bluuebunny/crossref_metadata_2025_split huggingface dataset:
2449 The Great Mimicker Syphilis and a Case of Hepatitis
Authors: Raman Daya 
Published: October 2019

INTRODUCTION:
Early syphilitic hepatitis during the stage of secondary syphilis is often an overlooked presentation of syphilis. Liver involvement occurs in 0.2–3% of patients with syphilis.
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CASE DESCRIPTION/METHODS:
Here, we present the case of a 31 yr old male with type 1 diabetes and hyperlipidemia who presented with epigastric abdominal pain that began on morning of admission. Patient denied any change in bowel movements and, on initial exam, was found to be tachycardic and hypotensive. On physical exam, patient had tenderness in the epigastrium without any skin changes noted. On evaluation of lab work, patient was found to be in DKA with moderately elevated LFTs, ALP 273, AST 400 and ALT 200, Bilirubin 0.2. Hepatitis panel and Acetaminophen level were negative. A right upper quadrant ultrasound was performed that did not show any evidence of cholelithiasis or passed stone. Patients DKA resolved one day into admission, however AST/ALT more than doubled from admission to 4000/1000 respectively, with rising ALP of 320. Bilirubin remained normal. Given elevated liver enzymes, there was concern for possible passed stone and therefore MRCP was pursued. MRCP showed incidental finding of hepatomegaly without any evidence of cholelithiasis. Acute hepatitis panel was also found to be negative. On further history, patient reported that he did engage in unprotected sexual intercourse approximately 1 month prior, with another male. Patient's abdominal pain resolved and he was discharged with HIV, HSV, RPR, GC Chlamydia studies pending. Lab workup revealed RPR to be reactive, 1:16 TU with reactive Treponema pallidum particle agglutination assay (TPPA), and negative HIV testing. Patient was diagnosed with secondary syphilis with hepatitis and treated with Benzathine Penicillin 2.4 MU IM in 2 divided doses. AST/ALT trended down to 116/43 and ALP 242.

DISCUSSION:
This case highlights the critical importance of a broad differential in workup of hepatitis.
2450 Gastroenterology, a Guiding Light in Hemophagocytic Lymphohistiocytosis
Authors: Viswanathan Sahityan , Capati Jeffrey , Hoang Long 
Published: October 2019

INTRODUCTION:
Transaminitis is not an uncommon consult for a gastroenterologist. The presence of profound transaminitis in the setting of septic shock often skews our etiology towards shock liver and other more common causes of acute liver injury in the absence of previous known liver pathology. HLH is a rare cause of acute liver injury but lack of prompt diagnosis and treatment can make the difference in outcome.

CASE DESCRIPTION/METHODS:
Patient is a 27 year old male with no known past medical history who presented with a two week history of fever in which he took acetaminophen every four hours. Reported history of alcohol consumption of 12 beers per day for three years. History remarkable for a camping trip to Oklahoma two to three weeks prior to admission. No history of NSAID or herbal use. No personal or family history of liver disease. Patient admitted to the ICU and treated for septic shock and subsequently started on N-acetylcysteine in the setting of acetaminophen use and acute liver injury. Patient had worsening renal function requiring initiation of dialysis, acute respiratory failure requiring intubation during evaluation. Gastroenterology consulted on hospital day two in which complete hepatic evaluation was initiated with remarkable findings of markedly elevated ferritin and positive Ebstein-Barr virus (EBV). Suspicion of Hemophagocytic lymphohistiocytosis (HLH) noted per Gastroenterology given high ferritin and diagnostic criteria being met. This prompted recommendations for evaluation by Hematology in which bone marrow biopsy confirmed HLH and prompt treatment with Rituxan, Dexamethasone and subsequent clinical improvement.

DISCUSSION:
EBV-HLH is an uncommon entity that is encountered on a daily basis. However, the mortality associated with the disease process is high if timely intervention is not implemented. A constellation of clinical features and laboratory results comprise clinical diagnosis of HLH combined with multi-speciality contribution to formulate the diagnosis. Transaminitis, elevated ferritin, cytopeniae should not skew a clinician's thought process towards shock liver altogether and EBV-HLH should be high on the differential early in the clinical course. Gastroenterology will be involved frequently in management due to transaminits and gastrointestinal bleeding manifesting frequently. Early treatment of EBV-HLH with immunosuppressants has shown to reduce mortality by 40% mandating more awareness about the disease in Gastroenterology and be a guiding light in the management.
2452 Intrahepatic Portosystemic Venous Shunt: A Clinically Significant Rare Anomaly
Authors: Nagaraj Savitha V. , John Febin , Etienne Denzil 
Published: October 2019

INTRODUCTION:
Intrahepatic Portosystemic Venous Shunts (IPSVS) are rare vascular malformations between the hepatic and portal veins. Most of the porto-venous shunts are asymptomatic and found incidentally on radiology imaging. These can be either congenital or acquired. Acquired shunts are typically less than 2 mm in size. Occasionally, complications such as encephalopathy, heart failure, pulmonary hypertension and elevated ammonia levels can occur. We report a case of incidental IPSVS in a patient who presented with complaints of fatigue and failure to thrive.

CASE DESCRIPTION/METHODS:
82-year-old African American male with hypertension, diabetes mellitus and prostate cancer (treated) presented with complaints of fatigue and loss of appetite for one-month. Laboratory work up showed a hemoglobin of 11.6 g/dl, normal platelet count, and elevated aspartate aminotransferase of 45 U/L and decreased albumin of 2.0 g/dl. Serum ammonia level was normal. He was immune to hepatitis A, non immune to hepatitis B and hepatitis C antibody was negative. A computed tomography scan of the abdomen showed a contrast enhanced tubular shaped vascular connection between middle hepatic vein and the anterior branch of the right portal vein. The size of the shunt was approximately 10 mm (Figure 1). Liver appeared normal except for small calcifications within the parenchyma. Ultrasound Doppler of hepatic and portal veins were performed, and the porto-hepatic shunt was visualized (Figure 2). Patient had a complicated hospital course secondary to sepsis; however, he did not develop hyperammonemia or hepatic encephalopathy during the hospital stay.

DISCUSSION:
IPSVS in our patient was likely a congenital anomaly given the presentation, size and lack of related symptoms. IPSVS is divided into 4 types; Type 1- Shunt between portal vein and IVC; Type 2- connections between peripheral branches of portal and hepatic veins in one hepatic segment; Type 3- Aneurysmal connection between portal and hepatic vein; Type 4- multiple communications of portal and hepatic veins in both major lobes of the liver. Doppler sonogram with pulse doppler is highly sensitive and specific to evaluate for IPSVS. In symptomatic IPSVS, shunt closure has shown improvement in symptoms and correction of hyperammonemia. Clinicians should consider this rare vascular malformation in patients presenting with hepatic encephalopathy or hyperammonemia in the absence of cirrhosis where shunt closure can be curative.
2457 Khat-Induced Hepatotoxicity
Authors: Kumar Kishore , Bhandari Peter , Essrani Rajesh Kumar , Chime Chukwunonso U. , Ahmed Rafeeq , Tariq Hassan , Patel Harish 
Published: October 2019

INTRODUCTION:
Khat is a commonly used recreational substance in Somali, Yemeni and East African countries due to its stimulant properties. The active ingredients of Khat are structurally similar to amphetamine and phenylethylamines respectively. Physiologic effects of Khat includes increased alertness, euphoria and hypertension. Khat use has been associated with subacute hepatitis and severe hepatitis leading to adverse outcomes including death. Rarely it can cause psychosis, acute coronary syndrome and stroke. Therefore, many countries have banned the possession and use of Khat, because of its potential for health and social harm. Here we present a case of hepatoxicity in Yemeni man after Khat consumption.

CASE DESCRIPTION/METHODS:
32-year-old man with no prior medical co-morbid condition presented to the ER with right upper quadrant abdominal pain for 1 day. The review of system is negative for fever, nausea, vomiting, diarrhea and weight loss. He denies use of herbal medication or over the counter medication. He has no personal or family history of liver disease. Social history was significant for recreational use of Khat and last use was few months prior to current illness. He denied alcohol and tobacco use. The physical examination revealed normal vital signs and right upper quadrant tenderness. Initial workup showed hepatocellular liver injury, indirect hyperbilirubinemia and gallstone on abdominal sonogram (Table 1). He had normal biliary tree on ultrasound and MRCP. Comprehensive workup for liver disease remained unrevealing subsequently he underwent liver biopsy which revealed lobular hepatitis with many eosinophils consistent with drug induced liver disease. The liver biopsy was negative for iron overload and significant steatosis. The presence of lobular hepatitis on liver biopsy and chronologic use of Khat in the absence of alternate etiology suggest Khat related hepatoxicity in our patient.

DISCUSSION:
The WHO classified Khat as a substance of abuse that can lead to severe dependence. While viral and alcoholic etiologies are the most common cause of hepatitis, Khat induced hepatitis is easily missed and often undiscovered. The mechanism of Khat-related hepatotoxicity remains to be elucidated though histopathologic evidence showed central vein congestion and hepatocellular degeneration and regeneration. The characteristic clinical picture is of acute or subacute hepatitis that resolves on cessation of Khat use. It is essential that other causes for liver disease be ruled out.
2459 Is Shorter Course of Gleceprevir and Pibrentasvir (MAVYRET) for Hepatitis C Treatment Possible? A Case Series
Authors: Jindal Robin , Sidhu Rajvinder , Martinez Anthony 
Published: October 2019

INTRODUCTION:
Direct-acting oral antiviral agents (DAA) including a combination of Gleceprevir and Pibrentasvir (Mavyret), among others, is the current standard of care therapy for chronic hepatitis C (HCV) infection. These medications have demonstrated high rates of sustained virological response at 12 weeks post-therapy (SVR12) in all genotypes of HCV. More specifically for Mavyret, multiple clinical trials have demonstrated SVR12 in HCV patients after 8, 12, or 16 weeks of therapy. There is, however, no data to support a shorter therapy course. In this small case series, we report 5 subjects who achieved SVR12 with Mavyret after 2 or 4 weeks of therapy.

CASE DESCRIPTION/METHODS:
Clinical data from a large cohort of patients treated for HCV was retrospectively reviewed for patients treated from 2015-2019. Data extracted and analyzed included patient demographics, HCV treatment regimen, HCV genotype, HCV RNA levels, fibrosis state and sustained virological response (SVR).

DISCUSSION:
Results: Among the 5 patients identified there were 4 males and 1 female. In total, 4 patients had genotype 1a and 1 patient had genotype 1b. The single patient with SVR12 after 2 weeks of therapy was genotype 1a. The remaining patients received 4 weeks of therapy. Fibrosis Score ranged from F0 to F1, HCV RNA levels ranged from 83,400-8,420,000 and all patients had a documented history of intravenous drug use (Table 1). Conclusion: In this small case series, we present 5 patients who achieved SVR12 after only 2 or 4 weeks of Mavyret therapy. When considering factors such as the cost of therapy, possible history of substance abuse, and/or poor socioeconomic status, opting for a shorter course of therapy may be beneficial. Further large-scale prospective clinical trials are needed to support these findings.
2460 A Father-Son Pair With APOB and PNPLA3 Double Heterozygosity Resulting in Advanced Nonalcoholic Fatty Liver Disease
Authors: Jansson-Knodell Claire L. , Gawrieh Samer , McIntyre Adam D. , Hegele Robert A. , Chalasani Naga P. 
Published: October 2019

INTRODUCTION:
Mutations in apolipoprotein (apo) B cripple the machinery and transport needed for lipoprotein export from the liver resulting in familial hypobetalipoproteinemia (FHBL). In heterozygotes, the normal allele allows for sufficient transport and mobilization of fat from the liver, but homozygotes are unable to compensate, leading to elevated intrahepatic triglycerides, NASH, and cirrhosis. We report a father-son pair with heterozygote FHBL who developed advanced NASH which we believe is due to its compound heterozygosity with PNPLA3, a gene important in the pathogenesis of NAFLD and NASH.

CASE DESCRIPTION/METHODS:
A Caucasian father and son of European ancestry were referred to our hepatology clinic. The son is a 46-year-old male who was evaluated for elevated transaminases and very low LDL-cholesterol. There was no significant alcohol consumption. Family history was significant for a father with cirrhosis and paternal grandmother who died of an unknown liver complication. Physical exam revealed obesity with hepatomegaly and acanthosis nigricans. Laboratory studies were notable for an LDL-cholesterol 15 mg/dL, triglycerides 30 mg/dL, and ALT 109 U/L. Increased liver echogenicity was found on ultrasound. Transient Elastography showed CAP of 327 dB/m (severe steatosis) and liver stiffness of 8.5 kPa (F2 fibrosis). Genetic testing revealed heterozygosity for both APOB (c.1830 -1G >A) and PNPLA3 (p.I148M) genes. The father is a 71-year-old male who was diagnosed with liver disease during evaluation for thrombocytopenia. There was no significant alcohol consumption. Physical examination revealed abdominal obesity and splenomegaly. Laboratory studies were notable for an LDL-cholesterol of 28 mg/dL and platelets of 83 k/mm3. Liver biopsy confirmed cirrhosis due to steatohepatitis and his HVPG was 12 mmHg. Recent imaging identified two small liver lesions with arterial enhancement, concerning for early liver cancer. Genetic testing revealed variants in both APOB and PNPLA3 genes identical to his son's mutations.

DISCUSSION:
We describe a father-son pair with double heterozygosity for APOB and PNPLA3 genes in the setting of obesity who developed progressive NASH. Neither APOB heterozygosity nor PNPLA3 heterozygosity is associated with advanced NAFLD, but their double heterozygosity may cause progressive NASH and cirrhosis, as illustrated here. A strong family history and very low serum LDL-cholesterol levels were helpful in working up this father-son pair.
2463 Infliximab Drug-Induced Autoimmune Hepatitis in Patient With Crohn's Ileocolitis
Authors: Karanfilian Briette , Mahpour Noah , Reja Debashis , Sarkar Avik 
Published: October 2019

INTRODUCTION:
Infliximab is an anti-tumor necrosis factor alpha inhibitor commonly used in the treatment of Crohn's disease. It is known to produce mild elevations in liver enzymes, however, severe damage and marked elevations in transaminases related to drug-induced autoimmune hepatitis (AIH) are rare. We present a case of a patient with Crohn's disease who recently began infliximab and presented with severe infliximab-induced autoimmune hepatitis.

CASE DESCRIPTION/METHODS:
A 37-year-old man with Crohn's ileocolitis, diagnosed at age 28 and recently inducted with infliximab, presented with fatigue, weakness, and epigastric pain. He had previously been on 6-mercaptopurine, adalimumab, and ustekinumab, but had most recently been on azathioprine (AZT) and began infliximab two months prior to presentation. Approximately one month prior to presentation, he developed elevated transaminases with aspartate transaminase (AST) and alanine transaminase (ALT) in the 300s, so AZT was discontinued as this was thought to be the cause. Despite discontinuation of AZT, his liver function tests (LFTs) rose dramatically, prompting hospitalization. Labs on admission revealed a hepatocellular pattern with total bilirubin 7.1, AST 2153, ALT 2931, alkaline phosphatase 156, and international normalized ratio 1.25. Notably, antinuclear antibody was positive 1:1280, smooth muscle antibody was weakly positive, and cytomegalovirus (CMV) IgM was elevated. Hepatobiliary scan and magnetic resonance cholangiopancreatography were consistent with hepatocellular disease. Core liver biopsy revealed active hepatitis with moderate activity (interface moderate), cholestasis, and an infiltrate of eosinophils and plasma cells. CMV staining was negative. Overall, the testing was consistent with drug-induced AIH. Infliximab was stopped and steroids were begun, resulting in normalization of LFTs.

DISCUSSION:
Infliximab has been implicated in mild cases of hepatotoxicity, and therefore, it is recommended to check LFTs before and after beginning the medication. There are very few reports of severe AIH that have been linked specifically to infliximab, as cases of hepatotoxicity are often secondary to reactivation of viral hepatitis or CMV infections, or related to other biologic therapies. This is a rare case of infliximab-induced AIH, and it is an important consideration in patients with elevated LFTs after starting this drug. Treatment involves cessation of infliximab and treatment with steroids, which usually leads to resolution of the hepatitis.
2470 Mysterious Ascites
Authors: Fernandez Carlos , Diaz Carolina , Bofill Aliana , De Jesús Henry , Caceres William , Martin-Ortiz Jose , Santiago Loscar 
Published: October 2019

INTRODUCTION:
Hypereosinophilic Syndrome (HES) is a rare disorder most commonly diagnosed in patients 20-50 years of age. It is defined as a persistent absolute eosinophil count greater than 1500 cells/microL and/or pathologic confirmation of tissue hypereosinophilia, as well as evidence of end-organ dysfunction. Prior to making a diagnosis, allergic, rheumatologic, infectious, and neoplastic etiologies should be excluded.

CASE DESCRIPTION/METHODS:
This is the case of an 87 -year-old male with past medical history of malaria treated with chloroquine who presented with altered mental status, dry cough and ascites de novo. Associated symptoms included unintentional weight loss of 10-15 lbs and intermittent episodes of non- bloody, watery diarrhea. He denied history of alcohol or drug use. Physical exam was remarkable for a distended abdomen with a positive fluid wave and pitting edema +1 in the lower extremities bilaterally. Labs showed a platelet count of 53,000, hypoalbuminemia (1.9 g/dl), increasing creatinine (2.1 mg/dl), alkaline phosphatase of 444 u/l, total bilirubin of 2.8 mg/dl with indirect predominance, AST of 59 u/l, ALT of 55 u/l, and eosinophilia of 72% (absolute eosinophil count of 6,048). Abdominal sonogram with doppler failed to show obstructive pathology and no evidence of hepatic vein thrombosis. Paracentesis showed absence of malignant cells, SAAG >1.1, 0.79 g/dl of protein and abundant eosinophils. Hepatitis, Iron Saturation levels, AMA, ASMA, Schistosoma and Strongyloides workup were negative. Hematology oncology service was consulted for bone marrow biopsy to asses for myeloid and lymphoid disorders. Results showed a hypercellular bone marrow with marked eosinophilia and 50% nucleated cells but C-KIT, PGFR Alpha, PGFRB, BCR/ABL, JAK 2 and TCR were all negative. A diagnosis of Hypereosinophilic Syndrome with suspected eosinophilic liver infiltration was made and the patient was started on prednisone therapy. All prior described multisystemic symptoms resolved. Eosinophilia, thrombocytopenia and all liver enzymes normalized within 24-48 hours after initiation of therapy. Ascites also resolved for which diuretic therapy was discontinued.

DISCUSSION:
This case is interesting as HES has an incidence of 0.36 per 100,000, and hepatic involvement accounts solely for an estimated 30% of patients. This serves to illustrate that HES should form part of the differential diagnosis when hepatic involvement and eosinophilia are concerned as prompt evaluation and treatment can lead to rapid resolution.
2471 UnDRESSing Elevated Liver Enzymes
Authors: Hakim Christopher M. , Melitas Constantine , Ngo Kha 
Published: October 2019

INTRODUCTION:
The differential diagnosis for liver transaminases over 1000 units/liters typically includes liver ischemia, acute viral hepatitis, acetaminophen-induced liver toxicity, autoimmune hepatitis, Wilsonian crisis, or acute Budd-Chiari. Prompt evaluation and exclusion of these causes must be completed as many of these etiologies can ultimately lead to fulminant liver failure. We present a case of liver transaminases over 1000 units/liter from an atypical etiology.

CASE DESCRIPTION/METHODS:
A 52-year-old male with hypertension, CKD stage 5, gastroesophageal reflux disease, and gout presented with intermittent fevers of 39C. He was recently hospitalized for Clostridium difficile colitis and treated with oral vancomycin. During this hospitalization he was started on allopurinol for an acute gout flare. Soon after starting allopurinol, he developed a diffuse morbilliform rash. On initial exam, vitals were within normal limits and temperature was 39C. Laboratory data revealed white blood cell count within normal limits, hemoglobin 7.9 gm/dL, platelet count 71 K/mcl, INR 1.1, AST 878 unit/L, ALT 1,117 unit/L, AKI, and peripheral eosinophilia. Viral hepatitis serologies, autoimmunity studies, as well as viral markers for EBV, CMV, HSV, and varicella were negative. Ceruloplasmin, Alpha 1 antitrypsin, and acetaminophen levels were within normal limits. N-acetylcysteine was administered due to suspicion for drug-induced liver injury. Given that he had taken allopurinol two weeks before presentation, presented with fevers, rash, and renal impairment and now developed peripheral eosinophilia, DRESS syndrome secondary to allopurinol was diagnosed.

DISCUSSION:
Drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare, potentially life-threatening, drug-induced hypersensitivity reaction. Diagnosis is suspected in a patient who received a drug treatment in the previous two to six weeks and subsequently presents with characteristic symptoms including skin rash, liver involvement, hypereosinophilia, and lymphadenopathy. Dress typically causes transaminase elevations, but usually not in the 1000s, as was seen in our patient. The pathogenesis is hypothesized to involve a genetically deficient detoxifying enzyme resulting in an accumulation of drug metabolites. Supportive therapy and prompt withdrawal of the offending agent is the primary treatment for drug hypersensitivity reactions. Additionally, N-acetylcysteine has been shown to significantly increase survival in cases of drug-induced liver failure.