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au:Marson, Fernando Augusto de Lima

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Combined multi-channel intraluminal impedance measurement and pHmetry in the detection of gastroesophageal reflux disease in children with cystic fibrosis multichannel multi channel
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Gonçalves, Emília da Silva

; Ribeiro, José Dirceu

; Marson, Fernando Augusto Lima

; Montes, Ciro Garcia

; Ribeiro, Antônio Fernando

; Mainz, Jochen Georg

; Toro, Adyléia Aparecida Dalbo Contrera

; Zamariola, Juliana Helena

; Borgli, Daniela Souza Paiva

; Lomazi, Elizete Aparecida

; Servidoni, Maria de Fátima Corrêa Pimenta

Jornal de Pediatria

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jun 2023, Volume 99 N. 3 Pages 269 - 277

Abstract: En | Text: En | PDF: En

Abstract Objective To assess the prevalence of GERD exclusively by means of multichannel intraluminal impedanciometry associated with pH monitoring (MIIpH) and compare it with respiratory symptoms in children with CF. To compare MIIpH with pHmetry alone to perform GERD diagnosis. Methods An analytical cross-sectional study was conducted with children diagnosed with CF who underwent MIIpH. Clinical and laboratory markers, including respiratory and digestive symptoms, were used for comparative analyses. High-resolution chest computed tomography was performed on patients with symptoms of chronic lung disease. Severity was classified according to the Bhalla score. Results A total of 29 children < 10 yo (18 girls) were evaluated; 19 of whom with physiological GER and 10 with GERD. Of the children with GERD, seven had predominantly acid GER, two acid+non-acid GER, and one non-acid GER. Three patients had GERD diagnosed only by MIIpH. Bhalla scores ranged from seven to 17.75 with no significant relationship with GERD. The number of pulmonary exacerbations was associated with a decrease in esophageal clearance regardless of the position in pHmetry and MIIpH. Conclusions The prevalence of GERD was 34% in children with CF. There was no association between respiratory disease severity and GER types. MIIpH detected 30% more patients with GERD than pHmetry. (MIIpH diagnosis crosssectional cross sectional markers analyses Highresolution High resolution score 2 1 18 (1 girls evaluated acid+nonacid acidnonacid acid+non non nonacid 1775 17 75 17.7 34 types 30 ( acidnon 177 7 17. 3

https://doi.org/10.1016/j.jped.2022.11.007

Cross-sectional evaluation of the saccharin transit time test for primary ciliary dyskinesia: did we discard this tool too soon? Crosssectional Cross sectional dyskinesia soon
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Toro, Mariana Dalbo Contrera

; Ortiz, Erica

; Marson, Fernando Augusto Lima

; Pinheiro, Laíza Mohana

; Toro, Adyléia Aparecida Dalbo Contrera

; Ribeiro, José Dirceu

; Sakano, Eulália

Sao Paulo Medical Journal

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2023, Volume 141 N. 6 elocation e2022508

Abstract: En | Text: En | PDF: En

ABSTRACT BACKGROUND: Primary ciliary dyskinesia (PCD) is a rare and heterogeneous disease that is difficult to diagnose and requires complex and expensive diagnostic tools. The saccharin transit time test is a simple and inexpensive tool that may assist in screening patients with PCD. OBJECTIVES: This study aimed to compare changes in the electron microscopy findings with clinical variables and saccharin tests in individuals diagnosed with clinical PCD (cPCD) and a control group. DESIGN AND SETTING: An observational cross-sectional study was conducted in an otorhinolaryngology outpatient clinic from August 2012 to April 2021. METHOD: Patients with cPCD underwent clinical screening questionnaires, nasal endoscopy, the saccharin transit time test, and nasal biopsy for transmission electron microscopy. RESULTS: Thirty-four patients with cPCD were evaluated. The most prevalent clinical comorbidities in the cPCD group were recurrent pneumonia, bronchiectasis, and chronic rhinosinusitis. Electron microscopy confirmed the clinical diagnosis of PCD in 16 of the 34 (47.1%) patients. CONCLUSION: The saccharin test could assist in screening patients with PCD due to its association with clinical alterations related to PCD. BACKGROUND (PCD tools OBJECTIVES (cPCD SETTING crosssectional cross sectional 201 2021 METHOD questionnaires endoscopy RESULTS Thirtyfour Thirty four evaluated pneumonia bronchiectasis rhinosinusitis 1 3 47.1% 471 47 (47.1% CONCLUSION 20 202 47.1 4 (47.1 2 47. (47. (47 (4 (

https://doi.org/10.1590/1516-3180.2022.0508.r2.13032023

Correlation and agreement of olfactory perception assessed by the Connecticut Chemosensory Clinical Research Center olfactory test and the Brief-Smell Identification Test
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Aniteli, Marcello Bailarini

; Marson, Fernando Augusto Lima

; Cunha, Fernanda Rodrigues

; Sakano, Eulália

Brazilian Journal of Otorhinolaryngology

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nov 2022, Volume 88 N. 6 Pages 858 - 866

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

Resumo Introdução: Avaliar a percepção olfativa em distúrbios olfativos é de extrema importância para a correta conduta terapêutica. No entanto, apenas o teste University of Pennsylvania smell identification test e o teste sniffin’sticks são validados no Brasil. Objetivos: Avaliar a correlação e concordância entre os testes Connecticut chemosensory clinical research center e do brief-smell identification test e University of Pennsylvania smell identification test em participantes saudáveis e em participantes com distúrbios olfativos de acordo com os resultados e aspectos técnicos dos dois testes. Método: Cinquenta participantes sem queixas olfativas e 50 participantes com distúrbios olfativos submetidos ao teste Connecticut chemosensory clinical research center e ao brief-smell identification test foram incluídos. Os seguintes testes foram usados para análise estatística: teste U de Mann-Whitney, correlação de Spearman, coeficiente de correlação intraclasse e plotagem de Bland-Altman. Um erro alfa (nível de significância) de 0,05 foi considerado nas análises estatísticas feitas no estudo. Resultados: Ambos os testes foram eficazes para diferenciar os grupos sem a presença de valores sobrepostos para os marcadores medidos. Além disso, houve uma forte correlação entre a correlação de Spearman e o coeficiente de correlação intraclasse entre os testes e para as duas narinas. Entretanto, as correlações foram menores quando os grupos foram avaliados individualmente. O gráfico de Bland-Altman não mostrou viés quando todos os participantes foram avaliados simultaneamente. Conclusões: Os testes para avaliar a percepção olfativa apresentaram um elevado nível de concordância. Em nossa amostra, podemos inferir que o Connecticut chemosensory clinical research center é equivalente ao brief-smell identification test e pode ser usado no diagnóstico de rotina de pacientes com queixas de distúrbios olfativos, considerando a vantagem de seu baixo custo.

Abstract Introduction: Assessing olfactory perception in olfactory disorders is of utmost importance in therapy management. However, the University of Pennsylvania Smell Identification Test and the Sniffin’ Sticks are the only tests validated in Brazil. Objectives: To evaluate the correlation and agreement between the Chemosensory Clinical Research Center olfactory test and the Brief-Smell Identification Test – University of Pennsylvania Smell Identification Test – in healthy participants and in participants with olfactory disorders based on the results and technical aspects of both tests. Methods: Fifty participants without olfactory complaints and 50 participants with olfactory disorders who underwent the Chemosensory Clinical Research Center olfactory test and the Brief-Smell Identification Test were included. The following tests were used for statistical analysis: Mann-Whitney U test, Spearman’s correlation, intraclass correlation coefficient and Bland-Altman plot. An alpha error (significance level) of 0.05 was considered in the statistical analysis. Results: Both tests were effective in distinguishing the groups without the presence of overlapping values for the measured markers. Additionally, there was a strong correlation between Spearman’s correlation and intraclass correlation coefficient between the tests and for both nostrils. However, the correlations were lower when the groups were individually evaluated. The Bland-Altman plot showed no bias when all participants were simultaneously evaluated. Conclusions: The tests to assess olfactory perception presented a high level of agreement. In our sample, we could infer that the Connecticut Chemosensory Clinical Research Center olfactory test is similar to the Brief-Smell Identification Test and can be used in the routine diagnosis of patients with complaints of olfactory disorders, considering the advantage of its low cost.

https://doi.org/10.1016/j.bjorl.2020.11.013

Rhinoviruses as critical agents in severe bronchiolitis in infants
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Bastos, Juliana Cristina Santiago

; Simas, Paulo Vitor Marques

; Caserta, Leonardo Cardia

; Bragunde, Alfonso Eduardo Alvarez

; Marson, Fernando Augusto de Lima

; Martini, Matheus Cavalheiro

; Padilla, Marina Aiello

; Ribeiro, José Dirceu

; Santos, Márcia Mercês Aparecida Bianchi dos

; Arns, Clarice Weis

Jornal de Pediatria

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jul 2022, Volume 98 N. 4 Pages 362 - 368

Abstract: En | Text: En | PDF: En

Abstract Objectives: To detect RSV or other thirteen respiratory viruses as possible causer agent of bronchiolitis in infants. Method: This is an epidemiological analytical study, conducted using a nasopharyngeal aspirate of 173 hospitalized children younger than two years old with severe bronchiolitis in three hospitals in the Campinas Metropolitan Region (CMR) during 2013-14. The data was statically evaluated by Pearson’s chi-squared test with statistical significance of 0.05 and 95% confidence level. Results: As expected, the most prevalent viruses detected were RSV A and B in 47% and 16% of the samples, respectively. However, almost a third of severe bronchiolitis cases there were no detection of RSV, and the viruses more commonly detected were rhinoviruses, which were identified in almost a quarter of all positive samples for at least a viral agent. Conclusions: Although nothing could be concluded from the disease severity and clinicalepidemiological data, the present study’s results indicate that severe bronchiolitis is not always related to RSV infections in children younger than two years old, and the rhinoviruses were more prevalent in these cases. These findings reinforce the need to carry out a

https://doi.org/10.1016/j.jped.2021.11.006

Can continuous glucose monitoring predict cystic fibrosis-related diabetes and worse clinical outcome?
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Zorron, Mariana

; Marson, Fernando Augusto Lima

; Morcillo, André Moreno

; Gonçalves, Aline Cristina

; El Beck, Mayra de Souza

; Ribeiro, José Dirceu

; Ribeiro, Antonio Fernando

Jornal Brasileiro de Pneumologia

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2022, Volume 48 N. 2 elocation e20210307

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

RESUMO Objetivo: Verificar se leituras de continuous glucose monitoring (CGM, monitoramento contínuo da glicose) anormais (hipoglicemia/hiperglicemia) podem prever o aparecimento de diabetes relacionado à fibrose cística (DRFC) e/ou comprometimento clínico (declínio do IMC e/ou do VEF1) em pacientes pediátricos com fibrose cística (FC). Métodos: Estudo de coorte longitudinal prospectivo envolvendo pacientes com FC sem diabetes no início do estudo. O tempo médio de acompanhamento foi de 3,1 anos. Os pacientes foram submetidos a CGM de três dias, teste oral de tolerância à glicose (TOTG) e medida de VEF1 e IMC no início do estudo. TOTG, VEF1 e IMC foram reavaliados ao final do acompanhamento. Resultados: Trinta e nove pacientes com FC (10-19 anos de idade) apresentaram leituras de CGM válidas no início do estudo, e 34 completaram o acompanhamento (média = 3,1 ± 0,5 anos). Nenhuma das variáveis estudadas previu evolução para DRFC ou apresentou associação com eventos hipoglicêmicos. O CGM conseguiu detectar anormalidades glicêmicas não reveladas pelo TOTG. Pacientes com níveis de glicose ≥ 140 mg/dL no CGM, comparados àqueles com níveis menores, apresentaram valores de IMC e escores z de IMC menores no início do estudo - 17,30 ± 3,91 kg/m2 vs. 19,42 ± 2,07 kg/m2; p = 0,043; e −1,55 ± 1,68 vs. −0,17 ± 0,88; p = 0,02, respectivamente - e no final do acompanhamento - 17,88 ± 3,63 kg/m2 vs. 19,95 ± 2,56 kg/m2; p = 0,039; e −1,65 ± 1,55 vs. −0,42 ± 1,08; p = 0,039. Na comparação dos pacientes com e sem DRFC, os primeiros apresentaram pior VEF1 (em % do previsto) - 22,67 ± 5,03 vs. 59,58 ± 28,92; p = 0,041 - e maior declínio do VEF1 (−36,00 ± 23,52 vs. −8,13 ± 17,18; p = 0,041) no final do acompanhamento. Conclusões: O CGM foi capaz de identificar anormalidades glicêmicas não detectadas pelo TOTG que se relacionaram com reduções precoces do IMC. O CGM foi ineficaz na previsão do aparecimento de diabetes nesta população com FC. Diferentes critérios diagnósticos para diabetes podem ser necessários para indivíduos com FC.

ABSTRACT Objective: To determine whether abnormal continuous glucose monitoring (CGM) readings (hypoglycemia/hyperglycemia) can predict the onset of cystic fibrosis-related diabetes (CFRD) and/or clinical impairment (decline in BMI and/or FEV1) in pediatric patients with cystic fibrosis (CF). Methods: This was a longitudinal prospective cohort study involving CF patients without diabetes at baseline. The mean follow-up period was 3.1 years. The patients underwent 3-day CGM, performed oral glucose tolerance test (OGTT), and had FEV1 and BMI determined at baseline. OGTT, FEV1, and BMI were reassessed at the end of the follow-up period. Results: Thirty-nine CF patients (10-19 years of age) had valid CGM readings at baseline, and 34 completed the follow-up period (mean = 3.1 ± 0.5 years). None of the study variables predicted progression to CFRD or were associated with hypoglycemic events. CGM could detect glucose abnormalities not revealed by OGTT. Patients with glucose levels ≥ 140 mg/dL, as compared with those with lower levels, on CGM showed lower BMI values and z-scores at baseline-17.30 ± 3.91 kg/m2 vs. 19.42 ± 2.07 kg/m2; p = 0.043; and −1.55 ± 1.68 vs. −0.17 ± 0.88; p = 0.02, respectively-and at the end of follow-up-17.88 ± 3.63 kg/m2 vs. 19.95 ± 2.56 kg/m2; p = 0.039; and −1.65 ± 1.55 vs. −0.42 ± 1.08; p = 0.039. When comparing patients with and without CFRD, the former were found to have worse FEV1 (in % of predicted)-22.67 ± 5.03 vs. 59.58 ± 28.92; p = 0.041-and a greater decline in FEV1 (−36.00 ± 23.52 vs. −8.13 ± 17.18; p = 0.041) at the end of follow-up. Conclusions: CGM was able to identify glucose abnormalities not detected by OGTT that were related to early-stage decreases in BMI. CGM was ineffective in predicting the onset of diabetes in this CF population. Different diagnostic criteria for diabetes may be required for individuals with CF.

https://doi.org/10.36416/1806-3756/e20210307

GASTROESOPHAGEAL REFLUX DISEASE IN INFANTS WHO PRESENTED BRIEF RESOLVED UNEXPLAINED EVENT (BRUE)
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BELLOMO-BRANDÃO, Maria Angela

; STRANGUETTI, Fernanda Maso

; LOPES, Iara Ferreira

; PEIXOTO, Andressa Oliveira

; MARSON, Fernando Augusto Lima

; LOMAZI, Elizete Aparecida

Arquivos de Gastroenterologia

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oct 2021, Volume 58 N. 4 Pages 424 - 428

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

RESUMO CONTEXTO: O termo Eventos Resolvidos Breves Não Explicados (Brief Resolved Unexplained Event - BRUE) é uma descrição do evento agudo que ocorre em lactentes menores de 1 ano de idade que inclui pelo menos uma das seguintes características: cianose ou palidez; respiração ausente, diminuída ou irregular, alteração acentuada no tônus ou nível alterado de responsividade. É necessário um procedimento investigativo para identificar o fenômeno desencadeante naqueles que apresentam alto risco de complicações. A pHmetria esofágica prolongada tem sido usada como uma ferramenta na pesquisa de doença do refluxo gastroesofágico (DRGE) como uma das etiologias subjacentes. OBJETIVO: Este estudo tem como objetivo verificar a frequência da DRGE em lactentes de até 1 ano de idade, quando a pHmetria foi realizada para investigação da BRUE de alto risco, e analisar se alguma característica clínica ou sintoma particular relatado pelos cuidadores durante a BRUE poderia estar correlacionado a DRGE. MÉTODOS: Foi realizado um estudo observacional, transversal, cujos dados foram coletados retrospectivamente de pacientes menores de 1 ano de idade, que realizaram pHmetria em hospital terciário para investigação de BRUE de alto risco de outubro de 2008 e janeiro de 2018. Para a análise dos prontuários, um protocolo de coleta de dados incluiu: sexo, idade no primeiro episódio de BRUE de alto risco, idade no momento da pHmetria, idade gestacional, tipo de parto (normal ou cesárea), peso ao nascer e sintomas associados a alto risco-BRUE relatado por cuidadores. A relação entre as variáveis foi avaliada por meio do teste exato de Fisher, qui-quadrado e teste de Mann-Whitney. O nível de significância foi estabelecido em 0,05. RESULTADOS: Foram incluídos 54 lactentes (pré-termo 25, termo 29), 62,9% do sexo masculino, idade mediana na BRUE de alto risco foi de 36 dias. De acordo com o relatório do cuidador, 53,7% dos episódios de BRUE de alto risco ocorreram durante ou logo após a alimentação. Resultados da pHmetria: nove resultados da pHmetria foram considerados inconclusivos, refluxo fisiológico (n=30) e DRGE (n=15). A frequência de DRGE diagnosticada por pHmetria foi de 33%. A DRGE não foi estatisticamente relacionada ao sexo (P=0,757), idade no primeiro episódio de BRUE de alto risco (P=0,96), idade no momento da pHmetria (P=0,72) prematuridade (P=0,321) ou tipo de parto (P=0,738). A DRGE foi estatisticamente relacionada ao baixo peso ao nascer (P=0,023). Não houve associação entre os sintomas relatados pelos cuidadores durante BRUE de alto risco e o diagnóstico de DRGE. CONCLUSÃO: A DRGE diagnosticada pela pHmetria foi encontrada em um terço dos lactentes que vivenciaram BRUE de alto risco, mostrando a importância da investigação adequada. Em metade das crianças, o evento ocorreu durante ou logo após a alimentação. Além do baixo peso ao nascer, não foi possível selecionar outros dados da história clínica que sugiram que esses pacientes terão maior probabilidade de apresentar DRGE.

ABSTRACT BACKGROUND: The term brief resolved unexplained events (BRUE) is a description of the acute event occurring in infants less than 1-year-old that includes at least one of the following characteristics: cyanosis or pallor; absent, decreased, or irregular breathing; marked change in tone or altered level of responsiveness. An investigative proceeding is required to identify the triggering phenomenon in those who are at high risk of complications. Prolonged esophageal pHmetry has been used as a tool in searching for gastroesophageal reflux disease (GERD) as one of the underlying etiologies. OBJECTIVE: The study aims to verify the frequency of GERD in infants up to 1-year-old, when pHmetry has been performed for investigating high-risk BRUE (HR-BRUE) and to analyze if clinical characteristics or any particular symptom related by caregivers during BRUE could be correlated to GERD. METHODS: It was performed a cross-sectional study. The data was collected retrospectively of patients less than 1-year-old, who had performed pHmetry in a tertiary hospital for investigating HR-BRUE between October 2008 and January 2018. For the analysis of medical records, a data collection protocol included: gender, age at the first HR-BRUE episode, age at the time of the pHmetry, gestational age, type of delivery (normal or caesarean) and birth weight and symptoms associated to HR-BRUE related by caregivers. Relation between variables were assessed using Fisher’s exact test and Mann-Whitney test. The significance level was set at 0.05. RESULTS: A total of 54 infants were included (preterm 25, term 29), 62.9% males, median age at the HR-BRUE was 36 days, 53.7% HR-BRUE episodes had occurred during or right after feeding. According to pHmetry results: nine pHmetry results were considered inconclusive, physiological reflux (n=30) and GERD (n=15). The frequency of GERD diagnosed by pHmetry was 33%. GERD was not statistically related to gender (P-value=0.757), age at first HR-BRUE episode (P-value=0.960), age at the time of the pHmetry (P-value=0.720), prematurity (P-value=0.120) or type of delivery (P-value=0.738). GERD was statistically related to low birth weight (P-value=0.023). There was no association between symptoms reported by caregivers during HR-BRUE and GERD. CONCLUSION: GERD diagnosed by the pHmetry was found in one third of infants that experiencing a HR-BRUE, showing the importance of properly investigation. In half of infants BRUE occurred during or right after feeding. Besides low birth weight, it was not possible to select other data from the clinical history that suggest that these patients would be more likely to have GERD.

https://doi.org/10.1590/s0004-2803.202100000-77 1 downloads

Volumetric capnography versus spirometry for the evaluation of pulmonary function in cystic fibrosis and allergic asthma,
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Almeida-Junior, Armando

; Marson, Fernando Augusto Lima

; Almeida, Celize Cruz Bresciani

; Ribeiro, Maria Ângela Gonçalves Oliveira

; Paschoal, Ilma Aparecida

; Moreira, Marcos Mello

; Ribeiro, José Dirceu

Jornal de Pediatria

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apr 2020, Volume 96 N. 2 Pages 255 - 264

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

Resumo Objetivo Comparar os valores dos marcadores para capnografia volumétrica e espirometria e sua capacidade de classificar crianças e adolescentes com asma, fibrose cística (FC) e controles saudáveis. Métodos Foi realizado um estudo transversal que incluiu 103 pacientes com asma alérgica persistente controlada, 53 com FC e um grupo controle saudável com 40 voluntários (6 a 15 anos), de ambos os sexos. Os indivíduos foram submetidos a capnografia volumétrica e espirometria. Resultados O slope da fase III (SIII), SIII padronizada pelo volume tidal exalado (SIII/VT) e o índice capnográfico (SIII/SII) × 100 (KPIv) foram diferentes entre os três grupos avaliados, com maiores valores para o grupo FC. A relação entre o volume expiratório forçado no primeiro segundo e a capacidade vital forçada (VEF1/CVF) foi o único marcador de espirometria com diferenças nos três grupos. Nos indivíduos com espirometria normal, o KPIv e VEF1/CVF foram diferentes entre os três grupos. A curva ROC diferenciou os indivíduos com asma ou FC daqueles do grupo controle, ambos através da capnografia volumétrica (melhor para identificar a FC em relação aos controles pelo KPIv) e por meio da espirometria (melhor para identificar a asma em relação aos controles). O KPIv foi o melhor parâmetro para distinguir a asma da FC, mesmo em indivíduos com espirometria normal. Conclusão A capnografia volumétrica e a espirometria identificaram diferentes alterações de função pulmonar na asma, na FC e nos controles saudáveis, permitiram que os três grupos fossem diferenciados.

Abstract Objective: To compare the values of the markers for volumetric capnography and spirometry and their ability to classify children and adolescents with asthma, cystic fibrosis (CF), and healthy controls. Methods: This was a cross-sectional study that included 103 patients with controlled persistent allergic asthma, 53 with CF and a healthy control group with 40 volunteers (aged 6 to 15 years), of both sexes. The individuals underwent volumetric capnography and spirometry. Results: Phase III slope (SIII), SIII standardized by exhaled tidal volume (SIII/TV) and capnographic index (SIII/SII) × 100 (KPIv) were different among the three groups assessed, with highest values for CF. The relation between the forced expiratory volume in one second and the forced vital capacity (FEV1/FVC) was the only spirometric marker that presented difference on the three groups. On individuals with normal spirometry, KPIv and FEV1/FVC were different among the three groups. The ROC curve identified the individuals with asthma or CF from the control group, both through volumetric capnography (better to identify CF in relation to the control using KPIv) and through spirometry (better to identify asthma in relation to the control). KPIv was the best parameter to distinguish asthma from CF, even in individuals with normal spirometry. Conclusion: Volumetric capnography and spirometry identified different alterations in lung function on asthma, CF, and healthy controls, allowing the three groups to be distinguished.

https://doi.org/10.1016/j.jped.2018.10.008 725 downloads

Cost of precision medicine at a referral center for cystic fibrosis
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Marson, Fernando Augusto Lima

Jornal Brasileiro de Pneumologia

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2020, Volume 46 N. 2 elocation e20190308

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

https://doi.org/10.36416/1806-3756/e20190308 876 downloads

Chloride and sodium ion concentrations in saliva and sweat as a method to diagnose cystic fibrosis
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Gonçalves, Aline Cristina

; Marson, Fernando Augusto Lima

; Mendonça, Regina Maria Holanda

; Bertuzzo, Carmen Sílvia

; Paschoal, Ilma Aparecida

; Ribeiro, José Dirceu

; Ribeiro, Antônio Fernando

; Levy, Carlos Emílio

Jornal de Pediatria

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aug 2019, Volume 95 N. 4 Pages 443 - 450

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

Resumo Objetivo: O diagnóstico da fibrose cística depende do valor da concentração de íons de cloreto no teste do suor (≥ 60 mEq/mL - reconhecido como o indicador-padrão para o diagnóstico da doença). Além disso, as glândulas salivares expressam a proteína RTFC igualmente às glândulas sudoríparas. Nesse contexto, nosso objetivo foi verificar a correlação da concentração de cloreto na saliva e a concentração de cloreto no suor e entre a concentração de sódio na saliva e a concentração de sódio no suor em pacientes com fibrose cística e indivíduos controles saudáveis, como uma ferramenta para diagnóstico de fibrose cística. Métodos: Contamos com a participação de 160 indivíduos [57/160 (35,70%) com fibrose cística e duas mutações no gene RTFC conhecidas e 103/160 (64,40%) indivíduos controles saudáveis]. A concentração de íons na saliva foi analisada pelo equipamento ABL 835 da Radiometer® e a concentração de cloreto no suor e sódio no suor, respectivamente, por titulação manual utilizando o método mercurimétrico de Schales & Schales e fotometria de chama. A análise estatística foi realizada pelo teste qui-quadrado, pelo teste de Mann-Whitney e pela correlação de Spearman. Alpha = 0,05. Resultados: Os pacientes com fibrose cística apresentaram maiores valores na concentração de cloreto no suor, concentração de sódio no suor, concentração de cloreto na saliva e concentração de sódio na saliva do que os indivíduos-controle saudáveis (valor de p < 0,001). A correlação entre as concentrações de cloreto na saliva e cloreto no suor mostrou Rho de Spearman (coeficiente de correlação) positivo = 0,475 (IC de 95% = 0,346 a 0,587). Além disso, a correlação entre concentração de sódio na saliva e concentração de sódio no suor mostrou Rho de Spearman positivo = 0,306 (IC de 95% = 0,158 a 0,440). Conclusões: A concentração de cloreto na saliva e a concentração de sódio na saliva são candidatas a ser usadas como diagnóstico de fibrose cística, principalmente em casos em que é difícil atingir a quantidade correta de suor, e/ou o exame da mutação RTFC é difícil e/ou o método de referência para o teste do suor não se encontra disponível ou não é de fácil acesso ao público em geral.

Abstract Objective: Cystic fibrosis diagnosis is dependent on the chloride ion concentration in the sweat test (≥ 60 mEq/mL - recognized as the gold standard indicator for cystic fibrosis diagnosis). Moreover, the salivary glands express the CFTR protein in the same manner as sweat glands. Given this context, the objective was to verify the correlation of saliva chloride concentration and sweat chloride concentration, and between saliva sodium concentration and sweat sodium concentration, in patients with cystic fibrosis and healthy control subjects, as a tool for cystic fibrosis diagnosis. Methods: There were 160 subjects enrolled: 57/160 (35.70%) patients with cystic fibrosis and two known CFTR mutations and 103/160 (64.40%) healthy controls subjects. Saliva ion concentration was analyzed by ABL 835 Radiometer® equipment and, sweat chloride concentration and sweat sodium concentration, respectively, by manual titration using the mercurimetric procedure of Schales & Schales and flame photometry. Statistical analysis was performed by the chi-squared test, the Mann -Whitney test, and Spearman's correlation. Alpha = 0.05. Results: Patients with cystic fibrosis showed higher values of sweat chloride concentration, sweat sodium concentration, saliva chloride concentration, and saliva sodium concentration than healthy controls subjects (p-value < 0.001). The correlation between saliva chloride concentration and sweat chloride concentration showed a positive Spearman's Rho (correlation coefficient) = 0.475 (95% CI = 0.346 to 0.587). Also, the correlation between saliva sodium concentration and sweat sodium concentration showed a positive Spearman's Rho = 0.306 (95% CI = 0.158 to 0.440). Conclusions: Saliva chloride concentration and saliva sodium concentration are candidates to be used in cystic fibrosis diagnosis, mainly in cases where it is difficult to achieve the correct sweat amount, and/or CFTR mutation screening is difficult, and/or reference methods for sweat test are unavailable to implement or are not easily accessible by the general population.

https://doi.org/10.1016/j.jped.2018.04.005 2718 downloads

10.

Influence of AIDS antiretroviral therapy on the growth pattern,
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Golucci, Ana Paula Brigatto Simões

; Marson, Fernando Augusto Lima

; Valente, Mariana Freitas Fedato

; Branco, Maira Migliari

; Prado, Camila Carbone

; Nogueira, Roberto José Negrão

Jornal de Pediatria

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feb 2019, Volume 95 N. 1 Pages 7 - 17

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

Resumo Objetivos: A infecção pelo vírus da imunodeficiência humana pode comprometer, precocemente, os indicadores antropométricos de crianças e adolescentes. No entanto, a terapia antirretroviral combinada tem melhorado, além da resposta imunológica e da infecção viral, o ganho pôndero-estatural dos infectados. Dessa forma, nosso objetivo foi avaliar o efeito da terapia antirretroviral combinada no crescimento, de crianças e adolescentes, infectadas pelo vírus da imunodeficiência humana. Fonte dos dados: Foi realizada uma revisão sistemática. No estudo, adotou-se como critério de elegibilidade dos artigos, a estratégia PRISMA (preferred reporting items for systematic reviews and meta-analyses). Foram consultadas as bases de dados MEDLINE-PubMed e LILACS pelos descritores: HIV (vírus da imunodeficiência humana), children, growth, antiretroviral therapy. O objetivo foi definido pela estratégia PICO (population, intervention, comparison/control, outcome). Critérios de inclusão e exclusão foram aplicados na seleção dos estudos. Síntese dos dados: Dos 549 estudos indexados no MEDLINE-PubMed e LILACS, 73 foram lidos na íntegra – 44 incluídos na revisão (33 demonstraram impacto positivo da terapia antirretroviral combinada no ganho pôndero-estatural, dez no ganho de peso e um no de estatura, em crianças e adolescentes, infectados com vírus da imunodeficiência humana). No entanto, o incremento no crescimento não foi o suficiente para normalizar a estatura de crianças infectadas, quando comparado com crianças da mesma idade e sexo, sem infecção pelo vírus da imunodeficiência humana. Conclusões: A terapia antirretroviral combinada que, conhecidamente, atua na melhora de marcadores virais e imunológicos, pode influenciar no ganho pôndero-estatural de crianças infectadas com vírus da imunodeficiência humana. Quanto mais precoce o diagnóstico da infecção e, concomitante, desnutrição e início da terapia antirretroviral combinada, menores serão os prejuízos no crescimento, quando comparado às crianças saudáveis.

Abstract Objectives: Human immunodeficiency virus infection can result in the early impairment of anthropometric indicators in children and adolescents. However, combined antiretroviral therapy has improved, in addition to the immune response and viral infection, the weight and height development in infected individuals. Therefore, the objective was to evaluate the effect of combined antiretroviral on the growth development of human immunodeficiency virus infected children and adolescents. Source of data: A systematic review was performed. In the study, the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) strategy was used as the eligibility criterion. The MEDLINE-PubMed and LILACS databases were searched using these descriptors: HIV, children, growth, antiretroviral therapy. The objective was defined by the population, intervention, comparison/control, and outcome (PICO) technique. Inclusion and exclusion criteria were applied for study selection. Synthesis of data: Of the 549 studies indexed in MEDLINE-PubMed and LILACS, 73 were read in full, and 44 were included in the review (33 showed a positive impact of combined antiretroviral therapy on weight/height development, ten on weight gain, and one on height gain in children and adolescents infected with human immunodeficiency virus). However, the increase in growth was not enough to normalize the height of infected children when compared to children of the same age and gender without human immunodeficiency virus infection. Conclusions: Combined antiretroviral therapy, which is known to play a role in the improvement of viral and immunological markers, may influence in the weight and height development in children infected with human immunodeficiency virus. The earlier the infection diagnosis and, concomitantly, of malnutrition and the start of combined antiretroviral therapy, the lower the growth impairment when compared to healthy children.

https://doi.org/10.1016/j.jped.2018.02.006 1049 downloads

11.

Lung ultrasound assessment of response to antibiotic therapy in cystic fibrosis exacerbations: a study of two cases
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Peixoto, Andressa Oliveira

; Marson, Fernando Augusto Lima

; Souza, Tiago Henrique

; Fraga, Andrea de Melo Alexandre

; Ribeiro, José Dirceu

Jornal Brasileiro de Pneumologia

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2019, Volume 45 N. 6 elocation e20190128

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

https://doi.org/10.1590/1806-3713/e20190128 649 downloads

12.

Correlation between acoustic rhinometry, computed rhinomanometry and cone-beam computed tomography in mouth breathers with transverse maxillary deficiency
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Sakai, Raquel Harumi Uejima Satto

; Marson, Fernando Augusto Lima

; Sakuma, Emerson Taro Inoue

; Ribeiro, José Dirceu

; Sakano, Eulália

Brazilian Journal of Otorhinolaryngology

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jan 2018, Volume 84 N. 1 Pages 40 - 50

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

Resumo Introdução Numerosos exames podem ser realizados para fornecer informações clínicas e diagnósticas em respiradores bucais com atresia maxilar e mordida cruzada posterior, entretanto a correlação entre esses exames ainda é incerta. Objetivo Avaliar a correlação entre rinometria acústica, rinomanometria computadorizada e tomografia computadorizada por feixe cônico em respiradores bucais com atresia maxilar. Método Um estudo de corte transversal foi realizado em 30 respiradores bucais com atresia maxilar (7-13 anos) com mordida cruzada posterior. Os exames avaliados foram: (i) rinomanometria acústica: volumes nasais (0-5 cm e 2-5 cm) e áreas mínimas de corte transversal 1 e 2 da cavidade nasal; (ii) rinomanometria computadorizada: fluxo nasal e resistências médias inspiratórias e expiratórias; (iii) tomografia computadorizada por feixe cônico: corte coronal na cabeça da concha inferior (larguras 1 e 2), concha média (larguras 3 e 4) e na maxila (Largura 5). Rinomanometria acústica e rinomanometria computadorizada foram avaliadas antes e depois da administração de vasoconstritor. Os resultados foram comparados pelo teste de correlação de Spearman e pelo teste de Mann-Whitney (α = 0,05). Resultados Foram encontradas correlações positivas entre: (i) fluxo antes da administração de vasoconstritor e largura 4 (Rho = 0,380) e largura 5 (Rho = 0,371); (ii) largura 2 e área mínima de corte transversal 1 antes da administração de vasoconstritor (Rho = 0,380); (iii) fluxo antes da administração de vasoconstritor e volumes nasais de 0-5 cm (Rho = 0,421), 2-5 cm (Rho = 0,393) e área mínima de corte transversal 1 (Rho = 0,375); (iv) largura 4 e volume nasal de 0-5 cm antes da administração do vasoconstritor (Rho = 0,376), volume nasal de 2-5 cm antes do uso de vasoconstritor (Rho = 0,376), áreas mínimas de corte transversal 1 antes da administração de vasoconstritor (Rho = 0,410) e áreas mínimas de corte transversal 1 após o uso do vasoconstritor (Rho = 0,426); (v) largura 5 e largura 1 (Rho = 0,542), largura 2 (Rho = 0,411) e largura 4 (Rho = 0,429). Foram encontradas correlações negativas: (i) largura 4 e resistência inspiratória média (Rho = −0,385); (ii) resistência inspiratória média antes da administração de vasoconstritor e volume de 0-5 cm (Rho = −0,382) e resistência expiratória média antes da administração de vasoconstritor e área mínima de corte transversal 1 (Rho = −0,362). Conclusão Correlações foram encontradas entre a rinometria acústica, a rinomanometria computadorizada e a tomografia computadorizada de feixe cônico em respiradores bucais com atresia maxilar.

Abstract Introduction To provide clinical information and diagnosis in mouth breathers with transverse maxillary deficiency with posterior crossbite, numerous exams can be performed; however, the correlation among these exams remains unclear. Objective To evaluate the correlation between acoustic rhinometry, computed rhinomanometry, and cone-beam computed tomography in mouth breathers with transverse maxillary deficiency. Methods A cross-sectional study was conducted in 30 mouth breathers with transverse maxillary deficiency (7-13 y.o.) patients with posterior crossbite. The examinations assessed: (i) acoustic rhinometry: nasal volumes (0-5 cm and 2-5 cm) and minimum cross-sectional areas 1 and 2 of nasal cavity; (ii) computed rhinomanometry: flow and average inspiratory and expiratory resistance; (iii) cone-beam computed tomography: coronal section on the head of inferior turbinate (Widths 1 and 2), middle turbinate (Widths 3 and 4) and maxilla levels (Width 5). Acoustic rhinometry and computed rhinomanometry were evaluated before and after administration of vasoconstrictor. Results were compared by Spearman's correlation and Mann-Whitney tests (α = 0.05). Results Positive correlations were observed between: (i) flow evaluated before administration of vasoconstrictor and Width 4 (Rho = 0.380) and Width 5 (Rho = 0.371); (ii) Width 2 and minimum cross-sectional areas 1 evaluated before administration of vasoconstrictor (Rho = 0.380); (iii) flow evaluated before administration of vasoconstrictor and nasal volumes of 0-5 cm (Rho = 0.421), nasal volumes of 2-5 cm (Rho = 0.393) and minimum cross-sectional areas 1 (Rho = 0.375); (iv) Width 4 and nasal volumes of 0-5 cm evaluated before administration of vasoconstrictor (Rho = 0.376), nasal volumes of 2-5 cm evaluated before administration of vasoconstrictor (Rho = 0.376), minimum cross-sectional areas 1 evaluated before administration of vasoconstrictor (Rho = 0.410) and minimum cross-sectional areas 1 after administration of vasoconstrictor (Rho = 0.426); (v) Width 5 and Width 1 (Rho = 0.542), Width 2 (Rho = 0.411), and Width 4 (Rho = 0.429). Negative correlations were observed between: (i) Width 4 and average inspiratory resistance (Rho = −0.385); (ii) average inspiratory resistance evaluated before administration of vasoconstrictor and nasal volumes of 0-5 cm (Rho = −0.382), and average expiratory resistance evaluated before administration of vasoconstrictor and minimum cross-sectional areas 1 (Rho = −0.362). Conclusion There were correlations between acoustic rhinometry, computed rhinomanometry, and cone-beam computed tomography in mouth breathers with transverse maxillary deficiency.

https://doi.org/10.1016/j.bjorl.2016.10.015 1137 downloads

13.

Variants in the interleukin 8 gene and the response to inhaled bronchodilators in cystic fibrosis,
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Furlan, Larissa Lazzarini

; Ribeiro, José Dirceu

; Bertuzzo, Carmen Sílvia

; Salomão Junior, João Batista

; Souza, Dorotéia Rossi Silva

; Marson, Fernando Augusto Lima

Jornal de Pediatria

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dec 2017, Volume 93 N. 6 Pages 639 - 648

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

Resumo Objetivo: A proteína interleucina 8 promove respostas inflamatórias, o que inclui sua atuação nas vias aéreas. A presença de variantes no gene da interleucina 8 causa respostas inflamatórias alteradas e possivelmente respostas variadas ao uso de broncodilatadores inalatórios. Assim, este estudo analisou as variantes da interleucina 8 (rs4073, rs2227306, rs2227307) e sua associação à resposta a broncodilatadores inalatórios em pacientes com fibrose cística. Métodos: Foi feita análise das variantes genéticas da interleucina 8 por restriction fragment length polymorphism da reação em cadeia da polimerase. A associação entre os marcadores da espirometria e a resposta a broncodilatadores inalatórios foi feita pelos testes de Mann-Whitney e Kruskal-Wallis. A análise incluiu todos os pacientes com fibrose cística e posteriormente pacientes com duas mutações no gene cystic fibrosis transmembrane conductance regulator pertencentes às Classes I a II. Resultados: Este estudo incluiu 186 pacientes com fibrose cística. Não houve associação da variante rs2227307 à resposta a broncodilatadores inalatórios. A variante rs2227306 foi associada a FEF50% no grupo dominante e no grupo com duas mutações identificadas no gene cystic fibrosis transmembrane conductance regulator. A variante rs4073 foi associada a marcadores da espirometria em quatro modelos genéticos: codominante (FEF25-75% e FEF75%), dominante (VEF1, FEF50%, FEF75% e FEF25-75%), recessivo (FEF75% e FEF25-75%) e overdominante (VEF1/CVF). Conclusões: Este estudo destaca, principalmente, a importância da variante rs4073 do gene da interleucina 8, na resposta a broncodilatadores inalatórios, concomitantemente ao genótipo das mutações no gene cystic fibrosis transmembrane conductance regulator.

Abstract Objective: Interleukin 8 protein promotes inflammatory responses, even in airways. The presence of interleukin 8 gene variants causes altered inflammatory responses and possibly varied responses to inhaled bronchodilators. Thus, this study analyzed the interleukin 8 variants (rs4073, rs2227306, and rs2227307) and their association with the response to inhaled bronchodilators in cystic fibrosis patients. Methods: Analysis of interleukin 8 gene variants was performed by restriction fragment length polymorphism of polymerase chain reaction. The association between spirometry markers and the response to inhaled bronchodilators was evaluated by Mann-Whitney and Kruskal-Wallis tests. The analysis included all cystic fibrosis patients, and subsequently patients with two mutations in the cystic fibrosis transmembrane conductance regulator gene belonging to classes I to III. Results: This study included 186 cystic fibrosis patients. There was no association of the rs2227307 variant with the response to inhaled bronchodilators. The rs2227306 variant was associated with FEF50% in the dominant group and in the group with two identified mutations in the cystic fibrosis transmembrane conductance regulator gene. The rs4073 variant was associated with spirometry markers in four genetic models: co-dominant (FEF25-75% and FEF75%), dominant (FEV1, FEF50%, FEF75%, and FEF25-75%), recessive (FEF75% and FEF25-75%), and over-dominant (FEV1/FVC). Conclusions: This study highlighted the importance of the rs4073 variant of the interleukin 8 gene, regarding response to inhaled bronchodilators, and of the assessment of mutations in the cystic fibrosis transmembrane conductance regulator gene.

https://doi.org/10.1016/j.jped.2017.03.005 948 downloads

14.

Brazilian guidelines for the diagnosis and treatment of cystic fibrosis
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Athanazio, Rodrigo Abensur

; Silva Filho, Luiz Vicente Ribeiro Ferreira da

; Vergara, Alberto Andrade

; Ribeiro, Antônio Fernando

; Riedi, Carlos Antônio

; Procianoy, Elenara da Fonseca Andrade

; Adde, Fabíola Villac

; Reis, Francisco José Caldeira

; Ribeiro, José Dirceu

; Torres, Lídia Alice

; Fuccio, Marcelo Bicalho de

; Epifanio, Matias

; Firmida, Mônica de Cássia

; Damaceno, Neiva

; Ludwig-Neto, Norberto

; Maróstica, Paulo José Cauduro

; Rached, Samia Zahi

; Melo, Suzana Fonseca de Oliveira

; Pinto, Leonardo Araújo

; Monte, Luciana Freitas Velloso

; Higa, Laurinda Yoko Shinzato

; Folescu, Tania Wrobel

; Marson, Fernando Augusto de Lima

; Sad, Isabela

; Servidoni, Maria de Fátima Correa Pimenta

; Kussek, Paulo

; Raskin, Salmo

; Zuana, Adriana Della

; Augustin, Albin

; Hoffmann, Anneliese

; Barbisan, Beatriz

; Hochhegger, Bruno

; Levy, Carlos Emilio

; Veiga, Claudine Sarmento da

; Ricachinevsky, Claudio

; Esposito, Concetta

; Escuissato, Dante

; Brandemburgo, Diego

; Marques, Elisabeth

; Aquino, Evanirso de

; Fischer, Gilberto Bueno

; Rodrigues, Joaquim Carlos

; Machado, Leticia

; Muramato, Lucia

; Costa, Lusmaia Damasceno Camargo

; Donadio, Marcio

; Castro, Marcos César Santos de

; Ribeiro, Maria Angela

; Santana, Maria Angélica

; Canan, Mariane

; Almeida, Marina Buarque de

; Britto, Murilo

; Dalcin, Paulo Roth Tarso

; Ramos, Regina Terse Trindade

; Chiba, Sonia

; Martins, Valéria de Carvalho

; Lacerda, Claudine

; Barbosa, Eliana

; Guimarães, Elizabet Vilar

; Hessel, Gabriel

; Gurmini, Jocemara

; Neri, Lenycia

; Nogueira, Marcelo Coelho

; Wayhs, Mônica Chang

; Simon, Miriam Isabel Santos

; Fernandes, Arlene Gonçalves dos Santos

; Silva, Claudia de Castro de

; Albuquerque, Cristiano Túlio Maciel

; Souza, Edna Lúcia

; Silva, Fernando Antonio de Abreu e

; Dalcin, Paulo de Tarso

; Noronha, Renata Maria de

; Teixeira, Ricardo

; Machado, Sandra Helena

; Camargo, Spencer Marcantonio

; Rozov, Tatiana

; Rodrigues, Ticiana da Costa

Jornal Brasileiro de Pneumologia

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jun 2017, Volume 43 N. 3 Pages 219 - 245

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

RESUMO A fibrose cística (FC) é uma doença genética autossômica recessiva caracterizada pela disfunção do gene CFTR. Trata-se de uma doença multissistêmica que ocorre mais frequentemente em populações descendentes de caucasianos. Nas últimas décadas, diversos avanços no diagnóstico e tratamento da FC mudaram drasticamente o cenário dessa doença, com aumento expressivo da sobrevida e qualidade de vida. Atualmente, o Brasil dispõe de um programa de ampla cobertura para a triagem neonatal de FC e centros de referência distribuídos na maior parte desses estados para seguimento dos indivíduos. Antigamente confinada à faixa etária pediátrica, tem-se observado um aumento de pacientes adultos com FC tanto pelo maior número de diagnósticos de formas atípicas, de expressão fenotípica mais leve, assim como pelo aumento da expectativa de vida com os novos tratamentos. Entretanto, ainda se observa uma grande heterogeneidade no acesso aos métodos diagnósticos e terapêuticos para FC entre as diferentes regiões brasileiras. O objetivo dessas diretrizes foi reunir as principais evidências científicas que norteiam o manejo desses pacientes. Um grupo de 18 especialistas em FC elaborou 82 perguntas clínicas relevantes que foram divididas em cinco categorias: características de um centro de referência; diagnóstico; tratamento da doença respiratória; tratamento gastrointestinal e nutricional; e outros aspectos. Diversos profissionais brasileiros atuantes na área da FC foram convidados a responder as perguntas formuladas pelos coordenadores. A literatura disponível foi pesquisada na base de dados PubMed com palavras-chave, buscando-se as melhores respostas às perguntas dos autores.

ABSTRACT Cystic fibrosis (CF) is an autosomal recessive genetic disorder characterized by dysfunction of the CFTR gene. It is a multisystem disease that most often affects White individuals. In recent decades, various advances in the diagnosis and treatment of CF have drastically changed the scenario, resulting in a significant increase in survival and quality of life. In Brazil, the current neonatal screening program for CF has broad coverage, and most of the Brazilian states have referral centers for the follow-up of individuals with the disease. Previously, CF was limited to the pediatric age group. However, an increase in the number of adult CF patients has been observed, because of the greater number of individuals being diagnosed with atypical forms (with milder phenotypic expression) and because of the increase in life expectancy provided by the new treatments. However, there is still great heterogeneity among the different regions of Brazil in terms of the access of CF patients to diagnostic and therapeutic methods. The objective of these guidelines was to aggregate the main scientific evidence to guide the management of these patients. A group of 18 CF specialists devised 82 relevant clinical questions, divided into five categories: characteristics of a referral center; diagnosis; treatment of respiratory disease; gastrointestinal and nutritional treatment; and other aspects. Various professionals working in the area of CF in Brazil were invited to answer the questions devised by the coordinators. We used the PubMed database to search the available literature based on keywords, in order to find the best answers to these questions.

https://doi.org/10.1590/s1806-37562017000000065 75529 downloads

15.

Sweat test and cystic fibrosis: overview of test performance at public and private centers in the state of São Paulo, Brazil
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Servidoni, Maria Fátima

; Gomez, Carla Cristina Souza

; Marson, Fernando Augusto Lima

; Toro, Adyléia Aparecida Dalbo Contrera

; Ribeiro, Maria Ângela Gonçalves de Oliveira

; Ribeiro, José Dirceu

; Ribeiro, Antônio Fernando

Jornal Brasileiro de Pneumologia

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apr 2017, Volume 43 N. 2 Pages 121 - 128

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

RESUMO Objetivo: O teste do suor (TS) mede os níveis de cloro no suor e é considerado o padrão ouro para o diagnóstico da fibrose cística (FC). Contudo, a confiabilidade do TS depende de sua realização por técnicos experientes e segundo diretrizes rígidas. Nosso objetivo foi avaliar como são realizadas a estimulação e coleta do suor e a dosagem de cloro em 14 centros (9 públicos e 5 privados) que realizam TS rotineiramente no estado de São Paulo, que possui a maior frequência de FC do Brasil. Métodos: Estudo de coorte transversal utilizando um questionário padronizado aplicado in loco ao pessoal responsável pela realização dos TS. Resultados: Não houve uniformidade entre os centros quanto aos procedimentos. A maioria dos centros não era aderente às diretrizes internacionais, especialmente quanto à coleta do suor (amostras insuficientes em 10-50% dos indivíduos testados), disponibilidade de equipamentos de estimulação (limitada em 2 centros), modernidade e certificação dos mesmos (a maioria utilizada há 3-23 anos) e protocolos escritos (ausentes em 12 centros). Avaliou-se o conhecimento sobre diretrizes para TS em apenas 1 centro. Conclusões: Nossos resultados mostram que, nos centros participantes, os TS estão muito distantes das diretrizes internacionalmente aceitas. Portanto, há necessidade urgente de padronização dos TS, de treinamento de pessoal qualificado e de aquisição/certificação de equipamentos adequados. Essas são condições essenciais para um diagnóstico confiável de FC, especialmente com a crescente demanda resultante da triagem neonatal em todo o país, e para a avaliação do possível benefício clínico do uso de moduladores.

ABSTRACT Objective: The sweat test (ST) measures chloride levels in sweat and is considered the gold standard for the diagnosis of cystic fibrosis (CF). However, the reliability of a ST depends on their being performed by experienced technicians and in accordance with strict guidelines. Our aim was to evaluate how sweat stimulation, sweat collection, and chloride measurement are performed at 14 centers (9 public centers and 5 private centers) that routinely perform STs in the state of São Paulo, which has the highest frequency of CF in Brazil. Methods: This was a cross-sectional cohort study, using a standardized questionnaire administered in loco to the staff responsible for conducting STs. Results: No uniformity regarding the procedures was found among the centers. Most centers were noncompliant with the international guidelines, especially regarding the collection of sweat (the samples were insufficient in 10-50% of the subjects tested); availability of stimulation equipment (which was limited at 2 centers); modernity and certification of stimulation equipment (most of the equipment having been used for 3-23 years); and written protocols (which were lacking at 12 centers). Knowledge of ST guidelines was evaluated at only 1 center. Conclusions: Our results show that STs largely deviate from internationally accepted guidelines at the participating centers. Therefore, there is an urgent need for standardization of STs, training of qualified personnel, and acquisition/certification of suitable equipment. These are essential conditions for a reliable diagnosis of CF, especially with the increasing demand due to newborn screening nationwide, and for the assessment of a possible clinical benefit from the use of modulator drugs.

https://doi.org/10.1590/s1806-37562016000000076 9668 downloads

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