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1.
Cytokine ; 83: 27-32, 2016 07.
Artigo em Inglês | MEDLINE | ID: mdl-27026419

RESUMO

BACKGROUND AND OBJECTIVE: Cytokines have an important role in the pathogenesis of rheumatoid arthritis (RA). Although plasma levels of IL-6 have been related to musculoskeletal ultrasound (MSUS) synovitis in early DMARD-naïve RA, there are no similar studies in established disease. METHODS: 64 RA patients treated with non-biological DMARDs and 30 healthy controls were included in this prospective cross-sectional study. A blood sample was taken before evaluation of disease activity (DAS28) and ultrasonography (all tests performed in a blinded fashion). MSUS was performed by one of two ultrasound-trained rheumatologists on 10 joints of both hands. Gray scale (GS) and pD (power Doppler) synovitis were evaluated using a semi-quantitative scale (0-3) in individual joints, and their sum (score 10) was calculated. Plasma cytokines (IL-2, IL-4, IL-6, IL-10, IL-17, TNF, IFN-γ, and VEGF) were quantified by flow cytometry. RESULTS: Levels of all cytokines, excepting VEGF, were significantly higher in RA patients than in controls (P⩽0.05). In RA patients, IL-6, but not other cytokines, correlated positively with DAS28 and swollen joint count (P⩽0.01), as well as with 10-joint pD score, and GS and pD of both wrists (P<0.01 for all tests). In multiple linear regression, the association of IL-6 with 10-joint pD score was maintained even after adjustment for DAS28. However, there was no correlation of IL-6 with tender joint count, 10-joint GS score, or presence of erosions. CONCLUSION: We demonstrated an association of inflammatory findings on MSUS and plasma IL-6 independently of DAS28 in established RA.


Assuntos
Artrite Reumatoide/sangue , Artrite Reumatoide/diagnóstico por imagem , Interleucina-6/sangue , Sinovite/sangue , Sinovite/diagnóstico por imagem , Ultrassonografia Doppler , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade
3.
Adv Rheumatol ; 61(1): 47, 2021 07 20.
Artigo em Inglês | MEDLINE | ID: mdl-34284822

RESUMO

PURPOSE: To evaluate the quality of referrals for a first Rheumatology consultation at a tertiary care center in a southern Brazilian capital (Porto Alegre, RS), having as background findings from a similar survey performed in 2007/2008. Since then, our state has implemented referral protocols and a triage system with teleconsulting support exclusively for referrals from locations outside the capital, permitting a comparison between patients screened and not screened by the new system. METHODS: Physicians of the Rheumatology Service at Hospital Nossa Senhora da Conceição prospectively collected information regarding first visits over a 6-month period (Oct 2017 to March 2018). We recorded demographic characteristics, diagnostic hypotheses, date of referral, and the municipality of origin (within the state of Rio Grande do Sul). We considered adequate referrals from primary health care when a systemic autoimmune inflammatory disease (SIRD) was suspected at first evaluation by the attending rheumatologist. RESULTS: Three hundred fifty-seven patients/appointments were eligible for analysis (193 from the capital and 164 from small and medium towns). In 2007/2008, suspected SIRD occurred in 76/260 (29.2%) and 73/222 (32.9%) among patients from the capital and outside counties, respectively (P = 0.387). In 2017/2018, suspected SIRD occurred in 75/193 (38.9%) and 111/164 (67.7%) in patients from the capital and outside counties, respectively (difference: 28.8, 95% CI: 19.0 to 38.9, P < 0.001), indicating a marked improvement in referrals submitted to the new triage system. CONCLUSION: The quality of Rheumatology referrals in our state improved over the 10-year interval under study, particularly among patients from locations submitted to referral protocols and teleconsulting support.


Assuntos
Encaminhamento e Consulta , Reumatologia , Telemedicina , Triagem , Humanos , Encaminhamento e Consulta/normas , Telemedicina/organização & administração , Triagem/organização & administração
4.
Adv Rheumatol ; 59(1): 26, 2019 07 02.
Artigo em Inglês | MEDLINE | ID: mdl-31266534

RESUMO

BACKGROUND: Imbalance and disfuntion in regulatory T-cells (Tregs) and IL-17 producer lymphocytes (Th17) have been implicated in the pathogenesis of rheumatoid arthritis (RA). Gray scale synovial proliferation (GS), power Doppler signal (pD) and bone erosions seen on high resolution muskuloskeletal ultrasound (MSUS) are hallmarks of destructive articular disease. OBJECTIVE: To evaluate the association of peripheral Tregs and Th17 with MSUS findings in RA. METHODS: RA patients (1987 ACR criteria) treated with disease-modifying antirheumatic drugs (DMARDs) were included. Lymphocytes were isolated and immunophenotyped by flow cytometry to investigate regulatory FoxP3+ T cells and IL-17+ cells. MSUS (MyLab 60, Esaote, Genova, Italy, linear probe 6-18 MHz) was performed on hand joints, and a 10-joint US score was calculated for each patient. RESULTS: Data on lymphocytes subsets were avaiable for 90 patients. The majority of patients were Caucasian women with a median disease duration of 6 years (interquartile range: 2-13 years). Mean DAS28 was 4.28 (SD ± 1.64) and mean HAQ score was 1.11 (SD ± 0.83). There was no significant correlation of 10-joint GS score (rS = 0.122, 95% CI: - 0.124 to 0.336, P = 0.254) and 10-joint pD score (rS = 0.056, 95% CI: - 0.180 to 0.273, P = 0.602) with the mean percentage of peripheral Treg cells. Also, 10-joint GS score (rS = 0.083, 95% CI: - 0.125 to 0.302, P = 0.438) and 10-joint pD score 10 (rS = - 0.060, 95% CI: - 0.271 to 0.150, P = 0.575); did not correlate to Th17 profile. No association of bone erosions on MSUS with Treg and Th17 profiles (P = 0.831 and P = 0.632, respectively) was observed. CONCLUSION: In this first study addressing MSUS features and lymphocytes subtypes in established RA, data did not support an association of circulating Tregs and Th17 lymphocytes with inflammatory and structural damage findings on MSUS.


Assuntos
Artrite Reumatoide/diagnóstico por imagem , Artrite Reumatoide/imunologia , Sistema Musculoesquelético/diagnóstico por imagem , Linfócitos T Reguladores , Células Th17 , Antirreumáticos/uso terapêutico , Artrite Reumatoide/tratamento farmacológico , Feminino , Humanos , Contagem de Linfócitos , Masculino , Ultrassonografia Doppler
6.
Clin Rheumatol ; 37(3): 855, 2018 03.
Artigo em Inglês | MEDLINE | ID: mdl-29098474

RESUMO

The original version of this article, unfortunately, contained an error. One of the author's name on this article was incorrectly spelled as "José Alexandre de Mendonça". The correct spelling is "José Alexandre Mendonça" and is now presented correctly in this article.

7.
Clin Rheumatol ; 36(4): 947-951, 2017 04.
Artigo em Inglês | MEDLINE | ID: mdl-27995383

RESUMO

Ultrasonography (US) is a useful tool for the evaluation of sinovial vascularization and proliferation in rheumatoid arthritis (RA). Accordingly, resistive index (RI) on spectral Doppler (sD) US provides a quantitative analysis of vascular inflammation, but its utility in the evaluation of RA activity has not been established. Our objective was to determine the association of RI with other US parameters of synovitis and with clinical disease activity in established RA. Patients with positive power Doppler (pD) were included in a prospective cross-sectional study. Disease activity and disability were evaluated using the Disease Activity Score in 28-joints (DAS28) and Health Assessment Questionnaire (HAQ), respectively. Gray scale (GS) synovitis, pD, and sD analyses were performed by one of two examiners in wrists and the second and third metacarpophalangeal and proximal interphalangeal joints. The 10-joint GS and 10-joint pD scores and mean RI were then calculated. Weighted kappa (WK) values were employed to assess interobserver reability, and correlations were tested using the Spearman coefficient. Ninety-five RA patients (median duration of disease of 7 years and mean DAS28 of 4.32 ± 1.66) were included. WK values in real-time US were 0.77 for synovitis, 0.87 for pD, and 0.68 for RI. There were no significant correlations of RI with 10-joint GS, 10-joint pD, DAS28, joint counts, or HAQ (P > 0.10 for all tests). Patients in remission had a mean RI similar to those with high disease activity (0.62 ± 0.10, n = 15 versus 0.63 ± 0.13, n = 34, respectively). The addition of the RI score did not seem to improve US performance in patients with established RA.


Assuntos
Artrite Reumatoide/diagnóstico por imagem , Articulações/diagnóstico por imagem , Sinovite/diagnóstico por imagem , Ultrassonografia Doppler , Idoso , Brasil , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Índice de Gravidade de Doença
8.
Clin Rheumatol ; 36(9): 1959-1968, 2017 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-28656478

RESUMO

Rheumatoid arthritis (RA) has been associated with cognitive impairment and peripheral production of autoantibodies. Autoantibodies against central nervous system (CNS) proteins and S100 calcium-binding ß (S100ß) were found increased in diseases characterized by cognitive impairment like Alzheimer disease and Neuropsychiatric Systemic Lupus Erythematosus (NPSLE). The aim of this study was to investigate the plasma levels of autoantibodies against myelin basic protein (anti-MBP), myelin oligodendrocyte glycoprotein (anti-MOG) and S100ß, and their relationships with cognitive performance in RA patients. Twenty patients with active rheumatoid arthritis and 19 age-, sex-, and schooling-matched healthy controls were recruited. Multiple dimensions of cognitive function were evaluated by structured clinical questionnaires. Autoantibodies and S100ß levels were assessed by ELISAs. Patients had significantly higher levels of anti-MBP IgG (17.51 ± 1.36 vs. 5.24 ± 0.53 ng/mL), anti-MOG IgG (5.68 ± 1.34 vs. 0.51 ± 0.49 ng/mL), and S100ß protein (2.24 ± 0.50 vs. 0.47 ± 0.06) than controls (all p < 0.0001). After adjusting for potential confounders, RA group presented worse cognitive performance involving the working memory and executive functions such as inhibition, flexibility, and mental control in parallel to higher autoantibodies and S100ß levels than healthy controls (all p < 0.001). Levels of anti-MBP were negatively associated with delayed verbal recall (DVR; r = -0.42, p = 0.005), Stroop Color-Word (r = -0.48, p = 0.004), and N-Back Total scores (r = -0.59, p < 0.0001) and positively with Trail Making Test B (TMB, r = 0.53, p = 0.001). Negative correlation was found between levels of anti-MOG and DVR (r = -0.64, p < 0.0001), N-Back Total scores (r = -0.35, p = 0.03), Stroop Color-Word (r = -0.51, p = 0.001), and positively with TMB (r = 0.50, p = 0.003). S100ß levels were associated with DVR (r = -0.51, p = 0.002), TMB (r = 0.46, p = 0.008), Stroop Color-Word (r = -0.67, p < 0.0001), and N-Back Total (r = -0.52, p = 0.003). RA is associated with impaired cognitive performance associated with higher levels of CNS-related autoantibodies and S100ß levels. Given the importance of myelin integrity to cognition, our data indicate that these autoantibodies may be harmful to proper cognitive function.


Assuntos
Artrite Reumatoide/imunologia , Artrite Reumatoide/psicologia , Autoanticorpos/sangue , Disfunção Cognitiva/sangue , Idoso , Brasil , Estudos de Casos e Controles , Estudos Transversais , Ensaio de Imunoadsorção Enzimática , Feminino , Humanos , Modelos Lineares , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Proteína Básica da Mielina/imunologia , Glicoproteína Mielina-Oligodendrócito/imunologia , Subunidade beta da Proteína Ligante de Cálcio S100/imunologia
9.
Adv Rheumatol ; 61: 47, 2021. tab
Artigo em Inglês | LILACS-Express | LILACS | ID: biblio-1284986

RESUMO

Abstract Purpose: To evaluate the quality of referrals for a first Rheumatology consultation at a tertiary care center in a southern Brazilian capital (Porto Alegre, RS), having as background findings from a similar survey performed in 2007/2008. Since then, our state has implemented referral protocols and a triage system with teleconsulting support exclusively for referrals from locations outside the capital, permitting a comparison between patients screened and not screened by the new system. Methods: Physicians of the Rheumatology Service at Hospital Nossa Senhora da Conceição prospectively collected information regarding first visits over a 6-month period (Oct 2017 to March 2018). We recorded demographic characteristics, diagnostic hypotheses, date of referral, and the municipality of origin (within the state of Rio Grande do Sul). We considered adequate referrals from primary health care when a systemic autoimmune inflammatory disease (SIRD) was suspected at first evaluation by the attending rheumatologist. Results: Three hundred fifty-seven patients/appointments were eligible for analysis (193 from the capital and 164 from small and medium towns). In 2007/2008, suspected SIRD occurred in 76/260 (29.2%) and 73/222 (32.9%) among patients from the capital and outside counties, respectively (P = 0.387). In 2017/2018, suspected SIRD occurred in 75/193 (38.9%) and 111/164 (67.7%) in patients from the capital and outside counties, respectively (difference: 28.8, 95% CI: 19.0 to 38.9, P < 0.001), indicating a marked improvement in referrals submitted to the new triage system. Conclusion: The quality of Rheumatology referrals in our state improved over the 10-year interval under study, particularly among patients from locations submitted to referral protocols and teleconsulting support.

10.
Adv Rheumatol ; 59: 26, 2019. tab, graf
Artigo em Inglês | LILACS | ID: biblio-1088601

RESUMO

Abstract Background: Imbalance and disfuntion in regulatory T-cells (Tregs) and IL-17 producer lymphocytes (Th17) have been implicated in the pathogenesis of rheumatoid arthritis (RA). Gray scale synovial proliferation (GS), power Doppler signal (pD) and bone erosions seen on high resolution muskuloskeletal ultrasound (MSUS) are hallmarks of destructive articular disease. Objective: To evaluate the association of peripheral Tregs and Th17 with MSUS findings in RA. Methods: RA patients (1987 ACR criteria) treated with disease-modifying antirheumatic drugs (DMARDs) were included. Lymphocytes were isolated and immunophenotyped by flow cytometry to investigate regulatory FoxP3+ T cells and IL-17+ cells. MSUS (MyLab 60, Esaote, Genova, Italy, linear probe 6-18 MHz) was performed on hand joints, and a 10-joint US score was calculated for each patient. Results: Data on lymphocytes subsets were avaiable for 90 patients. The majority of patients were Caucasian women with a median disease duration of 6 years (interquartile range: 2-13 years). Mean DAS28 was 4.28 (SD ± 1.64) and mean HAQ score was 1.11 (SD ± 0.83). There was no significant correlation of 10-joint GS score (rS = 0.122, 95% CI: - 0.124 to 0.336, P = 0.254) and 10-joint pD score (rS = 0.056, 95% CI: - 0.180 to 0.273, P = 0.602) with the mean percentage of peripheral Treg cells. Also, 10-joint GS score (rS = 0.083, 95% CI: - 0.125 to 0.302, P = 0.438) and 10-joint pD score 10 (rS = - 0.060, 95% CI: - 0.271 to 0.150, P = 0.575); did not correlate to Th17 profile. No association of bone erosions on MSUS with Treg and Th17 profiles (P = 0.831 and P = 0.632, respectively) was observed. Conclusion: In this first study addressing MSUS features and lymphocytes subtypes in established RA, data did not support an association of circulating Tregs and Th17 lymphocytes with inflammatory and structural damage findings on MSUS.


Assuntos
Humanos , Artrite Reumatoide/fisiopatologia , Linfócitos T Reguladores/imunologia , Células Th17/imunologia , Ultrassonografia/métodos
12.
J Bras Pneumol ; 32(1): 1-9, 2006.
Artigo em Inglês, Português | MEDLINE | ID: mdl-17273562

RESUMO

OBJECTIVE: To evaluate clinical and pulmonary function measurements taken in the first fifteen minutes of the assessment of acute asthma in the emergency room and used for prognostic purposes. METHODS: A prospective cohort study involving consecutive patients with acute asthma. Only patients who were between the ages of 12 and 55 and presented peak expiratory flow rates < or = 50% of predicted were included. Evaluations were performed upon admission, then again at 15 minutes and 4 hours after the initiation of treatment. Treatment included albuterol and ipratropium delivered by metered-dose inhaler with a spacer, together with 100 mg of intravenous hydrocortisone. Favorable outcomes were defined as peak expiratory flow > or = 50% of predicted after 4 hours of treatment, and unfavorable outcomes were defined as peak expiratory flow < 50% after 4 hours of treatment. RESULTS: Favorable outcomes were seen in 27 patients, and unfavorable outcomes were seen in 24 patients. In the multivariate analysis, peak expiratory flow as percentage of predicted was identified as the variable with the highest predictive value. A peak expiratory flow > or = 40% after 15 minutes of treatment showed significant power in predicting a favorable outcome (sensitivity = 0.74, specificity = 1.00, and positive predictive value = 1.00). A peak expiratory flow < 30% after 15 minutes of treatment was predictive of a poor outcome (sensitivity = 0.54, specificity = 0.93, and positive predictive value = 0.87). CONCLUSION: Our results suggest that measuring peak expiratory flow after 15 minutes of management in the emergency room is a useful tool for predicting outcomes in cases of acute asthma.


Assuntos
Asma/diagnóstico , Broncodilatadores/administração & dosagem , Pico do Fluxo Expiratório , Doença Aguda , Adolescente , Adulto , Albuterol/administração & dosagem , Asma/tratamento farmacológico , Asma/fisiopatologia , Criança , Emergências , Serviço Hospitalar de Emergência , Métodos Epidemiológicos , Feminino , Humanos , Hidrocortisona/administração & dosagem , Ipratrópio/administração & dosagem , Masculino , Pessoa de Meia-Idade , Prognóstico , Fatores de Tempo
13.
J. bras. pneumol ; 32(1): 1-9, jan.-fev. 2006. tab, graf
Artigo em Português | LILACS | ID: lil-430871

RESUMO

OBJETIVO: Estudar medidas clínicas e funcionais pulmonares utilizadas nos primeiros quinze minutos de manejo da asma aguda em um serviço de emergência, para predição prognóstica. MÉTODOS: Estudo de coorte, prospectivo, que incluiu pacientes consecutivos com asma aguda, com idades entre doze e 55 anos e medida do pico de fluxo expiratório menor ou igual a 50 por cento do previsto. Realizaram-se avaliações na admissão, aos quinze minutos e em quatro horas após o início do tratamento. O tratamento incluiu salbutamol e ipratrópio, administrados por aerossol dosimetrado com espaçador, e 100 mg de hidrocortisona intravenosa. O desfecho favorável foi definido pelo pico de fluxo expiratório maior ou igual a 50 por cento do previsto após a quarta hora de tratamento, e o desfecho desfavorável pelo pico de fluxo expiratório menor que 50 por cento do previsto. RESULTADOS: Tiveram desfecho favorável 27 pacientes e desfavorável 24. A análise multivariada identificou o pico de fluxo expiratório em porcentagem do previsto aos quinze minutos como variável mais preditiva. O pico de fluxo expiratório maior ou igual a 40 por cento aos quinze minutos mostrou significativa contribuição em predizer desfecho favorável (sensibilidade = 0,74, especificidade = 1,00 e valor preditivo positivo = 1,00). O pico de fluxo expiratório menor que 30 por cento aos quinze minutos contribuiu para predizer desfecho desfavorável (sensibilidade = 0,54, especificidade = 0,93 e valor preditivo positivo = 0,87). CONCLUSÃO: O estudo sugeriu que a medida do pico de fluxo expiratório aos quinze minutos do manejo da asma aguda em um serviço de emergência é um instrumento útil para avaliação prognóstica.


Assuntos
Humanos , Masculino , Feminino , Criança , Adolescente , Adulto , Pessoa de Meia-Idade , Albuterol/administração & dosagem , Asma/diagnóstico , Broncodilatadores/administração & dosagem , Hidrocortisona/administração & dosagem , Ipratrópio/administração & dosagem , Pico do Fluxo Expiratório , Doença Aguda , Asma/tratamento farmacológico , Estudos de Coortes , Serviço Hospitalar de Emergência , Análise Multivariada , Valor Preditivo dos Testes , Prognóstico , Estudos Prospectivos , Sensibilidade e Especificidade , Índice de Gravidade de Doença , Fatores de Tempo
14.
J. bras. pneumol ; 30(2): 94-101, mar.-abr. 2004. tab
Artigo em Português | LILACS | ID: lil-360375

RESUMO

INTRODUÇAO: Existe grande variabilidade de prática clínica no tratamento da asma aguda na sala de emergência, o que interfere na qualidade de atendimento. OBJETIVO: Avaliar o efeito da implantação de um protocolo assistencial de asma aguda no Serviço de Emergência do Hospital de Clínicas de Porto Alegre. MÉTODO: Estudo transversal, antes e após a implantação do protocolo assistencial de asma aguda no setor de adultos (idade > 12 anos) do referido serviço, avaliando o efeito das recomendações sobre a avaliação objetiva da gravidade, solicitações de exames, uso de terapêutica recomendada, uso de terapêutica não-recomendada e desfechos da crise. RESULTADOS: Na fase pré-implantação, foram estudados 108 pacientes e, na fase pós-implantação, 96 pacientes. Houve aumento na utilização da oximetria de pulso (de 8 por cento para 77 por cento, p < 0,001) e do pico de fluxo expiratório (de 5 por cento para 21 por cento, p < 0,001). Ocorreu aumento na utilização de recursos radiológicos (de 33 por cento para 66 por cento, p < 0,001) e de hemograma (de 11 por cento para 25 por cento, p = 0,016). Houve aumento no número de pacientes que receberam as três nebulizações preconizadas para a primeira hora de tratamento (de 22 por cento para 36 por cento, p=0,04). Embora a utilização geral de corticóide não se tenha modificado, houve aumento no uso de corticóide oral (de 8,3 por cento para 28 por cento, p < 0,001). Não houve alteração significativa na utilização de medidas terapêuticas não-preconizadas, no tempo de permanência na sala de emergência, nem nas taxas de internações e de altas. CONCLUSAO: A aplicação do protocolo assistencial de asma aguda na sala de emergência obteve efeito positivo, com maior utilização de medidas objetivas na avaliação da gravidade e de medidas terapêuticas recomendadas, porém não teve repercussão sobre tratamento e desfechos.


Assuntos
Humanos , Masculino , Feminino , Asma/terapia , Avaliação de Processos e Resultados em Cuidados de Saúde , Doença Aguda , Protocolos Clínicos , Estudos Transversais , Serviço Hospitalar de Emergência , Hospitais Universitários , Índice de Gravidade de Doença
15.
Artigo em Português | LILACS | ID: lil-360270

RESUMO

A administração de broncodilatadores através de dispositivos com aerossol dosimetrado (spray) ou através de nebulizador é equivalente em eficácia no tratamento da asma aguda. Alguns estudos sugerem que o spray tem um custo menor. A escolha do método de administração do aerossol depende de considerações sobre o custo. O objetivo deste estudo é comparar os custos entre nebulização intermitente com fluxo de ar comprimido versus spray acoplado a espaçador valvulado na administração do aerossol broncodilatador no tratamento da asma aguda no setor de adultos da sala de emergência. Comparamos os custos de diferentes agentes beta-adrenérgicos, com e sem brometo de ipratrópio, administrados por nebulização intermitente versus spray acoplado a espaçador valvulado no setor de adultos da emergência do Hospital de Clínicas de Porto Alegre. As comparações foram feitas para 1,6 e 12 horas de tratamento. Foram considerados os custos de todos os materiais utilizados. O spray com espaçador consistiu em um método mais barato de administrar o broncodilatador em comparação com a nebulização intermitente para diferentes agentes beta-agonistas, com e sem ipratrópio. A administração do broncodilatador por spray com espaçador valvulado foi mais econômica em comparação à administração por nebulização intermitente no tratamento da asma aguda na sala de emergência. O tratamento com spray/espaçador é progressivamente mais barato à medida que o tempo de permanência no setor de emergência aumenta.


Assuntos
Administração por Inalação , Asma , Análise Custo-Eficiência , Análise Custo-Benefício , Medicina de Emergência , Nebulizadores e Vaporizadores
16.
Rev. AMRIGS ; 48(3): 162-170, jul.-set. 2004. tab
Artigo em Português | LILACS | ID: biblio-876127

RESUMO

Introdução: A expectativa de vida dos pacientes com fibrose cística (FC) tem aumentado progressivamente nas últimas décadas. Objetivos: Determinar as características clínicas dos pacientes com FC em acompanhamento com a equipe de adultos do Hospital de Clínicas de Porto Alegre (HCPA) e determinar quais características estão associadas com a gravidade da obstrução do fluxo aéreo. Pacientes e métodos: Estudo transversal dos pacientes com FC (idade ≥ 16 anos) em acompanhamento na equipe de adultos do HCPA. Foram coletados dados demográficos, clínicos, nutricionais, função pulmonar, testes laboratoriais, achados radiológicos e microbiologia do escarro. Resultados: Trinta e nove pacientes (21 masculinos/18 femininos) consultavam com a equipe de adultos em 2003. A idade mediana foi 22,3 anos e a freqüência da raça branca, 97,4%. Análise genética foi realizada em 27 pacientes. Sete pacientes (25,9%) foram homozigotos para mutação delta F508 e 10 (37%) tinham apenas uma mutação delta F508. O escore clínico de Shwachman-Kulczycki mediano foi 80, o escore de Brasfield mediano foi 14 e o VEF1 médio foi 51,1% do previsto. Quatorze pacientes tinham distúrbio ventilatório obstrutivo (DVO) ausente ou leve, 11 tinham DVO moderado e 14 tinham DVO grave. A gravidade do DVO associou-se com a saturação de oxigênio, escore de dispnéia, escore clínico e escore radiológico. Conclusão: Descrevemos um grupo jovem de pacientes adultos com FC com doença pulmonar moderada a grave, mas com performance boa a excelente na sua atividade diária. A obstrução do fluxo aéreo associou-se à saturação de oxigênio, escore de dispnéia, escore clínico e escore radiológico (AU)


Introduction: Life expectancy of cystic fibrosis (CF) patients has been greatly increased over past decades. Objective: To determine the clinical characteristics of CF patients attending the adult team of Hospital de Clínicas de Porto Alegre (HCPA) and to determine which characteristics are associated with the severity of the air-flow obstruction. Patients and methods: The study comprised a cross-sectional survey of CF patients (age ≥16 years) attending to the adult team of HCPA. Data collected included patient demographics, clinical data, nutritional status, pulmonary function, laboratory tests, radiological findings and sputum microbiology. Results: Thirty-nine patients (21 male/18 female) were attending to the adult team during 2003. The median age was 22.3 years and the frequency of white race was 97.4%. Genetic analysis was performed in 27 patients. Seven patients (25.9%) were homozygous for delta F508 mutation and 10 (37%) had one delta F508 mutation. The median Shwachman-Kulczycki clinical score was 80, the median Brasfield score was 14 and the mean FEV1 was 51.1% predicted. Fourteen patients had normal or mild obstructive ventilatory disorder (OVD), 11 patients had moderate OVD and 14 patients had severe OVD. The severity of the OVD was associated with oxygen saturation, dyspnea score, clinical score and radiographic score. Conclusion: We describe a young group of CF adults with moderate to severe pulmonary disease, but with a good to excellent performance in their daily activity. The air-flow obstruction was associated with oxygen saturation, dyspnea score, clinical score and radiographic score (AU)


Assuntos
Humanos , Masculino , Feminino , Adolescente , Adulto , Pessoa de Meia-Idade , Fibrose Cística/epidemiologia , Brasil/epidemiologia , Estudos Transversais , Estudos Retrospectivos , Fibrose Cística/fisiopatologia , Pulmão/fisiopatologia
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