Refractory Hypernatremia and Osmotic Demyelination Syndrome After Liver Transplantation: A Case Report.

Osmotic demyelination syndrome is an uncommon neurologic condition, characterized by noninflammatory demyelination involving the pons and other areas of the central nervous system. As chronic hyponatremia is frequently associated with cirrhosis, patients undergoing liver transplantation are at an increased risk for developing this condition. We report the case of a patient who developed refractory hypernatremia and osmotic demyelination syndrome after liver transplantation. The patient was a 40-year-old man, who underwent liver transplantation for the treatment of cryptogenic cirrhosis, and had a preoperative sodium level of 128 mmol/L. Although there were no intraoperative complications, the patient showed signs of mental confusion and drowsiness in the second postoperative day, and we noticed an increase to 136 mmol/L in his serum sodium. Treatment with 5% dextrose and desmopressin was initiated, but his serum sodium continued to increase steadily, while his neurologic condition gradually worsened. Serum sodium rose to 157 mmol/L, and a magnetic resonance imaging of the brain showed extensive lesions consistent with osmotic demyelination syndrome. The clinical condition of the patient continued to deteriorate until his death 17 days after the transplant. Although the occurrence of this syndrome after liver transplantation is well described, the steady increase in serum sodium despite early treatment, as described in this case, is highly unusual, and highlights the great attention that must be taken with monitoring and control of serum sodium in patients who undergo liver transplant in the context of chronic hyponatremia. This manuscript is compliant with the Helsinki Congress and the Istanbul Declaration.

Incidental Finding of Multiple Synchronous Neoplasms in Explanted Liver After Transplantation: A Case Report.

Liver transplantation is the only potentially curative treatment for patients with end-stage liver disease. After the procedure, histopathologic analysis of the liver explant may reveal neoplasms that were not previously diagnosed in preoperative imaging examinations. This incidental finding of primary liver neoplasms in the explant is not an uncommon situation in liver transplant, and hepatocarcinomas and cholangiocarcinomas are the types of tumors most frequently encountered in this scenario. These are the most common primary neoplasms of the liver, and liver transplantation is often a curative treatment for these types of tumors when they are in their earlier stages. In contrast, liver plasmacytoma is a rare type of plasma cell neoplasm, consisting of a single mass of monoclonal plasma cells, which is treated primarily by radiotherapy and is seldom encountered in the setting of liver transplant. We report the case of a patient who underwent liver transplantation for the treatment of cryptogenic cirrhosis, with no preoperative diagnosis of liver tumors. Analysis of the liver explant revealed the presence of three synchronous neoplasms with different histologic origins: a 27-mm hepatocellular carcinoma, a 17-mm intrahepatic cholangiocarcinoma, and a 25-mm solitary hepatic plasmacytoma. The patient received no further adjuvant treatment and remained well and with no signs of disease recurrence over an observation period of 44 months. We found no previous report in the literature of the synchronous presence of these three types of liver neoplasms.

Progressive Familial Intrahepatic Cholestasis Type 2 and Recurrence After Liver Transplantation: A Case Report.

Progressive familial intrahepatic cholestasis type 2 (PFIC2) is a rare autosomal recessive disorder caused by mutations in the ABCB11 gene. Clinical manifestations include cholestasis with low γ-glutamyltransferase (GGT), hepatosplenomegaly, and severe pruritus. Liver transplantation is required for individuals with progressive liver disease or failure of the bypass procedure and has been considered curative. However, in the case of PFIC2, although bile salt excretory pump (BSEP) deficiency is a liver-specific condition rather than a systemic disease, evidence of recurrent BSEP disease has been shown in a small proportion of allografts. We describe an unusual case of a 21-year-old individual with PFIC2 and evidence of recurrent BSEP disease after liver transplantation, with clinical and laboratory improvement after pulse therapy with methylprednisolone for 3 days and adjustment of oral immunosuppression. This case report highlights the recurrence of PFIC2 in patients post liver transplant. It also emphasizes the importance of clinical suspicion, which should be considered in cases of posttransplant cholestasis in PFIC2 patients, especially those with low γ-glutamyltransferase (GGT) and without signs of acute graft rejection. Having knowledge of the condition favors a targeted diagnostic approach and contributes to early therapeutic management and a higher success rate.

Preoperative Alpha-Fetoprotein and Radiological Total Tumor Diameter as Predictors of Hepatocellular Carcinoma Recurrence After Liver Transplantation.

BACKGROUND: Liver transplantation is a unique treatment opportunity for patients with chronic liver disease and hepatocellular carcinoma (HCC). Selection of HCC patients for transplantation was revolutionized by Milan-based criteria, but tumor recurrence and shortage of organs are still a major concern. Nowadays, additional preoperative tumor parameters can help to refine the graft allocation process. The objective of this study was to evaluate the prognostic value and cut-off points of pretransplant serum alpha-fetoprotein (AFP) levels and radiological tumor parameters on liver transplantation outcomes. METHODS: This is a single-team retrospective cohort of 162 consecutive deceased donor liver transplants (DDLT) with pathologically confirmed HCC. Pretransplant serum AFP levels and radiological tumor parameters were retrieved from a preoperative follow-up. Receiver-operating characteristics (ROC) curves were used to evaluate cut-off points for each outcome. Multivariate Cox regression model was used to assess the predictors of HCC relapse and recipient mortality. RESULTS: Twelve recipients (7.4%) had HCC recurrence after transplantation, with median survival time of 5.8 months. Pretransplant AFP ≥30 ng/mL (hazard ratio [HR]: 13.84, P = .003) and radiological total tumor diameter (TTD) ≥5 cm (HR: 12.89, P = .005) were independent predictors for HCC relapse. Moreover, pretransplant AFP ≥150 ng/mL was independently associated with recipient mortality (HR: 4.45, P = .003). CONCLUSIONS: Pretransplant AFP levels and radiological TTD were independently associated with HCC relapse and recipient mortality after DDLT, with different cut-off points predicting different outcomes. These findings may contribute to improving decision-making in the context of liver transplantation for HCC patients.

Liver Transplant and Active Ulcerative Colitis: A Case Report.

BACKGROUND: The association between ulcerative colitis (UC) and primary sclerosing cholangitis has been described for several years and can be classified as having a distinct disease phenotype from inflammatory bowel diseases (IBD). The simultaneous occurrence of decompensated liver disease requiring liver transplant and active IBD is a management challenge, considering that these patients may be at increased risk of infections, thromboembolic events, bleeding, and drug hepatotoxicity. CASE PRESENTATION: We describe a case of a 37-year-old patient with UC and sclerosing cholangitis presenting with severe decompensated rectocolitis complicated with thromboembolic phenomena and severe liver dysfunction who underwent liver transplant while using biological therapy to control bowel disease. CONCLUSIONS: This case highlights the evolution of sclerosing cholangitis to liver transplant in patients with decompensated UC. Despite the risk of recurrence, primary sclerosing cholangitis has excellent results after liver transplant. Despite the use of immunosuppression after liver transplant, biological therapy may be necessary to control IBD.

Liver Transplantation After Acute Portal Vein Thrombosis: Case Report.

BACKGROUND: Portal vein thrombosis is a relatively frequent complication in patients with liver cirrhosis. Its detection and management are essential to avoid worsening portal hypertension or liver function complications. This complication can also negatively impact or even preclude liver transplant. CASE PRESENTATION: We report the case of a patient who presented with acute portal vein thrombosis, which allowed the diagnosis of liver cirrhosis and hepatocarcinoma within the Milan criteria. Chemical thrombolysis was performed with a mechanical aspiration of the thrombus, and in a second moment, the patient was submitted to a liver transplant. CONCLUSIONS: Advances in the therapeutic approach to portal vein thrombosis and surgical techniques have allowed the condition to no longer be an absolute contraindication to liver transplantation. Diagnosis in the acute phase is associated with greater therapeutic success, aiming to avoid the extension of thrombosis and achieve portal vein recanalization.

Hemophagocytic Lymphohistiocytosis Secondary to Tuberculosis After Liver Transplantation: A Case Report.

Hemophagocytic lymphohistiocytosis (HL) is a rare syndrome characterized by a hyperinflammatory state, resulting from an excessive but ineffective immune response. There is a continuous stimulation of TCD8 + lymphocytes, associated with an uncontrolled release of cytokines, causing the infiltration of multiple organs by histiocytes and activated lymphocytes. HL can be a primary condition as a consequence of genetic disorder that most often affects children, or it can be secondary to neoplasms, autoimmune conditions or various infectious diseases in patients of all ages. HL caused by infection by Mycobacterium tuberculosis is highly unusual, with few cases reported in the literature. There is no clinical manifestation or laboratorial finding that is specific to HL, and a high index of clinical suspicion is necessary for the correct diagnosis, which is usually confirmed by biopsy. Treatment consists of controlling the causative event and the use of immunosuppressant drugs such as corticosteroids, etoposide, and cyclosporine to suppress the exacerbated immune response. We report the case of a patient who developed HL 2 months after liver transplantation. The initial presentation was persistent fever, prompting a search for a site of infection and the use of broad-spectrum antibiotics. As the clinical condition of the patient continued to deteriorate, HL was diagnosed through a bone marrow biopsy, and a cerebrospinal fluid culture positive for M. tuberculosis established the diagnosis of disseminated tuberculosis. Despite optimal treatment with immunosuppressors and antituberculosis drugs, there was no significant response and the patient died. This article is compliant with the Helsinki Congress and the Istanbul Declaration.

500 Consecutive Liver Transplants: The Outcomes of a New Transplantation Program in the Middle West of Brazil.

INTRODUCTION: Liver transplantation is the standard treatment for end-stage liver disease. Brazil holds the third highest number of liver transplants performed per year, but center maldistribution results in high discrepancies in accessing this treatment. In 2012, an interstate partnership successfully implemented a new liver transplantation program in the middle west of Brazil. Here, we report the results of the first 500 liver transplants performed in this new program and discuss the impacts of a new transplant center in regional transplantation dynamics. METHODS: We reviewed data from the first 500 consecutive deceased donor liver transplants performed in the new program during an 8-year period. We analyzed data on patients' clinical and demographic profiles, postoperative outcomes, and graft and recipient survival rates. Univariate survival analysis was conducted using log-rank tests to compare the groups. RESULTS: Almost half (48%) of the procured organs and 40% of the recipients transplanted in our center were from outside our state. Recipient 30-day mortality was 9%. Overall recipient survival at 1 year and 5 years was 85% and 80%, respectively. Mortality was significantly associated with higher Model for End-Stage Liver Disease (P < .001) but not with the presence of hepatocellular carcinoma (P = .795). DISCUSSION: The new transplantation program treated patients from different regions of Brazil and became the reference center in liver transplantation for the middle west region. Despite the recent implementation, our outcomes are comparable to experienced centers around the world. This model can inspire the creation of new transplantation programs aiming to democratize access to liver transplantation nationwide.

Transmission of cryptococcosis by liver transplantation: A case report and review of literature.

BACKGROUND: Cryptococcosis is a fungal infection caused by the yeast-like encapsulated basidiomycetous fungus of the Cryptococcus neoformans (C. neoformans) species complex. These fungi are ubiquitous in soil and bird droppings, and infection by them is an important global health concern, particularly in immunosuppressed patients, such as organ transplant recipients and those infected by the human immunodeficiency virus. The fungus usually enters the body through the respiratory tract, but extremely rare cases of infection acquired by transplantation of solid organs have been reported. CASE SUMMARY: We report a case of disseminated cryptococcosis in a liver transplant recipient, diagnosed 2 wk after the procedure. The patient initially presented with fever, hyponatremia and elevated transaminase levels, manifesting intense headache after a few days. Blood cultures were positive for C. neoformans. Liver biopsy showed numerous fungal elements surrounded by gelatinous matrix and sparse granulomatous formations. Magnetic resonance imaging of the brain showed multiple small lesions with low signal in T2, peripheric enhancement and edematous halo, diffuse through the parenchyma but more concentrated in the subcortical regions. Treatment with amphotericin B for 3 wk, followed by maintenance therapy with fluconazole, led to complete resolution of the symptoms. The recipients of both kidneys from the same donor also developed disseminated cryptococcosis, confirming the transplant as the source of infection. The organ donor lived in a rural area, surrounded by tropical rainforest, and had negative blood cultures prior to organ procurement. CONCLUSION: This case highlights the risk of transmission of fungal diseases, specifically of C. neoformans, through liver graft during liver transplantation.

Visceral Leishmaniasis in a Liver Transplant Patient: A Case Report.

Leishmaniasis is an infection caused by protozoa of the genus Leishmania, transmitted by sandflies and endemic to more than 88 countries. Visceral leishmaniasis in immunosuppressed patients is a growing concern. We report the case of a 61-year-old male patient with a previous history of alcoholic cirrhosis and portal vein thrombosis who underwent liver transplantation for the treatment of hepatocellular carcinoma. Thirty-six days after the procedure, the patient showed an increase in liver enzymes and was diagnosed with moderate acute rejection of the graft. He was treated with high-dose intravenous corticosteroids, and while showing improvement in biochemical markers, he became febrile 12 days after corticosteroid treatment. He presented daily episodes of fever, even after the use of several antimicrobial, antiviral, and antifungal agents, and a number of negative cultures from different sites were obtained. A bone marrow biopsy was then performed, showing a large number of amastigote forms of Leishmania spp. Treatment with liposomal amphotericin B was initiated; however, the patient progressed to refractory septic shock and death. This case highlights several aspects of visceral leishmaniasis in liver transplant recipients, such as the association of malnutrition to Leishmania infection and the challenges of diagnosing leishmaniasis in cirrhotic patients in which splenomegaly and pancytopenia, the hallmarks of leishmaniasis, may also be attributed to portal hypertension and end-stage liver disease. A high index of suspicion is necessary for the correct diagnosis and treatment of leishmaniasis in this group of patients. This study is compliant with the Helsinki Congress and the Istanbul Declaration.

Impact of Donor Positive Blood Culture in Deceased Donor Liver Transplantation.

INTRODUCTION: In the era of shortage of organs for donation, transplantation from suboptimal donors is an expanding alternative to minimize waitlist mortality. In that sense, the safety of using organs from bacteremic donors has been a recurrent matter of discussion. We aimed to evaluate the influence of donor positive blood culture in the recipient and graft outcomes after liver transplantation from deceased donors. MATERIAL AND METHODS: Blood culture results from 255 deceased liver donors were retrospectively reviewed. Patients were categorized into 2 groups based on the recipients who obtained a graft from a donor with negative or positive blood culture. Graft and recipient outcomes were compared between the 2 groups using univariate survival analysis and multivariate regression models. Transmission of bloodstream infection from donor to recipient was assessed by reviewing recipients' microbiologic status when there was evidence of infection. RESULTS: Positive blood culture in donors was not associated with negative outcomes after transplantation. Death within 30 days after transplantation and overall recipient and graft survival did not differ between the 2 groups. Only Child-Pugh score ≥10 and retransplantation status were considered independent predictors of recipient death and graft failure. We identified 1 potential case of bacteremia transmission from donor to recipient. CONCLUSION: Donor positive blood culture was not associated with negative outcomes after liver transplantation. Transmission of infection from donor to recipient is possible, but rare. The results support the usage of bacteremic donors as a safe alternative to the scarcity of optimal donors.