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Gastrointestinal manifestations in myotonic muscular dystrophy

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World Journal of Gastroenterology 2006年第12期12卷 1821-1828页

作者： Massimo Bellini Sonia Biagi Cristina Stasi francesco costa Maria Gloria Mumolo Angelo Ricchiuti Santino Marchi Gastroenterology Unit Department of Internal MedicineUniversity of PisaItaly

Myotonic dystrophy （MD） is characterized by myotonic phenomena and progressive muscular weakness. Involvement of the gastrointestinal tract is frequent and may occur at any level. The clinical manifestations have previously been attributed to motility disorders caused by smooth muscle damage, but histologic evidence of alterations has been scarce and conflicting. A neural factor has also been hypothesized. In the upper digestive tract, dysphagia, heartburn, regurgitation and dyspepsia are the most common complaints, while in the lower tract, abdominal pain, bloating and changes in bowel habits are often reported. Digestive symptoms may be the first sign of dystrophic disease and may precede the rnusculo-skeletal features. The impairment of gastrointestinal function may be sometimes so gradual that the patients adapt to it with little awareness of symptoms. In such cases routine endoscopic and ultrasonographic evaluations are not sufficient and targeted techniques （electrogastrography, manornetry, electromyography, functional ultrasonography, scintigraphy, etc.） are needed. There is a low correlation between the degree of skeletal muscle involvement and the presence and severity of gastrointestinal disturbances whereas a positive correlation with the duration of the skeletal muscle disease has been reported. The drugs recommended for treating the gastrointestinal complaints such as prokinetic, antidyspeptic drugs and laxatives, are mainly aimed at correcting the motility disorders. Gastrointestinal involvement in MD remains a complex and intriguing condition since many important problems are still unsolved. Further studies concentrating on genetic aspects, early diagnostic techniques and the development of new therapeutic strategies are needed to improve our management of the gastrointestinal manifestations of MD.

关键词： Myotonic dystrophy Digestive disorders Gastrointestinal symptoms

From bench to bedside: Fecal calprotectin in inflammatory bowel diseases clinical setting

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World Journal of Gastroenterology 2018年第33期24卷 3681-3694页

作者： Maria Gloria Mumolo Lorenzo Bertani Linda Ceccarelli Gabriella Laino Giorgia Di Fluri Eleonora Albano Gherardo Tapete francesco costa Department of General Surgery and Gastroenterology Gastroenterology Unit Azienda Ospedaliero Universitaria Pisana Department of New Technologies and Translational Research in Medicine and Surgery University of Pisa

Fecal calprotectin(FC) has emerged as one of the most useful tools for clinical management of inflammatory bowel diseases(IBD). Many different methods of assessment have been developed and different cutoffs have been suggested for different clinical settings. We carried out a comprehensive literature review of the most relevant FC-related topics: the role of FC in discriminating between IBD and irritable bowel syndrome(IBS) and its use in managing IBD patients In patients with intestinal symptoms, due to the high negative predictive value a normal FC level reliably rules out active IBD. In IBD patients a correlation with both mucosal healing and histology was found, and there is increasing evidence that FC assessment can be helpful in monitoring disease activity and response to therapy as well as in predicting relapse, post-operative recurrence or pouchitis. Recently, its use in the context of a treat-to-target approach led to a better outcome than clinically-based therapy adjustment in patients with early Crohn's disease. In conclusion, FC measurement represents a cheap, safe and reliable test, easy to perform and with a good reproducibility. The main concerns are still related to the choice of the optimal cut-off, both for differentiating IBD from IBS, and for the management of IBD patients.

关键词： Fecal calprotectin Inflammatory bowel diseases Crohn's disease Ulcerative colitis Irritable

Evaluation of latent links between irritable bowel syndrome and sleep quality

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World Journal of Gastroenterology 2011年第46期17卷 5089-5096页

作者： Massimo Bellini Angelo Gemignani Dario Gambaccini Simona Toti Danilo Menicucci Cristina Stasi francesco costa Maria Gloria Mumolo Angelo Ricchiuti Remo Bedini Nicola de Bortoli Santino Marchi Gastrointestinal Unit Department of GastroenterologyUniversity of Pisa Institute of Clinical Physiology National Research Council Extreme Centre Scuola Superiore S Anna Department of Physiological Science University of Pisa Italian National Institute of Statistics

AIM:To examine the links between quality of sleep and the severity of intestinal symptoms in irritable bow-el syndrome(IBS).METHODS:One hundred and forty-two outpatients(110female,32 male)who met the Rome Ⅲ criteria for IBS with no psychiatric comorbidity were consecutively en-rolled in this *** on age,body mass index(BMI),and a set of life-habit variables were recorded,and IBS symptoms and sleep quality were evaluated using the questionnaires IBS Symptom Severity Score(IBS-SSS)and Pittsburgh Sleep Quality Index(PSQI).The associa-tion between severity of IBS and sleep disturbances was evaluated by comparing the global IBS-SSS and PSQI score(Pearson's correlation and Fisher's exact test)and then analyzing the individual items of the IBS-SSS and PSQI questionnaires by a unitary bowel-sleep model based on item response theory(IRT).RESULTS:IBS-SSS ranged from mild to severe(120-470).The global PSQI score ranged from 1 to 17(median 5),and 60 patients were found to be poor sleepers(PSQI>5).The correlation between the global IBS-SSS and PSQI score indicated a weak association(r=0.2 and 95% CI:-0.03 to 0.35,P<0.05),which becomes stronger using our unitary ***,the IBS and sleep disturbances severities,estimated as latent variables,resulted significantly high intra-subject cor-relation(posterior mean of r=0.45 and 95% CI:0.17 to 0.70,P<0.05).Moreover,the correlations between patient features(age,sex,BMI,daily coffee and alcohol intake)and IBS and sleep disturbances were also ana-lyzed through our unitary *** was a signif icant regressor,with patients≤50 years old showing more severe bowel disturbances(posterior mean=-0.38,P<0.05)and less severe sleep disturbances(posterior mean=0.49,P<0.05)than older *** daily coffee intake was correlated with a lower sever-ity of bowel disturbances(posterior mean=-0.31,P<0.05).Sex(female)and daily alcohol intake(modest)were correlated with less severe sleep ***:The unitary bowel-sleep mod

关键词： Irritable bowel syndrome Sleep disorders Item response theory model Bayesian model

How many cases of laryngopharyngeal reflux suspected by laryngoscopy are gastroesophageal reflux disease-related?

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World Journal of Gastroenterology 2012年第32期18卷 4363-4370页

作者： Nicola de Bortoli Andrea Nacci Edoardo Savarino Irene Martinucci Massimo Bellini Bruno Fattori Linda Ceccarelli francesco costa Maria Gloria Mumolo Angelo Ricchiuti Vincenzo Savarino Stefano Berrettini Santino Marchi Division of Gastroenterology University of Pisa56124 PisaItaly Ear Nose and Throat Audiology Phoniatrics UnitUniversity of Pisa56124 PisaItaly Division of Gastroenterology University of Padua35100 PaduaItaly Division of Gastroenterology University of Genoa16100 GenoaItaly

AIM:To investigate the prevalence of gastroesophageal reflux disease(GERD) in patients with a laryngoscopic diagnosis of laryngopharyngeal reflux(LPR).METHODS:Between May 2011 and October 2011,41 consecutive patients with laryngopharyngeal symptoms(LPS) and laryngoscopic diagnosis of LPR were empirically treated with proton pump inhibitors(PPIs) for at least 8 wk,and the therapeutic outcome was assessed through validated questionnaires(GERD impact scale,GIS;visual analogue scale,VAS).LPR diagnosis was performed by ear,nose and throat specialists using the reflux finding score(RFS) and reflux symptom index(RSI).After a 16-d wash-out from PPIs,all patients underwent an upper endoscopy,stationary esophageal manometry,24-h multichannel intraluminal impedance and pH(MII-pH) esophageal monitoring.A positive correlation between LPR diagnosis and GERD was supposed based on the presence of esophagitis(ERD),pathological acid exposure time(AET) in the absence of esophageal erosions(NERD),and a positive correlation between symptoms and refluxes(hypersensitive esophagus,HE).RESULTS:The male/female ratio was 0.52(14/27),the mean age ± SD was 51.5 ± 12.7 years,and the mean body mass index was 25.7 ± 3.4 kg/m *** subjects reported one or more ***-five out of 41 patients also had typical GERD symptoms(heartburn and/or regurgitation).The most frequent laryngoscopic findings were posterior laryngeal hyperemia(38/41),linear indentation in the medial edge of the vocal fold(31/41),vocal fold nodules(6/41) and diffuse infraglottic oedema(25/41).The GIS analysis showed that 10/41 patients reported symptom relief with PPI therapy(P < 0.05);conversely,23/41 did not report any clinical *** the same time,the VAS analysis showed a significant reduction in typical GERD symptoms after PPI therapy(P < 0.001).A significant reduction in LPS *** the other hand,such result was not recorded for *** was detected in 2/41 patients,and ineffective esophageal motility

关键词： Laryngopharyngeal reflux Gastroesophageal reflux Multichannel impedance and pH monitoring Extra-esophageal reflux syndromes Chronic laryngitis

Intestinal pseudo-obstruction in inactive systemic lupus erythematosus: An unusual finding

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World Journal of Gastrointestinal Pharmacology and Therapeutics 2010年第6期1卷 135-136页

作者： Giulia Leonardi Nicola de Bortoli Massimo Bellini Maria Gloria Mumolo francesco costa Angelo Ricchiuti Stefano Bombardieri Santino Marchi Gastroenterology Unit Cisanello Hospital University of Pisa Reumatology Unit Hospital of Santa Chiara University of Pisa

Chronic intestinal pseudo-obstruction (CIP) is an infre-quent complication of an active systemic lupus erythema-tosus (SLE). We illustrate a case of SLE inactive-related CIP. A 51-year old female with inactive SLE (ECLAM score 2) was hospitalized with postprandial fullness, vomiting, abdominal bloating and abdominal pain. She had had no bowel movements for five days. Plain abdominal X-ray revealed multiple fluid levels and dilated small and large bowel loops with air-fluid levels. Intestinal contrast radiology detected dilated loops. CIP was diagnosed. The patient was treated with prokinetics, octreotide, claritromycin, rifaximin, azathioprine and tegaserod without any clinical improvement. Then methylprednisolone (500 mg iv daily) was started. After the first administration, the patient showed peristaltic movements. A bowel movement was reported after the second administration. A plain abdominal X-ray revealed no air-fluid levels. Steroid therapy was slowly reduced with complete resolution of the symptoms. The patient is still in a good clinical condition. SLE-related CIP is generally reported as a complication of an active disease. In our case, CIP was the only clinical demonstration of the SLE.

关键词： Chronic intestinal pseudo-obstruction Systemic lupus erythematosus

Cognitive training in neurodegenerative diseases: a way to boost neuroprotective molecules?

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Neural Regeneration Research 2015年第11期10卷 1754-1755页

作者： francesco Angelucci Carlo Caltagirone Alberto costa IRCCS Santa Lucia Foundation Department of Clinical and Behavioural Neurology Department of Systemic Medicine Rome University Tor Vergata Niccolò Cusano University

Neurotrophins,and growth factors in general,are proteins which exert many actions in central nervous system *** promote the survival and neuronal function of neurons and exert neuroprotective effects in pathological models of neurodegenerative *** humans several clinical trials based on the use of these proteins have been carried out. Among them, it is worth to mention gene ther- apy especially in Parkinson＇s disease （PD）（Kordower and Bjorklund, 2013）, and a series of experiments using vectors capable of carrying neurotrophins in the selected target re- gion where they can exert their beneficial action （Ghosh et al., 2014）.

关键词： BDNF a way to boost neuroprotective molecules Cognitive training in neurodegenerative diseases

Metabolomics characterization of colostrum in three sow breeds and its influences on piglets' survival and litter growth rates

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Journal of Animal Science and Biotechnology 2018年第3期9卷 618-629页

作者： Gianfranco Picone Martina Zappaterra Diana Luise Alessia Trimigno francesco Capozzi Vincenzo Motta Roberta Davoli Leonardo Nanni costa Paolo Bosi Paolo Trevisi Department of Agricultural and Food Sciences (DISTAL) Alma Mater Studiorum-University of Bologna

Background: Colostrum is the first secretion produced by mammary glands during the hours immediately preceding and succeeding parturition. This secretion differs from milk and represents an essential vehicle of passive immunity,prebiotic compounds and growth factors involved in intestinal development. Most of the literature concerning colostrum composition refers mainly to human and cow; and little is known about pig colostrum metabolome and how it varies between pig breeds and different farrowing parity. Thus, the aim of the present research is to provide new information about pig colostrum composition and the associations between metabolites, the sows' breed and the survival and growth rates of their ***: Colostrum samples were gathered from 58 parturitions of sows belonging to three different breeds chosen for their importance in Italian heavy pig production: 31 Large White, 15 Landrace and 12 Duroc respectively. The defatted and ultrafiltered colostrum samples were analysed using1 H–NMR spectroscopy. Principal Components Analysis(PCA) was assessed on the obtained spectra. In addition, using a Stepwise Regression and a Linear Regression analyses the metabolites named after the signals assignment were tested for their associations with piglets' ***-five metabolites were identified, comprehending monosaccharides, disaccharides(such as lactose), organic acids(lactate, citrate, acetate and formate), nitrogenous organic acids(such as creatine) and other compounds,including nucleotides. PCA results evidence a clustering due to breed and season effects. Lactose was the main compound determining the assignment of the samples into different clusters according to the sow breed. Furthermore, some metabolites showed to be associated with piglets' performance and survival traits: acetate and taurine were positively related to litter weight gain and piglets' survival rate, respectively, while dimethylamine and cis-aconitate were linked to new-borns'

关键词： Colostrum 1H–NMR spectroscopy Metabolome Pig breeds Piglets survival

Knockdown of MLO genes reduces susceptibility to powdery mildew in grapevine

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Horticulture Research 2016年第1期3卷 248-256页

作者： Stefano Pessina Luisa Lenzi Michele Perazzolli Manuela Campa Lorenza Dalla costa Simona Urso Giampiero Valè francesco Salamini Riccardo Velasco Mickael Malnoy Research and Innovation Centre Fondazione Edmund Machvia Edmund Mach 1San Michele all’Adige38010Italy Wageningen UR Plant Breeding Wageningen University and Research CentrePO Box 386Wageningen6700AJThe Netherlands Department of Ecological and Biological Sciences Universitàdella TusciaVia San Camillo de LellisViterbo01100Italy Council for Agricultural Research and Economics(CREA) Rice Research UnitS.S.11 per Torino km 25Vercelli13100Italy Council for Agricultural Research and Economics(CREA) Genomics Research CentreVia San Protaso302Fiorenzuola d’Arda29017PiacenzaItaly

Erysiphe necator is the causal agent of powdery mildew(PM),one of the most destructive diseases of *** is controlled by sulfur-based and synthetic fungicides,which every year are dispersed into the *** is why PM-resistant varieties should become a priority for sustainable grapevine and wine *** resistance can be achieved in other crops by knocking out susceptibility S-genes,such as those residing at genetic loci known as MLO(Mildew Locus O).All MLO S-genes of dicots belong to the phylogenetic clade V,including grapevine genes VvMLO7,11 and 13,which are upregulated during PM infection,and VvMLO6,which is not *** adopting a gene-editing approach to knockout candidate S-genes,the evidence that loss of function of MLO genes can reduce PM susceptibility is *** paper reports the knockdown through RNA interference of VvMLO6,7,11 and *** knockdown of VvMLO6,11 and 13 did not decrease PM severity,whereas the knockdown of VvMLO7 in combination with VvMLO6 and VvMLO11 reduced PM severity up to 77%.The knockdown of VvMLO7 and VvMLO6 seemed to be important for PM resistance,whereas a role for VvMLO11 does not seem *** wall appositions(papillae)were present in both resistant and susceptible lines in response to PM *** genes involved in defense were less upregulated in infected mlo plants,highlighting the early mlo-dependent disruption of PM invasion.

关键词： MLO crops mildew

Nutritional value of a partially defatted and a highly defatted black soldier fly larvae (Hermetia illucens L.) meal for broiler chickens: apparent nutrient digestibility,apparent metabolizable energy and apparent ileal amino acid digestibility

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Journal of Animal Science and Biotechnology 2017年第4期8卷 897-905页

作者： Achille Schiavone Michele De Marco Silvia Martínez Sihem Dabbou Manuela Renna Josefa Madrid Fuensanta Hernandez Luca Rotolo Pierluca costa francesco Gai Laura Gasco Department of Veterinary Sciences University of Turin Largo Paolo Braccini 2 10095 Grugliasco TO Italy Department of Animal Production University of Murcia Campus de Espinardo 30071 Murcia Spain Department of Agricultural Forest and Food Sciences University of Turin Largo Paolo Braccini 2 10095 Grugliasco TO Italy Institute of Science of Food Production National Research Council Largo Paolo Braccini 2 10095 Grugliasco TO Italy

Background: The study aimed to determine the apparent total tract digestibility coefficients(ATTDC) of nutrients, the apparent metabolizable energy(AME and AMEn) and the amino acid(AA) apparent ileal digestibility coefficients(AIDC)of a partially defatted(BSFp) and a highly defatted(BSFh) black soldier fly larvae meal. The experimental diets were: a basal diet and two diets prepared by substituting 250 g/kg(w/w) of the basal diet with BSFp or BSFh, ***: Significant differences were found between BSFp and BSFh meals for ATTDC of the nutrients: BSFp resulted more digestible than BSFh, except for ATTDC of CP which did not differed between meals, while a statistical trend was observed for ATTDC of DM and EE. The AME and AMEn values were significantly(P < 0.05) different between the two BSF meals, with higher levels for BSFp(16.25 and 14.87 MJ/kg DM, respectively). The AIDC of the AA in BSFp ranged from 0.44 to 0.92, while in BSFh they ranged from 0.45 to 0.99. No significant differences were observed for the AA digestibility(0.77 and 0.80 for BSFp and BSFh, respectively), except for glutamic acid, proline and serine that were more digestible in the BSFh meal(P < 0.05).Conclusions: Defatted BSF meals can be considered as an excellent source of AME and digestible AA for broilers with a better efficient nutrient digestion. These considerations suggested the effective utilization of defatted BSF larvae meal in poultry feed formulation.

关键词： Amino acid Apparent digestibility Black soldier fly meal Broiler chicken Metabolizable energy