bluuebunny/crossref_metadata_2025_split huggingface dataset:
3187 Rare Presentation of Hereditary Hemochromatosis Brought to Light by Disseminated Mycoplasmosis
Authors: Khorasani-Zadeh Arshia , Shahi Diwas , Joshi Sumendra 
Published: October 2019

INTRODUCTION:
Hereditary hemochromatosis is commonly caused by mutation of the HFE protein (C282Y/H63D), leading to iron overload and deposition in the liver, heart, pancreas, and pituitary. Manifestations of Hereditary hemochromatosis differ based on the primary site of iron deposition, ranging from cirrhosis, cardiomyopathy, to diabetes and immunosuppression. which increases susceptibility to opportunistic infections including disseminated mycoplasma. Even with its estimated prevalence of 15% of all community acquired pneumonias, mycoplasma pneumonia is not well recognized for its multitude of rare extrapulmonary manifestations (Figure 1).

CASE DESCRIPTION/METHODS:
43 year old male was found unresponsive at home. On admission, diffuse mucosal sloughing and bleeding with numerous small, flat, erythematous lesions on the trunk and extremities. Throat swab Polymerase Chain Reaction: positive for mycoplasma pneumonia, with IgM serum confirmatory test. These skin lesions would blister and ulcerate on following days. Skin biopsy revealed Steven Johnson Syndrome. On hospital day 6 patient was noted to have left hemiplegia, Head imaging, Right Middle Cerebral Artery ischemic stroke and hemorrhagic conversion. Cardiac Imaging showed No patent Foramen Ovale, no atrial thrombi or valvular pathology, leading to a diagnosis of cryptogenic stroke. In the presence of elevated liver function testing and diffuse hyperattenuation of the liver on non-contrast CT, Iron studies were obtained. Ferritin of 10,086 ng/ml, % Iron saturation of 78.7. Genetic testing returned homozygous for C282Y gene. Liver biopsy confirmed hemochromatosis. Appropriate antibiotics and serial phlebotomies started, after which he clinically improved.

DISCUSSION:
The liver plays a central role in the clearance of bacteria from the bloodstream as hepatic phagocytosis accounts for more than 80% of all reticuloendothelial system function. In hemochromatosis, patients have increased portocaval shunting from liver injury. Therefore, bypassing the liver, and reducing clearance of mycoplasma, predisposing patients to a more virulent clinical course. Mycoplasma's activity in an Iron rich environment is poorly understood. It is known that mycoplasma binds to lactoferrin, a protein largely responsible for iron sequestration in the body for bacteriostasis. This leaves room for further research regarding its evolutionary pathogenicity. Therefore, patients with unexplained opportunistic infections and elevated transaminases should be investigated for hemochromatosis.
3188 Alcoholic Hepatitis and the Need to Focus on Abstinence From Alcohol
Authors: Lewis Justin , Collins Judy 
Published: October 2019

INTRODUCTION:
Alcoholic hepatitis (AH) results from long-term alcohol abuse that leads to liver inflammation and impaired hepatic function. Abstinence from drinking is of utmost significance for these patients.

CASE DESCRIPTION/METHODS:
A 56 year-old man with a history of severe alcohol use disorder presented with subacute vomiting, abdominal pain, dark stools, and fatigue. He drank 10 beers/day for 15 years. HR was 110 and BP 105/75. He was jaundiced, tremulous, and had moderate tenderness in his right upper quadrant. Labs showed WBC 12.7, Hgb 6.6 (baseline 8 - 9), AST 230/ALT 61, total bilirubin (TB) 8.1, ALP 295, INR 1.3, and EtOH level 156. CT demonstrated low liver attention consistent with hepatic steatosis. He was started on IV PPI, CIWA protocol, and given 1u pRBCs. EGD and colonoscopy revealed no source of GI bleed. His hemoglobin and transaminitis improved, TB peaked at 8.8 and declined, and he was discharged with outpatient alcohol counseling and PPI BID. He presented four weeks later in alcohol relapse and is now admitted for detoxification.

DISCUSSION:
This patient presented with classic findings of AH, but his case represents how alcohol abstinence is paramount for these patients. The recent literature for AH centers upon the mortality benefit (or lack thereof) of steroids. Had our patient's Maddrey score been ≥32 (his was 24), this would have been considered, but this misses the larger issue of alcohol in AH. Abstinence is the only independent predictor of long-term survival in AH, with one study demonstrating 5-year survival of 75% in abstainers vs 26% in relapsed drinkers.
1
While most of our patient's care focused on his anemia workup, the highest mortality benefit gained is through resources to maintain sobriety, likely needing more than just outpatient counseling (as is demonstrated by his relapse). To this end, integrated psychotherapy is effective for achieving abstinence in AH patients. A systematic review found that 45% of patients in a psychosocial intervention group achieved abstinence vs 36% in the control group, with one study showing 74% vs 45% abstinence.
2
Getting AH patients to stop drinking is the most beneficial treatment for their health, and a deliberate effort utilizing integrated, multidisciplinary care can achieve this.
3199 Acute Decompensation of Cirrhosis Complicated by Simultaneous Portal Vein Thrombosis and Hepatocellular Carcinoma
Authors: Paz Sandra Gomez , Jiyani Rucha , Kumar Swati , Jacob Bobby , Rizvon Kaleem , Mustacchia Paul 
Published: October 2019

INTRODUCTION:
Prognostic factors differ significantly between those with compensated and decompensated cirrhosis. We present the case of a patient with decompensated cirrhosis who was found to have portal vein thrombosis and hepatocellular carcinoma simultaneously.

CASE DESCRIPTION/METHODS:
68 year old Indian male with history of alcohol abuse, liver cirrhosis and gastroesophageal varices (GOV) type 2, presents with 4 days of diffuse abdominal pain, abdominal distension, fatigue and mild shortness of breath. Collateral information included recent episodes of confusion. Physical exam showed moderate abdominal distension with shifting dullness and diffuse tenderness. Patient was oriented only to person and space. Laboratory examination showed AST 166 U/L, ALT 58 U/L, total bilirubin 20 mg/dL, Ammonia 43 ummol/L and AFP 6.8 ng/mL. CT scan of the abdomen revealed heterogeneous nodular liver with enhancing nodule in the right hepatic lobe measuring 1.8 cm; portal hypertension, paraesophageal varices, and moderate intrabdominal ascites. Portal vein thrombosis (PVT) of intrahepatic portion was noted. Abdominal ultrasound was consistent with advanced cirrhosis and common bile duct (CBD) dilatation of 1.2 cm. Abdominal MRI and MRCP were performed, confirming liver lesion. Ascitic fluid analysis ruled out spontaneous bacterial peritonitis. CT- guided biopsy of hepatic lesion confirmed Hepatocellular Carcinoma (HCC). Patient underwent successful Transarterial Chemoembolization (TACE) of right hepatic tumor and was eventually discharged and followed by Gastroenterology as outpatient.

DISCUSSION:
Literature review stipulate that in the compensated patients, factors related to the tumor in HCC would be more important, whereas in the decompensated patient, both liver and tumor related factors would be important. However, the number of studies is too small to be conclusive, particularly regarding patients with mostly decompensated cirrhosis and those with non-advanced tumor stage. When it comes to PVT as a predictor of prognosis and mortality in patients with decompensated cirrhosis, recent studies strengthen the idea that PVT simply arises in the context of severe liver failure, and does not significantly worsen hepatic function. In our case, the simultaneous finding of PVT and HCC in setting of decompensation may be indicative of dismal outcome. The significance of HCC and PVT as predictors of prognosis and mortality, should be better evaluated in cirrhotic patients in compensated versus decompensated states independently.
3201 Acute Portal Vein Thrombosis Presenting With Atypical Abdominal Pain: A Rare Presentation
Authors: Randhawa Tejinder , Vohra Ishaan , Omar Yazan Abu , Patel Ricky , Flores Estefania 
Published: October 2019

INTRODUCTION:
Portal vein thrombosis (PVT) is an uncommon etiology of transient abdominal pain that left undiagnosed can present with severe complications including variceal hemorrhage, intestinal ischemia, and portal biliopathy. The clinical presentation of PVT is highly variable and depends on the speed of development and extent of occlusion. Rarely do partial acute PVTs present with symptoms. As a result, many PVTs are found incidentally on CT scanning performed for other indications. Management of acute PVT relies on anticoagulation over a three- to six-month period, however this practice has not been evaluated in randomized trials. Current practices rely on the principal that spontaneous recanalization of PVT is rare compared to rates of recanalization as high as 40% in anticoagulated patients. We present a rare case of partial PVT presenting with localized abdominal pain and fever.

CASE DESCRIPTION/METHODS:
A 63-year-old male was admitted from the Emergency Department with right upper quadrant pain and subjective fever. Past histories remarkable for hypertension and alcohol use disorder. Patients examination revealed a soft abdomen with severe right upper quadrant tenderness. On initial presentation the patient had a fever of 100.5 degrees Fahrenheit sustained for 1 hour that self-resolved within 4 hours of admission. CT scan of the abdomen and pelvis revealed linear hypodensities in portal vein branches to the right anterior hepatic lobe and lateral segment of the left hepatic lobe and was otherwise unremarkable. Follow-up duplex ultrasound of the hepatic portal veins corroborated the CT findings. Anticoagulation was started initially with heparin and then subsequently transitioned to rivaroxaban with plans to continue anticoagulation for six months. Patient had complete resolution of abdominal pain within 24 hours of initial presentation. The patient was evaluated for underlying prothrombotic disorders and investigations revealed positive lupus anticoagulant. Patient currently awaits repeat imaging to document resolution of the PVT.

DISCUSSION:
PVT can present a diagnostic challenge as presentation, though typically asymptomatic, can also be highly variable. Review of the literature demonstrates atypical presentations including nonspecific dyspepsia, epigastric pain mimicking pancreatitis, and colicky abdominal pain and diarrhea. Recognition and early treatment of PVT is important to prevent portal hypertension and its associated long-term complications.
3202 Where Is the Coin?
Authors: Widner Justin , Jain Rekha , Malkani Anjali 
Published: October 2019

INTRODUCTION:
Foreign body ingestions are a common presenting complaint in the emergency department. Nearly 80 percent of the greater than 100,000 cases annually are in children. Coins are the most frequently ingested item in children. Most coins pass uneventfully, however they may get lodged at the level of the thoracic inlet, at the level of the arch of the aorta or at the gastroesophageal junction. Urgent evaluation involves obtaining a chest radiograph. Ingested coins found in the esophagus are oriented coronally on a posteroanterior (PA) radiograph. However, coins lodged in the trachea are oriented sagitally (due to the longitudinal orientation of the larynx and the incomplete C rings of the trachea, allowing a longer antero-posterior diameter of the trachea). We present a child with esophageal coin oriented sagitally on PA chest X-ray.

CASE DESCRIPTION/METHODS:
A healthy six year old boy presented to the Emergency Room with severe chest pain an hour after swallowing a nickel. There were no respiratory symptoms. On chest X-ray the coin was seen “on edge” on the PA view and “en-face” on the lateral view, below the level of carina. CT scan revealed the coin sagitally oriented in the esophagus at the level of the gastroesophageal junction. Due to persistence of chest pain he underwent endoscopy immediately after CT scan. At endoscopy under general anesthesia the coin- a nickel, was seen in the stomach and retrieved. The esophagus appeared normal.

DISCUSSION:
Even though most ingested coins are oriented coronally in the esophagus, our case demonstrates that coins in the esophagus may be oriented sagitally as well. Review of the literature revealed a study with 8 cases of sagittally oriented esophageal coins. In these children, 7 of the 8 were at the level of the aortic arch, while the other was at the distal esophagus, as was seen in our patient. The cause of the sagittal orientation is speculative; and may be related to peristalsis and a smaller sized coin.
3205 A Rare Case of Salmonella Gastroenteritis Presenting as Aspiration Pneumonia With Pleural Empyema in Immunocompetent Patient
Authors: Mehmood Asif , Ullah Waqas , Ashraf Aniza , Essrani Rajesh 
Published: October 2019

INTRODUCTION:
Salmonellae are gram negative rods belongs to Enterobacteriaceae. They commonly cause enterocolitis, enteric fever, endovascular infection and bacteremia with localized infection which can occur at any site. Pulmonary involvement especially empyema is very rare and usually reported in immunosuppressed patients secondary to co-morbidities such as HIV,malignancy,diabetes and iatrogenic (steroids/chemotherapy).

CASE DESCRIPTION/METHODS:
76-year-old male with past medical history of Ischemic cardiomyopathy with 35% EF status post AICD, CKD stage 3, dementia and stroke presented to ER for generalized weakness, fever, coughing, right sided lower chest pain, abdominal pain, non-bloody watery diarrhea and vomiting for 3 days. Physical examination revealed new oxygen requirement with respiratory rate 24/min, blood pressure 110/75 mmgh, heart rate 70/min and temperature 100.9 F. He was ill appearing, lethargic with +1 pedal edema. Abdomen was soft but with epigastric/right upper quadrant tenderness. Decreased breath sounds were present at bases. Initial laboratory results showed leukocytosis of 12000 K/ul, normal AST/ALT, elevated bilirubin 2.1 mg/dl, Alkaline phosphatase 324 u/l, BUN 37 mg/dl and Creatinine of 1.8 (at baseline). Chest x-ray showed right greater than left small bilateral pleural effusions and bibasilar atelectasis/infiltrates. Ultrasound RUQ negative for acute cholecystitis. Patient was started on intravenous lasix was started given concern for volume overload and IV antibiotics for possible aspiration pneumonia. Low grade fevers persisted for next 24-36 hrs along with worsening breathing. Repeat chest x-ray showed worsening right pleural effusion. CT chest revealed large loculated left pleural effusion with infiltrates, small right effusion with nearby atelectasis and distended esophagus. Chest tube was placed along with injecting tPA/DNase to help break up loculations. Patient passed swallow evaluation and further testing was negative like blood/sputum cultures and stool studies. Pleural fluid was exudative and grew few non-typhoid salmonella species without reporting of exact identification by laboratory. Patient was discharged in stable condition on 4 weeks of Amoxicillin/clavulanic acid after consultation with Infectious disease specialist.

DISCUSSION:
Non-typhi salmonella causing pneumonia and empyema is very rare. Most common presentation is gastroenteritis. Patients presenting with gastroenteritis and pulmonary involvement, clinicians should keep salmonella as a differential diagnosis.
3216 SMA Syndrome and Small Bowel Obstruction
Authors: Kapadia Deena , Pham Michael 
Published: October 2019

INTRODUCTION:
Superior mesenteric artery (SMA) syndrome is a condition in which the third portion of the duodenum, between the abdominal aorta and SMA, is compressed. The disease is rare, as incidence rates range from 0.1% to 0.3%. It is more prevalent in females aged 10 to 39 years old. Symptoms include severe weight loss, epigastric pain, nausea, and vomiting. SMA syndrome is an uncommon cause of small bowel obstruction (SBO). This case describes a patient with known SMA syndrome who presented with acute symptoms of SBO.

CASE DESCRIPTION/METHODS:
A 36-year-old male with past medical history of polysubstance abuse, hypertension, GERD, psychiatric disorders (bipolar, anxiety, depression), GI bleeds, and SMA syndrome presented with worsening abdominal pain for two weeks. Pain was described as dull and constant with radiation to his lower back. Associated symptoms included worsening nausea and coffee ground hematemesis, as well as dark stools and decreased appetite. The patient was hemodynamically stable. NG tube was placed to help with decompression. He was given IV fluids and started on pantoprazole 40 mg BID IV. CT abdomen with contrast showed a 4 mm obstruction at the third portion of the duodenum consistent with SMA syndrome and a second jejunal area of transition. General surgery deemed him to not be an appropriate surgical candidate, and medical management was recommended. EGD demonstrated a collapse of the duodenal mucosa at the third to fourth portion of the duodenum with moderate chronic appearing dilation of the duodenum proximal to this area. A small sliding hiatal hernia and LA class D esophagitis were also seen. He was started on sucralfate 1 gm QID PO and switched to pantoprazole 40 mg daily PO upon discharge. Liquid diet was advanced as tolerated. Once his symptoms improved, he was discharged and instructed to follow up with a GI physician.

DISCUSSION:
SMA syndrome should be considered in the differential for young adults presenting with symptoms of SBO due to the morbidity and mortality associated with malnutrition, dehydration, and other complications, such as gastric perforation and hemorrhage. Diagnosis is made based on clinical symptoms and radiologic imaging. Initial treatment is non-operative medical management with fluid resuscitation, TPN, NG tube, and electrolyte correction. Laparoscopic duodenojejunostomy is the most common surgical intervention used when medical management fails. In this case, the patient was able to have improvement in symptoms with medical management alone.
3221 A Rare Case of Collagenous Sprue
Authors: Gade Ajay , Rubery Bradley 
Published: October 2019

INTRODUCTION:
Collagenous Sprue (CS) is a rare clinicopathological malabsorptive disorder characterized by mucosal villi atrophy and sub-epithelial collagen deposition. It is very important to consider the possibility of CS in patients diagnosed with celiac disease not improving on the gluten-free diet.

CASE DESCRIPTION/METHODS:
We present an elderly caucasian male with clinicopathological manifestations of Collagenous Sprue, as well as the successful treatment course that he underwent.

DISCUSSION:
Collagenous Sprue (CS) is a rare clinicopathological malabsorptive disorder characterized by mucosal villi atrophy which was first described in 1947. Approximately 60 cases have been reported so far in the medical literature. Replacement of the crypts with subepithelial collagen deposition in the small intestine made some experts believe CS to be a histopathologic variant of celiac disease with a poorer outcome. Although it's not true in all patients, diagnosis of CS should be considered in a patient whose symptoms are not getting better despite a gluten-free diet. Unlike in celiac disease, the collagenous deposits in CS doesn't regress with a gluten-free diet. Thus far, the etiology of CS is not clearly understood and it is very essential to initiate treatment at an early stage of the sprue to prevent fibrotic progression. Multiple treatment strategies were advised in the past but there is no standard therapy.
3223 Knot in the Stomach: Metastatic DLBCL Disguised as an Ulcer
Authors: Prakash Sameer , Banwait Amrit , Khandheria Bharat , Banwait Kuldip 
Published: October 2019

INTRODUCTION:
Early primary gastrointestinal non-Hodgkin's lymphomas can be difficult to diagnose and can be mistaken for diseases such as Crohn's disease or intestinal tuberculosis. We present a 73-year-old patient who had large duodenal bulb ulcer on endoscopy that resulted in metastatic DLBCL.

CASE DESCRIPTION/METHODS:
A 73-year-old female with history of osteoporosis, Vitamin B12 deficiency, and leukopenia presented to her primary care physician complaining of feeling knot in upper abdomen for 1 month. She felt the knot was getting progressively bigger with mild abdominal discomfort. She had also complained of early satiety, abdominal bloating, and weight loss of 3-4 pounds in past few months. She denied diarrhea or blood in the stools. Physical exam was significant for minimal distension with prominent palpable pass in right upper abdomen with tenderness to palpation and no lymphadenopathy. Labs showed WBC 4.0, Hb 11.6, Plt 417, Alkaline Phosphatase 235, Alb 3.3. CT scan of abdomen pelvis showed circumferential mass like thickening along gastric antrum, mesenteric implants, and periaortic lymphadenopathy concerning for gastric carcinoma or lymphoma. She was referred to oncologist and gastroenterologist for evaluation with upper endoscopy. EGD showed large duodenal bulb ulcer with necrotic base and small ulcer in antrum. Duodenal biopsy showed newly diagnosed stage IV Diffuse Large B-Cell Lymphoma that also involved gastric, gallbladder, and pancreas. She completed 3 cycles of R-CHOP chemotherapy and was started on high-dose methotrexate for CNS prophylaxis. She had Whipple procedure done after leak of gastrografin and noted to have perforated duodenum.

DISCUSSION:
Diagnosis of primary GI lymphoma is difficult as it can be nonspecific and often benign gross appearance on endoscopy or colonoscopy. The duodenum represents roughly 6-8% of sites involving small intestine lymphoma. DLBCL of the intestine treated with surgery plus CHOP or R-CHOP chemotherapy has very good prognosis. Early diagnosis and treatment is crucial due to the aggressive nature of this malignancy.