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Clinical spectrum and currently available treatment of typeⅠinterferonopathy Aicardi-Goutières syndrome

World Journal of Pediatrics 2023年第7期19卷 635-643页

作者： Giovanni Battista Dell'Isola gianluca dini Kaleb Logan Culpepper Katherin Elizabeth Portwood Pietro Ferrara Giuseppe Di Cara Alberto Verrotti Mauro Lodolo Department of Pediatrics University of PerugiaGiorgio Menghini Square06129 PerugiaItaly Department of Neurology Jacksonville University of FloridaFloridaUSA Department of Pediatrics Division of Child NeurologyUniversity of FloridaUF Health Shands Children's HospitalGainesvilleFLUSA Unit of Pediatrics Campus Bio-Medico UniversityRomeItaly

Background Aicardi-Goutières syndrome(AGS)is a genetically determined disorder with a variable *** the original description of AGS,advances in gene sequencing techniques have resulted in a significant broadening of the phenotypic spectrum associated with AGS genes,and new clinical pictures have emerged beyond the classic *** aim of this review is to provide a comprehensive analysis of the clinical spectrum of AGS and report currently available treatments and new immunosuppressive *** sources Literature reviews and original research articles were collected from databases,including PubMed and *** articles about AGS were *** The involvement of the nervous system certainly represents the major cause of mortality and morbidity in AGS ***,other clinical manifestations,such as chilblains,hepatosplenomegaly,and hematological disturbances,may lead to the diagnosis and considerably impact the prognosis and overall quality of life of these *** approaches of AGS are limited to interventions aimed at specific symptoms and the management of multiple ***,advances in understanding the pathogenesis of AGS could open new and more effective *** The over-activation of innate immunity due to upregulated interferon production plays a critical role in AGS,leading to multi-organ damage with the main involvement of the central nervous *** date,there is no specific and effective treatment for *** drugs specifically targeting the interferon pathway may bring new hope to AGS patients.

关键词： Aicardi-Goutieres syndrome Immunosuppressive drugs Interferon-α Neuroinflammation Systemic lupus erythematosus

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ARBITRARILY HIGH-ORDER ENERGY-CONSERVING METHODS FOR HAMILTONIAN PROBLEMS WITH QUADRATIC HOLONOMIC CONSTRAINTS

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Journal of Computational Mathematics 2024年第4期42卷 1145-1171页

作者： Pierluigi Amodio Luigi Brugnano gianluca Frasca-Caccia Felice Iavernaro Dipartimento di Matematica Università di BariItaly Dipartimento di Matematica e Informatica"U.dini" Università di FirenzeItaly Dipartimento di Matematica Università di SalernoItaly

In this paper,we define arbitrarily high-order energy-conserving methods for Hamilto-nian systems with quadratic holonomic *** derivation of the methods is made within the so-called line integral *** tests to illustra... 详细信息

关键词： Constrained Hamiltonian systems Quadratic holonomic constraints Energy-conserving methods Line integral methods Hamiltonian Boundary Value Methods HB-VMs

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