bluuebunny/crossref_metadata_2025 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.
2243 Abdominal Pain Born From Migrating IUD to the Liver
Authors: Pacha Faris , Matsuzak Francis , Skrove Jamie L. , Paniker Linoj , Sobrado Javier , Rana Zaid
Published: October 2019

INTRODUCTION:

The use of intrauterine devices are a core of contraception practiced globally. There are several different types and brands that are FDA Approved for use in the United States. The advantages include their length of efficacy, one cost, and safe for breastfeeding. The risks of using IUDs include infection and migration. These risks have been as high as 3% but more consistently at one to two percent. Usual mechanisms for migration include erosion through uterine tissue or expulsion from vaginal canal. Once migrated the device is removed and another is placed as efficacy drops.

CASE DESCRIPTION/METHODS:

Patient is a 43-year-old female Hispanic American who presented to the hospital with complaints of generalized abdominal pain and low back pain for one year. The patient has no past medical history and does not take any medication besides ibuprophen for minor aches and pains. The patient has a surgical history of copper IUD placed five years prior. The patient mentions at a recent appointment with her primary care doctor her “liver tests” were “a tad elevated.” The patient has no family history of cancer or gastrointestinal history. Labs were performed and were WNL other than moderately elevated transaminases. Imaging demonstrating the patient’s intrauterine device had migrated to the anterior portion of the liver.

DISCUSSION:

There have been less than three cases including this one where an IUD migrated to the liver. The migration of the copper IUD was causing the transaminitis and general abdominal pain. This Case was chosen because it demonstrates a complication that is less than one percent. There have been very limited cases of an IUD migrating to the liver and even lower causing transaminitis. The mechanism of the migration in this case is unknown and thus makes this case very unusual. The proposed mechanism is erosion through the uterus and migration to the anterior portion of the liver.
2247 Transfusion Associated Babesiosis: An Uncommon Cause of Acute Liver Failure
Authors: Brandt Daniel R. , Balasubramanian Manjula , Navarro Victor J. , Kalman Richard
Published: October 2019

INTRODUCTION:

Babesiosis is a zoonotic disease caused by obligate intracellular protozoa that reproduce in erythrocytes. We present a case of transfusion-transmitted babesiosis (TTB) leading to acute liver failure (ALF). Whereas mild liver chemistry abnormalities are common in babesiosis, to the authors’ knowledge this is the first case of TTB directly leading to ALF.

CASE DESCRIPTION/METHODS:

A 79-year-old man from New Jersey with end stage renal disease presented with two weeks of flu-like illness and was found to have profound liver function abnormalities consistent with ALF. Six weeks prior the patient was hospitalized for a diverticular bleed at which time he received multiple packed red blood cell transfusions and recovered to his baseline health. At that time, liver chemistries were normal. On arrival, he was afebrile and normotensive. He was oriented but lethargic. Skin exam notable for pallor. His abdomen was benign. Initial investigations revealed AST 3497 IU/L, ALT 2719 IU/L, ALP 259 IU/L, TB 5.2mg/dl, DB 3.6mg/dl. INR was 4.1, Hgb 8.7g/dL, and fibrinogen 252mg/dl. LDH 2003 IU/L with haptoglobin < 8.0mg/dl. Peripheral smear revealed ring-shaped intra-erythrocytic trophozoites (Figure 1). Babesia microti PCR was positive. Atovaquone and azithromycin were started and exchange transfusion performed. Fevers and worsening encephalopathy progressed despite directed treatment. Multi-system organ failure developed and care was withdrawn. Further testing determined our patient was a recipient of babesia microti contaminated transfusion.

DISCUSSION:

Babesiosis is a world-wide emerging disease caused by intra-erythrocytic protozoa that are transmitted primarily through Ixodes ticks but also through blood transfusion. TTB is often severe in presentation with a mortality rate of approximately 20%. Marked hemolytic anemia, disseminated intravascular coagulation, and adult respiratory distress syndrome may develop. Case series have established that patients with babesiosis exhibit abnormal liver chemistries with median values notable for ALT of 59 IU/L and AST 79 IU/L. A literature review reveals just one published case of babesiosis presenting as ALF, and no such cases when babesia is transmitted through transfusion. Babesiosis should be considered in the differential diagnosis of ALF in patients living in or having recently travelled to an endemic area or who have recently received transfusions. In such patients, we recommend performing blood smear to prevent delay in treatment.
2253 Is There a Role for TAVR in Cirrhotics With Severe Aortic Stenosis? A Case Report
Authors: Hyder Jeffrey , Srinivasan Sachin , Tofteland Nathan , Salyers William , Fanari Zaher
Published: October 2019

INTRODUCTION:

Patients with cirrhosis requiring transplant are usually fraught with co-existing co-morbidities that make them poor surgical transplant patients. One such common co-morbidity is severe aortic stenosis. Trans-catheter aortic valve replacement (TAVR) is a promising alternative to surgical aortic valve replacement (SAVR) in patients at intermediate to high surgical risk. A recent meta-analysis by the above authors (Srinivasan, Fanari et al) has shown improved medical and surgical outcomes in patients undergoing TAVR. We here describe the case of a patient with cirrhosis and severe aortic stenosis who had significantly improved outcomes post-TAVR.

CASE DESCRIPTION/METHODS:

A 73-year-old white male with past medical history significant for CAD status post-CABG, congestive heart failure with reduced ejection fraction (EF 30%), end-stage renal disease on dialysis (EGFR- 20 mL/min per 1.73 m2 ) and cirrhosis secondary to hepatitis C (MELD-24) presented to the clinic for increased shortness of breath and fluid retention over the past two days. Most recent ECHO demonstrated severe aortic stenosis (AS), moderate to severe mitral valve regurgitation, and atrial fibrillation. The patient had a balloon valvuloplasty one-year prior for his AS. On CT, the aortic valve was found to be tricuspid and have bulky calcifications of the aortic valvular leaflets with a calcium score of 2000. He was deemed a poor surgical candidate given his co-morbidities and it was discussed that he might benefit from TAVR. He underwent the procedure without any complications. Post-procedure ECHO demonstrated no evidence of aortic stenosis or para-valvular leak. The patient’s mitral valve regurgitation was reduced from severe to mild to moderate. His LVEF was increased mildly to 35%. One day after the procedure the patient’s EGFR increased to 50 mL/min per 1.73 m2 and the patient no longer needed dialysis. The patient’s MELD score significantly decreased from 24 to 11. His calculated MAYO post-operative mortality risk at 7, 30, and 90 days decreased from 22%, 65%, and 81% to 4%, 15%, and 24% respectively. The patient tolerated the procedure well and was discharged to home two days post-operatively.

DISCUSSION:

Patients with cirrhosis and co-existent co-morbidities (esp. AS) are poor surgical candidates. TAVR in such patients could significantly improve their post-operative mortality risk and potentially improve their candidacy for liver transplantation.
2254 A Rare Case of Valproate-Induced Encephalopathy Requiring Hemofiltration
Authors: Myat Thin , Win Min
Published: October 2019

INTRODUCTION:

We present a rare case of acute encephalopathy due to Valproate induced hyperammonemia required Hemofiltration (HF).

CASE DESCRIPTION/METHODS:

45-year-old male was brought in by caregiver to the hospital with acute altered mental status, somnolent but arousable. PMHx of CP, Epilepsy, Left sided hemiplegia, Mental retardation, Abscess of thigh and wrist. Medications were Valproate and Phenytion. PE showed stable vitals, confused, was spitting to staff required restraints. Pupils equal, reactive to light. Neurological exam- left sided hemiplegia. Lab showed Na 140, K 4.0, HC03 30, BUN 7, Cr 1.18, Glucose 101, Osmolarity 287, ALT 24, AST 24, T Bil 1.1, Albumin 3.5, Al phos 79, Mg 2.0, lactic 1.2. WBC 6.9, hemoglobin 15.2, platelet 291, INR 1.2, Ammonia 402. Blood & urine culture and toxicology were negative. Acute hepatitis panel was non- reactive, Ceruloplasmin 32, ferritin 33.7, AMA < 20.1, Smooth muscle antibody < 1:40. Valproic acid level 125 mcg/ml (therapeutic level-50-100 mcg/ml). ECG was normal. Head CT showed no acute abnormality. Ultrasound showed fatty infiltration & Cholelithiasis. Unremarkable EGD. Lactulose & xifaxan were given for hyperammonemia, recurrent seizures occurred.

RESULTS:

Valproate was discontinued. L-carnitine 100 mg/kg IV loading dose was given. Received HF for 3hrs , and supportive care. Ammonia at 2hours post HF was 80. Patient’s mental status improved back to baseline over 24 hours.

DISCUSSION:

Acute encephalopathy due to valproate induced hyperammonemia required Hemofiltration is very rare. The incidence of valproate-induced hyperammonemia in the general population of patients taking VPA (Valproate acid) is not well established. Most cases of Valproate acid- toxicity are rather benign and resolve with supportive therapy. It is generally accepted that serum level >180 mcg/ml represents a high risk of toxicity. Our case was a first patient without chronic liver disease and valproate level was below high risk for toxicity who presented with acute encephalopathy due to valproate induced hyperammonemia and required Hemofiltration. Care of patients depends upon recognition of those at increased risk, anticipation & prevention, and appropriate response when they occur. Discontinuation of Valproate may require. L-carnitine has a significant mortality benefit in patient severe Valproate toxicity. HF is reserved for severely ill patients who have not responded to less invasive treatment.
2258 Cascara Sagrada: A Dangerous Herbal Stool Softener: A Case Report and Literature Review
Authors: Flores Estefania , Mbachi Chimezie , Randhawa Tejinder , Vohra Ishaan , Agrawal Rohit , Mba Benjamin
Published: October 2019

INTRODUCTION:

The percentage of drug-induced liver injury due to the use of herbal supplements has exponentially increased over the last decade from 7% to about 20%. Liver injury attributed to supplement use is associated with more deaths than cases attributed to anabolic steroid use or even allopathic medications.

CASE DESCRIPTION/METHODS:

53-year-old female presented with jaundice, pruritus, fatigue, clay-colored stools and dark-colored urine for 4 days. She reported taking an herbal supplement called cascara sagrada for constipation over an 8-week period. On physical examination she appeared jaundiced, abdomen was non-tender, non-distended, and there was no hepatomegaly. Laboratory tests were significant for total bilirubin 8.2 mg/dL, direct bilirubin 5.5 mg/dL, ALP 459 U/L, GGT 733 U/L, AST 1274 U/L, ALT 1711 U/L, INR 0.97, ferritin 1409 ng/ml, transferrin saturation 59%, viral hepatitis antigens and antibodies were negative and an autoimmune panel was unremarkable. Liver ultrasound revealed coarse echotexture with no hepatomegaly and no biliary dilatation. Patient was discharged with close follow up. Five days after discharge, total bilirubin was 19 mg/dL with worsening symptoms necessitating re-admission. Further workup with liver biopsy showed marked portal and lobular chronic inflammatory infiltrate and focal bile duct injury and cholestasis; iron stain showed minimal Kupffer cell iron deposition but no hepatocellular deposition. Patient improved clinically and biochemically after discontinuation of cascara sagrada, with normalization of liver function tests after 3 months of the initial presentation.

DISCUSSION:

Cascara Sagrada is a herbal medication used for short term treatment of constipation, generally well tolerated but is known to cause liver injury when used in higher doses than recommended. Anthraquinone derivatives are the active laxative components; promoting peristalsis and inhibiting reabsorption of water and electrolytes. Cascara is slightly absorbed, and typical recommended courses are for less than 7 days. The mechanism of liver injury is not well understood but it has been hypothesized that it is mediated by the direct toxicity of anthraquinone derivatives. Liver injury can present with a pattern of serum enzyme elevation that can be either hepatocellular or cholestatic and ranges in severity from mild transaminase elevation to severe hepatitis with associated portal hypertension and ascites. Nevertheless, injury is usually self-limited and typically reversible upon discontinuation.
2262 Too Much TAE: A Case of Tumor Lysis Syndrome After Repeat Transarterial Embolization of Hepatocellular Carcinoma
Authors: Henry George
Published: October 2019

INTRODUCTION:

Hepatocellular carcinoma (HCC) is the sixth most common cancer in the world & the second highest cause of cancer-related deaths globally. In the US, HCC incidence has almost tripled since the 1980s & has become the fastest rising etiology of cancer-related deaths. Tumor lysis syndrome (TLS) is a condition that results when malignant cells release their intracellular contents, either spontaneously or in response to cytoxic treatment. The cellular contents result in hyperuricemia, hyperkalemia, hyperphosphatemia, & hypocalcemia; these disturbances can lead to arrhythmias, AKI & death. TLS is common in leukemias & lymphomas but is rare in solid tumors.

CASE DESCRIPTION/METHODS:

A 50-year-old female with biopsy proven HCC without cirrhosis was admitted for planned transarterial chemoembolization (TACE) of her large right lobe & medial left lobe HCC, measuring 13.3 × 17.9 × 21.8 cm (Image 1), as too advanced for surgical resection or transplant & she had large liver-lung shunt preventing radioembolization treatment. Interventional Radiology (IR) placed two vials of LC beads containing doxirubicin 100mg & bland transarterial embolization (TAE) via 2.5 vials of Embospheres into right hepatic artery. Afterwards, she had acute drop in Hgb with CTA revealing multiple areas of active bleeding in the liver & new hematoma centrally in the mass. IR performed complete embolization of the right hepatic artery using coils & gelfoam to achieve hemostasis; patient was transferred to ICU. She received blood products & lab monitoring, the latter demonstrated new hyperkalemia of 5.5 mmol/L, previously normal. Creatinine increased from 0.55mg/dL to 1.23, thought to be multifactorial. Additional workup revealed uric acid of 12.5mg/dL, calcium 6.9mg/dL & phosphorous 7.4mg/dL. TLS was diagnosed & treated with intravenous fluids for dilution, rasburicase for hyperuricemia, furosemide for renal dysfunction & minimal urine output, & insulin for hyperkalemia.

DISCUSSION:

HCC is a growing health burden, & advanced tumors that are not candidates for surgical resection are commonly treated with radiofrequency ablation, TACE, TAE, & chemotherapy. Early recognition of TLS is key for treatment & involves awareness of the propensity for development of TLS in large tumor burdens, rapidly dividing tumors, multiple treatment sessions, & pretreatment renal dysfunction. Oliguria after IR treatment of HCC should raise the suspicion for possible TLS & not be easily dismissed as the common but less fatal contrast induced nephropathy.
2264 Hyperammonemia Secondary to an Acquired Urea Cycle Disorder in a Patient With Metastatic Hepatocellular Carcinoma
Authors: Sen Ahana , McNutt Markey , Singal Amit G. , Lee William M. , Rich Nicole E.
Published: October 2019

INTRODUCTION:

Urea cycle disorders are rare but critically important to consider in the differential diagnosis of hyperammonemia. Delayed treatment may result in disastrous neurologic consequences.

CASE DESCRIPTION/METHODS:

A 45-year-old woman with past history of gastric sleeve, no known liver disease, and biopsy-proven hepatocellular carcinoma (HCC) with peritoneal metastases on sorafenib presented with acute onset confusion and seizures. She was found to have severe hyperammonemia to 331 µMol/L. Labs were also notable for normal urine organic acids and low plasma citrulline (Table 1). She started on emergent dialysis as well as levetiracetam, lactulose, rifaximin, citrulline, and a low protein diet; sorafenib was discontinued. Her ammonia levels and mentation gradually improved over 3 days. She underwent genetic testing for 14 genes associated with primary urea cycle defects and further testing for another 72 genes associated with secondary hyperammonemia, all of which were negative.

DISCUSSION:

Urea cycle disorders (UCDs), whether genetic or acquired, can lead to life-threatening hyperammonemia. While classically seen in children, an increasing number of late-onset genetic UCDs have been reported. Ornithine transcarbamylase (OTC) deficiency in particular is X-linked and late-onset, and mild forms are more common than in other genetic UCDs. Individuals may also develop acquired urea cycle defects, with symptoms and lab results consistent with the genetic forms but without an identifiable mutation, as seen in this patient. Acquired UCDs have been reported secondary to tyrosine kinase inhibitors such as sorafenib, and a few cases described in untreated fibrolamellar-type HCC (Table 2). Whether these are primary acquired deficiencies in the setting of hypermetabolic states, as seen in advanced tumors, or an unmasking of an underlying genetic predisposition remains unknown. Further, this patient’s bariatric surgery could have resulted in nutritional deficiencies, revealing an occult UCD. Regardless of etiology, treatment for UCDs includes aggressive ammonia control with nitrogen scavengers, amino acid supplements, and appropriate dietary protein intake. Suspected offending agents should be stopped immediately and emergent hemodialysis initiated if hyperammonemia does not improve with conservative measures. Empiric therapy should be initiated even without confirmation of the diagnosis, as prolonged exposure to high ammonia can lead to irreversible neurological ramifications.
2265 Umbilical Hernia Rupture Complicated by Enterocutaneous Fistula in 63-Year-Old Female With Decompensated NASH Cirrhosis
Authors: Kloska Michal , Sullivan Matthew J. , Peyton Adam , Adeel Waqas
Published: October 2019

INTRODUCTION:

Spontaneous umbilical hernia rupture, known commonly as Flood syndrome, is a rare complication of severe ascites in end stage liver disease. Leakage of ascitic fluid leads to skin ulceration and necrosis which poses a threat of infection in addition to risk of evisceration or incarceration of bowels. Surgical repair is often deferred due to high mortality risk in decompensated cirrhosis which further complicates management.

CASE DESCRIPTION/METHODS:

A 63-year-old female with decompensated NASH cirrhosis, type 2 diabetes, atrial fibrillation, chronic heart failure, and morbid obesity was admitted after she developed sudden rupture of umbilical hernia with large amount of fluid discharge after defecation. She was afebrile and normotensive and was found to have ulcerated, ruptured umbilical hernia with active leakage of yellow fluid. Labs were significant for thrombocytopenia (115 × 10^3/µL), mild creatinine elevation (1.11 mg/dl), hyperbilirubinemia (2.8 mg/dl) and hypoalbuminemia (2.2 mg/dl). CT of the abdomen and pelvis revealed large abdominal wall hernia containing large and small bowels with questionable low-grade partial small bowel obstruction. General Surgery was involved but as she didn’t want to undergo any invasive treatment and due to high surgical risk, conservative treatment was recommended. Two days later she became febrile and hypotensive, accompanied by acute kidney injury (1.40 mg/dl) and worsening bilirubin (3.5 mg/dl). Due to high likelihood of SBP, diuretics were held, and ceftriaxone and albumin were initiated. Her INR increased to 6.3 and thrombocytopenia progressed (56 × 10^3/µL). Blood cultures showed no growth, but ascitic fluid cultures revealed polymicrobial flora including Bacteroides fragilis raising concerns of enterocutaneous fistula. Fortunately, she improved with conservative management and was safely discharged.

DISCUSSION:

Flood syndrome is caused by increased intraabdominal pressure during coughing, vomiting or defecating, like in our patient. Treatment is challenging as surgical repair is associated with high mortality despite known benefits of hernia repair in cirrhotic patients, however, these studies did not include decompensated cirrhosis. Patients are at increased risk of intraperitoneal infection, which was likely the case in our patient, however formation of enterocutaneous fistula is extremely rare.
2268 Rituximab for the Treatment of Refractory Autoimmune Hepatitis
Authors: Lourdusamy Vennis , Kemmer Nyingi , Albers Christopher , Syed Rashid , Malespin Miguel , Agrawal Saurabh
Published: October 2019

INTRODUCTION:

Autoimmune hepatitis can be refractory to steroids and traditional immunosuppressant agents especially in the setting of coexisting autoimmune conditions. We describe a patient with autoimmune hepatitis and Neuromyelitis Optica with elevated liver enzymes who was refractory to the traditional immunosuppressant management, but responded to therapy with Rituximab.

CASE DESCRIPTION/METHODS:

Our patient is a 57-year-old male who was diagnosed with Autoimmune hepatitis after he presented with elevated liver enzymes. Just prior to being evaluated for autoimmune hepatitis, he was diagnosed with Neuromyelitis Optica after extensive investigation when he presented with recurrent symptoms of diffuse paresthesia and muscle weakness distributed all over the body. His liver enzymes at the time of presentation were ALT (1688), AST (656), ALP (193), Bilirubin (1). Autoimmune work up was positive for NMO IgG antibody and ANA, but negative for AMA and ASMA. Liver biopsy was consistent with autoimmune hepatitis showing interface hepatitis with necroinflammatory activity and presence of plasma cells with lobular disarray. Work up for other chronic liver diseases was negative. He initially improved with pulse dose of IV steroids and was later transitioned to maintenance therapy with oral prednisone and Imuran. But, he continued to have moderately elevated liver enzymes with relapses of both autoimmune hepatitis and neurological symptoms on combination of prednisone and Imuran, and also experienced adverse effects from Imuran. He was later transitioned from Imuran to Cellcept without significant improvement. He was finally started on Rituximab infusion due to severe neurologic symptoms with complete normalization of liver enzymes and remission of Neurological manifestations.

DISCUSSION:

Rituximab is an anti CD 20 monoclonal antibody that depletes B cells. It has been shown to improve many autoimmune conditions and should be considered in refractory cases of autoimmune hepatitis especially in the setting of coexisting autoimmune conditions. Although prior case reports and case series have demonstrated the efficacy and safety of Rituximab in difficult to treat autoimmune hepatitis, it is not currently FDA approved for this purpose and payers will usually deny coverage. Prospective clinical trials of Rituximab in refractory autoimmune hepatitis should be considered for further validation.