Abstract Introduction: Due to lack of data, the management of air leaks in children and adolescents is based on protocols for adults. In this study, the authors review and report their institutional experience in the area. Methods: Retrospective and descriptive study of spontaneous air leak cases diagnosed in a Portuguese Pediatric Emergency Department (PED) between January 2007 and December 2018. Results: Twenty-one episodes of spontaneous air leak were diagnosed in 16 patients (87.5% male), with a mean (± standard deviation) age of 14.3 (± 5.1) years. Eighteen cases of spontaneous pneumothorax were reported, fifteen of which primary (eleven first-time episodes and four recurrences) and three secondary (two first-time episodes and one recurrence) to asthma. Three cases of pneumomediastinum with subcutaneous emphysema were identified, two of which related to infection. Smoking habits and/or recent physical activity were major triggers. Pleuritic chest pain was the most frequent symptom on admission. Conservative treatment was the only approach used in six cases. Most cases required thoracic drainage, five of which required surgical intervention. Discussion: This study shows similar demographic features, triggers, and clinical presentation to those reported in the literature for these cases. The management of the condition was based on recommendations established for adults. Conclusion: Although spontaneous air leak is an uncommon condition, it is a reality in PED. Prospective studies in pediatric age are required to develop adequate recommendations for children and adolescents.

Resumo Introdução: Devido à escassez de dados em idade pediátrica, a abordagem da síndrome de fuga de ar em crianças e adolescentes baseia-se em protocolos estabelecidos para adultos. Neste estudo, os autores descrevem a sua experiência institucional na área. Métodos: Estudo retrospetivo descritivo de episódios espontâneos de síndroma de fuga de ar diagnosticados num Serviço de Urgência Pediátrico (SUP) em Portugal entre 1 de janeiro de 2007 e 31 de dezembro de 2018. Resultados: Foram diagnosticados vinte e um episódios de síndroma de fuga de ar em dezasseis crianças, 87.5% das quais do género masculino, com uma idade média (± desvio padrão) de 14,3 (± 5,1) anos. Foram reportados 18 casos de pneumotórax espontâneo, quinze dos quais primários (onze primeiros episódios e quatro recorrências) e três secundários (dois primeiros episódios e uma recorrência) a asma. Foram contabilizados três episódios de pneumomediastino com enfisema subcutâneo, dois dos quais secundários a infeção. A exposição ativa a fumo de tabaco e/ou esforço físico recente foram fatores etiopatogénicos preponderantes na maioria dos casos. Dor torácica pleurítica foi o sintoma mais frequente na apresentação clínica. A terapêutica conservadora foi a única abordagem utilizada em seis casos, com os restantes a necessitar de drenagem transtorácica. Cinco episódios exigiram intervenção cirúrgica. Discussão: Este estudo evidencia fatores demográficos e etiopatogénicos e apresentação clínica semelhantes aos descritos na literatura para estes casos. Relativamente à abordagem, foram seguidas as recomendações existentes para a idade adulta. Conclusão: Apesar de pouco frequente, a síndrome de fuga de ar é uma realidade no SUP. São necessários estudos prospetivos especificamente em idade pediátrica para permitir o desenvolvimento de recomendações adequadas às particularidades desta faixa etária.

RESUMO Para avaliar o impacto do ensino da técnica inalatória no controle clínico e funcional de pacientes com asma ou DPOC, incluíram-se 44 participantes antes e após essa intervenção. Houve uma diminuição significativa no número de erros cometidos, sendo que 20 pacientes (46%) melhoraram significativamente sua técnica na expiração prévia e apneia final. No grupo asma, houve significativa melhora nas médias de FEV1 (6,4%), CVF (8,6%) e PFE (8,3%), e essa melhora correlacionou-se com os resultados no Control of Allergic Rhinitis and Asthma Test, mas não com os do Asthma Control Test. No grupo DPOC, não houve variações significativas. O ensino da técnica inalatória parece melhorar seu desempenho e os controles clínico e funcional em pacientes com asma.

ABSTRACT To assess the impact that educational interventions to improve inhaler techniques have on the clinical and functional control of asthma and COPD, we evaluated 44 participants before and after such an intervention. There was a significant decrease in the number of errors, and 20 patients (46%) significantly improved their technique regarding prior exhalation and breath hold. In the asthma group, there were significant improvements in the mean FEV1, FVC, and PEF (of 6.4%, 8.6%, and 8.3% respectively). Those improvements were accompanied by improvements in Control of Allergic Rhinitis and Asthma Test scores but not in Asthma Control Test scores. In the COPD group, there were no significant variations. In asthma patients, educational interventions appear to improve inhaler technique, clinical control, and functional control.

OBJECTIVE: To evaluate the prognostic value of platelet counts in acute kidney injury patients requiring renal replacement therapy. METHODS: This prospective cohort study was performed in three tertiary-care hospitals. Platelet counts were obtained upon admission to the intensive care unit and during the first week of renal replacement therapy on days 1, 3, 5 and 7. The outcome of interest was the hospital mortality rate. With the aim of minimizing individual variation, we analyzed the relative platelet counts on days 3, 5, 7 and at the point of the largest variation during the first week of renal replacement therapy. Logistic regression analysis was used to test the prognostic value of the platelet counts. RESULTS: The study included 274 patients. The hospital mortality rate was 62%. The survivors had significantly higher platelet counts upon admission to the intensive care unit compared to the non-survivors [175.5×103/mm3 (108.5-259×103/mm3) vs. 148×103/mm3 (80−141×103/mm3)] and during the first week of renal replacement therapy. The relative platelet count reductions were significantly associated with a higher hospital mortality rate compared with the platelet count increases (70% vs. 44% at the nadir, respectively). A relative platelet count reduction >60% was significantly associated with a worse outcome (mortality rate = 82.6%). Relative platelet count variations and the percentage of reduction were independent risk factors of hospital mortality during the first week of renal replacement therapy. CONCLUSION: Platelet counts upon admission to the intensive care unit and at the beginning of renal replacement therapy as well as sequential platelet count evaluation have prognostic value in acute kidney injury patients requiring renal replacement therapy.

As próteses na via aérea têm a função de manter as estruturas tubulares abertas e estáveis. A sua colocação está indicada essencialmente na obstrução intrínseca ou compressão extrínseca da via aérea, fístulas ou traqueobroncomalacia. Com este estudo pretendeu-se determinar a tolerabilidade a eficácia de próteses na via aérea nas situações em que a sua colocação era imprescindível. Procedeu-se ao estudo retrospectivo dos processos clínicos de 23 doentes em que se tinha procedido à colocação de próteses traqueobrônquicas através de broncoscopia rígida, durante dois anos consecutivos (2006-2007), na Unidade Funcional de Técnicas de Diagnóstico e Terapêutica, tendo sido avaliadas a indicação, a eficácia, a tolerabilidade, as complicações e a localização exacta da sua inserção, tendo em conta a informação imagiológica fornecida por TC do tórax. Em todas as situações foram utilizadas próteses flexíveis de silicone tipo Dumon (Tracheobronxane®), sendo previamente avaliada, através de broncoscopia flexível, a necessidade de técnicas complementares, nomeadamente laserterapia e dilatação mecânica. O estudo efectuado permitiu concluir que a inserção de próteses não apresentou complicações, demonstrando boa tolerabilidade, tendo em conta a maioria das situações, de natureza neoplásica em estádio avançado, apenas com indicação terapêutica paliativa.

The function of airway stents is to keep the tubular structures open and stable. Their insertion is essentially indicated for intrinsic obstruction or extrinsic compression of the airway, fistulae or tracheobronchomalacia. The aim of this study was to determine the tolera bility and efficacy of airway stents in situations in which their insertion was vital. A retrospective study of airway stent insertion with rigid bronchoscopy (23 patients) was carried out over a two year period (2006-2007) at the Diagnostic and Therapeutic Techniques Unit. We assessed indication, efficacy, tolerability, complications and exact insertion based on chest CT imaging. In all situations Dumon flexible silicon stents (Tracheobronxane ®) were used, with the need for complementary techniques such as laser therapy and mechanical dilation having been previously evaluated by flexible bronchoscopy. The authors conclude that stent insertion has no complications and good tolerability in the majority of advanced stage oncological situations with indication for palliative management.

O hemangioendotelioma epitelióide (HEE) é um tumor de origem vascular, de grau intermédio de malignidade, cuja apresentação primária na pleura é rara. Pelo reduzido número de casos descritos na literatura, os autores apresentam o caso de uma doente de 65 anos, que recorreu ao serviço de urgência por toracalgia à direita, com sete meses de evolução, na sequência de um traumatismo torácico. Radiologicamente apresentava sinais sugestivos de derrame pleural direito de volume moderado. A biópsia pleuropulmonar, efectuada por toracotomia, permitiu estabelecer o diagnóstico histológico de HEE da pleura. Atendendo ao estádio localmente avançado do tumor, a doente iniciou quimioterapia com carboplatina e etoposido, tendo falecido 6 meses após o diagnóstico. Constata-se, com este caso, que a manifestação pleural do HEE tem um comportamento agressivo, semelhante ao do angiossarcoma, com uma sobrevida de apenas alguns meses após o diagnóstico.

Epithelioid Hemangioendothelioma (EHE) is a vascular tumour with rare pleural presentation. As there are a small number of cases described in the literature the authors present the case of a 65-year old woman, who was admitted to the Emergency Department for a right-sided chest pain, which progressed over 7 months, after a thoracic trauma. The chest X-ray showed signs of a moderate right-sided pleural effusion. A pleural-pulmonary biopsy carried out by toracotomy established the histological diagnosis of EHE of the pleura. Due to the locally advanced stage of the tumour, chemotherapy with carboplatin and etoposide was prescribed and the patient died 6 months later. This case confirms that pleural EHE has an aggressive behaviour, similar to an angiossarcoma, with a median survival of only a few months after diagnosis.

A linfangioleiomiomatose (LAM) é uma doença rara, de etiologia desconhecida, caracterizada pela proliferação anormal de células musculares lisas nas regiões perilinfática, perivascular e peribrônquica. A LAM pode ocorrer esporadicamente ou associada ao complexo esclerose tuberosa (CET) e hamartose hereditária multiorgânica. Em ambas as situações a LAM afecta principalmente mulheres jovens em idade fértil, sendo que aproximadamente 1/3 das mulheres com CET têm LAM. A propósito desta patologia, os autores elaboram uma revisão da literatura e descrevem os casos clínicos de três doentes do sexo feminino com o diagnóstico de LAM com base nos achados clínicos e imagiológicos.

Pulmonary lymphangioleiomyomatosis (LAM) is a rare disease of unknown aetiology. It is characterized by proliferation of abnormal smooth-muscle cells throughout the peribronchial, perivascular and perilymphatic regions of the lung. LAM may occur sporadically, in association with tuberous sclerosis complex (TSC) or inheritable multiorgan hamartomatosis. In either situation, LAM occurs almost exclusively in women of reproductive age, and approximately one third of the patients with TSC have LAM. The authors review the cases of three female patients diagnosed with LAM based on clinical and radiological findings. A brief review of the disease is then presented.

A ectopia de tireoide é rara, e a sua localização no mediastino anterior é excepcional, estando descritos apenas 5 casos nos últimos 30 anos. Os autores apresentam 2 casos clínicos, além de uma revisão da literatura abordando a etiologia, a embriologia e manifestações clínicas de ectopia de tireoide.

Ectopic thyroid is a rare condition, and its location in the anterior mediastinum is even rarer, there having been only 5 reported cases in the past 30 years. Here, we describe 2 clinical cases and present a review of the literature regarding the etiology, embryology and clinical manifestations of ectopic thyroid.

As pneumonias eosinofílicas constituem um grupo heterogéneo de quadros clínicos que podem comprometer unicamente as vias aéreas, o parênquima pulmonar, ou ambos, caracterizado pela presença de eosinofilia alveolar e infiltrados pulmonares, com ou sem evidência de eosinofilia no sangue periférico¹. A pneumonia eosinofílica crónica idiopática (PECI), descrita por Carrington5 em 1969, é uma doença pulmonar eosinofílica rara, de etiologia desconhecida, caracterizada por eosinofilia periférica, infiltrados pulmonares na telerradiografia torácica e excelente resposta à corticoterapia. A PECI afecta geralmente mulheres de meia idade, sendo os sintomas mais comuns a tosse, a dispneia, a febre e a perda de peso ². Os autores apresentam um caso de PECI numa mulher jovem, de 21 anos, raça caucasiana, não fumadora e sem antecedentes patológicos relevantes.

The eosinophilic pneumonias are a heterogeneous group of pulmonary disorders, which may compromise only the air ways, the pulmonary parenchyma, or both, characterised by alveolar eosinophils and infiltration of pulmonary tissue, with or without peripheral blood eosinophilia¹. Idiopathic Chronic Eosinophilic Pneumonia (ICEP), detailed description was by Carrington5 in 1969, is a rare eosinophilic lung disease, of unknown aetiology, characterised by peripheral blood eosinophilia, chest radiograph infiltrates and prompt response to corticosteroid therapy. ICEP most commonly affects women of middle age and usual symptoms are cough, dyspnea, fever and weight loss². The authors present a case of ICEP in a young woman, 21 years old, non -smoker and previously healthy.

JUSTIFICATIVA E OBJETIVOS: Não existe consenso sobre qual modelo prognóstico deva ser utilizado em pacientes com disfunção renal aguda (DRA). O objetivo deste estudo foi avaliar o desempenho de seis escores de prognóstico em pacientes que necessitaram de suporte renal. MÉTODO: Coorte prospectiva de pacientes internados nas unidades de terapia intensiva (UTI) de três hospitais terciários que necessitaram de suporte renal por DRA durante 32 meses. Foram excluídos os pacientes crônicos em programa de diálise ou com < 24h de internação na UTI. Os dados das primeiras 24h de UTI foram utilizados no cálculo do SAPS II e do APACHE II, e os dados das primeiras 24h de suporte renal foram utilizados no cálculo dos escores LOD, ODIN, Liaño e Mehta. A discriminação foi avaliada através da área sobre a curva ROC (AUROC) e a calibração através do teste do goodness-of-fit de Hosmer-Lemeshow. A letalidade hospitalar foi o desfecho de interesse. RESULTADOS: Quatrocentos e sesseta e sete pacientes foram incluídos e a letalidade hospitalar foi 75%. Os valores dos escores SAPS II, APACHE II e LOD foram 48,5 ± 11,2, 27,4 ± 6,3, 7 (5-8) pontos, respectivamente. A calibração foi adequada para todos os escores, com exceção do Mehta (p = 0,001). Entretanto, a discriminação foi ruim para todos os modelos, com AUROC variando entre 0,60 para o ODIN e 0,72 para o SAPS II e Mehta. Com exceção do Mehta, todos os modelos subestimaram a letalidade. CONCLUSÕES: Todos os seis modelos estudados foram inadequados na predição prognóstica de pacientes graves com DRA e necessidade de suporte renal.

BACKGROUND AND OBJECTIVES: There is no consensus about prognostic scores for use in patients with acute kidney injury (AKI). The aim of this study was to evaluate the performance of six prognostic scores in predicting hospital mortality in patients with AKI and need for renal replacement therapy (RRT). METHODS: Prospective cohort of patients admitted to the intensive care units (ICU) of three tertiary care hospitals that required RRT for AKI over a 32-month period. Patients with end-stage renal disease and those with ICU stay < 24h were excluded. Data from the first 24h of ICU admission were used to calculate SAPS II and APACHE II scores, and data from the first 24h of RRT were used in the calculation of LOD, ODIN, Liaño and Mehta scores. Discrimination was evaluated using the area under ROC curve (AUROC) and calibration using the Hosmer-Lemeshow goodness-of-fit test. The hospital mortality was the end-point of interest. RESULTS: 467 patients were evaluated. Hospital mortality rate was 75%. Mean SAPS II and APACHE II scores were 48.5 ±11.2 and 27.4 ± 6.3 points, and median LOD score was 7 (5-8) points. Except for Mehta score (p = 0.001), calibration was appropriate in all models. However, discrimination was uniformly unsatisfactory; AUROC ranged from 0.60 for ODIN to 0.72 for SAPS II and Mehta scores. In addition, except for Mehta, all models tended to underestimate hospital mortality. CONCLUSIONS: Organ dysfunction, general and renal-specific severity-of-illness scores were inaccurate in predicting outcome in ICU patients in need for RRT.

INTRODUCTION: Acute kidney injury usually develops in critically ill patients in the context of multiple organ dysfunctions. OBJECTIVE: To evaluate the effect of changes in associated organ dysfunctions over the first three days of renal replacement therapy on the outcomes of patients with acute kidney injury. METHODS: Over a 19-month period, we evaluated 260 patients admitted to the intensive care units of three tertiary-care hospitals who required renal replacement therapy for > 48 h. Organ dysfunctions were evaluated by SOFA score (excluding renal points) on the first (D1) and third (D3) days of renal replacement therapy. Absolute (A-SOFA) and relative (D-SOFA) changes in SOFA scores were also calculated. RESULTS: Hospital mortality rate was 75%. Organ dysfunctions worsened (A-SOFA>0) in 53%, remained unchanged (A-SOFA=0) in 17% and improved (A-SOFA<0) in 30% of patients; and mortality was lower in the last group (80% vs. 84% vs. 61%, p=0.003). SOFA on D1 (p<0.001), SOFA on D3 (p<0.001), A-SOFA (p=0.019) and D-SOFA (p=0.016) were higher in non-survivors. However, neither A-SOFA nor D-SOFA discriminated survivors from non-survivors on an individual basis. Adjusting for other covariates (including SOFA on D1), A-SOFA and D-SOFA were associated with increased mortality, and patients in whom SOFA scores worsened or remained unchanged had poorer outcomes. CONCLUSIONS: In addition to baseline values, early changes in SOFA score after the start of renal replacement therapy were associated with hospital mortality. However, no prognostic score should be used as the only parameter to predict individual outcomes.