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5. Additional booster doses in patients with chronic lymphocytic leukemia induce humoral and cellular immune responses to SARS-CoV-2 similar to natural infection regardless ongoing treatments : A study by ERIC, the European Research Initiative on CLL

Author

Campanella, A., Capasso, A., Heltai, S., Taccetti, C., Albi, E., Herishanu, Y., Haggenburg, S., Chatzikonstantinou, T., Doubek, M., Kättström, Magdalena, Giannopoulos, K., Simkovic, M., Moreno, C., Massaia, M., Bumbea, H., Alshemmari, S., Ranghetti, P., Perotta, E., Martini, F., Sant'Antonio, E., Colia, M., Combi, C., Levi, S., Kater, A. P., Hazenberg, M., Nijhof, I. S., Hofsink, Q., Demosthenous, C., Kotaskova, J., Zaleska, J., Vrbacky, F., Raya, A. Mora, Bisogno, D., Tripoli, I. E., Popov, V. M., Roman, V., Stavroyianni, N., Karypidou, M., Scarano, E., Locatelli, M., Frenquelli, M., Scarfò, L., Stamatopoulos, K., Ghia, P., Campanella, A., Capasso, A., Heltai, S., Taccetti, C., Albi, E., Herishanu, Y., Haggenburg, S., Chatzikonstantinou, T., Doubek, M., Kättström, Magdalena, Giannopoulos, K., Simkovic, M., Moreno, C., Massaia, M., Bumbea, H., Alshemmari, S., Ranghetti, P., Perotta, E., Martini, F., Sant'Antonio, E., Colia, M., Combi, C., Levi, S., Kater, A. P., Hazenberg, M., Nijhof, I. S., Hofsink, Q., Demosthenous, C., Kotaskova, J., Zaleska, J., Vrbacky, F., Raya, A. Mora, Bisogno, D., Tripoli, I. E., Popov, V. M., Roman, V., Stavroyianni, N., Karypidou, M., Scarano, E., Locatelli, M., Frenquelli, M., Scarfò, L., Stamatopoulos, K., and Ghia, P.

Abstract

Profound immune dysregulation and impaired response to the SARS-CoV-2 vaccine put patients with chronic lymphocytic leukemia (CLL) at risk of severe COVID-19. We compared humoral memory and T-cell responses after booster dose vaccination or breakthrough infection. (Green) Quantitative determination of anti-Spike specific antibodies. Booster doses increased seroconversion rate and antibody titers in all patient categories, ultimately generating humoral responses similar to those observed in the postinfection cohort. In detail, humoral response with overscale median antibody titers arose in >80% of patients in watch and wait, off-therapy in remission, or under treatment with venetoclax single-agent. Anti-CD20 antibodies and active treatment with BTK inhibitors (BTKi) represent limiting factors of humoral response, still memory mounted in ~40% of cases following booster doses or infection. (Blue) Evaluation of SARS-CoV-2-specific T-cell responses. Number of T-cell functional activation markers documented in each patient. The vast majority of patients, including those seronegative, developed T-cell responses, qualitatively similar between treatment groups or between vaccination alone and infection cases. These data highlight the efficacy of booster doses in eliciting T-cell immunity independently of treatment status and support the use of additional vaccination boosters to stimulate humoral immunity in patients on active CLL-directed treatments., The project was supported in part by Fondazione Veronesi (ID 1852164), Janssen (IBR-I-20-EMEA-014-V01/54179060CLL4024; NOPRODCLL4001), and MH CZ—DRO (FNBr, 65269705).

Published
2024
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7. Additional booster doses in patients with chronic lymphocytic leukemia induce humoral and cellular immune responses to SARS‐CoV‐2 similar to natural infection regardless ongoing treatments: A study by ERIC, the European Research Initiative on CLL

Author

Campanella, A., primary, Capasso, A., additional, Heltai, S., additional, Taccetti, C., additional, Albi, E., additional, Herishanu, Y., additional, Haggenburg, S., additional, Chatzikonstantinou, T., additional, Doubek, M., additional, Kättström, M., additional, Giannopoulos, K., additional, Simkovic, M., additional, Moreno, C., additional, Massaia, M., additional, Bumbea, H., additional, Alshemmari, S., additional, Ranghetti, P., additional, Perotta, E., additional, Martini, F., additional, Sant'Antonio, E., additional, Colia, M., additional, Combi, C., additional, Levi, S., additional, Kater, A. P., additional, Hazenberg, M., additional, Nijhof, I. S., additional, Hofsink, Q., additional, Demosthenous, C., additional, Kotaskova, J., additional, Zaleska, J., additional, Vrbacky, F., additional, Raya, A. Mora, additional, Bisogno, D., additional, Tripoli, I. E., additional, Popov, V. M., additional, Roman, V., additional, Stavroyianni, N., additional, Karypidou, M., additional, Scarano, E., additional, Locatelli, M., additional, Frenquelli, M., additional, Scarfò, L., additional, Stamatopoulos, K., additional, and Ghia, P., additional

Published
2024
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9. Epilepsy is an important feature of KBG syndrome associated with poorer developmental outcome.

Author

Buijsse, N., Jansen, Femke, Ockeloen, C.W., Kempen, M.J.P. van, Zeidler, S., Willemsen, M.H., Scarano, E., Monticone, S., Zonneveld-Huijssoon, E., Low, K.J., Bayat, A., Sisodiya, S.M., Samanta, D., Lesca, G., Jong, D. de, Giltay, J.C., Verbeek, N.E., Kleefstra, T., Brilstra, E.H., Vlaskamp, D.R.M., Buijsse, N., Jansen, Femke, Ockeloen, C.W., Kempen, M.J.P. van, Zeidler, S., Willemsen, M.H., Scarano, E., Monticone, S., Zonneveld-Huijssoon, E., Low, K.J., Bayat, A., Sisodiya, S.M., Samanta, D., Lesca, G., Jong, D. de, Giltay, J.C., Verbeek, N.E., Kleefstra, T., Brilstra, E.H., and Vlaskamp, D.R.M.

Abstract

Contains fulltext : 300069.pdf (Publisher’s version ) (Open Access), OBJECTIVE: The aim of this study was to describe the epilepsy phenotype in a large international cohort of patients with KBG syndrome and to study a possible genotype-phenotype correlation. METHODS: We collected data on patients with ANKRD11 variants by contacting University Medical Centers in the Netherlands, an international network of collaborating clinicians, and study groups who previously published about KBG syndrome. All patients with a likely pathogenic or pathogenic ANKRD11 variant were included in our patient cohort and categorized into an "epilepsy group" or "non-epilepsy group". Additionally, we included previously reported patients with (likely) pathogenic ANKRD11 variants and epilepsy from the literature. RESULTS: We included 75 patients with KBG syndrome of whom 26 had epilepsy. Those with epilepsy more often had moderate to severe intellectual disability (42.3% vs 9.1%, RR 4.6 [95% CI 1.7-13.1]). Seizure onset in patients with KBG syndrome occurred at a median age of 4 years (range 12 months - 20 years), and the majority had generalized onset seizures (57.7%) with tonic-clonic seizures being most common (23.1%). The epilepsy type was mostly classified as generalized (42.9%) or combined generalized and focal (42.9%), not fulfilling the criteria of an electroclinical syndrome diagnosis. Half of the epilepsy patients (50.0%) were seizure free on anti-seizure medication (ASM) for at least 1 year at the time of last assessment, but 26.9% of patients had drug-resistant epilepsy (failure of ≥2 ASM). No genotype-phenotype correlation could be identified for the presence of epilepsy or epilepsy characteristics. SIGNIFICANCE: Epilepsy in KBG syndrome most often presents as a generalized or combined focal and generalized type. No distinctive epilepsy syndrome could be identified. Patients with KBG syndrome and epilepsy had a significantly poorer neurodevelopmental outcome compared with those without epilepsy. Clinicians should consider KBG syndrome as a causal, 01 december 2023

Published
2023

10. Management of intracranial hypertension following traumatic brain injury: a best clinical practice adoption proposal for intracranial pressure monitoring and decompressive craniectomy. Joint statements by the Traumatic Brain Injury Section of the Italian Society of Neurosurgery (SINch) and the Neuroanesthesia and Neurocritical Care Study Group of the Italian Society of Anesthesia, Analgesia, Resuscitation and Intensive Care (SIAARTI)

Author

Iaccarino C., Lippa L., Munari M., Castioni C. A., Robba C., Caricato A., Pompucci A., Signoretti S., Zona G., Rasulo F. A., Aimar E., Amato S., Angileri F. F., Anile C., Assietti R., Baratto V., Barbanera A., Basile L., Battaglia R., Bellocchi S., Bertuccio A., Blanco S., Bolognini A., Boniferro B., Bordi L., Bortolotti C., Brandini V., Broger M., Brollo M., Caffarella D. D., Caggiano C., Cantisani P. L., Capone C., Cappelletto B., Capuano C., Carangelo B., Caruselli G., Chessa M. A., Chiara M., Chibbaro S., Cioffi V., Ciprianocecchi P., Colistra D., Conti C., Contratti F., Costella G. B., Cuoci A., D'Avella D., D'Ercole M., Deangelis M., Defalco R., de Luca G., de Marinis P., Del Vecchio C., Delfinis C., Denaro L., Deodato F., Desogus N., Disomma A., Domenicucci M., Dones F., Fina M., Fiori L., Fricia M., Gaetani P., Gazzeri R., Gentile M., Germano A., Ghadirpour R., Gianfreda C. D., Gigante N., Gigli R., Giorgetti J., Giusa M., Gravina U. G., Grippi L., Guida F., Guizzardi G., Iannuzzo G., Kropp M., Lattanzi L., Lucantoni D., Maffei L., Magliulo M., Marconi F., Marruzzo D., Martellotta N., Marton E., Maugeri R., Mauro G., Meli F., Menniti A., Merciadri P., Milanese L., Nardacci B., Nasi D., Orvieto P., Pacca P., Pansini G., Panzarasa G., Passanisi M., Pavesi G., Pizzoni C., Pulera F., Rapana A., Ricci A., Rispoli R., Rotondo M., Russo N., Santilli S., Scarano E., Schwarz A., Servadei F., Simonetti G., Stefini R., Talamonti G., Turrisi A., Valente V. M., Villa A., Vindigni M., Visocchi M., Vitali M., Wierzbicki V., Zambon G., Zanotti B., Zenga F., Alampi D., Alessandri F., Aloj F., Amigoni A., Aspide R., Bertuetti R., Betti V., Bilotta F., Bonato V., Bosco E., Brita M., Buscema G., Cafiero T., Cappuccio D., Caradonna M., Caria C. G., Casartelliliviero M., Ciritella P., Cirrincione S., Citerio G., Colelli S., Coletta F., Concordia L., Congedo E., Covotta M., Crimella F., Dall'Acqua G., De Cassai A., Defulviis S., Deperi E., Deana C., Delgaudio A., Denittis N., Dicolandrea S., Divezza F., Ferri F., Flocco R., Fontana C., Forastierimolinari A., Frangiosa A., Fumagalli P., Fuselli E., Garbarino M. M., Gelormini D., Geraci C., Geraldini F., Giacomucci A., Giampaoli V., Giorgetti D., Gritti P., Gualdani S., Iacovazzo C., Iermano C., Latronico N., Lugari S., Lusenti F., Maglione C., Magnoni S., Maiarota F., Malla M., Marchesi M., Martino C., Matteotti I., Mazzeo A. T., Morello G., Nardiello I., Paticchio F., Pegoli M., Perotti V., Piazzolla M., Picciafuochi F., Rachedi N., Radolovich D. K., Recchia A., Riccardi S., Romagnoli S., Sala S., Scafuro M. A., Sgarlata P., Soragni A., Stefani F., Stival E., Stofella G., Terranova F., Tinturini R., Togni T., Toto R., Trapani D., Tringali E., Tullo L., Valente A., Valeo T., Varelli G., Villani R., Zamacavicchi F., Zanello M., Zarrillo N., Zugni N., Iaccarino, C, Lippa, L, Munari, M, Castioni, C, Robba, C, Caricato, A, Pompucci, A, Signoretti, S, Zona, G, Rasulo, F, Aimar, E, Amato, S, Angileri, F, Anile, C, Assietti, R, Baratto, V, Barbanera, A, Basile, L, Battaglia, R, Bellocchi, S, Bertuccio, A, Blanco, S, Bolognini, A, Boniferro, B, Bordi, L, Bortolotti, C, Brandini, V, Broger, M, Brollo, M, Caffarella, D, Caggiano, C, Cantisani, P, Capone, C, Cappelletto, B, Capuano, C, Carangelo, B, Caruselli, G, Chessa, M, Chiara, M, Chibbaro, S, Cioffi, V, Ciprianocecchi, P, Colistra, D, Conti, C, Contratti, F, Costella, G, Cuoci, A, D'Avella, D, D'Ercole, M, Deangelis, M, Defalco, R, de Luca, G, de Marinis, P, Del Vecchio, C, Delfinis, C, Denaro, L, Deodato, F, Desogus, N, Disomma, A, Domenicucci, M, Dones, F, Fina, M, Fiori, L, Fricia, M, Gaetani, P, Gazzeri, R, Gentile, M, Germano, A, Ghadirpour, R, Gianfreda, C, Gigante, N, Gigli, R, Giorgetti, J, Giusa, M, Gravina, U, Grippi, L, Guida, F, Guizzardi, G, Iannuzzo, G, Kropp, M, Lattanzi, L, Lucantoni, D, Maffei, L, Magliulo, M, Marconi, F, Marruzzo, D, Martellotta, N, Marton, E, Maugeri, R, Mauro, G, Meli, F, Menniti, A, Merciadri, P, Milanese, L, Nardacci, B, Nasi, D, Orvieto, P, Pacca, P, Pansini, G, Panzarasa, G, Passanisi, M, Pavesi, G, Pizzoni, C, Pulera, F, Rapana, A, Ricci, A, Rispoli, R, Rotondo, M, Russo, N, Santilli, S, Scarano, E, Schwarz, A, Servadei, F, Simonetti, G, Stefini, R, Talamonti, G, Turrisi, A, Valente, V, Villa, A, Vindigni, M, Visocchi, M, Vitali, M, Wierzbicki, V, Zambon, G, Zanotti, B, Zenga, F, Alampi, D, Alessandri, F, Aloj, F, Amigoni, A, Aspide, R, Bertuetti, R, Betti, V, Bilotta, F, Bonato, V, Bosco, E, Brita, M, Buscema, G, Cafiero, T, Cappuccio, D, Caradonna, M, Caria, C, Casartelliliviero, M, Ciritella, P, Cirrincione, S, Citerio, G, Colelli, S, Coletta, F, Concordia, L, Congedo, E, Covotta, M, Crimella, F, Dall'Acqua, G, De Cassai, A, Defulviis, S, Deperi, E, Deana, C, Delgaudio, A, Denittis, N, Dicolandrea, S, Divezza, F, Ferri, F, Flocco, R, Fontana, C, Forastierimolinari, A, Frangiosa, A, Fumagalli, P, Fuselli, E, Garbarino, M, Gelormini, D, Geraci, C, Geraldini, F, Giacomucci, A, Giampaoli, V, Giorgetti, D, Gritti, P, Gualdani, S, Iacovazzo, C, Iermano, C, Latronico, N, Lugari, S, Lusenti, F, Maglione, C, Magnoni, S, Maiarota, F, Malla, M, Marchesi, M, Martino, C, Matteotti, I, Mazzeo, A, Morello, G, Nardiello, I, Paticchio, F, Pegoli, M, Perotti, V, Piazzolla, M, Picciafuochi, F, Rachedi, N, Radolovich, D, Recchia, A, Riccardi, S, Romagnoli, S, Sala, S, Scafuro, M, Sgarlata, P, Soragni, A, Stefani, F, Stival, E, Stofella, G, Terranova, F, Tinturini, R, Togni, T, Toto, R, Trapani, D, Tringali, E, Tullo, L, Valente, A, Valeo, T, Varelli, G, Villani, R, Zamacavicchi, F, Zanello, M, Zarrillo, N, Zugni, N, Iaccarino, C., Lippa, L., Munari, M., Castioni, C. A., Robba, C., Caricato, A., Pompucci, A., Signoretti, S., Zona, G., Rasulo, F. A., Aimar, E., Amato, S., Angileri, F. F., Anile, C., Assietti, R., Baratto, V., Barbanera, A., Basile, L., Battaglia, R., Bellocchi, S., Bertuccio, A., Blanco, S., Bolognini, A., Boniferro, B., Bordi, L., Bortolotti, C., Brandini, V., Broger, M., Brollo, M., Caffarella, D. D., Caggiano, C., Cantisani, P. L., Capone, C., Cappelletto, B., Capuano, C., Carangelo, B., Caruselli, G., Chessa, M. A., Chiara, M., Chibbaro, S., Cioffi, V., Ciprianocecchi, P., Colistra, D., Conti, C., Contratti, F., Costella, G. B., Cuoci, A., D'Avella, D., D'Ercole, M., Deangelis, M., Defalco, R., de Luca, G., de Marinis, P., Del Vecchio, C., Delfinis, C., Denaro, L., Deodato, F., Desogus, N., Disomma, A., Domenicucci, M., Dones, F., Fina, M., Fiori, L., Fricia, M., Gaetani, P., Gazzeri, R., Gentile, M., Germano, A., Ghadirpour, R., Gianfreda, C. D., Gigante, N., Gigli, R., Giorgetti, J., Giusa, M., Gravina, U. G., Grippi, L., Guida, F., Guizzardi, G., Iannuzzo, G., Kropp, M., Lattanzi, L., Lucantoni, D., Maffei, L., Magliulo, M., Marconi, F., Marruzzo, D., Martellotta, N., Marton, E., Maugeri, R., Mauro, G., Meli, F., Menniti, A., Merciadri, P., Milanese, L., Nardacci, B., Nasi, D., Orvieto, P., Pacca, P., Pansini, G., Panzarasa, G., Passanisi, M., Pavesi, G., Pizzoni, C., Pulera, F., Rapana, A., Ricci, A., Rispoli, R., Rotondo, M., Russo, N., Santilli, S., Scarano, E., Schwarz, A., Servadei, F., Simonetti, G., Stefini, R., Talamonti, G., Turrisi, A., Valente, V. M., Villa, A., Vindigni, M., Visocchi, M., Vitali, M., Wierzbicki, V., Zambon, G., Zanotti, B., Zenga, F., Alampi, D., Alessandri, F., Aloj, F., Amigoni, A., Aspide, R., Bertuetti, R., Betti, V., Bilotta, F., Bonato, V., Bosco, E., Brita, M., Buscema, G., Cafiero, T., Cappuccio, D., Caradonna, M., Caria, C. G., Casartelliliviero, M., Ciritella, P., Cirrincione, S., Citerio, G., Colelli, S., Coletta, F., Concordia, L., Congedo, E., Covotta, M., Crimella, F., Dall'Acqua, G., De Cassai, A., Defulviis, S., Deperi, E., Deana, C., Delgaudio, A., Denittis, N., Dicolandrea, S., Divezza, F., Ferri, F., Flocco, R., Fontana, C., Forastierimolinari, A., Frangiosa, A., Fumagalli, P., Fuselli, E., Garbarino, M. M., Gelormini, D., Geraci, C., Geraldini, F., Giacomucci, A., Giampaoli, V., Giorgetti, D., Gritti, P., Gualdani, S., Iacovazzo, C., Iermano, C., Latronico, N., Lugari, S., Lusenti, F., Maglione, C., Magnoni, S., Maiarota, F., Malla, M., Marchesi, M., Martino, C., Matteotti, I., Mazzeo, A. T., Morello, G., Nardiello, I., Paticchio, F., Pegoli, M., Perotti, V., Piazzolla, M., Picciafuochi, F., Rachedi, N., Radolovich, D. K., Recchia, A., Riccardi, S., Romagnoli, S., Sala, S., Scafuro, M. A., Sgarlata, P., Soragni, A., Stefani, F., Stival, E., Stofella, G., Terranova, F., Tinturini, R., Togni, T., Toto, R., Trapani, D., Tringali, E., Tullo, L., Valente, A., Valeo, T., Varelli, G., Villani, R., Zamacavicchi, F., Zanello, M., Zarrillo, N., and Zugni, N.

Subjects
medicine.medical_specialty, Decompressive Craniectomy, Consensus development conference, Decompressive craniectomy, Guideline, Traumatic brain injuries, Critical Care, Intracranial Pressure, medicine.medical_treatment, MEDLINE, Neurosurgery, Traumatic brain injurie, law.invention, 03 medical and health sciences, 0302 clinical medicine, Randomized controlled trial, law, Intensive care, Settore MED/41 - ANESTESIOLOGIA, Brain Injuries, Traumatic, medicine, Humans, Anesthesia, Intensive care medicine, Randomized Controlled Trials as Topic, business.industry, Neurointensive care, Evidence-based medicine, Italy, 030220 oncology & carcinogenesis, Intracranial pressure monitoring, Surgery, Neurology (clinical), Analgesia, Intracranial Hypertension, business, 030217 neurology & neurosurgery, Human
Abstract

No robust evidence is provided by literature regarding the management of intracranial hypertension following severe traumatic brain injury (TBI). This is mostly due to the lack of prospective randomized controlled trials (RCTs), the presence of studies containing extreme heterogeneously collected populations and controversial considerations about chosen outcome. A scientific society should provide guidelines for care management and scientific support for those areas for which evidence-based medicine has not been identified. However, RCTs in severe TBI have failed to establish intervention effectiveness, arising the need to make greater use of tools such as Consensus Conferences between experts, which have the advantage of providing recommendations based on experience, on the analysis of updated literature data and on the direct comparison of different logistic realities. The Italian scientific societies should provide guidelines following the national laws ruling the best medical practice. However, many limitations do not allow the collection of data supporting high levels of evidence for intracranial pressure (ICP) monitoring and decompressive craniectomy (DC) in patients with severe TBI. This intersociety document proposes best practice guidelines for this subsetting of patients to be adopted on a national Italian level, along with joint statements from "TBI Section" of the Italian Society of Neurosurgery (SINch) endorsed by the Neuroanesthesia and Neurocritical Care Study Group of the Italian Society of Anesthesia, Analgesia, Resuscitation and Intensive Care (SIAARTI). Presented here is a recap of recommendations on management of ICP and DC supported a high level of available evidence and rate of agreement expressed by the assemblies during the more recent consensus conferences, where members of both groups have had a role of active participants and supporters. The listed recommendations have been sent to a panel of experts consisting of the 107 members of the "TBI Section" of the SINch and the 111 members of the Neuroanesthesia and Neurocritical Care Study Group of the SIAARTI. The aim of the survey was to test a preliminary evaluation of the grade of predictable future adherence of the recommendations following this intersociety proposal. The following recommendations are suggested as representing best clinical practice, nevertheless, adoption of local multidisciplinary protocols regarding thresholds of ICP values, drug therapies, hemostasis management and perioperative care of decompressed patients is strongly recommended to improve treatment efficiency, to increase the quality of data collection and to provide more powerful evidence with future studies. Thus, for this future perspective a rapid overview of the role of the multimodal neuromonitoring in the optimal severe TBI management is also provided in this document. It is reasonable to assume that the recommendations reported in this paper will in future be updated by new observations arising from future trials. They are not binding, and this document should be offered as a guidance for clinical practice through an intersociety agreement, taking in consideration the low level of evidence.

Published
2021

11. Management of intracranial hypertension following traumatic brain injury: A best clinical practice adoption proposal for intracranial pressure monitoring and decompressive craniectomy: Joint statements by the Traumatic Brain Injury Section of the Italian Society of Neurosurgery (SINch) and the Neuroanesthesia and Neurocritical Care Study Group of the Italian Society of Anesthesia, Analgesia, Resuscitation and Intensive Care (SIAARTI)

Author

Iaccarino, C, Lippa, L, Munari, M, Castioni, C, Robba, C, Caricato, A, Pompucci, A, Signoretti, S, Zona, G, Rasulo, F, Aimar, E, Amato, S, Angileri, F, Anile, C, Assietti, R, Baratto, V, Barbanera, A, Basile, L, Battaglia, R, Bellocchi, S, Bertuccio, A, Blanco, S, Bolognini, A, Boniferro, B, Bordi, L, Bortolotti, C, Brandini, V, Broger, M, Brollo, M, Caffarella, D, Caggiano, C, Cantisani, P, Capone, C, Cappelletto, B, Capuano, C, Carangelo, B, Caruselli, G, Chessa, M, Chiara, M, Chibbaro, S, Cioffi, V, Ciprianocecchi, P, Colistra, D, Conti, C, Contratti, F, Costella, G, Cuoci, A, D'Avella, D, D'Ercole, M, Deangelis, M, Defalco, R, de Luca, G, de Marinis, P, Del Vecchio, C, Delfinis, C, Denaro, L, Deodato, F, Desogus, N, Disomma, A, Domenicucci, M, Dones, F, Fina, M, Fiori, L, Fricia, M, Gaetani, P, Gazzeri, R, Gentile, M, Germano, A, Ghadirpour, R, Gianfreda, C, Gigante, N, Gigli, R, Giorgetti, J, Giusa, M, Gravina, U, Grippi, L, Guida, F, Guizzardi, G, Iannuzzo, G, Kropp, M, Lattanzi, L, Lucantoni, D, Maffei, L, Magliulo, M, Marconi, F, Marruzzo, D, Martellotta, N, Marton, E, Maugeri, R, Mauro, G, Meli, F, Menniti, A, Merciadri, P, Milanese, L, Nardacci, B, Nasi, D, Orvieto, P, Pacca, P, Pansini, G, Panzarasa, G, Passanisi, M, Pavesi, G, Pizzoni, C, Pulera, F, Rapana, A, Ricci, A, Rispoli, R, Rotondo, M, Russo, N, Santilli, S, Scarano, E, Schwarz, A, Servadei, F, Simonetti, G, Stefini, R, Talamonti, G, Turrisi, A, Valente, V, Villa, A, Vindigni, M, Visocchi, M, Vitali, M, Wierzbicki, V, Zambon, G, Zanotti, B, Zenga, F, Alampi, D, Alessandri, F, Aloj, F, Amigoni, A, Aspide, R, Bertuetti, R, Betti, V, Bilotta, F, Bonato, V, Bosco, E, Brita, M, Buscema, G, Cafiero, T, Cappuccio, D, Caradonna, M, Caria, C, Casartelliliviero, M, Ciritella, P, Cirrincione, S, Citerio, G, Colelli, S, Coletta, F, Concordia, L, Congedo, E, Covotta, M, Crimella, F, Dall'Acqua, G, De Cassai, A, Defulviis, S, Deperi, E, Deana, C, Delgaudio, A, Denittis, N, Dicolandrea, S, Divezza, F, Ferri, F, Flocco, R, Fontana, C, Forastierimolinari, A, Frangiosa, A, Fumagalli, P, Fuselli, E, Garbarino, M, Gelormini, D, Geraci, C, Geraldini, F, Giacomucci, A, Giampaoli, V, Giorgetti, D, Gritti, P, Gualdani, S, Iacovazzo, C, Iermano, C, Latronico, N, Lugari, S, Lusenti, F, Maglione, C, Magnoni, S, Maiarota, F, Malla, M, Marchesi, M, Martino, C, Matteotti, I, Mazzeo, A, Morello, G, Nardiello, I, Paticchio, F, Pegoli, M, Perotti, V, Piazzolla, M, Picciafuochi, F, Rachedi, N, Radolovich, D, Recchia, A, Riccardi, S, Romagnoli, S, Sala, S, Scafuro, M, Sgarlata, P, Soragni, A, Stefani, F, Stival, E, Stofella, G, Terranova, F, Tinturini, R, Togni, T, Toto, R, Trapani, D, Tringali, E, Tullo, L, Valente, A, Valeo, T, Varelli, G, Villani, R, Zamacavicchi, F, Zanello, M, Zarrillo, N, Zugni, N, Iaccarino C., Lippa L., Munari M., Castioni C. A., Robba C., Caricato A., Pompucci A., Signoretti S., Zona G., Rasulo F. A., Aimar E., Amato S., Angileri F. F., Anile C., Assietti R., Baratto V., Barbanera A., Basile L., Battaglia R., Bellocchi S., Bertuccio A., Blanco S., Bolognini A., Boniferro B., Bordi L., Bortolotti C., Brandini V., Broger M., Brollo M., Caffarella D. D., Caggiano C., Cantisani P. L., Capone C., Cappelletto B., Capuano C., Carangelo B., Caruselli G., Chessa M. A., Chiara M., Chibbaro S., Cioffi V., Ciprianocecchi P., Colistra D., Conti C., Contratti F., Costella G. B., Cuoci A., D'Avella D., D'Ercole M., Deangelis M., Defalco R., de Luca G., de Marinis P., Del Vecchio C., Delfinis C., Denaro L., Deodato F., Desogus N., Disomma A., Domenicucci M., Dones F., Fina M., Fiori L., Fricia M., Gaetani P., Gazzeri R., Gentile M., Germano A., Ghadirpour R., Gianfreda C. D., Gigante N., Gigli R., Giorgetti J., Giusa M., Gravina U. G., Grippi L., Guida F., Guizzardi G., Iannuzzo G., Kropp M., Lattanzi L., Lucantoni D., Maffei L., Magliulo M., Marconi F., Marruzzo D., Martellotta N., Marton E., Maugeri R., Mauro G., Meli F., Menniti A., Merciadri P., Milanese L., Nardacci B., Nasi D., Orvieto P., Pacca P., Pansini G., Panzarasa G., Passanisi M., Pavesi G., Pizzoni C., Pulera F., Rapana A., Ricci A., Rispoli R., Rotondo M., Russo N., Santilli S., Scarano E., Schwarz A., Servadei F., Simonetti G., Stefini R., Talamonti G., Turrisi A., Valente V. M., Villa A., Vindigni M., Visocchi M., Vitali M., Wierzbicki V., Zambon G., Zanotti B., Zenga F., Alampi D., Alessandri F., Aloj F., Amigoni A., Aspide R., Bertuetti R., Betti V., Bilotta F., Bonato V., Bosco E., Brita M., Buscema G., Cafiero T., Cappuccio D., Caradonna M., Caria C. G., Casartelliliviero M., Ciritella P., Cirrincione S., Citerio G., Colelli S., Coletta F., Concordia L., Congedo E., Covotta M., Crimella F., Dall'Acqua G., De Cassai A., Defulviis S., Deperi E., Deana C., Delgaudio A., Denittis N., Dicolandrea S., Divezza F., Ferri F., Flocco R., Fontana C., Forastierimolinari A., Frangiosa A., Fumagalli P., Fuselli E., Garbarino M. M., Gelormini D., Geraci C., Geraldini F., Giacomucci A., Giampaoli V., Giorgetti D., Gritti P., Gualdani S., Iacovazzo C., Iermano C., Latronico N., Lugari S., Lusenti F., Maglione C., Magnoni S., Maiarota F., Malla M., Marchesi M., Martino C., Matteotti I., Mazzeo A. T., Morello G., Nardiello I., Paticchio F., Pegoli M., Perotti V., Piazzolla M., Picciafuochi F., Rachedi N., Radolovich D. K., Recchia A., Riccardi S., Romagnoli S., Sala S., Scafuro M. A., Sgarlata P., Soragni A., Stefani F., Stival E., Stofella G., Terranova F., Tinturini R., Togni T., Toto R., Trapani D., Tringali E., Tullo L., Valente A., Valeo T., Varelli G., Villani R., Zamacavicchi F., Zanello M., Zarrillo N., Zugni N., Iaccarino, C, Lippa, L, Munari, M, Castioni, C, Robba, C, Caricato, A, Pompucci, A, Signoretti, S, Zona, G, Rasulo, F, Aimar, E, Amato, S, Angileri, F, Anile, C, Assietti, R, Baratto, V, Barbanera, A, Basile, L, Battaglia, R, Bellocchi, S, Bertuccio, A, Blanco, S, Bolognini, A, Boniferro, B, Bordi, L, Bortolotti, C, Brandini, V, Broger, M, Brollo, M, Caffarella, D, Caggiano, C, Cantisani, P, Capone, C, Cappelletto, B, Capuano, C, Carangelo, B, Caruselli, G, Chessa, M, Chiara, M, Chibbaro, S, Cioffi, V, Ciprianocecchi, P, Colistra, D, Conti, C, Contratti, F, Costella, G, Cuoci, A, D'Avella, D, D'Ercole, M, Deangelis, M, Defalco, R, de Luca, G, de Marinis, P, Del Vecchio, C, Delfinis, C, Denaro, L, Deodato, F, Desogus, N, Disomma, A, Domenicucci, M, Dones, F, Fina, M, Fiori, L, Fricia, M, Gaetani, P, Gazzeri, R, Gentile, M, Germano, A, Ghadirpour, R, Gianfreda, C, Gigante, N, Gigli, R, Giorgetti, J, Giusa, M, Gravina, U, Grippi, L, Guida, F, Guizzardi, G, Iannuzzo, G, Kropp, M, Lattanzi, L, Lucantoni, D, Maffei, L, Magliulo, M, Marconi, F, Marruzzo, D, Martellotta, N, Marton, E, Maugeri, R, Mauro, G, Meli, F, Menniti, A, Merciadri, P, Milanese, L, Nardacci, B, Nasi, D, Orvieto, P, Pacca, P, Pansini, G, Panzarasa, G, Passanisi, M, Pavesi, G, Pizzoni, C, Pulera, F, Rapana, A, Ricci, A, Rispoli, R, Rotondo, M, Russo, N, Santilli, S, Scarano, E, Schwarz, A, Servadei, F, Simonetti, G, Stefini, R, Talamonti, G, Turrisi, A, Valente, V, Villa, A, Vindigni, M, Visocchi, M, Vitali, M, Wierzbicki, V, Zambon, G, Zanotti, B, Zenga, F, Alampi, D, Alessandri, F, Aloj, F, Amigoni, A, Aspide, R, Bertuetti, R, Betti, V, Bilotta, F, Bonato, V, Bosco, E, Brita, M, Buscema, G, Cafiero, T, Cappuccio, D, Caradonna, M, Caria, C, Casartelliliviero, M, Ciritella, P, Cirrincione, S, Citerio, G, Colelli, S, Coletta, F, Concordia, L, Congedo, E, Covotta, M, Crimella, F, Dall'Acqua, G, De Cassai, A, Defulviis, S, Deperi, E, Deana, C, Delgaudio, A, Denittis, N, Dicolandrea, S, Divezza, F, Ferri, F, Flocco, R, Fontana, C, Forastierimolinari, A, Frangiosa, A, Fumagalli, P, Fuselli, E, Garbarino, M, Gelormini, D, Geraci, C, Geraldini, F, Giacomucci, A, Giampaoli, V, Giorgetti, D, Gritti, P, Gualdani, S, Iacovazzo, C, Iermano, C, Latronico, N, Lugari, S, Lusenti, F, Maglione, C, Magnoni, S, Maiarota, F, Malla, M, Marchesi, M, Martino, C, Matteotti, I, Mazzeo, A, Morello, G, Nardiello, I, Paticchio, F, Pegoli, M, Perotti, V, Piazzolla, M, Picciafuochi, F, Rachedi, N, Radolovich, D, Recchia, A, Riccardi, S, Romagnoli, S, Sala, S, Scafuro, M, Sgarlata, P, Soragni, A, Stefani, F, Stival, E, Stofella, G, Terranova, F, Tinturini, R, Togni, T, Toto, R, Trapani, D, Tringali, E, Tullo, L, Valente, A, Valeo, T, Varelli, G, Villani, R, Zamacavicchi, F, Zanello, M, Zarrillo, N, Zugni, N, Iaccarino C., Lippa L., Munari M., Castioni C. A., Robba C., Caricato A., Pompucci A., Signoretti S., Zona G., Rasulo F. A., Aimar E., Amato S., Angileri F. F., Anile C., Assietti R., Baratto V., Barbanera A., Basile L., Battaglia R., Bellocchi S., Bertuccio A., Blanco S., Bolognini A., Boniferro B., Bordi L., Bortolotti C., Brandini V., Broger M., Brollo M., Caffarella D. D., Caggiano C., Cantisani P. L., Capone C., Cappelletto B., Capuano C., Carangelo B., Caruselli G., Chessa M. A., Chiara M., Chibbaro S., Cioffi V., Ciprianocecchi P., Colistra D., Conti C., Contratti F., Costella G. B., Cuoci A., D'Avella D., D'Ercole M., Deangelis M., Defalco R., de Luca G., de Marinis P., Del Vecchio C., Delfinis C., Denaro L., Deodato F., Desogus N., Disomma A., Domenicucci M., Dones F., Fina M., Fiori L., Fricia M., Gaetani P., Gazzeri R., Gentile M., Germano A., Ghadirpour R., Gianfreda C. D., Gigante N., Gigli R., Giorgetti J., Giusa M., Gravina U. G., Grippi L., Guida F., Guizzardi G., Iannuzzo G., Kropp M., Lattanzi L., Lucantoni D., Maffei L., Magliulo M., Marconi F., Marruzzo D., Martellotta N., Marton E., Maugeri R., Mauro G., Meli F., Menniti A., Merciadri P., Milanese L., Nardacci B., Nasi D., Orvieto P., Pacca P., Pansini G., Panzarasa G., Passanisi M., Pavesi G., Pizzoni C., Pulera F., Rapana A., Ricci A., Rispoli R., Rotondo M., Russo N., Santilli S., Scarano E., Schwarz A., Servadei F., Simonetti G., Stefini R., Talamonti G., Turrisi A., Valente V. M., Villa A., Vindigni M., Visocchi M., Vitali M., Wierzbicki V., Zambon G., Zanotti B., Zenga F., Alampi D., Alessandri F., Aloj F., Amigoni A., Aspide R., Bertuetti R., Betti V., Bilotta F., Bonato V., Bosco E., Brita M., Buscema G., Cafiero T., Cappuccio D., Caradonna M., Caria C. G., Casartelliliviero M., Ciritella P., Cirrincione S., Citerio G., Colelli S., Coletta F., Concordia L., Congedo E., Covotta M., Crimella F., Dall'Acqua G., De Cassai A., Defulviis S., Deperi E., Deana C., Delgaudio A., Denittis N., Dicolandrea S., Divezza F., Ferri F., Flocco R., Fontana C., Forastierimolinari A., Frangiosa A., Fumagalli P., Fuselli E., Garbarino M. M., Gelormini D., Geraci C., Geraldini F., Giacomucci A., Giampaoli V., Giorgetti D., Gritti P., Gualdani S., Iacovazzo C., Iermano C., Latronico N., Lugari S., Lusenti F., Maglione C., Magnoni S., Maiarota F., Malla M., Marchesi M., Martino C., Matteotti I., Mazzeo A. T., Morello G., Nardiello I., Paticchio F., Pegoli M., Perotti V., Piazzolla M., Picciafuochi F., Rachedi N., Radolovich D. K., Recchia A., Riccardi S., Romagnoli S., Sala S., Scafuro M. A., Sgarlata P., Soragni A., Stefani F., Stival E., Stofella G., Terranova F., Tinturini R., Togni T., Toto R., Trapani D., Tringali E., Tullo L., Valente A., Valeo T., Varelli G., Villani R., Zamacavicchi F., Zanello M., Zarrillo N., and Zugni N.

Abstract

No robust evidence is provided by literature regarding the management of intracranial hypertension following severe traumatic brain injury (TBI). This is mostly due to the lack of prospective randomized controlled trials (RCTs), the presence of studies containing extreme heterogeneously collected populations and controversial considerations about chosen outcome. A scientific society should provide guidelines for care management and scientific support for those areas for which evidence-based medicine has not been identified. However, RCTs in severe TBI have failed to establish intervention effectiveness, arising the need to make greater use of tools such as Consensus Conferences between experts, which have the advantage of providing recommendations based on experience, on the analysis of updated literature data and on the direct comparison of different logistic realities. The Italian scientific societies should provide guidelines following the national laws ruling the best medical practice. However, many limitations do not allow the collection of data supporting high levels of evidence for intracranial pressure (ICP) monitoring and decompressive craniectomy (DC) in patients with severe TBI. This intersociety document proposes best practice guidelines for this subsetting of patients to be adopted on a national Italian level, along with joint statements from “TBI Section” of the Italian Society of Neurosurgery (SINch) endorsed by the Neuroanesthesia and Neurocritical Care Study Group of the Italian Society of Anesthesia, Analgesia, Resuscitation and Intensive Care (SIAARTI). Presented here is a recap of recommendations on management of ICP and DC supported a high level of available evidence and rate of agreement expressed by the assemblies during the more recent consensus conferences, where members of both groups have had a role of active participants and supporters. The listed recommendations have been sent to a panel of experts consisting of the 107 members of the “T

Published
2021

12. Minimal residual disease–driven treatment intensification with sequential addition of ibrutinib to venetoclax in R/R CLL

Author

Scarfo, L., Heltai, S., Albi, E., Scarano, E., Schiattone, L., Farina, L., Moia, R., Deodato, M., Ferrario, Alberto Alfredo, Motta, M., Reda, G., Sancetta, R., Coscia, M., Rivela, P., Laurenti, Luca, Varettoni, M., Perotta, E., Capasso, A., Ranghetti, P., Colia, M., Ghia, P., Ferrario A., Laurenti L. (ORCID:0000-0002-8327-1396), Scarfo, L., Heltai, S., Albi, E., Scarano, E., Schiattone, L., Farina, L., Moia, R., Deodato, M., Ferrario, Alberto Alfredo, Motta, M., Reda, G., Sancetta, R., Coscia, M., Rivela, P., Laurenti, Luca, Varettoni, M., Perotta, E., Capasso, A., Ranghetti, P., Colia, M., Ghia, P., Ferrario A., and Laurenti L. (ORCID:0000-0002-8327-1396)

Abstract

Undetectable measurable residual disease (uMRD) is achievable in patients with chronic lymphocytic leukemia (CLL) with the BCL2-inhibitor venetoclax alone or combined with the Bruton's tyrosine kinase inhibitor ibrutinib. This phase 2, multicenter, MRD-driven study was designed to discontinue treatment upon reaching uMRD4 (<10−4) in patients with relapsed/refractory CLL receiving venetoclax monotherapy or after the addition of ibrutinib. Primary end point of the study was proportion of uMRD4 with venetoclax ± ibrutinib. Secondary end points were overall response rate, partial response, complete response, progression-free survival, duration of response, overall survival, and safety of venetoclax ± ibrutinib. Patients with uMRD4 at Cycle 12 Day 1 discontinued venetoclax. MRD+ patients added ibrutinib and continued both drugs up to Cycle 24 Day 28/uMRD4/progression/toxicity. After Cycle 24 Day 28, MRD+ patients continued ibrutinib. Thirty-eight patients (29% with TP53 aberrations; 79% with unmutated IGHV) started venetoclax. Overall response rate with venetoclax was 36 (95%) of 38 patients (20 complete; 16 partial response). Seventeen patients (45%) with uMRD4 at Cycle 12 Day 1 discontinued venetoclax. Nineteen (55%) MRD+ subjects added ibrutinib. After a median of 7 months (range, 3-10 months) of combined treatment, 16 (84%) of 19 achieved uMRD4, thus stopping both drugs. Two MRD+ patients at Cycle 24 Day 28 continued ibrutinib until progression/toxicity. After a median follow-up of 36.5 months, median progression-free survival was not reached; 10 patients progressed (4 restarted venetoclax, 3 without treatment need, 2 developed Richter transformation, and 1 dropped out). Seven (22%) of 32 patients remain uMRD4 after 3 years of follow-up. Neutropenia was the most frequent grade 3 to 4 adverse event; no grade 5 events occurred on study. This sequential MRD-guided approach led to uMRD4 in 33 (87%) of 38 patients, with venetoclax monotherapy or combined with ibrutinib

Published
2022

13. Long-term auditory follow-up in the management of pediatric platinum-induced ototoxicity

Author

Fetoni, Anna Rita, Brigato, F., De Corso, Eugenio, Lucidi, D., Sergi, Bruno, Scarano, E., Galli, Jacopo, Ruggiero, Antonio, Fetoni A. R. (ORCID:0000-0001-5405-4301), De Corso E., Sergi B. (ORCID:0000-0001-8648-5966), Galli J. (ORCID:0000-0001-6353-6249), Ruggiero A. (ORCID:0000-0002-6052-3511), Fetoni, Anna Rita, Brigato, F., De Corso, Eugenio, Lucidi, D., Sergi, Bruno, Scarano, E., Galli, Jacopo, Ruggiero, Antonio, Fetoni A. R. (ORCID:0000-0001-5405-4301), De Corso E., Sergi B. (ORCID:0000-0001-8648-5966), Galli J. (ORCID:0000-0001-6353-6249), and Ruggiero A. (ORCID:0000-0002-6052-3511)

Abstract

Purpose: Irreversible bilateral sensorineural hearing loss is a common side effect of platinum compounds. Because of the extended overall survival, a prolonged hearing surveillance and management of hearing impairments are emerging concerns for pediatric oncology. Methods: In this retrospective observational study, we enrolled 38 children out of 116 treated at our institution by chemotherapy (cisplatin and/or carboplatin) with or without irradiation between 2007 and 2014, submitted to hearing monitoring before every cycle of chemotherapy, and who completed a 5-year long-term audiological follow-up. Chemotherapy regimens, demographic findings, cumulative doses, and cranial irradiation were compared. Results: At the end of 5-year follow-up, ototoxicity was significantly increased compared to that observed at the end of chemotherapy (52.5% vs 39.5%, p < 0.001). A late onset of hearing loss was experienced in 13.1% of children, while in 26.3% progressive hearing loss was measured. Deafness at the end of chemotherapy and irradiation were significant prognostic factors for late ototoxicity outcomes (Odds Ratio 7.2—CI 1.67–31.1—p < 0.01 and 5.25—CI 1.26–21.86—p < 0.01 respectively). No significant differences were found between cisplatin and combined treatment (i.e., cisplatin shifted to carboplatin during monitoring for the onset of ototoxicity) and ototoxicity was not associated with platinum compounds cumulative dose (p > 0.05). 13.1% of children needed hearing aids at the end of follow-up. Conclusion: Long-term monitoring of at least 5 years prevents the harmful effects of hearing deprivation identifying late onset/progressive hearing loss after platinum compound chemotherapy in children thanks to early hearing rehabilitation, especially in those who underwent multimodal therapy or subjected to irradiation.

Published
2022

14. Article craniopharyngioma, chronotypes and metabolic risk profile

Author

Di Somma C., Scarano E., Barrea L., Solari D., Riccio E., Arianna R., Cavallo L. M., Romano F., Di Benedetto E., Rodriguez A., de Alteriis G., Colao A., Di Somma, C., Scarano, E., Barrea, L., Solari, D., Riccio, E., Arianna, R., Cavallo, L. M., Romano, F., Di Benedetto, E., Rodriguez, A., de Alteriis, G., and Colao, A.

Subjects
Adult, Male, Chronotype, Risk Factor, Blood Pressure, Body Weights and Measure, Biomarker, Middle Aged, Risk Assessment, Circadian Rhythm, Pituitary neoplasm, Craniopharyngioma, Hypothalamu, Female, Obesity, Disease Susceptibility, Case-Control Studie, Energy Metabolism, Human
Abstract

Aim: To investigate the potential association among Craniopharyngioma (CP), chronotypes and metabolic risk profile. Subjects and Methods: The study population included 28 patients (46.4% males; 42.6 ± 15.8 years) and 28 controls, age, gender and BMI matched (46.4% males; 46.5 ± 12.9 years). In this study sample, we evaluated: anthropometric measurements (waist circumference, WC; BMI), plasma glucose, lipid profile, and systolic (SBP) and diastolic (DBP) blood pressure. Morningness-Eveningness was measured with the Horne-Ostberg Morningness-Eveningness Questionnaire (MEQ), which included 19 questions about preferred sleep time and daily performance. Results: in both patients and controls grade I obesity was detected in 15 subjects (53.6%), grade II obesity in 13 subjects (46.4%). In the patient group, the mean score of chronotype was 47.8 ± 12.6. In particular, 9 patients (32.1%) exhibited the morning chronotype, 6 (21.4%) the intermediate chronotype and 13 (46.4.%) the evening chronotype. No significant difference was found in gender and age among the chronotype categories. Patients with the evening chronotype had higher blood pressure values and worse metabolic parameters than those with the morning chronotype. In the control group, the mean score of the chronotype was 57.6 ± 9.5. In particular, 16 (57.1%) subjects exhibited the morning chronotype, 10 (35.7%) the intermediate chronotype and only 2 (7.1.%) the evening chronotype. The prevalence of intermediate and evening chronotypes was higher in females than males (p = 0.021), while males have a higher prevalence of the morning chronotype. Subjects with intermediate and evening chronotypes had worse metabolic parameters than those with the morning chronotype. In patients, the chronotype score was inversely correlated to WC, BMI, SBP, DBP, plasma glucose, total cholesterol, triglycerides, LDL cholesterol and positively correlated with HDL cholesterol. No correlation was found between age and chronotype. In controls, the chronotype score was inversely correlated to WC, BMI, plasma glucose, total cholesterol, LDL cholesterol. No correlation was found among chronotype and age, blood pressure, triglycerides, HDL cholesterol. Considering the whole population of the study (patients and controls), at logistic regression the chronotype score was significantly associated with the presence of CP. Conclusions: for the first time thus far, our study puts the light on the association of the CP with chronotypes and metabolic alterations in this disease, which are the main determinants of the reduced quality of life, higher morbidity and mortality in this setting of patients. This finding suggests that alterations of chronotype might represent an adjunctive risk for CP patients and a possible target for their integrate management.

Published
2021

15. Expanding the phenotype associated to KMT2A variants: overlapping clinical signs between Wiedemann–Steiner and Rubinstein–Taybi syndromes

Author

Di Fede E., Massa V., Augello B., Squeo G., Scarano E., Perri A. M., Fischetto R., Causio F. A., Zampino G., Piccione M., Curridori E., Mazza T., Castellana S., Larizza L., Ghelma F., Colombo E. A., Gandini M. C., Castori M., Merla G., Milani D., Gervasini C., Di Fede E., Massa V., Augello B., Squeo G., Scarano E., Perri A.M., Fischetto R., Causio F.A., Zampino G., Piccione M., Curridori E., Mazza T., Castellana S., Larizza L., Ghelma F., Colombo E.A., Gandini M.C., Castori M., Merla G., Milani D., and Gervasini C.

Subjects
Adult, Male, Rubinstein-Taybi Syndrome, Adolescent, Histone-Lysine N-Methyltransferase, Wiedemann–Steiner, Article, Phenotype, Settore MED/03 - Genetica Medica, Settore MED/38 - PEDIATRIA GENERALE E SPECIALISTICA, KMT2A variants, Mutation, Humans, Female, Epigenetics, Rubinstein–Taybi syndromes, Child, KMT2A Gene, Wiedemann–Steiner syndrome, Rubinstein–Taybi syndrome, Myeloid-Lymphoid Leukemia Protein
Abstract

Lysine-specific methyltransferase 2A (KMT2A) is responsible for methylation of histone H3 (K4H3me) and contributes to chromatin remodeling, acting as “writer” of the epigenetic machinery. Mutations in KMT2A were first reported in Wiedemann–Steiner syndrome (WDSTS). More recently, KMT2A variants have been described in probands with a specific clinical diagnosis comprised in the so-called chromatinopathies. Such conditions, including WDSTS, are a group of overlapping disorders caused by mutations in genes coding for the epigenetic machinery. Among them, Rubinstein–Taybi syndrome (RSTS) is mainly caused by heterozygous pathogenic variants in CREBBP or EP300. In this work, we used next generation sequencing (either by custom-made panel or by whole exome) to identify alternative causative genes in individuals with a RSTS-like phenotype negative to CREBBP and EP300 mutational screening. In six patients we identified different novel unreported variants in KMT2A gene. The identified variants are de novo in at least four out of six tested individuals and all of them display some typical RSTS phenotypic features but also WDSTS specific signs. This study reinforces the concept that germline variants affecting the epigenetic machinery lead to a shared molecular effect (alteration of the chromatin state) determining superimposable clinical conditions.

Published
2021

16. Mowat-Wilson syndrome:growth charts

Author

Ivanovski I., Djuric O., Broccoli S., Caraffi S. G., Accorsi P., Adam M. P., Avela K., Badura-Stronka M., Bayat A., Clayton-Smith J., Cocco I., Cordelli D. M., Cuturilo G., Di Pisa V., Dupont Garcia J., Gastaldi R., Giordano L., Guala A., Hoei-Hansen C., Inaba M., Iodice A., Nielsen J. E. K., Kuburovic V., Lazalde-Medina B., Malbora B., Mizuno S., Moldovan O., Moller R. S., Muschke P., Otelli V., Pantaleoni C., Piscopo C., Poch-Olive M. L., Prpic I., Marin Reina P., Raviglione F., Ricci E., Scarano E., Simonte G., Smigiel R., Tanteles G., Tarani L., Trimouille A., Valera E. T., Schrier Vergano S., Writzl K., Callewaert B., Savasta S., Street M. E., Iughetti L., Bernasconi S., Giorgi Rossi P., Garavelli L., HUSLAB, Clinicum, Department of Medical and Clinical Genetics, Helsinki University Hospital Area, Ivanovski I., Djuric O., Broccoli S., Caraffi S.G., Accorsi P., Adam M.P., Avela K., Badura-Stronka M., Bayat A., Clayton-Smith J., Cocco I., Cordelli D.M., Cuturilo G., Di Pisa V., Dupont Garcia J., Gastaldi R., Giordano L., Guala A., Hoei-Hansen C., Inaba M., Iodice A., Nielsen J.E.K., Kuburovic V., Lazalde-Medina B., Malbora B., Mizuno S., Moldovan O., Moller R.S., Muschke P., Otelli V., Pantaleoni C., Piscopo C., Poch-Olive M.L., Prpic I., Marin Reina P., Raviglione F., Ricci E., Scarano E., Simonte G., Smigiel R., Tanteles G., Tarani L., Trimouille A., Valera E.T., Schrier Vergano S., Writzl K., Callewaert B., Savasta S., Street M.E., Iughetti L., Bernasconi S., Giorgi Rossi P., and Garavelli L.

Subjects
Male, 0301 basic medicine, BIOMEDICINE AND HEALTHCARE. Clinical Medical Sciences, Pediatrics, Microcephaly, FEATURES, lcsh:Medicine, CHILDREN, 030105 genetics & heredity, Head circumference, DISEASE, 0302 clinical medicine, Intellectual disability, Medicine and Health Sciences, Genetics(clinical), Pharmacology (medical), Mowat-Wilson syndrome, Child, Genetics (clinical), Body mass index, ZEB2, 2. Zero hunger, education.field_of_study, 0303 health sciences, BIOMEDICINA I ZDRAVSTVO. Kliničke medicinske znanosti, Mowat-Wilson syndrome, ZEB2, Growth charts, Weight, Length, Height, Head circumference, Body mass index, BM, 1184 Genetics, developmental biology, physiology, General Medicine, STATISTICS, 3. Good health, MORFOMETRIA, Italy, 030220 oncology & carcinogenesis, Female, medicine.medical_specialty, Mowat–Wilson syndrome, Length, Population, BMI, Growth charts, Height, Weight, Growth chart, 03 medical and health sciences, AGE, Intellectual Disability, medicine, Humans, In patient, Hirschsprung Disease, education, Zinc Finger E-box Binding Homeobox 2, 030304 developmental biology, Homeodomain Proteins, Physical development, MUTATIONS, business.industry, Research, lcsh:R, Infant, Newborn, Facies, Infant, medicine.disease, Repressor Proteins, DELINEATION, INDIVIDUALS, 030104 developmental biology, 3111 Biomedicine, business
Abstract

Background: Mowat–Wilson syndrome (MWS; OMIM #235730) is a genetic condition caused by heterozygous mutations or deletions of the ZEB2 gene. It is characterized by moderate-severe intellectual disability, epilepsy, Hirschsprung disease and multiple organ malformations of which congenital heart defects and urogenital anomalies are the most frequent ones. To date, a clear description of the physical development of MWS patients does not exist. The aim of this study is to provide up-to-date growth charts specific for infants and children with MWS. Charts for males and females aged from 0 to 16 years were generated using a total of 2,865 measurements from 99 MWS patients of different ancestries. All data were collected through extensive collaborations with the Italian MWS association (AIMW) and the MWS Foundation. The GAMLSS package for the R statistical computing software was used to model the growth charts. Height, weight, body mass index (BMI) and head circumference were compared to those from standard international growth charts for healthy children.Results: In newborns, weight and length were distributed as in the general population, while head circumference was slightly smaller, with an average below the 30th centile. Up to the age of 7 years, weight and height distribution was shifted to slightly lower values than in the general population; after that, the difference increased further, with 50% of the affected children below the 5th centile of the general population. BMI distribution was similar to that of non-affected children until the age of 7 years, at which point values in MWS children increased with a less steep slope, particularly in males. Microcephaly was sometimes present at birth, but in most cases it developed gradually during infancy; many children had a small head circumference, between the 3rd and the 10th centile, rather than being truly microcephalic (at least 2 SD below the mean). Most patients were of slender build. Conclusions: These charts contribute to the understanding of the natural history of MWS and should assist pediatricians and other caregivers in providing optimal care to MWS individuals who show problems related to physical growth. This is the first study on growth in patients with MWS.

Published
2020

18. Neutrophil-to-lymphocyte ratio (NLR) and platelet-to-lymphocyte ratio (PLR) in Ménière’s disease and vestibular neuritis

Author

Quaranta, N. A. A., Salzo, A. E., Pontillo, V., Scarano, Emanuele, Sergi, Bruno, Picciotti, Pasqualina Maria, Scarano E. (ORCID:0000-0003-2570-1121), Sergi B. (ORCID:0000-0001-8648-5966), Picciotti P. M. (ORCID:0000-0002-1502-6508), Quaranta, N. A. A., Salzo, A. E., Pontillo, V., Scarano, Emanuele, Sergi, Bruno, Picciotti, Pasqualina Maria, Scarano E. (ORCID:0000-0003-2570-1121), Sergi B. (ORCID:0000-0001-8648-5966), and Picciotti P. M. (ORCID:0000-0002-1502-6508)

Abstract

Objective: several hypothesis including viral infection, vascular disturbance, and immune-mediated mechanisms have been proposed in Menière disease (MD) and vestibular neuritis (VN) and the role of inflammation has been also investigated. To evaluate the role of neutrophil to lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR) and metabolic parameters in MD and VN patients. Methods: we retrospectively studied 120 subjects (40 affected by MD, 40 by VN and 40 normal controls). We analyzed glycaemia, Haematocrit Blood Test (HCT), leukocytes, neutrophils, lymphocytes, platelets, cholesterol, prothrombin time and the related international normalized ratio (PT-INR), fibrinogen, erythrocyte sedimentation rate (ESR), NLR and PLR. Results: Neutrophils, leukocytes, cholesterol, ESR, NLR and PLR values of MD patients are significantly higher than normal controls. Higher significant values are reported for glycaemia, neutrophils, lymphocytes, leucocyte, fibrinogen, ESR, NLR and PLR in VN patients compared to NC. Conclusions: High levels of NLR and PLR indicate the presence of an acute inflammatory state in both MD and VN. Our data confirm the role of proinflammatory state leading to microvascular injury together with risk factors for atherogenesis. © 2021 International Association of Physicians in Audiology.

Published
2021

19. Prevalence of signs and symptoms of temporo-mandibular disorder in patients with sleep apnea

Author

Alessandri-Bonetti, A., Scarano, Emanuele, Fiorita, Antonella, Cordaro, Massimo, Gallenzi, Patrizia, Scarano E. (ORCID:0000-0003-2570-1121), Fiorita A., Cordaro M. (ORCID:0000-0002-0797-5172), Gallenzi P. (ORCID:0000-0001-9805-4522), Alessandri-Bonetti, A., Scarano, Emanuele, Fiorita, Antonella, Cordaro, Massimo, Gallenzi, Patrizia, Scarano E. (ORCID:0000-0003-2570-1121), Fiorita A., Cordaro M. (ORCID:0000-0002-0797-5172), and Gallenzi P. (ORCID:0000-0001-9805-4522)

Abstract

Purpose: The aim of the present study was to detect the prevalence of temporomandibular disorders (TMD) in patients with untreated obstructive sleep apnea (OSA) and to compare the results with healthy controls, matched for sex and age. Methods: Forty-one consecutive patients with OSA were prospectively recruited from the Department of Otorhinolaryngology at the A. Gemelli Hospital prior to undergoing any treatment for OSA and independently of OSA severity. All patients underwent a complete TMD examination according to the diagnostic criteria for temporomandibular disorders (DC/TMD) protocol. The same examination was performed on 41 healthy controls matched for sex and age. Chi-squared test was used to compare results between the two groups. Results: Of the 41 patients with OSA, 21 (51%) presented signs and/or symptoms of TMD compared to 13 of 41 subjects (32%) from the control group. Headache attributed to TMD and disc displacement with reduction were the most common diagnoses, with a statistically significant difference between the two groups (p<0.05). Conclusions: The prevalence of TMD signs and symptoms is significantly higher in untreated patients with OSA compared to healthy controls.

Published
2021

20. Multidisciplinary approach to orbital decompression. A review

Author

Parrilla, Claudio, Mele, Dario Antonio, Gelli, Silvia, Zelano, Lorenzo, Bussu, Francesco, Rigante, Mario, Savino, Gustavo, Scarano, Emanuele, Parrilla C., Mele D. A., Gelli S., Zelano L., Bussu F. (ORCID:0000-0001-6261-2772), Rigante M. (ORCID:0000-0002-6111-0786), Savino G. (ORCID:0000-0002-9993-5986), Scarano E. (ORCID:0000-0003-2570-1121), Parrilla, Claudio, Mele, Dario Antonio, Gelli, Silvia, Zelano, Lorenzo, Bussu, Francesco, Rigante, Mario, Savino, Gustavo, Scarano, Emanuele, Parrilla C., Mele D. A., Gelli S., Zelano L., Bussu F. (ORCID:0000-0001-6261-2772), Rigante M. (ORCID:0000-0002-6111-0786), Savino G. (ORCID:0000-0002-9993-5986), and Scarano E. (ORCID:0000-0003-2570-1121)

Abstract

Endoscopic orbital surgery has become a highly evolving multidisciplinary surgical field thanks to development in technical skills of ophthalmologists and otolaryngologists. These advances expanded the clinical application of orbital decompression, with a growing body of literature describing the multidisciplinary management of thyroid eye disease and com-pressive optic neuropathy, since 1990. Although techniques have improved considerably, only few Randomized Control Trials (RCT) provide evidence to support recommendations in clinical practice. This review provides an overview of the current knowledge of orbital decompression to clarify which is the most standardized therapeutic strategy. In the literature, we observed several approaches with contradicting results and the comparison of different surgical techniques was biased by inclusion of patients at different stage of disease (active or inactive), different surgical indications (dysthyroid neuropathy or disfiguring proptosis) and measures of outcomes (such as different system for ocular motility evaluation). The timing of surgical decompression is one of the debated issues. One RCT focusing on Graves’ orbitopathy showed how intravenous corticosteroids achieve better visual recovery than surgical orbital decompression; but in case of absent or poor response to medical therapy the patient should undergo surgery within two weeks. There is slight evidence that the removal of the medial and lateral wall (so-called balanced decompression) with or without fat removal could be the most effective surgical technique, with low com-plication rate, but an increasing number of authors are promoting, for selected cases, a pure endoscopic surgical approach (with removal of medial and infero-medial orbital wall), less invasive than the balanced one; the latter indicated to more severe proptosis or diplopia after endoscopic procedure. Three-wall decompression is chosen for high degrees of proptosis, but complications are more fr

Published
2021

21. Management of intracranial hypertension following traumatic brain injury: A best clinical practice adoption proposal for intracranial pressure monitoring and decompressive craniectomy: Joint statements by the Traumatic Brain Injury Section of the Italian Society of Neurosurgery (SINch) and the Neuroanesthesia and Neurocritical Care Study Group of the Italian Society of Anesthesia, Analgesia, Resuscitation and Intensive Care (SIAARTI)

Author

Iaccarino, C., Lippa, L., Munari, M., Castioni, C. A., Robba, C., Caricato, Anselmo, Pompucci, Angelo, Signoretti, S., Zona, G., Rasulo, F. A., Aimar, E., Amato, S., Angileri, F. F., Anile, Carmelo, Assietti, R., Baratto, V., Barbanera, A., Basile, L., Battaglia, R., Bellocchi, S., Bertuccio, A., Blanco, S., Bolognini, A., Boniferro, B., Bordi, L., Bortolotti, C., Brandini, V., Broger, Maximilian, Brollo, M., Caffarella, D. D., Caggiano, Cinzia, Cantisani, P. L., Capone, C., Cappelletto, B., Capuano, C., Carangelo, B., Caruselli, G., Chessa, M. A., Chiara, M., Chibbaro, S., Cioffi, V., Ciprianocecchi, P., Colistra, D., Conti, C., Contratti, F., Costella, G. B., Cuoci, A., D'Avella, D., D'Ercole, Manuela, Deangelis, M., Defalco, R., de Luca, G., de Marinis, P., Del Vecchio, C., Delfinis, C., Denaro, Luca, Deodato, F., Desogus, N., Disomma, A., Domenicucci, M., Dones, F., Fina, M., Fiori, L., Fricia, M., Gaetani, P., Gazzeri, R., Gentile, M., Germano, A., Ghadirpour, R., Gianfreda, C. D., Gigante, N., Gigli, R., Giorgetti, J., Giusa, M., Gravina, U. G., Grippi, L., Guida, F., Guizzardi, G., Iannuzzo, G., Kropp, M., Lattanzi, L., Lucantoni, D., Maffei, L., Magliulo, M., Marconi, F., Marruzzo, D., Martellotta, N., Marton, E., Maugeri, R., Mauro, G., Meli, F., Menniti, A., Merciadri, P., Milanese, L., Nardacci, B., Nasi, D., Orvieto, P., Pacca, P., Pansini, G., Panzarasa, G., Passanisi, M., Pavesi, G., Pizzoni, C., Pulera, F., Rapana, A., Ricci, A., Rispoli, R., Rotondo, M., Russo, N., Santilli, S., Scarano, E., Schwarz, A., Servadei, Franco, Simonetti, G., Stefini, R., Talamonti, G., Turrisi, A., Valente, V. M., Villa, A., Vindigni, M., Visocchi, Massimiliano, Vitali, M., Wierzbicki, V., Zambon, G., Zanotti, B., Zenga, F., Alampi, D., Alessandri, F., Aloj, F., Amigoni, A., Aspide, R., Bertuetti, R., Betti, V., Bilotta, F., Bonato, V., Bosco, E., Brita, M., Buscema, G., Cafiero, T., Cappuccio, D., Caradonna, M., Caria, C. G., Casartelliliviero, M., Ciritella, P., Cirrincione, S., Citerio, G., Colelli, S., Coletta, F., Concordia, L., Congedo, E., Covotta, M., Crimella, F., Dall'Acqua, G., De Cassai, A., Defulviis, S., Deperi, E., Deana, C., Delgaudio, A., Denittis, N., Dicolandrea, S., Divezza, F., Ferri, F., Flocco, R., Fontana, C., Forastierimolinari, A., Frangiosa, A., Fumagalli, P., Fuselli, E., Garbarino, M. M., Gelormini, D., Geraci, C., Geraldini, F., Giacomucci, A., Giampaoli, V., Giorgetti, D., Gritti, P., Gualdani, S., Iacovazzo, C., Iermano, C., Latronico, N., Lugari, S., Lusenti, F., Maglione, C., Magnoni, S., Maiarota, F., Malla, M., Marchesi, M., Martino, C., Matteotti, I., Mazzeo, A. T., Morello, G., Nardiello, I., Paticchio, F., Pegoli, M., Perotti, Valerio, Piazzolla, M., Picciafuochi, F., Rachedi, N., Radolovich, D. K., Recchia, A., Riccardi, S., Romagnoli, S., Sala, S., Scafuro, M. A., Sgarlata, P., Soragni, A., Stefani, F., Stival, Eleonora, Stofella, G., Terranova, F., Tinturini, R., Togni, T., Toto, R., Trapani, D., Tringali, E., Tullo, L., Valente, A., Valeo, T., Varelli, G., Villani, R., Zamacavicchi, F., Zanello, M., Zarrillo, N., Zugni, N., Caricato A. (ORCID:0000-0001-5929-120X), Pompucci A. (ORCID:0000-0002-5427-9719), Anile C. (ORCID:0000-0002-0481-9713), Broger M., Caggiano C., D'Ercole M., Denaro L., Servadei F., Visocchi M. (ORCID:0000-0003-1087-0491), Perotti V. (ORCID:0000-0001-9461-2101), Stival E., Iaccarino, C., Lippa, L., Munari, M., Castioni, C. A., Robba, C., Caricato, Anselmo, Pompucci, Angelo, Signoretti, S., Zona, G., Rasulo, F. A., Aimar, E., Amato, S., Angileri, F. F., Anile, Carmelo, Assietti, R., Baratto, V., Barbanera, A., Basile, L., Battaglia, R., Bellocchi, S., Bertuccio, A., Blanco, S., Bolognini, A., Boniferro, B., Bordi, L., Bortolotti, C., Brandini, V., Broger, Maximilian, Brollo, M., Caffarella, D. D., Caggiano, Cinzia, Cantisani, P. L., Capone, C., Cappelletto, B., Capuano, C., Carangelo, B., Caruselli, G., Chessa, M. A., Chiara, M., Chibbaro, S., Cioffi, V., Ciprianocecchi, P., Colistra, D., Conti, C., Contratti, F., Costella, G. B., Cuoci, A., D'Avella, D., D'Ercole, Manuela, Deangelis, M., Defalco, R., de Luca, G., de Marinis, P., Del Vecchio, C., Delfinis, C., Denaro, Luca, Deodato, F., Desogus, N., Disomma, A., Domenicucci, M., Dones, F., Fina, M., Fiori, L., Fricia, M., Gaetani, P., Gazzeri, R., Gentile, M., Germano, A., Ghadirpour, R., Gianfreda, C. D., Gigante, N., Gigli, R., Giorgetti, J., Giusa, M., Gravina, U. G., Grippi, L., Guida, F., Guizzardi, G., Iannuzzo, G., Kropp, M., Lattanzi, L., Lucantoni, D., Maffei, L., Magliulo, M., Marconi, F., Marruzzo, D., Martellotta, N., Marton, E., Maugeri, R., Mauro, G., Meli, F., Menniti, A., Merciadri, P., Milanese, L., Nardacci, B., Nasi, D., Orvieto, P., Pacca, P., Pansini, G., Panzarasa, G., Passanisi, M., Pavesi, G., Pizzoni, C., Pulera, F., Rapana, A., Ricci, A., Rispoli, R., Rotondo, M., Russo, N., Santilli, S., Scarano, E., Schwarz, A., Servadei, Franco, Simonetti, G., Stefini, R., Talamonti, G., Turrisi, A., Valente, V. M., Villa, A., Vindigni, M., Visocchi, Massimiliano, Vitali, M., Wierzbicki, V., Zambon, G., Zanotti, B., Zenga, F., Alampi, D., Alessandri, F., Aloj, F., Amigoni, A., Aspide, R., Bertuetti, R., Betti, V., Bilotta, F., Bonato, V., Bosco, E., Brita, M., Buscema, G., Cafiero, T., Cappuccio, D., Caradonna, M., Caria, C. G., Casartelliliviero, M., Ciritella, P., Cirrincione, S., Citerio, G., Colelli, S., Coletta, F., Concordia, L., Congedo, E., Covotta, M., Crimella, F., Dall'Acqua, G., De Cassai, A., Defulviis, S., Deperi, E., Deana, C., Delgaudio, A., Denittis, N., Dicolandrea, S., Divezza, F., Ferri, F., Flocco, R., Fontana, C., Forastierimolinari, A., Frangiosa, A., Fumagalli, P., Fuselli, E., Garbarino, M. M., Gelormini, D., Geraci, C., Geraldini, F., Giacomucci, A., Giampaoli, V., Giorgetti, D., Gritti, P., Gualdani, S., Iacovazzo, C., Iermano, C., Latronico, N., Lugari, S., Lusenti, F., Maglione, C., Magnoni, S., Maiarota, F., Malla, M., Marchesi, M., Martino, C., Matteotti, I., Mazzeo, A. T., Morello, G., Nardiello, I., Paticchio, F., Pegoli, M., Perotti, Valerio, Piazzolla, M., Picciafuochi, F., Rachedi, N., Radolovich, D. K., Recchia, A., Riccardi, S., Romagnoli, S., Sala, S., Scafuro, M. A., Sgarlata, P., Soragni, A., Stefani, F., Stival, Eleonora, Stofella, G., Terranova, F., Tinturini, R., Togni, T., Toto, R., Trapani, D., Tringali, E., Tullo, L., Valente, A., Valeo, T., Varelli, G., Villani, R., Zamacavicchi, F., Zanello, M., Zarrillo, N., Zugni, N., Caricato A. (ORCID:0000-0001-5929-120X), Pompucci A. (ORCID:0000-0002-5427-9719), Anile C. (ORCID:0000-0002-0481-9713), Broger M., Caggiano C., D'Ercole M., Denaro L., Servadei F., Visocchi M. (ORCID:0000-0003-1087-0491), Perotti V. (ORCID:0000-0001-9461-2101), and Stival E.

Abstract

No robust evidence is provided by literature regarding the management of intracranial hypertension following severe traumatic brain injury (TBI). This is mostly due to the lack of prospective randomized controlled trials (RCTs), the presence of studies containing extreme heterogeneously collected populations and controversial considerations about chosen outcome. A scientific society should provide guidelines for care management and scientific support for those areas for which evidence-based medicine has not been identified. However, RCTs in severe TBI have failed to establish intervention effectiveness, arising the need to make greater use of tools such as Consensus Conferences between experts, which have the advantage of providing recommendations based on experience, on the analysis of updated literature data and on the direct comparison of different logistic realities. The Italian scientific societies should provide guidelines following the national laws ruling the best medical practice. However, many limitations do not allow the collection of data supporting high levels of evidence for intracranial pressure (ICP) monitoring and decompressive craniectomy (DC) in patients with severe TBI. This intersociety document proposes best practice guidelines for this subsetting of patients to be adopted on a national Italian level, along with joint statements from “TBI Section” of the Italian Society of Neurosurgery (SINch) endorsed by the Neuroanesthesia and Neurocritical Care Study Group of the Italian Society of Anesthesia, Analgesia, Resuscitation and Intensive Care (SIAARTI). Presented here is a recap of recommendations on management of ICP and DC supported a high level of available evidence and rate of agreement expressed by the assemblies during the more recent consensus conferences, where members of both groups have had a role of active participants and supporters. The listed recommendations have been sent to a panel of experts consisting of the 107 members of the “T

Published
2021

22. Not only gustatory sweating and flushing: Signs and symptoms associated to the Frey syndrome and the role of botulinum toxin A therapy

Author

Marchese, Maria Raffaella, Bussu, Francesco, Settimi, Stefano, Scarano, Emanuele, Almadori, Giovanni, Galli, Jacopo, Marchese M. R. (ORCID:0000-0003-0751-0882), Bussu F. (ORCID:0000-0001-6261-2772), Settimi S. (ORCID:0000-0003-0104-1501), Scarano E. (ORCID:0000-0003-2570-1121), Almadori G. (ORCID:0000-0002-4605-2442), Galli J. (ORCID:0000-0001-6353-6249), Marchese, Maria Raffaella, Bussu, Francesco, Settimi, Stefano, Scarano, Emanuele, Almadori, Giovanni, Galli, Jacopo, Marchese M. R. (ORCID:0000-0003-0751-0882), Bussu F. (ORCID:0000-0001-6261-2772), Settimi S. (ORCID:0000-0003-0104-1501), Scarano E. (ORCID:0000-0003-2570-1121), Almadori G. (ORCID:0000-0002-4605-2442), and Galli J. (ORCID:0000-0001-6353-6249)

Abstract

Background: The classic symptoms of Frey syndrome are gustatory sweating and flushing. Aims of the study were to describe prevalenceand severity of typical and atypical presentations of the disorder and to assess the effects of botulinum neurotoxin A (BoNT-A) therapy in patients with Frey syndrome after parotidectomy. Methods: In this prospective, observational study on 18 patients, we assessed symptom severity before therapy, after 15 days, 1, 3 and 6 months' follow-up with the sweating-flushing-itch-paresthesia-pain (SFIPP) Frey scale specifically designed by the authors themselves for this study. Results: Before BoNT-A injection, all patients (100%) complained gustatory sweating, 80% paresthesia, 77% gustatory flushing, 60% pain and 60% gustatory itch. The SFIPP-Frey overall score and the symptom-specific ones decreased significantly at each post-therapy control. Conclusions: The prevalence of “unusual” manifestations is not negligible. BoNT-A improves symptoms severity. The SFIPP-Frey scale may be useful to assess symptoms and to monitor post-therapy outcomes.

Published
2021

23. Periprosthetic Leakage in Tracheoesophageal Prosthesis: Proposal of a Standardized Therapeutic Algorithm

Author

Parrilla, Claudio, Longobardi, Ylenia, Galli, Jacopo, Rigante, Mario, Paludetti, Gaetano, Bussu, Francesco, Scarano, E., Parrilla C., Longobardi Y., Galli J. (ORCID:0000-0001-6353-6249), Rigante M. (ORCID:0000-0002-6111-0786), Paludetti G. (ORCID:0000-0003-2480-1243), Bussu F. (ORCID:0000-0001-6261-2772), Parrilla, Claudio, Longobardi, Ylenia, Galli, Jacopo, Rigante, Mario, Paludetti, Gaetano, Bussu, Francesco, Scarano, E., Parrilla C., Longobardi Y., Galli J. (ORCID:0000-0001-6353-6249), Rigante M. (ORCID:0000-0002-6111-0786), Paludetti G. (ORCID:0000-0003-2480-1243), and Bussu F. (ORCID:0000-0001-6261-2772)

Abstract

Objective: Periprosthetic leakage represents the most demanding long-term complication in the voice prosthesis rehabilitation. The aim of this article is to discuss the various causes of periprosthetic leakage and to propose a systematic management algorithm. Study Design: Retrospective cohort study. Setting: Otolaryngology clinic of the University Polyclinic A. Gemelli–IRCCS Foundation. Methods: The study included 115 patients with voice prosthesis who were treated from December 2014 to December 2019. All patients who experienced periprosthetic leakage were treated with the same step-by-step therapeutic approach until it was successful. Incidence, management, and success rate of every attempt are analyzed and discussed. Results: Periprosthetic leakage was reported 330 times by 82 patients in 1374 clinic accesses. Radiotherapy, timing of tracheoesophageal puncture, and type of total laryngectomy (primary or salvage) did not influence the incidence of periprosthetic leakage. Salvage total laryngectomy increases the risk of more clinically relevant leakages. Conclusion: By using a systematic algorithm with a step-by-step standardized approach, periprosthetic leakage management could become a less treacherous issue.

Published
2021

24. Expanding the phenotype associated to KMT2A variants: overlapping clinical signs between Wiedemann–Steiner and Rubinstein–Taybi syndromes

Author

Di Fede, E., Massa, V., Augello, B., Squeo, G., Scarano, E., Perri, A. M., Fischetto, R., Causio, F. A., Zampino, G., Piccione, M., Curridori, E., Mazza, T., Castellana, S., Larizza, L., Ghelma, F., Colombo, E. A., Gandini, M. C., Castori, M., Merla, G., Milani, D., Gervasini, C., Zampino G. (ORCID:0000-0003-3865-3253), Milani D., Di Fede, E., Massa, V., Augello, B., Squeo, G., Scarano, E., Perri, A. M., Fischetto, R., Causio, F. A., Zampino, G., Piccione, M., Curridori, E., Mazza, T., Castellana, S., Larizza, L., Ghelma, F., Colombo, E. A., Gandini, M. C., Castori, M., Merla, G., Milani, D., Gervasini, C., Zampino G. (ORCID:0000-0003-3865-3253), and Milani D.

Abstract

Lysine-specific methyltransferase 2A (KMT2A) is responsible for methylation of histone H3 (K4H3me) and contributes to chromatin remodeling, acting as “writer” of the epigenetic machinery. Mutations in KMT2A were first reported in Wiedemann–Steiner syndrome (WDSTS). More recently, KMT2A variants have been described in probands with a specific clinical diagnosis comprised in the so-called chromatinopathies. Such conditions, including WDSTS, are a group of overlapping disorders caused by mutations in genes coding for the epigenetic machinery. Among them, Rubinstein–Taybi syndrome (RSTS) is mainly caused by heterozygous pathogenic variants in CREBBP or EP300. In this work, we used next generation sequencing (either by custom-made panel or by whole exome) to identify alternative causative genes in individuals with a RSTS-like phenotype negative to CREBBP and EP300 mutational screening. In six patients we identified different novel unreported variants in KMT2A gene. The identified variants are de novo in at least four out of six tested individuals and all of them display some typical RSTS phenotypic features but also WDSTS specific signs. This study reinforces the concept that germline variants affecting the epigenetic machinery lead to a shared molecular effect (alteration of the chromatin state) determining superimposable clinical conditions.

Published
2021

27. Management of vertigo in pregnancy

Author

Salvati, Alessandra, Apa, Rosanna, Loperfido, A., Scarano, E., Paludetti, Gaetano, Tropea, A., Picciotti, Pasqualina Maria, Salvati A., Apa R. (ORCID:0000-0003-0143-9114), Paludetti G. (ORCID:0000-0003-2480-1243), Picciotti P. M. (ORCID:0000-0002-1502-6508), Salvati, Alessandra, Apa, Rosanna, Loperfido, A., Scarano, E., Paludetti, Gaetano, Tropea, A., Picciotti, Pasqualina Maria, Salvati A., Apa R. (ORCID:0000-0003-0143-9114), Paludetti G. (ORCID:0000-0003-2480-1243), and Picciotti P. M. (ORCID:0000-0002-1502-6508)

Abstract

Background. Pregnancy is a specific female physiological period characterized by significant changes, included oto-logical and neurotological manifestations. Vestibular disorders like vertigo and dizziness, are common complaints from pregnant women to primary care. Objectives. The aim of this paper is to describe clinical pictures, evaluation methods and therapeutic options of acute vertigo in pregnancy with the related pathogenetic hypotheses. Method. We describe 11 cases of vertigo in pregnancy. All patients underwent audio-vestibular evaluation, consisting of pure-tone audiometry, impedance and clinical testing of the vestibular function, the “bed side examination”. Results. Audiological evaluation showed normal pure-tone audiometry and impedance in 10 patients. Only in one case a sudden right total deafness was highlighted with vestibular areflexia, showing a secondary positional vertigo. Seven patients presented a benign paroxysmal positional vertigo (BPPV) effectively treated by liberatory maneuvers. Three patients had a diagnosis of vestibular neuritis and they were treated with corticosteroids therapy with a complete resolution of dizziness and vertigo in one month. Conclusions. Our results point out the importance of multidisciplinarity between otolaryngologist, neurologist and gynecologist. From a pathogenic point of view, the vascular etiology, strictly related to the gravidic hormonal variations, is often hypothesized.

Published
2020

28. A one-year time frame for voice prosthesis management. What should the physician expect? is it an overrated job?

Author

Parrilla, Claudio, Longobardi, Ylenia, Paludetti, Gaetano, Marenda, Maria Elisabetta, D'Alatri, Lucia, Bussu, Francesco, Scarano, Emanuele, Galli, Jacopo, Parrilla C., Longobardi Y., Paludetti G. (ORCID:0000-0003-2480-1243), Marenda M. E., D'alatri L. (ORCID:0000-0003-3104-958X), Bussu F. (ORCID:0000-0001-6261-2772), Scarano E. (ORCID:0000-0003-2570-1121), Galli J. (ORCID:0000-0001-6353-6249), Parrilla, Claudio, Longobardi, Ylenia, Paludetti, Gaetano, Marenda, Maria Elisabetta, D'Alatri, Lucia, Bussu, Francesco, Scarano, Emanuele, Galli, Jacopo, Parrilla C., Longobardi Y., Paludetti G. (ORCID:0000-0003-2480-1243), Marenda M. E., D'alatri L. (ORCID:0000-0003-3104-958X), Bussu F. (ORCID:0000-0001-6261-2772), Scarano E. (ORCID:0000-0003-2570-1121), and Galli J. (ORCID:0000-0001-6353-6249)

Abstract

Management of late complications represents the main reason for reluctance in using voice prosthesis rehabilitation. The aim of this paper is to report our experience by describing the one-year management of a large cohort of patients in order to clarify how demanding management is in terms of burden on clinicians. Between June 2017 and June 2018, each access made at the Otolaryngology Clinic of our Institute for issues related to prosthesis by 70 laryngectomised patients rehabilitated by voice prosthesis was registered in a specific database. A review of the data provided information on the incidence, management and outcomes of adverse events encountered during the selected time frame. In addition, a T test was used to evaluate the differences between irradiated and non-irradiated patients and between primary and secondary tracheo-oesophageal-puncture. Leakage through the prosthesis was the most common cause for access (51.86%). The median number of accesses per patient per year was 3.47. The speech therapist autonomously managed 18.1% of accesses. The median number of accesses per patient per year needing a physician was 2.84. The median lifetime of the prosthesis was 4.85 months. Radiotherapy or modality (primary or secondary) of the puncture did not influence the number of accesses per year or the prosthesis lifetime. This retrospective analysis of results highlighted the most frequent issues and the most effective measures to deal with them, which allowed us to define a systematic algorithm to standardise and ease long-term outpatient management.

Published
2020

29. Adult phenotype in Koolen-de Vries/KANSL1 haploinsufficiency syndrome

Author

Amenta, S., Frangella, S., Marangi, G., Lattante, S., Ricciardi, S., Doronzio, P. N., Orteschi, D., Veredice, C., Contaldo, I., Zampino, G., Gentile, M., Scarano, E., Graziano, C., Zollino, M., Amenta S., Frangella S., Marangi G. (ORCID:0000-0002-6898-8882), Lattante S. (ORCID:0000-0003-2891-0340), Doronzio P. N., Veredice C., Contaldo I., Zampino G. (ORCID:0000-0003-3865-3253), Gentile M., Zollino M. (ORCID:0000-0003-4871-9519), Amenta, S., Frangella, S., Marangi, G., Lattante, S., Ricciardi, S., Doronzio, P. N., Orteschi, D., Veredice, C., Contaldo, I., Zampino, G., Gentile, M., Scarano, E., Graziano, C., Zollino, M., Amenta S., Frangella S., Marangi G. (ORCID:0000-0002-6898-8882), Lattante S. (ORCID:0000-0003-2891-0340), Doronzio P. N., Veredice C., Contaldo I., Zampino G. (ORCID:0000-0003-3865-3253), Gentile M., and Zollino M. (ORCID:0000-0003-4871-9519)

Abstract

Background: Koolen-de Vries syndrome (KdVS) is a multisystem neurodevelopmental disorder caused by 17q21.31 deletions or mutations in KANSL1. It was mainly described in children. Methods: A retrospective study on 9 subjects aged 19-45 years and revision of 18 literature patients, with the purpose to get insights into the phenotypic evolution with time, and into the clinical manifestations in adulthood. Results: Seven patients had a 17q21.31 deletion and two a point mutation in KANSL1. All had intellectual disability, which was mild in five (56%) and moderate in four (44%). Epilepsy was diagnosed in four subjects (44%), with onset from 1 to 7 years and full remission before 9 years in 3/4 patients. Scoliosis affected seven individuals (77.7%) and it was substantially stable with age in 5/7 patients, allowing for simple daily activities. Two subjects had severely progressive scoliosis, which was surgically corrected. Overweight or true obesity did occur after puberty in six patients (67%). Behaviour abnormalities were recorded in six patients (67%). The facial phenotype slightly evolved with time to include thick eyebrows, elongated nose and pronounced pointed chin. Despite behaviour abnormalities, happy disposition and sociable attitudes were common. Half of patients had fluent language and were good at writing and reading. Rich language, although limited to single words or short sentences, and very limited or absent skills in writing and reading were observed in the remaining patients. Autonomy in daily activities and personal care was usually limited. Conclusions: Distinctive features in adult KdVS subjects include intellectual disability, overweight/obesity, behaviour abnormalities with preserved social interest, ability in language, slight worsening of the facial phenotype and no seizures.

Published
2020

33. Management of vertigo in pregnancy

Author

Salvati, A., primary, Apa, R., additional, Loperfido, A., additional, Scarano, E., additional, Paludetti, G., additional, Tropea, A., additional, and Picciotti, P.M., additional

Published
2020
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36. HDAC8 Loss of Function and SHOX Haploinsufficiency: Two Independent Genetic Defects Responsible for a Complex Phenotype

Author

Severi G., Bonora E., Perri A., Scarano E., Mazzanti L., Isidori F., Zuntini R., Menabo S., Graziano C., Severi G., Bonora E., Perri A., Scarano E., Mazzanti L., Isidori F., Zuntini R., Menabo S., and Graziano C.

Subjects
Haploinsufficiency, Osteochondrodysplasias, Whole Exome Sequencing, Histone Deacetylases, Brachydactyly-mental retardation syndrome, Short Stature Homeobox Protein, Growth Disorder, Histone Deacetylase, De Lange Syndrome, Exome Sequencing, Humans, Osteochondrodysplasia, Child, Frameshift Mutation, Growth Disorders, Comparative Genomic Hybridization, Repressor Protein, Dual molecular diagnosi, Pedigree, Repressor Proteins, Phenotype, HDAC8, Female, Gene Deletion, SHOX, Human
Abstract

We report a patient with developmental delay, brachydactyly type E, short stature, and tetralogy of Fallot. Brachydactyly-mental retardation syndrome (BDMR) was suspected based on the phenotype; however, array CGH excluded a 2q37 deletion, but identified a deletion encompassing the SHOX gene. BDMR is characterized by cognitive impairment, skeletal abnormalities involving hands and feet, short stature, and overweight. Most affected individuals carry relatively large 2q37 deletions encompassing HDAC4. This gene encodes a histone deacetylase involved in epigenetic regulation of cell growth and differentiation, specifically during endochondral bone formation in chondrocyte hypertrophy. Since SHOX haploinsufficiency can cause skeletal defects and short stature but would not fully explain the clinical picture of this patient, exome sequencing was performed, and a heterozygous HDAC8 frameshift mutation was identified. HDAC8 is a distinct histone deacetylase involved in cohesin recycling and is responsible for an X-linked dominant Cornelia de Lange-like phenotype. A new blended clinical phenotype may be explained by the result of a dual molecular diagnosis, which represents a combination of 2 independent genetic defects, with relevant implications for genetic counseling, clinical management, and prognosis.

Published
2018

45. Anthropometric characteristics of newborns with Prader-Willi syndrome

Author

Salvatoni, A, Moretti, A, Grugni, G, Agosti, M, Azzolini, S, Bonaita, V, Cianci, P, Corica, D, Crinò, A, Delvecchio, M, Ferraris, S, Greggio, N, Iughetti, L, Licenziati, M, Madeo, S, Nosetti, L, Pajno, R, Rutigliano, I, Sacco, M, Salvatore, S, Scarano, E, Trifirò, G, Wasniewska, M, Salvatoni, Alessandro, Moretti, Alex, Grugni, Graziano, Agosti, Massimo, Azzolini, Sara, Bonaita, Valentina, Cianci, Paola, Corica, Domenico, Crinò, Antonino, Delvecchio, Maurizio, Ferraris, Silvio, Greggio, Nella A, Iughetti, Lorenzo, Licenziati, Maria R, Madeo, Simona F, Nosetti, Luana, Pajno, Roberta, Rutigliano, Irene, Sacco, Michele, Salvatore, Silvia, Scarano, Emanuela, Trifirò, Giuliana, Wasniewska, Malgorzata, Salvatoni, A, Moretti, A, Grugni, G, Agosti, M, Azzolini, S, Bonaita, V, Cianci, P, Corica, D, Crinò, A, Delvecchio, M, Ferraris, S, Greggio, N, Iughetti, L, Licenziati, M, Madeo, S, Nosetti, L, Pajno, R, Rutigliano, I, Sacco, M, Salvatore, S, Scarano, E, Trifirò, G, Wasniewska, M, Salvatoni, Alessandro, Moretti, Alex, Grugni, Graziano, Agosti, Massimo, Azzolini, Sara, Bonaita, Valentina, Cianci, Paola, Corica, Domenico, Crinò, Antonino, Delvecchio, Maurizio, Ferraris, Silvio, Greggio, Nella A, Iughetti, Lorenzo, Licenziati, Maria R, Madeo, Simona F, Nosetti, Luana, Pajno, Roberta, Rutigliano, Irene, Sacco, Michele, Salvatore, Silvia, Scarano, Emanuela, Trifirò, Giuliana, and Wasniewska, Malgorzata

Abstract

This is a retrospective multicenter nationwide Italian study collecting neonatal anthropometric data of Caucasian subjects with Prader–Willi syndrome (PWS) born from 1988 to 2018. The aim of the study is to provide percentile charts for weight and length of singletons with PWS born between 36 and 42 gestational weeks. We collected the birth weight and birth length of 252 male and 244 female singleton live born infants with both parents of Italian origin and PWS genetically confirmed. Percentile smoothed curves of birth weight and length for gestational age were built through Cole's lambda, mu, sigma method. The data were compared to normal Italian standards. Newborns with PWS showed a lower mean birth weight, by 1/2 kg, and a shorter mean birth length, by 1 cm, than healthy neonates. Females with a 15q11-13 deletion were shorter than those with maternal uniparental maternal disomy of chromosome 15 (p <.0001). The present growth curves may be useful as further traits in supporting a suspicion of PWS in a newborn. Because impaired prenatal growth increases risk of health problems later in life, having neonatal anthropometric standards could be helpful to evaluate possible correlations between the presence or absence of small gestational age and some clinical and metabolic aspects of PWS.

Published
2019

46. Treatment-emergent central sleep apnoea after surgery for obstructive sleep apnoea

Author

Testani, Elisa, De Corso, Eugenio, Losurdo, A., Fiorita, Antonella, Vollono, Catello, Della Marca, Giacomo, Scarano, Emanuele, Testani E., DE CORSO E., Fiorita A., Vollono C., della Marca G. (ORCID:0000-0001-6914-799X), Scarano E. (ORCID:0000-0003-2570-1121), Testani, Elisa, De Corso, Eugenio, Losurdo, A., Fiorita, Antonella, Vollono, Catello, Della Marca, Giacomo, Scarano, Emanuele, Testani E., DE CORSO E., Fiorita A., Vollono C., della Marca G. (ORCID:0000-0001-6914-799X), and Scarano E. (ORCID:0000-0003-2570-1121)

Abstract

Central sleep apnoea (CSA) is a lack of drive to breathe during sleep, which can occur in physiologic as well as in pathologic conditions. A particular type of CSA, defined treatment-emergent CSA (TECSA), may occur after the treatment of obstructive sleep apnoea syndrome (OSAS), either with CPAP or surgery. TECSA is transitory and seems to be related to the severity of OSAS. We describe a 51-year-old man affected by severe OSAS who developed severe, transient CSA immediately after upper airways surgery. We believe that CSA was triggered by the sudden variation in nocturnal arterial PCO 2 , which decreased from 52.3 mmHg before surgery to 42.0 mmHg after surgery. It is conceivable that, due to long-lasting severe OSAS, our patient lowered his chemosensitivity to PCO 2 . Consequently, the resolution of obstructive apnoeas and the restoration of normal nocturnal values of PCO 2 may have reduced the nocturnal PCO 2 to the point of being inadequate to stimulate ventilation.

Published
2019

48. Radiologist-rheumatologist multidisciplinary approach in the management of axial spondyloarthritis: a Delphi consensus statement

Author

Marchesoni, A., D Angelo, S., Anzidei, M., Bortolotti, R., Cantini, F., Caramella, D., Carotti, M., Maria Sole Chimenti, Delle Sedie, A., Egan, C. G., Fabbroni, M., Frediani, B., Fusaro, E., Galeazzi, M., Gallazzi, M. B., Gentileschi, S., Gentili, F., Gerli, R., Gilio, M., Iannone, F., La Paglia, E., Lubrano, E., Macarini, L., Olivieri, I., Pellerito, R., Ramonda, R., Salvarani, C., Scarano, E., Scarpa, R., Spaggiari, L., Spanò, A., Zawaideh, J. P., Mazzei, M. A., Marchesoni, Antonio, D'Angelo, Salvatore, Anzidei, Michele, Bortolotti, Roberto, Cantini, Fabrizio, Caramella, Davide, Carotti, Marina, Chimenti, Maria Sole, Delle Sedie, Andrea, Egan, Colin Gerard, Fabbroni, Marta, Frediani, Bruno, Fusaro, Enrico, Galeazzi, Mauro, Gallazzi, Mauro Battista, Gentileschi, Stefano, Gentili, Francesco, Gerli, Roberto, Gilio, Michele, Iannone, Florenzo, La Paglia, Ernesto, Lubrano, Ennio, Macarini, Luca, Olivieri, Ignazio, Pellerito, Raffaele, Ramonda, Roberta, Salvarani, Carlo, Scarano, Enrico, Scarpa, Raffaele, Spaggiari, Lucia, Spanò, Angelo, Zawaideh, Jeries Paolo, and Mazzei, Maria Antonietta

Subjects
Consensus, Delphi Technique, Consensu, Humans, Interdisciplinary Communication, Italy, Radiologists, Rheumatologists, Spondylarthritis, Settore MED/16 - Reumatologia, Radiologist, Rheumatologist, Human
Abstract

The aim of this study was to develop a Delphi consensus statement between rheumatologists and radiologists for the diagnosis and monitoring of axial spondyloarthritis (axial-SpA).Following an extensive literature search to identify unmet needs and potential goals for a multidisciplinary approach, a scientific board comprising 28 Italian hospital-based rheumatologists (n=19) and radiologists (n=9) identified 8 "starting points", resulting in the development of 23 consensus statements covering issues from current practice guidelines to specific MRI protocols for the assessment of axial-SpA. Each participant anonymously expressed a level of agreement for each statement using a 5-point Likert scale (1="strongly disagree"; 5="strongly agree") via an online Delphi method.Total cumulative agreement (TCA) was defined as the sum of the percentage of response to items 4 ("agree") and 5 ("absolutely agree"). Consensus was defined as ≥80% total cumulative agreement for each statement.After the first round of voting (28 participants), positive consensus was reached for 28/31 (90.3%) statements. Statements without consensus (n=3) were discussed in a face-to-face plenary session prior to the second vote (28 participants). After the second round voting, positive consensus was attained for all 31 statements, with mean final TCA of 95.5% (range 82.1-100%).This Delphi consensus statement provides an aid to rheumatologists and radiologists for the diagnosis and monitoring of axial-SpA.

Published
2018

50. R-CHOP PRECEDED BY ENGINEERED TUMOR NECROSIS FACTOR (TNF) IN RELAPSED OR REFRACTORY PRIMARY DIFFUSE LARGE B-CELL LYMPHOMA OF THE CNS (rPCNSL): FINAL RESULTS OF THE INGRID TRIAL

Author

Ferreri, A. J., primary, Calimeri, T., additional, Conte, G., additional, Ponzoni, M., additional, Fallanca, F., additional, Cattaneo, D., additional, Scarano, E., additional, Flavio, C., additional, Sassone, M., additional, Foppoli, M., additional, Perrone, S., additional, Cecchetti, C., additional, Lopedote, P., additional, Gritti, G., additional, Castellino, C., additional, Verga, L., additional, Olcese, F., additional, Mazza, R., additional, Ciceri, F., additional, Bordignon, C., additional, Anzalone, N., additional, and Corti, A., additional

Published
2019
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