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    Iperenzimemia Pancreatica in Pazienti Asintomatici: Alterazioni Morfo-Funzionali del Pancreas e Studio di Patologie Associate.Follow-up.

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    Premesse: L’ iperenzimemia pancreatica cronica asintomatica (CAPH) è stata descritta dal 1996 come una condizione benigna. Recenti studi descrivono reperti patologici alla Risonanza Magnetica Colangiopancreatografia con stimolo secretinico (s-MRCP) in più della metà dei soggetti portatori di CAPH. Nessuno studio di follow-up è stato condotto finora in tali soggetti. Obiettivo: indagare la frequenza e la rilevanza clinica dei reperti alla s-MRCP in pazienti con CAPH; rivalutare i soggetti dopo anni per valutare eventuali modificazioni radiologiche nel tempo e/o comparsa di sintomi. Metodi: I soggetti, arruolati dal Gennaio 2005 al Dicembre 2010, sono stati sottoposti a s-MRCP ed esami bioumorali, e successivamente una parte di essi rivalutati in follow-up. Risultati: Sono stati analizzati i dati relativi a 160 soggetti (94 maschi, 66 femmine, età 49,6 ± 13,6 anni). Cinquantuno (32%) soggetti avevano iperamilasemia, 9 (6%) iperlipasemia, 100 (62%), un aumento in entrambi i livelli degli enzimi. Una iperenzimemia pancreatica familiare è stata osservata in 26 dei 133 soggetti (19,5%). Anomalie anatomiche del sistema duttale pancreatico alla s-MRCP sono state riscontrate in 24 dei 160 soggetti (15%). Risultati patologici erano presenti in 44 soggetti (27,5%) prima e in 80 (50%) dopo somministrazione di secretina (p <0,0001). Cinque soggetti (3,1%) sono stati sottoposti a intervento chirurgico, 3 per il tumore del pancreas endocrino, 1 per l'adenocarcinoma del pancreas e 1 per neoplasia mucinosa papillare intraduttale (IPMN) del dotto pancreatico principale e in 18 pazienti (11,3%) è stato indicato un follow-up (17 per IPMN e 1 per tumore endocrino). Nello studio di follow-up abbiamo ottenuto dati clinici di 107 soggetti e una rivalutazione radiologica mediante MRCP in 66 soggetti. Un solo soggetto ha presentato una pancreatite acuta dopo 5 anni dal primo riscontro di iperenzimemia. Un solo soggetto su 11 con IPMN aveva una progressione delle dimensioni della cisti, 1 su 10 con SOD presentava peggioramento della dilatazione del dotto di Wirsung, mentre si assisteva a progressione della pancreatite cronica nel 11% dei soggetti dopo follow-up medio di 3 ± 2 anni. Conclusioni: alterazioni del sistema dotto pancreatico alla s-MRCP in soggetti con CAPH possono essere osservate nel 50% dei soggetti e sono clinicamente rilevanti nel 14,4% dei casi. Le alterazioni funzionali riscontrate (SOD, sospetta pancreatite cronica) solo in modesta parte evolvono verso la cronicizzazione di malattia.Background: Chronic asymptomatic pancreatic hyperenzymemia (CAPH) has been described since 1996 as a benign disease. Recent studies described pathological findings at Magnetic Resonance Cholangio-Pancreatography with secretin stimulation in more than a half of the CAPH subjects. No study of follow-up was conducted so far in these subjects. Aim: to investigate the frequency and clinical relevance of s-MRCP findings in patients with CAPH; reevaluate the subjects after years to assess any radiological changes in time and / or appearance of symptoms. Methods: Subjects prospectively enrolled from January 2005 to December 2010 underwent s-MRCP and biochemical tests routinely performed, and then a part of them re-evaluated in follow-up. Results: Data relative to 160 subjects (94 males, 66 females, age 49.6 ± 13.6 years) were analyzed. Fifty-one (32%) subjects had hyperamylasemia, 9 (6%) hyperlipasemia, 100 (62%) an increase in both enzyme levels. Familial pancreatic hyperenzymemia was observed in 26 out of 133 subjects (19.5%). Anatomic abnormalities of the pancreatic duct system at s-MRCP were found in 24 out of 160 subjects (15%). Pathological MRCP findings were present in 44 subjects (27.5%) before and in 80 (50%) after secretin administration (p<0.0001). Five subjects (3.1%) underwent surgery, 3 for pancreatic endocrine tumor, 1 for pancreatic adenocarcinoma and 1 for intraductal papillary-mucinous neoplasia (IPMN) involving the main pancreatic duct and 18 patients (11.3%) needed a follow-up (17 for IPMN and 1 for endocrine tumor). In the follow-up study we obtained clinical data of 107 subjects and a radiological evaluation by MRCP in 66 subjects. One subject experienced acute pancreatitis after 5 years from the first detection of hyperenzymemia. Only one subject out of 11 with IPMN had progression of the size of the cyst, 1 out of 10 with SOD showed worsening of Wirsung duct dilation, while we observed a progression of chronic pancreatitis in 11% of subjects after mean follow-up of 3 ± 2 years. Conclusions: Alterations of the pancreatic duct system at s-MRCP in subjects with CAPH can be observed in 50% of the subjects and are clinically relevant in 14.4% of cases. Only a small part of the “functional” alterations observed at S-MRCP (SOD, suspected chronic pancreatitis) evolves into a chronic form of the disease

    Systemic fibroinflammatory disorders

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    clinical aspects, diagnosis, therapy., and clinical outcome of autoimmune pancreatiti

    Italian Experience

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    Autoimmune pancreatitis (AIP) is now a well-defined disease of the pancreas that differs clinically, radiologically, and therapeutically from all the other inflammatory diseases of the pancreas. It has been recently histologically classified in type 1 and type 2 AIPs, not different at imaging, but with a better clinical outcome in type 2 compared to type 1. From a clinical point of view, the key point in Italy is to differentiate focal form of the disease from pancreatic cancer. The Italian diagnostic algorithm is therefore addressed to exclude cancer in this setting and, therefore, to safely use steroids. In diffuse form of the disease, the clinical problem is to differentiate AIP from acute nonnecrotizing pancreatitis and to exclude neoplasia (cholangiocarcinoma or lymphoma) in some cases. In this chapter, we report the clinical profile of an Italian series of patients with AIP and highlight the main radiological and pathological findings

    Le malattie autoimmuni

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    Aspetti patologici, criteri diagnostici, epidemiologia e manifestazioni cliniche; imaging e terapia della pancreatite autoimmun

    Going Beyond Counting First Authors in Author Co-citation Analysis

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    The present study examines one of the fundamental aspects of author co-citation analysis (ACA) - the way co-citation counts are defined. Co-citation counting provides the data on which all subsequent statistical analyses and mappings are based, and we compare ACA results based on two different types of co-citation counting - the traditional type that only counts the first one among a cited work's authors on the one hand and a non-traditional type that takes into account the first 5 authors of a cited work on the other hand. Results indicate that the picture produced through this non-traditional author co-citation counting contains more coherent author groups and is therefore considerably clearer. However, this picture represents fewer specialties in the research field being studied than that produced through the traditional first-author co-citation counting when the same number of top-ranked authors is selected and analyzed. Reasons for these effects are discussed

    Updates in the field of autoimmune pancreatitis: a clinical guide

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    Autoimmune Pancreatitis (AIP) is a peculiar form of pancreatitis different from all other type of pancreatitis ('one like no one pancreatitis') and characterized by a dramatic response to steroids. AIP can be classified histologically in type 1, included in a larger group of diseases named IgG4-related disease, and type 2. At imaging, AIP may involve all (diffuse form) or only a part (focal form) of the pancreatic gland. Areas covered: In this article, the clinical approach to diagnosis and different therapeutic strategies of this complicated disease are reviewed. Literature search was undertaken using PubMed database entering autoimmune pancreatitis [title] or IgG4-related diseases [title], and selecting the more relevant papers for diagnosis and therapy of AIP in clinical practice. We focus on diagnosis of AIP in focal and diffuse form, and how to achieve diagnosis for type 1 and type 2 AIP. We finally analyzed the different strategies proposed in induction of remission and maintenance therapy (long-term low-dose steroids, immunosuppressants or biologics). Expert commentary: The main issue in clinical practice is how to achieve the diagnosis. A second key point is how to prevent the disease relapse
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