Living donor liver transplantation for patients with portal vein thrombosis: high-volume single center experience.

OBJECTIVE: End-stage liver disease is commonly associated with portal vein thrombosis (PVT). Lastly, PVT is no longer an absolute contraindication for liver transplantation, and many centers adopt portal vein thrombectomy. PVT imposes special technical difficulties during living donor liver transplantation (LDLT). In this research, the experience with PVT cases during LDLT in a high-volume center is introduced. PATIENTS AND METHODS: Between January 2018 and July 2023, 312 patients underwent LDLT. After 88 cases were excluded, 224 cases were included, and their incidence of pre-transplant PVT was 16.5% (37/224). Demographic and clinical features, perioperative variables, and post-transplant outcomes of patients with PVT (PVT group, n=37) were compared to patients who had no PVT (non-PVT group, n=187). RESULTS: According to Yerdel classification, 16, 16, 2, and 3 patients had PVT grade I, II, III, and IV, respectively. Complete venous thrombectomy was accomplished in 34 patients, while for three patients, thrombectomy was not feasible, and graft inflow was established by interposition vascular graft. For portal flow modulation, splenectomy and splenic artery ligation were performed in 7 and 4 patients, respectively, while two patients underwent post-transplant splenic artery embolization. The PVT group had longer operation time (p<0.001), longer warm ischemia time (p=0.031), longer anhepatic phase (p<0.001), and intraoperatively required more than 3 packed RBCs units (p=0.029) and ≥1 platelet unit transfusion (p=0.021) than the non-PVT group. No statistically significant difference was found between groups in terms of re-exploration (p=0.954), post-transplant PVT (p=0.375), biliary (p=0.253) and arterial complications (p=0.593), ICU stay (p=0.633), hospital stay (p=896), and 30-day mortality (p=1.000). Survival analysis showed no statistically significant difference regarding 1-year survival (p=0.176) between both groups. CONCLUSIONS: This study showed that patients with different stages of PVT can successfully undergo LDLT in experienced centers and that they do not differ from patients without PVT in terms of post-transplant complications.

Effect of terlipressin infusion therapy on recipient's hepatic and renal functions in living donor liver transplantations: Experience from a tertiary hospital.

BACKGROUND: Patients with end-stage liver disease are prone to hemodynamic disturbances which may be aggravated with liver transplantation. Blood pooling in splanchnic area and portal hypertension cause reduction in central blood volume. Terlipressin reduces mesenteric and hepatic blood flow, causing vasoconstriction in the smooth muscles of the arteries in the splanchnic region. OBJECTIVE: We investigated the efficacy of perioperative terlipressin infusion in patients who received living donor liver transplantation (LDLT) on hepatic and renal functions. DESIGN: Retrospective. SETTING: University hospital. METHOD: The study included 86 adult patients who received LDLT, due to end-stage hepatic disease, between April 2014 and July 2016 in our institute. Data were collected by searching the medical archives of patients. A standard anesthesia protocol was administered to all patients. In a selected group of patients, terlipressin infusion was initiated at 3 µg/kg/h, immediately after anesthesia was induced. The dose was halved following arterial anastomosis and was continued at this dose for the subsequent 3 days. Patients who received terlipressin infusion were compared with patients who did not receive it. MAIN OUTCOME MEASURES: There is no evidence in this trial to show evidence of effectiveness as a result of terlipressin infusion. RESULTS: Patients in the terlipressin group were statistically significantly older. Central venous pressure, cardiac index, global end diastolic volume, and extravascular lung volume did not show significant differences between the groups. Urine output was similar in both groups; however, regarding the use of packed red blood cells and fresh frozen plasma, terlipressin group patients needed more packs. Perioperative liver function tests were similar between the groups except for aspartate aminotransferase and alanine aminotransferase values on the first and third postoperative days. CONCLUSION: Terlipressin infusion was not found to be significantly effective among the liver and kidney function tests. LIMITATIONS: This may be a result of randomization defect of our retrospective study design. Many prospective randomized studies should be planned to reach more accurate results.

Four Separate Hepatic Vein Reconstructions in Living-Donor Right-Lobe Liver Transplantation: Case Report.

Living-donor liver transplantation (LDLT) with the use of a partial liver graft was established as an option to overcome the donor pool shortage, especially in developing countries. When right-lobe grafts are used for LDLT, appropriate venous drainage of the anterior segment is critical for maximizing the graft capacity. Here, we report a successful LDLT case using a right-lobe graft with 4 hepatic veins that were anastomosed separately to obtain adequate blood flow through the vena cava.

MID TERM RESULTS AFTER OPEN HEART SURGERY IN HEMODIALYSIS PATIENTS AWAITING KIDNEY TRANSPLANT: DOES CARDIOVASCULAR SURGICAL INTERVENTION PRIOR TO TRANSPLANTATION PROLONG SURVIVAL?

The aim of this study was to compare the mid and long term postoperative outcomes between the hemodialysis-dependent patients awaiting kidney transplantat who underwent open heart surgery in our department during the last five years, and those who did not receive a renal transplant, to determine the predictors of mortality, and assess the possible contribution of post heart surgery kidney transplantation to survival. The patients were separated into two groups: those who underwent a transplantation after open heart surgery were included in the Tp+ group, and those who did not in the Tp- group Between June 2008 and December 2012, 127 dialysis dependent patients awaiting kidney transplant and who underwent open heart surgery were separated into two groups. Those who underwent transplantation after open heart surgery were determined as Tp+ (n=33), and those who did not as Tp- (n=94). Both groups were compared with respect to preoperative paramaters including age, sex, diabetes mellitus (DM), hypertension (HT), hyperlipidemia (HL), obesity, smoking, chronic obstructive pulmonary disease (COPD), peripheral vascular disease (PVD), left ventricle ejection fraction (EF), Euroscore; operative parameters including cross clamp time, perfusion time, number of grafts, use of internal mammary artery (IMA); postoperative parameters including revision, blood transfusion, ventilation time, use of inotropic agents, length of stay in the intensive care unit and hospital, and follow up findings. Problems encountered during follow up were recorded. Predictors of mortality were determined and the survival was calculated. Among the preoperative parameters, when compared with the Tp- group, the Tp+ group had significantly lower values in mean age, presence of DM, obesity, PVD, and Euroscore levels, and higher EF values. Assessment of postoperative values showed that blood transfusion requirement and length of hospital stay were significantly lower in the Tp+ group compared to the Tp- group, whereas the length of follow up was significantly higher in the Tp+ group. The use of inotropic agents was significantly higher in the Tp- group. A logistic regression analysis was made to determine the factors affecting mortality. Revision (p=0.013), blood transfusion (p=0.017), ventilation time (p=0.019), and length of stay in the intensive care unit (p=0.009) were found as predictors of mortality. Survival rates at years 1, 2 and 3 were 86.1%, 81%, 77.5% in the Tp- group, and 96.0%, 96.3%, 90.4% in the Tp+ group. Median survival rate was 41.35±2.02 in the Tp- group, and 49.64±1.59 in the Tp+ group which was significantly higher compared to the Tp- group (p=0.048). Chronic renal failure is among the perioperative risk factors for patients undergoing open heart surgery. Transplantation is still an important health issue due to insufficiency of available transplant organs. Patients with chronic renal failure are well known to have higher risks for coronary artery disease. A radical solution of the cardiovascular system problems prior to kidney transplantation seems to have a significant contribution to the post transplant survival.

Effect of prostaglandin E-1 on Wisconsin University and histidine-tryptophan-ketoglutarate preservation solutions on preservation injury of the perfused liver.

BACKGROUND: The aim of this study was to investigate the effects of prostaglandin E-1 (PGE-1) on preservation injury in livers perfused with the University of Wisconsin (UW) or histidine-tryptophan-ketoglutarate (HTK) solutions. MATERIALS AND METHODS: Five groups each including six rats included. Ringer's lactate RL (group 1), HTK (group 2), HTK + PGE-1 (group 3), UW (group 4), or UW PGE-1 (group 5). Liver tissue and preservation fluid samples were obtained from the perfused lives for pathological and biochemical examinations respectively at 0, 6 and 12 hours. RESULTS: Upon biochemical examination, aspartate aminotrasnferase and alanine aminotransferase values were highest among the group with RL solution and lowest with PGE-1. Liver structure was found to be damaged immediately after RL solution, whereas it was preserved in the other four groups. Fewer cellular changes were reported at the end of 12 hours in the groups administered PGE-1 compared with the other groups. CONCLUSIONS: PGE-1 when applied before preservation protected liver functions, decreased pathologic injury, and delayed changes that occur under cold ischemic conditions.

Factors associated with hyperbilirubinemia after living donor hepatectomy.

PURPOSE: Increased serum bilirubin levels are common after living-donor hepatectomy. Little information is available on the characteristics and clinical significance of serum bilirubin levels soon after donor hepatectomy. MATERIALS AND METHODS: Since September 2001, we performed 229 living donor hepatectomies for living-donor liver transplantations. The 128 men and 101 women had a mean age of 34.4±8.9 years (range, 19-66). Most donors were parents (n=110; 48%). We transplanted 110 right lobes, 46 left lobes, and 73 left lateral segments. Donors were divided into 2 groups: Group 1 consisted of 181 donors who showed total bilirubin levels of <3 mg/dL, and group 2, 48 donors with levels of ≥3 mg/dL on postoperative day 3. Preoperative total bilirubin level, ratio of preoperatively estimated remnant liver volume, surgical duration, gender, age, graft type, blood transfusions, and preoperative liver biopsy findings were evaluated as risk factors for hyperbilirubinemia. RESULTS: The mean postoperative maximum total bilirubin level was 2.26±1.49 mg/dL (range, 0.36-9.9). Remnant liver volume<40%, preoperative bilirubin levels>1 mg/dL, right lobe donor hepatectomy, male donor, and abnormal liver biopsy findings were significant risk factors for postoperative hyperbilirubinemia (P=.015, P=.02, P<.01, P=.008, and P=.023 respectively). Also donor age>50 years showed a slight effect on hyperbilirubinemia (P=.052). Blood transfusions and surgical times were not significant factors. CONCLUSION: Donor safety is paramount, requiring thorough donor evaluation. Extensive liver resection may result in transient functional impairment. Several factors are believed to play roles in the development of postoperative hyperbilirubinemia after living-donor hepatectomy.

Comparison of basiliximab and daclizumab with triple immunosuppression in renal transplantation.

PURPOSE: Graft rejection is a serious problem despite immunosuppressive agents. Immunosuppression has been achieved with monoclonal antibodies (mAb) that bind specifically to the α subunit of the interleukin (IL)-2 receptor present on activated T lymphocytes. We explored the effects of two of the mAbs-daclizumab and basiliximab-on graft function. MATERIALS AND METHODS: Our 1543 renal transplant recipients received baseline therapy with cyclosporine or tacrolimus plus corticosteroids and mycophenolate mofetil. In addition standard dosages intravenously of daclizumab (n=156) or basiliximab (n=45) in were administered intravenously to 201 renal transplant patients who included 122 men and 79 women of overall mean age of 30±13.7 years. RESULTS: Patient and donor characteristics including age, sex, causes of renal failure, presence of comorbidities, panel-reactive antibodies, and numbers of human leukocyte antigen-mismatched were similar between the groups. During a mean follow-up of 27±20 months, biopsy-proven acute rejection was observed in three patients in the basiliximab group and 23 in the daclizumab group. Cytomegalovirus infection occurred in 13 patients. There was no case of posttransplant lymphoproliferative disorder. Three polyoma BK nephropathies were detected in the daclizumab group. No hypersensitivity reaction occurred in either group. One-year patient survival was 100% in the basiliximab group and 99% in the daclizumab group, with graft survivals of 95% versus 94%, respectively. The mean creatinine levels at discharge were 2 mg/dL versus 2.3 mg/dL and at 12 months, 1.3 mg/dL versus 1.2 mg/dL, respectively. CONCLUSIONS: Acute rejection episodes remain a significant risk factor for the development of graft dysfunction and poor long-term graft survival. IL-2R antagonists were effective antibody therapies. There was no apparent difference between basiliximab and daclizumab treatment.

Analysis of vascular complications after renal transplantation.

PURPOSE: Despite medical and surgical advances, vascular complications remain common after renal transplant, occurring among 3%-15% of patients. These complications may compromise graft function. This study sought to evaluate the frequency and management of vascular complications after renal transplant. MATERIALS AND METHODS: We retrospectively analyzed the 1843 transplantations performed at 2 centers by our team since November 1975. The 1349 male and 494 female patients had an overall mean age of 31.5±11.2 years; (range, 3-66). Grafts were obtained from a living-related donor in 1406 (76.29%) or a deceased donor in the remaining 437 (23.71%). The mean donor age was 40.7±13.7 years (range, 2-76). Of 1843 transplants, multiple vascular anastomoses were performed in 155 cases (8.4%), including 130 involving renal arteries and 25 renal veins. RESULTS: Forty-seven vascular complications (2.55%) were observed in 43 procedures (2.33%), most frequently renal artery stenosis (n=14). It was followed by allograft renal artery kinking (n=7), renal vein kinking (n=7), renal artery thrombosis (n=5), renal vein laceration (n=4), renal artery laceration (n=3), renal vein thrombosis (n=2), renal artery disruption (n=2), renal and iliac vein obstructions owing to pressure from a lymphocele (n=1), renal artery and vein obstruction owing to pressure from a hematoma (n=1), or an arteriovenous fistula after percutaneous graft biopsy (n=1). Fifteen of these 47 complications were treated by interventional radiologic procedures. CONCLUSION: The vascular complication rates in our patients were somewhat lower than those reported in the literature. A thorough understanding of how complications impair allograft function and survival is essential for adequate treatment. Interventional radiology is invaluable in the postoperative management of transplant-related complications.

Duct-to-duct biliary reconstruction without a stent in pediatric living-donor liver transplantation.

PURPOSE: In pediatric liver transplantation, Roux-en-Y hepaticojejunostomy is often preferred for biliary reconstruction, especially in living-donor liver transplantation (LDLT). Limited numbers of duct-to-duct biliary reconstructions have been presented in pediatric recipients. We retrospectively reviewed our experiences with duct-to-duct biliary reconstruction without a stent in pediatric LDLT recipients. MATERIALS AND METHODS: Since September 2006, 32 LDLTs were performed using a duct-to-duct biliary reconstruction without a stent in 31 children (16 boys and 15 girls; overall mean age, 8.3±5.1 years). We transplanted 19 left lobe grafts, 11 left lateral segments, 1 monosegment, and 1 reduced-size right lobe graft. Twenty-eight grafts had a single bile duct; the remaining 4, two bile ducts. We created a single orifice at the back table for the grafts that had 2 bile ducts. RESULTS: Two recipients developed bile leakage in the early postoperative period; 3 bile duct stenoses occurred in the late postoperative period. All biliary complications were successfully treated with interventional radiologic or endoscopic approaches. There was no morbidity and no graft loss owing to biliary complications. During a mean follow-up of 23.5±13.6 months (range, 4-44), 4 children died and the remaining 27 (88%) are doing well with satisfactory liver function. CONCLUSION: Our results showed that duct-to-duct biliary reconstruction without a stent was a safe technique for biliary reconstruction even among pediatric cases.

Long-term results of hepatitis B immunoglobulin and lamuvidine for hepatitis B prophylaxis after liver transplantation.

PURPOSE: Recurrence of hepatitis B virus after a liver transplantation (OLT) is a risk factor affecting graft and patient survivals. Short-term hepatitis B virus reactivation rates after OLT range between 3% and 15%. Using combination prophylaxis, the outcomes of OLT among patients with liver disease related to hepatitis B virus have improved to levels comparable to those whose disease is not related to hepatitis B virus. MATERIALS AND METHODS: Since September 2001, we performed 288 OLT in 282 patients including 74 who had liver failure related to hepatitis B virus among whom 58 were followed for >12 months and analyzed retrospectively. Our protocol included lamivudine (100 mg orally per day beginning the day after surgery) and hepatitis B immunoglobulin (10,000 IU IV during the anhepatic phase, 2000 IU/d IV during the first week after surgery, 2000 IU IV/month from postoperative months 1 to 12). Using our protocol, the anti-hepatitis B surface antibodies (HBsAb) serum titer was maintained up to 100 IU/mL. The female:male ratio was 11:47. The mean age of patients was 43±12.8 years. RESULTS: Five patients died of causes unrelated to hepatitis B virus. At the time of death, their hepatitis B surface antigens were negative, and serum titers of anti-HBsAb were 45, 35.3, 56.4, 79.6, and 123 IU/mL. Mean follow-up was 46.5±18.9 months (range, 12-79). The hepatitis B surface antigen became positive in 4 patients; the remaining 49 had no evidence of hepatitis B surface antigen. In 18 patients, serum titer of anti-hepatitis B surface antigen was 0; in the remaining 31 patients, it was 69.2±133 IU/mL. CONCLUSION: Our combination protocol with hepatitis B immunoglobulin and lamivudine is a safe, cost-saving, and effective treatment for hepatitis B virus prophylaxis after liver transplantation.

Diagnosis and treatment of late-onset portal vein stenosis after pediatric living-donor liver transplantation.

PURPOSE: Portal vein stenosis is a relatively rare complication after living-donor liver transplantation, which sometimes leads to a life-threatening event owing to gastrointestinal bleeding or graft failure. This study sought to evaluate the diagnoses and management of late-onset portal vein stenosis in pediatric living-donor liver transplants. MATERIALS AND METHODS: Since September 2001, we performed 123 living-donor liver transplant procedures in 120 children, among which 109 children with a functioning graft at 6 months after living-donor liver transplant are included in this analysis. Seven instances of portal vein stenosis were diagnosed and were analyzed retrospectively. RESULTS: The median age of the children was 5.3 years, and the median body weight was 19.2 kg. Portal vein stenosis was diagnosed at 11.2±3.1 months after living-donor liver transplantation. Whereas 3 children were asymptomatic, splenomegaly and/or massive ascites were observed in the remaining 4. Additionally, platelet counts were below the normal limit in 4 children. All children were treated with transhepatic balloon dilatation except 1. Intraluminal stent placement was needed in 1 child owing to resistance of balloon dilatation. The mean pressure gradient decreased from 12.4 to 3.2 mmHg after successful treatment. We did not observe any treatment-related complications. Portal venous patency was maintained in all children during posttreatment follow-up of 43.2±20.4 months. There were no recurrences of portal vein stenosis. One child died; the remaining 6 children are alive with good graft function at 49.8±23.9 months of follow-up. CONCLUSION: Although most portal vein stenosis is asymptomatic, splenomegaly and platelet counts are 2 important markers for portal vein stenosis. Early detection of portal vein stenosis with these 2 markers can lead to successful interventional percutaneous approaches and avoid graft loss.

Management of early hepatic arterial thrombosis after pediatric living-donor liver transplantation.

PURPOSE: Early hepatic arterial thrombosis after living-donor liver transplantation is a cause of graft loss and patient mortality. We analyzed early hepatic arterial thrombosis after pediatric living-donor liver transplantation. MATERIALS AND METHODS: Since September 2001, we performed 122 living-donor liver transplants on 119 children. Ten hepatic arterial thromboses developed in the early postoperative period. The 7 male and 4 female patients of overall mean age of 6.3±6.1 years underwent 5 left lateral segment, 3 right lobe, and 2 left lobe transplantations. RESULTS: Among 10 children with hepatic arterial thrombosis, 8 diagnoses were made before any elevation of liver function tests. One child displayed fever at the time of the hepatic arterial thrombosis. The median time for diagnosis was 5 days. Hepatic arterial thrombosis was treated with interventional radiologic techniques in 9 children, with 1 undergoing surgical exploration owing to failed radiologic approaches, and a reanastomosis using a polytetrafluoroethylene graft. Successful revascularization was achieved in all children, except 1. Four children died, the remaining 6 are alive with good graft function. During the mean follow-up of 52.7±18.8 months, multiple intrahepatic biliary stenoses were identified in 1 child. CONCLUSION: Routine Doppler ultrasonography is effective for the early diagnosis of hepatic arterial thrombosis. Interventional radiologic approaches such as arterial thrombolysis and intraluminal stent placement should be the first therapeutic choices for patients with early hepatic arterial thrombosis; if radiologic methods fail, one must consider surgical exploration or retransplantation.

Role of postreperfusion subcapsular wedge biopsies in predicting initially poor graft function after liver transplantation.

Preservation injury is a major contributing factor to primary allograft failure or poor initial graft function after an orthotopic liver transplant (OLT). We examined the histopathological findings from postreperfusion wedge biopsy specimens in relation to early graft function during the first postoperative week among OLT patients at our center. We reanalyzed subcapsular postreperfusion biopsy specimens from 88 patients to histologically grade the lesions. Grafts were grouped as good function, initial poor function (an alanine aminotransferase or aspartate aminotransferase level >1500 IU/L during week 1), or primary nonfunction (death or retransplantation). Only 1 patient experienced primary nonfunction; the remaining patients fell into the other 2 groups: ie, good function or initial poor function. When patients were compared using numerous morphologic and clinical features, no statistical relation was observed regarding clinical data on bile duct complications, donor type, graft volume, patient age, or type of stent. Histological features of neutrophilic infiltration of the subcapsular region, hepatocellular ballooning, and macro/microvesicular steatosis were not related to initial poor graft function; in contrast, there were prominent sinusoidal neutrophilic infiltrations and hepatocellular necrosis. Preservation-reperfusion injury (grade 2 or grade 3 neutrophilic infiltration) occurred in 78.6% of initial poor function patients and in 39.7% of good function patients. Subcapsular neutrophilic infiltration, a sign of surgical hepatitis, did not provide prognostic information about graft survival. Similar to other studies, we observed neutrophilic infiltration and necrosis away from the capsule to predict subsequent graft function.

Preemptive living donor renal transplantation: a single-center experience.

Renal transplantation is considered preemptive if it occurs before initiation of dialysis. In our experience and in the literature, preemptive transplantation has been shown not only to reduce the costs of renal replacement therapy but also to avoid the long-term adverse effects of dialysis. Preemptive renal transplantation therefore is associated with better survival of both the allograft and the recipient. Our aim was to evaluate the outcomes of preemptive renal transplantation experience at our center. Since 1985, 1385 renal transplantations have been performed at our center. We retrospectively analyzed the 16/1385 recipients (11 male, 5 female) of overall mean age of 28.5 +/- 15 years who underwent preemptive procedures. The causes of end-stage renal failure were focal segmental glomerulosclerosis (n = 5), vesicular ureteral reflux (n = 4), Berger disease (n = 2), polycystic renal disease (n = 2), and others (n = 3). Ten patients were adults, the remaining six, children. The mean creatinine clearance and plasma creatinine levels of the recipients before renal transplantation were 13.5 +/- 8.5 mL/min and 6.7 +/- 2.4 mg/dL, respectively. All renal transplantations were performed from living related donors. The mean preoperative serum creatinine levels, mean glomerular filtration rate, and creatinine clearance rates of the donors were 0.8 +/- 0.1 mg/dL, 61.6 +/- 6.5 mL/min, and 112.5 12 mL/min, respectively. Two episodes of acute cellular rejection and one of humoral rejection occurred during a mean follow-up of 48.7 +/- 14 months (range = 25-76 months). The two patients who experienced graft losses due to humoral rejection or chronic rejection were retransplanted 2 and 48 months thereafter, respectively. At this time all patients are alive with good renal function. In conclusion, our single-center results are promising for preemptive renal transplantation as the optimal, least-expensive mode of treatment for end-stage renal disease.

Conversion to sirolimus for chronic allograft nephropathy and calcineurin inhibitor toxicity and the adverse effects of sirolimus after conversion.

BACKGROUND: Chronic allograft nephropathy and calcineurin inhibitor toxicity may cause graft loss. After kidney transplantation, especially among those patients with chronic allograft nephropathy, sirolimus may be a good alternative to calcineurin inhibitors. Unlike calcineurin inhibitors, sirolimus is devoid of significant nephrotoxicity, but approximately 30% to 50% of patients on sirolimus therapy display mild or severe adverse effects. We sought to report our experience with sirolimus conversion among patients with chronic allograft nephropathy as well as the mild versus severe adverse effects that limit the drug's use. MATERIALS AND METHODS: We analyzed the outcomes of 88 patients (64 men and 24 women) of overall mean age of 35.9 +/- 9.9 years (range, 21-59 years) who had undergone kidney transplantation. Immunosuppressive therapy had been converted from a calcineurin inhibitor to sirolimus because of biopsy-proven chronic allograft nephropathy, calcineurin inhibitor toxicity, or presence of malignancy. We excluded patients with prior acute rejection episodes. Subjects were divided into two groups with respect to their creatinine levels: Group A < 2 mg/dL and Group B >or= 2 mg/dL. After conversion to sirolimus, possible adverse effects of sirolimus were evaluated at the follow-up inset. Each patient underwent a physical examination, and estimation of serum lipid and electrolyte levels as well as hemoglobin concentration. RESULTS: At the time of conversion of the 88 renal transplant patients, their mean duration after grafting was 48 +/- 15 months (range, 4-296). The prior treatment consisted of a calcineurin inhibitor, prednisolone, and mycophenolate mofetil. After conversion, the calcineurin inhibitor was stopped and sirolimus was begun. The 48 Group 2 patients (34 men, 14 women) of overall mean posttransplant time of 22.7 +/- 14.6 months who underwent conversion displayed a mean serum creatinine increase to 3.2 +/- 1.4 mg/dL, including 17 subjects who underwent rejection. The 40 Group 1 patients (30 men, 10 women) with a mean overall posttransplant period of 67.6 +/- 49.9 months showed an fall in serum creatinine level to 1.4 +/- 0.5 mg/dL among only 3 patients. While 5/88 patients showed no increase in proteinuria (5.6%); 83 (94.4%) did experience it. Proteinuria increased from a mean of 192 +/- 316 to 449 +/- 422 mg/d. Only three patients displayed heavy proteinuria (>3 g/d); sirolimus was discontinued for this reason. Proteinuria was well controlled in the other patients with angiotensin-converting enzyme and/or angiotensin II receptor inhibitor agents. After sirolimus conversion, serum cholesterol levels increased from 187 +/- 42 to 214 +/- 52 mg/dL, and serum triglyceride levels increased from 161 +/- 61 to 194 +/- 102 mg/dL. All but four patients responded to statin therapy, with serum lipid levels falling to acceptable levels. Another four patients developed unilateral lower extremity edema with sirolimus discontinued for this reason. One patient displayed generalized arthralgia. CONCLUSION: Chronic allograft nephropathy or calcineurin inhibitor toxicity can lead to loss of graft kidney function. Calcineurin inhibitor toxicity can lead to chronic allograft nephropathy. Patients with a low baseline serum creatinine level who undergo sirolimus conversion showed stabilized kidney function. Late conversion of patients with a serum creatinine above 2 mg/dL face a risk of graft failure. Sirolimus displayed a limited incidence of serious adverse effects; mild or moderate adverse effects, such as hyperlipidemia and proteinuria, were easily controlled with countermeasure therapy.

Living related liver transplantation in Crigler-Najjar syndrome type 1.

Four children underwent living related liver transplantation because of Crigler-Najjar syndrome type 1. Three were infants aged 2, 8(1/2), and 15 months, and weighed 5, 8, and 10 kg, respectively. Pretransplantation unconjugated bilirubin concentration was 22 to 30 mg/dL despite 12 to 14 hours of phototherapy daily. Patient 1, the 2-month-old infant, with unconjugated bilirubin concentration of 30 mg/dL, had a high-pitched cry, suggestive of bilirubin encephalopathy; results of neurologic examination were normal. Plasmapheresis and urgent liver transplantation were performed. Patient 4, a 13-year-old girl, had learning difficulties at school and attended a special class. Three patients received left lateral liver segments, and 1 patient received a left lobe. Biliary reconstruction was completed with duct-to-duct anastomosis. Bile leakage developed at the anastomosis in 2 patients, which was treated successfully with cholangioplasty. In all patients, the unconjugated bilirubin concentration normalized by day 1 posttransplantation, and no phototherapy was necessary. After transplantation, the 2-month-old infant with suspected encephalopathy exhibited hypotonia, spasticity of the lower extremities, and lack of head control. He died after vomitus aspiration during sleep at 10 months posttransplantation. The other 3 patients are alive with normal neurodevelopmental milestones. Irreversible brain damage may occur early in the course of Crigler-Najjar syndrome type 1. Urgent treatment including plasmapheresis, exchange transfusion, phototherapy, and liver transplantation may not reverse brain damage. Young infants must be evaluated carefully for subtle signs and symptoms of bilirubin encephalopathy. Liver transplantation is curative if performed before development of neurologic dysfunction.

The role of valacyclovir on Epstein-Barr virus viral loads in pediatric liver transplantation patients.

INTRODUCTION: Epstein-Barr virus (EBV) infection occurring in the postoperative period represents a significant risk for pediatric transplant recipients. It presents in various manners, including a mononucleosis-like syndrome, hepatitis, encephalopathy, or posttransplant lymphoproliferative disease (PTLD). Valacyclovir has in vitro activity against EBV. We sought to review our experience with valacyclovir on peripheral blood EBV viral loads among a group of EBV-infected patients after liver transplantation (OLT). PATIENTS AND METHODS: Twelve children of ages 6-36 months (median, 12 months), underwent OLT. Eight (66%) were EBV immunoglobulin (Ig)G seronegative at the time of the operation. Eight patients developed primary infection and 4 patients developed reactivation of a post primary infection. Valacyclovir was prescribed immediately to 3 patients when we detected an acute-primary EBV infection. Valacyclovir was prescribed for 2 patients who had primary EBV infections followed by PTLD. Three patients who had primary EBV infection were administered valacyclovir after they became chronically EBV PCR positive for more than 1 year. Four out of 12 cases (33%) were EBV seropositive at the time of OLT, and underwent postprimary EBV reactivation displaying chronic EBV carrier state for 8-10 months before valacyclovir treatment. Peripheral blood EBV viral loads were tested every 2 months. The primary outcome was the proportion of subjects with EBV viremia who had a >or=2 log 10 decrease in EBV copies/mL after valacyclovir treatment. The duration of valacyclovir treatment was a median of 10 months (range, 8-11 months). At the beginning of the treatment period the median level of EBV viral load was 1.1 x 10(4) (range, 1 x 10(4) to 1 x 10(7)). EBV virus was cleared in only 1 patient with primary acute EBV infection. EBV viral loads did not change in 7 of 12 patients and decreased only 1 log 10 (n = 2) or 2 log 10 (n = 2). CONCLUSION: In this small, non-placebo-controlled study, valacyclovir treatment was not effective to decrease peripheral blood EBV viral loads.

Posttransplant lymphoproliferative disease in pediatric liver transplant recipients.

Posttransplant lymphoproliferative disease was first reported in 1968. Posttransplant lymphoproliferative disease encompasses a range of abnormalities from benign infectious mononucleosis-like illnesses to non-Hodgkin's lymphomas with nodal and extranodal site involvement. We evaluated five children who had posttransplant lymphoproliferative disease after liver transplantation. Since 2001, we have performed 118 liver transplantations in 115 children. Five children (4.6%), including three girls and two boys of overall mean age, 3.9 year, developed posttransplant lymphoproliferative diseases. The indications for liver transplant were hepatoblastoma in one recipient and cholestatic liver disease in the remaining four subjects. Posttransplant lymphoproliferative disease was diagnosed at 6, 11, 17, 22, and 27 months after the liver transplantation. Imaging modalities identified generalized lymphadenopathy in one, multiple liver masses in one, a large portal mass in one, multiple stomach ulcers in one, and a large mediastinal mass in one recipient. At diagnosis, the recipient with the large mediastinal mass displayed cough; the remaining four recipients were asymptomatic. Histological findings showed B-cell lymphomas in three recipients and T-cell lymphomas in two. The results of in situ hybridization for Epstein-Barr virus were negative in one recipient and positive in four. Four recipients were treated with chemotherapy; the remaining recipient was treated with anti-CD20 monoclonal antibodies. The one recipient who had a large mediastinal mass died at 2 months after receiving the diagnosis of chemotherapy-related sepsis; the remaining four children are alive at 9, 11, 18, and 34 months after treatment. Our rate of posttransplant lymphoproliferative disease was similar to that in the literature. From a few months to several years after liver transplantation, radiologists must be alert to the possibility of posttransplant lymphoproliferative disease. Thorough imaging is required to detect the wide variety of potential presentations.

Liver transplantation in management of alveolar echinococcosis: two case reports.

Hepatic alveolar echinococcosis is an infectious disease caused by the larval stage of Echinococcus multilocularis, which grows primarily in the liver of an infected person and develops as a tumorlike lesion. In advanced cases, the organisms infiltrate every organ neighboring the liver and spread hematogenously to distant organs such as lungs and brain. Surgical resection and liver transplantation are accepted treatment options for early and advanced disease, respectively. Herein, we present case reports of 2 patients with advanced alveolar echinococcal disease that invaded both lobes of the liver and neighboring vital structures including the inferior vena cava. Despite the technical difficulty of the surgery, both patients were successfully treated with living donor liver transplantation. Liver transplantation should be accepted as a life-saving treatment of choice in patients with alveolar echinococcosis for whom there is no other medical or surgical treatment options.