bluuebunny/crossref_metadata_2025_split huggingface dataset:
1275 Intestinal-Type Intracholecystic Papillary Tubular Neoplasm (ICPN) Presenting as Acute Hemorrhagic Cholecystitis
Authors: Gill Jasmeet , Sannapaneni Shilpa , Feldman Mark 
Published: October 2019

INTRODUCTION:
The term Intracholecystic Papillary Tubular Neoplasm (ICPN) was coined by Adsay et al (2012). ICPN includes mass-forming (papillary or polypoid), intramucosal and preinvasive gallbladder neoplasms larger than one cm in size.

CASE DESCRIPTION/METHODS:
A 73-year-old man presented with acute and sharp right upper quadrant abdominal pain for one day. He denied nausea, vomiting, diarrhea, fever or chills. Past medical history included COPD, non-Hodgkin lymphoma in remission and hypertension. He was not receiving anticoagulants or antiplatelet medications. On abdominal examination, there was marked tenderness in the right upper quadrant area with a positive Murphy's sign. Laboratory testing revealed leukocytosis of 22,000/µL with neutrophilia and normal liver chemistry tests and a serum lipase level. Abdominal ultrasonography showed a markedly distended gallbladder with mural thickening of up to 1.0 cm and numerous gallstones with a positive sonographic Murphy's sign. Non-contrast CT showed mixed attenuation suggesting hemorrhage into the gallbladder lumen and suggestive of acute hemorrhagic cholecystitis. He underwent open cholecystectomy. Surgical findings were a distended gallbladder with a thickened and inflamed wall. Half of the gallbladder lumen was occupied by stones and the remainder was filled with detached, friable, tan-red hemorrhagic material. Microscopic examination of several of detached fragments and intact mucosa showed an abundant ICPN of intestinal-type origin and a tubular-papillary growth pattern. There was marked acute and chronic inflammation of the gallbladder wall without mural perforation.

DISCUSSION:
ICPNs are usually seen in middle-aged individuals, with a 2- fold higher prevalence in women. Macroscopically, ICPNs are exophytic tumors: tubular/lobulated types are soft, friable and can get easily detached appearing as sludge or debris, while granular or papillary types are sessile and feathery filling the gallbladder lumen. ICPN exhibits a variety of cell lineages such as biliary (50%), gastric (25%), intestinal (8%, as in our patient), oncocytic (6%) and mixtures of lineages (∼10%). In our patient, the two most plausible pathogenic processes are either diagnosis of an incidental ICPN due to the associated acute calculus hemorrhagic cholecystitis or acute “acalculous” cholecystitis from tumor shedding and bleeding into the gallbladder lumen and causing cystic duct obstruction associated with asymptomatic (incidental) cholelithiasis.
1280 Biliary Sludge and Acute Pancreatitis Following Treatment of Sexually Transmitted Infection
Authors: Kwak Hakyong Gloria , Al Momani Laith , Bokhari Ali , Alomari Mohammad , Young Mark 
Published: October 2019

INTRODUCTION:
Ceftriaxone has been shown to cause transient biliary and cholecystic sludge evident on imaging in asymptomatic subjects. This sludge may result in obstruction, and hence, acute pancreatitis and cholecystitis. Here we report a case of acute pancreatitis presenting three days following treatment with ceftriaxone which may be attributed to this phenomenon. No other risk factors could be identified.

CASE DESCRIPTION/METHODS:
A 34-year-old male patient with no past medical history presented to the hospital with the complaint of stabbing epigastric abdominal pain radiating to the back with associated nausea and vomiting of one day duration. He was seen by his primary care doctor three days prior to presentation for urethral discharge and was given an intramuscular injection of 250 mg ceftriaxone in addition to 1 g oral azithromycin with complete resolution of symptoms. Physical examination was notable for epigastric tenderness without rebound tenderness or guarding and was otherwise normal. Laboratory work up was remarkable for WBC 15200 per microliter and lipase 1961 U/L. Liver function test, serum calcium, triglycerides, and IgG4 were all within normal limit. Computed tomography (CT) scan of the abdomen confirmed findings of acute pancreatitis with well-defined pancreatic borders. This was followed by a right upper quadrant ultrasound which showed biliary and cholecystic sludge. Upon further questioning the patient denied alcohol and tobacco use, recent travel, or any prior history of mumps and herpes simplex virus infections. Furthermore, no family history of acute pancreatitis was reported. The time frame of symptoms and the absence of other risk factors point towards a drug adverse reaction. The patient was managed conservatively and discharged the following day with significant clinical improvement.

DISCUSSION:
Younger patients, patients receiving a prolonged or larger dose and patients with impaired gallbladder emptying are at a greater risk for developing sludge secondary to ceftriaxone use. This is often asymptomatic with discontinuation of the drug usually resulting in resolution of this phenomenon. This case highlights the fact that ceftriaxone induced biliary sludge may rarely result in potentially serious adverse events including acute cholecystitis and pancreatitis.
1283 Liver Abscesses as a Complication of Side-to-Side Choledochoduodenostomy
Authors: Gonzaga Ernesto Robalino , Bashir Khawaja , Bhatti Hammad , Bracha Adam S. , Vanar Vishwas 
Published: October 2019

INTRODUCTION:
Side-to-side Choledochoduodenostomy (CDD) is a low morbidity surgical procedure commonly performed in the pre-Endoscopic Retrograde Cholangiopancreatography (ERCP) era to provide permanent relief and drainage of the common bile duct (CBD) in patients with retained or recurrent CBD stones, ampullary stenosis or stricture of the CBD. In side-to-side CDD, the distal bile duct becomes a poorly drained reservoir or “sump” as bile is unable to be drained from the area. Bile, stones, or debris may accumulate and lead to inflammation and bacterial growth. In many cases the inflammation in the sump can cause biliary obstruction, recurrent cholangitis, or pancreatitis, incurring the name “sump syndrome”. Although it is rare complication, sump syndrome carries a high mortality. In addition, hepatic abscesses have been reported as a complication.

CASE DESCRIPTION/METHODS:
A 61-year-old man, with a surgical history of cholecystectomy 15 years ago, presented with acute epigastric pain and severe sepsis. Laboratory studies suggested a biliary source of infection. A computed tomography showed multiple liver abscesses and pneumobilia. Urgent percutaneous biliary drainage with CBD sweep was performed. Cholangiogram demonstrated side-to-side CDD with residual debris in the sump. ERCP revealed choledolithiasis. Sphincterotomy and balloon extraction was performed to remove residual stones and debris. Distal biliary stricture was found and biliary stent was placed. The presentation of biliary disease complicated by liver abscess secondary to retrograde intrahepatic flow and history of side-to-side CDD with typical ERCP findings, established the diagnosis of sump syndrome.

DISCUSSION:
Hepatic abscesses have an incidence of 3.6 per 100,000 in the US. The majority of these cases are secondary to biliary tree disease, with pyogenic abscesses being the most common. There is limited data regarding abscesses as a late complication of sump syndrome, with few cases reported in the literature. This complication may be due to an obstruction between proximal CBD to duodenum, which leads to regurgitation of bile into intrahepatic bile ducts. Although the incidence of those abscesses is low, mortality remains as high as 12%. It is important to recognize the underlying cause of hepatic abscesses, as timely drainage and antibiotic therapy are essential to prevent significant morbidity. Furthermore, the restoration of normal bile flow from CBD via an ERCP is needed to eliminate any culprit source of infection and reduce recurrence.
1287 Metastatic Cholangiocarcinoma in a Healthy 27-Year-Old Man: A Case Report
Authors: Gribbin Caitlin , Niec Rachel , Mehta Amit , Tafesh Zaid , Wan David , Crawford Carl V. , Soumekh Amir 
Published: October 2019

INTRODUCTION:
Cholangiocarcinoma is a rare, aggressive cancer of the biliary tract most often found in men between the ages of 50 and 70. Risk factors include genetic disorders, such as Lynch syndrome and cystic fibrosis, primary hepatobiliary disease, such as primary sclerosing cholangitis (PSC), and chronic liver disease. This malignancy is highly uncommon in young individuals without PSC. Here we describe the case of a young man with cholangiocarcinoma with recurrent ascites in the setting of a portal vein thrombosis (PVT).

CASE DESCRIPTION/METHODS:
A 27-year-old man with a history of metastatic cholangiocarcinoma status-post hemihepatectomy with Roux-en-Y hepaticojejunostomy and adjuvant chemoradiation presented with rapidly accumulating ascites secondary to a portal vein thrombosis (PVT) discovered on abdominal imaging. A large-volume paracentesis was performed followed by thrombus recanalization and placement of a portal vein stent. The patient was discharged and presented two weeks later with recurrent ascites and evidence of occlusion of the portal vein stent as well as a new thrombus in the superior mesenteric vein (SMV) and a possible hepatic abscess seen on abdominal imaging. Clinical examination demonstrated a distended abdomen with a positive fluid wave. Laboratory studies revealed elevated white blood cell count (WBC) as well as Klebsiella, Candida, and gram-positive rods (GPR) bacteremia. The benefits versus risks of thrombectomy for recurrent PVT occlusion were considered as though this procedure could ameliorate ascites, the risk of rapid reocclusion was deemed to be high. Furthermore, the patient was at risk for intra- and post-operative complications due to an anticoagulant regimen and unstable clinical status. Ultimately, the patient received repeat LVP and remained on anticoagulants with no additional interventions for recurrent PVT. The patient and his family were discharged home with supportive care.

DISCUSSION:
Cholangiocarcinoma is a rare, aggressive malignancy of the biliary tract predominantly found in middle-aged men with a high risk of metastasis and a rapidly progressive course. The diagnosis is made on imaging and confirmed on biopsy. Treatment is dependent on disease staging and includes surgery, chemotherapy, and radiation. The disease is rapidly progressive and may predispose patients to pulmonary vein obstruction, leading to portal hypertension and associated sequelae secondary to tumor burden or PVT.
1295 A Unique BRIC Case With Response to Glucocorticoids
Authors: Lorio Eric , Valadez David , Alkhouri Naim , Loo Nicole 
Published: October 2019

INTRODUCTION:
Benign Recurrent Intrahepatic Cholestasis (BRIC) represents a rare class of autosomal recessive chronic cholestasis disorders usually presenting with intense pruritus and jaundice. BRIC type 2 (BRIC 2) results from a defect in the ABCB11 gene, which encodes the ATP dependent bile salt export pump (BSEP).
1
Here we report a unique case of a 27-year-old heterozygous female presenting with BRIC 2 and a response to steroids that may have implications for future treatment.

CASE DESCRIPTION/METHODS:
A 27-year-old female presented with 4 weeks of progressive jaundice, pruritus, and weakness. A prior episode one year earlier was notable for similar symptoms that resolved with supportive measures and a liver biopsy that showed nonspecific cholestasis with features suggesting extrahepatic duct obstruction. Presenting labs were notable for AST 63, ALT 74, total bilirubin (TB) 9.1, direct bilirubin (DB) 6.7, GGT 22, and alkaline phosphatase 227. Hepatitis serology, α-1 antitrypsin, hemochromatosis, and autoimmune workup were negative. Abdominal ultrasound and CT were unremarkable. MRCP revealed mild liver enlargement (19 cm), but no biliary stricture or dilatation. Liver biopsy showed extensive canalicular cholestasis with associated mild hepatocyte pericentral and perisinusoidal fibrosis. Her symptoms and labs continued to worsen, with TB peaking at 14.2 and DB at 11.5. On day 18, she was started on methylprednisolone 40 mg IV daily and achieved modest symptomatic improvement. On day 21, TB had downtrended to 8.1, and she was discharged home with a five-day course of prednisone. Genetic testing revealed heterozygosity for ABCB11.

DISCUSSION:
This report demonstrates a fairly typical presentation of BRIC, but in an older patient than typically seen. Though BRIC classically presents with its first episode prior to second decade of life, it can present in patients of any age.1 Our patient's heterozygosity for CFTR, NPHP4, and A1ATD (SERPINA1) may explain the severe nature of her disease expression, as heterozygosity in each of these genes has been associated with cholestasis. Her steroid response suggests a potentially novel treatment for BRIC and highlights the need for further consideration of steroid therapy in BSEP-related diseases.
1296 Elevated CA 19-9 Associated With Heavy Black Tea Consumption
Authors: Jiang Alice C. , Chan Edie Y. , Jakate Shriram , Shah Nikunj 
Published: October 2019

INTRODUCTION:
CA 19-9 is an important tumor marker for pancreatic cancer, but is also seen in non-malignant conditions such as cholelithiasis, cholangitis, pancreatitis, and other non-pancreaticobiliary conditions such as interstitial lung disease. This is a case of markedly elevated CA 19-9 found in the setting of a simple biliary cyst in the liver and heavy black tea consumption.

CASE DESCRIPTION/METHODS:
A 34-year-old woman with no significant past medical history presented to the ER for acute onset RUQ pain and nausea with vomiting. She had an ultrasound showing a 10.8 × 16.1 × 9.5 cm cystic lesion. Follow-up CT described the lesion as a large non-enhancing, hypoattenuating mass within the medial segment of the left lobe, most likely representing a cyst. AST, ALT, alkaline phosphatase, total bilirubin, albumin, INR and CBC were normal. AFP was <2.0 and CA 19-9 was 45,266 U/mL. On further interview, she reported drinking large amounts of black tea daily. She underwent a left lobectomy, with pathology showing simple biliary cyst. After surgery, her symptoms resolved and she reduced tea consumption to once weekly. Her CA 19-9 level decreased to 29 U/mL by 1 month post-resection, and remains normal 1 year later.

DISCUSSION:
The first case report of an association between heavy black tea consumption and elevated CA 19-9 hypothesized that the high gallated flavenoid content in black tea can impact a range of molecular targets influencing cell growth through the angiogenesis pathway.
1,2
In a study of 270 healthy individuals with heavy black tea consumption, 43.3% had elevated levels of CA 19-9 between 69-105 U/mL. The remainder of individuals had levels of 40 U/mL or lower. The proposed mechanism was stimulated production of CA 19-9 in epithelial tissues. While the exact mechanisms remain unknown, the presence of heavy black tea consumption should be considered in patients with elevated CA 19-9 in the absence of pancreaticobiliary disease because it is an easily modifiable factor.
1299 Sorafenib Induce Acute Pancreatitis: A Seldom Reported Side Effect
Authors: Then Eric O. , Lopez Michell , Ofosu Andrew , Are Vijay S. , Culliford Andrea , Gaduputi Vinaya 
Published: October 2019

INTRODUCTION:
Sorafenib is a multikinase inhibitor that has been used successfully in patients with hepatocellular carcinoma. Given its recent approval in the market, many of its side effects are still being uncovered. Here we present a rare case of Sorafenib induced acute pancreatitis.

CASE DESCRIPTION/METHODS:
The patient is a 60-year-old male with a medical history of hepatocellular carcinoma (stage IV with metastasis to bone), Hepatitis C virus, hypertension and rheumatoid arthritis who presented to the emergency department complaining of slowly progressive epigastric abdominal pain for 2 weeks. Of note the patient was started on Sorafenib 400 mg twice daily three weeks prior as an outpatient. Notable laboratory workup included a lipase of 189 U/L and a triglyceride level of 70 mg/dL. Notable imaging included a computed tomography of the abdomen which showed mild peri-pancreatic haziness consistent with acute pancreatitis and a ill defined hypodense lesion in the right hepatic lobe that was unchaged from previous imaging. The patient was then admitted under the impression of acute pancreatitis secondary to Sorafenib. Prior to reaching this diagnosis, alternate etiologies of acute pancreatitis were ruled out. This included ingestion of alcohol, hypertrigliceridemia, cholelithiasis and other medications. During his admission the patients diet was held and he was given early intravenous fluid resuscitation. Two days later his abdominal pain resolved and he was safely discharged home with follow up appointments. On follow up visit the patient was restarted on Soraenib at a lower dose (200 mg twice daily) with no recurrence of symptoms.

DISCUSSION:
Sorafenib induced pancreatitis is a rare complication that has seldom been reported in the literature and whose pathogenesis has not been completely elucidated. One theory suggests that it results from pancreatic ischemia that occurs secondary to sorafenib's anti-androgenic effect on vascular endothelial growth factor (VEGRF). A second theory suggests that sorafenib's effect on gastrointestinal dysmotility results in reflux of duodenal contents into the pancreatic duct. Diagnosis of sorafenib induced pancreatitis can be done after ruling out secondary causes of pancreatitis (ie: alcohol, hypertriglyceridemia). Treatment of sorafenib induced pancreatitis includes cessation of the offending agent. There have been reported cases where sorafenib was resumed at a lower dose after initial cessation, with no clinical sequelae as was the case with our patient.
1300 Disconnected Pancreatic Duct Syndrome a Rare Complication
Authors: Truscello David , Pandey Nishi , Shah Maulik , Itidiare Michael , Chiesa Drew J. , DeRosa Justin 
Published: October 2019

INTRODUCTION:
What follows is a case study of a patient with a pancreatic pseudocyst who developed a complication of Disconnected Pancreatic Duct Syndrome (DPDS). Pseudocysts are a collection of fluid and tissue that forms as result of trauma or repeated spells of pancreatitis. A rare complication of these cysts is disruption of the pancreatic duct termed (DPDS). This case report will discuss the diagnosis and management DPDS along with highlighting the use of endoscopic stenting for the management.

CASE DESCRIPTION/METHODS:
The patient is a 49-year-old male who presented to the hospital with sudden onset epigastric pain and distention. His past medical history includes chronic pancreatitis, and alcoholic cirrhosis with ascites, requiring large volume paracenteses. On admission, the patient's physical exam was remarkable for diffuse abdominal tenderness and ascites. Labwork was significant for a lipase of 329 U/L, albumin of 2.5 U/L, and a hemoglobin of 8.4 g/dL. Given the patient's abdominal tenderness, a paracentesis was performed with a resulting cell count concerning for Spontaneous Bacterial Peritonitis. A week later, patient had marked improvement in his abdominal exam. However, a CBC drawn on day 7 revealed a hemoglobin of 6.9 g/dL. A CT scan was ordered revealing an 8 cm inflammatory mass/pseudocyst in the head of the pancreas compressing the main portal vein. Another paracentesis was performed which showed high amylase consistent with a pancreatic source. Given these findings, GI performed a magnetic resonance cholangiopancreatography which showed pancreatic duct disconnection complicated by a large 6.4 × 5.5 cm cystic collection at the pancreatic head. An ERCP was performed with the implementation of a lumen apposing stent.

DISCUSSION:
DPDS is usually diagnosed with contrast enhanced CT which shows an absence of the pancreatic neck, body or tail. ERCP is usually able to show cut-off of the pancreatic duct or extravasation of contrast. ERCP with stenting is one minimally invasive intervention that has shown to reduce the recurrence rate of fluid collection. There are two challenges that are commonly encountered: high rates of stent migration and inability to pass a stent between the two disconnected ends of the duct. Due to the fact that there is still a lot that is not known about this disease state the consensus on optimal management remains to be determined. DPDS is a disease state that can be difficult to manage which is why early identification is the mainstay of successful treatment.
1307 A Rare Case of Metastatic Adenocarcinoma to the Pancreas Secondary to a Primary Lung Malignancy
Authors: Vangimalla Shiva , Mirza Muhammad U. , Agostini Tandy , Patel Mitesh R. 
Published: October 2019

INTRODUCTION:
Metastatic pancreatic tumors are an infrequent occurrence. Pancreatic metastasis from non-small-cell lung cancer (NSCLC) is rare and might present with few symptoms when the tumor is small. Here, we report such an occurrence of metastatic pancreatic adenocarcinoma secondary to primary lung malignancy.

CASE DESCRIPTION/METHODS:
A 62-year-old male presented with cough, shortness of breath, early satiety and abdominal pain for a few days. His medical history was notable for hypertension, congestive heart failure, emphysema, and heavy cigarette use. He was hemodynamically stable on presentation. Initial labs were pertinent for NT-proBNP 969 pg/ml, low white blood cells 3.5 K/uL, low hemoglobin 11.7 gm/dL, elevated lipase 566 units/L, and elevated INR 3.2. Chest X-ray showed left basilar consolidation with pleural effusion. CT chest/abdomen/pelvis revealed 3 cm × 4 cm × 5 cm lower lobe consolidation in left lung (Figure 1), 2 cm × 2 cm × 2 cm low-attenuation lesion in pancreatic head, and multiple sclerotic lesions of thoracolumbar spine. Preliminary infectious workup was negative. He was started on empiric antibiotics for community-acquired pneumonia. Further testing for malignancy revealed a normal level of CA19-9 22.9 IU/ml, CEA 1.3 ng/ml, and PSA 0.83 ng/ml. He underwent upper endoscopic ultrasound (EUS) which showed 28 mm × 25 mm hypoechoic mass in the pancreatic head (Figure 2). The preliminary diagnosis was consistent with “adenocarcinoma”. Further immunostaining analysis of pancreatic mass was positive for TTF1 (Figure 3) and PD-L1, negative for p63 and CK5/6 compatible with primary lung adenocarcinoma. The lung mass was not biopsied in view of the presumptive diagnosis - primary lung adenocarcinoma with secondary metastasis to pancreas. After discharge, he was due to follow up in the Oncology clinic for more definitive treatment.

DISCUSSION:
There have been only a few reported cases of pancreatic metastases in the setting of primary lung malignancy. Among the subtypes of lung malignancy, pancreatic involvement is more common in small cell carcinoma whereas it is much less common in adenocarcinoma. Clinical presentation may range from asymptomatic to acute pancreatitis. EUS guided FNA and immunostaining studies tend to be the diagnostic tool of choice to differentiate between primary and secondary tumors. Identification of pancreatic metastases is important as it confers poorer prognosis and worse survival.