Co-existence of chronic renal failure, renal clear cell carcinoma, and Blau syndrome

Blau syndrome is a rare, multisystem, autosomal-dominant, and granulomatous disorder caused by susceptibility variants in the NOD2 gene. We describe here a 14-year-old girl with Blau syndrome with incidentally diagnosed renal carcinoma. The index case presented with growth retardation and recurrent symmetric arthritis. Her clinical symptoms included bilateral cataract due to recurrent uveitis, camptodactyly, and persistent erythematous rash with ichthyosis. Her two sisters and her mother were affected with combinations of these conditions—symmetric polyarthritis, uveitis, and skin involvement—suggesting an autosomal dominant trait. The index case developed a chronic renal insufficiency, and an abdominal computerized tomography scan revealed a 2.5-cm mass in the left kidney. The histopathological examination showed renal clear cell carcinoma, chronic tubulointerstitial nephritis, and giant cell granulomas in both the tumor and non-neoplastic renal tissue. Granulomatous inflammation was observed in the skin biopsy specimen. The patient was diagnosed with Blau syndrome based on her family history, uveitis, granulomatous inflammation proved by skin biopsy, and polyarthritis. Sequencing of the NOD2 gene showed a heterozygous p.R334Q mutation in all affected family members. To the best of our knowledge, this is the first reported case of a patient with Blau syndrome accompanied by chronic renal failure and renal carcinoma.     

Immunoprophylaxis with basiliximab,a chimeric anti[ndash ]interleukin-2 receptor monoclonal antibody,in combination with azathioprine-containing triple therapy in liver transplant recipients

Acute graft rejection remains a major problem among additional sequelae in liver transplant recipients. Basiliximab, a chimeric monoclonal antibody with high affinity for the CD25 chain of the interleukin-2 receptor, has significantly reduced the incidence of acute rejection episodes in renal transplant recipients. This single-arm, open-label, multicenter study investigated the efficacy and tolerability of basiliximab immunoprophylaxis in adult patients undergoing first elective liver transplantation. One hundred one patients (70 hepatitis C virus [HCV]-negative patients, 31 HCV-positive patients) were administered basiliximab, 20 mg, by intravenous bolus injection the day of transplantation (day 0) and day 4. In addition, all patients were administered triple immunosuppressive therapy with cyclosporine, steroids, and azathioprine. The efficacy of basiliximab was assessed by conventional parameters, and tolerability was assessed by the incidence of adverse events, infections, and laboratory test result abnormalities. At 6 months, the incidence of first acute biopsy-confirmed rejection episodes was 22.8%. Rejections were more frequent in the HCV-positive (29.0%) than HCV-negative subgroup (20.0%; P = .441). No rejection episode was graded histologically as severe, and no patient required antibody therapy for the management of acute rejection. Ten patients (9.9%) required treatment with tacrolimus for acute rejection episodes. Patient and graft survival rates at 12 months were 90.1% and 88.1%, respectively. Basiliximab caused no injection-site reactions, anaphylaxis, or cytokine release syndrome. Five malignancies were reported at 12 months: of these, three malignancies predated transplantation surgery. Compared with earlier studies, the addition of basiliximab immunoprophylaxis to triple immunosuppressive therapy provides increased efficacy in reducing the incidence of acute rejection episodes, with no clinically significant increase in adverse events. (Liver Transpl 2002;8:123-131.)     

Sinus of valsalva aneurysm in Blau's syndrome

Blau syndrome is a rare granulomatous disorder inherited in an autosomal dominant manner characterized by the early appearance of granulomatous arthritis, skin rash and anterior uveitis. There are very few data on the cardiovascular manifestations of Blau syndrome. Here we report the first case of sinus of valsava aneurysm in Blau syndrome. In isolated unruptured aneurysms of a sinus of Valsalva without compromise of the aortic valve and/or the coronary ostia, repair may be accomplished by simple placation of the aneurysm or excision of the aneurysm(s) and patch closure of the defect(s) between the aortic annulus and the sinu-vascular ridge. Because of the particular conditions in our case, the repair was performed with replacement of the aortic valve and root using a composite graft employing a modified Bentall's technique.     

Thrombotic Microangiopathy After Kidney Transplantation

Thrombotic microangiopathy (TMA) is a severe complication of kidney transplantation that often causes graft failure. TMA may occur de novo, often triggered by immunosuppressive drugs and acute antibody‐mediated rejection, or recur in patients with previous history of hemolytic uremic syndrome (HUS). Recurrent TMA is very rare in patients who had developed end‐stage renal failure following HUS caused by Shiga‐toxin producing E. scherichia coli, whereas disease recurrence is common in patients with atypical HUS (aHUS). The underlying genetic defect greatly impacts the risk of posttransplant recurrence in aHUS. Indeed recurrence is almost the rule in patients with mutations in genes encoding factor H or factor I, whereas patients with a mutation in membrane‐cofactor‐protein gene have a good transplant outcome. Prophylactic and therapeutic options for posttransplant TMA, including plasma therapy, combined kidney and liver transplantation and targeted complement inhibitors are discussed in this review.     

Auxiliary partial orthotopic liver transplantation for Crigler-Najjar syndrome type I

OBJECTIVE: To determine if auxiliary partial orthotopic liver transplantation (APOLT) has the long-term potential to correct the underlying abnormality in Crigler-Najjar syndrome type 1 (CNS1) without the need for total liver replacement. BACKGROUND: Orthotopic liver transplantation has been used successfully to replace the defective enzyme in CNS1. Experimental studies have shown that only 1% to 2% of the normal hepatocyte mass is needed for bilirubin conjugation. If APOLT corrects the underlying metabolic abnormality, it has the advantage of preserving the native liver, which would serve as a "safety net" should the graft fail, and there is the potential for gene therapy in the future with possible withdrawal of immunosuppression. METHODS: Seven APOLT procedures were performed in six recipients with CNS1. Median age at transplantation was 10.5 years. Six transplants were performed as a left auxiliary liver transplant, and one was performed as a right auxiliary liver transplant. Median serum bilirubin level at transplantation was 320 micromol/L. All patients required 12 to 16 hours of phototherapy daily before the transplant to maintain serum bilirubin levels between 250 and 350 micromol/L. RESULTS: Median serum bilirubin level was 50 micromol/L at day 5 after the transplant and 23 micromol/L at a median follow-up of 32 months. In four children, early severe acute rejection developed, requiring conversion to tacrolimus; one underwent a second transplant for chronic rejection and graft atrophy but died from lymphoproliferative disease 6 months after the second transplant. CONCLUSIONS: This report shows that APOLT is technically feasible and provides adequate hepatocyte mass to correct the underlying metabolic abnormality in CNS1.     

Disseminated Bartonella infection following liver transplantation.

Bartonella henselae has not only been identified as the causative agent of cat scratch disease, but it is also associated with other significant infectious syndromes in the immunocompromised population. We describe two cases of B. henselae associated diseases in liver transplant recipients who both had contact with cats. The first recipient developed localized skin manifestation of bacillary angiomatosis in association with granulomatous hepatitis. He tested positive for Immunoglobulin G (IgG) antibodies against B. henselae. The second patient developed axillary lymphadenopathy, with biopsy showing necrotizing granulomatous inflammation and polymerase chain reaction studies were positive for B. henselae DNA. Her serology for bartonellosis showed a fourfold rise in antibody titers during her hospitalization. Both patients responded to treatment with Azithromycin in combination with Doxycycline. These were the only cases within a series of 467 consecutive liver transplants performed in 402 patients performed during a 4-year period. Although bartonellosis is a rare infection in liver transplantation recipients, it should always be included in the differential diagnosis of patients presenting with fever, central nervous system (CNS) symptoms, skin lesions, lymphadenopathy, and hepatitis especially if prior contact with cats is reported.     

The role of transplantation in liver disease

Liver transplantation is rapidly emerging as the most effective treatment pathway for a growing number of acute and chronic liver disease states. Indications and contraindications to transplant are undergoing continuous revision and clarification as experience is accrued in the expanding number of treatment centers. For some disorders such as primary biliary cirrhosis, sclerosing cholangitis, and chronic active hepatitis with cirrhosis, the role of transplantation in patient management is obvious. For other hepatic diseases such as primary hepatic neoplasm, clear definition of the role of transplantation is likely to await development of improved early diagnostic techniques and more effective chemotherapy regimens. Standardization of the technical aspects of liver transplant and recent advances in graft preservation have led to reduction in the logistical problems that previously plagued this complex therapy. Refinements in immunosuppression with the introduction of cyclosporine and monoclonal antibody therapy have extended chances for survival and contributed to considerable improvement in quality of life following transplant. Further extension of transplantation as a treatment option to individuals with liver disease will require the concerned effort of the primary care or referral physician in the early recognition and management of patients with liver disease.     

Hepatic artery thrombosis and liver malignancy in pediatric liver transplantation

BackgroundHepatic artery thrombosis (HAT) remains a significant cause of graft failure and mortality after pediatric liver transplantation. Conditions not associated with hepatic failure, such as liver tumors, may be more prone to thrombotic problems after transplant. We hypothesized that liver transplant for hepatic malignancies may be associated with increased rates of HAT in the posttransplant period.MethodsWe conducted a retrospective review of pediatric patients (age, 0-21 years) who underwent primary liver transplantation at a free-standing children's hospital from 1990 to 2009. We reviewed cause of underlying liver disease, age, sex, weight, occurrence of HAT, use of antiplatelets and anticoagulants perioperatively, as well as reintervention, retransplant, and death.ResultsA total of 129 children underwent 146 liver transplants, and 15 (12%) patients developed HAT. Nine liver transplants were performed for hepatic malignancy, and 4 (44%) of these patients developed HAT (relative risk, 4.85; 95% confidence interval, 1.9-12.2; P = .0015). All 4 children with hepatic malignancy and HAT required reintervention, including 3 retransplants (75%). One of these patients died.ConclusionsHepatic artery thrombosis occurs approximately 5 times more often and appears to be more morbid in children with hepatic malignancy after transplantation. Prospective evaluation of prophylactic anticoagulation regimens in the setting of hepatic malignancy requiring transplantation is warranted.     

Selection of live-related liver transplantation candidates

Purpose

Living donor liver transplantation (LR) is an important alternative for children. We compared our outcomes of LR and cadaveric (CAD) graft recipients, with attention to the pediatric end-stage liver disease (PELD) score and perioperative morbidity and mortality to identify appropriate candidates for LR.

Methods

Our transplant database and electronic medical records were searched for demographics and outcome measures.

Results

From 2000 to 2008, 81 children underwent liver transplantation from 37 LR and 44 CAD donors. There were no significant differences in graft or overall survival at 3 months or 1 year. The LR group was significantly younger (4.46 ± 5.2 years vs 7.41 ± 6.6 years; P = .03) and had a significantly lower PELD score (12.7 ± 13 vs 22 ± 12; P = .001) at the time of transplantation. Ten patients were transplanted for unresectable tumor in the LR group vs 4 CAD (P = .03). Significantly fewer LR recipients required return to the operating room in the first 30 days posttransplant (13.9% vs 34.1%; P = .03). The LR recipients had a higher rate of biliary stricture requiring reoperation (22.2% vs 2.3%; P = .005).

Conclusions

The LR liver transplantation is highly selected for patients with a parent donor who will need transplant but do not yet have a high PELD score. A lower PELD score at operation may have contributed to the lower incidence of postoperative complications requiring reoperation.     

Successful staged kidney and liver transplantation for glycogen storage disease type Ib: A case report

Glycogen storage disease type Ib is a rare metabolic disease caused by a defect of the G6P transporter. Patients suffer from hypoglycemic episodes; growth and developmental delay; osteoporosis; neutropenia; and tendency to infections, ovarian cysts, and liver adenomas. Terminal kidney disease is a rare complication. Liver transplantation has been performed to prevent malignant transformation of hepatic adenomas. We present the case of a female patient with glycogenosis type Ib who had severe hypoglycemic episodes and recurrent infections since early childhood. She became dialysis dependent at the age of 24 years. Kidney transplantation was performed at age 30, and liver transplantation 2 years later. The main indication for liver transplantation were the persistent, therapy-refractory hypoglycemic episodes. The transplanted kidney function is stable. The liver transplantation resulted in the disappearance of hypoglycemic episodes, with the patient leading a normal life and eating a normal diet. The neutropenia did not recover, but there were no more significant infectious episodes after liver transplantation. This is, to the best of our knowledge, the first communication of a dual kidney and liver transplant performed in a patient with glycogenosis type Ib. It confirmed the beneficial effect of liver transplantation on the quality of life of patients with severe hypoglycemia. The transplantation should be attempted earlier in the course of the disease to reduce complications and allow catch-up growth. Hepatocyte transplantation may be considered; however, long-term results seem to be rather poor in the few documented cases.     

Model for End-Stage Liver Disease score does not predict patient or graft survival in living donor liver transplant recipients

Although living donor liver transplantation (LDLT) is a successful procedure for most recipients, outcomes in patients who undergo transplantation as United Network for Organ Sharing status 2A are marginal. There are no published data on living donor liver transplant recipient outcomes relative to Model for End-Stage Liver Disease (MELD) scores. Such information could be useful in living donor liver transplant recipient selection. We retrospectively analyzed all non-fulminant hepatic failure, right hepatic lobe, adult-to-adult living donor liver transplant recipients at our center between August 1997 and March 2002. We calculated MELD scores at the time of LDLT and correlated scores with 1-year patient and graft survival and hospital days during the 90-day post-LDLT period. There were 62 recipients with greater than 6 months of follow-up: 38 men, 24 women; mean age, 47.9 years; 42 white, 1 black, 17 Hispanic, and 2 Asian patients. Twenty-nine patients had hepatitis C virus infection; 4 patients, hepatitis C virus infection and alcoholic liver disease; 4 patients, alcoholic liver disease; 4 patients, cryptogenic cirrhosis; 13 patients, primary sclerosing cholangitis; 5 patients, autoimmune hepatitis; and 3 patients, primary biliary cirrhosis. Mean and median MELD scores were 15.2 and 13, respectively (range, 6 to 40). One-year patient and graft survival were 59 of 62 patients (95%) and 52 of 62 patients (84%), respectively. There was no statistically significant difference between median MELD scores of dead versus living patients (15 v 13; P = .15) or patients who underwent retransplantation versus those who did not (16.5 v 13; P = .30). Mean and median hospital days in the 90-day post-LDLT period were 23.7 and 16.0 days, respectively. Living donor liver transplant recipients with a MELD score of 18 or greater had significantly more hospital days compared with recipients with a MELD score less than 18 (35.2 v 19.8 days; P = .01). In conclusion, MELD scores did not predict post-LDLT patient or graft survival at 1 year. However, higher MELD scores (≥18) were associated with more hospital days during the 3-month post-LDLT period. (Liver Transpl 2003;9:737-740.)     

Living donor liver transplantation for end-stage liver disease with severe hepatopulmonary syndrome: report of a case

Hepatopulmonary syndrome (HPS) is a serious complication of terminal liver disease, which manifests as severe hypoxia without any pulmonary anatomic or functional causes. The precise indications for liver transplantation in patients with severe HPS also remain unclear. A 49-year old woman was referred to our department for investigation and management of liver cirrhosis with severe hypoxia (PaO₂, 38 mmHg). A pulmonary perfusion scintigram showed an intrapulmonary shunt ratio of 40%, confirming a diagnosis of severe HPS. She underwent living donor liver transplantation using a right lobe graft donated by her 27-yearold daughter. Her post-transplant graft function was excellent, and her oxygenation recovered slowly but steadily. She was discharged from hospital on posttransplant day 39 with an SpO₂ of 94% under 3 l/min of O₂, delivered nasally. Despite the severity of the HPS, her postoperative recovery was smooth after the liver transplant.     

Use of eculizumab and plasma exchange in successful combined liver–kidney transplantation in a case of atypical HUS associated with complement factor H mutation

Background

Atypical hemolytic uremic syndrome (aHUS) evolves into end-stage renal failure in nearly half of affected patients and is associated with defective regulation of the alternative complement pathway. Patients with a complement factor H (CFH) mutation have a 30–100% risk of graft loss due to aHUS recurrence or graft thrombosis. Since CFH is produced predominantly by the liver, combined liver–kidney transplant is a curative treatment option. One major unexpected risk includes liver failure secondary to uncontrolled complement activation. We report a successful combined liver–kidney transplantation with perioperative plasma exchange and use of the humanized anti-C5 monoclonal antibody eculizumab.

Case Diagnosis/Treatment

An 11-month-old female presented with oliguric renal failure after 3 weeks of flu-like symptoms in the absence of diarrhea. Following the identification of Escherichia coli 0157:H7 in her stool, she was discharged home on peritoneal dialysis with a diagnosis of Shiga toxin-associated HUS. Three months later, she developed severe anemia, thrombocytopenia, and neurological involvement. aHUS was diagnosed and confirmed, and genetic testing revealed a mutation in CFH SCR20. Once donor organs became available, she received preoperative plasma exchange followed by eculizumab infusion with intra-operative fresh frozen plasma prior to combined liver–kidney transplant. At 19 months post-transplant, she continues to have excellent allograft and liver function without signs of disease recurrence.

Conclusion

Perioperative use of eculizumab in conjunction with plasma exchange during simultaneous liver–kidney transplant can be used to inhibit terminal complement activity, thereby optimizing successful transplantation by reducing the risk of graft thrombosis.     

Long-term outcomes of retransplantation after live donor liver transplantation: A Western experience

BackgroundDespite most liver transplants in North America being from deceased donors, the number of living donor liver transplants has increased over the last decade. Although outcomes of liver retransplantation after deceased donor liver transplantation have been widely published, outcomes of retransplant after living donor liver transplant need to be further elucidated.MethodWe aimed to compare waitlist outcomes and survival post-retransplant in recipients of initial living or deceased donor grafts. Adult liver recipients relisted at University Health Network between April 2000 and October 2020 were retrospectively identified and grouped according to their initial graft: living donor liver transplants or deceased donor liver transplant. A competing risk multivariable model evaluated the association between graft type at first transplant and outcomes after relisting. Survival after retransplant waitlisting (intention-to-treat) and after retransplant (per protocol) were also assessed. Multivariable Cox regression evaluated the effect of initial graft type on survival after retransplant.ResultsA total of 201 recipients were relisted (living donor liver transplants, n = 67; donor liver transplants, n = 134) and 114 underwent retransplant (living donor liver transplants, n = 48; deceased donor liver transplants, n = 66). The waitlist mortality with an initial living donor liver transplant was not significantly different (hazard ratio = 0.51; 95% confidence interval, 0.23–1.10; P = .08). Both unadjusted and adjusted graft loss risks were similar post-retransplant. The risk-adjusted overall intention-to-treat survival after relisting (hazard ratio = 0.76; 95% confidence interval, 0.44–1.32; P = .30) and per protocol survival after retransplant (hazard ratio:1.51; 95% confidence interval, 0.54–4.19; P = .40) were equivalent in those who initially received a living donor liver transplant.ConclusionPatients requiring relisting and retransplant after either living donor liver transplants or deceased donor liver transplantation experience similar waitlist and survival outcomes.     

Deferred versus prophylactic therapy with gancyclovir for cytomegalovirus in allograft liver transplantation

OBJECTIVE: The aim of this study was to assess the efficacy of deferred versus prophylactic therapy with gancyclovir to prevent cytomegalovirus (CMV) infection or disease in liver transplantation recipients, and to alter the timing of infection or the incidences of acute rejection, chronic rejection, or death. METHODS: We retrospectively studied 89 consecutive liver transplant recipients with a minimum of 1 year follow-up. CMV early antigen detection (pp65) was performed weekly for the first 2 months and thereafter monthly for an additional 10 months. Forty-one recipients were administered prophylactic treatment and (48 recipients) deferred therapy for positive antigenemia. RESULTS: During the first year after transplantation, CMV infection or disease developed in 61% or 12.2% of those treated with prophylactic therapy and 54.1% or 31.3% of those treated with deferred therapy (P = 0.51 or P = 0.032, respectively). The mean time to CMV disease in the prophylactic group was 161 +/- 33 days compared with 82 +/- 27 days for the deferred therapy arm (P < 0.001). Subgroup analysis based on CMV serological status also showed prophylactic treatment significantly diminished CMV disease in the CMV IgG antibody negative group. No patients died in the prophylactic group, and one died in the deferred group (P = 0.54). The incidence of acute rejection episodes was 34% in the prophylactic and 46% in the deferred group (P = 0.26). Chronic rejection was observed in two recipients in the prophylactic group versus one recipient in the deferred arm (P = 0.35). CONCLUSION: Compared with deferred therapy prophylactic therapy with gancyclovir decreased CMV disease and delayed the onset of CMV disease after liver transplantation.     

Living donor liver transplantation using a right lobe graft in an adult with situs inversus.

Situs inversus totalis is a rare anatomic variant in which there is a complete mirror image of the thoracic and abdominal viscera. The common association of situs inversus and biliary atresia has led to a variety of modifications of surgical techniques utilizing both living donor and deceased donor liver grafts, with mixed results in the pediatric liver transplant population. The use of a living donor liver graft in an adult with situs inversus has not yet been described. However, living donor liver transplantation (LDLT) has produced excellent results in the adult population, particularly in the cholestatic population, which may be disadvantaged by the model for end-stage liver disease system. This is the first report of a successful living donor right liver graft in an adult with end-stage liver disease secondary to primary sclerosing cholangitis and situs inversus totalis.     

Living-donor liver transplantation for homozygous familial hypercholesterolemia from a donor with heterozygous hypercholesterolemia

Familial hypercholesterolemia is a rare inherited disease with an incidence of approximately one per million. Severe hypercholesterolemia is observed from the time of birth onwards. It is associated with severe atherosclerosis in childhood, leading to death from myocardial infarction before the age of 20 years. Liver transplantation is the only effective treatment for this disease. We experienced the case of an infant aged 2 years 5 months who had homozygous familial hypercholesterolemia and who received a liver graft from his father, who had familial heterozygous hypercholesterolemia. The pre-operative plasma cholesterol level was >800 mg/dl. After liver transplantation, the recipient's cholesterol level decreased to 250 mg/dl after we administered the HMG-CoA reductase inhibitor. At present, 6 months after transplantation, the patient is doing well and free from a special diet. We can thus conclude that the combination therapy of liver transplantation from a donor with heterozygous familial hypercholesterolemia on cholesterol-lowering drugs is an effective therapy for a patient with the homozygous type of hypercholesterolemia.     

Potential immunological advantage of intravenous mycophenolate mofetil with tacrolimus and steroids in primary deceased donor liver transplantation and live donor liver transplantation without antibody induction.

With the current immunosuppressive regimens, graft loss secondary to immunological reasons after successful liver transplantation is a rarity; acute rejections, however, do occur, with the majority of them being steroid-responsive. The aim of the present study is to examine the rate of acute rejection with tacrolimus, intravenous (IV) mycophenolate mofetil (MMF), and steroids in primary deceased donor liver transplant (DDLT) and live donor liver transplant (LDLT) recipients. During the year 2005, 130 patients (mean age: 54.9 +/- 10.8, males: 84, females: 46, 112 DDLT and 18 LDLT) received primary liver transplantation. They were followed up for the incidence of acute rejection in the first 12 months. Liver biopsies were performed as clinically indicated; protocol liver biopsies were never performed. A total of 127 liver biopsies were performed. Thirty-two had a rejection activity index (RAI) score of > or =3, of which 24 biopsies in 20 patients were not treated with a steroid bolus. Eight (6.1%) patients (mean RAI score: 5.1 +/- 1.4) received 750 to 1500 mg of methylprednisolone over 3 days. Out of these, 2 were noncompliant, 4 were off MMF, and 1 was on cyclosporine. All patients responded to steroid therapy. None of the patients required any antibody preparation. In conclusion, IV MMF with tacrolimus and steroids is useful and required antirejection therapy in 6.1% of liver transplant recipients.     

Long-term analysis of combined liver and kidney transplantation at a single center

OBJECTIVE: To analyze use of combined liver and kidney transplantation (CLKT) for patients with chronic primary diseases of both organs and for patients with hepatorenal syndrome. DESIGN: Retrospective case series. SETTING: Multiorgan transplantation service in a large university medical center. PATIENTS: A total of 98 patients underwent 99 CLKTs during a 16-year period; 76 had primary renal diseases, and 22 had hepatorenal syndrome. Patients receiving isolated liver and kidney transplants were analyzed for comparison. MAIN OUTCOME MEASURES: Patient and graft survival, rejection rates, and need for hemodialysis before and after transplantation. RESULTS: Overall patient survival was 76%, 72%, and 70% at 1, 3, and 5 years, respectively; liver graft survival was 70%, 65%, and 65%; and kidney graft survival was 76%, 72%, and 70%. No risk factors analyzed for recipients or donors were associated significantly with early posttransplantation mortality or graft loss. In 28 patients who received monoclonal antibody induction therapy with interleukin 2 blockers, there were significantly fewer episodes of acute liver rejection. For patients with hepatorenal syndrome, CLKT did not confer a survival advantage over liver-only transplantation (1-year patient survival was 72% vs 66%; P = .88). The 1-year acute kidney rejection rate in the adult CLKT group was 14% vs 23% in a 5-year cadaveric renal transplantation cohort (P<.01). CONCLUSIONS: First, CLKT is indicated in patients with dual organ disease and achieves excellent results. Second, CLKT for hepatorenal syndrome is indicated in patients receiving hemodialysis for longer than 8 weeks and confers advantages in patient survival and use of hospital resources. Third, the liver is immunoprotective for the kidney.     

Pediatric liver transplantation with daclizumab induction

BACKGROUND: A new class of monoclonal antibodies (non-T-cell depleting) has gained favor for induction therapy after transplantation. This study evaluated the non-T-cell depleting antibody to the CD25 cell, daclizumab, as a single-dose induction agent immediately after pediatric liver transplantation to spare the use of the calcineurin inhibitor, tacrolimus, for 7 days in respect to both efficacy and renal function. METHODS: From January 1998 to November 2001, 81 pediatric orthotopic liver transplant recipients receiving 89 liver grafts were evaluated. The treatment arm (n=61) received daclizumab 1 mg/kg immediately after liver transplantation along with mycophenolate, steroids, and, on postoperative day 7, tacrolimus. The control group did not receive induction therapy, whereas tacrolimus, mycophenolate, and steroids were started immediately after surgery. RESULTS: The induction group had fewer patients with rejection within the first 30 days after liver transplantation (9 [14.8%] vs. 10 [50%]; P=0.003). The mean time to first rejection was similar between groups (12.1 [+/-7.8] days vs. 18.5 [+/-8.1] days; P=not significant). There was a 3.39 increase in relative risk to develop rejection within the first 30 days after orthotopic liver transplantation if the patient did not receive induction therapy (relative risk=3.39; 95% confidence interval [1.61, 7.14]). Two-year actuarial survival for the induction group was 93.2% compared with 85% in the control; graft survival was also similar between groups (87.8% vs. 72.7%) at 2 years. CONCLUSION: Daclizumab 1 mg/kg given immediately after pediatric liver transplantation and withholding tacrolimus, is safe, efficacious, and reduces rejections within the first 30 days after surgery.