SIM - Sistema Imune (Doenças autoimunes, imunodeficiências e alergias)
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- The family physician and the human immunodeficiency virus seropositive patientPublication . MARCOS, T.; BARBOSA, A.; ALMEIDA, I.; BARBOSA, P.; VASCONCELOS, C.Acta Med Port. 2000 Jul-Aug;13(4):173-9. [The family physician and the human immunodeficiency virus seropositive patient]. [Article in Portuguese] Marcos T, Barbosa A, Almeida I, Barbosa P, Vasconcelos C. SourceCentro de Saúde da Batalha, Porto. Abstract OBJECTIVES: To characterize a subject who is HIV positive and closely observed in the consultation of clinical immunology (Santo António General Hospital), from a demographic and socio-economic point of view; to determine the percentage of these subjects who have a family doctor; how often they go to a family doctor; the reasons for a more frequent visit to the doctor; if there has been any alteration in the reasons for consulting the family doctor after establishing the diagnosis of HIV infection; who made the diagnosis of HIV infection; to evaluate, from these patient's point of view, if there has been any alteration in the family doctor's attitude or vice versa after the diagnosis. CHARACTERISATION OF THE STUDY: A descriptive, transversal study was carried out from 30/01/97 to 13/03/1997. METHOD: A questionnaire with twenty-two questions was used in a personal interview of a random sample of 100 patients observed in the Consultation of Clinical Immunology at Santo António General Hospital and represented 40% of the population studied. RESULTS: One hundred patients answered the questionnaire, 73 were male and 27 female, with an mean age of 34.73 years. The majority were single, representing 44% of the random sample, 33% had completed primary education (or equivalent), and 28% had attended secondary school (complete or incomplete), 35% of the subjects were employed and 34% unemployed. In what concerns area of residence, the majority live in the metropolitan area of Oporto. The majority of the patients (85%) had a family doctor. Nevertheless, 1/3 had never paid a visit to their doctor and, as for the others, the majority rarely did. After the diagnosis of HIV infection, the reasons that led patients to see their doctors were merely administrative (prescriptions--24.56%, sick leave--21.05%). On the other hand, before the diagnosis, health problems were their major concern and priority (27.54%). The diagnosis was made in equal proportion (28%) by the hospital services and by institutions providing health care for drug addicts. In most cases, from the patient's point of view, there had not been any change in the family doctor's attitude after the diagnosis of HIV infection and vice versa. COMMENTS: Although the majority of the subjects have a family doctor, their demand is very low, therefore, there is a waste of opportunities in terms of primary, secondary and tertiary medical care.
- Prolidase deficiency with hyperimmunoglobulin E: a case reportPublication . LOPES, I.; MARQUES, L.; NEVES, E.; SILVA, A.; TAVEIRA, M.; PENA, R.; VILARINHO, L.; MARTINS, E.
- Immunosuppressive therapy in lupus nephritis: the Euro‐Lupus Nephritis Trial, a randomized trial of low‐dose versus high‐dose intravenous cyclophosphamide.Publication . Houssiau, F.A.; Vasconcelos, C.; D'Cruz, D.; Sebastiani, G.D.; Garrido Ed Ede, R.; Danieli, M.G.; Abramovicz, D.; Blockmans, D.; Mathieu, A.; Direskeneli, H.; Galeazzi, M.; Gül, A.; Levy, Y.; Petera, P.; Popovic, R.; Petrovic, R; Sinico, R.A.; Cattaneo, R.; Font, J.; Depresseux, G.; Cosyns, J.P.; Cervera, R.Arthritis Rheum. 2002 Aug;46(8):2121-31. Immunosuppressive therapy in lupus nephritis: the Euro-Lupus Nephritis Trial, a randomized trial of low-dose versus high-dose intravenous cyclophosphamide. Houssiau FA, Vasconcelos C, D'Cruz D, Sebastiani GD, Garrido Ed Ede R, Danieli MG, Abramovicz D, Blockmans D, Mathieu A, Direskeneli H, Galeazzi M, Gül A, Levy Y, Petera P, Popovic R, Petrovic R, Sinico RA, Cattaneo R, Font J, Depresseux G, Cosyns JP, Cervera R. Cliniques Universitaires St. Luc, Université Catholique de Louvain, Brussels, Belgium. houssiau@ruma.ucl.ac.be Comment in: Arthritis Rheum. 2003 May;48(5):1466; author reply 1466-7. Abstract OBJECTIVE: Glomerulonephritis is a severe manifestation of systemic lupus erythematosus (SLE) that is usually treated with an extended course of intravenous (IV) cyclophosphamide (CYC). Given the side effects of this regimen, we evaluated the efficacy and the toxicity of a course of low-dose IV CYC prescribed as a remission-inducing treatment, followed by azathioprine (AZA) as a remission-maintaining treatment. METHODS: In this multicenter, prospective clinical trial (the Euro-Lupus Nephritis Trial [ELNT]), we randomly assigned 90 SLE patients with proliferative glomerulonephritis to a high-dose IV CYC regimen (6 monthly pulses and 2 quarterly pulses; doses increased according to the white blood cell count nadir) or a low-dose IV CYC regimen (6 fortnightly pulses at a fixed dose of 500 mg), each of which was followed by AZA. Intent-to-treat analyses were performed. RESULTS: Followup continued for a median of 41.3 months in the low-dose group and 41 months in the high-dose group. Sixteen percent of those in the low-dose group and 20% of those in the high-dose group experienced treatment failure (not statistically significant by Kaplan-Meier analysis). Levels of serum creatinine, albumin, C3, 24-hour urinary protein, and the disease activity scores significantly improved in both groups during the first year of followup. Renal remission was achieved in 71% of the low-dose group and 54% of the high-dose group (not statistically significant). Renal flares were noted in 27% of the low-dose group and 29% of the high-dose group. Although episodes of severe infection were more than twice as frequent in the high-dose group, the difference was not statistically significant. CONCLUSION: The data from the ELNT indicate that in European SLE patients with proliferative lupus nephritis, a remission-inducing regimen of low-dose IV CYC (cumulative dose 3 gm) followed by AZA achieves clinical results comparable to those obtained with a high-dose regimen. PMID: 12209517 [PubMed - indexed for MEDLINE]
- Self‐reported drug allergy in a general adult Portuguese populationPublication . GOMES, E.; CARDOSO, M.F.; PRAÇA, F.; GOMES, L.; MARIÑO, E.; DEMOLY, P.Clin Exp Allergy. 2004 Oct;34(10):1597-601. Self-reported drug allergy in a general adult Portuguese population. Gomes E, Cardoso MF, Praça F, Gomes L, Mariño E, Demoly P. Serviço de Imunoalergologia, Hospital Maria Pia, Porto, Portugal. evamariasrg@yahoo.com Abstract AIM: To estimate the prevalence of self-reported drug allergy in adults. METHODS: Cross-sectional survey of a general adult population from Porto (all of whom were living with children involved in the International Study of Asthma and Allergies in Childhood-phase three), during the year 2002, using a self-administered questionnaire. RESULTS: The prevalence of self-reported drug allergy was 7.8% (181/2309): 4.5% to penicillins or other beta-lactams, 1.9% to aspirin or other non-steroidal anti-inflammatory drugs (NSAIDs) and 1.5% to other drugs. In the group 'allergic to beta-lactams', the most frequently implicated drug was penicillin G or V (76.2%) followed by the association of amoxicillin and clavulanic acids (14.3%). In the group 'allergic to NSAIDs', acetylsalicylic acid (18.2%) and ibuprofen (18.2%) were the most frequently identified drugs, followed by nimesulide and meloxicam. Identification of the exact name of the involved drug was possible in less than one-third of the patients, more often within the NSAID group (59.5%). Women were significantly more likely to claim a drug allergy than men (10.2% vs. 5.3%). The most common manifestations were cutaneous (63.5%), followed by cardiovascular symptoms (35.9%). Most of the reactions were immediate, occurring on the first day of treatment (78.5%). Only half of the patients were submitted to drug allergy investigations. The majority (86.8%) completely avoided the suspected culprit drug thereafter. CONCLUSIONS: The results showed that self-reported allergy to drugs is highly prevalent and poorly explored. Women seem to be more susceptible. beta-lactams and NSAIDs are the most frequently concerned drugs. PMID: 15479276 [PubMed - indexed for MEDLINE]
- EarlyPublication . HOUSSIAU, F.A.; VASCONCELOS, C; D'CRUZ, D.; SEBASTIANI, G.D.; DE RAMON GARRIDO, E.; DANIELI, M.G.; ABRAMOVICZ, D.; BLOCKMANS, D.; MATHIEU, A.; DIRESKENELI, H.; GALEAZZI, M.; GUL, A.; LEVY, Y.; PETERA, P.; POPOVIC, R.; PETROVIC, R.; SINICO, R.A.; CATTANEO, R.; FONT, J.; DEPRESSEUX, G.; COSYNS, J.P.; CERVERA, R.Arthritis Rheum. 2004 Dec;50(12):3934-40. Early response to immunosuppressive therapy predicts good renal outcome in lupus nephritis: lessons from long-term followup of patients in the Euro-Lupus Nephritis Trial. Houssiau FA, Vasconcelos C, D'Cruz D, Sebastiani GD, de Ramon Garrido E, Danieli MG, Abramovicz D, Blockmans D, Mathieu A, Direskeneli H, Galeazzi M, Gül A, Levy Y, Petera P, Popovic R, Petrovic R, Sinico RA, Cattaneo R, Font J, Depresseux G, Cosyns JP, Cervera R. Université Catholique de Louvain, Brussels, Belgium. houssiau@ruma.ucl.ac.be Abstract OBJECTIVE: In the Euro-Lupus Nephritis Trial (ELNT), 90 patients with lupus nephritis were randomly assigned to a high-dose intravenous cyclophosphamide (IV CYC) regimen (6 monthly pulses and 2 quarterly pulses with escalating doses) or a low-dose IV CYC regimen (6 pulses of 500 mg given at intervals of 2 weeks), each of which was followed by azathioprine (AZA). After a median followup of 41 months, a difference in efficacy between the 2 regimens was not observed. The present analysis was undertaken to extend the followup and to identify prognostic factors. METHODS: Renal function was prospectively assessed quarterly in all 90 patients except 5 who were lost to followup. Survival curves were derived using the Kaplan-Meier method. RESULTS: After a median followup of 73 months, there was no significant difference in the cumulative probability of end-stage renal disease or doubling of the serum creatinine level in patients who received the low-dose IV CYC regimen versus those who received the high-dose regimen. At long-term followup, 18 patients (8 receiving low-dose and 10 receiving high-dose treatment) had developed permanent renal impairment and were classified as having poor long-term renal outcome. We demonstrated by multivariate analysis that early response to therapy at 6 months (defined as a decrease in serum creatinine level and proteinuria <1 g/24 hours) was the best predictor of good long-term renal outcome. CONCLUSION: Long-term followup of patients from the ELNT confirms that, in lupus nephritis, a remission-inducing regimen of low-dose IV CYC followed by AZA achieves clinical results comparable with those obtained with a high-dose regimen. Early response to therapy is predictive of good long-term renal outcome. PMID: 15593207 [PubMed - indexed for MEDLINE]
- Early response to immunosuppressive therapy predicts good renal outcome in lupus nephritis: lessons from long-term followup of patients in the Euro-Lupus Nephritis Trial.Publication . Houssiau, F.A.; Vasconcelos, C.; D'Cruz, D.; Sebastiani, G.D.; de Ramon Garrido, E.; Danieli, M.G.; Abramovicz, D.; Blockmans, D.; Mathieu, A.; Direskeneli, H.; Galeazzi, M.; Gül, A.; Levy, Y.; Petera, P.; Popovic, R.; Petrovic, R.; Sinico, R.A.; Cattaneo, R.; Font, J.; Depresseux, G.; Cosyns, J.P.; Cervera, R.Arthritis Rheum. 2004 Dec;50(12):3934-40. Early response to immunosuppressive therapy predicts good renal outcome in lupus nephritis: lessons from long-term followup of patients in the Euro-Lupus Nephritis Trial. Houssiau FA, Vasconcelos C, D'Cruz D, Sebastiani GD, de Ramon Garrido E, Danieli MG, Abramovicz D, Blockmans D, Mathieu A, Direskeneli H, Galeazzi M, Gül A, Levy Y, Petera P, Popovic R, Petrovic R, Sinico RA, Cattaneo R, Font J, Depresseux G, Cosyns JP, Cervera R. Université Catholique de Louvain, Brussels, Belgium. houssiau@ruma.ucl.ac.be Abstract OBJECTIVE: In the Euro-Lupus Nephritis Trial (ELNT), 90 patients with lupus nephritis were randomly assigned to a high-dose intravenous cyclophosphamide (IV CYC) regimen (6 monthly pulses and 2 quarterly pulses with escalating doses) or a low-dose IV CYC regimen (6 pulses of 500 mg given at intervals of 2 weeks), each of which was followed by azathioprine (AZA). After a median followup of 41 months, a difference in efficacy between the 2 regimens was not observed. The present analysis was undertaken to extend the followup and to identify prognostic factors. METHODS: Renal function was prospectively assessed quarterly in all 90 patients except 5 who were lost to followup. Survival curves were derived using the Kaplan-Meier method. RESULTS: After a median followup of 73 months, there was no significant difference in the cumulative probability of end-stage renal disease or doubling of the serum creatinine level in patients who received the low-dose IV CYC regimen versus those who received the high-dose regimen. At long-term followup, 18 patients (8 receiving low-dose and 10 receiving high-dose treatment) had developed permanent renal impairment and were classified as having poor long-term renal outcome. We demonstrated by multivariate analysis that early response to therapy at 6 months (defined as a decrease in serum creatinine level and proteinuria <1 g/24 hours) was the best predictor of good long-term renal outcome. CONCLUSION: Long-term followup of patients from the ELNT confirms that, in lupus nephritis, a remission-inducing regimen of low-dose IV CYC followed by AZA achieves clinical results comparable with those obtained with a high-dose regimen. Early response to therapy is predictive of good long-term renal outcome. PMID: 15593207 [PubMed - indexed for MEDLINE]
- Alergia a fármacos na criançaPublication . Gomes, E.RESUMO As reacções alérgicas a fármacos fazem parte do universo mais alargado das reacções adversas a fármacos e a sua classificação implica a demonstração de um mecanismo imunológico especifico subjacente. Estas reacções são frequentes quer em adultos quer em idades pediátricas e acarretam morbilidade e mesmo mortalidade apreciável. A sua investigação, a ser realizada em meio hospitalar, assenta numa anamenese cuidadosa complementada por testes in vivo e ocasionalmente por testes in vitro. Só o estabelecimento de um diagnóstico correcto permite o aconselhamento do doente relativamente a futuras opções terapêuticas, evitando riscos de novas reacções e o uso indiscriminado de fármacos menos eficazes e mais dispendiosos. Esta investigação é também fundamental para o progresso na compreensão dos mecanismos fisiopatológicos envolvidos o que permitirá implementar melhores medidas preventivas.
- ErisipelaPublication . Caetano, M.; Amorim, I.A erisipela é uma infecção dermo-hipodérmica aguda, não necrosante, geralmente causada pelo estreptococo β–hemolítico do grupo A. Em mais de 80% dos casos situa-se nos membros inferiores e são factores predisponentes a existência de solução de continuidade na pele, o linfedema crónico e a obesidade. O seu diagnóstico é essencialmente clínico e baseia-se na presença de placa inflamatória associada a febre,linfangite, adenopatia e leucocitose. Os exames bacteriológicos têm baixa sensibilidade ou positividade tardia. Nos casos atípicos é importante o diagnóstico diferencial com a fasceíte necrosante e a trombose venosa profunda. A penicilina continua a ser o antibiótico de referência, embora actualmente diversos fármacos, com propriedades farmacodinâmicas mais favoráveis, possam ser utilizados. A recidiva constitui a complicação mais frequente, sendo fundamental o correcto tratamento dos factores de risco.
- Mutations in STAT3 and IL12RB1 impair the development of human IL-17 – producing T cellsPublication . Beaucoudrey, L.; Puel, A.; Filipe-Santos, O.; Cobat, A.; Ghandil, P.; Chrabieh, M.; Feinberg, J.; Bernuth, H.; Samarina, A.; Jannière, L.; Fieschi, C.; Stéphan, J.; Boileau, C.; Lyonnet, S.; Jondeau, G.; Cormier-Daire, V.; Merrer, M.; Hoarau, C.; Lebranchu, Y.; Lortholary, O.; Chandesris, M.; Tron, F.; Gambineri, E.; Bianchi, L.; Rodriguez-Gallego, C.; Zitnik, S.; Vasconcelos, J.; Guedes, M.; Vitor, A.; Marodi, L.; Chapel, H.; Reid, B.; Roifman, C.; Nadal, D.; Reichenbach, J.; Caragol, I.; Garty, B.; Dogu, F.; Camcioglu, Y.; Gülle, S.; Sanal, O.; Fischer, A.; Abel, L.; Stockinger, B.; Picard, C.; Casanova, J.Abstract The cytokines controlling the development of human interleukin (IL) 17--producing T helper cells in vitro have been difficult to identify. We addressed the question of the development of human IL-17--producing T helper cells in vivo by quantifying the production and secretion of IL-17 by fresh T cells ex vivo, and by T cell blasts expanded in vitro from patients with particular genetic traits affecting transforming growth factor (TGF) beta, IL-1, IL-6, or IL-23 responses. Activating mutations in TGFB1, TGFBR1, and TGFBR2 (Camurati-Engelmann disease and Marfan-like syndromes) and loss-of-function mutations in IRAK4 and MYD88 (Mendelian predisposition to pyogenic bacterial infections) had no detectable impact. In contrast, dominant-negative mutations in STAT3 (autosomal-dominant hyperimmunoglobulin E syndrome) and, to a lesser extent, null mutations in IL12B and IL12RB1 (Mendelian susceptibility to mycobacterial diseases) impaired the development of IL-17--producing T cells. These data suggest that IL-12Rbeta1- and STAT-3--dependent signals play a key role in the differentiation and/or expansion of human IL-17-producing T cell populations in vivo.
- Cobertura vacinal das vacinas meningocócica e pneumocócica num centro de saúde de Vila Nova de GaiaPublication . Santos, H.; Pinto, E.; Valente, I.; Marinheiro, M.; Almeida, S.; Pinto, M. E.Objectivos: A vacinação é um dos melhores exemplos de prevenção primária em saúde. Este estudo pretendeu estimar a cobertura das vacinas meningocócica (MnC) e pneumocócica (Pn7) numa coorte de crianças utentes de um centro de saúde do Norte de Portugal. Métodos: Realização de um estudo longitudinal de uma amostra representativa das crianças nascidas entre 2000 e 2003. Os dados foram recolhidos dos registos de vacinação do centro de saúde. Resultados: Foram recolhidos dados de 290 crianças, 41,4% raparigas. A cobertura vacinal 2000-2003 foi de 76,9% para a MnC e 41,4% para a Pn7. Quarenta e sete por cento tinham ambas as vacinas. O número das crianças que fizeram pelo menos uma dose de MnC ou Pn7 foi máximo em 2002. No período estudado, 3,8% (MnC) e 21.5% (Pn7) das 290 crianças iniciou mas não terminou a vacinação. A mediana da primeira dose foi de 11 meses para a MnC, e 6,5 meses para a Pn7. Noventa e sete por cento das crianças com vacinação completa para Pn7 também a tinham para a MnC, mas somente em 52% das crianças acontecia o inverso (p<0,001). A proporção das crianças que receberam outras vacinas extra plano foi de 24.4% dentro das crianças que receberam MnC e Pn7 e de 4,0% nas restantes (p<0,001). Conclusões: Encontrou-se uma cobertura vacinal elevada para ambas as vacinas, apesar dos custos para os utentes. A maioria da cobertura para a MnC foi devido à vacinação no segundo ano da vida, exigindo apenas 1 dose. Um número considerável de crianças iniciou mas não terminou a programa de vacinação. A idade da primeira toma diminuiu entre 2000 e 2003, proporcionando uma melhor cobertura no período mais vulnerável da doença. Observou-se uma associação entre a administração de Pn7 e MnC, e outras vacinas extra plano. Palavras-chave: Cobertura vacinal, vacina meningocócica, vacina pneumocócica, vacinas conjugadas.