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Objectives Dysregulation of hepcidin-iron axis is presumed to account for abnormal iron status in patients with chronic liver disease (CLD). Our aim is to determine the effect of specific etiologies of CLD and of cirrhosis on serum hepcidin levels. Methods Pubmed, Embase, Web of Science were searched for studies comparing serum hepcidin levels in patients with CLD to that in controls using enzyme-linked immunosorbent assay. The study was conducted in accordance with the Preferred Reporting Items for Systematic Review and Meta-Analysis Guidelines. Statistical analysis was carried out with STATA using random effects model to calculate the mean difference (MD) between two groups. Results Hepcidin levels were significantly lower in subjects with hepatitis C virus (16 studies) [MD −1.6 (95 % CI: −2.66 to −0.54), p<0.01] and alcoholic liver disease (3 studies) [MD −0.84 (95 % CI: −1.6 to −0.07), p=0.03] than controls. Serum hepcidin was significantly higher in subjects with non-alcoholic fatty liver disease (12 studies) [MD 0.62 (95 % CI: 0.21 to 1.03), p<0.01], but did not differ in subjects with hepatitis B and controls (eight studies) [MD −0.65 (95 % CI: −1.47 to 0.16), p=0.12]. Hepcidin levels were significantly lower in patients with cirrhosis of any etiology (four studies) [MD −1.02 (CI: −1.59 to −0.45), p<0.01] vs. controls (CI: confidence interval). Conclusions Serum hepcidin levels are altered in common forms of CLD albeit not in a consistent direction. Additional study is needed to determine how changes in hepcidin levels are related to dysregulation of iron metabolism in CLD.

A 56-year-old man presented with recurrent gastrointestinal obstruction. Computed tomography showed fluid-filled, distended stomach, small intestine, and large intestine. Extensive workup including esophagogastroduodenoscopy, colonoscopy, magnetic resonance enterography, push enteroscopy, and video capsule enteroscopy showed no mechanical obstruction. Endoscopic ultrasound-guided biopsy of peripancreatic nodes detected on 18 F-fluorodeoxyglucose positron emission tomography revealed a duodenal neuroendocrine tumor. The lesion showed intense uptake on gallium-68 DOTATOC positron emission tomographycomputed tomography scan. The patient underwent surgical resection of the tumor with resolution of bowel obstruction events. He had elevated pancreatic polypeptide levels, which are known to delay gastric emptying and could explain his symptoms.Previous computed tomography (CT) scans of the abdomen showed variable distension of the stomach, or fluid-filled small intestine with concern for transition point at the ileum, or distended large intestine. He had formerly undergone 2 esophagogastroduodenoscopies, which did not reveal gastric outlet obstruction. His current CT abdomen showed a fluid-filled stomach and duodenum, suggestive of gastric outlet obstruction (Figure 1). Magnetic resonance enterography (MRE) did not show obstruction, but demonstrated a 2.1 3 2.3-cm right mesenteric nodule with similar enhancement as the spleen, raising the possibility of ectopic splenic tissue, and a 0.7-cm nodule posterior to the uncinate process.Push enteroscopy revealed esophagitis and severe aphthous ulcerations in the small intestine, but no obstruction. Colonoscopy did not reveal any obstructive pathology. Video capsule enterography revealed erythematous mucosa with villous blunting and nonobstructive luminal narrowing in the duodenum through which the capsule passed easily.An 18 F-fluorodeoxyglucose positron emission tomography ( 18 FDG PET)/CT scan was performed to further evaluate the mesenteric lesion seen on MRE. It showed multiple mild to moderately FDG avid cervical lymph nodes (LN), the largest a 4-cm left supraclavicular LN. It redemonstrated the soft-tissue lesion anterior to the duodenum with mild to moderate FDG uptake and multiple

Background Erdheim-Chester disease (ECD) is a rare histiocytic disorder recently recognized as a neoplasm due to the discovery of activating MAPK pathway mutations. Hepatic involvement by ECD is extremely rare. Case presentation We describe a case of a 64-year-old male who presented with pruritis, weight loss, and cholestatic liver function tests. Magnetic resonance imaging of the abdomen showed beaded appearance of the intrahepatic biliary tree. A liver biopsy was suggestive of primary or secondary sclerosing cholangitis. Computerized tomography (CT) of the abdomen showed perinephric and periaortic soft tissue stranding suggestive of ECD. 18F-fluorodeoxyglucose positron emission/computerized tomography scan showed a mediastinal hilar mass which turned out to be follicular lymphoma. Histopathology of molluscum-like skin lesions showed CD68 + , Factor XIIIa + , and CD1a-foamy histiocytes with multiple giant cells suggestive of ECD. The patient developed recurrent episodes of ascending cholangitis and his hyperbilirubinemia continued to worsen despite stenting of a common hepatic duct stricture found on endoscopic retrograde cholangiopancreatography. Conclusions The absence of associated inflammatory bowel disease and anti-neutrophil cytoplasmic antibody, as well as the rapidity of disease progression, makes us consider the possibility of hepatic involvement by ECD or an overlap syndrome. We want to highlight that negative histopathology should not delay the diagnosis of ECD as effective and potentially lifesaving therapies with BRAF or MEK pathway inhibitors are now available for these patients.

Introduction: Primary duodenal neuroendocrine carcinoma (NEC) is a rare and highly aggressive malignancy with very poor prognosis. There is no established treatment due to its rarity. Treatment regimens used for small cell lung cancer (SCLC) are used to treat neuroendocrine carcinoma, due to its clinical and histopathological similarities. Therapeutic strategies are poorly understood and not well defined. There is no standardization of therapy even for limited disease and usually multimodal treatment approach is used. Etoposide based treatment regimens have been used mostly in advanced stages. We herein report a case of an aggressive primary duodenal neuroendocrine tumor with excellent response to oxaliplatin-based chemotherapy regimen. Case Description/Methods: We describe a case of a 72-year-old caucasian male who presented to emergency room with abdominal bloating and constipation. He was found to have peritoneal carcinomatosis and marked hydronephrosis in computed tomography (CT) of the chest/abdomen/pelvis. He was further evaluated by urology, oncology, and gastroenterology team. He had paracentesis with removal of 4 L of ascitic fluid. He underwent esophagogastroduodenoscopy, endoscopic ultrasound, and a colonoscopy for further assessment. He was found to have a large mass in the duodenum which upon biopsy was consistent with grade 4 poorly differentiated neuroendocrine carcinoma. Positron emission tomography dotadate scan, peritoneal biopsy and peritoneal fluid cytology further confirmed metastatic neuroendocrine carcinoma. His tumor markers showed elevated Ca 19-9 and chromogranin levels at presentation. Due to his poor performance status and concerns for intolerability to etoposide, he was started on FOLFOX chemotherapy. He completed 12 cycles of chemotherapy with near complete resolution of his disease as evidence by his positron emission tomography dotadate scan and improvements in his tumor markers. Due to excellent response and disease remission, he is currently on xeloda with omission of oxaliplatin. Discussion: Primary duodenal neuroendocrine carcinoma is a relatively rare malignancy with reported incidence of 0.4-2% among all duodenal malignancies. The prognosis is dismal due to presentation as advanced stage at diagnosis. Oxaliplatin based regiments have been shown to have promising anti-tumor activity in gastrointestinal neuroendocrine cancers, however the available data in duodenal gastrointestinal neuroendocrine cancers are very limited.