bluuebunny/crossref_metadata_2025_split huggingface dataset:
2420 A Rare Cause of Portal Vein Thrombosis
Authors: Loganathan Danekka , Shah Gunjan 
Published: October 2019

INTRODUCTION:
Hormone therapy is the mainstay adjuvant therapy for patients with estrogen receptor positive early stage breast cancer. Anastrozole is generally considered to have lower risk of venous thromboembolism compared to tamoxifen, albeit not negligible.

CASE DESCRIPTION/METHODS:
81-year-old female with history of breast cancer on anastrozole presented to the hospital with high-grade fever. Physical exam was significant for RUQ tenderness. Her labs were significant for elevated transaminases, ALP and direct hyperbilirubinemia without leukocytosis. US abdomen showed enlarged gallbladder with stones. MRI was positive for portal vein thrombosis in the main portal vein and extending into right portal vein, with possible central anterior segment of right liver lobe infarct. She had no history of liver disease or venous thromboembolism. There is no clinical or radiological evidence of recurrence of malignancy. Patient was started on anticoagulation and was discharged on apixaban. APLA testing is pending and hypercoagulability workup was otherwise negative. It was concluded that the portal vein thrombosis was secondary to anastrozole due to lack of alternative etiologies.

DISCUSSION:
To our knowledge, our case of hepatic portal vein thrombosis suspected to be secondary to anastrozole therapy is the first to be reported in the medical literature. A case report suggesting a causal association between pulmonary embolism and anastrozole in a patient with no prior VTE and negative hypercoagulable workup was published in 2006 by Lycette et al. It is particularly important to consider the causative role of Anastrozole in patients with otherwise unexplained venous thromboembolism in unusual vascular beds as seen in our patient.
2429 Acute Severe Cholestatic Hepatitis Secondary to Emerge Weight Loss Supplement
Authors: Mukherjee Sandeep , Vellanki Meghana 
Published: October 2019

INTRODUCTION:
Herbal and dietary supplements (HDS) are an important cause of liver injury. EmergeR weight loss supplement is advertised as a product which burns fat and stimulates weight loss. We report a case of acute severe cholestatic hepatitis after consumption of EmergeR in a 32 year-old man. This case highlights a previously unreported and important association between this supplement and hepatotoxicity and the importance of obtaining a through medication history including HDS.

CASE DESCRIPTION/METHODS:
A 32-year-old male presented to hepatology clinic for evaluation of jaundice and elevated liver tests. He was well until 1 week ago when he developed flu-like symptoms, fatigue, jaundice, pruritus, dark urine and pale stools. He denied other symptoms, recent travel, reported rare alcohol use and did not take any other medications. However, he started a new weight loss supplement, EmergeR 3 weeks ago and had normal liver tests prior (Table 1). Exam was remarkable for a BMI of 35 and scleral icterus. Liver tests were elevated with AST 987 IU/L, ALT 2305 IU/L, total bilirubin 7.2 mg/dl and INR 1.1 (Table 1). Testing for other viral causes was negative. Abdominal ultrasound showed hepatic steatosis. The patient was advised to stop EmergeR immediately and undergo liver tests and INR weekly. 4 weeks later (7 weeks after starting EmergeR), patient was asymptomatic with resolution of prior symptoms.

DISCUSSION:
Despite the widespread use and potential severe side effects associated with weight loss supplements, HDS do not require FDA approval prior to marketing.3 This emphasizes the need for physicians to report potential side effects associated with weight loss products as continued use without awareness or proper monitoring can lead to severe liver injury. To our knowledge, this is the first reported case of acute cholestatic hepatitis from EmergeR use. The temporal relationship of acute liver injury with the use of Emerge, improvement after withdrawal and absence of other possible causes strongly suggested that liver injury was secondary to this supplement. As EmergeR contains a variety of ingredients which may also be contaminated by an unknown toxic botanical or synthetic chemical, it remains unclear which component of EmergeR caused liver injury.4 As patients may exhibit subtle symptoms such as fatigue and nausea at the time of presentation, a high index of suspicion is required for diagnosis of HDS induced liver injury along with a through HDS use history.
2433 Sub-Acute Liver Failure as the Initial Manifestation of Metastatic Small Cell Lung Cancer
Authors: Agarwal Amit K. , Yoo Joseph , Halegoua-DeMarzio Dina 
Published: October 2019

INTRODUCTION:
The liver is a common site of metastasis in small cell lung carcinoma (SCLC), with 20% of cases metastasizing to the liver. However, most liver metastases are discovered at time of autopsy and not at initial diagnosis. Given the scarcity of literature regarding sub-acute liver failure as the presenting sign of metastatic SCLC, this case explores a patient who presented with sub-acute liver failure secondary to diffuse, infiltrative metastatic SCLC.

CASE DESCRIPTION/METHODS:
A 54 year old Caucasian male with no significant past medical history presented with jaundice and ascites. He denied any history of alcohol consumption, any personal or family history of liver disease, and consumption of any supplements or over the counter medications. Physical examination was notable for marked abdominal distension with shifting dullness, grade I hepatic encephalopathy, and mild asterixis. He did not have findings of chronic liver disease. Labs were significant for a creatinine of 1.1, sodium of 128, total bilirubin of 8.9, ALT 85, AST 227, alkaline phosphatase 153, and an INR of 3.03, with a MELD-Na score of 32. Laboratory workup was negative for causes of liver failure, including infectious and autoimmune etiologies. A triple phase contrast enhanced CT abdomen showed multiple hypodense lesions too small to characterize, without any large enhancing liver mass seen. Given the patient's high MELD-Na score and an initial concern for liver failure, the patient was transferred to our institution for liver transplant. To determine the etiology of his suspected cirrhosis after negative serologic workup, a transjugular liver biopsy was obtained. Pathology results were not consistent with cirrhosis, and instead revealed diffuse, infiltrative metastatic small cell carcinoma from a suspected primary lung cancer in the setting of a 1.8 cm lung lesion later seen on CT chest. He was subsequently removed from liver transplant consideration. The patient unfortunately expired due to liver failure.

DISCUSSION:
This is a case of a patient who presented with sub-acute liver failure due to metastatic small cell carcinoma. Liver failure is a rare presentation of metastatic disease but has been reported in malignancies such as metastatic breast cancer. While SCLC metastasis to the liver is common, it has only rarely been described to cause sub-acute liver failure.
2435 An Uncommon Case of Hepatic Abscess From Fish Bone Ingestion: A Case Report With Successful Conservative Management
Authors: Maruvada Smita , Vuppala Anusha , Hafiz Nazar , Sabillo Sheryl 
Published: October 2019

INTRODUCTION:
Accidental ingestion of foreign bodies is infrequently seen in clinical practice and can be fatal. Liver abscess from enterohepatic migration of an ingested foreign body is extremely rare and is usually associated with perforation, peritonitis from rupture of the hepatic abscess and sepsis. Lack of specific symptoms, unacknowledged ingestion by the patient and a low clinical suspicion, complicate our arrival at an accurate diagnosis. Here, we report a case of hepatic abscess secondary to fish bone ingestion, without peritonitis, that was managed conservatively and without extraction of the foreign body.

CASE DESCRIPTION/METHODS:
Our patient is a 64 y/o lady who came in with sharp, right sided abdominal pain, fever and anorexia for a week. She was febrile upon arrival with leukocytosis and mild transaminitis with total bilirubin 2.7. Other monitored vitals were stable. CT abdomen revealed a 6.9 cm abscess at the junction of the right and left hepatic lobes which was promptly drained percutaneously by IR. Despite clinical improvement with drainage and antibiotics, persistent leukocytosis was appreciated. A repeat CT showed a small residual liver abscess and a sharp, linear radiopaque foreign body adjacent to the pylorus with perigastric stranding. Upon further questioning, patient recollected having had fish prior to onset of symptoms. Endoscopy was unsuccessful in identifying the exit tract from the upper GI tract. Reimaging confirmed migration of the fishbone towards the falciform fissure and gastrohepatic ligament. The position of the fish bone posed a challenge for percutaneous extraction and Surgery recommended against exploratory laparotomy as patient had marked clinical improvement. Follow up imaging a month later showed the fish bone to be in stable position, in an asymptomatic patient.

DISCUSSION:
80% – 90% of ingested foreign bodies usually pass through the gut without any intervention. Foreign body ingestion is generally an unconscious event, that the patient rarely recalls, which complicates the preoperative diagnosis in most cases. In the case of liver abscess unresponsive to drainage and antibiotic therapy, this possibility needs to be entertained, despite its low incidence. Besides antibiotics and abscess drainage for size >5 cm, surgical or endoscopic retrieval of the foreign body has always been the conventional treatment. However, medical management should be considered for patients, like ours, without signs of severe sequelae.
2436 Lysosomal Acid Lipase Deficiency: A Missed Diagnosis Spanning All Ages
Authors: Filo Jean , Ascher Bartlett Johanna M. , Mittal Naveen , Rodas Fabian , Rosas Christy , Alkhouri Naim 
Published: October 2019

INTRODUCTION:
Lysosomal acid lipase deficiency (LAL-D) is a rare autosomal recessive disease caused by mutations in the lysosomal acid lipase (LIPA) gene and characterized by cholesteryl ester accumulation in the liver and other body parts. Depending on the lipase enzyme residual activity level, severity of LAL-D can be low enough and patients may present with nonspecific symptoms; LAL-D can then be easily confused with more common causes of hepatic steatosis such as nonalcoholic fatty liver disease leading to underdiagnosis or even misdiagnosis well into adulthood. Here we present two cases of patients whose diagnoses of LAL-D were delayed for several years due to initial misdiagnosis.

CASE DESCRIPTION/METHODS:
A 59-year-old male, initially presented in childhood with hepatomegaly and elevated liver enzymes and was diagnosed with type 3 glycogen storage disease due to findings of liver biopsy done in 1975. He presented to our practice with worsening fatigue, muscle wasting and abdominal distension despite being on cornstarch therapy. His labs showed ALT of 75 U/L (0-44), AST of 73 U/L (0-40), and LDL cholesterol of 302 mg/dL (<110). LAL activity was low at 4 pmol/h/punch and liver biopsy showed diffuse microvesicular steatosis and foamy macrophages with stage 2 fibrosis confirming the diagnosis of LAL-D. His diagnosis was missed for at least 44 years from his initial liver biopsy. The second patient, a 14-year-old male, was not diagnosed for 8 years after repeatedly presenting to the emergency room with abdominal pain, diarrhea, and mild elevation in ALT for years. He required a cholecystectomy at the age of 13 years and the surgeon noted an “orange” liver. A biopsy showed diffuse microvesicular steatosis with stage 3 fibrosis suggestive of lipid storage disorder. His labs at presentation to our clinic were: ALT of 225, AST of 111, LDL cholesterol at 192. Genetic testing confirmed the diagnosis of LAL-D (E8SJM homozygous) and he was started on sebelipase alfa enzyme replacement with normalization of ALT and LDL cholesterol.

DISCUSSION:
Gastroenterologists should suspect the diagnosis of LAL-D in any patient presenting with hepatomegaly, fatty infiltration of the liver and elevated LDL. Prompt diagnosis of LAL-D could have helped decrease morbidity and improve these patients' quality of life by initiating sebelipase alfa enzyme replacement therapy to reduce the known long-term complications of advanced liver fibrosis such as cirrhosis and liver failure, and the many effects of progressive atherosclerosis.
2443 Dealing With DILI: When the Diagnosis Is Not Always Clear Cut
Authors: Hashmi Irma , Hsu Andrew , Shor Julia , Devuni Deepika 
Published: October 2019

INTRODUCTION:
Drug induced liver injury (DILI) is a rare, mostly reversible cause of liver failure (ALF); however, in rare cases DILI can unmask or trigger underlying chronic liver disease. We present a case of DILI with two novel findings: one, the patient consumed tea extract not yet cited on the LiverTox registry, and two, the patient's DILI likely triggered autoimmune hepatitis (AIH), an occurrence noted only a few times in the literature.

CASE DESCRIPTION/METHODS:
Patient is a 43-year-old female with no medical history who presented with acute onset jaundice and dark urine. She endorsed a “tea cleanse” which included dandelion roots, milk thistle and barks one month prior to admission. Her jaundice persisted despite cessation of the herbal tea intake. Liver function tests (LFTs) showed AST 2553, ALT 3022, alkaline phosphatase 135, total bilirubin 23.8, direct bilirubin 15.5, INR 1.8 and MELD 25. MRCP was consistent with acute hepatitis. ALF workup was negative except for elevated IgG1 and IgG4. Liver biopsy revealed panlobular mixed inflammatory cell infiltrate and necrosis but absent fibrosis, concerning for DILI, viral infection or AIH. Patient was managed supportively for DILI with downtrending LFTs. However, her LFTs worsened with total bilirubin 20 and INR 2.1. She developed ascites and lower extremity edema. The patient received corticosteroids for AIH despite negative ANA, anti-smooth muscle and anti-LKM antibodies with marked improvement in synthetic liver function.

DISCUSSION:
Although acetaminophen is the most common offending drug associated with DILI (40-50% of ALF cases annually), herbal or dietary supplements are not an uncommon cause, seen in 11-12% of cases per year. Accurate diagnosis of DILI can be made with clinical history, exclusion of other etiologies of ALF on laboratory evaluation, and with liver biopsy. The database LiverTox documents etiologies of DILI. “Be Well Teas,” consumed by our patient, yields no results but should be included to prevent future DILI cases. Further, despite biopsy consistent with DILI, rebound of total bilirubin and INR in our patient demanded further investigation. Likely the patient had pre-existent AIH triggered by DILI given her improvement with immunosuppression, cited previously in only in a few case reports. Concurrent, underlying AIH should be considered with relapse of liver dysfunction despite cessation of the inciting drug.
2445 All Liver, No Lungs: An Unusual Presentation of Sarcoidosis
Authors: Gurm Hashroop , Jamorabo Daniel S. , Renelus Benjamin D. , Mohanty Smruti R. 
Published: October 2019

INTRODUCTION:
Sarcoidosis primarily isolated to the liver—hepatic sarcoidosis (HS)—is rare and typically presents with non-specific gastrointestinal (GI) symptoms, abnormal liver enzyme levels, and radiological findings. We present a patient whom we determined to have HS.

CASE DESCRIPTION/METHODS:
A 33-year-old woman without significant past medical history presented with 3 months of worsening right upper quadrant pain and 35-40 lb weight loss. She had no other specific complaints. Her exam was notable for epigastric and RUQ tenderness. Her liver was palpable 4 cm below the costal margin. Lab results included Hgb 12.5 g/dL with MCV 79, AST 60, ALT 69, AP 629, and GGT 773. CT scan showed diffuse cyst-like lesions throughout the liver and spleen with peri-portal lymphadenopathy (Figure 1). The chest X-ray was unrevealing. Abdominal ultrasound showed hepatomegaly to 16.1 cm, splenomegaly to 13.1 cm, and periportal and peripancreatic lymphadenopathy. Her viral hepatitis workup, Chromogranin A, CEA, CA-19-9, CA-125, and AFP levels were unremarkable. Autoimmune hepatitis workup was negative. Her angiotensin converting enzyme (ACE) was elevated to 261 IU/L. EGD and colonoscopy were unremarkable, but we took random biopsies. Liver biopsy showed confluent, non-caseating epithelioid granulomas. Biliary destruction with lymphocytic cholangitis was noted (Figure 2). Our gastric biopsies showed non-necrotizing granulomas. We diagnosed her with HS without clinically or radiographically evident lung involvement. Since her symptoms later resolved, we opted against starting steroids and we referred her to the rheumatology and pulmonology services.

DISCUSSION:
Lung involvement is seen in 90% of sarcoidosis patients, usually in the upper lobes, hilar and mediastinal lymph nodes. The diagnostic criteria for sarcoidosis include clinical and radiographic signs, non-caseating granulomas, and exclusion of other diseases. Elevated ACE levels and non-caseating granulomas are not specific to sarcoidosis and can also be seen in primary biliary cholangitis. What clinched our diagnosis was the gastric biopsies that also showed granulomas. Those with asymptomatic liver disease can be monitored until liver enzymes normalize. Hepatomegaly does not necessitate medical treatment, although patients with weight loss and lymphadenopathy should have prednisone, generally for 12 months. Ursodeoxycholic acid (UDCA) can be given for pruritus and liver enzyme elevations. In advanced HS cases with cirrhosis, liver transplantation is the only recourse.
2446 Idiopathic Non-Cirrhotic Portal Hypertension: A Teaching Case
Authors: Gurm Hashroop , Jamorabo Daniel S. , Renelus Benjamin D. , Guss Debra , Mohanty Smruti R. 
Published: October 2019

INTRODUCTION:
Idiopathic non-cirrhotic portal hypertension (INCPH) is characterized by portal hypertension without cirrhosis in setting of patent hepatic and portal veins, preserved hepatic synthetic function, and no established etiology. We present a case of idiopathic non-cirrhotic portal hypertension.

CASE DESCRIPTION/METHODS:
A 25-year-old Hispanic man presented with elevated liver enzymes. He was asymptomatic and not taking any medications. His family history was non-contributory. His BMI was 23 and he rarely drank alcohol. His examination was unremarkable. Lab data showed leukopenia to 2700, thrombocytopenia to 96,000, normal INR, AST 80, ALT 114, and alkaline phosphatase (AP) 128. His Alpha 1- Antitrypsin (A1AT), alpha-fetoprotein (AFP), and serum IgG were all normal. The viral hepatitis and HIV serologies were negative. His ANA, ASMA, AMA, anti-LKM titers were also negative. An abdominal sonogram showed liver 14.5 cm and splenomegaly to 14.8 cm; no ascites was seen. A Fibroscan showed F3-F4 consistent with advanced liver fibrosis, but our liver biopsy had <5% fatty change, negative iron stain, and overall normal histology. This was confirmed by a second outside pathologist. Testing for other diagnoses including Budd-Chiari, Epstein-Barr virus, cytomegalovirus, and Schistosomiasis returned negative.

DISCUSSION:
Non-cirrhotic portal hypertension can present with lab, imaging, and endoscopic findings suggestive of cirrhosis, yet liver biopsy shows normal histology. Our patient underwent extensive workup for cirrhosis and causes of non-cirrhotic portal hypertension before we diagnosed him with INCPH. This is a pre-sinusoidal entity that involves: 1) presence of portal hypertension, 2) absence of cirrhosis or advanced liver disease, and 3) lack of hepatic or portal vein thrombosis. Unlike in cirrhosis, liver failure, ascites, and hepatic encephalopathy are not typically present. Patients have preserved liver function and often normal LFTs, but can have splenomegaly (95%), hepatomegaly (50%), varices, and thrombocytopenia. The most common complication in these patients is variceal bleeding. Thus, primary and secondary prevention of esophageal bleeding via non-selective beta blockers and endoscopic therapy is beneficial. Of note, there is no role for screening patients for hepatocellular carcinoma (HCC) in INCPH since the absence of cirrhosis renders the HCC risk very low. INCPH is important to consider in patients with no clear cause for portal hypertension or LFT abnormalities.
2451 Living Donation: A Liver Saved or a Liver Compromised
Authors: Garkusha Larisa , Ho Tai Hang , Kalia Harmit 
Published: October 2019

INTRODUCTION:
When compared to deceased donor liver transplants (DDLT), living donor liver transplants (LDLT) have been associated with longer operative times, higher rates of biliary and vascular complications, and higher rates of small-for-size syndrome (SFSS). We present a case in which multiple complications and related diagnostic dilemmas are manifested over the course of several months following LDLT.

CASE DESCRIPTION/METHODS:
A 55 year old woman with decompensated alcohol-related cirrhosis with MELD of 24 was admitted and underwent living donor liver transplantation. Donor anatomy limited R lobe hepatectomy. Intraoperatively, portocaval shunt was left intact due to concern for small graft size (graft to recipient weight ratio was 0.7) as left lobe graft appeared congested post-anastomosis. Postoperatively, her transaminases trended down slowly, but she developed a worsening direct hyperbilirubinemia, peaking at Tbili 16.2 on POD #9. She was subsequently taken back to the OR for portocaval shunt closure and liver biopsy which was complicated by large hematoma. Following closure of portocaval shunt, her total and direct bilirubin continued to fluctuate but stabilized at Tbili 5.3 at which point she was discharged. Her biopsy was consistent with moderate preservation injury. Following discharge, she continued to require periodic paracentesis and thoracentesis for her refractory ascites. The patient's postoperative course was further complicated by persistent cholestatic liver tests concerning for bile cast syndrome and an admission for cholangitis two months post-op. She had multiple ERCPs showing biliary anastomotic strictures requiring stent placement. She continued to have persistent cholestatic labs despite these interventions and subsequently underwent a percutaneous liver biopsy. A venogram done at time of biopsy was revealing for severe short segment narrowing of the infrahepatic IVC s/p stent placement. Her repeat liver biopsy was suggestive of acute cell mediated rejection. Following pulsed steroid therapy, her liver tests improved.

DISCUSSION:
This case demonstrates the complications that are more likely to arise in LDLT compared to DDLT. Despite this, LDLT outcomes remain superior when compared to those who received DDLT; however this observation is complicated by selection bias in that younger, less critically ill patients with lower MELD scores are usually chosen for LDLT.