94 research outputs found
Guidelines for the use of human immunoglobulin therapy in patients with primary immunodeficiencies in Latin America
Antibodies are an essential component of the adaptative immune response and hold long-term memory of the immunological experiences throughout life. Antibody defects represent approximately half of the well-known primary immunodeficiencies requiring immunoglobulin replacement therapy. In this article, the authors review the current indications and therapeutic protocols in the Latin American environment. Immunoglobulin replacement therapy has been a safe procedure that induces dramatic positive changes in the clinical outcome of patients who carry antibody defects.Fil: Condino Neto, A.. Universidade de Sao Paulo; BrasilFil: Costa Carvalho, B. T.. Universidade Federal de Sao Paulo; BrasilFil: Grumach, A. S.. No especifĂca;Fil: King, A.. No especifĂca;Fil: Bezrodnik, Liliana. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños "Ricardo GutiĂ©rrez". Ărea de InmunologĂa; Argentina. Consejo Nacional de Investigaciones CientĂficas y TĂ©cnicas; ArgentinaFil: Oleastro, M.. Gobierno de la Ciudad de Buenos Aires. Hospital de PediatrĂa "Juan P. Garrahan"; ArgentinaFil: Leiva, L.. Louisiana Tech University; Estados UnidosFil: Porras, O.. Hospital Nacional de Ninos Ë Dr Carlos SĂĄenz Herrera"; Costa RicaFil: Espinosa Rosales, F. J.. Instituto Nacional de PediatrĂa; MĂ©xicoFil: Franco, J. L.. Universidad de Antioquia; ColombiaFil: Sorensen, R. U.. Universidad de La Frontera; Chil
Variability of disease activity in patients with hereditary angioedema type 1/2: longitudinal data from the Icatibant Outcome Survey.
Funder: Takeda Pharmaceuticals International AG, Zurich, SwitzerlandBACKGROUND: Hereditary angioedema due to C1 inhibitor deficiency (HAE-1/2) is a chronic and debilitating disease. The unpredictable clinical course represents a significant patient burden. OBJECTIVE: To analyse longitudinal registry data from the Icatibant Outcome Survey (IOS) in order to characterize temporal changes in disease activity in patients with HAE-1/2. METHODS: Icatibant Outcome Survey (NCT01034969) is an international observational registry monitoring the clinical outcomes of patients eligible for icatibant treatment. The current analyses are based on data collected between July 2009 and July 2019. Retrospective data for attacks recorded in the 12âmonths prior to IOS enrolment and for each 12-month period up to 7âyears were analysed. RESULTS: Included patients reported angioedema attacks without long-term prophylaxis (LTP; nâ=â315) and with LTP (nâ=â292) use at the time of attack onset. Androgens were the most frequently used LTP option (80.8%). At the population level, regardless of LTP use, most patients (52-80%) reporting <5 attacks in Year 1 continued experiencing this rate; similarly, many patients (25-76%) who reported high attack frequency continued reporting â„10 attacks/year. However, year on year, 31-51% of patients experienced notable changes (increase/decrease of â„5 attacks) in annual attack frequency. Of patients who reported an absolute change of â„10 attacks from Year 1 to 2, 17-50% continued to experience a change of this magnitude in subsequent years. CONCLUSION: At the population level, attack frequency was generally consistent over 7âyears. At the small group level, 28.8-34.5% of patients reported a change in attack frequency of â„5 attacks from Year 1 to Year 2; up to half of these patients continued to experience this magnitude of variation in disease activity in later years, reflecting high intra-patient variability
Population Prevalence of Diagnosed Primary Immunodeficiency Diseases in the United States
Controlo quĂmico de infestantes
Uma planta Ă© considerada infestante quando nasce espontaneamente num local e momento indesejados, podendo interferir negativamente com a cultura instalada.
As infestantes competem com as culturas para o espaço, a luz, ågua e nutrientes, podendo atrasar e prejudicar as operaçÔes de colheita, depreciar o produto final e assegurarem a reinfestação nas culturas seguintes.
Dado o modo de propagação diferenciado das diversas espĂ©cies de infestantes, com as anuais a propagarem-se por semente e as perenes ou vivazes a assegurarem a sua propagação atravĂ©s de ĂłrgĂŁos vegetativos (rizomas, bolbos, tubĂ©rculos, etc.), assim, tambĂ©m o seu controlo quer quĂmico, quer mecĂąnico terĂĄ que ser diferenciado, ou seja, para controlar infestantes anuais serĂĄ suficiente destruir a sua parte aĂ©rea, enquanto para controlar infestantes perenes teremos que destruir os seus ĂłrgĂŁos reprodutivos.
O controlo de infestantes poderĂĄ ser quĂmico, atravĂ©s da utilização de herbicidas, ou mecĂąnico pela utilização de alfaias agrĂcolas, tais como a charrua de aivecas, a charrua de discos, a grade de discos, o escarificador e a fresa. Quando a tĂ©cnica utilizada na instalação das culturas Ă© a sementeira directa, o controlo das infestantes terĂĄ que ser obrigatoriamente quĂmico, enquanto se o recurso Ă mobilização do solo for a tĂ©cnica mais utilizada (sistema de mobilização tradicional ou sistema de mobilização reduzida), o controlo das infestantes tanto poderĂĄ ser quĂmico como mecĂąnico.
Neste trabalho iremos abordar apenas, o controlo quĂmico de infestantes
Attending to warning signs of primary immunodeficiencies disease across the range of clinical practices
Purpose: Patients with primary immunodeficiency diseases (PIDD) may present with recurrent infections affecting different organs, organ-specific inflammation/autoimmunity, and also increased cancer risk, particularly hematopoietic malignancies. The diversity of PIDD and the wide age range over which these clinical occurrences become apparent often make the identification of patients difficult for physicians other than immunologists. The aim of this report is to develop a tool for educative programs targeted to specialists and applied by clinical immunologists.
Methods: Considering the data from national surveys and clinical reports of experiences with specific PIDD patients, an evidence-based list of symptoms, signs, and corresponding laboratory tests were elaborated to help physicians other than immunologists look for PIDD.
Results: Tables including main clinical manifestations, restricted immunological evaluation, and possible related diagnosis were organized for general practitioners and 5 specialties. Tables include information on specific warning signs of PIDD for pulmonologists, gastroenterologists, dermatologists, hematologists, and infectious disease specialists.
Conclusions: This report provides clinical immunologists with an instrument they can use to introduce specialists in other areas of medicine to the warning signs of PIDD and increase early diagnosis. Educational programs should be developed attending the needs of each specialty.Fil: Costa Carvalho, Beatriz Tavares. Universidade Federal de SĂŁo Paulo; BrasilFil: Sevciovic Grumach, Anete. Fundação ABC. Faculdade de Medicina; BrasilFil: Franco, JosĂ© Luis. Universidad de Antioquia; ColombiaFil: Espinosa Rosales, Francisco Javier. Instituto Nacional de PediatrĂa. Unidad de InvestigaciĂłn en Inmunodeficiencias; MĂ©xicoFil: Leiva, Lily E.. State University of Louisiana; Estados UnidosFil: King, Alejandra. Hospital de Niños Doctor Luis Calvo Mackenna. Unidad de InmunologĂa; ChileFil: Porras, Oscar. Hospital Nacional de Niños âDr. Carlos SĂĄenz Herreraâ; Costa RicaFil: Bezrodnik, Liliana. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños "Ricardo GutiĂ©rrez"; Argentina. Consejo Nacional de Investigaciones CientĂficas y TĂ©cnicas; ArgentinaFil: Oleastro, Mathias. Gobierno de la Ciudad de Buenos Aires. Hospital de PediatrĂa "Juan P. Garrahan"; ArgentinaFil: Sorensen, Ricardo U.. State University of Louisiana; Estados Unidos. Universidad de La Frontera. Facultad de Medicina; MĂ©xicoFil: Condino Neto, Antonio. Universidade de Sao Paulo; Brasi
TRIAGEM NEONATAL DE IMUNODEFICIĂNCIAS GRAVES COMBINADAS POR MEIO DE TRECS E KRECS: SEGUNDO ESTUDO PILOTO NO BRASIL
RESUMO Objetivo: Validar a quantificação de T-cell receptor excision circles (TRECs) e kappa-deleting recombination circles (KRECs) por reação em cadeia de polimerase (polymerase chain reaction, PCR) em tempo real (qRT-PCR), para triagem neonatal de imunodeficiĂȘncias primĂĄrias que cursam com defeitos nas cĂ©lulas T e/ou B no Brasil. MĂ©todos: Amostras de sangue de recĂ©m-nascidos (RN) e controles foram coletadas em papel-filtro. O DNA foi extraĂdo e os TRECs e KRECs foram quantificados por reação duplex de qRT-PCR. O valor de corte foi determinado pela anĂĄlise de Receiver Operating Characteristics Curve, utilizando-se o programa Statistical Package for the Social Sciences (SSPS) (IBMÂź, Armonk, NY, EUA). Resultados: 6.881 amostras de RN foram analisadas quanto Ă concentração de TRECs e KRECs. Os valores de TRECs variaram entre 1 e 1.006 TRECs/”L, com mĂ©dia e mediana de 160 e 139 TRECs/”L, respectivamente. TrĂȘs amostras de pacientes diagnosticados com imunodeficiĂȘncia grave combinada (severe combined immunodeficiency, SCID) apresentaram valores de TRECs abaixo de 4/”L e um paciente com SĂndrome de DiGeorge apresentou TRECs indetectĂĄveis. Os valores de KRECs encontraram-se entre 10 e 1.097 KRECs/”L, com mĂ©dia e mediana de 130 e 108 KRECs/”L, e quatro pacientes com diagnĂłstico de agamaglobulinemia tiveram resultados abaixo de 4 KRECs/”L. Os valores de corte encontrados foram 15 TRECs/”L e 14 KRECs/”L, e foram estabelecidos de acordo com a anĂĄlise da Receiver Operating Characteristics Curve, com sensibilidade de 100% para detecção de SCID e agamaglobulinemia, respectivamente. ConclusĂ”es: A quantificação de TRECs e KRECs foi capaz de diagnosticar crianças com linfopenias T e/ou B em nosso estudo, validando a tĂ©cnica e dando o primeiro passo para a implementação da triagem neonatal em grande escala no Brasil
Definition, aims, and implementation of GA2LEN/HAEi Angioedema Centers of Reference and Excellence
Primary complement and antibody deficiencies in autoimmune rheumatologic diseases with juvenile onset: a prospective study at two centers
Breastfeeding: making the difference in the development, health and nutrition of term and preterm newborns
- âŠ