bluuebunny/crossref_metadata_2025 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.
2478 Approach to a Foreign Body (Fish Bone) Resulting in a Liver Abscess With Antibiotic-Evading Filamentation of Bacterial Organisms Resembling Fungal Hyphae
Authors: Membreno Raul F. , Pena Miren M. , Chorath Kevin T. , Kang William D. , Membreno Fernando
Published: October 2019

INTRODUCTION:

Foreign bodies that are ingested may migrate from the gastrointestinal (GI) tract into different organs of the body and cause a variety of clinical manifestations including fever of unknown origin. In this vignette we describe the case of a transmural fish bone migration from the stomach to the liver causing a recurrent liver abscess.

CASE DESCRIPTION/METHODS:

A 76 year old Caucasian male with a past medical history of hypertension presented to the hospital with a left liver lobe abscess that was aspirated six years ago. All cultures at that time were negative and apparently resolved with antibiotics. About two months prior to his hospital admission, he started developing subjective fevers and malaise, with CT Scan of the abdomen showing a possible liver mass in the left lobe. He underwent a liver biopsy of this lesion that showed multiple caseating granulomas. Infectious work up was negative. Despite broad spectrum antibiotics, the patient continued with low grade fevers. Pancultures were negative and a left liver lobectomy and cholecystectomy was pefomed. CT imaging of the liver showed a multiloculated 7.2 cm lesion in the left lobe of the liver consistent with an abscess, with a linear foreign body which extended from the stomach into the left lobe liver lesion (Figure 1). During surgery, the foreign body was identified and removed. Surgical histology of the foreign body showed bone fragments lined by osteocytes and calcifications, which was identified as a “fish bone.” Furthermore, the left liver lobe showed prominent granulomatous inflammation with necrosis and microabscesses with no evidence of malignancy. Special stain GMS-F was positive for filamentous organisms that most closely resembled nocardia or streptomyces (Figure 2).

DISCUSSION:

This is the first case report showing filamentation of bacteria resembling a fungal infection causing a liver abscess due to a fish bone that penetrated the left lobe of the liver. Filamentation is a bacterial defense mechanism against eradication by antibiotics, which explains failure of resolution of the infection with antimicrobials. Studies have reported incomplete bacterial replication leading to the appearance of filamentation resembling fungal species. This phenomenon should be considered by clinicians when treating challenging liver foreign body abscesses.
2504 Successful Budesonide Monotherapy for Autoimmune Hepatitis/Hepatic Sarcoidosis Overlap Syndrome in a Patient With Drug Reaction With Eosinophilia and Systemic Symptoms (DRESS) From Azathioprine
Authors: Goff Sara , Liaquat Hammad , Jhala Nirag , Kiyani Amirali
Published: October 2019

INTRODUCTION:

Drug reaction with eosinophilia and systemic symptoms (DRESS) is considered a severe idiosyncratic drug hypersensitivity which can be fatal if not promptly diagnosed and treated. Here, we describe a case of hepatic sarcoidosis and autoimmune hepatitis (AIH) overlap syndrome who was found to have DRESS from taking azathioprine (AZA).

CASE DESCRIPTION/METHODS:

A 63-year-old African-American female with a diagnosis of AIH and hepatic sarcoidosis overlap based on liver biopsy (chronic hepatitis with moderate activity (grade 2/4), mild fibrosis (stage 1/4), non-caseating granulomata, and interface hepatitis with dense lymphoplasmacytic infiltration) (Figure 1), positive ANA (1:160), and elevated IgG (2140) was started on treatment with AZA and budesonide (intolerance to prednisone). She had normal TPMT activity. Patient stopped taking AZA few days later because of feeling “jittery.” She presented to the ED five weeks later with lip swelling and desquamating rash and was discharged with antihistamines. She returned to the ED two days later with worsening rash, fever, and dyspnea. Labs showed leukocytosis, eosinophilia (56.1%, 13,400/mm3), acute hepatitis, acute kidney injury, and respiratory distress with concern for DRESS. Her initial LFTs were ALT 96, AST 65, and Alk Phos 633, which were increased from her baseline. Imaging ruled out biliary obstruction and bone marrow biopsy was negative for myeloproliferative disorders such as hypereosinophilic syndrome. She was diagnosed with DRESS secondary to AZA and was treated with a short course of prednisone with transition to budesonide monotherapy. Eosinophilia and other organ dysfunction resolved. Her most recent LFTs are ALT 37, AST 42, and Alk Phos 277.

DISCUSSION:

While AZA hypersensitivity syndrome is a well-known disorder presenting with drug eruption and flu-like symptoms, to our knowledge this is the first case of DRESS secondary to AZA. Additionally, this occurred in the setting of hepatic sarcoidosis and AIH overlap syndrome which itself is a rare entity. DRESS is characterized by a long latency time between exposure and disease (2-8 weeks) and presents with end organ dysfunction requiring steroid treatment. Based on the European Registry of Severe Cutaneous Adverse Reactions (RegiSCAR) scoring system, our patient's score was 7, where a score of 6 or higher is considered definite for DRESS. This case also stresses the value of budesonide monotherapy for treating AIH and hepatic sarcoidosis overlap when other first line medications cannot be used.
2507 Amoebic Liver Abscess With Synchronous Proctitis—Presenting With Massive Lower Gastrointestinal Bleeding: A Case Report of a Rare Presentation of Amebiasis
Authors: Nalinthassanai Nutbordee , Chaiteerakij Roongruedee , Sanpavat Anapat
Published: October 2019

INTRODUCTION:

Amoebic colitis and liver abscess caused by Entamoeba histolytica is one of the most important enteropathogen, leading to 40-73 per 100,000 deaths annually worldwide.1,2 Transmission generally occurs by fecal-oral route and during male homosexual activity.3 Colonic involvement was seen in 58% patients with amoebic liver abscess, but only 7% presents with diarrhea and bleeding.4

CASE DESCRIPTION/METHODS:

A 25-year-old MSM patient with newly diagnosed HIV infection had persistent low grade fever and diarrhea 3-4 times/day for 1 month. He sometimes noticed a small amount of fresh blood in the stool and had mild epigastric discomfort for 1 week. He had worsening abdominal pain, radiating to back and passed 1000 mL fresh blood per rectum. Physical examination showed fever (38.9oC), pallor and markedly enlarged liver with tenderness. Lab showed hemoglobin 6.1 g/dl, white blood cell count 25350 cell/ul (N 89%, L 4%, M 7%), total/direct bilirubin 1.8/1.1 mg/dL, SGOT/SGPT 353/111 U/L, albumin/globulin 2.7/6.6 g/dL, ALP 436 U/L. Colonoscopy showed a 3-cm hemi-circumferential ulcer covered by blood clots and exudative materials with friable mucosa at rectum (Figure 1). Biopsy was done at the ulcer. Abdominal CT showed a 15-cm rim-enhancing cystic lesion with internal septation occupying right liver lobe. Capsule disruption at segment VIII/V anterior aspect of liver was noted (Figure 2). Percutaneous drainage was done and found markedly turbid gray pus 500 ml. Histopathologic findings revealed severe active proctitis with ulcer and few trophozoites ingested red blood cells on the surface, compatible with amoebic proctitis (Figure 3a). The pus aspirated from the abscess showed Charcot Leyden crystals (Figure 3b). Serum for E. histolytica antigen and PCR were both positive. The final diagnosis was amoebic liver abscess with proctitis. The patient was treated with metronidazole for 2 months, with a decreased size of abscess on follow-up CT. No recurrent bleeding occurred.

DISCUSSION:

Although synchronous colonic lesions in amoebic liver abscess are present in over 50% patient, diarrhea and bleeding are uncommon, without unclear reasons. Caecum is the most commonly affected site (70%). Left sided ulcers are seen in elderly and those presenting with diarrhea. Oral or intravenous metronidazole or tinidazole leads to rapid clinical improvement of invasive amoebic colitis and liver abscess. Large abscess (>7.7 cm) with abnormal liver tests were predictive of drainage intervention.
2531 A Bloody Mess: A Rare Cause of Ascites From a Different Field of Medicine
Authors: Simmelink Andrew , Spence Linsey , Brown Jubilee , Kasper Rebecca E. , Scobey Martin
Published: October 2019

INTRODUCTION:

After exclusion of a traumatic etiology, the development of progressive bloody ascites in a patient usually portends an ominous diagnosis. We present a rare, benign case of progressive bloody ascites due to a gynecologic cause.

CASE DESCRIPTION/METHODS:

A 40 yo healthy African American lady presented with 3 months of worsening abdominal enlargement and nausea. Physical exam and CT scan of the abdomen revealed massive ascites but were otherwise normal. Bloodwork showed a Hgb of 5.7 gm/dL, and normal PLT. Ferritin level, liver panel, hepatitis and HIV studies were all normal. Paracentesis showed 26,000 RBCs, 88 nucleated cells, 79 cholesterol crystals in pigmented macrophages, a SAAG of 0.5, a fluid protein of 5.5 g/dL, and negative cytology. Further testing showed a negative quantiferon-gold along with a normal CEA level and ANA. A CA-125 was mildly elevated at 82 U/mL. CT scan post-paracentesis showed diffusely thickened peritoneum without nodularity, and a 5 cm right pelvic cystic mass. Diagnostic laparoscopy found 4L bloody ascites and complete fibrotic abdominal structure encasement. Peritoneal biopsies and right oophorectomy showed endometriosis. She was treated with a high dose steroid taper and leuprolide with resolution of her ascites.

DISCUSSION:

Our case is one of a few reports describing endometriosis inducing encapsulating peritoneal sclerosis (EPS), and is the first to show asymptomatic endometriosis as the etiology. (EPS) is a syndrome of intestinal obstruction from diffuse thickened peritoneum. Typical symptoms are intermittent abdominal pain with nausea and vomiting related to the intermittent obstruction and reduced gut motility. Symptoms average 3.9 years before diagnosis. Primary EPS is extremely rare and while secondary EPS is usually due to chronic peritoneal dialysis (PD). Other causes include malignancy, infections, and various rheumatologic conditions. Management is based on the offending cause (e.g., stopping PD) but is largely empiric, based on case reports and series utilizing prednisolone and/or tamoxifen.
2551 A Case of Auto-Brewery Syndrome: Probably an Under-Diagnosed Medical Condition
Authors: Malik Fahad , Wickremesinghe Prasanna
Published: October 2019

INTRODUCTION:

Auto-brewery syndrome (ABS), also known as gut fermentation syndrome, is a rarely diagnosed medical condition in which the ingestion of carbohydrates results in endogenous alcohol production. The patient in this case report had fungal yeast forms in the upper small bowel and cecum, which likely fermented carbohydrates to alcohol. Treatment with antifungal agents allowed subsequent ingestion of carbohydrates without symptoms. He had been exposed to a prolonged course of antibiotics before this occurred. We postulate that the antibiotic altered his gut microbiome, allowing fungal growth.

CASE DESCRIPTION/METHODS:

A healthy 46-year-old male who complained of memory loss, mental changes, and episodes of depression for over three years after antibiotic therapy (cephalexin) for a traumatic thumb injury. He was given a carbohydrate meal and blood alcohol level became elevated to 57 mg/dL without any exogenous alcohol while under observation. Saccharomyces cerevisiae (brewer's yeast) and Saccharomyces boulardii were detected in his stool. To investigate further, an upper and lower endoscopy were performed. Fungal cultures from cecal secretions grew Candida Albicans and Candida Parapsilosis. He was started on a carbohydrate free diet and intravenous micafungin therapy for six weeks after which secretions from a repeat endoscopy showed absence of fungal growth. He was also started on a probiotic to competitively inhibit fungal growth in his gut. A repeat carbohydrate challenge test was negative for endogenous alcohol production now. Approximately 2 years later, he remains asymptomatic and has resumed his previous lifestyle, including eating a normal diet.

DISCUSSION:

A large Middle Eastern study involving 1400 subjects who were teetotalers detected very small endogenously produced alcohol levels using gas chromatography and mass spectrometry. Saccharomyces cerevisiae which can convert carbohydrates to endogenous alcohol, was detected in our patient's stool. This fungus uses acetate for anaerobic alcohol fermentation. It should be noted that the earliest symptoms of ABS may be mood changes, delirium, and brain fog rather than the medical manifestation of alcohol inebriation. A multidisciplinary approach with early psychiatrist involvement is suggested. Any patient denying alcohol ingestion but who has elevated blood alcohol levels or positive breathalyzer results should be investigated for ABS. This could be a treatable condition with dietary modifications, appropriate antifungal therapy and probiotic use.
2619 Persistently Elevated Intestinal Alkaline Phosphatase in an Individual With AB Blood Group: A Rare Presentation
Authors: Mantri Nikhitha , Miranda Jeirym , Jyala Abhilasha , Badipatla Kanthi Rekha , Nayudu Suresh Kumar
Published: October 2019

INTRODUCTION:

Elevated alkaline phosphatase (ALP) is seen in hepatobiliary and bone diseases. However, elevated intestinal ALP has been reported in the literature in association with blood group B or O, whose clinical significance is not known. We present a rare case of persistently elevated intestinal ALP in an individual with blood group AB.

CASE DESCRIPTION/METHODS:

A 56-year-old female presented to our ambulatory clinic to establish health care. Her medical history included peptic ulcer disease and dyslipidemia. She underwent appendectomy in childhood and cholecystectomy around 20 years ago. she denied smoking, alcohol consumption or use of recreational drugs. Family history was significant for type 1 DM in mother and brother. Her only medication included ranitidine. General and systemic examination was normal. Her initial labs were normal except elevated alkaline phosphatase of 359 units/liter. The patient was seen by the gastroenterology team. Further laboratory workup including viral hepatitis, metabolic and autoimmune markers of liver disease were normal. Gamma-glutamyltransferase (GGT), Vitamin D and parathyroid profile were normal. Ultrasound abdomen did not show any significant lesion in the liver or biliary tree. As no evidence of liver disease was noted isoenzyme electrophoresis of ALP was ordered which showed elevated intestinal ALP fraction of 64% (normal 1–24%). Patient blood group was tested which showed group AB. On further follow up in gastroenterology clinic persistently elevated ALP noted.

DISCUSSION:

Elevated intestinal alkaline phosphatase has been reported in maintaining of gut hemostasis and elevated levels noted in various pathological conditions of the gastrointestinal tract. Meal induced elevation of intestinal ALP has been proposed as a treatment modality to preserve gut hemostasis and minimize inflammation. To the best of our knowledge, our case is an extremely rare presentation of elevated intestinal ALP in an individual with blood group AB. Further molecular and genetic studies in the future may help elaborate functions and therapeutic intervention of intestinal ALP
2629 A Benign Tumor With a Malignant Course
Authors: Zackria Rasiq , Hwang Andrew W.
Published: October 2019

INTRODUCTION:

Mesenteric fibromatosis is a rare, locally invasive, non-metastasizing intra-abdominal fibroma with a high rate of recurrence. These tumors pose a diagnostic and therapeutic challenge due to its rarity and wide-ranging clinical presentation.

CASE DESCRIPTION/METHODS:

A 43-year-old man with a medical history of hypertension and no prior surgeries who presented with acutely worsening diffuse abdominal pain with associated bloating, nausea, and vomiting for 2 days. Physical exam revealed abdominal tenderness with deep palpation with the absence of peritoneal signs. MRI revealed a large liver mass with central necrosis suspicious of hepatocellular carcinoma, however, the biopsy was consistent with a hemangioma. Colonoscopy visualized a colonic mass with difficulty maneuvering the scope through the entire colon raising concern for primary versus metastatic colon cancer. Tumor markers were unremarkable. Gastrografin radiography demonstrated a high-grade obstructing annular mucosal lesion at the splenic flexure. He underwent left hemicolectomy with colostomy placement. Pathology demonstrated a spindle cell neoplasm with a fascicular architecture surrounded by abundant collagen fibers with no necrosis. Further histological staining was positive for vimentin, beta-catenin, and desmin conforming to mesenteric fibromatosis.

DISCUSSION:

Mesenteric fibromatosis is part of the clinical-pathologic spectrum of deep fibromatosis encompassing a group of benign fibroproliferative processes that are locally aggressive and have the capacity to infiltrate or recur, but not metastasize. Most cases manifest sporadically, however, there is a link with familial adenomatous polyposis (FAP), specifically Gardner syndrome. In addition, they can be locally aggressive showing recurrence even after excision. Mesenteric fibromatosis may be confused radiologically and histologically with a gastrointestinal stromal tumor (GIST), however immunohistochemical profile is helpful in differentiation. Mesenteric fibromatosis is typically negative for CD34 and CD117, which are almost universally positive in GISTs. This case illustrates the difficulty in diagnosing mesenteric fibromatosis, and a secondary lesion can complicate diagnosis. Mesenteric fibromatosis with intestinal involvement can be easily confused with other primary gastrointestinal tumors, especially with mesenchymal origin such as GIST. Hence, it should be considered in the differential diagnosis of mechanical intestinal obstruction as well as intra-abdominal masses.
2662 A Unique Presentation of Concurrent Duodenal and Peritoneal Metastasis From Head and Neck Cancer
Authors: Ahmed Ahmed , Nasir Umair M. , Delle Donna Paul , Ahmed Shahida , Lenza Christopher
Published: October 2019

INTRODUCTION:

It is very rare for head and neck squamous cell carcinoma (HNSCC) to metastasize to the duodenum or the peritoneum. Only a few cases have been reported for each metastatic site, however, there have been no reported cases of concurrent duodenal and peritoneal metastasis (PM) from HNSCC. Such a presentation enhances the literature on HNSCC and illustrates the need for further studies to understand the mechanism of HNSCC metastasis.

CASE DESCRIPTION/METHODS:

68-year-old male with history of HNSCC presented with worsening nausea, postprandial vomiting, and abdominal pain for over 2 months. 3 months prior he was worked up for several postauricular lumps, with computerized tomography (CT) scan showing a supraglottic mass and excisional biopsy of a nodule confirming metastatic HNSCC. Imaging upon admission showed new peritoneal carcinomatosis and worsening diffuse lymphadenopathy (LAD). Esophagogastroduodenoscopy (EGD) showed a large nodular lesion occupying one third of the lumen circumference. Like the neck nodule, biopsies of the mass showed metastatic, poorly differentiated SCC that was strongly positive for p63 and p16. The patient was not deemed a candidate for chemotherapy due to poor prognosis and poor functional status and was eventually discharged on home hospice.

DISCUSSION:

We hereby described the first case of concurrent duodenal and peritoneal metastasis from a HNSCC, with both being independently rare sites of metastasis. The patient’s history of chronic tobacco use increased his risk of SCC. Chemotherapy and peritoneal excision, in certain cases, have shown to be a viable treatment option and have a beneficial impact on survival and quality of life. In select cases of obstruction, palliative stenting can be an option to improve quality of life. Due to rapid disease progression and the patient’s poor functional status, these treatment options were deemed not feasible. There are many speculated mechanisms regarding intestinal and PM such as direct extension, tumor rupture or trauma leading to seeding of the peritoneum, hematogenous dissemination, and even via the lymphatics. In our case, the patient did not have any abdominal surgeries and had multiple metastatic sites, including the duodenum, making hematogenous or lymphatic spread more likely. In any case, further studies are needed to understand the mechanism involved in HNSCC metastasis.
2670 A Highly Lethal Case of Gastric Pneumatosis
Authors: Multani Monica , Kantor Micaella , Anyu Tse , Sobrado Javier
Published: October 2019

INTRODUCTION:

While gas can accumulate in the intestinal wall at multiple sites, the stomach is the rarest site because of its acidity and very efficient mucosal barrier. In has been proposed in one retrospective study that 46% of their cases involved the colon, 27% the small bowel, 14% in whom the whole gastrointestinal tract was infected including the portal venous system, 7% the small bowel with the colon, and only 5% the stomach as in our case.

CASE DESCRIPTION/METHODS:

An 83 year old male with history of coronary artery disease, diabetes mellitus, bullous pemphigoid and smoking history presented for shortness of breath over one week. Patient was tachypnic and using accessory muscles on exam. During his hospital course, he had abdominal pain. CT Scan showed fluid filled distal esophagus, distended stomach, air-fluid levels and peripheral lucencies concerning for intramural gas. Nasogastric tube put out a large amount of red emesis after which he expired.

DISCUSSION:

There are two distinct pathological processes involved in air within the lumen of the stomach which is termed gastrointestinal pneumatosis. The first type is called gastric emphysema which often has a benign course and constitutes 9% of all gastrointestinal pneumatosis cases. Gastric emphysema is classified as traumatic, pulmonary or obstructive types. There have only been 5 recorded cases of gastric emphysema with a pulmonary etiology and four of those cases were secondary to bullous emphysema. The second type is emphysematous gastritis which involves gas forming organisms including Pseudomonas aeruginosa, Candida albicans, and Staphylococcus aureus. The risk factors for this dangerous type of gastrointestinal pneumatosis include a history of abdominal surgery or manipulation, bowel infarction/ischemia, immunosuppression which include disease processes including diabetes, alcohol abuse or corrosive material ingestion. There is a mortality of 16% in those receiving surgical treatment versus 6% who received only conservative treatment. In stable patients, improvement in clinical condition or emphysema resolution within 3 days occurs with nasogastric catheter decompression as attempted in our case. This becomes important in identifying between gastric emphysema, a relatively benign course, or emphysematous gastritis, which has a poor prognosis. As time becomes an increasingly prognostic factor for these individuals, differentiation in treatments based on type could lead to faster implementation which could ultimately help to prevent demise.