bluuebunny/crossref_metadata_2025_split huggingface dataset:
2251 An Atypical Case of Recurrent Intrahepatic Cholestasis of Pregnancy
Authors: Shah Pir Ahmad , Campoverde Reyes Karen J. , Aslam Aysha , Suri Jaspreet , Wu Lily , Chie Lucy , Lau Daryl T. 
Published: October 2019

INTRODUCTION:
Intrahepatic cholestasis of pregnancy (ICP) usually manifests in the second to third trimester with pruritus, jaundice and abnormal liver tests. It can increase risks of preterm birth, respiratory distress syndrome, and stillbirth. Timely diagnosis and planned delivery are crucial. We hereby describe an atypical case of recurrent ICP.

CASE DESCRIPTION/METHODS:
Patient is a 31-year-old Chinese female who presented to hepatology clinic at week-13 of her second pregnancy. During her first pregnancy 4 years ago, she experienced pruritus associated with significant elevation of bile acids and serum aminotransferases at about 20-week gestation. Diagnosis of ICP was made and she was promptly started on ursodeoxycholic acid (URSO) [Figure 1]. Bile acids improved and pruritus resolved but she had a preterm delivery at 30-week gestation. URSO was discontinued after delivery and bile acids were completely normal by 6 weeks postpartum. The ALT levels, however, increased to >200 U/L and remained abnormal for 10 months post-partum [Figure 1]. Other common causes of liver diseases such as viral hepatitis, autoimmune and drug-related liver diseases were ruled out. Her liver biopsy reported only non-specific resolving inflammation. For her second pregnancy, bile acids were checked at 14-week gestation and were mildly elevated at 14.7umol/L; they increased to 20.8 umol/L by 26 weeks. At that time, she experienced mild pruritus. URSO was initiated at 28-week gestation for recurrent ICP. Despite being on URSO for 3 weeks, ALT normalized but bile acid increased further to 53.5 umol/L without worsening of pruritus. Induced labor was planned at week-37 and the delivery was uneventful.URSO was subsequently discontinued. At week-3 postpartum, URSO was reinitiated for her ALT significantly elevated at 259 U/L even though bile acids continued to decrease. About 1 week after starting URSO, ALT decreased to 62 U/L and bile acids completely normalized.

DISCUSSION:
ICP usually resolves after delivery and URSO can be discontinued. Our case was atypical for her serum aminotransferases actually markedly increased during early postpartum in both pregnancies despite improvement of the bile acid levels. URSO was promptly reinitiated postpartum that resulted in reduction of ALT. For patients with elevated liver tests associated with ICP, physicians need to be vigilant in monitoring the patient closely during postpartum period and consider continuing URSO therapy until both serum aminotransferases and bile acids return to normal range.
2260 Management of a Massive Liver Hemangioma: Does Size Matter?
Authors: Fazio Richard M. , Waintraub Daniel J. , Rahmani Rabin , Hajdu Cristina H. , Park James S. 
Published: October 2019

INTRODUCTION:
The majority of “giant” hemangiomas remain asymptomatic with no cause for surgical intervention; however, this may not hold true for massive tumors. The following case will review the challenges facing both patients and physicians when managing these atypical tumors.

CASE DESCRIPTION/METHODS:
A 49-year-old male with no medical problems presented with complaints of post-prandial bloating, early satiety and mild epigastric discomfort. The bloating was intermittent for several years; however, symptoms have recently curtailed his eating habits. CBC, BMP and liver enzymes were unremarkable. An ultrasound highlighted a massively enlarged liver extending into the pelvis and displacing surrounding organs. The liver parenchyma appeared to be replaced with a homogenous, hyperechoic lesion. An MRI then illustrated a 29.5 × 20.1 × 19.4 cm, strongly hyperintense mass on T-2 weighted sequences consistent with the diagnosis of a hemangioma. He was referred to a hepatobiliary surgeon and an extended right hepatectomy was eventually performed. Histopathology results described vast endothelial lined channels supported by thin fibrous stroma without features of malignancy. The patient returned to clinic four weeks after surgery reporting complete resolution of his symptoms.

DISCUSSION:
Hemangiomas are the most common benign solid tumor of the liver with little to no risk of malignant transformation. Often discovered incidentally on imaging studies, the majority of these tumors remain indolent without the need for routine surveillance. Rarely these tumors become symptomatic, often correlating with tumor size. The definition of “giant” liver hemangioma remains controversial, with most authors assigning the label to tumors greater than 4cm or 5cm in size. It is for this reason that management of giant hemangiomas remains highly debated (i.e. observation versus resection). Recent studies have shown that tumors greater than 20cm in size pose a higher risk for GI symptoms related to mass effect on surrounding organs as well as causing a disturbance in the hematologic and coagulation systems. Surgical resection should be considered for symptomatic or complicated lesions, or when the diagnosis remains inconclusive. It is our belief that size classifications for giant hemangiomas requires further subgrouping to consider the danger of these massive tumors as well as the increased morbidity of surgery. Proper management of these tumors should be individualized to each patient and include a multidisciplinary team approach.
2273 A Rare Case of Very Late Liver Metastasis in Resected Neuroendocrine Tumor
Authors: Kochar Tanureet , Dhingra Parminder , Sankari Mohamad 
Published: October 2019

INTRODUCTION:
Carcinoid tumors are neuroendocrine tumors derived from enterochromaffin or Kulchitsky cells. They occur predominantly in foregut, midgut and hindgut. The annual incidence of carcinoid tumors is around two cases per 100,000. Although generally slowly progressing, hepatic metastases are the major cause of carcinoid syndrome and ultimately lead to liver dysfunction, cardiac insufficiency.

CASE DESCRIPTION/METHODS:
This is 72-year-old male with PMH carcinoid tumor involving right colon and terminal ileum T3N1M0 present in 6 ileum ileocolic lymph nodes s/p resection in 2006, chronic kidney disease stage III, diabetes mellitus type II, hypertension presented watery diarrhea with 5-6 bowel movements/day from last 2-3 months, 30 pound unintentional weight loss in last 2 months, occasional night sweats. He denied any episodes of flushing. On presentation, vitals were stable. Other labs included CBC- wnl, BUN - 25 mg/dl with creatinine of 3 (baseline 2), albumin – 2.4, AST – 19u/l, ALT- 26u/l, ALP- 210 u/l, total bilirubin 0.9 mg/dl. Clostridium difficle screen and GI panel were negative. Last colonoscopy, done one month prior to presentation was normal. He was started on Imodium without any relief. Gastroenterology service was consulted for further management. His chromogranin A was found to be 782 (< 93), 5HIAAA was 50 (< 8). He underwent ultrasound of the abdomen for this acute kidney injury which revealed 5.9 × 5.9 × 5.9 cm hyperechoic mass with central decreased echogenicity located within the liver. AFP was 1.1 ng/ml (< 8 ng/ml), CEA 7.4 ng/ml (3 ng/ml). Meanwhile, he was started on sandostatin which helped a lot with his diarrhea. Further imaging of this abdomen was deferred due to kidney injury. He had previous PET scan done 3-4 years ago revealed increased uptake in liver but were inconsistent of any metastatis. His last abdominal CT scan done 2-3 years ago was negative of any metastasis. Two weeks after his discharge, he was readmitted to intensive care unit (ICU) with septic shock and unfortunately he passed away.

DISCUSSION:
Carcinoid tumors are relatively slow growing compared to adenocarcinomas of the same site and have a tendency for recurrence. The NANETS Consensus Guideline for the Diagnosis and Management of Neuroendocrine Tumors recommends an office follow up every 6 to12 months for 7 years. Either radiographic (CT/MRI) or endoscopic methods could be used for surveillance. The secretory tumors can be followed up by monitoring the levels of urinary 5- HIAA and serum chromogranin A.
2276 Malignant Hepatic Epitheliod Hemangioendothelioma in a Patient With a Prior Liver Transplant
Authors: Ellison Parker L. , Willner Ira 
Published: October 2019

INTRODUCTION:
While liver transplantation itself is an invasive and specialized procedure, often with an arduous medical course preceding the surgery; post-transplant life is rarely without complication. Permanent immune suppression introduces its own cohort of increased risks including unique infections, malignancies, and side effects of immunosuppressive or prophylactic therapies. Special consideration should be given to these patients when potential complications arise.

CASE DESCRIPTION/METHODS:
A 51-year-old male with a an orthotopic liver transplant 6 years prior due to NASH cirrhosis presents with rapid significant weight loss and worsening abdominal pain. MRI revealed development of T2 hyper-intense peripherally enhancing lesions throughout the liver, as well as massive splenomegaly with possible areas of splenic infarct. Transjugular liver biopsy was expedited and ultimately revealed epitheliod hemangioendothelioma (EHE). Initial infectious work up was unrevealing outside of EBV viral load returning weakly positive; however, given possible association with EHE with EBV, treatment was initiated with valgancyclovir. Immunosuppression was also adjusted from tacrolimus to everolimus given anti-neoplastic properties of mTOR inhibitors over calcineurin inhibitors. He was started on pazopanib as an outpatient given anecdotal evidence of VEGF inhibitors in treating EHE, however, treatment was complicated shortly thereafter by syncope and refractory TTP, and palliative care was ultimately pursued.

DISCUSSION:
Epithelioid hemangioendothelioma is a rare vascular tumor originating from mesenchymal cells. While risk of malignancy is increased post transplant due to immunomodulators, post transplant Hepatic EHE remains an exceedingly rare diagnosis. EHE is considered to have intermediate behavior between hemangioma’s and hemangiosarcoma’s however HEHE is metastatic at diagnosis in 50% of diagnoses. Common presenting symptoms include vague abdominal pain, weight loss, and weakness/fatigue. Histopathological examination via biopsy is the gold standard of diagnosis and positivity of factor VIII-dependent antigen, CD-34, and CD-31 are important. Course is variable however prognosis is poor and there is no standardized management plan. VEGF inhibitors including Bevacizumab and Pazopanib have been a target of therapy given histologic positivity for target receptors, however results have been mixed to date.
2287 HELLP My Liver Hematoma
Authors: Abdalla Abubaker O. , Abdallah Mohamed , Narala Sai B. , Gullapalli Nageshwara 
Published: October 2019

INTRODUCTION:
Pregnant females are at increased risk of hypertensive conditions including preeclampsia, eclampsia, and hemolysis, elevated liver enzymes, low platelet (HELLP) syndrome. Complications related to these disorders can be life-threatening and can result in multi organs failure. These complications involve disseminated intravascular coagulation (DIC), stroke, acute renal failure, acute liver injury, hepatic hematoma, and rupture.

CASE DESCRIPTION/METHODS:
We describe a 34-year-old G2P1 female at 36-weeks gestation with no significant past medical history, who presented with abdominal pain for the last couple of days prior to her emergency room (ER) visit. She described the pain as dull, constant, and worsening right upper quadrant pain associated with nausea but no vomiting, fever, chills, jaundice or changes to bowel habits. On presentation, she was noted to be in a stable condition. The physical exam was consistent with tight upper quadrant tenderness with no signs of acute abdomen. The initial workup was significant for leukocytosis of 13.8, hemoglobin of 11.9, mildly elevated liver enzymes with AST of 270, ALT of 346, ALP of 165 (normal liver tests during her recent pre-natal visits). Abdominal ultrasound revealed a normal appearance of the liver with no signs of gallbladder disease. The rest of her work up was unremarkable on admission. Her condition got worse with markedly elevated liver enzymes that peaked at 2500s range and diminished platelet count concerning for the diagnosis of HELLP syndrome. She underwent a successful urgent cesarean section in the setting of deceleration of fetal heart tracing that was complicated by hypotension requiring pressor support. She was noted to have a liver hematoma intra-operatively. Abdominal CT post-operatively confirmed extensive heterogeneous sub-capsular hepatic hematoma with a small amount of ascites but not evidence of extravasation (Figure 1,2). The patient was managed conservatively in the intensive care unit (ICU) after her operation and her condition improved gradually.

DISCUSSION:
HELLP syndrome is a life-threating condition that carries a high morbidity and mortality burden. Hepatic hematoma and spontaneous liver rupture have been described previously as fatal complications. A high index of suspicion is required for early diagnosis and prompt treatment of such conditions.
2299 Resolution of Acute Cellular Rejection Using Irradiating Packed Red Blood Cells for Exchange Transfusion in a Patient With Sickle Cell Disease and a History of Orthotopic Liver Transplantation
Authors: Nayi Vipul , Htut Alvin 
Published: October 2019

INTRODUCTION:
Liver transplantation in patients with Sickle Cell Disease (SCD) is fairly unique; a review article from 2013 noted only 15 reported cases within the literature. This case highlights a patient with SCD who underwent orthotopic liver transplantation. His course was complicated by iron overload due to recurrent exchange transfusions, as well as biopsy-proven acute cellular rejection (ACR), that was inadequately treated with corticosteroids, with no recurrence of rejection once the patient was switched to irradiated red cell exchange transfusions.

CASE DESCRIPTION/METHODS:
A 32-year-old male with HbSS disease, who had also undergone orthotopic liver transplantation at the age of 26 due to complications of Caroli’s disease presented with persistent abnormal liver tests over the course of several weeks. Graft function was normal during post-transplant until monthly exchange transfusions were initiated to manage his sickle cell crises. During this period he had two episodes of ACR and was noted to have persistent liver test abnormalities despite adequate therapeutic levels of tacrolimus. The patient was switched to receiving irradiated packed red blood cells for his subsequent exchange transfusions, which led to improvement of his liver tests and resolution of his acute cellular rejection.

DISCUSSION:
Only a limited number of patients with sickle cell disease have undergone liver transplantation, and this is the first case of using irradiated packed red cells for transfusion to help resolve acute cellular rejection. Although it is not thoroughly understood how blood transfusions could induce acute cellular rejection, it may be related to passenger donor leukocytes within the donor blood products triggering an immune response within the transplanted liver. Given our patient’s exposure to higher volumes of transfusions, there may have been enough donor lymphocytes to trigger this response. The use of irradiated blood products is common place in patients that have received hematopoietic stem cell transplants. Current recommendations do not standardize use of irradiated red cells for solid organ transplant (SOT) recipients. Our patient presents an unusual indication for use of irradiated red cells in a SOT patient.
2309 Vasculitic Mononeuritis Multiplex Mimicking Bilateral Carpal Tunnel Syndrome: A Diagnostic Challenge
Authors: Vohra Ishaan , Randhawa Tejinder , Katiyar Vatsala , Desai Parth M. , Lingamaneni Prasanth , Palacios Pedro , Flores Estefania , Haque Zohaib , Nwaichi Chineme , Majeed Muhammad , Elkhouly Mohamed A. , Attar Bashar M. 
Published: October 2019

INTRODUCTION:
Cryoglobulinemic vasculitis(CV) can have variety of presentations including palpable purpura, arthralgias, glomerulonephritis, peripheral neuropathy, lymphadenopathy, hepatosplenomegaly, and hypocomplementemia. We present a patient with bilateral hand mononeuritis multiplex secondary to hepatitis C related CV.

CASE DESCRIPTION/METHODS:
A 52-year-old man with untreated hepatitis C (Viral load 520388, Genotype 1b), diabetes mellitus type II, hypertension and chronic kidney disease, presented with two months of bilateral hand pain. He also complained of numbness and tingling over the palmar aspects of the medial three digits of his hands, in addition to his medial left foot. He had no rash, arthritis, fevers, or weight loss. Physical exam was significant for positive Tinel's sign bilaterally, with weakness on finger extension, flexion and decreased power of interossei. Fundoscopy demonstrated non proliferative diabetic retinopathy (NPDR).Laboratory work-up revealed negative ANA, C-ANCA, MPO/PR3, HIV and RPR. Vitamin B12 and TSH levels were normal. However, serum cryoglobulins were positive with low C3, C4 and nephrotic range proteinuria Electromyography showed non-length-dependent neuropathy, which was more suggestive of mononeuritis multiplex rather than carpal tunnel syndrome (CTS). Sural nerve biopsy was performed and revealed vasculopathic changes consistent with vasculitis. He was diagnosed with mononeuritis multiplex secondary to hepatitis C related cryoglobulinemia. Immunosuppresive treatment was withheld because the patient tested positive for latent Tuberculosis (TB). He is currently undergoing treatment for the latent TB.

DISCUSSION:
CV is a small-to-medium-vessel vasculitis that occurs in 10 to 15 percent of patients chronically infected with hepatitis C virus (HCV). The two competing diagnosis for our patient’s neurological symptoms were diabetic neuropathy and CV secondary to untreated Hepatitis C. NPDR favored the microvascular complications of diabetes but the rapid onset of symptoms, positive cryoglobulins in the setting of HCV infection and sural nerve biopsy helped to establish the diagnosis of CV. This case is especially unique because it’s a very rare extrahepatic manifestation of HCV infection .Management of HCV cryoglobulinemic vasculitis involves administration of immunosuppressive therapy such as rituximab and antivirals but all patients should be tested and treated for latent TB prior to treatment initiation.
2311 Syphilis Hepatitis Presenting as a Mimic of Primary Biliary Cholangitis
Authors: Kern Cody , Blake Caroline M. , Hoellein Andrew R. 
Published: October 2019

INTRODUCTION:
Primary Biliary Cholangitis (PBC) is a progressive cholestatic liver disease defined by autoimmune destruction of intrahepatic bile ducts. Patients typically present with a cholestatic liver injury pattern. Anti-mitochondrial antibody (AMA) is present in 95% of PBC diagnoses thus, an AMA titer of 1:40 in addition to alkaline phosphatase (ALP) greater than 1.5 times the upper limit of normal is diagnostic of PBC (1). Syphilis is a sexually transmitted infectious disease caused by transmission of Treponema pallidum. The highest risk populations are men who have sex with men (MSM) and persons living with HIV. Syphilis has been further associated with HIV infection considering shared risk factors and transmission routes. While the staged progression of syphilis is well-documented, not all presentations are readily observed by clinicians considering the variability of the disease. One variation that is sparingly seen is syphilis presenting as acute hepatitis.

CASE DESCRIPTION/METHODS:
A 54 year-old man living with HIV presented to University of Kentucky Hospital with one week of abdominal pain, vomiting, and a diffuse non-pruritic rash. Upon admission he was noted to have a cholestatic liver injury pattern. Laboratory evaluation revealed AST 91, ALT 120, ALP 832, total bilirubin 6.4, and CD4 count 336. The acute hepatitis panel was negative. Abdominal CT and ultrasound failed to demonstrate biliary ductal dilation. Further investigation revealed an RPR titer 1:256 and an anti-mitochondrial M2 antibody IgG of 83.5U. The patient was treated for syphilis with doxycycline due an allergy to penicillin. Subsequently there was complete resolution of the cholestatic liver injury and a final diagnosis of syphilis hepatitis.

DISCUSSION:
In this report, we present a case of syphilis that presented similar to PBC with a consistent serologic pattern and a positive AMA. Other reports have recognized syphilis as a reversible cause of cholestatic liver injury. One study in particular, recognized a high false positive AMA rate in patients with syphilis (2). As, syphilis is a easily targeted cause of liver injury, we suggest testing high-risk patients with a presumed diagnosis of PBC.
2318 A Rare Case of Cytomegalovirus Hepatitis Complicated by Secondary Syphilis Hepatitis in an Immunocompetent Patient
Authors: Qiu He , Lim Huei-Wen , Lee Tai-Ping 
Published: October 2019

INTRODUCTION:
Cytomegalovirus (CMV) hepatitis most commonly presents with subclinical transaminitis and less commonly with the elevation of alkaline phosphatase (ALP) and total bilirubin (TB). Syphilis hepatitis, on the other hand, generally has a non-specific presentation with a more cholestatic picture. We present a rare case of cholestatic hepatitis from acute CMV infection complicated by syphilis hepatitis.

CASE DESCRIPTION/METHODS:
A 57-year-old homosexual man presented to the emergency room with complaints of intermittent fever and general malaise for the past two weeks. Blood work showed aspartate aminotransferase (AST) of 235 U/L, alanine aminotransferase (ALT) of 284 U/L, ALP of 940 U/L. TB and coagulation profile were normal. Acute hepatitis A, B, C, Epstein-Barr virus, and herpes simplex virus were ruled out. HIV was negative. Workup for autoimmune hepatitis, primary biliary cholangitis, alpha-1-antitrypsin deficiency, and Wilson’s disease was all negative. Abdominal CT scan with contrast was notable for hepatosplenomegaly without liver lesion. CMV IgM was positive with PCR of 7490 copies/mL, indicating acute CMV infection. The patient subsequently improved without treatment. He presented two months later with worsening liver tests with a peaked ALP of 1981 U/L, TB of 1.6 mg/dL, AST 230 U/L, ALT 195 U/L, associated with the macular rash on his trunk, abdomen, both palms, and soles. Abdominal MRI with contrast showed three < 2cm liver lesions. The patient was subsequently diagnosed with secondary syphilis with rapid plasma reagin (RPR) of 1:16 and confirmatory positive fluorescent treponemal antibody absorption (FTA-ABS). Biopsy of the liver lesions showed granulomatous inflammation with negative immunohistochemistry for CMV which was compatible with clinical syphilis hepatitis. The patient was treated with penicillin G. His rash resolved in 2 months with significant improvement of liver tests. The CMV PCR and RPR were negative with normalization of liver enzymes in 4 months. Follow-up MRI showed resolution of the liver lesions.

DISCUSSION:
Our patient initially had an atypical presentation of acute CMV hepatitis with cholestatic features. He subsequently developed secondary syphilis associated hepatitis with severe intrahepatic cholestasis and liver lesions. This is, to our knowledge, the first case of CMV hepatitis complicated by syphilis hepatitis. Resolving CMV hepatitis with recurrent elevation in liver tests warrant the workup for possible superimposed liver disease.