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.

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.

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.

Colonic Infection by Histoplasma capsulatum in a Liver Transplant Patient: A Case Report.

Histoplasmosis is an infection caused by the dimorphic fungus Histoplasma capsulatum. While the lungs are the most common site of infection, disseminated disease affecting multiple organs can occur, particularly in immunocompromised patients. Gastrointestinal histoplasmosis is usually diagnosed in the context of disseminated disease and can present in any part of the digestive system, the ileum being the most frequently affected. We report the case of a 60-year-old female patient who underwent liver transplant for alcoholic liver cirrhosis. The patient had a 10 mm polypoid lesion in the sigmoid colon diagnosed in a screening colonoscopy performed 8 months prior to the transplant, but biopsy was not done for fear of bleeding due to extensive anorectal varices. There were no other lesions in the rest of the colon at that time. Four months after the transplant, the patient was asymptomatic and was submitted to a control colonoscopy, which showed 8 polypoid lesions in different parts of the colon, all of which were biopsied. Histologic results showed extensive infiltration of the colonic mucosa by Histoplasma capsulatum. Imaging and laboratorial screening for other sites of infection was negative, and the patient was treated with itraconazole for 12 months. A marked reduction in the dose of tacrolimus was necessary to maintain therapeutic levels during itraconazole treatment. Asymptomatic isolated colonic histoplasmosis is an uncommon manifestation of infection by Histoplasma capsulatum, with no previous reports in the literature of this condition affecting liver transplant recipients. This manuscript is compliant with the Helsinki Congress and the Istanbul Declaration.

Acute Liver Failure Caused by Use of Fat Burner: A Case Report.

Acute liver failure is a rare condition consisting of abrupt and extensive hepatocyte injury, leading to significant liver dysfunction associated with a high mortality. Liver transplantation is the most effective treatment in severe cases. The most common cause of acute liver failure in Western countries is drug-induced liver injury caused by prescription drugs and herbal and dietary supplements. Thermogenics, or fat burners, are a category of dietary supplements that claim to increase the resting metabolic rate, leading to weight loss. There are previous reports of acute liver failure associated with specific thermogenic formulations. We report the case of a 36-year-old male patient who developed jaundice 7 days after he started taking a thermogenic dietary supplement (Thermo Gun), with progressive deterioration of hepatic function and development of hepatic encephalopathy 19 days after the beginning of the symptoms. He had a Model for End-Stage Liver Disease score of 38 and fulfilled 4 of the King's College Criteria for poor prognosis in patients with acute liver failure. He underwent liver transplantation, receiving a graft from a cadaveric donor, and is alive with good liver graft function 2 years after the transplant. No possible causes for acute liver injury were identified other than the use of the supplement, which contained N-acetyl-L-tyrosine; 1,3,7-trimenthylxanthine; white willow; and 1-hydroxypholedrine. We found no previous reports in the literature of acute liver failure associated with those particular substances. This manuscript is compliant with the Helsinki Congress and the Istanbul Declaration.

Leukocytoclastic vasculitis caused by hepatitis C virus in a liver transplant recipient: A case report.

BACKGROUND: Infection by the hepatitis C virus (HCV) is currently considered to be a global health issue, with a high worldwide prevalence and causing chronic disease in afflicted individuals. The disease largely involves the liver but it can affect other organs, including the skin. While leukocytoclastic vasculitis has been reported as one of the dermatologic manifestations of HCV infection, there are no reports of this condition as the first symptom of HCV recurrence after liver transplantation. CASE SUMMARY: We report here a case of leukocytoclastic vasculitis in a liver transplant recipient on maintenance immunosuppression. The condition presented as a palpable purpura in both lower extremities. Blood and urine cultures were negative and all biochemical tests were normal, excepting evidence of anemia and hypocomplementemia. Imaging examination by computed tomography showed a small volume of ascites, diffuse thickening of bowel walls, and a small bilateral pleural effusion. Skin biopsy showed leukocytoclasia and fibrinoid necrosis. Liver biopsy was suggestive of HCV recurrence in the graft, and HCV polymerase chain reaction yielded 11460 copies/mL and identified the genotype as 1A. Treatment of the virus with a 12-wk direct-acting antiviral regimen of ribavirin, sofosbuvir and daclatasvir led to regression of the symptoms within the first 10 d and subsequent complete resolution of the symptoms. CONCLUSION: This case highlights the difficulties of diagnosing skin lesions caused by HCV infection in immunosuppressed patients.