bluuebunny/crossref_metadata_2025_split huggingface dataset:
2182 Endoscopic Challenges in a Patient With Ansa Pancreatica and Recurrent Pancreatitis
Authors: Aaron Jacob , Ahmad Dina , Smith Zachary L. 
Published: October 2019

INTRODUCTION:
Ansa pancreatica is a rare anatomical variation of the pancreatic duct where there is communication between the ventral pancreatic duct, which forms an ansa loop, and the accessory pancreatic duct. Its prevalence is estimated to be 1% in general population, and its presence can predispose to acute pancreatitis. We present an interesting and rare case of chronic pancreatitis with a pancreatic duct stricture in the setting of ansa pancreatica.

CASE DESCRIPTION/METHODS:
A 54-year-old female with a heterozygous CFTR mutation and a long-standing history of recurrent acute and now chronic pancreatitis. Two weeks after an episode of acute pancreatitis, a magnetic resonance cholangiopancreatography (MRCP) was performed showing a new stricture in the pancreatic duct near the genu, with pre-stenotic ductal dilation. Two prior endoscopic retrograde cholangiopancreatographies had been performed to attempt pancreatic duct endotherapy, but these were unsuccessful in achieving deep wire cannulation beyond the ansa loop. (image 1A-1B). The pancreatic duct in the genu of the pancreas was noted to have moderate stenosis on MRCP (image 1C). After a discussion with the patient, the decision was made to re-attempt endoscopic therapy. The ventral pancreatic duct was initially cannulated. The 0.025 inch angled guidewire was again unable to navigate the ansa loop and into the distal pancreas. Thereafter, we attempted dorsal pancreatic duct cannulation via the monir papilla. The guidewire was advanced successfully beyond the stricture and into the opacified body and tail (image 1D). The minor papilla and the stricture in the genu were dilated with a 7 Fr catheter dilator. After a minor papillotomy a 7 Fr by 8 cm plastic stent was placed into the dorsal pancreatic duct (image 1E, 2). The stent was removed 6 weeks later, and the duct remained patent. The patient has had no recurrence of acute pancreatitis to date.

DISCUSSION:
While the prevalence of ansa pancreatica is low, its presence can have great impact on the necessary approach for endoscopic therapy in chronic pancreatitis. In our case, the patient failed several ERCP attempts via ventral duct cannulation, and endoscopic therapy was successful only when cannulation of dorsal pancreatic duct was performed. In conclusion, in patients with chronic pancreatitis, recognition of ansa pancreatica, when present, is important to guide the endoscopic approach to treatment as minor papilla cannulation, a necessary skill for the interventional endoscopist, may be required.
2200 Ischemic Cholangiopathy Complicating an Orthotopic Liver Transplantation in Donation After Circulatory Death
Authors: Serrao Steve , Strilaeff Ryan , Che Kendrick , Rodrigues Medora , Bharadwaj Shishira 
Published: October 2019

INTRODUCTION:
Livers from donation after circulatory death (DCD) donors have a higher incidence of poor functional status and ischemic cholangiopathy as highlighted in our case report below.

CASE DESCRIPTION/METHODS:
Here we present a case of a 64 year old male with end stage liver disease due to Hepatitis C cirrhosis complicated by HCC, with three episodes of TACE with a pretransplant MELD-Na 28 who underwent OLT from DCD donor with replaced left and right hepatic arteries. He presented with worsening liver enzymes on post-operative day (POD) 19. Initial labs suggested a hepatocellular liver injury pattern with total bilirubin 0.7 mg/dl, aspartate aminotransferase 116 U/L, alanine aminotransferase 286 U/L, and alkaline phosphatase 473 U/L. A post-transplant ultrasound indicated nonvascular hypoechoic areas in the right hepatic lobe possibly representing abscesses or areas of infarction. A vascular scan indicated decreased resistive indices and elevated systolic rise time in the right hepatic artery indicating hepatic arterial stenosis. A CT scan showed mild intrahepatic biliary ductal dilation with decompressed distal common bile duct (CBD) representing a possible anastomotic stricture. The liver biopsy showed minimal-mild portal infiltrates, without venulitis or ductulitis with mild lobular disarray and focal, mild cholestasis. Rare necrotic and degenerating hepatocytes, with focal drop-out were noted which showed active hepatitis. An ERCP was performed on POD 20. Necrotic and denuded biliary mucosa with a distal CBD stricture (Figure 1) was noted on cholangioscopy. Fluoroscopic images showed multiple intrahepatic strictures and cystic dilatation of several intrahepatic biliary ducts (Figure 2). Given the tightness of the stricture, only a A 7Fr x 7 cm plastic biliary stent could be placed across the stricture. Angiogram showed severe focal stenosis of the proximal right hepatic artery, just distal the anastomosis related to “kinking” of the vessel. Percutaneous Transluminal balloon Angioplasty resulted in wide patency after balloon dilation and placement of a 4 mm × 15 mm stent within the vessel (Figure 3). The middle and left hepatic arteries were patent.

DISCUSSION:
This case of DCD OLT complicated by ischemic cholangiopathy resulted in intrahepatic and CBD strictures requiring biliary stenting. What is unique in this case is the additionally stenosis of right hepatic artery, the inciting trigger which was re-perfused with a vascular stent and resulted in the stabilization of liver function.
2213 Graft-versus-Host Disease: A Rare but Lethal Complication After Liver Transplantation
Authors: Hasan Badar , Yim Yunjoo , Castaneda Daniel , Ur Rashid Mamoon , Al Khalloufi Kawtar 
Published: October 2019

INTRODUCTION:
The Acute Graft-versus-Host Disease (GVHD) is one of the rare complications after liver transplant (LT) with prevalence up to 2%. Among solid organ transplant, intestinal transplant has the highest incidence followed by LT. Unfortunately, no universal treatment guidelines are available for this disease with poor outcomes and mortality up to 85%.

CASE DESCRIPTION/METHODS:
67-year-old male underwent orthotopic LT for end-stage liver failure due to Nonalcoholic steatohepatitis. CMV- patient received ABO identical cadaveric donor liver with CMV + serology. He had no peri-operative complications and was started on immunosuppression and antimicrobial therapies. Within 40 days of LT, he developed leukopenia and early transplant rejection, which was treated with medications. Within 100 days, patient developed rash on extremities and trunk that later spread to face, palms and soles. His course was further complicated by CMV seroconversion around 130 days post-LT. On day 168, patient presented with pancytopenia and continuing skin rash. The initial skin biopsy indicated drug induced reaction, but subsequent biopsy was consistent with GVHD. His treatment regime included steroid, tacrolimus, and rabbit Anti-thymocyte globulin (ATG) with broad-spectrum antimicrobials. Chimerism was initially 19% donor cells in the blood and it was 55% after 4 doses of ATG. He became septic and on day 209 succumbed to massive brain hemorrhage.

DISCUSSION:
GVHD after LT is in of the lethal complication that usually develops within 3-5 weeks. The key is to have a high index of suspicion with recognition of clinical signs; characteristic itchy palmar skin rash, diarrhea, fever, pancytopenia with normal LFTs. The symptoms are not specific and often diagnosed as infection or drug allergies. GVHD diagnosis can be established with skin lesion or GI tract biopsy and further confirmed with chimerism >30% in the blood of recipient. There is no consensus on treatment. Various strategies are adopted to suppress activated donor lymphocytes, including but not limited to, reduction or even withdrawal of immunosuppression, change in regimen with or without adding steroid boluses, IL-2 antagonist, antithymocyte/lymphocyte globulin, or use of OKT3. Broad-spectrum antimicrobial coverage is pivotal to prevent superimposed infection which is the main cause of death. Further studies are needed to raise the awareness among clinicians and to improve outcomes in patients who develop GVHD following LT.
2214 Acetaminophen-Induced Liver Toxicity in a Patient With Undetectable Acetaminophen Levels
Authors: Penmetcha Akhil , Goolsarran Nirvani , Namn Yunseok 
Published: October 2019

INTRODUCTION:
Acetaminophen is a common cause of overdose related acute liver injury (ALI). However, patients using acetaminophen chronically may have undetectable levels on presentation. It is important to recognize acetaminophen induced ALI early during clinical presentation and treat it promptly to prevent acute liver failure (ALF).

CASE DESCRIPTION/METHODS:
72-year-old female with a history of osteoarthritis relieved by acetaminophen presented with severe right upper quadrant (RUQ) pain. On admission, temperature 38°C, blood pressure 119/73, and heart rate 111. Physical exam shows tenderness to palpation in RUQ, no guarding or rebound tenderness, negative murphy sign, complete alertness and orientation, and no asterixis. Laboratory findings show total bilirubin 1.6, direct bilirubin 1.0, ALT 2,133, AST 36,600, alkaline phosphatase (AP) 92, albumin 3.7, INR 1.6, ferritin 77,183, acetaminophen level < 5, alcohol levels undetectable. RUQ ultrasound showed reduced liver echogenicity. Given history of chronic acetaminophen use, patient started on n-acetylcysteine (NAC) protocol for 72 hours. During hospitalization, diagnostic testing showed: Iron, TIBC, and transferrin all within normal limits, hemochromatosis DNA testing negative, alpha-1 antitrypsin within normal limits, ceruloplasmin within normal limits, IgG serum within normal limits, ANA Ab screen negative, hepatitis A Ab IgM negative, Hepatitis B Core IgM, Surface Ag, Surface Ab all negative, Hepatitis C Ab negative, EBV and CMV negative, HSV IgM negative, anti-liver kidney microsomal antibody negative, anti-smooth Muscle Ab negative, AMA negative, MRI of liver not concerning for iron overload. While on NAC therapy, liver enzymes and function tests trended to normal limits and RUQ pain and fevers resolved. On discharge, bilirubin and AP were within normal limits, ALT 305, AST 61, albumin 3.6, ferritin 8002 (Figures 1 and 2). 2-week post discharge follow up with primary care provider showed liver enzymes within normal limits.

DISCUSSION:
In this case, although acetaminophen levels were undetectable, careful elicitation of patient history gave clinicians more reason to suspect acetaminophen related liver toxicity. This led to prompt initiation of NAC therapy and prevention of ALF. This case demonstrates the importance of not excluding acetaminophen toxicity because of undetectable levels, especially in patients who use acetaminophen chronically, and initiating NAC therapy early for patients with ALI or ALF when acetaminophen toxicity is suspected.
2218 Recurrent Hepatocellular Carcinoma From Needle Tract Seeding 13 Years Post-Transplant
Authors: Palmer Quinton D. , Perisetti Abhilash , Broadfoot Brannon , Johnson Kemmian D. , Garcia-Saenz-de-Sicilia Mauricio 
Published: October 2019

INTRODUCTION:
Liver biopsy (LB) is a known risk factor for tumor seeding and hepatocellular carcinoma (HCC) recurrence. The time from LB to confirmed needle tract seeding (NTS) varies from 3 months to 4 years. We present case of recurrent HCC due to NTS 13 years after LB who presented with a right intercostal wall mass.

CASE DESCRIPTION/METHODS:
A 32-year-old Caucasian male presented to his primary care provider (PCP) in 2004 with recurrent right upper abdominal pain. A CT abdomen & pelvis showed 15 × 12 × 12 cm lesion in the right lobe of the liver with central hypodensity and 1-2 cm solid peripheral rim. His PCP requested CT guided percutaneous needle biopsy. Cytologic analysis was negative for malignant cells. After referral to our institution he underwent posterior right hepatectomy. A 14 × 12 × 7 cm well circumscribed tumor was removed with histopathology confirming well-differentiated HCC with negative margins (Figure 1A). In 2007, new hepatic lesions were noted during HCC surveillance for which he underwent orthotopic liver transplantation (OLT). He continued HCC surveillance which included CT abdomen, pelvis and thorax every 1- and 3-years, whole body bone scintigraphy every 3 years and abdominal US every 6-month intervals; which were negative for HCC recurrence. In 2017 (13 years post OLT), he returned with six months of right intercostal pain and swelling. CT chest & abdomen showed a large mass involving the right 10th rib, soft tissue, and lung nodules (Figure 2). Biopsy of the mass showed well-differentiated HCC, suspicious for vascular invasion (Figures 1B and 3). He was initially started sorafenib however could not tolerate it. He could not receive nivolumab due to risk of rejection and is currently on cabozatinib. He received palliative radiation for pain related to metastasis.

DISCUSSION:
This case highlights NTS from the LB resulting in HCC recurrence and potentially compromising curative therapies such as hepatectomy and OLT. NTC in HCC is rare with a reported incidence from 1 to 5% and could potential implant 1,000 to 100,000 malignant cells along the needle tract. Due to the risk of NTS, percutaneous biopsy is avoided in indeterminate nodules and should be used only in selected cases. Prompt referral to an experienced hepatologist is imperative. LB should be reserved for lesions in which no definitive surgical intervention is planned, and pathological confirmation is necessary for non-surgical therapy.
2230 Benign Intermittent Intestinal Alkaline Phosphatase Elevation
Authors: Mansoor Muhammad Sohail , Tageldin Omar , Parsa Nour , Itani Wafic , Alexandre Cassidy , Ells Peter F. 
Published: October 2019

INTRODUCTION:
Liver function tests (LFTs) are commonly checked during routine clinical encounters without specific signs or symptoms. An elevated alkaline phosphatase (AP) level is followed by investigative algorithms to identify the etiology. Total AP comprises liver, bone and intestinal fractions. Elevated intestinal alkaline phosphatase (IAP) can occur as a rare benign condition or due to intestinal pathologies. Unlike previously published reports of benign persistent IAP elevation, our patient had intermittent elevation of IAP.

CASE DESCRIPTION/METHODS:
A 48-year-old obese female was seen in liver clinic for evaluation of elevated AP for the last two years. She had undergone work-up for elevated AP on several occasions but the exact etiology was not found. She did not report any prior history of jaundice, pruritis, nausea, vomiting, diarrhea, abdominal pain, arthralgias or arthritis. She denied any family history of elevated IAP. She was found to have elevated total AP on 6 separate occasions over the last 18 months ranging from 186 to 239 U/l (normal < 117). Her fasting isozyme fractionation was checked 4 times. She had elevated total AP and normal liver/bone fractions on all occasions but twice had elevated IAP of 62 and 93 U/l (normal < 15 U/l), 26-33% of total AP (normal < 14%). Her other LFTs, TFT, PTH, Ca, CBC, BMP, GGT, viral hepatitis serologies, AMA and Vit D were unremarkable. Abdominal imaging revealed a fatty liver while a bone scan was normal. Our patient remained asymptomatic and did not require any treatment.

DISCUSSION:
Elevated AP can be physiological during rapid bone growth and pregnancy or pathological due to hepatobiliary disease or abnormal bone metabolism. Non-hepatic causes of elevated AP include heart failure, renal failure, hyperthyroidism, hyperparathyroidism and certain malignancies. IAP normally accounts for up to 14% of total AP. IAP can be elevated in conditions such as intestinal perforation, intestinal ischemia, extensive erosions/ulceration in GI tract, liver cirrhosis, familial benign elevation of IAP or post-prandial rise after a fatty meal particularly in patients with blood group B or O. Our patient did not have any of the above-listed conditions. Although she was blood group B, the blood draws were done after an overnight fast. While investigating elevated AP clinicians should be aware of pathological and benign causes of IAP elevation including benign intermittent IAP elevation which may require repeated testing of AP isozymes.
2231 Spontaneous Fungal Peritonitis- A Rare Complication of Ascites Secondary to Right Heart Failure: A Case Report
Authors: Siddiqui Salaah , Iqbal Arshad Muhammad , Patel Dharti , Mubarik Ateeq , Jupalli Aamani , Zafar Fahad , Muddassir Salman 
Published: October 2019

INTRODUCTION:
Spontaneous fungal peritonitis is an infection which occurs in patients with liver cirrhosis.Spontaneous fungal peritonitis is uncommon in a patient with cardiac ascites because of high protein content and low risk of infections.We present the second known case of SFP in a patient with cardiogenic ascites.

CASE DESCRIPTION/METHODS:
52-year-old female with past medical history of COPD was admitted to the hospital with a two-week history of abdominal pain and dyspnea.Labratory findings showed WBC count of 7,900/mm3, ANC of 6936/mm3, hemoglobin 15.8, platelet count 406 × 103/mm3, creatinine 0.9mg/dL, total bilirubin 3.40 mg/dL, direct bilirubin 2.30 mg/dL, ammonia of 51. Initial imaging of CT abdomen and pelvis showed bilateral pleural effusions, moderate ascites, and liver cirrhosis. ECHO showed left ventricular ejection fraction of 55%, dilated right ventricle, moderate tricuspid regurgitation with right ventricular systolic pressure of 76 mm Hg. Diagnostic and therapeutic ultrasound guided paracentesis was done and 400 mL of fluid was removed and sent for analysis and culture. Patient was empirically started on ceftriaxone for possible spontaneous bacterial peritonitis. Ascitic fluid analysis showed hazy fluid with WBC count of 23,000, 92% of polymorphic cells and 8% of mononuclear cells. Preliminary cultures from ascitic fluid grew Candida glabrata, and patient was started on caspofungin, and diagnosis of spontaneous fungal peritonitis was made. The patient gradually responded to antifungals.

DISCUSSION:
Cardiac cirrhosis is defined by signs and symptoms of chronic liver disease along with history of heart failure. Ascites due to heart failure is considered low risk for infections because of the high protein content in the ascitic fluid. Ascitic fluid retrieved by diagnostic paracentesis should be analyzed for lactate dehydrogenase, cytology, total cell count and differential, total protein, albumin, bacterial and fungal culture sensitivities. Along with bacterial gram stain and culture sensitivity, fungal stain and culture sensitivities are necessary due to an increasing prevalence of SFP in patients with cirrhosis. Common species causing SFP is Candida albicans, and other causative fungal agents being Candida glabrata, Candida krusei. Risk factors for SFP include elevated Child-Pugh and MELD scores, use of prophylactic antibiotics for SBP, low ascitic fluid protein (< 1 g/dL), hepatorenal syndrome. Treatment with echinocandins is recommended as soon as the diagnosis is made.
2234 Sorafenib-Induced Nephrotic Syndrome in Recurrent Post-Transplant Hepatocellular Carcinoma
Authors: Kumar Shiva 
Published: October 2019

INTRODUCTION:
An oral antiangiogenic tyrosine kinase inhibitor (TKI), Sorafenib (Nexavar®; Bayer Healthcare, Whippany, NJ) was the first systemic agent to be approved for treatment of unresectable hepatocellular carcinoma (HCC) in 2007 and remains an important first line treatment option. Common treatment related side-effects include diarrhea, fatigue, hand-foot skin reaction and hypertension. Limited data exists regarding safety and efficacy in post-transplant (LT) recurrent HCC. Sorafenib-induced nephrotoxicity is rare and has never been previously reported in patients with HCC or post LT. We report a case of Sorafenib-induced nephrotic syndrome in a patient with recurrent HCC post-LT, which promptly resolved with drug discontinuation.

CASE DESCRIPTION/METHODS:
A 64-year-old male was noted to have evidence of multi-focal recurrent HCC on surveillance imaging two years post-LT. He was treated with loco-regional and systemic therapy. At baseline, he had mild albuminuria from diabetic nephropathy. He developed increasing pedal edema and weight gain 4 weeks after starting Sorafenib. Repeat testing revealed marked increase in proteinuria (6 gm/24 hr), which continued to worsen (8.3 gm/24 hr in 6 weeks). Kidney biopsy revealed diffuse, severe podocyte foot process effacement with segmental sclerosing glomerulopathy (Figure 1). Sorafenib induced nephrotic syndrome was diagnosed based on temporal relationship to drug initiation and biopsy findings. Drug discontinuation resulted in prompt improvement, with return of proteinuria to baseline 16 weeks later.

DISCUSSION:
Nephrotic syndrome is an infrequently reported side effect of TKI such as sorafenib and its pathophysiologic mechanism remains unclear. Sorafenib inhibits vascular endothelial growth factor (VEGF) receptors and platelet-derived growth factor receptor. VEGF, produced by podocytes, plays an important role in development and maintenance of glomerular endothelium and its inhibition may help explain the development of podocyte and endothelial cell damage and consequent alterations in glomerular vascular permeability. All reported cases of TKI induced nephrotic syndrome have occurred in patients with advanced renal cell carcinoma. This is the first report of nephrotoxicity due to podocyte damage induced by Sorafenib treatment in HCC and in the post-transplant setting. All patients starting Sorafenib should undergo baseline urinalysis with periodic reassessment thereafter, since medication induced nephrotic syndrome appears to be reversible if detected early.
2238 An Unusual Case of Direct Acting Antiviral Potentiating Angioedema
Authors: Lambert Karissa , Hamed Ahmed Said , Khalid Hamza 
Published: October 2019

INTRODUCTION:
Hepatitis C Virus (HCV) is a leading cause of chronic liver disease and the most common reason for liver transplantation in the United States (US). The aim of direct acting antiviral (DAA) agents is to reduce progression to cirrhosis. There are newer well tolerated all-oral DAA agents, such as glecaprevir and pibrentasvir (GLE/PIB). We report an unreported adverse event in a treatment naive chronic hepatitis C genotype 1a patient: GLE/PIB induced angioedema.

CASE DESCRIPTION/METHODS:
A 63-year-old African American Male with a history of chronic hepatitis C infection (genotype 1a) without cirrhosis and hypertension on chronic lisinopril presented to the Emergency Room (ER) with acute onset facial and lip edema. Patient denied known environmental, food, or medication allergies. He denied unusual food intake. He recently started GLE/PIB. Vital signs were unremarkable. On physical examination, there was significant facial and lip swelling. Given the timing of symptoms, the side effect was suspected to be secondary to GLE/PIB. At this time, it was decided to stop the DAA and he was treated with steroids and antihistamine agents. At follow-up, the facial and lip edema had completely resolved. The HCV RNA level was obtained and surprisingly undetectable after only 4 days of therapy with a baseline of 5,080,000 IU/mL. However, at 12 week follow-up, he was again positive.

DISCUSSION:
Although GLE/PIB has a relatively good safety profile, angioedema is a potentially life threatening side effect that has not been reported as commonly. The underlying mechanism is not known, however it was hypothesized that co-administration with an Angiotensin Converting Enzyme (ACE) inhibitor may be related. We would recommend avoiding co-administration to prevent the rare but significant side effect, eliminating a potentially curative treatment regimen and the financial aspects of wasted expensive medication and expenses associated with hospitalization.