bluuebunny/crossref_metadata_2025_split huggingface dataset:
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].
2352 Herbal Teas: More Harmful Than Helpful?
Authors: Gampa Anuhya , Shah Ayush N. , Shah Dhiren 
Published: October 2019

INTRODUCTION:
The objective of this case is to become aware of the increasing number of agents with the potential to cause acute liver injury, in the modern age of combined allopathic and naturopathic medicine.

CASE DESCRIPTION/METHODS:
A 45 year old woman, recently emigrated from Honduras, presented with three weeks of epigastric pain and progressive jaundice. She denied high risk behaviors and endorsed NSAID use for headaches. Her exam was notable for marked scleral icterus and jaundice, but no stigmata of chronic liver disease. Labs were notable for AST 1893 IU/L, ALT 1454 IU/L, total bilirubin 16 mg/dL (direct bilirubin 12.4), ALP 209 IU/L, protein 6.4 g/dL, albumin 3.1 g/dL, INR 1.2. Urine drug screen, ethanol and acetaminophen levels were undetectable. A complete acute hepatitis viral panel and ceruloplasmin was negative except for a borderline anti smooth muscle antibody (1:40). Further workup was done including an abdominal ultrasound with dopplers showing normal gallbladder, no gallstones, and patent hepatic and portal vasculature, and an MRCP with small hepatic cysts and normal liver. No etiology was identified and her transaminases continued to rise as to AST 2230 IU/L and ALT 1895 IU/L. Liver biopsy showed periportal and cholestatic hepatitis with bridging necrosis consistent with drug induced liver injury. Further detailed history eventually yielded that she started an herbal remedy, arnica, around the same time she was developing jaundice. She was treated with N-acetylcysteine, and with continued abstinence of arnica, her liver enzymes normalized.

DISCUSSION:
There are innumerable supplements available to patients with purported health benefits, making it challenging to identify safety of use. arnica herbs are used as a topical agent to help with joint and muscle aches given its anti-inflammatory properties. Our patient had been using the herbs to create a concentrated tea. It is likely that the ingestion and systemic absorption led to her liver injury. This emphasizes the importance of thorough history taking with direct questioning, especially when diagnostic testing is unrevealing. Treatment is avoidance of the agent with expected improvement in liver enzymes.
2358 Zieve's Syndrome: A Case of an Unusually Profound Hemolysis in the Setting of Alcoholic Liver Disease
Authors: Ali Farhan , Steimle Cassandra , Kelts Andrew , Jeng Jennifer , Kamjula Pallavi , Reddy Dayakar , Livingston Wesley , Li Zhezheng , Kochik Michael , Golikov Edwin , Raborn Eric 
Published: October 2019

INTRODUCTION:
Zieve’s Syndrome is a cause of hemolytic anemia in alcoholic liver disease, thought to arise from altered erythrocyte metabolism (pyruvate kinase instability) and hyperlipidemia (lysolecithin may trigger hemolysis). It is an under recognized syndrome, and often times leads to patients undergoing extensive clinical work ups.

CASE DESCRIPTION/METHODS:
A 73-year-old male with a history of alcohol abuse presented to the emergency room for acute jaundice associated with melenic stools, and dark urine. Physical exam was notable for dry mucous membranes, scleral icterus and conjunctival pallor, No oral ulcers, rashes or petechiae were found. Initial labs included a hemoglobin of 5.5G/DL, LDH 753IU/L, and a negative Coombs test. Based on his liver function tests, his R factor was 1.6, consistent with a cholestatic pattern of injury. Through his hospital course he had a rising alkaline phosphatase as high as 226 Units/L, and total bilirubin of 11.5 MG/DL. Triglycerides were elevated (289MG/DL) compared to prior lipid study. Abdominal ultrasound revealed an echogenic liver consistent with fatty infiltration. Lab workup was negative for HFE (hemochromatosis) mutations, autoimmune hepatitis, HIV, CMV, HCV, and EBV. Prior to coming to the hospital, the patient spent time out in the woods. Given risk of tick exposure, in the setting of hemolytic anemia, he was evaluated for tick borne illness. He was started on Doxycycline, and Atovaquone to empirically cover Lyme and Babesia, as well as Piperacillin-Tazobactam while blood and urine cultures were pending. Parasite studies and blood/urine cultures returned negative. His hemolytic anemia was refractory to 8U of packed red blood cells, and 1U of fresh frozen plasma. Eventually, the patient was diagnosed with Zieve's Syndrome.

DISCUSSION:
Zieve’s Syndrome is characterized by a triad of alcoholic steatohepatitis, jaundice, and transient hyperlipidemia. The challenging aspect of this case lies in the fact that these findings are often seen under the constellation of other diseases, leading patients to unnecessary tests and studies. Immediate management of the syndrome is comprised of supportive care, while definitive treatment is alcohol cessation.
2364 Case Studies: Hepatitis B Surface Antigen Positive or DNA Detectable After Vaccination?
Authors: Xu Binghong , Houng Mindy S. , Wang Su 
Published: October 2019

INTRODUCTION:
After 1980s, hepatitis B (Hep B) vaccine reduced 80% of the incidence of acute infection. It has not been necessary to check blood work after immunization, but we happened to have two patients who had blood work after Hep B vaccination.

CASE DESCRIPTION/METHODS:
1)A 59 years old female, found to be HBsAg positive in the ED when she had GERD and presented with atypical chest pain. In her hepatitis linkage-to-care visit, we found she had her first dose of Hep B vaccine 3 days before her ED visit. We rechecked the blood work one month after her vaccine, and it showed low surface antibody titer and no antigen at all. 2)A 62 years old male was found to have positive isolated total core antibody (anti-HBc) on 4/27/2015 from his physical checkup. Other history included type II DM and insomnia. He received a Hep B booster on 5/21 and repeated blood work on 6/25 found still isolated anti-HBc. Further blood work in August, including Hep B e Ag, e Ab and viral load, verified this patient didn't have an active infection. He finished the three-dose vaccine series. Incidental Hep B panel drawn on 12/23 found detectable DNA (< 20) with negative HBsAg and positive anti-HBs.

DISCUSSION:
The phenomenon of HBsAg being positive after vaccination is reported in several studies; however, most of them are hemodialysis patients. The surface Ag could be positive for 6 weeks after vaccination. The incidence of positive isolated anti-HBc was found higher in endemic countries than in low prevalence areas. Since the second patient is from China, a hyperendemic area, the possible interpretation of his blood work is either, after many years of recovering from chronic Hep B, his anti-HBs waned down or his HBsAg fell to undetected with his chronic infection. The last possibility will be antigen mutations inducing false-negative HBsAgresults. Studies found HBV DNA detected in anti-HBc positive patients, and lower prevalence in combined anti-HBc and anti-HBs positive patients than in isolated anti-HBc positive patients. The covalently closed circular DNA (cccDNA) in the nuclei of infected hepatocytes persist in chronic infection. When the HBV-infected hepatocytes are destroyed by the immune system, the cccDNA is released. This explains how HBV DNA can be detected or not in patients with isolated positive anti-HBc and how the levels could change in the same patient. In summary, HBsAg can be positive for a short period after vaccination and when infected from HBV, cccDNA stays persistent in the hepatocytes.