The Journal of allergy and clinical immunology · 2019
Latin American consensus on the supportive management of patients with severe combined immunodeficiency.
СтатьяПоддержка исследования: вне правительства СШАConsensus StatementНазвание и аннотация: Перевод пока не подготовлен
Обработка статьи · этапы, ошибки и управление
Загрузка состояния…
Ошибки и ограничения
История · было → стало
Состояние обновляется каждые 5 секунд, пока блок открыт. Процент показывается только там, где известен объём работы. Пауза останавливает продолжение этапа; текущий запрос к источнику может завершиться.
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
Аннотация
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.