48 results on '"Pironi, Loris"'
Search Results
2. Liraglutide 3.0 mg and mental health: can psychiatric symptoms be associated to adherence to therapy? Insights from a clinical audit
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Tempia Valenta, Silvia, Stecchi, Michele, Perazza, Federica, Nuccitelli, Chiara, Villanova, Nicola, Pironi, Loris, Atti, Anna Rita, and Petroni, Maria Letizia
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- 2023
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3. Symptoms of SARS-CoV-2 infection and vaccine status of sixty-seven adult patients affected by inherited metabolic diseases: a phone survey
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Brodosi, Lucia, Stecchi, Michele, Mita, Dorina, Marchignoli, Francesca, Guarneri, Valeria, Agnelli, Giulio, Osti, Valentino, Perazza, Federica, Sacilotto, Federica, Pession, Andrea, and Pironi, Loris
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- 2023
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4. Baseline Characteristics of Adult Patients Treated and Never Treated with Teduglutide in a Multinational Short Bowel Syndrome and Intestinal Failure Registry.
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Gondolesi, Gabriel E., Pape, Ulrich-Frank, Mason, Joel B., Allard, Johane P., Pironi, Loris, Casas, María Núria Virgili, Schwartz, Lauren K., Joly, Francisca, Gabriel, André, Sabrdaran, Sasan, Zhang, Pinggao, Kohl-Sobania, Martina, Huang, Yi-Wen, and Jeppesen, Palle B.
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The Short Bowel Syndrome (SBS) Registry (NCT01990040) is a multinational real-world study evaluating the long-term safety of teduglutide in patients with SBS and intestinal failure (SBS-IF) in routine clinical practice. This paper describes the study methodology and baseline characteristics of adult patients who have (ever-treated) or have never (never-treated) received teduglutide. A total of 1411 adult patients (679 ever-treated; 732 never-treated) were enrolled at 124 sites across 17 countries. The mean (standard deviation [SD]) age at enrollment was 55.4 (15.46) years, and 60.2% of patients were women. Crohn's disease was the most common cause of major intestinal resection in both ever-treated (34.1%) and never-treated patients (20.4%). A similar proportion of ever-treated and never-treated patients had a prior history of colorectal polyps (2.7% vs. 3.6%), whereas proportionally fewer ever-treated patients reported a history of colorectal cancer (1.8% vs. 6.2%) or any malignancy (17.7% vs. 30.0%) than never-treated patients. Never-treated patients received a numerically greater mean (SD) volume of parenteral nutrition and/or intravenous fluids than ever-treated patients (12.4 [8.02] vs. 10.1 [6.64] L/week). Ever-treated patients received a mean teduglutide dosage of 0.05 mg/kg/day. This is the first report of patient baseline characteristics from the SBS Registry, and the largest cohort of patients with SBS-IF to date. Overall, ever-treated and never-treated patients had similar baseline characteristics. Differences between treatment groups may reflect variations in patient selection and degree of monitoring. [ABSTRACT FROM AUTHOR]
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- 2024
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5. NEUROD1 mutation in an Italian patient with maturity onset diabetes of the young 6: a case report
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Brodosi, Lucia, Baracco, Bianca, Mantovani, Vilma, and Pironi, Loris
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- 2021
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6. Home parenteral nutrition with an omega-3-fatty-acid-enriched MCT/LCT lipid emulsion in patients with chronic intestinal failure (the HOME study): study protocol for a randomized, controlled, multicenter, international clinical trial
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Klek, Stanislaw, Chambrier, Cécile, Cooper, Sheldon C., Gabe, Simon, Kunecki, Marek, Pironi, Loris, Rahman, Farooq, Sobocki, Jacek, Szczepanek, Kinga, Wanten, Geert, Lincke, Nicole, Glotzbach, Bernhard, and Forbes, Alastair
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- 2019
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7. Experience and opinions relating to pregnancy in patients with chronic intestinal failure: an international survey
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Bond, Ashley, primary, Allan, Philip, additional, Conley, Thomas Edward, additional, Farrer, Kirstine, additional, Mackillop, Lucy, additional, Bozzetti, Federico, additional, Cuerda, Cristina, additional, Jeppesen, Palle, additional, Joly, Francisca, additional, Lamprecht, Georg, additional, Mundi, Manpreet, additional, Szczepanek, Kinga, additional, Van Gossum, Andre, additional, Wanten, Geert, additional, Pironi, Loris, additional, and Lal, Simon, additional
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- 2023
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8. Anatomical Laser Microdissection of the Ileum Reveals mtDNA Depletion Recovery in A Mitochondrial Neuro-Gastrointestinal Encephalomyopathy (MNGIE) Patient Receiving Liver Transplant
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Boschetti, Elisa, primary, Caporali, Leonardo, additional, D’Angelo, Roberto, additional, Malagelada, Carolina, additional, Accarino, Anna, additional, Dotti, Maria Teresa, additional, Costa, Roberta, additional, Cenacchi, Giovanna, additional, Pironi, Loris, additional, Rinaldi, Rita, additional, Stanghellini, Vincenzo, additional, Ratti, Stefano, additional, Manzoli, Lucia, additional, Carelli, Valerio, additional, and De Giorgio, Roberto, additional
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- 2022
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9. Chronic Intestinal Failure in Children: An International Multicenter Cross-Sectional Survey
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Lezo, Antonella, primary, Diamanti, Antonella, additional, Marinier, Evelyne M., additional, Tabbers, Merit, additional, Guz-Mark, Anat, additional, Gandullia, Paolo, additional, Spagnuolo, Maria I., additional, Protheroe, Sue, additional, Peretti, Noel, additional, Merras-Salmio, Laura, additional, Hulst, Jessie M., additional, Kolaček, Sanja, additional, Ee, Looi C., additional, Lawrence, Joanna, additional, Hind, Jonathan, additional, D’Antiga, Lorenzo, additional, Verlato, Giovanna, additional, Pukite, Ieva, additional, Di Leo, Grazia, additional, Vanuytsel, Tim, additional, Doitchinova-Simeonova, Maryana K., additional, Ellegard, Lars, additional, Masconale, Luisa, additional, Maíz-Jiménez, María, additional, Cooper, Sheldon C., additional, Brillanti, Giorgia, additional, Nardi, Elena, additional, Sasdelli, Anna S., additional, Lal, Simon, additional, and Pironi, Loris, additional
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- 2022
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10. Evidence of enteric angiopathy and neuromuscular hypoxia in patients with mitochondrial neurogastrointestinal encephalomyopathy
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Boschetti, Elisa, primary, D’Angelo, Roberto, additional, Tardio, Maria Lucia, additional, Costa, Roberta, additional, Giordano, Carla, additional, Accarino, Anna, additional, Malagelada, Carolina, additional, Clavenzani, Paolo, additional, Tugnoli, Vitaliano, additional, Caio, Giacomo, additional, Righi, Valeria, additional, Garone, Caterina, additional, D'Errico, Antonietta, additional, Cenacchi, Giovanna, additional, Dotti, Maria Teresa, additional, Stanghellini, Vincenzo, additional, Sternini, Catia, additional, Pironi, Loris, additional, Rinaldi, Rita, additional, Carelli, Valerio, additional, and De Giorgio, Roberto, additional
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- 2021
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11. Home parenteral nutrition provision modalities for chronic intestinal failure in adult patients: An international survey
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Pironi, Loris, Steiger, Ezra, Brandt, Chrisoffer, Joly, Francisca, Wanten, Geert, Chambrier, Cecile, Aimasso, Umberto, Sasdelli, Anna Simona, Zeraschi, Sarah, Kelly, Darlene, Szczepanek, Kinga, Jukes, Amelia, Di Caro, Simona, Theilla, Miriam, Kunecki, Marek, Daniels, Joanne, Serlie, Mireille, Poullenot, Florian, Wu, Jian, Cooper, Sheldon C, Rasmussen, Henrik H, Compher, Charlene, Seguy, David, Crivelli, Adriana, Pagano, Maria C, Hughes, Sarah-Jane, Guglielmi, Francesco W, Kozjek, Nada Rotovnik, Schneider, Stéphane M, Gillanders, Lyn, Ellegard, Lars, Thibault, Ronan, Matras, Przemysław, Zmarzly, Anna, Matysiak, Konrad, Van Gossum, Andrè, Forbes, Alastair, Wyer, Nicola, Taus, Marina, Virgili, Nuria M, O'Callaghan, Margie, Chapman, Brooke, Osland, Emma, Cuerda, Cristina, Sahin, Peter, Jones, Lynn, Won Lee, Andre Dong, Masconale, Luisa, Orlandoni, Paolo, Izbéki, Ferenc, Spaggiari, Corrado, Bueno, Marta, Doitchinova-Simeonova, Maryana, Garde, Carmen, Serralde-Zúñiga, Aurora E, Olveira, Gabriel, Krznaric, Zeljko, Czako, Laszlo, Kekstas, Gintautas, Sanz-Paris, Alejandro, Jáuregui, Estrella Petrina, Murillo, Ana Zugasti, Schafer, Eszter, Arends, Jann, Suárez-Llanos, José P, Lal, Simon, Home Artificial Nutrition and Chronic Intestinal Failure Special Interest Group of ESPEN, European Society for Clinical Nutrition and Metabolism, Pironi L., Steiger E., Brandt C., Joly F., Wanten G., Chambrier C., Aimasso U., Sasdelli A.S., Zeraschi S., Kelly D., Szczepanek K., Jukes A., Di Caro S., Theilla M., Kunecki M., Daniels J., Serlie M., Poullenot F., Wu J., Cooper S.C., Rasmussen H.H., Compher C., Seguy D., Crivelli A., Pagano M.C., Hughes S.-J., Guglielmi F.W., Kozjek N.R., Schneider S.M., Gillanders L., Ellegard L., Thibault R., Matras P., Zmarzly A., Matysiak K., Van Gossum A., Forbes A., Wyer N., Taus M., Virgili N.M., O'Callaghan M., Chapman B., Osland E., Cuerda C., Sahin P., Jones L., Won Lee A.D., Masconale L., Orlandoni P., Izbeki F., Spaggiari C., Bueno M., Doitchinova-Simeonova M., Garde C., Serralde-Zuniga A.E., Olveira G., Krznaric Z., Czako L., Kekstas G., Sanz-Paris A., Jauregui E.P., Murillo A.Z., Schafer E., Arends J., Suarez-Llanos J.P., Lal S., St. Orsola University Hospital, Cleveland Clinic, Rigshospitalet [Copenhagen], Copenhagen University Hospital, Radboud University Medical Center [Nijmegen], Hospices Civils de Lyon, Departement de Neurologie (HCL), Leeds Teaching Hospitals NHS Trust, University Hospital of Wales, Rabin Medical Center, Nottingham University Hospital NHS Trust, Department of Infectious Diseases [Amsterdam, Netherlands] (Academic Medical Center), University of Amsterdam [Amsterdam] (UvA)-Center for Tropical and Travel Medicine [Amsterdam, Netherlands], University Hospital Southampton NHS Foundation Trust, University Hospitals Birmingham NHS Foundation Trust, 'Federico II' University of Naples Medical School, Sahlgrenska University Hospital [Gothenburg], Nutrition, Métabolismes et Cancer (NuMeCan), Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES), CHU Pontchaillou [Rennes], University Hospitals Birmingham NHS Foundation Trust Institut National de la Santé et de la Recherche Médicale University of Pennsylvania, PennRoyal Marsden NHS Foundation TrustNottingham University Hospitals NHS TrustCleveland Clinic FoundationUniversity Hospital Southampton NHS Foundation TrustChung Hua University Fondazione Città della SperanzaInstitut National de la Recherche Agronomique Hospices Civils de Lyon European Society for Clinical Nutrition and Metabolism, Endocrinology, AGEM - Endocrinology, metabolism and nutrition, University Hospital of Wales (UHW), Nottingham University Hospitals NHS Trust (NUH), and Université de Rennes (UR)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE)
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0301 basic medicine ,Male ,medicine.medical_specialty ,Internationality ,[SDV]Life Sciences [q-bio] ,030209 endocrinology & metabolism ,Pharmacy ,[SDV.CAN]Life Sciences [q-bio]/Cancer ,Critical Care and Intensive Care Medicine ,03 medical and health sciences ,0302 clinical medicine ,Internal medicine ,Health care ,Chronic renal failure ,Medicine ,Humans ,Medical prescription ,Cancer ,Home parenteral nutrition ,030109 nutrition & dietetics ,Nutrition and Dietetics ,Modalities ,Adult patients ,Intestinal failure ,Intravenous supplementation ,business.industry ,International survey ,[SDV.MHEP.HEG]Life Sciences [q-bio]/Human health and pathology/Hépatology and Gastroenterology ,Middle Aged ,Health Surveys ,3. Good health ,Chronic intestinal failure ,Intestinal Diseases ,lnfectious Diseases and Global Health Radboud Institute for Health Sciences [Radboudumc 4] ,Parenteral nutrition ,Cross-Sectional Studies ,Treatment Outcome ,Alimentació parenteral ,Chronic Disease ,Parenteral feeding ,Insuficiència renal crònica ,Female ,business ,Parenteral Nutrition, Home ,[SDV.AEN]Life Sciences [q-bio]/Food and Nutrition - Abstract
Contains fulltext : 220087.pdf (Publisher’s version ) (Closed access) BACKGROUND & AIMS: The safety and effectiveness of a home parenteral nutrition (HPN) program depends both on the expertise and the management approach of the HPN center. We aimed to evaluate both the approaches of different international HPN-centers in their provision of HPN and the types of intravenous supplementation (IVS)-admixtures prescribed to patients with chronic intestinal failure (CIF). METHODS: In March 2015, 65 centers from 22 countries enrolled 3239 patients (benign disease 90.1%, malignant disease 9.9%), recording the patient, CIF and HPN characteristics in a structured database. The HPN-provider was categorized as health care system local pharmacy (LP) or independent home care company (HCC). The IVS-admixture was categorized as fluids and electrolytes alone (FE) or parenteral nutrition, either commercially premixed (PA) or customized to the individual patient (CA), alone or plus extra FE (PAFE or CAFE). Doctors of HPN centers were responsible for the IVS prescriptions. RESULTS: HCC (66%) was the most common HPN provider, with no difference noted between benign-CIF and malignant-CIF. LP was the main modality in 11 countries; HCC prevailed in 4 European countries: Israel, USA, South America and Oceania (p
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- 2020
12. A Call to Action: Now Is the Time to Screen Elderly and Treat Osteosarcopenia, a Position Paper of the Italian College of Academic Nutritionists MED/49 (ICAN-49)
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Montalcini, Tiziana, primary, Pujia, Arturo, additional, Donini, Lorenzo M., additional, Frittitta, Lucia, additional, Galvano, Fabio, additional, Natali, Andrea, additional, Pironi, Loris, additional, Porrini, Marisa, additional, Riso, Patrizia, additional, Rivellese, Angela Albarosa, additional, Russo, Diego, additional, Scapagnini, Giovanni, additional, Serafini, Mauro, additional, Tagliabue, Anna, additional, and De Lorenzo, Antonino, additional
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- 2020
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13. Su1722 GASTROINTESTINAL VASCULAR CHANGES IN GENETIC AND IDIOPATIC FORMS OF CRONIC INTESTINAL-PSEUDO OBSTRUCTION
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Boschetti, Elisa, primary, Costa, Roberta, additional, Papa, Valentina, additional, Cenacchi, Giovanna, additional, Rinaldi, Rita, additional, D'Angelo, Roberto, additional, carelli, valerio, additional, Pironi, Loris, additional, Accarino, Anna, additional, Malagelada, Carolina, additional, Sternini, Catia, additional, Stanghellini, Vincenzo, additional, and De Giorgio, Roberto, additional
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- 2020
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14. Teduglutide for the treatment of adults with intestinal failure associated with short bowel syndrome: pooled safety data from four clinical trials
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Pape, Ulrich-Frank, primary, Iyer, Kishore R., additional, Jeppesen, Palle B., additional, Kunecki, Marek, additional, Pironi, Loris, additional, Schneider, Stéphane M., additional, Seidner, Douglas L., additional, Lee, Hak-Myung, additional, and Caminis, John, additional
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- 2020
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15. MOESM2 of Home parenteral nutrition with an omega-3-fatty-acid-enriched MCT/LCT lipid emulsion in patients with chronic intestinal failure (the HOME study): study protocol for a randomized, controlled, multicenter, international clinical trial
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Klek, Stanislaw, CéCile Chambrier, Cooper, Sheldon, Gabe, Simon, Kunecki, Marek, Pironi, Loris, Rahman, Farooq, Sobocki, Jacek, Szczepanek, Kinga, Wanten, Geert, Lincke, Nicole, Glotzbach, Bernhard, and Forbes, Alastair
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education ,Data_FILES ,food and beverages ,behavioral disciplines and activities ,humanities - Abstract
Additional file 2. Standard Protocol Items: Recommendations for Interventional Trials (SPIRIT) Checklist.
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- 2019
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16. Intestinal Transplantation for Chronic Intestinal Pseudo-Obstruction in Adult Patients
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Masetti, Michele, Di Benedetto, Fabrizio, Cautero, Nicola, Stanghellini, Vincenzo, De Giorgio, Roberto, Lauro, Augusto, Begliomini, Bruno, Siniscalchi, Antonio, Pironi, Loris, Cogliandro, Rosanna, and Pinna, Antonio D.
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- 2004
17. PROGNOSTIC FACTORS OF SURVIVAL ON HOME ARTIFICIAL NUTRITION IN PATIENTS WITH ADVANCED CANCER
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Pironi, Loris, Ruggeri, Enrico, Paganelli, Federica, Mirri, Rossana, Fortuzzi, Anna, and Pannuti, Franco
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- 2003
18. Development of home artificial nutrition in Italy over a seven year period: 2005–2012
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PIRONI, LORIS, Regional Coordinators of SINPE, and Pironi, Loris
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0301 basic medicine ,medicine.medical_specialty ,Population ageing ,Pediatrics ,Endocrinology, Diabetes and Metabolism ,Prevalence ,Epidemiology, Enteral nutrition, Parenteral nutrition, Home, Home care ,Medicine (miscellaneous) ,Artificial nutrition ,Clinical nutrition ,Disease ,03 medical and health sciences ,0302 clinical medicine ,Epidemiology ,medicine ,030109 nutrition & dietetics ,Nutrition and Dietetics ,business.industry ,Public health ,Public Health, Environmental and Occupational Health ,nutritional and metabolic diseases ,stomatognathic diseases ,Parenteral nutrition ,population characteristics ,030211 gastroenterology & hepatology ,Home care ,Enteral nutrition ,Parenteral nutrition, Home ,business ,geographic locations ,Demography - Abstract
BACKGROUND: In 2005, the Italian Society for Artificial Nutrition and Metabolism carried out a national survey on the prevalence of home artificial nutrition (HAN), enteral (HEN) and parenteral (HPN). A second survey was carried out in 2012 to investigate the development of HAN in Italy. METHODS: The Local Health Care Units (LHCUs) were required to record the ongoing cases of HAN using a structured questionnaire. The point prevalence (n./million inhabitants) was analysed according to patient age and disease categories. Data were compared with those obtained in 2005. RESULTS: Data were obtained from 65% of the LHCUs, representing 73% of the Italian population. The prevalence of HAN was 325.5 (90.3% adults, 84.6% HEN). As in 2005, a wide range of prevalence was observed among the administrative regions. The most frequent disease category for HPN was oncological in adults and intestinal failure in children; for HEN, it was neurological in both age groups. A positive correlation was observed between the regional prevalence of HAN and the regional population aging index (p = 0.018). The prevalence of HAN in 2012 was 66% higher than that in 2005. In the individual regions, a negative association was observed between the 2012% prevalence increase of HAN (2012:2005 ratio) and the HAN prevalence in 2005 (p = 0.020). CONCLUSIONS: A two-thirds increase in HAN prevalence was observed over seven years, probably due to improved HAN organisation and management in those regions which showed a low prevalence in 2005. Population aging seemed to be one of the main factors necessitating HAN.
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- 2017
19. Review article: diagnosis and management of intestinal failure‐associated liver disease in adults
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Bond, Ashley, primary, Huijbers, Angelique, additional, Pironi, Loris, additional, Schneider, Stephane M., additional, Wanten, Geert, additional, and Lal, Simon, additional
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- 2019
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20. Mo1619 – An International Survey on the Diagnosis and Management of Severe Gastrointestinal Dysmotility
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Vasant, Dipesh H., primary, Pironi, Loris, additional, Barbara, Giovanni, additional, Bozzetti, Federico, additional, Cuerda, Cristina, additional, Joly, Francisca, additional, Kelly, Darlene G., additional, Paine, Peter, additional, Staun, Michael, additional, Szczepanek, Kinga, additional, Van Gossum, André, additional, Wanten, Geert, additional, and Lal, Simon, additional
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- 2019
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21. Lipid peroxidation and antioxidant status in adults receiving lipid-based home parenteral nutrition
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Pironi, Loris, Ruggeri, Enrico, Zolezzi, Carola, Savarino, Lucia, Incasa, Elena, Belluzzi, Andrea, Munarini, Alessandra, Piazzi, Sandro, Tolomelli, Maurizio, Pizzoferrato, Arturo, and Miglioli, Mario
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Lipid peroxidation -- Causes of ,Parenteral feeding -- Physiological aspects ,Antioxidants -- Physiological aspects ,Food/cooking/nutrition ,Health - Abstract
Background: Infusion of lipid emulsions rich in polyunsaturated fatty acids (PUFAs) may increase lipid peroxidation, which is counteracted mainly by superoxide dismutase (SOD) (a zinc-, copper-, and manganese-dependent enzyme), selenium-dependent glutathione peroxidase (Se-GSHPx), and [Alpha]-tocopherol. Objective: We investigated lipid peroxidation and antioxidant status in patients receiving home parenteral nutrition (HPN) providing variable amounts of a lipid emulsion rich in PUFAs, and [Alpha]-tocopherol, zinc, copper, and manganese as recommended by the American Medical Association, and no selenium. Design: Serum malondialdehyde, plasma [Alpha]-tocopherol, selenium, Se-GSHPx, PUFAs, and red blood cell Se-GSHPx and SOD were evaluated in 12 patients and in 25 healthy control subjects. Malondialdehyde was also assessed in a group of 40 healthy control subjects. Results: Patients had significantly higher concentrations of malondialdehyde and SOD and lower [Alpha]-tocopherol concentrations and selenium nutritional status. Linear regression analysis showed that malondialdehyde was associated with the daily PUFA load (r = 0.69, P [is less than] 0.03) and with plasma [Alpha]-tocopherol (r = -0.59, P [is less than] 0.05), but stepwise multiple regression analysis confirmed only the association between malondialdehyde and [Alpha]-tocopherol; plasma [Alpha]-tocopherol was associated with the daily PUFA load (r = -0.65, P [is less than] 0.04) and with the duration of HPN (r = -0.74, P [is less than] 0.02). Conclusions: In HPN patients, the peroxidative stress due to lipid emulsions rich in PUFAs is counteracted primarily by [Alpha]-tocopherol. The dosages of [Alpha]-tocopherol, zinc, copper, and manganese Recommended by the American Medical Association appear sufficient to sustain SOD activity but inadequate to maintain [Alpha]-tocopherol nutritional status. HPN formulations should be supplemented with selenium. Am J Clin Nutr 1998;68:888-93.
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- 1998
22. Home artificial nutrition in advanced cancer
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Pironi, Loris, Ruggeri, Enrico, Tanneberger, Stephan, Giordani, Stefano, Pannuti, Franco, and Miglioli, Mario
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- 1997
23. Two novel PRNP truncating mutations broaden the spectrum of prion amyloidosis
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Capellari, Sabina, primary, Baiardi, Simone, additional, Rinaldi, Rita, additional, Bartoletti-Stella, Anna, additional, Graziano, Claudio, additional, Piras, Silvia, additional, Calandra-Buonaura, Giovanna, additional, D'Angelo, Roberto, additional, Terziotti, Camilla, additional, Lodi, Raffaele, additional, Donadio, Vincenzo, additional, Pironi, Loris, additional, Cortelli, Pietro, additional, and Parchi, Piero, additional
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- 2018
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24. Functional lipidomics in patients on home parenteral nutrition: Effect of lipid emulsions
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Pironi, Loris, primary, Guidetti, Mariacristina, additional, Verrastro, Ornella, additional, Iacona, Claudia, additional, Agostini, Federica, additional, Pazzeschi, Caterina, additional, Sasdelli, Anna Simona, additional, Melchiorre, Michele, additional, and Ferreri, Carla, additional
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- 2017
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25. Progression of micro-anatomical, molecular and metabolic changes affecting the liver of patients with TYMP derived mitochondrial disease.
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Boschetti, Elisa, Neri, Irene, Caporali, Leonardino, Fiorini, Claudio, Ormanbekova, Danara, Righi, Valeria, Rinaldi, Rita, D’Angelo, Roberto, Malagelada, Carolina, Costa, Roberta, Cenacchi, Giovanna, Pironi, Loris, Carelli, Valerio, De Giorgio, Roberto, Ratti, Stefano, and Manzoli, Lucia
- Abstract
The article focuses on the progression of micro-anatomical, molecular, and metabolic changes affecting the liver of patients with TYMP derived mitochondrial disease (MNGIE), revealing that the liver is a target organ in MNGIE with significant morphological and metabolic alterations.
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- 2023
26. Glutamitaly 2003: consensus paper SINPE sull'impiego della glutamina nella nutrizione artificiale dell'adulto
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Braga, M., Muscaritoli, M., Pittiruti, M., Biffi, Roberto, Biolo, Gianni, Bobbio Pallavicini, Franco, Bossola, Maurizio, Bozzetti, Federico, Candusso, Manila, Carideo, Pietro, Contaldo, Franco, Cozzaglio, Luca, DeGaudio, A. Raffaele, Ficari, Ferdinando, Gallitelli, Livia, Gavazzi, Cecilia, Giacosa, Attilio, Gianotti, Luca, Guglielmi, Francesco W., Laviano, Alessandro, Paccagnella, Agostino, Palmo, Augusta, Pironi, Loris, Radrizzani, Danilo, Salis, Carlo, Sukkar, Samir G., Uderzo, Cornelio, Vannucci, Andrea, Zanello, Marco, Braga, M., Muscaritoli, M., Pittiruti, M., Biffi, Roberto, Biolo, Gianni, Bobbio Pallavicini, Franco, Bossola, Maurizio, Bozzetti, Federico, Candusso, Manila, Carideo, Pietro, Contaldo, Franco, Cozzaglio, Luca, DeGaudio, A. Raffaele, Ficari, Ferdinando, Gallitelli, Livia, Gavazzi, Cecilia, Giacosa, Attilio, Gianotti, Luca, Guglielmi, Francesco W., Laviano, Alessandro, Paccagnella, Agostino, Palmo, Augusta, Pironi, Lori, Radrizzani, Danilo, Salis, Carlo, Sukkar, Samir G., Uderzo, Cornelio, Vannucci, Andrea, and Zanello, Marco
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Surgery ICU ,Oncology ,Glutamine ,SINPE ,Artificial nutrition ,Gastroenterology ,Nutrition and Dietetic ,Consensu ,Anatomy ,Critical Care and Intensive Care Medicine ,Food Science - Abstract
From among the several nutrients believed to exert pharmacological actions, the amino acid Glutamine (GLN) is by far the most extensively studied during the last fifteen years. As an example, more than 970 papers appeared in Medline® between 1990 and 2003, matching the terms glutamine and nutrition, more than 300 of which in the form of review articles. The interest for this 'conditionallly essential' amino acid stems from the observation that it fulfills a number of biochemical needs. It operates as a nitrogen shuttle, may contribute to the formation of other amino acids, glucose, nucleotides, protein and glutathione, and represents the primary energy fuel for rapidly dividing cells, such as enterocytes, lymphocytes, macrophages and fibroblasts. In situations where a certain tissue is in greater need of GLN, inter-organ transfer of GLN usually fulfills site-specific requirements. Under certain conditions, however, endogenous GLN synthesis is not adequate to satisfy tissue needs and exogenous supplementation becomes necessary. Although exogenous parenteral and enteral GLN administation has been proven safe in the clinical setting, precise guidelines and recommendations for its use in clinical practice are still lacking, due to the non univocal interpretation of the clinical trials so far performed. The present article represents the Consensus Paper based on the results of the Glutamitaly 2003 SINPE Consensus Meeting on GLN in adult artificial nutrition. Rationale of use, indications, results of available clinical trials on GLN supplementation have been critically reviewed by a board of experts in the field of clinical nutrition with the aim to develop the methodology for possible future clinical trials evaluating the efficacy of parenteral or entreral GLN supplementation in surgery, ICU, gastroenterology, oncology and haematology patients.
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- 2004
27. Liver as a Source for Thymidine Phosphorylase Replacement in Mitochondrial Neurogastrointestinal Encephalomyopathy
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Boschetti, Elisa, primary, D’Alessandro, Roberto, additional, Bianco, Francesca, additional, Carelli, Valerio, additional, Cenacchi, Giovanna, additional, Pinna, Antonio D., additional, Gaudio, Massimo Del, additional, Rinaldi, Rita, additional, Stanghellini, Vincenzo, additional, Pironi, Loris, additional, Rhoden, Kerry, additional, Tugnoli, Vitaliano, additional, Casali, Carlo, additional, and De Giorgio, Roberto, additional
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- 2014
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28. In Patients on Long-Term Home Parenteral Nutrition (HPN), Transient Elastography (FibroScan®) Correlates With Cholestasis but Not With Liver Fibrosis
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Van Gossum, Andre M., primary, Pironi, Loris, additional, Messing, Bernard, additional, Moreno, Christophe, additional, D'Errico, Antonietta, additional, Colecchia, Antonio, additional, Demetter, Pieter, additional, Ozenne, Violaine, additional, Cazals-Hatem, Dominique, additional, and Joly, Francisca, additional
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- 2011
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29. M2014 Evidence of Neurological Impairment in Patients With Severe Gut Dysmotility
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De Giorgio, Roberto, primary, Rinaldi, Rita, additional, Pironi, Loris, additional, Cenacchi, Giovanna, additional, De Giorgi, Lucilla Badiali, additional, Liguori, Rocco, additional, Carelli, Valerio, additional, D'Alessandro, Roberto, additional, Lodi, Raffaele, additional, Cogliandro, Rosanna, additional, Barbara, Giovanni, additional, Corinaldesi, Roberto, additional, and Stanghellini, Vincenzo, additional
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- 2010
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30. 210 Survival Rate and Causes of Death on Home Parenteral Nutrition for Irreversible Intestinal Failure: Implications for Intestinal Transplantation
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Pironi, Loris, primary, Joly, Francisca, additional, Forbes, Alastair, additional, Colomb, Virginie, additional, Lyszkowska, Malgorzata, additional, Baxter, Janet, additional, Gabe, Simon M., additional, Hebuterne, Xavier, additional, Gambarara, Manuela, additional, Gottrand, Frederic, additional, Cuerda, Cristina, additional, Goulet, Olivier, additional, Messing, Bernard, additional, Staun, Michael, additional, and Van Gossum, Andre M., additional
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- 2010
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31. T1278 Is TRANSIENT ELASTOGRAPHY (FibroScan®) Valuable for Assessing Liver Fibrosis in Patients with Long-Term Home Parenteral Nutrition (HPN)? Preliminary Results
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Van Gossum, Andre M., primary, Pironi, Loris, additional, Messing, Bernard, additional, Moreno, Christophe, additional, Colecchia, Antonio, additional, Demetter, Pieter, additional, Degos, Francoise, additional, Cazals-Hatem, Dominique, additional, and Joly, Francisca, additional
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- 2009
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32. Parvovirus B19 Infection Localized in the Intestinal Mucosa and Associated with Severe Inflammatory Bowel Disease
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Pironi, Loris, primary, Bonvicini, Francesca, additional, Gionchetti, Paolo, additional, D'Errico, Antonia, additional, Rizzello, Fernando, additional, Corsini, Catia, additional, Foroni, Laura, additional, and Gallinella, Giorgio, additional
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- 2009
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33. Short Bowel Syndrome: clinical management
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Pironi, Loris, primary, Guidetti, Mariacristina, additional, Lanzoni, Elisabetta, additional, Pazzeschi, Caterina, additional, and Corsini, Catia, additional
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- 2008
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34. Survival of Patients Identified as Candidates for Intestinal Transplantation: A 3-Year Prospective Follow-Up
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Pironi, Loris, primary, Forbes, Alastair, additional, Joly, Francisca, additional, Colomb, Virginie, additional, Lyszkowska, Malgorzata, additional, Van Gossum, André, additional, Baxter, Janet, additional, Thul, Paul, additional, Hébuterne, Xavier, additional, Gambarara, Manuela, additional, Gottrand, Frederic, additional, Moreno Villares, José M., additional, Messing, Bernard, additional, Goulet, Olivier, additional, and Staun, Michael, additional
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- 2008
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35. Management of intestinal failure in Europe. A questionnaire based study on the incidence and management
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Staun, Michael, primary, Hebuterne, Xavier, additional, Shaffer, Jon, additional, Haderslev, Kent V, additional, Bozzetti, Frederico, additional, Pertkiewicz, Marek, additional, Micklewright, Ann, additional, Moreno, Jose, additional, Thul, Paul, additional, and Pironi, Loris, additional
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- 2007
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36. Splanchnic haemodinamic in short bowel syndrome (SBS): Color-doppler evaluation in fasting and post-prandial conditions
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Serra, Carla, primary, Pironi, Loris, additional, Merli, Cecilia, additional, Volpe, Laura, additional, Morelli, Cristina, additional, Paganelli, Federica, additional, and Miglioli, Mario, additional
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- 2001
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37. Cellular and metabolic changes in the liver tissue of patients with Mitochondrial Neurogastrointestinal Encephalomyopathy.
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Boschetti, Elisa, Caporali, Leonardo, D'Angelo, Roberto, Tardio, Maria Lucia, Costa, Roberta, Accarino, Anna, Malagelada, Carolina, Righi, Valeria, Costanzini, Anna, D'Errico, Antonietta, Cenacchi, Giovanna, Sternini, Catia, Stanghellini, Vincenzo, Pironi, Loris, Rinaldi, Rita, Ratti, Stefano, Manzoli, Lucia, Carelli, Valerio, and De Giorgio, Roberto
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URIDINE ,VASCULAR endothelial growth factors ,HYPOXIA-inducible factor 1 ,HEMATOXYLIN & eosin staining ,LIVER ,ENDOTHELIAL cells ,MITOCHONDRIA - Abstract
Objective: Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) is an extremely rare disease caused by a genetically-driven thymidine phosphorylase (TP) defect. TP converts the nucleosides thymidine and deoxyuridine into respective nucleotides. In MNGIE, these nucleosides accumulate promoting tissue damage and mtDNA abnormalities responsible for neurological impairment and severe gastrointestinal dysmotility. The resulting clinical phenotype has a tremendous impact on patients' poor quality of life and fatal outcome.1;2 One possible treatment strategy to MNGIE patients is the stable reduction of circulating toxic nucleosides via liver transplantation. This approach has been successfully achieved in MNGIE patients in Italy and in many Western Countries.3 Although the liver has never been described as a target organ of the disease, explanted livers of MNGIE patients show clear macroscopic changes. Aim: The objective of our study was to provide an accurate morphologic and metabolic analysis of MNGIE liver tissue vs. controls (CTR). Methods: Liver tissue biopsies (1x1 cm each sample) were collected from n=7 MNGIE (2F; 20-38 yrs) and n=7 CTR (2F; 22-67 yrs) subjects. Formalin fixed-paraffin embedded tissue sections were processed for: 1) Hematoxylin and Eosin staining to evaluate architectural changes; 2) Sirius Red/Fast Green collagen assay to localize and quantify fibrosis; 3) Orcein, vascular endothelial cells growth factor and CD31 immunostaining to assess vascular changes; 4) hypoxia-inducible-factor-1a (HIF-1a) immunostaining to test hypoxia. Part of the liver tissue biopsies (0.5x0.5 cm each sample) were collected and snap frozen from n=3 MNGIE and n=3 CTR for metabolomic NMR assay. Results: MNGIE liver was characterized by intense steatosis. Fibrosis increases (P=0.0025) with patient age leading to the formation of porto-portal and porto-central collagen septa. The vascular and biliary branches were altered and these findings correlated with the increased hypoxia in hepatocytes, as indicated by HIF-1a positive staining. Preliminary analysis performed with NMR indicated a rearrangement characterized by triglyceride production and a significant decline in aerobic metabolism. Conclusions: Our data, showing marked morphological and metabolic changes, indicate that the liver is a target organ of MNGIE. Liver alterations should be carefully taken into account as a compromised liver may interfere with the clinical response to targeted treatments. [ABSTRACT FROM AUTHOR]
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- 2021
38. Fat-induced heal brake in humans: A dose-dependent phenomenon correlated to the plasma levels of peptide YY
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Pironi, Loris, primary, Stanghellini, Vincenzo, additional, Miglioli, Mario, additional, Corinaldesi, Roberto, additional, De Giorgio, Roberto, additional, Ruggeri, Enrico, additional, Tosetti, Cesare, additional, Poggioli, Gilberto, additional, Morselli^Labate, Antonio M., additional, Monetti, Nino, additional, Gozzetti, Giuseppe, additional, Barbara, Luigi, additional, and Go, Vay Liang W., additional
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- 1993
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39. Brief Communication
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Masetti, Michele, Benedetto, Fabrizio Di, Cautero, Nicola, Stanghellini, Vincenzo, Giorgio, Roberto De, Lauro, Augusto, Begliomini, Bruno, Siniscalchi, Antonio, Pironi, Loris, Cogliandro, Rosanna, and Pinna, Antonio D.
- Abstract
Intestinal transplantation (ITx) has become a life-saving procedure for patients with irreversible intestinal failure who can no longer be maintained on parenteral nutrition (PN). This report presents the results of our experience on ITx in patients suffering from chronic intestinal pseudo-obstruction (CIPO). Between December 30, 2000 and May 30, 2003 six adult patients affected by CIPO underwent primary ITx at our Center. Pre-transplant evaluation, indication for ITx and surgical technique are reported. On December 30 2003, the mean follow-up was 25.0 months. No peri-operative deaths occurred in the study population and five out of six patients are alive, with 1-year patient and graft survival of 83.3% and 66.6%. Although our series is limited by the number of patients, our experience suggests that ITx transplantation should be considered in adult patients suffering from CIPO and PN life-threatening complication.
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- 2004
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40. Guía Práctica ESPEN: nutrición enteral domiciliaria.
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Bischoff, Stephan C., Austin, Peter, Boeykens, Kurt, Chourdakis, Michael, Cuerda, Cristina, Jonkers-Schuitema, Cora, Lichota, Marek, Nyulasi, Ibolya, Schneider, Stéphane M., Stanga, Zeno, Pironi, Loris, and Cantón, Ana
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ENTERAL feeding , *PARENTERAL feeding , *PHARMACISTS , *DIETITIANS , *PHYSICIANS , *SCIENCE publishing , *NUTRITION - Abstract
This ESPEN practical guideline will inform physicians, nurses, dieticians, pharmacists, caregivers and other home enteral nutrition (HEN) providers in a concise way about the indications and contraindications for HEN, as well as its implementation and monitoring. This guideline will also inform interested patients requiring HEN. Home parenteral nutrition is not included but will be addressed in a separate ESPEN guideline. The guideline is based on the ESPEN scientific guideline published before, which consists of 61 recommendations that have been reproduced and renumbered, along with the associated commentaries that have been shorted compared to the scientific guideline. Evidence grades and consensus levels are indicated. The guideline was commissioned and financially supported by ESPEN and the members of the guideline group were selected by ESPEN. [ABSTRACT FROM AUTHOR]
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- 2023
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41. COVID-19 Vaccination Willingness and Hesitancy in Patients With Inflammatory Bowel Diseases: Analysis of Determinants in a National Survey of the Italian IBD Patients’ Association
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Simona Radice, Francesco Conforti, Claudio Costantino, Marina Aloi, Ferdinando Ficari, S. Leone, A. Armuzzi, Francesco Manguso, Andrea Costantino, Giammarco Mocci, Maurizio Vecchi, Loris Pironi, Ambrogio Orlando, Daniele Noviello, Alessandra Tongiorgi, Fernando Rizzello, Fabrizio Bossa, Flavio Caprioli, Costantino A., Noviello D., Conforti F.S., Aloi M., Armuzzi A., Bossa F., Ficari F., Leone S., Manguso F., Mocci G., Orlando A., Pironi L., Radice S., Rizzello F., Tongiorgi A., Costantino C., Vecchi M., Caprioli F., and Costantino Andrea, Noviello Daniele, Conforti Francesco Simone, Aloi Marina, Armuzzi Alessandro, Bossa Fabrizio, Ficari Ferdinando, Leone Salvo, Manguso Francesco, Mocci Giammarco, Orlando Amrbogio, Pironi Loris, Radice Simona, Rizzello Fernando, Tongiorgi Alessandra, Costantino Claudio, Vecchi Maurizio, Caprioli Flavio
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medicine.medical_specialty ,2019-20 coronavirus outbreak ,COVID-19 Vaccines ,Coronavirus disease 2019 (COVID-19) ,Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) ,IBD ,Brief Report - Clinical ,Internal medicine ,medicine ,Humans ,Immunology and Allergy ,In patient ,AcademicSubjects/MED00260 ,SARS-CoV-2 ,business.industry ,Vaccination ,Gastroenterology ,COVID-19 ,Inflammatory Bowel Diseases ,COVID-19 Vaccines Human ,IBD, Crohn, Ulcerative Colitis, Vaccination, Covid 19, Sars-Cov 2 ,COVID-19 vaccine ,vaccine hesitancy ,business - Abstract
The coronavirus disease 2019 (COVID-19) pandemic has not finished yet, and the most promising option towards its ending is widespread vaccination. Because patients with inflammatory bowel diseases (IBDs), namely Crohn’s disease (CD) and ulcerative colitis (UC), often require immune-modifying treatment, which might increase the risk of opportunistic infection,1 their vaccination history for several infectious diseases is routinely checked, and when inadequate, vaccination is performed at diagnosis or ideally before immune suppressive treatment is started.1 However, IBD patients were not found to be at an increased risk of developing COVID-19 or of experiencing a more severe disease course.2
- Published
- 2021
42. Effects of antidiabetic agents on steatosis and fibrosis biomarkers in type 2 diabetes: A real‐world data analysis
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Federico Ravaioli, Maria Letizia Petroni, Santo Colosimo, Anna Simona Sasdelli, Loris Pironi, Giulio Marchesini, Francesca Marchignoli, Lucia Brodosi, Francesca Alessandra Barbanti, Colosimo, Santo, Ravaioli, Federico, Petroni, Maria L, Brodosi, Lucia, Marchignoli, Francesca, Barbanti, Francesca A, Sasdelli, Anna S, Marchesini, Giulio, and Pironi, Loris
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Data Analysis ,endocrine system ,medicine.medical_specialty ,Glucagon like peptide-1 receptor agonist ,Type 2 diabetes ,Dipeptidyl-peptidase-4 inhibitor ,Gastroenterology ,Sodium-glucose cotransporter-2 inhibitor ,03 medical and health sciences ,Liver disease ,0302 clinical medicine ,Internal medicine ,NAFLD ,Nonalcoholic fatty liver disease ,glucagon like peptide‐1 receptor agonists ,sodium‐glucose cotransporter‐2 inhibitors ,Medicine ,dipeptidyl‐peptidase‐4 inhibitors ,Humans ,Hypoglycemic Agents ,Metabolic & Toxic Liver Diseases ,Sodium-Glucose Transporter 2 Inhibitors ,Retrospective Studies ,Hepatology ,business.industry ,Fatty liver ,medicine.disease ,Fibrosis ,Metformin ,Fatty Liver ,Diabetes Mellitus, Type 2 ,030220 oncology & carcinogenesis ,030211 gastroenterology & hepatology ,Original Article ,Steatosis ,business ,Pioglitazone ,Body mass index ,surrogate biomarkers ,Biomarkers ,medicine.drug - Abstract
Background & Aims There is intense research for drugs able to reduce disease progression in nonalcoholic fatty liver disease. We aimed to test the impact of novel antidiabetic drugs (dipeptidyl‐peptidase‐4 inhibitors – DPP‐4Is, glucagon‐like peptide‐1 receptor agonists – GLP‐1RAs, sodium‐glucose cotransporter‐2 inhibitors – SGLT‐2Is) on non‐invasive biomarkers of steatosis (fatty liver index, FLI) and fibrosis (Fibrosis‐4 score, FIB‐4) in patients with type 2 diabetes (T2D). Methods Clinical, anthropometric and biochemical parameters were retrospectively analysed in 637 consecutive T2D patients switched from metformin w/wo sulfonylureas and/or pioglitazone to DPP‐4Is, GLP‐1RAs and SGLT‐2Is in a tertiary care setting. 165 patients maintained on original treatments served as controls. The effects on FLI and FIB‐4 at 6‐ and 12‐month follow‐up were analysed by logistic regression after adjustment for baseline differences, computed by propensity scores, and additional adjustment for changes in glycosylated hemoglobin (HbA1c) and body mass index. Results Body mass index, HbA1c and aminotrasferases significantly decreased following switching to GLP‐1RAs and SGLT2‐Is, compared with both controls and DPP‐4Is, whereas only HbA1c was reduced on DPP‐4Is. FLI and FIB‐4 were reduced on GLP‐1RA and SGLT‐2I; logistic regression analysis confirmed a significant improvement of both biomarkers after adjustment for propensity score. The shift of FIB‐4 values towards the category ruling out advanced fibrosis was maintained after additional adjustment for confounders. These effects were confirmed in a sensitivity analysis on effect size. Conclusions Glucagon‐like peptide‐1 receptor agonists and SGLT‐2Is improve biomarkers of steatosis and fibrosis, in keeping with beneficial effects on liver disease progression, and should be considered the treatment of choice in T2D.
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- 2021
43. Chronic Intestinal Failure in Children: An International Multicenter Cross-Sectional Survey
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Antonella Lezo, Antonella Diamanti, Evelyne M. Marinier, Merit Tabbers, Anat Guz-Mark, Paolo Gandullia, Maria I. Spagnuolo, Sue Protheroe, Noel Peretti, Laura Merras-Salmio, Jessie M. Hulst, Sanja Kolaček, Looi C. Ee, Joanna Lawrence, Jonathan Hind, Lorenzo D’Antiga, Giovanna Verlato, Ieva Pukite, Grazia Di Leo, Tim Vanuytsel, Maryana K. Doitchinova-Simeonova, Lars Ellegard, Luisa Masconale, María Maíz-Jiménez, Sheldon C. Cooper, Giorgia Brillanti, Elena Nardi, Anna S. Sasdelli, Simon Lal, Loris Pironi, Regione Piemonte Azienda Ospedaliera - S. Anna [Turin, Italy] (OIRM-S), Ospedale Bambin Gesù [Rome, Italy] (OBG), Centre de Référence des Maladies Digestives Rares [AP-HP Hôpital Robert-Debré] (CRMDR), AP-HP Hôpital universitaire Robert-Debré [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Emma Children’s Hospital, Amsterdam UMC - Amsterdam University Medical Center, Schneider Children’s Medical Center [Petah Tikva, Israel] (SCMC), Tel Aviv University (TAU), IRCCS Istituto Giannina Gaslini [Genoa, Italy], University of Naples Federico II = Università degli studi di Napoli Federico II, Birmingham Women's and Children's NHS Foundation Trust, Cardiovasculaire, métabolisme, diabétologie et nutrition (CarMeN), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Hospices Civils de Lyon (HCL)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE), Hôpital Femme Mère Enfant [CHU - HCL] (HFME), Hospices Civils de Lyon (HCL), Helsinki University Hospital [Finland] (HUS), Eramus MC-Sophia Children’s Hospital, Partenaires INRAE, Children’s Hospital Srebrnjak [Zagreb, Croatia], Children’s Health Queensland [Brisbane] (CHQ), Royal Children’s Hospital & Department of Paediatrics [Parkville, VIC, Australia], King‘s College London, Hospital Papa Giovanni XXIII (Hosp P Giovanni XXIII), Azienda Ospedale Università di Padova = Hospital-University of Padua (AOUP), Children's Clinical University Hospital [Riga, Latvia] (CCUH), Institute for Maternal and Child Health - IRCCS 'Burlo Garofolo' [Trieste], Leuven Intestinal Failure and Transplantation [Leuven, Belgium] (LIFT), University Hospitals Leuven [Leuven], Bulgarian Association of Patients with Malnutrition [Sofia, Bulgaria] (BAPM), Sahlgrenska University Hospital [Gothenburg], Ospedale Orlandi [Bussolengo, Italy] (2O), Hospital Universitario 12 de Octubre [Madrid], Alma Mater Studiorum University of Bologna (UNIBO), University of Bologna/Università di Bologna, Azienda Ospedaliero-Universitaria di Bologna [Bolohna, Italy] (AOUB), Salford Royal NHS Foundation Trust [Salford, UK], CarMeN, laboratoire, Pediatrics, Pediatric surgery, Clinicum, HUS Children and Adolescents, Children's Hospital, University of Helsinki, Paediatric Gastroenterology, AGEM - Amsterdam Gastroenterology Endocrinology Metabolism, ARD - Amsterdam Reproduction and Development, Lezo A., Diamanti A., Marinier E.M., Tabbers M., Guz-Mark A., Gandullia P., Spagnuolo M.I., Protheroe S., Peretti N., Merras-Salmio L., Hulst J.M., Kolacek S., Ee L.C., Lawrence J., Hind J., D'antiga L., Verlato G., Pukite I., Di Leo G., Vanuytsel T., Doitchinova-Simeonova M.K., Ellegard L., Masconale L., Maiz-Jimenez M., Cooper S.C., Brillanti G., Nardi E., Sasdelli A.S., Lal S., Pironi L., Lezo, Antonella, Diamanti, Antonella, Marinier, Evelyne M., Tabbers, Merit, Guz-Mark, Anat, Gandullia, Paolo, Spagnuolo, Maria I., Protheroe, Sue, Peretti, Noel, Merras-Salmio, Laura, Hulst, Jessie M., Kola( (c))ek, Sanja, Ee, Looi C., Lawrence, Joanna, Hind, Jonathan, D'Antiga, Lorenzo, Verlato, Giovanna, Pukite, Ieva, Di Leo, Grazia, Vanuytsel, Tim, Doitchinova-Simeonova, Maryana K., Ellegard, Lar, Masconale, Luisa, Ma('(i))z-Jim('(e))nez, Mar('(i))a, Cooper, Sheldon C., Brillanti, Giorgia, Nardi, Elena, Sasdelli, Anna S., Lal, Simon, and Pironi, Loris
- Subjects
Adult ,Male ,Short Bowel Syndrome ,[SDV]Life Sciences [q-bio] ,YOUNG-PEOPLE ,ENTERAL NUTRITION ,CLASSIFICATION ,home parenteral nutrition ,Intestinal Failure ,children ,body growth ,Humans ,chronic intestinal failure ,intravenous supplementation ,intestinal transplantation ,transition ,ADULT PATIENTS ,HEPATOLOGY ,Child ,Cross-Sectional Studie ,Nutrition and Dietetics ,Science & Technology ,Intestinal Disease ,Nutrition & Dietetics ,GASTROENTEROLOGY ,PREVALENCE ,[SDV] Life Sciences [q-bio] ,Intestinal Diseases ,Cross-Sectional Studies ,ITALIAN SOCIETY ,Chronic Disease ,HOME PARENTERAL-NUTRITION ,Female ,REHABILITATION PROGRAMS ,3143 Nutrition ,Parenteral Nutrition, Home ,Life Sciences & Biomedicine ,Food Science ,Human - Abstract
Background: The European Society for Clinical Nutrition and Metabolism database for chronic intestinal failure (CIF) was analyzed to investigate factors associated with nutritional status and the intravenous supplementation (IVS) dependency in children. Methods: Data collected: demographics, CIF mechanism, home parenteral nutrition program, z-scores of weight-for-age (WFA), length or height-for-age (LFA/HFA), and body mass index-for-age (BMI-FA). IVS dependency was calculated as the ratio of daily total IVS energy over estimated resting energy expenditure (%IVSE/REE). Results: Five hundred and fifty-eight patients were included, 57.2% of whom were male. CIF mechanisms at age 1−4 and 14−18 years, respectively: SBS 63.3%, 37.9%; dysmotility or mucosal disease: 36.7%, 62.1%. One-third had WFA and/or LFA/HFA z-scores < −2. One-third had %IVSE/REE > 125%. Multivariate analysis showed that mechanism of CIF was associated with WFA and/or LFA/HFA z-scores (negatively with mucosal disease) and %IVSE/REE (higher for dysmotility and lower in SBS with colon in continuity), while z-scores were negatively associated with %IVSE/REE. Conclusions: The main mechanism of CIF at young age was short bowel syndrome (SBS), whereas most patients facing adulthood had intestinal dysmotility or mucosal disease. One-third were underweight or stunted and had high IVS dependency. Considering that IVS dependency was associated with both CIF mechanisms and nutritional status, IVS dependency is suggested as a potential marker for CIF severity in children. ispartof: NUTRIENTS vol:14 issue:9 ispartof: location:Switzerland status: published
- Published
- 2022
44. An international study of the quality of life of adult patients treated with home parenteral nutrition
- Author
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Darlene G. Kelly, Cristina Cuerda, Peter Fayers, Geert J. A. Wanten, C. Jonkers, Manon Jobin, Alastair Forbes, Ceferino Martinez Faedo, J.P. Baxter, Loris Pironi, Federico Bozzetti, André Van Gossum, Luigi Mariani, Marie-France Boudreault, Francisca Joly, Lyn Gillanders, Cinzia Brunelli, Margie O'Callaghan, Stanislaw Klek, Michael Staun, Alain Gilbert, Baxter, Janet P., Fayers, Peter M., Bozzetti, Federico, Kelly, Darlene, Joly, Francisca, Wanten, Geert, Jonkers, Cora, Cuerda, Cristina, van Gossum, Andre, Klek, Stanislaw, Boudreault, Marie-France, Gilbert, Alain, Jobin, Manon, Staun, Michael, Gillanders, Lyn, Forbes, Alastair, O'Callaghan, Margie, Faedo, Ceferino Martínez, Brunelli, Cinzia, Mariani, Luigi, Pironi, Loris, and Other departments
- Subjects
Adult ,Male ,0301 basic medicine ,Quality of life ,medicine.medical_specialty ,Adolescent ,030209 endocrinology & metabolism ,Disease ,Critical Care and Intensive Care Medicine ,Young Adult ,03 medical and health sciences ,0302 clinical medicine ,Internal consistency ,LIVING STATUS ,medicine ,Nutrition and Dietetic ,Humans ,Patient Reported Outcome Measures ,Patient reported outcomes ,Aged ,Aged, 80 and over ,Home parenteral nutrition ,030109 nutrition & dietetics ,Nutrition and Dietetics ,Multivariable linear regression ,Adult patients ,business.industry ,Australia ,Discriminant validity ,Middle Aged ,Intestinal failure ,Patient reported outcome ,humanities ,Europe ,Intestinal Diseases ,lnfectious Diseases and Global Health Radboud Institute for Health Sciences [Radboudumc 4] ,Cross-Sectional Studies ,Parenteral nutrition ,North America ,Physical therapy ,Female ,Parenteral Nutrition, Home ,business - Abstract
Contains fulltext : 205187.pdf (Publisher’s version ) (Closed access) BACKGROUND & AIMS: Home parenteral nutrition-quality of life (HPN-QOL((c))) is a self-assessment tool for the measurement of QOL in patients on HPN. The aims of this study were: to re-assess the basic psychometric properties of the HPN-QOL((c)) in a multinational sample of adult patients; to provide a description of QOL dimensions by short and long HPN treatment duration; to explore clinical factors potentially associated to QOL scores. METHODS: Patients (n = 699) from 14 countries completed the HPN-QOL((c)). The questionnaires were analysed to evaluate data completeness, convergent/discriminant validity and internal-consistency reliability. The association of overall QOL and HPN treatment duration as well as other clinical factors were investigated using multivariable linear regression models. RESULTS: The analysis of the multitrait-scaling and internal consistency indicates a good fit with the questionnaire structure for most items. Item discriminant validity correlation was satisfactory and psychometric evaluation of the HPN-QOL((c)) in the different English, French and Italian language patient sub-groups confirmed psychometric equivalence of the three questionnaire versions. The results of the multivariable linear regression showed that QOL scores were significantly associated with HPN duration (better in long-term), underlying disease (better in Crohn's disease and mesenteric ischaemia) and living status (worse in living alone) and, after adjusting for the other factors, with the number of days of HPN infusion per week. CONCLUSIONS: The HPN-QOL((c)), is a valid tool for measurement of QOL in patients on HPN, to be used in the clinical practice as well as in research.
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- 2019
45. Intestinal failure in adults: Recommendations from the ESPEN expert groups
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Simon Lal, Mette Holst, Annika Reintam Blaser, Katie E. Rollins, Geert J. A. Wanten, Chiara Zanfi, Olivier Corcos, C. Jonkers, Jon Shaffer, Francisca Joly, Stanislaw Klek, Anna Simona Sasdelli, Alastair Forbes, Loris Pironi, André Van Gossum, Dileep N. Lobo, Espen Acute, Pironi, Loris, Corcos, Olivier, Forbes, Alastair, Holst, Mette, Joly, Francisca, Jonkers, Cora, Klek, Stanislaw, Lal, Simon, Blaser, Annika Reintam, Rollins, Katie E., Sasdelli, Anna S., Shaffer, Jon, Van Gossum, Andre, Wanten, Geert, Zanfi, Chiara, and Lobo, Dileep N.
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Adult ,0301 basic medicine ,medicine.medical_specialty ,definitions ,media_common.quotation_subject ,Definitions ,Acute ,Critical Care and Intensive Care Medicine ,03 medical and health sciences ,All institutes and research themes of the Radboud University Medical Center ,0302 clinical medicine ,Quality of life (healthcare) ,Risk Factors ,Multidisciplinary approach ,Patient-Centered Care ,Epidemiology ,Nutrition and Dietetic ,Humans ,Medicine ,Quality (business) ,Chronic ,Intensive care medicine ,media_common ,Government ,030109 nutrition & dietetics ,Nutrition and Dietetics ,business.industry ,Short bowel syndrome ,Definition ,Water-Electrolyte Balance ,Intestinal failure ,Management ,Chronic intestinal failure ,Europe ,Gastrointestinal Tract ,Intestines ,Intestinal Diseases ,lnfectious Diseases and Global Health Radboud Institute for Health Sciences [Radboudumc 4] ,Intestinal Absorption ,Hydroxyzine ,General partnership ,Acute Disease ,Chronic Disease ,Quality of Life ,Life expectancy ,Interdisciplinary Communication ,030211 gastroenterology & hepatology ,Nutrition Therapy ,business - Abstract
BACKGROUND & AIMS: Intestinal failure (IF) is defined as "the reduction of gut function below the minimum necessary for the absorption of macronutrients and/or water and electrolytes, such that intravenous supplementation is required to maintain health and/or growth". Functionally, it may be classified as type I acute intestinal failure (AIF), type II prolonged AIF and type III chronic intestinal failure (CIF) The ESPEN Workshop on IF was held in Bologna, Italy, on 15-16 October 2017 and the aims of this document were to highlight the current state of the art and future directions for research in IF.METHODS: This paper represents the opinion of experts in the field, based on current evidence. It is not a formal review, but encompasses the current evidence, with emphasis on epidemiology, classification, diagnosis and management.RESULTS: IF is the rarest form of organ failure and can result from a variety of conditions that affect gastrointestinal anatomy and function adversely. Assessment, diagnosis, and short and long-term management involves a multidisciplinary team with diverse expertise in the field that aims to reduce complications, increase life expectancy and improve quality of life in patients.CONCLUSIONS: Both AIF and CIF are relatively rare conditions and most of the published work presents evidence from small, single-centre studies. Much remains to be investigated to improve the diagnosis and management of IF and future studies should rely on multidisciplinary, multicentre and multinational collaborations that gather data from large cohorts of patients. Emphasis should also be placed on partnership with patients, carers and government agencies in order to improve the quality of research that focuses on patient-centred outcomes that will help to improve both outcomes and quality of life in patients with this devastating condition.
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- 2018
46. Five-year survival and causes of death in patients on home parenteral nutrition for severe chronic and benign intestinal failure
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Stanislaw Klek, Michael Staun, L. Chicharro, Lidia Santarpia, Cristina Cuerda, Francisca Joly, Olivier Corcos, Loris Pironi, Yi Lisa Hwa, Franco Contaldo, J. Baxter, Geert J. A. Wanten, Nuria Virgili, Darlene G. Kelly, Antonella De Francesco, Rosa Burgos, André Van Gossum, Cora Jonker, Federica Agostini, Joly, Francisca, Baxter, Janet, Staun, Michael, Kelly, Darlene G, Hwa, Yi Lisa, Corcos, Olivier, De Francesco, Antonella, Agostini, Federica, Klek, Stanislaw, Santarpia, Lidia, Contaldo, Franco, Jonker, Cora, Wanten, Geert, Chicharro, Luisa, Burgos, Rosa, Van Gossum, Andre, Cuerda, Cristina, Virgili, Nuria, Pironi, Loris, Kelly, Darlene G., and Other departments
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0301 basic medicine ,Adult ,Male ,Pediatrics ,medicine.medical_specialty ,Adolescent ,Critical Care and Intensive Care Medicine ,03 medical and health sciences ,Young Adult ,0302 clinical medicine ,Intestinal failure ,Nutrition and Dietetic ,Medicine ,Humans ,In patient ,Cause of death ,Aged ,Retrospective Studies ,Home parenteral nutrition ,030109 nutrition & dietetics ,Nutrition and Dietetics ,business.industry ,Short bowel syndrome ,Middle Aged ,Chronic intestinal failure ,Log-rank test ,Transplantation ,Intestinal Diseases ,lnfectious Diseases and Global Health Radboud Institute for Health Sciences [Radboudumc 4] ,Parenteral nutrition ,Cohort ,Chronic Disease ,030211 gastroenterology & hepatology ,Female ,business ,Parenteral Nutrition, Home - Abstract
Contains fulltext : 193361.pdf (Publisher’s version ) (Closed access) BACKGROUND & AIM: Home parenteral nutrition (HPN) is the primary treatment for chronic intestinal failure (IF). Intestinal transplantation (ITx) is indicated when there is an increased risk of death due to HPN complications or to the underlying disease. Age, pathophysiologic conditions and underlying disease are known predictors of HPN dependency and overall survival. Although the cause of death on HPN is mostly related to underlying disease in these patients, the relationship between mortality and duration of HPN use remains unclear. The purpose of the present study is to describe factors associated with survival and HPN dependency as well as causes of death in adult patients requiring HPN for chronic intestinal failure during the first 5 years of treatment with HPN. METHODS: A multicenter international (European and USA) questionnaire-based retrospective follow-up of a cohort of 472 IF patients who started HPN was conducted between June and December 2000. Study endpoint was either end of 5-year follow-up, weaned-off HPN, ITx, or death on HPN. Data were analyzed for HPN dependence and overall survival using Kaplan-Meier models and log rank tests. RESULTS: The overall survival probability was 88%, 74% and 64% at 1, 3 and 5 years respectively. Survival was inversely related to age (p < .001) and higher in patients with Crohn's disease or chronic idiopathic pseudo-obstruction. A total of 169 (36.5%) patients were weaned-off HPN mainly (80%) within the first year and most frequently in patients with fistulae. Five of the 14 patients who underwent ITx died. By the end of the study, 104 (23%) of patients died on HPN; 65% of deaths occurred within the first 2.5 years of HPN. CONCLUSIONS: Younger ages at HPN initiation and underlying pathologies are significantly predictive of survival on HPN. Risk of death is greatest during the first 2 years of HPN.
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- 2018
47. Management of intestinal failure in Europe. A questionnaire based study on the incidence and management
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Jonathan Shaffer, P. Thul, Xavier Hébuterne, F. Bozzetti, A. Micklewright, Michael Staun, José Moreno, Kent V. Haderslev, Marek Pertkiewicz, Loris Pironi, Staun, M., Hebuterne, X., Shaffer, J., Haderslev, K. V., Bozzetti, F., Pertkiewicz, M., Micklewright, A., Moreno, J., Thul, P., and Pironi, Loris
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Pediatrics ,medicine.medical_specialty ,Referral ,Sports medicine ,Physiology ,business.industry ,Research ,Incidence (epidemiology) ,Human physiology ,Bioinformatics ,INTESTINAL FAILURE ,Enteral administration ,HOME PARENTERAL NUTRITION ,Intestinal failure ,Treatment practice ,MANAGEMENT ,Medicine ,Orthopedics and Sports Medicine ,Lost to follow-up ,business - Abstract
Background Intestinal failure is the outcome of a number of gastrointestinal diseases and characterized by significant reduction in functional gut mass. If not resolved patients often face long-term nutritional support. This study gathered information about how patients referred with intestinal failure are managed in specialised European centres. Methods A questionnaire was circulated in 7 European countries via representatives of the ESPEN-HAN working group to seek information about experience in treating patients with intestinal failure. We asked about clinical outcome, information about structure and organisation of the department, referral criteria, treatment procedures and guidelines. Results 17 centres in 6 European countries completed the questionnaire: UK, n = 6, France, n = 4, Spain, n = 3, Denmark, n = 2, Italy, n = 1, Poland, n = 1. The experience of the centres in treating patients was in the range 12–30 years. The total number of patients on HPN in all centres was 590. The number of patients referred to centres with intestinal failure during the period January to December 2000 was n = 882: UK, n = 375 (range 2–175), France, n = 308 (range 24–182), Italy and Spain, n = 43 (range 9–52), Denmark n = 51 (range 14–37), the centre in Poland included 53 patients. Comparing all centres the following distribution among patients (median % (range%)) with regard to the endpoints were reported: Oral nutrition 32% (23–50%), enteral/tube feeding 11% (4–23%), HPN 36% (15–57%), lost to follow up 10% (0–35%), dead 9% (5–18%). No patients had an intestinal transplant. Conclusion The study provides information about how patients with intestinal failure are managed across Europe and the data indicates that treatment practice varies between countries.
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- 2007
48. Evidence of enteric angiopathy and neuromuscular hypoxia in patients with mitochondrial neurogastrointestinal encephalomyopathy.
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Boschetti E, D'Angelo R, Tardio ML, Costa R, Giordano C, Accarino A, Malagelada C, Clavenzani P, Tugnoli V, Caio G, Righi V, Garone C, D'Errico A, Cenacchi G, Dotti MT, Stanghellini V, Sternini C, Pironi L, Rinaldi R, Carelli V, and De Giorgio R
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- Gastrointestinal Tract pathology, Humans, Intestinal Pseudo-Obstruction pathology, Mitochondrial Encephalomyopathies pathology, Muscular Dystrophy, Oculopharyngeal pathology, Neovascularization, Pathologic pathology, Ophthalmoplegia metabolism, Ophthalmoplegia pathology, Thymidine Phosphorylase metabolism, Gastrointestinal Tract metabolism, Intestinal Pseudo-Obstruction metabolism, Mitochondrial Encephalomyopathies metabolism, Muscular Dystrophy, Oculopharyngeal metabolism, Neovascularization, Pathologic metabolism, Ophthalmoplegia congenital
- Abstract
Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) is a rare autosomal recessive disease caused by thymidine phosphorylase (TP) enzyme defect. As gastrointestinal changes do not revert in patients undergone TP replacement therapy, one can postulate that other unexplored mechanisms contribute to MNGIE pathophysiology. Hence, we focused on the local TP angiogenic potential that has never been considered in MNGIE. In this study, we investigated the enteric submucosal microvasculature and the effect of hypoxia on fibrosis and enteric neurons density in jejunal full-thickness biopsies collected from patients with MNGIE. Orcein staining was used to count blood vessels based on their size. Fibrosis was assessed using the Sirius Red and Fast Green method. Hypoxia and neoangiogenesis were determined via hypoxia-inducible-factor-1α (HIF-1α) and vascular endothelial cell growth factor (VEGF) protein expression, respectively. Neuron-specific enolase was used to label enteric neurons. Compared with controls, patients with MNGIE showed a decreased area of vascular tissue, but a twofold increase of submucosal vessels/mm
2 with increased small size and decreased medium and large size vessels. VEGF positive vessels, fibrosis index, and HIF-1α protein expression were increased, whereas there was a diminished thickness of the longitudinal muscle layer with an increased interganglionic distance and reduced number of myenteric neurons. We demonstrated the occurrence of an angiopathy in the GI tract of patients with MNGIE. Neoangiogenetic changes, as detected by the abundance of small size vessels in the jejunal submucosa, along with hypoxia provide a morphological basis to explain neuromuscular alterations, vasculature breakdown, and ischemic abnormalities in MNGIE. NEW & NOTEWORTHY Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) is characterized by a genetically driven defect of thymidine phosphorylase, a multitask enzyme playing a role also in angiogenesis. Indeed, major gastrointestinal bleedings are life-threatening complications of MNGIE. Thus, we focused on jejunal submucosal vasculature and showed intestinal microangiopathy as a novel feature occurring in this disease. Notably, vascular changes were associated with neuromuscular abnormalities, which may explain gut dysfunction and help to develop future therapeutic approaches in MNGIE.- Published
- 2021
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