bluuebunny/crossref_metadata_2025 huggingface dataset:
2352 Herbal Teas: More Harmful Than Helpful?
Authors: Gampa Anuhya , Shah Ayush N. , Shah Dhiren
Published: October 2019

INTRODUCTION:

The objective of this case is to become aware of the increasing number of agents with the potential to cause acute liver injury, in the modern age of combined allopathic and naturopathic medicine.

CASE DESCRIPTION/METHODS:

A 45 year old woman, recently emigrated from Honduras, presented with three weeks of epigastric pain and progressive jaundice. She denied high risk behaviors and endorsed NSAID use for headaches. Her exam was notable for marked scleral icterus and jaundice, but no stigmata of chronic liver disease. Labs were notable for AST 1893 IU/L, ALT 1454 IU/L, total bilirubin 16 mg/dL (direct bilirubin 12.4), ALP 209 IU/L, protein 6.4 g/dL, albumin 3.1 g/dL, INR 1.2. Urine drug screen, ethanol and acetaminophen levels were undetectable. A complete acute hepatitis viral panel and ceruloplasmin was negative except for a borderline anti smooth muscle antibody (1:40). Further workup was done including an abdominal ultrasound with dopplers showing normal gallbladder, no gallstones, and patent hepatic and portal vasculature, and an MRCP with small hepatic cysts and normal liver. No etiology was identified and her transaminases continued to rise as to AST 2230 IU/L and ALT 1895 IU/L. Liver biopsy showed periportal and cholestatic hepatitis with bridging necrosis consistent with drug induced liver injury. Further detailed history eventually yielded that she started an herbal remedy, arnica, around the same time she was developing jaundice. She was treated with N-acetylcysteine, and with continued abstinence of arnica, her liver enzymes normalized.

DISCUSSION:

There are innumerable supplements available to patients with purported health benefits, making it challenging to identify safety of use. arnica herbs are used as a topical agent to help with joint and muscle aches given its anti-inflammatory properties. Our patient had been using the herbs to create a concentrated tea. It is likely that the ingestion and systemic absorption led to her liver injury. This emphasizes the importance of thorough history taking with direct questioning, especially when diagnostic testing is unrevealing. Treatment is avoidance of the agent with expected improvement in liver enzymes.
2358 Zieve's Syndrome: A Case of an Unusually Profound Hemolysis in the Setting of Alcoholic Liver Disease
Authors: Ali Farhan , Steimle Cassandra , Kelts Andrew , Jeng Jennifer , Kamjula Pallavi , Reddy Dayakar , Livingston Wesley , Li Zhezheng , Kochik Michael , Golikov Edwin , Raborn Eric
Published: October 2019

INTRODUCTION:

Zieve’s Syndrome is a cause of hemolytic anemia in alcoholic liver disease, thought to arise from altered erythrocyte metabolism (pyruvate kinase instability) and hyperlipidemia (lysolecithin may trigger hemolysis). It is an under recognized syndrome, and often times leads to patients undergoing extensive clinical work ups.

CASE DESCRIPTION/METHODS:

A 73-year-old male with a history of alcohol abuse presented to the emergency room for acute jaundice associated with melenic stools, and dark urine. Physical exam was notable for dry mucous membranes, scleral icterus and conjunctival pallor, No oral ulcers, rashes or petechiae were found. Initial labs included a hemoglobin of 5.5G/DL, LDH 753IU/L, and a negative Coombs test. Based on his liver function tests, his R factor was 1.6, consistent with a cholestatic pattern of injury. Through his hospital course he had a rising alkaline phosphatase as high as 226 Units/L, and total bilirubin of 11.5 MG/DL. Triglycerides were elevated (289MG/DL) compared to prior lipid study. Abdominal ultrasound revealed an echogenic liver consistent with fatty infiltration. Lab workup was negative for HFE (hemochromatosis) mutations, autoimmune hepatitis, HIV, CMV, HCV, and EBV. Prior to coming to the hospital, the patient spent time out in the woods. Given risk of tick exposure, in the setting of hemolytic anemia, he was evaluated for tick borne illness. He was started on Doxycycline, and Atovaquone to empirically cover Lyme and Babesia, as well as Piperacillin-Tazobactam while blood and urine cultures were pending. Parasite studies and blood/urine cultures returned negative. His hemolytic anemia was refractory to 8U of packed red blood cells, and 1U of fresh frozen plasma. Eventually, the patient was diagnosed with Zieve's Syndrome.

DISCUSSION:

Zieve’s Syndrome is characterized by a triad of alcoholic steatohepatitis, jaundice, and transient hyperlipidemia. The challenging aspect of this case lies in the fact that these findings are often seen under the constellation of other diseases, leading patients to unnecessary tests and studies. Immediate management of the syndrome is comprised of supportive care, while definitive treatment is alcohol cessation.
2364 Case Studies: Hepatitis B Surface Antigen Positive or DNA Detectable After Vaccination?
Authors: Xu Binghong , Houng Mindy S. , Wang Su
Published: October 2019

INTRODUCTION:

After 1980s, hepatitis B (Hep B) vaccine reduced 80% of the incidence of acute infection. It has not been necessary to check blood work after immunization, but we happened to have two patients who had blood work after Hep B vaccination.

CASE DESCRIPTION/METHODS:

1)A 59 years old female, found to be HBsAg positive in the ED when she had GERD and presented with atypical chest pain. In her hepatitis linkage-to-care visit, we found she had her first dose of Hep B vaccine 3 days before her ED visit. We rechecked the blood work one month after her vaccine, and it showed low surface antibody titer and no antigen at all. 2)A 62 years old male was found to have positive isolated total core antibody (anti-HBc) on 4/27/2015 from his physical checkup. Other history included type II DM and insomnia. He received a Hep B booster on 5/21 and repeated blood work on 6/25 found still isolated anti-HBc. Further blood work in August, including Hep B e Ag, e Ab and viral load, verified this patient didn't have an active infection. He finished the three-dose vaccine series. Incidental Hep B panel drawn on 12/23 found detectable DNA (< 20) with negative HBsAg and positive anti-HBs.

DISCUSSION:

The phenomenon of HBsAg being positive after vaccination is reported in several studies; however, most of them are hemodialysis patients. The surface Ag could be positive for 6 weeks after vaccination. The incidence of positive isolated anti-HBc was found higher in endemic countries than in low prevalence areas. Since the second patient is from China, a hyperendemic area, the possible interpretation of his blood work is either, after many years of recovering from chronic Hep B, his anti-HBs waned down or his HBsAg fell to undetected with his chronic infection. The last possibility will be antigen mutations inducing false-negative HBsAgresults. Studies found HBV DNA detected in anti-HBc positive patients, and lower prevalence in combined anti-HBc and anti-HBs positive patients than in isolated anti-HBc positive patients. The covalently closed circular DNA (cccDNA) in the nuclei of infected hepatocytes persist in chronic infection. When the HBV-infected hepatocytes are destroyed by the immune system, the cccDNA is released. This explains how HBV DNA can be detected or not in patients with isolated positive anti-HBc and how the levels could change in the same patient. In summary, HBsAg can be positive for a short period after vaccination and when infected from HBV, cccDNA stays persistent in the hepatocytes.
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.
2398 Are Herbal and Dietary Supplements Really Safe?
Authors: Goel Siddharth , Garg Chandi , Debnath Dipanjan , Shrimanker Isha , Nookala Vinod , McLaughlin John
Published: October 2019

INTRODUCTION:

The use of herbal and dietary supplements (HDS) has been on a rise in the United States in the last couple of decades with roughly one half of the population consuming it. Artemisinin is found in the leaves of Artemisia annua and has been in use for a long time for its medicinal purposes. It is also a key ingredient in HDS and is generally considered safe and effective with no major side effects but very rarely, may lead to liver injury. Due to the HDS being unregulated, its increased use has led to an increase in adverse effects with hepatic injury being the most notable one. Here we present a case of HDS causing severe hepatic injury.

CASE DESCRIPTION/METHODS:

A 63-year-old female with a past medical history of generalized anxiety disorder and recurrent major depression, and with a recent history of over the counter supplement Microb-Clear consumption, presented with pruritus and generalized rash, of one week duration, associated with malaise, anorexia, intermittent nausea, yellow discoloration of the skin, dark urine and light stools. Her physical examination was significant for jaundice and diffuse maculopapular rash. Laboratory investigations revealed direct hyperbilirubinemia (7.3 mg/dl) with a cholestatic pattern of liver injury, with an upward trend in direct bilirubin and alkaline phosphatase noticed during the treatment course. The patient improved following discontinuation of the over the counter supplement, leading to a diagnosis of exclusion of HDS induced liver injury.

DISCUSSION:

The current regulations regarding supplements are inadequate and irrational. The general perception about safety and easy availability without the requirement of a medical prescription is one of the major causes of their misuse. They have unpredictable pharmacokinetics and pharmacodynamics as well as unknown drug interactions, increasing the probability of toxicity. As with our patient, discontinuing the supplements caused rapid symptomatic relief with improvement in liver function, which points towards a causal relationship between consumption of artemisinin-containing supplements and hepatic injury. The mechanism of the liver injury caused by artemisinin is still unknown. Further studies are required to study the relationship between HDS consumption and hepatic injury. A high index of suspicion is required to identify and minimize such adverse reactions in the future.
2403 Adefovir-Induced Fanconi Syndrome: Missed Diagnosis and Misdiagnosis
Authors: Weeber Kaitlyn , Kluesner Joseph , Whang Charles , Viswanathan Lavanya
Published: October 2019

INTRODUCTION:

Nucleotide reverse transcriptase inhibitors (NRTIs), e.g., adefovir (ADV), are commonly used to treat chronic hepatitis B, which affects approximately 240 million worldwide and 2.2 million in the US. We report a case of ADV-induced Fanconi Syndrome (AIFS) in a patient who was undiagnosed for 5 y.

CASE DESCRIPTION/METHODS:

A 75 yo Spanish female with history of osteoporosis and CHB on ADV 10 mg/d for 15 y presents to the ED for bilateral hip pain. Over the past 2 y, her hip pain was refractory to steroid injections and complicated by traumatic bilateral hip and atraumatic clavicular fractures. One year ago she was diagnosed with osteoporosis and started on teriparatide (synthetic parathyroid hormone). Exam reveals bilateral hip tenderness and she is unable to bear weight. Labs reveal renal phosphorous wasting and glucosuria, proteinuria, hyperaminoaciduria and low fibroblast growth factor 23 confirm diagnosis of FS (Table 1). Bone scan is consistent with osteomalacia (Figure 1). Teriparatide is stopped to prevent worsening of osteomalacia. Lab review shows otherwise normal phosphorous prior to starting ADV (Table 2), and patient is diagnosed with AIFS. Phosphorous supplementation is started and ADV is changed to entacavir (ETV) due to decreased renal toxicity. Patient reports complete resolution of hip pain within 1 week, over which time labs also improve (Table 2).

DISCUSSION:

heavy metal or drug toxicity. Classic features include hyperphosphaturia, hyperuricosuria, hypercalciuria, modest proteinuria and glycosuria. In addition to hyperchloremic acidosis, rickets or osteomalacia are the predominant effects of FS. Osteomalacia can exist despite normal calcium and vitamin D levels. FS and osteomalacia affect 30% and 10% of patients on long-term NRTI therapy, respectively. Nephrotoxicity is reported at dosages of 10-120 mg. Risk factors for developing AIFS include old age, low BMI and >8 y treatment with ADV, all of which apply to this patient. While this patient's CMP was checked yearly, her metabolic derangements were overlooked for 5 y. Treatment of her osteoporosis likely worsened her underlying FS. This highlights the need for awareness of this clinically significant syndrome as a more timely diagnosis may have prevented fractures. While AIFS is well reported in Endocrine literature, it is not as widely reported in GI literature, which may contribute to this knowledge gap in Gastroenterologists. GI clinicians should consider FS in patients on longstanding ADV with low phosphorous.
2411 Acute Liver Injury in a Patient Treated With Rosuvastatin: A Rare Adverse Effect
Authors: Shah Jamil M. , Lingiah Vivek A. , Pyrsopoulos Nikolaos T. , Galan Mark A.
Published: October 2019

INTRODUCTION:

As recently as 2014, Rosuvastatin was the most prescribed brand name drug in the US, with 22.3 million prescriptions filled and $5.8 billion in sales. Its use is associated with mild, asymptomatic, and usually transient, serum aminotransferase elevations, occurring in 1-3% of patients. However, levels above 3 times the ULN occur no more frequently among rosuvastatin-treated [0.2%] as placebo-treated [0.3%] recipients. Rarely, patients can present with clinically apparent acute liver injury, occurring in 1/10,000 patients.

CASE DESCRIPTION/METHODS:

A 47 y/o Peruvian man was told by his PCP that he had high cholesterol, and he was started on rosuvastatin 5 mg daily. At the time, his LFTs showed AST 17 and ALT 19. However, labs performed a month later showed AST 1282, ALT 2245, ALP 295, total bilirubin (TB) 7.1 (direct 5.5). He was advised to visit his local ER. He was admitted, the rosuvastatin was stopped, he received mainly supportive care, his LFTs continued to rise, and he underwent a liver biopsy. He was then transferred to a tertiary care hospital. His labs upon arrival showed AST 1400, ALT 2253, ALP 277, TB 9.9, and INR 1.1. He was admitted for acute liver failure due to drug-induced liver injury from rosuvastatin. Despite IV NAC therapy for five days and initiation of Ursodiol, his LFTs showed no recovery. His liver biopsy showed acute severe hepatitis with a mixed inflammatory infiltrate with many plasma cells. There was interface activity and bile duct damage. The histopathology was consistent with an immune-mediated drug reaction. He was approved to be listed for liver transplantation due to a high MELD score of 21. Before discharge, his labs improved to AST 942, ALT 1312, and ALP 167, but the TB rose to 17.2. He remained hemodynamically stable with no mental status changes during the hospital course. Afterward, repeated labs showed improvement in the LFTs, except for increasing hyperbilirubinemia. He was re-admitted and given a trial course of IV methylprednisolone after which the TB proceeded to decrease. Ultimately, his liver enzymes improved, he remained asymptomatic, and he was removed from the liver transplantation waitlist.

DISCUSSION:

The acute liver failure attributed to rosuvastatin is often accompanied by autoimmune features and may be caused by immune mechanisms. Corticosteroids have been used when recovery does not occur promptly. If used, the dose and duration of treatment should be kept to a minimum, and careful follow up after discontinuation is essential.
2423 A Case of Budd-Chiari in the Setting of Paroxysmal Nocturnal Hemoglobinuria
Authors: Parker Kasey
Published: October 2019

INTRODUCTION:

Paroxysmal nocturnal hemoglobinuria has an incidence of 1.3 case per 1 million, but the incidence is significantly increased in patients with aplastic anemia. We present a patient with a history of aplastic anemia who presents with findings concerning for Budd-Chiari.

CASE DESCRIPTION/METHODS:

A 44 year old man with a history of aplastic anemia presented with dyspnea and abdominal distention that started over the course of several days. At time of presentation, he appeared uncomfortable, but was hemodynamically stable. Due to initial concern for PE, CT chest was obtained and negative for pulmonary process. CT did note liver changes concerning for cirrhosis, however patient denied history of liver disease or risk factors associated with cirrhosis. Work up for causes of cirrhosis was initiated, and lab studies were unrevealing. SAAG score of 2.1 indicating portal hypertension as a cause of ascites. Due patient's fairly rapid onset of symptoms and otherwise unremarkable work up, CT liver protocol was performed with concern for Budd-Chiari. Imaging was suggestive of cirrhosis, and could not rule out Budd-Chiari, however no evidence of thrombosis was identified. Portal venous duplex suggested patent portal system without thrombosis. Given high level of suspicion for Budd-Chiari, hepatic venography was obtained, and significant for partial or complete thrombosis of all hepatic veins. In the setting of a patient with aplastic anemia and Budd-Chiari, work up for PNH was initiated and consistent with PNH. With diagnosis of cirrhosis due to Budd-Chiari, patient was referred to transplant clinic. With new diagnosis of PNH, he was started on Eculizumab with Hematology follow up.

DISCUSSION:

If there is concern for Budd-Chiari, diagnosis is generally with doppler ultrasonography. If US unremarkable, CT or MRI can be performed. If noninvasive diagnostic tools are nondiagnostic, hepatic venography can be performed given there is strong clinical suspicion. In PNH, thrombosis is the leading cause of death, though rare as a presenting symptom. Thromboses typically occur in unusual locations, and approximately two-thirds of thrombosis occur in intra-abdominal locations. The incidence of PNH is significantly increased in patients with aplastic anemia. In a patient with high concern for Budd-Chiari and unremarkable noninvasive testing, hepatic venography is a helpful diagnostic tool. In a patient with significant thrombosis such as this with a history of aplastic anemia, it is important to consider PNH.
2458 Autoimmune Hepatitis-Induced Hepatic Lobar Atrophy
Authors: Anucha Jeff C. , Pinto Jonathan , Culpepper-Morgan Joan
Published: October 2019

INTRODUCTION:

Lobar atrophy of the liver can be a morphologic manifestation of benign and malignant liver or biliary duct disease. Rarely has it been associated with autoimmune hepatitis (AIH).

CASE DESCRIPTION/METHODS:

A 26 year old woman presented with a 2 month history of severe RUQ abdominal pain associated with anorexia, 10 lb weight loss, pruritus, and scleral icterus. Physical exam was notable for cachexia, jaundice, abdominal distension and tender hepatomegaly. Initial labs revealed: hemoglobin 10.1 g/dl, MCV 80, platelets 107, AST 968 U/L, ALT 487 U/L, ALP 369 U/L, total bilirubin 12.0 mg/dl, DB 9.0 mg/dl, total protein 6.4 g/dL, albumin 2.1 g/dl and INR 1.65. Serological data were as follows: AMA <1:20; ANA 1:160; and anti-smooth muscle antibody 1:20. Viral markers for hepatitis A, B and C were negative. CT abdomen revealed a large area of infiltrative hypoattenuation of the right hepatic lobe. Abdominal MRI noted a diffuse edema within both hepatic lobes. N-acetylcysteine was started for impending acute liver failure and prednisone for AIH. She improved clinically. A liver biopsy done showed diffuse liver cell necrosis with lobular collapse, acute and chronic inflammation in the portal tracts, and in the interface. She was deemed stable for discharge to return to clinic but she was lost to follow up. A year later, she returned with worsening RUQ pain, further weight loss of 30 pounds. Her LFTs were within normal limits but a CT abdomen showed portal hypertension, hypoattenuation of the right lobe, and cirrhosis with left hepatic lobe atrophy. Azathioprine was started in addition to Prednisone.

DISCUSSION:

This patient's left lobar atrophy was not deemed to be congenital due to the presence of the left hepatic lobe on prior imaging studies. Hepatic neoplasms which can as well cause lobar atrophy was also ruled out with imaging. This is one of the few reported cases of hepatic lobar atrophy attributed to AIH which is a chronic disease characterized by increased autoantibodies in circulation and inflammation around the portal vein. This inflammation can result in lobar atrophy via occlusion of the portal vein resulting in impairment of liver blood flow as well as a reduction in the supply of hepatotrophic substances, particularly insulin. This may cause ischemia in certain areas of the liver and subsequently, atrophy secondary to necrosis and apoptosis. This patient also had significant cholestasis on initial presentation which could also contribute to cellular apoptosis through the Fas ligand pathway.
2466 Surprisingly Uncommon Trigger of Massive Ascites
Authors: Benrajab Karim , Ali Saad Emhmed , Frandah Wesam , Almuwaqqat Zakaria , Ismail Bahaaeldeen
Published: October 2019

INTRODUCTION:

Systemic Mastocytosis (SM) is a group of disorders characterized by excessive mast cells accumulation into single or multiple organs. It affects mainly skin, but it may involve extracutaneous sites, such as the bone, liver, spleen, GI tract, and lymph node. If it involves the liver, it can lead to noncirrhotic portal hypertension. We present a case of systemic mastocytosis with an associated hematologic neoplasm (SM-AHN) that mimics a decompensated cirrhosis with massive ascites.

CASE DESCRIPTION/METHODS:

A 73-year-old male presented with progressive abdominal distention, lower extremity swelling for 3 months. Also, he had a poor appetite, 20 pounds of weight loss, shortness of breath, diarrhea, and recurrent skin bruises. He was a non-smoker, denied any alcohol or illicit drugs. Past medical history was remarkable for chronic myelomonocytic leukemia. Vitals were normal. Physical exam was remarkable for the presence of significant ascites & legs edema. Labs revealed ALP 456 U/L, ALT 33 U/L, AST 36 U/L, bilirubin 2.8 mg/dl, albumin 3.3 g/dl, Hgb 10.5 g/dl, WBC 3.9 k/uL, and Platelet 37 k/uL. Hepatitis A, B, and C serology was negative. The abdominal US showed normal liver morphology, splenomegaly, and ascites. The echocardiogram showed LVEF of 60% and no abnormalities. Paracentesis was performed with removal of 8.5 liters. The ascitic fluid analysis was negative for infection with a low total protein, and SAAG was >1.1. Trans-jugular liver biopsy was significant for HVPG 15 mmHg, and pathology showed dense portal and sinusoidal infiltration by clusters of atypical cells with relatively abundant cytoplasm and irregular nuclei. These atypical cells stained positive for CD117, CD 25, and mast cell tryptase by immunohistochemistry, consistent with systemic mastocytosis. Serum tryptase was 230. The patient was going to follow up with hematologist for cytoreductive therapy and evaluation for hematopoietic stem cell transplantation.

DISCUSSION:

Liver involvement in a patient with systemic mastocytosis is a poor prognostic factor associated with increased mortality due to disease progression. Our patient presented with massive ascites and hepatic involvement due to systemic mastocytosis with an associated hematologic neoplasm (SM-AHN). The diagnosis is with high clinical suspicion and aided with liver biopsy. The survival rate is months to a year in case of (SM-AHN). Early diagnosis could limit unnecessary management and lessen any potential harm.