bluuebunny/crossref_metadata_2025 huggingface dataset:
2231 Spontaneous Fungal Peritonitis- A Rare Complication of Ascites Secondary to Right Heart Failure: A Case Report
Authors: Siddiqui Salaah , Iqbal Arshad Muhammad , Patel Dharti , Mubarik Ateeq , Jupalli Aamani , Zafar Fahad , Muddassir Salman
Published: October 2019

INTRODUCTION:

Spontaneous fungal peritonitis is an infection which occurs in patients with liver cirrhosis.Spontaneous fungal peritonitis is uncommon in a patient with cardiac ascites because of high protein content and low risk of infections.We present the second known case of SFP in a patient with cardiogenic ascites.

CASE DESCRIPTION/METHODS:

52-year-old female with past medical history of COPD was admitted to the hospital with a two-week history of abdominal pain and dyspnea.Labratory findings showed WBC count of 7,900/mm3, ANC of 6936/mm3, hemoglobin 15.8, platelet count 406 × 103/mm3, creatinine 0.9mg/dL, total bilirubin 3.40 mg/dL, direct bilirubin 2.30 mg/dL, ammonia of 51. Initial imaging of CT abdomen and pelvis showed bilateral pleural effusions, moderate ascites, and liver cirrhosis. ECHO showed left ventricular ejection fraction of 55%, dilated right ventricle, moderate tricuspid regurgitation with right ventricular systolic pressure of 76 mm Hg. Diagnostic and therapeutic ultrasound guided paracentesis was done and 400 mL of fluid was removed and sent for analysis and culture. Patient was empirically started on ceftriaxone for possible spontaneous bacterial peritonitis. Ascitic fluid analysis showed hazy fluid with WBC count of 23,000, 92% of polymorphic cells and 8% of mononuclear cells. Preliminary cultures from ascitic fluid grew Candida glabrata, and patient was started on caspofungin, and diagnosis of spontaneous fungal peritonitis was made. The patient gradually responded to antifungals.

DISCUSSION:

Cardiac cirrhosis is defined by signs and symptoms of chronic liver disease along with history of heart failure. Ascites due to heart failure is considered low risk for infections because of the high protein content in the ascitic fluid. Ascitic fluid retrieved by diagnostic paracentesis should be analyzed for lactate dehydrogenase, cytology, total cell count and differential, total protein, albumin, bacterial and fungal culture sensitivities. Along with bacterial gram stain and culture sensitivity, fungal stain and culture sensitivities are necessary due to an increasing prevalence of SFP in patients with cirrhosis. Common species causing SFP is Candida albicans, and other causative fungal agents being Candida glabrata, Candida krusei. Risk factors for SFP include elevated Child-Pugh and MELD scores, use of prophylactic antibiotics for SBP, low ascitic fluid protein (< 1 g/dL), hepatorenal syndrome. Treatment with echinocandins is recommended as soon as the diagnosis is made.
2234 Sorafenib-Induced Nephrotic Syndrome in Recurrent Post-Transplant Hepatocellular Carcinoma
Authors: Kumar Shiva
Published: October 2019

INTRODUCTION:

An oral antiangiogenic tyrosine kinase inhibitor (TKI), Sorafenib (Nexavar®; Bayer Healthcare, Whippany, NJ) was the first systemic agent to be approved for treatment of unresectable hepatocellular carcinoma (HCC) in 2007 and remains an important first line treatment option. Common treatment related side-effects include diarrhea, fatigue, hand-foot skin reaction and hypertension. Limited data exists regarding safety and efficacy in post-transplant (LT) recurrent HCC. Sorafenib-induced nephrotoxicity is rare and has never been previously reported in patients with HCC or post LT. We report a case of Sorafenib-induced nephrotic syndrome in a patient with recurrent HCC post-LT, which promptly resolved with drug discontinuation.

CASE DESCRIPTION/METHODS:

A 64-year-old male was noted to have evidence of multi-focal recurrent HCC on surveillance imaging two years post-LT. He was treated with loco-regional and systemic therapy. At baseline, he had mild albuminuria from diabetic nephropathy. He developed increasing pedal edema and weight gain 4 weeks after starting Sorafenib. Repeat testing revealed marked increase in proteinuria (6 gm/24 hr), which continued to worsen (8.3 gm/24 hr in 6 weeks). Kidney biopsy revealed diffuse, severe podocyte foot process effacement with segmental sclerosing glomerulopathy (Figure 1). Sorafenib induced nephrotic syndrome was diagnosed based on temporal relationship to drug initiation and biopsy findings. Drug discontinuation resulted in prompt improvement, with return of proteinuria to baseline 16 weeks later.

DISCUSSION:

Nephrotic syndrome is an infrequently reported side effect of TKI such as sorafenib and its pathophysiologic mechanism remains unclear. Sorafenib inhibits vascular endothelial growth factor (VEGF) receptors and platelet-derived growth factor receptor. VEGF, produced by podocytes, plays an important role in development and maintenance of glomerular endothelium and its inhibition may help explain the development of podocyte and endothelial cell damage and consequent alterations in glomerular vascular permeability. All reported cases of TKI induced nephrotic syndrome have occurred in patients with advanced renal cell carcinoma. This is the first report of nephrotoxicity due to podocyte damage induced by Sorafenib treatment in HCC and in the post-transplant setting. All patients starting Sorafenib should undergo baseline urinalysis with periodic reassessment thereafter, since medication induced nephrotic syndrome appears to be reversible if detected early.
2238 An Unusual Case of Direct Acting Antiviral Potentiating Angioedema
Authors: Lambert Karissa , Hamed Ahmed Said , Khalid Hamza
Published: October 2019

INTRODUCTION:

Hepatitis C Virus (HCV) is a leading cause of chronic liver disease and the most common reason for liver transplantation in the United States (US). The aim of direct acting antiviral (DAA) agents is to reduce progression to cirrhosis. There are newer well tolerated all-oral DAA agents, such as glecaprevir and pibrentasvir (GLE/PIB). We report an unreported adverse event in a treatment naive chronic hepatitis C genotype 1a patient: GLE/PIB induced angioedema.

CASE DESCRIPTION/METHODS:

A 63-year-old African American Male with a history of chronic hepatitis C infection (genotype 1a) without cirrhosis and hypertension on chronic lisinopril presented to the Emergency Room (ER) with acute onset facial and lip edema. Patient denied known environmental, food, or medication allergies. He denied unusual food intake. He recently started GLE/PIB. Vital signs were unremarkable. On physical examination, there was significant facial and lip swelling. Given the timing of symptoms, the side effect was suspected to be secondary to GLE/PIB. At this time, it was decided to stop the DAA and he was treated with steroids and antihistamine agents. At follow-up, the facial and lip edema had completely resolved. The HCV RNA level was obtained and surprisingly undetectable after only 4 days of therapy with a baseline of 5,080,000 IU/mL. However, at 12 week follow-up, he was again positive.

DISCUSSION:

Although GLE/PIB has a relatively good safety profile, angioedema is a potentially life threatening side effect that has not been reported as commonly. The underlying mechanism is not known, however it was hypothesized that co-administration with an Angiotensin Converting Enzyme (ACE) inhibitor may be related. We would recommend avoiding co-administration to prevent the rare but significant side effect, eliminating a potentially curative treatment regimen and the financial aspects of wasted expensive medication and expenses associated with hospitalization.
2250 An Unusual Case of Elevated AFP in a Patient With Chronic Hepatitis B
Authors: Shah Pir Ahmad , Onken Allison , Campoverde Reyes Karen J. , Maqsood Muhammad Haisum , Ishtiaq Rizwan , Herskovits Adrianna , Lau Daryl T.
Published: October 2019

INTRODUCTION:

Alpha-fetoprotein (AFP) is commonly used as a screening tool for hepatocellular carcinoma (HCC) among at-risk populations. It is, however, not specific for HCC detection. In addition, the assays can generate false-positive and false-negative AFP results. This case illustrated the dilemma and uncertainties of the AFP results.

CASE DESCRIPTION/METHODS:

The patient is a 50-year-old Chinese female with stable HBeAg negative chronic hepatitis B and was antiviral treatment naïve. She was first noted to have an asymptomatic elevation of AFP to 1,156ng/ml when she had her tests performed in a new clinic. Initial abdominal MRI did not reveal focal hepatic lesion. The patient was extremely depressed and anxious about the AFP result. She had a suicide attempt and was briefly hospitalized. One month later, she was evaluated at our Liver Center and her AFP remained high at 1,028ng/ml (Roche Assay). MRI of the abdomen and pelvic reported only a 6mm hepatic hemangioma. Other causes of elevated AFP such as embryonic cancers and pregnancy were ruled out. Interestingly, her same day blood sample yielded a normal AFP value of 3ng/ml by the Wako Assay performed in Quest Diagnostic. Over the course of 36 months, her abdominal imaging remained unremarkable and ALT levels were stable. At each follow-up visit, AFP was concurrently tested by both Roche and Wako assay platforms. The discrepant results between the 2 assays persisted after complete HBV DNA suppression by antiviral therapy (Figure 1). With time, the patient gained back confidence with her health and returned to work even though the AFP dilemma continued. We further evaluated her stored samples by 3 other assay platforms (Abbott, Beckman, and Siemens) and they again yielded discordant results (Table 1). Assay interference was systematically evaluated by various local and national laboratories. Presence of heterophilic antibodies most likely explained the elevated AFP values but they could not be confirmed definitively in this case.

DISCUSSION:

There are a number of lessons to learn from this unusual case of elevated AFP. While diagnostic assays are valuable for medical practice, they have limitations. It is important to understand the potential false- positive and false-negative values of a given test. In this case, it has been very informative to apply multiple assay platforms to unmask the discordant results. That was tremendously helpful to lessen the patient’s anxiety and to prevent unnecessary medical evaluations and procedures.
2256 A Unique Case of Concurrent Langerhans Cell Histiocytosis and Autoimmune Hepatitis Complicated by a Lower Gastrointestinal Bleed
Authors: Ahmed Ahmed , Nasir Umair M. , Delle Donna Paul , Galan Mark A. , Jiang Jie-Gen , Lingiah Vivek A.
Published: October 2019

INTRODUCTION:

Autoimmune hepatitis (AIH) and Langerhans Cell Histiocytosis (LCH) are two independently rare disease processes that can have similar presentations. There have been no documented cases of concurrent AIH and LCH. We illustrate a complex case that required an interdisciplinary approach to ultimately diagnose and treat the patient. This unique presentation contributes to the medical literature of the two rare diseases.

CASE DESCRIPTION/METHODS:

20-year-old male with no significant history presented with worsening jaundice, diffuse, pruritic rash, and abdominal pain over one month. On admission, the patient’s labs showed significantly elevated liver function tests (LFTs), eosinophilia, and anemia. Exam was notable for diffuse lymphadenopathy (LAD), hepatosplenomegaly, and a diffuse, non-blanching, morbilliform rash. Interdisciplinary workup was notable for positive anti smooth muscle antibody (ASMA), and anti-neutrophilic antibody (ANA). A liver biopsy was done and showed findings of severe inflammation with interface activity, consistent with AIH. A lymph node (LN) biopsy showed findings consistent with LCH, including histiocyte clusters (HC). He was started on high dose steroids with LAD/LFT improvement; yet, his hospital stay was complicated by a gastrointestinal (GI) bleed requiring a hemicolectomy. The patient was ultimately transferred to a larger referral center where he continued to improve with steroids and was ultimately discharged with close follow up.

DISCUSSION:

LCH tends to affect skin or bones; however, LN and GI involvement are relatively uncommon. This case was notable for a LN biopsy showing HC with reniform nuclei, nuclear grooves and eosinophils with immunohistochemical stains positive for S-100, CD1a, fascin, langerin, CD45, and CD68, consistent with LCH. The resected colon showed atypical histiocyte proliferation positive for fascin, CD4, and CD68. Key findings can help support a diagnosis of AIH, such as elevated LFTs, ASMA, and ANA, as well as a liver biopsy showing inflammation with interface activity, eosinophils, plasma cells, and characteristic fibrosis, all of which were found in this patient. In either case, steroids were indicated and ultimately helped the patient clinically improve. Such a case is distinct since the LCH appeared to be a systemic process, with findings of diffuse LAD, questionable GI involvement, and no liver involvement, but rather concurrent AIH. This case was further notable for a complication of a lower GI bleed requiring a hemicolectomy.
2261 GIST or MPNST or Both? An Enigmatic Mass
Authors: Agha Yasmine Hussein , Srinivasan Sachin , Hyder Jeffrey , Salyers William , Tofteland Nathan , Ahmad Ali
Published: October 2019

INTRODUCTION:

Neurofibromatosis (NF) has been frequently associated with multiple cancers and the expansive growth of these tumors often is one of the reasons for their short life expectancy. The incidence of gastrointestinal stromal tumors (GIST) and malignant peripheral nerve sheath tumors (MPNST) in patients with NF tends to be higher than the general population. The literature describes patients with only one of these tumors. The patient presented in our report likely had both tumors concomitantly, which makes our case rare.

CASE DESCRIPTION/METHODS:

A 39-year-old male with neurofibromatosis type 2 affecting the spine presented to the ED with RUQ abdominal pain, nausea, vomiting and jaundice. Abdominal CT and MRCP showed intrahepatic bile duct dilation with obstruction of the common duct at the gallbladder level. ERCP showed a localized biliary stricture with dilation of the common hepatic, left and right hepatic, and intrahepatic ducts. A stent was placed, but brush cytology was non-diagnostic. EUS with FNA was performed, which was also non-diagnostic. Repeat MRI a month later revealed interval growth, compressing the porta hepatis. Exploratory laparotomy demonstrated a perihilar mass (5 cm × 5 cm) involving the entire CBD and part of the left lobe of the liver. A second small bowel tumor (2 cm × 2 cm) was also present. He underwent a radical resection of the peri-hilar mass, CBD, Roux-en-Y hepatico-jejunostomy to the intra-hepatic bile ducts, retroperitoneal and peri-portal lymphadenectomy, left partial hepatectomy and small bowel resection. The small bowel mass showed a GIST with typical morphology, a low mitotic rate and a conventional immunophenotype, with expression of CD117 and DOG-1, negative for epithelial membrane antigen, rare expression of SOX10 and retention of H3K27me3. The hilar mass was mitotically active, malignant-appearing spindle cell neoplasm with necrosis. Its immunophenotype was not concordant with its morphologic features since it was found to be positive for both CD117 and DOG-1, scattered expression of SOX, diagnostic for GIST. The hilar mass showed complete loss of expression of H3K27me3, an immunohistochemical finding typically associated with malignant peripheral nerve sheath tumors.

DISCUSSION:

GIST and MPNSTs are reported in patients with NF. Our patient presented with a mass that had features of both in the same tumor making the diagnosis difficult and its management uncertain. Reporting of other such cases may help establish a pattern regarding this entity.
2263 A Case of Dasatinib-Induced Liver Injury
Authors: Bertran-Rodriguez Carlos E. , Sethi Sajiv , Pagan Andrea , Arroyo Yadis , Kemmer Nyingi
Published: October 2019

INTRODUCTION:

Dasatinib is a tyrosine kinase inhibitor approved by the FDA for the treatment of chronic myeloid leukemia (CML) with resistance or intolerance to prior therapy. Elevations in serum aminotransferase levels during dasatinib therapy occurs in up to 50% of patients, but were usually mild and self-limited. Histological findings are described for certain agents. However, to our knowledge, there has been no histologic classification of Dasatinib-induced hepatotoxicity.

CASE DESCRIPTION/METHODS:

We present a case of a 32-year-old male with B-cell acute lymphocytic leukemia who was undergoing consolidative chemotherapy with dasatinib. He presented to our tertiary care center with progressive jaundice. Laboratory work-up was remarkable for a cholestatic pattern of liver enzymes. Patient reported nausea but denied abdominal pain, fevers, and vomiting. On physical exam, his abdomen was non-tender with a negative murphy’s sign, and his skin did not show evidence of rash. Laboratory testing showed initial results of aspartate aminotransferase 155, alanine aminotransferase 291, Alkaline phosphatase 1272, total bilirubin 7.7 (peaked at 11.6 during hospitalization). One month prior to presentation, a complete metabolic panel was performed and was unremarkable. A liver ultrasound was performed and showed changes consistent with hepatic steatosis. MRCP was negative for biliary obstruction and also noted hepatic steatosis. A transjugular liver biopsy was performed which demonstrated severe steatosis with marked cholestasis, along with mixed micro and macrovesicular steatosis involving greater than 90% of sampled parenchyma. There was minimal bile ductular reaction observed. Dasatinib was thought to be the most likely culprit after other etiologies were ruled out. The medication was held and time was given for liver function to recuperate. Repeat analysis in one month, including AST, ALT, and total bilirubin, demonstrated near-complete resolution of liver injury.

DISCUSSION:

We report the first histopathologic plausibility related to dasatinib in the literature. This helps elucidate the underling pathogenic mechanism for Dasatinib. Furthermore, liver biopsy may have important prognostic considerations. Analyses have shown that the degree of necrosis, fibrosis stage, microvesicular steatosis, cholangiolar cholestasis, ductular reaction, neutrophils, and portal venopathy were all associated with a higher degree of severity of drug-induced liver injury and more likely to progress to chronic form of disease.
2289 Clostridioides difficile-Associated Hemolytic Uremic Syndrome After Orthotopic Liver Transplantation
Authors: Chatila Ahmed , Merwat Shehzad N. , Bilal Mohammad , Merwat Sheharyar , Kulkarni Rupak , Mujtaba Muhammad
Published: October 2019

INTRODUCTION:

Clostridium Difficile infection (CDI) has previously been reported as a rare cause of possible Hemolytic Uremic Syndrome (HUS) in both adults and children. We present a unique case of CDI related HUS in a patient post orthotopic liver transplantation (OLT).

CASE DESCRIPTION/METHODS:

A 40-year-old African American male presented with acute decompensation of alcoholic cirrhosis. After evaluation, the patient underwent successful OLT. Post-transplant course was complicated by post-operative bleeding requiring multiple exploratory laparotomies. Patient developed new-onset acute renal failure post transplantation requiring hemodialysis and prolonged courses of antibiotics. On post op day 15, patient developed diarrhea and tested positive for CDI. Physical examination was significant for jaundice, multiple ecchymoses, and a large abdominal wound without signs of excessive erythema. Laboratory data at that time showed hemoglobin drop to 5 g/dL from 8.7 g/dL, thrombocytopenia with a drop in platelets from 282 × 103/uL to 15 × 103/uL, and blood smear consistent with presence of schistocytes raising the concern for a hemolytic process. Lactate dehydrogenase (LDH) was greater than 6450 U/L, haptoglobin was less than 6 mg/dL, and reticulocytes were elevated at 8.06%. ADAMSTS-13 resulted at 26% with a negative ADMASTS-13 antibody screen suggestive against thrombotic thrombocytopenic purpura. Serotonin release assay antibodies and heparin induced thrombocytopenia antibodies were not present. A diagnosis HUS secondary to CDI was made, and the patient was started on treatment for severe CDI with oral vancomycin, vancomycin enemas, and intravenous metronidazole. The patient also underwent emergent plasma exchange receiving 4 treatments, blood transfusions, and prednisone therapy (1 mg/kg). The patient had improvement in his hemoglobin and platelets following therapy and eventual resolution of diarrhea. His mental status as well returned to baseline. Patient’s hospital course was later complicated by aright hemicolectomy, end ileostomy secondary to toxic megacolon, and pathology was consistent with pseudomembranous colitis.His renal function did not recover and he was declared end-stage renal disease.

DISCUSSION:

CDI related HUS is exceedingly rare. The pathogenesis of CDI related HUS has not been well defined. To our knowledge, this is the first case of CDI related HUS in a patient post OLT. It is important for gastroenterologists and hepatologists to be aware that although rare, CDI is a possible cause of HUS.
2296 Hepatic Rupture Due to Subcapsular Liver Hematoma in HELLP Syndrome
Authors: Webb Alexander , Sethi Sajiv , Malespin Miguel
Published: October 2019

INTRODUCTION:

Subcapsular liver hematoma (SLH) is a rare complication that occurs in about 1 in 45,000 pregnancies almost exclusively in the setting of preeclampsia or HELLP syndrome. Subcapsular and intraparenchymal hemorrhage can lead to hepatic capsular rupture which is associated with significant maternal and fetal mortality. We present a rare case of a pregnant woman who developed HELLP syndrome complicated by ruptured subcapsular liver hematoma.

CASE DESCRIPTION/METHODS:

A 33-year-old woman at 31 weeks of pregnancy presented with generalized abdominal pain. She was hypertensive and urinalysis showed proteinuria thus the clinical scenario was concerning for preeclampsia. Development of non-reassuring fetal heart tones led to an emergent C-section with delivery of a healthy infant. Within hours she became hypotensive and somnolent requiring vasopressors and intubation. The patient developed significantly elevated liver enzymes (peak ALT 15,463 U/L and AST 36,653 U/L), hyperbilirubinemia, oliguric renal failure, and hemolysis. A CT abdomen was obtained which demonstrated hepatomegaly with an extensive subcapsular and intraparenchymal hematoma and large volume hemoperitoneum, consistent with hepatic capsular rupture. Angiography showed extravasation of contrast from a branch of the right hepatic artery, which was embolized. The hospital course was complicated by disseminated intravascular coagulation, liver and bowel necrosis due to microthrombi requiring multiple laparotomies and resections, and persistent respiratory and renal failure. Ultimately the patient expired as a result of these complications.

DISCUSSION:

SLH can lead to rupture of the Glisson capsule and subsequent intraperitoneal bleeding and hemorrhagic shock. Hemodynamically stable patients can be managed conservatively. Unstable patients require hepatic artery embolization or surgical ligation, hepatic resection, or in severe cases liver transplantation. This case demonstrates a severe episode of hepatic rupture complicated by disseminated intravascular coagulation and thrombosis leading to patient mortality.
2301 Hepatic Adenoma Misdiagnosed as Biliary Colic
Authors: Pitlick Mitchell , Stephenson Christopher
Published: October 2019

INTRODUCTION:

We present the case of a woman who presented on multiple occasions with abdominal pain attributed to biliary colic that was eventually diagnosed as a hemorrhagic hepatic adenoma. We emphasize the importance of keeping a wide differential diagnosis for a common gastrointestinal complaint, the importance of medication review, and the benefit of patient-centered care.

CASE DESCRIPTION/METHODS:

A 37 year old female presented to the emergency department with right upper quadrant pain. She had pain for the past month that was more severe and constant in the past week. She had no fevers, chills, vomiting, or diarrhea. Her only medication was ethinyl estradiol for endometriosis. Her pain had been attributed to biliary colic at prior clinic appointments. She had right upper quadrant tenderness without peritoneal signs. A complete blood count and complete metabolic panel were unremarkable. A CT scan showed a 4.9 × 3.9 × 3.9 cm hepatic mass, with subsequent MRI demonstrating intrinsic T1 hyperintense signal consistent with a hepatic adenoma with recent hemorrhage. The patient declined to stop her estrogen without further endometriosis therapy, so she underwent simultaneous laparoscopic liver wedge resection and robotic hysterectomy with bilateral salpingo-oopherectomy. She now has some minor residual pain but is doing well.

DISCUSSION:

Hepatic adenomas are uncommon liver tumors classically associated with oral contraceptive (OCP) use. The annual incidence is 1 in 1,000,000 in non OCP users, but increases to 30-40 per 1,000,000 in those with long term OCP use. The risk increases with both increasing dose and duration of therapy. OCPs not only increase the incidence of hepatic adenomas, but they tend to be larger, more numerous, and more likely to bleed in patients who take OCPs. In this case, premature closure by multiple providers led to anchoring on the diagnosis of biliary colic. The association between her use of ethinyl estradiol and the possibility of hepatic adenoma was not recognized until her adenoma had begun to bleed, which could have resulted in a catastrophic outcome. This highlights the need to keep a broad differential and perform an adequate medication review for patients that present with this common gastrointestinal complaint. Additionally, prioritizing the patient’s preference to control her endometriosis pain led to consultation with the appropriate surgical teams that ultimately led to a positive outcome for the patient.
2305 HBSAg Seroclearance and Seroconversion Among Male Filipino Americans With Chronic Hepatitis B
Authors: Fajardo Noel R.
Published: October 2019

INTRODUCTION:

Worldwide prevalence of HBV infection, defined as presence of HBSAg is estimated at 257 million, and a 2009 study on HBV prevalence in the United States is estimated to be 2.2 million individuals. The chronic effects of HBV infection include cirrhosis and liver cancer and the disease burden impacts health care resources. Therefore, in 2009, the Centers for Disease Control has recommended HBV screening for patients who were born in geographic areas that has high HBV prevalence. The Philippines has approximately 8-12% HBV infection, and Filipino Americans account for a significant number of individuals migrating to the USA. HBSAg seroclearance (loss of HBSAg) and seroconversion (development of HBSAb) is described to be rare, and to our knowledge, has not been documented among patients who reside in the United States. In this case series, we describe our experience of 5 patients with this rare event.

CASE DESCRIPTION/METHODS:

Electronic medical records from our clinic Las Vegas Gastroenterology (Clark County, Las Vegas, NV) were retrospectively reviewed with ICD 9 and 10 codes (070.30, 070.32, B16.9, B19.10, B19.11). Of these we describe 5 HBSAg positive, HBeAg negative, HBeAb positive patients: 2 patients received treatment and 3 patients were treatment naive. All of these patients were of Asian descent (Filipino American), all males, with ages 45-55 years when initially seen in clinic. The 2 patients who received treatment, both had seroclearance while only 1 had seroconversion. Both patients were treated with TDF, with average treatment duration of 8 years prior to seroclearance. The 3 patients who were treatment naive all did not meet treatment criteria on presentation, and were followed for average duration of 6 years (3-8 years). All patients had spontaneous seroclearance and seroconversion.

DISCUSSION:

Our study describes HBSAg loss in patients with chronic HBV infection, which is relatively sparsely documented among chronic HBV patients in the United States. Interestingly, these patients were all males, all Filipino Americans and majority of the patients described in this case series were treatment naive. Further studies on the effect of gender, race and ethnicity are essentially in understanding the natural history of chronic HBV infection.
2307 Liver Abscess Complicated by Invasive Klebsiella Syndrome Resulting in Endophthalmitis
Authors: Naseer Usman , Wazir Ali , Murawski Shannon , Chieng Hau C. , Medarov Boris
Published: October 2019

INTRODUCTION:

Invasive Klebsiella pneumoniae Syndrome is characterized by liver abscesses, bacteremia, and metastatic infection – particularly endogenous endophthalmitis. First described in Southeast Asia in the 1980s, this syndrome has been increasingly identified in North America and is linked to virulent strains defined by hypermucoviscosity specific capsular serotypes, especially serotype K1 and K2. Following the development of a liver abscess, bile leak is a potential complication that may benefit from endoscopic management.

CASE DESCRIPTION/METHODS:

A 68-year-old female presented with 5 days of fevers, rigors, & chills. Blood cultures grew mucoid Klebsiella pneumonia, and a large hepatic abscess was noted on abdominal imaging. Shortly after admission, she developed left-sided visual deficits, with floaters and decreased visual acuity. She was found to have endogenous endophthalmitis and received intravitreal antibiotics. A percutaneous hepatic drain was placed, and she was treated with IV ceftriaxone. At a one-week follow-up, she reported decreased drain output and right flank pain. The drain had bilious output and was concerning for bile leak. ERCP showed a contrast leak at the level of the common bile duct, and a stent was placed. Repeat cultures were sterile, with no output following stent placement, so the drain was removed. She completed 6 weeks of antibiotics, and follow up imaging demonstrated marked improvement. She underwent vitrectomy with anterior chamber washout. Despite the resolution of abscess and bacteremia, she had no recovery of vision in the affected eye. Although the Klebsiella strain was of mucoid nature, the traditional K1 hypermucoviscosity serotype was negative. Only K1 serotype was tested for in our patient.

DISCUSSION:

Klebsiella pneumoniae is known to result in highly invasive community-acquired infection in immunocompetent patients without underlying hepatobiliary or intestinal disease, particularly the K1 and K2 subtypes, which are the most virulent of the known serotypes. Their capsules essentially protect the bacteria from phagocytosis. Endophthalmitis is a common complication, with high morbidity. Liver abscesses are treated with antibiotics and percutaneous drainage. Development of bilious drain output may signal the development of a bile leak, which can be successfully managed with endoscopic stenting.
2324 Right Upper Quadrant Pain in a Young Woman Isn't Always Biliary in Origin: A Case of Fitz-Hugh–Curtis Syndrome (FHCS)
Authors: Kassim Gassan , Liu Yingheng , Smith Michael S.
Published: October 2019

INTRODUCTION:

Fitz-Hugh–Curtis syndrome (FHCS) is a rare complication of pelvic inflammatory disease (PID) causing inflammation of the liver capsule and peri-hepatic peritoneal surfaces. It usually is caused by Neisseria gonorrhoeae or Chlamydia trachomatis ascending from genital tract infections. Here we present a case of right upper quadrant (RUQ) abdominal pain initially thought to be biliary in origin, with the etiology ultimately thought to be FHCS.

CASE DESCRIPTION/METHODS:

A 24-year-old woman with no significant past medical history presented to the emergency department with RUQ abdominal pain for one week. The pain was described as sharp, constant, “pinching and pulling” in quality, worsened by coughing and deep breathing, and associated with nausea and vomiting. While she reported being sexually active, there were no genital or urinary symptoms or any history of sexually transmitted infections. Physical examination was notable for RUQ tenderness with positive Murphy’s sign. Pelvic exam was negative for cervical motion tenderness (CMT), adnexal or uterine tenderness, cervical or vaginal discharge. Labs were significant only for leukocytosis of 13.5 K/uL and an elevated total bilirubin to 1.7 mg/dL, both of which resolved within 24 hours. Abdominal ultrasound and magnetic resonance cholangiopancreatography showed no evidence of cholecystitis or gallstones. Given persistent symptoms despite improving blood work and unremarkable imaging, the leading concern was biliary colic or sludge. Diagnostic laparoscopy was performed, showing an injected peri-hepatic diaphragmatic peritoneum and focal adhesions between the liver capsule and anterior abdominal wall, suggesting FHCS. Of note, all gynecologic organs were inspected and appeared normal. Gallbladder was removed during the procedure as a precaution, and pain resolved shortly after surgery. Nucleic acid amplification test for Chlamydia trachomatis ultimately was positive, confirming the diagnosis of FHCS.

DISCUSSION:

FHCS usually presents with RUQ abdominal pain that mimics hepatobiliary etiologies, and usually is associated with symptoms of PID including pelvic/lower abdominal pain, vaginal discharge or CMT. This case of FHCS is atypical in that the patient presented only with RUQ pain in the absence of PID symptoms, which may be due to bacteria bypassing pelvic structures on the way to the liver capsule. FHCS should be considered in the differential diagnosis of RUQ abdominal pain in young women of reproductive age.
2329 Recurrent Hepatic Sarcoidosis in Post-Liver Transplant
Authors: Jogimahanti Anusha , Bourgoyne Kesler , Jogimahanti Anusha , Samant Hrishikesh , Morris James D.
Published: October 2019

INTRODUCTION:

Sarcoidosis is a systemic disease that is characterized by presence of non-caseating granulomas which can affect multiple organ systems which include lungs, eyes and skin. Involvement of the liver is common in sarcoidosis but typically patients are asymptomatic and as a result underdiagnosed. Abnormalities of liver enzymes is seen only in thirty five percent of patients. Only one percent of these patients will progress to end stage liver disease requiring orthotopic liver transplant (OLT). This is a unique case of recurrent sarcoidosis in a patient who was compliant with anti-rejection immunosuppressive medication and had a recurrent flare of sarcoidosis in the liver fou years after transplant.

CASE DESCRIPTION/METHODS:

We present a case of a 59-year-old African American lady with medical history remarkable for DM2, psoriasis, and systemic sarcoidosis s/p OLT 4 years ago for hepatic sarcoidosis presented to the emergency department for increasing abdominal pain. She is complaint with her immunosuppressive medications, which included prednisone, tacrolimus and mycophenolate. Work-up showed hypercalcemia, which prompted imaging. Imaging showed typical changes for systemic sarcoidosis consistent with retroperitoneal and pelvic adenopathy. A liver biopsy was prompted which showed both cirrhosis and granuloma formation consistent with sarcoidosis. She was discharged with a significant increase in immunosuppressive medications especially her prednisone after an appropriate response in her liver chemistries. She follows with hepatology clinic for long-term care of her liver sarcoidosis.

DISCUSSION:

This is a unique case of recurrent sarcoidosis in a patient who was compliant with medication including steroids and had a recurrent flare of sarcoidosis in the liver 4 years after OLT. According to our review of literature there has only been a handful of cases of hepatic sarcoidosis following OLT. Most of these reports involved the recurrence of sarcoidosis within 8 months and up to 5 years ,but most of them had early cessation of steroids. Recurrent hepatic sarcoidosis is a rare occurrence which likely under diagnosed. Future studies implementing protocol liver biopsies in this population could lead to the discovery of the true prevalence along with further insights into the prevention of recurrent disease long-term.
2330 Acute Leukemia Developing in a Patient With Advanced Hepatocellular Carcinoma
Authors: Ramachandran Preethi , Erdinc Burak , Morcus Rewais , Gotlieb Vladimir
Published: October 2019

INTRODUCTION:

Patients with acute myeloid leukemia and myelodysplastic syndrome frequently present with infections. However, there is not enough data to prove that infectious pathogens may act as pathogenic triggers. We present here a case of acute myeloid leukemia triggered by cytomegalovirus infection in a patient with advanced hepatocellular cancer. He was at a high risk of infections due to chronic immunosuppression secondary to malignancy. In addition, him being a Jehovah's witness further complicated his management as he refused any type of blood products.

CASE DESCRIPTION/METHODS:

75-year-old male Jehovah’s witness with progressively worsening advanced hepatocellular carcinoma presented to the emergency room with worsening hepatic encephalopathy. He also had a past medical history of cirrhosis, prostate cancer and end-stage renal disease requiring hemodialysis. His white cell count was normal on admission. However, within a week into admission, his white blood count started to progressively increase (graph 1) which after further workup revealed a diagnosis of acute myeloid leukemia. He had high titers of CMV IgM antibodies during this presentation. The patient was given hydroxyurea to suppress the white blood count, erythropoietin analogs and iron supplementation to support red blood cell production as he refused to have any blood product support. Hydroxyurea could not be continued due to the cytopenias. Due to this complicated situation of liver and renal dysfunction in addition to him being a Jehovah’s witness in the setting of aggressive leukemia did not make him a good candidate for any AML chemotherapy and a decision of comfort care was made due to his complex clinical situation.

DISCUSSION:

Chronic immune suppression either by malignancy or by infections may act as a trigger for AML/MDS development. Treating leukemia in a Jehovah’s witness becomes a challenge without the support of blood product support. Less aggressive myelosuppressive therapy and erythropoiesis optimization becomes the mainstay of treatment in these population. However, it becomes even more challenging and complex to treat two aggressive cancers in these patient population and is predictive of poor prognosis.
2334 Dapagliflozin, Diabetes, Liver Disease: With New Medications Come New Responsibilities
Authors: John Jason J. , John Elizabeth S. , Pagadala Mangesh
Published: October 2019

INTRODUCTION:

Dapagliflozin, a sodium-glucose co-transporter 2 (SGLT-2) inhibitor, is an oral hypoglycemic agent that was released in 2011. FDA approval was initially delayed due to concerns of hepatotoxicity. We present a case of drug induced liver injury (DILI) from dapagliflozin in a patient with newly diagnosed non-alcoholic fatty liver disease (NAFLD).

CASE DESCRIPTION/METHODS:

A 54-year-old Caucasian female with a history of obesity, diabetes mellitus, hyperlipidemia, and macular edema presented to the emergency room with new onset jaundice and intermittent episodes of confusion. She denied history of liver disease, recent travel, new or unusual foods, tattoos, incarcerations, previous blood transfusions, alcohol abuse, illicit substance use, or intake of herbal preparations. She started dapagliflozin one month prior for her diabetes. Her physical exam revealed scleral icterus and jaundice. Laboratory workup revealed total bilirubin 20.5, direct bilirubin 18.8, AST 228, ALT 222, alkaline phosphatase 1,145. She had a normal CBC, INR, ferritin, negative viral hepatitis panel, ASMA, AMA, and ANA. MRCP was unremarkable. Liver biopsy revealed a free hepatic venous pressure of 11 and wedged hepatic venous pressure of 14, and gradient was 3 mm Hg. Histology revealed cholestasis, several missing bile ducts, bile duct proliferation, portal edema from neutrophil related inflammation, and 35% steatosis with ballooning cells and stage 3-4 fibrosis. Dapagliflozin was stopped, and the patient's bilirubin and LFTs normalized over the next few months.

DISCUSSION:

With the incidence of type 2 diabetes mellitus projected to rise, novel medications are being introduced to treat patients. Obesity, metabolic syndrome, and diabetes are all risk factors for NAFLD, and currently the prevalence of NAFLD in this group is estimated at 75%. As patients with chronic liver disease have less hepatic reserve, physicians must identify signs of NAFLD or other chronic liver diseases prior to starting potentially hepatotoxic medications. Dapagliflozin is 1,200 times more specific for SGLT-2, but also has some inhibitory effects on SGLT-1, a cotransporter responsible for reabsorption of glucose from bile. SGLT-1 inhibition causes increased glucose saturation of bile, which can result in hepatocyte injury. It is pivotal to screen diabetics beforehand for NAFLD when considering hypoglycemic agents like dapagliflozin that may be hepatotoxic given the potential for acute liver failure as was seen in our patient.
2337 Secondary Sclerosing Cholangitis and Nodular Regenerative Hyperplasia Caused by Hereditary Spherocytosis
Authors: Shah Maulik , Salim Summar , Malek Ashraf
Published: October 2019

INTRODUCTION:

Hereditary spherocytosis is a hemolytic anemia that leads to premature cell destruction due to red cell membrane defect. It causes release of unconjugated bilirubin, and subsequent bilirubin gallstone formation occurs. Chronic recurrent choledocholithiasis can cause secondary sclerosing cholangitis (SSC). Hemolysis can lead to chronic vascular injury and portal vein thrombosis, resulting in Nodular Regenerative Hyperplasia (NRH). Here, we report a case of HS causing SSC with concurrent NRH.

CASE DESCRIPTION/METHODS:

A 37-year-old female with a history of hereditary spherocytosis with thrombocytosis presented to the office for abnormal liver function tests (LFTs). History of splenectomy and cholecystectomy in 2002. Patient was noted to have abnormal LFTs in 2009 (Figure 1). Her LFTs were routinely monitored until 2016 when she presented with epigastric pain, jaundice, and abnormal LFTs. Viral and autoimmune hepatitis serologies were negative. Iron studies were normal. Right upper quadrant (RUQ) ultrasound (US) showed mildly dilated intrahepatic ducts. Magnetic resonance cholangiopancreatography(MRCP) was performed which showed a questionable stricture in the proximal CBD and mild dilatation of the central intrahepatic duct. ERCP showed normal caliber CBD with multiple filling defects. A biliary sphincterotomy was performed and the stone was removed. ERCP performed in 2016 and 2018 revealed multiple stones in the biliary ductal system, which were removed and the CBD was dilated. Liver biopsy showed mild portal and lobular inflammation with mild portal fibrosis and changes suggestive of NRH.

DISCUSSION:

HS caused the patient's recurrent choledocholithiasis and contributed to SSC. Pathologically, SSC causes inflammation, obliterative fibrosis, stricture formation, and destruction of the biliary tree, which leads to biliary cirrhosis. Although often reversible, outcomes may not be as favorable if it is not recognized quickly, particularly in critical illness induced SSC. NRH is a liver disease characterized by many hyperplastic nodules without separation by fibrotic tissue. It is associated with any systemic disease that causes vascular injury, in this case our patient’s HS. Pathology in our case revealed only “mild” changes suggestive of NRH, so the patient hasn’t yet developed portal hypertension. There are no cases in literature that document HS as a factor contributing to either of these disease states. Early suspicion and liver biopsy are necessary for prompt diagnosis and improved outcomes.
2349 Flood Syndrome, a Rare Complication of Cirrhosis
Authors: Gil Arcelia Guerson , Rockwell Bradley , Galen Benjamin
Published: October 2019

INTRODUCTION:

“Flood syndrome,” also known as spontaneous umbilical hernia rupture, is a rare complication of end-stage liver disease and persistent ascites. We present a case of a patient who developed Flood syndrome prompting emergent surgical management.

CASE DESCRIPTION/METHODS:

A 63-year-old man with cryptogenic cirrhosis and ascites presented with worsening diffuse abdominal pain. Physical exam revealed a massively distended abdomen with generalized tenderness and a large umbilical hernia (UH) with ulceration of the overlying skin. Abdominal CT scan revealed large, loculated, abdominopelvic ascites with a large ventral hernia containing ascites (Figure 1). Despite undergoing two large-volume therapeutic paracentesis, he experienced spontaneous rupture of an ulcerated UH while attempting to defecate during this hospitalization. Likely greater than 10 L of ascitic fluid poured out leading to hypotension and prompt emergent exploratory laparotomy with repair of the defect in the abdominal wall.

DISCUSSION:

UH is seen in up to 20% of patients with cirrhosis and persistent ascites [1]. It is the result of the chronically weakened state of the abdominal wall surrounding the umbilicus in the setting of increased intra-abdominal pressure. Flood syndrome can occur when the tense pressure of ascites leads to spontaneous rupture of an umbilical hernia site with drainage of a large volume of ascitic fluid. It is a serious condition, with a mortality of over 60%. Rupture of an umbilical hernia site in Flood syndrome can be provoked by vomiting, coughing, or straining with defecation. In Flood syndrome, treatment begins with fluid resuscitation and antibiotics to prevent infection. Once stable, emergent surgical repair of the abdominal wall defect is essential. Non-surgical management is not advised and is associated with higher mortality. To prevent complications and improve mortality, these patients should be treated immediately with surgical intervention [2].