Fabrizio De Benedetti

Summary

Country: Italy

Publications

  1. ncbi request reprint Mechanistic associations of a mild phenotype of immunodysregulation, polyendocrinopathy, enteropathy, x-linked syndrome
    Fabrizio De Benedetti
    Direzione Scientifica, IRCCS Ospedale Pediatrico Bambino Gesù, Roma, Italy
    Clin Gastroenterol Hepatol 4:653-9. 2006
  2. doi request reprint Randomized trial of tocilizumab in systemic juvenile idiopathic arthritis
    Fabrizio De Benedetti
    Division of Rheumatology, Department of Medicine, Istituto Di Ricovero e Cura a Carattere Scientifico IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy
    N Engl J Med 367:2385-95. 2012
  3. pmc Increased muscle expression of interleukin-17 in Duchenne muscular dystrophy
    L De Pasquale
    Division of Rheumatology, Bambino Gesu Children s Hospital, Rome, Italy
    Neurology 78:1309-14. 2012
  4. doi request reprint Targeting interleukin-6 in pediatric rheumatic diseases
    Fabrizio De Benedetti
    IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy
    Curr Opin Rheumatol 21:533-7. 2009
  5. doi request reprint Association between mannose-binding lectin gene polymorphisms and necrotizing enterocolitis in preterm infants
    Giusi Prencipe
    Laboratory of Rheumatology, Bambino Gesu Children s Hospital, Rome, Italy
    J Pediatr Gastroenterol Nutr 55:160-5. 2012
  6. doi request reprint Safety and efficacy of etanercept in a cohort of patients with juvenile idiopathic arthritis under 4 years of age
    Claudia Bracaglia
    Division of Rheumatology, Department of Pediatric Medicine, Ospedale Pediatrico Bambino GesùIRCCS, Rome, Italy
    J Rheumatol 39:1287-90. 2012
  7. ncbi request reprint A novel mutation in the CIAS1/NLRP3 gene associated with an unexpected phenotype of cryopyrin-associated periodic syndromes
    Antonella Insalaco
    Division of Rheumatology, Department of Pediatric Medicine, Bambino Gesu Children s Hospital, IRCCS, Rome, Italy
    Clin Exp Rheumatol 32:123-5. 2014
  8. doi request reprint Association of the macrophage migration inhibitory factor -173*C allele with childhood nephrotic syndrome
    Marina Vivarelli
    Unita Operativa di Nefrologia e Dialisi, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy
    Pediatr Nephrol 23:743-8. 2008
  9. pmc Deregulation of the IL-1β axis in chronic recurrent multifocal osteomyelitis
    Roberta Scianaro
    Rheumatology Unit, Bambino Gesu Children s Hospital, Rome, Italy
    Pediatr Rheumatol Online J 12:30. 2014
  10. ncbi request reprint Impaired skeletal development in interleukin-6-transgenic mice: a model for the impact of chronic inflammation on the growing skeletal system
    Fabrizio De Benedetti
    Ospedale Pediatrico Bambino Gesu, Rome, Italy
    Arthritis Rheum 54:3551-63. 2006

Collaborators

Detail Information

Publications23

  1. ncbi request reprint Mechanistic associations of a mild phenotype of immunodysregulation, polyendocrinopathy, enteropathy, x-linked syndrome
    Fabrizio De Benedetti
    Direzione Scientifica, IRCCS Ospedale Pediatrico Bambino Gesù, Roma, Italy
    Clin Gastroenterol Hepatol 4:653-9. 2006
    ..We describe 2 unrelated patients with IPEX syndrome with a mild clinical phenotype and with novel FOXP3 mutations and the phenotypic and functional characterization of their Treg cells...
  2. doi request reprint Randomized trial of tocilizumab in systemic juvenile idiopathic arthritis
    Fabrizio De Benedetti
    Division of Rheumatology, Department of Medicine, Istituto Di Ricovero e Cura a Carattere Scientifico IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy
    N Engl J Med 367:2385-95. 2012
    ..Systemic juvenile idiopathic arthritis (JIA) is the most severe subtype of JIA; treatment options are limited. Interleukin-6 plays a pathogenic role in systemic JIA...
  3. pmc Increased muscle expression of interleukin-17 in Duchenne muscular dystrophy
    L De Pasquale
    Division of Rheumatology, Bambino Gesu Children s Hospital, Rome, Italy
    Neurology 78:1309-14. 2012
    ..We analyzed muscle biopsy samples of DMD-affected children to characterize interleukin (IL)-17 and Forkhead box P3 (Foxp3) expression levels and to identify possible correlations with clinical status...
  4. doi request reprint Targeting interleukin-6 in pediatric rheumatic diseases
    Fabrizio De Benedetti
    IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy
    Curr Opin Rheumatol 21:533-7. 2009
    ....
  5. doi request reprint Association between mannose-binding lectin gene polymorphisms and necrotizing enterocolitis in preterm infants
    Giusi Prencipe
    Laboratory of Rheumatology, Bambino Gesu Children s Hospital, Rome, Italy
    J Pediatr Gastroenterol Nutr 55:160-5. 2012
    ....
  6. doi request reprint Safety and efficacy of etanercept in a cohort of patients with juvenile idiopathic arthritis under 4 years of age
    Claudia Bracaglia
    Division of Rheumatology, Department of Pediatric Medicine, Ospedale Pediatrico Bambino GesùIRCCS, Rome, Italy
    J Rheumatol 39:1287-90. 2012
    ..To evaluate safety, tolerability, and efficacy of etanercept in a cohort of patients with juvenile idiopathic arthritis (JIA) under 4 years of age...
  7. ncbi request reprint A novel mutation in the CIAS1/NLRP3 gene associated with an unexpected phenotype of cryopyrin-associated periodic syndromes
    Antonella Insalaco
    Division of Rheumatology, Department of Pediatric Medicine, Bambino Gesu Children s Hospital, IRCCS, Rome, Italy
    Clin Exp Rheumatol 32:123-5. 2014
    ..Cryopyrin-associated periodic syndromes (CAPS) comprise a spectrum of distinct, rare, autosomal dominant autoinflammatory disorders of increasing severity caused by NLRP3 gene mutations...
  8. doi request reprint Association of the macrophage migration inhibitory factor -173*C allele with childhood nephrotic syndrome
    Marina Vivarelli
    Unita Operativa di Nefrologia e Dialisi, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy
    Pediatr Nephrol 23:743-8. 2008
    ....
  9. pmc Deregulation of the IL-1β axis in chronic recurrent multifocal osteomyelitis
    Roberta Scianaro
    Rheumatology Unit, Bambino Gesu Children s Hospital, Rome, Italy
    Pediatr Rheumatol Online J 12:30. 2014
    ..This study aims to investigate the inflammasome response in peripheral blood mononuclear cells (PBMCs) and the expression of inflammasome components in bone biopsies from patients with chronic recurrent multifocal osteomyelitis (CRMO)...
  10. ncbi request reprint Impaired skeletal development in interleukin-6-transgenic mice: a model for the impact of chronic inflammation on the growing skeletal system
    Fabrizio De Benedetti
    Ospedale Pediatrico Bambino Gesu, Rome, Italy
    Arthritis Rheum 54:3551-63. 2006
    ....
  11. doi request reprint Catch-Up Growth During Tocilizumab Therapy for Systemic Juvenile Idiopathic Arthritis: Results From a Phase III Trial
    Fabrizio De Benedetti
    IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy
    Arthritis Rheumatol 67:840-8. 2015
    ..To investigate the impact of tocilizumab treatment on growth and growth-related laboratory parameters in patients with systemic juvenile idiopathic arthritis (JIA) enrolled in a phase III clinical trial...
  12. doi request reprint Relapsing polychondritis: new therapeutic strategies with biological agents
    Paola Sabrina Buonuomo
    Department of Pediatrics, Pediatric Rheumatology, Ospedale Pediatrico Bambino Gesù IRCCS, Piazza S Onofrio, 4, 00135, Rome, Italy
    Rheumatol Int 30:691-3. 2010
    ..We describe a young girl with RP unresponsive to conventional therapy...
  13. pmc IL-6 amplifies TLR mediated cytokine and chemokine production: implications for the pathogenesis of rheumatic inflammatory diseases
    Ivan Caiello
    Division of Rheumatology, Bambino Gesu Children s Hospital, Rome, Italy
    PLoS ONE 9:e107886. 2014
    ..Our results suggest that IL-6 amplifies TLR-induced inflammatory response. This effect may be relevant in the presence of high IL-6 and sIL-6R levels, such as in arthritic joints in the context of stimulation by endogenous TLR ligands. ..
  14. doi request reprint Reaching the threshold: a multilayer pathogenesis of macrophage activation syndrome
    Raffaele Strippoli
    Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy
    J Rheumatol 40:761-7. 2013
    ..Once the "threshold" has been reached, inflammatory cytokines are the final effectors, independent of the interplay between different upstream pathogenic factors...
  15. doi request reprint Role of mannose-binding lectin in nosocomial sepsis in critically ill neonates
    Cinzia Auriti
    Department of Medical and Surgical Neonatology, Bambino Gesu Children s Hospital, Rome, Italy
    Hum Immunol 71:1084-8. 2010
    ..Moreover, no association was found between MBL2 genotypes and death...
  16. doi request reprint A6: Tapering and Withdrawal of Tocilizumab in Patients With Systemic Juvenile Idiopathic Arthritis in Inactive Disease: Results From an Alternative Dosing Regimen in the TENDER Study
    Fabrizio De Benedetti
    IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy
    Arthritis Rheumatol 66:S8-9. 2014
    ..Herein we describe the patients registered to participate in the optional alternative dosing schedule in the TENDER study...
  17. doi request reprint Amplification of the response to Toll-like receptor ligands by prolonged exposure to interleukin-6 in mice: implication for the pathogenesis of macrophage activation syndrome
    Raffaele Strippoli
    Ospedale Pediatrico Bambino Gesu, Rome, Italy
    Arthritis Rheum 64:1680-8. 2012
    ..To investigate whether prolonged exposure to interleukin-6 (IL-6) affects the inflammatory response induced by Toll-like receptor (TLR) ligands...
  18. ncbi request reprint Functional and prognostic relevance of the -173 polymorphism of the macrophage migration inhibitory factor gene in systemic-onset juvenile idiopathic arthritis
    Fabrizio De Benedetti
    IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy
    Arthritis Rheum 48:1398-407. 2003
    ....
  19. ncbi request reprint Abnormal production of tumor necrosis factor (TNF) -- alpha and clinical efficacy of the TNF inhibitor etanercept in a patient with PAPA syndrome [corrected]
    Elisabetta Cortis
    Department of Medicine, Division of Rheumathology and Laboratory of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy
    J Pediatr 145:851-5. 2004
    ..Production of tumor necrosis factor-alpha by mononuclear cells of the proband and of the affected relatives was abnormally elevated...
  20. pmc Interleukin-1β and interleukin-6 in arthritis animal models: roles in the early phase of transition from acute to chronic inflammation and relevance for human rheumatoid arthritis
    Gianfranco Ferraccioli
    Division of Rheumatology, School of Medicine, Catholic University of the Sacred Heart, Rome, Italy
    Mol Med 16:552-7. 2010
    ..In the other models, IL-1β and IL-6, more than TNF-α, appear to be relevant in driving the transition, which suggests that these should be the targets of an early intervention to stop the course toward the chronic form of the disease...
  21. doi request reprint Kikuchi-Fujimoto disease in patient with systemic phacomatosis pigmentovascularis
    Hyppolite K Tchidjou
    aUniversity Department of Pediatrics bRare Diseases and Medical Genetics Unit, Department of Pediatric Medicine cDivision of Rheumatology, Department of Pediatric Medicine dUnit of Pathology, Bambino Gesu Children s Hospital, Rome, Italy
    Blood Coagul Fibrinolysis 25:783-5. 2014
    ....
  22. doi request reprint A14: neutropenia with tocilizumab treatment is not associated with increased infection risk in patients with systemic juvenile idiopathic arthritis
    Fabrizio De Benedetti
    IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy
    Arthritis Rheumatol 66:S23-4. 2014
    ....
  23. ncbi request reprint Association of serum interleukin-8 levels with the degree of fibrosis in infants with chronic liver disease
    Valerio Nobili
    Department of Liver Disease, Laboratory of Rheumatology, Research Institute, Bambino Gesu Children s Hospital, Rome, Italy
    J Pediatr Gastroenterol Nutr 39:540-4. 2004
    ..To determine the proinflammatory cytokine profile in children with biliary atresia, we measured circulating levels of interleukin (IL)-1beta, IL-6, tumor necrosis factor-alpha and IL-8...