bluuebunny/crossref_metadata_2025 huggingface dataset:
2216 Toxic Epidermal Necrolysis and Drug-Induced Liver Injury Secondary to Oseltamivir
Authors: Shaikh Abdullah S. , Zaibaq-Krill Jenine , Merwat Shehzad N. , Merwat Sheharyar
Published: October 2019

INTRODUCTION:

Multiple antibiotics have the well documented adverse reactions of Stevens-Johnson syndrome, toxic epidermal necrolysis, and liver injury. Oseltamivir is not known to cause any of the above reactions. We present a patient who suffered both toxic epidermal necrolysis and drug induced liver injury (DILI) secondary to oseltamivir.

CASE DESCRIPTION/METHODS:

A 40-year-old female with systemic lupus erythematosus (SLE) on hydroxychloroquine and azathioprine for several years presented with community acquired pneumonia and rash concerning for Stevens-Johnson syndrome (SJS). She was also found to have newly elevated liver chemistries in a hepatocellular pattern. Her only new medication was oseltamivir which had been started 48 hours prior to presentation for empiric treatment of influenza. She was transferred to our tertiary care academic medical center burn unit for further management of SJS which eventually progressed to toxic epidermal necrolysis (TEN) (confirmed via dermatopathology). On arrival to our institution, her pattern of abnormal liver chemistries evolved into that of a cholestatic hepatitis with a peak conjugated hyperbilirubinemia of 16.7mg/dL, alkaline phosphatase of 1087 U/L, AST of 400 U/L, and ALT of 708 U/L. Her TEN started to improve with supportive care and cessation of oseltamivir. She underwent MRCP which revealed possible bile duct stricturing without biliary ductal dilatation. Her ANA was positive with a titer of 1:640, anti-smooth muscle antibody was negative, anti-mitochondrial antibody was negative, and IgG level was within normal limits. She underwent liver biopsy which was consistent with drug induced liver injury. Her liver chemistries eventually started to improve and she was discharged from the hospital with continued improvement on follow up.

DISCUSSION:

Oseltamivir is an antiviral sometimes used for influenza infection. Very few reports exist regarding oseltamivir’s association with SJS/TEN, and even less on its association with liver injury. Our case is unique in that a patient developed both TEN as well as drug induced liver injury secondary to oseltamivir which are rare but important differential diagnoses to be aware of for patients who develop rash or elevated liver enzymes while being treated with oseltamivir.
2221 A Case of Rare Escape Mutation Type G130N and Conundrum of Heptitis B Serology
Authors: Lakkasani Saraswathi , Jayasingam Rajasingam M. , Barham Shaker , Siegel Arthur M. , Depasqule Joseph
Published: October 2019

INTRODUCTION:

Hepatitis B (HB) virus is a global public health problem as it affects more than 300 million patients around the world. It's well known that once we have Anti-HBs, seroconversion takes place, and that's a marker of viral clearance and immunity. In view of their important protective role, occurrence of anti-HBs and HBsAg is perplexing. Although this has been reported in 5-20% of cases of Chronic Hep B, it remains a confusing finding to many physicians who are unaware of the existence, explanation and implications of this finding. Herein, we will present a case of such a pattern.

CASE DESCRIPTION/METHODS:

A 49-year-old male with diabetes and hypertension was found to have elevated liver enzymes on routine blood work. Further workup resulted in diagnosing chronic hepatitis B. The patient’s viral serology results were positive for HB core antibody, HB surface antibody, HB surface antigen, HB e antigen, and a viral load of 1,500,000 IU/ml. Liver enzymes were elevated with ALT of 250U/L and AST of 180U/L. No masses were detected on liver ultrasound. Blood sample was sent to Center for Disease Control and analysis revealed a mutation G130N within the major hydrophilic region of surface antigen. The patient was started on Entecavir. After 12 months, the VL became undetectable. Serology was still positive for both HB s Ag and Ab but he successfully achieved HB e Ab +ve status. Liver enzymes normalized.

DISCUSSION:

The expected pattern in chronic HB is the presence of HBsAg and lack of anti-HBs. The mechanism underlying the coexistence of HBsAg and anti-HBs remains unclear. Mutations in the viral DNA were initially suggested by researchers to explain how the subtypes of the HBsAg and anti-HBs were heterologous. It was shown that there is a relation between this special serological profile and mutations in S gene region, particularly in the 'a' determinant of the major hydrophilic region of the surface antigen, including one located at G130N. Aside from mutations in the S gene region, the presence of heterologous subtype-specific antibodies, superinfection with a new HB strain, occult HB reactivation and false positivity for anti-HBs are other possible explanations. These patients might harbor an augmented HBV replicative capacity and HBV reactivation upon receiving immunosuppression. Antiviral prophylaxis must be considered before starting immunosuppressive drugs. It was also reported that coexistent HBsAg and anti-HBs independently increased the risk of advanced fibrosis and hepatocellular carcinoma.
2240 Hepatocellular Carcinoma With Vascular Invasion and Cardiac Metastasis: A Case of Veno-Occlusive Disease Causing Progressive Liver and Renal Failure
Authors: Dasu Neethi R. , Khalid Yaser , Walters Richard , Lal Kailash , Parikh Nishant
Published: October 2019

INTRODUCTION:

Hepatocellular carcinoma (HCC) is the most common primary tumor of the liver. The most frequent sites of metastasis are the lungs, the bones, the lymphatics, and the brain. We present a case of a 61-year-old female found to have advanced stage HCC with isolated metastasis to the right atrium and IVC, which is extremely rare with incidence of 2.7-4.1%. Prognosis is very poor.

CASE DESCRIPTION/METHODS:

A 61-year-old female presented with two weeks of generalized weakness and diarrhea. Physical exam revealed bilateral lower extremity edema. Laboratory data revealed a cholestatic injury pattern with an R factor of 1.1. CT of the abdomen and pelvis showed abdominal lymphadenopathy and a 3-cm mass in the right hepatic lobe. Diagnostic paracentesis showed a serum-ascites albumin gradient (SAAG) of 1.9 and a fluid protein of 2.7 g/dL. Treatment with lactulose, fluids, and albumin improved her mental status, but the patient’s renal function and diffuse edema continued worsening. Following a therapeutic paracentesis of 4 liters of peritoneal fluid, she had a primary hepatocellular injury pattern with R factor 14. With an alpha-fetoprotein (AFP) level of 1700, the diagnosis of HCC was confirmed. An echocardiogram showed a bilobed mass in the inferior portion of the right atrium. Magnetic resonance venography revealed occlusion of the right intrahepatic portal vein. Fine needle aspiration biopsy confirmed hepatocellular carcinoma. Due to having end-stage HCC, Child-Pugh Class C with expected survival less than 3 months, only palliative treatment was offered.

DISCUSSION:

HCC with isolated metastasis to the right atrium presents with right heart failure, manifesting as lower extremity edema, jugular venous distention, and ascites as seen in our patient. Only 1.2-4.1% of all HCC have metastasis to the heart, with around 17 reported cases in the literature of isolated metastasis to the heart. Our patient had an extremely rare case of an isolated right atrium metastasis of HCC. Sorafenib is the first line treatment option for advanced HCC but the delay in diagnosis, as well as our patient’s complex presentation, limited treatment. We recommend that echocardiography should be routinely performed for all patients with advanced HCC to prevent unexpected and fatal cardiovascular complications. This case illustrates the subtle presentation of HCC and that early diagnosis is key for treatment. Further studies need to investigate the current and novel treatment options for both HCC and cardiac metastasis.
2251 An Atypical Case of Recurrent Intrahepatic Cholestasis of Pregnancy
Authors: Shah Pir Ahmad , Campoverde Reyes Karen J. , Aslam Aysha , Suri Jaspreet , Wu Lily , Chie Lucy , Lau Daryl T.
Published: October 2019

INTRODUCTION:

Intrahepatic cholestasis of pregnancy (ICP) usually manifests in the second to third trimester with pruritus, jaundice and abnormal liver tests. It can increase risks of preterm birth, respiratory distress syndrome, and stillbirth. Timely diagnosis and planned delivery are crucial. We hereby describe an atypical case of recurrent ICP.

CASE DESCRIPTION/METHODS:

Patient is a 31-year-old Chinese female who presented to hepatology clinic at week-13 of her second pregnancy. During her first pregnancy 4 years ago, she experienced pruritus associated with significant elevation of bile acids and serum aminotransferases at about 20-week gestation. Diagnosis of ICP was made and she was promptly started on ursodeoxycholic acid (URSO) [Figure 1]. Bile acids improved and pruritus resolved but she had a preterm delivery at 30-week gestation. URSO was discontinued after delivery and bile acids were completely normal by 6 weeks postpartum. The ALT levels, however, increased to >200 U/L and remained abnormal for 10 months post-partum [Figure 1]. Other common causes of liver diseases such as viral hepatitis, autoimmune and drug-related liver diseases were ruled out. Her liver biopsy reported only non-specific resolving inflammation. For her second pregnancy, bile acids were checked at 14-week gestation and were mildly elevated at 14.7umol/L; they increased to 20.8 umol/L by 26 weeks. At that time, she experienced mild pruritus. URSO was initiated at 28-week gestation for recurrent ICP. Despite being on URSO for 3 weeks, ALT normalized but bile acid increased further to 53.5 umol/L without worsening of pruritus. Induced labor was planned at week-37 and the delivery was uneventful.URSO was subsequently discontinued. At week-3 postpartum, URSO was reinitiated for her ALT significantly elevated at 259 U/L even though bile acids continued to decrease. About 1 week after starting URSO, ALT decreased to 62 U/L and bile acids completely normalized.

DISCUSSION:

ICP usually resolves after delivery and URSO can be discontinued. Our case was atypical for her serum aminotransferases actually markedly increased during early postpartum in both pregnancies despite improvement of the bile acid levels. URSO was promptly reinitiated postpartum that resulted in reduction of ALT. For patients with elevated liver tests associated with ICP, physicians need to be vigilant in monitoring the patient closely during postpartum period and consider continuing URSO therapy until both serum aminotransferases and bile acids return to normal range.
2260 Management of a Massive Liver Hemangioma: Does Size Matter?
Authors: Fazio Richard M. , Waintraub Daniel J. , Rahmani Rabin , Hajdu Cristina H. , Park James S.
Published: October 2019

INTRODUCTION:

The majority of “giant” hemangiomas remain asymptomatic with no cause for surgical intervention; however, this may not hold true for massive tumors. The following case will review the challenges facing both patients and physicians when managing these atypical tumors.

CASE DESCRIPTION/METHODS:

A 49-year-old male with no medical problems presented with complaints of post-prandial bloating, early satiety and mild epigastric discomfort. The bloating was intermittent for several years; however, symptoms have recently curtailed his eating habits. CBC, BMP and liver enzymes were unremarkable. An ultrasound highlighted a massively enlarged liver extending into the pelvis and displacing surrounding organs. The liver parenchyma appeared to be replaced with a homogenous, hyperechoic lesion. An MRI then illustrated a 29.5 × 20.1 × 19.4 cm, strongly hyperintense mass on T-2 weighted sequences consistent with the diagnosis of a hemangioma. He was referred to a hepatobiliary surgeon and an extended right hepatectomy was eventually performed. Histopathology results described vast endothelial lined channels supported by thin fibrous stroma without features of malignancy. The patient returned to clinic four weeks after surgery reporting complete resolution of his symptoms.

DISCUSSION:

Hemangiomas are the most common benign solid tumor of the liver with little to no risk of malignant transformation. Often discovered incidentally on imaging studies, the majority of these tumors remain indolent without the need for routine surveillance. Rarely these tumors become symptomatic, often correlating with tumor size. The definition of “giant” liver hemangioma remains controversial, with most authors assigning the label to tumors greater than 4cm or 5cm in size. It is for this reason that management of giant hemangiomas remains highly debated (i.e. observation versus resection). Recent studies have shown that tumors greater than 20cm in size pose a higher risk for GI symptoms related to mass effect on surrounding organs as well as causing a disturbance in the hematologic and coagulation systems. Surgical resection should be considered for symptomatic or complicated lesions, or when the diagnosis remains inconclusive. It is our belief that size classifications for giant hemangiomas requires further subgrouping to consider the danger of these massive tumors as well as the increased morbidity of surgery. Proper management of these tumors should be individualized to each patient and include a multidisciplinary team approach.
2273 A Rare Case of Very Late Liver Metastasis in Resected Neuroendocrine Tumor
Authors: Kochar Tanureet , Dhingra Parminder , Sankari Mohamad
Published: October 2019

INTRODUCTION:

Carcinoid tumors are neuroendocrine tumors derived from enterochromaffin or Kulchitsky cells. They occur predominantly in foregut, midgut and hindgut. The annual incidence of carcinoid tumors is around two cases per 100,000. Although generally slowly progressing, hepatic metastases are the major cause of carcinoid syndrome and ultimately lead to liver dysfunction, cardiac insufficiency.

CASE DESCRIPTION/METHODS:

This is 72-year-old male with PMH carcinoid tumor involving right colon and terminal ileum T3N1M0 present in 6 ileum ileocolic lymph nodes s/p resection in 2006, chronic kidney disease stage III, diabetes mellitus type II, hypertension presented watery diarrhea with 5-6 bowel movements/day from last 2-3 months, 30 pound unintentional weight loss in last 2 months, occasional night sweats. He denied any episodes of flushing. On presentation, vitals were stable. Other labs included CBC- wnl, BUN - 25 mg/dl with creatinine of 3 (baseline 2), albumin – 2.4, AST – 19u/l, ALT- 26u/l, ALP- 210 u/l, total bilirubin 0.9 mg/dl. Clostridium difficle screen and GI panel were negative. Last colonoscopy, done one month prior to presentation was normal. He was started on Imodium without any relief. Gastroenterology service was consulted for further management. His chromogranin A was found to be 782 (< 93), 5HIAAA was 50 (< 8). He underwent ultrasound of the abdomen for this acute kidney injury which revealed 5.9 × 5.9 × 5.9 cm hyperechoic mass with central decreased echogenicity located within the liver. AFP was 1.1 ng/ml (< 8 ng/ml), CEA 7.4 ng/ml (3 ng/ml). Meanwhile, he was started on sandostatin which helped a lot with his diarrhea. Further imaging of this abdomen was deferred due to kidney injury. He had previous PET scan done 3-4 years ago revealed increased uptake in liver but were inconsistent of any metastatis. His last abdominal CT scan done 2-3 years ago was negative of any metastasis. Two weeks after his discharge, he was readmitted to intensive care unit (ICU) with septic shock and unfortunately he passed away.

DISCUSSION:

Carcinoid tumors are relatively slow growing compared to adenocarcinomas of the same site and have a tendency for recurrence. The NANETS Consensus Guideline for the Diagnosis and Management of Neuroendocrine Tumors recommends an office follow up every 6 to12 months for 7 years. Either radiographic (CT/MRI) or endoscopic methods could be used for surveillance. The secretory tumors can be followed up by monitoring the levels of urinary 5- HIAA and serum chromogranin A.
2276 Malignant Hepatic Epitheliod Hemangioendothelioma in a Patient With a Prior Liver Transplant
Authors: Ellison Parker L. , Willner Ira
Published: October 2019

INTRODUCTION:

While liver transplantation itself is an invasive and specialized procedure, often with an arduous medical course preceding the surgery; post-transplant life is rarely without complication. Permanent immune suppression introduces its own cohort of increased risks including unique infections, malignancies, and side effects of immunosuppressive or prophylactic therapies. Special consideration should be given to these patients when potential complications arise.

CASE DESCRIPTION/METHODS:

A 51-year-old male with a an orthotopic liver transplant 6 years prior due to NASH cirrhosis presents with rapid significant weight loss and worsening abdominal pain. MRI revealed development of T2 hyper-intense peripherally enhancing lesions throughout the liver, as well as massive splenomegaly with possible areas of splenic infarct. Transjugular liver biopsy was expedited and ultimately revealed epitheliod hemangioendothelioma (EHE). Initial infectious work up was unrevealing outside of EBV viral load returning weakly positive; however, given possible association with EHE with EBV, treatment was initiated with valgancyclovir. Immunosuppression was also adjusted from tacrolimus to everolimus given anti-neoplastic properties of mTOR inhibitors over calcineurin inhibitors. He was started on pazopanib as an outpatient given anecdotal evidence of VEGF inhibitors in treating EHE, however, treatment was complicated shortly thereafter by syncope and refractory TTP, and palliative care was ultimately pursued.

DISCUSSION:

Epithelioid hemangioendothelioma is a rare vascular tumor originating from mesenchymal cells. While risk of malignancy is increased post transplant due to immunomodulators, post transplant Hepatic EHE remains an exceedingly rare diagnosis. EHE is considered to have intermediate behavior between hemangioma’s and hemangiosarcoma’s however HEHE is metastatic at diagnosis in 50% of diagnoses. Common presenting symptoms include vague abdominal pain, weight loss, and weakness/fatigue. Histopathological examination via biopsy is the gold standard of diagnosis and positivity of factor VIII-dependent antigen, CD-34, and CD-31 are important. Course is variable however prognosis is poor and there is no standardized management plan. VEGF inhibitors including Bevacizumab and Pazopanib have been a target of therapy given histologic positivity for target receptors, however results have been mixed to date.
2287 HELLP My Liver Hematoma
Authors: Abdalla Abubaker O. , Abdallah Mohamed , Narala Sai B. , Gullapalli Nageshwara
Published: October 2019

INTRODUCTION:

Pregnant females are at increased risk of hypertensive conditions including preeclampsia, eclampsia, and hemolysis, elevated liver enzymes, low platelet (HELLP) syndrome. Complications related to these disorders can be life-threatening and can result in multi organs failure. These complications involve disseminated intravascular coagulation (DIC), stroke, acute renal failure, acute liver injury, hepatic hematoma, and rupture.

CASE DESCRIPTION/METHODS:

We describe a 34-year-old G2P1 female at 36-weeks gestation with no significant past medical history, who presented with abdominal pain for the last couple of days prior to her emergency room (ER) visit. She described the pain as dull, constant, and worsening right upper quadrant pain associated with nausea but no vomiting, fever, chills, jaundice or changes to bowel habits. On presentation, she was noted to be in a stable condition. The physical exam was consistent with tight upper quadrant tenderness with no signs of acute abdomen. The initial workup was significant for leukocytosis of 13.8, hemoglobin of 11.9, mildly elevated liver enzymes with AST of 270, ALT of 346, ALP of 165 (normal liver tests during her recent pre-natal visits). Abdominal ultrasound revealed a normal appearance of the liver with no signs of gallbladder disease. The rest of her work up was unremarkable on admission. Her condition got worse with markedly elevated liver enzymes that peaked at 2500s range and diminished platelet count concerning for the diagnosis of HELLP syndrome. She underwent a successful urgent cesarean section in the setting of deceleration of fetal heart tracing that was complicated by hypotension requiring pressor support. She was noted to have a liver hematoma intra-operatively. Abdominal CT post-operatively confirmed extensive heterogeneous sub-capsular hepatic hematoma with a small amount of ascites but not evidence of extravasation (Figure 1,2). The patient was managed conservatively in the intensive care unit (ICU) after her operation and her condition improved gradually.

DISCUSSION:

HELLP syndrome is a life-threating condition that carries a high morbidity and mortality burden. Hepatic hematoma and spontaneous liver rupture have been described previously as fatal complications. A high index of suspicion is required for early diagnosis and prompt treatment of such conditions.
2299 Resolution of Acute Cellular Rejection Using Irradiating Packed Red Blood Cells for Exchange Transfusion in a Patient With Sickle Cell Disease and a History of Orthotopic Liver Transplantation
Authors: Nayi Vipul , Htut Alvin
Published: October 2019

INTRODUCTION:

Liver transplantation in patients with Sickle Cell Disease (SCD) is fairly unique; a review article from 2013 noted only 15 reported cases within the literature. This case highlights a patient with SCD who underwent orthotopic liver transplantation. His course was complicated by iron overload due to recurrent exchange transfusions, as well as biopsy-proven acute cellular rejection (ACR), that was inadequately treated with corticosteroids, with no recurrence of rejection once the patient was switched to irradiated red cell exchange transfusions.

CASE DESCRIPTION/METHODS:

A 32-year-old male with HbSS disease, who had also undergone orthotopic liver transplantation at the age of 26 due to complications of Caroli’s disease presented with persistent abnormal liver tests over the course of several weeks. Graft function was normal during post-transplant until monthly exchange transfusions were initiated to manage his sickle cell crises. During this period he had two episodes of ACR and was noted to have persistent liver test abnormalities despite adequate therapeutic levels of tacrolimus. The patient was switched to receiving irradiated packed red blood cells for his subsequent exchange transfusions, which led to improvement of his liver tests and resolution of his acute cellular rejection.

DISCUSSION:

Only a limited number of patients with sickle cell disease have undergone liver transplantation, and this is the first case of using irradiated packed red cells for transfusion to help resolve acute cellular rejection. Although it is not thoroughly understood how blood transfusions could induce acute cellular rejection, it may be related to passenger donor leukocytes within the donor blood products triggering an immune response within the transplanted liver. Given our patient’s exposure to higher volumes of transfusions, there may have been enough donor lymphocytes to trigger this response. The use of irradiated blood products is common place in patients that have received hematopoietic stem cell transplants. Current recommendations do not standardize use of irradiated red cells for solid organ transplant (SOT) recipients. Our patient presents an unusual indication for use of irradiated red cells in a SOT patient.
2309 Vasculitic Mononeuritis Multiplex Mimicking Bilateral Carpal Tunnel Syndrome: A Diagnostic Challenge
Authors: Vohra Ishaan , Randhawa Tejinder , Katiyar Vatsala , Desai Parth M. , Lingamaneni Prasanth , Palacios Pedro , Flores Estefania , Haque Zohaib , Nwaichi Chineme , Majeed Muhammad , Elkhouly Mohamed A. , Attar Bashar M.
Published: October 2019

INTRODUCTION:

Cryoglobulinemic vasculitis(CV) can have variety of presentations including palpable purpura, arthralgias, glomerulonephritis, peripheral neuropathy, lymphadenopathy, hepatosplenomegaly, and hypocomplementemia. We present a patient with bilateral hand mononeuritis multiplex secondary to hepatitis C related CV.

CASE DESCRIPTION/METHODS:

A 52-year-old man with untreated hepatitis C (Viral load 520388, Genotype 1b), diabetes mellitus type II, hypertension and chronic kidney disease, presented with two months of bilateral hand pain. He also complained of numbness and tingling over the palmar aspects of the medial three digits of his hands, in addition to his medial left foot. He had no rash, arthritis, fevers, or weight loss. Physical exam was significant for positive Tinel's sign bilaterally, with weakness on finger extension, flexion and decreased power of interossei. Fundoscopy demonstrated non proliferative diabetic retinopathy (NPDR).Laboratory work-up revealed negative ANA, C-ANCA, MPO/PR3, HIV and RPR. Vitamin B12 and TSH levels were normal. However, serum cryoglobulins were positive with low C3, C4 and nephrotic range proteinuria Electromyography showed non-length-dependent neuropathy, which was more suggestive of mononeuritis multiplex rather than carpal tunnel syndrome (CTS). Sural nerve biopsy was performed and revealed vasculopathic changes consistent with vasculitis. He was diagnosed with mononeuritis multiplex secondary to hepatitis C related cryoglobulinemia. Immunosuppresive treatment was withheld because the patient tested positive for latent Tuberculosis (TB). He is currently undergoing treatment for the latent TB.

DISCUSSION:

CV is a small-to-medium-vessel vasculitis that occurs in 10 to 15 percent of patients chronically infected with hepatitis C virus (HCV). The two competing diagnosis for our patient’s neurological symptoms were diabetic neuropathy and CV secondary to untreated Hepatitis C. NPDR favored the microvascular complications of diabetes but the rapid onset of symptoms, positive cryoglobulins in the setting of HCV infection and sural nerve biopsy helped to establish the diagnosis of CV. This case is especially unique because it’s a very rare extrahepatic manifestation of HCV infection .Management of HCV cryoglobulinemic vasculitis involves administration of immunosuppressive therapy such as rituximab and antivirals but all patients should be tested and treated for latent TB prior to treatment initiation.
2311 Syphilis Hepatitis Presenting as a Mimic of Primary Biliary Cholangitis
Authors: Kern Cody , Blake Caroline M. , Hoellein Andrew R.
Published: October 2019

INTRODUCTION:

Primary Biliary Cholangitis (PBC) is a progressive cholestatic liver disease defined by autoimmune destruction of intrahepatic bile ducts. Patients typically present with a cholestatic liver injury pattern. Anti-mitochondrial antibody (AMA) is present in 95% of PBC diagnoses thus, an AMA titer of 1:40 in addition to alkaline phosphatase (ALP) greater than 1.5 times the upper limit of normal is diagnostic of PBC (1). Syphilis is a sexually transmitted infectious disease caused by transmission of Treponema pallidum. The highest risk populations are men who have sex with men (MSM) and persons living with HIV. Syphilis has been further associated with HIV infection considering shared risk factors and transmission routes. While the staged progression of syphilis is well-documented, not all presentations are readily observed by clinicians considering the variability of the disease. One variation that is sparingly seen is syphilis presenting as acute hepatitis.

CASE DESCRIPTION/METHODS:

A 54 year-old man living with HIV presented to University of Kentucky Hospital with one week of abdominal pain, vomiting, and a diffuse non-pruritic rash. Upon admission he was noted to have a cholestatic liver injury pattern. Laboratory evaluation revealed AST 91, ALT 120, ALP 832, total bilirubin 6.4, and CD4 count 336. The acute hepatitis panel was negative. Abdominal CT and ultrasound failed to demonstrate biliary ductal dilation. Further investigation revealed an RPR titer 1:256 and an anti-mitochondrial M2 antibody IgG of 83.5U. The patient was treated for syphilis with doxycycline due an allergy to penicillin. Subsequently there was complete resolution of the cholestatic liver injury and a final diagnosis of syphilis hepatitis.

DISCUSSION:

In this report, we present a case of syphilis that presented similar to PBC with a consistent serologic pattern and a positive AMA. Other reports have recognized syphilis as a reversible cause of cholestatic liver injury. One study in particular, recognized a high false positive AMA rate in patients with syphilis (2). As, syphilis is a easily targeted cause of liver injury, we suggest testing high-risk patients with a presumed diagnosis of PBC.
2318 A Rare Case of Cytomegalovirus Hepatitis Complicated by Secondary Syphilis Hepatitis in an Immunocompetent Patient
Authors: Qiu He , Lim Huei-Wen , Lee Tai-Ping
Published: October 2019

INTRODUCTION:

Cytomegalovirus (CMV) hepatitis most commonly presents with subclinical transaminitis and less commonly with the elevation of alkaline phosphatase (ALP) and total bilirubin (TB). Syphilis hepatitis, on the other hand, generally has a non-specific presentation with a more cholestatic picture. We present a rare case of cholestatic hepatitis from acute CMV infection complicated by syphilis hepatitis.

CASE DESCRIPTION/METHODS:

A 57-year-old homosexual man presented to the emergency room with complaints of intermittent fever and general malaise for the past two weeks. Blood work showed aspartate aminotransferase (AST) of 235 U/L, alanine aminotransferase (ALT) of 284 U/L, ALP of 940 U/L. TB and coagulation profile were normal. Acute hepatitis A, B, C, Epstein-Barr virus, and herpes simplex virus were ruled out. HIV was negative. Workup for autoimmune hepatitis, primary biliary cholangitis, alpha-1-antitrypsin deficiency, and Wilson’s disease was all negative. Abdominal CT scan with contrast was notable for hepatosplenomegaly without liver lesion. CMV IgM was positive with PCR of 7490 copies/mL, indicating acute CMV infection. The patient subsequently improved without treatment. He presented two months later with worsening liver tests with a peaked ALP of 1981 U/L, TB of 1.6 mg/dL, AST 230 U/L, ALT 195 U/L, associated with the macular rash on his trunk, abdomen, both palms, and soles. Abdominal MRI with contrast showed three < 2cm liver lesions. The patient was subsequently diagnosed with secondary syphilis with rapid plasma reagin (RPR) of 1:16 and confirmatory positive fluorescent treponemal antibody absorption (FTA-ABS). Biopsy of the liver lesions showed granulomatous inflammation with negative immunohistochemistry for CMV which was compatible with clinical syphilis hepatitis. The patient was treated with penicillin G. His rash resolved in 2 months with significant improvement of liver tests. The CMV PCR and RPR were negative with normalization of liver enzymes in 4 months. Follow-up MRI showed resolution of the liver lesions.

DISCUSSION:

Our patient initially had an atypical presentation of acute CMV hepatitis with cholestatic features. He subsequently developed secondary syphilis associated hepatitis with severe intrahepatic cholestasis and liver lesions. This is, to our knowledge, the first case of CMV hepatitis complicated by syphilis hepatitis. Resolving CMV hepatitis with recurrent elevation in liver tests warrant the workup for possible superimposed liver disease.
2338 Drug-Induced Liver Injury From Prolonged Kava Kava and Kratom Use
Authors: Shah Maulik , Ali Hasan , Elgenaidi Hisham
Published: October 2019

INTRODUCTION:

Several herbal supplements carry the risk of drug-induced liver injury (DILI).Kava Kava is a herbal compound derived from the root of the Piper methysticum plant that is indigenous to the South and Western Pacific. Kratom is a herbal extract derived from the leaves of Mitragyna speciosa, a evergreen tree indigenous to Southeast Asia. Here we report a case of Kava and Kratom induced hepatocellular hepatitis in a male with complex regional pain syndrome (CRPS).

CASE DESCRIPTION/METHODS:

A 48-year-old male with history of CRPS and rheumatoid arthritis presented with a 7-day history of nausea, vomiting, dark urine, and jaundice. He denied alcohol, tobacco, or IV drug abuse. Denied use of medications. On exam, he was jaundiced. His labs on admission were- WBC 10.8, HGB 17.2, INR 1.4, creatinine 0.97, Total Bilirubin 21.8, Conjugated Bilirubin 16.7, ALP 293, ALT 1805, AST 1539. Viral hepatitis, HIV, HSV, EBV, and CMV were ruled out. Autoimmune serologies and ceruloplasmin were negative. Abdominal ultrasound showed a dilated common bile duct measuring 9.6mm. MRCP ruled out biliary ductal abnormality. Patient refused liver biopsy. Further questioning revealed prior use of opioids for pain, but in the last 9 months he had been substituting with a combination of Kava Kava and Kratom tea.

DISCUSSION:

Herbal supplementation use is increasing, and often, not discussed with medical providers. Kava Kava is comprised of kavalactones, which have similar effects to alcohol with regards to relaxation, sociability and euphoria. Kratom is used for its opioid-like ability to increase stamina and treat chronic pain. Its highly potent agonist activity on the mu, kappa and delta opioid-receptors have led to its popularity as a recreational drug. Formulations of Kava Kava and Kratom can be found for sale in herbal supplement stores and on the internet. Although a few case reports exist of these supplements leading to hepatocellular damage, the exact mechanism of injury is still not well understood and more research is necessary. Health care professionals should be aware of the hepatotoxic potential associated with Kratom and Kava Kava use and should be vigilant in identifying patients who would benefit from education regarding supplement use and discontinuation. Although these herbal supplements can cause hepatotoxicity, it’s important to exclude other possible causes of hepatic injury, including infection, vascular, autoimmune, and biliary etiologies.
2345 Do You Wish to Have a Liver Transplant? A Case of Testosterone Booster-Induced Toxic Hepatitis
Authors: Patel Nehal , Choksi Vivek , Rivas John , Yella Prashanth Reddy , Priyadarshni Shivani , Surapaneni Balarama K. , Cantave Rulz T.
Published: October 2019

INTRODUCTION:

Drug-induced hepatitis is inflammation of the liver that is caused by a toxic amount of certain medicines, vitamins, herbal or food supplements. We present a case of a young adult male with testosterone booster induced acute hepatitis, abnormally high liver enzymes but otherwise relatively asymptomatic presentation.

CASE DESCRIPTION/METHODS:

35 year old man, who presented to the hospital with flu like symptoms for 3 days. Gastroenterology was consulted for urgent evaluation of new onset nausea, vomiting and fatigue, associated with a >60-fold increase in his transaminases and alkaline phosphatase. His liver function was: AST U/L 2450, ALT 3300 U/L, Alkaline Phosphatase 200 U/L, LDH 1660 U/L, GGT 750 U/L, Indirect bilirubinemia, with abnormally high ferritin levels of >6000 ng/ml. His further workup including viral serology of HSV, Hepatitis, EBV, CMV, Influenza; Urine toxicology; ANA; Anti Smooth Muscle Antibody, Ceruloplasmin, Alpha-1 Antitrypsin was negative. Liver function including protein and PT/INR was normal. With an unremarkable spleen and portal vein ultrasound, he also underwent CT abdomen and pelvis without contrast on day 3 of admission which was not rewarding. With uptrending liver enzymes, liver biopsy was done and pathology showed findings consistent with drug-induced hepatitis- collapsed portal tracts with inflammation comprising of histiocytes, lymphocytes, scattered eosinophils with few plasma cells. On detailed questioning, he reported endorsing high dose testosterone booster orally, to improve his muscle mass. The medication was stopped. Liver enzymes started down trending over a period of 1 week. He was eventually discharged on oral steroids. Patient was thereafter lost to follow up outpatient.

DISCUSSION:

Drug induced liver injury (DILI) is mostly a diagnosis of exclusion with supporting liver biopsy findings. DILI has several clinical manifestations ranging from asymptomatic elevations in liver enzymes to fulminant liver failure culminating in death or liver transplantation. DILI may be predictable and dose-dependent, or may be idiosyncratic. Rapid and severe recurrence may occur with re-exposure and stopping the drug or supplement is of utmost importance. Our patient had mixed hepatocellular-cholestatic injury. General guidelines involve giving gluccocorticoids to patients with hypersensitivity reactions who have progressive cholestasis or no significant improvement despite withdrawal of causative agent. Patient counselling for avoidance is of utmost importance.
2355 Rare on Rare: A Simultaneous Presentation of SLE and PBC
Authors: Javed Abid T. , Koch David
Published: October 2019

INTRODUCTION:

Though autoimmune diseases commonly co-exist, systemic lupus erythematosus and concomitant primary biliary cirrhosis are uncommon. A recent literature review encompassing 34 cases noted that PBC was diagnosed in 69% of cases; in 5/34 cases, SLE and PBC were diagnosed simultaneously.

CASE DESCRIPTION/METHODS:

A 51 year old African American female with chronic kidney disease stage 3, hypertension, vitiligo presented with fatigue, anorexia, and >20 lbs weight loss over 3-4 months. Vitals were notable for blood pressure of 91/47, pulse 62, T 97.3F, and she was breathing room air. On exam, she appeared lethargic and fatigued. Scleral icterus and sublingual jaundice were noted. Discoid rash involving both ears was noted. Vitiligo of lower extremities was noted. Initial labs noted leukocytosis of 13.5 k/cumm, normocytic anemia, BUN 125 mg/dL, K 6.7 mmol/L, Cr 5.5 mg/dL, total bilirubin of 5.0, AST 86 U/L, ALT 218 U/L, ALP 1363 U/LDouble stranded DNA antibody was >13.0 IU/mL. F-actin and anti-smooth muscle antibody were negative. Anti-nuclear antibody was 1:2560. Anti-mitochondrial antibody (IgG) was 3.5. The patient’s hyperkalemia was treated, and after stabilization, patient underwent kidney biopsy and transjugular liver biopsy. These resulted, respectively, nephritis ISN/RPS Class III (C) and a cirrhotic liver severe bile ductular proliferation of the portal triads with absent bile ducts. Diagnoses of SLE with lupus nephritis and AMA negative PBC were made, and the patient was started on plaquenil and ursodiol.

DISCUSSION:

Liver involvement in patients with SLE can include primary liver disease as well as involvement due to SLE itself. Distinguishing between the true etiology of liver damage in these patients can be challenging, often due to confounding serological assays and use of immunosuppressive therapy in SLE. It is currently unknown whether SLE and PBC concurrence shares a common genetic or environmental factor, or if the diseases co-occur by chance. While liver dysfunction is not a diagnostic criteria for SLE, SLE related hepatitis (subclinical hepatopathy) has been described, particularly as a hypertransaminasemia. This is often noted during SLE flares, and improves or resolves with steroid therapy. Unfortunately, steroid therapy independent of other immunosuppressive therapies is associated with worsening outcomes in PBC, as well as development of fatty liver disease. Further exploration regarding co-occurrence and comanagement of SLE and PBC is needed, despite its rare incidence.
2363 Symptomatic Hepatic Sarcoidosis in a Hispanic Patient With Severely Elevated Liver Biochemical Tests
Authors: Harris Michael , Pottebaum Benjamin , Settergren Noah , Ruzkowski Charles
Published: October 2019

INTRODUCTION:

Sarcoidosis is a systemic granulomatous disease of unknown etiology that typically predominates as a respiratory disease process. Hepatic involvement is uncommon and reported in only 11.5% of cases and even rarer is symptomatic hepatic involvement that occurs in approximately 5 to 30% of those cases. Epidemiological studies show substantial variations in extrapulmonary involvement based on race, with few studies on the Hispanic population. This case report presents a Hispanic female who underwent extensive workup and was diagnosed with symptomatic hepatic sarcoidosis.

CASE DESCRIPTION/METHODS:

A 27-year-old Hispanic woman with history of a mediastinal mass presented to the emergency department with an abrupt onset of right upper quadrant (RUQ) abdominal pain and nausea. Liver biochemical tests were severely elevated in a hepatocellular pattern. A RUQ ultrasound showed a dilated common bile duct (CBD) with a questionable stone in the CBD. Next, a magnetic resonance cholangiopancreatography was performed and demonstrated a normal CBD without biliary ductal dilatation or choledocholithiasis. Workup for chronic liver disease was negative, as well as extensive workup for other causes (Table 1). Then a liver biopsy was performed with histology demonstrating scattered non-necrotizing granulomas (Figure 1) with negative stains for mycobacterial and fungal organisms. Review of past medical records revealed a biopsy of her mediastinal mass with histology showing a non-necrotizing granulomas (Figure 2). A diagnosis of sarcoidosis was established and she was started on oral prednisone and referred to pulmonology.

DISCUSSION:

A diagnosis of sarcoidosis involves a suggestive history, granulomas in two organs, negative staining for acid fast bacilli, and a lack of drug-induced disease. The majority of patients with hepatic granulomas have primary biliary cholangitis (PBC). Screening for anti-mitochondrial antibodies and elevated IgM levels is important as these are present in >90% of those with PBC. Infectious diseases should be ruled out including tuberculosis, AIDS-related diseases, Q fever, and Brucellosis. Race is the most important risk factor for developing extrapulmonary sarcoidosis, possibly due to involvement of the reticuloendothelial system. Hispanics account for 18.1% of the U.S population. It is possible that Hispanics are at increased risk for extrapulmonary sarcoidosis compared to whites, but more Hispanic epidemiology studies are warranted.
2367 A Rare Case of Primary Glomus Tumor of Liver
Authors: Sonti Sravanthi , Shreenath Aparna , Parsa Laxmi , Bohman Keith
Published: October 2019

INTRODUCTION:

Glomus tumors are very rare mesenchymal perivascular tumors arising in glomus body. They are mainly found in skin of extremities but rarely arise in visceral organs like gastrointestinal tract, mediastinum, bladder, and kidney. However, glomus tumors occurring in liver is extremely rare. We found only seven case reports in English literature so far. Our case is incidentally found after imaging was obtained to evaluate significantly elevated inflammatory markers.

CASE DESCRIPTION/METHODS:

A 60-Yr-old African American female with medical history of atrial fibrillation, hypertension, rheumatoid arthritis, anxiety, depression, adenomatous polyps was referred for evaluation of an abnormal PET-CT scan. She was seen by her rheumatologist with a complaint of worsening pain in her hips and lower back and 5lbs weight loss over past 3months. Laboratory evaluation showed normal complete blood counts and liver function tests but CRP 14.8mg/L, sedimentation rate 100mm/hr. A PET-CT scan showed an intensely hyper metabolic, peripherally enhancing, 2 cm × 1.8 cm, left hepatic lobe lesion concerning for malignancy. Moderately increased uptake was noted in cecal area suspicious for a primary site. No FDG avid nodal metastases, or vasculitis noted. A colonoscopy showed 1cm adenomatous polyp in cecum but no malignancy seen. A CT guided biopsy of the liver mass was performed which showed sheets of small bland cells with scant to moderate eosinophilic cytoplasm with centrally placed small nuclei.There is prominent vascular network throughout the tumor and the cells are positive for CD34 stain, smooth muscle actin, collagen IV, but negative for glycipan-3, arginase, AE1/3, pCEA, synaptophysin, chromogranin, KIT and DOG1. A diagnosis of glomus tumor was made. She was discussed in hepatobiliary conference and a recommendation was made for a surgical excision. She was seen by a surgeon but she denied surgery.

DISCUSSION:

Glomus tumor in liver is very rare and we report eighth case. Our patient is asymptomatic and immunohistochemical stains confirmed glomus tumor. It is vascular surrounded by epithelioid cells with round to oval nuclei, and eosinophilic cytoplasm. Tumor cells are positive for for markers like SMA, desmin , vimentin; may be positive for CD34/31, but negative for cytokeratins, CD117, neuroendocrine markers like synaptophysin, chromogranin, or protein S-100. The natural course and management options are not clear though surgical resection is recommended. The tumor is benign and has favorable prognosis.
2376 Diffuse Large B-Cell Lymphoma Presenting With Typical Features of HCC on Liver Imaging: A Diagnostic Conundrum
Authors: Liaquat Hammad , Goff Sara , Jhala Nirag , Kiyani Amirali
Published: October 2019

INTRODUCTION:

Hepatocellular carcinoma (HCC) is the most common primary liver malignancy. HCC can be solely diagnosed on cross-sectional imaging (CT or MRI) if a lesion meets the diagnostic criteria according to the Liver Imaging Reporting and Data System (LI-RADS), which has above 90% specificity. HCC commonly metastasizes to lungs, regional lymph nodes, kidney, bone marrow and adrenals but metastases to the spleen are rare. We present a case of liver masses with typical HCC characteristics on liver MRI which was ultimately proven to be diffuse large B-cell lymphoma (DLBCL) on liver biopsy.

CASE DESCRIPTION/METHODS:

38-year-old Vietnamese male with no past medical history was admitted with significant weight loss, fatigue and abdominal discomfort. He did not have a history of high risk behaviors or recent foreign travel. Physical exam revealed hepatomegaly. On labs, CBC was normal, T-bili 0.7, Alk Phos 147, AST 89, ALT 50, Albumin 3 g/dL, INR 1.1, creatinine 0.7. HBV serology was positive for HBsAg, anti-HBc and anti-HBe and was negative for HBeAg. HBV DNA was 497 IU/ml. HIV, HCV and HDV serology were negative. CT abdomen showed numerous liver lesions, largest of which was 10.4 cm and a single 8.1 cm splenic lesion. MRI showed several large, rounded and well-circumscribed liver lesions demonstrating arterial hyperenhancement, washout and pseudocapsule consistent with diagnosis of HCC (LI-RADS 5). MRI confirmed a single lesion on spleen (Figure 1), suspicious for metastasis. Tumor markers (AFP, CEA, CA 19-9) were within normal range. Lactate dehydrogenase (LDH) was 1200. Given elevated LDH and a spleen lesion, liver biopsy was done which showed high grade DLBCL (Figure 2). Systemic staging tests did not reveal any other tumor sites. Patient was started on tenofovir and chemotherapy with excellent response (Figure 3).

DISCUSSION:

Liver biopsy is rarely required to diagnose HCC if certain diagnostic criteria are met on cross sectional imaging, even without significantly elevated AFP. Extranodal lymphoma with liver and spleen involvement can present with lab and imaging abnormalities which can mimic HCC, as in our case. Markedly elevated LDH and spleen lesion raised suspicion for a different diagnosis. Undiagnosed or untreated advanced DLBCL can limit survival to a few months while hepatic involvement can also cause liver failure. In conclusion, a biopsy should be considered for liver lesions diagnosed as HCC on cross sectional imaging when atypical features, in our case elevated LDH and spleen lesion, are present.