bluuebunny/crossref_metadata_2025_split huggingface dataset:
2274 Splenic Infarction in Alcoholic Hepatitis
Authors: Aijazi Muaz , Gill Brendan , Kalaria Arjun , Ahuja Ashish 
Published: October 2019

INTRODUCTION:
Case reports can be found in the literature describing elderly cirrhotic patients developing splenic infarctions (1,2). Herein, we describe a relatively young female who developed splenic infarction likely secondary to alcoholic hepatitis (AH).

CASE DESCRIPTION/METHODS:
A 40-year-old Caucasian female with past medical history of alcohol abuse and irritable bowel syndrome presented with jaundice, nausea, and vomiting. Laboratory showed ALT 54, AST 246, alkaline phosphatase 589, total bilirubin 14.0, INR 1.9, and ethanol level 240. WBC count was elevated to 20,900 with a 92% neutrophilic predominance. Leukocytosis was initially thought to be secondary to UTI as she also presented with urinary symptoms and urinalysis concerning for UTI. After a 5 day course of IV ceftriaxone, her WBC count decreased only to 18,400. Her abdominal exam began to worsen. RUQ ultrasound showed hepatic steatosis. CT scan with contrast showed moderate hepatomegaly and borderline splenomegaly with mild ascites. Abdominal pain, leukocytosis, and total bilirubin continued to worsen. Because of her severe abdominal pain and WBC count >50,000 despite a 7-day course of meropenem, contrast CT scan was repeated. At this time, lesions in the spleen concerning for infarction were seen. From here on, she was managed conservatively and her condition improved until discharge.

DISCUSSION:
Splenic function has been shown to decrease in patients with AH, but the exact pathophysiology is unclear (3). Splenic infarctions have been described in the literature in cirrhotic patients with co-morbidities (e.g. primary biliary cirrhosis), but to the authors’ knowledge there is not much mention in the literature of spontaneous splenic infarction in AH. In the right clinical context, physicians should consider splenic infarction in AH patients, especially in those with persistent leukocytosis which could be classified as a leukemoid reaction. Our report shows splenic vasculature infarction may be worth investigating in the future as an explanation for hyposplenia in AH.
2280 Propafenone-Induced Hepatocellular and Cholestatic Injury
Authors: Rivas Cynthia , Castano Juan J. , Pozo Leonardo , Huang Rex , Rapoport Grigoriy , Zamir Asif 
Published: October 2019

INTRODUCTION:
Propafenone, an antiarrhythmic medication, decreases cardiac automaticity and increases refractory periods slowing cardiac conduction. Propafenone-induced hepatic injury is rare with only eight reported cases in the literature. We report a case of propafenone-induced hepatic injury in a patient presenting with painless jaundice.

CASE DESCRIPTION/METHODS:
A 60-year-old woman presented with sudden onset jaundice and a one week history of 10-pound weight loss, right upper quadrant abdominal pain, nausea and vomiting. Two months prior to presentation, she was diagnosed with atrial fibrillation and was begun on Propafenone 325 mg oral twice daily. She had no history of alcohol, drugs, or herbal medication use. Physical exam was significant only for generalized jaundice. On admission, laboratory studies were significant for AST 304 IU/L, ALT 601 IU/L, ALP 334 IU/L, total bilirubin 7.9 mg/dL, and direct bilirubin 5.4 mg/dL.Abdominal ultrasound revealed fatty liver without biliary obstruction. CT abdomen was unremarkable for hepatic or biliary pathology. Viral serologic markers and acetaminophen levels were negative as well as workup for Autoimmune hepatitis, and Primary biliary cholangitis. Liver biopsy revealed steatohepatitis with superimposed ductular and centrilobular cholestasis suggestive of drug-induced injury.The patient was begun on N-Acetylcysteine and Propafenone was discontinued. Several days after withdrawal of Propafenone, liver function tests began to improve slowly. The patient was discharged home and one month later, transaminases returned to normal and jaundice had resolved.

DISCUSSION:
Propafenone is a Class 1C antiarrhythmic indicated for symptomatic atrial fibrillation without structural abnormalities. The mechanism for hepatic injury is unknown. Like in our patient, the injury is self-limited and has not been linked to acute liver failure, chronic liver injury, or vanishing bile duct syndrome. Once identified as the cause, the medication should be immediately stopped and never prescribed because re-exposure is associated with recurrence of symptoms. As in other reports, our patient’s liver injury occurred within a 2-6-week period after exposure and, resolved 3-5 weeks after cessation of the offending medication. Propafenone-induced hepatic injury should be suspected in a patient presenting with hepatocellular and cholestatic injury, especially in a patient recently begun on that medication after exclusion of other causes of abnormal liver function.
2283 Herpes Simplex Virus Hepatitis in Immunocompetent Hosts - A Case Series Demonstrating the Importance of Early Recognition and Empiric Antiviral Therapy
Authors: Douglas Scott R. , Miller Jennifer , Henry Zachary 
Published: October 2019

INTRODUCTION:
Herpes simplex virus (HSV) is a highly prevalent virus but only rarely results in fulminant hepatitis. The prevalence of HSV1 and HSV2 are reportedly 53.9% and 15.7% in the general United States population. However, despite the high prevalence it is only responsible for 1% of all acute liver failure. Immunocompetent individuals represent only 24% of these cases. We present three cases of HSV hepatitis in the presumed immunocompetent host.

CASE DESCRIPTION/METHODS:
Case 1: A 70 y.o male with type II diabetes presented with high fevers. His transaminases peaked at AST 973 and ALT 1183. His INR and T Bili both peaked at 1.4. Due to the anicteric nature of his hepatitis, he was started on empiric acyclovir. He developed nonspecific encephalopathy, later attributed to hospital acquired delirium. Cerebrospinal fluid was negative for HSV. His HSV serum PCR was positive for HSV1 on day 8. He later developed painful oropharyngeal ulcers. His liver enzymes and mental status would normalize. Case 2: A 19 y.o female with osteogenesis imperfecta and lupus (treatment naive) presented after a sexual assault with fevers. She developed right upper quadrant pain and a pustular rash on upper extremities. Her liver enzymes peaked at AST 2,480 and ALT 824 on day five of admission. Her INR and total bilirubin peaked at 2.8 and 1.5, respectively. Due to the anicteric nature of her hepatitis, she was empirically treated with acyclovir. Her serum HSV PCR returned positive for HSV2 on day twenty. Liver enzymes improved and she was discharged on PO valganciclovir. Case 3: A 21 y.o female with history of chlamydial infection presented with a diffuse urticarial rash, fever and right upper quadrant pain. Her AST was 2389 and ALT 1896. Total bilirubin peaked at 1.3. Due to the anicteric nature of her hepatitis, she was empirically treated with acyclovir. PCR of an oral lesion was positive for HSV2 and her serum PCR was positive for HSV2. She recovered completely.

DISCUSSION:
Despite low occurrence rates, our cases demonstrate HSV hepatitis must be kept in the differential diagnosis of hepatitis in the immunocompetent host. Progression to acute liver failure (ALF) occurs in 74% of HSV hepatitis cases. ALF from HSV has a mortality rate of 90%. Therefore, high clinical suspicion must be maintained for favorable outcomes. Fortunately, our patients had resolution of their HSV hepatitis due to early recognition of anicteric hepatitis and treatment with empiric acyclovir.
2284 Coagulopathy and Jaundice in Alagille Syndrome, Not All That Glitters Is Gold
Authors: Altamimi Barakat Aburajab , Vancura James , Sanchez Antonio J. 
Published: October 2019

INTRODUCTION:
Alagille-Watson syndrome is an autosomal dominant disease associated with cardiac, hepatic and kidney abnormalities. Estimated prevalence of disease is 1 in 100,000 live births. Symptoms and severity of Alagille syndrome range from mild to cases requiring organ transplantation.

CASE DESCRIPTION/METHODS:
32-year-old male patient with history of Alagille Syndrome s/p biliary diversion and right hemicolectomy at age 5 who was lost to follow up after he turned 18. Patient known to have Allele 1: 551 G >A (R184H), Allele 2 with no mutation. Patient was admitted to the hospital with one month of worsening jaundice, fever and two episodes of non-bloody vomiting. Pertinent history included alcohol use 6-8 beers over the weekend for several years. Patient had baseline bilirubin of 2.4 and had elevated bilirubin for most of his life. On admission his AST/ALT were 209/143 U/L, bilirubin 18.6 mg/dl, direct 13.6 mg/dl; total bilirubin peaked at 24.4 mg/dl , INR was 10 and PTT was 51s. CBC and chemistries were normal. After vitamin K administration, INR corrected to 1.5. Patient received antibiotics and had negative blood and urine cultures. US done showed splenomegaly. MRCP showed a cirrhotic liver and nondilated bile ducts. Liver biopsy showed biliary cirrhosis, absent interlobular bile ducts, bile stasis and prominent Mallory Hyaline. Patient bilirubin levels improved and patient is currently undergoing liver transplant workup.

DISCUSSION:
90% of patients with Alagille syndrome have bile duct paucity. This usually presents as cholestasis in the first four months of life, in our patient this issue improved with the biliary diversion. However this was complicated by low absorption of fat soluble vitamins. It is likely that infection and alcohol use contributed to his worsening liver disease. Pulmonic stenosis is the most common cardiac anomaly and most patients have classical syndromic facial features and butterfly vertebrae as did our patient. Posterior embryotoxon is also common. Conclusion: While 15% of nontransplanted patients with Alagille disease progress to cirrhosis, this case highlights that common liver diseases are still common in unique patient populations and should always be considered. Our patient alcohol intake contributed to his liver disease, his elevated INR was related to vitamin K deficiency rather than synthetic liver dysfunction, and marked improvement in his liver enzymes followed with alcohol cessation.
2285 HSV Hepatitis in an Immune-Competent Patient
Authors: Francis Pilin D. , Tosounian Shannon L. , Kalman Richard 
Published: October 2019

INTRODUCTION:
A rare complication of herpes simplex virus (HSV) infection is hepatitis, especially in an immunocompetent host. It is important to recognize this diagnosis, as HSV hepatitis is potentially fatal if untreated due to the risk of acute liver failure. We present a case of HSV hepatitis in an immunocompetent patient with a benign clinical course.

CASE DESCRIPTION/METHODS:
A 58-year-old female with history of diabetes mellitus, breast cancer with distant history of chemotherapy, presented to the emergency department for one week history of right upper quadrant pain and fevers at home. On presentation she was febrile with a temperature of 39.3 degrees Celcius, blood pressure 118/72 mmHg, heart rate 100 bpm. Physical exam findings included tenderness to palpation of upper quadrants, flanks, and suprapubic area. Laboratory findings revealed alanine aminotransferase (ALT) 139 IU/L, aspartate aminotransferase (AST) 172 IU/L, and alkaline phosphatase 73 IU/L. She was negative for Hepatitis A, B, C, HIV, and influenza. A liver ultrasound showed an enlarged liver (19.5cm) with homogeneous parenchyma and increased echogenicity consistent with hepatic steatosis. The liver contour was smooth and there was no duct dilation. Her liver tests worsened with ALT and AST peaking at 822 and 1558, respectively. Due to persistent fevers, the patient was evaluated for HSV, and was found to be HSV-2 IgM positive (titer >1:320) and HSV-2 DNA was detected. The patient was started on intravenous acyclovir. A liver biopsy showed findings of periportal and pericentral necrosis, favoring infection. An immunohistochemical stain for HSV was positive. She completed 2 weeks of acyclovir, achieving improvement in liver tests to normal range.

DISCUSSION:
HSV is a common infection, but a rare cause of hepatitis, comprising < 2% of cases and < 1% of all causes of acute liver failure. HSV hepatitis is usually described in immunocompromised hosts, pregnant women, and neonates. Presentation is vague, but most commonly with fever, abdominal pain, and only 30% with typical oral or genital lesions. Diagnostic tools are serological testing for HSV and liver biopsy. Treatment with acyclovir is effective in preventing transplant or can be life saving in 51% if initiated promptly, and thus, a high index of suspicion is warranted in patients with appropriate risk factors or with an unclear etiology of severe transaminitis. Given our immunocompetent patient’s relatively benign course, this is a unique presentation of a potentially fatal disease.
2286 Cholestatic Hepatitis as a Paraneoplastic Syndrome of Endometrial Carcinoma
Authors: Wade Francis , Odufalu Florence-Damilola , Prather Charlene , Marsicano Elizabeth 
Published: October 2019

INTRODUCTION:
Cholestatic hepatitis is a rare manifestation of both solid organ and hematologic malignancies. It has been reported to occur with ovarian cancer, but not other female genitourinary tract malignancies to our knowledge. We report a case of simultaneous cholestatic hepatitis and acute sensorimotor polyneuropathy as paraneoplastic syndromes of endometrial adenocarcinoma.

CASE DESCRIPTION/METHODS:
A 66-year-old woman with past medical history of newly diagnosed stage IIIC endometrial adenocarcinoma and cholecystectomy presented with ascending bilateral lower extremity weakness and numbness for 14 days. Physical exam revealed bilateral foot drop and distal lower extremity numbness. Patient did not have jaundice or scleral icterus. Laboratory assessment showed ALT 172 U/L (normal < 61 U/L), AST 149 U/L (normal < 40U/L), ALP 243 U/L (normal < 126 U/L), and GGT 461 U/L (normal < 85 U/L). Lumbar puncture showed CSF albuminocytologic disassociation. Further neurologic workup was consistent with an acute sensorimotor polyneuropathy, which was attributed to a paraneoplastic process of her cancer. During investigation of her polyneuropathy, transaminases and ALP continued to rise to peak values of ALT 245 U/L, AST 202 U/L, and ALP 659 U/L so workup of cholestatic hepatitis was pursued. Total bilirubin and INR remained normal. Fractionated ALP showed a majority liver fraction. Infectious, autoimmune, pharmacologic, malignant, metabolic, and drug-induced causes of cholestatic hepatitis were screened for and ruled out. Liver ultrasound with doppler was negative for acute biliary or liver pathology. CT of the chest, abdomen, and pelvis with IV contrast showed a normal liver. Additional workup for cholestatic hepatitis, including liver biopsy, was not pursued as ascending paralysis progressed to paraplegia and patient opted for hospice care. Patient was diagnosed with paraneoplastic, acute cholestatic hepatitis from endometrial adenocarcinoma.

DISCUSSION:
To our knowledge, this is the first case report of cholestatic hepatitis as a paraneoplastic syndrome of endometrial adenocarcinoma. Our patient’s liver injury may have occurred due to paraneoplastic proinflammatory state or tumor antigen-antibody cross reactivity with liver cell antigens, or a combination of both. Gastroenterologists should include paraneoplastic processes of cancer in the differential diagnosis for liver injury, especially when workup of alternative causes is unrevealing.
2288 Diabetic Hepatosclerosis Presenting as Severe Pruritis
Authors: Long Amanda , Yalamanchili Silpa , Heightchew Kimberly , Wong Patricia 
Published: October 2019

INTRODUCTION:
Diabetic hepatosclerosis (DH) is an uncommon type of hepatic injury associated with poorly controlled diabetes mellitus (DM). Unlike steatohepatitis or glycogenic hepatopathy, DH is a form of end-organ damage due to microvascular injury. This case illustrates a patient with advanced DM who presented with cholestatic liver injury and histologic findings of DH.

CASE DESCRIPTION/METHODS:
A 34 yo woman with poorly controlled type 1 DM (HgA1C 17%) complicated by diabetic nephropathy presented with one month of generalized pruritis. Symptoms were most severe after hot showers and at night. She denied rash, fevers, unusual exposures, ingestions, or new medications. On exam, she appeared well, had no visible skin lesions, no scleral icterus, and a soft abdomen without organomegaly. Labs were notable for ALP 612 U/L, ALT 205 IU/L, AST 109 IU/L, TB 0.9 mg/dL, WBC 8.3 K/uL, PLT 306 K/uL. Abdominal ultrasound revealed a normal appearing liver and biliary ducts. Ultrasound-guided liver biopsy showed perivenular and perisinusoidal fibrosis without inflammation, steatosis, or bile duct injury. These findings were consistent with DH. After one year of aggressive glycemic control, her pruritus resolved. Her HgA1C was 7.6%, ALP 313 IU/L, ALT 32 IU/L, AST 29 IU/L.

DISCUSSION:
While non-alcoholic fatty liver disease (NAFLD) and glycogenic hepatopathy are more commonly seen in patients with advanced DM, DH is a distinct form of microangiopathic liver injury characterized by dense perisinusoidal fibrosis (Figure 1), lack of steatosis (Figure 2), and basement membrane deposition of laminin and type IV collagen1,2. DH can be seen in both type 1 and type 2 DM, and rarely occurs in the absence of other microvascular complications. DH is characterized by a predominantly elevated ALP, suggesting a primary bile duct or canalicular injury. Diabetics with hypertension seem to be at increased risk for DH3. The long term consequences of DH remain unclear. In our patient, a decrease in HgA1c level resulted in normalization of AST and ALT levels, a marked reduction in ALP, and resolution of pruritus.
2290 A Unique Presentation of Severe Cholestatic Autoimmune Hepatitis With an Associated Biliary Stricture
Authors: Reinhart Evan , Garner Jessica , Jeffries Mark A. , Sing James 
Published: October 2019

INTRODUCTION:
Autoimmune hepatitis, usually associated with hepatocellular injury patterns, has rarely been described in current literature presenting with severe cholestasis. Even fewer cases have associated autoimmune hepatitis in patients with Mixed Connective Tissue Disease (MCTD). We describe a rare case report of a young male with suspected MCTD who presented with severe cholestatic autoimmune hepatitis with an associated biliary stricture.

CASE DESCRIPTION/METHODS:
A 33 yo AA male veteran with no significant PMHx presented with a 3 months of RUQ abdominal pain with associated jaundice, fatigue, and subjective fevers. On further review, he endorsed dry mouth, joint pain, and pain in his fingers in cold temperatures. He denied alcohol use, taking herbals or prescription medications. He endorsed travel to Korea. Physical examination was notable for RUQ abdominal tenderness and jaundice. Initial labs were significant for: Bilirubin: 11.6, Alk Phos: 338, AST/ALT: 1086, 116, Tylenol level: below assay, INR 1.2, platelets 213, Ferritin: 6,767, and Ceruloplasmin: 32. Acute viral hepatitis testing for A, B, C, and E were negative. HFE gene mutation was negative. Autoimmune markers included: positive ANA 1:320, positive RNP Ab, positive Smooth Muscle Ab 1:80; elevated IgG subclass 1 (2,010) and IgG subclass 2 (589). RUQ ultrasound and CT Abdomen/Pelvis showed evidence of cirrhosis. MRCP and ERCP revealed: gallbladder distension, cirrhosis of the right hepatic lobe, biliary strictures of the common hepatic and right intrahepatic bile ducts. The biliary stricture brushings were negative for malignancy. Ileocolonoscopy was unremarkable without inflammatory bowel disease. He underwent laparoscopic cholecystectomy with intraoperative liver biopsies. Interestingly, his liver biopsies revealed marked cholestasis with lymphoplasmacytic portal inflammation and minimal fibrosis (stage 1-2) despite imaging suggestive of cirrhosis. Ultimately , these findings were consistent with severe cholestatic autoimmune hepatitis with an associated biliary stricture in a patient with suspected underlying MCTD.

DISCUSSION:
Our patients demographics, travel and pattern of liver injury posed a diagnostic dilemma for diagnosing autoimmune hepatitis. After a thorough evaluation for the etiology, his liver histopathology, positive autoimmune markers, and favorable response to steroids were all compatible with the diagnosis of cholestatic autoimmune hepatitis with a biliary stricture in a patient with suspected MCTD.
2294 The Unusual Wolf in Sheep's Clothing
Authors: Khan Zarak H. , Korpole Pranay R. , Zavoyskikh Anton , Paul Jonathan R. , Misirliyan Roy , Salah Zahra'a , Wolok Ryan 
Published: October 2019

INTRODUCTION:
Dronedarone is frequently employed for rhythm control in patients with atrial fibrillation (AF). Chronic therapy with Dronedarone has been associated with mild liver enzymes elevations in up to 12% of patients. There are also several reports of dronedarone induced Acute Renal Failure (ARF) on Adverse Drug Reaction reporting databases worldwide.

CASE DESCRIPTION/METHODS:
An 86-year-old male with past history of AF, COPD, CKD and HTN presented with 1 day duration of dyspnea and palpitations. He was found to be in AF with rapid ventricular response with a heart rate of 127. Vitals were otherwise stable. He was also suspected to have acute on chronic diastolic CHF likely precipitated by atrial fibrillation. Initial labs were pertinent for creatinine of 2.02 (baseline-1.60). Patient was initially started on Diltiazem drip in the ED which was later discontinued due to an IV site reaction. Upon transfer to the medical floor, Cardiology was consulted who recommended Dronedarone for rhythm control. Patient received a total of 3 doses of Dronedarone but did not cardiovert, therefore electrical cardioversion was performed. He developed fatigue few hours after the cardioversion. Liver function tests (LFTs) one day after the last dose of dronedarone and post cardioversion showed elevated ALT (3155), AST (3930), Alkaline Phosphatase (144), total (2.4) and direct (1.0) bilirubin levels. INR was also high (6.1). Viral hepatitis testing was negative. The patient's course was further complicated by acute renal failure (ARF) with creatinine rising to 5.38. Dronedarone mediated hepatic and renal injury was suspected. Dronedarone was discontinued and supportive measures were initiated. Patient's LFTs and creatinine trended down with improvement of symptoms four days following discontinuation of Dronedarone. Patient was discharged home with complete resolution of hepatic and renal failure noted during outpatient follow up.

DISCUSSION:
Dronedarone mediated acute liver toxicity is rare. Almost none of the cases involved rapid onset of liver injury after drug initiation, which resolved quickly after cessation of the agent. The onset of injury in reported cases ranged from 2 to 11 months. Besides reports on pharmacovigilance databases, two cases of dronedarone-associated ARF have also been published, one of which required hemodialysis. Only one of these cases reported a combined hepatic and renal failure. Our case is unique due to its early onset and severe features with concurrent involvement of both organ systems.