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Latin American consensus on the supportive management of patients with severe combined immunodeficiency.
Bustamante Ogando, Juan Carlos; Partida Gaytán, Armando; Aldave Becerra, Juan Carlos; Álvarez Cardona, Aristóteles; Bezrodnik, Liliana; Borzutzky, Arturo; Blancas Galicia, Lizbeth; Cabanillas, Diana; Condino-Neto, Antonio; De Colsa Ranero, Agustín; Espinosa Padilla, Sara; Fernandes, Juliana Folloni; García Campos, Jorge Alberto; Gómez Tello, Héctor; González Serrano, María Edith; Gutiérrez Hernández, Alonso; Hernández Bautista, Víctor Manuel; Ivankovich Escoto, Gabriele; King, Alejandra; Lessa Mazzucchelli, Juliana; Llamas Guillén, Beatriz Adriana; Lugo Reyes, Saul Oswaldo; Moreno Espinosa, Sarbelio; Oleastro, Matías; Otero Mendoza, Francisco; Poli Harlowe, María Cecilia; Porras, Oscar; Ramirez Uribe, Nideshda; Regairaz, Lorean; Rivas Larrauri, Francisco; Saracho Weber, Federico José; Grumach, Anete S; Staines Boone, Tamara; Tavares Costa-Carvalho, Beatriz; Yamazaki Nakashimada, Marco Antonio; Espinosa Rosales, Francisco Javier.
Afiliación
  • Bustamante Ogando JC; Immunodeficiencies Research Unit, Instituto Nacional de Pediatria, Mexico City, Mexico. Electronic address: drbustamante_inp@hotmail.com.
  • Partida Gaytán A; Immunodeficiencies Research Unit, Instituto Nacional de Pediatria, Mexico City, Mexico.
  • Aldave Becerra JC; Division of Allergy and Immunology, Hospital Nacional Edgardo Rebagliati Martins Lima, Lima, Peru.
  • Álvarez Cardona A; Unidad de Investigación en Inmunología Clínica y Alergia, Aguascalientes, Mexico.
  • Bezrodnik L; Immunology Department, Hospital de Niños "Ricardo Gutiérrez", Buenos Aires, Argentina; Centro de Inmunología Clínica "Dra. Bezrodnik", Buenos Aires, Argentina.
  • Borzutzky A; Department of Pediatric Infectious Diseases and Immunology, School of Medicine, Pontificia Universidad Católica de Chile, Santiago, Chile.
  • Blancas Galicia L; Immunodeficiencies Research Unit, Instituto Nacional de Pediatria, Mexico City, Mexico.
  • Cabanillas D; Immunology Unit, Children's Hospital "Superiora Sor Maria Ludovica", La Plata, Argentina.
  • Condino-Neto A; Department of Immunology, Institute of Biomedical Sciences, University of Sao Paulo, Sao Paulo, Brazil.
  • De Colsa Ranero A; Department of Infectious Diseases, Instituto Nacional de Pediatria, Mexico City, Mexico.
  • Espinosa Padilla S; Immunodeficiencies Research Unit, Instituto Nacional de Pediatria, Mexico City, Mexico.
  • Fernandes JF; Departamento do Hematología e Transplante de Medula Óssea, Hospitalo Israelita Albert Einstein, Sao Paulo, Brazil.
  • García Campos JA; Infectious Disease Department, Hospital de Especialidades, Monterrey, Mexico.
  • Gómez Tello H; Immunology Department, Hospital para el Niño Poblano, Puebla, Mexico.
  • González Serrano ME; Immunodeficiencies Research Unit, Instituto Nacional de Pediatria, Mexico City, Mexico.
  • Gutiérrez Hernández A; Department of Clinical Immunology, Instituto Nacional de Pediatria, Mexico City, Mexico.
  • Hernández Bautista VM; Department of Clinical Immunology, Instituto Nacional de Pediatria, Mexico City, Mexico.
  • Ivankovich Escoto G; School of Medicine, University of Costa Rica, San José, Costa Rica; Pediatric Immunology and Rheumatology, Hospital Nacional de Niños "Dr. Carlos Sáenz Herrera", San José, Costa Rica.
  • King A; Division of Pediatric Immunology, Hospital Luis Calvo Mackenna, Santiago, Chile.
  • Lessa Mazzucchelli J; Department of Pediatrics, Universidade Federal de Sao Paulo, Sao Paulo, Brazil.
  • Llamas Guillén BA; Immunology Department, Hospital del Niño Morelense, Morelos, Mexico.
  • Lugo Reyes SO; Immunodeficiencies Research Unit, Instituto Nacional de Pediatria, Mexico City, Mexico.
  • Moreno Espinosa S; Department of Infectious Diseases, Hospital Infantil de México "Federico Gómez", Mexico City, Mexico.
  • Oleastro M; Department of Immunology and Rheumatology, Hospital de Pediatría J. P. Garrahan, Buenos Aires, Argentina.
  • Otero Mendoza F; Department of Infectious Diseases, Instituto Nacional de Pediatria, Mexico City, Mexico.
  • Poli Harlowe MC; Instituto de ciencias e innovación en medicina, Universidad del Desarrollo-Clinica Alemana, Santiago, Chile.
  • Porras O; Pediatric Immunology and Rheumatology, Hospital Nacional de Niños "Dr. Carlos Sáenz Herrera", San José, Costa Rica.
  • Ramirez Uribe N; the Hematopoietic Stem Cell Transplantation Unit, Instituto Nacional de Pediatria, Mexico City, Mexico.
  • Regairaz L; Immunology Unit, Children's Hospital "Superiora Sor Maria Ludovica", La Plata, Argentina.
  • Rivas Larrauri F; Department of Clinical Immunology, Instituto Nacional de Pediatria, Mexico City, Mexico.
  • Saracho Weber FJ; Hospital Infantil de Especialidades, Chihuahua, Mexico.
  • Grumach AS; Division of Clinical Immunology, Facultade de Medicina ABC, Sao Paulo, Brazil.
  • Staines Boone T; Clinical Immunology Department, Hospital de Especialidades, Monterrey, Mexico.
  • Tavares Costa-Carvalho B; Allergy and Immunology Unit, Department of Pediatrics, Universidade Federal de Sao Paulo, Santo Andre, Sao Paulo, Brazil.
  • Yamazaki Nakashimada MA; Department of Clinical Immunology, Instituto Nacional de Pediatria, Mexico City, Mexico.
  • Espinosa Rosales FJ; Fundación Mexicana para Niñas y Niños con Inmunodeficiencias Primarias (FUMENI), Mexico City, Mexico.
J Allergy Clin Immunol ; 144(4): 897-905, 2019 10.
Article en En | MEDLINE | ID: mdl-31419546
Severe combined immunodeficiency (SCID) represents the most lethal form of primary immunodeficiency, with mortality rates of greater than 90% within the first year of life without treatment. Hematopoietic stem cell transplantation and gene therapy are the only curative treatments available, and the best-known prognostic factors for success are age at diagnosis, age at hematopoietic stem cell transplantation, and the comorbidities that develop in between. There are no evidence-based guidelines for standardized clinical care for patients with SCID during the time between diagnosis and definitive treatment, and we aim to generate a consensus management strategy on the supportive care of patients with SCID. First, we gathered available information about SCID diagnostic and therapeutic guidelines, then we developed a document including diagnostic and therapeutic interventions, and finally we submitted the interventions for expert consensus through a modified Delphi technique. Interventions are grouped in 10 topic domains, including 123 "agreed" and 38 "nonagreed" statements. This document intends to standardize supportive clinical care of patients with SCID from diagnosis to definitive treatment, reduce disease burden, and ultimately improve prognosis, particularly in countries where newborn screening for SCID is not universally available and delayed diagnosis is the rule. Our work intends to provide a tool not only for immunologists but also for primary care physicians and other specialists involved in the care of patients with SCID.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Inmunodeficiencia Combinada Grave / Guías de Práctica Clínica como Asunto Tipo de estudio: Guideline / Prognostic_studies Límite: Humans Idioma: En Revista: J Allergy Clin Immunol Año: 2019 Tipo del documento: Article Pais de publicación: Estados Unidos

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Inmunodeficiencia Combinada Grave / Guías de Práctica Clínica como Asunto Tipo de estudio: Guideline / Prognostic_studies Límite: Humans Idioma: En Revista: J Allergy Clin Immunol Año: 2019 Tipo del documento: Article Pais de publicación: Estados Unidos