bluuebunny/crossref_metadata_2025 huggingface dataset:
2244 Getting to the Root of the Matter: A Case of Valerian Root-Induced Hepatitis and Pancreatitis
Authors: Bergagnini Isabella , Kadosh David , Nguyen Andrew , Swaminath Arun
Published: October 2019

INTRODUCTION:

The use of herbal supplements has increased substantially in recent years for amelioration of common ailments. Valerian root, a common over-the-counter supplement (OTC) used for insomnia and anxiety, has been in rare instances linked to hepatotoxicity and pancreatitis, as depicted in the case below.

CASE DESCRIPTION/METHODS:

A 36-year-old gentleman with no significant medical history presented with worsening burning abdominal pain, nausea and vomiting for one day with similar symptoms three months prior. Labs were notable for elevated AST/ALT 682/619, GGT 100, and lipase 220. Abdominal ultrasound showed mildly echogenic liver with no intrahepatic or extrahepatic ductal dilatation or cholelithiasis, and patent hepatic and portal veins. CT abdomen and pelvis demonstrated acute edematous interstitial pancreatitis with a small heterogeneous area concerning for necrosis. The patient revealed routine use of multiple OTC supplements including Driftoff and Epi-dry, and Valerian root, each of which contain Valerian officinalis. The remaining workup was negative for infectious, autoimmune, and other toxic etiologies. On day two, AST/ALT peaked at 2398/3132 and N-acetylcysteine (NAC) was initiated. Labs and symptoms improved and he was discharged home on day four. At follow-up in 2 weeks, he was asymptomatic, remained off supplements, with normalization of his transaminases.

DISCUSSION:

Valerian officinalis is increasingly used for its sleep aid properties and anxiolytic effects. Its interaction with gamma-aminobutyric acid (GABA) receptor produces effects similar to benzodiazepines though clinical data is scant. Presentation of valerian root induced hepatotoxicity varies, ranging from no symptoms to overt jaundice, with abdominal pain as the most common symptom. Liver enzyme elevation is of hepatocellular or hepatocellular-cholestatic pattern with aberrancies seen 3-12 weeks later. While the mechanism of hepatotoxicity remains unknown, the literature suggests gallstone formation may be responsible for valerian root induced pancreatitis unlike our case which suggests a drug induced injury. Effects are self-limited though abstaining from the causative agent is paramount. Management often remains supportive. Although data is limited, NAC has been used in non-acetaminophen induced hepatotoxicity and aided in improvement in liver injury in our patient. We present this case to raise awareness of this potential toxin and consider this in the differential when evaluating concomitant hepatitis and pancreatitis.
2245 Thoracic Neuroendocrine Tumor With Hepatic Metastases: An Ideal Case for Radioembolization
Authors: Kolli Sindhura , Aamar Ali , Etienne Denzil
Published: October 2019

INTRODUCTION:

This rare case of a thoracic neuroendocrine tumor (NET) metastasizing to the liver is meant to bring attention to the lag in diagnosis until symptoms from the metastases become discernible. It also acknowledges recent literature on radioembolization efficacy in disease control rate as well as symptom resolution for NET with liver metastases, in particular.

CASE DESCRIPTION/METHODS:

A 56-year-old male patient with cirrhosis secondary to hepatitis C s/p treatment presented with RUQ abdominal pain, fever, chills, and fatigue. Labs show elevated AST/ALT/ALP of 64/72/227, respectively. A CT abdomen and MRCP demonstrated a cirrhotic liver with multiple hypodense ill-defined hepatic lesions with portocaval lymphadenopathy. An EGD discovered 4 non-bleeding esophageal varices and hypertensive portal gastropathy (Figure 2). EUS-guided liver and periportal lymph node biopsies returned positive for a high grade poorly differentiated metastatic NET with greater than 50% positive for KI-67. Octreotide scan was negative. PET scan indicated the primary source as possibly thoracic given mediastinal involvement. Currently, patient is receiving cycles of carboplatin and etoposide.

DISCUSSION:

NETs arise from neurological and hormonal cells within any organ system and are classified by their anatomical location. Incidence is 5/100,000 with total prevalence 35/100,000, with the highest proportion reported in African Americans. Genetically, they are associated with MEN1, VHL, and tuberous sclerosis. Symptoms are present for 5-7 years before finally being diagnosed and include abdominal pain, diarrhea and fatigue. They are detected sooner if they are functional tumors that secrete hormones, such as serotonin, causing carcinoid syndrome. A majority, however, are non-secretory or non-functional and found incidentally. For diagnosis and staging, a CT or MRI and Ki-67 IHC staining. An octreotide scan is sensitive for a well-differentiated NET, whereas a PET scan is more sensitive for poorly differentiated or high grade tumors. Treatment entails laparoscopic removal or debulking, local hepatic ablation via radiofrequency, cryotherapy or electroporation, or embolization of the hepatic artery. For those with metastases or poor surgical candidates, chemotherapy with cisplatin and streptozocin are widely used. A recent study showed that radioembolization for NETs with hepatic metastases had a 91% disease control rate and 79% had resolution of NET-related symptoms.
2248 Primary Malignant Mesothelioma of Liver: A Rare Liver Tumor
Authors: Ghimire Subash , Yang Tsujung , Shah Harshil , Srivatana Ukorn , Sarker Ashit
Published: October 2019

INTRODUCTION:

Primary malignant mesothelioma of liver is an extremely rare condition. Less than twenty cases have been reported so far in literature. This presents as liver mass and differentials include primary and secondary tumors of the liver. We describe the case of a 70-year-old male with asbestos exposure who was incidentally found to have primary malignant mesothelioma of liver in screening CT scan.

CASE DESCRIPTION/METHODS:

A 70-year-old non-smoker, nonalcoholic male with past medical history of pulmonary asbestosis (had a stable calcified pleural plaques, no evidence of metastatic disease in the chest) being followed with annual low dose CT scan for asbestosis exposure management was found to have an incidental liver mass in the CT scan. He did not have icterus, itching of the skin or change in his stool color or weight loss. Upon examination, he was found to be hemodynamically stable, there was no jaundice. There was no right upper quadrant tenderness on palpation. CT scan of abdomen with contrast was obtained that showed an 8 cm mass involving the right lobe of liver predominantly the segment 6 (Figure 1). This mass was highly concerning for liver malignancy. A bone scan was obtained for metastatic workup which did not reveal any metastatic disease. CT scan of the chest was done that did not show any evidence of hematogenous metastasis or mesothelioma in the thorax. His hepatitis panel, AFP level were negative. There was no elevation of liver enzymes. The patient underwent partial hepatectomy (segments V/VI), diaphragm resection and cholecystectomy. Biopsy of the liver mass showed a poorly differentiated malignancy, showing areas of epitheloid and spindle cell morphology. The tumor cells were positive for keratin AE1/AE3, keratin CAM 5.2, WT1, calretinin, CK20 and D2-40. Tumor cells were negative for arginase, CK 5/6, ERG, mucicarmine, MOC 31, BerEp4, desmin, S–100, and albumin in situ hybridization. The overall morphology as well as immunohistochemical staining pattern were most consistent with malignant mesothelioma. The patient was started on adjuvant chemotherapy with Pemetrexed and Cisplatin.

DISCUSSION:

Primary malignant mesothelioma of liver is an extremely rare condition. This presents a solitary lesion in the liver. Treatment includes surgical resection followed by adjuvant chemotherapy with Pemetrexed and Cisplatin. It should be considered as a differential in patients with solitary liver mass.
2249 Rectus Sheath Hematoma Post Abdominal Paracentesis: A Rare Complication
Authors: Laoveeravat Passisd , Tikue Alay M. , Thavaraputta Subhanudh , Suchartlikitwong Sakolwan , Mingbunjerdsuk Thammasak , Thongtan Thanita , Nugent Kenneth
Published: October 2019

INTRODUCTION:

Rectus sheath hematoma (RSH) is a very rare complication after abdominal paracentesis especially in patients who do not take antiplatelet or anticoagulants. We presented a case of rectus sheath hematoma post paracentesis in a patient without obvious risk factors.

CASE DESCRIPTION/METHODS:

A 56-year-old male with past medical histories of chronic diastolic congestive heart failure and end-stage renal disease (ESRD) on hemodialysis presented with abdominal pain after abdominal paracentesis for ascites. 4.3 L of yellow ascitic fluid was drained in the first attempt of ultrasound-guided paracentesis. Abdominal distension on the right flank was also noticed. Vital signs were stable. Abdominal exam showed moderate tenderness on right flank, no guarding, no rebound tenderness, no bruising. ESRD was believed to be the cause of ascites. He received hemodialysis one day prior to the procedure. He is not on any antiplatelet or anticoagulation therapy. Computed tomography scan of abdomen showed right rectus sheath hematoma 14 × 9 cm (Figure 1). Labs on admission showed Hemoglobin (Hb) of 9.2 g/dL (compared to baseline Hb of 10.8 g/dL), Platelet count 192000 /uL, PT 13.9 secs, PTT 34.6 secs, and INR 1.22, BUN 42 mg/dL, Creatinine (Cr) 5.6 mg/dL (similar to baseline Cr). Interventional radiologist was emergently consulted and performed embolization which found extravasation from the right iliolumbar artery (Figure 2). Coil embolization was done successfully. On post-embolization day 3, Hb continued to drop to 5.7 g/dL. He developed hypotension. Surgery team was notified and brought the patient to operation room for exploratory laparotomy. Intraoperative findings revealed that 2 L of hematoma was evacuated from the patient’s rectus sheath. No active bleeding was immediately noted. Upon the exploration of the anterior wall toward the medial side of the incision, a small bleeding vein was noted and ligated. Vital signs became stable after the operation. He was discharged without postoperative complications.

DISCUSSION:

Large RSH is a rare complication of paracentesis. It can also develop in a patient without obvious bleeding risk factors. Clinicians should watch for this potential complication when a patient presents with an abdominal mass or abdominal pain without other particular symptoms after paracentesis. RSH is a self-limited condition; however, our case suggests that RSH may take an unpredictable course and may not be successfully managed with a conservative approach.
2252 From Lungs to Liver: Acute Budd-Chiari Syndrome Secondary to Small Cell Lung Carcinoma
Authors: Weismiller Scott , Ganapini Vincent , Ramasamy Kovil
Published: October 2019

INTRODUCTION:

Budd-Chiari syndrome (BCS) is any process that inhibits hepatic venous outflow. It is caused by malignancy in 10% of all cases, either through hepatic vein / inferior vena cava (IVC) thrombosis and / or vascular compression due to mass effect. Clinical suspicion should remain high when abdominal pain, jaundice, and hepatomegaly are present with elevated transaminases, alkaline phosphatase, and bilirubin. This is a rare case of acute BCS caused by metastatic small cell lung carcinoma (SCLC) compressing the IVC.

CASE DESCRIPTION/METHODS:

A 61 y/o male with hypertension, dyslipidemia, asthma, and nonalcoholic fatty liver disease presented to the ED for URI symptoms refractory to antibiotics and steroids. He denied weight changes, hemoptysis, nausea, abdominal pain, sick contacts, and recent travel. The patient reported 22 years of second-hand smoke exposure as a police officer. He denied alcohol, tobacco, or illicit drug use. On exam he had a firm, distended abdomen. Laboratory exam showed: WBC: 18.8 103/µL, lactate: 2.8 mmol/L, BUN: 25 mg/dL, Tbili: 3.0 mg/dL, AST: 637 U/L, ALT: 606 U/L, ALP: 362 U/L, and LDH: 11173 U/L. Autoimmune, infectious, and toxicology workup was negative. Abdominal US and CT revealed hypoechoic liver masses and hilar / mediastinal lymphadenopathy. His liver biopsy identified a primary small cell carcinoma. The patient developed severe elevation of transaminases and acute kidney injury. RUQ tenderness, hepatomegaly, and jaundice correlated with worsening liver and kidney tests (Figures 1, 2). Abdominal CT revealed extra-luminal tumor compression of the IVC (Figure 3). The oncologist began carboplatin / etoposide in the ICU with rapid improvement of his labs and symptoms. The final diagnosis was stage IV SCLC complicated by BCS. The patient expired six months later.

DISCUSSION:

BCS caused by primary lung carcinoma (PLC) is rare; less than a dozen cases are reported in the literature. Prompt diagnosis of PLC allowed for targeted chemotherapy, which reduced tumor burden, improved symptoms, prevented acute liver and renal failure, and prolonged life. Rapid treatment may have prevented ascites by restoring hepatic venous pressures. Clinical suspicion of BCS should be based on physical exam and lab findings even with negative doppler US or CT. Past cases showed post-mortem tumor invasion of hepatic vasculature despite negative imaging. The overall prognosis for stage IV SCLC is poor, making early immunohistochemical analysis, tumor identification, and treatment method paramount.
2255 Acute on Chronic Hepatic Failure From Primary Amyloidosis (AL): A Fatal Complication
Authors: Rashid Mamoon Ur , Khan Muzammil Muhammad , Ullah Waqas , Zafar Hammad , Hussain Ishtiaq , Hasan Badar , Ur Rehman Fnu Asad , Hurairah Abu
Published: October 2019

INTRODUCTION:

Hepatic involvement in primary amyloidosis (AL) is common but rarely causes severe cholestasis. We are here reporting a case of acute on chronic hepatic failure from amyloidosis leading to a severe decline in patient’s clinical status and fatality.

CASE DESCRIPTION/METHODS:

44 years old man was initially seen for persistent cough and recurrent right-sided pleural effusion. Thoracentesis showed exudative effusion. A right VATS with decortication was done. Pathology showed extensive caseating granulomas and tissue cultures came back positive for MTB. Initial labs showed elevated LFT’s with a cholestatic pattern. The patient was started on levofloxacin, rifabutin and ethambutol due to less hepatotoxic profile. During hospitalization, patient LFTs kept on worsening. Medications were stopped and a liver biopsy was done. There was significant involvement of the liver by amyloidosis (AL). Patient further workup included a bone marrow biopsy which revealed 30-40% cellularity, 5-10% plasma cells, amyloidosis AL (Kappra type) consistent with multiple myeloma. Oncology team recommended chemotherapy with CyborD (Bortezomib, dexamethasone and cyclophosphamide) for multiple myeloma. In spite of chemotherapy patient hepatic, renal and cardiac functions were worsening. Patient developed abdominal pain, moderate ascites and hypotension with elevated INR and bilirubin of 30. He was ultimately transferred to ICU where he developed cardiac arrest and could not survive.

DISCUSSION:

Primary amyloidosis (AL) is the most common form of systemic amyloidosis with mean age of diagnosis 65 years and less than 10% of patients being 50 years of age. As compared to myeloproliferative disease, leukemias and lymphomas, hepatic involvement is less common in multiple myeloma (32%). Amyloid deposits may be seen in the liver with hepatomegaly and normal to mild elevation of hepatic enzymes but serious manifestations such as hepatic failure and portal hypertension are rare. Previously in literature very few cases of acute hepatic failure from AL amyloidosis have been reported. Diagnosis is made by biopsy of the involved site showing amyloid deposits. There has been reported limited success for liver transplantation as the disease progression is unchanged in the presence of plasma cell dyscrasias. In the literature combined approach with autologous stem cell transplant and liver transplant have been suggested but the prognosis still remains poor.
2257 Hepatic Dysfunction as a Result of Multiple Myeloma
Authors: Taborda Cesar , Barrett Amanda , Li Shiyong , Vora Ravi
Published: October 2019

INTRODUCTION:

Plasma cell infiltration of the liver can be detected in up to 45% of patients with multiple myeloma (MM) at autopsy. However, only a few cases have been reported of plasma cell infiltration of the liver leading to plasmacytoma formation and non-obstructive cholestasis with progression to liver failure.

CASE DESCRIPTION/METHODS:

A 53-year-old African American male with history of IgG multiple myeloma status post AlloimmuneStem Cell transplant (ASCT) in 2010 was admitted for intermittent epistaxis, hemoptysis and fatigue found to have relapse. Patient was started on salvage therapy with bortezomib, dexamethasone, cyclophosphamide, etoposide and cisplatin (VDCEP). Physical exam revealed an alert and lethargic male in no acute distress but with asterixis. Sclera were icteric. Abdominal exam revealed marked hepatomegaly without tenderness. Laboratory data showed an ALT of 60 IU/L, total bilirubin of 11mg/dL and normal AST and ALP . INR was 3.0, platelets 38 × 103 mcL, Hgb 7.6gm/dL and WBC 1.6 × 103 mcL. Factor VIII activity was 311.1%. Ammonia levels were 67mcmol/L. Imaging with MRI showed an enlarged liver measuring 22.4cm without mass lesions. A transjugular liver biopsy was then performed revealing atypical mononuclear cell infiltration with positive stain for CD138 and MUM1 consistent with patient’s known history of labda-restricted plasma cell neoplasm (Figures 1 and 2). His clinical course deteriorated with worsening total bilirubin, hepatic encephalopathy and coagulopathy consistent with liver dysfunction and acute liver failure. Eventually there was development of multiorgan failure and death. Autopsy confirmed presence of myeloma cells in the liver forming a subcapsular plasmacytoma with associated extensive cholestasis and hepatocyte necrosis.

DISCUSSION:

Hepatic involvement of multiple myeloma most commonly presents with hepatomegaly and portends poor prognosis. It is important to recognize that patients may present with a cholestatic pattern of liver injury which can lead to severe hepatic dysfunction and liver failure such as the case of our patient. The histological pattern of liver involvement in MM can be in the form of light chain deposition disease, extramedullary plasmacytoma, amyloidosis, or a diffuse infiltrative pattern. Liver biopsy is thus an imperative way of diagnosis particularly to differentiate it from other entities causing similar pattern of injury such as sinusoidal occlusive syndrome or drug induced liver injury.
2259 Severely Jaundiced Body Builder: A Case of Acute Cholestatic Syndrome With Anabolic Androgenic Steroid Use
Authors: Flores Estefania , Fu Damon , Attar Bashar M. , Palacios Pedro , Vohra Ishaan , Demetria Melchor V.
Published: October 2019

INTRODUCTION:

Illicit use of anabolic androgenic steroids (AAS) has been growing among athletes and bodybuilders for cosmetic and performance purposes. We report a case of one of the possible hepatic complications of AAS, acute cholestatic syndrome (ACS), in a male with anabolic steroid abuse.

CASE DESCRIPTION/METHODS:

27-year-old Polish man with no past medical history presented with jaundice, itching of the body, dark-colored urine, clay-colored stools for 5 weeks. No abdominal pain was reported. Patient reported taking for 4 months 4 types of AAS (testosterone, oxandrolone, methasterone and trenbolone) for body building, which he stopped taking 6 weeks prior to initial presentation due to fatigue. Vitals were normal, and on exam he appeared to be a muscular, jaundiced male with scratch marks on his skin. Abdominal exam revealed a non-tender, non-distended abdomen, with difficulty palpating the liver edge due to the muscularity. Laboratory tests were significant for total bilirubin of 34.8 mg/dL with direct of 23.5 mg/dL, AST 55 U/L, ALT 70 U/L, ALP 212 U/L, GGT 57 U/L, INR 1, creatinine 1.1 mg/dL. Liver ultrasound showed hepatomegaly with no biliary dilation and patent hepatic and portal veins. A liver biopsy was obtained showing portal neutrophil predominant inflammation with occasional eosinophils, mild portal fibrosis and cholestasis, suggestive of drug-induced hepatotoxicity. Liver function tests normalized after 4 months of cessation of AAS use, with resolution of his jaundice and pruritis.

DISCUSSION:

Body builders usually prefer polydrug use of AAS to maximize the desired effects and hypothetically reduce side effects at the same time. But contrary to their belief, the concomitant use of different illicit AAS may be a factor in their toxicity, making difficult to link an adverse effect with a specific substance. Acute cholesta tic syndrome, has been associated with use of the 17α – alkylated anabolic steroids ( i.e. oxandrolone, methasterone), thought related to an increase of reactive oxygen. This leads to prolonged direct toxic effects and intrahepatic cholestasis, though the pathophysiology remains unclear. ACS usually occurs 1-4 months after steroid initiation presenting with nausea, fatigue, pruritis; later, jaundice, dark urine, with significantly elevated total bilirubin and ALP but mild aminotransferase elevation can be seen. Management consists of stopping anabolic steroid use, supportive care and symptomatic treatment of pruritis.
2266 Simultaneous Coronary Artery Bypass Grafting and Orthotopic Liver Transplantation
Authors: Lourdusamy Vennis , Albers Christopher , Kemmer Nyingi , Syed Rashid , Malespin Miguel , Dhanireddy Kiran , Lu Amy , Porubsky Marian , Dunning John , Agrawal Saurabh
Published: October 2019

INTRODUCTION:

Patients with Decompensated Cirrhosis requiring Coronary artery bypass grafting (CABG) before liver transplantation are at significant risk of liver failure and death. We discuss a simultaneous CABG and liver transplantation approach to offer the best therapeutic option in such cases.

CASE DESCRIPTION/METHODS:

Our patient is a 56-year-old male with decompensated cirrhosis related to Ethanol and Fatty liver. He had severe coronary artery disease of the proximal, mid and distal Right coronary artery (RCA) not amenable to PCI. Once a donor liver allograft was found to be suitable, simultaneous CABG with liver transplantation was performed. Initially after procuring the saphenous vein from the right leg, a median sternotomy incision was performed and the pericardium was opened. Single vessel CABG to RCA was performed using the Saphenous vein graft off cardiopulmonary bypass. Chest was left open for the liver transplantation surgery. A bilateral subcostal incision with midline extension was performed. After explantation of the native liver, donor liver implantation was performed in a piggyback fashion where the venocavoplasty formed using middle and left hepatic vein of the recipient was anastomosed with the donor IVC. Venovenous bypass was not used. A Choledochocholedochostomy was performed. There were no major perioperative complications. A month after surgery, he has normal liver function and is recovering at home.

DISCUSSION:

In advanced liver disease patients needing liver transplantation and with concurrent CAD not amenable to PCI, it is not always possible to perform staged surgeries. In our case the anticipated morbidity and mortality rate was high with either surgery performed alone. Our patient’s probability of post operative mortality one year after major surgery according to the Mayo Clinic risk calculator was 32%. Recipient hepatectomy during liver transplantation in the background of severe CAD and with already reduced systemic pressure could result in major hemodynamic instability and acute coronary ischemia. He underwent simultaneous CABG and liver transplantation successfully. Survival rates of 80% have been reported in case series from more than a decade ago (Table 1). All patients should have preserved ventricular function. Simultaneous CABG and liver transplantation can offer the best therapeutic option with good perioperative and long term survival rate for patients with advanced liver disease who have severe CAD not amenable to PCI.
2267 Drug-Induced Autoimmune Hepatitis From Valerian Root Tea
Authors: Lourdusamy Vennis , Albers Christopher , Malespin Miguel , Kemmer Nyingi , Agrawal Saurabh , Syed Rashid
Published: October 2019

INTRODUCTION:

Differentiating drug induced liver injury from other causes of acute hepatitis, especially autoimmune hepatitis can be difficult secondary to overlapping histological features and confounding laboratory abnormalities. Stopping the offending drug should usually result in resolution of liver injury. We describe a rare case of drug induced liver injury secondary to valerian root tea with overlapping features of autoimmune hepatitis which resolved with limited duration steroid therapy.

CASE DESCRIPTION/METHODS:

Our patient is a 47-year-old female with hypothyroidism who presented with acute onset of generalized abdominal discomfort, jaundice and vomiting without fever or confusion. She denied prior similar episodes. She reported regular use of valerian tea for insomnia for a duration of 3 months prior to presentation. Physical examination revealed jaundice, mild diffuse abdominal tenderness and no features of chronic liver disease. Labs showed elevated alanine aminotransferase of 2741 U/L, aspartate aminotransferase of 1847 U/L, alkaline phosphatase of 199 U/L, total bilirubin of 9.2 mg/dL, albumin of 3.4 g/dL and International normalized ratio of 1.4. Autoimmune work up revealed positive smooth muscle antibody, mildly elevated Immunoglobulin G of 1980 mg/dL and negative antinuclear and mitochondrial antibodies. Viral hepatitis panel was negative. Imaging of the abdomen was unremarkable. Liver biopsy showed severe acute hepatitis with extensive necroinflammatory activity with plasma cells and bridging necrosis without significant fibrosis. With no significant improvement in liver enzymes after stopping valerian root tea and with concern for autoimmune hepatitis, she was started on prednisone 60 mg daily with improvement of transaminitis. Transaminases have remained normal for several months after stopping prednisone (Table 1, Figure 1).

DISCUSSION:

Diagnosing autoimmune like drug induced liver injury and differentiating it from autoimmune hepatitis can be difficult. Steroids should be considered in cases with severe or prolonged elevation of liver enzymes that do not resolve after stopping the offending drug. Valerian root can cause severe form of autoimmune like drug induced liver injury that responds to steroids. Complete recovery is possible with steroids for limited duration. Relapse following steroid cessation should prompt further evaluation for autoimmune hepatitis.
2269 An Unprovoked Crisis: Hemophagocytic Lymphohistiocytosis
Authors: Zghyer Fawzi , Buffie Charlie , Wan David
Published: October 2019

INTRODUCTION:

Hemophagocytic Lymphohistiocytosis (HLH) is a syndrome of dysregulated immune response due to abnormal immune activation which can be life threatening and very aggressive. Here we report a woman with jaundice found to have hemophagocytosis on a bone marrow aspirate.

CASE DESCRIPTION/METHODS:

A 48-year-old woman with no past medical history presented with a diffuse erythematous morbilliform rash, fever, chills, and headache for one week. The headache is described as throbbing with associated photophobia. There was no history of travel, or hiking. On physical exam, the patient was afebrile but tachycardic, her BP was 85/64 mmHg, she was ill appearing, lethargic, and had scleral icterus. Her abdomen was tender to palpation with hepatosplenomegaly. She had a diffuse maculopapular rash. Labs were notable for hypoproliferative anemia, bandemia of 36%, thrombocytopenia, elevated AST/ALT, total bilirubin with predominance of direct bilirubin, alkaline phosphatase, and lactate. CT abdomen revealed moderate diffuse colonic wall thickening from the cecum to rectum. In the meantime, a very extensive infectious workup was sent. A bone marrow biopsy revealed a mildly hypercellular marrow with hemophagocytosis. Soluble CD-25 was found to be significantly elevated. The patient’s mental status continued to deteriorate and had to be transferred to MICU for airway protection. She received steroids, etoposide, and was eventually started on emapalumab due to progression in symptoms. The patient significantly improved, and was extubated. Over two weeks her mental status returned back to baseline.

DISCUSSION:

HLH results from the inability to downregulate active macrophages and lymphocytes. The primary tissue damage mechanism is thought to be due to cytokine release secondary to immune activation. The instigating factor for HLH is typically an infection or an altered immune homeostasis state. The typical presentation of HLH involves fevers, bicytopenia, splenomegaly, hemophagocytosis, elevated soluble CD25, hypertriglyceridema, hepatitis, and elevated ferritin. The dilemma in the case we present remains in the trigger for HLH; the extensive infectious workup was completely unrevealing, with the patient being negative to some of the very common viral pathogens. There is no family history of HLH or any immune related disorders, and she had no past medical history. With that being said, an episode of HLH of unknown etiology seems to have been the cause of her symptoms.
2270 The Path to Encephalopathy: A Case of Spontaneous Portosystemic Shunt Embolization
Authors: Jenkins Alexander , Skaret Michael M. , Hussell John J. , Kwok Ryan
Published: October 2019

INTRODUCTION:

Hepatic encephalopathy (HE) is a debilitating complication of cirrhosis that can lead to alterations in consciousness, behavior, and overall functional decline. It has been suggested that up to 70% of patients with HE refractory to medical treatment have evidence of spontaneous portosystemic shunts (SPSS). While this is a relatively well-known sequela of longstanding cirrhosis, and current guidelines recommend at least consideration of closure of SPSS if found, the literature is limited regarding procedural occlusion of these potentially harmful vascular phenomena.

CASE DESCRIPTION/METHODS:

We present the case of a 70-year-old female with cirrhosis secondary to non-alcoholic steatohepatitis and progressive ascites requiring repeated therapeutic paracentesis. Over a short period, her need for paracentesis inexplicably declined and her HE simultaneously worsened, despite appropriately titrated lactulose. Imaging at this point revealed spontaneous splenosystemic and intrahepatic shunts. At the time of presentation, the patient’s Model for End-Stage Liver Disease (MELD) score was 14. Despite optimization of medical therapy and shunt embolization, the patient’s mental status did not improve. Upon further review of post-embolization imaging, there was evidence of shunt collateralization that still appeared to be patent. This patient’s lack of clinical improvement may have been due to several factors, but incomplete embolization of known shunts or presence of additional undiagnosed shunts seem most likely.

DISCUSSION:

As hepatic fibrosis progresses, portal hypertension rises steadily to the point where otherwise insignificant portosystemic microvasculature can engorge to sizes greater than 1 centimeter. This phenomenon can relieve portal hypertension at the expense of releasing higher levels of ammonia and other neurotoxins into systemic circulation, resulting in more pronounced HE. While the decision to pursue embolization should be individualized, the presence of SPSS should always be considered in the setting of refractory HE. A MELD score of 11 or less may be associated with more favorable outcomes following embolization of SPSS and a majority of patients show rapid clinical improvement of HE following successful shunt closure. Dedicated post-embolization imaging should be obtained, especially if a patient’s HE remains refractory after intervention.
2271 A Case of Portomesenteric Venous Thrombosis After Laparoscopic Sleeve Gastrectomy
Authors: Ramachandran Rajarajeshwari , Jani Niraj
Published: October 2019

INTRODUCTION:

Portomesenteric venous thrombosis (PMVT) after laparoscopic sleeve gastrectomy (LSG) is exceedingly rare. We present the case of a 42-year-old woman who underwent an uncomplicated LSG and developed PMVT.

CASE DESCRIPTION/METHODS:

42-year-old woman with morbid obesity was admitted to the hospital with nausea, vomiting and epigastric pain on the 11th post-operative day after undergoing an uncomplicated LSG. Examination: Vital signs - BP 130/82 mmHg, pulse 100/min and temperature 36.9 °C. BMI – 45 kg/m2. Tenderness in the epigastric and umbilical area. No rebound or guarding. Bowel sounds were absent. Laboratory data: White blood cell count - 16.22 × 10³/microliter with neutrophil predominance, hemoglobin 12.3 g/deciliter, platelets 242 × 10³/microliter, lipase 15 IU/L, normal electrolyte levels/renal and hepatic function. Imaging studies: CT scan of the abdomen with contrast - portal and superior mesenteric thrombosis accompanied by peri-pancreatic edema and thickened small bowel loops. These findings were highly suggestive of venous ischemia. Anticoagulation with unfractionated heparin was initiated immediately and patient was aggressively volume resuscitated. Thrombophilia workup – negative factor V Leiden mutation, anti-cardiolipin antibodies and prothrombin/factor II gene mutation. Patient’s condition steadily improved and she was discharged home on oral anti-coagulation.

DISCUSSION:

PMVT is a rare complication of LSG with non-specific clinical presentation. Prompt diagnosis requires a high index of suspicion. Common clinical presentation includes abdominal pain, nausea, vomiting and low-grade fever. During LSG, there is increased intraabdominal pressure with carbon dioxide pneumoperitoneum. It is suspected that a decrease in the portal blood flow may contribute to the prothrombotic state in the portomesenteric circulation. The definitive diagnosis of PMVT is made with noninvasive imagining such as contrast-enhanced computed tomography (CT) or color Doppler ultrasonography. Therapeutic anticoagulation is recommended in stable patients with acute PMVT for 6-12 months. Acquired and inherited hypercoagulable conditions should be considered as predisposing factors for PMVT and require lifelong anticoagulation.
2272 A Case of Cryptococcal Hepatitis in HIV Patient With a Negative Serum Cryptococcal Antigen
Authors: Estifan Elias , Laxina Ian , Hlayhel Ahmad , Adib Sami , Baddoura Walid
Published: October 2019

INTRODUCTION:

Infectious Cryptococcus neoformans occurs primarily in immunocompromised patients. The primary organ affected is the lungs, but CNS and skin involvement can also be seen. Disseminated Cryptococcosis can involve any organ in the body. However, hepatic involvement is rare. Here we offer a case of Cryptococcus hepatitis in a patient who presented with persistently elevated liver enzymes.

CASE DESCRIPTION/METHODS:

A 56-year-old Hispanic female with no known past medical history presented with fever, abdominal pain, nausea, unintentional weight loss, and diarrhea for two months. On physical examination, she was tachycardic with a heart rate of 110 bpm, and her abdomen was soft but tender to palpation in the right upper quadrant. Her liver function tests (LFTs) revealed elevated Aspartate Aminotransferase (AST) (415 U/L), elevated Alanine Aminotransferase (ALT) (201 U/L), elevated Alkaline Phosphatase (ALP) (763 U/L), normal total Bilirubin (0.9 mg/dl), and additional laboratory results in Table 1. Her HIV antigen screening was reactive, and absolute CD4 count was 22/ mcL. CT scan of the chest revealed two small calcified granulomas, for which she was started prophylactically on Azithromycin and TMP/SMZ. Over the course of her hospital stay, the patient's liver enzymes continued to trend upward while further workup ruled out hepatitis A, B, C, Wilson's disease, Hemochromatosis, AIH, PBC, PSC, other causes of viral hepatitis (CMV, EBV, HSV), with negative Histoplasma antibodies and negative serum Cryptococcal antigen titers. During the second week of hospitalization, her liver enzymes continued to rise with an ALP of 4046 U/L, AST of 407 U/L, and ALT of 276 U/L. With a persistent elevation of the liver enzymes without any definitive cause, an ultrasound-guided biopsy was warranted and performed. Pathology revealed Cryptococcal hepatitis (Figures 1 and 2), and the patient was started on a 15-day course of Amphotericin B with an 8-week course of Fluconazole 400 mg with LFTs nearly normalizing at 6 weeks.

DISCUSSION:

This case demonstrates an unusual manifestation of Cryptococcosis. Our patient did not present with the typical Cryptococcal pulmonary or central nervous system infection. As well, our patient's serum Cryptococcal antigen titers were negative, but biopsy results revealed Cryptococcal hepatitis, despite a very high sensitivity and specificity of the serum Cryptococcal antigen test. This case demonstrates the importance of maintaining a broad differential, specifically in immunocompromised patients.
2275 Anoxic Brain Injury From Hepatic Encephalopathy in Acute on Chronic Liver Failure: A Case Report
Authors: Aijazi Muaz , Noor Ahmed
Published: October 2019

INTRODUCTION:

Hepatic encephalopathy (HE) is a complication seen in both acute liver failure and acute on chronic liver failure (ACLF). There is a paucity of data regarding IV HE resulting in global anoxic brain injury in those with ACLF. We present a case of anoxic brain injury from severe HE in a patient with ACLF.

CASE DESCRIPTION/METHODS:

74-year-old male with a past medical history of hypertension and CAD had recently been diagnosed with NASH cirrhosis when he presented to an outside hospital with ascites. Two months later he presented to our hospital with acute change in mental status. On presentation, the patient had normal vital signs. Physical examination revealed obtundation with response to noxious stimuli. There was no scleral icterus and labs were significant for WBC 5.1, platelet count 82, BUN/Cr 32/1.81, lactate 3.2, NH3 248, LFTs unremarkable, MELD Na 23. CT head and chest X-ray were negative for acute processes. On physical exam, patient was non-jaundiced with a mildly distended abdomen. He was started on lactulose every 6 hours. His mentation continuously deteriorated and he was intubated for airway protection and transferred to ICU 8 hours after admission, where he was started on rifaximin. Nephrology was consulted and CRRT was initiated after NH3 was noted to be >500 in the ICU. Repeat CT head now showed increased intracranial pressure, and subsequent MRI one day later showed global hypoxic ischemic injury. EEG with global slowing pattern signifying anoxic brain injury. Ammonia levels normalized on CRRT (although patient required dialysis for anuria after completion of 6 days of CRRT) and SSEP was done which did show evoked potentials, however no improvement was noted on neurological exam. Family eventually made the decision to transition care to comfort measures and the patient expired soon after.

DISCUSSION:

Overt cerebral edema leading to coma in HE and ACLF is rare given the chronicity of high ammonia and brain adaptation. The pathophysiologic mechanism for HE in ACLF is thought to be a combination of elevated serum ammonia and systemic inflammatory changes. Our attempts with hemodialysis did not change the clinical course suggesting irreversible tissue damage. Advanced therapies including embolization of portosystemic shunts was not pursued as none were identified on contrast enhanced imaging. Preventing decompensation and early recognition may be the modifiable factor in this high mortality disease.
2277 Multiple Liver Abscesses Caused by Streptococcus Intermedius Bacteremia in the Setting of a Routine Dental Cleaning
Authors: Hanna Angy , Odish Fadi , Imam Zaid , Dalal Bhavinkumar
Published: October 2019

INTRODUCTION:

Streptococcus intermedius is a member of the Streptococcus anginosus group, also referred to as the “Streptococcus milleri” group. The S anginosus group is part of the oral cavity and GI tract normal flora. S intermedius has been known to cause abscesses in various locations in the body, most commonly in the liver and brain, however this is uncommon in healthy individuals. We report the clinical case of an immunocompetent 21-year-old male found to have multiple hepatic abscesses associated with streptococcus intermedius in the setting of a recent dental cleaning.

CASE DESCRIPTION/METHODS:

Patient is a 21-year-old immunocompetent male who presented to the hospital with complaint of right upper quadrant pain, fevers, and chills. He underwent a routine dental cleaning 3 months prior. On admission, his vitals were BP 88/53 mmHg, HR 142 beats/minute, RR 41 breaths/min and temperature 38.7C. His exam was remarkable for right upper quadrant tenderness and splenomegaly. The remainder of his diagnostic evaluation revealed (a) leukocytosis of 16,000 cells/mm3 with left shift, (b) elevated creatinine of 2.66 mg/dl, (c) elevated alkaline phosphatase of 573 U/L, aspartate aminotransferase of 102 U/L and alanine aminotransferase of 65 U/L with a bilirubin of 1.8 mg/dl. Computed-tomography (CT) of the abdomen and pelvis demonstrated multiple right lobe hepatic abscesses with the largest measuring 7.3 cm. Two sets of blood and fluid cultures from an ultrasound guided aspiration returned positive for S. Intermedius. The patient underwent 2 subsequent drainage procedures for the larger lesion. The patient demonstrated remarkable clinical recovery and was discharged with a 6-week course of PO ertapenem therapy. Follow-up laboratory values demonstrated resolution of his hematological abnormalities, acute kidney injury and transaminitis.

DISCUSSION:

Although S. intermedius has been been associated with pyogenic liver abscesses, it typically occurs in the setting of immunocompromised states. This case was particularly interesting in that it occurred in an immunocompetent young male in the setting of routine dental cleaning 3 months prior. While other case studies have reported liver abscesses from S. intermedius after dental cleaning, typical latency is 1 month from the dental cleaning while much longer latency is noted in the reported case. This raises the question of how long after a dental cleaning a patient may have an increased risk of developing abscesses, even if the patient is thought to be immunocompetent.
2278 Rapidly Progressive Hepatitis C After Liver Transplantation Treated With Glecaprevir/Pibrentasvir
Authors: Abdulla Hamza , Merwat Shehzad N. , Stevenson Heather , Kulkarni Rupak
Published: October 2019

INTRODUCTION:

Hepatitis C recurrence following liver transplantation occurs in all patients with chronic HCV infection who have detectable serum HCV RNA levels prior to transplant. Less than 10% of these patients develop an acute and severe phenotype called fibrosing cholestatic hepatitis, which often involves fibrosis, rapid graft loss and decreased survival. The introduction of direct-acting antiviral agents (DAAs) have changed the paradigm of treatment of these patients.

CASE DESCRIPTION/METHODS:

A 62-year-old woman with a medical history of hepatitis C infection, hepatocellular carcinoma status post orthotropic liver transplantation, diabetes mellitus and hypertension who was hospitalized for fatigue and elevated liver chemistries following her liver transplantation. Laboratory tests showed a white cell count 6.67 × 103/μL, creatinine 1.1 mg/dL, aspartate aminotransferase 592 U/L, alanine aminotransferase 504U/L, alkaline phosphatase 312 U/L, bilirubin 3.5 mg/dL, albumin 2.9 g/dL. Acute hepatitis work up showed a positive hepatitis C virus antibodies with genotype 1B, with a viral load of 80 million IU/mL HCV RNA. HBsAg, HBc IgG, HBc IgM, and hepatitis A virus IgM were negative. Evaluation for Epstein-Barr virus, cytomegalovirus and herpes simplex virus were negative. Ultrasound of the abdomen revealed that the liver is normal in size, echotexture and contours. No focal hepatic lesions. Normal hepatic blood flow. The patient underwent liver biopsy that showed lymphocytic portal inflammation with frequent lobular apoptosis consistent with active chronic hepatitis C (MHAI: 5-6/18). Mild portal fibrosis (ISHAK FIBROSIS STAGE: 1/6) and minimal evidence of acute t cell-mediated rejection (rejection activity index: 1/9). During the patient hospitalization her liver chemistries significantly worsened and peaked to aspartate aminotransferase 740 U/L, alanine aminotransferase 511U/L, alkaline phosphatase 312 U/L, bilirubin 3.5 mg/dL. We decided to start the patient on Glecaprevir/Pibrentasvir for acute and severe hepatitis. Fourteen days after starting Glecaprevir/Pibrentasvir, the patient viral load decreased to 782 IU/mL HCV RNA and normalized liver chemistries with AST19 and ALT 27. The patient denied any adverse events due to the treatment.

DISCUSSION:

To our knowledge, this is the first case report of treating early and aggressive form of HCV recurrence with Glecaprevir/Pibrentasvir. The dramatic improvement of the patient suggest that DAA might be safe and effective in this situation.
2279 DRESS Causing a Subacute Fulminant Hepatitis Secondary to Sulfasalazine
Authors: Zaibaq-Krill Jenine , Sydor Michael , Merwat Sheharyar , Merwat Shehzad N. , Luthra Gurinder
Published: October 2019

INTRODUCTION:

Drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare and life-threatening hypersensitivity reaction most often due to anti-epileptics. It is characterized by a maculopapular rash, eosinophilia, lymphadenopathy, and visceral organ involvement. Here we present a case of DRESS caused by sulfasalazine which resulted in subacute fulminant hepatitis.

CASE DESCRIPTION/METHODS:

A 24-year-old male with ulcerative colitis was started on sulfasalazine. After being on sulfasalazine for one month, he ran out of the medication. Ten days later, he developed a diffuse pruritic morbilliform rash. He initially presented with the following lab values: total bilirubin 0.7 mg/dL, AST 175 U/L, ALT 354 U/L, alkaline phosphatase 208 U/L, and INR of 1.1. Skin biopsy revealed focal vacuolar interface dermatitis and superficial perivascular lymphocytic infiltrate with neutrophils and eosinophils, consistent with drug eruption. He was started on prednisone 50mg daily and was discharged home. Ten days later, he returned to the hospital with jaundice and the following lab values suggestive of subacute fulminant hepatitis: total bilirubin 6.5 mg/dL (conjugated bilirubin 3.4 mg/dL), AST 3475 U/L, ALT 4171 U/L, alkaline phosphatase 308 U/L, and INR 2.6. Due to the degree of his hepatocellular injury, he was started on prednisone 2mg/kg. The rash, liver chemistries and INR improved over the next week.

DISCUSSION:

Sulfasalazine is a rare, but known cause of DRESS and idiosyncratic liver injury. This case is notable because he developed a subacute fulminant hepatitis 10 days after initial presentation and 20 days after discontinuation of sulfasalazine. This highlights the fact that liver injury can evolve into acute liver failure despite withdrawal of the culprit drug several days prior; this latency period is characteristic of sulfasalazine induced acute liver injury and thus supports our diagnosis. AST and bilirubin levels are the most important prognostic indicators for liver transplantation and/or death. DRESS due to sulfasalazine with significantly elevated transaminase levels ( >2000 U/L) has been reported, however, to our knowledge, this is the third reported case with this degree of hepatocellular injury that did not result in liver transplantation. Given the fact that acute liver failure is often the cause of mortality in patients with DRESS, this case illustrates that serial lab monitoring is essential in such patients as liver injury can persist long after the offending agent has been discontinued.