bluuebunny/crossref_metadata_2025_split huggingface dataset:
2232 Peribiliary Cysts Mimicking Primary Sclerosing Cholangitis and Cholangiocarcinoma
Authors: Kumar Shiva 
Published: October 2019

INTRODUCTION:
Peribiliary cysts are cystic dilatations resulting from obstruction of extramural peribiliary glands. They are usually benign, may arise from the intrahepatic and extrahepatic bile ducts and do not communicate with the biliary tree. They may, however, be confused with alternative diagnoses, resulting in unnecessary investigations with profound clinical implications.

CASE DESCRIPTION/METHODS:
A 42-year-old male was referred after recent diagnosis of cirrhosis, presumed secondary to primary sclerosing cholangitis (PSC), complicated by suspected cholangiocarcinoma. He presented with jaundice, weight loss and was noted to have nodular, cirrhotic liver with diffusely beaded appearance of the intra-hepatic biliary tree and central hilar prominence suggestive of mass lesion on outside imaging studies. ERCP was also interpreted as PSC with suspected cholangiocarcinoma. Brushing was negative for malignancy. Repeat MRI with MRCP showed multiple grape-like cystic structures surrounding the intrahepatic portal vein branches, resulting in compression of the intrahepatic bile ducts (Figure 1). These findings were consistent with peribiliary cysts. He subsequently underwent deceased donor liver transplantation. Gross examination of the explant revealed multiple benign bile duct cysts, which were focally multi-cystic with an exophytic semitranslucent cystic lesion in the hilum, which likely explains the prior imaging misdiagnosis as cholangiocarcinoma (Figure 2). Histologically, multiple cystic biliary structures were noted, lined by non-dysplastic cuboidal biliary type epithelium (Figure 3).

DISCUSSION:
Originally described in 1984, Peribiliary cysts are usually asymptomatic and often under- and misdiagnosed. The clinical importance of these structures arises from the fact that they may at times cluster together resembling multi-locular malignancy and mimic a dilated biliary tree. They may consequently be mistaken for entities such as biliary intraductal papillary mucinous neoplasm, cystic metastases, PSC, Caroli’s disease and importantly cholangiocarcinoma. In addition, they may also occasionally grow to compress the neighboring structures, resulting in biliary obstruction. High-resolution imaging with MRCP is a good modality for establishing the diagnosis. As highlighted by our case, given the prevalence of these lesions in patients with cirrhosis, awareness of this entity is critical to avoid misdiagnosis leading to inappropriate exclusion from transplantation in otherwise acceptable candidates.
2282 Hepatocellular Carcinoma With Rare Duodenal Metastases
Authors: Leroy Kyley , Long Amanda , Zitin Marc 
Published: October 2019

INTRODUCTION:
Hepatocellular carcinoma is a decompensation of cirrhosis. We present a patient with HCC, presenting with anemia, found to have metastatic disease to the duodenum.

CASE DESCRIPTION/METHODS:
67 y/o male with hepatitis C cirrhosis, complicated by HCC, presented with anemia and melena. HCC was diagnosed 1 year prior, with a 3.8 cm left lobe lesion, portal venous thrombus, and a LIRADS-4 right lobe lesion, initiated on palliative chemotherapy. Recent MRI demonstrated infiltrative HCC involving nearly the entire left lobe of the liver; tumor and bland thrombus within the portal system involving the confluence of the portal veins, left, and to a lesser extent, right portal vein. Exam revealed guaiac positive brown stool. Labs revealed hemoglobin 6.0 g/dL (baseline 12g/dL), MCV 93, BUN 17 mg/dL, Cr 1.2 mg/dL. Endoscopy demonstrated 3 superficial duodenal bulb ulcers, 2mm-3mm, with a visible vessel, treated; no varices seen. Repeat EGD demonstrated multiple closely associated pedunculated polypoid lesions with superimposed ulcerations with irregular appearing surrounding mucosa; biopsies without evidence of malignancy. Given the abnormal appearance, repeat EGD with biopsies was performed, revealing metastatic hepatocellular carcinoma in multiple fragments of the duodenal mucosa.

DISCUSSION:
Hepatocellular carcinoma is the most common primary liver malignancy. The most significant risk factor for HCC is pre-existing cirrhosis. Lung, bone and lymph nodes are the most common metastatic sites of HCC; metastatic disease to the duodenum is exceedingly rare (1). Of >7000 cases of HCC identified in Taiwan between 1996-2009, < 1%, or just 21 cases, revealed duodenal disease (1). GI involvement is most frequently a result of direct invasion by contiguous tumor, with right lobe disease most commonly extending to the duodenum and left lobe disease most commonly extending to the stomach (1). Rarely, as seen in our patient, duodenal disease can be a location of metastatic disease without direct contact. Wall thickening appreciated on imaging should prompt consideration of gastrointestinal involvement. It has been suggested that portal vein thrombosis may be an indication of hematogenous spread of tumor, in addition to being a poor prognostic indicator. Duodenal masses, ulcerations or bleeding should raise suspicion for duodenal metastasis, and portends a very poor prognosis.
2317 Accidentally Discovered Cystic Echinococcosis of the Liver
Authors: Elkhouly Mohamed A. , Attar Bashar M. , Elkafrawy Ahmed A. , Vohra Ishaan , Salazar Miguel , Mutneja Hemant , Mishra Satya 
Published: October 2019

INTRODUCTION:
Cystic echinococcosis (CE) is a complex, chronic and neglected disease with a worldwide distribution. The liver is the most frequent location of parasitic cysts. In humans, its clinical spectrum ranges from asymptomatic infection to severe, potentially fatal disease. We are presenting a case report of a patient with incidentally discovered liver hydatid disease after he was found to have suspicious liver cystic liver on renal ultrasound.

CASE DESCRIPTION/METHODS:
A 57-years-old male with history of hypertension and penile pain was referred to the Gastroenterology clinic after he was found to have a possible cystic liver lesion on renal ultrasound. He was originally from Palestine and moved to US 10 years ago. He did not have abdominal pain, icterus, nausea, vomiting or history of recent travel or liver disease. Examination was only relevant for non-tender, non-pulsatile, hepatomegaly with no palpable masses. Labs showed normal liver enzymes. CT Abdomen followed by MRCP showed 11.7 × 10.6 × 9.7 cm cystic mass within the right hepatic lobe, demonstrating multiple non enhancing daughter cysts within in, suggestive of hydatid disease. His serology was positive for Echinococcus granulosus antibodies. After discussion with the patient, he agreed for total cystectomy due to large size of the cyst. During the surgery, once the large cyst was mobilized, daughter cysts were first suctioned through a little puncture hole on the top of the cyst, until the large cavity was totally empty. During excision of the cyst wall, it was found to be tightly adherent to the liver tissue allowing only for excision of anterior cyst wall and omentoplasty. Pathology showed numerous scolexes consistent with Echinococcus Granulosus. He was given Albendazole 2 weeks prior the surgery and 4 weeks after with absence of recurrence on repeated imaging.

DISCUSSION:
Cystic echinococcosis is often asymptomatic and diagnosed accidentally during radiographic examination, surgery, or during evaluation of other clinical diagnoses. Potential presentations may be due to the mechanical effect of a large cyst on surrounding tissues, rupture of a cyst causing an acute hypersensitivity reaction, or complications such as biliary obstruction. Surgery has long been considered the best, if not the only, option in the treatment. However, in the past two decades, medical treatment, percutaneous procedures, and a “watch and wait” approach have been successfully introduced and have replaced surgery as the treatment of choice in selected cases.
2369 Intrahepatic Cholangiocarcinoma Presenting as Liver Abscess: A Rare Presentation of Common Cancer in Thailand
Authors: Sritunyarat Yingluk , Chaiteerakij Roongruedee , Wisedopas Nareumon 
Published: October 2019

INTRODUCTION:
Although cholangiocarcinoma is common in Thailand, it is uncommon to present as liver abscess1. Both conditions may be difficult to differentiate by clinical, laboratory and radiological findings2.

CASE DESCRIPTION/METHODS:
A 61-year-old-male with underlying type-2 diabetes presented with RUQ abdominal pain with low grade fever for 1 month. Two weeks later, he developed jaundice after taking herbal medicine. He visited a nearby hospital. Abdominal CT showed a 7.5 × 8.5 cm hypodensity lesion with rim enhancing and internal septation at right lobe liver (Figure 1) with no evidence of intrahepatic or common bile duct dilatation. He was treated as liver abscess with ceftazidime and metronidazole for a week, but not improved. Hence, he was referred to our hospital. At our hospital, physical examination revealed mildly pale, markedly icteric sclerae, tender hepatomegaly, but no splenomegaly. Lab showed Hb 10.7 g/dL, WBC 11.8 × 103/mL, platelets 140 × 103/mL, PT 24.8 sec, INR 2.4, PTT 35.5 sec, serum TB/DB 12.4/ 9.2 mg/dL, AST 80, ALT 51, ALP 134 IU/L, albumin/globulin 2.5/4.2 g/dL. Hemoculture, melioidosis and amoebic serologies, HBV, HCV and HIV tests were negative. Follow-up abdominal CT found a heterogeneous hypodensity lesion, 7.5 × 9 cm in size, with rim enhancing and internal air bubble at right lobe liver (Figure 2), suggestive of liver abscess. AFP was 11 IU/mL and CA 19-9 was 183 IU/mL. Provisional diagnosis was liver abscess with jaundice suspected from herbal drug induced liver injury (DILI). On USG-guided aspiration, 30 ml purulent material was found, which was negative for gram, AFB and fungal stain, including culture and bacterial identification. Ceftazidime and metronidazole were continued for 1 week, subsequently switched to imipenem for 1 week, however his fever did not resolve. The USG-guided aspiration was performed again, which found only 2 ml of pus. Biopsy of abscess wall was done. Pathologic results showed moderately differentiated adenocarcinoma (Figure 3),positive for CK7 and negative for CK20. The definite diagnosis was intrahepatic cholangiocarcinoma.

DISCUSSION:
Cholangiocarcinoma may present as liver abscess secondary to biliary tract obstruction or tumor necrosis with infection. In this case, CT findings of cholangiocarcinoma mimic liver abscess and history of herbal use misleads to diagnose jaundice from DILI. Thus, malignancy should be suspected when liver abscess does not resolve after treatment with antibiotics and adequate drainage.
2417 PEG-Asparaginase (PEG-ASP) Induced Liver Injury: Case Report and Systematic Review of the Literature
Authors: Rasor Madalyn , Zakharia Kais , Murali Arvind , Gunderson Alan 
Published: October 2019

INTRODUCTION:
Drug induced liver injury (DILI) is the most common cause of acute liver failure (ALF) in the USA. L-asparaginase (L-ASP) and its pegylated form (PEG-ASP) are antineoplastic agents used in acute leukemias. Here we present a case of severe DILI secondary to PEG-ASP and a systematic review of published cases.

CASE DESCRIPTION/METHODS:
Methods: PubMed search detected 343 abstracts related to ASP, of which 18 were relevant to our case. We used descriptive statistics to compare the published experience with our patient. Case: A 45 yo female with acute lymphoblastic leukemia (ALL) was admitted with a cholestatic liver injury and jaundice 14 days after starting PEG-ASP. She had normal liver enzymes prior to onset of treatment. Tests for viral and autoimmune hepatitis were unremarkable. Diagnostic ultrasound suggested only a fatty liver parenchyma. Liver biopsy revealed severe steatosis and mild cholestasis without fibrosis. Naranjo scale was 6 consistent with probable adverse drug reaction. PEG-ASP was stopped, steroids and L-carnitine infusion were started, however DILI continued to worsen. At their peak Total/direct bilirubin were 21/17 mg/dL, ALP 1,504 U/L, GGT 2,329 U/L and AST/ALT 153/50 U/L. Patient never developed ALF. Liver enzyme panel normalized over the next 8 weeks. Results: In our review of 18 reported cases, most were females (72%), adults (72%), with 32% being adults <50 years. The patterns of liver enzyme abnormality were cholestatic (26%), hepatocellular (21%), or mixed (21%) (unknown in 32%). Median time from starting ASP to DILI was 17 days. When biopsy was performed (11 patients) macrovesicular steatosis was uniformly present. Overall 6 patients died (one died of ALF) and 12 recovered with normalization of liver enzymes over the course of 2-26 weeks.

DISCUSSION:
Our patient is a classic case of DILI given onset of injury within 2-3 weeks of initiation of treatment, exclusion of alternate etiologies, histopathology showing severe steatosis, and complete normalization of liver enzymes following drug discontinuation. It is important to recognize ASP-DILI as 30% of cases end in ALF or death; nearly all cases will delay important treatment of acute leukemia. L-carnitine was reported as a potential rescue therapy, though its efficacy is uncertain.

CONCLUSION:
ASP related DILI is marked by severe steatosis and significant liver enzyme abnormalities occurring within weeks of starting treatment and resolving over many weeks after. Prompt recognition and drug discontinuation are imperative.
2421 A Case of Acute Liver Injury Caused by an Herbal Supplement Containing Saw Palmetto: “Good for the Prostate but Not for the Liver”
Authors: Wadood Romaisa , Nawaz Waqas , Hertan Hilary 
Published: October 2019

INTRODUCTION:
Saw palmetto (Serenoa repens), an herbal product used for benign prostatic hyperplasia, is a part of many herbal and dietary supplements (HDS) used for bodybuilding. It is associated with a hepatocellular pattern of acute liver injury. We present a case of liver injury secondary to supplement containing saw palmetto, which improved upon discontinuation.

CASE DESCRIPTION/METHODS:
A 36-year-old man with history of gastric bypass surgery was admitted with vomiting and diarrhea for the last 2 days. Abdominal examination was unremarkable. Labs revealed ALT of 2200 U/L, AST of 800 U/L with normal bilirubin, alkaline phosphatase and INR. Abdominal ultrasound revealed gallstones with a normal bile duct. Work up for infectious diarrhea was unremarkable. The symptoms were attributed to acute viral gastroenteritis. A diagnosis of acute liver injury was made, and further tests were performed to establish the etiology. Viral markers for Hepatitis A, B, C, E, Epstein Barr, Herpes simplex and Cytomegalovirus were all negative. The ANA, ASMA, ceruloplasmin and acetaminophen levels were unremarkable. He denied any alcohol use. Further history revealed that he had been using an over the counter herbal product to boost the testosterone levels to increase the muscle mass for the last one month. The ingredients of the product included saw palmetto and other herbal constituents. Review of the literature showed an association between saw palmetto and hepatocellular type of liver injury. The liver injury was attributed to the supplement and the cessation was advised. Follow up 4 weeks later showed normalization of the liver profile.

DISCUSSION:
HDS induced liver injury accounts for 20% cases of hepatotoxicity and 13% cases of acute liver failure in America. Many such cases are due to MINS (multi ingredient nutritional supplements), and the component responsible for the toxicity is usually unknown or can only be suspected. One of such ingredients used in these products is sawPalmetto which may cause supplement-induced liver injury. The latency to onset is within 1 to 2 weeks, and the clinical features resemble acute viral hepatitis with a hepatocellular pattern of liver enzyme elevations. Liver injury is probably an idiosyncratic phenomenon and usually resolves in 1 to 3 months. HDS-induced liver injury presents many challenges in diagnosis, identification of the responsible constituents, treatment and prevention. The regulation of non-prescription products to guarantee public safety is very important.
2442 Garcinia Cambogia, Weight Loss at What Price?
Authors: Park Angela , Castineira Janyll , Bournique Marissa , Babcock Heather , Miller Rebecca T. , Thomas Miriam 
Published: October 2019

INTRODUCTION:
As obesity levels in the United States continue to rise, more Americans are turning to the use of weight loss supplements. The perception of herbal supplements as an “all-natural” alternative has led to an increase in their use as well as a false sense of security in their safety profile. Garcinia cambogia (GC) is one such weight loss supplement. GC has been a topic of controversy, being scrutinized in multiple case studies as a potential cause of herb induced liver injury (HILI), a subset of drug induced liver injury (DILI). We present a case of DILI secondary to the ingestion of GC in the setting of alcohol and quetiapine use.

CASE DESCRIPTION/METHODS:
A 46-year-old female with past medical history significant for bipolar disorder and depression presented to the emergency department with complaints of acute right upper quadrant abdominal pain and distention for two days. Initial blood tests showed transaminitis, hyperbilirubinemia, and coagulopathy. The abdomen was mildly tender to deep palpation within the right upper quadrant; no jaundice or scleral icterus was noted. Imaging showed evidence of fatty infiltration of the liver. The patient denied prior history of liver disease, however she admitted to increased daily alcohol consumption over the last several months and long-term high-dose quetiapine use. An exhaustive work-up for alternative etiologies of liver disease was negative. The patient's liver injury was deemed to be drug-induced, secondary to the synergistic effects of GC in the setting of high-dose quetiapine ingestion and alcohol use. Supportive care was continued with improvement in the liver enzymes and bilirubin, and patient was discharged home with a decreased dose of quetiapine and instructions to follow up with psychiatry. One-week follow up labs showed continued down trending of liver enzymes and bilirubin.

DISCUSSION:
We present this case to emphasize the importance of considering the compounding effects of multiple hepatotoxic substances when mitigating a patient's risks for long-term liver damage. Our case highlights how multiple hepatotoxic insults can create a synergistic effect great enough to push the liver beyond its detoxification limits. Additionally, it demonstrates the importance of healthcare providers acting as diligent investigators of a patient’s home medications, supplements and lifestyle habits. Heightened sense of vigilance is necessary in order to prevent liver injury and its subsequent complications.
2454 Hepatocellular Carcinoma After Fontan Surgery
Authors: Kanagalingam Gowthami , Desai Anish 
Published: October 2019

INTRODUCTION:
Tricuspid atresia is a congenital cyanotic heart disease characterized by inability of the blood to flow from the right atrium to the right ventricle. The Fontan operation was first described for tricuspid atresia in 1971. The surgery creates an extra cardiac conduit to maintain circulation in the pulmonary artery without the pump function of the ventricle. This leads to low cardiac output and an increased central venous pressure mostly due to the resistance in the pulmonary circulation. Post- surgery, patients are at risk for liver cirrhosis and possibly hepatocellular carcinoma (HCC), due to venous congestion. Official guidelines do not exist for the screening of patient's for hepatocellular carcinoma after Fontan surgery. We present a case report of a woman with HCC and a history of Fontan surgery.

CASE DESCRIPTION/METHODS:
A 46-year-old woman with a past medical history of Fontan surgery for tricuspid atresia at age nine presented to the hospital with right upper quadrant discomfort. The patient had a negative history for alcohol abuse, hepatitis or family history for liver disease. Physical examination revealed jaundice and a palpable mass in the right upper quadrant. Laboratory data demonstrated elevated liver enzymes, bilirubin of 1.8 (mg/dl) and an alpha fetoprotein of 1386 (ng/ml). Computed tomographic (CT) scan of the abdomen demonstrated a subcapsular mass with multiple satellite lesions, in the liver. Liver biopsy confirmed moderately differentiated hepatocellular carcinoma. CT scan of lung was compatible with multiple metastatic lesions. She was started on chemotherapy with sorafenib and underwent radio-embolization. She had a poor clinical response to this initial treatment and was subsequently started on nivolumab. The patient was later admitted to the medical intensive care unit with decompensated liver disease and unfortunately, died soon after.

DISCUSSION:
Her only identifiable risk factor for hepatocellular carcinoma was her history of Fontan surgery. Current opinion is to screen patients for liver disease after Fontan procedure with magnetic resonance imaging (MRI) or CT scan of the liver every 3-5 years, starting at five years after surgery. We aim to increase awareness about the association between Fontan surgery and HCC with the help of our case report.
2461 Atraumatic Chylous Ascites in a Cirrhotic Patient
Authors: Karim Syed S. , Me Hay Me , Hein Aung M. , Wankhade Charudatta , Fernandez Damian , Rizvon Kaleem , Mustacchia Paul 
Published: October 2019

INTRODUCTION:
Only 0.5 to 1% of cirrhotic patients has chylous ascites (CA). CA is a rare form of ascites with milky peritoneal fluid that is rich in triglycerides. One study showed that the incidence is approximately 1/20,000 admissions at a university hospital over a 20 year period. Here, we report a case of atraumatic CA found in a cirrhotic patient.

CASE DESCRIPTION/METHODS:
A 66 year old man with cirrhosis secondary to alcohol abuse and hepatitis C virus, presented with marked abdominal distention and abdominal pain. On examination, the abdomen was soft, diffusely tender, distended with shifting dullness. Laboratory findings showed anemia, leukocytosis, normal AST, ALT, lipase and INR ratio. Paracentesis was performed and showed milky fluid. Ascites fluid analysis revealed leukocytosis with increased neutrophil count, triglyceride of 284 mg/dl and negative adenosine deaminase level and cytology. The results were consistent with spontaneous bacterial peritonitis and antibiotics was started. However, he deteriorated with intractable vomiting and was found to have high grade small bowel obstruction from CT imaging. He was taken to the OR for laparotomy but continued to deteriorate and expired.

DISCUSSION:
The most common causes of atraumatic chylous ascites are lymphatic anomalies (32%), malignancy (17%), cirrhosis (11%), Mycobacterium (15%), and a variety of uncommon causes (21%). Our patient had grade 3 ascites by International Ascites Club Grading system, cirrhotic liver with portal hypertension and high grade small bowel obstruction. All of those factors increased the pressure on the intestinal lymphatic system leading to CA. Paracentesis showing triglycerides above 200 mg/dl gives the diagnosis. Management consists of treating the underlying etiology. Pharmacologic treatment with Orlistat and octreotide were reported to be effective in CA. Transjugular intrahepatic portosystemic shunt and peritoneovenous shunting have been used in refractory cases. However, our patient had sepsis from spontaneous bacterial peritonitis with Child Pugh class C, and high grade small bowel obstruction which excluded him from the insertion of shunts. Repeated large-volume paracentesis is a reasonable option for patients who have end-stage disease not amenable to medical or surgical treatment. It is imperative to realize the critical condition in patients with CA as the one year mortality rate is high (69%) and appropriate studies should be done immediately to find and treat the underlying etiology.