bluuebunny/crossref_metadata_2025 huggingface dataset:
2387 Desmoplastic Tumor: Rare and Advanced Presentation
Authors: Khudyakov Aleksandr , Arroyo-Mercado Fray , Sumbly Vikram , Ahmed Rafsan , Wang Donghai , Shao Charles , Frank David
Published: October 2019

INTRODUCTION:

Desmoplastic small round cell tumors (DSRCT) are a rare and aggressive variant of soft-tissue sarcomas. Data suggest that occurs as a consequence of a translocation of (11;22) (p13; q12) resulting in a fusion of EWS/WT1 genes. The incidence of DSRCT is more common in young Caucasian males between the ages of 15 and 25 [6,7].

CASE DESCRIPTION/METHODS:

A 26-year-old male with no past medical history complaining of shortness of breath, anorexia, unintentional weight loss of 20 pounds and increased abdominal girth associated with pain starting two months prior to presentation. Physical exam was remarkable for cachexia, mild diffuse tenderness to palpation, protuberant abdomen without a distinct mass. Initial workup showed the following abnormality; normocytic anemia with hemoglobin of 12.6, ALP 189, AST 58, ALT 73 and HIV negative. Abdominopelvic CT scan revealed a large mass measuring 20 × 10 × 10 cm in the left upper quadrant, nodular deposits in the anterior abdomen concerning for carcinomatosis and multiple liver metastases, largest measuring 10 × 8 cm (Figure 1). Mesenteric lymph node biopsy showed findings consistent with desmoplastic fibromyxoid stroma and immunohistochemically positive for AE1/AE3, EMA, vimentin, desmin, synaptophysin, and CD99 (Figures 2–3).

DISCUSSION:

DSRCT's are aggressive soft tissue sarcomas that are extremely rare. They predominantly involve the pelvis, retroperitoneum, and mesentery. Upon diagnosis, it is crucial to exclude desmoid tumors and Ewing sarcomas, since, these malignancies that can be confused with DSRCT. Accumulating data suggest that is more prevalent in young adults, making the diagnosis challenging at the early stages, since, it can be potentially confused with less aggressive conditions such as gastritis, dyspepsia or gastroesophageal reflux disease. Prognosis is frequently poor given a late presentation of disease; five-year survival is estimated at 15 to 30% [2]. The exact mechanism of DSRCT is not well understood, but research suggest it arises from a translocation. Some promising therapies are undergoing phase trials as surgical interventions and hyperthermic intraperitoneal chemotherapy [2]. Systemic chemotherapy with aggressive surgical resection has shown improved outcomes [6]. Nonetheless, systemic therapy tends to be very toxic, requiring frequent admissions to control potential adverse events. Further research is needed to characterize the exact pathogenesis and treatment of this uncommon malignancy.
2388 Peliosis Hepatis Progressing to Acute Liver Failure
Authors: Engelberg Rachel , Mehta Amit , Wan David
Published: October 2019

INTRODUCTION:

Peliosis hepatis is characterized as a vascular condition in which blood-filled cystic cavities are distributed diffusely throughout the liver parenchyma. The condition is rare, with the pathogenesis poorly described in the literature. It is believed that this disease is secondary to an underlying malignancy, immune disorder, infection, or a drug/toxin mediated effect. We present a case of a patient diagnosed with peliosis hepatis complicated by the development of acute liver failure.

CASE DESCRIPTION/METHODS:

A 71-year-old man with a past medical history of coronary artery disease, hypertension, hyperlipidemia, and prostate cancer treated with radiosurgery was admitted to our hospital with symptoms of fatigue, dysphagia and anorexia. He was in his usual state of health until four weeks prior to admission, when he began experiencing early satiety with poor oral intake, nausea, diarrhea, right upper quadrant abdominal pain, and weight loss. He was evaluated by his primary care provider who prescribed him a five day course of levofloxacin with no resolution of symptoms. Upon admission, labs were notable for leukocytosis and a mild transaminitis. Imaging revealed hepatomegaly with hepatic hemangiomatosis and splenic hemangiomas. A liver MRI was performed which returned consistent with diffuse hepatic peliosis with splenic peliosis. He underwent an intensive hematologic/oncologic, rheumatologic, and infectious workup that was significant for a positive quantiferon TB gold and he was subsequently started on empiric TB therapy. Synthetic liver function continued to worsen consistent with acute liver injury, which progressed to acute liver failure. The patient continued to decline and ultimately passed from multi-organ system failure.

DISCUSSION:

The majority of patients with peliosis hepatis will present asymptomatically and are found incidentally, while a subset of patients will present with abdominal pain or stigmata of liver disease. A liver biopsy is useful for diagnosis and is used in the context of the clinical picture, laboratory data, and imaging findings. Once the underlying offending etiology is recognized and appropriately treated, symptoms can improve and regression of the hepatic lesions can be seen. Our patient is unique in that his presentation was atypical and rapid, and a true causal etiology was never concretely identified, leading to progression to acute hepatic failure.
2391 When Breast Cancer Comes Back With Vengeance: Death Within Three Weeks Secondary to Acute Liver Failure
Authors: Rozner Raquel N. , Wan David
Published: October 2019

INTRODUCTION:

Breast cancer (BC) is the most common cancer in women in the developed and developing world. Here we report a patient with treated stage II HER2+ BC presenting with abnormal liver enzymes and jaundice, found to have innumerable hepatic lesions consistent with metastatic BC, with rapid progression to acute liver failure(ALF).

CASE DESCRIPTION/METHODS:

A 63-year-old female with history of stage II HER2+ BC who underwent six cycles of neoadjuvant chemotherapy, lumpectomy, radiation, letrozole for four years, presented with one week of abdominal distension, dark urine, pale stools, and jaundice. She denied a history of hepatitis, intravenous drug use, or herbal supplement use but used marijuana and was a former smoker and heavy alcohol user. Six years prior to admission, two tubulovillous adenomas, 1.0 × 1.0 × 0.8 cm and 1.5 × 1.5 × 1.2 cm were resected. Surveillance colonoscopy, six months later, revealed a tubular adenoma, 0.2 × 0.2 × 0.2 cm and a tubulovillous adenoma, 1.2 × 1.2 × 1.2 cm. She missed her repeat surveillance colonoscopy. Physical examination was notable for jaundice, scleral icterus, hepatomegaly, and abdominal distension. Workup included negative hepatitis panel, platelet count 121,000/uL, total bilirubin 14.2mg/dL, direct bilirubin 10.7mg/dL, alkaline phosphatase 1367 IU/L, aspartate aminotransferase 621 U/L, alanine aminotransferase 252 U/L, albumin 1.5 g/dL, prothrombin time 14.7 seconds, activated partial thromboplastin time 41.5 seconds, international normal ratio 1.2, CA 15-3 3900 U/mL, CA 19-9 7200 U/mL, and CEA 844 U/mL. CT Abdomen/Pelvis revealed innumerable hepatic metastases, hepatomegaly, and small volume ascites (image 1). She underwent IR-guided biopsy of a hepatic lesion, confirmed as metastatic HER2 equivocal BC. Within one week, she became encephalopathic and died whilst deciding between hospice and systemic chemotherapy.

DISCUSSION:

BC represents about 30% of all cancer diagnoses in women each year. Of those diagnosed at early stages, 20-30% will relapse with metastatic BC. HER2+ BC has a higher risk of relapse than other subtypes, and is more likely to spread to the brain, liver, bone, and lung. This patient’s hepatic involvement was concerning for metastatic BC or colon cancer, based on past tubulovillous adenomas and missed surveillance colonoscopy. Within three weeks of presentation, she died. This case highlights the fulminant progression of HER2+ BC causing ALF due to tumor infiltration of liver parenchyma with cessation of synthetic capacity.
2394 Development of Hepatic Infarct After Successful Transjugular Intrahepatic Portosystemic Shunt (TIPS) Placement
Authors: Mavuram Meher S. , Herlevic Vincent , Samant Hrishikesh , Morris James D. , Pandit Sudha , Manas Kenneth J. , Jordan Paul
Published: October 2019

INTRODUCTION:

Transjugular intrahepatic portosystemic shunt (TIPS) placement has now been well established as an effective and considered relatively safe treatment for the management of sequelae of portal hypertension. Hepatic infarction is an uncommon, potentially fatal complication after a TIPS procedure but rarely reported in cirrhosis patients with a low MELD (Model for End Stage Liver Disease). Here, we present a case of large hepatic infarct developing within a day of successful placement of TIPS in a patient with low MELD, which resolved with supportive treatment.

CASE DESCRIPTION/METHODS:

We present a 55 y/o Caucasian lady with Laennec cirrhosis with MELD 15 and normal bilirubin, who underwent elective TIPS placement for refractory ascites at our tertiary care center. Patient tolerated the procedure well with normal monitored vitals post procedure. However, she started complaining of right upper quadrant abdominal pain on next day with elevated transaminases and INR (40- fold and 2- fold increase from her baseline, respectively) with a stable bilirubin, CBC and creatinine. CT triple phase of the liver revealed a large hypodensity involving segments 6, 7 of the liver, suggestive of massive hepatic infarction and a patent TIPS. Patient not being a suitable candidate for liver transplant (with a history of recently diagnosed bladder carcinoma), was managed with supportive measures- Fluid, albumin, pain control and antibiotics. Liver enzymes continued to improve with return to baseline in 2 weeks. Subsequent clinic follow up showed complete control of ascites.

DISCUSSION:

Potential sequelae of TIPS are reported more frequently in cirrhotic with a MELD score greater than 18. Hepatic infarction is an uncommon development after a TIPS procedure. However, physicians must be aware of this virtually serious, post TIPS complication in cirrhotics even with a low MELD, which may progress to acute liver failure, with a need for liver transplant. Though our patient responded to supportive care, we think, an ideal patient planned for elective TIPS should be aware of this rare phenomenon. Prompt diagnosis is imperative to decrease the mortality from hepatic infarction.
2402 A Rare Case of IPEX Syndrome With Hepatic Complications
Authors: Ahmad Bareeqah , Masood Umair , Sharma Anuj , John Savio
Published: October 2019

INTRODUCTION:

Immunodysregulation, polyendocrinopathy and enteropathy X-linked (IPEX) Syndrome is a rare autoimmune illness affecting only males starting in the early few months of life. It can be associated with autoimmune hepatitis among other systemic ailments and ultimate treatment is immunosuppression or bone marrow transplant. We present a case of undiagnosed IPEX Syndrome leading to liver failure requiring eventual liver transplantation.

CASE DESCRIPTION/METHODS:

A 19 year-old male patient had undergone a liver transplant at age 2 for suspected autoimmune hepatitis resulting in acute liver failure. No specific cause was identified at time of diagnosis. He remained on chronic immunosuppression thereafter, eventually progressing to chronic active autoimmune hepatitis in the transplanted liver. He was diagnosed with IPEX Syndrome at age 16, for which he underwent a bone marrow transplant. He presented to the hospital with neutropenia, and hepatology was consulted for liver transplant rejection in light of mild transaminitis. On admission, vital signs were normal and pertinent labs include: AST/ALT 50/74 units/L, Alkaline Phosphatase 811 IU/L, Albumin 3.4 g/dL, WBC 0.6 × 109 cells/L. He reportedly had multiple episodes of acute rejection in the past which were treated with steroids. Chronically, he was on mycophenolate mofetil, tacrolimus, and prednisone. His liver biopsy did not show evidence of acute rejection, but suggested chronic rejection and liver fibrosis. A prior liver biopsy had suggested autoimmune hepatitis, but no evidence of a flare on current biopsy. He was continued on his current immunosuppression regimen, and he was subsequently discharged home.

DISCUSSION:

Due to its rarity, incidence of IPEX syndrome is not entirely known, but is estimated to be 1 in 1,609,490. Generally, it is diagnosed in infancy and is often fatal. Our case is unique as the diagnosis was missed for more than a decade during which period the patient had even received a liver transplant for autoimmune hepatitis. Autoimmune hepatitis reportedly occurs in 20% of patients with IPEX Syndrome. There are currently no reports of liver failure requiring liver transplant in such scenarios. The fact that our patient developed recurrent autoimmune hepatitis in the transplanted liver highlights the underlying defect in his genetic disorder. This case should serve as a reminder to clinicians to consider IPEX syndrome in the differential for early onset autoimmune hepatitis, as early diagnosis could reduce significant morbidity.
2407 Clinical Challenges of Diagnosis and Management: A Case of Malignant Insulinoma With Hepatic Metastasis
Authors: Khan Adil , Lu Zheyu , Panghal Ritvika , Naeem Raza
Published: October 2019

INTRODUCTION:

Insulinoma is a rare type of neuroendocrine tumor, about 4 cases per 1 million person-years. It can present at varied sites of body and metastasize extensively and progress rapidly. We present a case of rare and complicated duodenal insulinoma with significant hepatic metastases requiring integrated diagnostic workup and multidisciplinary management with specialties- gastroenterology, pulmonary, oncology, endocrinology and interventional radiology.

CASE DESCRIPTION/METHODS:

70 year old male with PMHx. of tobacco who presented with fall. When EMS arrived, patient was found to be hypoglycemic. Noted to have increased liver enzymes, CT of Abdomen showing multiple liver lesions and heterogenous lesion near or arising from the distal stomach/pylorus. Due to refractory hypoglycemia, he was started on D20W on a high rate. GI performed EGD which showed large penetrating duodenal ulcer at posterior wall of duodenal bulb and biopsy showed reactive changes but negative for intestinal metaplasia. IR guided Liver Biopsy was performed which showed Metastatic Neuroendocrine tumor, grade 3. Immunostains reveal neoplastic cells positive for synaptophysin. Ki67 show high proliferation (up to 50%). B-OH gastrin level, VIP, proinsulin were normal, insulin and c-peptide were interestingly low. Also, ACTH sim test was negative. Chromogranin was elevated. On repeat EGD with EUS and biopsy obtained directly from duodenal bulb was consistent with neuroendocrine tumor diagnosis. Also, Octreoscan scan did not display of Octreoscan uptake. Given this finding and the high KI-67 index, the hepatic metastases were thought likely somatostatin receptor negative. IR decided to perform embolization. IR angiography, showed very large hyper vascular tumor occupying the entire left and anterior right lobe of the liver and 2/3 left hepatic artery. It did persist despite left, right gastric artery and 2/3 left hepatic artery embolization. Later, patient was weaned off dextrose drip and blood sugars stabilized. Patient discharged home with follow up with IR, gastroenterology and Oncology team.

DISCUSSION:

This is rare case of hepatic metastatic insulinoma with paradoxical negative diagnostic parameters and atypical location, successfully diagnosed by EUS endoscope and liver biopsy and managed by multidisciplinary specialties.
2409 A Rare Complication of Gastric Bypass in the Setting of Chronic Liver Disease
Authors: Kaur Randeep , Naffouj Sandra , Jafri Syed-Mohammed
Published: October 2019

INTRODUCTION:

Porto-mesenteric venous thrombosis (PMVT) is a known and potentially fatal complication of general surgery. However, it has been rarely reported as a complication of worsening liver disease after undergoing bariatric surgery. This is a case of a patient who presented with mesenteric thrombosis following Roux-en-y gastric bypass (RYGB).

CASE DESCRIPTION/METHODS:

Patient is a 42-year-old African American female with a history of RYGB who presented with episodes of post-operative abdominal pain for 5 years. She was found to have recurrent high-grade small bowel obstruction's (SBO) necessitating a small bowel resection. Her course was further complicated by intra-abdominal abscesses requiring computed tomography (CT)-guided drainage, and wound dehiscence requiring open surgical intervention and debridement. Four months after discharge, she presented with right-sided abdominal pain and acute encephalopathy. Liver enzymes were elevated. Abdominal ultrasound revealed portal vein thrombosis (PVT). Dual-phase CT of the abdomen revealed sub-acute to chronic complete diffuse mesenteric thrombosis with collateralizations involving the portal, splenic, and distal superior mesenteric veins. Given that previous imaging suggested steatosis and hepatomegaly, sub-acute liver injury secondary to thrombosis was established in the setting of chronic liver disease. The patient's underlying non-alcoholic fatty liver disease was likely worsened by severe malnutrition and short gut syndrome following the RYGB. Her hepatic encephalopathy resolved after initiating lactulose and rifaximin. Outpatient hematology evaluation for all causes of thrombophilia remained inconclusive and the patient had no recurrences of thrombosis.

DISCUSSION:

RYGB remains the gold standard for bariatric surgery as it results in the most durable weight loss. However, complications may occur such as gastrointestinal leak, fistulas, marginal ulcers, SBO, and dumping syndrome. Mechanisms of thrombosis formation in this setting could be related to intra-operative vascular manipulation, increased intra-abdominal pressure, venous stasis, and possibly deteriorating liver function secondary to rapid weight loss and malnutrition. PMVT hasn't been reported consistently as a complication in various bariatric surgery studies, which leads us to conclude that it's either under or mis-diagnosed. It is extremely important to consider PMVT as a complication of RYGB in patients who have evidence of underlying liver disease prior to undergoing RYGB.
2416 A Case of Fever, Rash, and Abdominal Pain in an Immunocompromised Host
Authors: Colbenson Gretchen , Kamboj Amrit K. , Dines Virginia
Published: October 2019

INTRODUCTION:

Varicella Zoster Virus (VZV) is a DNA virus that leads to two clinical syndromes: varicella (primary) and herpes zoster or shingles (secondary). Disseminated infection is most common in immunocompromised hosts and can lead to multi-organ involvement including hepatitis, pneumonitis, encephalitis and others. Here, we describe a case of a young woman with human immunodeficiency virus (HIV) and primary varicella infection leading to VZV hepatitis.

CASE DESCRIPTION/METHODS:

A 35-year-old woman presented with a 1-week history of fevers, right upper quadrant and epigastric pain, and a non-pruritic, non-painful rash. Her past medical history was significant for a diagnosis of HIV 5 years prior, not currently on treatment. Upon presentation, vital signs were within normal limits except for a fever of 38.8°C. Physical examination was notable for multiple crusted erosions and papules present on her face, trunk and extremities (Figures 1 and 2). Laboratory evaluation was notable for alanine aminotransferase level 291 U/L, aspartate aminotransferase level 260 U/L, alkaline phosphatase level 135 U/L, total bilirubin 0.4 mg/dL, leukocytes 8 × 109/L, and platelet count 132 × 109/L. CD4 count was 118 cells/mcL and HIV-1 RNA 4,120,000 copies/mL. Computed tomography abdomen/pelvis demonstrated diffuse heterogeneity of the hepatic parenchyma (Figure 3). She underwent liver biopsy and pathology demonstrated hepatic parenchyma with portal and lobular inflammation but no necrosis. VZV polymerase chain reaction (PCR) from the liver and facial lesion swab returned positive. She was treated with 4 days of IV acyclovir with improvement in her fevers and transaminitis and then transitioned to oral valacyclovir. HAART was also initiated two days after the start of IV acyclovir.

DISCUSSION:

The differential for fever and widespread rash in an immunocompromised individual is broad, and includes disseminated herpes simplex virus, VZV, secondary syphilis, disseminated gonococcal infection, parvovirus, cryptococcosis and others. While uncomplicated VZV can be treated with supportive care or oral antivirals, such as acyclovir, valacyclovir, or famciclovir, an immunocompromised host with disseminated VZV should typically be admitted to the hospital for intravenous acyclovir. Biopsy proven VZV hepatitis is rare and is associated with a high mortality. Notably, our patient's VZV IgM and IgG were negative, indicating the need for a high degree of suspicion if the clinical scenario is consistent with VZV.
2418 Abdominal Wall Hematoma Due to Dissection of the Deep Circumflex Iliac Artery: A Rare Complication of Ultrasound Guided Paracentesis
Authors: Amarnath Shivantha , Daoud Magda , Mulrooney Stephen
Published: October 2019

INTRODUCTION:

Aspiration of ascitic fluid via paracentesis is performed for both diagnostic and therapeutic purposes, using a landmark based approach (LBA) or the safer ultrasound guided method. Complication rate for paracentesis is less than 2% and LBA carries a nearly four-fold increase as opposed to the sonographic approach. Complications include bowel perforation, infection/peritonitis, and bleeding. It is well documented that hemorrhagic complications due to accidental transection of the inferior epigastric artery (IEA) or its branches can lead to abdominal wall hematomas, pseudoaneurysms and hemoperitoneum. We report a rare complicated case of accidental iatrogenic laceration of the deep circumflex iliac artery (DCIA) due to altered anatomy in a patient with ascites.

CASE DESCRIPTION/METHODS:

50-year-old female with decompensated alcoholic liver cirrhosis, coagulopathy and thrombocytopenia (Child-Pugh class C and MELD 29) presented with abdominal distention due to worsening abdominal ascites. Vitals were normal. Physical exam demonstrated fluid shift and mental status was intact. Laboratory findings reported thrombocytopenia of 118 K/uL, INR of 1.8 and Albumin of 2.2 g/dL. Ultrasound revealed extensive abdomino-pelvic ascites with the largest pocket of 19 cm in diameter in the right lower quadrant. She underwent sonographic diagnostic paracentesis of the right lower quadrant with drainage of 60 cc of serous fluid. Spontaneous bacterial peritonitis was ruled out. The next day, she endorsed sharp pain near the incision site and her hemoglobin dropped by 2 units. Patient was hypotensive and underwent CT abdominal angiography. This revealed a large right anterior abdominal wall hematoma measuring 24 cm × 9 cm with active arterial extravasation from the ascending branch of the right DCIA. She underwent angiographic transcatheter coil embolization of the DCIA. She remained stable and a repeat CT angiogram demonstrated reduced size of the hematoma and was discharged.

DISCUSSION:

The literature reveals that distribution of the abdominal wall vasculature, primarily the IEA is often displaced laterally in patients with ascites. Our case proves that this holds true for the DCIA, the second branch of the external iliac artery that travels along the iliac crest. Future studies should focus on incidence of the aberrant anatomy of abdominal wall vasculature, which might help clarify if pre-procedure precautions like the color doppler mode could possibly reduce risk of all paracentesis related morbidity and mortality.
2420 A Rare Cause of Portal Vein Thrombosis
Authors: Loganathan Danekka , Shah Gunjan
Published: October 2019

INTRODUCTION:

Hormone therapy is the mainstay adjuvant therapy for patients with estrogen receptor positive early stage breast cancer. Anastrozole is generally considered to have lower risk of venous thromboembolism compared to tamoxifen, albeit not negligible.

CASE DESCRIPTION/METHODS:

81-year-old female with history of breast cancer on anastrozole presented to the hospital with high-grade fever. Physical exam was significant for RUQ tenderness. Her labs were significant for elevated transaminases, ALP and direct hyperbilirubinemia without leukocytosis. US abdomen showed enlarged gallbladder with stones. MRI was positive for portal vein thrombosis in the main portal vein and extending into right portal vein, with possible central anterior segment of right liver lobe infarct. She had no history of liver disease or venous thromboembolism. There is no clinical or radiological evidence of recurrence of malignancy. Patient was started on anticoagulation and was discharged on apixaban. APLA testing is pending and hypercoagulability workup was otherwise negative. It was concluded that the portal vein thrombosis was secondary to anastrozole due to lack of alternative etiologies.

DISCUSSION:

To our knowledge, our case of hepatic portal vein thrombosis suspected to be secondary to anastrozole therapy is the first to be reported in the medical literature. A case report suggesting a causal association between pulmonary embolism and anastrozole in a patient with no prior VTE and negative hypercoagulable workup was published in 2006 by Lycette et al. It is particularly important to consider the causative role of Anastrozole in patients with otherwise unexplained venous thromboembolism in unusual vascular beds as seen in our patient.
2429 Acute Severe Cholestatic Hepatitis Secondary to Emerge Weight Loss Supplement
Authors: Mukherjee Sandeep , Vellanki Meghana
Published: October 2019

INTRODUCTION:

Herbal and dietary supplements (HDS) are an important cause of liver injury. EmergeR weight loss supplement is advertised as a product which burns fat and stimulates weight loss. We report a case of acute severe cholestatic hepatitis after consumption of EmergeR in a 32 year-old man. This case highlights a previously unreported and important association between this supplement and hepatotoxicity and the importance of obtaining a through medication history including HDS.

CASE DESCRIPTION/METHODS:

A 32-year-old male presented to hepatology clinic for evaluation of jaundice and elevated liver tests. He was well until 1 week ago when he developed flu-like symptoms, fatigue, jaundice, pruritus, dark urine and pale stools. He denied other symptoms, recent travel, reported rare alcohol use and did not take any other medications. However, he started a new weight loss supplement, EmergeR 3 weeks ago and had normal liver tests prior (Table 1). Exam was remarkable for a BMI of 35 and scleral icterus. Liver tests were elevated with AST 987 IU/L, ALT 2305 IU/L, total bilirubin 7.2 mg/dl and INR 1.1 (Table 1). Testing for other viral causes was negative. Abdominal ultrasound showed hepatic steatosis. The patient was advised to stop EmergeR immediately and undergo liver tests and INR weekly. 4 weeks later (7 weeks after starting EmergeR), patient was asymptomatic with resolution of prior symptoms.

DISCUSSION:

Despite the widespread use and potential severe side effects associated with weight loss supplements, HDS do not require FDA approval prior to marketing.3 This emphasizes the need for physicians to report potential side effects associated with weight loss products as continued use without awareness or proper monitoring can lead to severe liver injury. To our knowledge, this is the first reported case of acute cholestatic hepatitis from EmergeR use. The temporal relationship of acute liver injury with the use of Emerge, improvement after withdrawal and absence of other possible causes strongly suggested that liver injury was secondary to this supplement. As EmergeR contains a variety of ingredients which may also be contaminated by an unknown toxic botanical or synthetic chemical, it remains unclear which component of EmergeR caused liver injury.4 As patients may exhibit subtle symptoms such as fatigue and nausea at the time of presentation, a high index of suspicion is required for diagnosis of HDS induced liver injury along with a through HDS use history.
2433 Sub-Acute Liver Failure as the Initial Manifestation of Metastatic Small Cell Lung Cancer
Authors: Agarwal Amit K. , Yoo Joseph , Halegoua-DeMarzio Dina
Published: October 2019

INTRODUCTION:

The liver is a common site of metastasis in small cell lung carcinoma (SCLC), with 20% of cases metastasizing to the liver. However, most liver metastases are discovered at time of autopsy and not at initial diagnosis. Given the scarcity of literature regarding sub-acute liver failure as the presenting sign of metastatic SCLC, this case explores a patient who presented with sub-acute liver failure secondary to diffuse, infiltrative metastatic SCLC.

CASE DESCRIPTION/METHODS:

A 54 year old Caucasian male with no significant past medical history presented with jaundice and ascites. He denied any history of alcohol consumption, any personal or family history of liver disease, and consumption of any supplements or over the counter medications. Physical examination was notable for marked abdominal distension with shifting dullness, grade I hepatic encephalopathy, and mild asterixis. He did not have findings of chronic liver disease. Labs were significant for a creatinine of 1.1, sodium of 128, total bilirubin of 8.9, ALT 85, AST 227, alkaline phosphatase 153, and an INR of 3.03, with a MELD-Na score of 32. Laboratory workup was negative for causes of liver failure, including infectious and autoimmune etiologies. A triple phase contrast enhanced CT abdomen showed multiple hypodense lesions too small to characterize, without any large enhancing liver mass seen. Given the patient's high MELD-Na score and an initial concern for liver failure, the patient was transferred to our institution for liver transplant. To determine the etiology of his suspected cirrhosis after negative serologic workup, a transjugular liver biopsy was obtained. Pathology results were not consistent with cirrhosis, and instead revealed diffuse, infiltrative metastatic small cell carcinoma from a suspected primary lung cancer in the setting of a 1.8 cm lung lesion later seen on CT chest. He was subsequently removed from liver transplant consideration. The patient unfortunately expired due to liver failure.

DISCUSSION:

This is a case of a patient who presented with sub-acute liver failure due to metastatic small cell carcinoma. Liver failure is a rare presentation of metastatic disease but has been reported in malignancies such as metastatic breast cancer. While SCLC metastasis to the liver is common, it has only rarely been described to cause sub-acute liver failure.
2435 An Uncommon Case of Hepatic Abscess From Fish Bone Ingestion: A Case Report With Successful Conservative Management
Authors: Maruvada Smita , Vuppala Anusha , Hafiz Nazar , Sabillo Sheryl
Published: October 2019

INTRODUCTION:

Accidental ingestion of foreign bodies is infrequently seen in clinical practice and can be fatal. Liver abscess from enterohepatic migration of an ingested foreign body is extremely rare and is usually associated with perforation, peritonitis from rupture of the hepatic abscess and sepsis. Lack of specific symptoms, unacknowledged ingestion by the patient and a low clinical suspicion, complicate our arrival at an accurate diagnosis. Here, we report a case of hepatic abscess secondary to fish bone ingestion, without peritonitis, that was managed conservatively and without extraction of the foreign body.

CASE DESCRIPTION/METHODS:

Our patient is a 64 y/o lady who came in with sharp, right sided abdominal pain, fever and anorexia for a week. She was febrile upon arrival with leukocytosis and mild transaminitis with total bilirubin 2.7. Other monitored vitals were stable. CT abdomen revealed a 6.9 cm abscess at the junction of the right and left hepatic lobes which was promptly drained percutaneously by IR. Despite clinical improvement with drainage and antibiotics, persistent leukocytosis was appreciated. A repeat CT showed a small residual liver abscess and a sharp, linear radiopaque foreign body adjacent to the pylorus with perigastric stranding. Upon further questioning, patient recollected having had fish prior to onset of symptoms. Endoscopy was unsuccessful in identifying the exit tract from the upper GI tract. Reimaging confirmed migration of the fishbone towards the falciform fissure and gastrohepatic ligament. The position of the fish bone posed a challenge for percutaneous extraction and Surgery recommended against exploratory laparotomy as patient had marked clinical improvement. Follow up imaging a month later showed the fish bone to be in stable position, in an asymptomatic patient.

DISCUSSION:

80% – 90% of ingested foreign bodies usually pass through the gut without any intervention. Foreign body ingestion is generally an unconscious event, that the patient rarely recalls, which complicates the preoperative diagnosis in most cases. In the case of liver abscess unresponsive to drainage and antibiotic therapy, this possibility needs to be entertained, despite its low incidence. Besides antibiotics and abscess drainage for size >5 cm, surgical or endoscopic retrieval of the foreign body has always been the conventional treatment. However, medical management should be considered for patients, like ours, without signs of severe sequelae.
2436 Lysosomal Acid Lipase Deficiency: A Missed Diagnosis Spanning All Ages
Authors: Filo Jean , Ascher Bartlett Johanna M. , Mittal Naveen , Rodas Fabian , Rosas Christy , Alkhouri Naim
Published: October 2019

INTRODUCTION:

Lysosomal acid lipase deficiency (LAL-D) is a rare autosomal recessive disease caused by mutations in the lysosomal acid lipase (LIPA) gene and characterized by cholesteryl ester accumulation in the liver and other body parts. Depending on the lipase enzyme residual activity level, severity of LAL-D can be low enough and patients may present with nonspecific symptoms; LAL-D can then be easily confused with more common causes of hepatic steatosis such as nonalcoholic fatty liver disease leading to underdiagnosis or even misdiagnosis well into adulthood. Here we present two cases of patients whose diagnoses of LAL-D were delayed for several years due to initial misdiagnosis.

CASE DESCRIPTION/METHODS:

A 59-year-old male, initially presented in childhood with hepatomegaly and elevated liver enzymes and was diagnosed with type 3 glycogen storage disease due to findings of liver biopsy done in 1975. He presented to our practice with worsening fatigue, muscle wasting and abdominal distension despite being on cornstarch therapy. His labs showed ALT of 75 U/L (0-44), AST of 73 U/L (0-40), and LDL cholesterol of 302 mg/dL (<110). LAL activity was low at 4 pmol/h/punch and liver biopsy showed diffuse microvesicular steatosis and foamy macrophages with stage 2 fibrosis confirming the diagnosis of LAL-D. His diagnosis was missed for at least 44 years from his initial liver biopsy. The second patient, a 14-year-old male, was not diagnosed for 8 years after repeatedly presenting to the emergency room with abdominal pain, diarrhea, and mild elevation in ALT for years. He required a cholecystectomy at the age of 13 years and the surgeon noted an “orange” liver. A biopsy showed diffuse microvesicular steatosis with stage 3 fibrosis suggestive of lipid storage disorder. His labs at presentation to our clinic were: ALT of 225, AST of 111, LDL cholesterol at 192. Genetic testing confirmed the diagnosis of LAL-D (E8SJM homozygous) and he was started on sebelipase alfa enzyme replacement with normalization of ALT and LDL cholesterol.

DISCUSSION:

Gastroenterologists should suspect the diagnosis of LAL-D in any patient presenting with hepatomegaly, fatty infiltration of the liver and elevated LDL. Prompt diagnosis of LAL-D could have helped decrease morbidity and improve these patients' quality of life by initiating sebelipase alfa enzyme replacement therapy to reduce the known long-term complications of advanced liver fibrosis such as cirrhosis and liver failure, and the many effects of progressive atherosclerosis.
2443 Dealing With DILI: When the Diagnosis Is Not Always Clear Cut
Authors: Hashmi Irma , Hsu Andrew , Shor Julia , Devuni Deepika
Published: October 2019

INTRODUCTION:

Drug induced liver injury (DILI) is a rare, mostly reversible cause of liver failure (ALF); however, in rare cases DILI can unmask or trigger underlying chronic liver disease. We present a case of DILI with two novel findings: one, the patient consumed tea extract not yet cited on the LiverTox registry, and two, the patient's DILI likely triggered autoimmune hepatitis (AIH), an occurrence noted only a few times in the literature.

CASE DESCRIPTION/METHODS:

Patient is a 43-year-old female with no medical history who presented with acute onset jaundice and dark urine. She endorsed a “tea cleanse” which included dandelion roots, milk thistle and barks one month prior to admission. Her jaundice persisted despite cessation of the herbal tea intake. Liver function tests (LFTs) showed AST 2553, ALT 3022, alkaline phosphatase 135, total bilirubin 23.8, direct bilirubin 15.5, INR 1.8 and MELD 25. MRCP was consistent with acute hepatitis. ALF workup was negative except for elevated IgG1 and IgG4. Liver biopsy revealed panlobular mixed inflammatory cell infiltrate and necrosis but absent fibrosis, concerning for DILI, viral infection or AIH. Patient was managed supportively for DILI with downtrending LFTs. However, her LFTs worsened with total bilirubin 20 and INR 2.1. She developed ascites and lower extremity edema. The patient received corticosteroids for AIH despite negative ANA, anti-smooth muscle and anti-LKM antibodies with marked improvement in synthetic liver function.

DISCUSSION:

Although acetaminophen is the most common offending drug associated with DILI (40-50% of ALF cases annually), herbal or dietary supplements are not an uncommon cause, seen in 11-12% of cases per year. Accurate diagnosis of DILI can be made with clinical history, exclusion of other etiologies of ALF on laboratory evaluation, and with liver biopsy. The database LiverTox documents etiologies of DILI. “Be Well Teas,” consumed by our patient, yields no results but should be included to prevent future DILI cases. Further, despite biopsy consistent with DILI, rebound of total bilirubin and INR in our patient demanded further investigation. Likely the patient had pre-existent AIH triggered by DILI given her improvement with immunosuppression, cited previously in only in a few case reports. Concurrent, underlying AIH should be considered with relapse of liver dysfunction despite cessation of the inciting drug.
2445 All Liver, No Lungs: An Unusual Presentation of Sarcoidosis
Authors: Gurm Hashroop , Jamorabo Daniel S. , Renelus Benjamin D. , Mohanty Smruti R.
Published: October 2019

INTRODUCTION:

Sarcoidosis primarily isolated to the liver—hepatic sarcoidosis (HS)—is rare and typically presents with non-specific gastrointestinal (GI) symptoms, abnormal liver enzyme levels, and radiological findings. We present a patient whom we determined to have HS.

CASE DESCRIPTION/METHODS:

A 33-year-old woman without significant past medical history presented with 3 months of worsening right upper quadrant pain and 35-40 lb weight loss. She had no other specific complaints. Her exam was notable for epigastric and RUQ tenderness. Her liver was palpable 4 cm below the costal margin. Lab results included Hgb 12.5 g/dL with MCV 79, AST 60, ALT 69, AP 629, and GGT 773. CT scan showed diffuse cyst-like lesions throughout the liver and spleen with peri-portal lymphadenopathy (Figure 1). The chest X-ray was unrevealing. Abdominal ultrasound showed hepatomegaly to 16.1 cm, splenomegaly to 13.1 cm, and periportal and peripancreatic lymphadenopathy. Her viral hepatitis workup, Chromogranin A, CEA, CA-19-9, CA-125, and AFP levels were unremarkable. Autoimmune hepatitis workup was negative. Her angiotensin converting enzyme (ACE) was elevated to 261 IU/L. EGD and colonoscopy were unremarkable, but we took random biopsies. Liver biopsy showed confluent, non-caseating epithelioid granulomas. Biliary destruction with lymphocytic cholangitis was noted (Figure 2). Our gastric biopsies showed non-necrotizing granulomas. We diagnosed her with HS without clinically or radiographically evident lung involvement. Since her symptoms later resolved, we opted against starting steroids and we referred her to the rheumatology and pulmonology services.

DISCUSSION:

Lung involvement is seen in 90% of sarcoidosis patients, usually in the upper lobes, hilar and mediastinal lymph nodes. The diagnostic criteria for sarcoidosis include clinical and radiographic signs, non-caseating granulomas, and exclusion of other diseases. Elevated ACE levels and non-caseating granulomas are not specific to sarcoidosis and can also be seen in primary biliary cholangitis. What clinched our diagnosis was the gastric biopsies that also showed granulomas. Those with asymptomatic liver disease can be monitored until liver enzymes normalize. Hepatomegaly does not necessitate medical treatment, although patients with weight loss and lymphadenopathy should have prednisone, generally for 12 months. Ursodeoxycholic acid (UDCA) can be given for pruritus and liver enzyme elevations. In advanced HS cases with cirrhosis, liver transplantation is the only recourse.
2446 Idiopathic Non-Cirrhotic Portal Hypertension: A Teaching Case
Authors: Gurm Hashroop , Jamorabo Daniel S. , Renelus Benjamin D. , Guss Debra , Mohanty Smruti R.
Published: October 2019

INTRODUCTION:

Idiopathic non-cirrhotic portal hypertension (INCPH) is characterized by portal hypertension without cirrhosis in setting of patent hepatic and portal veins, preserved hepatic synthetic function, and no established etiology. We present a case of idiopathic non-cirrhotic portal hypertension.

CASE DESCRIPTION/METHODS:

A 25-year-old Hispanic man presented with elevated liver enzymes. He was asymptomatic and not taking any medications. His family history was non-contributory. His BMI was 23 and he rarely drank alcohol. His examination was unremarkable. Lab data showed leukopenia to 2700, thrombocytopenia to 96,000, normal INR, AST 80, ALT 114, and alkaline phosphatase (AP) 128. His Alpha 1- Antitrypsin (A1AT), alpha-fetoprotein (AFP), and serum IgG were all normal. The viral hepatitis and HIV serologies were negative. His ANA, ASMA, AMA, anti-LKM titers were also negative. An abdominal sonogram showed liver 14.5 cm and splenomegaly to 14.8 cm; no ascites was seen. A Fibroscan showed F3-F4 consistent with advanced liver fibrosis, but our liver biopsy had <5% fatty change, negative iron stain, and overall normal histology. This was confirmed by a second outside pathologist. Testing for other diagnoses including Budd-Chiari, Epstein-Barr virus, cytomegalovirus, and Schistosomiasis returned negative.

DISCUSSION:

Non-cirrhotic portal hypertension can present with lab, imaging, and endoscopic findings suggestive of cirrhosis, yet liver biopsy shows normal histology. Our patient underwent extensive workup for cirrhosis and causes of non-cirrhotic portal hypertension before we diagnosed him with INCPH. This is a pre-sinusoidal entity that involves: 1) presence of portal hypertension, 2) absence of cirrhosis or advanced liver disease, and 3) lack of hepatic or portal vein thrombosis. Unlike in cirrhosis, liver failure, ascites, and hepatic encephalopathy are not typically present. Patients have preserved liver function and often normal LFTs, but can have splenomegaly (95%), hepatomegaly (50%), varices, and thrombocytopenia. The most common complication in these patients is variceal bleeding. Thus, primary and secondary prevention of esophageal bleeding via non-selective beta blockers and endoscopic therapy is beneficial. Of note, there is no role for screening patients for hepatocellular carcinoma (HCC) in INCPH since the absence of cirrhosis renders the HCC risk very low. INCPH is important to consider in patients with no clear cause for portal hypertension or LFT abnormalities.
2451 Living Donation: A Liver Saved or a Liver Compromised
Authors: Garkusha Larisa , Ho Tai Hang , Kalia Harmit
Published: October 2019

INTRODUCTION:

When compared to deceased donor liver transplants (DDLT), living donor liver transplants (LDLT) have been associated with longer operative times, higher rates of biliary and vascular complications, and higher rates of small-for-size syndrome (SFSS). We present a case in which multiple complications and related diagnostic dilemmas are manifested over the course of several months following LDLT.

CASE DESCRIPTION/METHODS:

A 55 year old woman with decompensated alcohol-related cirrhosis with MELD of 24 was admitted and underwent living donor liver transplantation. Donor anatomy limited R lobe hepatectomy. Intraoperatively, portocaval shunt was left intact due to concern for small graft size (graft to recipient weight ratio was 0.7) as left lobe graft appeared congested post-anastomosis. Postoperatively, her transaminases trended down slowly, but she developed a worsening direct hyperbilirubinemia, peaking at Tbili 16.2 on POD #9. She was subsequently taken back to the OR for portocaval shunt closure and liver biopsy which was complicated by large hematoma. Following closure of portocaval shunt, her total and direct bilirubin continued to fluctuate but stabilized at Tbili 5.3 at which point she was discharged. Her biopsy was consistent with moderate preservation injury. Following discharge, she continued to require periodic paracentesis and thoracentesis for her refractory ascites. The patient's postoperative course was further complicated by persistent cholestatic liver tests concerning for bile cast syndrome and an admission for cholangitis two months post-op. She had multiple ERCPs showing biliary anastomotic strictures requiring stent placement. She continued to have persistent cholestatic labs despite these interventions and subsequently underwent a percutaneous liver biopsy. A venogram done at time of biopsy was revealing for severe short segment narrowing of the infrahepatic IVC s/p stent placement. Her repeat liver biopsy was suggestive of acute cell mediated rejection. Following pulsed steroid therapy, her liver tests improved.

DISCUSSION:

This case demonstrates the complications that are more likely to arise in LDLT compared to DDLT. Despite this, LDLT outcomes remain superior when compared to those who received DDLT; however this observation is complicated by selection bias in that younger, less critically ill patients with lower MELD scores are usually chosen for LDLT.