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4. Impact of 2021 European Academy of Neurology/Peripheral Nerve Society diagnostic criteria on diagnosis and therapy of chronic inflammatory demyelinating polyradiculoneuropathy variants

Author

De&nbsp, Lorenzo, A, Liberatore, G, Doneddu, P, Manganelli, F, Cocito, D, Briani, C, Fazio, R, Mazzeo, A, Schenone, A, Di&nbsp, Stefano, V, Cosentino, G, Marfia, G, Benedetti, L, Carpo, M, Filosto, M, Antonini, G, Clerici, A, Luigetti, M, Matà, S, Rosso, T, Lucchetta, M, Siciliano, G, Lauria&nbsp, Pinter, G, Cavaletti, G, Inghilleri, M, Cantisani, T, Notturno, F, Ricciardi, D, Habetswallner, F, Spina, E, Peci, E, Salvalaggio, A, Falzone, Y, Strano, C, Gentile, L, Vegezzi, E, Mataluni, G, Cotti&nbsp, Piccinelli, S, Leonardi, L, Romano, A, Nobile-Orazio, E, Lorenzo A., Liberatore G., Doneddu P. E., Manganelli F., Cocito D., Briani C., Fazio R., Mazzeo A., Schenone A., Stefano V., Cosentino G., Marfia G. A., Benedetti L., Carpo M., Filosto M., Antonini G., Clerici A. M., Luigetti M., Matà S., Rosso T., Lucchetta M., Siciliano G., Pinter G., Cavaletti G., Inghilleri M., Cantisani T., Notturno F., Ricciardi D., Habetswallner F., Spina E., Peci E., Salvalaggio A., Falzone Y., Strano C., Gentile L., Vegezzi E., Mataluni G., Piccinelli S., Leonardi L., Romano A., Nobile-Orazio E., De&nbsp, Lorenzo, A, Liberatore, G, Doneddu, P, Manganelli, F, Cocito, D, Briani, C, Fazio, R, Mazzeo, A, Schenone, A, Di&nbsp, Stefano, V, Cosentino, G, Marfia, G, Benedetti, L, Carpo, M, Filosto, M, Antonini, G, Clerici, A, Luigetti, M, Matà, S, Rosso, T, Lucchetta, M, Siciliano, G, Lauria&nbsp, Pinter, G, Cavaletti, G, Inghilleri, M, Cantisani, T, Notturno, F, Ricciardi, D, Habetswallner, F, Spina, E, Peci, E, Salvalaggio, A, Falzone, Y, Strano, C, Gentile, L, Vegezzi, E, Mataluni, G, Cotti&nbsp, Piccinelli, S, Leonardi, L, Romano, A, Nobile-Orazio, E, Lorenzo A., Liberatore G., Doneddu P. E., Manganelli F., Cocito D., Briani C., Fazio R., Mazzeo A., Schenone A., Stefano V., Cosentino G., Marfia G. A., Benedetti L., Carpo M., Filosto M., Antonini G., Clerici A. M., Luigetti M., Matà S., Rosso T., Lucchetta M., Siciliano G., Pinter G., Cavaletti G., Inghilleri M., Cantisani T., Notturno F., Ricciardi D., Habetswallner F., Spina E., Peci E., Salvalaggio A., Falzone Y., Strano C., Gentile L., Vegezzi E., Mataluni G., Piccinelli S., Leonardi L., Romano A., and Nobile-Orazio E.

Abstract

Background and purpose: There are different criteria for the diagnosis of different variants of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). The 2021 European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) guidelines provide specific clinical criteria for each CIDP variant even if their therapeutical impact has not been investigated. Methods: We applied the clinical criteria for CIDP variants of the 2021 EAN/PNS guidelines to 369 patients included in the Italian CIDP database who fulfilled the 2021 EAN/PNS electrodiagnostic criteria for CIDP. Results: According to the 2021 EAN/PNS clinical criteria, 245 patients achieved a clinical diagnosis of typical CIDP or CIDP variant (66%). We identified 106 patients with typical CIDP (29%), 62 distal CIDP (17%), 28 multifocal or focal CIDP (7%), four sensory CIDP (1%), 27 sensory-predominant CIDP (7%), 10 motor CIDP (3%), and eight motor-predominant CIDP (2%). Patients with multifocal, distal, and sensory CIDP had milder impairment and symptoms. Patients with multifocal CIDP had less frequently reduced conduction velocity and prolonged F-wave latency and had lower levels of cerebrospinal fluid protein. Patients with distal CIDP more frequently had reduced distal compound muscle action potentials. Patients with motor CIDP did not improve after steroid therapy, whereas those with motor-predominant CIDP did. None of the patients with sensory CIDP responded to steroids, whereas most of those with sensory-predominant CIDP did. Conclusions: The 2021 EAN/PNS criteria for CIDP allow a better characterization of CIDP variants, permitting their distinction from typical CIDP and more appropriate treatment for patients.

Published
2024

6. Italian Real-Life Experience of Patients with Hereditary Transthyretin Amyloidosis Treated with Patisiran

Author

Di Stefano V, Fava A, Gentile L, Guaraldi P, Leonardi L, Poli L, Tagliapietra M, Vastola M, Fanara S, Ferrero B, Giorgi M, Perfetto F, Russo M, and Russo D

Subjects
hereditary transthyretin amyloidosis, patisiran, real-life, case reports, Therapeutics. Pharmacology, RM1-950
Abstract

Vincenzo Di Stefano,1 Antonella Fava,2 Luca Gentile,3 Pietro Guaraldi,4 Luca Leonardi,5 Loris Poli,6 Matteo Tagliapietra,7 Michele Vastola,8,9 Salvatore Fanara,1 Bruno Ferrero,10 Mauro Giorgi,2 Federico Perfetto,8 Massimo Russo,3 Domitilla Russo11 1Department of Biomedicine, Neuroscience and Advanced Diagnostic (BIND), University of Palermo, Palermo, Italy; 2Division of Cardiology, Heart Vascular and Thoracic Department, Città della Salute e della Scienza (Molinette Hospital- University of Turin), Turin, Italy; 3Unit of Neurology and Neuromuscular Disease, Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy; 4IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy; 5Department of Neuroscience, Mental Health and Sensory Organs (NESMOS), Sapienza University of Rome Sant’Andrea Hospital, Rome, Italy; 6Department of Neurology, ASST Spedali Civili, Brescia, Italy; 7Department of Neurosciences, Biomedicine and Movement Sciences, University of Verona, Verona, Italy; 8Tuscan Regional Amyloidosis Centre, Careggi University Hospital, Florence, Italy; 9Neurosciences Department, Florence University, Rome, Italy; 10SC Neurology 2U, Department of Neuroscience “Rita Levi Montalcini”, Città della Salute e della Scienza (Molinette Hospital -University of Turin), Turin, Italy; 11Division of Cardiology, Department of Clinical and Molecular Medicine, St. Andrea Hospital, Sapienza University, Rome, ItalyCorrespondence: Vincenzo Di Stefano, Department of Biomedicine, Neuroscience and advanced Diagnostic (BIND), University of Palermo Palermo, Italy, Via del Vespro 143, Palermo, 90127, Italy, Tel +39 3285781786, Fax +390916552974, Email vincenzo19689@gmail.comAbstract: Hereditary transthyretin amyloidosis (ATTRv) is a multisystemic, rare, inherited, progressive and adult-onset disease, affecting the sensorimotor nerves, heart, autonomic function and other organs. The actual scenario of pharmaceutical approaches for ATTRv amyloidosis includes five main groups: TTR stabilizers, TTR mRNA silencers, TTR fibril disruptors, inhibitor of TTR fibril seeding and gene therapy. Patisiran is a small, double-stranded interfering RNA encapsulated in a lipid nanoparticle, able to penetrate into hepatocytes, where it selectively targets TTR mRNA, reducing TTR production. We report and discuss 9 cases of different patients with ATTRv amyloidosis successfully managed with patisiran in the real clinical practice. Literature data, as well as the above presented case reports, show that this drug is effective and safe in improving both neurological and cardiovascular symptoms of ATTRv amyloidosis, and to maintain a good QoL, independently form the stage of the disease and the involved mutation. Recent studies correlated improved functional and biochemical outcomes with a regression of amyloid burden, especially at the cardiac level. Today, patisiran can be considered a valid therapeutic option for the management of patients with ATTRv amyloidosis and polyneuropathy and cardiovascular symptoms.Keywords: hereditary transthyretin amyloidosis, patisiran, real-life, case reports

Published
2022

7. Compatibility of PiC Insupen Needles with a Broad Range of Pens for the Injection of Subcutaneously Administered Drugs for Diabetes

Author

Nicolucci A and Leonardi L

Subjects
diabetes mellitus, injection therapy, pen needle, needle compatibility, Medical technology, R855-855.5
Abstract

Antonio Nicolucci,1 Luca Leonardi2 1Center for Outcomes Research and Clinical Epidemiology (CORESEARCH Srl), Pescara (PE), Italy; 2Pikdare S.p.A., Casnate Con Bernate (CO), ItalyCorrespondence: Antonio Nicolucci, Center for Outcomes Research and Clinical Epidemiology (CORESEARCH Srl), Corso Umberto I, Pescara (PE), 103 – 65122, Italy, Tel +39 085 9047114, Fax +39 085 9047113, Email nicolucci@coresearch.itPurpose: To test the compatibility of PiC Insupen needles with a broad range of pens produced by different manufacturers for the injection of subcutaneously administered drugs.Patients and Methods: The “extreme” products in PiC pen needles range were considered (G33x4 mm and G29x12 mm), to verify that the compatibility was not affected by the cannula diameter and length. Following the launch of the G34x3.5mm needle, additional tests were performed comparing G33x4 and G34x3.5 pen needles. A test medium with viscosity similar to insulin was used. Additional tests were performed with a liquid with a much higher viscosity. All the requirements of the technical standard ISO 11608-2 were satisfied, and the differences between G29, G33 and G34 were negligible. Therefore, the PiC Insupen needle G33x4mm was chosen as representative of the PiC pen needles. Dose accuracy and needle hub torque were assessed, according to the ISO 11608-2:2012 norms. For pens with variable volume, two different volumes were tested (Vlow and Vhigh) in random order, testing 60 needles with Vlow and 60 with Vhigh.Results: Overall, 50 different pens were tested. Dose accuracy acceptance criteria were met for all the pens tested, with the only exception of Vhigh for Berlipen Precision pen. The removal torque was adequate for all pens, with the only exception of Berlipen 301 and Berlipen 302 pens.Conclusion: We documented that Pikdare needles are compatible with a large array of different pens for the injection of insulin and other drugs administered subcutaneously.Keywords: diabetes mellitus, injection therapy, pen needle, needle compatibility

Published
2022

8. Unclassified clinical presentations of chronic inflammatory demyelinating polyradiculoneuropathy

Author

Doneddu, P, Akyil, H, Manganelli, F, Briani, C, Cocito, D, Benedetti, L, Mazzeo, A, Fazio, R, Filosto, M, Cosentino, G, Di Stefano, V, Antonini, G, Marfia, G, Inghilleri, M, Siciliano, G, Clerici, A, Carpo, M, Schenone, A, Luigetti, M, Lauria, G, Mata, S, Rosso, T, Minicuci, G, Lucchetta, M, Cavaletti, G, Liberatore, G, Spina, E, Campagnolo, M, Peci, E, Germano, F, Gentile, L, Strano, C, Cotti Piccinelli, S, Vegezzi, E, Leonardi, L, Mataluni, G, Ceccanti, M, Schirinzi, E, Romozzi, M, Nobile-Orazio, E, Doneddu P. E., Akyil H., Manganelli F., Briani C., Cocito D., Benedetti L., Mazzeo A., Fazio R., Filosto M., Cosentino G., Di Stefano V., Antonini G., Marfia G. A., Inghilleri M., Siciliano G., Clerici A. M., Carpo M., Schenone A., Luigetti M., Lauria G., Mata S., Rosso T., Minicuci G. M., Lucchetta M., Cavaletti G., Liberatore G., Spina E., Campagnolo M., Peci E., Germano F., Gentile L., Strano C., Cotti Piccinelli S., Vegezzi E., Leonardi L., Mataluni G., Ceccanti M., Schirinzi E., Romozzi M., Nobile-Orazio E., Doneddu, P, Akyil, H, Manganelli, F, Briani, C, Cocito, D, Benedetti, L, Mazzeo, A, Fazio, R, Filosto, M, Cosentino, G, Di Stefano, V, Antonini, G, Marfia, G, Inghilleri, M, Siciliano, G, Clerici, A, Carpo, M, Schenone, A, Luigetti, M, Lauria, G, Mata, S, Rosso, T, Minicuci, G, Lucchetta, M, Cavaletti, G, Liberatore, G, Spina, E, Campagnolo, M, Peci, E, Germano, F, Gentile, L, Strano, C, Cotti Piccinelli, S, Vegezzi, E, Leonardi, L, Mataluni, G, Ceccanti, M, Schirinzi, E, Romozzi, M, Nobile-Orazio, E, Doneddu P. E., Akyil H., Manganelli F., Briani C., Cocito D., Benedetti L., Mazzeo A., Fazio R., Filosto M., Cosentino G., Di Stefano V., Antonini G., Marfia G. A., Inghilleri M., Siciliano G., Clerici A. M., Carpo M., Schenone A., Luigetti M., Lauria G., Mata S., Rosso T., Minicuci G. M., Lucchetta M., Cavaletti G., Liberatore G., Spina E., Campagnolo M., Peci E., Germano F., Gentile L., Strano C., Cotti Piccinelli S., Vegezzi E., Leonardi L., Mataluni G., Ceccanti M., Schirinzi E., Romozzi M., and Nobile-Orazio E.

Abstract

Background To assess the ability of the 2021 European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) clinical criteria for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) to include within their classification the whole spectrum of clinical heterogeneity of the disease and to define the clinical characteristics of the unclassifiable clinical forms. Methods The 2021 EAN/PNS clinical criteria for CIDP were applied to 329 patients fulfilling the electrodiagnostic (and in some cases also the supportive) criteria for the diagnosis of CIDP. Clinical characteristics were reviewed for each patient not strictly fulfilling the clinical criteria ('unclassifiable'). Results At study inclusion, 124 (37.5%) patients had an unclassifiable clinical presentation, including 110 (89%) with a typical CIDP-like clinical phenotype in whom some segments of the four limbs were unaffected by weakness ('incomplete typical CIDP'), 10 (8%) with a mild distal, symmetric, sensory or sensorimotor polyneuropathy confined to the lower limbs with cranial nerve involvement ('cranial nerve predominant CIDP') and 4 (1%) with a symmetric sensorimotor polyneuropathy limited to the proximal and distal areas of the lower limbs ('paraparetic CIDP'). Eighty-one (65%) patients maintained an unclassifiable presentation during the entire disease follow-up while 13 patients progressed to typical CIDP. Patients with the unclassifiable clinical forms compared with patients with typical CIDP had a milder form of CIDP, while there was no difference in the distribution patterns of demyelination. Conclusions A proportion of patients with CIDP do not strictly fulfil the 2021 EAN/PNS clinical criteria for diagnosis. These unclassifiable clinical phenotypes may pose diagnostic challenges and thus deserve more attention in clinical practice and research.

Published
2023

10. Correction: Denosumab improves trabecular bone score in relationship with decrease in fracture risk of women exposed to aromatase inhibitors

Author

Antonini, S., primary, Pedersini, R., additional, Birtolo, M. F., additional, Baruch, N. L., additional, Carrone, F., additional, Jaafar, S., additional, Ciafardini, A., additional, Cosentini, D., additional, Laganà, M., additional, Torrisi, R., additional, Farina, D., additional, Leonardi, L., additional, Balzarini, L., additional, Vena, W., additional, Bossi, A. C., additional, Zambelli, A., additional, Lania, A. G., additional, Berruti, A., additional, and Mazziotti, G., additional

Published
2023
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11. Penetration force and cannula sliding profiles of different pen needles: the PICASSO study

Author

Leonardi L, Viganò M, and Nicolucci A

Subjects
Penetration force, maximum load, average sliding, Medical technology, R855-855.5
Abstract

Luca Leonardi,1 Mara Viganò,1 Antonio Nicolucci21Pikdare S.p.A., Casnate Con Bernate 22070, CO, Italy; 2CORESEARCH, Center for Outcomes Research and Clinical Epidemiology, Pescara 65124, ItalyCorrespondence: Luca LeonardiPikdare Research & Development, Via F. Catelli, 10, Casnate Con Bernate 22070, CO, ItalyTel +39 031 729 7111Fax +39 031 729 7100Email Luca.Leonardi@pikdare.comPurpose: Pen needles used for insulin injections can have different characteristics that affect a patient’s injection experience. The aim of the study was to investigate in a standardized laboratory setting the penetration force and sliding force of different 31/32/33/34 gauge pen needles available in 3.5/4/5/8 mm length and 3/5 bevel tips for subcutaneous injection through pen needles and injection pens.Methods: Eight different commercially available pen needles were tested in this experimental study. The needle was inserted into a polyurethane substrate at a specific constant speed and the force for insertion was recorded as a function of penetration depth. A load cell was utilized to measure force during the different stages of insertion.Results: Maximum load was lower with the PiC G32×4 when compared with the G32×4 5-bevel needle (p

Published
2019

12. Denosumab improves trabecular bone score in relationship with decrease in fracture risk of women exposed to aromatase inhibitors

Author

Antonini, S., primary, Pedersini, R., additional, Birtolo, M. F., additional, Baruch, N. L., additional, Carrone, F., additional, Jaafar, S., additional, Ciafardini, A., additional, Cosentini, D., additional, Laganà, M., additional, Torrisi, R., additional, Farina, D., additional, Leonardi, L., additional, Balzarini, L., additional, Vena, W., additional, Bossi, A. C., additional, Zambelli, A., additional, Lania, A. G., additional, Berruti, A., additional, and Mazziotti, G., additional

Published
2023
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13. Safety of COVID-19 Vaccines Among the Paediatric Population: Analysis of the European Surveillance Systems and Pivotal Clinical Trials

Author

Ahmadizar F., Luxi N., Raethke M., Schmikli S., Riefolo F., Saraswati P. W., Bucsa C., Osman A., Liddiard M., Maques F. B., Petrelli G., Sonderlichova S., Thurin N. H., Villalobos F., Trifiro G., Sturkenboom M., Moretti U., Bellitto C., Ciccimarra F., Gonella L. A., Arzenton E., Chiamulera C., Lora R., Bellantuono D., Sabaini A., Firenze A., Zodda D., Guidotti F., Zappone M., Alagna B., Cutroneo P. M., Minore C., Costantino C., Vitale F., D'Alessandro G., Morreale I., Marsala L., Farinella D., Bavetta S., Fantini M. P., Reno C., Raschi E., Poluzzi E., Sapigni E., Potenza A. M., Podetti D., Nikitina V., Ricciardelli R., Mogheiseh N., Croce S., Paltrinieri B., Castellani S., Sangiorgi E., Selleri M., Lucchesi S., Catucci G., Savini D., Sacripanti C., Faccioli M., Romio M. S., Rossi L., Radici S., Negri G., Fares L., Ajolfi C., Fadda A., Chiarello A., Pieraccini F., Gavioli B., Palazzi S., Tuccori M., Vannacci A., Bonaiuti R., Ravaldi C., Lombardi N., Crescioli G., Gori F., Tessari R., Zandona E., Zanoni G., Senna G., Crivellaro M. A., Cancian M., Venturini F., Ferri M., Leonardi L., Orzetti S., Caccin E., Baldo P., Capuano A., Rafaniello C., Ferrajolo C., Pagliaro C., Mercaldo M., di Giorgio A., Tari M., Manna S., Farina G., Di Mauro C., De Carlo I., Senesi I., Pileggi C., Palleria C., Gallelli L., De Sarro G., de Sarro C., Verduci C., Papadopoli R., Trabace L., Morgese M., Schiavone S., Tucci P., Bove M., Lapi F., Cricelli C., Racagni G., Tonolo S., Fava G., Giuffrida S., Amato V., Gambera M., Montresor V., Mastropasqua D., Ahmadizar F., Luxi N., Raethke M., Schmikli S., Riefolo F., Saraswati P.W., Bucsa C., Osman A., Liddiard M., Maques F.B., Petrelli G., Sonderlichova S., Thurin N.H., Villalobos F., Trifiro G., Sturkenboom M., Moretti U., Bellitto C., Ciccimarra F., Gonella L.A., Arzenton E., Chiamulera C., Lora R., Bellantuono D., Sabaini A., Firenze A., Zodda D., Guidotti F., Zappone M., Alagna B., Cutroneo P.M., Minore C., Costantino C., Vitale F., D'Alessandro G., Morreale I., Marsala L., Farinella D., Bavetta S., Fantini M.P., Reno C., Raschi E., Poluzzi E., Sapigni E., Potenza A.M., Podetti D., Nikitina V., Ricciardelli R., Mogheiseh N., Croce S., Paltrinieri B., Castellani S., Sangiorgi E., Selleri M., Lucchesi S., Catucci G., Savini D., Sacripanti C., Faccioli M., Romio M.S., Rossi L., Radici S., Negri G., Fares L., Ajolfi C., Fadda A., Chiarello A., Pieraccini F., Gavioli B., Palazzi S., Tuccori M., Vannacci A., Bonaiuti R., Ravaldi C., Lombardi N., Crescioli G., Gori F., Tessari R., Zandona E., Zanoni G., Senna G., Crivellaro M.A., Cancian M., Venturini F., Ferri M., Leonardi L., Orzetti S., Caccin E., Baldo P., Capuano A., Rafaniello C., Ferrajolo C., Pagliaro C., Mercaldo M., di Giorgio A., Tari M., Manna S., Farina G., Di Mauro C., De Carlo I., Senesi I., Pileggi C., Palleria C., Gallelli L., De Sarro G., de Sarro C., Verduci C., Papadopoli R., Trabace L., Morgese M., Schiavone S., Tucci P., Bove M., Lapi F., Cricelli C., Racagni G., Tonolo S., Fava G., Giuffrida S., Amato V., Gambera M., Montresor V., and Mastropasqua D.

Subjects
COVID-19 Vaccines, safety, Surveillance Systems, Pivotal Clinical Trials
Abstract

Background and Objectives: The European Medicine Agency extended the use of Comirnaty, Spikevax, and Nuvaxovid in paediatrics; thus, these vaccines require additional real-world safety evidence. Herein, we aimed to monitor the safety of COVID-19 vaccines through Covid-19 Vaccine Monitor (CVM) and EudraVigilance surveillance systems and the published pivotal clinical trials. Methods: In a prospective cohort of vaccinees aged between 5 and 17 years, we measured the frequency of commonly reported (local/systemic solicited) and serious adverse drug events (ADRs) following the first and second doses of COVID-19 vaccines in Europe using data from the CVM cohort until April 2022. The results of previous pivotal clinical trials and data in the EudraVigilance were also analysed. Results: The CVM study enrolled 658 first-dose vaccinees (children aged 5–11 years; n = 250 and adolescents aged 12–17 years; n = 408). Local/systemic solicited ADRs were common, whereas serious ADRs were uncommon. Among Comirnaty first and second dose recipients, 28.8% and 17.1% of children and 54.2% and 52.2% of adolescents experienced at least one ADR, respectively; injection-site pain (29.2% and 20.7%), fatigue (16.1% and 12.8%), and headache (22.1% and 19.3%) were the most frequent local and systemic ADRs. Results were consistent but slightly lower than in pivotal clinical trials. Reporting rates in Eudravigilance were lower by a factor of 1000. Conclusions: The CVM study showed high frequencies of local solicited reactions after vaccination but lower rates than in pivotal clinical trials. Injection-site pain, fatigue, and headache were the most commonly reported ADRs for clinical trials, but higher than spontaneously reported data.

Published
2023

14. Prolonged distal motor latency of median nerve does not improve diagnostic accuracy for CIDP

Author

Spina, E, Doneddu, P, Liberatore, G, Cocito, D, Fazio, R, Briani, C, Filosto, M, Benedetti, L, Antonini, G, Cosentino, G, Jann, S, Mazzeo, A, Cortese, A, Marfia, G, Clerici, A, Siciliano, G, Carpo, M, Luigetti, M, Lauria, G, Rosso, T, Cavaletti, G, Peci, E, Tronci, S, Ruiz, M, Piccinelli, S, Schenone, A, Leonardi, L, Gentile, L, Piccolo, L, Mataluni, G, Santoro, L, Nobile-Orazio, E, Manganelli, F, Spina E., Doneddu P. E., Liberatore G., Cocito D., Fazio R., Briani C., Filosto M., Benedetti L., Antonini G., Cosentino G., Jann S., Mazzeo A., Cortese A., Marfia G. A., Clerici A. M., Siciliano G., Carpo M., Luigetti M., Lauria G., Rosso T., Cavaletti G., Peci E., Tronci S., Ruiz M., Piccinelli S. C., Schenone A., Leonardi L., Gentile L., Piccolo L., Mataluni G., Santoro L., Nobile-Orazio E., Manganelli F., Spina, E, Doneddu, P, Liberatore, G, Cocito, D, Fazio, R, Briani, C, Filosto, M, Benedetti, L, Antonini, G, Cosentino, G, Jann, S, Mazzeo, A, Cortese, A, Marfia, G, Clerici, A, Siciliano, G, Carpo, M, Luigetti, M, Lauria, G, Rosso, T, Cavaletti, G, Peci, E, Tronci, S, Ruiz, M, Piccinelli, S, Schenone, A, Leonardi, L, Gentile, L, Piccolo, L, Mataluni, G, Santoro, L, Nobile-Orazio, E, Manganelli, F, Spina E., Doneddu P. E., Liberatore G., Cocito D., Fazio R., Briani C., Filosto M., Benedetti L., Antonini G., Cosentino G., Jann S., Mazzeo A., Cortese A., Marfia G. A., Clerici A. M., Siciliano G., Carpo M., Luigetti M., Lauria G., Rosso T., Cavaletti G., Peci E., Tronci S., Ruiz M., Piccinelli S. C., Schenone A., Leonardi L., Gentile L., Piccolo L., Mataluni G., Santoro L., Nobile-Orazio E., and Manganelli F.

Abstract

Compression of the median nerve at the carpal tunnel can give demyelinating features and result in distal motor latency (DML) prolongation fulfilling the EFNS/PNS demyelinating criteria for chronic inflammatory demyelinating polyneuropathy (CIDP). Accordingly, being carpal tunnel syndrome (CTS) common in the general population, the EFNS/PNS guidelines recommend excluding the DML of the median nerve when DML prolongation may be consistent with median neuropathy at the wrist from CTS. The main aims of this study were to verify whether the inclusion of DML of the median nerve (when consistent with CTS) could improve electrophysiological diagnostic accuracy for CIDP and if the median nerve at the carpal tunnel was more prone to demyelination. We analyzed electrophysiological data from 499 patients included consecutively into the Italian CIDP Database. According to the EFNS/PNS criteria, 352 patients had a definite, 10 a probable, and 57 a possible diagnosis of CIDP, while 80 were not fulfilling the diagnostic criteria. The inclusion of DML prolongation of median nerve did not improve significantly the diagnostic accuracy for CIDP; overall diagnostic class changed in 6 out of 499 patients (1.2%) and electrodiagnostic class of CIDP changed from not fulfilling to possible in only 2 patients (2.5% of not-fulfilling patients). In conclusion, we can infer that excluding DML prolongation of median nerve does not increase the risk of missing a diagnosis of CIDP thus corroborating the current EFNS/PNS criteria.

Published
2022

15. The neurophysiological lesson from the Italian CIDP database

Author

Spina, E, Doneddu, P, Liberatore, G, Cocito, D, Fazio, R, Briani, C, Filosto, M, Benedetti, L, Antonini, G, Cosentino, G, Jann, S, Mazzeo, A, Cortese, A, Marfia, G, Clerici, A, Siciliano, G, Carpo, M, Luigetti, M, Lauria, G, Rosso, T, Cavaletti, G, Peci, E, Tronci, S, Ruiz, M, Piccinelli, S, Schenone, A, Leonardi, L, Gentile, L, Piccolo, L, Mataluni, G, Santoro, L, Nobile-Orazio, E, Manganelli, F, Spina E., Doneddu P. E., Liberatore G., Cocito D., Fazio R., Briani C., Filosto M., Benedetti L., Antonini G., Cosentino G., Jann S., Mazzeo A., Cortese A., Marfia G. A., Clerici A. M., Siciliano G., Carpo M., Luigetti M., Lauria G., Rosso T., Cavaletti G., Peci E., Tronci S., Ruiz M., Piccinelli S. C., Schenone A., Leonardi L., Gentile L., Piccolo L., Mataluni G., Santoro L., Nobile-Orazio E., Manganelli F., Spina, E, Doneddu, P, Liberatore, G, Cocito, D, Fazio, R, Briani, C, Filosto, M, Benedetti, L, Antonini, G, Cosentino, G, Jann, S, Mazzeo, A, Cortese, A, Marfia, G, Clerici, A, Siciliano, G, Carpo, M, Luigetti, M, Lauria, G, Rosso, T, Cavaletti, G, Peci, E, Tronci, S, Ruiz, M, Piccinelli, S, Schenone, A, Leonardi, L, Gentile, L, Piccolo, L, Mataluni, G, Santoro, L, Nobile-Orazio, E, Manganelli, F, Spina E., Doneddu P. E., Liberatore G., Cocito D., Fazio R., Briani C., Filosto M., Benedetti L., Antonini G., Cosentino G., Jann S., Mazzeo A., Cortese A., Marfia G. A., Clerici A. M., Siciliano G., Carpo M., Luigetti M., Lauria G., Rosso T., Cavaletti G., Peci E., Tronci S., Ruiz M., Piccinelli S. C., Schenone A., Leonardi L., Gentile L., Piccolo L., Mataluni G., Santoro L., Nobile-Orazio E., and Manganelli F.

Abstract

Introduction: Electrophysiological diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) may be challenging. Thus, with the aim ofproviding some practical advice in electrophysiological approach to a patient with suspected CIDP, we analyzed electrophysiological data from 499 patients enrolled inthe Italian CIDP Database. Methods: We calculated the rate of each demyelinating feature, the rate of demyelinating features per nerve, the diagnostic rate for upper andlower limb nerves, and, using a ROC curve analysis, the diagnostic accuracy of each couple of nerves and each demyelinating feature, for every CIDP subtype.Moreover, we compared the electrophysiological data of definite and probable CIDP patients with those of possible and not-fulfilling CIDP patients, and by a logisticregression analysis, we estimated the odds ratio (OR) to make an electrophysiological diagnosis of definite or probable CIDP. Results: The ulnar nerve had the highestrate of demyelinating features and, when tested bilaterally, had the highest diagnostic accuracy except for DADS in which peroneal nerves were the most informative.In possible and not-fulfilling CIDP patients, a lower number of nerves and proximal temporal dispersion (TD) measurements had been performed compared to definiteand probable CIDP patients. Importantly, OR for each tested motor nerve and each TD measurement was 1.59 and 1.33, respectively. Conclusion: Our findingsdemonstrated that the diagnosis of CIDP may be missed due to inadequate or incomplete electrophysiological examination or interpretation. At the same time, thesedata taken together could be useful to draw a thoughtful electrophysiological approach to patients suspected of CIDP.

Published
2022

16. Recommendations on Complementary Feeding as a Tool for Prevention of Non-Communicable Diseases (NCDs)—Paper Co-Drafted by the SIPPS, FIMP, SIDOHaD, and SINUPE Joint Working Group

Author

Caroli, M, Vania, A, Verga, M, Di Mauro, G, Bergamini, M, Cuomo, B, D'Anna, R, D'Antonio, G, Iacono, I, Dessi, A, Doria, M, Fanos, V, Fiore, M, Francavilla, R, Genovesi, S, Giussani, M, Gritti, A, Iafusco, D, Leonardi, L, Miniello, V, Del Giudice, E, Palma, F, Pastore, F, Scotese, I, Simeone, G, Squicciarini, M, Tezza, G, Troiano, E, Umano, G, Caroli M., Vania A., Verga M. C., Di Mauro G., Bergamini M., Cuomo B., D'anna R., D'antonio G., Iacono I. D., Dessi A., Doria M., Fanos V., Fiore M., Francavilla R., Genovesi S., Giussani M., Gritti A., Iafusco D., Leonardi L., Miniello V. L., Del Giudice E. M., Palma F., Pastore F., Scotese I., Simeone G., Squicciarini M., Tezza G., Troiano E., Umano G. R., Caroli, M, Vania, A, Verga, M, Di Mauro, G, Bergamini, M, Cuomo, B, D'Anna, R, D'Antonio, G, Iacono, I, Dessi, A, Doria, M, Fanos, V, Fiore, M, Francavilla, R, Genovesi, S, Giussani, M, Gritti, A, Iafusco, D, Leonardi, L, Miniello, V, Del Giudice, E, Palma, F, Pastore, F, Scotese, I, Simeone, G, Squicciarini, M, Tezza, G, Troiano, E, Umano, G, Caroli M., Vania A., Verga M. C., Di Mauro G., Bergamini M., Cuomo B., D'anna R., D'antonio G., Iacono I. D., Dessi A., Doria M., Fanos V., Fiore M., Francavilla R., Genovesi S., Giussani M., Gritti A., Iafusco D., Leonardi L., Miniello V. L., Del Giudice E. M., Palma F., Pastore F., Scotese I., Simeone G., Squicciarini M., Tezza G., Troiano E., and Umano G. R.

Abstract

Adequate and balanced nutrition is essential to promote optimal child growth and a long and healthy life. After breastfeeding, the second step is the introduction of complementary feeding (CF), a process that typically covers the period from 6 to 24 months of age. This process is, however, still highly controversial, as it is heavily influenced by socio-cultural choices, as well as by the availability of specific local foods, by family traditions, and pediatrician beliefs. The Società Italiana di Pediatria Preventiva e Sociale (SIPPS) together with the Federazione Italiana Medici Pediatri (FIMP), the Società Italiana per lo Sviluppo e le Origine della Salute e delle Malattie (SIDOHaD), and the Società Italiana di Nutrizione Pediatrica (SINUPE) have developed evidence-based recommendations for CF, given the importance of nutrition in the first 1000 days of life in influencing even long-term health outcomes. This paper includes 38 recommendations, all of them strictly evidence-based and overall addressed to developed countries. The recommendations in question cover several top-ics such as the appropriate age for the introduction of CF, the most appropriate quantitative and qualitative modalities to be chosen, and the relationship between CF and the development of Non-Communicable Diseases (NCDs) later in life.

Published
2022

18. Metabolomics to identify omalizumab responders among children with severe asthma: A prospective study

Author

Carraro, S., Di Palmo, E., Licari, A., Barni, S., Caldarelli, V., De Castro, G., Di Marco, A., Fenu, G., Giordano, G., Lombardi, E., Pirillo, P., Stocchero, M., Volpini, A., Zanconato, S., Rusconi, F., Capizzi, A., Tosca, M. A., Cazzato, S., Cutrera, R., Leonardi, L., Leonardi, S., Marseglia, G. L., Mori, F., Patria, M. F., Piacentini, G., Tenero, L., Pisi, G., and Ricci, G.

Subjects
Immunology, Immunology and Allergy
Published
2022

22. CO-09.2 - SEMI-QUANTITATIVE ANALYSIS ON IOFLUPANE (I-123) BRAIN SPECT: A MULTICENTRE INTERCOMPARISON

Author

Campanaro, F., Morbelli, S., Dominguez, V.A., Mezzaglia, S., Bagnalasta, M., Bagnara, M.C., De Pascalis, G., Bianchi, C., Rosso, V., Grikke, L., Zito, F., Savi, A., Olivieri, M., D’Ambrosio, D., Zoccarato, O., Capotosti, A., Antunovic, L., Leonardi, L., Trifiro’, G., Campini, R., Calcagni, M.L., Chiti, A., Pellencin, E., Castellani, M., Perani, D., Tagliavini, F., Erba, A.P., and Poli, G.L.

Published
2023
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25. Risk of disease relapse, safety and tolerability of SARS-CoV-2 vaccination in patients with chronic inflammatory neuropathies

Author

Doneddu, P. E., Briani, C., Cocito, D., Manganelli, F., Fabrizi, G. M., Matà, S., Mazzeo, A., Fazio, R., Benedetti, L., Luigetti, M., Inghilleri, M., Ruiu, E., Siciliano, G., Cosentino, G., Marfia, G. A., Carpo, M., Filosto, M., Antonini, G., Notturno, F., Sotgiu, S., Cucurachi, L., Dell'Aquila, C., Bianchi, E., Rosso, T., Giordano, A., Fernandes, M., Campagnolo, M., Peci, E., Spina, E., Tagliapietra, M., Sperti, M., Gentile, L., Strano, C., Germano, F., Romozzi, M., Moret, F., Zarbo, I. R., Viola, D. V., Vegezzi, E., Mataluni, G., Cotti-Piccinelli, S., Leonardi, L., Carta, A., and Nobile-Orazio, E.

Subjects
COVID-19, SARS-CoV-2, chronic inflammatory demyelinating polyneuropathy, multifocal motor neuropathy, vaccination, Settore MED/26
Published
2023

26. Chronic inflammatory demyelinating polyradiculoneuropathy: can a diagnosis be made in patients not fulfilling electrodiagnostic criteria?

Author

Liberatore, G, Manganelli, F, Doneddu, P, Cocito, D, Fazio, R, Briani, C, Filosto, M, Benedetti, L, Mazzeo, A, Antonini, G, Cosentino, G, Jann, S, Cortese, A, Marfia, G, Clerici, A, Siciliano, G, Carpo, M, Luigetti, M, Lauria, G, Rosso, T, Cavaletti, G, Santoro, L, Peci, E, Tronci, S, Ruiz, M, Cotti Piccinelli, S, Schenone, A, Leonardi, L, Toscano, A, Mataluni, G, Spina, E, Gentile, L, Nobile-Orazio, E, Gallia, F, Beronio, A, Piccolo, L, Fierro, B, Verrengia, E, Bianchi, E, Beghi, E, Scrascia, F, Garnero, M, Sabatelli, M, Dacci, P, Schirinzi, E, Balducci, C, Liberatore G., Manganelli F., Doneddu P. E., Cocito D., Fazio R., Briani C., Filosto M., Benedetti L., Mazzeo A., Antonini G., Cosentino G., Jann S., Cortese A., Marfia G. A., Clerici A. M., Siciliano G., Carpo M., Luigetti M., Lauria G., Rosso T., Cavaletti G., Santoro L., Peci E., Tronci S., Ruiz M., Cotti Piccinelli S., Schenone A., Leonardi L., Toscano A., Mataluni G., Spina E., Gentile L., Nobile-Orazio E., Gallia F., Beronio A., Piccolo L., Fierro B., Verrengia E. P., Bianchi E., Beghi E., Scrascia F., Garnero M., Sabatelli M., Dacci P., Schirinzi E., Balducci C., Liberatore, G, Manganelli, F, Doneddu, P, Cocito, D, Fazio, R, Briani, C, Filosto, M, Benedetti, L, Mazzeo, A, Antonini, G, Cosentino, G, Jann, S, Cortese, A, Marfia, G, Clerici, A, Siciliano, G, Carpo, M, Luigetti, M, Lauria, G, Rosso, T, Cavaletti, G, Santoro, L, Peci, E, Tronci, S, Ruiz, M, Cotti Piccinelli, S, Schenone, A, Leonardi, L, Toscano, A, Mataluni, G, Spina, E, Gentile, L, Nobile-Orazio, E, Gallia, F, Beronio, A, Piccolo, L, Fierro, B, Verrengia, E, Bianchi, E, Beghi, E, Scrascia, F, Garnero, M, Sabatelli, M, Dacci, P, Schirinzi, E, Balducci, C, Liberatore G., Manganelli F., Doneddu P. E., Cocito D., Fazio R., Briani C., Filosto M., Benedetti L., Mazzeo A., Antonini G., Cosentino G., Jann S., Cortese A., Marfia G. A., Clerici A. M., Siciliano G., Carpo M., Luigetti M., Lauria G., Rosso T., Cavaletti G., Santoro L., Peci E., Tronci S., Ruiz M., Cotti Piccinelli S., Schenone A., Leonardi L., Toscano A., Mataluni G., Spina E., Gentile L., Nobile-Orazio E., Gallia F., Beronio A., Piccolo L., Fierro B., Verrengia E. P., Bianchi E., Beghi E., Scrascia F., Garnero M., Sabatelli M., Dacci P., Schirinzi E., and Balducci C.

Abstract

Background and purpose: The aim was to identify the clinical and diagnostic investigations that may help to support a diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in patients not fulfilling the European Federation of Neurological Societies and Peripheral Nerve Society (EFNS/PNS) electrodiagnostic criteria. Methods: The data from patients with a clinical diagnosis of CIDP included in a national database were retrospectively reviewed. Results: In all, 535 patients with a diagnosis of CIDP were included. This diagnosis fulfilled the EFNS/PNS criteria in 468 patients (87.2%) (definite in 430, probable in 33, possible in three, while two had chronic immune sensory polyradiculopathy). Sixty-seven patients had a medical history and clinical signs compatible with CIDP but electrodiagnostic studies did not fulfill the EFNS/PNS criteria for CIDP. These patients had similar clinical features and frequency of abnormal supportive criteria for the diagnosis of CIDP compared to patients fulfilling EFNS/PNS criteria. Two or more abnormal supportive criteria were present in 40 (61.2%) patients rising to 54 (80.6%) if a history of a relapsing course as a possible supportive criterion was also included. Increased cerebrospinal fluid proteins and response to immune therapy most frequently helped in supporting the diagnosis of CIDP. Response to therapy was similarly frequent in patients fulfilling or not EFNS/PNS criteria (87.3% vs. 85.9%). Conclusions: Patients with a clinical diagnosis of CIDP had similar clinical findings, frequency of abnormal supportive criteria and response to therapy compared to patients fulfilling EFNS/PNS criteria. The presence of abnormal supportive criteria may help in supporting the diagnosis of CIDP in patients with a medical history and clinical signs compatible with this diagnosis but non-diagnostic nerve conduction studies.

Published
2021

28. Chronic inflammatory demyelinating polyradiculoneuropathy: can a diagnosis be made in patients not fulfilling electrodiagnostic criteria?

Author

Liberatore, G., Manganelli, F., Doneddu, P. E., Cocito, D., Fazio, R., Briani, C., Filosto, M., Benedetti, L., Mazzeo, A., Antonini, G., Cosentino, G., Jann, S., Cortese, A., Marfia, G. A., Clerici, A. M., Siciliano, G., Carpo, M., Luigetti, M., Lauria, G., Rosso, T., Cavaletti, G., Santoro, L., Peci, E., Tronci, S., Ruiz, M., Cotti Piccinelli, S., Schenone, A., Leonardi, L., Toscano, A., Mataluni, G., Spina, E., Gentile, L., Nobile-Orazio, E., Gallia, F., Beronio, A., Piccolo, L., Fierro, B., Verrengia, E. P., Bianchi, E., Beghi, E., Scrascia, F., Garnero, M., Sabatelli, M., Dacci, P., Schirinzi, E., Balducci, C., Liberatore, G., Manganelli, F., Doneddu, P. E., Cocito, D., Fazio, R., Briani, C., Filosto, M., Benedetti, L., Mazzeo, A., Antonini, G., Cosentino, G., Jann, S., Cortese, A., Marfia, G. A., Clerici, A. M., Siciliano, G., Carpo, M., Luigetti, M., Lauria, G., Rosso, T., Cavaletti, G., Santoro, L., Peci, E., Tronci, S., Ruiz, M., Cotti-Piccinelli, S., Schenone, A., Leonardi, L., Toscano, A., Mataluni, G., Spina, E., Gentile, L., Nobile-Orazio, E., Gallia, F., Beronio, A., Piccolo, L., Fierro, B., Verrengia, E. P., Bianchi, E., Beghi, E., Scrascia, F., Garnero, M., Sabatelli, M., Dacci, P., Toni, G., Schirinzi, E., Balducci, C., Liberatore, G, Manganelli, F, Doneddu, P, Cocito, D, Fazio, R, Briani, C, Filosto, M, Benedetti, L, Mazzeo, A, Antonini, G, Cosentino, G, Jann, S, Cortese, A, Marfia, G, Clerici, A, Siciliano, G, Carpo, M, Luigetti, M, Lauria, G, Rosso, T, Cavaletti, G, Santoro, L, Peci, E, Tronci, S, Ruiz, M, Cotti Piccinelli, S, Schenone, A, Leonardi, L, Toscano, A, Mataluni, G, Spina, E, Gentile, L, Nobile-Orazio, E, Gallia, F, Beronio, A, Piccolo, L, Fierro, B, Verrengia, E, Bianchi, E, Beghi, E, Scrascia, F, Garnero, M, Sabatelli, M, Dacci, P, Schirinzi, E, and Balducci, C

Subjects
Pediatrics, medicine.medical_specialty, Response to therapy, Databases, Factual, Neural Conduction, Settore MED/26, 03 medical and health sciences, 0302 clinical medicine, Peripheral nerve, Retrospective Studie, Medicine, Humans, Medical history, In patient, 030212 general & internal medicine, Peripheral Nerves, Retrospective Studies, chronic inflammatory demyelinating polyradiculoneuropathy, business.industry, Polyradiculoneuropathy, Polyradiculopathy, medicine.disease, electrophysiology, Settore MED/26 - NEUROLOGIA, Neurology, Polyradiculoneuropathy, Chronic Inflammatory Demyelinating, Clinical diagnosis, Peripheral Nerve, diagnostic criteria, National database, Neurology (clinical), business, 030217 neurology & neurosurgery, Human
Abstract

Background and purpose The aim was to identify the clinical and diagnostic investigations that may help to support a diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in patients not fulfilling the European Federation of Neurological Societies and Peripheral Nerve Society (EFNS/PNS) electrodiagnostic criteria. Methods The data from patients with a clinical diagnosis of CIDP included in a national database were retrospectively reviewed. Results In all, 535 patients with a diagnosis of CIDP were included. This diagnosis fulfilled the EFNS/PNS criteria in 468 patients (87.2%) (definite in 430, probable in 33, possible in three, while two had chronic immune sensory polyradiculopathy). Sixty-seven patients had a medical history and clinical signs compatible with CIDP but electrodiagnostic studies did not fulfill the EFNS/PNS criteria for CIDP. These patients had similar clinical features and frequency of abnormal supportive criteria for the diagnosis of CIDP compared to patients fulfilling EFNS/PNS criteria. Two or more abnormal supportive criteria were present in 40 (61.2%) patients rising to 54 (80.6%) if a history of a relapsing course as a possible supportive criterion was also included. Increased cerebrospinal fluid proteins and response to immune therapy most frequently helped in supporting the diagnosis of CIDP. Response to therapy was similarly frequent in patients fulfilling or not EFNS/PNS criteria (87.3% vs. 85.9%). Conclusions Patients with a clinical diagnosis of CIDP had similar clinical findings, frequency of abnormal supportive criteria and response to therapy compared to patients fulfilling EFNS/PNS criteria. The presence of abnormal supportive criteria may help in supporting the diagnosis of CIDP in patients with a medical history and clinical signs compatible with this diagnosis but non-diagnostic nerve conduction studies.

Published
2021

29. Body composition, trabecular bone score and vertebral fractures in subjects with Klinefelter syndrome

Author

Vena, W., primary, Carrone, F., additional, Delbarba, A., additional, Akpojiyovbi, O., additional, Pezzaioli, L. C., additional, Facondo, P., additional, Cappelli, C., additional, Leonardi, L., additional, Balzarini, L., additional, Farina, D., additional, Pizzocaro, A., additional, Lania, A. G., additional, Mazziotti, G., additional, and Ferlin, A., additional

Published
2022
Full Text
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30. Frequency of diabetes and other comorbidities in chronic inflammatory demyelinating polyradiculoneuropathy and their impact on clinical presentation and response to therapy

Author

Doneddu, P, Cocito, D, Manganelli, F, Fazio, R, Briani, C, Filosto, M, Benedetti, L, Bianchi, E, Jann, S, Mazzeo, A, Antonini, G, Cosentino, G, Marfia, G, Cortese, A, Clerici, A, Carpo, M, Schenone, A, Siciliano, G, Luigetti, M, Lauria, G, Rosso, T, Cavaletti, G, Beghi, E, Liberatore, G, Santoro, L, Spina, E, Peci, E, Tronci, S, Ruiz, M, Cotti Piccinelli, S, Verrengia, E, Gentile, L, Leonardi, L, Mataluni, G, Piccolo, L, Nobile-Orazio, E, Doneddu P. E., Cocito D., Manganelli F., Fazio R., Briani C., Filosto M., Benedetti L., Bianchi E., Jann S., Mazzeo A., Antonini G., Cosentino G., Marfia G. A., Cortese A., Clerici A. M., Carpo M., Schenone A., Siciliano G., Luigetti M., Lauria G., Rosso T., Cavaletti G., Beghi E., Liberatore G., Santoro L., Spina E., Peci E., Tronci S., Ruiz M., Cotti Piccinelli S., Verrengia E. P., Gentile L., Leonardi L., Mataluni G., Piccolo L., Nobile-Orazio E., Doneddu, P, Cocito, D, Manganelli, F, Fazio, R, Briani, C, Filosto, M, Benedetti, L, Bianchi, E, Jann, S, Mazzeo, A, Antonini, G, Cosentino, G, Marfia, G, Cortese, A, Clerici, A, Carpo, M, Schenone, A, Siciliano, G, Luigetti, M, Lauria, G, Rosso, T, Cavaletti, G, Beghi, E, Liberatore, G, Santoro, L, Spina, E, Peci, E, Tronci, S, Ruiz, M, Cotti Piccinelli, S, Verrengia, E, Gentile, L, Leonardi, L, Mataluni, G, Piccolo, L, Nobile-Orazio, E, Doneddu P. E., Cocito D., Manganelli F., Fazio R., Briani C., Filosto M., Benedetti L., Bianchi E., Jann S., Mazzeo A., Antonini G., Cosentino G., Marfia G. A., Cortese A., Clerici A. M., Carpo M., Schenone A., Siciliano G., Luigetti M., Lauria G., Rosso T., Cavaletti G., Beghi E., Liberatore G., Santoro L., Spina E., Peci E., Tronci S., Ruiz M., Cotti Piccinelli S., Verrengia E. P., Gentile L., Leonardi L., Mataluni G., Piccolo L., and Nobile-Orazio E.

Abstract

Objectives To determine the prevalence of different comorbidities in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), and their impact on outcome, treatment choice and response. Methods Using a structured questionnaire, we collected information on comorbidities from 393 patients with CIDP fulfilling the European Federation of Neurological Societies and Peripheral Nerve Society criteria included in the Italian CIDP database. Results One or more comorbidities were reported by 294 patients (75%) and potentially influenced treatment choice in 192 (49%) leading to a less frequent use of corticosteroids. Response to treatment did not differ, however, from that in patients without comorbidities. Diabetes (14%), monoclonal gammopathy of undetermined significance (MGUS) (12%) and other immune disorders (16%) were significantly more frequent in patients with CIDP than expected in the general European population. Patients with diabetes had higher disability scores, worse quality of life and a less frequent treatment response compared with patients without diabetes. Patients with IgG-IgA or IgM MGUS had an older age at CIDP onset while patients with other immune disorders had a younger age at onset and were more frequently females. IgM MGUS was more frequent in patients with motor CIDP than in patients with typical CIDP. Conclusions Comorbidities are frequent in patients with CIDP and in almost 50% of them have an impact on treatment choice. Diabetes, MGUS and other immune diseases are more frequent in patients with CIDP than in the general population. Only diabetes seems, however, to have an impact on disease severity and treatment response possibly reflecting in some patients a coexisting diabetic neuropathy.

Published
2020

31. Impact of environmental factors and physical activity on disability and quality of life in CIDP

Author

Doneddu, P, Bianchi, E, Cocito, D, Manganelli, F, Fazio, R, Filosto, M, Beghi, E, Mazzeo, A, Cosentino, G, Cortese, A, Jann, S, Clerici, A, Antonini, G, Siciliano, G, Marfia, G, Briani, C, Lauria, G, Rosso, T, Cavaletti, G, Carpo, M, Benedetti, L, Schenone, A, Liberatore, G, Peci, E, Spina, E, Tronci, S, Cotti Piccinelli, S, Toscano, A, Gentile, L, Piccolo, L, Leonardi, L, Mataluni, G, Ruiz, M, Sabatelli, M, Santoro, L, Nobile-Orazio, E, Gallia, F, Velardo, D, Topa, A, Cotti-Piccinelli, S, Todeschini, A, Cabona, C, Zuppa, A, Callegari, I, Verrengia, E, Scrascia, F, Garnero, M, Luigetti, M, Dacci, P, Schirinzi, E, Balducci, C, Doneddu P. E., Bianchi E., Cocito D., Manganelli F., Fazio R., Filosto M., Beghi E., Mazzeo A., Cosentino G., Cortese A., Jann S., Clerici A. M., Antonini G., Siciliano G., Marfia G. A., Briani C., Lauria G., Rosso T., Cavaletti G., Carpo M., Benedetti L., Schenone A., Liberatore G., Peci E., Spina E., Tronci S., Cotti Piccinelli S., Toscano A., Gentile L., Piccolo L., Leonardi L., Mataluni G., Ruiz M., Sabatelli M., Santoro L., Nobile-Orazio E., Gallia F., Velardo D., Topa A., Cotti-Piccinelli S., Todeschini A., Cabona C., Zuppa A., Callegari I., Verrengia E. P., Scrascia F., Garnero M., Luigetti M., Dacci P., Schirinzi E., Balducci C., Doneddu, P, Bianchi, E, Cocito, D, Manganelli, F, Fazio, R, Filosto, M, Beghi, E, Mazzeo, A, Cosentino, G, Cortese, A, Jann, S, Clerici, A, Antonini, G, Siciliano, G, Marfia, G, Briani, C, Lauria, G, Rosso, T, Cavaletti, G, Carpo, M, Benedetti, L, Schenone, A, Liberatore, G, Peci, E, Spina, E, Tronci, S, Cotti Piccinelli, S, Toscano, A, Gentile, L, Piccolo, L, Leonardi, L, Mataluni, G, Ruiz, M, Sabatelli, M, Santoro, L, Nobile-Orazio, E, Gallia, F, Velardo, D, Topa, A, Cotti-Piccinelli, S, Todeschini, A, Cabona, C, Zuppa, A, Callegari, I, Verrengia, E, Scrascia, F, Garnero, M, Luigetti, M, Dacci, P, Schirinzi, E, Balducci, C, Doneddu P. E., Bianchi E., Cocito D., Manganelli F., Fazio R., Filosto M., Beghi E., Mazzeo A., Cosentino G., Cortese A., Jann S., Clerici A. M., Antonini G., Siciliano G., Marfia G. A., Briani C., Lauria G., Rosso T., Cavaletti G., Carpo M., Benedetti L., Schenone A., Liberatore G., Peci E., Spina E., Tronci S., Cotti Piccinelli S., Toscano A., Gentile L., Piccolo L., Leonardi L., Mataluni G., Ruiz M., Sabatelli M., Santoro L., Nobile-Orazio E., Gallia F., Velardo D., Topa A., Cotti-Piccinelli S., Todeschini A., Cabona C., Zuppa A., Callegari I., Verrengia E. P., Scrascia F., Garnero M., Luigetti M., Dacci P., Schirinzi E., and Balducci C.

Abstract

A few observational studies and randomized trials suggest that exercise and rehabilitation may improve activity limitation and quality of life (QoL) in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), but the impact of other modifiable factors on the severity of the disease is not well understood. Using a structured questionnaire, we collected data on lifestyle and dietary habits of the patients included in the Italian CIDP database to investigate the possible influence of modifiable lifestyle factors on disability and QoL. Questionnaire data were available for 323 patients. The effect of lifestyle and dietary exposures on impairment, disability and QoL was evaluated using logistic regression models, adjusting for age, sex, disease duration, physical activity and smoking. Physical activity was associated with lower sensory impairment by the ISS scale, less disability by the INCAT and RODS scale and a better QoL in all the domains of EURO-QoL scale with the exception of anxiety/depression. None of the other parameters had an impact on these scales. This study adds evidence to the possible role of physical activity in improving symptom severity, disability and QoL in patients with CIDP. None of the other environmental factors investigated appeared to have an impact on the severity and health perception of CIDP.

Published
2020

32. Relevance of diagnostic investigations in chronic inflammatory demyelinating poliradiculoneuropathy: Data from the Italian CIDP database

Author

Liberatore, G, Manganelli, F, Cocito, D, Fazio, R, Briani, C, Filosto, M, Benedetti, L, Antonini, G, Cosentino, G, Jann, S, Mazzeo, A, Cortese, A, Marfia, G, Clerici, A, Siciliano, G, Carpo, M, Sabatelli, M, Lauria, G, Rosso, T, Nobile Orazio, E, Doneddu, P, Gallia, F, Nobile-Orazio, E, Peci, E, Tronci, S, Santoro, L, Spina, E, Ruiz, M, Piccinelli, S, Beronio, A, Toscano, A, Gentile, L, Mataluni, G, Piccolo, L, Callegari, I, Fierro, B, Pinuccia, V, Bianchi, E, Beghi, E, Scrascia, F, Garnero, M, Schenone, A, Luigetti, M, Dacci, P, Leonardi, L, Schirinzi, E, Balducci, C, Cavaletti, G, Liberatore G., Manganelli F., Cocito D., Fazio R., Briani C., Filosto M., Benedetti L., Antonini G., Cosentino G., Jann S., Mazzeo A., Cortese A., Marfia G. A., Clerici A. M., Siciliano G., Carpo M., Sabatelli M., Lauria G., Rosso T., Nobile Orazio E., Doneddu P. E., Gallia F., Nobile-Orazio E., Peci E., Tronci S., Santoro L., Spina E., Ruiz M., Piccinelli S. C., Beronio A., Toscano A., Gentile L., Mataluni G., Piccolo L., Callegari I., Fierro B., Pinuccia V. E., Bianchi E., Beghi E., Scrascia F., Garnero M., Schenone A., Luigetti M., Dacci P., Leonardi L., Schirinzi E., Balducci C., Cavaletti G., Liberatore, G, Manganelli, F, Cocito, D, Fazio, R, Briani, C, Filosto, M, Benedetti, L, Antonini, G, Cosentino, G, Jann, S, Mazzeo, A, Cortese, A, Marfia, G, Clerici, A, Siciliano, G, Carpo, M, Sabatelli, M, Lauria, G, Rosso, T, Nobile Orazio, E, Doneddu, P, Gallia, F, Nobile-Orazio, E, Peci, E, Tronci, S, Santoro, L, Spina, E, Ruiz, M, Piccinelli, S, Beronio, A, Toscano, A, Gentile, L, Mataluni, G, Piccolo, L, Callegari, I, Fierro, B, Pinuccia, V, Bianchi, E, Beghi, E, Scrascia, F, Garnero, M, Schenone, A, Luigetti, M, Dacci, P, Leonardi, L, Schirinzi, E, Balducci, C, Cavaletti, G, Liberatore G., Manganelli F., Cocito D., Fazio R., Briani C., Filosto M., Benedetti L., Antonini G., Cosentino G., Jann S., Mazzeo A., Cortese A., Marfia G. A., Clerici A. M., Siciliano G., Carpo M., Sabatelli M., Lauria G., Rosso T., Nobile Orazio E., Doneddu P. E., Gallia F., Nobile-Orazio E., Peci E., Tronci S., Santoro L., Spina E., Ruiz M., Piccinelli S. C., Beronio A., Toscano A., Gentile L., Mataluni G., Piccolo L., Callegari I., Fierro B., Pinuccia V. E., Bianchi E., Beghi E., Scrascia F., Garnero M., Schenone A., Luigetti M., Dacci P., Leonardi L., Schirinzi E., Balducci C., and Cavaletti G.

Abstract

The objective of our work was to report the clinical features and the relevance of diagnostic investigations in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). We retrospectively reviewed data from patients with a clinical diagnosis of CIDP included in a national database. Among the 500 included patients with a clinical diagnosis of CIDP, 437 patients (87%) fulfilled the European Federation of Neurological Societies and Peripheral Nerve Society criteria for CIDP (definite in 407, probable in 26, possible in four). In 352 patients (86%) motor nerve conduction abnormalities consistent with demyelination were sufficient for the diagnosis of definite CIDP. In 55 patients, this diagnosis required the addition of one or two (from probable or from possible CIDP, respectively) supportive tests, while in 20 cases they improved the diagnosis from possible to probable CIDP, seven patients did not change diagnosis. Considering these 85 patients, cerebrospinal fluid studies were performed in 79 cases (93%) upgrading the certainty of diagnosis in 59% of examined patients. Sensory nerve conduction studies (NCS) were performed in 85% of patients with an improvement of diagnosis in 32% of cases. Nerve biopsy and ultrasound and magnetic resonance imaging (US/MRI) exams resulted positive in about 40% of examined patients, but they were performed in few patients (7 patients and 16 patients, respectively). A response to the therapy was present in 84% of treated patients (n = 77), contributing to support the diagnosis in 40 patients in whom the other supportive criteria were not sufficient. In most patients with CIDP the diagnosis is possible solely with motor NCS while other investigations may help improving the diagnosis in a minority of patients.

Published
2020

33. Risk factors for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): antecedent events, lifestyle and dietary habits. Data from the Italian CIDP Database

Author

Doneddu, P, Bianchi, E, Cocito, D, Manganelli, F, Fazio, R, Filosto, M, Mazzeo, A, Cosentino, G, Cortese, A, Jann, S, Clerici, A, Antonini, G, Siciliano, G, Luigetti, M, Marfia, G, Briani, C, Lauria, G, Rosso, T, Cavaletti, G, Carpo, M, Benedetti, L, Beghi, E, Liberatore, G, Santoro, L, Peci, E, Tronci, S, Cotti Piccinelli, S, Toscano, A, Piccolo, L, Verrengia, E, Leonardi, L, Schirinzi, E, Mataluni, G, Ruiz, M, Dacci, P, Nobile-Orazio, E, Doneddu P. E., Bianchi E., Cocito D., Manganelli F., Fazio R., Filosto M., Mazzeo A., Cosentino G., Cortese A., Jann S., Clerici A. M., Antonini G., Siciliano G., Luigetti M., Marfia G. A., Briani C., Lauria G., Rosso T., Cavaletti G., Carpo M., Benedetti L., Beghi E., Liberatore G., Santoro L., Peci E., Tronci S., Cotti Piccinelli S., Toscano A., Piccolo L., Verrengia E. P., Leonardi L., Schirinzi E., Mataluni G., Ruiz M., Dacci P., Nobile-Orazio E., Doneddu, P, Bianchi, E, Cocito, D, Manganelli, F, Fazio, R, Filosto, M, Mazzeo, A, Cosentino, G, Cortese, A, Jann, S, Clerici, A, Antonini, G, Siciliano, G, Luigetti, M, Marfia, G, Briani, C, Lauria, G, Rosso, T, Cavaletti, G, Carpo, M, Benedetti, L, Beghi, E, Liberatore, G, Santoro, L, Peci, E, Tronci, S, Cotti Piccinelli, S, Toscano, A, Piccolo, L, Verrengia, E, Leonardi, L, Schirinzi, E, Mataluni, G, Ruiz, M, Dacci, P, Nobile-Orazio, E, Doneddu P. E., Bianchi E., Cocito D., Manganelli F., Fazio R., Filosto M., Mazzeo A., Cosentino G., Cortese A., Jann S., Clerici A. M., Antonini G., Siciliano G., Luigetti M., Marfia G. A., Briani C., Lauria G., Rosso T., Cavaletti G., Carpo M., Benedetti L., Beghi E., Liberatore G., Santoro L., Peci E., Tronci S., Cotti Piccinelli S., Toscano A., Piccolo L., Verrengia E. P., Leonardi L., Schirinzi E., Mataluni G., Ruiz M., Dacci P., and Nobile-Orazio E.

Abstract

Background and purpose: The role of lifestyle and dietary habits and antecedent events has not been clearly identified in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Methods: Information was collected about modifiable environmental factors and antecedent infections and vaccinations in patients with CIDP included in an Italian CIDP Database. Only patients who reported not having changed their diet or the lifestyle habits investigated in the study after the appearance of CIDP were included. The partners of patients with CIDP were chosen as controls. Gender-matched analysis was performed with randomly selected controls with a 1:1 ratio of patients and controls. Results: Dietary and lifestyle data of 323 patients and 266 controls were available. A total of 195 cases and 195 sex-matched controls were used in the analysis. Patients eating rice at least three times per week or eating fish at least once per week appeared to be at decreased risk of acquiring CIDP. Data on antecedent events were collected in 411 patients. Antecedent events within 1–42 days before CIDP onset were reported by 15.5% of the patients, including infections in 12% and vaccinations in 1.5%. Patients with CIDP and antecedent infections more often had an acute onset of CIDP and cranial nerve involvement than those without these antecedent events. Conclusions: The results of this preliminary study seem to indicate that some dietary habits may influence the risk of CIDP and that antecedent infections may have an impact on the onset and clinical presentation of the disease.

Published
2020

34. Supporting Information Integration With Autonomous Agents

Author

Bergamaschi, S., Cabri, G., Guerra, F., Leonardi, L., Vincini, M., Zambonelli, F., Goos, G., editor, Hartmanis, J., editor, van Leeuwen, J., editor, Carbonell, Jaime G., editor, Siekmann, Jörg, editor, Klusch, Matthias, editor, and Zambonelli, Franco, editor

Published
2001
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35. Relationship between quality of life and behavioural disorders in children with persistent asthma: a Multiple Indicators Multiple Causes (MIMIC) model

Author

Montalbano, L., Ferrante, G., Montella, S., Cilluffo, G., Di Marco, A., Bozzetto, S., Di Palmo, E., Licari, A., Leonardi, L., Caldarelli, V., Ghezzi, M., La Grutta, S., Rusconi, F., Amarri, S., Barni, S., Capizzi, A., Cardinale, F., Carraro, S., Cazzato, S., Cutrera, R., Di Pillo, S., Duse, M., Fenu, G., Kantar, A., Leonardi, S., Lombardi, E., Marseglia, G. L., Nosetti, L., Novembre, E., Patria, M. F., Piacentini, G., Pisi, G., Ricci, G., Sacco, O., Santamaria, F., Tenero, L., Tosca, M. A., Tripodi, M. C., Volpini, A., Montalbano, L., Ferrante, G., Montella, S., Cilluffo, G., Di Marco, A., Bozzetto, S., Di Palmo, E., Licari, A., Leonardi, L., Caldarelli, V., Ghezzi, M., La Grutta, S., Rusconi, F., Amarri, S., Barni, S., Capizzi, A., Cardinale, F., Carraro, S., Cazzato, S., Cutrera, R., Di Pillo, S., Duse, M., Fenu, G., Kantar, A., Leonardi, S., Lombardi, E., Marseglia, G. L., Nosetti, L., Novembre, E., Patria, M. F., Piacentini, G., Pisi, G., Ricci, G., Sacco, O., Santamaria, F., Tenero, L., Tosca, M. A., Tripodi, M. C., Volpini, A., and Montalbano L, Ferrante G, Montella S, Cilluffo G, Di Marco A, Bozzetto S, Di Palmo E, Licari A, Leonardi L, Caldarelli V, Ghezzi M, La Grutta S, Rusconi F

Subjects
Male, Moderate asthma, Asthma severity, lcsh:Medicine, 010501 environmental sciences, Behavioural disorders, Logistic regression, Paediatric research, 01 natural sciences, Article, 03 medical and health sciences, 0302 clinical medicine, Medical research, Quality of life, children, Human behaviour, medicine, Humans, Psychology, Child, lcsh:Science, 0105 earth and related environmental sciences, Asthma, Problem Behavior, Multidisciplinary, business.industry, children asthma, lcsh:R, Case-control study, asthma, medicine.disease, humanities, behavioural disorder, Logistic Models, 030228 respiratory system, behavioural disorders, quality of life, Case-Control Studies, Female, lcsh:Q, Persistent asthma, business, Clinical psychology
Abstract

Knowledge on multiple interdependences between quality of life (QoL) and behavioural problems in relation to asthma severity and control is undetermined. The aims of the study were: (i) to assess the relationship of QoL and behavioural problems with asthma severity and control (ii) to predict children’s “abnormal/borderline” status with variation in QoL. For these purposes a multicenter case-control study on 47 Severe Asthma (SA) and 94 Moderate Asthma (MA) children was performed. The MIMIC approach was applied to investigate the effect of SA and non-controlled asthma (NC) on QoL and behavioural disorders. Logistic regression was used to estimate probabilities of having an “abnormal/borderline” status with variation in QoL. The MIMIC model showed that the magnitude of the effect of SA and NC was larger on QoL (β = −0.37 and β = −0.30, respectively) than on behavioural problems (β = 0.27). With regards to the probability of having a borderline status, in MA a QoL of 1 returned a probability of 0.81, whereas in SA a QoL of 1 returned a probability of 0.89. In conclusion, SA children are highly affected by impaired QoL and behavioural problems. The MIMIC model allowed us to obtain a comprehensive assessment of QoL and behavioural problems with asthma severity and control.

Published
2020

36. Relevance of diagnostic investigations in chronic inflammatory demyelinating poliradiculoneuropathy: Data from the Italian CIDP database

Author

Liberatore, G., Manganelli, F., Cocito, D., Fazio, R., Briani, C., Filosto, M., Benedetti, L., Antonini, G., Cosentino, G., Jann, S., Mazzeo, A., Cortese, A., Marfia, G. A., Clerici, A. M., Siciliano, G., Carpo, M., Sabatelli, M., Lauria, G., Rosso, T., Nobile Orazio, E., Doneddu, P. E., Gallia, F., Nobile-Orazio, E., Peci, E., Tronci, S., Santoro, L., Spina, E., Ruiz, M., Piccinelli, S. C., Beronio, A., Toscano, A., Gentile, L., Mataluni, G., Piccolo, L., Callegari, I., Fierro, B., Pinuccia, V. E., Bianchi, E., Beghi, E., Scrascia, F., Garnero, M., Schenone, A., Luigetti, M., Dacci, P., Leonardi, L., Schirinzi, E., Balducci, C., Cavaletti, G., Liberatore, G, Manganelli, F, Cocito, D, Fazio, R, Briani, C, Filosto, M, Benedetti, L, Antonini, G, Cosentino, G, Jann, S, Mazzeo, A, Cortese, A, Marfia, G, Clerici, A, Siciliano, G, Carpo, M, Sabatelli, M, Lauria, G, Rosso, T, Nobile Orazio, E, Doneddu, P, Gallia, F, Nobile-Orazio, E, Peci, E, Tronci, S, Santoro, L, Spina, E, Ruiz, M, Piccinelli, S, Beronio, A, Toscano, A, Gentile, L, Mataluni, G, Piccolo, L, Callegari, I, Fierro, B, Pinuccia, V, Bianchi, E, Beghi, E, Scrascia, F, Garnero, M, Schenone, A, Luigetti, M, Dacci, P, Leonardi, L, Schirinzi, E, Balducci, C, Cavaletti, G, Liberatore, G., Manganelli, F., Cocito, D., Fazio, R., Briani, C., Filosto, M., Benedetti, L., Antonini, G., Cosentino, G., Jann, S., Mazzeo, A., Cortese, A., Marfia, G. A., Clerici, A. M., Siciliano, G., Carpo, M., Sabatelli, M., Lauria, G., Rosso, T., Nobile Orazio, E., Doneddu, P. E., Gallia, F., Nobile-Orazio, E., Peci, E., Tronci, S., Santoro, L., Spina, E., Ruiz, M., Piccinelli, S. C., Beronio, A., Toscano, A., Gentile, L., Mataluni, G., Piccolo, L., Callegari, I., Fierro, B., Pinuccia, V. E., Bianchi, E., Beghi, E., Scrascia, F., Garnero, M., Schenone, A., Luigetti, M., Dacci, P., Leonardi, L., Schirinzi, E., Balducci, C., and Cavaletti, G.

Subjects
medicine.medical_specialty, peripheral neuropathy, Response to therapy, Motor nerve, CIDP, Settore MED/26, chronic inflammatory demyelinating neuropathy, diagnostic criteria, EMG, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, medicine, In patient, Nerve biopsy, medicine.diagnostic_test, business.industry, General Neuroscience, Polyradiculoneuropathy, medicine.disease, Chronic inflammatory demyelinating neuropathy, Diagnostic criteria, Peripheral neuropathy, medicine.anatomical_structure, 030220 oncology & carcinogenesis, Clinical diagnosis, Neurology (clinical), business, 030217 neurology & neurosurgery, Sensory nerve
Abstract

The objective of our work was to report the clinical features and the relevance of diagnostic investigations in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). We retrospectively reviewed data from patients with a clinical diagnosis of CIDP included in a national database. Among the 500 included patients with a clinical diagnosis of CIDP, 437 patients (87%) fulfilled the European Federation of Neurological Societies and Peripheral Nerve Society criteria for CIDP (definite in 407, probable in 26, possible in four). In 352 patients (86%) motor nerve conduction abnormalities consistent with demyelination were sufficient for the diagnosis of definite CIDP. In 55 patients, this diagnosis required the addition of one or two (from probable or from possible CIDP, respectively) supportive tests, while in 20 cases they improved the diagnosis from possible to probable CIDP, seven patients did not change diagnosis. Considering these 85 patients, cerebrospinal fluid studies were performed in 79 cases (93%) upgrading the certainty of diagnosis in 59% of examined patients. Sensory nerve conduction studies (NCS) were performed in 85% of patients with an improvement of diagnosis in 32% of cases. Nerve biopsy and ultrasound and magnetic resonance imaging (US/MRI) exams resulted positive in about 40% of examined patients, but they were performed in few patients (7 patients and 16 patients, respectively). A response to the therapy was present in 84% of treated patients (n = 77), contributing to support the diagnosis in 40 patients in whom the other supportive criteria were not sufficient. In most patients with CIDP the diagnosis is possible solely with motor NCS while other investigations may help improving the diagnosis in a minority of patients.

Published
2020

37. Muscle magnetic resonance imaging in myotonic dystrophy type 1 (DM1): Refining muscle involvement and implications for clinical trials

Author

Garibaldi, Ida Marina Elisabetta, Nicoletti, Tommaso Filippo, Bucci, E., Fionda, L., Leonardi, L., Morino, S., Tufano, L., Alfieri, G., Lauletta, A., Merlonghi, G., Perna, A., Rossi, S., Ricci, Enzo, Alonso Perez, J., Tartaglione, Tommaso, Petrucci, Andrea, Pennisi, E. M., Salvetti, Maria Cristina, Cutter, G., Diaz-Manera, J., Silvestri, Gabriella, Antonini, Gabriele, Garibaldi M., Nicoletti T., Ricci E. (ORCID:0000-0003-3092-3597), Tartaglione T. (ORCID:0000-0003-3896-4078), Petrucci A., Salvetti M., Silvestri G. (ORCID:0000-0002-1950-1468), Antonini G., Garibaldi, Ida Marina Elisabetta, Nicoletti, Tommaso Filippo, Bucci, E., Fionda, L., Leonardi, L., Morino, S., Tufano, L., Alfieri, G., Lauletta, A., Merlonghi, G., Perna, A., Rossi, S., Ricci, Enzo, Alonso Perez, J., Tartaglione, Tommaso, Petrucci, Andrea, Pennisi, E. M., Salvetti, Maria Cristina, Cutter, G., Diaz-Manera, J., Silvestri, Gabriella, Antonini, Gabriele, Garibaldi M., Nicoletti T., Ricci E. (ORCID:0000-0003-3092-3597), Tartaglione T. (ORCID:0000-0003-3896-4078), Petrucci A., Salvetti M., Silvestri G. (ORCID:0000-0002-1950-1468), and Antonini G.

Abstract

Background: Only a few studies have reported muscle imaging data on small cohorts of patients with myotonic dystrophy type 1 (DM1). We aimed to investigate the muscle involvement in a large cohort of patients in order to refine the pattern of muscle involvement, to better understand the pathophysiological mechanisms of muscle weakness, and to identify potential imaging biomarkers for disease activity and severity. Methods: One hundred and thirty-four DM1 patients underwent a cross-sectional muscle magnetic resonance imaging (MRI) study. Short tau inversion recovery (STIR) and T1 sequences in the lower and upper body were analyzed. Fat replacement, muscle atrophy and STIR positivity were evaluated using three different scales. Correlations between MRI scores, clinical features and genetic background were investigated. Results: The most frequent pattern of muscle involvement in T1 consisted of fat replacement of the tongue, sternocleidomastoideus, paraspinalis, gluteus minimus, distal quadriceps and gastrocnemius medialis. Degree of fat replacement at MRI correlated with clinical severity and disease duration, but not with CTG expansion. Fat replacement was also detected in milder/asymptomatic patients. More than 80% of patients had STIR-positive signals in muscles. Most DM1 patients also showed a variable degree of muscle atrophy regardless of MRI signs of fat replacement. A subset of patients (20%) showed a ‘marbled’ muscle appearance. Conclusions: Muscle MRI is a sensitive biomarker of disease severity alsofor the milder spectrum of disease. STIR hyperintensity seems to precede fat replacement in T1. Beyond fat replacement, STIR positivity, muscle atrophy and a ‘marbled’ appearance suggest further mechanisms of muscle wasting and weakness in DM1, representing additional outcome measures and therapeutic targets for forthcoming clinical trials.

Published
2022

38. Muscle magnetic resonance imaging in myotonic dystrophy type 1 (DM1): Refining muscle involvement and implications for clinical trials

Author

Garibaldi, M., Nicoletti, T., Bucci, E., Fionda, L., Leonardi, L., Morino, S., Tufano, L., Alfieri, G., Lauletta, A., Merlonghi, G., Perna, A., Rossi, S., Ricci, E., Alonso Perez, J., Tartaglione, T., Petrucci, A., Pennisi, E. M., Salvetti, M., Cutter, G., Diaz-Manera, J., Silvestri, G., Antonini, G., Nicoletti T., Perna A., Rossi S., Ricci E. (ORCID:0000-0003-3092-3597), Tartaglione T. (ORCID:0000-0003-3896-4078), Silvestri G. (ORCID:0000-0002-1950-1468), Garibaldi, M., Nicoletti, T., Bucci, E., Fionda, L., Leonardi, L., Morino, S., Tufano, L., Alfieri, G., Lauletta, A., Merlonghi, G., Perna, A., Rossi, S., Ricci, E., Alonso Perez, J., Tartaglione, T., Petrucci, A., Pennisi, E. M., Salvetti, M., Cutter, G., Diaz-Manera, J., Silvestri, G., Antonini, G., Nicoletti T., Perna A., Rossi S., Ricci E. (ORCID:0000-0003-3092-3597), Tartaglione T. (ORCID:0000-0003-3896-4078), and Silvestri G. (ORCID:0000-0002-1950-1468)

Abstract

Background: Only a few studies have reported muscle imaging data on small cohorts of patients with myotonic dystrophy type 1 (DM1). We aimed to investigate the muscle involvement in a large cohort of patients in order to refine the pattern of muscle involvement, to better understand the pathophysiological mechanisms of muscle weakness, and to identify potential imaging biomarkers for disease activity and severity. Methods: One hundred and thirty-four DM1 patients underwent a cross-sectional muscle magnetic resonance imaging (MRI) study. Short tau inversion recovery (STIR) and T1 sequences in the lower and upper body were analyzed. Fat replacement, muscle atrophy and STIR positivity were evaluated using three different scales. Correlations between MRI scores, clinical features and genetic background were investigated. Results: The most frequent pattern of muscle involvement in T1 consisted of fat replacement of the tongue, sternocleidomastoideus, paraspinalis, gluteus minimus, distal quadriceps and gastrocnemius medialis. Degree of fat replacement at MRI correlated with clinical severity and disease duration, but not with CTG expansion. Fat replacement was also detected in milder/asymptomatic patients. More than 80% of patients had STIR-positive signals in muscles. Most DM1 patients also showed a variable degree of muscle atrophy regardless of MRI signs of fat replacement. A subset of patients (20%) showed a ‘marbled’ muscle appearance. Conclusions: Muscle MRI is a sensitive biomarker of disease severity alsofor the milder spectrum of disease. STIR hyperintensity seems to precede fat replacement in T1. Beyond fat replacement, STIR positivity, muscle atrophy and a ‘marbled’ appearance suggest further mechanisms of muscle wasting and weakness in DM1, representing additional outcome measures and therapeutic targets for forthcoming clinical trials.

Published
2022

40. Inter-society consensus for the use of inhaled corticosteroids in infants, children and adolescents with airway diseases (vol 47, pg 1, 2021)

Author

Duse, M, Santamaria, F, Verga, M, Bergamini, M, Simeone, G, Leonardi, L, Tezza, G, Bianchi, A, Capuano, A, Cardinale, F, Cerimoniale, G, Landi, M, Malventano, M, Tosca, M, Varricchio, A, Zicari, A, Alfaro, C, Barberi, S, Becherucci, P, Bernardini, R, Biasci, P, Caffarelli, C, Caldarelli, V, Capristo, C, Castronuovo, S, Chiappini, E, Cutrera, R, De Castro, G, De Franciscis, L, Decimo, F, Iacono, I, Diaferio, L, Di Cicco, M, Di Mauro, C, Di Mauro, D, Di Mauro, F, Di Mauro, G, Doria, M, Falsaperla, R, Ferraro, V, Fanos, V, Galli, E, Ghiglioni, D, Indinnimeo, L, Kantar, A, Lamborghini, A, Licari, A, Lubrano, R, Luciani, S, Macri, F, Marseglia, G, Martelli, A, Masini, L, Midulla, F, Minasi, D, Miniello, V, Del Giudice, M, Morandini, S, Nardini, G, Nocerino, A, Novembre, E, Pajno, G, Paravati, F, Piacentini, G, Piersantelli, C, Pozzobon, G, Ricci, G, Spanevello, V, Turra, R, Zanconato, S, Borrelli, M, Villani, A, Corsello, G, and Peroni, D

Subjects
Settore MED/38
Published
2022

41. Inherited defects in the complement system

Author

Leonardi, L, La Torre, F, Soresina, A, Federici, S, Cancrini, C, Castagnoli, R, Cinicola, B, Corrente, S, Giardino, G, Lougaris, V, Volpi, S, Marseglia, G, Cardinale, F, and Immunology Task Force of the Italian Society of Pediatric Allergy and Immunology (SIAIP)

Subjects
Immunology, pathways, alternative, classical, complement, deficiency, inherited, lectin, system, Complement System Proteins, Settore MED/02, Pediatrics, Perinatology and Child Health, Humans, Immunology and Allergy
Abstract

The complement system plays an essential role in both innate and adaptive immune responses. Any dysregulation in this system can disturb normal host defense and alter inflammatory response leading to both infections and autoimmune diseases. The complement system can be activated through three different pathways. Inherited complement deficiencies have been described for all complement components and their regulators. Despite being rare diseases, complement deficiencies are often severe, with a frequent onset during childhood. We provide an overview of clinical disorders related to these disorders and describe current diagnostic strategies required for their comprehensive characterization and management.

Published
2022

43. Analysis of fatal adverse drug events recorded in several Italian emergency departments (the MEREAFaPS study)

Author

Pagani, S., Lombardi, N., Crescioli, G., Vighi, G. V., Spada, G., Romoli, I., Andreetta, P., Capuano, A., Marrazzo, E., Marra, A., Leoni, O., Vannacci, A., Venegoni, M., Vighi, G. D., Bettoni, D., Blandizzi, C., Bonaiuti, R., Borsi, V., Cecchi, E., Convertino, I., Del Lungo, M., Di Mauro, C., Farina, G., Ferraro, S., Fucile, A., Galfrascoli, E., Geninatti, E., Giovannetti, L., Leonardi, L., Liccardo, R., Monina, G., Mugelli, A., Parrilli, M., Rafaniello, C., Rossi, F., Rostan, S., Ruocco, M., Sironi, M., Sportiello, L., Tuccori, M., Pagani, S., Lombardi, N., Crescioli, G., Vighi, G. V., Spada, G., Romoli, I., Andreetta, P., Capuano, A., Marrazzo, E., Marra, A., Leoni, O., Vannacci, A., Venegoni, M., Vighi, G. D., Bettoni, D., Blandizzi, C., Bonaiuti, R., Borsi, V., Cecchi, E., Convertino, I., Del Lungo, M., Di Mauro, C., Farina, G., Ferraro, S., Fucile, A., Galfrascoli, E., Geninatti, E., Giovannetti, L., Leonardi, L., Liccardo, R., Monina, G., Mugelli, A., Parrilli, M., Rafaniello, C., Rossi, F., Rostan, S., Ruocco, M., Sironi, M., Sportiello, L., and Tuccori, M.

Subjects
Drug, Male, medicine.medical_specialty, Drug-Related Side Effects and Adverse Reactions, media_common.quotation_subject, Adverse drug reaction, 030204 cardiovascular system & hematology, 03 medical and health sciences, Appropriateness of drug use, Pharmacovigilance, 0302 clinical medicine, Older patients, Internal Medicine, Medicine, Humans, 030212 general & internal medicine, Adverse effect, Drug safety, media_common, Aged, Medication error, business.industry, Public health, Anticoagulants, Mean age, medicine.disease, Italy, Concomitant, Emergency medicine, Emergency Medicine, Female, business, Emergency Service, Hospital, Algorithms, Platelet Aggregation Inhibitors
Abstract

Fatal Adverse Events (FADEs) are a major public health problem, and some FADEs could be preventable. The aim of the present study is to describe the frequency, the drugs involved and the preventability in the FADEs collected through the MEREAFaPS Study between 2012 and 2018. All cases including the outcome “death” have been examined. We excluded cases with vaccine-related ADEs, overdose or suicide, and ADEs occurred during the hospitalisation. Two trained assessors evaluated all cases fulfilling the inclusion criteria. ADEs’ preventability was evaluated applying the Schumock and Thornton algorithm. During the study period, we observed 429 cases of death, 92 of which were excluded. The remaining 337 cases involved 187 women and 150 men, with a mean age of 79 and of 77years, respectively. For each report, the suspected drugs and concomitant ones were 1.26 and 4.20, respectively. Anticoagulants and antiplatelet agents account for more than 40% of FADE cases and the most frequent reactions are haemorrhages (37.5%). The 25% of the FADEs were preventable. This study confirms that FADEs are still a relevant clinical occurrence, and are often caused by widely used old drugs associated with adverse events. The death of one in four patients was preventable. Further efforts should be done to improve the appropriateness of the therapy, especially in older patients who are treated with anticoagulants.

Published
2021

44. COVID-19 Vaccination in Pregnancy, Paediatrics, Immunocompromised Patients, and Persons with History of Allergy or Prior SARS-CoV-2 Infection: Overview of Current Recommendations and Pre- and Post-Marketing Evidence for Vaccine Efficacy and Safety

Author

Luxi N., Giovanazzi A., Capuano A., Crisafulli S., Cutroneo P. M., Fantini M. P., Ferrajolo C., Moretti U., Poluzzi E., Raschi E., Ravaldi C., Reno C., Tuccori M., Vannacci A., Zanoni G., Trifiro G., Petrelli G., Girotti S., Arzenton E., Magro L., Lora R., Bellantuono D., Sabaini A., Firenze A., Zodda D., Guidotti F., Zappone M., Alagna B., Spina E., Minore C., Costantino C., Conforto A., Vitale F., Morreale I., Marsala L., Farinella D., Bavetta S., Sapigni E., Potenza A. M., Podetti D., Nikitina V., Ricciardelli R., Mogheiseh N., Croce S., Paltrinieri B., Castellani S., Sangiorgi E., Selleri M., Lucchesi S., Catucci G., Savini D., Sacripanti C., Faccioli M., Romio M. S., Rossi L., Radici S., Negri G., Fares L., Ajolfi C., Fadda A., Chiarello A., Pieraccini F., Pappalardo F., Bonaiuti R., Lombardi N., Crescioli G., Tessari R., Zandona E., Marchiori F., Chiamulera C., Senna G., Crivellaro M. A., Cancian M., Venturini F., Ferri M., Leonardi L., Orzetti S., Caccin E., Baldo P., Rafaniello C., Pagliaro C., Mercaldo M., Fucile A., di Giorgio A., Tari M., Manna S., Farina G., Di Mauro C., De Carlo I., Senesi I., Pileggi C., Palleria C., Gallelli L., De Sarro G., Trabace L., Morgese M., Schiavone S., Tucci P., Bove M., Lapi F., Cricelli C., Racagni G., Tonolo S., Leopardi E., Fava G., Giuffrida S., Amato V., Gambera M., Montresor V., Luxi N., Giovanazzi A., Capuano A., Crisafulli S., Cutroneo P.M., Fantini M.P., Ferrajolo C., Moretti U., Poluzzi E., Raschi E., Ravaldi C., Reno C., Tuccori M., Vannacci A., Zanoni G., Trifiro G., Luxi, N., Giovanazzi, A., Capuano, A., Crisafulli, S., Cutroneo, P. M., Fantini, M. P., Ferrajolo, C., Moretti, U., Poluzzi, E., Raschi, E., Ravaldi, C., Reno, C., Tuccori, M., Vannacci, A., Zanoni, G., Trifiro, G., Petrelli G., Girotti S., Arzenton E., Magro L., Lora R., Bellantuono D., Sabaini A., Firenze A., Zodda D., Guidotti F., Zappone M., Alagna B., Spina E., Minore C., Costantino C., Conforto A., Vitale F., Morreale I., Marsala L., Farinella D., Bavetta S., Sapigni E., Potenza A.M., Podetti D., Nikitina V., Ricciardelli R., Mogheiseh N., Croce S., Paltrinieri B., Castellani S., Sangiorgi E., Selleri M., Lucchesi S., Catucci G., Savini D., Sacripanti C., Faccioli M., Romio M.S., Rossi L., Radici S., Negri G., Fares L., Ajolfi C., Fadda A., Chiarello A., Pieraccini F., Pappalardo F., Bonaiuti R., Lombardi N., Crescioli G., Tessari R., Zandona E., Marchiori F., Chiamulera C., Senna G., Crivellaro M.A., Cancian M., Venturini F., Ferri M., Leonardi L., Orzetti S., Caccin E., Baldo P., Rafaniello C., Pagliaro C., Mercaldo M., Fucile A., di Giorgio A., Tari M., Manna S., Farina G., Di Mauro C., De Carlo I., Senesi I., Pileggi C., Palleria C., Gallelli L., De Sarro G., Trabace L., Morgese M., Schiavone S., Tucci P., Bove M., Lapi F., Cricelli C., Racagni G., Tonolo S., Leopardi E., Fava G., Giuffrida S., Amato V., Gambera M., and Montresor V.

Subjects
Allergy, IMPACT, COVID-19 Vaccine, Breastfeeding, Review Article, Toxicology, Settore MED/42 - Igiene Generale E Applicata, CLINICAL CHARACTERISTICS, Pregnancy, Pharmacology (medical), Pregnancy Complications, Infectious, Child, OUTCOMES, education.field_of_study, CANCER, Vaccination, Europe, CORONAVIRUS DISEASE 2019, CLINICAL CHARACTERISTICS, CANCER, RECIPIENTS, SEVERITY, OUTCOMES, IMPACT, RATES, Breast Feeding, Child, Preschool, Practice Guidelines as Topic, Female, 2019-nCoV Vaccine mRNA-1273, Human, Adult, medicine.medical_specialty, COVID-19 Vaccines, Adolescent, BNT162 Vaccine, COVID-19, ChAdOx1 nCoV-19, Humans, Infant, SARS-CoV-2, Hypersensitivity, Immunocompromised Host, Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), Population, MEDLINE, CORONAVIRUS DISEASE 2019, medicine, RATES, education, Pharmacology, business.industry, medicine.disease, Vaccine efficacy, RECIPIENTS, SEVERITY, Family medicine, Pregnancy Complications, Infectiou, business
Abstract

To date, four vaccines have been authorised for emergency use and under conditional approval by the European Medicines Agency to prevent COVID-19: Comirnaty, COVID-19 Vaccine Janssen, Spikevax (previously COVID-19 Vaccine Moderna) and Vaxzevria (previously COVID-19 Vaccine AstraZeneca). Although the benefit–risk profile of these vaccines was proven to be largely favourable in the general population, evidence in special cohorts initially excluded from the pivotal trials, such as pregnant and breastfeeding women, children/adolescents, immunocompromised people and persons with a history of allergy or previous SARS-CoV-2 infection, is still limited. In this narrative review, we critically overview pre- and post-marketing evidence on the potential benefits and risks of marketed COVID-19 vaccines in the above-mentioned special cohorts. In addition, we summarise the recommendations of the scientific societies and regulatory agencies about COVID-19 primary prevention in the same vaccinee categories. Supplementary Information The online version contains supplementary material available at 10.1007/s40264-021-01131-6.

Published
2021

45. The Future of Factories: Different Trends

Author

Salierno, G., Leonardi, L., and Cabri, G.

Subjects
Technology, Cloud manufacturing, QH301-705.5, Physics, QC1-999, virtual factory, Industry 4.0, Engineering (General). Civil engineering (General), digital factory, Digital factory, Chemistry, Virtual factory, China 2025, TA1-2040, Biology (General), QD1-999, cloud manufacturing
Abstract

The technological advancements promote the rise of the fourth industrial revolution, where key terms are efficiency, innovation, and enterprises’ digitalization. Market globalization, product mass customization, and more complex products need to reflect on changing the actual design methods and developing business processes and methodologies that have to be data-driven, AI-assisted, smart, and service-oriented. Therefore, there is a great interest in experimenting with emerging technologies and evaluating how they impact the actual business processes. This paper reports a comparison among the major trends in the digitalization of a Factory of the Future, in conjunction with the two major strategic programs of Industry 4.0 and China 2025. We have focused on these two programs because we have had experience with them in the context of the FIRST H2020 project. European industrialists identify the radical change in the traditional manufacturing production process as the rise of Industry 4.0. Conversely, China mainland launched its strategic plan in China 2025 to promote smart manufacturing to digitalize traditional manufacturing processes. The main contribution of this review paper is to report about a study, conducted and part of the aforementioned FIRST project, which aimed to investigate major trends in applying for both programs in terms of technologies and their applications for the factory’s digitalization. In particular, our analysis consists of the comparison between Digital Factory, Virtual Factory, Smart Manufacturing, and Cloud Manufacturing. We analyzed their essential characteristics, the operational boundaries, the employed technologies, and the interoperability offered at each factory level for each paradigm. Based on this analysis, we report the building blocks in terms of essential technologies required to develop the next generation of a factory of the future, as well as some of the interoperability challenges at a different scale, for enabling inter-factories communications between heterogeneous entities.

Published
2021

46. Immunological basis of virus-host interaction in COVID-19

Author

La Torre, F, Leonardi, L, Giardino, G, Volpi, S, Federici, S, Soresina, A, Cancrini, C, Lougaris, V, Castagnoli, R, Corrente, S, Cardinale, F, Immunology Commission of the Italian Society of Pediatric Allergy, I, La Torre, F., Leonardi, L., Giardino, G., Volpi, S., Federici, S., Soresina, A., Cancrini, C., Lougaris, V., Castagnoli, R., Corrente, S., and Cardinale, F.

Subjects
Coronavirus disease 2019 (COVID-19), Immunology, Inflammation, Virus-host interaction, Covid‐19 in Children and Adolescents, 03 medical and health sciences, 2020 Update from The Italian Society of Pediatric Allergy and Immunology, 0302 clinical medicine, Immune system, Host Microbial Interaction, children, COVID‐19, Medicine, Immunology and Allergy, Humans, 030212 general & internal medicine, Pediatrics, Perinatology, and Child Health, Host Microbial Interactions, Kawasaki disease, business.industry, SARS-CoV-2, COVID-19, cytokine storm, immunopathogenesis, Cytokine Release Syndrome, Pneumonia, medicine.disease, Cytokine release syndrome, Settore MED/02, 030228 respiratory system, immunopathogenesi, Pediatrics, Perinatology and Child Health, Etiology, Supplement Article, Viral disease, medicine.symptom, business, Cytokine storm, Human
Abstract

COVID‐19 is a complex new viral disease, in which a strict balance between anti‐viral immune response and the ensuing organ inflammation has a critical role in determining the clinical course. In adults, compelling evidence exists indicating that an uncontrolled inflammatory response ("cytokine storm") is pivotal in determining disease progression and mortality. Children may rarely present with severe disease. Modulating factors related to the host's genetic factors, age‐related susceptibility, and the capability to mount appropriate immune responses might play a role in control virus load at an early stage and regulating the inflammatory reaction. Elucidating these mechanisms seems crucial in developing target therapies according to patient's age, immunologic status, and disease evolution in COVID‐19.

Published
2020

49. Primary atopic disorders and chronic skin disease

Author

Cinicola, B, Corrente, S, Castagnoli, R, Lougaris, V, Giardino, G, Leonardi, L, Volpi, S, La Torre, F, Federici, S, Soresina, A, Cancrini, C, Marseglia, G, Cardinale, F, and Immunology Task Force of the Italian Society of Pediatric Allergy, I

Subjects
IgE, allergy, inborn errors of immunity, primary atopic disorders, skin, Hypersensitivity, Immediate, Immediate, Urticaria, Immunology, Eczema, Skin Diseases, Settore MED/02, Humans, Job Syndrome, Pediatrics, Perinatology and Child Health, Hypersensitivity, Immunology and Allergy
Abstract

Primary atopic disorders (PADs) are monogenic diseases characterized by allergy or atopy-related symptoms as fundamental features. In patients with PADs, primary immune deficiency and immune dysregulation symptoms are usually coexist. Chronic skin disease, manifesting with erythroderma, severe atopic dermatitis or eczema, and urticaria, is one of the main features observed in PADs, such as hyper-IgE syndromes, Omenn syndrome, Wiskott-Aldrich syndrome, IPEX-linked syndrome, skin barrier disorders, as well as some autoinflammatory diseases. The recognition of PADs in the context of an allergic phenotype is crucial to ensure prompt diagnosis and appropriate treatment. This article provides an overview of the main PADs with skin involvement.

Published
2021

50. Serum levels of polychlorinated biphenyls and risk of non-Hodgkin lymphoma: A hospital-based case-control study

Author

Magoni, M., Donato, F., Apostoli, P., Rossi, G., Comba, P., Fazzo, L., Speziani, F., Leonardi, L., Orizio, G., Scarcella, C., Anastasia, A., Tucci, A., Terraroli, C., Coniglio, A., Zarattini, G., Manca, G., Fostinelli, J., Festa, R., Conti, M., and Micheletti, S.

Subjects
Male, Lymphoma, Health, Toxicology and Mutagenesis, 0208 environmental biotechnology, Physiology, 02 engineering and technology, 010501 environmental sciences, 01 natural sciences, Risk Factors, immune system diseases, hemic and lymphatic diseases, 80 and over, Medicine, Aged, 80 and over, Air Pollutants, education.field_of_study, Non-hodgkin lymphoma, Lymphoma, Non-Hodgkin, Incidence, General Medicine, Hospital based, Middle Aged, Control subjects, Polychlorinated Biphenyls, Pollution, Occupational, Hospitals, Italy, Quartile, Female, Adult, Environmental Engineering, Population, Non-Hodgkin, Air Pollutants, Occupational, Young Adult, Occupational Exposure, Humans, Environmental Chemistry, Environmental risk factors, education, Aged, 0105 earth and related environmental sciences, business.industry, Public Health, Environmental and Occupational Health, Case-control study, Cancer, General Chemistry, medicine.disease, 020801 environmental engineering, Polychlorinated biphenyls, Case-Control Studies, Hodgkin lymphoma, business
Abstract

Polychlorinated biphenyls (PCB) have been classified by the International Agency for Research on Cancer (IARC) in Group 1 as carcinogenic to human, based on sufficient evidence in humans of an increased risk of cutaneous malignant melanoma and limited evidence for non-Hodgkin lymphoma (NHL). However present findings on the association of PCB exposure and NHL are still controversial. This study examined the relationship between PCB serum levels and risk of NHL in a Northern Italy area (Brescia province), where a chemical factory produced PCBs from 1938 to 1984, causing human contamination. A case–control study of 215 NHL patients and 215 control subjects was conducted. Cases and controls were assayed for serum levels of 33 PCB congeners. No associations were found between risk of NHL and serum levels of total PCBs (OR = 0.51; 95% CI: 0.25–1.04 for highest vs lowest quartile) or specific PCB congeners. The study confirmed a strong association of NHL with HCV infection (OR = 3.60; 95% CI: 1.30–10.02). This case-control study does not support the hypothesis of an association between current serum levels of PCBs and NHL development in the general population.

Published
2019

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