bluuebunny/crossref_metadata_2025_split huggingface dataset:
2215 An Interesting Case of Intrahepatic Porto-Hepatic Shunt as a Cause of Unexplained Encephalopathy
Authors: Alvi Arsalan , Gupta Sonali , Goyal Pradeep , Pichardo Jose , Mensah Edward 
Published: October 2019

INTRODUCTION:
Congenital portosystemic shunts or Abernethy malformations are rare anatomical abnormalities shunting blood from the portal venous system to the systemic venous system, thereby bypassing passage through the hepatic acinus. The presentation may range from incidental finding on imaging to hepatic encephalopathy and liver failure depending on the degree and type of shunt. Here, we describe an interesting case of unexplained encephalopathy in a patient without liver cirrhosis found to have congenital intrahepatic Type II Abernethy malformation.

CASE DESCRIPTION/METHODS:
A 73-year-old female with a past medical history of Rheumatoid arthritis and Gilbert syndrome was admitted to hospital with clinical evidence of encephalopathy. Vitals were stable and physical examination was unremarkable except for encephalopathy. Laboratory investigations were unremarkable except for elevated ammonia levels (165 µmol/L). Computed tomography (CT) of the brain was unremarkable. Abdominal ultrasonography revealed the normal size and echotexture of the liver. Abdominal CT angiography showed the presence of dilated portal vein measuring up to 1.8 cm at the porta-hepatis along with dilated superior mesenteric and splenic veins. Multiple dilated vascular channels were seen within the right hepatic lobe. An intrahepatic portosystemic shunt between an enlarged middle hepatic vein and 2 separate branches of the right portal vein was visualized. Pressure gradients were measured and were normal. She received conservative management with lactulose and rifaximin and discharged on day 6 after the resolution of encephalopathy.

DISCUSSION:
Encephalopathy associated with portal-systemic bypass and no intrinsic hepatocellular disease is often referred to as Type B hepatic encephalopathy as was in our patient. Ammonia which is the culprit for the neuropsychiatric manifestation is usually metabolized by the liver in normal conditions, however in cases of portosystemic shunting, whether congenital or not, ammonia bypasses the liver and enters the systemic circulation and directly reaches the brain. The degree of symptomatology associated with these venous shunts depends on the shunt ratio and patient age. Treatment options include medical management including use of lactulose and non-absorbable antibiotics or endovascular shunt closure. Our patient was managed medically with the plan to refer her for shunt closure if she continues to have recurrent episodes.
2217 Silent Propafenone-Induced Liver Injury: Case Report
Authors: Musmar Ahmad , Hammami Muhammad B. , Azhar Mishah , Aboushaar Reem , Alzaraq Saleh , Miller Stephen M. , Salomon Peter 
Published: October 2019

INTRODUCTION:
Propafenone is a class Ic antiarrhythmic. It is metabolized primarily by hepatic cytochrome P-450 2D6 and rarely associated with liver toxicity. We report a case of silent propafenone-induced liver injury.

CASE DESCRIPTION/METHODS:
An 82-year-old man with DM, HTN, COPD, moderate mitral valve regurgitation, CABG, and aortic valve replacement, presented with progressive SOB and orthopnea of 2-week duration. Two weeks prior, he had atrial fibrillation treated with ablation, apixaban, and propafenone. Other medications were losartan, amlodipine, and furosemide. He denied alcohol/illicit drug use. He was alert, oriented, in moderate respiratory distress, and non-icteric. Temperature was 37.4 C, BP 155/67, HR 112, and RR 18. He had shallow breath sounds with bilateral diffuse crackles, bilateral lower extremity pitting edema, and unremarkable abdominal exam. EKG showed atrial fibrillation. He was treated with IV diltiazem and furosemide with improvement of SOB. However, on routine investigations, AST was 2182 IU/L, ALT 1805 IU/L, alkaline phosphatase 210 IU/L, total/direct bilirubin 4.6/1.0 mg/dL, and INR 3.4. Urine and serum toxicology, IgM hepatitis A virus, IgM hepatitis B core, hepatitis B surface antigen, hepatitis C virus Ab, Epstein-Barr virus, and cytomegalovirus were negative. ANA, smooth muscle Ab, LKM Ab, celiac serology, ceruloplasmin, alpha 1 antitrypsin, iron studies, and acetaminophen levels were all normal. Ultrasounography showed normal common bile duct and no intrahepatic biliary dilatation and was consistent with hepatocellular disease. Hepatic vessels doppler was unremarkable. Propafenone was discontinued and 5 days later, AST was 148, ALT 581, alkaline phosphatase 132, total/direct bilirubin 1.1/0.5; all tests normalized within 2 weeks.

DISCUSSION:
Hepatotoxicity secondary to propafenone is rare; only 9 cases have been previously reported. Our patient developed hepatotoxicity 2 weeks after starting propafenone, had immediate improvement after its withdrawal, and work up was otherwise negative, strongly suggesting propafenone as the cause. Similar to previous cases, hepatotoxicity had a cholestatic pattern and was completely reversible. Unlike previous cases where jaundice, pruritis, and abdominal pain were presenting symptoms; hepatotoxicity was incidentally discovered in our patient. Although very rare, clinicians should be aware of propafenone-associated hepatotoxicity and routine monitoring of hepatic function may be warranted.
2219 Chronic Active Epstein-Barr Virus Presenting Primarily With Abnormal Liver Enzymes
Authors: Palmer Quinton D. , Perisetti Abhilash , Garcia-Saenz-de-Sicilia Mauricio , Allard Felicia D. , Yee Eric U. 
Published: October 2019

INTRODUCTION:
Epstein-Barr virus (EBV) usually causes asymptomatic infections in adults. In rare cases, infected individuals develop Chronic Active EBV (CAEBV), a systemic disorder characterized by elevated EBV DNA in blood and lymphocytes in tissues. We present a rare case of CAEBV in a 24-year-old woman with serology suggestive of autoimmune hepatitis (AIH).

CASE DESCRIPTION/METHODS:
A 24-year-old Guatemalan woman presented with RUQ abdominal pain, nausea, vomiting and subjective chills for two weeks. Labs were significant for AST 1,167 U/L, ALT 1,049 U/L, total bilirubin 2.8 mg/dL, INR 1.2, ALP 1,118 U/L, GGT 123 U/L, EBV PCR > 1,000,000 copies, EBV IgM positive, positive ANA, serum IgG 3,690 mg/dL (normal 700 – 1,600), weakly positive smooth muscle antibody (SMA) titers 1:40 (negative < 1:20), and F-actin antibodies 42 units (positive ≥ 31 units). Mild scleral icterus and RUQ abdominal tenderness noted on physical exam. CT abdomen & pelvis showed hepatosplenomegaly. Liver biopsy (LB) showed panlobular hepatitis with focal parenchymal necrosis and a prominent sinusoidal T-lymphocyte dominant infiltrate, many of which were positive for EBV-encoded small RNAs (EBER) on in situ hybridization (Figure 1). Bone marrow biopsy (BMB) showed mildly hypocellular bone marrow without evidence of lymphoproliferative disorder. She left against medical advice (AMA) 6 days later and did not follow up in clinic. Five months later, she returned with similar complaints with pancytopenia and febrile neutropenia. A repeat BMB showed a hypercellular bone marrow with findings suggestive of CAEBV of T-cell type. She left AMA shortly after initiating steroids. A referral to the National Institute of Health for stem cell transplant (SCT) was unsuccessful as she was lost to follow up.

DISCUSSION:
Hepatic manifestations of CAEBV include acute hepatitis in 47% of patients in the United States with a mixed hepatocellular-cholestatic pattern and hepatomegaly. Her labs were suggestive of AIH such as elevated IgG, positive ANA, weakly positive SMA titers & elevated F-actin antibodies. Several cases report false positive serology suggestive of AIH in patients with active EBV. Inappropriate and possibly dangerous treatment could result if one is unaware of this association. Allogenic hematopoietic SCT is the only known curative therapy to date and without treatment, the average time to death after disease onset is 6.2 years. Given the high mortality associated with delay in treatment, prompt recognition and diagnosis of CAEBV is imperative.
2220 Incidental Finding of Hepatocellular Neoplasms in Non-Cirrhotic Patients: A Case Series
Authors: Ling Christina , Khalid Sameen , Martin David , Hanson Joshua , Castresana Daniel 
Published: October 2019

INTRODUCTION:
About 20% of hepatocellular cancers (HCC) arise in the absence of cirrhosis, such as in the setting of glycogen storage disease (GSD) and anabolic steroid use. Non-cirrhotic HCC tends to have a lower male predominance, bimodal age distribution, and lower prevalence of hepatitis B, C, and alcohol abuse. There is a larger tumor burden at time of HCC diagnosis but a higher amenability to hepatic resection given preserved liver function and better overall survival than cirrhotic patients.

CASE DESCRIPTION/METHODS:
Case 1 A 16-year-old male presented after a motor vehicle accident for evaluation of traumatic injuries. He was a body-builder who used anabolic steroids. Physical exam showed a palpable liver edge. Labs were unremarkable. Computed tomography scan of the abdomen showed an incidentally discovered mass in the right hepatic lobe. Magnetic resonance imaging (MRI) of the abdomen showed a 15.1 × 15.0 × 10.8 cm right hepatic lobe mass concerning for a hepatic neoplasm. He underwent partial hepatectomy. Resected surgical specimen showed a β-catenin activated well-differentiated hepatocellular carcinoma. Case 2 A 26-year-old man with GSD type 1a was evaluated for iron-deficiency anemia. Physical exam showed tachycardia, conjunctival pallor, and palpable liver edge. Labs were notable for hemoglobin of 12.6 gm/dL, platelet count of 529 × 103/µL, alanine aminotransferase 92 U/L, and aspartate aminotransferase 82 U/L. MRI of the abdomen showed multiple large arterially enhancing liver masses within the right and left hepatic lobes. The largest lesion within the right lobe of the liver measured 20.2 × 17.7 × 13.5 cm. Endoscopic ultrasound showed a large right heterogenous hepatic lobe lesion, appearing malignant which underwent fine needle aspiration. Pathology was consistent with β-catenin activated hepatocellular neoplasm. The patient is scheduled for a right hepatectomy with possible living donor liver transplantation given the large and multifocal nature of his liver masses.

DISCUSSION:
There are four different phenotypes of hepatocellular adenomas; the β-catenin subtype has the highest malignant potential. Anemia in GSD appears to be related to large hepatic adenoma formation and warrants imaging of the abdomen to evaluate for a bleed or growth of the lesions. No effective imaging tool or biomarker separates hepatocellular adenoma from HCC, so it is important to keep a low threshold to biopsy a hepatic adenoma with suspicious imaging features.
2222 Primary Combined Hepatocellular Carcinoma-cholangiocarcinoma: A Case Report for Under-Diagnosed Primary Liver Cancer
Authors: Affas Saif , Malapati Sindhu , Chand Momal , Hadid Tarik 
Published: October 2019

INTRODUCTION:
Combined hepatocellular carcinoma- cholangiocarcinoma (HCC-CC) was first classified in 1949 by Allen and Lisa et al. It composes less than 1% of primary hepatic malignancies. HCC-CC is being increasingly identified given the advances in immunohistochemistry (IHC) techniques. Due to its rarity, there are no formal treatment guidelines. However, a national cancer database review reported a worse prognosis.

CASE DESCRIPTION/METHODS:
A 73-year-old man with history of alcohol dependence presented with 2-week history of sharp right upper abdominal pain (RUQ). Review of systems was negative. Examination revealed RUQ tenderness. Hepatitis B and C serology were negative. Abdominal ultrasound showed two heterogeneous, hypoechoic liver lesions. Abdominal MRI showed enhancing masses in the left and right liver lobes. CT-guided liver biopsy confirmed the diagnosis of HCC -CC. The left sided tumor was treated with yttrium 90 radioembolization. Unfortunately, his restaging MRI showed progressive enlargement of the right hepatic lobe tumor and complete venous thrombosis of the portal vein. With rapidly worsening performance status, he was not a candidate for any further treatment and was enrolled in hospice care.

DISCUSSION:
HCC-CC is a primary liver tumor which presents diagnostic challenges to the pathologist, radiologist and clinician. Adequate tissue sampling is crucial to capture enough tumor to allow recognition of both histologies. Hence, core needle sampling would be preferable to fine needle aspiration. Triphasic CT/ MRI imaging may have characteristics of either tumor type. Tumor markers are often discordant with the imaging findings, which should raise suspicion for this entity. Histology and IHC are the only definitive way to establish diagnosis, which reveal a spectrum from both histologies occurring independently yet concurrently in the liver, to mixed cells of both histologies in same lesion to a fibrolamellar HCC like histology. Notably, the World Health Organization histological classification does not have prognostic or treatment impact. Although the prognosis of HCC-CC tends to be worse than both HCC and CC, there is uncertainty if it should be treated more like HCC or CC. Surgical resection and liver transplantation are the only curative option. For resected patient, survival was substantially worse than those with HCC. Inoperable patient and unresectable tumors can be treated locally with trans-arterial chemoembolization, radioembolization, and systemic therapy.
2226 Hot Spot Sign on Tc-99m DISIDA Nuclear Hepatobiliary Scan
Authors: Fakhri Asif A. , Iqbal Sabah 
Published: October 2019

INTRODUCTION:
Hepatic Focal Nodular Hyperplasia (FNH) can be an unusual cause of abdominal pain. Prior to the advent of readily accessible diagnostic imaging techniques, patients with FNH primarily presented with vague symptoms of abdominal pain, then proceeded to imaging, which led to diagnosis. Today, most cases are found incidentally on imaging, in often asymptomatic patients. Here, we present a patient with significant symptoms in whom an identified FNH was considered inconsequential, but is eventually proven otherwise.

CASE DESCRIPTION/METHODS:
A previously healthy 31-year-old male presented with a one-month history of persistent postprandial right upper quadrant abdominal pain. Ultrasound showed cholelithiasis. MRI revealed FNH of the anteroinferior subsegment of the liver. Tc-99m DISIDA Cholescintigraphy showed a normal functioning gallbladder, but an abnormal delayed clearance of tracer from a focal area in inferior tip of the liver, corresponding to the FNH seen on MRI, consistent with a “hot spot sign”. Due to suspicion for symptomatic cholelithiasis, he underwent elective cholecystectomy. Symptoms persisted post-op, thus, his pain was thought to be from symptomatic FNH. After subsequent elective FNH resection, symptoms subsided.

DISCUSSION:
While cholescintigraphy typically evaluates for biliary tree dysfunction, it can also reveal abnormality in hepatic parenchymal tissue. Previously, Tc-99m Sulfur Colloid Liver-Spleen scans were used for diagnosis of FNH. However, the “hot spot sign” on cholescintigraphy has also been described as diagnostic for FNH. This case illustrates that a focal area of delayed tracer washout, or “hot spot”, can also be seen with Tc-99m DISIDA. Although the significance of delayed tracer washout is unclear, this finding suggests liver parenchyma’s inability to effectively clear bile from an FNH. This inability to adequately clear bile, may result in pain. The severity of impaired clearance may even correlate with the severity of symptoms. Although further investigation is necessary, cholescintigraphy may prove a useful tool in functional imaging for differentiating between symptomatic and asymptomatic FNH.
2237 Double Trouble: Coxsackie A and B Induced Hepatitis in an Adult
Authors: Yakubov Stanley , Weissman Simcha , Panitch Jonathan , Tsirlin Yuriy , Rahmani Rabin , Mayer Ira 
Published: October 2019

INTRODUCTION:
Coxsackie virus which was first isolated in 1948 is a relatively common virus predominantly effecting children. There are 23 serotypes of Coxsackie A and 6 serotypes of the Coxsackie B viral strains. Clinical manifestations are generally mild in nature. We present a rare case of a 41yo female with Coxsackie A and B virus induced hepatitis.

CASE DESCRIPTION/METHODS:
41yo F PMH DM, presented 36 weeks pregnant c/o 4 days of severe SOB and b/l LE edema. Home medications included metformin, insulin and pre-natals. No recent travel or use of herbal supplements. VS were T-98.3F, BP 130/92, HR 130, RR-22. PE showed LE edema and crackles on auscultation. Pt had severe features of preeclampsia and echo showed Global Cardiomyopathy (EF 21-25%). Labs showed Ph-7.21, CO2-33, HCO3-13, Cr-0.8, LDH-252, Alb-2.7, Bili-0.9/0.3, AST-40, ALT-23, ALP-184, WBC-11.8, Plt-172, INR-1.1, high urine protein. She was intubated for airway protection and taken for C-section. Over the next 3 days pt became febrile, hypotensive, tachycardic and worsening of her labs was noted, Cr-1.5, LDH-1528, Alb-2.1, Bili-0.9/0.5, AST-767, ALT-597, ALP-127, WBC-25.9, Plt-219, INR-1.3 before they started to improve. Pt was managed with aggressive diuresis, broad spectrum abx, and IV pressors for presumed septic/cardiogenic shock. Work up was positive for Coxsackie A and B virus. Pts bilirubin never increased beyond its current values. The rest of liver disease work up was unremarkable. Pt was discharged after stabilization and optimization of her medical condition.

DISCUSSION:
Coxsackie belongs to the Enterovirus genus, a member of the Picornaviridaefamily. It spreads via the fecal oral route, direct contact with bodily secretions or inhalation. Diagnosis is established with a positive viral culture or an increase in IgM titers. Coxsackie virus infection presents with nonspecific febrile illness, but may include pericarditis, myocarditis, hepatitis and encephalitis. Coxsackie A causes Hand foot and mouth disease, while Coxsackie B is the culprit of hepatitis. Hepatitis as a result of Coxsackie A strain is a very rare clinical phenomenon especially in the adult population. As seen in this case, the pts hepatitis and severe cardiomyopathy were likely secondary to Coxsackie virus. Treatment of Coxsackie virus infection is mainly supportive. Although commonly viral hepatitis is a results of Hepatitis A, B, C, D, E virus, HSV, EBV, and HIV, the possibility of Coxsackie virus induced hepatitis should always be considered.
2239 Rotavirus, a Gastroenteritis Associated With Acute Transaminitis in Adult
Authors: Ponniah Sandeep Anthony , Homsi Maher 
Published: October 2019

INTRODUCTION:
Rotavirus gastroenteritis is commonly seen in the pediatric population. In contrast, the role of rotavirus in adults is far less reported. Infection typically presents as traveler’s diarrhea, in household members of affected children, and in outbreaks of gastroenteritis in vulnerable resident populations such as colleges and nursing homes. Laboratory findings are usually normal and mild elevations of serum transaminases may be seen during acute illness. The hepatic injury seen in adults and more severe elevations in transaminases have not been extensively reported.

CASE DESCRIPTION/METHODS:
We present a case of a 44-year-old female who presented to our emergency department with complaints of intractable vomiting, watery diarrhea and abdominal pain for a few hours. The patient had just returned to the United States from vacation in Mexico on the day prior to presentation. Patient was admitted for dehydration and symptomatic management. Laboratory workup was significant for leukocytosis of 17.400, normal hepatic transaminase and bilirubin. Gastrointestinal PCR panel was ordered and showed rotavirus A infection. On the next day, the patient noted to have significantly elevated transaminase with an AST of 504, an ALT of 444 with normal bilirubin and alkaline phosphatase. Transaminase continued to trend up and peaked the next morning at an AST of 689 and an ALT of 798. Acute hepatitis workup including HAV IgM, HBsAg, HBsAb, HCV antibody, HIV, EBV IgM, CMV IgM, and CK were all within normal limits. Ultrasound of the liver showed cholelithiasis without cholecystitis or common bile duct dilation. The patient was subsequently discharged the following day as transaminase started to trend down. Upon follow up a few days after discharge, laboratory workup showed near normal transaminase.

DISCUSSION:
We present a unique case of a middle aged female with travelers’ diarrhea due to Rotavirus A infection with moderately elevated transaminases. Transaminitis had been reported in literature but usually as only a mild elevation of transaminase. Rotavirus testing and diagnosis in the appropriate clinical setting can change management and prevent nosocomial spread. We would like to emphasize that viral gastroenteritis and specifically Rotavirus could be correlated to not only mild but also moderate and severe elevations of transaminases. Rotavirus A infection should be considered in the differential diagnosis of adults with acute gastroenteritis and elevated liver enzymes.
2241 Isolated Polycystic Liver Disease: Case of Long-Term Progression of Hepatic Cyst
Authors: Ali Sara I. , Zhang Hao Chi , Guha Sushovan 
Published: October 2019

INTRODUCTION:
Isolated polycystic liver disease (IPLD) is a rare congenital anomaly characterized by cystic lesions throughout the hepatic parenchyma. With advances and availability of imaging techniques, incidental findings have led to early diagnosis of asymptomatic patients. Currently, there are no universally accepted guidelines for monitoring patients with IPLD. We present a 51-year-old woman with an incidental finding of IPLD, the progression of disease, and management over a ten-year period in effort to elucidate course of disease.

CASE DESCRIPTION/METHODS:
51-year-old Hispanic woman with a history of choledocholithiasis requiring endoscopic retrograde cholangiopancreatography and open cholecystectomy presented to clinic for intraoperative finding of hepatic cysts. She had no known family history of polycystic liver or kidney disease. Intraoperative inspection of the abdominal cavity revealed marked hepatomegaly with multiple cysts consistent with pre-operative computed tomography. Her presentation was most consistent with IPLD. Over the course of ten years, she was monitored with liver biochemical testing and interval surveillance by cross-sectional imaging. With progressive enlargement of hepatic cysts, she developed symptoms of right upper quadrant fullness and abdominal distension attributed to constipation. There has been no abnormal liver biochemical testing, and her hepatic function remained preserved. Non-invasive methods with pain management and promoting regular bowel function have been sufficient to manage her symptoms.

DISCUSSION:
In 1906, pathologist Moschcowitz described IPLD as a phenotypic variant of polycystic kidney disease. The two entities were thought to coexist on a spectrum until the 1950s when IPLD was proposed to be a distinct condition. The progressive nature leads to a variety of symptoms: abdominal pain, early satiety, dyspnea, edema or jaundice. The broad clinical spectrum results from variable size, distribution and rate of progression of cysts. Current literature recommends conservative management with serial monitoring of synthetic hepatic function and assessment of symptoms. Complications of hepatic cysts include intracystic hemorrhage, infection and post-traumatic rupture; however, less than 5% of these patients require intervention. Although invasive therapies exist, the high rates of mortality and morbidity discourage their use and management prioritizes symptom management and quality of life.