bluuebunny/crossref_metadata_2025 huggingface dataset:
2281 Symptomatic Isolated Polycystic Liver Disease Causing Respiratory Compromise
Authors: Rivas Cynthia , Castano Juan J. , Pozo Leonardo , GrigoriyRapoport MD , Zamir Asif
Published: October 2019

INTRODUCTION:

Isolated Polycystic Liver Disease (PCLD) is defined by the presence of more than 20 hepatic cysts. It has an autosomal dominant inheritance and is largely asymptomatic with only 5% of patients who report pain or other complaints. The disorder is considered rare with an estimated incidence of < 0.01%. We present a case of isolated PCLD causing respiratory failure.

CASE DESCRIPTION/METHODS:

A 45-year-old woman with history of an isolated liver cyst, was admitted for acute respiratory failure. No significant family history. On physical exam, oxygen saturation was 98% on 2 liters nasal cannula. She had prolonged expiratory phase, decreased breath sounds and marked hepatomegaly. FEV1 = 23%, FEV1/FVC = 117%. Liver studies were significant for alkaline phosphatase 131 IU/L and albumin 3.3 gm/dl. Abdominal CT showed a markedly enlarged liver measuring 21 cm, liver volume of 3588 ml and the presence of numerous thin-walled-fluid-filled cysts ranging from 2 mm to 4 cm, consistent with PCLD. It was determined that the mass effect from the liver pressing against the diaphragm was the cause of her respiratory failure. She was started on BiPAP with improvement of respiratory function. She underwent evaluation for orthotopic liver transplantation(OLT) but was not a candidate due to high risk for hypercarbic respiratory failure, and prolonged mechanical ventilation. She was discharged on non-invasive ventilation with oxygen supplementation and was scheduled for re-evaluation for OLT.

DISCUSSION:

Isolated PCLD is caused by germline mutations of the PRKCSH and SEC63 genes. Genetic testing is rarely necessary and does not affect management. Liver function is often altered once symptoms develop. The number and size of liver cysts often increase with age. Women often present with a massive and symptomatic cystic liver more frequently than men and symptoms are likely attributed to the compression of adjacent organs due to mass effect, which often leads to poor body image and depression. When symptoms develop they include pain, nausea, early satiety, vomiting, or dyspnea. Complications are rare and can include infection, portal hypertension, and Budd-Chiari Syndrome. Treatment includes aspiration-sclerotherapy, laparoscopic or open surgical fenestration techniques, and hepatectomy. Somatostatin analogues show promise. Liver transplantation may be required in extensive disease. One should be cognizant that symptomatic isolated PCLD often leads to greatly diminished quality of life.
2292 A Case Report of Glycogen Hepatopathy: A Reversible, yet Relapsing Cause of Hepatitis in Type 1 Diabetics
Authors: Azhar Mishah , Hammami Muhammad B. , Musmar Ahmad , Bromer Matthew
Published: October 2019

INTRODUCTION:

Glycogen hepatopathy (GH), a rare glycogen storage disease caused by genetic or acquired overactivation of hepatic glycogen synthesis enzymes, can mimic non-alcoholic fatty liver disease (NAFLD). We describe a case of biopsy proven GH in an adult with Type 1 DM (T1DM). Similar to cases described in the current literature, our case not only portrays the challenges faced by physicians when diagnosing this condition, but also the importance of distinguishing this from NAFLD.

CASE DESCRIPTION/METHODS:

A 33-year-old Honduran woman with a 25-year history of T1DM complicated by gastroparesis, multiple episodes of DKA and hypoglycemia, and recurrent pancreatitis was referred for abnormal liver enzymes. Family history was negative for liver disease. There was no history of alcohol or recreational drug use. Patients medications included insulin and thyroxine. Physical exam showed hepatomegaly but no stigmata of chronic liver disease. AST and ALT had ranged from 100’s to over 7000 while ALP was elevated to over 400. Albumin, Total bilirubin, platelets, INR, eosinophils, viral hepatitis panel, ANA, smooth muscle AB, LKM Ab, celiac serologies, ceruloplasmin, alpha 1 antitrypsin, iron studies, and acetaminophen levels were all normal. An abdominal ultrasound showed “fatty liver” and an atrophic pancreas. CT abdomen showed hepatomegaly. CBD was found to be normal on EUS and MRCP. A liver biopsy demonstrated glycogenotic hepatocytes.

DISCUSSION:

GH is frequently misdiagnosed as NAFLD, a more common liver disease that is also associated with diabetes. While GH is known to be reversible with otherwise no known long-term complications, NAFLD has been shown to progress to cirrhosis and cause hepatocellular carcinoma. Definite diagnosis often requires liver biopsy because of overlapping clinical and radiographical pictures. Elevation of both glucose and insulin levels in the setting of fragile DM control is thought to play a role via overstimulation of glycogen synthesis. Recommended treatment is stable “tight” glycemic control; pancreatic transplantation has resulted in sustained GH remission in two case reports. The required degree of stability and tightness of glucose control is not yet known. An increased awareness of GH is needed in an attempt to prevent delay in diagnosis, in a condition with an otherwise unknown incidence.
2293 An Unusual Complication of Acute Viral Hepatitis A: Hemolytic Anemia
Authors: Korada Sai Krishna , Sobotka Lindsay , Wellner Michael , Michaels Anthony
Published: October 2019

INTRODUCTION:

Hepatitis A is one of the most prevalent causes of acute hepatitis worldwide. Given the availability and effectiveness of vaccination, rates of viral hepatitis A (HAV) decreased in the United States until recently with multiple areas across the United States experiencing outbreaks. Disease presentation can be variable with the majority of patients presenting with abdominal pain, nausea, vomiting, and jaundice. Few patients present with fulminate liver failure and have required liver transplantation. Other rare extrahepatic presentations of hepatitis A include acute kidney injury, neurologic complications, and anemia.

CASE DESCRIPTION/METHODS:

A 38-year-old female with a medical history of type 2 diabetes and hypertension presented with worsening anemia and fatigue. The patient was admitted to an outside hospital two months prior with abnormal liver function tests and was ultimately diagnosed with acute HAV. Physical exam revealed diffuse jaundice, scleral icterus, and abdominal tenderness. Lab results on admission are demonstrated in Table 1. She had no active signs of GI bleeding. Prednisolone 40mg daily was initiated given concern for hemolytic anemia. Analysis to evaluate for hemolysis is demonstrated in Table 2. Steroids were stopped due to side effects. The patient was treated supportively and hemoglobin stabilized.

DISCUSSION:

Rare extrahepatic presentations of acute hepatitis A occur in less than 10% of affected patients. Prompt recognition of hemolytic anemia is crucial. Patients recently diagnosed with HAV that develop acute anemia without overt signs of bleeding should be evaluated for hemolysis. Analysis with haptoglobin, lactate dehydrogenase, direct Coombs test, reticulocyte count and peripheral blood smear should be performed to confirm the diagnosis. No studies have been completed to determine factors that could predispose specific patients to develop hemolytic anemia but multiple studies have suggested increased frequency in patients with underlying glucose-6-phosphate dehydrogenase deficiency. Multiple treatment options have been proposed for patients that have developed hemolytic anemia as a complication of HAV, with most patients receiving steroids. Other studies highlight the use of plasmapheresis though the effectiveness of this intervention remains uncertain. Prior literature suggests that supportive care or a shorter course of steroids may be sufficient. However, no studies prove which intervention is the most effective. Anemia typically recovers as acute HAV resolves.
2300 Chronic Lymphocytic Leukemia of the Liver: A Rare Cause of Elevated Liver Function Tests
Authors: Buckshaw Ricky , Malik Anam , Kloska Michal , Vaze Reema , Wong She-Yan
Published: October 2019

INTRODUCTION:

Chronic lymphocytic leukemia is a lymphocytic proliferative malignancy that can infiltrate multiple different organs. It is one of the most common forms of leukemia in the adult population. CLL affecting the liver is historically rare, however since recent it has been becoming more prevalent due to certain advancements in chemotherapy. With metastatic CLL becoming more prevalent, we should focus expansion of research to this subset of patients.

CASE DESCRIPTION/METHODS:

A 76-year-old male with a past medical history of CLL 13q14 mutation+ and a recent diagnosis of renal insufficiency was evaluated by his Gastroenterologist for elevated liver enzymes. He was found to have an AST of 112 and an ALT of 348. His ferritin was also elevated to 953 with a transferrin saturation of 27%. He was referred to a Hepatologist for a more in-depth workup, which revealed worsening lymphadenopathy on CT scan along with worsening hepatomegaly and splenomegaly on abdominal ultrasound. Blood work was ordered for evaluation of infectious, inflammatory and autoimmune causes of his elevated liver enzymes. The patient’s blood work showed a negative ANA/SMA/AMA, a negative HAV/HBV/HCV. The patient underwent percutaneous liver biopsy for further evaluation. The biopsy showed involvement of his liver with dense lymphoid infiltrate of the portal tracts, consistent with chronic lymphocytic leukemia. The pathology also resulted showing the liver parenchyma showed the same biomarkers as the patients serum studies. The positivity of CD5, CD23 and PAX5 was consistent with the previous serum studies. The patient was initiated on Imbruvica, which provided improvement in his liver function tests. He continues to follow with Oncology for continued treatment on Imbruvica.

DISCUSSION:

The increased prevalence of liver dysfunction brings up new conversations in CLL treatment. Liver infiltration has not been studied as much as other organ systems and this limits our understanding of this chronic, debilitating disease process. There has been a documented shorter survival for patients diagnosed with liver infiltration of CLL, which limits our patient’s quality of life if we do not better grasp this potential complication of CLL.
2303 A Rare Case of Atraumatic Intercostal and Intrathoracic Liver Herniation Related to Influenza A
Authors: Harne Prateek S. , Mukherjee Samiran , Shepherd Zachary
Published: October 2019

INTRODUCTION:

A non-traumatic intercostal hernia is sparsely reported and is defined as a protrusion of intra-abdominal contents through acquired or congenital defect of the abdominal and thoracic walls. There have been individual cases reporting such herniation of lungs, small and large bowel, omentum, and liver. We describe a case in which a patient had both intercostal and diaphragmatic liver herniation secondary to increased cough. The literature relies on CT for diagnosis and for assessing damage to liver parenchyma which guides management. While intercostal liver herniations can be surgical emergencies, our case was managed conservatively given minimal liver injury and rapidly improving symptoms.

CASE DESCRIPTION/METHODS:

A 58-year-old female with a past medical history of hypothyroidism and essential hypertension presented with severe dry cough and nasal congestion for two weeks. Three days before presentation, she noticed a bruise on the right lateral chest wall which gradually increased and became painful with radiation to her back. The patient denied any recent history of trauma to the area. Physical exam revealed dry cough, large palpable tender bulging ecchymosis over right 9th and 10th ribs, and clear lungs. Vitals were unremarkable. Labs were significant for ALT 41, AST 62, CK 493, white count 17.8, platelets 567, INR 0.9 and positive nasal swab for influenza A. CT abdomen showed herniation of uninjured liver between 9th and 10th ribs with abdominal wall soft tissue stranding (or edema) with part of liver herniating through a diaphragmatic defect into the thoracic cavity (“hump sign”). Surgical services were consulted, and the patient was managed conservatively with opiate analgesia, IV hydration with normal saline, cough suppression, Tamiflu, and discharged with medical and surgical follow up.

DISCUSSION:

In patients with poor abdominal muscle strength having sudden worsening of pain after coughing and physical exam findings consistent with herniation, there should be high suspicion for abdominal and transdiaphragmatic herniation of liver. Given the rarity of such events, these patients can present as a diagnostic challenge and delay in care can result in poor outcomes especially in patients with organ damage. A consensus for management can be difficult due to sparsely reported cases, however, while injured or strangulated liver herniation is a surgical emergency, a conservative approach can be considered for uninjured liver herniation with close medical and surgical follow up.
2304 Glycogen Hepatopathy: An Unusual Cause of Liver Enzymes Elevation in Diabetic Patients
Authors: Sourabh Fnu , Jain Anubhav , Aggarwal Ankita , Kumar Sarwan , Ralh Neeharika
Published: October 2019

INTRODUCTION:

Liver enzyme elevation is a common condition among diabetic patients and often Nonalcoholic Fatty Liver Disease is responsible for this,which can lead to fibrosis and cirrhosis. GH should be one of the differential diagnoses upon exam of a patient with uncontrolled DM with elevated transaminases and hepatomegaly. Compared to other liver diseases associated with DM, GH is a favorable diagnosis due to its benign nature and good prognosis.

CASE DESCRIPTION/METHODS:

An 18 years old female with type I diabetes had been non compliant with her basal insulin frequently. This had led to 7-8 hospitalizations within last year. This time, she was in the hospital for a ketoacidosis episode with HbA1C of 17%. During P/E, her liver was enlarged. Liver enzymes were, ALP-201 U/L, AST-120 U/L, ALT-125 U/L, GGT 98 U/L).Lipid profile- Cholesterol 315 mg/dl, HDL 50.4 mg/dl, LDL 205 mg/dl, Triglyceride 298 mg/dl. Her USG showed 19 cm liver with normal parenchyma. CT scan reiterated the findings with hyperdense liver. It was noted that the liver enzymes had great fluctuations within the last year, when she was hospitalized due to hyperglycemia ,and her liver enzymes declined when blood sugar was regulated. The patient was examined with regard to chronic hepatitis differential diagnosis since the aminotransferase levels were found to be elevated , intermittently from last year. Serology for viral hepatitis and autoimmune hepatitis had normal results. So, a liver biopsy was performed. Significant Glycogen rich hepatocytes were observed in the tissue, without fibrosis or inflammation. The case was diagnosed with GH due to the present clinical, laboratory, and pathological findings. Patient was advised diet and intensive insulin regimen. Though she remained non compliant. 6 years follow up revealed persistent elevated enzymes with CT scan showing liver size of 21 cm with liver enzymes elevation.

DISCUSSION:

Glycogenic hepatopathy is a rare complication of the poorly controlled diabetes mellitus characterized by the liver dysfunction with elevated liver enzymes and hepatomegaly caused by the reversible accumulation of excess glycogen in the hepatocytes. One of the essential elements in the pathophysiology of development of GH is the wide fluctuation in both glucose and insulin levels. Future progress is required in understanding the biochemical defects underlying GH and an ideal diagnostic test instead of biopsy to avoid the extensive workup in evaluating suspected cases of GH.
2306 Hydroxychloroquine-Induced Acute Liver Injury
Authors: Cheema Bahar , Triplett Drew , Krishnamurthy Padmini
Published: October 2019

INTRODUCTION:

Hydroxychloroquine is a commonly prescribed medication with antimalarial and anti-inflammatory properties. A very rarely reported side effect of this medication is drug induced acute liver injury.

CASE DESCRIPTION/METHODS:

A 62-year old female with a history of rheumatoid arthritis, on long term prednisone, presented to the emergency department with two day history of arthralgias, generalized weakness, and fever of 104 F. She had no history of alcohol use or any risk factors for viral hepatitis. Clinical exam was unremarkable. Laboratory data showed Aspartate aminotransferase (AST) or 798 IU/L and Alanine aminotransferase (ALT) of 1254 IU/L, with normal serum bilirubin, alkaline phosphatase and coagulation profile. Serologic tests for viral etiology including Hepatitis A, Hepatitis B, Hepatitis C, Cytomegalovirus, Epstein-Barr virus, and Human immunodeficiency virus were non-reactive. Anti-nuclear antibody, smooth muscle antibody, and anti-double-stranded DNA antibody were unremarkable. Her urine and blood cultures showed no growth. Abdominal ultrasound showed an echogenic liver consistent with hepatic steatosis and no evidence of biliary obstruction. Chest X-ray was normal. Upon review of her medications, patient had started hydroxychloroquine two weeks prior to admission. The medication was discontinued upon admission, after which she continued to improve. At follow up ten days later, she remained asymptomatic and her liver function tests continued to improve with AST of 46 and ALT of 161.

DISCUSSION:

Acute transaminitis due to hydroxychloroquine is rare. Only four reported cases have been noted per literature review. Mechanism of injury to hepatic cells is unknown however the drug is metabolized in the liver and may alter the metabolism of other drugs. Porphyria cutanea tarda is a disease that may be treated with low doses of hydroxychloroquine. It is known that higher doses of hydroxychloroquine usually greater than 100 mg twice a week can trigger acute liver injury in patients with history of Porphyria cutana tarda. These patients present with sudden onset of fever and marked serum enzyme elevations with increased excretion of porphyrins and photosensitivity. The presented patient in the case report did not have underlying Porphyria cutanea tarda.
2310 A Mysterious Case of Non-Cirrhotic Recurrent Severe Hyperammonemia: What Urea-lly Need to Know
Authors: Pendela Venkata Satish , Munoz Anisleidys , Razzouk Gaby
Published: October 2019

INTRODUCTION:

Ammonia is a well-known toxin, mostly studied in Hepatic encephalopathy. While, it is related to severe liver disease in 90% cases, the remaining 10% is constituted by disturbances in its excretion or related to over production from other etiologies. [1] Awareness of hyperammonemia in absence of severe hepatic disease may lead to lifesaving prompt diagnosis and treatment. We present a mysterious case of recurrent severe hyperammonemia without known liver disease.

CASE DESCRIPTION/METHODS:

A 53 year old male with prior history of well controlled hypertension presented to the emergency department with unresponsiveness. On exam, he was unconscious with occasional spontaneous eye opening and a left gaze preference. CT head and CT angiogram of neck did not reveal any acute abnormalities. Liver enzymes and Bilirubin were normal. Blood alcohol level was 0.089gm/dl%. EEG showed diffuse encephalopathy. Serum ammonia was 705 umol/L. He was started on lactulose. His mentation improved in a day and repeat serum ammonia was 25umol/L. Ultrasound liver and CT abdomen ruled out cirrhosis and gall bladder disease. He was discharged home with a prescription of lactulose. He returned to the ED with another episode of unresponsiveness a month later and was found to have elevated serum ammonia of 213 umol/L. Levetiracetam was started empirically with suspicion of recurrent seizures causing this unresponsiveness. He continued to have similar episodes of hyperammonemic encephalopathy. Orotic acid in urine was high (>200 mmol/mol creatinine ), clinching the diagnosis of ornithine transcarbamylase (OTC) deficiency. He was advised to consume a low protein diet and was prescribed L-carnitine along with lactulose.

DISCUSSION:

Ammonia directly affects neuronal electric activity by inhibiting the generation of both excitatory and inhibitory postsynaptic potentials. Secondary hyperammonemia occurs commonly in the presence of hepatic disorders leading to portosystemic encephalopathy. Less common causes include drugs like valproate, post-chemotherapy, gastric bypass surgery. Causes of primary hyperammonemia in adults include milder forms of urea cycle enzyme deficiencies like OTC, argininosuccinate lyase. Noncirrhotic hyperammonemia as a cause of altered mental status remains a diagnosis of exclusion and requires high index suspicion. Treatment options include lactulose, l-carnitine, and drugs to lower cerebral edema if patient has MRI findings. Liver transplantation is an important treatment modality in urea-cycle disorders.
2312 Using Serum Transaminase Ratios to Recognize Herpes Simplex Hepatitis
Authors: Kern Cody , Schaberg Kurt , Deep Kristy
Published: October 2019

INTRODUCTION:

Herpes simplex virus (HSV) hepatitis is an uncommon cause of acute liver failure (ALF). HSV hepatitis accounts for less than 2% of all cases of viral ALF. The clinical presentation is non-specific including encephalopathy, leukopenia, and fever. Typical HSV skin manifestations are present in less than 50% of cases. Diagnosis is challenging due to the lack of timely and reliable serologic testing. Liver biopsy can help with diagnosis however is not always available. Reported mortality is 88% for untreated HSV hepatitis. Early treatment with acyclovir reduces mortality to 51%. Current guidelines recommend starting acyclovir if HSV hepatitis is suspected (1).

CASE DESCRIPTION/METHODS:

A 67 year-old female presented with a four day history of nausea, vomiting, diarrhea, and progressive weakness. On admission, she was hemodynamically stable with a fever of 102.8F. Physical exam revealed RUQ tenderness. No labial or genital lesions were present. Laboratory testing revealed WBC 2500 IU/L, platelets 108,000/mm3, AST 3038 IU/L, ALT 696 IU/L, ALP 181 IU/L, TB 0.2 mg/dL, and INR 1.5. Viral hepatitis serologies were negative. CT abdomen revealed a 4.9 × 3.7 × 3.5 cm enhancing lesion in the liver dome. Liver biopsy of lesion showed multifocal non-zonal massive necrosis consistent with HSV hepatitis. The patient was started on IV acyclovir at 7mg/kg/8hr on hospital day twelve for a total of fourteen days. The patient improved and liver enzymes normalized prior to discharge.

DISCUSSION:

A prior review of sixteen case reports described HSV hepatitis presenting with a hepatocellular liver injury pattern of AST significantly more elevated than ALT (2). This case presented similarly, with the AST:ALT ratio being nearly 5:1. Despite this pattern previously being recognized, it is not regularly described in review articles or practice guidelines. Early treatment with acyclovir lowers mortality of HSV hepatitis, yet there are no clear recommendations on when we should suspect HSV hepatitis. Clinicians should consider early empiric acyclovir for ALF when traditional viral hepatitis serologies are negative and give special consideration for hepatocellular liver injuries with significantly elevated AST:ALT ratios as this may represent a pattern for HSV hepatitis.
2313 Graft-versus-Host-Disease Following Liver Transplant
Authors: Stratton Leland , Wentworth Brian , Intagliata Nicolas M.
Published: October 2019

INTRODUCTION:

Graft versus host disease (GVHD) is a rare, life-threatening complication of liver transplantation that may affect the skin, GI tract and bone marrow. Donor T-lymphocytes remaining in the transplanted graft primarily target the recipient causing organ injury. Presentation may be non-specific and mimic other post-transplant complications, leading to delay in diagnosis and a mortality rate of greater than 80%. There is no consensus regarding optimal management of GVHD in the setting of liver transplantation.

CASE DESCRIPTION/METHODS:

A 65-year-old male with a history of NASH cirrhosis underwent deceased-donor liver transplant. Induction therapy was given with basiliximab and solumedrol then immunosuppression was maintained with tacrolimus, mycophenolate mofetil (MMF) and prednisone. Three weeks post-transplant, diarrhea developed and MMF was dose-reduced. Given persistence of diarrhea, fever, neutropenia and bilateral lower extremity petechial rash, additional work-up was performed. Imaging revealed colonic inflammation and subsequent flexible sigmoidoscopy demonstrated diffuse inflammation in the sigmoid. Biopsies of the colonic mucosa and lower extremity skin rash were consistent with GVHD. Peripheral blood confirmed the presence of donor chimerism. The patient was started on empiric antibiotics for febrile neutropenia, received granulocyte stimulating factor for leukopenia, intravenous steroids, and oral budesonide. MMF was initially discontinued but later resumed at increased dose; tacrolimus was continued. The patient's condition continued to deteriorate with diffuse mucosal GI bleeding. He received antithymocyte globulin and etanercept but his illness progressed secondary to Enterococcus bacteremia leading to septic shock. The patient died 8-weeks post-transplant in the setting of multiorgan failure.

DISCUSSION:

While rare, GVHD after liver transplantation should be considered in patients with new onset, unexplained symptoms. By virtue of its rarity after liver transplantation, data are sparse and management decisions are challenging. This case illustrates the need for increased awareness and standardized treatment guidelines. Management decisions are currently based on small case series often with discordant strategies including increasing or decreasing immunosuppression exposure. Global registries and multicenter collaboration will be essential to increase knowledge and improve outcomes.
2314 Pyogenic Liver Abscess in the Setting of Recent Polypectomy
Authors: Thomas Mathew J. , Kilpatrick Dustin , Mohammed Suneel , Krogel Nathaniel
Published: October 2019

INTRODUCTION:

Pyogenic liver abscess (PLA) is a bacterial infection of the liver parenchyma with reported mortality of 6-14%. Early detection is vital to reduce overall morbidity and mortality. Due variability in clinical presentation, a high index of suspicion is necessary to avoid delay in diagnosis and treatment. Intra-abdominal infections such as diverticulitis or pathologic conditions of the biliary tract are the most common etiologies of PLA but micro-perforations in the setting of recent polypectomy leading to bacterial seeding through the portal circulation may account as an under-reported precipitant.

CASE DESCRIPTION/METHODS:

A 63-year-old male with history of chronic alcohol abuse who was admitted to the ICU on broad spectrum antibiotics after developing acute respiratory failure requiring mechanical ventilation and septic shock. The patient had several days of vague abdominal pain which led to acute worsening with encephalopathy. Initial evaluation in the ED revealed temperature of 106.2 and laboratory results revealed WBC 27.8, lactic acid of 3.6, procalcitonin level 52 ng/mL, alkaline phosphatase 131, AST 90, and ALT 65. CT of the abdomen and pelvis revealed multiloculated cystic mass in the dome of right liver measuring 8.6 cm × 8.1 cm x 7.2 cm. It was noted that patient had underwent a screening colonoscopy approximately three weeks prior to admission and multiple polyps were removed with a hot snare in the ascending, descending and sigmoid colon. Admission blood cultures revealed polymicrobial bacteremia and grew streptococcus and E. coli. Due to concern for underlying liver abscess from recent bacterial seeding from polypectomy, patient underwent IR guided drainage and placement of drain which relieved purulent fluid that cultures eventually became positive for Streptococcus intermedius. Despite appropriate antibiotic coverage, patient had persistent fevers and required transfer to hepatobiliary service for definitive surgical management. Patient underwent hepatic lobectomy and laparoscopic marsupialization of the liver abscess with dramatic improvement of fever curve and leukocytosis.

DISCUSSION:

PLA in the setting of recent colonoscopy is a known phenomenon, however the incidence is unclear. As colorectal cancer screening rates continue to increase in practice, awareness of the development of PLA in the setting of immunocompromised patients must be highlighted. Patient counseling on this potentially fatal complication should occur especially to immunocompromised patients.
2315 Early Detection and IR Intervention in a Case of Fulminant Budd-Chiari Syndrome
Authors: Rezaie Aida , Ahmed Shifat , Al-Jashaami Layth , Patel Nayan
Published: October 2019

INTRODUCTION:

Budd-Chiari syndrome (BCS) is a relatively rare cause of abnormal LFTs, and infrequently progresses to fulminant hepatic failure. BCS requires a high degree of suspicion and can be easily confused with other causes of rapidly worsening liver enzymes. We present a case that illustrates successful management of fulminant hepatic failure by interventional radiology (IR) guided thrombolysis and thrombectomy, followed by TIPS placement in a rapidly decompensating 34-year-old woman who initially presented with mildly abnormal LFTs.

CASE DESCRIPTION/METHODS:

A 34-year-old white woman with past medical history of acid reflux, and family history of venous thromboembolism, presented to an outside hospital with a 5-day history of nausea, vomiting, diarrhea. She was subsequently was transferred to our facility for a higher level of care after worsening liver function testing and mentation. Patient had reported 5 days of epigastric tenderness, nausea, and abdominal distension prior to admission. Her LFTs continued to worsen, and clinically, she became progressively more hypotensive and tachycardic (BP 74/44, HR 112) requiring vasopressor support without significant improvement in her vital signs. Within 48 hours, she had acute onset of ascites and worsening encephalopathy. On arrival to our facility, she underwent CT abdomen/pelvis with and without IV contrast showing complete thrombosis of the right, left, and middle hepatic veins. Her liver function and transaminases continued to worsen prompting IR intervention. She underwent IR-guided thrombolysis and thrombectomy and TIPS with pre- and post- intervention pressures showing marked improvement in the transhepatic pressures (Table 1). (Pre- and post-intervention imaging in Figures 1 and 2). As part of her hypercoagulable workup, she was found to be deficient in protein C activity (46%). She had no OCP use for over 10 years.

DISCUSSION:

It was later discovered that patient’s mother had a history of saddle pulmonary embolism and other maternal relatives have had a pro-coagulative history, most of whom presented with significant lower extremity DVTs. This case illustrates that a high clinical suspicion is needed to make an early diagnosis of BCS, which can lead to early intervention and minimize the need for emergent liver transplantation. This case emphasizes the significant of a thorough history, and particularly a family history of venous thromboembolism.
2316 Gangrenous Cholecystitis and Revelation of Gallbladder Metastasis From Primary Breast Cancer
Authors: Minupuri Arun , Gudipally Sindusha , Nair Jayamohan , Walli Omar , Modi Simple , Salas Noain Jesus M.
Published: October 2019

INTRODUCTION:

Breast cancer metastasis seeds various organs in the body by gaining access to blood and lymphatic vessels. The preferential sites involve the lung, liver, bone, and brain, and symptoms usually compromise of chest pain, cough, shortness of breath, abdominal pain, and joint pain. Metastasis to the gallbladder is of a rare phenomenon, and here in our case report we present an unusual discovery of gangrenous cholecystitis through diagnostic laparoscopy; with eventual histopathological findings revealing metastatic primary breast cancer to the gallbladder.

CASE DESCRIPTION/METHODS:

A 59-year-old African American female presented to our institution with 1-day history of abdominal pain. The abdominal pain is of sudden onset, mainly located in the right upper quadrant, sharp/stabbing in nature, intermittent, radiating towards the right flank and was associated with non-bilious/non-bloody vomiting. Medical history included hypertension, hyperlipidemia, myocardial infarction X2, and notably a recent diagnosis of metastatic breast cancer. She was diagnosed 2 months prior to admission with T4N3M1 breast cancer. Labs were significant for AST 89, ALT 68, Alk Phos 178, and albumin 3.4. CT of the chest and abdomen was done which showed re-demonstration of metastatic breast cancer along with marked gallbladder wall thickening without obvious gallbladder calculi. A HIDA scan was performed which excluded cholecystitis. Patient was admitted and was started on intravenous fluids, antibiotics, and pain medications. General surgery was consulted, and decision was made for diagnostic laparoscopy. Laparoscopy revealed perforated acute gangrenous cholecystitis, leading to the removal of gallbladder along with appropriate cleansing of the abdomen. Histopathological examination of gallbladder shows tumor cells positive for GATA3 and ER (greater than 90%) [Figures 1 and 2]. Post operatively patient had no complications and was successfully discharged with close outpatient follow up.

DISCUSSION:

Metastasis (mets) to the gallbladder is rare, and from breast primary it is considered an extremely rare occurrence. In our case report, we see a patient with history of breast cancer developing symptoms of sudden onset right upper quadrant pain with vomiting and liver derangements. Treatment plans differ for primary gallbladder cancer vs mets to gallbladder; as histochemical testing is the key to accurate diagnosis. Hence, for appropriate treatment plans, clinicians should be aware of unusual presentation of metastatic breast cancer.
2319 A Case of Cryptogenic Liver Abscess in a Young Uncontrolled Diabetic
Authors: Mahmood Sharmeen , Kaur Randeep , Sayf Alaa Abu
Published: October 2019

INTRODUCTION:

Pyogenic liver abscess (PLA) is a rare complication of Diabetes Mellitus, usually seen in older adults who have had diabetes for many years. There is little information regarding the effects of glycemic control on the characteristics of PLA in diabetic patients. Actinomycosis is an infrequent invasive bacterial disease with indolent and non-specific presentation, therefore diagnosis is quite difficult to make for clinicians. Here, we report an uncontrolled diabetic patient who developed cryptogenic Actinomyces odontolyticus bacteremia and concurrent polymicrobial liver abscess.

CASE DESCRIPTION/METHODS:

A 41-year-old male with a history of uncontrolled Diabetes Mellitus Type 2, presented with severe, dull, epigastric pain. He was found to be in septic shock and diabetic ketoacidosis, requiring admission to the medical intensive care unit. Initial laboratory tests showed a white blood cell count of 16,800/mm3 (87% neutrophils), bicarbonate 14mmol/L, AST 147, ALT 126, alkaline phosphatase 193, beta hydroxybutyrate 5.04, and anion gap 17. HIV testing was negative. Hemoglobin A1c was 13%. A computed tomography (CT) image of his abdomen revealed a 7cm gas containing lesion in the left hepatic lobe concerning for hepatic abscess. He was started on empirica vancomycin and piperacillin/tazobactam and subsequently underwent CT-guided pigtail catheter insertion for drainage of the abscess. Microbiological examination of the drainage revealed polymicrobial pus including rare actinomyces. His blood cultures revealed actinomyces odontolyticus. He improved hemodynamically and repeat blood cultures were negative. He was discharged on IV Penicillin G and Metronidazole with duration dependent on resolution of abscess. Once infection is resolved, he will undergo colonoscopy and dental examination to identify possible source for infection.

DISCUSSION:

The incidence and morbidity of patients with PLA and diabetes has recently increased. Hyperglycemia can promote bacterial growth in tissues, and metabolic disorders impact the gastrointestinal tract. Diabetes can also promote infection through reduced immunity, neutrophil chemotaxis, mononuclear phagocyte activation, and opsonization. Actinomyces Odontolyticus is rare, especially in the absence of other pathology, and many conditions have been described as a possible initiating event including diverticulitis, appendicitis or peptic ulcer disease. A multi-team approach is always recommended when disseminated actinomycotic infection is observed.
2321 Treatment of Hepatitis C in an Immunosuppressed Patient With Malignancy
Authors: Coburn Elliot , Drinane Mary
Published: October 2019

INTRODUCTION:

The prevalence of hepatitis C (HCV) is estimated to be 1-1.5% in patients with cancer. Direct-acting antivirals (DAA’s) have been shown to be a safe and effective option for treatment of HCV in patients with concurrent malignancy. However evidence for the use of DAAs in combination with b-cell depleting agents is lacking. We present a complicated case of a patient with lymphoma and HCV, who was treated simultaneously with rituximab and DAA therapy.

CASE DESCRIPTION/METHODS:

A 57-year-old male presented with recurrent angioedema due to acquired C1 esterase deficiency. Workup led to a bone marrow biopsy showing marginal zone lymphoma (MZL). He was pulsed with dexamethasone with plan to start rituximab. HCV screening was negative. Just prior to rituximab dose, he was found to have jaundice. Labs revealed an AST 2195, ALT 4828, total bili 5.1, alk phos 120, platelets 9k. Liver ultrasound showed hepatomegaly. He reported recent high risk sexual behavior. Initial workup revealed negative hepatitis A/B/C antibodies, ferritin 10.6k, negative autoimmune workup, and an HCV viral load of 31 million copies. His liver tests improved and he was seen in clinic where liver elastography showed a kPa of 17.7 (IQR 21%). He was started on a 12-week course of glecaprevir/pibrentasvir. Follow up HCV antibody was positive three weeks later. His clinical course was further complicated by ITP treated with IVIG, dexamethasone, rituximab and ultimately splenectomy. Course was also complicated by renal failure due to secondary minimal change disease and neuropathy due to AIDP/CIDP. He received additional dexamethasone and IVIG for these conditions. One month later he completed DAA therapy, LFTs normalized, and his HCV viral load was < 12. Two days after completing HCV therapy he was admitted to the ICU with septic shock of unclear etiology, ultimately resulting in death.

DISCUSSION:

Our patient’s workup was suggestive of acute HCV given initial negative antibody with eventual seroconversion. A 12-week course of glecaprevir/pibrentasvir was chosen, as there was uncertainty regarding whether or not cirrhosis was present. Aggressive immunosuppression was given concomitantly for the MZL, as well as other immune-mediated diseases. His sepsis-related death was felt to be due to prolonged hospitalization in an immunosuppressed state rather than DAA therapy. Larger scale studies are needed to further inform optimal treatment of HCV in patients receiving immunosuppressive therapy, in the setting of malignancy.
2323 Bone Metastasis as Index Presentation of Hepatocellular Carcinoma
Authors: Then Eric O. , Ofosu Andrew , Gayam Vijay , Maddika Srikanth , Chaudhri Ali , Gaduputi Vinaya
Published: October 2019

INTRODUCTION:

Hepatocellular carcinoma (HCC) is the most common primary tumor of the liver, and the fifth most common cancer worldwide Extrahepatic mestastasis rarely occurs in patients with early course HCC. Bone metastasis as the initial presentation of HCC is even more rare. Here we present an unusual case of Bone metastasis to the ninth rib as the index presentation of HCC.

CASE DESCRIPTION/METHODS:

The patient is a 60 year old male with a medical history of hypertension, hepatitis C virus, hyperlipidemia, rheumatoid arthritis and chronic obstructive pulmonary disease who presented to the emergency department complaining of right upper quadrant pain for one week. Physical exam was notable for tenderness over the patients right ninth rib. The rest of the physical exam was unremarkable. Notable laboratory workup included a complete blood count and comprehensive metabolic panel within normal limits. Notable imaging included a computed tomography of the abdomen showing a mass over the ninth rib with multiple foci in liver. The next day an alpha fetoprotein (AFP) level was ordered and returned elevated at 1139.0 ng/ml. Other tumor markers including carcinoembryonic antigen (CEA), carbohydrate antigen 19 9 (CA 19 9) and prostate specific antigen (PSA) were within normal limits. Interventional radiology was then consulted and performed a biopsy of the ninth rib mass which showed metastatic carcinoma with a primary of hepatocellular malignancy. This diagnosis was supported by positive staining with Hep-Par 1. Following this diagnosis the oncology service was consulted and recommended palliative radiotherapy to the ninth rib and starting treatment with Sorafenib. The patient was then discharged with pain medications and follow up appointments.

DISCUSSION:

Common risk factors for the development of HCC include Hepatitis B, Hepatitis C, Aflatoxin exposure, and cirrhosis of any cause (including alcohol). The most common sites of extra-hepatic metastasis of HCC are lung, lymph node, musculoskeletal, and adrenal gland. Isolated bone metastasis of HCC is rare occurring in only 9.5% of patients. Symptoms associated with HCC include right upper quadrant pain, jaundice and ascites in more advanced cases. Treatment of metastatic HCC to bone includes chemotherapy and targeted radiotherapy. More recently Bisphosphonates have also been shown to have success.
2331 Post-Infantile Giant Cell Hepatitis Leading to Severe Acute Liver Injury in Chronic Lymphocytic Leukemia (CLL): Progression of CLL vs. Drug Induced Liver Injury (DILI)
Authors: Saeed Naba , Warrier Govind , Likhitsup Alisa , Hassan Ammar , Choi Eun-Young K. , Conjeevaram Hari
Published: October 2019

INTRODUCTION:

Post-infantile giant cell hepatitis (PIGCH) is a form of hepatocyte degeneration, triggered by noxious injury. Underlying etiologies are autoimmune, drug-induced, virus mediated and miscellaneous e.g. sickle cell, CLL, post-transplant. While only occurring in 5 per 100,000 adults, when present, rapid progression to liver failure can result in a 50% mortality rate.

CASE DESCRIPTION/METHODS:

A 76 yo lady with a history of anemia, remote breast cancer in remission, and CLL (not on treatment), presented with a 3-week history of fatigue, nausea and jaundice following a 5-day course of azithromycin and prednisone (20 mg OD), and one dose of ceftriaxone, for acute bronchitis. Exam revealed jaundice and abdominal distension. Admission labs: AST 4233U/L, ALT 3175U/L, ALP 187IU/L, total bilirubin 9.4 (7.1 direct) mg/dl, sodium 131mEq/L, BUN 27, Cr 0.7 mg/dl. INR 1.4. WBC 68.3/µL, hemoglobin 8.2 g/dl, platelets 233/µL. CT Abd/Pelvis without contrast: mild periportal edema, with enlarged liver, otherwise normal. Workup for viral hepatitis, other viruses and autoimmune serologies was negative, other than ANA titer of 1:640. Tox screen and mass spectrometry were negative. Given worsening liver injury, liver biopsy was obtained, which had active hepatitis with giant cell transformation, patchy necrosis, and CLL in portal areas (Figure 1). The patient was recommended high-dose prednisone, but she deferred therapy. Labs were followed closely, with remarkable improvement within a few days. She was discharged on ursodiol. On follow-up 2 months later, a mild transaminitis was persistent, so prednisone therapy was started with subsequent resolution of her hepatitis. However, subsequent imaging revealed development of cirrhosis.

DISCUSSION:

Treatment of PIGCH is dependent on underlying etiology, with autoimmune pathology having the best prognosis described in the literature versus variable courses for drug induced PIGCH, once the offending agent is stopped. In our patient, the trigger for giant cell transformation was suspected to be drug induced liver injury, from treatment with azithromycin/ceftriaxone. Interestingly, CLL itself can spontaneously transform to PIGCH as well, with treatment of CLL improving prognosis. Our patient did not want immunosuppressive therapy initially. Although her acute liver injury spontaneously improved, she did develop cirrhosis as a long-term complication. PIGCH must be aggressively treated/monitored to prevent life-threatening fulminant hepatic failure, or subsequent cirrhosis.
2332 Large Hepatic Arteriovenous Fistula Leading to Non-Cirrhotic Portal Hypertension and Associated Complications
Authors: Le Thanh-Truc , Jencks Kara J. , Parupudi Sreeram
Published: October 2019

INTRODUCTION:

Portal hypertension (and its complications including bleeding, ascites, encephalopathy) often occur in patients with liver cirrhosis as a result of intrahepatic resistance to portal blood flow and increased portal collateral blood flow. Non-cirrhotic portal hypertension (NCPH) is less common, but can also lead to similar complications. Arteriovenous fistulas are an uncommon presinusoidal intrahepatic cause of NCPH. We present a rare case of a patient with a remote history of a liver biopsy who presented with new onset ascites and found on imaging to have a large hepatic arteriovenous fistula which was subsequently treated with embolization.

CASE DESCRIPTION/METHODS:

A 61 year old male with no significant past medical history presented to clinic for evaluation of progressively worsening abdominal and lower extremity swelling. Laboratory findings on presentation were remarkable for hemoglobin 7.4 g/dL, platelets 59,000/microliter, INR 1.6, Na 137mmol/L, Cr 1.56 mg/dL T bilirubin 2.4 mg/dL. Paracentesis fluid studies showed high serum-ascites albumin gradient (SAAG) and high protein without evidence of heart failure on echocardiogram. Magnetic resonance imaging of the abdomen showed a 6.3 × 4 centimeters hepatic arteriovenous fistula within segment VIII of the liver; the portal vein was noted to be patent. Liver biopsy showed focal perivenular and subsinusoidal fibrosis without bridging (fibrosis stage 1/6) and nodular regenerative hyperplasia changes with bands of hepatocyte atrophy without any evidence of cirrhosis. Right hepatic arteriogram demonstrated a dilated and tortuous vessel with early filling of the portal venous system and a large aneurysm at the communication point between the arterial and venous fistula. Successful embolization of the anterior segmental branch of the right hepatic artery was achieved. The patient’s hospital course was also complicated by upper gastrointestinal bleeding secondary to esophageal varices which required banding x5.

DISCUSSION:

The presence of portal hypertensive changes with minimal hepatic fibrosis on liver biopsy is best categorized as non-cirrhotic portal hypertension (NCPH). This patient manifested complications of portal hypertension including variceal bleeding and recurrent ascites secondary to a large hepatic arteriovenous fistula which likely formed following his liver biopsy. Closure of large arteriovenous fistulas can be beneficial in preventing worsening portal hypertension and associated potentially fatal complications.