Timing for orthotopic liver transplantation in children with biliary atresia: a single-center experience.

INTRODUCTION: Biliary atresia is the most common indication for orthotopic liver transplantation (OLT) in childhood. The purpose of this study was to determine predictive prognostic factors for children with biliary atresia related to the timing for OLT within 15 months after hepatoportoenterostomy (HPE). PATIENTS AND METHODS: We retrospectively analyzed the medical records of 25 children (7 boys and 18 girls) who underwent HPE because of biliary atresia between January 1990 and December 2005 at our center. Data examined included age and pathologic findings at HPE, Pediatric End-Stage Liver Disease score at first admission, whether phototherapy was given, liver function test results and total bilirubin level before and 30 days after HPE, and number of cholangitis events. RESULTS: Twelve children were alive with their native liver, 8 had undergone living donor OLT (all children alive), and 5 had died without OLT. Five- and 10-year survival rates without OLT after HPE were 47.4% and 26.3%, respectively. At univariate analysis, the predictive prognostic factors for children with biliary atresia were total bilirubin level at 30 days after HPE and Pediatric End-Stage Liver Disease score before HPE. At multivariate analysis, the only prognostic factor was total bilirubin level at 30 days after HPE. CONCLUSIONS: In this study, the predictive prognostic factor was total bilirubin level at 30 days after HPE. Orthotopic liver transplantation within 15 months after HPE is needed in children with biliary atresia with a high total bilirubin level at 30 days after HPE.

Living donor liver transplantation for a child with recurrent pediatric adult-type hepatocellular carcinoma.

INTRODUCTION: Pediatric hepatocellular carcinoma (HCC) is an uncommon disease with a poor prognosis. There are few reports about liver transplantation for pediatric adult-type HCC. We experienced a case of living donor liver transplantation (LDLT) for a child with recurrent pediatric adult-type HCC. CASE REPORT: A 12-year-old boy was admitted to the Department of Pediatrics in our institution due to HCC in May 2005. He underwent hepatectomy after 3 courses of chemotherapy in July 2005. After the operation, he had 2 more courses of the same chemotherapy. His posttheraputic course was uneventful for 1 year. However, his alpha-fetoprotein level increased and a computed tomography (CT) scan showed recurrent tumor in his remnant liver in October 2006. He underwent another chemotherapy session immediately. However, CT revealed multiple liver tumors after chemotherapy in December 2006. His mother requested to be an LDLT donor, which was performed on January 23, 2007. The donor operation was a right hepatic lobectomy. The postoperative course of the donor was unremarkable and she has now returned to work. The recipient's posttransplantation course was uneventful and he was discharged at postoperative day 53 and is currently doing well. CONCLUSION: Liver transplantation in conjunction with chemotherapy may have an increasing role in the management of pediatric HCC.

Long-term Changes in Spleen Volume After Living Donor Liver Transplantation in Pediatric Recipients.

BACKGROUND: Living donor liver transplantation (LDLT) is a definitive procedure for splenomegaly caused by liver cirrhosis and portal hypertension, but splenomegaly persists in some patients. The aim of this study was to clarify the long-term changes in the spleen volume after LDLT. METHODS: The 13 pediatric patients who survived for >8 years after LDLT were retrospectively analyzed. We calculated the spleen volume/standard spleen volume (SV/SSV) ratio by automated computed tomography (CT) volumetry. We assessed the spleen volumes before LDLT, at roughly postoperative week (POW) 4, at postoperative year (POY) 1, at POY 5, and at POY 10. RESULTS: With regard to SV as evaluated by CT volumetry, there were no consistent trends, with median values as follows: before LDLT, 282.5 (71-641) cm3; POW 4, 252 (109-798) cm3; POY 1, 222.5 (97-948) cm3; POY 5, 263.5 (123-564) cm3; and POY 10, 377 (201-1080) cm3. In contrast, the SV/SSV ratio decreased chronologically as follows: before LDLT, 5.0 (0.7-6.0); POW 4, 3.7 (2.3-4.3); POY 1, 2.2 (1.7-6.3); POY 5, 1.7 (1.1-5.4); and POY 10, 1.4 (1.1-6.9). In the remote phase after LDLT, many cases showed a trend toward an improved SV/SSV ratio, but splenomegaly was prolonged without improvement in 3 cases (23.1%) with portal vein complications and advanced fibrosis. Furthermore, all 3 cases showed a decreased platelet count due to hypersplenism. CONCLUSION: Splenomegaly requires a long time to demonstrate an improvement. In cases without an improvement of splenomegaly, we should suspect abnormalities in the graft liver and portal hemodynamics.

Impact of Smoking Cessation in Donor Candidates for Living Donor Liver Transplantation.

BACKGROUND: The relationship between smoking cessation and weight gain is well recognized. Examining the link between smoking cessation and weight gain in donor candidates for living donor liver transplantation (LDLT) is an important topic because of the influence of weight gain on the liver. This study assessed body weight (BW) changes after smoking cessation in donor candidates for LDLT. METHODS: The 27 donor candidates were retrospectively analyzed. The smoking status was determined based on questionnaires administered at the initial presentation, and the candidates were divided into 2 groups: recent quitters and nonsmokers. The changes in BW were compared between the groups. RESULTS: The recent quitters group included 10 (37.0%) candidates, and the nonsmokers group included 17 (63.0%). In the nonsmokers group, 1 candidate had gained weight since the initial presentation. In contrast, in the recent quitters group, 70.0% of candidates had gained weight since the initial presentation (P < .01). The change in BW from the initial presentation was greater in recent quitters than in nonsmokers (+1.6 kg [+2.4%] vs -0.5 kg [-0.9%]; P < .01). Two candidates in the recent quitters group gained ≥ 5 kg [8%] of weight. One of these 2 candidates was judged to be in a donor-inadequate status because of the appearance of fatty liver. CONCLUSIONS: Weight gain due to smoking cessation was observed in donor candidates for LDLT. The amount of weight gain after smoking cessation is highly individualized, so everyone concerned with LDLT must be alert to its potential development.

Improved survival and ammonia metabolism by intraperitoneal transplantation of microencapsulated hepatocytes in totally hepatectomized rats.

BACKGROUND: We evaluated the effects of intraperitoneal transplantation of microencapsulated hepatocytes in a 3-stage total hepatectomy rat model. METHODS: A new model of total hepatectomy was created as follows. First, the infrahepatic inferior vena cava was ligated just above the right renal vein. Seven days later, the portal vein was ligated and a portacaval shunt was established using a Teflon catheter over a venipuncture needle. Another 7 days later, total hepatectomy was completed by ligating and dividing the suprahepatic inferior vena cava, the hepatic artery, and the bile duct. Next, 4 x 10(7) hepatocytes (4% of the normal liver hepatocyte mass) isolated from male Wistar rats were microencapsulated within a collagen matrix enveloped by a 3-layer membrane of sodium alginate-poly-L-lysine-sodium alginate copolymer. Capsules containing hepatocytes (diameter, 500-800 microm) and empty capsules (control) were transplanted intraperitoneally 4 days before the total hepatectomy. Survival time and selected blood chemistry concentrations after the total hepatectomy were measured. The capsules were also examined histologically with hematoxylin and eosin staining and modified Gmelin's stain for bile pigments. RESULTS: The survival time was greater in the rats given the microencapsulated hepatocytes than in the control rats (17.3 +/- 3 vs 3.7 +/- 0.1 hours; P <.01). The blood ammonia concentrations increased soon after total hepatectomy but remained significantly lower in the rats with microencapsulated hepatocytes (P <.05). The microcapsules contained numerous viable hepatocytes with abundant bile pigments and no lymphocytic infiltration. CONCLUSIONS: Microencapsulated hepatocytes with an ultrathin polymer layer that protects them from inflammatory and lymphocytic reactions may facilitate their ability to function. In this study, 4 x 10(7) hepatocytes significantly prolonged the survival of rats that underwent hepatectomy and supported ammonia metabolism. Further development of this technique may permit its use in patients with hepatic failure.

Rectal cancer associated with acquired hypertrichosis lanuginosa as a possible cutaneous marker of internal malignancy.

We report a case of acquired hypertrichosis lanuginosa associated with rectal cancer and discuss the cutaneous symptoms as a marker of malignancy. A 75-year-old man had noticed increasing growth of hair over his entire body 4 months before he was admitted to our hospital because of bloody stools. Advanced rectal cancer was diagnosed, and he underwent sigmoidocolostomy. After the operation, levels of serum adrenocorticotrophic hormone and cortisone were normal, as were urinary levels of 17-ketosteroids and 17-hydroxycorticosteroids. Levels of carcinoembryonic antigen and carbohydrate antigen 19-9 were slightly elevated. The patient died 5 months after the operation because of peritonitis carcinomatosa. We suggest that hypertrichosis lanuginosa is a marker of internal malignant tumors.

Primary immunosuppression regimen of rapid steroid withdrawal after living related liver transplantation: a single-center experience.

AIM: Corticosteroids have been considered the mainstay of immunosuppressive therapy after liver transplantation. However, the side effects of long-term steroid use such as diabetes, infections, and bone disease, including growth retardation in children, are serious problems. Our immunosuppression regimen includes FK506 and steroid withdrawal by 30 days after transplantation. The aim of this study was to determine the outcomes of liver transplant, using this immunosuppressive regimen. PATIENTS: Fifteen primary liver transplant recipients were performed between January 1994 and May 2003 and data were reviewed retrospectively. Eight pediatric and four adult recipients, who had survived more than 3 months after transplantation, were included in this sample. The immunosuppressive regimen consisted of FK 506 (Prograf), initially at doses of 0.03 mg/kg, with dose adjustments to achieve daily trough levels of approximately 10 to 12 ng/mL, and predonisone, initially at 4 mg/kg/d, with a taper and cessation by 30 days when the graft was stable. RESULTS: All recipients were successfully withdrawn by 30 days. Acute rejection episodes occurred in three patients, no patient was diagnosed with chronic rejection. The acute rejection-free rate at 5 year was 74.1%. No recipient had diabetes, serious infections or bone disease. CONCLUSION: Our primary immunosuppressive regimen of rapid steroid withdrawal is safe with regard to acute and chronic rejection with benefits upon steroid-related side effects.

Hepatic clearance improves after angioplasty of the hepatic vein.

Anastomotic stricture sometimes causes hepatic congestion leading to decreased hepatic clearance of drugs. We herein describe a correlation between trough levels of tacrolimus and an anastomotic stricture of the hepatic vein. A 13-year-old boy underwent living donor liver transplant with a left lobe graft from his mother. Outflow blockage due to an anastomotic stricture of the hepatic vein developed 3 months after transplant. His anastomotic site had been repeatedly treated with percutaneous transvenous angioplasty (PTA) by balloon dilation. About 1 year after transplant, his trough level of tacrolimus promptly decreased after balloon dilation (from 15.7 to 5.6 ng/dL). Liver function tests showed abnormalities, which were diagnosed as acute cellular rejection, and he was treated with pulse steroid therapy. The calculated half-life of tacrolimus (T1/2) showed marked improvement after PTA (from 35 to 22 hours). A 45-year-old woman underwent living donor transplantation due to alcoholic liver cirrhosis with a left lobe graft from her brother. An anastomotic stricture of the hepatic vein developed 4 months after transplant. She was treated with balloon dilation, which caused an abrupt decrease in the trough level of tacrolimus (12 to 4 ng/dL). Her alkaline phosphatase was elevated and she was diagnosed with rejection, which was treated with an increase of dosage of tacrolimus. In outflow block, the T1/2 of tacrolimus increases probably due to decreased hepatic clearance by stagnation or congestion of the liver. However, hepatic clearance of drugs quickly recovers after PTA. Close monitoring of immunosuppressive agents is fundamental at the time of angioplasty to avoid acute cellular rejection as developed in our two cases.

Efficacy of cutting balloon for anastomotic stricture of the hepatic vein.

Anastomotic stricture of the hepatic vein is an annoying complication, especially in living donor liver transplantation. Balloon dilation has been utilized but is sometimes associated with recurrences. Recently, a cutting balloon was invented for treatment of arteriosclerosis. Herein we report the results of application of this device for treatment of anastomotic strictures of the hepatic vein in two living donor liver transplant recipients who underwent percutaneous dilation of the hepatic vein with a cutting balloon (8 x 10 mm, Atherotome, Boston Scientific). Case 1, a 26-year-old woman transplanted for subacute fulminant hepatitis, had been treated for an anastomotic stricture by balloon dilation on 15 occasions over a 2- to 3-month interval. Case 2, a 13-year-old boy transplanted for cryptogenic liver cirrhosis, had been treated for an anastomotic stricture by balloon dilation biannually. The cutting balloon was applied safely without severe complications. The first case showed a recurrent anastomotic stricture at 6 months after dilation. Follow-up at 6 months in the second case revealed a mild recurrence of the stricture. Anastomotic stricture of the hepatic vein jeopardizes the graft and the recipient. The reported treatments involve venoplastic surgery and expandable metallic stents. Application of a cutting balloon seemed to be a safe, convenient modality. However, its effect was not indefinite, so a cutting balloon of greater diameter or application of an expandable metallic stent may be considered for patients with multiple recurrences of their anastomotic stricture.

Intraoperative blood lactate level as an early predictor of initial graft function in human living donor liver transplantation.

UNLABELLED: This study was performed to investigate whether intraoperative changes in blood lactate levels after hepatic allograft reperfusion reflect initial graft function in living donor liver transplantation (LDLT). PATIENTS AND METHODS: From 1994 to 2003, 15 of LDLT cases were divided into two groups based on the intraoperative blood lactate levels. Group A consisted of seven recipients whose new liver grafts started to consume lactate immediately after portal perfusion. Group B consisted of the remaining eight recipients whose intraoperative blood lactate values showed no change or an elevation for 2 hours after graft revascularization. RESULTS: All Group A patients survived, whereas three out of eight patients in Group B died of infection and portal vein thrombosis within 3 months after LDLT. There was no significant difference in preoperative donor and recipient laboratory data. The recipient age and body size in Group B were significantly higher than those in Group A, indicating that Group B consisted of small-for-size liver transplant cases. Serum total bilirubin concentrations in Group B were significantly higher than Group A from postoperative day 5 to 23, whereas postoperative liver enzyme levels and prothrombin time were similar between the two groups. CONCLUSION: The change in intraoperative blood lactate after hepatic allograft reperfusion served as an accurate predictor of initial graft function which was associated with graft size in human LDLT.

Hepatic vein anastomotic stricture after living donor liver transplantation.

This report discusses the pathophysiology of and therapeutic methods to address hepatic vein anastomotic stricture after living donor liver transplantation (LDLT). From 1994 to 2002, our 15 LDLTs using the lateral segments or left lobes included four recipients who experienced 28 occurrences of this complication after the operation. The period between LDLT and the first stricture was 4.0 +/- 1.2 months. The age of the affected recipients (31.0 +/- 8.2 years) was significantly higher than that of the nonaffected patients (7.0 +/- 4.1 years, P < .05). Graft liver/standard liver volume ratio was 39.1% +/- 3.8% in the former and 77.9% +/- 12.7% in the latter cases (P < .05). Initial symptoms of stricture were ascites (42.9%), abdominal distention (42.9%), liver enzyme elevation (10.7%), and gastrointestinal bleeding (3.6%). In addition, 14 of 28 stricture cases (50%) showed increased blood trough levels of tacrolimus. Doppler ultrasonography was used for diagnosis, and balloon dilatations performed in all stricture patients, thereby hepatic significantly reducing venous blood pressure from 33.5 +/- 1.7 to 20.3 +/- 1.5 cmH2O. All patients finally resolved the strictures after several treatments. The stricture after LDLT was associated with small-for-size grafts, suggesting that liver regeneration may lead to anatomical changes and strictures. Since tacrolimus is metabolized by the liver, its blood trough level is one initial symptoms of stricture. Balloon dilatation was useful and safe as the treatment, while problems have been reported after stent insertion in the hepatic vein.

Analysis of clinical variables of donors and recipients with respect to short-term graft outcome in human liver transplantation.

UNLABELLED: Donor and recipient factors are closely associated with graft survival after orthotopic liver transplantation (OLT). This study was performed to analyze clinical characteristics of recipients and donors, which affect 30-day graft loss after OLT. MATERIALS AND METHODS: One hundred eighty-six livers from heart-beating donors were accepted between May 1997 and June 1998 at the University of Pittsburgh Medical Center. Donor variables that were analyzed included age, sex, cold ischemia time (CIT), warm ischemia time (WIT), imported versus local procurement, cardiopulmonary arrest, serum sodium level, and dopamine dose. The recipient characteristics included native liver disease and UNOS status. Two-sided Fisher exact test and stepwise logistic regression were used for univariate and multivariate analyses. P-values < .05 were considered statistically significant. RESULTS: Twenty-eight grafts (15.1%) were lost within 30 days of OLT. The following factors were found to adversely affect graft survival: donor sodium > 155 mEq/L (P = .002); CIT > 12 hours (P = .002); WIT > 45 minutes (P = .002); and imported liver graft (P = .048). Logistic regression revealed that donor sodium (odds ratio, 3.03; 95% CI, 1.05 to 8.74), CIT (OR 1.20; 95% CI 1.05 to 1.38), WIT (OR 1.06; 95% CI 1.01 to 1.09) were independent predictors of early graft loss. CONCLUSION: Donor hypernatremia as well as warm and cold ischemia times independently affect graft outcomes in the early postoperative period after OLT. Avoidance of long preservation and correction of donor sodium level are recommended to optimize results and survival in OLT.

A case of living-related partial liver transplantation using the right gastroepiploic artery for hepatic artery reconstruction.

A 13-year-old boy with liver cirrhosis underwent living-related partial liver transplantation with a left lobe from his mother. A standard hepatic artery reconstruction using the recipient right hepatic artery was anticipated. Unfortunately, the recipient hepatic artery was found to be severely arteriosclerotic and was unsuitable for reconstruction. Instead, the right gastroepiploic artery, measuring 2.0 mm in diameter, was mobilized and was anastomosed to the left hepatic artery of the graft in an end-to-end fashion. Arterial blood flow was satisfactory. The patient's postoperative course was uneventful, and he was transferred to a floor bed on the 5th postoperative day.

Semiquantitative evaluation of hepatic fibrosis by measuring tissue hydroxyproline.

BACKGROUND/AIMS: It is important to evaluate the degree of hepatic fibrosis when diagnosing and treating hepatic cirrhosis. We focused on hydroxyproline, which is detected specifically in collagen, which plays a major role in hepatic fibrosis. The correlations between liver tissue hydroxyproline residue levels and the degree of hepatic fibrosis were examined in dogs with dimethylnitrosamine-induced fibrotic livers. METHODOLOGY: Dimethylnitrosamine was administered to dogs to establish experimental hepatic fibrosis. Paraffinized sections of liver specimens, stained with hematoxylin-eosin and azan, were examined and the degree of hepatic fibrosis was graded. About three-milligram samples of liver tissue were loaded onto a fully automated liquid chromatograph and the levels of hydroxyproline residues were measured. RESULTS: The liver tissue hydroxyproline appeared to reflect the degree of hepatic fibrosis. The liver tissue hydroxyproline levels and pathological hepatic fibrosis grades correlated significantly (p<0.05). CONCLUSIONS: Tissue hydroxyproline appears to be a more useful fibrosis marker, because hydroxyproline is influenced less by other factors. Furthermore, our results demonstrate that a very small amount of liver tissue (wet weight 3 mg) was enough to enable the levels of hydroxyproline residues to be measured by an automated amino acid analyzer (JLC-3000) and hepatic fibrosis is expressed as the numerical value by this analysis.

Laparoscopic treatment for biliary ascariasis.

Biliary ascariasis is one of the most common types of ascaris infections. The current treatments are helminthic drug therapy, endoscopic extraction, and surgical extraction. A case of biliary ascariasis and cholecystocholedocholithiasis was successfully treated by laparoscopic extraction of the living worm and biliary stones. This procedure was found to be very effective for biliary ascariasis with biliary stones, and it holds promise for similar cases in the future.

Iron overload after pediatric liver transplantation: a case report.

Iron is an essential nutrient for living cells; however, an excessive accumulation of iron leads to organ damage and directly affects systemic immunity. Iron overload is clinically classified as hereditary or secondary. Most of secondary iron overload is caused by frequent blood transfusions because there is no active mechanism to excrete iron from the body. As recommended in various guidelines, chelation therapy is effective for reducing iron burden and improving organ function. There have been few reports on iron overload through blood transfusion during the perioperative period of liver transplantation. This report presents a case of iron overload due to repeated transfusions after pediatric liver transplantation managed by chelation therapy. The patient, an 11-month-old female with biliary atresia, underwent living donor liver transplantation. She revealed refractory anemia and required frequent blood transfusion. Both serum ferritin and transferrin saturation tended to increase after repeated transfusions, leading to secondary iron overload. Iron chelation therapy was started to prevent progression to organ failure and infection due to iron overload, and yielded a favorable outcome. It is crucial to consider the possibility of secondary iron overload and to achieve early detection and treatment to avoid progression to irreversible organ damage.

Liver transplantation versus conservative treatment for adult-onset type II citrullinemia: our experience and a review of the literature.

Adult-onset type II citrullinemia (CTLN2), an autosomal recessive disorder caused by a mutation in the SLC25A13 gene, is characterized by increased serum citrulline and ammonia levels. Patients with CTLN2 also display various neuropsychiatric symptoms. Many individuals with CTLN2 are fond of protein-rich and/or lipid-rich foods with an aversion to carbohydrate-rich foods. We herein report two cases of CTLN2 treated with living donor liver transplantation (LDLT) and provide a review of the pertinent literature. Case 1 was a 43-year-old man admitted to our hospital for repetitive episodes of consciousness disturbance. Case 2 was a 37-year-old man admitted to our hospital because of abnormal behavior associated with hyperammonemia. A definitive diagnosis of CTLN2 was accomplished by DNA analysis in both patients, who successfully underwent LDLT using liver segments from donor siblings with confirmed heterozygous gene expression. Case 2 also underwent conservative therapy with arginine and a high-fat, carbohydrate-restricted diet prior to LDLT. Postoperative recovery was uneventful and food was unrestricted in both patients. We also identified 77 cases of CTLN2 in the literature and reviewed them in terms of outcome of both liver transplantation and conservative therapy. The survival rate in patients treated by liver transplantation was 100%, whereas that in patients treated by conservative treatment showed improvement from 39.5% to 76.5% over the years. Liver transplantation is a practical treatment that fundamentally improves patient quality of life after transplantation. However, recent studies have suggested that arginine and sodium pyruvate administration combined with intensive nutritional support is also an effective therapy for CTLN2. Further development of conservative therapy may provide a safer, more affordable alternative to liver transplantation in the near future.

Importance of awareness of perioperative social and physical situations of living donors for liver transplantation.

INTRODUCTION: Transplantation in Japan still depends on living donors even after the new revised law. We must pay attention to protect living donors. PATIENTS AND METHODS: Perioperative qualities of life after living donation for liver transplantation were assessed with questionnaires including the Medical Outcomes Study 36-Item Short-Form Health Survey version 2 (SF36-v2). Nonparametric Mann-Whitney tests were used to determine statistical significance. P values<.05 were considered significant. RESULTS: Thirty-one among 33 donors answered questionnaires (93.9%). The 15 men and 16 women of average age of 39.7 years had a median hospital stay of 16 days and median duration after surgery of 78 months. Ten of 33 (35.7%) donors considered themselves to be the only possibility. The decision to a donor was established prior to informed consent in 23 donors (74.1%). Six months were required for them to experience a full recovery after donor surgery. Hamilton depression/anxiety score was significantly increased among donors who considered themselves to be the only possibility or those who had decided prior to informed consent. SF36-v2 revealed a significant decrease in social functioning among donors who did not have sufficient time to decide before surgery. General health was significantly decreased among donors who required more than 6 months for full recovery. Perioperative management of pain influenced general health, physical role, bodily pain, and physical functioning. CONCLUSION: We must pay attention to depression and anxiety among living donors. More care should be focused on pain control and sharing of information of postoperative courses.

Donor quality of life after living donor liver transplantation: single-institute experience.

AIM: Living donor liver transplantation (LDLT) has been widely accepted because of the severe shortage of hepatic grafts. However, the healthy donor is exposed to risks of morbidity and mortality. In this study, we analyzed medical, functional, and psychological outcomes of donors after hepatectomy for liver donation. PATIENTS AND METHODS: Among 41 donor hepatectomy cases for LDLT performed in our institute from January 1994 to May 2011, we reviewed the medical records (liver function tests, complications, etc) of 27 subjects who donated to recipients older than 12 years. We also performed a questionnaire survey based on the Japanese Short Form-36 version 2 Health Survey scales as a measure of physical and mental health, to which 31 subjects responded. RESULTS: Six of the 27 donors experienced prolonged jaundice. Their ratios of graft volume/standard donor liver volume (GV/SDLV) were higher than those of the 21 donors without prolonged jaundice (60.0% vs 41.5%). According to the questionnaires, social functioning among those having undergone emergency hepatectomy as well as general health perceptions declined in those with postoperative complications. Physical component summary declined among those having undergone emergency hepatectomy and with postoperative complications. CONCLUSION: In liver donation from a living donor, massive hepatectomy should be avoided. A ratio of GV/SDLV around 50% seems reasonable. Donors with emergency transplantations or postoperative complications must be more carefully followed after donor hepatectomy.

Liver transplantation for Wilson's disease in pediatric patients: decision making and timing.

Transplantation for Wilson's disease occupies 1/3 of the cases for metabolic diseases in Japan. At the end of 2009, 109 transplantations had been performed including three deceased donor cases in the Japanese registry. We herein discuss problems of transplantation for Wilson's disease as well as its indication, timing, and social care. We retrospectively reviewed four fulminant cases and two chronic cases who underwent living donor liver transplantation. There were two boys and two girls. Four adolescents of average age 11.3 years underwent living donor liver transplantation. Duration from onset to transplantation ranged from 10 to 23 days. Average Model for End-stage Liver Disease (MELD) score was 27.8 (range=24-31). All patients were administrated chelates prior to transplantation. MELD, New Wilson's index, Japanese scoring for liver transplantation, and liver atrophy were useful tools for transplantation decision making; however, none of them was an independent decisive tool. Clinical courses after transplantation were almost uneventful. One girl, however, developed an acute rejection episode due to noncompliance at 3 years after transplantation. All patients currently survive without a graft loss. No disease recurrence had been noted even using living related donors. Two adults evaluated for liver transplantation were listed for deceased donor liver transplantation. Both candidates developed cirrhosis despite long-term medical treatment. There were no appropriate living donors for them. There are many problems in transplantation for Wilson's disease. The indications for liver transplantation should be considered individually using some decision-making tools. The safety of the living donor should be paid the most attention.