Person: Sánchez Ramón, Silvia María
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First Name
Silvia María
Last Name
Sánchez Ramón
Affiliation
Universidad Complutense de Madrid
Faculty / Institute
Medicina
Department
Inmunología, Oftalmología y ORL
Area
Inmunología
Identifiers
6 results
Search Results
Now showing 1 - 6 of 6
- PublicationdIvergEnt: How IgE Axis Contributes to the Continuum of Allergic Asthma and Anti-IgE Therapies(MDPI, 2017-06-21) Palomares Gracia, Óscar; Sánchez Ramón, Silvia María; Dávila, Ignacio; Prieto, Luis; Pérez de Llano, Luis; Lleonart, Marta; Domingo, Christian; Nieto, AntonioAsthma is an airway disease characterised by chronic inflammation with intermittent or permanent symptoms including wheezing, shortness of breath, chest tightness, and cough, which vary in terms of their occurrence, frequency, and intensity. The most common associated feature in the airways of patients with asthma is airway inflammation. In recent decades, efforts have been made to characterise the heterogeneous clinical nature of asthma. The interest in improving the definitions of asthma phenotypes and endotypes is growing, although these classifications do not always correlate with prognosis nor are always appropriate therapeutic approaches. Attempts have been made to identify the most relevant molecular and cellular biomarkers underlying the immunopathophysiological mechanisms of the disease. For almost 50 years, immunoglobulin E (IgE) has been identified as a central factor in allergic asthma, due to its allergen-specific nature. Many of the mechanisms of the inflammatory cascade underlying allergic asthma have already been elucidated, and IgE has been shown to play a fundamental role in the triggering, development, and chronicity of the inflammatory responses within the disease. Blocking IgE with monoclonal antibodies such as omalizumab have demonstrated their efficacy, effectiveness, and safety in treating allergic asthma. A better understanding of the multiple contributions of IgE to the inflammatory continuum of asthma could contribute to the development of novel therapeutic strategies for the disease.
- PublicationExecutive Summary of the Consensus Document on the Diagnosis and Management of Patients with Primary Immunodeficiencies(Elsevier, 2020-12) Cordero, Elisa; Goycochea Valdivia, Walter; Mendez Echevarría, Ana; M. Allende, Luis; Alsina, Laia; Bravo García Morato, María; Gil Herrera, Juana; Gudiol, Carlota; Len Abad, Óscar; López Medrano, Francisco; Moreno Pérez, David; Muñoz, Patricia; Olbrich, Peter; Sánchez Ramón, Silvia María; Soler Paracín, Pere; Aguilera Cros, Clara; Arostegui, Juan Ignacio; Badell Serra, Isabel; Carbone, Javier; Fortún, Jesús; González Granado, Luis Ignacio; López Granados, Eduardo; Lucena, José Manuel; Parody, Rocío; Ramakers, Jan; Regueiro González-Barros, José Ramón; Rivière, Jacque G; Roca Oporto, Cristina; Rodríguez Pena, Rebeca; Santos Pérez, Juan Luis; Rodríguez Gallego, Carlos; Neth, OlafPrimary immunodeficiencies (PIDs) are rare, undiagnosed and potentially fatal diseases. Clinical manifestations of PID can be fatal or leave sequelae that worsen the quality of life of patients. Traditionally, the treatment of PIDs has been largely supportive, with the exception of bone marrow transplantation and, more recently, gene therapy. The discovering of new affected pathways, the development of new molecules and biologics, and the increasing understanding of the molecular basis of these disorders have created opportunities in PIDs therapy. This document aims to review current knowledge and to provide recommendations about the diagnosis and clinical management of adults and childre with PIDs based on the available scientific evidence taking in to account current practice and future challenges. A systematic review was conducted, and evidence levels based on the available literature are given for each recommendation where available. � 2020 American Academy of Allergy, Asthma & Immunology; Sociedad Española de Enfermedades Infecciosas y Microbiología Clínica [Published by Elsevier Inc./Elsevier Espana]. All rights reserved.
- PublicationCombined Immune Defect in B-Cell Lymphoproliferative Disorders Is Associated with Severe Infection and Cancer Progression(MDPI, 2022-08-19) Ochoa Grullón, Juliana Lucía; Guevara Hoyer, Kissy; Pérez López, Cristina; Pérez de Diego, Rebeca; Peña Cortijo, Ascensión; Polo, Marta; Mateo Morales, Marta; Anguita Mandley, Eduardo; Jiménez García, Carlos; Bolaños, Estefanía; Íñigo, Belén; Medina, Fiorella; Rodríguez de la Peña, Antonia; Izquierdo Delgado, Carmen; Fuente Muñoz, Eduardo de la; Mayol, Elsa; Fernandez Arquero, Miguel; González Fernández, Ataúlfo; Benavente Cuesta, Celina; Sánchez Ramón, Silvia MaríaB cell chronic lymphoproliferative diseases (B-CLPD) are associated with secondary antibody deficiency and other innate and adaptive immune defects, whose impact on infectious risk has not been systematically addressed. We performed an immunological analysis of a cohort of 83 B-CLPD patients with recurrent and/or severe infections to ascertain the clinical relevance of the immune deficiency expression. B-cell defects were present in all patients. Patients with combined immune defect had a 3.69-fold higher risk for severe infection (p = 0.001) than those with predominantly antibody defect. Interestingly, by Kaplan–Meier analysis, combined immune defect showed an earlier progression of cancer with a hazard ratio of 3.21, than predominantly antibody defect (p = 0.005). When B-CLPD were classified in low-degree, high-degree, and plasma cell dyscrasias, risk of severe disease and cancer progression significantly diverged in combined immune defect, compared with predominantly antibody defect (p = 0.001). Remarkably, an underlying primary immunodeficiency (PID) was suspected in 12 patients (14%), due to prior history of infections, autoimmune and granulomatous conditions, atypical or variegated course and compatible biological data. This first proposed SID classification might have relevant clinical implications, in terms of predicting severe infections and cancer progression, and might be applied to different B-CLPD entities.
- PublicationInherited BCL10 deficiency impairs hematopoietic and nonhematopoietic immunity(American Society for Clinical Investigation, 2014-11) Torres, Juan Manuel; Martínez Barricarte, Rubén; García Gómez, Sonia; Mazariegos, Marina S; Itan, Yuval; Boisson, Bertrand; Álvarez Carbajal, Rita Lucía; Jiménez Reinoso, Anaïs; del Pino, Lucía; Rodríguez Pena, Rebeca; Ferreira Martín, Antonio; Hernández Jiménez, Enrique; Toledano, Víctor; Cubillos Zapata, Carolina; Díaz Almirón, Mariana; López Collazo, Eduardo; Unzueta Roch, José L; Sánchez Ramón, Silvia María; Regueiro González-Barros, José Ramón; López Granados, Eduardo; Casanova, Jean-Laurent; Pérez de Diego, RebecaHeterotrimers composed of B cell CLL/lymphoma 10 (BCL10), mucosa-associated lymphoid tissue lymphoma translocation protein 1 (MALT1), and caspase recruitment domain–containing (CARD) family adaptors play a role in NF-κB activation and have been shown to be involved in both the innate and the adaptive arms of immunity in murine models. Moreover, individuals with inherited defects of MALT1, CARD9, and CARD11 present with immunological and clinical phenotypes. Here, we characterized a case of autosomal-recessive, complete BCL10 deficiency in a child with a broad immunodeficiency, including defects of both hematopoietic and nonhematopoietic immunity. The patient died at 3 years of age and was homozygous for a loss-of-expression, loss-of function BCL10 mutation. The effect of BCL10 deficiency was dependent on the signaling pathway, and, for some pathways, the cell type affected. Despite the noted similarities to BCL10 deficiency in mice, including a deficient adaptive immune response, human BCL10 deficiency in this patient resulted in a number of specific features within cell populations. Treatment of the patient’s myeloid cells with a variety of pathogen-associated molecular pattern molecules (PAMPs) elicited a normal response; however, NF-κB–mediated fibroblast functions were dramatically impaired. The results of this study indicate that inherited BCL10 deficiency should be considered in patients with combined immunodeficiency with B cell, T cell, and fibroblast defects.
- PublicationCost-minimization analysis of immunoglobulin treatment of primary immunodeficiency diseases in Spain(Springer Nature, 2021-09-21) Alsina, Laia; Montoro, J. Bruno; Moral, Pedro Moral; Neth, Olaf; Ortiz Pica, Marta; Sánchez Ramón, Silvia María; Presa, María; Oyagüez, Itziar; Casado, Miguel Ángel; González Granado, Luis IgnacioPrimary immunodefciency diseases (PID), which are comprised of over 400 genetic disorders, occur when a component of the immune system is diminished or dysfunctional. Patients with PID who require immunoglobulin (IG) replacement therapy receive intravenous IG (IVIG) or subcutaneous IG (SCIG), each of which provides equivalent efcacy. We developed a costminimization model to evaluate costs of IVIG versus SCIG from the Spanish National Healthcare System perspective. The base case modeled the annual cost per patient of IVIG and SCIG for the mean doses (per current expert clinical practice) over 1 year in terms of direct (drug and administration) and indirect (lost productivity for adults and parents/guardians of pediatric patients) costs. It was assumed that all IVIG infusions were administered in a day hospital, and 95% of SCIG infusions were administered at home. Drug costs were calculated from ex-factory prices obtained from local databases minus the mandatory deduction. Costs were valued on 2018 euros. The annual modeled costs were €4,266 lower for patients with PID who received SCIG (total €14,466) compared with those who received IVIG (total €18,732). The two largest contributors were diferences in annual IG costs as a function of dosage (– €1,927) and hospital administration costs (– €2,688). However, SCIG incurred training costs for home administration (€695). Sensitivity analyses for two dose-rounding scenarios were consistent with the base case. Our model suggests that SCIG may be a cost-saving alternative to IVIG for patients with PID in Spain.
- PublicationMetodología experimental aplicada a la Inmunología MolecularCruz Adalia, Aranzazu; Cabañas Gutiérrez, Carlos; Castillo Gonzalez, Raquel Ana; Lafuente Duarte, María Esther; Sánchez-Gómez, María José; Mazariegos León, Marina; Sancho Temiño, Lucía; Reche Gallardo, Pedro Antonio; Marín Marín, Ana Victoria; Garcia-Mauriño Muzquiz, Jose Enrique; Villa Gómez, Cristina Matilde; Fernando Peláez Prestel, Hector; Juárez Martín, Ignacio; Valle Noguera, Ana; Recio Hoyas, María José; Sánchez Ramón, Silvia María; Morlino, GiuliaEl objetivo general del proyecto es aplicar un modelo pedagógico en el que participen los alumnos de manera activa y apliquen el método científico en base a los conocimientos que han adquirido, resolviendo y realizando un caso práctico en el laboratorio. Integra una estrategia didáctica que va a fomentar la participación activa del alumnado provocando un aprendizaje significativo, ya que el alumno tiene que resolver mediante el razonamiento un caso práctico y luego integrarlo en el laboratorio con el uso de una técnica ampliamente utilizada en Inmunología, como es la citometría de flujo.