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1. Incontinentia pigmenti underlies thymic dysplasia, autoantibodies to type I IFNs, and viral diseases.

Author

Rosain, Jérémie, Le Voyer, Tom, Liu, Xian, Gervais, Adrian, Polivka, Laura, Cederholm, Axel, Berteloot, Laureline, Parent, Audrey, Pescatore, Alessandra, Spinosa, Ezia, Minic, Snezana, Kiszewski, Ana, Tsumura, Miyuki, Thibault, Chloé, Esnaola Azcoiti, Maria, Martinovic, Jelena, Philippot, Quentin, Khan, Taushif, Marchal, Astrid, Charmeteau-De Muylder, Bénédicte, Bizien, Lucy, Deswarte, Caroline, Hadjem, Lillia, Fauvarque, Marie-Odile, Dorgham, Karim, Eriksson, Daniel, Falcone, Emilia, Puel, Mathilde, Ünal, Sinem, Geraldo, Amyrath, Le Floch, Corentin, Li, Hailun, Rheault, Sylvie, Muti, Christine, Bobrie-Moyrand, Claire, Welfringer-Morin, Anne, Fuleihan, Ramsay, Lévy, Romain, Roelens, Marie, Gao, Liwei, Materna, Marie, Pellegrini, Silvia, Piemonti, Lorenzo, Catherinot, Emilie, Goffard, Jean-Christophe, Fekkar, Arnaud, Sacko-Sow, Aissata, Soudée, Camille, Boucherit, Soraya, Neehus, Anna-Lena, Has, Cristina, Hübner, Stefanie, Blanchard-Rohner, Géraldine, Amador-Borrero, Blanca, Utsumi, Takanori, Taniguchi, Maki, Tani, Hiroo, Izawa, Kazushi, Yasumi, Takahiro, Kanai, Sotaro, Migaud, Mélanie, Aubart, Mélodie, Lambert, Nathalie, Gorochov, Guy, Picard, Capucine, Soudais, Claire, LHonneur, Anne-Sophie, Rozenberg, Flore, Milner, Joshua, Zhang, Shen-Ying, Vabres, Pierre, Trpinac, Dusan, Marr, Nico, Boddaert, Nathalie, Desguerre, Isabelle, Pasparakis, Manolis, Miller, Corey, Poziomczyk, Cláudia, Abel, Laurent, Okada, Satoshi, Jouanguy, Emmanuelle, Cheynier, Rémi, Zhang, Qian, Cobat, Aurélie, Béziat, Vivien, Boisson, Bertrand, Steffann, Julie, Fusco, Francesca, Ursini, Matilde, Hadj-Rabia, Smail, Bodemer, Christine, Bustamante, Jacinta, Luche, Hervé, Puel, Anne, Courtois, Gilles, Bastard, Paul, Landegren, Nils, Anderson, Mark, and Casanova, Jean-Laurent

Subjects
Humans, Interferon Type I, Female, Autoantibodies, Thymus Gland, Child, Incontinentia Pigmenti, Child, Preschool, I-kappa B Kinase, Virus Diseases, Infant, Adult, Adolescent, Young Adult
Abstract

Human inborn errors of thymic T cell tolerance underlie the production of autoantibodies (auto-Abs) neutralizing type I IFNs, which predispose to severe viral diseases. We analyze 131 female patients with X-linked dominant incontinentia pigmenti (IP), heterozygous for loss-of-function (LOF) NEMO variants, from 99 kindreds in 10 countries. Forty-seven of these patients (36%) have auto-Abs neutralizing IFN-α and/or IFN-ω, a proportion 23 times higher than that for age-matched female controls. This proportion remains stable from the age of 6 years onward. On imaging, female patients with IP have a small, abnormally structured thymus. Auto-Abs against type I IFNs confer a predisposition to life-threatening viral diseases. By contrast, patients with IP lacking auto-Abs against type I IFNs are at no particular risk of viral disease. These results suggest that IP accelerates thymic involution, thereby underlying the production of auto-Abs neutralizing type I IFNs in at least a third of female patients with IP, predisposing them to life-threatening viral diseases.

Published
2024

2. Immunogenicity of a 2-Dose Regimen of Moderna mRNA Beta/Omicron BA.1 Bivalent Variant Vaccine Boost in a Randomized Clinical Trial.

Author

Rouphael, Nadine, Branche, Angela, Diemert, David, Falsey, Ann, Losada, Cecilia, Baden, Lindsey, Frey, Sharon, Whitaker, Jennifer, Little, Susan, Kamidani, Satoshi, Walter, Emmanuel, Novak, Richard, Rupp, Richard, Jackson, Lisa, Babu, Tara, Kottkamp, Angelica, Luetkemeyer, Anne, Immergluck, Lilly, Presti, Rachel, Bäcker, Martín, Winokur, Patricia, Mahgoub, Siham, Goepfert, Paul, Fusco, Dahlene, Atmar, Robert, Posavad, Christine, Netzl, Antonia, Smith, Derek, Telu, Kalyani, Mu, Jinjian, McQuarrie, Lisa, Makowski, Mat, Makhene, Mamodikoe, Crandon, Sonja, Montefiori, David, Roberts, Paul, and Beigel, John

Subjects
SARS-CoV-2, vaccine, variant, Adult, Humans, Vaccines, Combined, 2019-nCoV Vaccine mRNA-1273, Clinical Protocols, RNA, Messenger
Abstract

We compared the serologic responses of 1 dose versus 2 doses of a variant vaccine (Moderna mRNA-1273 Beta/Omicron BA.1 bivalent vaccine) in adults. A 2-dose boosting regimen with a variant vaccine did not increase the magnitude or the durability of the serological responses compared to a single variant vaccine boost.

Published
2023

3. Comparison of bivalent and monovalent SARS-CoV-2 variant vaccines: the phase 2 randomized open-label COVAIL trial.

Author

Branche, Angela, Rouphael, Nadine, Diemert, David, Falsey, Ann, Losada, Cecilia, Baden, Lindsey, Frey, Sharon, Whitaker, Jennifer, Anderson, Evan, Walter, Emmanuel, Novak, Richard, Rupp, Richard, Jackson, Lisa, Babu, Tara, Kottkamp, Angelica, Luetkemeyer, Anne, Immergluck, Lilly, Presti, Rachel, Bäcker, Martín, Winokur, Patricia, Mahgoub, Siham, Goepfert, Paul, Fusco, Dahlene, Malkin, Elissa, Bethony, Jeffrey, Walsh, Edward, Graciaa, Daniel, Samaha, Hady, Sherman, Amy, Walsh, Stephen, Abate, Getahun, Oikonomopoulou, Zacharoula, El Sahly, Hana, Kamidani, Satoshi, Smith, Michael, Ladner, Benjamin, Porterfield, Laura, Dunstan, Maya, Wald, Anna, Davis, Tamia, Atmar, Robert, Mulligan, Mark, Lyke, Kirsten, Posavad, Christine, Meagher, Megan, Stephens, David, Neuzil, Kathleen, Abebe, Kuleni, Hill, Heather, Albert, Jim, Telu, Kalyani, Mu, Jinjian, Lewis, Teri, Giebeig, Lisa, Eaton, Amanda, Netzl, Antonia, Wilks, Samuel, Türeli, Sina, Makhene, Mamodikoe, Crandon, Sonja, Montefiori, David, Makowski, Mat, Smith, Derek, Nayak, Seema, Roberts, Paul, Beigel, John, Little, Susan, and Martin, Thomas

Subjects
Humans, COVID-19 Vaccines, SARS-CoV-2, COVID-19, Broadly Neutralizing Antibodies
Abstract

Vaccine protection against severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection wanes over time, requiring updated boosters. In a phase 2, open-label, randomized clinical trial with sequentially enrolled stages at 22 US sites, we assessed safety and immunogenicity of a second boost with monovalent or bivalent variant vaccines from mRNA and protein-based platforms targeting wild-type, Beta, Delta and Omicron BA.1 spike antigens. The primary outcome was pseudovirus neutralization titers at 50% inhibitory dilution (ID50 titers) with 95% confidence intervals against different SARS-CoV-2 strains. The secondary outcome assessed safety by solicited local and systemic adverse events (AEs), unsolicited AEs, serious AEs and AEs of special interest. Boosting with prototype/wild-type vaccines produced numerically lower ID50 titers than any variant-containing vaccine against all variants. Conversely, boosting with a variant vaccine excluding prototype was not associated with decreased neutralization against D614G. Omicron BA.1 or Beta monovalent vaccines were nearly equivalent to Omicron BA.1 + prototype or Beta + prototype bivalent vaccines for neutralization of Beta, Omicron BA.1 and Omicron BA.4/5, although they were lower for contemporaneous Omicron subvariants. Safety was similar across arms and stages and comparable to previous reports. Our study shows that updated vaccines targeting Beta or Omicron BA.1 provide broadly crossprotective neutralizing antibody responses against diverse SARS-CoV-2 variants without sacrificing immunity to the ancestral strain. ClinicalTrials.gov registration: NCT05289037 .

Published
2023

4. Immunogenicity of the BA.1 and BA.4/BA.5 Severe Acute Respiratory Syndrome Coronavirus 2 Bivalent Boosts: Preliminary Results From the COVAIL Randomized Clinical Trial.

Author

Branche, Angela, Rouphael, Nadine, Losada, Cecilia, Baden, Lindsey, Anderson, Evan, Luetkemeyer, Anne, Diemert, David, Winokur, Patricia, Presti, Rachel, Kottkamp, Angelica, Falsey, Ann, Frey, Sharon, Rupp, Richard, Bäcker, Martín, Novak, Richard, Walter, Emmanuel, Jackson, Lisa, Little, Susan, Immergluck, Lilly, Mahgoub, Siham, Whitaker, Jennifer, Babu, Tara, Goepfert, Paul, Fusco, Dahlene, Atmar, Robert, Posavad, Christine, Netzl, Antonia, Smith, Derek, Telu, Kalyani, Mu, Jinjian, Makowski, Mat, Makhene, Mamodikoe, Crandon, Sonja, Montefiori, David, Roberts, Paul, and Beigel, John

Subjects
SARS-CoV-2, vaccine, variant, Humans, COVID-19, SARS-CoV-2, Antibodies, Neutralizing, Vaccines, Combined, Antibodies, Viral
Abstract

In a randomized clinical trial, we compare early neutralizing antibody responses after boosting with bivalent severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) messenger RNA (mRNA) vaccines based on either BA.1 or BA.4/BA.5 Omicron spike protein combined with wild-type spike. Responses against SARS-CoV-2 variants exhibited the greatest reduction in titers against currently circulating Omicron subvariants for both bivalent vaccines.

Published
2023

5. Emergence of an early SARS-CoV-2 epidemic in the United States.

Author

Zeller, Mark, Gangavarapu, Karthik, Anderson, Catelyn, Smither, Allison R, Vanchiere, John A, Rose, Rebecca, Snyder, Daniel J, Dudas, Gytis, Watts, Alexander, Matteson, Nathaniel L, Robles-Sikisaka, Refugio, Marshall, Maximilian, Feehan, Amy K, Sabino-Santos, Gilberto, Bell-Kareem, Antoinette R, Hughes, Laura D, Alkuzweny, Manar, Snarski, Patricia, Garcia-Diaz, Julia, Scott, Rona S, Melnik, Lilia I, Klitting, Raphaëlle, McGraw, Michelle, Belda-Ferre, Pedro, DeHoff, Peter, Sathe, Shashank, Marotz, Clarisse, Grubaugh, Nathan D, Nolan, David J, Drouin, Arnaud C, Genemaras, Kaylynn J, Chao, Karissa, Topol, Sarah, Spencer, Emily, Nicholson, Laura, Aigner, Stefan, Yeo, Gene W, Farnaes, Lauge, Hobbs, Charlotte A, Laurent, Louise C, Knight, Rob, Hodcroft, Emma B, Khan, Kamran, Fusco, Dahlene N, Cooper, Vaughn S, Lemey, Phillipe, Gardner, Lauren, Lamers, Susanna L, Kamil, Jeremy P, Garry, Robert F, Suchard, Marc A, and Andersen, Kristian G

Subjects
Humans, Risk Factors, Disease Outbreaks, Phylogeny, Travel, United States, Louisiana, Texas, Databases as Topic, Epidemics, COVID-19, SARS-CoV-2, genomic epidemiology, mobility, phylogenetics, viral emergence, viral sequencing, Emerging Infectious Diseases, Biodefense, Prevention, Vaccine Related, Lung, Developmental Biology, Biological Sciences, Medical and Health Sciences
Abstract

The emergence of the COVID-19 epidemic in the United States (U.S.) went largely undetected due to inadequate testing. New Orleans experienced one of the earliest and fastest accelerating outbreaks, coinciding with Mardi Gras. To gain insight into the emergence of SARS-CoV-2 in the U.S. and how large-scale events accelerate transmission, we sequenced SARS-CoV-2 genomes during the first wave of the COVID-19 epidemic in Louisiana. We show that SARS-CoV-2 in Louisiana had limited diversity compared to other U.S. states and that one introduction of SARS-CoV-2 led to almost all of the early transmission in Louisiana. By analyzing mobility and genomic data, we show that SARS-CoV-2 was already present in New Orleans before Mardi Gras, and the festival dramatically accelerated transmission. Our study provides an understanding of how superspreading during large-scale events played a key role during the early outbreak in the U.S. and can greatly accelerate epidemics.

Published
2021

6. Effectiveness and safety of oral anticoagulants in older adults with non-valvular atrial fibrillation and heart failure

Author

Amin, Alpesh, Reeves, Alessandra B Garcia, Li, Xiaoyan, Dhamane, Amol, Luo, Xuemei, Di Fusco, Manuela, Nadkarni, Anagha, Friend, Keith, Rosenblatt, Lisa, Mardekian, Jack, Pan, Xianying, Yuce, Huseyin, and Keshishian, Allison

Subjects
Biomedical and Clinical Sciences, Cardiovascular Medicine and Haematology, Heart Disease, Clinical Research, Cardiovascular, Hematology, Administration, Oral, Aged, Aged, 80 and over, Anticoagulants, Atrial Fibrillation, Dabigatran, Female, Heart Failure, Humans, Incidence, Kaplan-Meier Estimate, Male, Medicare, Patient Safety, Proportional Hazards Models, Pyrazoles, Pyridones, Retrospective Studies, Risk, Rivaroxaban, Treatment Outcome, United States, Warfarin, General Science & Technology
Abstract

Direct oral anticoagulants (DOACs) are at least as efficacious and safe as warfarin among non-valvular atrial fibrillation (NVAF) patients; limited evidence is available regarding NVAF patients with heart failure (HF). US Medicare enrollees with NVAF and HF initiating DOACs (apixaban, rivaroxaban, dabigatran) or warfarin were selected. Propensity score matching and Cox models were used to estimate the risk of stroke/systemic embolism (SE), major bleeding (MB), and major adverse cardiac events (MACE) comparing DOACs versus warfarin and DOACs versus DOACs. We identified 10,570 apixaban-warfarin, 4,297 dabigatran-warfarin, 15,712 rivaroxaban-warfarin, 4,263 apixaban-dabigatran, 10,477 apixaban-rivaroxaban, and 4,297 dabigatran-rivaroxaban matched pairs. Compared to warfarin, apixaban had lower rates of stroke/SE (hazard ratio = 0.64, 95% confidence interval = 0.48-0.85), MB (hazard ratio = 0.66, 0.58-0.76), and MACE (hazard ratio = 0.73,0.67-0.79); dabigatran and rivaroxaban had lower rates of MACE (hazard ratio = 0.87,0.77-0.99; hazard ratio = 0.84, 0.79-0.89, respectively). Rivaroxaban had a lower stroke/SE rate (hazard ratio = 0.65, 0.52-0.81) and higher MB rate (hazard ratio = 1.18, 1.08-1.30) versus warfarin. Compared to dabigatran and rivaroxaban, apixaban had lower MB (hazard ratio = 0.71, 0.57-0.89; hazard ratio = 0.55, 0.49-0.63) and MACE rates (hazard ratio = 0.80, 0.69-0.93; hazard ratio = 0.86, 0.79-0.94), respectively. All DOACs had lower MACE rates versus warfarin; differences were observed in stroke/SE and MB. Our findings provide insights about OAC therapy among NVAF patients with HF.

Published
2019

7. Collective motion conceals fitness differences in crowded cellular populations

Author

Kayser, Jona, Schreck, Carl F, Gralka, Matti, Fusco, Diana, and Hallatschek, Oskar

Subjects
Biochemistry and Cell Biology, Biological Sciences, Genetics, Generic health relevance, Biofilms, Biological Evolution, Biomechanical Phenomena, Humans, Microbiota, Models, Biological, Mutation, Saccharomyces cerevisiae, Ecology, Evolutionary biology, Environmental management
Abstract

Many cellular populations are tightly packed, such as microbial colonies and biofilms, or tissues and tumours in multicellular organisms. The movement of one cell in these crowded assemblages requires motion of others, so that cell displacements are correlated over many cell diameters. Whenever movement is important for survival or growth, these correlated rearrangements could couple the evolutionary fate of different lineages. However, little is known about the interplay between mechanical forces and evolution in dense cellular populations. Here, by tracking slower-growing clones at the expanding edge of yeast colonies, we show that the collective motion of cells prevents costly mutations from being weeded out rapidly. Joint pushing by neighbouring cells generates correlated movements that suppress the differential displacements required for selection to act. This mechanical screening of fitness differences allows slower-growing mutants to leave more descendants than expected under non-mechanical models, thereby increasing their chance for evolutionary rescue. Our work suggests that, in crowded populations, cells cooperate with surrounding neighbours through inevitable mechanical interactions. This effect has to be considered when predicting evolutionary outcomes, such as the emergence of drug resistance or cancer evolution.

Published
2019

8. Collective motion conceals fitness differences in crowded cellular populations.

Author

Kayser, Jona, Schreck, Carl F, Gralka, Matti, Fusco, Diana, and Hallatschek, Oskar

Subjects
Humans, Biofilms, Saccharomyces cerevisiae, Mutation, Models, Biological, Biological Evolution, Biomechanical Phenomena, Microbiota
Abstract

Many cellular populations are tightly packed, such as microbial colonies and biofilms, or tissues and tumours in multicellular organisms. The movement of one cell in these crowded assemblages requires motion of others, so that cell displacements are correlated over many cell diameters. Whenever movement is important for survival or growth, these correlated rearrangements could couple the evolutionary fate of different lineages. However, little is known about the interplay between mechanical forces and evolution in dense cellular populations. Here, by tracking slower-growing clones at the expanding edge of yeast colonies, we show that the collective motion of cells prevents costly mutations from being weeded out rapidly. Joint pushing by neighbouring cells generates correlated movements that suppress the differential displacements required for selection to act. This mechanical screening of fitness differences allows slower-growing mutants to leave more descendants than expected under non-mechanical models, thereby increasing their chance for evolutionary rescue. Our work suggests that, in crowded populations, cells cooperate with surrounding neighbours through inevitable mechanical interactions. This effect has to be considered when predicting evolutionary outcomes, such as the emergence of drug resistance or cancer evolution.

Published
2018

9. Multifunctional Pan-ebolavirus Antibody Recognizes a Site of Broad Vulnerability on the Ebolavirus Glycoprotein

Author

Gilchuk, Pavlo, Kuzmina, Natalia, Ilinykh, Philipp A, Huang, Kai, Gunn, Bronwyn M, Bryan, Aubrey, Davidson, Edgar, Doranz, Benjamin J, Turner, Hannah L, Fusco, Marnie L, Bramble, Matthew S, Hoff, Nicole A, Binshtein, Elad, Kose, Nurgun, Flyak, Andrew I, Flinko, Robin, Orlandi, Chiara, Carnahan, Robert, Parrish, Erica H, Sevy, Alexander M, Bombardi, Robin G, Singh, Prashant K, Mukadi, Patrick, Muyembe-Tamfum, Jean Jacques, Ohi, Melanie D, Saphire, Erica Ollmann, Lewis, George K, Alter, Galit, Ward, Andrew B, Rimoin, Anne W, Bukreyev, Alexander, and Crowe, James E

Subjects
Medical Microbiology, Biomedical and Clinical Sciences, Immunology, Orphan Drug, Infectious Diseases, Immunization, Prevention, Biodefense, Biotechnology, Rare Diseases, Emerging Infectious Diseases, Good Health and Well Being, 3T3 Cells, Adult, Animals, Antibodies, Monoclonal, Antibodies, Neutralizing, Antibodies, Viral, CHO Cells, Cell Line, Chlorocebus aethiops, Cricetulus, Disease Models, Animal, Drosophila, Ebolavirus, Female, Ferrets, Glycoproteins, Guinea Pigs, Hemorrhagic Fever, Ebola, Humans, Immunoglobulin G, Jurkat Cells, Male, Mice, Mice, Inbred BALB C, Mice, Knockout, THP-1 Cells, Vero Cells, Ebola hemorrhagic fever, cross protection, ebolavirus, epitope mapping, epitopes, glycoproteins, heterologous immunity, monoclonal antibodies, neutralizing antibodies, viral antibodies
Abstract

Ebolaviruses cause severe disease in humans, and identification of monoclonal antibodies (mAbs) that are effective against multiple ebolaviruses are important for therapeutics development. Here we describe a distinct class of broadly neutralizing human mAbs with protective capacity against three ebolaviruses infectious for humans: Ebola (EBOV), Sudan (SUDV), and Bundibugyo (BDBV) viruses. We isolated mAbs from human survivors of ebolavirus disease and identified a potent mAb, EBOV-520, which bound to an epitope in the glycoprotein (GP) base region. EBOV-520 efficiently neutralized EBOV, BDBV, and SUDV and also showed protective capacity in relevant animal models of these infections. EBOV-520 mediated protection principally by direct virus neutralization and exhibited multifunctional properties. This study identified a potent naturally occurring mAb and defined key features of the human antibody response that may contribute to broad protection. This multifunctional mAb and related clones are promising candidates for development as broadly protective pan-ebolavirus therapeutic molecules.

Published
2018

10. Insights into the anticancer properties of the first antimicrobial peptide from Archaea

Author

Gaglione, Rosa, Pirone, Luciano, Farina, Biancamaria, Fusco, Salvatore, Smaldone, Giovanni, Aulitto, Martina, Dell'Olmo, Eliana, Roscetto, Emanuela, Del Gatto, Annarita, Fattorusso, Roberto, Notomista, Eugenio, Zaccaro, Laura, Arciello, Angela, Pedone, Emilia, and Contursi, Patrizia

Subjects
Biochemistry and Cell Biology, Biological Sciences, Cancer, Aetiology, 2.1 Biological and endogenous factors, Generic health relevance, Animals, Antimicrobial Cationic Peptides, Antineoplastic Agents, BALB 3T3 Cells, Cell Death, Cell Membrane, Circular Dichroism, Humans, Mice, Nuclear Magnetic Resonance, Biomolecular, Protein Conformation, Sulfolobus, Antimicrobial peptide, Anticancer peptide, Archaea, Peptide-membrane interactions, Pharmacology and Pharmaceutical Sciences, Biochemistry & Molecular Biology, Biochemistry and cell biology
Abstract

BackgroundThe peptide VLL-28, identified in the sequence of an archaeal protein, the transcription factor Stf76 from Sulfolobus islandicus, was previously identified and characterized as an antimicrobial peptide, possessing a broad-spectrum antibacterial activity.MethodsThrough a combined approach of NMR and Circular Dichroism spectroscopy, Dynamic Light Scattering, confocal microscopy and cell viability assays, the interaction of VLL-28 with the membranes of both parental and malignant cell lines has been characterized and peptide mechanism of action has been studied.ResultsIt is here demonstrated that VLL-28 selectively exerts cytotoxic activity against murine and human tumor cells. By means of structural methodologies, VLL-28 interaction with the membranes has been proven and the binding residues have been identified. Confocal microscopy data show that VLL-28 is internalized only into tumor cells. Finally, it is shown that cell death is mainly caused by a time-dependent activation of apoptotic pathways.ConclusionsVLL-28, deriving from the archaeal kingdom, is here found to be endowed with selective cytotoxic activity towards both murine and human cancer cells and consequently can be classified as an ACP.General significanceVLL-28 represents the first ACP identified in an archaeal microorganism, exerting a trans-kingdom activity.

Published
2017

11. Human-started wildfires expand the fire niche across the United States

Author

Balch, Jennifer K, Bradley, Bethany A, Abatzoglou, John T, Nagy, R Chelsea, Fusco, Emily J, and Mahood, Adam L

Subjects
Geography, Human Activities, Humans, Seasons, Spatio-Temporal Analysis, United States, Wildfires, anthropogenic wildfires, fire starts, ignitions, modern fire regimes, wildfire causes
Abstract

The economic and ecological costs of wildfire in the United States have risen substantially in recent decades. Although climate change has likely enabled a portion of the increase in wildfire activity, the direct role of people in increasing wildfire activity has been largely overlooked. We evaluate over 1.5 million government records of wildfires that had to be extinguished or managed by state or federal agencies from 1992 to 2012, and examined geographic and seasonal extents of human-ignited wildfires relative to lightning-ignited wildfires. Humans have vastly expanded the spatial and seasonal "fire niche" in the coterminous United States, accounting for 84% of all wildfires and 44% of total area burned. During the 21-y time period, the human-caused fire season was three times longer than the lightning-caused fire season and added an average of 40,000 wildfires per year across the United States. Human-started wildfires disproportionally occurred where fuel moisture was higher than lightning-started fires, thereby helping expand the geographic and seasonal niche of wildfire. Human-started wildfires were dominant (>80% of ignitions) in over 5.1 million km2, the vast majority of the United States, whereas lightning-started fires were dominant in only 0.7 million km2, primarily in sparsely populated areas of the mountainous western United States. Ignitions caused by human activities are a substantial driver of overall fire risk to ecosystems and economies. Actions to raise awareness and increase management in regions prone to human-started wildfires should be a focus of United States policy to reduce fire risk and associated hazards.

Published
2017

12. Telementoring for communication between residents and faculty physicians: Results from a survey on attitudes and perceptions in an Academic Tertiary Urology Referral Department in Italy

Author

Vincenzo Mirone, Massimiliano Creta, Marco Capece, Giuseppe Celentano, Gianluigi Califano, Claudia Collà Ruvolo, Lorenzo Spirito, Giovanni Maria Fusco, Luigi Cirillo, Nicola Longo, Ferdinando Fusco, Claudia Mirone, Roberto La Rocca, Luigi Napolitano, Mirone, Vincenzo, Creta, Massimiliano, Capece, Marco, Celentano, Giuseppe, Califano, Gianluigi, Collà Ruvolo, Claudia, Spirito, Lorenzo, Fusco, Giovanni Maria, Cirillo, Luigi, Longo, Nicola, Fusco, Ferdinando, Mirone, Claudia, La Rocca, Roberto, and Napolitano, Luigi

Subjects
Pandemic, Patient-related care, Communication, Urology, Bladder cancer, Internship and Residency, Faculty, Telemedicine, Diseases of the genitourinary system. Urology, Attitude, Physician, Physicians, Surveys and Questionnaires, Surveys and Questionnaire, Humans, RC870-923, Telementoring, Referral and Consultation, Human
Abstract

Introduction: Telemedicine has been adopted successfully in various urological scenarios. The aim of the present study was to explore attitudes and perceptions by urology residents toward the use of telementoring in the context of residents-faculty physicians communication for patient-related care. Methods: An online survey consisting of 19 multiple choice questions was designed including three sections: respondents’ demographics, attitudes and perceptions towards the use of telementoring. Invitations to participate in this anonymous survey were e-mailed to urology residents at University of Naples Federico II.Results: In total 60 responses were received (participation rate 86%). The frequency of telementoring use was described as occasional, frequent, very frequent, and rare by 51,3%, 41.0%, 5,1%, and 2,6% of respondents, respectively. WhatsApp messenger was used by 89.5% of respondents and photos were the most common type of media content shared (73.7%). Most of respondents declared a moderate and a strong agreement with respect to the utility of telementoring in improving the communication in relation to the interpretation of clinical, radiological, endoscopic, and functional findings. Overall, 78% of participants individuated risks of information flow distortions and misinterpretations as the major limit of telementoring. Conclusions: The use of telementoring is widespread and perceived as useful by urology residents in the context of residentsfaculty physicians communication in multiple settings of patientrelated care.

Published
2021

13. Prevalence and features of delirium in older patients admitted to rehabilitation facilities

Author

Sidoli, Chiara, Zambon, Antonella, Tassistro, Elena, Rossi, Emanuela, Mossello, Enrico, Inzitari, Marco, Cherubini, Antonio, Marengoni, Alessandra, Morandi, Alessandro, Bellelli, Giuseppe, Tarasconi, A, Sella, M, Paternò, G, Faggian, G, Lucarelli, C, De Grazia, N, Alberto, C, Porcella, L, Nardiello, I, Chimenti, E, Zeni, M, Romairone, E, Minaglia, C, Ceccotti, C, Guerra, G, Mantovani, G, Monacelli, F, Candiani, T, Santolini, F, Rosso, M, Bono, V, Sibilla, S, Dal Santo, P, Ceci, M, Barone, P, Schirinzi, T, Formenti, A, Nastasi, G, Isaia, G, Gonella, D, Battuello, A, Casson, S, Calvani, D, Boni, F, Ciaccio, A, Rosa, R, Sanna, G, Manfredini, S, Cortese, L, Rizzo, M, Prestano, R, Greco, A, Lauriola, M, Gelosa, G, Piras, V, Arena, M, Cosenza, D, Bellomo, A, Lamontagna, M, Gabbani, L, Lambertucci, L, Perego, S, Parati, G, Basile, G, Gallina, V, Pilone, G, Giudice, C, Pietrogrande, L, Mosca, M, Corazzin, I, Rossi, P, Nunziata, V, D’Amico, F, Grippa, A, Giardini, S, Barucci, R, Cossu, A, Fiorin, L, Distefano, M, Lunardelli, M, Brunori, M, Ruffini, I, Abraham, E, Varutti, A, Fabbro, E, Catalano, A, Martino, G, Leotta, D, Marchet, A, Dell’Aquila, G, Scrimieri, A, Davoli, M, Casella, M, Cartei, A, Polidori, G, Brischetto, D, Motta, S, Saponara, R, Perrone, P, Russo, G, Del, D, Car, C, Pirina, T, Franzoni, S, Cotroneo, A, Ghiggia, F, Volpi, G, Menichetti, C, Bo, M, Panico, A, Calogero, P, Corvalli, G, Mauri, M, Lupia, E, Manfredini, R, Fabbian, F, March, A, Pedrotti, M, Veronesi, M, Strocchi, E, Borghi, C, Bianchetti, A, Crucitti, A, Difrancesco, V, Fontana, G, Geriatria, A, Bonanni, L, Barbone, F, Serrati, C, Ballardini, G, Simoncelli, M, Ceschia, G, Scarpa, C, Brugiolo, R, Fusco, S, Ciarambino, T, Biagini, C, Tonon, E, Porta, M, Venuti, D, Delsette, M, Poeta, M, Barbagallo, G, Trovato, G, Delitala, A, Arosio, P, Reggiani, F, Zuliani, G, Ortolani, B, Mussio, E, Girardi, A, Coin, A, Ruotolo, G, Castagna, A, Masina, M, Cimino, R, Pinciaroli, A, Tripodi, G, Cassadonte, F, Vatrano, M, Scaglione, L, Fogliacco, P, Muzzuilini, C, Romano, F, Padovani, A, Rozzini, L, Cagnin, A, Fragiacomo, F, Desideri, G, Liberatore, E, Bruni, A, Orsitto, G, Franco, M, Bonfrate, L, Bonetto, M, Pizio, N, Magnani, G, Cecchetti, G, Longo, A, Bubba, V, Marinan, L, Cotelli, M, Turla, M, Sessa, M, Abruzzi, L, Castoldi, G, Lovetere, D, Musacchio, C, Novello, M, Cavarape, A, Bini, A, Leonardi, A, Seneci, F, Grimaldi, W, Fimognari, F, Bambar, V, Saitta, A, Corica, F, Braga, M, Servi, Null, Ettorre, E, Camellini Bellelli, C G, Annoni, G, Marengoni, A, Crescenzo, A, Noro, G, Turco, R, Ponzetto, M, Giuseppe, L, Mazzei, B, Maiuri, G, Costaggiu, D, Damato, R, Formilan, M, Patrizia, G, Santuar, L, Gallucci, M, Paragona, M, Bini, P, Modica, D, Abati, C, Clerici, M, Barbera, I, Nigroimperiale, F, Manni, A, Votino, C, Castiglioni, C, Di, M, Degl’Innocenti, M, Moscatelli, G, Guerini, S, Casini, C, Dini, D, Denotariis, S, Bonometti, F, Paolillo, C, Riccardi, A, Tiozzo, A, Samysalamafahmy, A, Dibari, M, Vanni, S, Scarpa, A, Zara, D, Ranieri, P, Alessandro, M, Di, F, Pezzoni, D, Platto, C, D’Ambrosio, V, Ivaldi, C, Milia, P, Desalvo, F, Solaro, C, Strazzacappa, M, Cazzadori, M, Grasso, M, Troisi, E, Guerini, V, Bernardini, B, Corsini, C, Boffelli, S, Filippi, A, Delpin, K, Faraci, B, Bertoletti, E, Vannucci, M, Crippa, P, Malighetti, A, Caltagirone, C, Disant, S, Bettini, D, Maltese, F, Abruzzese, G, Cosimo, D, Azzini, M, Colombo, M, Procino, G, Fascendini, S, Barocco, F, Del, P, Mazzone, A, Cottino, M, Vezzadini, G, Avanzi, S, Brambilla, C, Orini, S, Sgrilli, F, Mello, A, Lombardi Muti, L E, Dijk, B, Fenu, S, Pes, C, Gareri, P, Passamonte, M, Rigo, R, Locusta, L, Caser, L, Rosso, G, Cesarini, S, Cozzi, R, Santini, C, Carbone, P, Cazzaniga, I, Lovati, R, Cantoni, A, Ranzani, P, Barra, D, Pompilio, G, Dimori, S, Cernesi, S, Riccò, C, Piazzolla, F, Capittini, E, Rota, C, Gottardi, F, Merla, L, Barelli, A, Millul, A, De, G, Morrone, G, Bigolari, M, Macchi, M, Zambon, F, Pizzorni, C, Dicasaleto, G, Menculini, G, Marcacci, M, Catanese, G, Sprini, D, Dicasalet, T, Bocci, M, Borga, S, Caironi, P, Cat, C, Cingolani, E, Avalli, L, Greco, G, Citerio, G, Gandini, L, Cornara, G, Lerda, R, Brazzi, L, Simeone, F, Caciorgna, M, Alampi, D, Francesconi, S, Beck, E, Antonini, B, Vettoretto, K, Meggiolaro, M, Garofalo, E, Notaro, S, Varutti, R, Bassi, F, Mistraletti, G, Marino, A, Rona, R, Rondelli, E, Riva, I, Cortegiani, A, Pistidda, L, D’Andrea, R, Querci, L, Gnesin, P, Todeschini, M, Lugano, M, Castelli, G, Ortolani, M, Cotoia, A, Maggiore, S, Ditizio, L, Graziani, R, Testa, I, Ferretti, E, Castioni, C, Lombardi, F, Caserta, R, Pasqua, M, Simoncini, S, Baccarini, F, Rispoli, M, Grossi, F, Cancelliere, L, Carnelli, M, Puccini, F, Biancofiore, G, Siniscalchi, A, Laici, C, Mossello, E, Torrini, M, Pasetti, G, Palmese, S, Oggioni, R, Mangani, V, Pini, S, Martelli, M, Rigo, E, Zuccalà, F, Cherri, A, Spina, R, Calamai, I, Petrucci, N, Caicedo, A, Ferri, F, Gritti, P, Brienza, N, Fonnesu, R, Dessena, M, Fullin, G, Saggioro, D, Sidoli, Chiara, Zambon, Antonella, Tassistro, Elena, Rossi, Emanuela, Mossello, Enrico, Inzitari, Marco, Cherubini, Antonio, Marengoni, Alessandra, Morandi, Alessandro, Bellelli, Giuseppe, A Tarasconi, M Sella, G Paternò, G Faggian, C Lucarelli, N De Grazia, C Alberto, L Porcella, I Nardiello, E Chimenti, M Zeni, E Romairone, C Minaglia, C Ceccotti, G Guerra, G Mantovani, F Monacelli, C Minaglia, T Candiani, F Santolini, C Minaglia, M Rosso, V Bono, S Sibilla, P Dal Santo, M Ceci, P Barone, T Schirinzi, A Formenti, G Nastasi, G Isaia, D Gonella, A Battuello, S Casson, D Calvani, F Boni, A Ciaccio, R Rosa, G Sanna, S Manfredini, L Cortese, M Rizzo, R Prestano, A Greco, M Lauriola, G Gelosa, V Piras, M Arena, D Cosenza, A Bellomo, M LaMontagna, L Gabbani, L Lambertucci, S Perego, G Parati, G Basile, V Gallina, G Pilone, C Giudice, L Pietrogrande, M Mosca, I Corazzin, P Rossi, V Nunziata, F D’Amico, A Grippa, S Giardini, R Barucci, A Cossu, L Fiorin, M Arena , M Distefano, M Lunardelli, M Brunori, I Ruffini, E Abraham, A Varutti, E Fabbro, A Catalano, G Martino, D Leotta, A Marchet, G Dell’Aquila, A Scrimieri, M Davoli, M Casella, A Cartei, G Polidori, G Basile, D Brischetto, S Motta, R Saponara, P Perrone, G Russo, D Del, C Car, T Pirina, S Franzoni, A Cotroneo, F Ghiggia, G Volpi, C Menichetti, M Bo, A Panico, P Calogero, G Corvalli, M Mauri, E Lupia, R Manfredini, F Fabbian, A March, M Pedrotti, M Veronesi, E Strocchi, C Borghi, A Bianchetti, A Crucitti, V DiFrancesco, G Fontana, A Geriatria, L Bonanni, F Barbone, C Serrati, G Ballardini, M Simoncelli, G Ceschia, C Scarpa, R Brugiolo, S Fusco, T Ciarambino, C Biagini, E Tonon, M Porta , D Venuti, M DelSette, M Poeta, G Barbagallo, G Trovato, A Delitala, P Arosio, F Reggiani, G Zuliani, B Ortolani, E Mussio, A Girardi, A Coin, G Ruotolo, A Castagna, M Masina, R Cimino, A Pinciaroli, G Tripodi, F Cassadonte, M Vatrano, L Scaglione, P Fogliacco, C Muzzuilini, F Romano, A Padovani, L Rozzini, A Cagnin, F Fragiacomo, G Desideri, E Liberatore, A Bruni, G Orsitto, M Franco, L Bonfrate, M Bonetto, N Pizio, G Magnani, G Cecchetti, A Longo, V Bubba, L Marinan, M Cotelli, M Turla, M Brunori, M Sessa, L Abruzzi, G Castoldi, D LoVetere, C Musacchio, M Novello, A Cavarape, A Bini, A Leonardi, F Seneci, W Grimaldi, F Seneci, F Fimognari, V Bambar, A Saitta, F Corica, M Braga, Servi, E Ettorre , C G Camellini Bellelli, G Annoni, A Marengoni, A Bruni, A Crescenzo, G Noro, R Turco, M Ponzetto, L Giuseppe, B Mazzei, G Maiuri, D Costaggiu, R Damato, E Fabbro, M Formilan, G Patrizia, L Santuar , M Gallucci, C Minaglia, M Paragona, P Bini, D Modica, C Abati, M Clerici, I Barbera, F NigroImperiale, A Manni, C Votino, C Castiglioni, M Di, M Degl’Innocenti, G Moscatelli, S Guerini, C Casini, D Dini, S DeNotariis, F Bonometti, C Paolillo, A Riccardi, A Tiozzo, A SamySalamaFahmy, A Riccardi, C Paolillo, M DiBari, S Vanni, A Scarpa, D Zara, P Ranieri, M Alessandro, P Calogero, G Corvalli, F Di, D Pezzoni, C Platto, V D’Ambrosio, C Ivaldi, P Milia, F DeSalvo, C Solaro, M Strazzacappa, M Bo, A Panico, M Cazzadori, M Bonetto, M Grasso, E Troisi, G Magnani, G Cecchetti, V Guerini, B Bernardini, C Corsini, S Boffelli, A Filippi, K Delpin, B Faraci, E Bertoletti, M Vannucci, P Crippa, A Malighetti, C Caltagirone, S DiSant, D Bettini, F Maltese, M Formilan, G Abruzzese, C Minaglia, D Cosimo, M Azzini, M Cazzadori, M Colombo, G Procino, S Fascendini, F Barocco, P Del, F D’Amico, A Grippa , A Mazzone, M Cottino, G Vezzadini, S Avanzi, C Brambilla, S Orini, F Sgrilli, A Mello, L E Lombardi Muti, B Dijk , S Fenu, C Pes, P Gareri, A Castagna, M Passamonte, R Rigo, L Locusta, L Caser, G Rosso, S Cesarini, R Cozzi, C Santini, P Carbone, I Cazzaniga, R Lovati, A Cantoni, P Ranzani, D Barra, G Pompilio, S Dimori, S Cernesi, C Riccò, F Piazzolla, E Capittini, C Rota, F Gottardi, L Merla, A Barelli, A Millul , G De, G Morrone, M Bigolari, C Minaglia, M Macchi, F Zambon, F D’Amico, F D’Amico, C Pizzorni, G DiCasaleto, G Menculini, M Marcacci, G Catanese, D Sprini, T DiCasalet, M Bocci, S Borga, P Caironi, C Cat, E Cingolani, L Avalli, G Greco, G Citerio, L Gandini, G Cornara, R Lerda, L Brazzi, F Simeone, M Caciorgna, D Alampi, S Francesconi, E Beck, B Antonini, K Vettoretto, M Meggiolaro, E Garofalo, A Bruni, S Notaro, R Varutti, F Bassi, G Mistraletti, A Marino, R Rona, E Rondelli, I Riva, A Cortegiani, L Pistidda, R D’Andrea, L Querci, P Gnesin, M Todeschini, M Lugano, G Castelli, M Ortolani, A Cotoia, S Maggiore, L DiTizio, R Graziani, I Testa, E Ferretti, C Castioni, F Lombardi, R Caserta, M Pasqua, S Simoncini, F Baccarini, M Rispoli, F Grossi, L Cancelliere, M Carnelli, F Puccini, G Biancofiore, A Siniscalchi, C Laici, E Mossello, M Torrini, G Pasetti, S Palmese, R Oggioni, V Mangani, S Pini, M Martelli, E Rigo, F Zuccalà , A Cherri, R Spina, I Calamai, N Petrucci, A Caicedo, F Ferri, P Gritti, N Brienza, R Fonnesu, M Dessena, G Fullin & D Saggioro, VU University medical center, Sidoli, C, Zambon, A, Tassistro, E, Rossi, E, Mossello, E, Inzitari, M, Cherubini, A, Marengoni, A, Morandi, A, Bellelli, G, Tarasconi, A, Sella, M, Paterno, G, Faggian, G, Lucarelli, C, De Grazia, N, Alberto, C, Porcella, L, Nardiello, I, Chimenti, E, Zeni, M, Romairone, E, Minaglia, C, Ceccotti, C, Guerra, G, Mantovani, G, Monacelli, F, Candiani, T, Santolini, F, Rosso, M, Bono, V, Sibilla, S, Dal Santo, P, Ceci, M, Barone, P, Schirinzi, T, Formenti, A, Nastasi, G, Isaia, G, Gonella, D, Battuello, A, Casson, S, Calvani, D, Boni, F, Ciaccio, A, Rosa, R, Sanna, G, Manfredini, S, Cortese, L, Rizzo, M, Prestano, R, Greco, A, Lauriola, M, Gelosa, G, Piras, V, Arena, M, Cosenza, D, Bellomo, A, Lamontagna, M, Gabbani, L, Lambertucci, L, Perego, S, Parati, G, Basile, G, Gallina, V, Pilone, G, Giudice, C, Pietrogrande, L, Mosca, M, Corazzin, I, Rossi, P, Nunziata, V, D'Amico, F, Grippa, A, Giardini, S, Barucci, R, Cossu, A, Fiorin, L, Distefano, M, Lunardelli, M, Brunori, M, Ruffini, I, Abraham, E, Varutti, A, Fabbro, E, Catalano, A, Martino, G, Leotta, D, Marchet, A, Dell'Aquila, G, Scrimieri, A, Davoli, M, Casella, M, Cartei, A, Polidori, G, Brischetto, D, Motta, S, Saponara, R, Perrone, P, Russo, G, Del, D, Car, C, Pirina, T, Franzoni, S, Cotroneo, A, Ghiggia, F, Volpi, G, Menichetti, C, Bo, M, Panico, A, Calogero, P, Corvalli, G, Mauri, M, Lupia, E, Manfredini, R, Fabbian, F, March, A, Pedrotti, M, Veronesi, M, Strocchi, E, Borghi, C, Bianchetti, A, Crucitti, A, Difrancesco, V, Fontana, G, Geriatria, A, Bonanni, L, Barbone, F, Serrati, C, Ballardini, G, Simoncelli, M, Ceschia, G, Scarpa, C, Brugiolo, R, Fusco, S, Ciarambino, T, Biagini, C, Tonon, E, Porta, M, Venuti, D, Delsette, M, Poeta, M, Barbagallo, G, Trovato, G, Delitala, A, Arosio, P, Reggiani, F, Zuliani, G, Ortolani, B, Mussio, E, Girardi, A, Coin, A, Ruotolo, G, Castagna, A, Masina, M, Cimino, R, Pinciaroli, A, Tripodi, G, Cassadonte, F, Vatrano, M, Scaglione, L, Fogliacco, P, Muzzuilini, C, Romano, F, Padovani, A, Rozzini, L, Cagnin, A, Fragiacomo, F, Desideri, G, Liberatore, E, Bruni, A, Orsitto, G, Franco, M, Bonfrate, L, Bonetto, M, Pizio, N, Magnani, G, Cecchetti, G, Longo, A, Bubba, V, Marinan, L, Cotelli, M, Turla, M, Sessa, M, Abruzzi, L, Castoldi, G, Lovetere, D, Musacchio, C, Novello, M, Cavarape, A, Bini, A, Leonardi, A, Seneci, F, Grimaldi, W, Fimognari, F, Bambar, V, Saitta, A, Corica, F, Braga, M, Servi, Ettorre, E, Camellini Bellelli, C, Annoni, G, Crescenzo, A, Noro, G, Turco, R, Ponzetto, M, Giuseppe, L, Mazzei, B, Maiuri, G, Costaggiu, D, Damato, R, Formilan, M, Patrizia, G, Santuar, L, Gallucci, M, Paragona, M, Bini, P, Modica, D, Abati, C, Clerici, M, Barbera, I, Nigroimperiale, F, Manni, A, Votino, C, Castiglioni, C, Di, M, Degl'Innocenti, M, Moscatelli, G, Guerini, S, Casini, C, Dini, D, Denotariis, S, Bonometti, F, Paolillo, C, Riccardi, A, Tiozzo, A, Samysalamafahmy, A, Dibari, M, Vanni, S, Scarpa, A, Zara, D, Ranieri, P, Alessandro, M, Di, F, Pezzoni, D, Platto, C, D'Ambrosio, V, Ivaldi, C, Milia, P, Desalvo, F, Solaro, C, Strazzacappa, M, Cazzadori, M, Grasso, M, Troisi, E, Guerini, V, Bernardini, B, Corsini, C, Boffelli, S, Filippi, A, Delpin, K, Faraci, B, Bertoletti, E, Vannucci, M, Crippa, P, Malighetti, A, Caltagirone, C, Disant, S, Bettini, D, Maltese, F, Abruzzese, G, Cosimo, D, Azzini, M, Colombo, M, Procino, G, Fascendini, S, Barocco, F, Del, P, Mazzone, A, Cottino, M, Vezzadini, G, Avanzi, S, Brambilla, C, Orini, S, Sgrilli, F, Mello, A, Lombardi Muti, L, Dijk, B, Fenu, S, Pes, C, Gareri, P, Passamonte, M, Rigo, R, Locusta, L, Caser, L, Rosso, G, Cesarini, S, Cozzi, R, Santini, C, Carbone, P, Cazzaniga, I, Lovati, R, Cantoni, A, Ranzani, P, Barra, D, Pompilio, G, Dimori, S, Cernesi, S, Ricco, C, Piazzolla, F, Capittini, E, Rota, C, Gottardi, F, Merla, L, Barelli, A, Millul, A, De, G, Morrone, G, Bigolari, M, Macchi, M, Zambon, F, Pizzorni, C, Dicasaleto, G, Menculini, G, Marcacci, M, Catanese, G, Sprini, D, Dicasalet, T, Bocci, M, Borga, S, Caironi, P, Cat, C, Cingolani, E, Avalli, L, Greco, G, Citerio, G, Gandini, L, Cornara, G, Lerda, R, Brazzi, L, Simeone, F, Caciorgna, M, Alampi, D, Francesconi, S, Beck, E, Antonini, B, Vettoretto, K, Meggiolaro, M, Garofalo, E, Notaro, S, Varutti, R, Bassi, F, Mistraletti, G, Marino, A, Rona, R, Rondelli, E, Riva, I, Cortegiani, A, Pistidda, L, D'Andrea, R, Querci, L, Gnesin, P, Todeschini, M, Lugano, M, Castelli, G, Ortolani, M, Cotoia, A, Maggiore, S, Ditizio, L, Graziani, R, Testa, I, Ferretti, E, Castioni, C, Lombardi, F, Caserta, R, Pasqua, M, Simoncini, S, Baccarini, F, Rispoli, M, Grossi, F, Cancelliere, L, Carnelli, M, Puccini, F, Biancofiore, G, Siniscalchi, A, Laici, C, Torrini, M, Pasetti, G, Palmese, S, Oggioni, R, Mangani, V, Pini, S, Martelli, M, Rigo, E, Zuccala, F, Cherri, A, Spina, R, Calamai, I, Petrucci, N, Caicedo, A, Ferri, F, Gritti, P, Brienza, N, Fonnesu, R, Dessena, M, Fullin, G, and Saggioro, D

Subjects
Aging, Disability, Rehabilitation, Delirium, Dementia, Physical restraint, Cross-Sectional Studies, Activities of Daily Living, mental disorders, Prevalence, Humans, Geriatrics and Gerontology, Aged
Abstract

Background: Delirium is thought to be common across various settings of care; however, still little research has been conducted in rehabilitation. Aim: We investigated the prevalence of delirium, its features and motor subtypes in older patients admitted to rehabilitation facilities during the three editions of the “Delirium Day project”. Methods: We conducted a cross-sectional study in which 1237 older patients (age ≥ 65 years old) admitted to 50 Italian rehabilitation wards during the three editions of the “Delirium Day project” (2015 to 2017) were included. Delirium was evaluated through the 4AT and its motor subtype with the Delirium Motor Subtype Scale. Results: Delirium was detected in 226 patients (18%), and the most recurrent motor subtype was mixed (37%), followed by hypoactive (26%), hyperactive (21%) and non-motor one (16%). In a multivariate Poisson regression model with robust variance, factors associated with delirium were: disability in basic (PR 1.48, 95%CI: 1.17–1.9, p value 0.001) and instrumental activities of daily living (PR 1.58, 95%CI: 1.08–2.32, p value 0.018), dementia (PR 2.10, 95%CI: 1.62–2.73, p value < 0.0001), typical antipsychotics (PR 1.47, 95%CI: 1.10–1.95, p value 0.008), antidepressants other than selective serotonin reuptake inhibitors (PR 1.3, 95%CI: 1.02–1.66, p value 0.035), and physical restraints (PR 2.37, 95%CI: 1.68–3.36, p value < 0.0001). Conclusion: This multicenter study reports that 2 out 10 patients admitted to rehabilitations had delirium on the index day. Mixed delirium was the most prevalent subtype. Delirium was associated with unmodifiable (dementia, disability) and modifiable (physical restraints, medications) factors. Identification of these factors should prompt specific interventions aimed to prevent or mitigate delirium.

Published
2022

14. Comparison between two packages for pectoral muscle removal on mammographic images

Author

Mario Sansone, Stefano Marrone, Giusi Di Salvio, Maria Paola Belfiore, Gianluca Gatta, Roberta Fusco, Laura Vanore, Chiara Zuiani, Francesca Grassi, Maria Teresa Vietri, Vincenza Granata, Roberto Grassi, Sansone, Mario, Marrone, Stefano, Di Salvio, Giusi, Belfiore, Maria Paola, Gatta, Gianluca, Fusco, Roberta, Vanore, Laura, Zuiani, Chiara, Grassi, Francesca, Vietri, Maria Teresa, Granata, Vincenza, Grassi, Roberto, Sansone, M., Marrone, S., Di Salvio, G., Belfiore, M. P., Gatta, G., Fusco, R., Vanore, L., Zuiani, C., Grassi, F., Vietri, M. T., Granata, V., and Grassi, R.

Subjects
Breast evaluation, Reproducibility of Result, Reproducibility of Results, Breast Neoplasms, General Medicine, Full-field digital mammography, Pectoralis Muscles, Algorithm, Radiographic Image Enhancement, Pectoral muscle removal, Humans, Radiographic Image Interpretation, Computer-Assisted, Female, Radiology, Nuclear Medicine and imaging, Breast Neoplasm, Algorithms, Human, Breast Density, Mammography
Abstract

Background Pectoral muscle removal is a fundamental preliminary step in computer-aided diagnosis systems for full-field digital mammography (FFDM). Currently, two open-source publicly available packages (LIBRA and OpenBreast) provide algorithms for pectoral muscle removal within Matlab environment. Purpose To compare performance of the two packages on a single database of FFDM images. Methods Only mediolateral oblique (MLO) FFDM was considered because of large presence of pectoral muscle on this type of projection. For obtaining ground truth, pectoral muscle has been manually segmented by two radiologists in consensus. Both LIBRA’s and OpenBreast’s removal performance with respect to ground truth were compared using Dice similarity coefficient and Cohen-kappa reliability coefficient; Wilcoxon signed-rank test has been used for assessing differences in performances; Kruskal–Wallis test has been used to verify possible dependence of the performance from the breast density or image laterality. Results FFDMs from 168 consecutive women at our institution have been included in the study. Both LIBRA’s Dice-index and Cohen-kappa were significantly higher than OpenBreast (Wilcoxon signed-rank test P P > 0.05). Conclusion: Libra has a better performance than OpenBreast in pectoral muscle delineation so that, although our study has not a direct clinical application, these results are useful in the choice of packages for the development of complex systems for computer-aided breast evaluation.

Published
2022

15. p100/IκBδ sequesters and inhibits NF-κB through kappaBsome formation

Author

Tao, Zhihua, Fusco, Amanda, Huang, De-Bin, Gupta, Kushol, Kim, Daniel Young, Ware, Carl F, Van Duyne, Gregory D, and Ghosh, Gourisankar

Subjects
1.1 Normal biological development and functioning, Underpinning research, 3T3 Cells, Animals, Crystallography, X-Ray, Humans, I-kappa B Proteins, Intracellular Signaling Peptides and Proteins, Mice, Mice, Knockout, Multiprotein Complexes, NF-kappa B p52 Subunit, Peptide Fragments, Protein Structure, Quaternary, Protein Structure, Tertiary, Proteins, Proteolysis, Transcription Factors, I kappa B, NF-kappa B, transcription, structure, kappaBsome, IκB, NF-κB
Abstract

Degradation of I kappaB (κB) inhibitors is critical to activation of dimeric transcription factors of the NF-κB family. There are two types of IκB inhibitors: the prototypical IκBs (IκBα, IκBβ, and IκBε), which form low-molecular-weight (MW) IκB:NF-κB complexes that are highly stable, and the precursor IκBs (p105/IκBγ and p100/IκBδ), which form high-MW assemblies, thereby suppressing the activity of nearly half the cellular NF-κB [Savinova OV, Hoffmann A, Ghosh G (2009) Mol Cell 34(5):591-602]. The identity of these larger assemblies and their distinct roles in NF-κB inhibition are unknown. Using the X-ray crystal structure of the C-terminal domain of p100/IκBδ and functional analysis of structure-guided mutants, we show that p100/IκBδ forms high-MW (IκBδ)4:(NF-κB)4 complexes, referred to as kappaBsomes. These IκBδ-centric "kappaBsomes" are distinct from the 2:2 complexes formed by IκBγ. The stability of the IκBδ tetramer is enhanced upon association with NF-κB, and hence the high-MW assembly is essential for NF-κB inhibition. Furthermore, weakening of the IκBδ tetramer impairs both its association with NF-κB subunits and stimulus-dependent processing into p52. The unique ability of p100/IκBδ to stably interact with all NF-κB subunits by forming kappaBsomes demonstrates its importance in sequestering NF-κB subunits and releasing them as dictated by specific stimuli for developmental programs.

Published
2014

16. Hotspot activating PRKD1 somatic mutations in polymorphous low-grade adenocarcinomas of the salivary glands

Author

Weinreb, Ilan, Piscuoglio, Salvatore, Martelotto, Luciano G, Waggott, Daryl, Ng, Charlotte KY, Perez-Ordonez, Bayardo, Harding, Nicholas J, Alfaro, Javier, Chu, Kenneth C, Viale, Agnes, Fusco, Nicola, da Cruz Paula, Arnaud, Marchio, Caterina, Sakr, Rita A, Lim, Raymond, Thompson, Lester DR, Chiosea, Simion I, Seethala, Raja R, Skalova, Alena, Stelow, Edward B, Fonseca, Isabel, Assaad, Adel, How, Christine, Wang, Jianxin, de Borja, Richard, Chan-Seng-Yue, Michelle, Howlett, Christopher J, Nichols, Anthony C, Wen, Y Hannah, Katabi, Nora, Buchner, Nicholas, Mullen, Laura, Kislinger, Thomas, Wouters, Bradly G, Liu, Fei-Fei, Norton, Larry, McPherson, John D, Rubin, Brian P, Clarke, Blaise A, Weigelt, Britta, Boutros, Paul C, and Reis-Filho, Jorge S

Subjects
Agricultural, Veterinary and Food Sciences, Biological Sciences, Bioinformatics and Computational Biology, Genetics, Agricultural Biotechnology, Cancer, Digestive Diseases, Adenocarcinoma, Amino Acid Sequence, Animals, Humans, Immunohistochemistry, Immunoprecipitation, Mice, Microscopy, Confocal, Models, Molecular, Molecular Sequence Data, Mutagenesis, Mutation, Missense, NIH 3T3 Cells, Protein Kinase C, Salivary Gland Neoplasms, Sequence Alignment, Sequence Analysis, DNA, Medical and Health Sciences, Developmental Biology, Agricultural biotechnology, Bioinformatics and computational biology
Abstract

Polymorphous low-grade adenocarcinoma (PLGA) is the second most frequent type of malignant tumor of the minor salivary glands. We identified PRKD1 hotspot mutations encoding p.Glu710Asp in 72.9% of PLGAs but not in other salivary gland tumors. Functional studies demonstrated that this kinase-activating alteration likely constitutes a driver of PLGA.

Published
2014

17. Role of serum‐free light chain assay for defining response and progression in immunoglobulin secretory multiple myeloma

Author

Paola Tacchetti, Serena Rocchi, Elena Zamagni, Simona Barbato, Ilaria Rizzello, Gabriella De Cicco, Lucia Pantani, Katia Mancuso, Alessio Fusco, Luca Dozza, Margherita Ursi, Emanuele Favero, Carolina Terragna, Nicoletta Testoni, Michele Cavo, Tacchetti P., Rocchi S., Zamagni E., Barbato S., Rizzello I., De Cicco G., Pantani L., Mancuso K., Fusco A., Dozza L., Ursi M., Favero E., Terragna C., Testoni N., and Cavo M.

Subjects
serum-free light chain, response, progression, multiple myeloma, Humans, Immunoglobulin Light Chains, Hematology, Multiple Myeloma, Prognosis, Retrospective Studies
Abstract

The International Myeloma Working Group (IMWG) guidelines recommend using electrophoresis and immunofixation to define response and progressive disease (PD) in immunoglobulin (Ig) secretory multiple myeloma (Ig-MM), whereas the role of serum-free light chain (sFLC) is controversial. We retrospectively analyzed the value of adding sFLC assays in the definition of response and PD according to IMWG criteria in 339 Ig-MM patients treated with a first-line novel agent-based therapy (median follow-up 54 months). sFLC PD was defined according to conventional criteria plus increased sFLC levels, or sFLC escape (sFLCe); progression/sFLCe-free survival (ePFS) was the time from the start of treatment to the date of first PD or sFLCe, or death; overall survival after PD/sFLCe (OS after Pe) was the time from first PD or sFLCe to the date of death. 148 (44%) patients achieved a complete response and 198 (60%) a normal sFLC ratio (sFLCR). sFLCR normalization was an independent prognostic factor for extended PFS (HR=0.46, p=0.001) and OS (HR=0.47, p=0.006) by multivariable analysis. 175 (52%) patients experienced PD according to the IMWG criteria, whereas 180 (53%) experienced PD or sFLCe. Overall, a sFLCe was observed in 31 (9%) patients. Median PFS and ePFS were both equal to 36 (95% CI=32–42, and 32–40, respectively) months. sFLC PD adversely affected the OS after Pe compared to PD with increasing monoclonal Ig only (HR=0.52, p=0.012). Our results support the inclusion of the sFLC assay for defining response and PD in Ig-MM.

Published
2022

18. Excessive Weight Gain: Current Antiretroviral Agents in Virologically Suppressed People with HIV

Author

Ricky K. Hsu, Laurence Brunet, Jennifer S. Fusco, Karam Mounzer, Joyce C. Lamori, and Gregory P. Fusco

Subjects
Infectious Diseases, Anti-Retroviral Agents, Anti-HIV Agents, Virology, Immunology, HIV-1, Humans, Emtricitabine, HIV Infections, Cobicistat, Tenofovir, Weight Gain, Darunavir
Abstract

An observational cohort study was conducted with data from the Observational Pharmaco-Epidemiology ResearchAnalysis (OPERA) cohort to investigate weight gain among virologically suppressed people with HIV (PWH) switching to regimens containing tenofovir alafenamide/emtricitabine/(TAF/FTC). Virologically suppressed, antiretroviral therapy (ART)-experienced PWH switching to TAF/FTC with darunavir/cobicistat (DRV/c), elvitegravir/cobicistat (EVG/c), dolutegravir (DTG), or bictegravir (BIC) were selected. Cox proportional hazards models were used to assess the risk of excessive weight gain (i.e., ≥5% gain within 28 weeks or ≥10% within 54 weeks), by regimen. A linear mixed effects model with random intercept and restricted cubic splines on time was used to assess continuous changes in weight. Confounding was controlled for with both inverse probability of treatment weighting and traditional covariate adjustment. Among 5,536 PWH, 18% gained ≥5% of their weight within 28 weeks, and 9% gained ≥10% within 54 weeks. There were no differences in the risk of excessive weight gain by regimen, although there was a nonstatistically significant 20% increase in the risk of gaining ≥10% within 54 weeks with all regimens compared to DRV/c. Throughout follow-up, the mean predicted weight remained fairly constant, with no notable differentiation between regimens. Expected weight gains ranged from +0.2 to +0.3 kg at 6 months and from +0.5 to +0.6 kg at 24 months. In conclusion, in this study of virologically suppressed, ART-experienced PWH switching to regimens containing TAF/FTC and DRV/c, EVG/c, DTG, or BIC, up to 18% experienced excessive levels of weight gain. However, no statistically significant difference was observed across regimens.

Published
2022

19. Premature ejaculation in the era of mobile health application: A current analysis and evaluation of adherence to EAU guidelines

Author

Luigi Napolitano, Luigi Cirillo, Giovanni Maria Fusco, Marco Abate, Alfonso Falcone, Vincenzo Morgera, Gianluigi Cacace, Luigi De Luca, Pasquale Reccia, Claudia Mirone, Felice Crocetto, Giuseppe Celentano, Simone Morra, Biagio Barone, Ciro Imbimbo, Nicola Longo, Vincenzo Mirone, Roberto La Rocca, Napolitano, Luigi, Cirillo, Luigi, Fusco, Giovanni Maria, Abate, Marco, Falcone, Alfonso, Morgera, Vincenzo, Cacace, Gianluigi, De Luca, Luigi, Reccia, Pasquale, Mirone, Claudia, Crocetto, Felice, Celentano, Giuseppe, Morra, Simone, Barone, Biagio, Imbimbo, Ciro, Longo, Nicola, Mirone, Vincenzo, and La Rocca, Roberto

Subjects
Male, Urology, Quality of Life, Humans, Premature Ejaculation, Mobile Applications, Telemedicine
Abstract

Introduction: Several mobile health applications (MHAs) have been developed to assist and improve the quality of life of patients affected by premature ejaculation, but the scientific quality and adherence to guidelines are not yet addressed. Materials and methods: On 25 May 2022, we conducted a search in the Apple App Store and Google Play Store. We reviewed all mobile apps from Apple App Store and Google Play Store for premature ejaculation and evaluated their usage in screening, prevention, management, and adherence to EAU guidelines. Results: In total 9 MHA were reviewed. All MHAs are geared towards the patient and provide information about diagnoses and treatment of PE. The mean score were 2.87, 3.69, 2.77, 2.55, 2.86 for Engagement, Functionality, Aesthetics, Information, and Subjective quality respectively. MHAs reported low and medium adherence to EAU guidelines. Conclusions: MHAs provide different services in many medical fields, including male sexual dysfunction. Their development is constantly increasing, but the problems of scientific validation, content, and quality are not yet solved. Much future research is necessary to improve the quality of the apps and promote new user designed, and high-quality apps.

Published
2022

20. A structural basis for IκB kinase 2 activation via oligomerization-dependent trans auto-phosphorylation.

Author

Polley, Smarajit, Huang, De-Bin, Hauenstein, Arthur V, Fusco, Amanda J, Zhong, Xiangyang, Vu, Don, Schröfelbauer, Bärbel, Kim, Youngchang, Hoffmann, Alexander, Verma, Inder M, Ghosh, Gourisankar, and Huxford, Tom

Subjects
Humans, Solutions, Chromatography, Gel, Crystallography, X-Ray, Transfection, Enzyme Activation, Protein Structure, Quaternary, Protein Structure, Tertiary, Protein Binding, Structure-Activity Relationship, Phosphorylation, Models, Molecular, I-kappa B Kinase, Protein Multimerization, HEK293 Cells, Chromatography, Gel, Crystallography, X-Ray, Protein Structure, Quaternary, Tertiary, Models, Molecular, Biological Sciences, Agricultural and Veterinary Sciences, Medical and Health Sciences, Developmental Biology
Abstract

Activation of the IκB kinase (IKK) is central to NF-κB signaling. However, the precise activation mechanism by which catalytic IKK subunits gain the ability to induce NF-κB transcriptional activity is not well understood. Here we report a 4 Å x-ray crystal structure of human IKK2 (hIKK2) in its catalytically active conformation. The hIKK2 domain architecture closely resembles that of Xenopus IKK2 (xIKK2). However, whereas inactivated xIKK2 displays a closed dimeric structure, hIKK2 dimers adopt open conformations that permit higher order oligomerization within the crystal. Reversible oligomerization of hIKK2 dimers is observed in solution. Mutagenesis confirms that two of the surfaces that mediate oligomerization within the crystal are also critical for the process of hIKK2 activation in cells. We propose that IKK2 dimers transiently associate with one another through these interaction surfaces to promote trans auto-phosphorylation as part of their mechanism of activation. This structure-based model supports recently published structural data that implicate strand exchange as part of a mechanism for IKK2 activation via trans auto-phosphorylation. Moreover, oligomerization through the interfaces identified in this study and subsequent trans auto-phosphorylation account for the rapid amplification of IKK2 phosphorylation observed even in the absence of any upstream kinase.

Published
2013

21. Diagnostic issues faced by a rare disease healthcare network during Covid-19 outbreak: data from the Campania Rare Disease Registry

Author

Limongelli G, Iucolano S, Monda E, Elefante P, De Stasio C, Lubrano I, Caiazza M, Mazzella M, Fimiani F, Galdo M, De Marchi G, Esposito M, Rubino M, Cirillo A, Fusco A, Esposito A, Trama U, Esposito S, Scarano G, Sepe J, Andria G, Orlando V, Menditto E, Chiodini P, Campania Rare Disease, Iolascon A, Franzese A, Sanduzzi Zamparelli A, Tessitore A, Romano A, Venosa A, Nunzia Olivieri A, Bianco A, La Manna A, Cerbone AM, Spasiano A, Agnese Stanziola A, Colao A, De Bellis A, Gambale A, Toriello A, Tufano A, Ciampa A, Maria Risitano A, Pisani A, Russo A, Volpe A, De Martino B, Amato B, De Fusco C, Piscopo C, Selleri C, Tucci C, Pignata C, Cioffi D, Melis D, Pasquali D, De Brasi D, Spitaleri D, Russo D, Martellotta D, De Michele E, Varricchio E, Miraglia Del Giudice E, Coscioni E, Cimino E, Pane F, Tranfa F, Pollio F, Lonardo F, Nuzzi F, Simonelli F, Trojsi F, Habetswallner F, Valentini G, Cerbone G, Parenti G, Tedeschi G, Capasso G, Battista Rossi G, Gaglione G, Sarnelli G, Argenziano G, Bellastella G, De Michele G, Fiorentino G, Spadaro G, Scala I, Santoro L, Zeppa L, Auricchio L, Elio Adinolfi L, Alessio M, Amitrano M, Savanelli MC, Russo MG, Ferrucci MG, Carbone MT, Pellecchia MT, Salerno M, Melone M, Del Donno M, Vitale M, Triggiani M, Della Monica M, Lo Presti M, Tenuta M, Mignogna MD, Schiavulli M, Zacchia M, Brunetti-Pierri N, Iovino P, Moscato P, Iandoli R, Scarpa R, Russo R, Troisi S, Sbordone S, Perrotta S, Fecarotta S, Sampaolo S, Cicalese V., Limongelli, Giuseppe, Iucolano, Stefano, Monda, Emanuele, Elefante, Pasquale, De Stasio, Chiara, Lubrano, Imma, Caiazza, Martina, Mazzella, Marialuisa, Fimiani, Fabio, Galdo, Maria, De Marchi, Giulia, Esposito, Martina, Rubino, Marta, Cirillo, Annapaola, Fusco, Adelaide, Esposito, Augusto, Trama, Ugo, Esposito, Salvatore, Scarano, Gioacchino, Sepe, Joseph, Andria, Generoso, Orlando, Valentina, Menditto, Enrica, Chiodini, Paolo, Iolascon, Achille, Franzese, Adriana, Sanduzzi Zamparelli, Alessandro, Tessitore, Alessandro, Romano, Alfonso, Venosa, Alfredo, Nunzia Olivieri, Alma, Bianco, Andrea, La Manna, Angela, Cerbone, Anna Maria, Spasiano, Anna, Agnese Stanziola, Anna, Colao, Annamaria, De Bellis, Annamaria, Gambale, Antonella, Toriello, Antonella, Tufano, Antonella, Ciampa, Antonio, Maria Risitano, Antonio, Pisani, Antonio, Russo, Antonio, Volpe, Antonio, De Martino, Bernardo, Amato, Bruno, De Fusco, Carmela, Piscopo, Carmelo, Selleri, Carmine, Tucci, Celeste, Pignata, Claudio, Cioffi, Daniela, Melis, Daniela, Pasquali, Daniela, De Brasi, Daniele, Spitaleri, Daniele, De Brasi, Davide, Russo, Domenico, Martellotta, Donata, De Michele, Elisa, Varricchio, Elziario, Miraglia Del Giudice, Emanuele, Coscioni, Enrico, Cimino, Ernesto, Pane, Fabrizio, Tranfa, Fausto, Pollio, Filiberto, Lonardo, Fortunato, Nuzzi, Francesca, Simonelli, Francesca, Trojsi, Francesca, Habetswallner, Francesco, Valentini, Gabriele, Cerbone, Gaetana, Parenti, Giancarlo, Tedeschi, Gioacchino, Capasso, Giovambattista, Battista Rossi, Giovanni, Gaglione, Giovanni, Sarnelli, Giovanni, Argenziano, Giuseppe, Bellastella, Giuseppe, De Michele, Giuseppe, Fiorentino, Giuseppe, Spadaro, Giuseppe, Scala, Iri, Santoro, Lucio, Zeppa, Lucio, Auricchio, Luigi, Elio Adinolfi, Luigi, Alessio, Maria, Amitrano, Maria, Savanelli, Maria Cristina, Russo, Maria Giovanna, Ferrucci, Maria Grazia, Carbone, Maria Teresa, Pellecchia, Maria Teresa, Salerno, Mariacarolina, Melone, Marina, Del Donno, Mario, Vitale, Mario, Triggiani, Massimo, Della Monica, Matteo, Lo Presti, Maurizio, Tenuta, Maurizio, Mignogna, Michele Davide, Schiavulli, Michele, Zacchia, Miriam, Brunetti-Pierri, Nicola, Iovino, Paola, Moscato, Paolo, Iandoli, Raffaele, Scarpa, Raffaele, Russo, Romualdo, Troisi, Salvatore, Sbordone, Sandro, Perrotta, Silverio, Fecarotta, Simona, Sampaolo, Simone, Cicalese, Virgilio, Limongelli, G, Iucolano, S, Monda, E, Elefante, P, De Stasio, C, Lubrano, I, Caiazza, M, Mazzella, M, Fimiani, F, Galdo, M, De Marchi, G, Esposito, M, Rubino, M, Cirillo, A, Fusco, A, Esposito, A, Trama, U, Esposito, S, Scarano, G, Sepe, J, Andria, G, Orlando, V, Menditto, E, Chiodini, P, Campania Rare, Disease, Iolascon, A, Franzese, A, Sanduzzi Zamparelli, A, Tessitore, A, Romano, A, Venosa, A, Nunzia Olivieri, A, Bianco, A, La Manna, A, Cerbone, Am, Spasiano, A, Agnese Stanziola, A, Colao, A, De Bellis, A, Gambale, A, Toriello, A, Tufano, A, Ciampa, A, Maria Risitano, A, Pisani, A, Russo, A, Volpe, A, De Martino, B, Amato, B, De Fusco, C, Piscopo, C, Selleri, C, Tucci, C, Pignata, C, Cioffi, D, Melis, D, Pasquali, D, De Brasi, D, Spitaleri, D, Russo, D, Martellotta, D, De Michele, E, Varricchio, E, Miraglia Del Giudice, E, Coscioni, E, Cimino, E, Pane, F, Tranfa, F, Pollio, F, Lonardo, F, Nuzzi, F, Simonelli, F, Trojsi, F, Habetswallner, F, Valentini, G, Cerbone, G, Parenti, G, Tedeschi, G, Capasso, G, Battista Rossi, G, Gaglione, G, Sarnelli, G, Argenziano, G, Bellastella, G, De Michele, G, Fiorentino, G, Spadaro, G, Scala, I, Santoro, L, Zeppa, L, Auricchio, L, Elio Adinolfi, L, Alessio, M, Amitrano, M, Savanelli, Mc, Russo, Mg, Ferrucci, Mg, Carbone, Mt, Pellecchia, Mt, Salerno, M, Melone, M, Del Donno, M, Vitale, M, Triggiani, M, Della Monica, M, Lo Presti, M, Tenuta, M, Mignogna, Md, Schiavulli, M, Zacchia, M, Brunetti-Pierri, N, Iovino, P, Moscato, P, Iandoli, R, Scarpa, R, Russo, R, Troisi, S, Sbordone, S, Perrotta, S, Fecarotta, S, Sampaolo, S, and Cicalese, V.

Subjects
Pediatrics, medicine.medical_specialty, Coronavirus disease 2019 (COVID-19), Home therapy, rare diseases, 030204 cardiovascular system & hematology, Campania region, Covid-19, Italy, patient registry, Disease Outbreaks, 03 medical and health sciences, COVID-19 Testing, 0302 clinical medicine, Pandemic, Health care, medicine, Retrospective analysis, Humans, AcademicSubjects/MED00860, Registries, 030212 general & internal medicine, Medical diagnosis, Pandemics, Retrospective Studies, business.industry, Public Health, Environmental and Occupational Health, Outbreak, General Medicine, Original Article, business, Delivery of Health Care, Rare disease
Abstract

Background The aims of this study were: to investigate the capacity of the rare disease healthcare network in Campania to diagnose patients with rare diseases during the outbreak of Covid-19; and to shed light on problematic diagnoses during this period. Methods To describe the impact of the Covid-19 pandemic on the diagnosis of patients with rare diseases, a retrospective analysis of the Campania Region Rare Disease Registry was performed. A tailored questionnaire was sent to rare disease experts to investigate major issues during the emergency period. Results Prevalence of new diagnoses of rare disease in March and April 2020 was significantly lower than in 2019 (117 versus 317, P Conclusions This study describes the effects of the Covid-19 outbreak on the diagnosis of rare disease in a single Italian region and investigates potential issues of diagnosis and management during this period.

Published
2021

22. Safety and efficacy of propranolol for treatment of familial cerebral cavernous malformations (Treat_CCM): a randomised, open-label, blinded-endpoint, phase 2 pilot trial

Author

Silvia Lanfranconi, Elisa Scola, Jennifer M T A Meessen, Roberto Pallini, Giulio A Bertani, Rustam Al-Shahi Salman, Elisabetta Dejana, Roberto Latini, Giorgia Abete Fornara, Nicolò M. Agnelli, Alessio Albanese, Issam Awad, Renzo Bagnati, Giovanna Balconi, Elena Ballabio, Ettore Beghi, Roberto Bernasconi, Giulio A. Bertani, Silvia Besana, Adriana Blanda, Chiara Bossi, Nereo Bresolin, Maria G. Buratti, Roberta Calabrese, Maria R. Carriero, Marco Castori, Elisa F. Ciceri, Rossella Ciurleo, Giacomo P. Comi, Valeria Contarino, Giorgio Conte, Leonardo D'Agruma, Giorgio Q. D'Alessandris, Ugo de Grazia, Rina Di Bonaventura, Piergiorgio d'Orio, Giuseppe Farago', Andreana Foresta, Carmela Fusco, Chiara Gaudino, Maria G. Lampugnani, Alessia Lanno, Francesca Lazzaroni, Cornelia Lee, Marco Locatelli, Aldo P. Maggioni, Peetra Magnusson, Matteo Malinverno, Maurizio Mangiavacchi, Antonella Mangraviti, Silvia Marino, Selene Mazzola, Enrico B. Nicolis, Deborah Novelli, Maria L. Ojeda Fernandez, Antonio Petracca, Fabrizio Pignotti, Simona Pogliani, Marco Poloni, Alessandro Prelle, Pamela Raggi, Franca Raucci, Caroline Regna-Gladin, Dario Ronchi, Emma Scelzo, Salim Seyfried, Anna Simeone, Carmelo L. Sturiale, Laura Tassi, Mauro Tettamanti, Valter Torri, Elisabeth Tournier-Lasserve, Rita Treglia, Fabio M. Triulzi, Celeste Ungaro, Elison Ursi, Gloria Valcamonica, Antonella Vasami', and Barbara Zarino

Subjects
Male, Treatment Outcome, Cerebral Hemorrhage/chemically induced, Humans, Female, Pilot Projects, Propranolol/pharmacology, Neurology (clinical), Middle Aged, Hemangioma, Cavernous, Central Nervous System/diagnostic imaging
Abstract

BACKGROUND: Observations in people with cerebral cavernous malformations, and in preclinical models of this disorder, suggest that the β-blocker propranolol might reduce the risk of intracerebral haemorrhage. We aimed to evaluate the safety and efficacy of prolonged treatment with propranolol to reduce the incidence of symptomatic intracerebral haemorrhage or focal neurological deficit in people with familial cerebral cavernous malformations.METHODS: We conducted a randomised, open-label, blinded-endpoint, phase 2 pilot trial (Treat_CCM) at six national reference centres for rare diseases in Italy. People aged 18 years or older with symptomatic familial cerebral cavernous malformation were eligible for enrolment. Participants were randomly assigned (2:1) to receive either oral propranolol (20-320 mg daily) plus standard care (intervention group), or standard care alone (control group), for 24 months. Participants, caregivers, and investigators were aware of treatment group assignment. Participants had clinical assessments and 3 T brain MRI at baseline and at 12 and 24 months. The primary outcome was new occurrence of symptomatic intracerebral haemorrhage or focal neurological deficit attributable to cerebral cavernous malformation over 24 months. Outcome assessors were masked to treatment group assignment. The primary analysis was done in the intention-to-treat population. Because of the pilot study design, we chose a one-sided 80% CI, which could either exclude a clinically meaningful effect or show a signal of efficacy. This trial is registered with EudraCT, 2017-003595-30, and ClinicalTrials.gov, NCT03589014, and is closed to recruitment.FINDINGS: Between April 11, 2018, and Dec 5, 2019, 95 people were assessed for eligibility and 83 were enrolled, of whom 57 were assigned to the propranolol plus standard care group and 26 to the standard care alone group. The mean age of participants was 46 years (SD 15); 48 (58%) were female and 35 (42%) were male. The incidence of symptomatic intracerebral haemorrhage or focal neurological deficit was 1·7 (95% CI 1·4-2·0) cases per 100 person-years (two [4%] of 57 participants) in the propranolol plus standard care group and 3·9 (3·1-4·7) per 100 person-years (two [8%] of 26) in the standard care alone group (univariable hazard ratio [HR] 0·43, 80% CI 0·18-0·98). The univariable HR showed a signal of efficacy, according to predefined criteria. The incidence of hospitalisation did not differ between groups (8·2 cases [95% CI 7·5-8·9] per 100 person-years in the propranolol plus standard care group vs 8·2 [95% CI 7·1-9·3] per 100 person-years in the standard care alone group). One participant in the standard care alone group died of sepsis. Three participants in the propranolol plus standard care group discontinued propranolol due to side-effects (two reported hypotension and one reported weakness).INTERPRETATION: Propranolol was safe and well tolerated in this population. Propranolol might be beneficial for reducing the incidence of clinical events in people with symptomatic familial cerebral cavernous malformations, although this trial was not designed to be adequately powered to investigate efficacy. A definitive phase 3 trial of propranolol in people with symptomatic familial cerebral cavernous malformations is justified.FUNDING: Italian Medicines Agency, Associazione Italiana per la Ricerca sul Cancro, Swedish Science Council, Knut and Alice Wallenberg Foundation, CARIPLO Foundation, Italian Ministry of Health.

Published
2023

23. Bisoprolol for treatment of symptomatic patients with obstructive hypertrophic cardiomyopathy. The BASIC (bisoprolol AS therapy in hypertrophic cardiomyopathy) study

Author

Emanuele Monda, Michele Lioncino, Giuseppe Palmiero, Francesco Franco, Marta Rubino, Annapaola Cirillo, Federica Verrillo, Adelaide Fusco, Martina Caiazza, Marialuisa Mazzella, Elisabetta Moscarella, Francesca Dongiglio, Joseph Sepe, Giuseppe Pacileo, Paolo Calabrò, Giuseppe Limongelli, Monda, E., Lioncino, M., Palmiero, G., Franco, F., Rubino, M., Cirillo, A., Verrillo, F., Fusco, A., Caiazza, M., Mazzella, M., Moscarella, E., Dongiglio, F., Sepe, J., Pacileo, G., Calabro, P., and Limongelli, G.

Subjects
Adult, Retrospective Studie, Bisoprolol, Humans, Therapy, Cardiomyopathy, Hypertrophic, Cardiology and Cardiovascular Medicine, Disopyramide, Hypertrophic cardiomyopathy, Human, Retrospective Studies, Ventricular Outflow Obstruction
Abstract

Aims: To evaluate the role of bisoprolol to control symptoms and left ventricular outflow tract obstruction (LVOTO) in a consecutive cohort of adults with hypertrophic cardiomyopathy (HCM). Methods and results: In this retrospective study, patients with HCM with an LVOT gradient ≥50 mmHg after Valsalva manoeuvre and New York Heart Association (NYHA) class II-III symptoms were assigned to receive bisoprolol (starting at 1.25 mg daily). The initial dose was increased every two weeks to achieve the target in LVOT gradient

Published
2022

24. Looking for cystoscopy on YouTube: Are videos a reliable information tool for internet users?

Author

Carmine Turco, Claudia Collà Ruvolo, Simone Cilio, Giuseppe Celentano, Gianluigi Califano, Massimiliano Creta, Marco Capece, Roberto La Rocca, Luigi Napolitano, Francesco Mangiapia, Lorenzo Spirito, Simone Morra, Alberto Melchionna, Ferdinando Fusco, Vincenzo Mirone, Nicola Longo, Turco, Carmine, Collà Ruvolo, Claudia, Cilio, Simone, Celentano, Giuseppe, Califano, Gianluigi, Creta, Massimiliano, Capece, Marco, La Rocca, Roberto, Napolitano, Luigi, Mangiapia, Francesco, Spirito, Lorenzo, Morra, Simone, Melchionna, Alberto, Fusco, Ferdinando, Mirone, Vincenzo, and Longo, Nicola

Subjects
Urology, Video Recording, Humans, Cystoscopy, Social Media, Human
Abstract

Objective: The Internet is an important and easily accessible source of information. The aim of the current study was to investigate the quality of YouTube videos on cystoscopy and to establish if they can be used as a reliable information tool for internet users. Materials and methods: The search term “cystoscopy” was used on YouTube platform and the first 120 YouTube videos were analyzed. To assess the video quality Patient Education Materials Assessment Tool (PEMAT) for Audiovisual (A/V) Materials (Understandability and Actionability sections), Misinformation score and Global Quality Score (GQS) were used. Results: Of all 120 videos, 72 were included in the analyses. Of all videos, 59.7% (n = 43), and 40.3% (n = 29) were targeted to General Public and Healthcare Workers. Moreover, “technical aspects” was the main topic addressed (n = 29, 40.3%). The median PEMAT A/V Understandability and Actionability scores were 50.0% (IQR: 39.1-70.0) and 66.7% (IQR: 33.3- 100.0), respectively. The median Misinformation score ranged from 1.0 to 3.0. According to GQS, 22 (30.6%), 26 (36.1%), 16 (22.2%), 8 (11.1%) videos were poor, generally poor, moderate, and good, respectively. No video was evaluated as excellent. Conclusions: Today, YouTube videos on cystoscopy are more frequently uploaded by healthcare workers, who share information about specific aspects of this procedure. However, the quality of YouTube contents on cystoscopy is still poor. Therefore, currently users interested in cystoscopy cannot rely on YouTube to get good informative material on this topic. In consequence, future authors should focus on improving the quality of video contents on cystoscopy

Published
2022

25. The preventive care of medication-related osteonecrosis of the jaw (MRONJ): a position paper by Italian experts for dental hygienists

Author

Rodolfo Mauceri, Rita Coniglio, Antonia Abbinante, Paola Carcieri, Domenico Tomassi, Vera Panzarella, Olga Di Fede, Francesco Bertoldo, Vittorio Fusco, Alberto Bedogni, Giuseppina Campisi, Mauceri, Rodolfo, Coniglio, Rita, Abbinante, Antonia, Carcieri, Paola, Tomassi, Domenico, Panzarella, Vera, Di Fede, Olga, Bertoldo, Francesco, Fusco, Vittorio, Bedogni, Alberto, and Campisi, Giuseppina

Subjects
Bone Density Conservation Agents, Diphosphonates, Osteonecrosis of the jaw, Risk factors, Oncology, Dental hygienists, Incidence, Prevention, Humans, MRONJ, Periodontal screening score, Bisphosphonate-Associated Osteonecrosis of the Jaw
Abstract

Purpose The prevention and early diagnosis of medication-related osteonecrosis of the jaw (MRONJ) is fundamental to reducing the incidence and progression of MRONJ. Many in the field believe that dental hygienists should play an integral role in primary and secondary MRONJ prevention. However, to date, very few publications in the literature have proposed standardised MRONJ protocols, which are dedicated to dental hygienists. The aim of this study was to provide guidance to the health care providers managing MRONJ. Methods The expert opinion in this study was developed by dental hygienists from the main Italian technical-scientific associations (Italian Dental Hygienists Association, AIDI and National Union of Dental Hygienists, UNID) and authors of the latest Italian recommendations regarding MRONJ from the field of dentistry and maxillofacial surgery. Results The oral care protocol outlined in this position paper is focused on the role of dental hygienist in patients at risk or affected by MRONJ, and it regards 3 main issues: primary prevention, secondary prevention and supporting the treatment of MRONJ. Each issue contains easy-to-apply indications and procedures, as described by the authors, regarding the role of the dental hygienist. Conclusion Referring to the main issues under consideration (primary prevention, secondary prevention and the treatment of MRONJ), a clinical examination of periodontal tissue is critical in preventing MRONJ. It is the opinion of the authors of this study that the application of a periodontal screening score is fundamental in defining personalised strategies for patients at risk of MRONJ. By means of these basic procedures, a protocol for assisting the health care provider and the presentation of a practical approach for patients at risk or affected by MRONJ are described in this study.

Published
2022

26. Natural history of left ventricular hypertrophy in infants of diabetic mothers

Author

Emanuele Monda, Federica Verrillo, Ippolita Altobelli, Michele Lioncino, Martina Caiazza, Marta Rubino, Annapaola Cirillo, Adelaide Fusco, Augusto Esposito, Francesco Di Fraia, Roberta Pacileo, Felice Gragnano, Annalisa Passariello, Paolo Calabrò, Maria Giovanna Russo, Giuseppe Limongelli, Monda, E., Verrillo, F., Altobelli, I., Lioncino, M., Caiazza, M., Rubino, Marta, Cirillo, A., Fusco, A., Esposito, A., Di Fraia, F., Pacileo, R., Gragnano, F., Passariello, Annalisa, Calabrò, Paolo, Russo, M. G., and Limongelli, G.

Subjects
Echocardiography, Diabetes Mellitus, Humans, Infant, Mothers, Left ventricular hypertrophy, Maternal diabetes, Female, Hypertrophy, Left Ventricular, Infants of diabetic mother, Cardiology and Cardiovascular Medicine, Retrospective Studies
Abstract

Background: This study sought to describe the characteristics and the natural course of left ventricular hypertrophy (LVH) in a well-characterized consecutive cohort of infants of diabetic mothers (IDMs). Methods: Sixty consecutive IDMs with LVH have been retrospectively identified and enrolled in the study. All IDMs were evaluated at baseline and every 6 months until LV wall thickness regression, defined as the decrease of wall thickness measurement into the normal reference range for cardiac parameters (z-score > −2 and < 2). A comprehensive assessment was performed in those patients with diagnostic markers suggestive of a different cause and/or without significant reduction of the LVH during follow-up. Results: At 1-year follow-up, all IDMs showed a significant reduction of maximal wall thickness MWT (6.00 mm [IQR 5.00–712] vs. 5.50 mm [IQR 5.00–6.00], p-value

Published
2022

27. Quantification of heterogeneity to classify benign parotid tumors: a feasibility study on most frequent histotypes

Author

Gabriele Maria Nicolino, Mario Petrillo, Giuseppe Franceschelli, Laura Tofanelli, Filippo Pesapane, Giorgio Buccimazza, Massimo Fusco, Federico Biglioli, Vissagan Gopalakrishnan, Roberta Fusco, Francesca Patella, Mario Sansone, Maurizio Cariati, Patella, F., Sansone, M., Franceschelli, G., Tofanelli, L., Petrillo, M., Fusco, M., Nicolino, G. M., Buccimazza, G., Fusco, R., Gopalakrishnan, V., Pesapane, F., Biglioli, F., and Cariati, M.

Subjects
Male, pleomorphic adenoma, Cancer Research, Multivariate analysis, 030218 nuclear medicine & medical imaging, 0302 clinical medicine, parotid tumor, volume of interest, Image Processing, Computer-Assisted, Medicine, Intravoxel incoherent motion, intravoxel incoherent motion, Aged, 80 and over, medicine.diagnostic_test, Histocytochemistry, Area under the curve, General Medicine, Middle Aged, Magnetic Resonance Imaging, Parotid Neoplasms, Oncology, 030220 oncology & carcinogenesis, Female, Warthin tumor, histogram, Algorithms, Adult, Adolescent, DCE-MRI, magnetic resonance, Young Adult, 03 medical and health sciences, DW-MRI, Histogram, Humans, Aged, Neoplasm Staging, Retrospective Studies, Receiver operating characteristic, business.industry, Warthin Tumor, Magnetic resonance imaging, Models, Theoretical, Biological Variation, Population, ROC Curve, Skewness, Feasibility Studies, Neoplasm Grading, business, Nuclear medicine
Abstract

Aim: To differentiate Warthin tumors (WTs) and pleomorphic adenomas (PAs) measuring heterogeneity of intravoxel incoherent motion (IVIM) and dynamic-contrast enhanced-magnetic resonance imaging biomarkers. Methods: Volumes of interest were traced on 18 WT and 18 PA in 25 patients. For each IVIM and dynamic-contrast enhanced biomarker, histogram parameters were calculated and then compared using the Wilcoxon-signed-rank test. Receiver operating characteristic curves and multivariate analysis were employed to identify the parameters and their pairs with the best accuracy. Results: Most of the biomarkers exhibited significant difference (p < 0.05) between PA and WT for histogram parameters. Time to peak median and skewness, and D* median and entropy showed the highest area under the curve. No meaningful improvement of accuracy was obtained using two features. Conclusion: IVIM and dynamic-contrast enhanced histogram descriptors may help in the classification of WT and PA.

Published
2020

28. Clinical Features in Aromatic L-Amino Acid Decarboxylase (AADC) Deficiency: A Systematic Review

Author

Susanna Rizzi, Carlotta Spagnoli, Daniele Frattini, Francesco Pisani, and Carlo Fusco

Subjects
Neuropsychology and Physiological Psychology, Neurology, newborn screening, Metabolic Disorder, Dopa Decarboxylase, Humans, Aromatic L-Amino Acid Decarboxylase Deficiency, Neurology (clinical), General Medicine, Aromatic L-Amino Acid Decarboxylase Deficiency, Metabolic Disorder, newborn screening, Amino Acids, Amino Acid Metabolism, Inborn Errors, Retrospective Studies
Abstract

Aromatic L-amino acid decarboxylase (AADC) deficiency is a rare congenital autosomal recessive metabolic disorder caused by pathogenic homozygous or compound heterozygous variants in the dopa decarboxylase (DDC) gene. Adeno-associated viral vector-mediated gene transfer of the human AADC gene into the putamina has become available. This systematic review on PubMed, Scopus databases, and other sources is aimed at describing the AADC whole phenotypic spectrum in order to facilitate its early diagnosis. Literature reviews, original articles, retrospective and comparative studies, large case series, case reports, and short communications were considered. A database was set up using Microsoft Excel to collect clinical, molecular, biochemical, and therapeutic data. By analysing 261 patients from 41 papers with molecular and/or biochemical diagnosis of AADC deficiency for which individuality could be determined with certainty, we found symptom onset to occur in the first 6 months of life in 93% of cases. Hypotonia and developmental delay are cardinal signs, reported as present in 73.9% and 72% of cases, respectively. Oculogyric crises were seen in 67% of patients while hypokinesia in 42% and ptosis in 26%. Dysautonomic features have been revealed in 53% and gastrointestinal symptoms in 19% of cases. With 37% and 30% of patients reported being affected by sleep and behavioural disorders, it seems to be commoner than previously acknowledged. Although reporting bias cannot be excluded, there is still a need for comprehensive clinical descriptions of symptoms at onset and during follow-up. In fact, our review suggests that most of the neurological and extraneurological symptoms and signs reported, although quite frequent in this condition, are not pathognomonic, and therefore, ADCC deficiency can remain an underdiscovered disorder.

Published
2022

29. Long-term neurological and psychiatric outcomes in patients with aromatic l-amino acid decarboxylase deficiency

Author

Filippo Manti, Mario Mastrangelo, Roberta Battini, Claudia Carducci, Carlotta Spagnoli, Carlo Fusco, Manuela Tolve, Carla Carducci, and Vincenzo Leuzzi

Subjects
Adult, Biogenic Amines, Adolescent, Dopamine, AADC deficiency Biogenic amines Intellectual disability Parkinsonism-dystonia Psychiatric disorders, Homovanillic Acid, Hydroxyindoleacetic Acid, Young Adult, Neurology, Aromatic-L-Amino-Acid Decarboxylases, Intellectual Disability, Humans, Neurology (clinical), Amino Acids, Geriatrics and Gerontology, Child
Abstract

l-amino acid decarboxylase deficiency (AADCD) is an ultrarare autosomal recessive defect of biogenic amine synthesis that presents with early-onset encephalopathy progressing to severe neurological impairment and intellectual disability. We aimed to explore neurocognitive and behavioral profiles associated with AADCD and possible factors predicting outcome in more detail.Nine AADCD patients (23.2 ± 10.3 years; range 8-40) underwent systematic clinical and neuropsychological assessment. Diagnostic levels of CSF 5-hydroxyindolacetic acid (5-HIAA) and homovanillic acid (HVA), and DDC genotype (as ascertained by American College of Medical Genetics and Genomics grading) were included in the data analysis.All AADCD patients were affected by intellectual disability and psychiatric disorders. Movement disorders included parkinsonism-dystonia, dysarthria, and oculogyric crises. CSF 5-HIAA and HVA levels at diagnosis had a significant influence on adaptive behavior and executive function performance. Patients homozygous for DDC pathogenetic variants showed lower CSF 5-HIAA and HVA levels and higher Unified Parkinson's Disease Rating Scale scores. The disease showed a self-limiting clinical course with partial improvement under pharmacological treatment (B6 and dopamine mimetic drugs).Patients with AADCD suffer from neuropsychological and psychopathological impairment, which may be improved but not reversed under the present therapeutic approach. However, cognitive functioning should be specifically examined in order to avoid its underestimation on the basis of movement disorder severity. Genotype and biogenic amine level at diagnosis have an important prognostic value.

Published
2022

30. Long-term results of teduglutide treatment for chronic intestinal failure – Insights from a national, multi-centric patient home-care service program

Author

Sophie Greif, Sebastian Maasberg, Jan Wehkamp, Stefano Fusco, Yurdagül Zopf, Hans Joachim Herrmann, Georg Lamprecht, Torid Jacob, Ingolf Schiefke, Martin W. von Websky, Janine Büttner, Elisabeth Blüthner, Frank Tacke, and Ulrich-Frank Pape

Subjects
Adult, Intestinal Failure, Short Bowel Syndrome, Intestinal Diseases, Nutrition and Dietetics, Gastrointestinal Agents, Endocrinology, Diabetes and Metabolism, Chronic Disease, Glucagon-Like Peptides, Quality of Life, Humans, Peptides, Retrospective Studies
Abstract

Teduglutide is a Glucagon-like peptide-2 (GLP-2) agonist indicated for the treatment of patients with parenteral support (PS) dependent short bowel syndrome (SBS) with chronic intestinal failure (cIF). Its application is accompanied by a structured nation-wide home-care service program in Germany. We investigated care characteristics and outcome parameters in a clinical real-world observational setting.Data generated within a therapy-accompanying home-care service program for adult SBS-cIF patients were analyzed retrospectively for patients treated up to 1 year (data cut: April 2020).In total, 52 teduglutide-treated patients were included by 6 German cIF centers. At teduglutide administration start, 49/52 patients were on PS, 3 of them without macronutrients. The majority of patients received individualized parenteral nutrition (PN) (n = 32/46), while 13/46 were on commercial premixed bags. PS application was done by patients themselves (37%), home-care nurses (19%), relatives (8%) or by a combination of those (16%). In patients with PS dependency at baseline and available follow-up data (n = 40-44), teduglutide treatment resulted in significantly reduced PN days, caloric needs, infusion time, and infusion volume after 6 and 12 months. After 1 year, reduction of infusion time was positively correlated with a reduction of PN calories and volume; 30 patients (68%) were responders (PS-volume reduction ≥20%), and 6 patients (14%) were completely weaned off PS. Sleep disturbances per night were significantly reduced after 3 months of treatment and stool characteristics improved in consistency and significantly in frequency, while meal frequency remained stable.Teduglutide treatment associated reduction in PS volume and calories was accompanied by reduced infusion days, infusion times, sleep disturbances, stable oral intake surrogates, and improved stool characteristics, all of these potential parameters for improving quality of life. Furthermore, analyzed care characteristics reflect SBS-cIF treatment as a complex, resource-intensive and demanding task for both, healthcare system and patients.

Published
2022

31. EEG at onset and MRI predict long-term clinical outcome in Aicardi syndrome

Author

Silvia Masnada, Enrico Alfei, Manuela Formica, Roberto Previtali, Patrizia Accorsi, Filippo Arrigoni, Paolo Bonanni, Renato Borgatti, Francesca Darra, Carlo Fusco, Valentina De Giorgis, Lucio Giordano, Francesca La Briola, Simona Orcesi, Elisa Osanni, Cecilia Parazzini, Lorenzo Pinelli, Erika Rebessi, Romina Romaniello, Antonino Romeo, Carlotta Spagnoli, Christian Uebler, Costanza Varesio, Maurizio Viri, Claudio Zucca, Anna Pichiecchio, and Pierangelo Veggiotti

Subjects
Epilepsy, Aicardi syndrome phenotypes, Clinical outcome, Long term electroencephalographic follow-up, Electroencephalography, Magnetic Resonance Imaging, Settore MED/39 - Neuropsichiatria Infantile, Sensory Systems, Aicardi Syndrome, Neurology, Physiology (medical), Humans, Neurology (clinical), Retrospective Studies
Abstract

Descriptions of electroencephalographic (EEG) patterns in Aicardi syndrome (AIC) have to date referred to small cohorts of up to six cases and indicated severe derangement of electrical activity in all cases. The present study was conducted to describe the long-term EEG evolution in a larger AIC cohort, followed for up to 23 years, and identify possible early predictors of the clinical and EEG outcomes.In a retrospective study, two experienced clinical neurophysiologists systematically reviewed all EEG traces recorded in 12 AIC cases throughout their follow-up, from epilepsy onset to the present. Clinical outcome was assessed with standardized clinical outcome scales.Analysis of the data revealed two distinct AIC phenotypes. In addition to the "classical severe phenotype" already described in the literature, we identified a new "mild phenotype". The two phenotypes show completely different EEG features at onset of epilepsy and during its evolution, which correspond to different clinical outcomes.Data from our long-term EEG and clinical-neuroradiological study allowed us to describe two different phenotypes of AIC, with different imaging severity and, in particular, different EEG at onset, which tend to remain constant over time.Together, these findings might help to predict long-term clinical outcomes.

Published
2022

32. Long-Term Outcomes After Atrial Switch Operation for Transposition of the Great Arteries

Author

Craig S. Broberg, Alexandra C. van Dissel, Jessica Minnier, Jamil Aboulhosn, Robert M. Kauling, Salil Ginde, Eric V. Krieger, Fred Rodriguez, Tripti Gupta, Sangeeta Shah, Anitha S. John, Timothy Cotts, W. Aaron Kay, Marissa Kuo, Cindy Dwight, Patricia Woods, Jeremy Nicolarsen, Berardo Sarubbi, Flavia Fusco, Petra Antonova, Susan Fernandes, Jasmine Grewal, Jonathan Cramer, Paul Khairy, Pastora Gallego, Clare O’Donnell, Jane Hannah, Mikael Dellborg, Carla P. Rodriguez-Monserrate, Isabelle Vonder Muhll, Stephen Pylypchuk, Anthony Magalski, Frank Han, Adam M. Lubert, Joseph Kay, Elizabeth Yeung, Jolien Roos-Hesselink, David Baker, David S. Celermajer, Luke J. Burchill, William M. Wilson, Joshua Wong, Shelby Kutty, Alexander R. Opotowsky, Cardiology, Graduate School, ACS - Heart failure & arrhythmias, and ACS - Pulmonary hypertension & thrombosis

Subjects
Adult, Heart Failure, Male, transposition of the great arteries, QRS duration, Transposition of Great Vessels, atrial arrhythmia, Arteries, congenital heart disease, Arterial Switch Operation, Treatment Outcome, congestive heart failure, systemic right ventricle, Humans, Female, Cardiology and Cardiovascular Medicine, Follow-Up Studies, Retrospective Studies
Abstract

Background: For patients with d-loop transposition of the great arteries (d-TGA) with a systemic right ventricle after an atrial switch operation, there is a need to identify risks for end-stage heart failure outcomes. Objectives: The authors aimed to determine factors associated with survival in a large cohort of such individuals. Methods: This multicenter, retrospective cohort study included adults with d-TGA and prior atrial switch surgery seen at a congenital heart center. Clinical data from initial and most recent visits were obtained. The composite primary outcome was death, transplantation, or mechanical circulatory support (MCS). Results: From 1,168 patients (38% female, age at first visit 29 ± 7.2 years) during a median 9.2 years of follow-up, 91 (8.8% per 10 person-years) met the outcome (66 deaths, 19 transplantations, 6 MCS). Patients experiencing sudden/arrhythmic death were younger than those dying of other causes (32.6 ± 6.4 years vs 42.4 ± 6.8 years; P < 0.001). There was a long duration between sentinel clinical events and end-stage heart failure. Age, atrial arrhythmia, pacemaker, biventricular enlargement, systolic dysfunction, and tricuspid regurgitation were all associated with the primary outcome. Independent 5-year predictors of primary outcome were prior ventricular arrhythmia, heart failure admission, complex anatomy, QRS duration >120 ms, and severe right ventricle dysfunction based on echocardiography. Conclusions: For most adults with d-TGA after atrial switch, progress to end-stage heart failure or death is slow. A simplified prediction score for 5-year adverse outcome is derived to help identify those at greatest risk.

Published
2022

33. Potential pathophysiologic mechanisms underlying the inherent risk of cancer in patients with atherosclerotic cardiovascular disease

Author

Stefania Angela, Di Fusco, Cinzia, Cianfrocca, Irma, Bisceglia, Antonella, Spinelli, Alessandro, Alonzo, Edoardo, Mocini, Michele Massimo, Gulizia, Domenico, Gabrielli, Fabrizio, Oliva, Giuseppe, Imperoli, and Furio, Colivicchi

Subjects
Inflammation, Cardiovascular Diseases, Neoplasms, Myocardial Infarction, Humans, Atherosclerosis, Cardiology and Cardiovascular Medicine
Abstract

Emerging evidence demonstrates an intimate interplay between cardiovascular disease and cancer pathophysiology. The aim of this review is to shed light on the common biological pathways underlying cardiovascular disease and cancer. These common pathways form the basis of "reverse cardio-oncology". We focus on the role of inflammation, stress response, cell proliferation, angiogenesis and tissue remodeling, neurohormonal system activation, and genomic instability as pathogenic pathways shared by cardiovascular disease and cancer. We also discuss shared mediators that may have a potential role as biomarkers for risk prediction in both diseases. Furthermore, we highlight current knowledge on biological pathways and mediators that are upregulated in diabetes and myocardial infarction and may be involved in tumorigenesis. On the basis of the shared pathophysiologic mechanisms, we also suggest an integrated approach to reduce the global burden of both cardiovascular disease and cancer.

Published
2022

34. The Effect of an Accelerated Renal Replacement Therapy Initiation Is Not Modified by Baseline Risk

Author

Federico Angriman, Bruno L. Ferreyro, Natalia Angeloni, Bruno R. da Costa, Ron Wald, Sean M. Bagshaw, Neill K.J. Adhikari, Rinaldo Bellomo, Didier Dreyfuss, Bin Du, Martin P. Gallagher, Stéphane Gaudry, François Lamontagne, Michael Joannidis, Kathleen D. Liu, Daniel F. McAuley, Shay P. McGuinness, Alistair D. Nichol, Marlies Ostermann, Paul M. Palevsky, Haibo Qiu, Ville Pettilä, Antoine G. Schneider, Orla M. Smith, Suvi T. Vaara, Matthew Weir, Glenn M. Eastwood, Leah Peck, Helen Young, Peter Kruger, Gordon Laurie, Emma Saylor, Jason Meyer, Ellen Venz, Krista Wetzig, Craig French, Forbes McGain, John Mulder, Gerard Fennessy, Sathyajith Koottayi, Samantha Bates, Miriam Towns, Rebecca Morgan, Anna Tippett, Andrew Udy, Chris Mason, Elisa Licari, Dashiell Gantner, Jason McClure, Alistair Nichol, Phoebe McCracken, Jasmin Board, Emma Martin, Shirley Vallance, Meredith Young, Chelsey Vladic, Steve McGloughlin, David Gattas, Heidi Buhr, Jennifer Coles, Debra Hutch, James Wun, Louise Cole, Christina Whitehead, Julie Lowrey, Kristy Masters, Rebecca Gresham, Victoria Campbell, David Gutierrez, Jane Brailsford, Loretta Forbes, Lauren Murray, Teena Maguire, Martina NiChonghaile, Neil Orford, Allison Bone, Tania Elderkin, Tania Salerno, Tim Chimunda, Jason Fletcher, Emma Broadfield, Sanjay Porwal, Cameron Knott, Catherine Boschert, Julie Smith, Angus Richardson, Dianne Hill, Graeme Duke, Peter Oziemski, Santiago Cegarra, Peter Chan, Deborah Welsh, Stephanie Hunter, Owen Roodenburg, John Dyett, Nicos Kokotsis, Max Moser, Yang Yang, Laven Padayachee, Joseph Vetro, Himangsu Gangopadhyay, Melissa Kaufman, Angaj Ghosh, Simone Said, Alpesh Patel, Shailesh Bihari, Elisha Matheson, Xia Jin, Tapaswi Shrestha, Kate Schwartz, Rosalba Cross, Winston Cheung, Helen Wong, Mark Kol, Asim Shah, Amanda Y. Wang, Zoltan Endre, Celia Bradford, Pierre Janin, Simon Finfer, Naomi Diel, Jonathan Gatward, Naomi Hammond, Anthony Delaney, Frances Bass, Elizabeth Yarad, Hergen Buscher, Claire Reynolds, Nerilee Baker, Romuald Bellmann, Andreas Peer, Julia Hasslacher, Paul Koglberger, Sebastian Klein, Klemens Zotter, Anna Brandtner, Armin Finkenstedt, Adelheid Ditlbacher, Frank Hartig, Dietmar Fries, Mirjam Bachler, Bettina Schenk, Martin Wagner, Philipp Eller, Thomas Staudinger, Esther Tiller, Peter Schellongowski, Andja Bojic, Eric A. Hoste, Stephanie Bracke, Luc De Crop, Daisy Vermeiren, Fernando Thome, Bianca Chiella, Lucia Fendt, Veronica Antunes, Frédérick D'Aragon, Charles St-Arnaud, Michael Mayette, Élaine Carbonnaeu, Joannie Marchand, Marie-Hélène Masse, Marilène Ladouceur, Alexis F. Turgeon, François Lauzier, David Bellemare, Charles Langis Francoeur, Guillaume LeBlanc, Gabrielle Guilbault, Stéphanie Grenier, Eve Cloutier, Annick Boivin, Charles Delisle-Thibault, Panagiota Giannakouros, Olivier Costerousse, Jean-François Cailhier, François-Martin Carrier, Ali Ghamraoui, Martine Lebrasseur, Fatna Benettaib, Maya Salamé, Dounia Boumahni, Ying Tung Sia, Jean-François Naud, Isabelle Roy, Henry T. Stelfox, Stacey Ruddell, Braden J. Manns, Shelley Duggan, Dominic Carney, Jennifer Barchard, Richard P. Whitlock, Emilie Belley-Cote, Nevena Savija, Alexandra Sabev, Troy Campbell, Thais Creary, Kelson Devereaux, Shira Brodutch, Claudio Rigatto, Bojan Paunovic, Owen Mooney, Anna Glybina, Oksana Harasemiw, Michelle Di Nella, John Harmon, Navdeep Mehta, Louis Lakatos, Nicole Haslam, Francois Lellouche, Mathieu Simon, Ying Tung, Patricia Lizotte, Pierre-Alexandre Bourchard, Bram Rochwerg, Tim Karachi, Tina Millen, John Muscedere, David Maslove, J. Gordon Boyd, Stephanie Sibley, John Drover, Miranda Hunt, Ilinca Georgescu, Randy Wax, Ilan Lenga, Kavita Sridhar, Andrew Steele, Kelly Fusco, Taneera Ghate, Michael Tolibas, Holly Robinson, Matthew A. Weir, Ravi Taneja, Ian M. Ball, Amit Garg, Eileen Campbell, Athena Ovsenek, Sean van Diepen, Nadia Baig, Sheldon Magder, Han Yao, Ahsan Alam, Josie Campisi, Erika MacIntyre, Ella Rokosh, Kimberly Scherr, Stephen Lapinsky, Sangeeta Mehta, Sumesh Shah, Daniel J. Niven, Michael Russell, Kym Jim, Gillian Brown, Kerry Oxtoby, Adam Hall, Luc Benoit, Colleen Sokolowski, Bhanu Prasad, Jag Rao, Shelley Giebel, Demetrios J. Kutsogiannis, Patricia Thompson, Tayne Thompson, Robert Cirone, Kanthi Kavikondala, Mark Soth, France Clarke, Alyson Takaoka, David Mazer, Karen Burns, Jan Friedrich, David Klein, Gyan Sandhu, Marlene Santos, Imrana Khalid, Jennifer Hodder, Peter Dodek, Najib Ayas, Victoria Alcuaz, Gabriel Suen, Oleksa Rewa, Gurmeet Singh, Sean Norris, Neil Gibson, Castro Arias, Aysha Shami, Celine Pelletier, Alireza Zahirieh, Andre Amaral, Nicole Marinoff, Navjot Kaur, Adic Perez, Jane Wang, Gregory Haljan, Christopher Condin, Lauralyn McIntyre, Brigette Gomes, Rebecca Porteous, Irene Watpool, Swapnil Hiremath, Edward Clark, Margaret S. Herridge, Felicity Backhouse, M. Elizabeth Wilcox, Karolina Walczak, Vincent Ki, Asheer Sharman, Martin Romano, R.T. Noel Gibney, Adam S. Romanovsky, Lorena McCoshen, Gordon Wood, Daniel Ovakim, Fiona Auld, Gayle Carney, Meili Duan, Xiaojun Ji, Dongchen Guo, Zhili Qi, Jin Lin, Meng Zhang, Lei Dong, Jingfeng Liu, Pei Liu, Deyuan Zhi, Guoqiang Bai, Yu Qiu, Ziqi Yang, Jing Bai, Zhuang Liu, Haizhou Zhuang, Haiman Wang, Jian Li, Mengya Zhao, Xiao Zhou, Xianqing Shi, Baning Ye, Manli Liu, Jing Wu, Yongjian Fu, Dali Long, Yu Pan, Jinlong Wang, Huaxian Mei, Songsong Zhang, Mingxiang Wen, Enyu Yang, Sijie Mu, Jianquan Li, Tingting Hu, Bingyu Qin, Min Li, Cunzhen Wang, Xin Dong, Kaiwu Wang, Haibo Wang, Jianxu Yang, Chuanyao Wang, Dongxin Wang, Nan Li, Zhui Yu, Song Xu, Lan Yao, Guo Hou, Zhou Liu, Liping Lu, Yingtao Lian, Chunting Wang, Jichen Zhang, Ruiqi Ding, Guoqing Qi, Qizhi Wang, Peng Wang, Zhaoli Meng, Man Chen, Xiaobo Hu, Xiandi He, Shibing Zhao, Lele Hang, Rui Li, Suhui Qin, Kun Lu, Shijuan Dun, Cheng Liu, Qi Zhou, Zhenzhen Chen, Jing Mei, Minwei Zhang, Hao Xu, Jincan Lin, Qindong Shi, Lijuan Fu, Qinjing Zeng, Hongye Ma, Jinqi Yan, Lan Gao, Hongjuan Liu, Lei Zhang, Hao Li, Xiaona He, Jingqun Fan, Litao Guo, Yu Liu, Xue Wang, Jingjing Sun, Zhongmin Liu, Juan Yang, Lili Ding, Lulu Sheng, Xingang Liu, Jie Yan, Quihui Wang, Yifeng Wang, Dan Zhao, Shuangping Zhao, Chenghuan Hu, Jing Li, Fuxing Deng, Haibo Qui, Yi Yang, Min Mo, Chun Pan, Changde Wu, Yingzi Huang, Lili Huang, Airan Liu, Anna-Maija Korhonen, Sanna Törnblom, Sari Sutinen, Leena Pettilä, Jonna Heinonen, Eliria Lappi, Taria Suhonen, Sari Karlsson, Sanna Hoppu, Ville Jalkanen, Anne Kuitunen, Markus Levoranta, Jaakko Långsjö, Sanna Ristimäki, Kaisa Malila, Anna Wootten, Simo Varila, Mikko J Järvisalo, Outi Inkinen, Satu Kentala, Keijo Leivo, Paivi Haltia, Jean-Damien Ricard, Jonathan Messika, Abirami Tiagarajah, Malo Emery, Aline Dechanet, Coralie Gernez, Damien Roux, Laurent Martin-Lefevre, Maud Fiancette, Isabelle Vinatier, Jean Claude Lacherade, Gwenhaël Colin, Christine Lebert, Marie-Ange Azais, Aihem Yehia, Caroline Pouplet, Matthieu Henry- Lagarrigue, Amélie Seguin, Laura Crosby, Julien Maizel, Dimitri Titeca-Beauport, Alain Combes, Ania Nieszkowska, Paul Masi, Alexandre Demoule, Julien Mayaux, Martin Dres, Elise Morawiec, Maxens Decalvele, Suela Demiri, Morgane Faure, Clémence Marios, Maxime Mallet, Marie Amélie Ordon, Laura Morizot, Marie Cantien, François Pousset, Florent Poirson, Yves Cohen, Laurent Argaud, Martin Cour, Laurent Bitker, Marie Simon, Romain Hernu, Thomas Baudry, Sylvie De La Salle, Adrien Robine, Nicholas Sedillot, Xavier Tchenio, Camille Bouisse, Sylvie Roux, Fabienne Tamion, Steven Grangé, Dorothée Carpentier, Guillaume Chevrel, Luis Ensenyat-Martin, Sophie Marque, Jean-Pierre Quenot, Pascal Andreu, Auguste Dargent, Audrey Large, Nicolas Chudeau, Mickael Landais, Benoit Derrien, Jean Christophe Callahan, Christophe Guitton, Charlène Le Moal, Alain Robert, Karim Asehnoune, Raphaël Cinotti, Nicolas Grillot, Dominique Demeure, Christophe Vinsonneau, Imen Rahmani, Mehdi Marzouk, Thibault Dekeyser, Caroline Sejourne, Mélanie Verlay, Fabienne Thevenin, Lucie Delecolle, Didier Thevenin, Bertrand Souweine, Elisabeth Coupez, Mireille Adda, Jean-Pierre Eraldi, Antoine Marchalot, Nicolas De Prost, Armand Mekontso Dessap, Keyvan Razazi, Ferhat Meziani, Julie Boisrame-Helms, Raphael Clere-Jehl, Xavier Delabranche, Christine Kummerlen, Hamid Merdji, Alexandra Monnier, Yannick Rabouel, Hassene Rahmani, Hayat Allam, Samir Chenaf, Vincenta Franja, Bertrand Pons, Michel Carles, Frédéric Martino, Régine Richard, Benjamin Zuber, Guillaume Lacave, Karim Lakhal, Bertrand Rozec, Hoa Dang Van, Éric Boulet, Fouad Fadel, Cedric Cleophax, Nicolas Dufour, Caroline Grant, Marie Thuong, Jean Reignier, Emmanuel Canet, Laurent Nicolet, Thierry Boulain, Mai-Anh Nay, Dalila Benzekri, François Barbier, Anne Bretagnol, Toufik Kamel, Armelle Mathonnet, Grégoire Muller, Marie Skarzynski, Julie Rossi, Amandine Pradet, Sandra Dos Santos, Aurore Guery, Lucie Muller, Luis Felix, Julien Bohé, Guillaume Thiéry, Nadia Aissaoui, Damien Vimpere, Morgane Commeureuc, Jean-Luc Diehl, Emmanuel Guerot, Orfeas Liangos, Monika Wittig, Alexander Zarbock, Mira Küllmar, Thomas van Waegeningh, Nadine Rosenow, Kathy Brickell, Peter Doran, Patrick T. Murray, Giovanni Landoni, Rosalba Lembo, Alberto Zangrillo, Giacomo Monti, Margherita Tozzi, Matteo Marzaroli, Gaetano Lombardi, Gianluca Paternoster, Michelangelo Vitiello, Shay McGuinness, Rachael Parke, Magdalena Butler, Eileen Gilder, Keri-Anne Cowdrey, Samantha Wallace, Jane Hallion, Melissa Woolett, Philippa Neal, Karina Duffy, Stephanie Long, Colin McArthur, Catherine Simmonds, Yan Chen, Rachael McConnochie, Lynette Newby, David Knight, Seton Henderson, Jan Mehrtens, Stacey Morgan, Anna Morris, Kymbalee Vander Hayden, Tara Burke, Matthew Bailey, Ross Freebairn, Lesley Chadwick, Penelope Park, Christine Rolls, Liz Thomas, Ulrike Buehner, Erin Williams, Jonathan Albrett, Simon Kirkham, Carolyn Jackson, Troy Browne, Jennifer Goodson, David Jackson, James Houghton, Owen Callender, Vicki Higson, Owen Keet, Clive Dominy, Paul Young, Anna Hunt, Harriet Judd, Cassie Lawrence, Shaanti Olatunji, Yvonne Robertson, Charlotte Latimer-Bell, Deborah Hendry, Agnes Mckay-Vucago, Nina Beehre, Eden Lesona, Leanlove Navarra, Chelsea Robinson, Ryan Jang, Andrea Junge, Bridget Lambert, Michel Thibault, Philippe Eckert, Sébastien Kissling, Erietta Polychronopoulos, Elettra Poli, Marco Altarelli, Madeleine Schnorf, Samia Abed Mallaird, Claudia Heidegger, Aurelie Perret, Philippe Montillier, Frederic Sangla, Seigenthaller Neils, Aude De Watteville, Mandeep-Kaur Phull, Aparna George, Nauman Hussain, Tatiana Pogreban, Steve Lobaz, Alison Daniels, Mishell Cunningham, Deborah Kerr, Alice Nicholson, Pradeep Shanmugasundaram, Judith Abrams, Katarina Manso, Geraldine Hambrook, Elizabeth McKerrow, Juvy Salva, Stephen Foulkes, Matthew Wise, Matt Morgan, Jenny Brooks, Jade Cole, Tracy Michelle Davies, Helen Hill, Emma Thomas, Marcela Vizcaychipi, Behrad Baharlo, Jaime Carungcong, Patricia Costa, Laura Martins, Ritoo Kapoor, Tracy Hazelton, Angela Moon, Janine Musselwhite, Ben Shelley, Philip McCall, Gill Arbane, Aneta Bociek, Martina Marotti, Rosario Lim, Sara Campos, Neus Grau Novellas, Armando Cennamo, Andrew Slack, Duncan Wyncoll, Luigi Camporota, Simon Sparkes, Rosalinde Tilley, Austin Rattray, Gayle Moreland, Jane Duffy, Elizabeth McGonigal, Philip Hopkins, Clare Finney, John Smith, Harriet Noble, Hayley Watson, Claire-Louise Harris, Emma Clarey, Eleanor Corcoran, James Beck, Clare Howcroft, Nora Youngs, Elizabeth Wilby, Bethan Ogg, Adam Wolverson, Sandra Lee, Susie Butler, Maryanne Okubanjo, Julia Hindle, Ingeborg Welters, Karen Williams, Emily Johnson, Julie Patrick-Heselton, David Shaw, Victoria Waugh, Richard Stewart, Esther Mwaura, Lynn Wren, Louise Mew, Sara-Beth Sutherland, Jane Adderley, Jim Ruddy, Margaret Harkins, Callum Kaye, Teresa Scott, Wendy Mitchell, Felicity Anderson, Fiona Willox, Vijay Jagannathan, Michele Clark, Sarah Purv, Andrew Sharman, Megan Meredith, Lucy Ryan, Louise Conner, Cecilia Peters, Dan Harvey, Ashraf Roshdy, Amy Collins, Malcolm Sim, Steven Henderson, Nigel Chee, Sally Pitts, Katie Bowman, Maria Dilawershah, Luke Vamplew, Elizabeth Howe, Paula Rogers, Clara Hernandez, Clara Prendergast, Jane Benton, Alex Rosenberg, Lui G. Forni, Alice Grant, Paula Carvelli, Ajay Raithatha, Sarah Bird, Max Richardson, Matthew Needham, Claire Hirst, Jonathan Ball, Susannah Leaver, Luisa Howlett, Carlos Castro Delgado, Sarah Farnell-Ward, Helen Farrah, Geraldine Gray, Gipsy Joseph, Francesca Robinson, Ascanio Tridente, Clare Harrop, Karen Shuker, Derek McLaughlan, Judith Ramsey, Sharon Meehan, Bernd Oliver Rose, Rosie Reece-Anthony, Babita Gurung, Tony Whitehouse, Catherine Snelson, Tonny Veenith, Andy Johnston, Lauren Cooper, Ron Carrera, Karen Ellis, Emma Fellows, Samanth Harkett, Colin Bergin, Elaine Spruce, Liesl Despy, Stephanie Goundry, Natalie Dooley, Tracy Mason, Amy Clark, Gemma Dignam, Geraldine Ward, Ben Attwood, Penny Parsons, Sophie Mason, Michael Margarson, Jenny Lord, Philip McGlone, Luke E. Hodgson, Indra Chadbourn, Raquel Gomez, Jordi Margalef, Rinus Pretorius, Alexandra Hamshere, Joseph Carter, Hazel Cahill, Lia Grainger, Kate Howard, Greg Forshaw, Zoe Guy, Kianoush B. Kashani, Robert C. Albright, Amy Amsbaugh, Anita Stoltenberg, Alexander S. Niven, Matthew Lynch, AnnMarie O'Mara, Syed Naeem, Sairah Sharif, Joyce McKenney Goulart, Ashita Tolwani, Claretha Lyas, Laura Latta, Azra Bihorac, Haleh Hashemighouchani, Philip Efron, Matthew Ruppert, Julie Cupka, Sean Kiley, Joshua Carson, Peggy White, George Omalay, Sherry Brown, Laura Velez, Alina Marceron, Javier A. Neyra, Juan Carlos Aycinena, Madona Elias, Victor M. Ortiz-Soriano, Caroline Hauschild, and Robert Dorfman

Subjects
Renal Replacement Therapy, Pulmonary and Respiratory Medicine, Critical Illness, Medicine and Health Sciences, Humans, Acute Kidney Injury
Published
2022

35. Impact of Circadian Desynchrony on Spermatogenesis: A Mini Review

Author

Ferdinando Fusco, Nicola Longo, Marco De Sio, Davide Arcaniolo, Giuseppe Celentano, Marco Capece, Roberto La Rocca, Francesco Mangiapia, Gianluigi Califano, Simone Morra, Carmine Turco, Gianluca Spena, Lorenzo Spirito, Giovanni Maria Fusco, Luigi Cirillo, Luigi De Luca, Luigi Napolitano, Vincenzo Mirone, Massimiliano Creta, Fusco, Ferdinando, Longo, Nicola, De Sio, Marco, Arcaniolo, Davide, Celentano, Giuseppe, Capece, Marco, La Rocca, Roberto, Mangiapia, Francesco, Califano, Gianluigi, Morra, Simone, Turco, Carmine, Spena, Gianluca, Spirito, Lorenzo, Fusco, Giovanni Maria, Cirillo, Luigi, De Luca, Luigi, Napolitano, Luigi, Mirone, Vincenzo, Creta, Massimiliano, Fusco, F., Longo, N., De Sio, M., Arcaniolo, D., Celentano, G., Capece, M., La Rocca, R., Mangiapia, F., Califano, G., Morra, S., Turco, C., Spena, G., Spirito, L., Fusco, G. M., Cirillo, L., De Luca, L., Napolitano, L., Mirone, V., and Creta, M.

Subjects
Male, circadian rhythm, fertility, Mini Review, Endocrinology, Diabetes and Metabolism, clock gene, circadian desynchrony, Chronobiology Disorders, RC648-665, spermatogenesis, Diseases of the endocrine glands. Clinical endocrinology, circadian desynchrony, circadian rhythms, clock genes, spermatogenesis, fertility, Semen Analysis, Endocrinology, circadian rhythms, clock genes, Animals, Humans, Gonadal Steroid Hormones, Infertility, Male
Abstract

The purpose of this mini review is to provide data about pre-clinical and clinical evidence exploring the impact of circadian desynchrony on spermatogenesis. Several lines of evidence exist demonstrating that disruption of circadian rhythms may interfere with male fertility. Experimental knock-out or knock-down of clock genes, physiologically involved in the regulation of circadian rhythms, are associated with impairments of fertility pathways in both animal and human models. Moreover, disruption of circadian rhythms, due to reduction of sleep duration and/or alteration of its architecture can negatively interfere in humans with circulating levels of male sexual hormones as well as with semen parameters. Unfortunately, current evidence remains low due to study heterogeneity.

Published
2021

36. Hyperbaric oxygen therapy as adjuvant treatment for surgical site infections after male-to-female gender affirmation surgery: A 10-year experience

Author

Marco Stizzo, Celeste Manfredi, Lorenzo Spirito, Carmine Sciorio, Javier Romero Otero, Juan Ignacio Martinez Salamanca, Felice Crocetto, Paolo Verze, Ciro Imbimbo, Ferdinando Fusco, Marco De Sio, Davide Arcaniolo, Stizzo, Marco, Manfredi, Celeste, Spirito, Lorenzo, Sciorio, Carmine, Romero Otero, Javier, Martinez Salamanca, Juan Ignacio, Crocetto, Felice, Verze, Paolo, Imbimbo, Ciro, Fusco, Ferdinando, De Sio, Marco, Arcaniolo, Davide, Stizzo, M., Manfredi, C., Spirito, L., Sciorio, C., Romero Otero, J., Martinez Salamanca, J. I., Crocetto, F., Verze, P., Imbimbo, C., Fusco, F., De Sio, M., and Arcaniolo, D.

Subjects
Male, vaginoplasty, Urology, Endocrinology, Diabetes and Metabolism, hyperbaric oxygen therapy, transgender, gender affirmation surgery, infection, male-to-female, Anti-Bacterial Agents, Oxygen, Endocrinology, Reproductive Medicine, Sex Reassignment Surgery, Humans, Surgical Wound Infection, Female, Retrospective Studies
Abstract

Introduction: Several urological conditions that share an impairment of tissue oxygenation can benefit from hyperbaric oxygen therapy; limited evidence is available of its impact on patients undergoing male-to-female gender affirmation surgery. Aim: To evaluate the efficacy and safety of hyperbaric oxygen therapy as adjuvant treatment for surgical site infections in patients undergoing male-to-female gender affirmation surgery. Methods: This research was conducted as an observational retrospective study. Patients undergoing male-to-female gender affirmation surgery at the principal investigators’ institution from January 2009 to September 2019, with a discharge diagnosis of complicated superficial or deep wound infections, were included. All patients underwent standard management of wound infection. Subjects received/not-received hyperbaric oxygen therapy at the surgeon's discretion and were assigned to the hyperbaric oxygen therapy versus non-hyperbaric oxygen therapy group accordingly. Complete wound healing rate (primary outcome), duration of antibiotic therapy, perineal drain time, bladder catheter time, and hospital stay were recorded. All adverse events that occurred during the study period were described. Results: A total of 156 patients underwent male-to-female gender affirmation surgery in the study period. Thirty-three patients were enrolled. Fifteen subjects belonged to the hyperbaric oxygen therapy group, the other 18 to the non-hyperbaric oxygen therapy group. No statistically significant differences were found between the two groups at baseline. Penile inversion vaginoplasty and intestinal vaginoplasty were performed in nine (60%) and six (40%) patients of the hyperbaric oxygen therapy group. Only penile inversion vaginoplasty was performed in subjects of the non-hyperbaric oxygen therapy group. Complete wound healing was obtained in 15 patients (100%) of the hyperbaric oxygen therapy group and 17 patients (94.4%) of the non-hyperbaric oxygen therapy group (p=0.35). Duration of antibiotic therapy, perineal drain time, bladder catheter time, and hospital stay were significantly lower in the hyperbaric oxygen therapy group (p&nbsp

Published
2022

37. Diagnosis and Management of Cardiovascular Involvement in Friedreich Ataxia

Author

Martina Caiazza, Adelaide Fusco, Silvia Passantino, Francesco Di Fraia, Alfredo Mauriello, Federica Amodio, Annapaola Cirillo, Michele Lioncino, Francesco Natale, Silvia Favilli, Fabio Fimiani, Giuseppe Limongelli, Federica Verrillo, Nunzia Borrelli, Emanuele Monda, Marta Rubino, Francesca Girolami, Gioacchino Scarano, Monda, E., Lioncino, M., Rubino, M., Passantino, S., Verrillo, F., Caiazza, M., Cirillo, A., Fusco, A., Di Fraia, F., Fimiani, F., Amodio, F., Borrelli, N., Mauriello, A., Natale, F., Scarano, G., Girolami, F., Favilli, S., and Limongelli, G.

Subjects
medicine.medical_specialty, Ataxia, Cardiomyopathy, Left ventricular hypertrophy, Asymptomatic, Ventricular Function, Left, Internal medicine, Humans, Medicine, Ejection fraction, biology, business.industry, Hypertrophic cardiomyopathy, Stroke Volume, General Medicine, medicine.disease, Friedreich ataxia, Heart failure, Frataxin, biology.protein, Cardiology, Therapy, medicine.symptom, Cardiomyopathies, Trinucleotide Repeat Expansion, Cardiology and Cardiovascular Medicine, business, Diagnosi
Abstract

Friedreich ataxia (FRDA) is an autosomal recessive neurodegenerative disorder caused by a homozygous GAA triplet repeat expansion in the frataxin gene. Cardiac involvement, usually manifesting as hypertrophic cardiomyopathy, can range from asymptomatic cases to severe cardiomyopathy with progressive deterioration of the left ventricular ejection fraction and chronic heart failure. The management of cardiac involvement is directed to prevent disease progression and cardiovascular complications. However, direct-disease therapies are not currently available for FRDA. The present review aims to describe the current state of knowledge regarding cardiovascular involvement of FRDA, focusing on clinical-instrumental features and management of cardiac manifestation.

Published
2022

38. Diagnosis and Management of Cardiovascular Involvement in Fabry Disease

Author

Adelaide Fusco, Eduardo Bossone, Emanuele Monda, Marta Rubino, Laura Capodicasa, Elena Biagini, Paolo Orabona, Francesca Dongiglio, Giuseppe Limongelli, Marialuisa Mazzella, Maurizio Pieroni, Dominique P. Germain, Martina Caiazza, Annapaola Cirillo, Giuseppe Palmiero, Antonio Pisani, Flavia Chiosi, Michele Lioncino, Paolo Calabrò, Arturo Cesaro, Rubino, M., Monda, E., Lioncino, M., Caiazza, M., Palmiero, G., Dongiglio, F., Fusco, A., Cirillo, A., Cesaro, A., Capodicasa, L., Mazzella, M., Chiosi, F., Orabona, P., Bossone, E., Calabro, P., Pisani, A., Germain, D. P., Biagini, E., Pieroni, M., and Limongelli, G.

Subjects
medicine.medical_specialty, medicine.medical_treatment, Left ventricular hypertrophy, Targeted therapy, Quality of life, Fibrosis, Internal medicine, medicine, Lysosomal storage disease, Humans, Enzyme Replacement Therapy, business.industry, Hypertrophic cardiomyopathy, General Medicine, Cardiomyopathy, Hypertrophic, medicine.disease, Fabry disease, Heart failure, Quality of Life, Cardiology, Fabry Disease, Hypertrophy, Left Ventricular, Therapy, Cardiology and Cardiovascular Medicine, business, Diagnosi
Abstract

Fabry disease (FD, OMIM 301500) is an X-linked lysosomal storage disease caused by pathogenic variants in the GLA gene. Cardiac involvement is common in FD and is responsible for impaired quality of life and premature death. The classic cardiac involvement is a nonobstructive form of hypertrophic cardiomyopathy, usually manifesting as concentric left ventricular hypertrophy, with subsequent arrhythmogenic intramural fibrosis. Treatment of patients with FD should be directed to prevent the disease progression to irreversible organ damage and organ failure. The aim of this review is to describe the current state of knowledge regarding cardiovascular involvement in FD, focusing on clinical and instrumental features, cardiovascular management, and targeted therapy.

Published
2022

39. The Heart Muscle and Valve Involvement in Marfan Syndrome, Loeys-Dietz Syndromes, and Collagenopathies

Author

Gioacchino Scarano, Francesco Natale, Giuseppe Palmiero, Guglielmina Pepe, Antonello Credendino, Adelaide Fusco, Giuseppe Limongelli, Alessandro Della Corte, Martina Caiazza, Giovanni Signore, Alfredo Mauriello, Chiara Granato, Flavia Chiosi, Michele Lioncino, Maria Giovanna Russo, Annapaola Cirillo, Fiorella Fratta, Emanuele Monda, Stefano Nistri, Fusco, A., Mauriello, A., Lioncino, M., Palmiero, G., Fratta, F., Granato, C., Cirillo, A., Caiazza, M., Monda, E., Credendino, A., Signore, G., Natale, F., Chiosi, F., Scarano, G., Della Corte, A., Nistri, S., Russo, M. G., Limongelli, G., and Pepe, G.

Subjects
Marfan syndrome, Pathology, medicine.medical_specialty, Fibrillinopathie, Connective tissue, Loeys–Dietz syndrome, Cardiomyopathy: mitral valve prolapse, Marfan Syndrome, medicine, Humans, Mitral valve prolapse, Ectopia lentis, Collagenophatie, Aortic dissection, Loeys-Dietz Syndrome, business.industry, Myocardium, TGFbetapathies, General Medicine, medicine.disease, medicine.anatomical_structure, Ehlers–Danlos syndrome, Heart failure, Ehlers-Danlos syndrome, Cardiology and Cardiovascular Medicine, business
Abstract

The inherited connective tissue disorders (Marfan syndrome, Loeys-Dietz syndrome [LDS], and Ehlers-Danlos syndrome [EDS]) involve connective tissue of various organ systems. These pathologies share many common features, nonetheless compared to Marfan syndrome, LDS' cardiovascular manifestations tend to be more severe. In contrast, no association is reported between LDS and the presence of ectopia lentis. The EDS are currently classified into thirteen subtypes. There is substantial symptoms overlap between the EDS subtypes, and they are associated with an increased incidence of cardiovascular abnormalities, such as mitral valve prolapse and aortic dissection.

Published
2022

40. [Cardiology as seen through social media]

Author

Castelletti, Silvia, Di Fusco, Stefania Angela, Borrelli, Nunzia, Gragnano, Felice, Perone, Francesco, Colivicchi, Furio, Castelletti, Silvia, Di Fusco, Stefania Angela, Borrelli, Nunzia, Gragnano, Felice, Perone, Francesco, and Colivicchi, Furio

Subjects
Cardiology, Humans, COVID-19, Social Media, Pandemics, Cardiovascular System
Abstract

The use of social media (SoMe) has spread worldwide among doctors, scientific societies, and researchers. SoMe offer a powerful platform to accelerate or create new contacts, spread scientific news, and increase visibility. A social media promotion strategy for cardiovascular medicine papers seems to be associated with increased online visibility and a higher number of citations. This effect is independent of the type of article and the total number of followers of the authors. Indeed, SoMe are democratic and even non-senior researchers may be popular on Twitter: your title is not as important as what you do on Twitter. Nevertheless, some physicians may be over-celebrated due to their presence on SoMe. This is why a new author index, the K-index, has been proposed. The K-index correlates the citations of a scientist with the number of Twitter followers. Even scientific journals and societies have recognized the importance of SoMe and in the last years they have appeared on SoMe with official accounts. Therefore, besides the classic impact factor, publishers now pay more and more attention to other parameters, such as the Altmetric score, which takes into account the number of citations, but also the number of downloads, mentions on SoMe, newspapers and tv news, web sites, and blogs. The COVID-19 pandemic has boosted the importance of SoMe for scientific content distribution, particularly for congresses. For all these reasons, it is important to understand the pros and cons of SoMe. It is also possible that SoMe will become a new education medium for continuing medical education.

Published
2022

41. COVID-19 Lockdown

Author

Jad Adrian Washif, Øyvind Sandbakk, Stephen Seiler, Thomas Haugen, Abdulaziz Farooq, Ken Quarrie, Dina C. Janse van Rensburg, Isabel Krug, Evert Verhagen, Del P. Wong, Iñigo Mujika, Cristina Cortis, Monoem Haddad, Omid Ahmadian, Mahmood Al Jufaili, Ramzi A. Al-Horani, Abdulla Saeed Al-Mohannadi, Asma Aloui, Achraf Ammar, Fitim Arifi, Abdul Rashid Aziz, Mikhail Batuev, Christopher Martyn Beaven, Ralph Beneke, Arben Bici, Pallawi Bishnoi, Lone Bogwasi, Daniel Bok, Omar Boukhris, Daniel Boullosa, Nicola Bragazzi, Joao Brito, Roxana Paola Palacios Cartagena, Anis Chaouachi, Stephen S. Cheung, Hamdi Chtourou, Germina Cosma, Tadej Debevec, Matthew D. DeLang, Alexandre Dellal, Gürhan Dönmez, Tarak Driss, Juan David Peña Duque, Cristiano Eirale, Mohamed Elloumi, Carl Foster, Emerson Franchini, Andrea Fusco, Olivier Galy, Paul B. Gastin, Nicholas Gill, Olivier Girard, Cvita Gregov, Shona Halson, Omar Hammouda, Ivana Hanzlíková, Bahar Hassanmirzaei, Kim Hébert-Losier, Hussein Muñoz Helú, Tomás Herrera-Valenzuela, Florentina J. Hettinga, Louis Holtzhausen, Olivier Hue, Antonio Dello Iacono, Johanna K. Ihalainen, Carl James, Saju Joseph, Karim Kamoun, Mehdi Khaled, Karim Khalladi, Kwang Joon Kim, Lian-Yee Kok, Lewis MacMillan, Leonardo Jose Mataruna-Dos-Santos, Ryo Matsunaga, Shpresa Memishi, Grégoire P. Millet, Imen Moussa-Chamari, Danladi Ibrahim Musa, Hoang Minh Thuan Nguyen, Pantelis T. Nikolaidis, Adam Owen, Johnny Padulo, Jeffrey Cabayan Pagaduan, Nirmala Panagodage Perera, Jorge Pérez-Gómez, Lervasen Pillay, Arporn Popa, Avishkar Pudasaini, Alizera Rabbani, Tandiyo Rahayu, Mohamed Romdhani, Paul Salamh, Abu-Sufian Sarkar, Andy Schillinger, Heny Setyawati, Navina Shrestha, Fatona Suraya, Montassar Tabben, Khaled Trabelsi, Axel Urhausen, Maarit Valtonen, Johanna Weber, Rodney Whiteley, Adel Zrane, Yacine Zerguini, Piotr Zmijewski, Helmi Ben Saad, David B. Pyne, Lee Taylor, Karim Chamari, Public and occupational health, AMS - Sports, and APH - Health Behaviors & Chronic Diseases

Subjects
Male, urheilulajit, sukupuolierot, crowd-sourced data, multinational sample, online survey, perception, remote training, Physical Therapy, Sports Therapy and Rehabilitation, crowd-sourced data, multinational sample, online survey, perception, remote training, male, Surveys and Questionnaires, Humans, harjoittelu, Orthopedics and Sports Medicine, humans, PESQUISA, athletes, communicable disease control, female, surveys and questionnaires, COVID-19, sports, 1106 Human Movement and Sports Sciences, 1116 Medical Physiology, 1701 Psychology, kansainvälinen vertailu, C600, Athletes, poikkeusolot, Communicable Disease Control, Settore M-EDF/02 - Metodi e Didattiche delle Attivita' Sportive, Female, Sport Sciences, survey-tutkimus, Sports, urheilijat
Abstract

Purpose: To investigate differences in athletes’ knowledge, beliefs, and training practices during COVID-19 lockdowns with reference to sport classification and sex. This work extends an initial descriptive evaluation focusing on athlete classification. Methods: Athletes (12,526; 66% male; 142 countries) completed an online survey (May–July 2020) assessing knowledge, beliefs, and practices toward training. Sports were classified as team sports (45%), endurance (20%), power/technical (10%), combat (9%), aquatic (6%), recreational (4%), racquet (3%), precision (2%), parasports (1%), and others (1%). Further analysis by sex was performed. Results: During lockdown, athletes practiced body-weight-based exercises routinely (67% females and 64% males), ranging from 50% (precision) to 78% (parasports). More sport-specific technical skills were performed in combat, parasports, and precision (∼50%) than other sports (∼35%). Most athletes (range: 50% [parasports] to 75% [endurance]) performed cardiorespiratory training (trivial sex differences). Compared to prelockdown, perceived training intensity was reduced by 29% to 41%, depending on sport (largest decline: ∼38% in team sports, unaffected by sex). Some athletes (range: 7%–49%) maintained their training intensity for strength, endurance, speed, plyometric, change-of-direction, and technical training. Athletes who previously trained ≥5 sessions per week reduced their volume (range: 18%–28%) during lockdown. The proportion of athletes (81%) training ≥60 min/session reduced by 31% to 43% during lockdown. Males and females had comparable moderate levels of training knowledge (56% vs 58%) and beliefs/attitudes (54% vs 56%). Conclusions: Changes in athletes’ training practices were sport-specific, with few or no sex differences. Team-based sports were generally more susceptible to changes than individual sports. Policy makers should provide athletes with specific training arrangements and educational resources to facilitate remote and/or home-based training during lockdown-type events.

Published
2022

42. A new score for life-threatening ventricular arrhythmias and sudden cardiac death in adults with transposition of the great arteries and a systemic right ventricle

Author

Magalie Ladouceur, Alexander Van De Bruaene, Robert Kauling, Werner Budts, Jolien Roos-Hesselink, Sandra Villagrá Albert, Inmaculada Sanchez Perez, Berardo Sarubbi, Flavia Fusco, Pastora Gallego, Maria Jose Rodriguez-Puras, Judith Bouchardy, Coralie Blanche, Tobias Rutz, Katja Prokselj, Fabien Labombarda, Laurence Iserin, Tom Wong, Michael A Gatzoulis, Cardiology, Fédération Française de Cardiologie, and Hôpitaux de Paris

Subjects
Adult, Male, Adolescent, Heart Ventricles, Transposition of Great Vessels, Systemic right ventricle, Arrhythmias, Cardiac, Arteries, Sudden cardiac death, Young Adult, Death, Sudden, Cardiac, Transposition of the great arteries, Humans, Female, Cardiology and Cardiovascular Medicine
Abstract

[Aims] To investigate the incidence of major adverse ventricular arrhythmias and related events (MAREs) and to develop a stratification tool predicting MAREs in adults with a systemic right ventricle (sRV)., [Methods and results] In a multicentre approach, all adults (≥16 years old) with a sRV undergoing follow-up between 2000 and 2018 were identified. The incidence of MAREs, defined as sudden cardiac death, sustained ventricular tachycardia, and appropriate implantable cardioverter-defibrillator (ICD) therapy, was analysed. The association of MAREs with clinical, electrical, and echocardiographic parameters was evaluated. A total of 1184 patients (median age 27.1 years; interquartile range 19.9–34.9 years; 59% male; 70% with atrial switch repair for D-transposition of the great arteries) were included. The incidence of MAREs was 6.3 per 1000 patient-years. On multivariate analysis, age, history of heart failure, syncope, QRS duration, severe sRV dysfunction and at least moderate left ventricular outflow tract obstruction were retained in the final model with a C-index of 0.78 [95% confidence interval (CI) 0.72–0.83] and a calibration slope of 0.93 (95% CI 0.64–1.21). For every five ICDs implanted in patients with a 5-year MARE risk >10%, one patient may potentially be spared from a MARE., [Conclusion] Sudden cardiac death remains a devastating cause of death in a contemporary adult cohort with a sRV. A prediction model based on clinical, electrocardiographic, and echocardiographic parameters was devised to estimate MARE risk and to identify high-risk patients who may benefit from primary prevention ICD implantation., This work was supported by the Fédération Française de Cardiologie and the Assistance Publique des Hôpitaux de Paris.

Published
2022

43. Management of cutaneous melanoma: radiologists challenging and risk assessment

Author

Vincenza Granata, Igino Simonetti, Roberta Fusco, Sergio Venanzio Setola, Francesco Izzo, Luigi Scarpato, Vito Vanella, Lucia Festino, Ester Simeone, Paolo Antonio Ascierto, and Antonella Petrillo

Subjects
Skin Neoplasms, Positron Emission Tomography Computed Tomography, Radiologists, Humans, Radiology, Nuclear Medicine and imaging, General Medicine, Melanoma, Risk Assessment, Neoplasm Staging
Abstract

Melanoma patient remains a challenging for the radiologist, due to the difficulty related to the management of a patient more often in an advanced stage of the disease. It is necessary to determine a stratification of risk, optimizing the means, with diagnostic tools that should be optimized in relation to the type of patient, and improving knowledge. Staging and risk assessment procedures are determined by disease presentation at diagnosis. Melanoma staging is a critical tool to assist clinical decision-making and prognostic assessment. It is used for clinical trial design, eligibility, stratification, and analysis. The current standard for regional lymph nodes staging is represented by the sentinel lymph node excision biopsy procedure. For staging of distant metastases, PET-CT has the highest sensitivity and diagnostic odds ratio. Similar trend is observed during melanoma surveillance. The advent of immunotherapy, which has improved patient outcome, however, has determined new issues for radiologists, partly due to atypical response patterns, partly due to adverse reactions that must be identified as soon as possible for the correct management of the patient. The main objectives of the new ir-criteria are to standardize the assessment between different trials. However, these ir-criteria do not take into account all cases of atypical response patterns, as hyperprogression or dissociated responses. None of these criteria has actually been uniformly adopted in routine. The immune-related adverse events (irAEs) can involve various organs from head to toe. It is crucial for radiologists to know the imaging appearances of this condition, to exclude recurrent or progressive disease and for pneumonitis, since it could be potentially life-threatening toxicity, resulting in pneumonitis-related deaths in early phase trials, to allow a proper patient management.

Published
2022

44. Cascade testing for inherited cardiac conditions: Risk perception and screening after a negative genetic test result

Author

Kelsey M. Fusco, Robyn J. Hylind, Allison L. Cirino, Stephanie L. Harris, Steven A. Lubitz, Dominic J.R. Abrams, and Neal K. Lakdawala

Subjects
Adult, Heart Diseases, Humans, Genetic Counseling, Family, Genetic Testing, Disclosure, Genetics (clinical)
Abstract

First-degree relatives of a proband with an inherited cardiac condition (ICC) are offered predictive genetic testing for the pathogenic or likely pathogenic (P/LP) cardiac gene variant (CGV) to clarify their risk for the familial condition. Relatives who test negative for a familial P/LP CGV typically do not require longitudinal cardiac surveillance. To our knowledge, no previous study has investigated adjustment to risk reduction and subsequent screening practices in genotype-negative relatives from an ICC population. We thus investigated risk perception and ongoing screening practices in genotype-negative adults who received cardiac genetic counseling. Correlations between clinical and demographic variables and risk perception and screening practices were also investigated. On average, participants (n = 71) reported a perceived 19.5% lifetime risk of developing the ICC in their family, despite their negative genetic test result. The majority (54%) of participants reported having undergone cardiac screening after disclosure of their negative result. There were no significant correlations between clinical and demographic variables and risk perception or screening practices. Furthermore, risk perception was not found to impact the likelihood of cardiac screening. These findings suggest that even with comprehensive cardiac genetic counseling, a proportion of this population did not accurately comprehend or recall their cardiac disease risk. Additional interventions beyond traditional result disclosure should be explored to help genotype-negative individuals adjust to their reduction in risk for a familial ICC.

Published
2022

46. Changes in Body Mass Index Associated with Antiretroviral Regimen Switch Among Treatment-Experienced, Virologically Suppressed People Living with HIV in the United States

Author

Jennifer S Fusco, Laurence Brunet, Vani Vannappagari, Melissa Crawford, Ricky Hsu, Jean van Wyk, Gregory Fusco, Karam Mounzer, Janet Lo, and Cassidy Henegar

Subjects
0301 basic medicine, medicine.medical_specialty, Anti-HIV Agents, Immunology, MEDLINE, Human immunodeficiency virus (HIV), Integrase inhibitor, HIV Infections, medicine.disease_cause, Body Mass Index, 03 medical and health sciences, 0302 clinical medicine, Virology, Internal medicine, Oxazines, medicine, Humans, 030212 general & internal medicine, Darunavir, business.industry, Rilpivirine, medicine.disease, Obesity, United States, Regimen, 030104 developmental biology, Infectious Diseases, Anti-Retroviral Agents, Cohort, medicine.symptom, business, Heterocyclic Compounds, 3-Ring, Body mass index, Weight gain
Abstract

With obesity on the rise among people living with HIV (PLWH), there is growing concern that weight gain may result as an undesired effect of antiretroviral therapy (ART). This analysis sought to assess the association between ART regimens and changes in body mass index (BMI) among ART-experienced, virologically suppressed PLWH. ART-experienced, virologically suppressed PLWH ≥18 years of age in the Observational Pharmacoepidemiology Research and Analysis (OPERA) cohort were included for analysis if prescribed a new regimen containing one of the following core agents: dolutegravir (DTG), elvitegravir/cobicistat (EVG/c), raltegravir (RAL), rilpivirine (RPV), or boosted darunavir (bDRV), for the first time between August 1, 2013 and December 31, 2017. Multivariable linear regression was used to assess the association between regimen and mean changes in BMI at 6, 12, and 24 months after switch. In unadjusted analyses, BMI increases ranged from 0.30 kg/m

Published
2021

47. Large-Scale Postmarketing Surveillance of Biological Drugs for Immune-Mediated Inflammatory Diseases Through an Italian Distributed Multi-Database Healthcare Network: The VALORE Project

Author

Trifiro, G., Isgro, V., Ingrasciotta, Y., Ientile, V., L'Abbate, L., Foti, S. S., Belleudi, V., Poggi, F., Fontana, A., Moretti, U., Lora, R., Sabaini, A., Senesi, I., Sorrentino, C., Puzo, M. R., Padula, A., Fusco, M., Giordana, R., Solfrini, V., Puccini, A., Rossi, P., Del Zotto, S., Leoni, O., Zanforlini, M., Ancona, D., Bavaro, V., Garau, D., Ledda, S., Scondotto, S., Allotta, A., Tuccori, M., Gini, R., Bucaneve, G., Franchini, D., Cavazzana, A., Biasi, V., Spila Alegiani, S., Massari, M., Andretta, I., Tanaglia, M., Carriero, A., Sassano, S., De Sarro, G., Mirarchi, S., Palleria, C., Sarro, C., Balestrieri, M., Rostan, S., Capuano, A., Bernardi, F. F., Trama, U., Russo, A., Fumo, M. G., Addis, A., Musicco, F., Sapigni, E., Mazzetti, I., Podetti, D., Potenza, A. M., Nikitina, V., Ricciardelli, R., Mogheiseh, N., Croce, S., Pettinelli, A., Ejlli, L., Fortino, I., Ercolanoni, M., Mazzone, A., Nisic, A., Schiatti, S., Ludergnani, M., Mancini, M., Patregnani, L., Fabbietti, P., Antonicelli, E., Mangano, A., Campomori, A., Urru, S. A., Costa, G., Guarrera, G. M., Stella, P., Serra, E., Carta, P., Vannacci, A., Lucenteforte, E., Parrilli, M., Convertino, I., De Giorgi, M., Rocchi, R. E., Rossi, M., Scroccaro, G., Deambrosis, P., Grindelli, G., Ferroni, E., Trifiro, G., Isgro, V., Ingrasciotta, Y., Ientile, V., L'Abbate, L., Foti, S. S., Belleudi, V., Poggi, F., Fontana, A., Moretti, U., Lora, R., Sabaini, A., Senesi, I., Sorrentino, C., Puzo, M. R., Padula, A., Fusco, M., Giordana, R., Solfrini, V., Puccini, A., Rossi, P., Del Zotto, S., Leoni, O., Zanforlini, M., Ancona, D., Bavaro, V., Garau, D., Ledda, S., Scondotto, S., Allotta, A., Tuccori, M., Gini, R., Bucaneve, G., Franchini, D., Cavazzana, A., Biasi, V., Spila Alegiani, S., Massari, M., Andretta, I., Tanaglia, M., Carriero, A., Sassano, S., De Sarro, G., Mirarchi, S., Palleria, C., Sarro, C., Balestrieri, M., Rostan, S., Capuano, A., Bernardi, F. F., Trama, U., Russo, A., Fumo, M. G., Addis, A., Musicco, F., Sapigni, E., Mazzetti, I., Podetti, D., Potenza, A. M., Nikitina, V., Ricciardelli, R., Mogheiseh, N., Croce, S., Pettinelli, A., Ejlli, L., Fortino, I., Ercolanoni, M., Mazzone, A., Nisic, A., Schiatti, S., Ludergnani, M., Mancini, M., Patregnani, L., Fabbietti, P., Antonicelli, E., Mangano, A., Campomori, A., Urru, S. A., Costa, G., Guarrera, G. M., Stella, P., Serra, E., Carta, P., Vannacci, A., Lucenteforte, E., Parrilli, M., Convertino, I., De Giorgi, M., Rocchi, R. E., Rossi, M., Scroccaro, G., Deambrosis, P., Grindelli, G., and Ferroni, E.

Subjects
Male, medicine.medical_specialty, Population, Postmarketing surveillance, Rate ratio, REGISTRIES, Retrospective Studie, Internal medicine, BIOSIMILARS, medicine, Adalimumab, Humans, Pharmacology (medical), Original Research Article, education, Adverse effect, ANTI-TNF THERAPY, RHEUMATOID-ARTHRITIS, RISK, PHARMACOVIGILANCE, BIOSIMILARS, EXPERIENCE, REGISTRIES, PSORIASIS, MEDICINES, ACCESS, Biosimilar Pharmaceuticals, Retrospective Studies, RISK, Delivery of Health Care, Female, Infliximab, Italy, SARS-CoV-2, COVID-19, Pharmacology, education.field_of_study, PSORIASIS, business.industry, Biosimilar, ANTI-TNF THERAPY, General Medicine, medicine.disease, RHEUMATOID-ARTHRITIS, PHARMACOVIGILANCE, MEDICINES, EXPERIENCE, Immune-mediated inflammatory diseases, ACCESS, business, Human, Biosimilar Pharmaceutical, Biotechnology, medicine.drug
Abstract

Background Biological drugs have improved the management of immune-mediated inflammatory diseases (IMIDs) despite being associated with important safety issues such as immunogenicity, infections, and malignancies in real-world settings. Objective The aim of this study was to explore the potential of a large Italian multi-database distributed network for use in the postmarketing surveillance of biological drugs, including biosimilars, in patients with IMID. Methods A retrospective cohort study was conducted using 13 Italian regional claims databases during 2010–2019. A tailor-made R-based tool developed for distributed analysis of claims data using a study-specific common data model was customized for this study. We measured the yearly prevalence of biological drug users and the frequency of switches between originator and biosimilars for infliximab, etanercept, and adalimumab separately and stratified them by calendar year and region. We then calculated the cumulative number of users and person-years (PYs) of exposure to individual biological drugs approved for IMIDs. For a number of safety outcomes (e.g., severe acute respiratory syndrome coronavirus 2 [SARS-COV-2] infection), we conducted a sample power calculation to estimate the PYs of exposure required to investigate their association with individual biological drugs approved for IMIDs, considering different strengths of association. Results From a total underlying population of almost 50 million inhabitants from 13 Italian regions, we identified 143,602 (0.3%) biological drug users, with a cumulative exposure of 507,745 PYs during the entire follow-up. The mean age ± standard deviation of biological drug users was 49.3 ± 16.3, with a female-to-male ratio of 1.2. The age-adjusted yearly prevalence of biological drug users increased threefold from 0.7 per 1000 in 2010 to 2.1 per 1000 in 2019. Overall, we identified 40,996 users of biosimilars of tumor necrosis factor (TNF)-α inhibitors (i.e., etanercept, adalimumab, and infliximab) in the years 2015–2019. Of these, 46% (N = 18,845) switched at any time between originator and biosimilars or vice versa. To investigate a moderate association (incidence rate ratio 2) between biological drugs approved for IMIDs and safety events of interest, such as optic neuritis (lowest background incidence rate 10.4/100,000 PYs) or severe infection (highest background incidence rate 4312/100,000 PYs), a total of 43,311 PYs and 104 PYs of exposure to individual biological drugs, respectively, would be required. As such, using this network, of 15 individual biological drugs approved for IMIDs, the association with those adverse events could be investigated for four (27%) and 14 (93%), respectively. Conclusion The VALORE project multi-database network has access to data on more than 140,000 biological drug users (and > 0.5 million PYs) from 13 Italian regions during the years 2010–2019, which will be further expanded with the inclusion of data from other regions and more recent calendar years. Overall, the cumulated amount of person-time of exposure to biological drugs approved for IMIDs provides enough statistical power to investigate weak/moderate associations of almost all individual compounds and the most relevant safety outcomes. Moreover, this network may offer the opportunity to investigate the interchangeability of originator and biosimilars of several TNFα inhibitors in different therapeutic areas in real-world settings. Supplementary Information The online version contains supplementary material available at 10.1007/s40259-021-00498-3.

Published
2021

48. Advanced Heart Failure in Special Population—Pediatric Age

Author

Francesco Di Fraia, Marta Rubino, Roberta Pacileo, Augusto Esposito, Emanuele Monda, Michele Lioncino, Paolo Calabrò, Martina Caiazza, Maria Giovanna Russo, Viviana Tessitore, Alfredo Mauriello, Annapaola Cirillo, Giuseppe Limongelli, Federica Verrillo, Arturo Cesaro, Adelaide Fusco, Monda, E., Lioncino, M., Pacileo, R., Rubino, M., Cirillo, A., Fusco, A., Esposito, A., Verrillo, F., Di Fraia, F., Mauriello, A., Tessitore, V., Caiazza, M., Cesaro, A., Calabro, P., Russo, M. G., and Limongelli, G.

Subjects
Inotrope, medicine.medical_specialty, Waiting Lists, medicine.medical_treatment, Heart failure, Mechanical circulatory support, Epidemiology, Health care, medicine, Humans, Child, Intensive care medicine, Children, Heart transplantation, business.industry, Mortality rate, Gold standard, General Medicine, medicine.disease, Circulatory system, Heart Transplantation, Cardiac transplantation, Heart-Assist Devices, Cardiology and Cardiovascular Medicine, business
Abstract

Heart failure (HF) is an important health care issue in children because of its considerable morbidity and mortality. Advanced HF encompasses patients who remained symptomatic despite optimal medical treatment and includes patients who require special management, such as continuous inotropic therapy, mechanical circulatory support, or heart transplantation (HT). HT is the gold standard for children with advanced HF; nonetheless, the number of suitable donors has not increased for decades, leading to prolonged waitlist times and increased mortality rates. Therefore, the role of pediatric mechanic circulatory support has been assessed as an alternative treatment in patients in whom heart transplant could not be performed. The authors discuss the epidemiology, causes, pathophysiology, clinical manifestation, medical treatment, device therapy, and HT in pediatric HF, and a particular emphasis was posed on patients with advanced HF.

Published
2021

49. Switch from Tenofovir Disoproxil Fumarate to Tenofovir Alafenamide in People Living with HIV: Lipid Changes and Statin Underutilization

Author

Laurence Brunet, Patrick W. G. Mallon, Michael Wohlfeiler, Jennifer S Fusco, Gregory Fusco, Andrew P Beyer, and Girish Prajapati

Subjects
medicine.medical_specialty, Statin, Tenofovir, medicine.drug_class, Anti-HIV Agents, Human immunodeficiency virus (HIV), HIV Infections, medicine.disease_cause, Tenofovir alafenamide, Pharmacotherapy, Internal medicine, medicine, Humans, Pharmacology (medical), Original Research Article, Retrospective Studies, Alanine, business.industry, General Medicine, Statin treatment, medicine.disease, Lipids, Cohort, Hydroxymethylglutaryl-CoA Reductase Inhibitors, business, Dyslipidemia, medicine.drug
Abstract

Background and Objective Many people living with HIV (PLWH) on stable tenofovir disoproxil fumarate (TDF)-containing regimens have switched to tenofovir alafenamide (TAF), despite the potential lipid-lowering effect of TDF. We aimed to assess the impact of switching from TDF to TAF on lipids in real-world clinical practice. Methods PLWH prescribed TDF for ≥ 4 weeks who switched to TAF were identified in the OPERA cohort. Patterns of dyslipidemia were compared before and after switch based on NCEP ATPIII guidelines. Elevated 10-year risk of atherosclerotic cardiovascular disease (ASCVD ≥ 7.5%) and statin use were assessed. Results Among 6423 PLWH switched from TDF to TAF, the proportion with dyslipidemia/severe dyslipidemia observed after switch from TDF to TAF increased statistically significantly (p < 0.0001) with total cholesterol (5–10%), low-density lipoprotein cholesterol (16–23%), and triglycerides (21–27%), but decreased statistically significantly with high-density lipoprotein cholesterol (35–30%, p < 0.0001). These patterns of dyslipidemia persisted in sensitivity analyses restricted to PLWH who maintained all other antiretrovirals (N = 4328) or stratified by pharmaco-enhancer use before and after switch. An elevated ASCVD risk was detected in 29% before and 31% after switch. As many as 59% of PLWH with an elevated ASCVD risk were not prescribed a statin after switch from TDF to TAF. Conclusions In this large, diverse population of PLWH in the USA, the switch from TDF to TAF was associated with development of less favorable lipid profiles, regardless of pharmaco-enhancers or third-agent use. Statins remained underutilized after a switch from TDF to TAF.

Published
2021

50. Introduction

Author

Fusco, Idamaria and Quagliarotti, Désirée A.L.

Published
2016

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