bluuebunny/crossref_metadata_2025_split huggingface dataset:
2327 Not Just a Cyst: Rare Presentation of LUQ Pain
Authors: Henriquez Richard , Comba Isin Y. , Kumar Sundeep , Bashir Khawaja , Wallis-Crespo Maria , Qazi Omar , Bhatia Lakhinder 
Published: October 2019

INTRODUCTION:
Lymphangioma is a benign congenital malformation of the lymphatic system that is relatively common in juvenile population. Rarely, it can be seen in adults and majority of the cases are reported in the head and neck region (70-75%). Typically, these malformations are asymptomatic. In this report, we present a rare case of splenic lymphangioma presenting with severe, intractable abdominal pain.

CASE DESCRIPTION/METHODS:
A 50-year-old female presented with three-month history of progressive abdominal pain located below the left subcostal margin. Initial vital signs and lab work were unremarkable. Physical exam was significant for tenderness in the left upper abdominal quadrant. Computed tomography (CT) scan of abdomen with contrast revealed a hypointense lesion measuring up to 1.8 cm in the posterior aspect of the spleen. Subsequently, a hypo-enhancing splenic mass in the corresponding location was demonstrated in T1-weighted MRI images (Image 1A). The spleen was normal-sized and there were no liver lesions. A total splenectomy was performed with resolution of the pain during the hospitalization. Histopathological examination of the lesion revealed multiple cystic cavities containing pale eosinophilic fluid (Image 1B) resembling lymph, consistent with splenic lymphangioma. The cystic cavities were lined with elongated cells with bland nuclei and scant cytoplasm (Image 1C). Immunohistochemically, the lesion stained positive with CD31 (Image 1D).

DISCUSSION:
Clinical presentation of splenic lymphangioma varies depending on the spleen size with majority of the patients being asymptomatic. The diagnosis is usually made incidentally on routine imaging studies. Based on the clinical data and imaging results, our differential diagnosis included hemangioma, complex splenic cyst, lymphangioma, lymphoma, and pseudocysts. The splenic lymphangioma was diagnosed on microscopic examination following a splenectomy which was done to avoid any further complications such as; infection, rupture, hemorrhage and pleural effusions. The treatment modality of choice was complete surgical resection in this patient given relevant history. Other therapeutic modalities such as aspiration or drainage have not shown effective results in the literature. Despite the rarity, early recognition of these lesions is important as the delay in surgical intervention can result in complications.
2357 Drug-Induced Liver Injury (DILI) Does Not Always Follow the Textbook
Authors: Ajayi Teminioluwa A. , Dawodu Ejemenare A. , Huh Won J. , Scanga Andrew E. , Izzy Manhal 
Published: October 2019

INTRODUCTION:
Drug-induced liver injury (DILI) is a leading cause of acute liver injury in the US, most commonly caused by antibiotics and NSAIDS. The clinical history and biochemical pattern are often the most important clues to the diagnosis and a classic pattern can save the patient an invasive procedure such as liver biopsy. It becomes diagnostically challenging when the biochemical presentation is inconsistent with the drug reported, such as in this case.

CASE DESCRIPTION/METHODS:
A 34-year old Caucasian man presented to the hospital with progressive jaundice, vomiting and malaise. Medical history is remarkable for Ankylosing Spondylitis (AS) for which he is on Secukinumab, and for acute sinusitis diagnosed 23 days prior in clinic for which he completed a 10-day course Amoxicillin-Clavulanate (AC). Liver tests from 15 months prior were normal. Liver tests on presentation were remarkable for total bilirubin of 4.5 mg/dL, AST of 163 U/L, ALT of 449 U/L, and ALP of 132 U/L and peaked at total bilirubin of 12.5mg/dL, AST of 702 U/L, ALT of 1,292 U/L and ALP of 184 U/L. Viral hepatitis panel was notable for Hepatitis A and B immunity and Hepatitis C IgG positive but with undetectable viral load. Autoimmune hepatitis markers were also normal. In view of the progressive uptrend in transaminases despite medication discontinuation, hepatocellular biochemical pattern, and history of autoimmune disease (i.e., AS), autoimmune hepatitis was suspected and a liver biopsy was done. Liver biopsy was notable for mild mixed portal inflammation and bile duct distortion. The overall impression was DILI. He resumed Secukinumab but not AC. Upon follow up one month later, his liver tests completely normalized.

DISCUSSION:
AC-DILI presents most frequently among elderly men with a cholestatic biochemical pattern and self-limited course. AC-DILI of hepatocellular pattern is rare in adult patients, although more common among pediatric patients. Our report, interestingly, describes a distinct hepatocellular biochemical pattern of AC-DILI in an adult male patient with an underlying autoimmune disease. HLA B-27 allele (class I) is a strong genetic risk factor for the development of AS. Studies have shown that predisposition to AC-DILI is influenced by certain HLA haplotypes perhaps suggesting an involvement of the adaptive immune system. However, further studies are needed to determine whether this association might increase risk for organ injury as in DILI or if it alters the pattern of presentation and expected clinical course.
2368 A Rare Case of Segmental Arterial Mediolysis in a Patient With Alpha-1 Antitrypsin Deficiency and Alcoholic Cirrhosis
Authors: Alimirah Maryam , Selim Ranya , Venkat Deepak 
Published: October 2019

INTRODUCTION:
Alpha-1 antitrypsin deficiency (AAT deficiency) is a systemic disease characterized by lack of the protease inhibitor, alpha-1 antitrypsin and typically manifests as severe emphysema and/or liver disease. There are several case reports describing vascular compromise in patients with AAT deficiency, however mostly in the context of systemic vasculitis and connective tissue disease. Segemental arterial medioloysis (SAM) is a rare non-vasculitis, non-atherosclerotic arteriopathy of middle and small arteries that can lead to aneurysmal dilation or arterial stenosis. We report the first case of a patient with cirrhosis secondary to AAT deficiency noted to have arteriopathy due to SAM.

CASE DESCRIPTION/METHODS:
A 30 year old patient with end stage renal disease (ESRD) on hemodialysis, alcoholic cirrhosis and underlying AAT deficiency presented to our tertiary care center for liver transplant evaluation with a presenting MELD score of 39. Evaluation for other causes of cirrhosis revealed AAT deficiency with a homozygous ZZ phenotype and undetectable serum levels. As part of his pre-transplantation workup at our institution, he underwent computed tomography angiography (CTA) of the abdomen and pelvis which revealed severe irregular narrowing of the celiac axis. Similar narrowing was noted of the renal arteries and splenic artery and focal aneurysmal dilation of the hepatic and gastroduodenal arteries was additionally seen (Image 1). Autoimmune serologies were negative. Patient proceeded to orthotropic liver transplantation during his hospitalization. Intraoperatively, hepatic artery appeared visually vasculitic but histopathologic report was consistent with diagnosis of SAM. Due to concern for potential graft compromise that could result from the recipient hepatic artery aneurysm, an aortic conduit was performed using the donor vessels with anastomosis to the recipient supraceliac aorta.

DISCUSSION:
To our knowledge, there is only one other published report describing SAM in a patient with co-existing AAT deficiency. SAM in this case resulted in hepatic aneurysm which complicated liver transplantation, necessitating alternative graft revascularization via an aortic conduit. Although doppler evaluation 3 days after transplantation showed hepatic artery anastomosis was still patent, the rate of complications related to aorto-hepatic bypass grafts is high, and close follow up remains prudent.
2413 Another Downside for Depression: A Case of SNRI-Induced Liver Injury
Authors: Odah Tarek , Sun Lishi , Treadwell Thomas 
Published: October 2019

INTRODUCTION:
Antidepressants are one of the most prescribed medications in the United States with duloxetine being reported as one of the top fifty drug prescriptions. Self-limited liver test abnormalities have been reported with duloxetine use but only a few case reports describe clinically apparent hepatitis following duloxetine use.

CASE DESCRIPTION/METHODS:
A 35-year-old African American woman with a past medical history of depression presented with few days of worsening upper abdominal pain, nausea and multiple episodes of non-bloody emesis. Initial laboratory tests revealed liver test abnormalities with ALT of 1176 U/L, AST of 596 U/L, bilirubin of 1.6 mg/dL (but with normal ALP) and INR of 1.65. Full workup including viral hepatitis, EBV, CMV, drug toxicology screen (including acetaminophen), lipase, and ceruloplasmin among others turned out to be negative. Autoimmune hepatitis markers were negative other than mild elevation of anti-smooth muscle antibody at 33 units (normal < 19). Abdominal ultrasound with Doppler showed no abnormalities without any evidence of biliary obstruction. The patient denied any alcohol or illicit drug use. Patient endorsed having a recent contact with a sick squirrel so leptospira IgM was ordered which was non-reactive. The patient denied taking any medications other than duloxetine which she was started on within the last two months but she stopped taking it when she started to vomit a few days before presentation. In the second day of hospitalization, patient's LFTs peaked with ALT above 7000 U/L and AST of 5409 U/L but it then started to decrease in the following days with normalization of her bilirubin and INR as well as improvement of her symptoms with supportive care. No corticosteroids or antibiotics were given. Patient's clinical presentation with the timeline of her duloxetine use and laboratory finding indicate that duloxetine was the likely cause of her acute hepatitis.

DISCUSSION:
Few cases described clinically evident hepatotoxicity following duloxetine treatment. Most duloxetine-induced hepatotoxicity was seen in females with symptoms starting in the first few months following initiation of duloxetine. Typically there was an elevation of ALT > AST and with some cases having bilirubin elevation as well as elevation of one of the autoimmune hepatitis markers in some cases. Clinicians should be vigilant toward possible hepatotoxicity following initiation of antidepressant even in patients with no pre-existing chronic liver disease or alcohol abuse history.
2419 Fatal Outcome Associated With Herbal Supplements
Authors: Ahmad Minhaz , Chowdhury Nazif , Saleem Atif 
Published: October 2019

INTRODUCTION:
Herbal and dietary supplements are widely used without prescription and used by the public as harmless remedies for a variety of ailments. It is difficult to determine causal relationships between herbal preparations and hepatotoxicity. We present a case of fulminant hepatic failure from over-the-counter hair regrowth supplements containing Polygonum multiflorum.

CASE DESCRIPTION/METHODS:
A 71-year-old male presented to the ER with complaints of abdominal discomfort and jaundice, associated with nausea and vomiting. He went to the ER ten days prior for abdominal discomfort and nausea, labs showed total bilirubin 1.8, ALP 141, AST 267, ALT 85, platelet 70, BUN 31 and Creatinine 1.2. CT abdomen showed duodenitis and was discharged with Metoclopramide. His symptoms continued to worsen. 12 weeks prior, he started a hair regrowth supplement which contained Polygonum multiflorum. Pertinent labs were-Total Bilirubin 9.8, AP 280, AST 445, ALT 156, platelet 49, BUN 85, creatinine 3.4 and INR 1.82. CT abdomen showed mild hepatic steatosis, mild peripancreatic and periduodenal inflammatory changes. Ultrasound of the gallbladder showed no gallstones or cholecystitis. Normal CBD and common hepatic duct. Hepatitis A, B, C, Anti-smooth muscle antibodies, anti-mitochondrial antibodies, smear for parasites and tick borne were negative, IgG, IgM, IgA – normal, Ceruloplasmin 45. Acetaminophen level <10. He was getting supportive treatment. Day 3 labs showed AST 556, ALT 151, AP 278, total bilirubin 15.1, direct bilirubin 14.6, PT 23.7, INR 2.03. platelets 24, BUN 97, creatinine 2.5. He was started on Acetylcysteine and Lactulose, then transferred to a hospital with a liver transplant facility, where toxicology screening was positive for P multiflorum. He was not a candidate for liver transplant and labs continued to worsen. Shortly thereafter he coded while getting an HD catheter for dialysis and expired. Liver biopsy after autopsy showed diffuse necrosis of hepatic cells.

DISCUSSION:
The mechanism of hepatotoxicity of P multiflorum is unknown but it is known to have anthraquinones including chrysophanol, emodin, and rhein which can induce hepatotoxicity. The pattern of enzyme elevations is typically hepatocellular or mixed and the clinically resembles acute viral hepatitis. The latency to onset ranges from a few days to as long as 6 months. Liver biopsies show changes typical of acute hepatitis. The course is usually self-limited but there are also a small number of patients with liver failure and even death.
2439 Cystic Fibrosis Liver Disease: A Curious Case of Atypical Cystic Fibrosis Diagnosed in Adulthood
Authors: Harriss Patrick B. , Jogimahanti Anusha , Herlevic Vincent , Samant Hrishikesh , Morris James D. , Jordan Paul 
Published: October 2019

INTRODUCTION:
Cystic fibrosis (CF) poses considerable morbidity and mortality. Largely identified in children with “typical” pulmonary problems, some present past adolescence and instead display “atypical” complaints. CF liver disease (CFLD) has a significant disease burden as a leading cause of death in CF. Symptom variation makes diagnosing CF in adults difficult. Our case report describes a diagnosis of atypical CF and CFLD in adulthood after nearly two decades of inconclusive findings. The case highlights hepatic sequelae of CF, disease diversity, and the impact of delayed diagnosis.

CASE DESCRIPTION/METHODS:
We present a 32-year-old African American female first noted to have elevated liver function tests (LFTs) on routine labs at age 16, yet was asymptomatic. Despite numerous clinical encounters over 17 years - including chemistry panels, imaging, and biopsies - the etiology remained unclear. In adulthood, she began experiencing chronic abdominal pain, and was later found to have critically low magnesium levels resulting in a seizure. The differential was expanded, and sweat chloride testing was positive, though genetic testing was negative. Given clinical history of high LFTs with abdominal pain and hypomagnesemia, appearance of symptoms past age 18, and unfixed results, she qualified for the diagnosis of atypical CF with CFLD. Her delayed diagnosis was further masked by unconventional misnomers not typically seen in CF. The patient was enlisted by our CF team for treatment and surveillance, and her LFTs improved.

DISCUSSION:
Our patient's clinical course made the diagnosis of CF less obvious, including: age, race, lack of family history, absence of pulmonary complications, presence of fertility, and vagueness of complaints. While sweat chloride testing was positive, genetic testing was negative. Retrospectively, each result lends itself to the diagnosis of atypical CF, which may show negative genetics despite sweat testing. The presence of a positive sweat test and clinical history is consistent with non-classic CF, and the chronically elevated LFTs and abdominal complaints were indicative of CFLD. Formal declaration of CFLD is important, as it leads to biliary cirrhosis and portal HTN, imposing high mortality. No gold standard modality exists for CFLD, further making diagnosis difficult in lieu of typical CF features. Our case emphasizes the importance of identifying both atypical CF and CFDL, how limited findings confound diagnosis, and the role of a multi-disciplinary team approach for CF patients.
2468 Humeral Fracture as the Only Presenting Symptom of a Metastatic Intra-Hepatic Cholangiocarcinoma: A Rare Cancer With Rare Presentation
Authors: Mehmood Asif , Ullah Waqas , Iqbal Umair , Patel Pranav , Essrani Rajesh 
Published: October 2019

INTRODUCTION:
Cholangiocarcinoma (CCA) is a malignant tumor which originates from the epithelial cells of bile ducts. Abdominal pain and jaundice are the most common presenting symptoms. It commonly metastasizes through lymphatics to regional lymph nodes, followed by involvement of liver, lungs and peritoneum via blood. Bone metastasis is very rare although it has been reported. We are presenting an unusual case with initial presentation of appendicular bony metastatic disease without any GI symptoms.

CASE DESCRIPTION/METHODS:
59-year-old non-smoker male with past medical history of obstructive sleep apnea initially presented to ER with right arm pain while standing from a chair and felt crack in his arm. Physical exam was unremarkable except right mid arm mild swelling and tenderness. Right arm x-ray showed an acute comminuted fracture of proximal humeral shaft with minimal displacement. A CT scan was obtained due to the low energy nature of this injury to rule out pathologic etiology, but this was negative. Blood work up was positive only for normocytic anemia with hemoglobin of 10 g/dl. He was placed in a sling and scheduled to follow-up with orthopedics as outpatient. Four weeks follow up x-ray showed persistent fracture and prominent bone resorption concerning for its pathological nature. Patient was referred to oncologist. Patient remained asymptomatic except right arm pain and some swelling. Extensive work up was initiated. Laboratory investigation showed elevated LFTs with cholestatic pattern. CT CAP with contrast showed significant metastatic abdominal lymphadenopathy, ill-defined mass in the right lobe of the liver and splenomegaly. A bone scan showed uptake in the right mid humerus but no additional findings. Patient underwent CT guided bone biopsy which was consistent with metastatic intrahepatic CCA. Given aggressive growth in short duration and refractory pain, intralesional debulking and metallic end prosthetic segmental reconstruction of the right humeral diaphysis was performed followed by radiation therapy. Oncology recommended to start chemotherapy although prognosis is grim.

DISCUSSION:
CCA is an aggressive tumor with poor prognosis. The pattern of metastasis is usually through lymphatics and further spread via hematogenous route. Appendicular bone metastasis is very rare phenomena and only few cases reported so far. Physicians should have low threshold to investigate low energy injuries leading to bone fractures, in depth for pathological fractures and possibly underlying malignancy.
2481 Severe Transaminitis in Intrahepatic Cholestasis of Pregnancy: An Atypical Presentation
Authors: Randhawa Tejinder , Almoghrabi Anas , Agrawal Rohit , Omar Yazan Abu , Vohra Ishaan 
Published: October 2019

INTRODUCTION:
Intrahepatic cholestasis of pregnancy (ICP) is a self-resolving hepatic condition noted during the second and third trimesters of pregnancy. ICP is a relatively benign condition. However in severe cases ICP can influence fetal outcomes resulting in prematurity and rarely intrauterine fetal demise. Diagnosis of ICP is clinical and suspected when pregnant patients present with moderate to severe pruritus and concomitant elevation in serum aminotransferases and/or bile acids. Total bile acid levels can rise by 10- to 25-fold, however aspartate aminotransferase (AST) and alanine aminotransferase (ALT) levels do not characteristically increase more than two times the upper limit of normal. The mainstay of treatment, ursodeoxycholic acid (UDCA) offers symptomatic relief of pruritus and may benefit fetal outcomes. Delivery of the fetus is followed by resolution of pruritus and normalization of transaminases and bile acids. Our case describes a rare presentation of ICP with marked 10-fold increase in AST and nearly 30-fold increase in ALT.

CASE DESCRIPTION/METHODS:
A 31-year-old female at 28 weeks gestation was admitted for two-day history of generalized pruritus and abnormal transaminases on surveillance testing. Past histories remarkable cholecystectomy following gallstone pancreatitis. Physical examination was benign and was without scleral icterus or jaundice. Investigations were remarkable for: alkaline phosphatase (ALK) 172, gamma glutamyl transferase (GGT) 20, AST 465, ALT 1023, and total bile acids 48.7. Further evaluations revealed negative hepatitis serologies and chronic liver disease work-up, liver doppler ultrasound without occlusion, MRCP without choledocholithiasis or other features of pancreaticobiliary pathology. The patient was started on UDCA and continued follow-up with Gastroenterology and Obstetric Clinics. Shortly after initiation of UDCA, transaminases began a steady down trend. After delivery of a healthy fetus, the patient's transaminases returned to baseline levels.

DISCUSSION:
Classically ICP presents with mild AST and ALT elevations no more than two times the upper limit of normal. In cases of ICP presenting with marked transaminitis it is important to exclude other causes of cholestasis and hepatic disease including viral hepatitis, gallstones, preeclampsia, and HELLP syndrome. With close multi-disciplinary monitoring, even severe cases of ICP can lead to favorable fetal and maternal outcomes including complete resolution of transaminitis and uncomplicated fetal delivery.