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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 phenotype.Since 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 presentation.The aim of this review is to provide a comprehensive analysis of the clinical spectrum of AGS and report currently available treatments and new immunosuppressive strategies.Data sources Literature reviews and original research articles were collected from databases,including PubMed and Clini-calTrials.gov.Relevant articles about AGS were included.Results The involvement of the nervous system certainly represents the major cause of mortality and morbidity in AGS patients.However,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 patients.Therapeutic approaches of AGS are limited to interventions aimed at specific symptoms and the management of multiple comorbidities.However,advances in understanding the pathogenesis of AGS could open new and more effective therapies.Conclusions 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 system.To date,there is no specific and effective treatment for AGS.New 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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Ripple Tectonics—When Subduction Is Interrupted

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Positioning 2020年第3期11卷 33-44页

作者： Zvi Ben-Avraham Gerald Schubert Emanuele lodolo Uri Schattner Department of Geophysics Porter School of the Environment and Earth Sciences Tel Aviv University Tel Aviv Israel Leon H. Charney School of Marine Sciences University of Haifa Haifa Israel Department of Earth Planetary and Space Sciences University of California Los Angeles USA National Institute of Oceanography and Experimental Geophysics Sgonico Italy Dr. Moses Strauss Department of Marine Geosciences Leon H. Charney School of Marine Sciences University of Haifa Haifa Israel

Subduction plays a fundamental role in plate tectonics and is a significant factor in modifying the structure and topography of the Earth. It is driven by convection forces that change over a >100 Myr time scale. However, when an oceanic plateau approaches, it plugs the subduction, and causes slab necking and tearing. This abrupt change may trigger a series of geodynamic (tectonic, volcanic) and sedimentary responses recorded across the convergence boundary and its surrounding regions by synchronous structural modifications. We suggest that a large enough triggering event may lead to a ripple tectonic effect that propagates outwards while speeding up the yielding of localized stress states that otherwise would not reach their threshold. The ripple effect facilitates tectonic, volcanic, and structural events worldwide that are seemingly unrelated. When the world’s largest oceanic plateau, Ontong Java Plateau (OJP), choked the Pacific-Australian convergence zone at ~6 Myr ago, it induced kinematic modifications throughout the Pacific region and along its plate margins. Other, seemingly unrelated, short-lived modifications were recorded worldwide during that time window. These modifications changed the rotation of the entire Pacific plate, which occupies ~20% of the Earth’s surface. In addition, the Scotia Sea spreading stopped, global volcanism increased, the Strait of Gibraltar closed, and the Mediterranean Sea dried up and induced the Messinian salinity crisis. In this paper, we attribute these and many other synchronous events to a new “ripple tectonics” mechanism. We suggest that the OJPincipient collision triggered the Miocene-Pliocene transition. Similarly, we suggest that innovative GPS-based studies conducted today may seek the connectivity between tectonic, seismic, and volcanic events worldwide.

关键词： Plate Tectonics Subduction-Collision Transition Miocene-Pliocene Transition

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