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Neuroprotective modulation of the unfolded protein response in Marinesco-Sj?gren syndrome: PERK signaling inhibition and beyond

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Neural Regeneration Research 2019年第1期14卷 62-64页

作者： Elena Restelli antonio masone Michele Sallese Roberto Chiesa Department of Neuroscience IRCCS–Istituto di Ricerche Farmacologiche Mario Negri Department of Medical Oral and Biotechnological Sciences Università "G. d’Annunzio" CeSI-MeT Center for Research on Ageing and Translational Medicine Università "G.d’Annunzio"

Individuals with Marinesco-Sj?gren syndrome(MSS;OMIM 248800),a genetic disease of infancy,suffer various disabilities,including loss of motor coordination due to cerebellar degeneration,and skeletal muscle *** a progressive phase,symptoms stabilize and patients live to old ***,any pharmacological treatment that delays or attenuates cerebellar degeneration and/or muscle pathology can significantly improve their quality of *** recently found that inhibiting the protein kinase RNA-like endoplasmic reticulum

关键词：蛋白质症候群信号抑制津贴反应调整治疗学调查结果

Intestinal amyloidosis:Two cases with different patterns of clinical and imaging presentation

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World Journal of Gastroenterology 2010年第20期16卷 2566-2570页

作者： Pier Paolo Mainenti Sabrina Segreto Marcello Mancini antonio Rispo Immacolata Cozzolino Stefania masone Ciro Roberto Rinaldi Gerardo Nardone Marco Salvatore IBB CNR NaplesVia Pansini 580131NaplesItaly Department of Biomorphological and Functional Sciences Section of RadiologyUniversity of Naples"Federico II"Via Pansini 580131NaplesItaly Department of Gastro-enterology University of Naples"Federico II"Via Pansini 580131NaplesItaly Department of Biomorphological and Functional Sciences Section of PathologyUniversity of Naples "Federico II"Via Pansini 580131NaplesItaly Department of General Surgery Geriatry and EndoscopyUniversity of Naples"Federico II"Via Pansini 580131NaplesItaly Department of Biotechnology Haema-tology UnitUniversity of Naples"Federico II"Via Pansini 580131NaplesItaly

The involvement of the small bowel in systemic forms of amyloidosis may be diffuse or very rarely *** cases of focal amyloidomas of the duodenum and jejunum without extraintestinal manifestations have been *** focal amyloidomas consisted of extensive amyloid infiltration of the entire intestinal wall *** barium studies,ultrasound and computed tomography(CT)patterns of diffuse small bowel amyloidosis have been described:the signs are non-specific and may include small-bowel dilatation,symmetric bowel wall thickening,mesenteric infiltration,and mesenteric *** data are available about the positron emission tomography (PET)/CT and magnetic resonance imaging(MRI)patterns of intestinal *** report two cases of small bowel amyloidosis:the former characterized by focal deposition of amyloid proteins exclusively within blood vessel walls of the terminal ileum,the latter characterized by diffuse intestinal involvement observed on MRI and PET/CT studies.

关键词： Amyloidosis Small bowel Positron emission tomography/computed tomography Magnetic resonance imaging Computed tomography

Lower gastrointestinal bleeding secondary to a rectal leiomyoma

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World Journal of Gastroenterology 2009年第14期15卷 1769-1770页

作者： Giovanni D De Palma Maria Rega Stefania masone Saverio Siciliano Marcello Persico Francesca Salvatori Francesco Maione Dario Esposito antonio Bellino Giovanni Persico Department of Surgery and Advanced Technologies Center for Technical Innovation in Surgery University of Naples Federico II School of Medicine Via Pansini 5 Naples 80131 Italy

The occurrence of leiomyoma of the rectum is uncommon. Most of these lesions are clinically silent and are found incidentally during laparotomy or endoscopic procedures for unrelated conditions. Symptomatic leiomyomas of the rectum are encountered less frequently, with only sporadic reports in the literature. We describe a case of a leiomyoma of the rectum presenting as recurrent lower gastrointestinal hemorrhage and secondary anemia.

关键词： Rectal leiomyoma Gastrointestinal bleeding Endoscopy Endoscopic ultrasonography Immunohistochemistry

Contrast-free endoscopic stent insertion in malignant biliary obstruction

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World Journal of Gastroenterology 2007年第29期13卷 3973-3976页

作者： Giovanni D De Palma Giovanni Lombardi Maria Rega Immacolata Simeoli Stefania masone Saverio Siciliano Francesco Maione Francesca Salvatori antonio Balzano Giovanni Persico Department of General Surgery Oncologic Surgery and Advanced Technologies-Center for Technical Innovation in Surgery University of Naples Federico Ⅱ School of Medicine Naples Italy Division of Gastroenterology General Hospital A. Cardarelli Naples Italy

AIM： To present a case series of MRCP-guided endoscopic biliary stent placement, performed entirely without contrast injection. METHODS： Contrast-free endoscopic biliary drainage was attempted in 20 patients with malignant obstruction, unsuitable for resection on the basis of tumor extent or medical illness. MRCP images were used to confirm the diagnosis of tumor, to exclude other biliary diseases and to demonstrate the stenoses as well as dilation of proximal liver segments. The procedure was carried out under conscious sedation. Patients were placed in the left lateral decubitus position. The endoscope was inserted, the papilla identified and cannulated by a papiUotome. A guide wire was inserted and guided deeply into the biliary tree, above the stenosis, by fluoroscopy. A papillotomy approximately 1 cm. long was performed and the papillotome was exchanged with a guiding-catheter. A 10 Fr＇ Amsterdam-type plastic stent, 7 to 15 cm long, was finally inserted over the guide wire/ guiding catheter by a pusher tube system. RESULTS： Successful stent insertion was achieved in all patients. There were no major complications. Successful drainage, with substantial reduction in bilirubin levels, was achieved in all patients. CONCLUSION： This new method of contrast-free endoscopic stenting in malignant biliary obstruction is a safe and effective method of palliation. However＇ a larger, randomized study comparing this new approach with the standard procedure is needed to confirm the findings of the present study.

关键词： Endoscopic retrograde cholangiopancreatography Biliary stenoses Biliary drainage Endoscopy Stents

Diagnostic accuracy of confocal laser endomicroscopy in diagnosing dysplasia in patients affected by long-standing ulcerative colitis

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World Journal of Gastrointestinal Endoscopy 2012年第9期4卷 414-420页

作者： antonio Rispo Fabiana Castiglione Stefania Staibano Dario Esposito Maria Siano Francesco Maione Francesca Salvatori Stefania masone Marcello Persico Giovanni Domenico De Palma Gastroenterology University 'Federico Ⅱ'of Naples80131 NaplesItaly Gastroenterology University 'Federico Ⅱ' of Naples80131 NaplesItaly Department of Biomorphological and Functional Sciences Section of PathologyUniversity 'Federico Ⅱ' of Naples80131 NaplesItaly Surgery and Advanced Technologies-University 'Federico Ⅱ' of Naples 80131 NaplesItaly Department of Biomorphological and Functional Sciences Section of PathologyUniversity'Federico Ⅱ'of Naples80131 NaplesItaly Surgery and Advanced Technologies-University 'Federico Ⅱ'of Naples 80131 NaplesItaly

AIM:To evaluate the diagnostic accuracy of confocal laser endomicroscopy(CLE) for the detection of dysplasia in long-standing ulcerative colitis(UC).METHODS:We prospectively performed a surveillance colonoscopy in 51 patients affected by long-standing ***,in the presence of macroscopic areas with suspected dysplasia,both targeted contrasted indigo carmine endoscopic assessment and probe-based CLE were *** mucosal biopsies and histology,utilised as the gold standard,were assessed randomly and on visible lesions,in accordance with current ***:Fourteen of the 51 patients(27%) showed macroscopic mucosal alterations with the suspected presence of dysplasia,needing chromoendoscopic and CLE *** 5 macroscopically suspected cases,the presence of dysplasia was confirmed by histology(3 flat dysplasia;2 DALMs).No dysplasia/cancer was found on any of the outstanding random *** diagnostic accuracy of CLE for the detection of dysplasia compared to standard histology was sensitivity 100%,specificity 90%,positive predictive value 83% and negative predictive value 100%.CONCLUSION:CLE is an accurate tool for the detection of dysplasia in long-standing UC and shows optimal values of sensitivity and negative *** scheduled combined application of chromoendoscopy and CLE could maximize the endoscopic diagnostic accuracy for diagnosis of dysplasia in UC patients,thus limiting the need for biopsies.

关键词： Ulcerative colitis Cancer Confocal Surveillance