bluuebunny/crossref_metadata_2025_split huggingface dataset:
2353 Tuberculosis: Another Great Masquerader of the GI Tract
Authors: Gampa Anuhya , Shah Ayush N. , Shah Dhiren 
Published: October 2019

INTRODUCTION:
This case underlines the role tuberculosis has in non-pulmonary settings. Elevated liver enzymes in any setting of tuberculosis should raise concern for TB hepatitis until proven otherwise.

CASE DESCRIPTION/METHODS:
A 48 year old man a with PMH of Crohn’s colitis s/p right hemicolectomy, latent tuberculosis (TB) and asthma presented for elevated liver enzymes on routine testing. He denied abdominal pain, jaundice, acholic stools or dark urine. He was taking Adalimumab, Isoniazid and Pyridoxine but ran out of medications. He emigrated from Mongolia a few years ago and endorsed heavy alcohol use (bottle of liquor 2-3 times per week) and daily herb use. His exam was notable for fever to 102.9 F, tachypnea 25 breaths/min with hypoxia SpO2 8o% and mild hepatomegaly. Labs were notable for AST 92 IU/L, ALT 157 IU/L, total bilirubin 0.9 mg/dL, ALP 476 IU/L, protein 6.5 g/dL, albumin 3.0 g/dL, INR 1.1. Viral hepatitis panel was negative and interferon gamma release assay was positive for TB. MRCP revealed an enlarged liver but otherwise normal biliary tree and gallbladder. CXR and CT chest showed innumerable pulmonary nodules and lymphadenopathy concerning for pulmonary TB, which was confirmed on bronchoscopy. Liver enzymes continued to stay elevated, so a liver biopsy obtained showed cholestatic hepatitis with no granulomas. PCR testing was added on which was positive for M. Tuberculosis DNA, consistent with TB hepatitis. The patient was started on anti-tubercolosis therapy with improvement in liver enzymes.

DISCUSSION:
Elevated liver enzyme testing in the setting any manifestation of tuberculosis should raise concern for TB hepatitis. The presentation is highly variable from an acute to chronic process, and the most common lab abnormality is ALP elevation. Biopsy findings with caseating granulomas is only 68% sensitive while PCR testing is 85% sensitive. Despite the multiple risk factors for acute hepatitis and nonspecific biopsy findings, if the suspicion for TB hepatitis is still high, PCR testing can be added.
2356 Severe Allopurinol-Induced Liver Injury
Authors: Guzik Paul , Kim Christopher , Kumbum Kavitha 
Published: October 2019

INTRODUCTION:
The severity of drug-induced liver injury (DILI), a diagnosis of exclusion, can vary greatly from mild to life-threatening. Allopurinol is a commonly used medication that rarely causes serious hepatotoxicity. Objective data, such as aspartate aminotransferase (AST), alanine aminotransferase (ALT), alkaline phosphatase (ALP), gamma-glutamyl transferase (GGT), and total serum bilirubin, have been used to grade the severity of liver injury. We present a case of idiosyncratic allopurinol-induced cholestatic injury that meets diagnostic criteria for severe and life-threatening.

CASE DESCRIPTION/METHODS:
A 70-year-old African American woman with gouty arthritis, hypertension, and dyslipidemia presented to the Emergency Room with two weeks of abdominal pain. The patient was started on allopurinol for acute gout flare one month prior to presentation. In the interim, she developed malaise, anorexia (3 kg weight loss), and pruritus. She had no personal or family history of liver disease. Physical examination was notable for scleral icterus and mild, diffuse abdominal tenderness. Complete blood count was remarkable for absolute eosinophilia of 3.0 thou/cu mm, confirmed on peripheral blood smear. Liver function tests revealed a cholestatic pattern, with AST 259 u/L (normal 5-34), ALT 411 u/L (10-60), ALP 817 u/L (4-121), GGT 766 u/L (9-36), total bilirubin 15.7 mg/dL (0.2-1.2) and direct bilirubin 10.6 mg/dL (0.1-0.5). Synthetic liver function was normal. Toxicology screens, ceruloplasmin level, hepatitis serologies, and autoimmune workup were unremarkable. Imaging studies did not reveal acute pathology. Liver biopsy showed mild/moderate expansion of mixed inflammatory infiltrate with increased number of eosinophils. After discontinuation of allopurinol, absolute eosinophil count and liver function tests trended down with clinical resolution of symptoms.

DISCUSSION:
Among DILI literature, few reports exist of severe, life-threatening cholestatic liver injury after allopurinol initiation. This patient had 8x the upper limit of normal AST, 7x ALT, 7x ALP, 21x GGT, and 21x direct bilirubin. According to multiple DILI severity grading tools, this case is classified between severe (grade 3) and life-threatening (grade 4). Beyond discontinuation of the medication, there are limited treatment options available for idiosyncratic DILI other than liver transplantation. Therefore, prompt discontinuation of the offending medication along with close monitoring for progression to fulminant liver failure is crucial.
2360 Progression of Post-Islet Cell Transplantation Hepatic Steatosis to Cirrhosis: A Rare Complication of a Common Finding
Authors: Condon Sally , Smart Laura , McClain Craig 
Published: October 2019

INTRODUCTION:
Non-alcoholic fatty liver disease (NAFLD) is a growing cause of liver disease in the USA and throughout the rest of the world and is predicted to soon become the leading indication for liver transplantation. NAFLD exists on a spectrum from hepatic steatosis to non-alcoholic steatohepatitis (NASH) to cirrhosis. It is closely related to metabolic syndrome and insulin resistance. A lesser known cause of hepatic steatosis is islet cell allotransplantation and autotransplantation, which are experimental treatments for Type 1 Diabetes Mellitus and FDA- approved treatment for patients with chronic pancreatitis who have undergone pancreatectomy, respectively. In this case, we present the first case of post-islet cell transplantation hepatic steatosis progressing to cirrhosis.

CASE DESCRIPTION/METHODS:
The patient is a 27 year old female with a history of chronic pancreatitis secondary to cystic fibrosis, status-post total pancreatectomy and islet cell autotransplantation, complicated by development of non-alcoholic steatohepatitis. Her NASH was treated with probiotics and vitamin E supplementation. Eight years after her islet cell transplant, she was hospitalized for nausea, vomiting, and abdominal pain. A CT scan showed that she had developed severe, diffuse hepatic steatosis. Her ALP and AST were mildly elevated. ALT and total bilirubin were normal. She had facial and pedal edema, but no other stigmata of cirrhosis. She underwent a liver biopsy which showed bridging fibrosis and a small regenerative nodule with steatohepatitis. It was with these findings that she was diagnosed with cirrhosis.

DISCUSSION:
Hepatic steatosis has been reported with a prevalence of 20-40% of patients following islet cell transplantation. These reports have not been associated with any long term liver or graft survival outcomes. The usual onset of histological findings in one study was approximately 3 years after transplant, and some of these cases resolved over time. These reported cases did not have any changes in total bilirubin, ALP, AST, or ALT. Hepatic steatosis from islet transplantation is thought to develop due to the paracrine effect of localized hyperinsulinemia and fatty acid infiltration from the islet cells, which are inserted into the portal vein and taken to the liver during transplant. To our knowledge, this is the only reported case in which post-islet cell transplantation hepatic steatosis progressed to NASH cirrhosis along with abnormal liver biomarkers.
2362 A Case of BRAF V600E Intrahepatic Cholangiocarcinoma
Authors: Ahmed Zarir , Doane Elizabeth , Antao Nirav , Chhaparia Anuj 
Published: October 2019

INTRODUCTION:
Intrahepatic cholangiocarcinoma is an uncommon hepatic malignancy and accounts for 3% of all gastrointestinal malignancies. Patients with this disease have a poor prognosis, with an average 5-year survival rate of 5 to 10%. Herein we describe a case of BRAF-related cholangiocarcinoma.

CASE DESCRIPTION/METHODS:
A 26 year-old female presented with right-upper quadrant pain and worsening nausea, vomiting for four months. On exam the abdomen was soft, non-tender and non-distended. A right upper quadrant ultra sound revealed a 7 cm mass in the inferior right hepatic lobe and ensuing magnetic resonance imaging of the liver should an 8 cm lesion with numerous satellite lesions. She underwent laparoscopic wedge excision of the liver mass which revealed poorly differentiated adenocarcinoma with features concerning for cholangiocarcinoma (See Images). PET revealed also portal hepatic lymph node metastasis, and metastatic lytic lesions at the clavicle and scapula. CEA and AFP were elevated. Comprehensive genetic analysis with next generation sequencing genomic panel was performed on her initial biopsy and confirmed BRAF V600E mutation. She was initiated on a phase II trial with trametinib and dabrafenib at a referral center. Despite this, repeat CT imaging revealed she had progression of metastatic cholangiocarcinoma. She was switched to FOLFIRI and vemurafineb. However, the patient developed worsening abdominal distension, hyperbilirubinemia and pancytopenia. At that point, the family elected for hospice care whereupon after a few weeks, the patient expired at home.

DISCUSSION:
Currently the only curative therapy is surgical resection or transplant. However there has been research in molecular targeted therapies to treat BRAF cholangiocarcinoma with optimal response. BRAF V600 mutation results in activation of down-stream signaling though MAPK pathway and can contribute to malignancies such as melanoma, hairy cell leukemia, or non-small cell lung cancer. The frequency of BRAF V600E mutation has been reported to be 0-22% in intrahepatic cholangiocarcinoma. Inhibitors of BRAF V600 kinase such as vemurafenib and dabrafenib can be used to target these aberrations. Although this patient failed treatment with molecular targeting, precision medicine may change the trajectory in management of intrahepatic cholangiocarcinoma. The implementation of these therapies will require multi-team and institutional collaborations and will positively impact these patients.
2371 Non-Cirrhotic Portal Hypertension Presenting With Variceal Bleeding in a Patient With Breast Cancer on Capecitabine
Authors: Luft Suzannah , Calderwood Audrey H. 
Published: October 2019

INTRODUCTION:
We describe a case of variceal bleeding in a cancer patient with improving liver metastases without radiographic evidence of cirrhosis.

CASE DESCRIPTION/METHODS:
A 66 year-old woman with stage IV bilateral breast cancer presented to the hospital with hematemesis. Platelet count was 98,000/mcL and INR 1.2 with normal albumin and liver enzymes. Upper endoscopy showed large varices with stigmata of recent bleeding and portal hypertensive gastropathy. Varices were banded without further bleeding. CT abdomen/pelvis with contrast showed left hepatic lobe atrophy, normal spleen, normal liver contour and no portal venous thrombosis (PVT). She was diagnosed with breast cancer 26 years prior and underwent lumpectomy and radiation. Eight years ago, she had recurrence, underwent mastectomy and then was diagnosed with contralateral breast cancer followed by mastectomy. Six years ago, she had recurrence requiring axillary dissection with chemotherapy and radiation. Four years ago, imaging showed new metastases with heavy left liver tumor burden with left portal vein cut off, ascites and bony metastases. Liver lesion biopsy showed breast cancer. She started doxorubicin with resolution of ascites and marked left lobe atrophy by imaging. For two years, she was remained on doxorubicin without evidence of disease. Three months prior to admission, PET CT showed two small right hepatic lobe metastasis, and she was started on capecitabine.

DISCUSSION:
Non-cirrhotic portal hypertension in the setting of liver metastases occurs for various reasons. One etiology is PVT, which imaging ruled out. Pseudocirrhosis describes a radiographic appearance of cirrhosis without histopathologic cirrhosis due to capsular retraction from metastases shrinkage after therapy, nodular regenerative hyperplasia (NRH) due to chemotherapy and fibrotic changes around metastases. While this is reported with capecitabine, our patient did not appear cirrhotic on imaging. Portal hypertension due to micronodular metastases is reported, but this is unlikely with improved liver lesions on imaging. Hepatic steatosis with elevated transaminases is reported with capecitabine. Several chemotherapies cause NRH, including doxorubicin. We suspect underlying NRH from previous doxorubicin and left hepatic lobe atrophy from shrinkage of metastases combined with new capecitabine caused an increased in liver stiffness. This case demonstrates the complex effects on the liver from both metastases and cancer treatments in a patient without known liver disease.
2379 Biopsy Confirmed Infiltrating Breast Cancer in the Liver Not Captured by MRI Abdomen
Authors: Stewart Sarah , Reddy Shravani , Kesar Varun , Sahebjam Farhad , Grider Douglas J. 
Published: October 2019

INTRODUCTION:
Early detection of liver metastases (mets) has the potential to improve patient outcomes, and those with breast cancer are at increased risk for liver mets. Literature has shown that computed tomography (CT) or magnetic resonance imaging (MRI) techniques detect most liver mets with a high level of accuracy; however, small mets or non-mass mets in sinusoidal spaces, are less likely to be recognized on imaging studies. Of the imaging studies available, MRI is toted to be more sensitive than CT or positron emission tomography in detecting small lesions. Here we present a case of metastatic breast cancer not detected by MRI.

CASE DESCRIPTION/METHODS:
A 50-year-old female with a history of cirrhosis, invasive lobular carcinoma of the left breast (T3N3aM1) following unilateral mastectomy and axillary dissection, and mets to uterus status post total abdominal hysterectomy with bilateral salpingo-oophorectomy presented with complaints of confusion and abdominal distension. She was admitted for acute on chronic decompensated liver failure with exam significant for jaundice, asterixis, distended abdomen, and pitting edema to bilateral extremities. MELD score on admission was 23 with Child-Pugh Class C, and labs were notable for hyperbilirubinemia (15.9) and elevated alkaline phosphatase (950). Abdominal ultrasound demonstrated a nodular heterogenous liver consistent with cirrhosis. Ascitic fluid was felt to be secondary to portal hypertension (SAAG 2.4g/dL, ascitic protein 0.5 g/dL) and negative for spontaneous bacterial peritonitis. The patient was negative for hepatitis A, B, C, or D, antimitochondrial antibodies, and acetaminophen levels were unremarkable. MRI of the abdomen indicated hypertrophy of the left lobe of the liver and an irregular hepatic capsule contour, but no distinct reticular enhancement pattern or focal abnormalities. Transjugular liver biopsy was, however, positive for a large burden of metastatic lobular breast carcinoma, mostly confined to sinusoidal spaces, in a background of end-stage liver disease and acute cholestasis.

DISCUSSION:
The presence of liver mets that were undetectable by MRI, the imaging technique of choice metastatic lesions, demonstrates this case’s uniqueness. The size and/or location of the metastasis is directly related to the ability of anatomic imaging to detect metastatic lesions. This patient’s presentation and lack of radiographic evidence of metastasis demonstrates the importance of tissue pathology studies in patients with suspected metastatic processes.
2380 Liver Cirrhosis of Unknown Etiology With Iron Overload in a Patient Heterozygous for Hereditary Hemochromatosis and Alpha-1 Antitrypsin
Authors: Shastri Pinang , Ghazaleh Sami , Khader Yasmin , Moussa Zeinab , Nehme Christian , Awad Mohammed , Nawras Ali 
Published: October 2019

INTRODUCTION:
Abnormal liver function tests are frequently detected in routine laboratory work. The differential diagnosis is broad and further evaluation is required to identify and treat the underlying cause. We present a patient with abnormal liver function tests who was found to have a unique genetic makeup.

CASE DESCRIPTION/METHODS:
A 68-year-old female patient presented with jaundice and dark urine for 3 months. Past medical history was significant for paroxysmal atrial fibrillation and nephrolithiasis. Past surgical history and family history were insignificant. Home medications included aspirin and atenolol. She used alcohol socially and denied IV drug use. Review of systems was unremarkable. On physical exam, the patient was diffusely jaundiced and had scleral icterus. Vital signs were within normal limits. Abdominal exam showed a soft, non-tender, and non-distended abdomen without organomegaly. Skin exam was significant for multiple spider angiomas. She had elevated AST of 518 U/L, ALT of 246 U/L, alkaline phosphatase of 185 U/L, total bilirubin of 9.5 mg/dL, and direct bilirubin of 4.5 mg/dL. Workup was negative for viral hepatitis, Wilson’s disease, autoimmune hepatitis, primary biliary cirrhosis, and primary sclerosing cholangitis. She had elevated Ferritin of 3547 ng/mL and iron saturation of 83%. MRI showed features of intrinsic liver disease without a focal mass. Next, upper endoscopy revealed Grade 1 esophageal varices. Subsequent liver biopsy revealed cirrhosis and increased iron stores. The patient underwent genetic testing for hemochromatosis and alpha-1 antitrypsin deficiency. She had a heterozygous C282Y mutation of the HFE gene. In addition, she had a heterozygous PI*MS mutation of SERPINA1 - the gene that codes for alpha-1 antitrypsin. The patient experienced a slow improvement in her abnormal labs over a period of a few months.

DISCUSSION:
Hereditary hemochromatosis and alpha-1 antitrypsin deficiency are inherited conditions that result in liver damage and eventually liver cirrhosis. Our patient was heterogenous for the HFE gene and developed iron overload on liver biopsy. She was also heterozygous for PI*MS, a less common mutation for SERPINA1 that causes isolated lung disease. Therefore, we can conclude that our patient’s condition was primarily related to iron overload as a result of a mutated HFE gene.
2381 Abnormal Aminotransferases, Think Beyond the Liver
Authors: Ghazaleh Sami , Khader Yasmin , Ghanim Marcel , Shastri Pinang , Alhmoud Tarik 
Published: October 2019

INTRODUCTION:
Liver aminotransferases are frequently utilized to evaluate liver and biliary tract diseases. Elevated aminotransferases are non-specific and further testing is usually necessary to identify the underlying cause. We present a puzzling case of abnormal aminotransferases.

CASE DESCRIPTION/METHODS:
A 28-year-old male patient was referred to hepatology clinic for evaluation of abnormal laboratory findings. Routine blood tests revealed elevated AST of 67 U/L and ALT of 77 U/L. Alkaline phosphatase was 36 U/L and total bilirubin, total protein, and albumin were within normal limits. Review of systems was unremarkable. Past medical and surgical histories were insignificant. His only home medication was diphenhydramine. Family history was negative for liver disease or malignancies. He reported drinking 5-6 drinks per week and denied the use of tobacco or illicit drugs. On physical exam, body mass index (BMI) was 28. Abdomen was soft, non-tender, and slightly distended. He had normal bowel sounds and no organomegaly. Abdominal ultrasound showed a normal liver and gallbladder. Hepatitis A, hepatitis B, and hepatitis C serology were unremarkable. Serum ceruloplasmin and alpha-1 antitrypsin levels were normal. Antinuclear antibody (ANA) and antimitochondrial antibodies were negative. Anti-smooth muscle antibody (ASMA) was borderline positive with a titer of 1:20. Ferritin level was 100 and transferrin saturation (TSAT) was elevated at 59%. Subsequently, a liver biopsy was performed and revealed normal liver architecture with intact hepatic lobules. Additional history revealed that the patient gets leg cramps while walking and could not run as fast as the other kids in school. Upper musculoskeletal exam was unremarkable. However, lower musculoskeletal exam revealed a 4/5 weakness in addition to bulky calf muscles. Laboratory workup showed elevated CK of 1002 U/L and aldolase of 11.2 U/L. The patient was referred to rheumatology and neurology for further evaluation of myopathy.

DISCUSSION:
The evaluation of elevated aminotransferases can be challenging especially in asymptomatic patients. In our patient, extensive liver work-up came back normal. Although he had elevated BMI, there was no evidence of non-alcoholic steatohepatitis (NASH). Muscle disorders/myopathies may present with elevated aminotransferases. A good history and physical exam can provide important clues to reach the correct diagnosis.
2382 Successful Hepatitis C Virus Antibody-Positive Donor Liver Transplantation in Acute Sickle Cell Intrahepatic Cholestasis: A Case Report
Authors: Alkhayyat Motasem , Saleh Mohannad Abou , Sanghi Vedha , Singh Tavankit , Rouphael Carol , Simons-Linares Roberto , Lindenmeyer Christina C. , Romero-Marrero Carlos , Carey William 
Published: October 2019

INTRODUCTION:
Sickle cell hepatopathy (SCH) is the umbrella term referring to liver dysfunction in sickle cell disease (SCD) patients. We report a case of acute intrahepatic cholestasis (ASCIH) with underlying cirrhosis managed with hepatitis C virus (HCV) antibody-positive donor liver transplantation (LT).

CASE DESCRIPTION/METHODS:
A 29-year-old African American male with known medical history of SCD (Hb SS) and cirrhosis presented to the medical intensive liver unit with vaso-occlusive pain crises and jaundice. Patient was afebrile with benign exam. Labs were consistent with obstructive jaundice (total serum bilirubin of 57 mg/dL, direct serum bilirubin of 30 mg/dL), and elevated liver enzymes (alkaline phosphatase 306 U/L, aspartate transaminase 227 U/L and alanine transaminase 54 U/L). He had significant leukocytosis 38.6 k/uL, low hemoglobin 6.3 g/dL and thrombocytopenia 39 k/uL. Sodium level was 149 mg/dl and INR was 2.3. Computed tomography scan showed changes consistent with cirrhosis but no evidence of ductal dilatation. He developed encephalopathy after 24 hours of presentation, acute kidney injury (serum creatinine 3.57 mg/dl) and respiratory failure requiring endotracheal intubation. He was diagnosed with acute liver failure secondary to ASCIH after a non-revealing extensive workup for acute liver failure. Exchange transfusion was started without improvement in hepatic function or mental status. His MELD-Na (Model for End-Stage Liver Disease-Sodium) score was 43 and thus patient was listed for urgent LT. He received an HCV positive donor liver after 9 days of hospitalization. Explanted liver biopsy showed widespread sinusoidal dilatation with innumerable clusters of sickled red blood cells, scattered pigmented histiocytes, and cholestasis. Following transplant, exchange transfusion was resumed and immunosuppression treatment started with mycophenolate, tacrolimus and steroid taper. Glecaprevir/Pibrentasvir started for high HCV RNA titer. He was discharged after 23 days with total bilirubin of 4.2 mg/dL and normalization of his liver enzymes. He was doing well during his 4 months follow up.

DISCUSSION:
We present a rare case of acute sickle cell intrahepatic cholestasis treated with successful LT. This represents the 1st case of a positive HCV donor LT and the 20th LT case in SCD. ASCIH is the most severe, often fatal, form of SCH. Our goal with this case is to increase the awareness of this important condition and emphasize the role of liver transplantation as a potential treatment.