Abbreviations and Acronyms: AN = anorexia nervosa; MRI = magnetic resonance imaging; WE = Wernicke encephalopathy

Elevated liver enzymes are a common scenario encountered by physicians in clinical practice. For many physicians, however, evaluation of such a problem in patients presenting with no symptoms can be challenging. Evidence supporting a standardized approach to evaluation is lacking. Although alterations of liver enzymes could be a normal physiological phenomenon in certain cases, it may also reflect potential liver injury in others, necessitating its further assessment and management. In this article, we provide a guide to primary care clinicians to interpret abnormal elevation of liver enzymes in asymptomatic patients using a step-wise algorithm. Adopting a schematic approach that classifies enzyme alterations on the basis of pattern (hepatocellular, cholestatic and isolated hyperbilirubinemia), we review an approach to abnormal alteration of liver enzymes within each section, the most common causes of enzyme alteration, and suggest initial investigations.

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INTRODUCTION: Neuroendocrine carcinomas (NECs) are defined epithelial neoplasms with predominant neuroendocrine differentiation. They primarily arise from the gut and bronchopulmonary systems with the possibility of distant metastasis. We present a case of an esophageal neuroendocrine neoplasm with unique endoscopic/radiographic features on presentation disguised as a food impaction. CASE DESCRIPTION/METHODS: A 50-year-old Caucasian male with past medical history of tobacco dependence; presented to the ED with complaints of solid food dysphagia for 1.5 months with unintentional weight loss of 15 lbs and intractable hiccups. On arrival, he was hemodynamically stable. Physical exam was unremarkable. Laboratory testing revealed mild normocytic anemia of 12.3, otherwise unremarkable. An upper endoscopy was performed revealing a large amount of meat in the mid-esophagus; however, after further manipulation there appeared to be bleeding from the mucosa (Figure 1). Upon closer inspection, a mass originating from the lateral esophageal wall was caked with food debris giving it the appearance of meat. CT Chest was performed showing a large heterogenous esophageal mass (8.1 cm v 5.7 cm) extending from the mid-esophagus to the GE junction with no evidence of direct invasion (Figure 2). Biopsies returned positive for large cell neuro-endocrine carcinoma with diffuse synaptophysin and Ki67 positivity. PET-CT performed as outpatient showed prominent peri-gastric lymphadenopathy. EUS was attempted but failed due to inability to pass the ultrasound scope. CT surgery subsequently performed a McKeown transthoracic esophagectomy for definitive therapy as outpatient. He recovered well from the procedure with positive radial margins and lymph nodes on pathology and is now undergoing adjuvant radiation and chemotherapy. DISCUSSION: Esophageal NEC is a rare entity with a generally dismal prognosis when high-grade. A study out of a large volume, tertiary center reported approximately 40 cases over a 20-year period. Given the sparsity of esophageal NECs, most studies are retrospective case series and are from outside the USA. It is reported that esophageal NECs represent only 0.4-1.4% of the gastrointestinal NECs a majority of which arise from the mid-esophagus. To our knowledge, there is no reports of a single case with similar endoscopic and radiographic findings which makes it unique. There is also limited data reported on the endoscopic/phenotypic appearance of large cell neuroendocrine carcinomas of the esophagus.

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INTRODUCTION: Pantoprazole is a widely used proton pump inhibitor that is used to treat variety of acid related disorders. It is well tolerated with excellent safety profile. Mild transient elevations in serum aminotransferase can be seen with pantoprazole use that resolves without dose modification. Pantoprazole induced clinically apparent liver injury on the other hand is exceedingly rare. CASE DESCRIPTION/METHODS: A 47-year-old woman with no prior liver disease was referred to the hospital for acute hepatitis. She started pantoprazole 2 months prior to admission for gastritis. Patient had nausea, fatigue, abdominal pain, jaundice and dark urine 2 weeks after starting pantoprazole and stopped it a week prior to hospital admission. She denied alcohol use. Her current medications were levothyroxine, enalapril/hydrochlorothiazide and aspirin and no use of herbal supplements. Physical exam was notable for conjunctival icterus. Baseline liver enzymes were normal. Labs on admission were: AST 681 U/L (< 32 U/L), ALT 2100 U/L (< 36 U/L), total bilirubin 12.3 mg/dL (0.2-1.2 mg/dL), direct bilirubin 8.3 mg/dL (< 0.5 mg/dL), alkaline phosphatase 96 U/L (45-117 U/L) and INR 1.9 (0.8-1.2). Urine drug screen was negative for acetaminophen toxicity. Imaging excluded biliary obstruction and hepatic venous thrombosis. Laboratory investigations for viral and autoimmune hepatitis, wilson’s disease and hemochromatosis were negative. Liver biopsy (Figure 1) reported portal areas with edema and a minimal amount of inflammation composed of mixed lymphocytes, occasional eosinophils and neutrophils. The lobular parenchyma showed cholestasis with lymphocytic and neutrophilic inflammation. Patient noted improvement of symptoms with supportive care (Table 1). Two months after stopping pantoprazole, liver enzymes were normal. DISCUSSION: DILI (drug-induced liver injury) is a significant cause of acute hepatitis. To our knowledge, only 7 case reports of pantoprazole induced liver injury have been published. The timing of starting pantoprazole prior to acute hepatitis, negative serologies, and normalized liver enzymes after stopping suggests pantoprazole as the cause of DILI. Based on its pharmacokinetics, timeline and pattern of insult, an idiosyncratic reaction has been suggested as the mechanism for hepatotoxicity. We have described a rare case of acute mixed (hepatocellular and cholestatic) liver injury from pantoprazole use suggesting its consideration in the differential diagnosis of DILI.

INTRODUCTION: Celiac disease is an immune-mediated disorder triggered by a reaction to gluten with elevated levels of IgA antibodies. Symptoms typically resolve with a gluten free diet. However, refractory cases have been reported despite dietary adherence. Our discussion involves an elderly woman with severe, refractory celiac disease with concomitant IgA deficiency. CASE DESCRIPTION/METHODS: An 80-year-old woman presented our clinic with exacerbation of chronic, non-bloody diarrhea. She has a history of celiac disease diagnosed 20 years ago after an episode of acute pancreatitis. She also has IgA deficiency and her total IgA level remains low at 37, despite infusions 3 times per week. She is strictly adherent to a gluten free diet, but continues to have multiple episodes of diarrhea and has lost over 100 pounds since her diagnosis. Patient had bidirectional endoscopy notable for antral gastropathy as well as fissures and flattening of the duodenal mucosa. Biopsies were consistent with celiac sprue. Capsule endoscopy showed diffuse flattening of the small bowel villi with erythema and cracked, scalloped mucosa as well as multiple, small, aphthous ulcers throughout the bowel (Figures 1, 2, and 3). Patient has had an extensive workup, but everything else have been unrevealing. Steroids have been initiated as a last-ditch effort, but she continues to have “more bad days than good days” despite being on a high dose prednisone. DISCUSSION: The diagnosis and treatment of celiac disease can be complicated by coexisting immunodeficiencies. Usually, titers of tissue transglutaminase IgA will be elevated and are involved in the immune reaction that causes damage to the lining of the small bowel with characteristic flattening of the villi as well as chronic diarrhea and malabsorption. A connection between IgA deficiency and celiac disease has been well established and likely contributes to the refractory nature of our patient’s disease. In patients with IgA deficiency it is important to utilize other forms of serologic testing, typically with IgG tissue transglutaminase, in addition to duodenal biopsies. Symptoms typically resolve with a gluten free diet. However, refractory cases have been reported with persistent diarrhea and villous atrophy for over 1–2 years. Symptom management can be difficult for these patients and may require the use of steroids as the last resource.