bluuebunny/crossref_metadata_2025_split huggingface dataset:
2365 A Rare Case of Severe Primary Polycystic Liver Disease
Authors: Diallo Souleymane Y. , Briones Brenda M. , Thompson Adrianne K. , Kundrotas Leon 
Published: October 2019

INTRODUCTION:
Polycystic Liver Disease (PLD) is found mainly in association with autosomal dominant polycystic kidney disease (ADPKD). Isolated PLD is rare with a prevalence of less than 0.01%. Massive hepatomegaly causing symptoms associated with PLD is even rarer. We present a patient with severe PLD requiring surgical intervention.

CASE DESCRIPTION/METHODS:
A 55-year-old man had an incidental finding of multiple liver cysts on an ultrasound done for investigation of a superficial abdominal wall mass. He reported left upper quadrant pressure. Two siblings had hepatic cysts but no family history of kidney cysts. He had been in South Korea and central America in the past. Physical exam showed hepatomegaly, splenomegaly and a bulge in the right upper quadrant (Figure 1). Hepatic synthetic, excretory function and aminotransferases were normal. CT abdomen revealed too numerous to count hepatic cysts, some measuring up to 11cm in diameter, renal cysts and a 1.4 cm splenic cyst. MRI showed numerous intrahepatic cysts, measuring up to 14 cm, a small number of bilateral renal cysts and splenic cyst (Figure 2). AFP, Echinococcus IgG and chronic hepatitis panel were negative. He did not met criteria for ADPKD as the renal his cysts were simple and age-related according to nephrology. He reported worsening symptoms abdominal pressure and postprandial pain. He underwent laparoscopic fenestration of liver cysts and specimens were consistent with simple hepatic cysts.

DISCUSSION:
PLD is a rare condition that is often asymptomatic. At least one-third of PLD may also have a few kidney cysts that do not meet criteria for ADPKD. Gigot’s classification is based on imaging findings and can used to help determine management choice based on total liver involvement, cyst size and number (Table 1). Asymptomatic PLD patients do not require treatment. Symptomatic patients may be treated medically with somatostatin analogues or surgically including fenestration +/- hepatic resection or liver transplantation. Severe PLD causing bulging on the abdomen is rare. Our patient benefited from fenestration.
2370 Acute Liver Failure After Foraging for Wild Mushrooms: A Family Affair?
Authors: Flocco Gianina , Mastroianni Barbara , Lindenmeyer Christina C. 
Published: October 2019

INTRODUCTION:
Ingestion of cytotoxic mushrooms is associated with a high mortality rate due to acute liver failure (ALF). Amanita phalloides (AP) and related species account for over 90% of fatal cases, and can be easily mistaken with edible species by inexperienced foragers. There is currently no gold standard of therapy, and often liver transplantation (LT) is required. We present 5 cases of mushroom ingestion and associated ALF who were treated at a quaternary LT center in Northeast Ohio.

CASE DESCRIPTION/METHODS:
Five members of a family were referred to our Medical Intensive Liver Unit in September 2018 after ingesting a shared meal prepared with wild mushrooms from a neighbor’s garden. Median age was 43.6 years (32-61 years) and all patients were female. All patients presented to a local hospital with nausea, vomiting, diarrhea and abdominal pain approximately 6 hours after ingestion and were transferred to our center 1 day later. Mean peak laboratory values were as follows: alanine aminotransferase 6530 U/L (range 3750-10506 U/L), aspartate aminotransferase 10974 U/L (3780-19737 U/L), bilirubin 7.2 mg/dL (4.6-11.3 mg/dL) and international normalized ratio 3.4 (1.8-7.1). Mushroom specimens were obtained for inspection and visually identified as AP. The 5 patients were treated with aggressive supportive care, including N-Acetylcysteine, octreotide and penicillin G infusions, followed by experimental silibinin obtained under emergent institutional review board approval. All patients progressed to ALF and underwent emergent LT evaluation; all 5 were listed for LT (3 as status 1A). Three patients improved with medical therapy, while 2 underwent successful LT. Pathology of the explanted livers revealed extensive necrosis. The local departments of health were notified and educational programs implemented throughout Northeast Ohio.

DISCUSSION:
We present 3 cases of successful medical therapy associated with transplant-free survival, and 2 cases of LT for severe ALF related to mushroom ingestion. In some Southeast Asia cultures, foraging for wild mushrooms is common practice. Mortality has been reduced in recent decades due to improved supportive and intensive care, as well as the introduction of experimental therapies. A high index of suspicion for diagnosis is important, as timing of initiation of therapy is paramount. Public education for populations in which foraging is common will be important to promote proper identification of wild toxic mushrooms.
2373 Failure of Occam’s Razor: A Rare Trifecta of Causes for Elevated Liver Enzymes
Authors: Khiew Yii Chun , Weinberg Ethan 
Published: October 2019

INTRODUCTION:
Unexplained abnormal liver function tests in a patient are a common reason for referral to a hepatologist. Patients may often have coexisting liver diseases, and require a liver biopsy for definitive diagnosis to guide treatment. We present a patient with celiac disease, fatty liver disease, and a delayed diagnosis of autoimmune hepatitis, highlighting the need to recognize anchoring bias.

CASE DESCRIPTION/METHODS:
A 55 year-old male, with a history of eosinophilic esophagitis and obesity, was referred a hepatologist for elevated liver enzymes. The patient was asymptomatic and consumed two units of alcohol per week. His BMI was 30, and he did not have stigmata of chronic liver disease and hepatomegaly. Labs showed total bilirubin 2.0mg/dL, ALP 59U/L, AST 132U/L, ALT 392U/L, albumin 4.3g/dL, and INR 1.3. Complete serologic evaluation for secondary causes of liver disease including viral hepatitis and autoimmune screen, alpha 1 antitrypsin, iron studies and ceruloplasmin were negative. Tissue transglutaminase (tTG) antibody was greater than 150U/mL and EGD revealed Modified-Marsh 3 pathologic changes suggestive of celiac disease. Liver ultrasound showed steatosis without cirrhosis and transient hepatic elastography suggested mild fibrosis (6.7kPa). Transaminases and tissue tTG antibody increased to more than 10 times the upper limit of normal despite adherence to a gluten-free diet. Liver biopsy confirmed evidence of autoimmune hepatitis with mild fibrosis and steatosis. The patient was commenced on immunosuppression with significant improvement in liver function tests.

DISCUSSION:
Liver involvement in celiac disease accounts for 9% of patients with unexplained, mildly elevated transaminases and has the potential to impact the clinical course of established liver disease. Primary biliary cholangitis and autoimmune hepatitis are also associated with celiac disease. Due to the high prevalence of elevated liver enzymes in celiac disease, it is reasonable to delay liver biopsy and monitor for improvement of liver enzymes with exclusion of dietary gluten. A liver biopsy to rule out coexisting liver diseases is warranted in patients with celiac disease with severely elevated transaminases or failure to respond to a gluten free diet. Clinicians must maintain a high index of suspicion for concurrent autoimmune liver disease especially in patients with a history of other autoimmune diseases. 1) Rubio-Tapia, A., Murray, JA. Liver involvement in celiac disease. Minerva Medica, 2008 Dec; 99(6): 595–604.
2375 Unusual Presentation of Spontaneous Bacterial Empyema in Cirrhosis
Authors: Agrawal Neha , Liaquat Hammad , Mansour Natalie , Shaker Mina 
Published: October 2019

INTRODUCTION:
Spontaneous bacterial empyema (SBEM) is defined as spontaneous infection of the pleural fluid in the absence of underlying pneumonia. Diagnostic criteria include i) serum/pleural fluid albumin gradient (SPAG) >1.1 g/dL, ii) polymorphonuclear (PMN) cells >500/mm3 or positive fluid culture with PMN cell count >250 cells/mm3 and iii) absence of pneumonia or malignancy. SBEM is an under-diagnosed entity, prevalence being 5-12% overall in cirrhotics and 16% in hepatic hydrothorax (HH). HH is usually right-sided (65-85%), however it may be left sided or bilateral. Most patients with SBEM present with dyspnea or pleuritic chest pain. We present a case of SBEM with an unusual presenting complaint.

CASE DESCRIPTION/METHODS:
59-year-old male with decompensated alcoholic cirrhosis presented with abdominal pain. Physical exam significant for tachycardia, fever (102.3 F), jaundice, diminished breath sounds over the right lung base and diffuse abdominal tenderness. Lab data notable for mild leukocytosis and deranged liver functions (Total bilirubin 5.8 mg/dl, Albumin 1.8 g/dl, INR 2.3). A chest radiograph showed moderate right sided pleural effusion without pneumonia or lung mass, confirmed on CT. Abdominal CT showed absence of ascites. Thoracentesis yielded 1.5 L of clear yellow fluid, with 7000 WBC (90% PMNs) and albumin was 0.6 g/dl. SPAG was greater than 1.1 g/dl. Cytology and cultures were negative. This confirmed the diagnosis of SBEM. Thoracentesis lead to resolution of abdominal pain and patient was discharged to complete a 7 day course of Ciprofloxacin.

DISCUSSION:
Pathogenesis of SBEM is unclear, proposed mechanisms being direct bacterial spread from the peritoneal cavity or transient bacteremia in the absence of ascites. Risk factors include low pleural fluid protein, low serum albumin levels, presence of SBP and high Child-Pugh scores. Factors which portend poor prognosis are high MELD-Na, early ICU admission and antibiotic failure. Treatment includes thoracentesis and third generation cephalosporins. Chest tube insertion is not recommended. Role of long term antibiotic prophylaxis is unclear. In conclusion, SBEM is associated with high mortality and often under diagnosed due to lack of routine chest imaging in patients with ascites or suspicion for SBP. Majority of SBEM cases are associated with ascites (88%) and SBP (47%). Our case is unique because patient presented with abdominal pain in absence of ascites and symptom resolution post thoracentesis.
2377 A Rare Case of Klebsiella Pneumoniae Liver Abscess With Subsequent Invasive Disease
Authors: McCarty Daniel , Castineira Janyll , Muso Ergen , Beljic Steven 
Published: October 2019

INTRODUCTION:
Klebsiella pneumoniae is a gram-negative bacterium, native to the common flora of the human digestive tract and skin. This organism can cause opportunistic infection in at risk populations, such as those who are immunocompromised. Less commonly, monomicrobial abscess formation can develop without underlying hepatobiliary disease.

CASE DESCRIPTION/METHODS:
A 76-year-old Polish male presented for the evaluation of worsening mentation, generalized weakness and anorexia that began two months prior to presentation. The patient was admitted when Chest X-ray revealed a near complete opacification of the right hemithorax. CT imaging of both chest and abdomen/pelvis were obtained showing a large, loculated pleural effusion of the right lung as well as findings consistent with a probable liver abscess. Intra-operatively, an associated diaphragmatic fistula was repaired, two right sided chest tubes were placed. Cultures of both pleural and hepatic fluid collections yielded Klebsiella pneumoniae. Blood and urine cultures remained negative. CT Brain was obtained and negative for acute findings. ENT was also consulted to rule out endophthalmitis. The patient continued to clinically improve with supportive care and IV antibiotics. Approximately one week after surgery, chest tubes were removed and the patient was discharged home to complete IV antibiotic therapy.

DISCUSSION:
This case highlights a rare formation of liver abscess with hepatothoracic fistulation in an immunocompetent patient, of non-Asian descent, without underlying hepatobiliary disease. Pyogenic liver abscess is usually associated with complication from intra-abdominal or hepatobiliary disease. Infection from monoisolate Klebsiella pneumoniae has been increasingly prevalent in the Taiwanese population and extensively studied among certain Asian demographics. It has been postulated that some groups may be predisposed to intestinal colonization of more invasive Klebsiella species, suggesting a possible genetic etiology. Metastatic infection secondary to particularly virulent strains, K1/K2 capsular serotypes, can be potentially life-threatening as it is associated with CNS infection and endophthalmitis. Thus far, regional distribution of KLA shows a preference for South Africa and Taiwan. With the rising epidemic of obesity and insulin resistance in the United States, we encourage clinicians with similar presentations to consider possible KLA infection. Early identification and eradication of the infection is key in preventing fatal outcomes.
2385 Multifocal Hepatocellular Carcinoma Masquerading as Cirrhosis
Authors: Humphrey Kyle , Henderson Phillip 
Published: October 2019

INTRODUCTION:
Hepatocellular carcinoma (HCC) is a primary tumor of the liver that is usually seen in patients with chronic liver disease, such as cirrhosis. The only curative therapy for HCC is liver resection or liver transplantation. For patients with HCC that undergo liver transplantation, immunosuppressive therapy is necessary but associated with a risk for tumor recurrence given the intrinsic hepatoproliferative effects. Here, we present a case of a patient who underwent simultaneous liver and kidney transplantation and was found to have multifocal hepatocellular carcinoma that mimicked cirrhosis.

CASE DESCRIPTION/METHODS:
A 58-year-old male with a past medical history of hepatitis B and C associated liver cirrhosis and CKD-IV presents to the transplant clinic. The patient completed a course of ledipasvir/sofosbuvir therapy for his hepatitis C with sustained virologic response. He also has chronic hepatitis B which is treated with tenofovir alafenamide. His presumed cirrhosis was complicated by recurrent ascites requiring scheduled weekly paracenteses. Ascites fluid studies were bloody but negative for malignancy on path review. Multiple CT and MRI of the abdomen without contrast due to eGFR showed signs of portal hypertension but no liver lesions. A blood type and HLA matched donor was found, and the patient underwent successful simultaneous liver and kidney transplant. He was subsequently started on standard 3 drug immunosuppressive therapy after induction. Upon path review of the explant, more than 150 small hepatocellular carcinomas were discovered. On review of his liver no fibrosis was found and these innumerable HCC’s created the illusion of cirrhosis on imaging. Secondary to this discovery, tacrolimus was decreased and everolimus was started in hopes to decrease risk for recurrent HCC. The patient is doing well without recurrence 18 months post-transplant.

DISCUSSION:
In patients with HCC who undergo liver transplantation, it is important to consider alternative immunosuppresives. Studies have shown in animal models the potential positive effects of mTOR inhibitors in decreasing recurrence; however human data is controversial. There is some thought that the benefit of the mTOR inhibitor is to decrease the dose of calcineurin agents with known hepatoproliferative properties.
2386 Shock Liver: A Complication of Gastroduodenal Artery Embolization
Authors: Husain Faiza , Dwairy Ahmad , Che John , El Zein Mohamad , Nawras Ali , Goyal Tanvi , Kobeissy Abdallah 
Published: October 2019

INTRODUCTION:
PUD is the most common cause of acute upper GI bleeding. Duodenal ulcers are 4x more likely to bleed than gastric ulcers. Posterior duodenal ulcers, are more likely to erode into the gastroduodenal artery (GDA) resulting in massive bleeding requiring intervention. Liver failure is a known complication of gastroduodenal artery embolization due to inadvertent embolization of the main hepatic artery [1,2].

CASE DESCRIPTION/METHODS:
A 71 year old lady presented with a hemoglobin of 5.7 g/dL with a baseline of 10.7 g/dL. She was resuscitated with pRBCs and fluids to a hemoglobin of 8.7 g/dL. EGD showed an active 3 cm bleeding ulcer at the duodenal bulb with a visible vessel and adherent clot. Hemostasis was achieved with epinephrine, two hemoclips and gold probe cautery. On POD# 5, she had two melenic stools and an acute drop in hemoglobin reaching a nadir of 6.4 g/dL which prompted a repeat EGD. An ulcerated area on the posterior wall of the bulb was bleeding, hemostasis with epinephrine and three hemoclip deployment failed. Subsequently, she underwent gastroduodenal artery coiling. During the embolization, the coil was partially protruding into the hepatic artery. Coil repositioning failed. POD# 1, AST and ALT trended up to 2814 IU/L and 1375 IU/L from a baseline of 14 IU/L and 10 IU/L on admission, respectively. INR trended up from 1.23 to 1.8, she developed encephalopathy, was diagnosed with shock liver and transferred to outside liver transplant center.

DISCUSSION:
Technical success rates of transcatheter arterial embolization are extremely high (>90%) while clinical success rates vary from 60%-90% [3]. This difference may be attributed to multiorgan failure, coagulopathy, and bleeding from trauma or invasive procedures [4]. A lack of understanding of the factors that influence clinical outcome also plays a role. Transcatheter therapeutic embolization has a very high rate of success and should be considered as an alternative to surgery in high-risk populations. [1,2]. Endoscopic embolization usually has a low rate of complications of rebleeding, infarction, and rarely coil migration [3,5,]. Coil migration has been described after embolization of the GDA, but infrequently been reported to be fatal. Our patient had movement of the sheath used to protect the hepatic artery and the catheter resulting in partial migration of the coils into the hepatic artery which led to shock liver. To our knowledge, few cases of coil migration inducing hepatic failure have been reported in the literature.
2395 Treating Acute Hepatitis C Virus Infections: Is It Cost Effective?
Authors: Koppala Jahnavi , Buaisha Haitam M. , Rangray Rajani , Mukherjee Sandeep 
Published: October 2019

INTRODUCTION:
One- fourth of acute HCV patients spontaneously clear the virus. Direct antiviral agents(DAA) decreased the duration of treatment, cost-effective, and reduce the transmission risk. Of 2 cases we present here, one achieved SVR with DAA and another cleared the virus spontaneously.

CASE DESCRIPTION/METHODS:
1) 51 y/o F presented with RUQ pain & jaundice. Labs: AST 1633, ALT 2214, ALP 345, T.bili 6.4. Imaging: no biliary obstruction. Acute hepatitis panel was positive for HCV antibodies. These were negative 3 months ago. Genotype was 1a and viral load was 1,000,000. Biopsy showed acute hepatitis with lobular and portal inflammation. She was treated with ledipasvir 90 mg/sofosbuvir 400 mg daily for 12 weeks and SVR was achieved. 2) 29 y/o M presented with RUQ and jaundice. He is an IVDU. Labs: AST 1338, ALT 2921 T bili at 14.8 and ALP 244. Imaging: negative for biliary obstruction. He was tested positive for HCV RNA titers . His prior HCV Ab was negative 2 weeks ago.Genotype was 1a and viral titers were1,894,692. He was given Oxcarbazapine recently. Biopsy showed lobular inflammation with hepatocyte necrosis, eosinophils, granulomas and portal inflammation consistent with acute hepatitis. Though the presence of the eosinophils and granulomas suggest Oxcarbazepine induced liver injury, the marked hepatocyte injury coincides with acute HCV. He cleared the HCV spontaneously after 4 months.

DISCUSSION:
Liver tests and HCV RNA monitoring for 6 months is recommended for acute HCV treatment. when the quality adjusted life years (QALY) and costs were measured in a microsimulation model, treatment of acute HCV,when compared to chronic HCV in non- high-risk behaviors, QALY increased by 0.02. The increased cost was 483 dollars with a resulted incremental cost effectiveness ratio (ICER) of $19,9991 per QALY, demonstrating that this treatment is cost effective. In high risk patients the QALY increased by 0.03 and decreased costs up to $3,655, substantiating this as a cost saving outcome. Although more studies are needed to demonstrate the effectiveness of DAAS,this particular study seemed to be promising.
2396 Idiopathic Granulomatous Hepatitis: A Case of Episodic Fever of Unknown Origin
Authors: Anugwom Chimaobi M. , Thompson Julie 
Published: October 2019

INTRODUCTION:
We present a case of a patient with episodes of fever and weight loss, due to a second episode of idiopathic granulomatous hepatitis (IGH), 12 years after the first episode.

CASE DESCRIPTION/METHODS:
A 63-year-old female with a history of hyperlipidemia and reflux disease was referred to the hepatology clinic with an elevated alkaline phosphatase (ALP) of 326. The rest of her hepatic panel was normal. She reported a 3-month history of intermittent fevers up to 103 degrees Fahrenheit and a 35-pound weight loss over 5 months. She had a similar episode over 10 years ago, with an extensive negative work-up. A liver biopsy was obtained at that time and it showed extensive necrotizing granulomas with portal and lobular involvement. She responded to a short course of prednisone with resolution of symptoms and normalization of ALP. With her present episode, viral serology was obtained and found to be negative. A repeat liver biopsy was done, with evaluation for fungal and mycobacterium components, as well as iron overload. It showed extensive sclerosed and calcified granulomas without any evidence of active alcoholic hepatitis. A diagnosis of relapsing fever of unknown origin due to idiopathic granulomatous hepatitis (IGH) was made and she was started on prednisone 40 mg daily with resolution of her symptoms.

DISCUSSION:
Hepatic granulomas are found in up to 10% of liver biopsies and may be incidental. The etiology of hepatic granulomas is diverse, and search for cause should include an exhaustive infectious, autoimmune and rheumatology work up, as well as careful medication review. Presentation of IGH is variable and most symptoms reported by patients include subjective fevers, chills and night sweats, weight loss, hepatosplenomegaly, or elevation of alkaline phosphatase (ALP) or gamma-glutamyl transferase (GGT). IGH has been noted to occur mostly in middle-aged women with primarily cholestatic liver enzymes and has a favorable prognosis. Corticosteroids are the main-stay of treatment of IGH, and methotrexate has also been shown to be effective in those with significant relapse, in steroid-refractory cases or in patients who do not tolerate steroids.