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Background/Aims: Specific serum markers reflecting hepatic inflammation and fibrosis are required to tailor the treatment strategies in non-alcoholic steatohepatitis (NASH). We aimed to investigate the roles of myeloperoxidase (MPO) and calprotectin in predicting the hepatic inflammation status and disease severity in NASH. Materials and Methods: A total of 48 patients with biopsy-proven NASH and 25 healthy volunteers with normal weight were prospectively enroiled Serum MPO and calprotectin levels were compared between the NASH and control groups. Hepatic MPO and calprotectin expressions were compared in terms of histologic non-alcoholic fatty liver disease activity scores (NAS) (low NAS [5]) and fibrosis stage (insignificant [F0-1]/significant [F2-4]). Results: Serum MPO and calprotectin levels were not significantly different between the NASH and control groups. In the subgroup analysis, hepatic MPG expression was significantly increased in patients with NASH with significant fibrosis than in those with insignificant fibrosis (F2-4: 7.04 +/- 3.61 vs. F0-1: 4.83 +/- 2.42, p=0.01). We found no difference between the groups with low and high NAS with regard to serum MPG and calprotectin levels and hepatic MPG and calprotectin expressions. Conclusion: This study demonstrated that hepatic MPG expression can reflect advanced fibrosis in NASH. However, when serum MPO and calprotectin levels were evaluated as potential serum markers, both did not associate with hepatic inflammation status and fibrosis stage in NASH. Therefore, our study results preclude their use as serum markers for hepatic inflammation in NASH.

BACKGROUND/AIMS: Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant syndrome characterized by tumors arising from endocrine glands with no specific genotype-phenotype correlation. Here, we report the largest Turkish kindred with MEN1 syndrome which inherited a scarce MEN1 mutation gene. MATERIALS AND METHODS: A 64-year-old man, referred to our gastroenterology outpatient clinic for evaluation of a pancreatic mass lesion, was diagnosed with MEN1 syndrome after endoscopic ultrasound-guided sampling of the mass revealed pancreatic neuroendocrine tumor (pNET) and accompanying primary hyperparathyroidism (PHPT) and pituitary tumor. Genetic analysis by whole gene Sanger sequencing of the MEN1 gene identified a frame-shift mutation in exon 10 (c.1680_1683delTGAG). All the relatives of the index case were proposed for clinical and genetic evaluation for MEN1 syndrome. RESULTS: Of the 25 relatives of the index case, 17 were diagnosed with the MEN1 syndrome. Eighteen members among all relatives consented to genetic analysis, and 11 had the same mutation as the index case. All the mutation positive members had MEN1, while none of mutation-negative subjects had any sign of MEN1 syndrome. The frequencies of PHPT, pNET, and pituitary tumors in this kindred were 94.1% (16/17), 29.4% (5/17), and 29.4% (5/17) respectively. CONCLUSION: We report a rare MEN1 gene mutation which has been descibed in a single sporadic patient earlier. It was inherited by at least three generations of a large family, proving the strong dominant effect of the MEN1 phenotype. Further research may be conducted to clarify potential candidacy of this mutation as a hotspot for MEN1 patients, especially in the Turkish population.

Frulloni, Luca/0000-0001-7417-2655; Hart, Phil/0000-0003-4346-6196; Capurso, Gabriele/0000-0002-0019-8753; de-Madaria, Enrique/0000-0002-2412-9541; Lohr, Matthias/0000-0002-7647-198X; Frokjaer, Jens Brondum/0000-0001-8722-0070 WOS:000542363500001 PubMed: 32552502 The overall objective of these guidelines is to provide evidence-based recommendations for the diagnosis and management of immunoglobulin G4 (IgG4)-related digestive disease in adults and children. IgG4-related digestive disease can be diagnosed only with a comprehensive work-up that includes histology, organ morphology at imaging, serology, search for other organ involvement, and response to glucocorticoid treatment. Indications for treatment are symptomatic patients with obstructive jaundice, abdominal pain, posterior pancreatic pain, and involvement of extra-pancreatic digestive organs, including IgG4-related cholangitis. Treatment with glucocorticoids should be weight-based and initiated at a dose of 0.6-0.8 mg/kg body weight/day orally (typical starting dose 30-40 mg/day prednisone equivalent) for 1 month to induce remission and then be tapered within two additional months. Response to initial treatment should be assessed at week 2-4 with clinical, biochemical and morphological markers. Maintenance treatment with glucocorticoids should be considered in multi-organ disease or history of relapse. If there is no change in disease activity and burden within 3 months, the diagnosis should be reconsidered. If the disease relapsed during the 3 months of treatment, immunosuppressive drugs should be added. National Societies Committee of the United European Gastroenterology (UEG) We gratefully acknowledge the support from the National Societies Committee of the United European Gastroenterology (UEG) for the conduct of these guidelines independent from other sources. No other funding was received.

Pancreatic cystic lesions can be benign, premalignant or malignant. The recent increase in detection and tremendous clinical variability of pancreatic cysts has presented a significant therapeutic challenge to physicians. Mucinous cystic neoplasms are of particular interest given their known malignant potential. This review article provides a brief but comprehensive review of premalignant pancreatic cystic lesions with advanced endoscopic ultrasound (EUS) management approaches. A comprehensive literature search was performed using PubMed, Cochrane, OVID and EMBASE databases. Preneoplastic pancreatic cystic lesions include mucinous cystadenoma and intraductal papillary mucinous neoplasm. The 2012 International Sendai Guidelines guide physicians in their management of pancreatic cystic lesions. Some of the advanced EUS management techniques include ethanol ablation, chemotherapeutic (paclitaxel) ablation, radiofrequency ablation and cryotherapy. In future, EUS-guided injections of drug-eluting beads and neodymium:yttrium aluminum agent laser ablation is predicted to be an integral part of EUS-guided management techniques. In summary, International Sendai Consensus Guidelines should be used to make a decision regarding management of pancreatic cystic lesions. Advanced EUS techniques are proving extremely beneficial in management, especially in those patients who are at high surgical risk.