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Long-term clinical outcomes of elexacaftor/tezacaftor/ivacaftor therapy in adults with cystic fibrosis and advanced pulmonary disease.
Savi D, Lucca F, Tridello G, Meneghelli I, Comello I, Tomezzoli S, Signorini M, Proietti E, Cucchetto G, Volpi S, Cipolli M. Savi D, et al. Among authors: cipolli m. Respir Med. 2023 Nov-Dec;219:107406. doi: 10.1016/j.rmed.2023.107406. Epub 2023 Sep 9. Respir Med. 2023. PMID: 37690570
BACKGROUND: The combination of cystic fibrosis transmembrane conductance regulator (CFTR) modulators elexacaftor, tezacaftor and ivacaftor (ELX/TEZ/IVA) has been approved for treatment of cystic fibrosis (CF) patients (pwCF) homozygous and heterozygous for Phe508del. We aim to as …
BACKGROUND: The combination of cystic fibrosis transmembrane conductance regulator (CFTR) modulators elexacaftor, tezacaftor and ivacaftor ( …
[Italian Cystic Fibrosis Registry (ICFR). Report 2019-2020].
Campagna G, Amato A, Majo F, Ferrari G, Quattrucci S, Padoan R, Floridia G, Salvatore D, Carnovale V, Puppo Fornaro G, Taruscio D, Salvatore M; Gruppo di lavoro RIFC. Campagna G, et al. Epidemiol Prev. 2022 Jul-Aug;46(4 Suppl 2):1-38. doi: 10.19191/EP22.4S2.060. Epidemiol Prev. 2022. PMID: 36102313 Free article. Italian.
INTRODUCTION: Italian cystic fibrosis registry (ICFR) collects data from cystic fibrosis (CF) patients through the collaboration with Italian CF referral and support Centres (Italian law 548/93). ICFR contributes: to the analysis of medium and long term clinical and epidem …
INTRODUCTION: Italian cystic fibrosis registry (ICFR) collects data from cystic fibrosis (CF) patients through the collaboration with Italia …
Long-term amikacin liposome inhalation suspension in cystic fibrosis patients with chronic P. aeruginosa infection.
Bilton D, Fajac I, Pressler T, Clancy JP, Sands D, Minic P, Cipolli M, Galeva I, Solé A, Quittner AL, Jumadilova Z, Ciesielska M, Konstan MW; CLEAR-110 Study Group. Bilton D, et al. Among authors: cipolli m. J Cyst Fibros. 2021 Nov;20(6):1010-1017. doi: 10.1016/j.jcf.2021.05.013. Epub 2021 Jun 16. J Cyst Fibros. 2021. PMID: 34144923 Free PMC article. Clinical Trial.
The CLEAR-110 extension study (ClinicalTrials.gov: NCT01316276; EudraCT: 2011-000443-24) assessed long-term safety, tolerability, and efficacy of ALIS in eligible patients who completed CLEAR-108. ...Mean change from baseline in sputum density of P. aeruginosa at day 672 w …
The CLEAR-110 extension study (ClinicalTrials.gov: NCT01316276; EudraCT: 2011-000443-24) assessed long-term safety, tolerability, and …
[Italian Cystic Fibrosis Registry (ICFR). Report 2017-2018].
Campagna G, Amato A, Majo F, Ferrari G, Quattrucci S, Padoan R, Floridia G, Salvatore D, Carnovale V, Puppo Fornaro G, Taruscio D, Salvatore M; Gruppo di lavoro RIFC. Campagna G, et al. Epidemiol Prev. 2021 May-Jun;45(3 Suppl 1):1-37. doi: 10.19191/EP21.3.S1.050. Epidemiol Prev. 2021. PMID: 34132083 Free article. Italian.
In particular, this Report should contribute to the following objectives: to analyse the medium- and long-term clinical and epidemiological trends of the disease; to identify the main healthcare needs at regional and national level, in order to contribute to the healthcare …
In particular, this Report should contribute to the following objectives: to analyse the medium- and long-term clinical and epidemiol …
SARS-CoV-2 infection in cystic fibrosis: A multicentre prospective study with a control group, Italy, February-July 2020.
Colombo C, Alicandro G, Daccó V, Gagliano V, Morlacchi LC, Casciaro R, Pisi G, Francalanci M, Badolato R, Bignamini E, Messore B, Lucanto MC, Leonetti G, Maschio M, Cipolli M; Italian Cystic Fibrosis Society (SIFC) Study Group for COVID-19. Colombo C, et al. Among authors: cipolli m. PLoS One. 2021 May 13;16(5):e0251527. doi: 10.1371/journal.pone.0251527. eCollection 2021. PLoS One. 2021. PMID: 33984027 Free PMC article.
Two adults with a history of lung transplant required non-invasive ventilation, none required ICU admission and all patients fully recovered without short-term sequelae. CONCLUSIONS: The course of SARS-CoV-2 in our patients was relatively favorable. ...
Two adults with a history of lung transplant required non-invasive ventilation, none required ICU admission and all patients fully recovered …
Cystic Fibrosis: Recent Insights into Inhaled Antibiotic Treatment and Future Perspectives.
Taccetti G, Francalanci M, Pizzamiglio G, Messore B, Carnovale V, Cimino G, Cipolli M. Taccetti G, et al. Among authors: cipolli m. Antibiotics (Basel). 2021 Mar 22;10(3):338. doi: 10.3390/antibiotics10030338. Antibiotics (Basel). 2021. PMID: 33810116 Free PMC article. Review.
In this review, the efficacy and safety of the currently available inhaled antibiotics for lung infection treatment in CF patients are discussed, with a particular focus on strategies for eradicating P. aeruginosa and other pathogens. Moreover, the effects of long-term inh …
In this review, the efficacy and safety of the currently available inhaled antibiotics for lung infection treatment in CF patients are discu …
A Prospective Study of Hematologic Complications and Long-Term Survival of Italian Patients Affected by Shwachman-Diamond Syndrome.
Cesaro S, Pegoraro A, Sainati L, Lucidi V, Montemitro E, Corti P, Ramenghi U, Nasi C, Menna G, Zecca M, Danesino C, Nicolis E, Pasquali F, Perobelli S, Tridello G, Farruggia P, Cipolli M. Cesaro S, et al. Among authors: cipolli m. J Pediatr. 2020 Apr;219:196-201.e1. doi: 10.1016/j.jpeds.2019.12.041. Epub 2020 Feb 6. J Pediatr. 2020. PMID: 32037152
OBJECTIVE: To describe the hematologic outcome and long-term survival of patients enrolled in the Shwachman-Diamond syndrome Italian Registry. ...Further studies are needed to optimize type and modality of hematopoietic stem cell transplantation and to assess the long-t …
OBJECTIVE: To describe the hematologic outcome and long-term survival of patients enrolled in the Shwachman-Diamond syndrome Italian …
[Italian Cystic Fibrosis Registry (ICFR). Report 2015-2016].
Giordani B, Amato A, Majo F, Ferrari G, Quattrucci S, Minicucci L, Padoan R, Floridia G, Salvatore D, Carnovale V, Puppo Fornaro G, Taruscio D, Salvatore M; Gruppo di lavoro RIFC. Giordani B, et al. Epidemiol Prev. 2019 Jul-Aug;43(4S1):1-36. doi: 10.19191/EP19.4.S1.067. Epidemiol Prev. 2019. PMID: 31370382 Free article. Italian.
In particular, the Report should contribute to the following objectives: * to analize medium- and long-term clinical and epidemiological trends of the disesase; * to identify the main health care needs at regional and national level in order to contribute to the healthcare …
In particular, the Report should contribute to the following objectives: * to analize medium- and long-term clinical and epidemiologi …
Mesenchymal stem cells from Shwachman-Diamond syndrome patients display normal functions and do not contribute to hematological defects.
André V, Longoni D, Bresolin S, Cappuzzello C, Dander E, Galbiati M, Bugarin C, Di Meglio A, Nicolis E, Maserati E, Serafini M, Warren AJ, Te Kronnie G, Cazzaniga G, Sainati L, Cipolli M, Biondi A, D'Amico G. André V, et al. Among authors: cipolli m. Blood Cancer J. 2012 Oct 12;2(10):e94. doi: 10.1038/bcj.2012.40. Blood Cancer J. 2012. PMID: 23064742 Free PMC article.
We demonstrated in an in vitro coculture system that SDS-MSCs, significantly inhibited neutrophil apoptosis probably through interleukin-6 production. In a long-term coculture with CD34(+)-sorted cells, SDS-MSCs were able to sustain CD34(+) cells survival and to preserve t …
We demonstrated in an in vitro coculture system that SDS-MSCs, significantly inhibited neutrophil apoptosis probably through interleukin-6 p …
Liprotamase long-term safety and support of nutritional status in pancreatic-insufficient cystic fibrosis.
Borowitz D, Stevens C, Brettman LR, Campion M, Wilschanski M, Thompson H; Liprotamase 767 Study Group. Borowitz D, et al. J Pediatr Gastroenterol Nutr. 2012 Feb;54(2):248-57. doi: 10.1097/MPG.0b013e31823315d1. J Pediatr Gastroenterol Nutr. 2012. PMID: 22266487 Clinical Trial.
Liprotamase, a nonporcine, highly purified biotechnology-derived PERT, has demonstrated significant efficacy in fat and protein malabsorption in patients with EPI compared to placebo. This study of liprotamase is the first ever long-term trial of a PERT to evaluate safety …
Liprotamase, a nonporcine, highly purified biotechnology-derived PERT, has demonstrated significant efficacy in fat and protein malabsorptio …
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