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; Sallum, Adriana M. E. Brentani, Alexandra V. M. Neto, Álvaro José S. Ihara, Amanda Santos, Andrea R. Canton, Ana Pinheiro M. Watanabe, Andreia Santos, Angélica C. dos Pastorino, Antonio C. Franco, Bernadette D. G. M. Caruzo, Bruna Ceneviva, Carina Martins, Carolina C. M. F. Prado, Danilo Abellan, Deipara M. Benatti, Fabiana B. Smaria, Fabiana Gonçalves, Fernanda T. Penteado, Fernando D. Castro, Gabriela S. F. de Gonçalves, Guilherme S. Roschel, Hamilton Disi, Ilana R. Marques, Isabela G. Castro, Inar A. Buscatti, Izabel M. Faiad, Jaline Z. Fiamoncini, Jarlei Rodrigues, Joaquim C. Carneiro, Jorge D. A. Paz, Jose A. Ferreira, Juliana C. Ferreira, Juliana C. O. Silva, Katia R. Bastos, Karina L. M. Kozu, Katia Cristofani, Lilian M. Souza, Lucas V. B. Campos, Lucia M. A. Silva Filho, Luiz Vicente R. F. Sapienza, Marcelo T. Lima, Marcos S. Garanito, Marlene P. Santos, Márcia F. A. Dorna, Mayra B. Aikawa, Nadia E. Litvinov, Nadia Sakita, Neusa K. Gaiolla, Paula V. V. Pasqualucci, Paula Toma, Ricardo K. Correa-Silva, Simone Sieczkowska, Sofia M. Imamura, Marta Forsait, Silvana Santos, Vera A. Zheng, Yingying

Clinics

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2021, Volume 76 elocation e3511

Abstract: En | Text: En | PDF: En

OBJECTIVES: To prospectively evaluate demographic, anthropometric and health-related quality of life (HRQoL) in pediatric patients with laboratory-confirmed coronavirus disease 2019 (COVID-19) METHODS: This was a longitudinal observational study of surviving pediatric post-COVID-19 patients (n=53) and pediatric subjects without laboratory-confirmed COVID-19 included as controls (n=52) was performed. RESULTS: The median duration between COVID-19 diagnosis (n=53) and follow-up was 4.4 months (0.8-10.7). Twenty-three of 53 (43%) patients reported at least one persistent symptom at the longitudinal follow-up visit and 12/53 (23%) had long COVID-19, with at least one symptom lasting for >12 weeks. The most frequently reported symptoms at the longitudinal follow-up visit were headache (19%), severe recurrent headache (9%), tiredness (9%), dyspnea (8%), and concentration difficulty (4%). At the longitudinal follow-up visit, the frequencies of anemia (11% versus 0%, p=0.030), lymphopenia (42% versus 18%, p=0.020), C-reactive protein level of >30 mg/L (35% versus 0%, p=0.0001), and D-dimer level of >1000 ng/mL (43% versus 6%, p=0.0004) significantly reduced compared with baseline values. Chest X-ray abnormalities (11% versus 2%, p=0.178) and cardiac alterations on echocardiogram (33% versus 22%, p=0.462) were similar at both visits. Comparison of characteristic data between patients with COVID-19 at the longitudinal follow-up visit and controls showed similar age (p=0.962), proportion of male sex (p=0.907), ethnicity (p=0.566), family minimum monthly wage (p=0.664), body mass index (p=0.601), and pediatric pre-existing chronic conditions (p=1.000). The Pediatric Quality of Live Inventory 4.0 scores, median physical score (69 [0-100] versus 81 [34-100], p=0.012), and school score (60 [15-100] versus 70 [15-95], p=0.028) were significantly lower in pediatric patients with COVID-19 at the longitudinal follow-up visit than in controls. CONCLUSIONS: Pediatric patients with COVID-19 showed a longitudinal impact on HRQoL parameters, particularly in physical/school domains, reinforcing the need for a prospective multidisciplinary approach for these patients. These data highlight the importance of closer monitoring of children and adolescents by the clinical team after COVID-19.

https://doi.org/10.6061/clinics/2021/e3511

Diffuse alveolar hemorrhage in childhoodonset systemic lupus erythematosus: a severe disease flare with serious outcome
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Blay, Gabriela

; Rodrigues, Joaquim C. Ferreira, Juliana C. O. Leal, Gabriela N. Gormezano, Natali W. Novak, Glaucia V. Pereira, Rosa M. R. Terreri, Maria T. Magalhães, Claudia S. Molinari, Beatriz C. Sakamoto, Ana P. Aikawa, Nadia E. Campos, Lucia M. A. Fernandes, Taciana A. P. Clemente, Gleice Peracchi, Octavio A. B. Bugni, Vanessa Marini, Roberto Sacchetti, Silvana B. Carvalho, Luciana M. Fraga, Melissa M. Castro, Tânia C. M. Ramos, Valéria C. Bonfá, Eloisa Silva, Clovis A.

Advances in Rheumatology

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2018, Volume 58 elocation 39

Abstract: En | Text: En | PDF: En

Abstract Objective: To evaluate prevalence, clinical manifestations, laboratory abnormalities and treatment in a multicenter cohort study including 847 childhood-onset systemic lupus erythematosus (cSLE) patients with and without diffuse alveolar hemorrhage (DAH), as well as concomitant parameters of severity. Methods: DAH was defined as the presence of at least three respiratory symptoms/signs associated with diffuse interstitial/alveolar infiltrates on chest x-ray or high-resolution computer tomography and sudden drop in hemoglobin levels. Statistical analysis was performed using Bonferroni correction (p < 0.0022). Results: DAH was observed in 19/847 (2.2%) cSLE patients. Cough/dyspnea/tachycardia/hypoxemia occurred in all cSLE patients with DAH. Concomitant parameters of severity observed were: mechanical ventilation in 14/19 (74%), hemoptysis 12/19 (63%), macrophage activation syndrome 2/19 (10%) and death 9/19 (47%). Further analysis of cSLE patients at DAH diagnosis compared to 76 cSLE control patients without DAH with same disease duration [3 (1–151) vs. 4 (1–151) months, p = 0.335], showed higher frequencies of constitutional involvement (74% vs. 10%, p < 0.0001), serositis (63% vs. 6%, p < 0.0001) and sepsis (53% vs. 9%, p < 0.0001) in the DAH group. The median of disease activity score(SLEDAI-2 K) was significantly higher in cSLE patients with DAH [18 (5–40) vs. 6 (0–44), p < 0.0001]. The frequencies of thrombocytopenia (53% vs. 12%, p < 0.0001), intravenous methylprednisolone (95% vs. 16%, p < 0.0001) and intravenous cyclophosphamide (47% vs. 8%, p < 0.0001) were also significantly higher in DAH patients. Conclusions: This was the first study to demonstrate that DAH, although not a disease activity score descriptor, occurred in the context of significant moderate/severe cSLE flare. Importantly, we identified that this condition was associated with serious disease flare complicated by sepsis with high mortality rate.

https://doi.org/10.1186/s42358-018-0038-4 467 downloads

Quality of life amongst adolescents and young adults with cystic fibrosis: correlations with clinical outcomes
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Gancz, Daniela W. Cunha, Maristela T. Leone, Claudio Rodrigues, Joaquim C. Adde, Fabíola V.

Clinics

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2018, Volume 73 elocation e427

Abstract: En | Text: En | PDF: En

OBJECTIVES: The current study sought to evaluate the quality of life of young patients with cystic fibrosis and correlate these results with the clinical parameters indicative of disease severity. METHODS: This cross-sectional study applied the validated Portuguese version of a cystic fibrosis specific quality of life questionnaire to clinically stable patients aged 14 to 21 years old. The correlations between the questionnaire domain scores and forced expiratory volume in one second (FEV1) values, the Shwachman-Kulczycki score, and body mass index were assessed, and correlations were considered as significant when p<0.05. RESULTS: A total of 31 patients (11 females; 16.4±2.3 years old) were evaluated, and the median scores on the questionnaire domains ranged from 66.7 to 100. A significant correlation was found between body mass index and the weight (r=0.43, p=0.016) and the eating questionnaire domains (r=0.44, p=0.013); between FEV1 and the physical (r=0.53, p=0.002) and treatment burden (r=0.41, p=0.023) domains; and between the Shwachman-Kulczycki score and the physical (r=0.39, p=0.03), health (r=0.41, p=0.023), and role (r=0.37, p=0.041) domains. A significant difference was found amongst patients with FEV1 values above or below 60% of the predicted value with regard to the role and health domains. No differences in the scores were found according to gender. CONCLUSIONS: The current cystic fibrosis specific quality of life questionnaire scores exhibited wide variability across all domains; however, they indicated a relatively satisfactory quality of life amongst the patients studied. Certain domains exhibited significant correlations with clinical parameters; thus, this instrument has consistent associations with clinical outcomes.

https://doi.org/10.6061/clinics/2017/e427 1043 downloads

Oxigenoterapia domiciliar prolongada em crianças e adolescentes: uma análise do uso clínico e de custos de um programa assistencial
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Munhoz, Andréa S. Adde, Fabiola V. Nakaie, Cleyde M. A. Doria Filho, Ulysses Silva Filho, Luiz V. R. F. Rodrigues, Joaquim C.

Jornal de Pediatria

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feb 2011, Volume 87 N. 1 Pages 13 - 18

Abstract: Pt En | Text: Pt En | PDF: Pt | PDF: En

OBJETIVOS: Descrever as características clínicas e laboratoriais dos pacientes em oxigenoterapia domiciliar prolongada acompanhados pelo programa de atendimento domiciliar do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, durante um período de 8 anos, e comparar os grupos com e sem hipertensão pulmonar secundária. Estimar o custo do programa utilizando concentradores versus cilindros de oxigênio arcados pela instituição. MÉTODOS: Estudo descritivo retrospectivo e de coorte dos pacientes em oxigenoterapia domiciliar prolongada, em seguimento no período de 2002 a 2009, na Unidade de Pneumologia do Instituto da Criança do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo. RESULTADOS: Foram estudados 165 pacientes, 53% do sexo masculino, com as medianas: idade de início da oxigenoterapia - 3,6 anos; tempo de oxigenoterapia - 7 anos; e tempo de sobrevida após início da oxigenoterapia - 3,4 anos. Os principais diagnósticos foram: fibrose cística (22%), displasia broncopulmonar (19%) e bronquiolite obliterante (15%). Dos 33 pacientes que realizaram espirometria, 70% apresentavam distúrbio ventilatório obstrutivo grave. O exame ecocardiográfico foi executado em 134 pacientes; 51% deles tinham hipertensão pulmonar secundária. Houve associação estatisticamente significante entre: presença de hipertensão pulmonar e necessidade de maiores fluxos de oxigênio (qui-quadrado, p = 0,011); e presença de hipertensão pulmonar e maior tempo de oxigenoterapia (Logrank, p = 0,0001). Não houve diferença estatisticamente significante entre tempo de sobrevida após início da oxigenoterapia e presença de hipertensão pulmonar. Os custos médios mensais do programa foram: US$ 7.392,93 para os concentradores e US$ 16.630,92 para cilindros. CONCLUSÕES: A oxigenoterapia domiciliar prolongada foi empregada em distintas doenças crônicas, predominantemente em lactentes e pré-escolares. Houve alta frequência de hipertensão pulmonar associada a maiores períodos de uso e fluxos de oxigênio, sem associação à sobrevida. A substituição dos cilindros por concentradores poderá reduzir custos significativamente.

OBJECTIVES: To describe the clinical and laboratory characteristics of patients on long-term home oxygen therapy followed up by the home care program of Hospital das Clínicas, School of Medicine, Universidade de São Paulo, during a period of 8 years; to compare groups with and without secondary pulmonary hypertension; and to estimate the cost of the program using oxygen concentrators versus oxygen cylinders provided by the hospital. METHODS: A descriptive, retrospective cohort study of patients on long-term home oxygen therapy followed up from 2002 to 2009 at the Unit of Pulmonology, Children's Institute, Hospital das Clínicas, School of Medicine, Universidade de São Paulo. RESULTS: We studied 165 patients, of whom 53% were male, with the following medians: age at the beginning of oxygen therapy - 3.6 years; duration of oxygen therapy - 7 years; and survival time after beginning of oxygen therapy - 3.4 years. The main diagnoses were: cystic fibrosis (22%), bronchopulmonary dysplasia (19%), and bronchiolitis obliterans (15%). Of the 33 patients who underwent spirometry, 70% had severe obstructive lung disease. Echocardiogram was performed in 134 patients; 51% of them had secondary pulmonary hypertension. There was a statistically significant association between pulmonary hypertension and need of higher oxygen flows (chi-square, p = 0.011), and between pulmonary hypertension and longer duration of oxygen therapy (Logrank, p = 0.0001). There was no statistically significant difference between survival time after the beginning of oxygen therapy and pulmonary hypertension. The average monthly costs of the program were US$ 7,392.93 for concentrators and US$ 16,630.92 for cylinders. CONCLUSIONS: Long-term home oxygen therapy was used to treat different chronic diseases, predominantly in infants and preschool children. There was a high frequency of pulmonary hypertension associated with longer periods of oxygen use and greater oxygen flow, without association with survival rate. The use of concentrators instead of cylinders may reduce costs significantly.

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Shwachman-Kulczycki score still useful to monitor cystic fibrosis severity
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Stollar, Fabíola Adde, Fabíola Villac Cunha, Maristela T. Leone, Claudio Rodrigues, Joaquim C.

Clinics

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2011, Volume 66 N. 6 Pages 979 - 983

Abstract: En | Text: En | PDF: En

INTRODUCTION: The Shwachman-Kulczycki score was the first scoring system used in cystic fibrosis to assess disease severity. Despite its subjectivity, it is still widely used. OBJECTIVE: To study correlations among forced expiratory volume in one second (FEV1), chest radiography, chest computed tomography, 6-minute walk test, and Shwachman-Kulczycki score in patients with cystic fibrosis and to test whether the Shwachman-Kulczycki score is still useful in monitoring the severity of the disease. METHODS: A cross-sectional prospective study was performed to analyze the correlations (Spearman). Patients with clinically stable cystic fibrosis, aged 3-21 years, were included. RESULTS: 43 patients, 19F/24M, mean age 10.5 + 4.7 years, with a median Shwachman-Kulczycki score of 70 were studied. The median Brasfield and Bhalla scores were 17 and 10, respectively. The mean Z score for the 6-minute walk test was -1.1 + 1.106 and the mean FEV1 was 59 + 26 (as percentage of predicted values). The following significant correlations versus the Shwachman-Kulczycki score were found: FEV1 (r = 0.76), 6-minute walk test (r = 0.71), chest radiography (r = 0.71) and chest computed tomography (r = -0.78). When patients were divided according to FEV1, a statistically significantly correlation with the Shwachman-Kulczycki score was found only in patients with FEV1 <70% (r = 0.67). CONCLUSIONS: The Shwachman-Kulczycki score remains an useful tool for monitoring the severity of cystic fibrosis, adequately reflecting the functional impairment and chest radiography and tomography changes, especially in patients with greater impairment of lung function. When assessing patients with mild lung disease its limitations should be considered and its usefulness in such patients should be evaluated in larger populations.

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Comparação entre o método clássico de Gibson e Cooke e o teste da condutividade no suor em pacientes com e sem fibrose cística
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Mattar, Ana C. V. Gomes, Eduardo N. Adde, Fabíola V. Leone, Claudio Rodrigues, Joaquim C.

Jornal de Pediatria

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apr 2010, Volume 86 N. 2 Pages 109 - 114

Abstract: Pt En | Text: Pt En | PDF: Pt | PDF: En

OBJETIVO: Comparar os valores de cloro no suor obtidos pelo teste quantitativo da iontoforese pela pilocarpina (teste clássico) com os valores de condutividade no suor obtidos pelo sistema de coleta por Macroduct® em pacientes com e sem fibrose cística (FC). O custo e tempo despendidos na execução de cada teste foram também analisados. MÉTODOS: O teste do suor pelas duas técnicas foi realizado simultaneamente, em pacientes com e sem FC. Os pontos de corte para a condutividade para excluir ou diagnosticar FC foram < 75 e > 90 mmol/L, respectivamente, e, para o teste clássico, cloro < 60 e > 60 mmol/L. RESULTADOS: Cinquenta e dois pacientes com FC (29 do sexo masculino e 23 do sexo feminino; de 1,5 a 18,2 anos) realizaram o teste do suor pelas duas técnicas, apresentando valores medianos de cloro e condutividade no suor de 114 e 122 mmol/L, respectivamente. Em todos eles, a condutividade foi > 95 mmol/L, o que conferiu ao teste 100% de sensibilidade (IC95% 93,1-100). Cinquenta pacientes sem FC (24 do sexo masculino e 26 do sexo feminino; de 0,5 a 12,5 anos) apresentaram valores medianos de cloro e condutividade no suor de 15,5 e 30 mmol/L, respectivamente. Em todos os casos, a condutividade foi < 70 mmol/L, o que conferiu ao teste 100% de especificidade (IC95% 92,9-100). O tempo despendido na execução dos testes foi significativamente menor com o teste da condutividade, e o seu custo também foi inferior. CONCLUSÕES: O teste da condutividade apresentou alta sensibilidade e especificidade, e houve boa correspondência entre os testes. O tempo de execução foi mais rápido e o custo inferior na aplicação do teste da condutividade em relação ao teste clássico.

OBJECTIVE: To compare sweat chloride values obtained by quantitative pilocarpine iontophoresis (classic test) with the sweat conductivity values obtained using Macroduct® collection system in patients with and without cystic fibrosis (CF). The cost and time spent to carry out each test were also analyzed. METHODS: The sweat test using both techniques was performed at the same time in patients with and without CF. Conductivity cutoff values to rule out or diagnose CF were < 75 and > 90 mmol/L, respectively, and for the classic test the chloride values were < 60 and > 60 mmol/L. RESULTS: Fifty-two patients with CF (29 males and 23 females; aged from 1.5 to 18.2 years) underwent the sweat test using both techniques, showing median sweat chloride and conductivity values of 114 and 122 mmol/L, respectively. In all of them, conductivity was > 95 mmol/L, which provided the test with 100% sensitivity (95%CI 93.1-100). Fifty patients without CF (24 males and 26 females; aged from 0.5 to 12.5 years) had median sweat chloride and conductivity values of 15.5 and 30 mmol/L, respectively. In all cases, conductivity was < 70 mmol/L, which provided the test with 100% specificity (95%CI 92.9-100). Time spent to perform the tests was significantly shorter for the conductivity test, and its cost was also lower. CONCLUSIONS: The conductivity test showed high sensitivity and specificity, and there was good correspondence between the tests. The time spent to carry out the conductivity test was shorter and the cost was lower in comparison with the classic test.

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Diagnóstico de ABPA em pacientes portadores de fibrose cística: utilidade clínica da pesquisa de IgE específica contra alérgenos recombinantes do Aspergillus fumigatus
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Almeida, Marina B. Bussamra, Maria Helena C. F. Rodrigues, Joaquim C.

Jornal de Pediatria

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jun 2006, Volume 82 N. 3 Pages 215 - 220

Abstract: Pt En | Text: Pt En | PDF: Pt | PDF: En

OBJETIVO: A aspergilose broncopulmonar alérgica (ABPA) é um fator complicador da fibrose cística que pode determinar uma combinação devastadora na evolução da doença pulmonar. A sobreposição de sinais e sintomas das duas enfermidades dificulta o diagnóstico, mesmo aplicando critérios padronizados. O objetivo deste trabalho foi identificar, em grupo de portadores de fibrose cística, os casos de ABPA através da detecção de IgE específica contra os alérgenos recombinantes do Aspergillus fumigatus e confrontar esse método com os critérios preconizados pela Cystic Fibrosis Foundation. MÉTODOS: Cinqüenta e quatro pacientes de 2 a 20 anos, com características que poderiam estar isoladamente presentes na ABPA, foram avaliados sistematicamente, incluindo: dados clínicos, tomografia computadorizada de tórax, teste cutâneo de hipersensibilidade imediata para A. fumigatus; dosagem de IgE sérica total, RAST para A. fumigatus, e IgE sérica específica para alérgenos recombinantes r Asp f1, f2, f3, f4 e f6. RESULTADOS: Foram elegíveis para o estudo 39 pacientes. Destes, 32 foram investigados. Houve sensibilização ao A. fumigatus em 34%. Ambos os métodos, o critério da Cystic Fibrosis Foundation e a pesquisa de IgE específica contra antígenos recombinantes, determinaram três casos de ABPA; entretanto, o diagnóstico foi concordante em apenas dois pacientes. CONCLUSÃO: A detecção de IgE específica contra antígenos recombinantes do A. fumigatus foi ferramenta útil para detecção precoce da sensibilização e diagnóstico de ABPA. No entanto, a confirmação diagnóstica não pôde ser desvinculada da condição clínica, e sua utilização para diagnóstico, detecção de recidivas e critério de cura ainda requer estudos longitudinais, envolvendo maior número de pacientes.

OBJECTIVE: Allergic bronchopulmonary aspergillosis (ABPA) is a complicating factor of cystic fibrosis which can result in a devastating combination as lung disease progresses. The overlap between the signs and symptoms of the two conditions makes diagnosis problematic, even if standardized criteria are used. The objective of this study was to identify, in a group of cystic fibrosis patients, cases of ABPA by assaying IgE specific to recombinant Aspergillus fumigatus antigens and to compare the method with the Cystic Fibrosis Foundation diagnostic criteria. METHODS: Fifty-four patients, aged 2 to 20 years, presenting characteristics that could occur with ABPA in isolation, were systematically assessed based on the following: clinical data, a chest CT scan, immediate hypersensitivity skin test for A. fumigatus; total serum IgE assay, RAST for A. fumigatus and serum IgE specific for the recombinant allergens Asp f1, f2, f3, f4 and f6. RESULTS: Thirty-nine patients were eligible for the study. Thirty-two of these were investigated. Sensitization to A. fumigatus was observed in 34%. Both the Cystic Fibrosis Foundation criteria and the recombinant antigen specific IgE assay defined three patients as suffering from ABPA; however, only two of these patients were diagnosed by both methods. CONCLUSIONS: The detection of A. fumigatus recombinant antigen specific IgE was a useful tool for the early detection of sensitization and diagnosis of ABPA. Nevertheless, diagnostic confirmation cannot be divorced from clinical findings, and before this method can be used for ABPA diagnosis, for detecting relapses and for defining cure criteria, longitudinal studies with larger numbers of patients are required.

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Escarro induzido em crianças e adolescentes com asma: segurança, aplicabilidade clínica e perfil de células inflamatórias em pacientes estáveis e durante exacerbação
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Palomino, Addy L. M. Bussamra, Maria Helena C. F. Saraiva-Romanholo, Beatriz M. Martins, Milton A. Nunes, Maria do Patrocínio T. Rodrigues, Joaquim C.

Jornal de Pediatria

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jun 2005, Volume 81 N. 3 Pages 216 - 224

Abstract: Pt En | Text: Pt En | PDF: Pt | PDF: En

OBJETIVOS: Verificar a segurança da indução de escarro em crianças e adolescentes asmáticos. Caracterizar a citologia do escarro durante a estabilidade clínica e exacerbação. Correlacionar a citologia do escarro com os eosinófilos no sangue periférico, IgE sérica e grau de obstrução brônquica. MÉTODOS: Foram recrutados 96 pacientes asmáticos, de 6 a 18 anos, para um estudo transversal. Foi realizada espirometria antes e, após, broncodilatador. A seguir, foi coletado o escarro, espontaneamente ou após indução com inalação de solução salina em concentrações crescentes. Foi coletada amostra de sangue para quantificar a IgE sérica e os eosinófilos. RESULTADOS: Obtiveram-se amostras adequadas de escarro para análise em 68 (70,8%) dos pacientes recrutados. Não houve broncoconstrição relevante durante a indução. A presença de maior número de eosinófilos no escarro de acordo com a maior gravidade clínica da asma não foi estatisticamente significante. Não houve correlação entre o grau de obstrução brônquica medido pelo VEF1 e as células inflamatórias do escarro. O mesmo foi observado em relação aos eosinófilos no sangue periférico e à IgE sérica. No grupo com exacerbação da asma, observou-se uma quantidade maior de neutrófilos (p < 0,05). CONCLUSÕES: A indução de escarro mostrou-se segura para obtenção de amostras clínicas em crianças e adolescentes, mesmo durante exacerbações, respeitando-se limitações clínicas e funcionais. O índice de sucesso da indução de 67% foi considerado satisfatório. A quantificação dos eosinófilos não discriminou a gravidade clínica e funcional da asma e mostrou-se independente do grau de obstrução das vias aéreas neste grupo de pacientes em tratamento com corticosteróides inalatórios. No escarro de pacientes em exacerbação da asma, houve um predomínio proporcional de neutrófilos.

OBJECTIVES: To determine the safety of sputum induction in asthmatic children and adolescents, to characterize sputum inflammatory cells while clinically stable and during exacerbation and to correlate sputum inflammatory cells with peripheral blood eosinophils, serum IgE and the degree of bronchial obstruction. METHODS: Ninety-six asthmatic patients aged 6 to 18 years were recruited for the present cross-sectional study. Spirometry was performed before and after administration of a bronchodilator. Sputum was collected spontaneously or after induction by the inhalation of saline solution at increasing concentrations. Blood samples were obtained for serum IgE and eosinophil quantification. RESULTS: Sputum samples adequate for analysis were obtained from 68 (70.8%) of the patients recruited. No relevant bronchoconstriction was observed during induction. The presence of a larger number of eosinophils in sputum did not correlate with more clinically severe asthma. No correlation was observed between the degree of bronchial obstruction, measured based on FEV1, and inflammatory cells in sputum, peripheral blood eosinophils or serum IgE. Larger numbers of neutrophils were observed in the asthma exacerbation group (p < 0.05). CONCLUSIONS: Sputum induction was found to be a safe procedure for obtaining clinical samples from children and adolescents even during exacerbations, allowing for clinical and functional limitations. The 67% induction success rate was considered satisfactory. In this group of patients, receiving inhaled corticosteroids, eosinophil quantification did not distinguish between the clinical and functional severity of asthma and was independent of the degree of airway obstruction. A proportional predominance of neutrophils was observed in the sputum of patients with asthma exacerbation.

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Seguimento nutricional de pacientes com fibrose cística: papel do aconselhamento nutricional
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Adde, Fabíola V. Rodrigues, Joaquim C. Cardoso, Ary L.

Jornal de Pediatria

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dec 2004, Volume 80 N. 6 Pages 475 - 482

Abstract: Pt En | Text: Pt En | PDF: Pt | PDF: En

OBJETIVO: Avaliar o estado nutricional de um grupo de pacientes com fibrose cística e analisar a repercussão do aconselhamento nutricional através de um estudo comparativo pré- e pós-intervenção. MÉTODOS: Todos os pacientes com fibrose cística em seguimento regular no ambulatório de pneumologia do Instituto da Criança no período de 1996-99 foram prospectivamente acompanhados durante 3,5 anos. Em quatro etapas (I = inicial, II = 7 meses, III = 13 meses, IV = 43 meses), foi realizada uma avaliação nutricional que consistia de medidas de peso, estatura/comprimento, circunferência do braço e pregas cutâneas, e cálculos de escores z para peso/idade, estatura/idade, peso/estatura, circunferência do braço e da prega cutânea tricipital, porcentagem de peso/estatura e porcentagem de gordura corpórea. Era feita verificação do uso das enzimas pancreáticas e do uso de suplementos nutricionais. Aconselhamento nutricional verbal e através de uma cartilha explicativa foi realizado em todos os pacientes. RESULTADOS: Foram avaliados 74 pacientes, 38 do sexo feminino e 36 do masculino, com idades de 6 meses a 18,4 anos. Na etapa inicial, os dados antropométricos revelaram: porcentagem de peso/estatura = 94±13, porcentagem de gordura corpórea = 15±7,1, escore z peso/idade = -1,13±1,3, escore z estatura/idade = -0,94±1,2, escore z peso/estatura = -0,69±,1, escore z circunferência do braço = -1,35±1,3, escore z prega cutânea tricipital = -0,74±0,9. A aderência ao uso de enzimas e suplementos melhorou durante o estudo. Houve um aumento significativo no escore z de peso/idade e da prega tricipital e na porcentagem de gordura corpórea durante todo o período de estudo. Dividindo-se os pacientes em três grupos etários, a melhora antropométrica só foi significativa nos menores de 5 anos. CONCLUSÕES: Desnutrição leve estava presente nesse grupo de pacientes com fibrose cística. O aconselhamento nutricional realizado possibilitou melhora na aderência ao uso de enzimas pancreáticas e de suplementos nutricionais e no estado nutricional, principalmente nos pacientes de baixa idade.

OBJECTIVE: To evaluate the nutritional status of a group of cystic fibrosis patients and establish the role of nutrition education addressed to them in a comparative study before and after intervention. METHODS: All cystic fibrosis patients in regular follow-up in the pulmonology clinic of Instituto da Criança during 1996-99 were prospectively monitored for 3.5 years. Measurements of weight, height, mid upper arm circumference, skinfolds and calculations of weight/age, height/age, weight/height, mid upper arm circumference and triceps z scores, percentage of ideal weight for height, percentage of body fat, check of the use of enzymes with meals and of the use of nutritional supplements were performed at four points in time: initial (I), 7 (II), 13 (III) and 43 (IV) months after the first evaluation. Nutritional counseling was given both verbally and in writing (booklet) to all patients. RESULTS: Seventy-four patients, 38F/36M, age range 6 months to 18.4 years were evaluated. At study entry the anthropometric data showed: percentage of ideal weight for height = 94±13, percentage of body fat = 15±7.1, z scores for weight/age = -1.13±1.3, z scores for height/age = -0.94±1.2, z scores for weight/height = -0.69±1.1, z scores for mid upper arm circumference = -1.35±1.3, triceps z scores = -0.74±0.9. Compliance with enzyme therapy and use of high-calorie supplements improved during the study period. There was a significant increase in weight/height and triceps z scores and percentage of body fat throughout the study period. After stratifying patients into three age groups the anthropometric improvement was only significant among children under 5 years of age. CONCLUSIONS: Mild malnutrition was present in this group of cystic fibrosis patients. The nutrition education led to an improvement in compliance with enzyme therapy, use of nutritional supplements and in nutritional status, mostly among the younger patients.

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10.

Use of selective medium for Burkholderia cepacia isolation in respiratory samples from cystic fibrosis patients
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SILVA FILHO, Luiz V.F. da VELLOSO, Luciana de F. BENTO, Christina N.O. GYTIN, Edelyn TATENO, Adriana F. LEVI, José E. RODRIGUES, Joaquim C. RAMOS, Sonia R.T.S.

Revista do Instituto de Medicina Tropical de São Paulo

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jul 2002, Volume 44 N. 4 Pages 203 - 208

Abstract: Pt En | Text: Pt En | PDF: Pt | PDF: En

A Burkholderia cepacia coloniza os pacientes portadores de fibrose cística (FC). Avaliamos o impacto do uso de um meio seletivo no isolamento de B. cepacia em amostras de secreção respiratória de pacientes portadores de FC. Durante um período de 6 meses, amostras de trato respiratório foram colhidas de 106 pacientes com FC e cultivadas em meios seletivos incluindo um meio para isolamento de B. cepacia. A identidade das cepas de B. cepacia foi confirmada através de PCR espécie específica e a determinação do genomovar ou subespécie realizada através de reações seqüenciais de PCR. Os resultados de isolamento de B. cepacia durante este período foram comparados com os dois anos precedentes, quando o processamento das amostras era idêntico, exceto pela utilização do meio seletivo para B. cepacia. B. cepacia foi isolada em 11/257 (4,2%) amostras usando o meio seletivo, e em apenas 6/1029 (0,58%) nos dois anos precedentes (p < 0,0001). A identidade destas 11 cepas foi confirmada e a determinação do genomovar obtida em 10/11. Estes resultados sugerem que o uso do meio seletivo aumenta a freqüência de isolamento de B. cepacia em amostras de trato respiratório de pacientes portadores de FC.

Burkholderia cepacia colonizes cystic fibrosis (CF) patients. We evaluated the impact of the use of a selective medium in the rate of B. cepacia recovery from respiratory samples of CF patients. During a 6-month period, respiratory samples were collected from 106 CF patients and cultivated on selective media including a B. cepacia selective medium. Confirmation of the identity of B. cepacia isolates was carried out by species specific PCR and determination of genomovar status performed by a sequential PCR approach. Results of B. cepacia isolation during this period were compared to the preceding two years, when the sample processing was identical except for the lack of the B. cepacia selective medium. B. cepacia was isolated in 11/257 (4.2%) of the samples using the selective medium, in contrast with the preceding two years, when it was isolated in 6/1029 samples (0.58%), p < 0.0001. Identity of all 11 isolates was confirmed by PCR and genomovar determination was accomplished in all but one isolate. These results suggest that the use of a selective medium increases recovery rate of B. cepacia from respiratory samples.

5684 downloads

11.

Traqueobroncomegalia em criança: relato de caso e revisão da literatura
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VARELLA, ELIANA H. MIHARA MIAMOTO, PATRÍCIA F. ADDE, FABÍOLA V. NAKAIE, CLEYDE M.A. RODRIGUES, JOAQUIM C.

Jornal de Pneumologia

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apr 2002, Volume 28 N. 2 Pages 103 - 106

Abstract: Pt En | Text: Pt En | PDF: Pt | PDF: En

Objetivo: Alertar para o diagnóstico de traqueobroncomegalia como um diagnóstico diferencial em pacientes pediátricos portadores de pneumopatias crônicas. Métodos: Revisão do prontuário de um paciente com traqueobroncomegalia em seguimento no ambulatório de Pneumologia do Instituto da Criança. Foi realizada uma revisão deste tema com base em pesquisa no banco de dados do sistema Medline, abrangendo as publicações dos últimos 10 anos. Resultados: Trata-se de um paciente de 15 anos, do sexo masculino, branco, que iniciou seguimento no ambulatório de Pneumologia do Instituto da Criança do HCFMUSP aos 10 anos de idade. Apresentava história de sibilância e infecções respiratórias de repetição desde os cinco meses de idade, sendo a princípio tratado como asmático, porém com pouca resposta. Durante a investigação foi realizada tomografia computadorizada de tórax, que revelou aumento dos diâmetros da traquéia e do brônquio fonte direito, sendo então confirmado o diagnóstico de traqueobroncomegalia. Conclusão: A traqueobroncomegalia é uma entidade rara em crianças. Deve ser investigada em pacientes com pneumopatias crônicas, sem diagnóstico etiológico definido. Até o momento não há tratamento específico.

Objective: To point out to tracheobronchomegaly as a differential diagnosis in children with chronic respiratory disorders. Methods: The patient's data were obtained from his chart review and bibliographical data were obtained from the Medline System. Results: This is a case report on a 15-year-old, white, male patient who had been followed up in the Pulmonology Unit of the Children' s Institute since he was 10 years old. His history included wheezing episodes and recurrent respiratory tract infections since he was 5 months old. The initial diagnosis was asthma, but, since the responded only partially to treatment, a chest computerized tomography was performed. It revealed an enlargement of the tracheal and right bronchial dimensions, confirming the diagnosis of tracheobronchomegaly. Conclusions: Tracheobronchomegaly is a rare disease in children. This entity should be thought in children with chronic respiratory diseases of unidentified etiology. To date, no definitive treament is avaliable.

5897 downloads

12.

É possível prever o número de nebulizações e o uso de corticosteróide intravenoso em crianças com crise asmática na unidade de emergência?
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Paro, Maria L. Z. Rodrigues, Joaquim C.

Jornal de Pediatria

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jun 2001, Volume 77 N. 3 Pages 219 - 226

Abstract: Pt En | Text: Pt En | PDF: Pt | PDF: En

OBJETIVO: identificar o número de nebulizações necessárias e a demanda de corticosteróide intravenoso em crianças em crise asmática, a partir de características clínicas e funcionais observadas no momento da admissão na unidade de emergência. MÉTODOS: Foram avaliadas prospectivamente 130 crianças em crise asmática, na faixa etária de 1 a 13 anos. No momento da admissão, as crianças foram avaliadas por meio de escore clínico e medidas de saturação arterial de oxigênio (por oximetria de pulso) e de pico de fluxo expiratório. Em seguida, receberam um tratamento padronizado e foram acompanhadas em relação à necessidade de nebulizações consecutivas com b2 agonista e costicosteróide intravenoso. Através de análise de regressão buscou-se uma correlação dos parâmetros avaliados com o número de nebulizações realizadas e o uso de corticosteróide. RESULTADOS: Oitenta e oito crianças (67,7%) receberam de uma até três nebulizações, e 42 crianças (32,3%) receberam seis nebulizações. Sessenta e oito crianças (52,3%) receberam corticosteróide. Os valores iniciais de escore clínico, saturação arterial de oxigênio e pico de fluxo expiratório mostraram uma correlação significativa com o número de nebulizações realizadas e com a necessidade do uso de corticosteróide. CONCLUSÃO: Com base em nossos resultados, é possível predizer e antecipar, no momento da admissão das crianças em crise asmática na Unidade de Emergência, a necessidade do uso de corticosteróide e de um maior número de nebulizações, o que pode alterar o prognóstico e o tempo de evolução da crise.

OBJECTIVE: to identify the number of nebulizations needed and the demand for intravenous corticosteroids in children with asthmatic attack, considering clinical and functional characteristics presented at the moment children were admitted to the emergency room. METHODS: we prospectively evaluated 130 children with asthmatic attack and from 1 to 13 years of age. At the moment children were admitted, they were evaluated according to clinical score, arterial oxygen saturation (pulse oximetry), and peak expiratory flow. Next, children received a standard treatment and were observed for the number of consecutive nebulizations required and the use of corticosteroid. Using regression analysis, we assessed the parameters evaluated for a correlation with the number of nebulizations and the use of intravenous corticosteroid. RESULTS: Eighty-eight children (67.7%) received one to three nebulizations, and 42 children (32.3%) received six nebulizations. Sixty-eight children (52.3%) received corticosteroids. The initial values for the clinical score, arterial oxygen saturation, and peak expiratory flow showed a significant correlation with the number of nebulizations required and the need for corticosteroids. CONCLUSIONS: According to our results, it is possible to predict and anticipate the need for corticosteroids and more nebulizations on admission of asthmatic children to the emergency room, allowing changes both in prognosis and in the time of evolution of the asthma attack.

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13.

Blood culture: specificity in childhood bacterial pneumonia
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Ejzenberg, Bernardo Rodrigues, Joaquim C. Vieira, Vera S. Dias Brandileone, Maria C. C. Baldacci, Evandro Roberto Okay, Yassuhiko

Revista do Instituto de Medicina Tropical de São Paulo

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dec 1995, Volume 37 N. 6 Pages 541 - 542

Abstract: En | Text: En | PDF: En

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14.

Dot-enzyme-linked immunosorbent assay (Dot-ELISA) for detection of pneumococcal polysaccharide antigens in pleural fluid effusion samples.: Comparison with bacterial culture, counterimmunoelectrophoresis and latex agglutination
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Requejo, Henry I.Z. Alkmin, Maria das Graças A. Almeida, Regina G. Casagrande, Silvana T. Cocozza, Ana Maria Lotufo, João Paulo B. Waetge, Aurora R.P. Rodrigues, Joaquim C.

Revista do Instituto de Medicina Tropical de São Paulo

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dec 1994, Volume 36 N. 6 Pages 531 - 537

Abstract: Pt En | Text: Pt En | PDF: Pt | PDF: En

Dot-ELISA para detecção de antígenos polissacarídicos de pneumococos foi padronizado em vista da necessidade de se ter um diagnóstico rápido e eficaz para pneumonia pneumocócica aguda. Um total de 480 amostras de líquido pleural sendo 442 de crianças com diagnóstico clínico e laboratorial de pneumonia bacteriana e 38 de pacientes com tuberculose, mais 20 amostras de soros sanguíneos de crianças sadias foram avaliadas no Dot-ELISA. As amostras foram tratadas previamente a 90°C por 10 min com EDTA 0,1 M de pH 7,5 e aplicadas sobre membrana de nitrocelulose. Para a detecção de antígeno pneumocócico foi empregado omniserum pneumocócico diluído a 1:200. Os resultados de Dot-ELISA avaliados em comparação com os resultados de cultura bacteriana, contra-imunoeletroforese e látex-aglutinação apresentaram índices de 0,940 para sensibilidade, 0,830 para especificidade e 0,760 para concordância. Omniserum pneumocócico mostrou ser um ótimo soro polivalente para a detecção de antígenos pneumocócicos em Dot-ELISA e, essa técnica provou ser uma alternativa prática e eficaz para o diagnóstico de pneumonias pneumocócicas.

A dot-enzyme-linked immunosorbent assay (Dot-ELISA) for pneumococcal antigen detection was standardized in view of the need for a rapid and accurate immunodiagnosis of acute pneumococcal pneumonia. A total of 442 pleural fluid effusion samples (PFES) from children with clinical and laboratory diagnoses of acute bacterial pneumonia, plus 38 control PFES from tuberculosis patients and 20 negative control serum samples from healthy children were evaluated by Dot-ELISA. The samples were previously treated with 0.1 M EDTA pH 7.5 at 90°C for 10 min and dotted on nitrocellulose membrane. Pneumococcal omniserum diluted at 1:200 was employed in this assay for antigen detection. When compared with standard bacterial culture, counterimmunoelectrophoresis and latex agglutination techniques, the Dot-ELISA results showed relative indices of 0.940 to sensitivity, 0.830 to specificity and 0.760 to agreement. Pneumococcal omniserum proved to be an optimal polyvalent antiserum for the detection of pneumococcal antigen by Dot-ELISA. Dot-ELISA proved to be a practical alternative technique for the diagnosis of pneumococcal pneumonia.

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