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81 results on '"Raffaele Iorio"'

1. Safety and efficacy of zilucoplan in patients with generalised myasthenia gravis (RAISE): a randomised, double-blind, placebo-controlled, phase 3 study

Author

James F Howard, Saskia Bresch, Angela Genge, Channa Hewamadduma, John Hinton, Yessar Hussain, Raul Juntas-Morales, Henry J Kaminski, Angelina Maniaol, Renato Mantegazza, Masayuki Masuda, Kumaraswamy Sivakumar, Marek Śmiłowski, Kimiaki Utsugisawa, Tuan Vu, Michael D Weiss, Małgorzata Zajda, Babak Boroojerdi, Melissa Brock, Guillemette de la Borderie, Petra W Duda, Romana Lowcock, Mark Vanderkelen, M Isabel Leite, Dylan Sembinelli, Jeanne Teitelbaum, Michael Nicolle, Emilien Bernard, Juliette Svahn, Marco Spinazzi, Tanya Stojkovic, Sophie Demeret, Nicolas Weiss, Loïc Le Guennec, Sihame Messai, Christine Tranchant, Aleksandra Nadaj-Pakleza, Jean-Baptiste Chanson, Muhtadi Suliman, Leila Zaidi, Celine Tard, Peggy Lecointe, Jana Zschüntzsch, Jens Schmidt, Stefanie Glaubitz, Rachel Zeng, Matthias Scholl, Markus Kowarik, Ulf Ziemann, Markus Krumbholz, Pascal Martin, Christoph Ruschil, Jutta Dünschede, Roswitha Kemmner, Natalie Rumpel, Benjamin Berger, Andreas Totzeck, Tim Hagenacker, Benjamin Stolte, Raffaele Iorio, Amelia Evoli, Silvia Falso, Carlo Antozzi, Rita Frangiamore, Fiammetta Vanoli, Elena Rinaldi, Kazushi Deguchi, Naoya Minami, Yuriko Nagane, Yasushi Suzuki, Sayaka Ishida, Shigeaki Suzuki, Jin Nakahara, Astushi Nagaoka, Shunsuke Yoshimura, Shingo Konno, Youko Tsuya, Akiyuki Uzawa, Tomoya Kubota, Masanori Takahashi, Tatsusada Okuno, Hiroyuki Murai, Nils Erik Gilhus, Marion Boldingh, Tone Hakvåg Rønning, Urszula Chyrchel-Paszkiewicz, Klaudiusz Kumor, Tomasz Zielinski, Krzysztof Banaszkiewicz, Michał Błaż, Agata Kłósek, Mariola Świderek-Matysiak, Andrzej Szczudlik, Aneta Paśko, Lech Szczechowski, Marta Banach, Jan Ilkowski, Solange Kapetanovic Garcia, Patricia Ortiz Bagan, Ana Belén Cánovas Segura, Joana Turon Sans, Nuria Vidal Fernandez, Elena Cortes Vicente, Patricia Rodrigo Armenteros, Mohammad Ashraghi, Ana Cavey, Liam Haslam, Anna Emery, Kore Liow, Sharon Yegiaian, Alexandru Barboi, Rosa Maria Vazquez, Joshua Lennon, Robert M Pascuzzi, Cynthia Bodkin, Sandra Guingrich, Adam Comer, Mark Bromberg, Teresa Janecki, Sami Saba, Marco Tellez, Bakri Elsheikh, Miriam Freimer, Sarah Heintzman, Raghav Govindarajan, Jeffrey Guptill, Janice M Massey, Vern Juel, Natalia Gonzalez, Ali A Habib, Tahseen Mozaffar, Manisha Korb, Namita Goyal, Hannah Machemehl, Georgios Manousakis, Jeffrey Allen, Emily Harper, Constantine Farmakidis, Lilli Saavedra, Mazen Dimachkie, Mamatha Pasnoor, Salma Akhter, Said Beydoun, Courtney McIlduff, Joan Nye, Bhaskar Roy, Bailey Munro Sheldon, Richard Nowak, Benjamin Barnes, Michael Rivner, Niraja Suresh, Jessica Shaw, Brittany Harvey, Lucy Lam, Nikki Thomas, Manisha Chopra, Rebecca E Traub, Sarah Jones, Mary Wagoner, Sejla Smajic, Radwa Aly, Jonathan Katz, Henry Chen, Robert G Miller, Liberty Jenkins, Shaida Khan, Bhupendra Khatri, Lisa Sershon, Pantelis Pavlakis, Shara Holzberg, Yuebing Li, Irys B Caristo, Robert Marquardt, Debbie Hastings, Jacob Rube, Robert P Lisak, Aparna Choudhury, Katherine Ruzhansky, Amit Sachdev, Susan Shin, Joan Bratton, Mary Fetter, Naya McKinnon, Jonathan McKinnon, Laura Sissons-Ross, Amos Sahu, and B Jane Distad

Subjects
Neurology (clinical)
Published
2023
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2. Safety and efficacy of rozanolixizumab in patients with generalised myasthenia gravis (MycarinG): a randomised, double-blind, placebo-controlled, adaptive phase 3 study

Author

Vera Bril, Artur Drużdż, Julian Grosskreutz, Ali A Habib, Renato Mantegazza, Sabrina Sacconi, Kimiaki Utsugisawa, John Vissing, Tuan Vu, Marion Boehnlein, Ali Bozorg, Maryam Gayfieva, Bernhard Greve, Franz Woltering, Henry J Kaminski, Angela Genge, Rami Massie, Maxime Berube, Lubna Daniyal, Shabber Mannan, Eduardo Ng, Ritesh Rohan Raghu Raman, Evelyn Sarpong, Monica Alcantara, Annie Dionne, Zaeem Siddiqi, Derrick Blackmore, Faraz Hussain, Genevieve Matte, Stephan Botez, Michaela Tyblova, Michala Jakubikova, Jana Junkerova, Nanna Witting, Sonja Holm-Yildiz, Mads Stemmerik, Henning Andersen, Izabella Obál, Guilhem Solé, Stéphane Mathis, Marie-Hélène Violleau, Christine Tranchant, Sihame Messai, Jean-Baptiste Chanson, Aleksandra Nadaj-Pakleza, Arnaud Verloes, Leila Zaidi, Manuela Gambella, Michele Cavalli, Tanya Stojkovic, Sophie Demeret, Loic Le Guennec, Giorgia Querin, Nicolas Weiss, Marion Masingue, Laurent Magy, Karima Ghorab, Ia Rukhadze, Alexander Tsiskaridze, Marina Janelidze, Temur Margania, Florian Then Bergh, Eike Hänsel, Andrea Kalb, Bianca Meilick, Mandy Reuschel, Lars-Malte Teußer, Astrid Unterlauft, Clemens Goedel, Tim Hagenacker, Andreas Totzeck, Benjamin Stolte, Franz Blaes, Christine Bindler, Vasilios Tsoutsikas, Annekathrin Roediger, Christian Geis, Jens Schmidt, Jana Zschüntzsch, Margret Schwarz, Stefanie Meyer, Karsten Kummer, Stefanie Glaubitz, Rachel Zeng, Heinz Wiendl, Luisa Klotz, Anna Lammerskitten, Jan Lünemann, Péter Diószeghy, Lorenzo Maggi, Elena Rinaldi, Matteo Gastaldi, Federico Mazzacane, Pietro Businaro, Raffaele Iorio, Giovanni Antonini, Laura Fionda, Rita Rinaldi, Simone Rossi, Francesco Habetswallner, Francesco Tuccillo, Haruna Umehara, Eiko Uenaka, Masanori Takahashi, Keiko Higashi, Makoto Kinoshita, Emika Yoneda, Noriko Nakamura, Saeka Fujita, Tomoya Kubota, Masami Ono, Sana Yamamoto, Taku Hatano, Kazuki Oikoshi, Kazumasa Yokoyama, Yutaka Oji, Yuji Tomizawa, Akiyuki Uzawa, Manato Yasuda, Sachiko Akita, Yukiko Ozawa, Yosuke Onishi, Miki Takaki, Hiromi Yamada, Kanako Minemoto, Miki Sanko, Nanae Izawa, Mayumi Nakayama, Masayuki Masuda, Rune Tsuji, Nobuhiro Ido, Yumi Hyodo, Yoshihiko Okubo, Akiko Minohara, Nana Haraguchi, Makiko Naito, Seiko Yoshida, Yuri Fukushige, Akira Tsujino, Atsushi Nagaoka, Teiichiro Miyazaki, Shunsuke Yoshimura, Takuro Hirayama, Tomoaki Shima, Naoko Okamoto, Riki Matsumoto, Kenji Sekiguchi, Takehiro Ueda, Norio Chihara, Mari Kirimura, Emi Sunagawa, Ayaka Suzuki, Shigeaki Suzuki, Aozora Wada, Kei Ishizuchi, Yasushi Suzuki, Mitsuo Yata, Yuka Komatsu, Kenichi Tsukita, Genya Watanabe, Kazuki Sato, Emiko Kawasaki, Naoki Yamamoto, Hirohiko Ono, Tomoko Tsuda, Shigeki Ohashi, Yuka Fujisawa, Yumiko Yokota, Yuriko Nagane, Kameda Ayumi, Yuka Takematsu, Hiroyuki Naito, Kumiko Kuwada, Konrad Rejdak, Sebastian Szklener, Monika Kitowska, Kandyda Derkacz, Tomasz Berkowicz, Paulina Budzinska, Marek Halas, Leonid Zaslavskiy, Evgeniya Skornyakova, Sergey Kotov, Ekaterina Novikova, Olga Sidorova, Vitalii Goldobin, Tatiana Alekseeva, Patimat Isabekova, Nadezhda Malkova, Denis Korobko, Gordana Djordjevic, Aleksandar Stojanov, Stojan Peric, Dragana Lavrnic, Ivo Bozovic, Aleksa Palibrk, Carlos Casasnovas, Velina Nedkova-Hristova, Nuria Vidal Fernández, Elena Cortés Vicente, Luis Querol Gutiérrez, Maria Salvadó Figueras, Anna Canovas Segura, Raúl Juntas Morales, Daniel Sanchez Tejerina, Albert Saiz, Yolanda Blanco Morgado, Sara Llufriú Durán, María Sepúlveda Gázquez, Eugenia María Martínez Hernández, Gerardo Gutiérrez Gutiérrez, Paqui Iniesta, José Meca Lallana, Yuh-Cherng Guo, Hou-Chang Chiu, Jiann-Horng Yeh, Ya Hui Chen, Mei Fen Lee, Yi-Chung Lee, Kuan Lin Lai, Said Beydoun, Salma Akhter, Lucy Lam, Alisha Thomas, Michael Rivner, Brandy Quarles, Dale Lange, Shara Holzberg, Pantelis Pavlakis, Ashwathy Goutham, Henry Kaminski, Radwa Aly, Lisa Ashworth, Kathryn Bender, Karie Bond, Joanne Buckner, Sara Byerly, James Caress, Jessyca Clemons, Asha Farmer, Catherine Franklin, Summer Harris, Meredith Hiatt, Rachana Gandhi Mehta, Gina Miller, Lynn Smith, Rose Smith, Brian Strittmatter, Tahseen Mozaffar, Isela Hernandez, Kelsey Moulton, Chafic Karam, Pranali Ravikumar, Catherine Lomen-Hoerth, Laura Rosow, Hannah George, Viktoriya Irodenko, Carol Denny, Bart Hanson, Sara Klein, Jennifer Martinez-Thompson, Elie Naddaf, Denny Padgett, Eric Sorenson, Jane L Sultze, Delena Weis, Kourosh Rezania, Jason Thonhoff, Sheetal Shroff, Robert Pascuzzi, Angela Micheels, Cynthia Bodkin, Adam Comer, Gelasio Baras, Renee Wagner, Zabeen Mahuwala, Stephen Ryan, Kai Su, Khema Sharma, Andrew Brown, and Kore Liow

Subjects
Neurology (clinical)
Published
2023
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3. Diagnostic approach to neonatal and infantile cholestasis: A position paper by the SIGENP liver disease working group

Author

Maurizio Fuoti, Mara Cananzi, Giulia Paolella, Manila Candusso, Paola Francalanci, Lidia Monti, Emanuele Nicastro, Lorenzo D'Antiga, Carlo Dionisi Vici, Michele Pinon, Lorenza Matarazzo, Irene Degrassi, P. Gaio, Angelo Di Giorgio, Giusy Ranucci, Pier Luigi Calvo, Giuseppe Indolfi, Claudia Mandato, Fabio Mosca, Pietro Vajro, Maria Pia Bondioni, Maria Iascone, Maria Grazia Clemente, Federica Nuti, Marco Sciveres, Jean de Ville de Goyet, Claudia Della Corte, Marco Spada, Chiara Grimaldi, Federica Ferrari, Gabriella Nebbia, Giuseppe Maggiore, Fabio Fusaro, Daniele Serranti, Daniele Alberti, Fabiola Di Dato, Paola Roggero, Raffaele Iorio, and Giovanni Boroni

Subjects
Male, medicine.medical_specialty, Genetic liver disease, Alagille syndrome, Biliary atresia, Diagnosis, Inborn errors of metabolism, Jaundice, Monogenic liver disease, Newborn, Female, Gastroenterology, Humans, Infant, Infant, Newborn, Cholestasis, Evidence-Based Medicine, Infant, Newborn, Diseases, Practice Guidelines as Topic, Diseases, Disease, Liver disease, Epidemiology, medicine, Intensive care medicine, Hepatology, business.industry, medicine.disease, Etiology, Position paper, medicine.symptom, business
Abstract

Neonatal and infantile cholestasis (NIC) can represent the onset of a surgically correctable disease and of a genetic or metabolic disorder worthy of medical treatment. Timely recognition of NIC and identification of the underlying etiology are paramount to improve outcomes. Upon invitation by the Italian National Institute of Health (ISS), an expert working grouped was formed to formulate evidence-based positions on current knowledge about the diagnosis of NIC. A systematic literature search was conducted to collect evidence about epidemiology, etiology, clinical aspects and accuracy of available diagnostic tests in NIC. Evidence was scored using the GRADE system. All recommendations were approved by a panel of experts upon agreement of at least 75% of the members. The final document was approved by all the panel components. This position document summarizes the collected statements and defines the best-evidence diagnostic approach to cholestasis in the first year of life.

Published
2022
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5. Unilateral and Multiple Cavitation Sounds During Lumbosacral Spinal Manipulation

Author

Raffaele Iorio, James Dunning, Firas Mourad, César Fernández-de-las-Peñas, Noah Zacharko, Raymond Butts, and Andrea Zingoni

Subjects
Male, Manipulation, Spinal, 030222 orthopedics, medicine.medical_specialty, Facet (geometry), Sound Spectrography, Single model, business.industry, Lumbosacral Region, Thrust, Spinal manipulation, Lumbosacral region, 03 medical and health sciences, Sound, 0302 clinical medicine, Physical medicine and rehabilitation, Lumbar, medicine, Humans, Female, Chiropractics, Manual therapy, business, 030217 neurology & neurosurgery, Lumbosacral joint
Abstract

Objective The purpose of this study was to determine from which side of the spine the popping sound (PS) emanates during side-lying, rotatory high-velocity low-amplitude (HVLA) thrust manipulation directed to the L5-S1 articulation using a time-frequency analysis. Secondary aims were to calculate the average number of PSs, the duration of lumbar thrust manipulation, and the duration of a single PS. Methods Thirty-four asymptomatic participants received 2 lumbar HVLA thrust manipulations targeting the right and left L5-S1 articulations. Two high sampling rate accelerometers were secured bilaterally 25 mm lateral to the midline of the L5-S1 interspace. For each manipulation, 2 audio signals were extracted and singularly processed via spectrogram calculation to obtain the release of energy over time on each side of the lumbosacral junction. Results During 60 HVLA thrust manipulations, it was measured a total of 320 PSs. Of those PSs, 176 occurred ipsilateral and 144 occurred contralateral to the targeted L5-S1 articulation; that is, the PS was no more likely to occur on the upside than the downside facet after right or left rotatory L5-S1 HVLA thrust manipulation. Moreover, PSs occurring on both sides at the same time were detected very rarely (ie, 2% of cases) with the lumbar HVLA thrust manipulations. The mean number of audible PSs per lumbosacral HVLA thrust manipulation was 5.27 (range 2-9). The mean duration of a single manipulation was 139.13 milliseconds (95% confidence interval: 5.61-493.79), and the mean duration of a single PS was 2.69 milliseconds (95% confidence interval: 0.95-4.59). Conclusion Based on our findings, spinal manipulative therapy practitioners should expect multiple PSs that most often occur on the upside or the downside facet articulations when performing HVLA thrust manipulation to the lumbosacral junction (ie, L5-S1). However, whether the multiple PSs found in this study emanated from the same joint or adjacent ipsilateral or contralateral facet joints remains unknown. A single model may not necessarily be able to explain all of the audible sounds during HVLA thrust manipulation.

Published
2019
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6. Safety profile of SARS-CoV-2 vaccination in patients with antibody-mediated CNS disorders

Author

Alessandro Dinoto, Matteo Gastaldi, Raffaele Iorio, Sofia Marini, Valentina Damato, Antonio Farina, Marco Zoccarato, Elia Sechi, Francesca Pinna, Giorgia Teresa Maniscalco, Ruggero Barnabei, Luigi Zuliani, Sergio Ferrari, and Sara Mariotto

Subjects
COVID-19 Vaccines, SARS-CoV-2, Vaccination, COVID-19, Viral Vaccines, General Medicine, Antibodies, Viral, Neurology, Humans, Neurology (clinical), Neoplasm Recurrence, Local, Autoimmune encephalitis, Safety, CNS autoantibodies, BNT162 Vaccine, Autoantibodies
Abstract

In this retrospective multicenter study, we evaluated the safety of SARS-CoV-2 vaccination in patients harboring autoantibodies targeting neuronal surface and/or synaptic antigens.From eight Italian Neurology Units, we included patients with: a) serum and/or CSF positivity for specific neuronal autoantibodies; b) a compatible neurological syndrome; and c) available follow-up ≥6 weeks after vaccination with any of the approved SARS-CoV-2 vaccines. Demographics, clinical data, and information regarding previous SARS-CoV-2 infection and vaccination were collected. Disease relapses were considered "post-infectious" or "post-vaccination" when occurring within 6 weeks from infection/vaccination.We included 66 patients; 7/66 (11%) had a previous history of SARS-CoV-2 infection and 1/7 (14%) had post-infection relapses. BNT162b2-Pfizer-BioNTec was administered in 55 cases (83.3%) and mRNA-1273-Moderna in 11 (16.7%). The median number of doses administered per patient was 2 (1-3) and50% of patients did not experience side effects. Five patients (8%) had post-vaccination relapses (seizure 3/5); 4/5 improved after immunotherapy, while one did not receive immunotherapy and worsened. Patients with post-vaccination relapses had higher disability scores at vaccination (p = 0.025), a trend favoring Leucine-rich glioma-inactivated protein 1 LGI1 glutamic acid decarboxylase 65 (GAD65) antibodies (p = 0.054) and shorter time from last relapse (p = 0.057).Our data support the safety of SARS-CoV-2 vaccines in patients with neurological disorders associated with antibodies to neuronal and synaptic antigens.

Published
2022
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7. Paraneoplastic Neuromyelitis Optica Spectrum Disorder: A single center cohort description with two cases of histological validation

Author

Philippe Beauchemin, Raffaele Iorio, Anthony Traboulsee, Robert Carruthers, Anna V. Tinker, and Thalia S. Field

Subjects
Adult, Male, Pathology, medicine.medical_specialty, Thymoma, Single Center, Cohort Studies, 03 medical and health sciences, 0302 clinical medicine, Ovarian carcinoma, medicine, Humans, Adrenocortical carcinoma, Autoantibodies, Aquaporin 4, Neuromyelitis optica, business.industry, Multiple sclerosis, Neuromyelitis Optica, Cancer, General Medicine, Middle Aged, medicine.disease, Serous fluid, Neurology, 030220 oncology & carcinogenesis, Female, Neurology (clinical), business, 030217 neurology & neurosurgery, Brain Stem, Paraneoplastic Syndromes, Nervous System
Abstract

Background Paraneoplastic syndromes are remote effects of cancer caused by an autoimmune response triggered by tumor cells. Paraneoplastic Neuromyelitis Optica Spectrum Disorders (NMOSD) has been previously described, but the underlying mechanism for these rare cases is not well characterized. This paper presents a newly described case series of paraneoplastic NMOSD, including 2 new histological types of cancer and histological validation. Methods The UBC NMO clinic database was surveyed and identified 6 patients with paraneoplastic NMOSD. In 2 cases, aquaporin-4 (AQP4) immunoreactivity was assessed on patients' tumor specimens. Results The mean age at NMOSD diagnosis was 56 years old and 5/6 patients were older than 50 years old. 4/6 patients with paraneoplastic NMOSD have positive AQP4 antibodies. The median time between NMOSD and cancer was 12 months. Two new cancer types- serous ovarian carcinoma and adrenocortical carcinoma - were found in paraneoplastic NMOSD cases. A serous ovarian carcinoma and a thymoma, found in patients with AQP4 serological evidence, showed a positive reactivity to AQP4 immunostaining. Conclusions Our findings will increase the recognition of NMOSD as a paraneoplastic syndrome. Cancer cells can express AQP4, increasing the likelihood of a direct mechanism between cancer cells and the development of NMOSD in paraneoplastic cases.

Published
2018
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8. Febrile infection-related epilepsy syndrome (FIRES) with super-refractory status epilepticus revealing autoimmune encephalitis due to GABA A R antibodies

Author

Lucia Fusco, Raffaele Iorio, Federico Vigevano, and D. Caputo

Subjects
0301 basic medicine, Drug Resistant Epilepsy, Adolescent, Hashimoto Disease, Status epilepticus, 03 medical and health sciences, Status Epilepticus, 0302 clinical medicine, Seizures, medicine, Humans, Autoantibodies, Autoimmune disease, Autoimmune encephalitis, biology, business.industry, General Medicine, Receptors, GABA-A, medicine.disease, Febrile infection related epilepsy syndrome, 030104 developmental biology, Pediatrics, Perinatology and Child Health, Immunology, Epilepsy syndromes, Etiology, biology.protein, Encephalitis, Anticonvulsants, Female, Ketamine, Steroids, Neurology (clinical), Antibody, medicine.symptom, business, Epileptic Syndromes, 030217 neurology & neurosurgery
Abstract

Background Febrile infection-related epilepsy syndrome (FIRES) has been described as an epileptic encephalopathy of unknown etiology affecting previously healthy children following febrile illness. Despite large investigations on autoimmune pathogenesis no membrane antibodies has been associated since now. Case study We report a 13 years-old girl with negative history for neurological or autoimmune disease that developed at the sixth day of high fever a super-refractory status epilepticus. All investigations, including the most common antibodies related to immune-mediated encephalitis were negative. Seizures continued despite several therapeutic trials with anesthetics (midazolam, propofol) and antiepileptic agents as well as i.v. immunoglobulins but responded, at day 10 from the onset, to ketamine and high dose i.v. steroids. Due the high suspicion of autoimmune encephalitis we tested patient's CSF and plasma on mouse brain with positive response. We subsequently detected a high titre of GABAAR antibodies. After the resolution of the status epilepticus the patient achieved complete recovery of neurological functions. Conclusion this is the first reported case of a FIRES-like condition due to autoimmune encephalitis mediated by GABAAR antibodies. Our case suggests that GABAAR antibodies should be investigated FIRES.

Published
2018
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9. Les marqueurs sériques sont-ils corrélés au caractère invasif de la procédure en arthroplastie primaire de hanche ? Étude prospective randomisée comparant les approches antérieures et latérales directes

Author

Alessandro Carrozzo, Ferdinando Iannotti, Ilaria Nicolosi, Edoardo Viglietta, Daniele Mazza, Raffaele Iorio, Andrea Ferretti, and Attilio Speranza

Subjects
Gynecology, medicine.medical_specialty, business.industry, law.invention, Randomized controlled trial, Harris Hip Score, law, Troponin I, medicine, Operative time, Orthopedics and Sports Medicine, Surgery, In patient, Prospective randomized study, Anterior approach, Implant, business
Abstract

Background Advantages of minimally invasive approaches for total hip arthroplasty are still matter of debate. Serum markers have been assessed as objective method to quantify muscle damage after surgery but in literature ambiguous results have been reported. The aim of this prospective randomized study was to: 1) compare serum markers elevation between a minimally invasive direct anterior approach (DAA) and a direct lateral approach (DLA), 2) to establish a correlation between serum markers increase and other peri-operative variables. Hypothesis A lesser elevation of markers could be found in patients who underwent a minimally invasive DAA. Patients and methods Seventy patients were enrolled and randomly divided in two groups according to the type of the approach. All patients were treated with the same implant by the same surgeon and received the same rehabilitation protocol. Demographic data, pre-operative Harris Hip Score (HHS) and operative time were recorded. Myoglobin, creatine kinase MB (CK-MB), troponin I, C-reactive protein (CRP), haemoglobin (HB) and pain levels were measured pre- and post-operatively. Results Mean post-operative rise were 524.9 ± 134.6 and 667.8 ± 409.5 for myoglobin, 4.8 ± 2.5 and 6.6 ± 3.7 for CK-MB, and 16.9 ± 5.3 and 15.4 ± 6.4 for PCR, in DAA and DLA groups respectively. In both groups, post-operatively values were significantly higher than pre-operatively (p 0.05). Conclusion Serum markers of muscle damage and inflammation increased in the post-operative period without significant differences between DAA and DLA, even though overall trend was higher in DLA group. The DAA group had significantly lower levels of post-operative pain. No significant correlation between pain and serum markers levels was found. Level of evidence I; randomized study.

Published
2021
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10. Clinical and neuroimaging characteristics of MOG autoimmunity in children with acquired demyelinating syndromes

Author

Claudia Ruscitto, Federico Vigevano, Lorenzo Figà Talamanca, Claudia Papi, Massimiliano Valeriani, Laura Papetti, Romina Moavero, Fabiana Ursitti, Michela Ada Noris Ferilli, and Raffaele Iorio

Subjects
Pathology, medicine.medical_specialty, Encephalomyelitis, Encephalopathy, Autoimmunity, Neuroimaging, Optic neuritis, Transverse myelitis, Myelin oligodendrocyte glycoprotein, 03 medical and health sciences, Acquired demyelinating syndromes, 0302 clinical medicine, immune system diseases, Acute disseminated encephalomyelitis, medicine, Humans, 030212 general & internal medicine, Child, Autoantibodies, Aquaporin 4, medicine.diagnostic_test, biology, business.industry, Lumbar puncture, Multiple sclerosis, Neuromyelitis Optica, Magnetic resonance imaging, General Medicine, Syndrome, medicine.disease, nervous system diseases, Settore MED/26 - NEUROLOGIA, nervous system, Neurology, biology.protein, Neuromyelitis optica spectrum disorders, Myelin-Oligodendrocyte Glycoprotein, Neurology (clinical), business, 030217 neurology & neurosurgery
Abstract

Background Myelin oligodendrocyte glycoprotein antibodies (MOG-IgG) have been recently reevaluated as a biomarker of acquired demyelinating syndromes (ADS) of the central nervous system (CNS). Here, we describe the clinical and neuroimaging features, and the long-term outcome of children with ADS of the CNS associated with MOG-IgG. Methods All patients underwent brain and spinal cord magnetic resonance imaging (MRI), lumbar puncture for cerebrospinal fluid (CSF) analysis and MOG-IgG and aquaporin-4 IgG (AQP4-IgG) testing. Results Forty-eight pediatric patients were recruited. MOG-IgG were detected in 11/48 (25%) patients with the following clinical presentations: encephalomyelitis (EM), 8/11 (73%); optic neuritis (ON), 2/11 (18%); transverse myelitis (TM), 1/11 (9%). Patients negative for MOG-IgG were diagnosed with Multiple Sclerosis (MS) (n=15), EM (n=7), ON (n=7), neuromyelitis optica spectrum disorders (NMOSD) (n=5), TM (n=2) and encephalitis (n=1). MOG-IgG positive patients were younger at disease onset and they more frequently experienced encephalopathy and epileptic seizures compared with negative patients. EM and inflammatory lesions involving optic nerves on MRI imaging were more frequent in MOG-IgG positive patients. None of the patients with MOG-IgG became persistently seronegative during the follow-up, although a decrease in MOG-IgG titer was observed. Patients with MOG-IgG showed a good response to therapy and only two patients presented relapses during follow-up. Conclusion This study supports the distinction of MOG autoimmune oligodendrocytopathy as a unique disease entity, with clinical features different from those of MS and AQP4-IgG-positive NMOSD.

Published
2021
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11. Wilson disease: Many guidelines but still many unsolved doubts

Author

Fabiola Di Dato, Raffaele Iorio, and Iorio, Raffaele

Subjects
medicine.medical_specialty, Hepatology, business.industry, Gastroenterology, MEDLINE, Disease, Zinc, Hepatolenticular Degeneration, Practice Guidelines as Topic, Humans, Medicine, business, Intensive care medicine, Human
Published
2021
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12. Safety and efficacy of eculizumab in anti-acetylcholine receptor antibody-positive refractory generalised myasthenia gravis (REGAIN): a phase 3, randomised, double-blind, placebo-controlled, multicentre study

Author

Kazumi Takada, Vladislav Abramov, Seiko Yoshida, Pinar Ozcelik, Carolina Miranda, Jennifer Kane, Kaitlyn McKenna, Natasha Campbell, Sharon P. Nations, Shitiz Kumar Sriwastava, Yuko Fujii, Mayumi Murata, Linda Wagemaekers, Angela Andoin, Mollie Vanderhook, Yoshinori Okubo, Martin Bilsker, Taira Uehara, Vera Bril, Julia Wanschitz, Stanislava Toncrova, Mariela Bettini, Kazumi Futono, Shachie Aranke, Yool-hee Kim, Hiroyuki Murai, Anne Nyrhinen, Vinay Chaudhry, Raffaele Iorio, Takashi Kanda, Brittany Harvey, Francisco Javier Rodriguez de Rivera, Henning Andersen, Marianne de Visser, Miwako Sato, Yasuhiro Maeda, Fabienne Deruelle, Marina Pozo, Adam Hart, Masaki Saitoh, Wladimir Bocca Vieira de Rezende Pinto, Said R. Beydoun, Lindsay Zilliox, Akihiro Mukaino, Cinzia Caserta, Mahi Jasinarachchi, Andrea M. Corse, Nikoletta Papadopoulou, JuYoung Kwon, Fernanda Carrara, Juliet Saba, Masayuki Makamori, Vittorio Frasca, Luciana Souza Duca, Hoo Nam Kang, C. Trebst, Celile Phan, Muzeyyen Ugur, Eduardo Ng, Jonathan McKinnon, Hila Bali Kuperman, David Feder, Judit Matolcsi, Jiri Pitha, Martin Stangel, Kate Beck, Gabriel Paiva, Diego Lopergolo, Katrien De Mey, Hidenori Matsuo, Lucas Eduardo Pazetto, Eugene Lai, Amanda Anderson, Ann D'Hondt, Tetsuya Akiyama, Beverly Fyfe, Bella Gross, Elisabet Arribas-Ibar, Kathy de Koning, Gulmohor Roy, Dmitry Pokhabov, Maria Johanna Keijzers, Nicholas Ventura, Tessa Marburger, John Loor, Ji Eun Lee, Alessandro Filla, Celal Tuga, Stephanie Scala, Rudy Mercelis, Marc H. De Baets, Hisako Kobayashi, Stanislav Vohanka, Ana Paula Macagnan, Ana Carolina Amaral de Andrade, Heike Arndt, Giovanni Antonini, Yumi Yamashita, Gwendal Le Masson, Sonia Garcia, Sarah Verjans, James F. Howard, Zaeem A. Siddiqi, Yuen T. So, Megumi Koga, Exuperio Diez Tejedor, Teresa Costabile, Mihoko Takada Takada, Steve Hopkins, Jonathan S. Katz, Charlene Hafer-Macko, Erica Nogueira Coelho, Hung Youl Seok, Carol Herbert, Yuriko Nagane, Didem Altiparmak, Sachiko Kamakura, Mohammad Sanjak, Caroline Moreau, Jordi Díaz-Manera, Sivakumar Sathasivam, Michael Vytopil, Amelia Evoli, Masakatsu Motomura, Ester Reggio, Guy Van den Abeele, Hélène Zéphir, Asya Yarmoschuk, Jasmine Hewlett, Amy Wilson, Sachie Fukui, Cavit Boz, Iandra Souza, Morgane Gaboreau, Ivana Jurajdova, Sonia Decressac, Yong Seo Koo, Valentina Pegoraro, Seung Min Kim, Benison Keung, Rosana Rocha, Nanna Witting, John Vissing, Elaine Weiner, Ali Malekniazi, Larisa Babenko, Amanda C. Guidon, Gal Maier, Charlotte Smetcoren, Robert M. Pascuzzi, Domenico Marco Bonifati, Yumiko Nakamura, Tamires Cristina Gomes da Silva, Takashi Murahara, Sarah Plevka, Tomoko Tsuda, John C. Kincaid, Arnaud Lacour, Ibrez Bandukwala, Alan R. Berger, Chang Nyoung Lee, Jae-Sung Lim, Vern C. Juel, Tulio E. Bertorini, Valeria Cavalcante Lino, Namie Taichi, Ju-Hong Min, Josep Gamez, Nelly Greenbereg, William S. David, Srikanth Muppidi, Husnu Efendi, Pedro Lopez Ruiz, Baki Dogan, Cansu Semiz, Natalia Julia Palacios, Sharon Downing, Paola Cudia, Daniel Jacobs, Can Ebru Bekircan-Kurt, Takayasu Fukudome, Kristen Roe, Lena Bjarbo, Nicole Kassebaum, Makoto Samukawa, Shizuka Asada, Christina Dheel, Fatima Maqsood, Eun Bi Hwang, Kevin Daniels, Sevim Erdem-Ozdamar, Olivier Stevens, Claudio Mazia, Karan Alcon, Sibel Gazioglu, Keiko Kikutake, Luis Lay, Petra Tilkin, Corrado Angelini, Derrick Blackmore, Kimiaki Utsugisawa, Despoina Charalambous, Tuula Harrison, Kristin Huynh, Huned S. Patwa, Laura Echevarria, Henrique Mohr, Christian Homedes-Pedret, Richard J. Barohn, Byung Jo Kim, Daniel DiCapua, Terry McClain, Debora Dada Martineli Torres, Maria Salvado Figueras, Ana Paula Melo, Riley Snook, Miki Ogawa, Marcelo Annes, Yuka Saito, Isabel Illa, Evanthia Bernitsas, Nicole Smalley, Molly Lindsay, Robert G. Miller, Olga Azrilin, Silvia Bonanno, Evgeniya Kosykh, Marcela Wolfova, Olivier Outteryck, Shirli Toska, Anna Kostera-Pruszczyk, HyeJin Ra, Rup Tandan, Sotirios Papagiannopoulos, Natasha Willlems, Anne Mette Ostergaard Autzen, Meinoshin Okumura, Patrick Vermersch, Sarada Sakamuri, Maria Antonia Alberti Aguilo, Shigemi Shimose, Cynthia Carter, Ira Blount, Lisa Thompson, Maurer Pereira Martins, Richard Nowak, Hyung Seok Lee, Anna Kaminska, Joan Bratton, Nazire Pinar Acar, Junichi Ogasawara, Mohamed Mahdi-Rogers, Teiichiro Mitazaki, Marek Čierny, Craig Donahue, Jaya Trivedi, Neelam Goyal, Gonzalo Vidal, Brandy Quarles, Akiko Kanzaki, Yasuko Ikeda, Tomomi Kobashikawa, Morris Brown, Daisuke Yamamoto, Michel Deneve, Denis Korobko, Beth DiSanzo, Benedikt Schoser, Heidi Boterhoven, Eri Kobayashi, Maoko Shirane, Cristiani Fernanda Butinhao, Eriko Higuchi, Takashi Hayashi, Masanori Takahashi, Anne-Cécile Wielanek-Bachelet, Benjamin Rix Brooks, Emanuela Onesti, Tahseen Mozaffar, Liang Lu, Sevasti Bostantzopoulou, Christophe Vial, Shawn J. Bird, Sandi Mumfrey-Thomas, Julie Khoury, Kara Patrick, Kenichi Tsukita, Yoshiko Sano, Hiroshi Nakazora, David P. Richman, Gavin Brown, Yoon-Ho Hong, Tomohiro Kawamura, Igor Dias Brockhausen, Ye Liu, Acary Souza Bulle Oliveira, Soichiro Funaka, Tomoya Hasuike, Frank Lin, Luis Antonio Querol Gutierrez, Namita Goyal, Elena Pinzan, Michelle Mellion, Silvia Messina, Christopher Lindberg, Csilla Rozsa, J. Chad Hoyle, Yoko Kaneko, Gustavo Duran, Francesco Patti, Arshira Seddigh, Ele Kim Perez, Jayashri Srinivasan, Michael Benatar, Philip Van Damme, Salma Akhter, Daniel Ambrosio, Maria Salvado, Floyd Jones, Mark Sivak, Anneke J. van der Kooi, Karen Callison, Catherine Nigro, Rebekah Garcia, Thomas Arnold, Hideki Arima, Brigid Crabtree, Mary Varghese, Aditya Kumar, Miri Kim, Fanny O'Brien, Naya McKinnon, Lauren Wheeler, Hong Vu, Shunsuke Yoshimura, Masatoshi Omoto, Jeffrey T. Guptill, Maria Gabriele, Francoise Bouhour, Veena Mathew, Ritsu Nakayama, Rosa Hasan, Francesco Saccà, Mohammed Salajegheh, Diana Dimitrova, Alzira Alves de Siqueira Carvalho, Maurizio Inghilleri, George Sachs, Rekha Pillai, Enrico Marano, Monika Konyane, Anh Tran, Seda Aydinlik, Kendrick Henderson, Fumie Meguro, Alexandre Guerreiro, Amaiak Chilingaryan, Tiyonnoh Cash, Jun Kawamata, Julie Steele, Helene Gervais-Bernard, Thomas Harbo, Alejandra Dalila Garcia, Musa Kazim Onar, Sabrina Sacconi, Carlos Casasnovas Pons, Nadezhda Malkova, Denis Sazonov, Mireya Fernandez-Fournier, Karin Fricke, Laurie Gutmann, Amy Saklad, Clara Schommer, Sandra Taber, Fiona Norwood, Tugce Kirbas Cavdar, Monique Miesen, Fernanda Troili, Masanori Watanabe, Ratna Bhavaraju-Sanka, Ted M. Burns, Sari Atula, Faisal Sohail, Barbora Kurkova, Brigitta Szabadosne, Luciana Renata Cubas Volpe, Jane Pedersen, Jing Jing Wang, Masashi Inoue, Antonella Di Pasquale, Megan Kramer, Magda Chmelikova, Mehran Soltani, Tuan Vu, Laura Fionda, Eliz Agopian, Susan Shin, Anthony A. Amato, Lotte Vinge, Hakan Cavus, Gil I. Wolfe, Joan Nye, Delphine Mahieu, Miguel Wilken, Markus Färkkilä, Catherine Faber, Erin Manning, Emiko Tsuda, Rami Massie, Paolo Emilio Alboini, Yasmeen Shabbir, Angela Campanella, Aikaterini Dimitriou, Marcelo Rugiero, Cynthia Bodkin, Gyorgyi Szabo, Sharon Halton, Akshay Shah, Yasuko Maeda, Hans D. Katzberg, Yagmur Caliskan, Jaimin Shah, Katsuhisa Masaki, Valentina Damato, Blanka Andersson, Aline de Cassia Santos, Masahiro Mori, Renato Mantegazza, Misa Shimpo, Joanne Nemeth, Livia Dezsi, Anna De Rosa, Doreen Ho, Julie Moutarde, Efstathia Mitropoulou, Amy Woodall, Angela Micheels, László Vécsei, Byoung Joon Kim, Lisa Smith, Tomihiro Imai, Harpreet Kaur, Lorenzo Maggi, Jane Distad, Anita Mogensen, Ericka Simpson, Anne Cooley, Eliana Reyes, Ha Young Shin, Da Yoon Koh, Stefan Gingele, Susan Strom, Ezgi Yilmaz, Manisha Chopra, Anna Melnikova, Edouard Millois, Ludwig Gutmann, Miriam Freimer, Hirokazu Shinozaki, Heena Olalde, Kerry Naunton, Shunya Nakane, Ihsan Sengun, Dimos-Dimitrios Mitsikostas, Edina Varga, Juha-Pekka Erälinna, Wolfgang Löscher, Jan De Bleecker, Elena Bravver, Ana Lazaro, Eun Bin Cho, Thomas Cochrane, Jonathan Goldstein, Lisa D. Hobson-Webb, Michaela Tyblova, Angela Marsil, J. 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Rosenfeld, Cigdem Ozen Aydin, Shara Holzberg, Hélène Merle, Olga Zapletalova, Kurt-Wolfram Suehs, Robert P. Lisak, Dale J. Lange, Albert Hietala, Sedat Sen, Elena Giacomelli, Akiyuki Uzawa, Tomás Augusto Suriane Fialho, Matteo Garibaldi, Nadia Sattar, Wai-Kuen Leong, Lindsay Kaplan, Tetsuya Kanai, Jaana Eriksson, Akiko Nagaishi, Khema Sharma, Tamar Gibson, Mohamed Kazamel, Yulia Nesterova, Sascha Alvermann, Murat Terzi, Taylor Darnell, Donna Carnes, Victor Balyazin, John T. Kissel, Waqar Waheed, Jana Junkerova, Kimberly Robeson, Nicholas Vlaikidis, Nicholas Silvestri, Fredrik Piehl, Maurício André Gheller Friedrich, Shun Shimohama, Nuria Vidal, Eleni Kasioti, H. James Jones, Michael K. Hehir, Luiz Augusto da Silva, Dave Watling, Leslie Roberts, Casey Faigle, Caroline Hourquin, Olli Oksaranta, Tomomi Imamura, Shin Hisahara, Dennis Jeffery, Marie-Hélène Soriani, M. Kawai, Chieko Yoshikawa, Roseann Keo, Angela Genge, Michelangelo Maestri Tassoni, Milvia Pleitez, Michael H. 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Kwan, Hidekazu Suzuki, Dai Matsuse, Kelly Jia, Ozlem Tun, Lara Katzin, Yasushi Suzuki, Shannon Lucy, Carlo Antozzi, ANS - Neuroinfection & -inflammation, Neurology, Howard, James F, Utsugisawa, Kimiaki, Benatar, Michael, Murai, Hiroyuki, Barohn, Richard J, Illa, Isabel, Jacob, Saiju, Vissing, John, Burns, Ted M, Kissel, John T, Muppidi, Srikanth, Nowak, Richard J, O'Brien, Fanny, Wang, Jing-Jing, Mantegazza, Renato, Mazia, Claudio Gabriel, Wilken, Miguel, Ortea, Carolina, Saba, Juliet, Rugiero, Marcelo, Bettini, Mariela, Vidal, Gonzalo, Garcia, Alejandra Dalila, Lamont, Phillipa, Leong, Wai-Kuen, Boterhoven, Heidi, Fyfe, Beverly, Roberts, Leslie, Jasinarachchi, Mahi, Willlems, Natasha, Wanschitz, Julia, Löscher, Wolfgang, De Bleecker, Jan, Van den Abeele, Guy, de Koning, Kathy, De Mey, Katrien, Mercelis, Rudy, Wagemaekers, Linda, Mahieu, Delphine, Van Damme, Philip, Smetcoren, Charlotte, Stevens, Olivier, Verjans, Sarah, D'Hondt, Ann, Tilkin, Petra, Alves de Siqueira Carvalho, Alzira, 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Michel, Deruelle, Fabienne, Schoser, Benedikt, Wenninger, Stephan, Stangel, Martin, Alvermann, Sascha, Gingele, Stefan, Skripuletz, Thoma, Suehs, Kurt-Wolfram, Trebst, Corinna, Fricke, Karin, Papagiannopoulos, Sotirio, Bostantzopoulou, Sevasti, Vlaikidis, Nichola, Zampaki, Martha, Papadopoulou, Nikoletta, Mitsikostas, Dimos-Dimitrio, Kasioti, Eleni, Mitropoulou, Efstathia, Charalambous, Despoina, Rozsa, Csilla, Horvath, Melinda, Lovas, Gabor, Matolcsi, Judit, Szabo, Gyorgyi, Szabadosne, Brigitta, Vecsei, Laszlo, Dezsi, Livia, Varga, Edina, Konyane, Monika, Gross, Bella, Azrilin, Olga, Greenbereg, Nelly, Bali Kuperman, Hila, Antonini, Giovanni, Garibaldi, Matteo, Morino, Stefania, Troili, Fernanda, Di Pasquale, Antonella, Filla, Alessandro, Costabile, Teresa, Marano, Enrico, Sacca, Francesco, Marsili, Angela, Puorro, Giorgia, Maestri Tassoni, Michelangelo, De Rosa, Anna, Bonanno, Silvia, Antozzi, Carlo, Maggi, Lorenzo, Campanella, Angela, Angelini, Corrado, Cudia, Paola, Pegoraro, 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Edward, Quarles, Brandy, Smalley, Nicole, Amato, Anthony, Cochrane, Thoma, Salajegheh, Mohammed, Roe, Kristen, Amato, Katherine, Toska, Shirli, Wolfe, Gil, Silvestri, Nichola, Patrick, Kara, Zakalik, Karen, Katz, Jonathan, Miller, Robert, Engel, Marguerite, Bravver, Elena, Brooks, Benjamin, Plevka, Sarah, Burdette, Maryanne, Sanjak, Mohammad, Kramer, Megan, Nemeth, Joanne, Schommer, Clara, Juel, Vern, Guptill, Jeffrey, Hobson-Webb, Lisa, Beck, Kate, Carnes, Donna, Loor, John, Anderson, Amanda, Lange, Dale, Agopian, Eliz, Goldstein, Jonathan, Manning, Erin, Kaplan, Lindsay, Holzberg, Shara, Kassebaum, Nicole, Pascuzzi, Robert, Bodkin, Cynthia, Kincaid, John, Snook, Riley, Guinrich, Sandra, Micheels, Angela, Chaudhry, Vinay, Corse, Andrea, Mosmiller, Betsy, Ho, Doreen, Srinivasan, Jayashri, Vytopil, Michael, Ventura, Nichola, Scala, Stephanie, Carter, Cynthia, Donahue, Craig, Herbert, Carol, Weiner, Elaine, Mckinnon, Jonathan, Haar, Laura, Mckinnon, Naya, Alcon, Karan, Daniels, Kevin, Sattar, Nadia, Jeffery, Denni, Mckenna, Kaitlyn, Guidon, Amanda, David, William, Dheel, Christina, Levine-Weinberg, Mark, Nigro, Catherine, Simpson, Ericka, Appel, Stanley H, Lai, Eugene, Lay, Lui, Pleitez, Milvia, Halton, Sharon, Faigle, Casey, Thompson, Lisa, Sivak, Mark, Shin, Susan, Bratton, Joan, Jacobs, Daniel, Brown, Gavin, Bandukwala, Ibrez, Brown, Morri, Kane, Jennifer, Blount, Ira, Freimer, Miriam, Hoyle, J. Chad, Agriesti, Julie, Khoury, Julie, Marburger, Tessa, Kaur, Harpreet, Dimitrova, Diana, Mellion, Michelle, Sachs, George, Crabtree, Brigid, Keo, Roseann, Perez, Ele Kim, Taber, Sandra, Gilchrist, Jame, Andoin, Angela, Darnell, Taylor, Goyal, Neelam, Sakamuri, Sarada, So, Yuen T, Welsh, Lesly Welsh, Bhavaraju-Sanka, Ratna, Tobon Gonzalez, Alejandro, Jones, Floyd, Saklad, Amy, Nations, Sharon, Trivedi, Jaya, Hopkins, Steve, Kazamel, Mohamed, Alsharabati, Mohammad, Lu, Liang, Mumfrey-Thomas, Sandi, Woodall, Amy, Richman, David, Butters, Janelle, Lindsay, Molly, Mozaffar, Tahseen, Cash, Tiyonnoh, Goyal, Namita, Roy, Gulmohor, Mathew, Veena, Maqsood, Fatima, Minton, Brian, Jones, H. Jame, Rosenfeld, Jeffrey, Garcia, Rebekah, Garcia, Sonia, Echevarria, Laura, Pulley, Michael, Aranke, Shachie, Berger, Alan Ro, Shah, Jaimin, Shabbir, Yasmeen, Smith, Lisa, Varghese, Mary, Gutmann, Laurie, Gutmann, Ludwig, Swenson, Andrea, Olalde, Heena, Hafer-Macko, Charlene, Kwan, Justin, Zilliox, Lindsay, Callison, Karen, Disanzo, Beth, Naunton, Kerry, Bilsker, Martin, Sharma, Khema, Reyes, Eliana, Cooley, Anne, Michon, Sara-Claude, Steele, Julie, Karam, Chafic Karam, Chopra, Manisha, Bird, Shawn, Kaufman, Jacob, Gallatti, Nichole, Vu, Tuan, Katzin, Lara, Mcclain, Terry, Harvey, Brittany, Hart, Adam, Huynh, Kristin, Beydoun, Said, Chilingaryan, Amaiak, Droker, Brian, Lin, Frank, Shah, Akshay, Tran, Anh, Akhter, Salma, Malekniazi, Ali, Tandan, Rup, Hehir, Michael, Waheed, Waqar, Lucy, Shannon, Weiss, Michael, Distad, Jane, Downing, Sharon, Strom, Susan, Lisak, Robert, Bernitsas, Evanthia, Khan, Omar, Kumar Sriwastava, Shitiz, Tselis, Alexandro, Jia, Kelly, Bertorini, Tulio, Arnold, Thoma, Henderson, Kendrick, Pillai, Rekha, Liu, Ye, Wheeler, Lauren, Hewlett, Jasmine, Vanderhook, Mollie, Dicapua, Daniel, Keung, Benison, Kumar, Aditya, Patwa, Huned, Robeson, Kimberly, Nye, Joan, Vu, Hong, Howard, J, Utsugisawa, K, Benatar, M, Murai, H, Barohn, R, Illa, I, Jacob, S, Vissing, J, Burns, T, Kissel, J, Muppidi, S, Nowak, R, O'Brien, F, Wang, J, Mantegazza, R, and Bonanno, S

Subjects
Male, 0301 basic medicine, medicine.medical_treatment, Drug Resistance, Adult, Aged, Antibodies, Monoclonal, Humanized, Autoantibodies, Double-Blind Method, Female, Humans, Middle Aged, Myasthenia Gravis, Receptors, Cholinergic, Outcome Assessment (Health Care), Severity of Illness Index, Neurology (clinical), law.invention, Complement inhibitor, 0302 clinical medicine, Randomized controlled trial, law, Monoclonal, Receptors, Clinical endpoint, Humanized, Cholinergic, education.field_of_study, Eculizumab, Autoantibodie, Myasthenia Gravi, Settore MED/26 - NEUROLOGIA, Human, medicine.drug, Meningitides, medicine.medical_specialty, Population, Placebo, Antibodies, 03 medical and health sciences, Internal medicine, medicine, education, business.industry, Surgery, Thymectomy, 030104 developmental biology, business, 030217 neurology & neurosurgery
Abstract

Background Complement is likely to have a role in refractory generalised myasthenia gravis, but no approved therapies specifically target this system. Results from a phase 2 study suggested that eculizumab, a terminal complement inhibitor, produced clinically meaningful improvements in patients with anti-acetylcholine receptor antibody-positive refractory generalised myasthenia gravis. We further assessed the efficacy and safety of eculizumab in this patient population in a phase 3 trial. Methods We did a phase 3, randomised, double-blind, placebo-controlled, multicentre study (REGAIN) in 76 hospitals and specialised clinics in 17 countries across North America, Latin America, Europe, and Asia. Eligible patients were aged at least 18 years, with a Myasthenia Gravis-Activities of Daily Living (MG-ADL) score of 6 or more, Myasthenia Gravis Foundation of America (MGFA) class II-IV disease, vaccination against Neisseria meningitides, and previous treatment with at least two immunosuppressive therapies or one immunosuppressive therapy and chronic intravenous immunoglobulin or plasma exchange for 12 months without symptom control. Patients with a history of thymoma or thymic neoplasms, thymectomy within 12 months before screening, or use of intravenous immunoglobulin or plasma exchange within 4 weeks before randomisation, or rituximab within 6 months before screening, were excluded. We randomly assigned participants (1:1) to either intravenous eculizumab or intravenous matched placebo for 26 weeks. Dosing for eculizumab was 900 mg on day 1 and at weeks 1, 2, and 3; 1200 mg at week 4; and 1200 mg given every second week thereafter as maintenance dosing. Randomisation was done centrally with an interactive voice or web-response system with patients stratified to one of four groups based on MGFA disease classification. Where possible, patients were maintained on existing myasthenia gravis therapies and rescue medication was allowed at the study physician's discretion. Patients, investigators, staff, and outcome assessors were masked to treatment assignment. The primary efficacy endpoint was the change from baseline to week 26 in MG-ADL total score measured by worst-rank ANCOVA. The efficacy population set was defined as all patients randomly assigned to treatment groups who received at least one dose of study drug, had a valid baseline MG-ADL assessment, and at least one post-baseline MG-ADL assessment. The safety analyses included all randomly assigned patients who received eculizumab or placebo. This trial is registered with ClinicalTrials.gov, number NCT01997229. Findings Between April 30, 2014, and Feb 19, 2016, we randomly assigned and treated 125 patients, 62 with eculizumab and 63 with placebo. The primary analysis showed no significant difference between eculizumab and placebo (least-squares mean rank 56·6 [SEM 4·5] vs 68·3 [4·5]; rank-based treatment difference -11·7, 95% CI -24·3 to 0·96; p=0·0698). No deaths or cases of meningococcal infection occurred during the study. The most common adverse events in both groups were headache and upper respiratory tract infection (ten [16%] for both events in the eculizumab group and 12 [19%] for both in the placebo group). Myasthenia gravis exacerbations were reported by six (10%) patients in the eculizumab group and 15 (24%) in the placebo group. Six (10%) patients in the eculizumab group and 12 (19%) in the placebo group required rescue therapy. Interpretation The change in the MG-ADL score was not statistically significant between eculizumab and placebo, as measured by the worst-rank analysis. Eculizumab was well tolerated. The use of a worst-rank analytical approach proved to be an important limitation of this study since the secondary and sensitivity analyses results were inconsistent with the primary endpoint result; further research into the role of complement is needed. Funding Alexion Pharmaceuticals.

Published
2017
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13. Endoscopic Excision of Symptomatic Os Trigonum in Professional Dancers

Author

Daniele Mazza, Andrea Ferretti, Cosma Calderaro, Marco Guidi, Federico Morelli, Pierluigi Serlorenzi, Raffaele Iorio, Emanuela Camerucci, and Matteo Guzzini

Subjects
Adult, Male, medicine.medical_specialty, Risk Assessment, Severity of Illness Index, Sampling Studies, Talus, Arthroscopy, 03 medical and health sciences, 0302 clinical medicine, Patient satisfaction, ankle, dancer, endoscopy, os trigonum, talus, surgery, orthopedics and sports medicine, Humans, Medicine, Orthopedics and Sports Medicine, Major complication, Dancing, Pain Measurement, Retrospective Studies, 030222 orthopedics, medicine.diagnostic_test, business.industry, Syndrome, 030229 sport sciences, Endoscopic excision, Arthralgia, Endoscopic Procedure, Osteotomy, Surgery, Endoscopy, Os Trigonum, Treatment Outcome, medicine.anatomical_structure, Female, Ankle, business, Tegner Activity Scale, Ankle Joint, Follow-Up Studies
Abstract

The present study reports the clinical results of excision of a symptomatic os trigonum using an endoscopic procedure in professional ballet dancers. The hypothesis was that posterior endoscopic excision of the os trigonum would be safe and effective in treating posterior ankle impingement syndrome related to the os trigonum. Twelve professional dancers underwent excision of a symptomatic os trigonum for PAIS using a posterior endoscopic technique after failure of conservative treatment. The patients were evaluated pre- and postoperatively according to the American Orthopaedic Foot and Ankle Society hindfoot scale score, the Tegner activity scale score, and the visual analog scale score. The surgical time, timing of a return to sports, patient satisfaction, and any complications related to the procedure were recorded. The average postoperative follow-up duration was 38.9 ± 20.6 (range 12 to 72) months. The mean Tegner activity scale score increased from 4.3 ± 0.8 (range 3 to 5) preoperatively to 9 ± 0.2 postoperatively (p

Published
2017
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14. 422 ORAL VANCOMYCIN THERAPY IS ASSOCIATED WITH IBD CLINICAL REMISSION IN PEDIATRIC PSC-IBD

Author

Katryn N. Furuya, Douglas Mogul, Madeleine Aumar, Kathleen B. Schwarz, Mercedes Martinez, Atsushi Tanaka, Kuan Liu, Kaija-Leena Kolho, Bernadette Vitola, Wael El-Matary, Tamir Miloh, Alexandre Rodrigues Ferreira, Smolka Vratislav, Trevor J. Laborda, Laura G. Draijer, Annemarie Broderick, Melissa Zerofsky, Nanda Kerkar, Sirish Palle, Simon Horslen, Maureen M. Jonas, Emily R. Perito, Kathleen M. Loomes, Christine K. Lee, Marek Woynarowski, Kyung Mo Kim, Eleonora Druve Tavares Fagundes, Veena Venkat, Nadia Ovchinsky, Amanda Ricciuto, Pamela L. Valentino, Binita M. Kamath, Mark Deneau, Federica Ferrari, Bart G. P. Koot, Cara L. Mack, Achiya Z. Amir, Girish S. Rao, Nitika A. Gupta, Saeed Mohammad, and Raffaele Iorio

Subjects
Ibd clinical, medicine.medical_specialty, Hepatology, business.industry, Internal medicine, Gastroenterology, Medicine, business, Oral vancomycin
Published
2021
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15. Combined Intra-articular and Extra-articular Reconstruction in Anterior Cruciate Ligament–Deficient Knee: 25 Years Later

Author

Andrea Ferretti, Ludovico Caperna, Luca Basiglini, Edoardo Monaco, Raffaele Iorio, Antonio Ponzo, and Fabio Conteduca

Subjects
Adult, Male, medicine.medical_specialty, Adolescent, Knee Joint, Anterior cruciate ligament reconstruction, Anterior cruciate ligament, medicine.medical_treatment, Hamstring Muscles, Osteoarthritis, Young Adult, 03 medical and health sciences, 0302 clinical medicine, medicine, Humans, Orthopedics and Sports Medicine, Autografts, Physical Therapy Modalities, Retrospective Studies, Postoperative Care, 030222 orthopedics, Lysholm Knee Score, Anterior Cruciate Ligament Reconstruction, Arthrometry, Articular, business.industry, Retrospective cohort study, 030229 sport sciences, Pivot-shift test, Middle Aged, Osteoarthritis, Knee, musculoskeletal system, medicine.disease, Surgery, Patient Outcome Assessment, medicine.anatomical_structure, Female, business, Hamstring, Follow-Up Studies
Abstract

Purpose To determine whether an anterior cruciate ligament (ACL)–reconstructed knee with hamstring autograft has a greater incidence of degenerative changes when an extra-articular reconstruction is added and to determine the effect of the combined reconstruction on knee stability and function at long-term follow-up. Methods For this retrospective study, patients who underwent ACL reconstruction between January 2002 and December 2003 were selected and classified into 2 groups, I and II. Group I consisted of patients operated by a standard ACL reconstruction, and group II consisted of patients in whom, owing to the presence of risk factors (severe pivot shift graded +++ or high-risk sports), an extra-articular reconstruction performed with ileo-tibial tract according to McIntosh as modified by Cocker Arnold technique, was used in conjunction with intra-articular ACL reconstruction. At final follow-up, Lysholm, International Knee Documentation Committee (IKDC), and Tegner scores were used. An arthrometric KT-1000 evaluation was done. Comparative weight-bearing radiographs were taken, including a skyline view for the patellofemoral joint and analyzed according to Fairbank, Kellgren, and IKDC classification. Results Seventy-two of the 75 (96%) patients in group I and 68 of the 75 patients in group II were available at the final follow-up (minimum 10 years). Subjective scores improved significantly in both groups, with no significant difference. Objectively, the number of patients receiving C and D IKDC objective activity scores in group I (7/56; 12.5%) was significantly higher than in group II (0/60) ( P = .01). Considering as a failure a side-to-side arthrometric difference more than 5 mm or a pivot shift test graded as ++ or +++, or any giving way episode occurring postoperatively, we found 8 cases in group I and no cases in group II ( P = .01) despite the presence of risk factors that group I did not include. Radiologic evaluation showed less arthritic changes in group II in both tibiofemoral and patellofemoral joints. Conclusions On the basis of the results of this study, adding an extra-articular reconstruction to an anatomically placed intra-articular ACL reconstruction, followed by a modern rehabilitation protocol, does not increase the risk of osteoarthritis and may be able to reduce the rate of failure. Level of Evidence Level III, retrospective comparative study.

Published
2016
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16. Nanohydroxyapatite promotes the healing process in open-wedge high tibial osteotomy: A CT study

Author

Andrea Ferretti, Daniele Mazza, P. Di Sette, Fabio Conteduca, Ludovico Caperna, Giuseppe Argento, and Raffaele Iorio

Subjects
Male, Bone density, medicine.medical_treatment, Dentistry, Biocompatible Materials, Bone healing, Osteotomy, open wedge high tibial osteotomy, Osseointegration, 03 medical and health sciences, 0302 clinical medicine, High tibial osteotomy, Bone Density, Hounsfield scale, Open wedge, medicine, Humans, Orthopedics and Sports Medicine, Prospective Studies, Tibia, Fracture Healing, Wound Healing, 030222 orthopedics, Bone Transplantation, business.industry, nanohydroxyapatite, osteointegration, 030229 sport sciences, Middle Aged, Osteoarthritis, Knee, Durapatite, Bone Substitutes, Female, Tomography, X-Ray Computed, business
Abstract

Background The aim of this prospective and randomized study was to evaluate the effectiveness of adding nanohydroxyapatite (NHA) to heterologous bone graft in open wedge high tibial osteotomy (OWHTO) by measuring the bone density of the tibial osteotomy gap. Methods Twenty-seven patients (26 knees) were operated by OWHTO and randomly divided into two groups: pure graft group, in which the osteotomy gap was filled with only heterologous bone graft; nanohydroxyapatite group, in which the osteotomy gap was filled with heterologous bone graft and NHA. All patients underwent computed tomography (CT) examination within one week after operation (Time 0), and after two months (Time 1) and 12months (Time 2). CT volume acquired Hounsfield Units (HU) were calculated and the mean value of bone density on three planes was measured. Results At Time 0, the mineral density of the nanohydroxyapatite group appeared significantly higher compared with the pure graft group, due to the presence of NHA. At Time 1, the mineral density of the nanohydroxyapatite group had decreased relative to Time 0, while in the pure graft group it remained unchanged. At Time 2, the mineral density in the nanohydroxyapatite group had further decreased, reaching values close to the mineral density of normal bone. In contrast, in the pure graft group the mineral density had increased, probably due to the lack of reabsorption of the graft and the development of sclerosis in the osteotomy borders. Conclusions The results of the present study show better osseointegration of the heterologous graft when nanohydroxyapatite is added.

Published
2016
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18. Efficacy of oral corticosteroids therapy in anti-glutamic acid decarboxylase antibodies cerebellar ataxia

Author

Gaetano Salomone, Raffaele Iorio, Lazzaro di Biase, Giovanni Assenza, Andrea Onetti Muda, J.M. Melgari, Vincenzo Di Lazzaro, and Massimo Marano

Subjects
0301 basic medicine, medicine.medical_specialty, Cerebellum, Ataxia, Glutamate decarboxylase, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, medicine, Gastric mucosa, Cerebellar ataxia, biology, medicine.diagnostic_test, business.industry, Autoantibody, Magnetic resonance imaging, 030104 developmental biology, medicine.anatomical_structure, Endocrinology, Neurology, biology.protein, Neurology (clinical), Geriatrics and Gerontology, medicine.symptom, Antibody, business, Neuroscience, 030217 neurology & neurosurgery
Published
2016
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19. Computer-assisted surgery: A teacher of TKAs

Author

Daniele Mazza, Fabio Conteduca, Andrea Ferretti, Andrea Redler, G. Bolle, J. Conteduca, and Raffaele Iorio

Subjects
Male, medicine.medical_specialty, Knee Joint, Post hoc, medicine.medical_treatment, Radiography, Lower limb, symbols.namesake, Prosthesis Fitting, medicine, Humans, Orthopedics and Sports Medicine, Arthroplasty, Replacement, Knee, Aged, Aged, 80 and over, Computer-assisted surgery, Orthodontics, Analysis of Variance, biology, business.industry, Navigation system, Middle Aged, biology.organism_classification, Surgery, Valgus, Bonferroni correction, Surgery, Computer-Assisted, Coronal plane, symbols, Female, Clinical Competence, Knee Prosthesis, business, Learning Curve
Abstract

Introduction The hypothesis of this study is that computer-aided navigation experience could improve the ability to better place components in the coronal plane and to improve visual/spatial awareness based on the ability of navigation to provide instant feedback. The purpose of this study is to demonstrate the educational role of the navigation system to obtain a better alignment of the prosthetic components with standard instrumentation after a computer-aided navigation experience. Materials and methods One hundred fifty patients were operated by the same surgeon, with more than 5years experience with TKA. They were equally divided in three groups: group A (operated with non-navigated technique by surgeon without computer-assisted experience); group B (operated with computer-assisted surgery by the same surgeon); group C (operated with non-navigated technique by the same surgeon after the computer-navigated experience). We evaluated by full-length weight-bearing radiographs the overall alignment of the lower limb in the coronal plane. The optimum placement of the components was considered when the angle was within the limits of ±3° varus/valgus on the coronal x-rays. Comparison between groups was done using one-way ANOVA followed by post hoc Bonferroni test and Pearson chi-square statistics for proportions of optimum placement ( P Results In the group A 34 patients (68%) had the optimum placement on the coronal x-rays; in the group B they were 46 (92%) and in the group 41 (82%). The difference is statistically significant in comparing group A and Group B ( 0.001), group A and group C ( P =0.04), but not for group B and C ( P =0.2). Conclusion We believe that the navigation system has an educational role to improve the ability of surgeon of positioning prosthetic components precisely in the coronal plane.

Published
2013
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20. Astrocytic autoantibody of neuromyelitis optica (NMO-IgG) binds to aquaporin-4 extracellular loops, monomers, tetramers and high order arrays

Author

Raffaele Iorio, Sean J. Pittock, Hartwig Wolburg, James P. Fryer, Vanda A. Lennon, Petra Fallier-Becker, and Shannon R. Hinson

Subjects
Male, Recombinant Fusion Proteins, Immunology, Biology, Crystallography, X-Ray, Article, Epitope, Cell membrane, Epitopes, medicine, Extracellular, Humans, Immunology and Allergy, Cells, Cultured, Autoantibodies, Aquaporin 4, Neuromyelitis optica, Cell Membrane, Neuromyelitis Optica, Autoantibody, medicine.disease, Molecular biology, medicine.anatomical_structure, IgG binding, Astrocytes, Female, sense organs, Intracellular
Abstract

The principal central nervous system (CNS) water channel, aquaporin-4 (AQP4), is confined to astrocytic and ependymal membranes and is the target of a pathogenic autoantibody, neuromyelitis optica (NMO)-IgG. This disease-specific autoantibody unifies a spectrum of relapsing CNS autoimmune inflammatory disorders of which NMO exemplifies the classic phenotype. Multiple sclerosis and other immune-mediated demyelinating disorders of the CNS lack a distinctive biomarker. Two AQP4 isoforms, M1 and M23, exist as homotetrameric and heterotetrameric intramembranous particles (IMPs). Orthogonal arrays of predominantly M23 particles (OAPs) are an ultrastructural characteristic of astrocytic membranes. We used high-titered serum from 32 AQP4-IgG-seropositive patients and 85 controls to investigate the nature and molecular location of AQP4 epitopes that bind NMO-IgG, and the influence of supramolecular structure. NMO-IgG bound to denatured AQP4 monomers (68% of cases), to native tetramers and high order arrays (90% of cases), and to AQP4 in live cell membranes (100% of cases). Disease-specific epitopes reside in extracellular loop C more than in loops A or E. IgG binding to intracellular epitopes lacks disease specificity. These observations predict greater disease specificity and sensitivity for tissue-based and cell-based serological assays employing “native” AQP4 than assays employing denatured AQP4 and fragments. NMO-IgG binds most avidly to plasma membrane surface AQP4 epitopes formed by loop interactions within tetramers and by intermolecular interactions within high order structures. The relative abundance and localization of AQP4 high order arrays in distinct CNS regions may explain the variability in clinical phenotype of NMO spectrum disorders.

Published
2013
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21. Recurrence of Primary Sclerosing Cholangitis after Liver Transplantation in Children: Data from the Pediatric PSC Consortium

Author

Wael El-Matary, Marcus Auth, Khaled Alqoaer, Anastasia Konidari, Binita M. Kamath, Bart G. P. Koot, Mounif El-Youssef, Albert C. Y. Chan, Lawrence J. Saubermann, Bernadette Vitola, Smolka Vratislav, Federica Ferrari, Achiya Z. Amir, Pushpa Sathya, Eyal Shteyer, Alexandra Papadopoulou, M.K. Jensen, Veena Venkat, Frédéric Gottrand, Oren Ledder, Kaija-Leena Kola Kaija-Leena Kolho, Katryn N. Furuya, Mercedes Martinez, Atsushi Tanaka, Tamir Miloh, Cara L. Mack, Raghu Varier, Mansi Amin, Parvathi Mohan, Jason Yap, Annemarie Broderick, Marek Woynarowski, Fateh Bazerbachi, Jillian M. Cotter, Raffaele Iorio, Reham Abdou, Miriam B. Vos, Pamela L. Valentino, Mark Deneau, Matjaz Homan, Sylvia Doan, Amanda Ricciuto, Niamh O'Cathain, Kyung Mo Kim, Madeleine Gottrand, and Nitika A. Gupta

Subjects
medicine.medical_specialty, Hepatology, business.industry, medicine.medical_treatment, Gastroenterology, Liver transplantation, medicine.disease, Primary sclerosing cholangitis, 03 medical and health sciences, 0302 clinical medicine, 030220 oncology & carcinogenesis, Internal medicine, medicine, 030211 gastroenterology & hepatology, business
Published
2017
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24. P028 Establishing a SIGENP Italian network for the study of biliary atresia

Author

Francesco Tandoi, Giulia Paolella, Mara Cananzi, Lorenza Matarazzo, Manila Candusso, P.G. Gamba, P. Bagolan, Valerio Nobili, E. La Pergola, Claudia Mandato, Federica Nuti, J. De Ville, P.L. Calvo, G. Ranucci, Daniele Alberti, Fabio Fusaro, Giuseppe Maggiore, Maria Grazia Clemente, Renato Romagnoli, Pietro Vajro, Raffaele Iorio, Gabriella Nebbia, Marco Spada, F. Parolini, Pietro Betalli, Lorenzo D'Antiga, P. Gaio, Mario Lima, Federica Ferrari, Marco Sciveres, Daniele Serranti, F. Fascetti Leon, F. di Francesco, Davide Liccardo, Giuseppe Indolfi, and Valeria Casotti

Subjects
medicine.medical_specialty, Hepatology, business.industry, Biliary atresia, General surgery, Gastroenterology, medicine, business, medicine.disease
Published
2018
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25. Clinical and radiological outcomes of postoperative septic arthritis after anterior cruciate ligament reconstruction

Author

Attilio Speranza, Antonio Vadalà, Luca Labianca, Andrea Ferretti, Barbara Maestri, Raffaele Iorio, and Edoardo Monaco

Subjects
Adult, medicine.medical_specialty, Adolescent, Knee Joint, Anterior cruciate ligament reconstruction, medicine.medical_treatment, Anterior cruciate ligament, Therapeutic irrigation, Arthritis, Knee Injuries, Young Adult, Postoperative Complications, Internal medicine, Staphylococcus epidermidis, medicine, Humans, Orthopedics and Sports Medicine, Anterior Cruciate Ligament, Therapeutic Irrigation, administration /&/ dosage, adolescent, adult, adverse effects, anterior cruciate ligament, anti-bacterial agents, arthritis, etiology/microbiology/therapy, female, humans, infectious, injuries/surgery, knee injuries, knee joint, microbiology/radiography, postoperative complications, radiography/surgery, reconstructive surgical procedures, staphylococcal infections, staphylococcus epidermidis, therapeutic irrigation, therapy, young adult, Arthritis, Infectious, business.industry, Anterior Cruciate Ligament Injuries, musculoskeletal, neural, and ocular physiology, Plastic Surgery Procedures, Staphylococcal Infections, musculoskeletal system, medicine.disease, Rheumatology, Anti-Bacterial Agents, Surgery, Radiography, medicine.anatomical_structure, Orthopedic surgery, Female, Septic arthritis, business, Complication, human activities
Abstract

Septic arthritis after arthroscopic anterior cruciate ligament (ACL) reconstruction is a rare complication. In the literature, several treatments have been proposed. The aim of the study was to report our results using ambulatory irrigation of the knee and parenteral and oral antibiotics.From January 2001 to December 2008, a total of 12 patients were treated for postoperative septic arthritis of the knee after ACL reconstruction in our hospital. The average age at trauma was 24 years (range 16-43 years). The treatment protocol included irrigation of the knee (for 2 days) and parenteral antibiotics and oral antibiotics subsequently. Repetitive irrigation was performed if necessary. The average duration of follow-up for our series was 38 months (range 6-54 months). Follow-up included International Knee Documentation Committee (IKDC) forms, radiography, Tegner and Lysholm scores, and KT-1000 arthrometric evaluation.In all cases treatment of infection was successful. Neither graft nor hardware removal was needed in any of the cases. At final examination, the pivot shift was negative in 10 of 12 patients, and it was 1+ in the other 2 patients; the Lachman was negative in all cases. The mean postoperative Tegner score was 7.2, and the mean Lysholm score was 98.3. In all, 10 of the 12 patients were graded as group A and the other 2 as group B using the IKDC form. The mean postoperative manual maximum KT-1000 side-to-side difference was 2.3. No significant bone tunnel enlargement was found at radiographic evaluation.The described treatment regimen gives reliable results for this complication. There were no recurrences of septic arthritis or bone infection. No further surgeries were required, and the graft was retained during the treatment of septic arthritis.

Published
2010
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26. Increased CD4+CD25+Foxp3+ T cells in peripheral blood of celiac disease patients: Correlation with dietary treatment

Author

Viviana Nociti, Domenico Plantone, Giovanni Cammarota, Bianco Assunta, Anna Paola Batocchi, Pietro Attilio Tonali, Giovanni Frisullo, Raffaele Iorio, Agata Katia Patanella, and Alessandro Marti

Subjects
Adult, CD4-Positive T-Lymphocytes, Male, Adolescent, T-Lymphocytes, Immunology, Population, chemical and pharmacologic phenomena, T-Lymphocytes, Regulatory, Coeliac disease, Diet, Gluten-Free, Young Adult, Immune system, Humans, Immunology and Allergy, Medicine, Enteropathy, Longitudinal Studies, IL-2 receptor, education, Cells, Cultured, Cell Proliferation, Autoimmune disease, education.field_of_study, business.industry, Interleukin-2 Receptor alpha Subunit, FOXP3, Forkhead Transcription Factors, hemic and immune systems, General Medicine, Middle Aged, medicine.disease, Celiac Disease, CD4 Antigens, Female, Gluten free, business
Abstract

Regulatory CD4+ CD25+Foxp3+ T cells are involved in the regulation of immune response and inhibit protective antitumor immunity. Celiac disease (CD), a food gluten-sensitive enteropathy, is considered a T-cell-mediated autoimmune disease and is generally associated with an overall increased risk of cancer in CD patients. We observed a higher percentage of circulating CD4+CD25+Foxp3+ T cells and an increased Foxp3 expression in CD4+CD25+ T cells from untreated than from treated CD patients. In co-culture, CD4+CD25+ T cells from both treated and untreated CD patients significantly suppressed the proliferation of autologous CD4+CD25(-) T cells similarly to values in healthy subjects. Our study suggests that Treg proportion and Foxp3 expression in circulating CD4+CD25+ T cells could justify the increased global risk of malignancy in CD population and support the efficacy of lifelong gluten-free diet in the reduction of the cancer risk.

Published
2009
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27. The persistency of high levels of pSTAT3 expression in circulating CD4+ T cells from CIS patients favors the early conversion to clinically defined multiple sclerosis

Author

Viviana Nociti, Giovanni Frisullo, Anna Paola Batocchi, Pietro Attilio Tonali, Alessandro Marti, Agata Katia Patanella, Raffaele Iorio, and Massimiliano Mirabella

Subjects
Adult, CD4-Positive T-Lymphocytes, Male, STAT3 Transcription Factor, Immunology, multiple sclerosis, Peripheral blood mononuclear cell, Young Adult, pSTAT3, medicine, Humans, Immunology and Allergy, STAT1, Young adult, STAT3, CIS, Clinically isolated syndrome, biology, business.industry, Multiple sclerosis, Case-control study, medicine.disease, Settore MED/26 - NEUROLOGIA, Interleukin 10, Neurology, Case-Control Studies, Disease Progression, biology.protein, Cytokines, Female, Neurology (clinical), business
Abstract

article i nfo STAT1 STAT3 T-bet IFNγ IL17 Not all patients with clinically isolated syndrome (CIS) suggestive of multiple sclerosis (MS) develop clinically defined MS (CDMS). At first clinical event we observed increased production of IL17, IFNγ and IL10 by peripheral blood mononuclear cells from patients with CIS that remained high in remission. In CD4+ T cells pSTAT3 expression was higher in patients who subsequently converted to CDMS than in patients who did not and controls. The persistency of high levels of pSTAT3 in circulating CD4+ T cells from CIS patients after the first clinical event may favor the early conversion to CDMS.

Published
2008
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28. Is exchange transfusion a possible treatment for neonatal hemochromatosis?

Author

Giuseppina Timpani, Antonino Nicolò, Francesca Foti, Emanuele Nicastro, Raffaele Iorio, and Pier Antonio Nicotina

Subjects
Male, Pediatrics, medicine.medical_specialty, Exchange transfusion, Iron, medicine.medical_treatment, Exchange Transfusion, Whole Blood, Liver transplantation, Antioxidants, Blood Coagulation Disorders, Inherited, Fatal Outcome, Neonatal hemochromatosis, Coagulopathy, Humans, Medicine, Hemochromatosis, Hepatology, business.industry, Liver Diseases, Infant, Newborn, Infant, Acute Kidney Injury, medicine.disease, Neonatal Hemochromatosis, Surgery, Treatment, Treatment Outcome, Immune System Diseases, Liver, Etiology, Neonatal Hemochromatosis, Exchange transfusion, Treatment, Antioxidant, Female, Antioxidant, business, Kidney disease, Congenital disorder
Abstract

Neonatal hemochromatosis is a rare congenital disorder of the liver associated to a poor prognosis. Liver transplantation is often required, since no effective medical treatment has been found. Despite mounting evidence of an alloimmune etiology of this condition, exchange transfusion has never been proposed as a specific treatment for neonatal hemochromatosis. Here we describe two siblings affected by neonatal hemochromatosis. The first, a female, died at 18 days of severe coagulopathy and acute renal failure, diagnosed as affected by neonatal hemochromatosis only when the second sibling was suspected as being affected by the same disease. The second child showed a rapidly worsening coagulopathy which was treated with two exchange transfusions, followed by rapid clinical and laboratory improvement, before reaching a definite diagnosis of neonatal hemochromatosis. He is healthy at present after a follow-up of 12 months. Although exchange transfusion has never been considered as treatment for neonatal hemochromatosis, this case suggests that it could be a feasible treatment option for children affected by this disease, as for other alloimmune conditions.

Published
2007
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29. Glucocorticoid treatment reduces T-bet and pSTAT1 expression in mononuclear cells from relapsing remitting multiple sclerosis patients

Author

Giovanni Frisullo, Massimiliano Mirabella, Raffaele Iorio, Marcella Caggiula, Cristina Sancricca, Anna Paola Batocchi, Pietro Attilio Tonali, Viviana Nociti, Assunta Bianco, and Agata Katia Patanella

Subjects
Adult, CD4-Positive T-Lymphocytes, Male, STAT3 Transcription Factor, medicine.medical_specialty, CD14, Immunology, Down-Regulation, CD8-Positive T-Lymphocytes, multiple sclerosis, Methylprednisolone, Peripheral blood mononuclear cell, corticosteroids, Multiple Sclerosis, Relapsing-Remitting, Immunopathology, Internal medicine, medicine, Humans, Immunology and Allergy, Annexin A5, Phosphorylation, Glucocorticoids, Cells, Cultured, transcription factor, business.industry, Multiple sclerosis, medicine.disease, Settore MED/26 - NEUROLOGIA, STAT1 Transcription Factor, Endocrinology, Mechanism of action, Apoptosis, Leukocytes, Mononuclear, Cytokines, Female, medicine.symptom, T-Box Domain Proteins, business, CD8, Glucocorticoid, Signal Transduction, medicine.drug
Abstract

High dose glucocorticoid (GC) treatment has been demonstrated to have a short-term beneficial effect on functional recovery in relapsing multiple sclerosis (MS) patients but the exact mechanism of action of GCs in MS is unclear. We found that high dose intravenous GCs strongly reduced T-bet and pSTAT1 expression in CD4+, CD8+, CD14+ circulating cells in RRMS patients in relapse. pSTAT1and T-bet reduction was associated with the decline of IFNgamma production by PBMCs. A significant increase of AV-positive CD4+ and CD8+ T cells was detectable after GC treatment without any variation in the percentage of annexin V-positive monocytes. By in vitro analysis, patients during relapse, either before or after GC treatment, exhibited a lower proportion of apoptotic lymphocytes than remitting patients and controls. Our study suggests that GCs can modulate T-bet and STAT1 expression and that IFNgamma signalling inhibition contributes to anti-inflammatory action of GCs in the treatment of relapses of MS patients.

Published
2007
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31. A Comparison of Primary Sclerosing Cholangitis with and Without Associated Inflammatory Bowel Disease: Data from the Pediatric PSC Consortium

Author

Smolka Vratislav, Mercedes Martinez, Atsushi Tanaka, Tamir Miloh, Katryn N. Furuya, Pushpa Sathya, Jason Yap, Annemarie Broderick, Sylvia Doan, Bernadette Vitola, Binita M. Kamath, Kaija-Leena Kola Kaija-Leena Kolho, Khaled Alqoaer, Mounif El-Youssef, Alexandra Papadopoulou, Achiya Z. Amir, Bart G. P. Koot, Miriam B. Vos, Albert C. Y. Chan, Fateh Bazerbachi, Jillian M. Cotter, Raffaele Iorio, Pamela L. Valentino, Reham Abdou, Mark Deneau, Amanda Ricciuto, Wael El-Matary, Marcus Auth, Veena Venkat, Niamh O'Cathain, Anastasia Konidari, Matjaz Homan, Mansi Amin, Kyung Mo Kim, Parvathi Mohan, Marek Woynarowski, Federica Ferrari, Madeleine Gottrand, Raghu Varier, Nitika A. Gupta, M.K. Jensen, Eyal Shteyer, Oren Ledder, Frédéric Gottrand, Cara L. Mack, and Lawrence J. Saubermann

Subjects
medicine.medical_specialty, Hepatology, business.industry, Gastroenterology, medicine.disease, Primary sclerosing cholangitis, 03 medical and health sciences, 0302 clinical medicine, 030220 oncology & carcinogenesis, Internal medicine, medicine, 030211 gastroenterology & hepatology, Associated inflammatory bowel disease, business
Published
2017
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38. Extra-hepatic portal vein obstruction in children: A multicentre national study

Author

Silvia Riva, Giuseppe Indolfi, Maurizio Baldi, Graziella Guariso, Paola De Angelis, Raffaele Iorio, M. Colusso, Pietro Vajro, Lorenzo D'Antiga, Angelo Di Giorgio, and Giuseppe Maggiore

Subjects
medicine.medical_specialty, Hepatology, Hepatic portal vein obstruction, business.industry, Gastroenterology, National study, Medicine, business, Surgery
Published
2014
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39. Su1766 Management of Portal Hypertension in Children: a Retrospective Italian Multicenter Study

Author

Chiara Russo, Paolo Gandullia, Claudio Romano, Salvatore Oliva, Fortunata Civitelli, Raffaele Iorio, Giovanni Di Nardo, Federica Ferrari, and Salvatore Cucchiara

Subjects
medicine.medical_specialty, Multicenter study, business.industry, Emergency medicine, Gastroenterology, medicine, Portal hypertension, Radiology, Nuclear Medicine and imaging, medicine.disease, business
Published
2014
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40. Paroxysmal ataxia and dysarthria in multiple sclerosis

Author

Anna Paola Batocchi, Fioravante Capone, Raffaele Iorio, and Domenico Plantone

Subjects
Adult, Male, congenital, hereditary, and neonatal diseases and abnormalities, Pediatrics, medicine.medical_specialty, Multiple Sclerosis, Paroxysmal dysarthria and ataxia, Audiology, Video imaging, Dysarthria, Physiology (medical), medicine, Humans, Multiple sclerosis, Paroxysmal dysarthria and ataxia, Relapse, Relapse, business.industry, Multiple sclerosis, food and beverages, General Medicine, Carbamazepine, medicine.disease, nervous system diseases, Neurology, Paroxysmal ataxia, Anticonvulsants, Ataxia, Surgery, Neurology (clinical), medicine.symptom, business, medicine.drug
Abstract

Paroxysmal ataxia and dysarthria are part of the spectrum of transient neurological disturbances that can be frequently encountered in multiple sclerosis (MS). Prompt recognition of these symptoms is important because they can be the only manifestation of a MS relapse and symptomatic therapy is often beneficial. We report a patient who developed paroxysmal ataxia and dysarthria, documented by video imaging, while he was recovering from a MS relapse. Treatment with carbamazepine resulted in the complete reversal of the paroxysmal ataxia and dysarthria.

Published
2014
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43. PO70 PRIMARY PROPHYLAXIS OF ESOPHAGEAL VARICES IN CHILDREN WITH PORTAL HYPERTENSION: PRELIMINARY DATA OF A RETROSPECTIVE ITALIAN MULTICENTER STUDY

Author

Corrado Romano, Raffaele Iorio, Federica Ferrari, Plinio Rossi, G. Di Nardo, Serena Arrigo, Salvatore Cucchiara, Arrigo Barabino, Paolo Gandullia, Saverio Mallardo, and Salvatore Oliva

Subjects
medicine.medical_specialty, Esophageal varices, Hepatology, Multicenter study, business.industry, General surgery, Gastroenterology, Medicine, Portal hypertension, business, medicine.disease
Published
2012
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44. A B3 Type-Thymoma in a 7-Year-Old Child with Myasthenia Gravis

Author

Raffaele Iorio, Amelia Evoli, Anna Paola Batocchi, and Libero Lauriola

Subjects
Male, Pulmonary and Respiratory Medicine, medicine.medical_specialty, Thymoma, business.industry, Thymus Neoplasms, Prognosis, medicine.disease, Combined Modality Therapy, Dermatology, Myasthenia gravis, Settore MED/26 - NEUROLOGIA, Oncology, Myasthenia Gravis, medicine, Humans, Child, business
Published
2012
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45. Sleep disorder associated with antibodies to IgLON5: parasomnia or agrypnia?

Author

Giovanni Frisullo, Anna Losurdo, Raffaele Iorio, Massimiliano Mirabella, and Giacomo Della Marca

Subjects
Male, Pediatrics, medicine.medical_specialty, Parasomnias, Cell Adhesion Molecules, Neuronal, agrypnia, Sleep spindle, medicine, Humans, Autoantibodies, Sleep Apnea, Obstructive, Sleep Stages, Sleep disorder, parasomnia, business.industry, Stupor, Sleep apnea, Dysautonomia, Chorea, Parasomnia, medicine.disease, Settore MED/26 - NEUROLOGIA, Female, Neurology (clinical), medicine.symptom, business
Abstract

We read with great interest the paper by Lidia Sabater and colleagues, who describe a novel neuroimmuno logical disease that is a complex, severe sleep disorder associated with antibodies to IgLON5. We have noted a clinically similar disorder in a single patient who had serum and CSF antibodies to the metabotropic GABAB receptor. Similar to the patients in Sabater and colleagues’ report, our patient developed a complex neurological syndrome at age 55 years that was characterised by diplopia, slight fl uctuating bilateral palpebral ptosis, and, later, dysphagia, dysphonia, tongue weakness, stridor, severe dysautonomia, and central hypoventilation, which required assisted ventilation during sleep. The outcome was also similar to the outcomes reported by Sabater and colleagues. Despite long periods of amelioration after plasma exchange and immunosuppressive treatment, the patient died suddenly during sleep 10 years after the onset of the disease. Sleep recordings showed a period of complete sleep loss in the acute phases of the disease. Agrypnia has been associated with neuroimmunological disorders, and particularly with Morvan’s chorea. Essentially, it is a complete loss of sleep, with the elimination of slowwave sleep and sleep spindles that are associated with sustained motor and autonomic activation. We believe that this peculiar sleep disorder described by Sabater and colleagues has several features in common with the syndrome described by Lugaresi and colleagues as agrypnia excitata: the presence of undiff erentiated nonrapid-eye movement sleep stages; the absence of the rapid-eye-movement atonia; the dysautonomia disorder; and the continuous motor activity during sleep. The fi nalistic movements described in Sabater and colleagues’ paper might be interpreted as episodes of oneiric stupor, which is probably an exclusive sign of agrypnia excitata and it is the recurrence of stereotyped gestures mimicking simple daily life activities. However, the patients had some episodes of N3 stage sleep, preserved K complexes slow-wave sleep, and spindles, which are not the typical fi ndings in agrypnia excitata. Nevertheless, in all disorders associated with agrypnia excitata, spindles, K complexes, and slow-wave sleep show a progressive reduction before disappearing. In our patient and other cases of agrypnia in our clinical experience, the sleep pattern and the other neurological symptoms had a relapsing-remitting course. In sleep recordings done serially and prolonged for several consecutive days, slow-wave sleep and spindles presented alternated periods of total suppression with periods of partial reduction, associated with the clinical fl uctuation. Therefore, we suggest that the disorder elegantly described by Sabater and colleagues, which was associated with anti-IgLON5 autoantibodies, could be regarded as a type of agrypnia excitata rather than as a parasomnia, similar to that seen in other neuroimmunological diseases.

Published
2014
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46. Hereditary fructose intolerance in children: Correlation between dietary intake of fructose and liver disease

Author

F. Chiatto, Simona Spadarella, Maria Immacolata Spagnuolo, Giancarlo Parenti, Raffaele Iorio, and Giovanna Puoti

Subjects
medicine.medical_specialty, Hepatology, business.industry, Hereditary fructose intolerance, Dietary intake, Gastroenterology, Fructose, medicine.disease, chemistry.chemical_compound, Liver disease, Endocrinology, chemistry, Biochemistry, Internal medicine, medicine, business
Published
2014
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47. Penicillamine-induced elastosis perforans serpiginosa: Is useful switching to zinc?

Author

Fabiola Di Dato, Maria Immacolata Spagnuolo, Maria Antonietta Tufano, Pietro Vajro, Giusy Ranucci, Raffaele Iorio, and Federica Leone

Subjects
medicine.medical_specialty, Hepatology, business.industry, Penicillamine, Gastroenterology, chemistry.chemical_element, Zinc, medicine.disease, Dermatology, chemistry, medicine, business, medicine.drug, Elastosis perforans serpiginosa
Published
2014
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48. Clinical experience with infantile hepatic hemangioendothelioma

Author

Maria Antonietta Tufano, Fabiola Di Dato, Gianfranco Vallone, Giusy Ranucci, R. Romano, Raffaele Iorio, Valentina Minicucci, Antonietta Giannattasio, and Francesco Nunziata

Subjects
Pathology, medicine.medical_specialty, Hepatology, business.industry, Gastroenterology, Medicine, business, Hepatic hemangioendothelioma
Published
2014
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