percent of the patients (166) were bridge-to-transplant (BTT) cases by several types of left ventricular assist devicess (LVAD), and mean support duration was 896 days. Most received a modified bicaval method of operation with Celsior for cardiac preservation, and all recipients were administered triple therapy with calcineurin inhibitor (cyclosporine or tacrolimus), mycophenolate mofetil, and steroid as initial immunosuppressive regimen. Two patients have been doing well >14 years after HTx, and ten-year survival rate was 89.8 %, which is superior to that of the international registry. Five pediatric HTx （<10 years: one, 10-18 years: 10） performed under modified transplant law. In this year, the number of HTxs in Japan exceeded the number of HTxs of Japanese patients in abroad. However, pediatric HTxs of Japanese patients abroad, especially <10 years old, still has much HTx in abroad. This surveillance documented that the results of HTx in Japan were excellent despite a severe shortage of donors and long waiting time with LVAS as BTT.

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Objective: To scrutinize the status of lung transplantation in Japan, the Japanese Society of Lung and Heart-Lung Transplantation started to collect and present registry data from 2005. This is the 10th official registry report of Japanese lung transplantation.
Design and Methods: The data of cadaveric lung transplantation and living-donor lobar lung transplantation performed by the end of 2013 were registered in the database and analyzed with respect to the number of transplants, recipient survival rates, recipient functional and working statuses, and causes of death after transplantations. Survival rates were calculated by the Kaplan-Meier method.
Results: A total of 197 cadaveric lung transplantation (104 single, 93 bilateral), 145 living-donor lobar lung transplantation and 2 heart-lung transplantation procedures were performed by the end of 2013. Five-year and 10-year survival rates of cadaveric lung transplantations were 73.2% and 64.0 %, which were superior to those in the International Registry (53.1% and 31.0%). Five -year and 10-year survival rates of living-donor lobar transplantations were similar to those of cadaveric lung transplantation with 73.2% and 66.7%. The recipients of 2 heart-lung transplantations have been survived. The functional status of around 80% of recipients was restored to Hugh-Jones I or II after transplantations. Infection was the leading cause of death after lung transplantation. Primary graft dysfunction accounted for about 30% of the causes of death after cadaveric lung transplantation.
Conclusion: The results of Japanese lung transplantation are so far satisfactory. Efforts must be made, however, to overcome early deaths resulting from primary graft dysfunctions. The modified Japanese transplantation law has been enforced since July, 2010, and an increase in the number of cadaveric organ transplantations has been achieved thereafter. The Japanese Society of Lung and Heart-Lung Transplantation will continue to present annual reports of Japanese lung transplantations.

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Two hundred and eight cases of pancreas transplantation from deceased, non-heart beating and living-related donors have been performed in 14 institutions in Japan until the end of 2013 since April, 2000. The following donor- and recipient-related factors were analyzed; i.e., age and gender of donor and recipient, cause of death, histories of diabetes and dialysis, waiting period, total cold ischemic time, operative procedure, immunosuppression and survival rates of patient and graft.
In spite of donor poor conditions which were mostly marginal in Japan, the outcome of pancreas transplants was considered to be comparable to that of the US and Europe.

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Islet transplantation can relieve glucose instability and problems with hypoglycemia while the graft is functioning. However, the long-term results of islet transplantation pointed to the need for progress improving islet graft function. We started the phase II clinical trial with type 1 diabetes patients for islet transplantation using both brain-dead donors and DCD to evaluate ATG induction and TNF inhibition protocol. Primary endpoints are the proportion of subjects with HbA1c<7.4% and who are free of severe hypoglycemic events one years after the first islet cell infusion. This trial would play a critical role in establishing islet transplantation in Japan.

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Twenty-five intestinal transplants were performed since 1996 in 5 institutions. There were 13 deceased donor and 12 living related donor transplants. Primary causes of intestinal transplants were short gut syndrome (n=9), intestinal mobility function disorder (n=12), others (n=1) and re- transplantation (n=3). 1 year patient survival was 86%, and 10-year patient survival was 66%. They were considered to be excellent results for a standard therapeutic option for intestinal failure if patients fail to maintain total parental nutrition.

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【Objective】This study examines the daily-life situation and the subjective-life satisfaction of living liver-transplanted preadolescent and adolescent children.
【Design】Cross-sectional survey
【Methods】Our study sample consists of 9 preadolescent and adolescent children who had undergone liver transplantation. Data were collected through semistructured interviews and questionnaires about their daily-life experiences. Descriptive statistics and qualitative descriptive research methods were used for the data analyse.
【Results】Our survey showed how elementary school children (fourth to sixth grades) could manage their health by themselves with parental support. They did know that they experienced an operation in their childhood period,but did not sufficiently understand the "transplantation" itself. Self-management of high-school children differs according to their transplantation period or their perception about the transplantation. In regard to subjective-life satisfaction, all the elementary school children had scores higher than the standard value; however, half of the high school children showed scores lower than that value. In particular, the subjective-life satisfaction did not always correspond with their physical situations.
【Conclusion】Preadolescent children could manage their health with parental support. Their satisfaction score was higher than the standard value. However, they did not sufficiently understand the "transplantation" itself. Self-management of adolescent children differs according to their transplantation period or their perception about the transplantation. The satisfaction score did not necessarily correspond to their physical situation. Medical staffs should support children with due consideration of their past experiences and understanding of the disease, the treatment, and the transplantation itself.

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