Fibrolamellar hepatocellular carcinoma is a rare hepatocellular tumor usually arising in noninfected and noncirrhotic livers. Only 2 cases accompanied by hyperammonemia due to intrahepatic shunting have been reported. A 23-year-old white woman presented with a 2-week history of nausea, vomiting, generalized weakness, and intermittent right upper quadrant pain. Abdominal computerized tomography revealed a 13 x 9-cm hepatic mass. Core-needle biopsy revealed fibrolamellar hepatocellular carcinoma. She presented with coma due to hyperammonemia levels (peak at 437 mcg/dL) but without metastatic disease. She was urgently transplanted, started on daily sorafenib 8 weeks after transplantation, and was free of disease at 1 year after transplantation.

BackgroundMost infections of Strongyloides stercoralis are asymptomatic but can be fulminant in the immunosuppressed. Fatal infections in transplant patients have been reported in United States but incidence estimates are lacking. Our protocol for Strongyloides until 2009 screened immigrants and those with travel history to endemic areas. In 2010, we began universal screening of SOT candidates due to a case of disseminated Strongyloidiasis in an unscreened lung transplant recipient with unknown risk factors. We calculated the incidence of Strongyloides stercoralis in our SOT candidates and associated risk factors, treatment, and outcomes since protocol change.MethodsA retrospective review was performed of patients who underwent transplant evaluation from January 2014 to July 2016. Patients positive for Strongyloides stercoralis were reviewed for age, sex, ethnicity, place of birth, travel history, occupation, eosinophilia, treatment, and outcome. We report descriptive statistics.ResultsOf a total of 2,351 SOT patients, 116 tested positive (heart 33, lung 24, kidney 26, liver 31, pancreas 2) with an incidence of 4.9%. A total of 113 charts were available for review. The characteristics of the patients are summarized in Table 1. Fifty patients had traditional risk factors (44%) and 63 lacked them (56%). Eosinophilia was present in 15% of cases. Of those transplanted, 87% received prophylaxis and none developed active Strongyloidiasis.ConclusionOur results show that S. stercoralis infection has a relatively high incidence in SOT patients and universal screening identified a substantial number that otherwise would go undetected, placing the transplant patient at risk of a fatal, yet preventable complication. Table 1. Characteristics of patientsPatient characteristics Number%Total screened2365Positive Strongyloides1164.9Age group60–70 years413650–59 years232040–49 years2219<402724SexMale9080Female2320EthnicityWhite8172Hispanic1816African American1412Occupation with soil or water contact2119Total SOT patients3887Treated before SOT3397Ivermectin323Albendazole1Travel or birth outside United States3531Puerto Rico1234Caribbean and South America1029Middle East38Africa26Europe and Australia38Asia514Eosinophilia >5%1715Disclosures All authors: No reported disclosures.

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INTRODUCTION: Differentiating drug induced liver injury from other causes of acute hepatitis, especially autoimmune hepatitis can be difficult secondary to overlapping histological features and confounding laboratory abnormalities. Stopping the offending drug should usually result in resolution of liver injury. We describe a rare case of drug induced liver injury secondary to valerian root tea with overlapping features of autoimmune hepatitis which resolved with limited duration steroid therapy. CASE DESCRIPTION/METHODS: Our patient is a 47-year-old female with hypothyroidism who presented with acute onset of generalized abdominal discomfort, jaundice and vomiting without fever or confusion. She denied prior similar episodes. She reported regular use of valerian tea for insomnia for a duration of 3 months prior to presentation. Physical examination revealed jaundice, mild diffuse abdominal tenderness and no features of chronic liver disease. Labs showed elevated alanine aminotransferase of 2741 U/L, aspartate aminotransferase of 1847 U/L, alkaline phosphatase of 199 U/L, total bilirubin of 9.2 mg/dL, albumin of 3.4 g/dL and International normalized ratio of 1.4. Autoimmune work up revealed positive smooth muscle antibody, mildly elevated Immunoglobulin G of 1980 mg/dL and negative antinuclear and mitochondrial antibodies. Viral hepatitis panel was negative. Imaging of the abdomen was unremarkable. Liver biopsy showed severe acute hepatitis with extensive necroinflammatory activity with plasma cells and bridging necrosis without significant fibrosis. With no significant improvement in liver enzymes after stopping valerian root tea and with concern for autoimmune hepatitis, she was started on prednisone 60 mg daily with improvement of transaminitis. Transaminases have remained normal for several months after stopping prednisone (Table 1, Figure 1). DISCUSSION: Diagnosing autoimmune like drug induced liver injury and differentiating it from autoimmune hepatitis can be difficult. Steroids should be considered in cases with severe or prolonged elevation of liver enzymes that do not resolve after stopping the offending drug. Valerian root can cause severe form of autoimmune like drug induced liver injury that responds to steroids. Complete recovery is possible with steroids for limited duration. Relapse following steroid cessation should prompt further evaluation for autoimmune hepatitis.

INTRODUCTION: Autoimmune hepatitis can be refractory to steroids and traditional immunosuppressant agents especially in the setting of coexisting autoimmune conditions. We describe a patient with autoimmune hepatitis and Neuromyelitis Optica with elevated liver enzymes who was refractory to the traditional immunosuppressant management, but responded to therapy with Rituximab. CASE DESCRIPTION/METHODS: Our patient is a 57-year-old male who was diagnosed with Autoimmune hepatitis after he presented with elevated liver enzymes. Just prior to being evaluated for autoimmune hepatitis, he was diagnosed with Neuromyelitis Optica after extensive investigation when he presented with recurrent symptoms of diffuse paresthesia and muscle weakness distributed all over the body. His liver enzymes at the time of presentation were ALT (1688), AST (656), ALP (193), Bilirubin (1). Autoimmune work up was positive for NMO IgG antibody and ANA, but negative for AMA and ASMA. Liver biopsy was consistent with autoimmune hepatitis showing interface hepatitis with necroinflammatory activity and presence of plasma cells with lobular disarray. Work up for other chronic liver diseases was negative. He initially improved with pulse dose of IV steroids and was later transitioned to maintenance therapy with oral prednisone and Imuran. But, he continued to have moderately elevated liver enzymes with relapses of both autoimmune hepatitis and neurological symptoms on combination of prednisone and Imuran, and also experienced adverse effects from Imuran. He was later transitioned from Imuran to Cellcept without significant improvement. He was finally started on Rituximab infusion due to severe neurologic symptoms with complete normalization of liver enzymes and remission of Neurological manifestations. DISCUSSION: Rituximab is an anti CD 20 monoclonal antibody that depletes B cells. It has been shown to improve many autoimmune conditions and should be considered in refractory cases of autoimmune hepatitis especially in the setting of coexisting autoimmune conditions. Although prior case reports and case series have demonstrated the efficacy and safety of Rituximab in difficult to treat autoimmune hepatitis, it is not currently FDA approved for this purpose and payers will usually deny coverage. Prospective clinical trials of Rituximab in refractory autoimmune hepatitis should be considered for further validation.