Routine spirometry in cystic fibrosis patients: impact on pulmonary exacerbation diagnosis and FEV1 decline / Espirometria de rotina em pacientes com fibrose cística: impacto no diagnóstico de exacerbação pulmonar e no declínio do VEF1

ABSTRACT Objective: Pulmonary disease in cystic fibrosis (CF) is characterised by recurrent episodes of pulmonary exacerbations (PExs), with acute and long-term declines in lung function (FEV1). The study sought to determine whether routine spirometry increases the frequency of PEx diagnosis, resulting in benefits to long-term pulmonary function. Methods: CF patients in the 5- to 18-year age bracket were followed for 1 year, during which they underwent spirometry before every medical visit. The main variables were the frequency of PEx diagnosis and use of antibiotics; the use of spirometry as a criterion for PEx diagnosis (a decline ≥ 10% in baseline FEV1); and median percent predicted FEV1 over time. The data were compared with those for the previous 24-month period, when spirometry was performed electively every 6 months. Results: The study included 80 CF patients. PExs were diagnosed in 27.5% of the visits, with a mean frequency of 1.44 PExs per patient/year in 2014 vs. 0.88 PExs per patient/year in 2012 (p = 0.0001) and 1.15 PExs per patient/year in 2013 (p = 0.05). FEV1 was used as a diagnostic feature in 83.5% of PExs. In 21.9% of PExs, the decision to initiate antibiotics was solely based on an acute decline in FEV1. The median percent predicted FEV1 during the follow-up year was 85.7%, being 78.5% in 2013 and 76.8% in 2012 (p > 0.05). The median percent predicted FEV1 remained above 80% during the two years after the study. Conclusions: Routine spirometry is associated with higher rates of diagnosis and treatment of PExs, possibly impacting long-term pulmonary function.

RESUMO Objetivo: A doença pulmonar na fibrose cística (FC) é caracterizada por episódios recorrentes de exacerbações pulmonares (EP), com declínio agudo e em longo prazo da função pulmonar (VEF1). O objetivo deste estudo foi determinar se a espirometria de rotina aumenta a frequência de diagnóstico de EP, beneficiando a função pulmonar em longo prazo. Métodos: Pacientes com FC na faixa etária de 5 a 18 anos foram acompanhados durante 1 ano, ao longo do qual foram submetidos a espirometria antes de cada consulta médica. As principais variáveis foram a frequência de diagnóstico de EP e uso de antibióticos; o uso da espirometria como critério de diagnóstico de EP (declínio do VEF1 basal ≥ 10%); e a mediana do VEF1 em porcentagem do previsto ao longo do tempo. Os dados foram comparados àqueles referentes aos 24 meses anteriores, período durante o qual a espirometria era realizada eletivamente a cada 6 meses. Resultados: O estudo incluiu 80 pacientes com FC. EP foram diagnosticadas em 27,5% das consultas, com média de frequência de 1,44 EP por paciente/ano em 2014 vs. 0,88 EP por paciente/ano em 2012 (p = 0,0001) e 1,15 EP por paciente/ano em 2013 (p = 0,05). O VEF1 foi usado como recurso diagnóstico em 83,5% das EP. Em 21,9% das EP, a decisão de iniciar a antibioticoterapia baseou-se exclusivamente no declínio agudo do VEF1. A mediana do VEF1 em porcentagem do previsto foi de 85,7% durante o ano de acompanhamento, de 78,5% em 2013 e de 76,8% em 2012 (p > 0,05). A mediana do VEF1 em porcentagem do previsto permaneceu acima de 80% durante os dois anos após o estudo. Conclusões: A espirometria de rotina está associada a taxas mais elevadas de diagnóstico e tratamento de EP e possivelmente tem impacto na função pulmonar em longo prazo.

Brazilian gastric cancer association guidelines (part 2): update on treatment / Diretrizes da associação brasileira de câncer gástrico (parte 2): atualização sobre o tratamento

ABSTRACT Background : The II Brazilian Consensus on Gastric Cancer of the Brazilian Gastric Cancer Association BGCA (Part 1) was recently published. On this occasion, countless specialists working in the treatment of this disease expressed their opinion in the face of the statements presented. Aim : To present the BGCA Guidelines (Part 2) regarding indications for surgical treatment, operative techniques, extension of resection and multimodal treatment. Methods: To formulate these guidelines, the authors carried out an extensive and current review regarding each declaration present in the II Consensus, using the Medline/PubMed, Cochrane Library and SciELO databases initially with the following descriptors: gastric cancer, gastrectomy, lymphadenectomy, multimodal treatment. In addition, each statement was classified according to the level of evidence and degree of recommendation. Results : Of the 43 statements present in this study, 11 (25,6%) were classified with level of evidence A, 20 (46,5%) B and 12 (27,9%) C. Regarding the degree of recommendation, 18 (41,9%) statements obtained grade of recommendation 1, 14 (32,6%) 2a, 10 (23,3%) 2b e one (2,3%) 3. Conclusion : The guidelines complement of the guidelines presented here allows surgeons and oncologists who work to combat gastric cancer to offer the best possible treatment, according to the local conditions available.

RESUMO Racional: O II Consenso Brasileiro de Câncer Gástrico da Associação Brasileira de Câncer Gástrico ABCG (Parte 1) foi recentemente publicado. Nesta ocasião inúmeros especialistas que atuam no tratamento desta doença expressaram suas opiniões diante declarações apresentadas. Objetivo: Apresentar as Diretrizes da ABCG (Parte 2) quanto às indicações de tratamento cirúrgico, técnicas operatórias, extensão de ressecção e terapia combinada. Métodos: Para formulação destas diretrizes os autores realizaram extensa e atual revisão referente a cada declaração presente no II Consenso, utilizando as bases Medline/PubMed, Cochrane Library e SciELO, inicialmente com os seguintes descritores: câncer gástrico, gastrectomia, linfadenectomia, terapia combinada. Ainda, cada declaração foi classificada de acordo com o nível de evidência e grau de recomendação. Resultados: Das 43 declarações presentes neste estudo, 11 (25,6%) foram classificadas com nível de evidência A, 20 (46,5%) B e 12 (27,9%) C. Quanto ao grau de recomendação, 18 (41,9%) declarações obtiveram grau de recomendação 1, 14 (32,6%) 2a, 10 (23,3%) 2b e um (2,3%) 3. Conclusão: O complemento das diretrizes aqui presentes possibilita que cirurgiões e oncologistas que atuam no combate ao câncer gástrico possam oferecer o melhor tratamento possível, de acordo com as condições locais disponíveis.

Persistent symptoms and decreased health-related quality of life after symptomatic pediatric COVID-19: A prospective study in a Latin American tertiary hospital

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.

Randomized trial of physiotherapy and hypertonic saline techniques for sputum induction in asthmatic children and adolescents

OBJECTIVES: This study aimed to analyze the efficiency of physiotherapy techniques in sputum induction and in the evaluation of pulmonary inflammation in asthmatic children and adolescents. Although hypertonic saline (HS) is widely used for sputum induction (SI), specific techniques and maneuvers of physiotherapy (P) may facilitate the collection of mucus in some asthmatic children and adolescents. METHODS: A randomized crossover study was performed in patients with well-controlled asthma, and 90 sputum samples were collected. Children and adolescents were assessed using spirometry and randomized at entry into one of three sputum induction techniques: (i) 3% hypertonic saline - HS technique; (ii) physiotherapy (oscillatory positive expiratory pressure, forced expiration, and acceleration of expiratory flow) - P technique; and (iii) hypertonic saline + physiotherapy - HSP technique. ClinicalTrials.gov: NCT03136042. RESULTS: The total cells (mL) and the percentage (%) of differential inflammatory cells were similar in all techniques. The sputum weight (g) in the HSP technique was significantly higher than that in the HS technique. In all techniques, the percentage of viable cells was >50%, and there was no difference between the HS and P techniques. Moreover, sputum induction did not cause any alterations in the pulmonary function of patients. CONCLUSION: The physiotherapy sputum collection technique was effective in obtaining viable cells from mucus samples and yielded the same amount of sputum as the gold standard technique (hypertonic saline). In addition, the physiotherapy maneuvers were both safe and useful for sputum induction in asthmatic children and adolescents with well-controlled asthma.

Ii brazilian consensus on gastric cancer by the brazilian gastric cancer association / Ii consenso brasileiro de câncer gástrico realizado pela associação brasileira de câncer gástrico

ABSTRACT Background: Since the publication of the first Brazilian Consensus on Gastric Cancer (GC) in 2012 carried out by the Brazilian Gastric Cancer Association, new concepts on diagnosis, staging, treatment and follow-up have been incorporated. Aim: This new consensus is to promote an update to professionals working in the fight against GC and to provide guidelines for the management of patients with this condition. Methods: Fifty-nine experts answered 67 statements regarding the diagnosis, staging, treatment and prognosis of GC with five possible alternatives: 1) fully agree; 2) partially agree; 3) undecided; 4) disagree and 5) strongly disagree A consensus was adopted when at least 80% of the sum of the answers "fully agree" and "partially agree" was reached. This article presents only the responses of the participating experts. Comments on each statement, as well as a literature review, will be presented in future publications. Results: Of the 67 statements, there was consensus in 50 (74%). In 10 declarations, there was 100% agreement. Conclusion: The gastric cancer treatment has evolved considerably in recent years. This consensus gathers consolidated principles in the last decades, new knowledge acquired recently, as well as promising perspectives on the management of this disease.

RESUMO Racional: Desde a publicação do primeiro Consenso Brasileiro sobre Câncer Gástrico em 2012 realizado pela Associação Brasileira de Câncer Gástrico (ABCG), novos conceitos sobre o diagnóstico, estadiamento, tratamento e seguimento foram incorporados. Objetivo: Promover uma atualização aos profissionais que atuam no combate ao câncer gástrico (CG) e fornecer diretrizes quanto ao manejo dos pacientes portadores desta afecção. Métodos: Cinquenta e nove especialistas responderam 67 declarações sobre o diagnóstico, estadiamento, tratamento e prognóstico do CG com cinco alternativas possíveis: 1) concordo plenamente; 2) concordo parcialmente; 3) indeciso; 4) discordo e 5) discordo fortemente. Foi considerado consenso a concordância de pelo menos 80% da soma das respostas "concordo plenamente" e "concordo parcialmente". Este artigo apresenta apenas as respostas dos especialistas participantes. Os comentários sobre cada declaração, assim como uma revisão da literatura serão apresentados em publicações futuras. Resultados: Das 67 declarações, houve consenso em 50 (74%). Em 10 declarações, houve concordância de 100%. Conclusão: O tratamento do câncer gástrico evoluiu consideravelmente nos últimos anos. Este consenso reúne princípios consolidados nas últimas décadas, novos conhecimentos adquiridos recentemente, assim como perspectivas promissoras sobre o manejo desta doença.

Quality of life amongst adolescents and young adults with cystic fibrosis: correlations with clinical outcomes

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.

Termo de consentimento livre e esclarecido (TCLE): fatores que interferem na adesão / The written informed consent form (WICF): factors that interfere with acceptance

RACIONAL: O Termo de Consentimento Livre e Esclarecido (TCLE) aborda informações que precisam estar descritas de forma clara e de fácil compreensão, destacando riscos, possíveis benefícios e procedimentos. Atualmente discute-se a possibilidade de sujeitos de pesquisa não entenderem totalmente o texto do TCLE nem seus direitos como participantes, mesmo tendo assinado o TCLE e aderido à pesquisa. OBJETIVOS: Avaliar a legibilidade dos TCLE, bem como correlacionar a aceitação do sujeito da pesquisa com estado demográfico, fatores sociais, relação risco-benefício e nível de instrução. MÉTODOS: Análise dos dados de 793 pacientes, que foram convidados a participar de diferentes protocolos de pesquisa clínica em hospitais privados e públicos. Foram revistos os dados dos prontuários médicos para obtenção dos dados demográficos e sociais. Foram usados os Índices de Legibilidade Flesch Reading Ease e Flesch-Kincaid para avaliar o nível de legibilidade dos textos dos TCLE. RESULTADOS: A adesão dos sujeitos de pesquisa aos protocolos propostos não teve influência dos fatores demográficos e sociais, no entanto, verificou-se maior adesão entre os pacientes de instituição de tratamento público (99,7%) em comparação com instituição de tratamento privada (93,7%). A adesão foi maior entre os pacientes que participaram de protocolos com menor risco (99,73%) em comparação com os que participaram de protocolos com maior risco (81,3%). Apesar da adesão não ter tido influência do nível de escolaridade, ele foi menor ou igual a oito anos de estudo para 462 pacientes (58,26%), entre os quais 444 (96,1%) eram de instituição de tratamento público. Os índices de legibilidade obtidos variaram de 9.9 a12 para o teste de Flesch-Kincaid e 33,1 a 51,3 para o teste de Flesch Reading Ease. CONCLUSÕES: Os TCLE apresentaram altos graus de dificuldade para leitura. Apesar da aceitação pelo paciente não estar relacionada com fatores sociais ou demográficos, foi influenciado pela relação risco-benefício.

BACKGROUND: The written informed consent form (WICF) provides information that must be written in simple, easily understood language, highlighting voluntary participation safeguards, risks, possible benefits, and procedures. Currently, the possibility that research subjects do not fully understand the text of the WICF or their rights as participants, despite having signed the WICF and agreed to participate in the study, has been a point of discussion. AIM: To evaluate the readability of the WICFs, as well as to correlate research subject acceptance of the WICF with demographic status, social factors, risk-benefit relationship, and education level. METHODS: The study involved 793 patients treated in public or private hospitals and asked to give informed consent for their inclusion. Were reviewed patient medical charts in order to obtain demographic and social data, and was used the Flesch Reading Ease and the Flesch-Kincaid Readability Indices to evaluate the reading level of the WICF texts. RESULTS: Acceptance was higher (99.7%) among patients treated in public health care facilities and among patients (99.73%) who participated in protocols involving lower risk. Although acceptance was not influenced by education level, 462 patients (58.26%) had eight or less years of schooling. The obtained readability index ranged from 9.9 to 12 on the Flesch-Kincaid test, and from 33.1 to 51.3 on the Flesch Reading Ease test. CONCLUSION: The WICFs had high degree of reading difficulty. Although patient acceptance was not found to be related to demographic or social factors, it was found to be influenced by the risk-benefit relationship.

Cylindrical abdominoperineal resection rationale, technique and controversies / Amputação abdominoperineal cilíndrica racional, técnica e controvérsias

Surgery remains the cornerstone in rectal cancer treatment. Abdominoperineal excision (APE), described more than 100 years ago, remains as an important procedure for the treatment of selected advanced distal tumors with direct invasion of the anal sphincter or preoperative fecal incontinence. Historically, oncological outcomes of patients undergoing APE have been worse when compared to sphincter preserving operations. More recently, it has been suggested that patients undergoing APE for distal rectal cancer are more likely to have positive circumferential resection margins and intraoperative perforation, known surrogate markers for local recurrence. Recently, an alternative approach known as "Extralevator Abdominoperineal Excision" has been described in an effort to improve rates of circumferential margin positivity possibly resulting in better oncological outcomes compared to the standard procedure. The objective of this paper is to provide a technical description and compare available data of both Extralevator and Standard abdominal perineal excision techniques. (AU)

Um dos pilares mais importantes no tratamento do câncer de reto ainda é a ressecção cirúrgica. A amputação de reto, ou excisão abdomino-perineal do reto (APE), descrita há mais de 100 anos, continua sendo um procedimento importante para o tratamento de tumores retais distais que invadem o aparelho esfincteriano ou em casos de incontinência pré-operatória. Entretanto, os resultados oncológicos dos pacientes submetidos à APE são piores quando comparados com os pacientes submetidos a procedimentos com preservação esfincteriana. Recentemente, foi sugerido que os pacientes submetidos à APE por câncer de reto distal apresentam mais frequentemente margem radial positiva, assim como perfuração intraoperatória do tumor, fatos reconhecidamente associados à recidiva local. Uma nova técnica cirúrgica conhecida como "Amputação de reto extraelevador ou cilíndrica" tem sido descrita em um esforço para reduzir as taxas de margem radial positivas, sugerindo melhores resultados oncológicos quando comparada com o procedimento convencional. O objetivo deste trabalho é descrever a técnica deste procedimento e comparar seus resultados com os obtidos com a técnica convencional de acordo com a evidência disponível. (AU)

Amputación abdominoperineal cilíndrica: racionalidad y aspectos técnicos / Cylindric Abdominoperineal Resection: rational and technique

La resección quirúrgica sigue siendo la piedra angular de la terapia curativa en el cáncer de recto. La amputación abdominoperineal implica la remoción en bloque del recto, mesorrecto, aparato esfinteriano y el ano; con la creación de una colostomía definitiva. Esta cirugía es la propuesta para pacientes sin posibilidad de conservación esfinteriana. Este grupo representa aproximadamente el 80% de los pacientes con lesiones a menos de 5 cm del margen anal y aquellos con una continencia preoperatoria deficiente. Recientemente una modificación de la técnica denominada “Amputación abdominoperineal extraelevador” destinada a la ampliación del margen circunferencial sugiere mejores resultados oncológicos que la técnica clásica. El siguiente trabajo tiene por objetivo caracterizar y describir a la amputación abdominoperineal cilíndrica en comparación a la técnica clásica.

Surgery remains the cornerstone in rectal cancer treatment. Abdominoperineal resection involves the en bloc removal of the rectum, mesorectum, sphincters and anus with confection of a definitive colostomy. This surgery is indicated in patients without the possibility of sphincter preservation. This group represents approximately 80% of patients with lesions <5 cm from the anal verge and those with preoperative incontinence. Recently “Extralevator Abdominoperineal Excision” has been described to improve rates of circumferential margin positivity suggesting better oncological outcomes compared to the standard procedure. The objective of this paper is to provide a technical description and compare available data of both Extralevator and Standard techniques.

Tumor maligno da bainha do nervo periférico: relato de caso / Malignant peripheral nerve sheath tumor: case report

JUSTIFICATIVA E OBJETIVOS: O tumor maligno da bainha do nervo periférico (TMBNP), anteriormente chamado de schwannoma maligno, é um tumor raro, representando até 10% dos sarcomas. Em aproximadamente 50% dos casos associam-se à neurofibromatose tipo 1 (NF1) ou doença de von Recklinghausen. São tumores agressivos e frequentemente resistentes à quimioterapia.O objetivo deste estudo foi a exposição de um caso de TMBNP enfatizando a importância da imuno-histoquímica para o diagnóstico. RELATO DO CASO: Paciente do sexo masculino, 54 anos, internado por dor abdominal, desconforto pós-prandial, lombociatalgia com irradiação ao membro inferior direito e lesões nodulares no fígado. A investigação revelou massa retroperitoneal com necrose e lesões líticas no sacro e coluna vertebral. As biópsias do sacro e hepática revelaram células mesenquimatosas, fusiformes, que coravam para S-100 na imuno-histoquímica. Admitido o diagnóstico de TMBNP com origem em massa retroperitoneal com metastatização óssea e hepática. O paciente foi referenciado à oncologia sendo tratado com quimioterapia paliativa. CONCLUSÃO: A raridade do TMBNP explica a importância da divulgação deste caso, informando os clínicos, diminuindo o tempo para o diagnóstico e melhorando substancialmente as probabilidades de cura e a sobrevida. Este caso é interessante também por não estar associado à NF1 e por não se ter determinado o nervo periférico de origem, assentando o diagnóstico no resultadodo estudo anatomo-histológico.

BACKGROUND AND OBJECTIVES: The malignant peripheral nerve sheath tumor (MPNST), previously designated malign schwannoma is a rare tumor, accounting up to 10% of the soft tissues sarcomas. In approximately 50% of the cases there is anassociation with neurofibromatosis -1 (NF1) or von Recklinghausen disease. This tumor is extremely aggressive and frequently resistant to chemotherapy. The purpose of this study is to describe a clinical case of MPNST emphasizing the importance of the immunohistochemistry to accomplish the diagnosis. CASE REPORT: A male patient, 54 years-old, admitted to the hospital with abdominal pain, postprandial epigastric discomfort, lumbosacral pain radiating to the right leg and nodular hepatic lesions. The clinical investigation revealed retroperitoneal mass with areas of necrosis and disperse lytic lesions on the sacrum and vertebral column. The hepatic and sacral biopsies revealed mesenchymal cells, spindled, which stained positively to S-100 in the immunohistochemistry. It was diagnosed MPNST,originated in the retroperitoneal mass metastasized to liver and bone. The patient was oriented to oncology being treated with palliative chemotherapy. CONCLUSION: The rarity of MPNST explains the importance of divulgating this case in order to inform the clinicians, diminishing the time to diagnosis, improving treatment and survival probabilities. This case report is also interesting because there was no association with NF1, and because it wasn't possible to identify the original peripheral nerve. The diagnosis was made due to the histological study performed.

Granulomatose de Wegener: relato de casos / Wegener's granulomatosis: cases report

JUSTIFICATIVA E OBJETIVOS: A granulomatose de Wegener (GW) é uma vasculite sistêmica de etiologia idiopática. É caracterizada por uma inflamação granulomatosa necrotizante, vasculite pauci-imune dos vasos de pequeno e médio calibres com compromisso predominantemente das vias respiratórias e dos rins. Está associada à presença de anticorpos anticitoplasma dos neutrófilos (ANCAs). O objetivo deste estudo foi a descrição de 2 casos de GW, salientando a importância do diagnóstico e terapêutica precoces para o prognóstico. RELATO DOS CASOS: Caso 1: Paciente do sexo feminino, 69 anos, com insuficiência renal rapidamente progressiva, glomerulonefrite pauci-imune com granulomas na biópsia renal e com positividade para o ANCA antimieloperoxidase (MPO). Caso 2:Paciente do sexo feminino, 26 anos, com otite refratária aos antibióticos, nódulos pulmonares e ANCA antiproteinase 3 (PR3)positivo. Foram tratadas com imunossupressores, mas em ambashouve lesão irreversível de órgãos (insuficiência renal terminal esurdez grave, respectivamente). CONCLUSÃO: A GW é uma doença pouco frequente, cujo diagnóstico permanece um desafio clínico. Este assenta na presença de sintomas constitucionais, compromisso das vias aéreas e renal, inflamação granulomatosa necrotizante e vasculite na histologia e presença de ANCAs. O tratamento baseia-se na imunossupressão. Salienta-se que a brevidade na determinação do diagnóstico e consequentemente no inicio da terapêutica é vital para o desfecho dos casos, reduzindo as sequelas irreversíveis e a morte.

BACKGROUND AND OBJECTIVES: Wegener's granulomatosis (WG) is a systemic vasculitis of unknown etiology. Characterized by necrotizing granulomatous inflammation and pauciimmune vasculitis of the small and medium-sized blood vessels affecting mainly the upper and lower respiratory tract and the kidneys. It's associated with antineutrophil cytoplasmic antibodies (ANCAs). The purpose of this study is to describe two clinical cases of WG emphasizing the importance of an early diagnosis and treatment in the prognosis.CASE REPORTS: Case 1: Female patient of 69 year-old, with rapidly progressive renal failure, pauci-immune glomerulonephritiswith granulomas in the renal biopsy and ANCA antimyeloperoxidase (MPO-ANCA). Case 2: Female patient of 26year-old with otitis media non responsive to antibiotics, pulmonary nodules, and ANCA directed against proteinase 3 (PR3).The patients were treated with immunosuppressive therapy, bothof them irreversible organ damage (renal failure and deafness). CONCLUSION: A WG it's an uncommon disease, which remains a clinical challenge. The diagnosis is based in the presence of constitutional symptoms, upper and lower airways and kidney involvement with necrotizing granulomatous inflammation and vasculitis, and associated with ANCAs. Therapy is based in immunossupression. We emphasize that early diagnosis and treatment is important to the outcome, minimizing irreversible organs damage and death.

Uso de inaladores dosimetrados em pacientes com asma: conhecimentos e efeitos de uma orientação teórico-prática para pediatras / Metered-dose inhaler for asthma patients: knowledge and effects of a theoretical and practical guidance for pediatricians

Objective: To evaluate the effect of a theoretical and practical guidance on knowledge of pediatricians regarding the use of metered dose inhalers with spacers. To identify major deficiencies, correct them and train these physicians on the correct use of the devices. Methods: Pediatricians who participated in a theoreticalpractical program focusing on the use of inhaler devices answered a questionnaire with five questions about the use of these devices before and after the program. A comparison of the scores obtained in the pre- and post-training tests was performed by Wilcoxon test for related samples, and a significance level of 0.05 was adopted. Results: Twenty pediatricians performed pre- and post-training tests. The performance of pediatricians in the post-training test was significantly better than baseline (p<0.001). Conclusion: A brief orientation program for pediatricians significantly improved their knowledge on the use of metered-dose inhalers with spacers, which may translate into an improvement in quality and quantity of prescriptions of these devices in clinical practice. The questions with higher rates of errors in the pre-training test were the questions about the waiting time between two sprays in successive applications and about the correct way to attach the inhaler to the spacer, both with high levels of success in the post-training test.

Objetivo: Avaliar o efeito de uma orientação teórico-prática sobre o conhecimento de pediatras a respeito do uso de inaladores dosimetrados com espaçadores. Detectar as principais deficiências, corrigi-las e capacitar esses médicos para a correta utilizaçãodesses inaladores. Métodos: Pediatras que participaram de um curso teórico-prático a respeito do uso de dispositivos inalatórios responderam a um questionário com cinco perguntas sobre o uso desses dispositivos antes e após o curso. A comparação entre asnotas obtidas no pré e no pós-teste foi feita por meio do teste de Wilcoxon para amostras relacionadas, sendo adotado um nível de significância de 0,05. Resultados: Vinte pediatras realizaram o pré e o pós-teste. O desempenho dos médicos no pós-teste foi significativamente melhor que o inicial (p<0,001). Conclusão: Um breve programa de orientação a pediatras melhorou de forma significativa o conhecimento desses médicos a respeito do uso de inaladores dosimetrados com espaçadores, fato que pode se traduzir em uma melhora na qualidade e na quantidade de prescrições desses dispositivos na prática clínica. As questões com maiores índices de erros no pré-teste foram a relativa ao tempo necessário entre dois jatos em aplicações sucessivas e a que diz respeito à maneira correta de se acoplar o inalador ao espaçador, todas com altos níveis de acertos no pós-teste.

Oxigenoterapia domiciliar prolongada em crianças e adolescentes: uma análise do uso clínico e de custos de um programa assistencial / Long-term home oxygen therapy in children and adolescents: analysis of clinical use and costs of a home care program

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 por cento 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 por cento), displasia broncopulmonar (19 por cento) e bronquiolite obliterante (15 por cento). Dos 33 pacientes que realizaram espirometria, 70 por cento apresentavam distúrbio ventilatório obstrutivo grave. O exame ecocardiográfico foi executado em 134 pacientes; 51 por cento 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 percent 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 percent), bronchopulmonary dysplasia (19 percent), and bronchiolitis obliterans (15 percent). Of the 33 patients who underwent spirometry, 70 percent had severe obstructive lung disease. Echocardiogram was performed in 134 patients; 51 percent 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.

Shwachman-Kulczycki score still useful to monitor cystic fibrosis severity

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 percent (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.

Comparação entre o método clássico de Gibson e Cooke e o teste da condutividade no suor em pacientes com e sem fibrose cística / Comparison between classic Gibson and Cooke technique and sweat conductivity test in patients with and without cystic fibrosis

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 por cento de sensibilidade (IC95 por cento 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 por cento de especificidade (IC95 por cento 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 percent sensitivity (95 percentCI 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 percent specificity (95 percentCI 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.

High rate of viral identification and coinfections in infants with acute bronchiolitis

OBJECTIVES: To determine the viruses and risk factors associated with hospital and intensive care unit (ICU) admissions in infants with acute bronchiolitis. INTRODUCTION: Bronchiolitis is a major cause of morbidity in infants. Widespread use of molecular-based methods has yielded new insights about its etiology, but the impact of viral etiologies on early outcomes is still unclear. METHODS: Seventy-seven infants with bronchiolitis who were under two years of age and visited an emergency unit were included. Using molecular-based methods, samples were tested for 12 different respiratory viruses. Logistic regression models were used to identify clinical and virological variables associated with the main endpoints: hospital admission and ICU admission. RESULTS: We identified at least one virus in 93.5 percent of patients, and coinfections were found in nearly 40 percent of patients. RSV was the most common pathogen (63.6 percent), followed by rhinovirus (39 percent). Identification of RSV was only associated with an increased risk of hospital admission in the univariate model. Younger age and enterovirus infection were associated with an increased risk of hospital admission, while atopy of a first-degree relative showed a protective effect. Prematurity was associated with an increased risk of admission to the ICU. Coinfections were not associated with worse outcomes. CONCLUSIONS: Molecular-based methods resulted in high rates of viral identification but did not change the significant role of RSV in acute bronchiolitis. Younger age and enterovirus infection were risk factors for hospital admission, while prematurity appeared to be a significant risk factor for admission to the ICU in acute viral bronchiolitis.

Gastric volvulus - A review of 38 cases / Volvo gástrico: revisão de 38 casos

BACKGROUND: Gastric volvulus is frequently an asymptomatic disease, and it is usually diagnosed during radiographic examination of the superior digestive tract. The acute form, however, can spawn serious and lethal clinical consequences. This disease is defined by the anomalous rotation of the stomach over itself, and it can be classified according to type, extension, direction, etiology, and clinical presentation. AIM: To review the records from 38 patients with gastric volvulus diagnosed in the Hospital das Clínicas of University of São Paulo between 1968 and 2001. METHODS: This is retrospective analysis of 38 patient records. It was collected from each patient: name, age of first symptom appearance, gender, main clinical findings and complementary exams, volvulus type, extension, direction, etiology, and clinical presentation, therapeutic procedures, type of surgery performed, eventual recurrence, and long-term evolution. RESULTS: It was observed that occurrences of gastric volvulus are mainly secondary (75.8%). For the majority of patients (n=33), surgery was chosen as the treatment option: chronic disease in 29 cases and acute in four. Conservative treatment was reserved only for patients with no clinical conditions to surgical treatment. Anterior gastropexy was associated to high recurrence rates. Suturing the low gastric curve to the hepatic capsule and the transverse colon to the left subphrenic space (Tanner´s operation) seemed to be the technical treatment of choice for primary gastric volvulus. CONCLUSION: Treatment of gastric volvolus must be tailored according the etiology of the disease.

RACIONAL: O volvo gástrico é frequentemente condição assintomática e diagnosticado em exame radiológico feito por outras causas. A forma aguda, no entanto, pode ter consequências graves e letais. Ele é definido como rotação anômala do estômago nele próprio e classificado de acordo com o tipo, extensão, direção, causa e apresentação clínica. OBJETIVO: Apresentar aspectos clínicos e morfológicos de volvos gástricos. em 38 pacientes. MÉTODOS: Análise retrospectiva onde foram coletadas informações registradas sobre 38 pacientes a cerda da idade, surgimento do primeiro sintoma, gênero, principais achados clínicos, de exames complementares, tipo do volvo, causa, procedimentos terapêuticos, recidivas e evolução tardia. RESULTADOS: Em 75,8% o volvo foi secundário Na maioria dos pacientes a opção de tratamento foi cirúrgica. Ele era crônico em 29 e agudo em quatro pacientes. O tratamento conservador foi indicado somente aos sem condições clínicas para operações. Gastropexia anterior foi associada à alta taxa de recidiva. A sutura da pequena curvatura gástrica à cápsula hepática e cólon transverso na fossa subfrência esquerda (operação de Tanner) parece ser o tratamento de escolha nos volvos primários. CONCLUSÃO: O tratamento do volvo gástrico deve ser feito sob medida caso a caso e de acordo com a causa da doença.