bluuebunny/crossref_metadata_2025 huggingface dataset:
2195 Expanding Therapeutic Options for Ileocecal Valve and Rectal Strictures With Lumen-Apposing Fully Covered Metal Stents
Authors: Vachaparambil Cicily , Abdelfatah Mohamed Magdy , Chawla Saurabh , Willingham Field , Mekaroonkamol Parit
Published: October 2019

INTRODUCTION:

Radiation-induced and Crohn’s-related colonic strictures are debilitating conditions requiring intensive serial endoscopic dilations or surgery. Use of colonic stents in benign strictures are limited by migration risk, lack of removability, and technical difficulties in placement. This is especially true of ileocecal valve (ICV) strictures given the anatomically challenging location and short stents are preferred. We report the cases of an ICV and distal rectal stricture, refractory to endoscopic dilation, that underwent successful lumen apposing fully covered metal stent (LAMS) placement.

CASE DESCRIPTION/METHODS:

A 32-year-old African American male with a history of Crohn’s disease complicated by ICV stenosis causing recurrent partial small bowel obstruction, was admitted with worsening nausea, vomiting, and abdominal pain. An abdominal CT showed dilated distal ileum to 4 cm with associated fecalization consistent with partial small bowel obstruction. A colonoscopy with balloon occlusion enterography confirmed a short <1 cm severe ICV stricture with non-inflamed colonic mucosa. He opted for an endoscopic dilation to 10 mm with good clinical response but this only lasted for 2 weeks. A stent placement was then performed using a 15 mm × 10 mm LAMS (Figure 1). His abdominal pain markedly improved with radiologic resolution of the small bowel obstruction. A 55-year-old African American female with a history of pelvic radiation for cervical cancer presented with tenesmus and alternating constipation and diarrhea. Barium enema and flexible sigmoidoscopy showed a focal rectal stricture at 10 cm proximal to the anal verge. She underwent serial endoscopic dilations up to 12-mm over a span of 6 months with no significant clinical response and the stricture kept recurring despite 2-3 week intervals between dilations. Ultimately, a 15 mm × 10 mm LAMS was placed (Figure 2). At 8-weeks, her tenesmus and post-constipation diarrhea were completely resolved.

DISCUSSION:

Our cases demonstrate the feasibility and efficacy of LAMS placement for refractory high-grade strictures even at anatomically challenging locations. LAMS was preferred here due to its removability and short length that could optimally fit in the ICV and distal rectum with an aim to minimize risk of stent migration due to its dumbbell configuration. However, the stricture site has to be reachable by a therapeutic gastroscope. Optimal duration for stent indwelling and risk of restenosis after stent removal remain unclear and warrant further study.
2196 A Case of Successful Endoscopic Management of Choledocholithiasis Complicated by Post-Transplant Biliary Stricture
Authors: Gollapudi Lakshmi Asritha , Kumar Anila , Bodin Roxana , Georgostathis Dimitrios , Lebovics Edward
Published: October 2019

INTRODUCTION:

Post Liver transplant (LT) biliary stones, sludge and casts are a challenge to both advanced endoscopists and patients in the setting of complex anatomy, variable pathologies and multiple co-morbidities. The incidence rate of biliary stones is widely variable from 3.8%-18% and are associated with biliary strictures in roughly 90% of cases, increasing the complexity of the procedure. Here we are presenting a case of elevated liver enzymes and normal bilirubin in a LT patient with a history of post-LT biliary stricture.

CASE DESCRIPTION/METHODS:

70 year old male with past medical history of fulminant hepatic failure status post orthotopic LT complicated by biliary anastomotic stricture, presented for management of new onset elevated liver enzymes ten years post LT. At the time of presentation he denied any complains of abdominal pain, nausea, vomiting, fevers, or chills. He denied any new medications or herbal supplements. Afebrile and hemodynamically stable at presentation. Labs showed WBC- 6.3, AST-202, ALT-133, GGT-1368, ALP-439 Total Bilirubin – 0.8 (Normal), INR-1.06 and Tacrolimus level -2.4. Liver biopsy showed bile ductular reaction and sparse to mild portal inflammation with minor ductulitis and endothelialitis. Abdomen ultrasound showed common bile duct with biliary ductal dilatation which may represent stones or sludge. Patient underwent ERCP revealing a large 3 × 2 cm stone in the common hepatic duct proximal to the anastomotic stenosis with marked upstream biliary dilation. The stone was partially crushed with electrohydraulic lithotripsy (EHL) via Spyglass cholangioscopy. The stone fragments were removed using an extraction balloon. Two 10 Fr x 12 cm long angled plastic stents were placed in the right and left biliary systems. A repeat ERCP was performed 8 weeks later and EHL under spyglass was performed. The ducts were subsequently cleared and stenosis resolved.

DISCUSSION:

Biliary stones and sludge can occur at any time after LT, with a reported incidence of 5%–10%. Our patient presented 10 years post-LT with transaminitis and normal bilirubin. Yu et al reported biliary strictures and cold ischemia time were significantly associated with bile duct stone formation after liver transplantation in a case-control study. Hence we conclude that in following patients post LT who are on immunosuppressants, the physician needs to be vigilant as patients can have a late presentation which can lead to graft loss and increased morbidity and mortality.
2197 Successful EUS-Guided Treatment of Duodenal Varices With Coil Embolization
Authors: Cortez Nathaly , Mahfouz Mahmoud , Berzosa Manuel , Nasir Ahmad Basil
Published: October 2019

INTRODUCTION:

Ectopic varices are unusual and can involve any site along the GI tract. Only 5% of variceal bleeding is related to ectopic varices such as duodenal varices. Ectopic variceal bleeding is usually massive, difficult to treat, and associated with high mortality rates. High clinical suspicion is warranted. No standardized treatment is recommended, and the management remains challenging with limited success in controlling recurrent bleeding. EUS-guided therapy has evolved as an effective therapeutic modality in gastric varices and this case demonstrates the role of EUS-guided coil embolization (EUS-CE) with cyanoacrylate (CYA) injection in the management of duodenal variceal bleeding.

CASE DESCRIPTION/METHODS:

81-year-old female with history of Diffuse large B cell lymphoma on chemotherapy presented with intermittent massive upper gastrointestinal bleeding. CT abdomen showed cirrhotic liver and portal vein thrombosis due to lymphoma involvement. Three endoscopies performed over a period of 5 days revealed 1 cm non-bleeding duodenal ulcer at the duodenal sweep without stigmata of recent bleeding. Due to recurrent bleeding, empiric coil embolization of the gastroduodenal artery and multiple duodenal branches through angiography was done by interventional radiology. However, severe bleeding recurred after the embolization, and the fourth urgent EGD showed large subepithelial violaceous lesions next to the deep penetrating ulcer, highly suspicious for duodenal varices. EGD-EUS confirmed these lesions were duodenal varices. By the fifth day of admission, patient had required 8 units of PRBC. Therefore, the decision was made to treat with EUS-CE. Two coils of 8 and 9 mm were deployed into the larger varix through a 19G EUS-FNA needle followed by 2 ml injection of CYA. A third 6 mm coil was also deployed in the feeder vessel also followed by CYA. Both lead to embolization and cessation of blood flow as demonstrated through Doppler investigation at the end of the procedure. Bleeding stopped and patient was discharged home to complete chemotherapy.

DISCUSSION:

Bleeding from duodenal varices require high suspicion as vessels could be miss if collapsed soon after bleeding. High clinical suspicion can result in early angiographic evaluation and intervention. Ideal management of ectopic variceal bleeding is still unclear. Multiple endoscopic technique modalities have been reported. EUS-CE with or without CYA injection is a safe endoscopic technique with effective variceal obliteration in most cases.
2199 Endoscopic Ultrasound-Guided Transrectal Drainage of a Tubo-Ovarian Abscess via a Lumen-Apposing Metal Stent
Authors: Shah Ruchit N. , West Sara , Sweeney Kaitlyn M. , Mehta Minesh , Diehl David L.
Published: October 2019

INTRODUCTION:

Tubo-ovarian abscess (TOA) is a potentially lethal condition requiring a combination of medical and surgical intervention. Endoscopic ultrasound (EUS)-guided drainage is a known modality for safe and effective management of pelvic collections, but its role in TOA drainage is not well documented. We present a case of a TOA treated successfully with EUS-guided transrectal drainage using a lumen apposing metal stent (LAMS).

CASE DESCRIPTION/METHODS:

A 45-year-old woman presented to the hospital with a 4-day history of left lower quadrant abdominal pain. Her medical history included a right-sided TOA treated with antibiotics one year ago, type II diabetes mellitus, and obesity. She was tachycardic and tachypneic on arrival with a leukocytosis and elevated inflammatory markers. Computed tomography (CT) revealed a 11.3 × 10.3 × 9.4 cm complex cystic structure within the left adnexa consistent with a TOA (Image 1). Due to worsening leukocytosis and abdominal pain, interventional radiology was consulted but could not find a safe window for percutaneous drainage. A rectal EUS subsequently revealed a 50 × 45 mm abscess 20 cm from the anal verge (Image 2). A 19-gauge EZ-Shot 3 FNA needle was advanced with return of purulent fluid. After injection of 15 cc ionic contrast to delineate the abscess and confirm positioning, EUS-guided transrectal drainage of the TOA with placement of a 15 × 10 mm AXIOS stent (Boston Scientific, Marlborough, MA, USA) was successfully performed. The distal flange was deployed within the abscess cavity and the proximal flange was deployed within the rectal lumen with copious amounts of purulent drainage through the AXIOS stent (Image 3). Repeat CT performed one week later revealed significant interval decrease in the size of the TOA.

DISCUSSION:

EUS-guided drainage of pelvic collections has been established as a lower-risk alternative to surgical interventions, especially for collections greater than 4 cm and within a 1-2 cm reach of the EUS transducer. Furthermore, use of LAMS can facilitate drainage through a wider conduit, promoting rapid evacuation and source control. To our knowledge, this is the first reported case of successful EUS-guided transrectal drainage of a TOA using a LAMS.
2201 Innovative Endoscopic Management of a Bariatric Surgery Complication
Authors: Herman Michael , Kakked Gaurav , Collins Kelsey , McCabe Evin , Kasmin Franklin
Published: October 2019

INTRODUCTION:

Vertical Banded gastroplasty (VBG) is a purely restrictive bariatric surgery that was the predominant weight loss procedure in the 1980s. The following case describes a well-known complication of VBG that was managed with an innovative endoscopic technique.

CASE DESCRIPTION/METHODS:

A 56-year-old lady with a history of open VBG 18 years ago presented with several years of recurrent vomiting, heartburn, and excessive weight loss. An upper GI series revealed a large distend proximal gastric pouch above the mesh with severe stasis that produced a mass effect and resultant compression of the rest of the stomach. She was offered a conversion to Roux en y gastric bypass, however she declined. She was subsequently referred to a GI for further evaluation and potential endoscopic intervention. An Upper GI endoscopy was performed, which revealed mild esophagitis, evidence of vertical banded gastroplasty, and gastric pouch distention with copious amount of food debris. The lumen through the band was identified and was noted to be very narrowed and was not easily traversable with the upper endoscope. Therefore, a decision was made to perform an EUS guided gastro-gastrostomy with placement of a lumen opposing metal stent. The distal pouch was punctured under EUS guidance and a 15 × 10 mm AXIOS Stent was deployed adjacent to the previously placed band, creating a larger lumen for food content to pass. The stent was serially dilated to 18 mm using a through the scope (TTS) dilator. Contrast injected proximally was seen to flow freely from the gastric pouch into the antrum and the duodenum through the stent. Patient had an uneventful post-procedure course and was discharged the next day. She later reported significant improvement in reflux symptoms, gained 15 pounds, and was more than elated.

DISCUSSION:

VBG quickly gained popularity in the 1980s because it was the only bariatric surgery that did not have malabsorptive side effects. Over time, however, patients began experiencing post procedural nausea, vomiting, and heartburn from pouch dilatation, band erosion, staple line disruption, marginal ulceration, and is now rarely used as a bariatric surgical option. The versatility of endoscopic stents have made them useful in the management of many bariatric surgical complications, including fistulas, leaks, obstructions, strictures, and stenoses. To our knowledge, this was the first case of severely symptomatic pouch dilatation following VBG that was successfully managed endoscopically.
2202  Liver Mass in Cirrhotic Patients: Is It Always Hepatocellular Carcinoma (HCC)?
Authors: Hayat Maham , Zia Hassaan , Hayat Muhammad Hashim , Fazili Javid
Published: October 2019

INTRODUCTION:

Pancreatic acinar cell carcinomas (PACCs) comprise only 1-2% of all pancreatic cancers. Patients with PACC have a prognosis better than pancreatic ductal adenocarcinomas but worse than pancreatic neuroendocrine tumors. They are easier to differentiate from the prior, but may have many similar morphological features to the latter. The majority of pancreatic tumors are adenocarcinomas of the ductal type; and carcinomas with multiple lineage differentiation are extremely rare. We present a rare case of PACC with neuroendocrine differentiation.

CASE DESCRIPTION/METHODS:

51-year-old male with history of chronic Hepatitis B infection, liver cirrhosis (CPT class B, MELD 15) was admitted for abdominal pain, leg swelling and worsening renal function. Pertinent physical exam revealed a tense abdomen and diffuse tenderness. An abdominal non-contrast computed tomography (CT) scan revealed a large cm ill-defined mass in the central liver involving both right and left liver lobes. A large mass medial to the splenic hilum measuring approximately 8.7 × 6.8 cm, abutting the stomach, pancreas and spleen was also seen. An Endoscopic Ultrasound (EUS) confirmed an anechoic splenic hilum mass measuring 8 cm in greatest dimension. A 2.4 cm × 2.2 cm anechoic left liver lobe mass was also appreciated. Fine needle aspiration (FNA) was performed for each of these masses. Histopathology revealed acinar-cell carcinoma with neuroendocrine differentiation. Positive Periodic acid-Schiff (PAS), Trypsin and Chromotrypsin stains on immunohistochemistry (IHC) supported the acinar differentiation. Positive synaptophysin and chromogranin stains supported neuroendocrine differentiation. Diagnosis of pancreatic neoplasm with metastasis to the liver was made and patient was referred to an oncologist.

DISCUSSION:

Forming the diagnosis of a mixed carcinoma requires thorough assessment of individual components in the tumor. Current theories suggest their origin from a stem cell with propensity for multi-directional differentiation. Close morphological examination and immunohistochemical staining are fundamental in making an accurate diagnosis. Better understanding of prognosis and treatment of pancreatic tumor with multi-lineage origin requires evaluation of additional cases due to their paucity.
2204 Non-Hepatic Hyperammonemia Associated With Acute Bowel Perforation
Authors: Melnyk Janet L. , Cavaliere Kimberly R. , Roth Nitzan C.
Published: October 2019

INTRODUCTION:

Hyperammonemia is often caused by impaired ammonia degradation in patients with cirrhosis or acute liver failure and can lead to acute mental status changes known as hepatic encephalopathy. Rarely, elevated ammonia levels occur in patients without liver disease. We report a case of non-hepatic hyperammonemia in a critically ill patient who was found to have bowel perforation.

CASE DESCRIPTION/METHODS:

A 62-year-old man with history of coronary artery disease and chronic systolic heart failure presented with chest pain and shortness of breath. He was found to have methicillin-sensitive Staphylococcus aureus bacteremia complicated by infective endocarditis requiring aortic valve replacement performed on hospital day (HD) 24. Post-operative course was complicated by altered mental status on HD 38. He later developed septic shock requiring pressor support and mechanical ventilation. Sedation was discontinued, but he remained unresponsive to verbal or noxious stimuli, a departure from a normal mental status a few days prior. Workup was significant for an elevated venous ammonia level of 118 umol/L on HD 48. Other than mildly elevated alkaline phosphatase 149 U/L and INR 1.66, his liver tests were within normal limits. Earlier in the admission, non-contrast CT showed hepatic steatosis with normal liver morphology and no features of cirrhosis or portal hypertension. Trial of therapy with rifaximin and lactulose HD 48-69 provided no benefit. On HD 52, the patient’s serum ammonia had increased to 231 umol/L, and his abdomen was found to be acutely distended and rigid with serum lactate 4.1 mmol/L. Repeat CT showed intraperitoneal free air, pneumatosis, and mesenteric venous gas. Emergent exploratory laparotomy revealed a perforated ascending colon, and hemicolectomy was performed. Post-surgery, serum ammonia levels normalized however the patient’s mental status did not improve. He was terminally extubated and expired HD 69.

DISCUSSION:

We present a unique case of non-hepatic hyperammonemia that preceded the discovery of acute bowel perforation, with serum ammonia levels normalizing after the damaged bowel was surgically removed. No other known causes of hyperammonemia were identified, particularly liver disfunction, ammonia-raising medications, infection with urease-producing organisms. Hyperammonemia has been shown to be associated with gastrointestinal hemorrhage, however that was not the case with this patient. Perforated viscus should be considered when non-hepatic hyperammonemia is encountered.
2208 Autoinflammation in an Immunocompromised Lymphoma Patient With Sepsis
Authors: Kraus Fabian B. T. , Mehta Amit , Wan David
Published: October 2019

INTRODUCTION:

Hemophagocytic lymphohistiocytosis (HLH) is a rare autoinflammatory, typically pediatric condition, often triggered by infectious or malignant diseases in the immunocompromised patient. In this case, we describe the coincidence of HLH and bacterial sepsis in an adult NK/T cell lymphoma (NKTCL) patient.

CASE DESCRIPTION/METHODS:

A 38-year-old man presented with weight loss, fevers, night sweats and jaundice. Dermatological findings, including petechiae on the chest and extremities as well as an oropharyngeal soft palate erosion, were biopsied and revealed extranodal small cell NKTCL. Treatment was initiated according to the SMILE protocol yet soon complicated by fever and hypotension, which made an empiric antibiotic therapy with meropenem and amikacin necessary. Initial blood tests showed transaminitis, an acute drop in hemoglobin and thrombocytopenia. This, in combination with progressive hypotension and abdominal pain, raised suspicions of an acute GI bleed, but an esophagogastroduodenoscopy was unremarkable. Abdominal ultrasound, chest X-ray and abdominal CT, also yielded no evidence for bleeding, but confirmed the disease’s progression and led to treatment expansion by pembrolizumab. An increased EBV load, hypofibrinogenemia, hypertriglyceridemia, lactic acidosis and elevated ferritin levels in combination with the described clinical findings finally led to the diagnosis of HLH, complicated by disseminated intravascular coagulopathy (DIC), and were treated with steroids and plasma products. In the days thereafter, the patient’s fever spiked to 39.8°C and abdominal pain increased. Blood cultures were positive for MRSA and an infection with Parainfluenza virus was diagnosed. Anti-infectious treatment was adjusted accordingly. Decompensating electrolyte disturbances due to renal failure tachycardia and hypotension required catecholamine-treatment and the patient was transferred to the ICU where he deceased six days after initial admission.

DISCUSSION:

The complexity of the phenotypical diagnosis as well as the current lack of time-tested therapeutics pose major challenges in the treatment of HLH. Here we describe the history and death of a NKTCL patient, whose diagnosis and treatment was complicated by DIC and bacterial sepsis. We want to highlight the importance of a quick diagnosis and the rapid treatment of HLH. Further research is needed to advance diagnostic tools, optimize current therapeutic approaches and prevent comorbidities.
2209 Abdominal Arterial Aneurysms in a Patient With Budd-Chiari Syndrome
Authors: Srivats Sudarshan , Niec Rachel , Wan David
Published: October 2019

INTRODUCTION:

Budd-Chiari syndrome (BCS) is defined as hepatic venous outflow tract obstruction. Chronic BCS eventually leads to post-sinusoidal hypertension resulting in the symptoms of the disease. There have been case reports of BCS leading to intra-abdominal bleeding from ectopic varices, however, these are cases of venous bleeding. Here, we report a unique case of a Chinese woman with BCS presenting with bleeding from arterial aneurysms.

CASE DESCRIPTION/METHODS:

A 61-year-old woman with BCS requiring multiple prior porto-systemic shunting procedures was brought by EMS for coffee ground emesis. At the ED, she was stuporous and developed bloody emesis and hypotension. She was intubated and transferred to the ICU. Exam was remarkable for abdominal distention (AD) with a fluid wave. Labs showed leukocytosis; prolonged coags, elevated troponins, lactate, LDH and decreased fibrinogen and haptoglobin. FAST showed intra-abdominal free fluid. EGD showed grade II varices in the esophagus that were banded. However, the patient continued to deteriorate with increasing AD. CT-AP revealed a cirrhotic liver with occluded left and middle hepatic veins and extravasation of contrast near the greater curvature of the stomach into a LUQ hematoma (Figure 1). IR embolized the left gastric artery and identified aneurysms along a branch of the SMA (Figure 2) in addition to an aneurysm at the celiac artery origin (Figure 3). On the 2nd post-op day, the patient again developed hypotension with AD. Attempted embolization of the SMA branch was unsuccessful. On the 5th post-op day, the family agreed to withdrawal of life support and the patient was extubated and vasopressors were stopped. The patient passed away in a few hours.

DISCUSSION:

Chronic BCS presents with complications of cirrhosis; i.e., ascites and rupture of esophageal varices, like in our patient. On CTA, both the hepatic veins and part of the IVC were occluded. In fact, 31% of BCS patients have outflow occlusion at both these locations. However, in our patient, CTA revealed aneurysms in the celiac artery and distal portions of an SMA branch along with left gastric artery bleeding. Multiple arterial aneurysms have been associated with disorders such as PAN, Behcet's, Ehler-Danlos etc. By comparison, there have not been many reports of multiple idiopathic arterial aneurysms in adults. Several of these possible causes were unable to be worked up for due to the acute presentation, although our patient’s history was not typical for these diseases.
2210 Resolution of Hepatocellular Carcinoma in a Patient With Chronic Hepatitis B Treated With Entecavir Therapy
Authors: Suri Jaspreet , Shah Pir Ahmad , Choudhry Saad , Nasser Imad A. , Lau Daryl T.
Published: October 2019

INTRODUCTION:

Hepatitis B virus (HBV) is a major cause of hepatocellular carcinoma (HCC) globally. Prolonged HBV DNA suppression with nucleos(t)ide analogues is known to regress hepatic fibrosis and reduce HCC risk. This is an unusual presentation of a patient with hepatitis B-associated HCC whose liver lesion resolved on antiviral therapy alone.

CASE DESCRIPTION/METHODS:

Patient is a 64-year-old Chinese male with HBeAg negative chronic hepatitis B with persistent normal serum aminotransferases and was antiviral treatment naïve. He had a positive family history of HCC and was first noted to have asymptomatic elevation of alpha-fetoprotein (AFP) to 648.9 ng/ml 6 months prior to his referral to Hepatology. During his initial Liver Center visit, the AFP increased to 1,900 ng/ml with elevated AFP-L3 at 18.4%. His hepatitis B remained inactive with normal ALT and minimal detectable HBV DNA at 1.6 log IU/ml (Table 1). He was started on entecavir 0.5 mg daily in view of his age and suspicious HCC complication. Initial MRI of the abdomen revealed lesions in segment 4 (2 cm) and segment 7 (2.1 cm) of the liver with arterial enhancement, venous washout and pseudocapsule. Targeted liver biopsy of segment 7 lesion and adjacent liver was performed within 2 weeks on entecavir therapy. The targeted liver specimen revealed a necrotic focus with loss of reticulin with strong immunoreactivity for cytokeratin Hep Par1 and Glypican-3; the findings were highly suspicious for HCC. The adjacent liver had only mild inflammation (grade 0-1) and minimal fibrosis (stage 0-1). Six weeks later, AFP decreased to 19 ng/dl but AFP-L3 remained elevated at 17%. On repeat CT scan, the segment 7 lesion decreased to 1.2 cm and no longer met OPTN HCC criteria. Patient remained clinically stable with optimal HBV DNA suppression. By 7-month follow-up, both AFP and AFP-L3 normalized and the liver lesions remained stable in size (Table 1). He continued to have regular monitoring and the segment 7 liver lesion was no longer visualized on abdominal MRI after 2 years.

DISCUSSION:

Nucleos(t)ide analogue therapy is known to suppress HBV replication but has not been reported to have direct effect on HCC. This HCC case is unique as the liver lesion regressed and resolved on entecavir therapy alone. While the molecular mechanism leading to the HCC resolution is currently unknown, this case highlights the beneficial role of prolonged HBV DNA suppression with antiviral therapy.
2212 Hemophagocytic Lymphohistiocytosis Disguised as Acute Liver Injury
Authors: Hasan Badar , Yim Yunjoo , Castaneda Daniel , Rashid Mamoon Ur , Al Khalloufi Kawtar
Published: October 2019

INTRODUCTION:

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disorder with unregulated immune activation and inflammation. Primary HLH occurs in infants or young children with or without family history whereas secondary HLH manifests in older children or adults without a family history or gene mutation. Concurrent infection, malignancy or rheumatological disorder usually trigger an uncontrolled immune response in the older age group. Our aim is to raise is awareness among physicians to recognize the early signs and symptoms of this life-threatening condition.

CASE DESCRIPTION/METHODS:

A 33-year-old female with a history of multiple sclerosis and rheumatoid arthritis presents with elevated LFTs, cholestatic jaundice and intermittent high-grade fever. Imaging studies showed no significant abnormality in the hepatobiliary system. Viral and autoimmune serologies were negative. Lab works revealed low fibrinogen and elevated ferritin and IL-2 receptor levels. Bone marrow biopsy was unremarkable; however, liver biopsy showed features suggestive of HLH. Steroid and etoposide therapy was started but treatment was complicated by pancytopenia and hemodynamic instability despite multiple transfusions and filgrastim administration. With worsening clinical pictures, emapalumab (Interferon gamma blocker) was started. However, the patient developed multiorgan failure with overwhelming sepsis and died within days of emapalumab administration.

DISCUSSION:

HLH has a wide spectrum of clinical manifestation often masquerading as sepsis or SIRS. Some studies have found that cholestasis and high AST levels are associated with poor outcomes in adult HLH patients. The goal of therapy is to suppress hyper inflammation with agents such as corticosteroids, calcineurin inhibitors, and immunoglobulins. When a patient presents with prolonged fever and liver dysfunction of unknown etiology, HLH should be considered in the differential diagnosis as early recognition and treatment can prevent mortality, which can be high up to 75 % in the adult age group.
2223 Zieve’s Syndrome: Case Report, Review of the Literature and Electronic Patient Database
Authors: Twohig Patrick , Desai Aakash , Sandhu Sonia , Sandhu Dalbir S.
Published: October 2019

INTRODUCTION:

Zieve’s syndrome (ZS) is rare condition of alcoholic hepatitis, hyperlipidemia and hemolytic anemia1. Clinicians need to be aware of ZS for several reasons: First, hemolysis increases unconjugated bilirubin levels, leading to false elevation of the Maddrey’s discriminant function (MDF), which may lead to the unnecessary prescription of steroids.2 Second, the anemia that occurs from hemolysis may be misinterpreted as a gastrointestinal bleed, leading to extra endoscopic procedures. Finally, since patients can present with fever and abdominal pain, diagnosis may be construed for other causes such as sepsis or acute cholecystitis.2-5

CASE DESCRIPTION/METHODS:

A 39-year-old female with long-standing alcohol abuse had three successive admissions resulting in refractory ZS. We performed a review of the literature to assess other case reports of patients with ZS. A total of 38 patients were included, 8 in women and 30 in men. Patients’ age ranged from 28 to 65 years old (mean 45.6 years). Average total bilirubin was 9.12. Average hemoglobin was 9.15. Average total cholesterol was 486. Average LDH was 243. Most patients did not receive prednisolone and fully recovered with strict alcohol cessation. A retrospective analysis using the IBM Explorys database of over 63 million de-identified, unique patients in the United States revealed that of 17,990 patients with alcoholic hepatitis, only 80 had ZS. The odds of a patient with alcoholic hepatitis also having hemolytic anemia is 7. ZS is rare, only seen in 0.4% of patient’s with alcoholic hepatitis.

DISCUSSION:

Our patient was 39, younger than the average age of 45.6 years. She was also female, a gender seen in a minority of previously reported cases. She had a total bilirubin 3 times above average, and a hemoglobin 33% lower than average. Despite 12 units of packed red blood cells, prednisolone, and pursuit of inpatient/outpatient alcohol rehabilitation, she ultimately succumbed to her liver injury 6 weeks after initially presenting to the hospital. This is the first reported case of death from ZS. Clinicians should maintain a high index of suspicion for ZS in patients with alcoholic hepatitis and transfusion refractory anemia. Evaluating for hemolysis and hyperlipidemia are recommended. Alcohol abstinence is essential. There is no role for steroids. Early diagnosis is essential. Our goal is that increased case reporting will raise clinician awareness.
2224 A Rare Complication of Post-TIPS Related Acute Cholecystitis
Authors: Chiejina Maria , Samant Hrishikesh , Morris James D.
Published: October 2019

INTRODUCTION:

Amongst the well documented TIPS-related biliary complications, there has been no reported case of acute cholecystitis. Multiple multicenter RCT comparing TIPS to large volume paracentesis reported better control of ascites, lower diuretic doses or cessation of diuretics in approximately 75% of the patients. Biliary related complications include biliary venous fistulas, biloma or biliary occlusion secondary to biliary compression. This may be the first reported case of Post TIPS acute cholecystitis.

CASE DESCRIPTION/METHODS:

67-year-old white male with end-stage NASH cirrhosis was hospitalized 6 months ago for diuretic-resistant ascites. Prior to admission, he has required weekly large volume paracentesis. While hospitalized, he underwent large-volume paracentesis and was treated for acute urinary tract infection. TIPS was placed two weeks post discharged and ascites improved. Three days later while undergoing further evaluation, a liver ultrasound showed gall bladder wall thickening and gall stones, suggestive of acute cholecystitis with cholelithiasis. He subsequently developed severe pancytopenia and worsening liver function test. A percutaneous cholecystostomy tube was placed for gall bladder decompression along with a 14-day course of IV antibiotics. He later developed Choledocholithiasis and required ERCP and cholangioscopy. Multiple stones were extracted from the bile duct and complete fibrosis of the cystic duct was noted. The patient required multiple hospitalizations since then, frequent paracentesis and treatment for SBP. He recently underwent OLT with cholecystectomy which was technically challenging.

DISCUSSION:

TIPS insertion has been established as an effective second-line treatment for refractory ascites. There are 3 documented category of TIPS complications; technical, portosystemic and unique shunt related but no known reported case of acute cholecystitis. Hepatic encephalopathy occurs in approximately 30% of patients, early thrombosis or delayed shunt stenosis occurred in 22- 50% of patients prior to the development of polytetrafluoroethylene-covered stents. The technical intricacy of this intervention poses a potential risk, and morbidity rates as high as 20%. A broad understanding of the uncommon and unique complications can potentially decrease post-op-related complications especially in a patient undergoing OLT It is necessary for clinicians who perform this procedure to be aware of the clinical spectrum of TIPS-related complications and their management.
2225 Hydrazine Sulfate: A Case Report of Drug-Induced Acute Hepatorenal Toxicity
Authors: Weigle Rachael L. , Carver Charles K. , Mank Gerald , Campanile Teresa , Burnette Tonya
Published: October 2019

INTRODUCTION:

Hydrazine sulfate (HS) is a synthetically manufactured ammonia derivative marketed in the US as a dietary supplement, though many patients still use it for its previously proposed anticancer properties. HS inhibits phophoenol pyruvate carboxykinase thereby blocking gluconeogenesis. In investigations since the 1980s, it has been proven ineffective with respect to its ability to improve cachexia and limit rate of tumor growth as previously postulated. Also, multiple animal trials have shown an increase in the incidence of new lung, liver, and breast tumors with use of HS. It has been listed by the IARC Working Group as a possible carcinogen to humans. Below we discuss a case of acute hepatic and renal failure from HS use after daily ingestion for 24 days.

CASE DESCRIPTION/METHODS:

Patient is a 74-year-old-female with history of metastatic large cell neuroendocrine rectal tumor status post chemoradiation and resection of colon with ileostomy placement and vaginectomy in 2013. In February 2019, CT chest imaging showed multiple bilateral pulmonary nodules, the largest measuring 2.3 cm, with extensive lymphadenopathy and bilateral pleural effusions. In April 2019, patient presented to the hospital with acute encephalopathy with associated transaminitis, hyperbilirubinemia, coagulopathy, and acute renal failure. Subsequent testing included negative hemolysis workup, hepatic viral and autoimmune panel, and MRCP abdomen. Patient had not followed up with oncology aboutpulmonary nodules, and instead took HS 60mg TID for 4 weeks prior to presentation. The patient’s encephalopathy improved after several days of supportive care. She was followed in clinic by gastroenterology with eventual resolution of hepatorenal toxicity after multiple weeks with cessation of HS.

DISCUSSION:

HS has been proven to be ineffective as any form of cancer treatment, though patients continue take it due to false hopes of cure and its ability to induce a state of euphoria with neurotropic effects. Unfortunately, many patients develop fulminant hepatorenal toxicity, severe gastrointestinal hemorrhage, hepatic necrosis, and autolysis of kidneys instead. The diagnosis of HS toxicity is often overlooked, and if diagnosed in a timely manner, extensive and costly workups could be avoided. Cancer patients with poor prognosis or limited chemotherapeutic options remain at risk for falling prey to seemingly benign naturalistic remedies. This population must be better educated on the dangers of hydrazine sulfate.
2227 Herpes Simplex Virus - 1 Related Acute Fulminant Liver Failure Successfully Treated With Medical Therapy
Authors: Kataria Archish , Speeg Kermit V. , Gonzalez Lisandro
Published: October 2019

INTRODUCTION:

Herpes simplex virus (HSV 1&2) is an uncommon cause of asymptomatic hepatitis and acute liver failure. Fatal disease often occurs in immunocompromised adults but is a rare event in immunocompetent patients. Early suspicion of infection is imperative as late diagnosis and treatment carries poor prognosis and high mortality. We describe a case of acute hepatic failure from HSV-1 virus in an immunocompetent adult successfully treated with medical therapy without need for liver transplantation.

CASE DESCRIPTION/METHODS:

A 55-year-old diabetic male with recent hospitalization for necrotizing fasciitis of the groin (discharged on amoxicillin/clavulanate 5 days ago) presented to the hospital with fever, vomiting, abdominal pain and was found to have severe elevation in liver enzymes. Patient denied prior history of liver disease. Physical exam was notable for fever: 102 degree F, tachycardia, multiple small circular lesions on the oral mucosa and tender hepatomegaly. Liver tests at baseline were normal and on admission showed: AST 1317 U/L (normal:< 32 U/L), ALT 966 U/L (normal:< 36 U/L), total bilirubin 0.4mg/dL (normal:0.2-1.2 mg/dL), alkaline phosphatase 86 U/L (normal:45-117 U/L) and INR 1.7 (normal:0.8-1.2). Urine drug screen was negative of acetaminophen. Imaging excluded hepatic venous thrombosis. Laboratory tests for viral and autoimmune hepatitis, wilson’s disease were negative. Patient was empirically started on IV acyclovir given concern for herpes hepatitis based on oral exam. Diagnosis was confirmed with a positive blood and oral swab HSV DNA PCR and a liver biopsy (Figures 1 and 2) showing herpes simplex viral inclusions. Due to progressive worsening of liver tests and patient’s condition while on treatment, intravenous immunoglobulin (IVIG) therapy was added eventually leading to clinical and laboratory signs of improvement (Table 1). He was discharged on an oral course of valganciclovir and continues to do well on follow up visit.

DISCUSSION:

Fulminant liver failure from herpes virus has been infrequently reported in immunocompetent adults and usually represents disseminated infection. It is a potentially treatable condition that requires high degree of diagnostic suspicion as presenting symptoms can be non-specific. Early empiric treatment with acyclovir while awaiting HSV PCR or liver biopsy has been shown to improve outcomes and decrease need for liver transplantation. We herein describe a novel approach to treat HSV hepatic failure with combination therapy of acyclovir and IVIG.
2228 A Rare Report of Pantoprazole-Induced Acute Hepatocellular and Cholestatic Hepatitis
Authors: Kataria Archish , Hubbard Hopethe , Page Robert
Published: October 2019

INTRODUCTION:

Pantoprazole is a widely used proton pump inhibitor that is used to treat variety of acid related disorders. It is well tolerated with excellent safety profile. Mild transient elevations in serum aminotransferase can be seen with pantoprazole use that resolves without dose modification. Pantoprazole induced clinically apparent liver injury on the other hand is exceedingly rare.

CASE DESCRIPTION/METHODS:

A 47-year-old woman with no prior liver disease was referred to the hospital for acute hepatitis. She started pantoprazole 2 months prior to admission for gastritis. Patient had nausea, fatigue, abdominal pain, jaundice and dark urine 2 weeks after starting pantoprazole and stopped it a week prior to hospital admission. She denied alcohol use. Her current medications were levothyroxine, enalapril/hydrochlorothiazide and aspirin and no use of herbal supplements. Physical exam was notable for conjunctival icterus. Baseline liver enzymes were normal. Labs on admission were: AST 681 U/L (< 32 U/L), ALT 2100 U/L (< 36 U/L), total bilirubin 12.3 mg/dL (0.2-1.2 mg/dL), direct bilirubin 8.3 mg/dL (< 0.5 mg/dL), alkaline phosphatase 96 U/L (45-117 U/L) and INR 1.9 (0.8-1.2). Urine drug screen was negative for acetaminophen toxicity. Imaging excluded biliary obstruction and hepatic venous thrombosis. Laboratory investigations for viral and autoimmune hepatitis, wilson’s disease and hemochromatosis were negative. Liver biopsy (Figure 1) reported portal areas with edema and a minimal amount of inflammation composed of mixed lymphocytes, occasional eosinophils and neutrophils. The lobular parenchyma showed cholestasis with lymphocytic and neutrophilic inflammation. Patient noted improvement of symptoms with supportive care (Table 1). Two months after stopping pantoprazole, liver enzymes were normal.

DISCUSSION:

DILI (drug-induced liver injury) is a significant cause of acute hepatitis. To our knowledge, only 7 case reports of pantoprazole induced liver injury have been published. The timing of starting pantoprazole prior to acute hepatitis, negative serologies, and normalized liver enzymes after stopping suggests pantoprazole as the cause of DILI. Based on its pharmacokinetics, timeline and pattern of insult, an idiosyncratic reaction has been suggested as the mechanism for hepatotoxicity. We have described a rare case of acute mixed (hepatocellular and cholestatic) liver injury from pantoprazole use suggesting its consideration in the differential diagnosis of DILI.
2236 Metastatic Melanoma: A Rare Case of Liver Involvement
Authors: Marusca Georgiana , Selim Ranya
Published: October 2019

INTRODUCTION:

Literature shows that cutaneous melanoma can metastasize hematogenously or via the lymphatic system. The reported median time course for distant metastases to develop for a tumor recurrence is 25 months. The anatomical location of the primary melanoma is considered the most important factor for the location of metastasis: 30% of patients with melanoma on the trunk or upper extremities develop direct distant metastases.

CASE DESCRIPTION/METHODS:

We present the case of a 68-year-old man with a history of diverticulosis, remote alcohol abuse and recently diagnosed melanoma of the scalp, who presented to the emergency room with complaints of sudden generalized abdominal discomfort, malaise, decreased appetite, fatigue and dark urine for one week. Eight months prior, the patient underwent a wide excision of a malignant melanoma on his left flank. In the emergency room, the patient was hemodynamically stable. Physical exam was notable for distended abdomen with dullness to percussion at the peripheries. Labs showed elevated liver function tests with ALT 98 IU/L, AST 144 IU/L, total bilirubin of 4.3 mg/dL, direct bilirubin of 2.8 mg/dL and INR of 1.46. Abdominal US showed right portal vein thrombosis. An abdominal MRI demonstrated hepatosplenomegaly with multiple lesions in the spleen and liver, small amount of perihepatic and perisplenic fluid with small right-sided pleural effusion as well as a 17 mm subcutaneous nodule in the left chest concerning for metastatic disease. A subsequent liver biopsy was consistent with metastatic melanoma. His hospital course was complicated by acute renal failure in the setting of hyperuricemia secondary to tumor lysis requiring dialysis. Unfortunately, he was unable to tolerate dialysis due to hypotension. Due to his overall clinical status, a family meeting was arranged and the decision was made to pursue hospice care. A few hours after enrolling in hospice, the patient expired.

DISCUSSION:

Cutaneous melanoma metastasizes to the lungs, lymph nodes and soft tissue, with only 10-20% of cases being liver metastasis. Liver metastasis, an important prognostic factor, occur more quickly after the primary lesion is detected. The median survival for patients with stage IV metastatic melanoma has been reported to be one year. Hepatic resection is considered the only potentially curative treatment for patients with liver metastases, with only 2-7% of patients being surgical candidates. Patients who are not surgical candidates can undergo systemic and/or regional therapies.
2242 I'm Starving: A Rare Case of Liver Autophagocytosis Secondary to Anorexia Nervosa
Authors: Rozenshteyn Frederick , Rastogi Neelesh , Collins Kelsey , Nagirimadugu Ankita , Ascunce Gil
Published: October 2019

INTRODUCTION:

Anorexia nervosa is a common eating disorder associated with fear of gaining weight and when severe can cause life-threatening complications. While mild elevations in liver enzymes can occur in anorexia, severe liver injury is rarely reported. Autophagy is a liposomal catabolic pathway that is crucial for cell survival under stressful conditions. We present the case of a young female with anorexia nervosa with severe malnutrition found to have significantly elevated liver enzymes concerning for autophagocytosis of the liver.

CASE DESCRIPTION/METHODS:

A 19-year-old female with a history of severe anorexia nervosa (BMI 11.5) presented with 3 days of abdominal pain. Basic lab testing was done which revealed significant elevations in LFTs in a hepatocellular pattern with both AST and ALT above 1000. The patient underwent abdominal imaging with no major abnormalities found and viral, drug-induced, and autoimmune causes were also ruled out. However, her LFTs continued to uptrend with a peak AST of 6328 and ALT of 5606 and her hospital course was complicated by hypotension requiring ICU care. Due to severe nutritional deficits and inability to tolerate oral intake, the decision was made to initiate supplemental nutrition first with TPN and subsequently with enteral feeding through a PEJ. Following nutrition initiation, the patient’s liver enzymes started to down-trend and ultimately normalized within a couple weeks of nutritional support. Therefore, it was determined that the patient’s acute liver elevations and abdominal pain were likely secondary to liver autophagocytosis in the setting of severe starvation from anorexia nervosa. Due to the normalization of her LFTS, liver biopsy was not pursued. The patient improved and was discharged to a specialized eating disorder rehabilitation center.

DISCUSSION:

Autophagy is a physiologic process the body activates under episodes of severe stress which can include severe malnutrition. While mild liver enzyme elevation can occur, there are only a few reported cases of profound LFT elevation such as in this patient. Initiation of enteral/parenteral nutrition appears to improve liver injury and stop autophagy. Therefore, careful implementation of nutritional support and close monitoring of LFTs is essential in anorexic patients with liver injury to help treat the underlying condition, avoid unnecessary invasive diagnostic testing, and prevent further autophagy.