Teto terapêutico e a adequação do tratamento no Serviço de Urgência: estudo retrospectivo / Ceilings of treatment and treatment adequacy in the Emergency department: retropective study

Introdução: no Serviço de Urgência vive-se um antagonismo constante pela sua natureza direcionada para a patologia aguda e a prestação de cuidados paliativos de qualidade. O nosso estudo tem como objetivo avaliar se a definição de teto terapêutico leva a diferenças na adequação da marcha diagnóstica e terapêutica instituída. Material e métodos: análise retrospetiva descritiva monocêntrica dos doentes que morreram nos primeiros seis meses de 2018 no serviço de urgência do Hospital do Espírito Santo de Évora. Resultados: compararam-se os três grupos de doentes o que não foi definido qualquer teto terapêutico, com o grupo em que iniciaram medidas paliativas e o grupo em que se tomou a Decisão de Não Reanimar. Verificou-se que não existem diferenças significativa entre as idades, o local de residência e as comorbilidades e, com exceção da demência (p= 0,006), existe sim uma diferença no grau de dependência nas atividades da vida diária (p<0,001). Verificou-se que não existem diferenças entre número ou tipo de exames complementares de diagnóstico, mas há algumas diferenças na terapêutica instituída já que no grupo dos doentes em cuidados paliativos a terapêutica com morfina (p<0,001), butilescopolamina (p=0,001) e paracetamol (p=0,004) foi mais frequente. A ventilação invasiva só ocorreu no grupo de doentes sem definição de teto terapêutico (p<0,001), enquanto a oxigénioterapia foi mais frequente nos grupos em Decisão de Não Reanimar ou em cuidados paliativos (p<0,001). Discussão e conclusão: os médicos do serviço de urgência reconhecem que os seus doentes estão em final de vida, adequando parcialmente a terapêutica com vista ao controlo de sintomas, dor e secreções.

Introduction: the Emergency Department is in a constant state of antagonism due to its nature towards the acute pathology, and quality of palliative care. Our study aims to assess whether the definition of a therapeutic ceiling leads to differences in the adequacy of the diagnostic methods and therapy instituted. Material and methods: retrospective descriptive monocentric analysis of patients who died the first 6 months of 2018 in the emergency department of Hospital do Espírito Santo de Évora. Results: the three groups of patients were compared, one without any therapeutic ceiling defined, with the group in which they started palliative measures and the group in which the Decision to Not Resuscitate was made. There were no significant differences between ages, place of residence and comorbidities except for dementia (p=0.006), but there is a difference in the degree of dependence on activities of daily living (p<0.001). It was found that there are no differences between the number or type of complementary diagnostic tests, but there are some differences in the therapy instituted since in the group of patients in palliative care, therapy with morphine (p<0.001), butylscopolamine (p=0.001) and paracetamol (p=0.004) was more frequent. Invasive ventilation only occurred in the group of patients without therapeutic ceiling (p<0.001), while oxygen therapy was more frequent in Decision to Not Resuscitate or in palliative care groups (p<0.001). Discussion and conclusion: the doctors of the emergency department recognize that their patients are at the end of their lives, partially adapting the therapy to control symptoms, pain and secretions.

Discrepancia entre SpO2 y SaO2: diagnóstico de cuatro generaciones / From SpO2 and SaO2 discrepancy to diagnosing four generations

Pulse oximetry measures the peripheral oxy-haemoglobin saturation (SpO2) which is a surrogate marker for arterial oxy-haemoglobin saturation (SaO2). SaO2 estimation is subjected to both oximeter proper functioning, patient characteristicsand haemoglobin disturbances. A 82-year-old man goes to the emergency with cough, dyspnoea and fever. He has haemolytic anaemia. His kids also have anaemia. Examination showed fine crackles in pulmonary auscultation of the lower two thirds of the right lung and splenomegaly. SpO2 was 80% (FiO2 21%). Arterial blood gas analysis: pH 7.514; PaCO2 23.4 mmHg; PaO2 43.2 mmHg; Hb 13.0 g/dL. Chest X-ray suggested an infectious process. He was admitted to the hospital with the diagnosis of pneumonia. During hospitalization we verify discrepancy between SpO2 and SaO2; haemolytic anaemia. The patient had a respiratory improvement and was discharged to external consult, dying months later. To clarify the discrepancy between SpO2 and SaO2 results; confirm the hereditary nature and identify the haemolytic anaemia, we conducted a retrospective familiar study based on the patient’s clinical processes. Three children were identified with anaemia. Two of the children have known their anaemia for 35 years - studied in the context of respiratory infections with haemolytic crisis due to Lepore haemoglobinopathy and β thalassemia, respectively. The patient previously diagnosed with Lepore haemoglobinopathy, currently undergoing hospital anaemia study, was diagnosed with Köln Hb. The discrepancy between SpO2 and SaO2 in association with a familiar haemolytic anaemia resulted in the diagnosis of autosomal dominant Köln haemoglobinopathy. The advances in the means of diagnosis enabled the probable diagnosis of 19 family members distributed over 4 generations. (AU)

A new dysferlin gene mutation in a Portuguese family with Miyoshi myopathy.

Dysferlinopathies are autosomal recessive muscular dystrophies caused by mutations in the dysferlin gene (DYSF). A 33-year-old man was born to a non-consanguineous couple. At the age of 25 he stared to feel weakness of the distal lower limbs and also experienced episodes of rhabdomyolysis. Electromyography showed a myopathic pattern, and muscle biopsy revealed dystrophic changes with absence of dysferlin. Genetic analysis was positive for a mutation in the c3367_3368del DYSF gene (p.Lys1123GLUFS*2). After 8 years of disease evolution the symptomatology worsened. This is the first report of this mutation of the DYSF gene identified in a non-consanguineous Portuguese family, studied over 8 years. We believe the mutation is responsible for the Miyoshi myopathy. Disease progression cannot be predicted in either the patient or carrier family because there are no similar cases previously described in the literature.

Aortitis as a Harbinger of Esophageal Cancer.

Aortitis is a rare diagnosis that requires a high index of suspicion due to nonspecific symptoms and its multiplicity of etiologies. An 80-year-old man, independent in activities of daily living (ADLs), had three consecutive hospitalizations in three months for fever, general malaise, anorexia associated with arthritis of the hands and feet with the inability to walk. Inflammatory markers were increased without a focus of infection identified. Upper digestive endoscopy (UDE), colonoscopy, blood cultures, thoracoabdominal-pelvic CT and transthoracic (TT) echocardiogram were performed without changes, with discharge for consultation after demonstrating apyrexia. At the patient second hospitalization for fever and arthritis, a transesophageal echocardiogram was performed that showed the presence of multiple complex atherosclerotic plaques, with associated thrombi in all segments of the aorta with a suspicious mass of vegetation on the aorta. Thoracic-abdominopelvic CT demonstrated calcified atheromatosis of the entire aorta with para-aortic nodes; MRI showed aortic thickening; and autoimmunity study was negative. Aortitis was the working diagnosis of possible infectious etiology and anticoagulation and antibiotic therapy were started. Fever recurred and a third admission led to a working diagnosis of inflammatory, non-infectious aortitis. The patient responded well to empiric corticosteroids. The patient followed up in consultation, remained asymptomatic under a low dose of corticosteroids with negative temporal artery biopsy. In the sixth month, he repeated UDE due to dysphagia, which showed the presence of esophageal neoformation with the histological diagnosis of squamous cell carcinoma, maintaining on the CT as alterations in the aorta. This is an unusual case of aortitis associated with arthritis with improvement after corticosteroids, which interestingly occurred before the progression of esophageal cancer. In hindsight, we think this may have been a large vessel paraneoplastic vasculitis that preceded the detection of esophageal squamous cell carcinoma.

Organizing Pneumonia After Exposure to Sodium Hypochlorite: A Case Report.

Organizing pneumonia (OP) is a rare inflammatory lung disease with a difficult diagnosis and sparse mentions in the literature. In most cases, the etiology is unknown but may be associated with infections, systemic disorders, exposure to industrial toxins and environmental pollutants, or even drug toxicity. This report describes a 77-year-old male who presented to the hospital with nonproductive cough, myalgias, fever, and progressive weight loss after prolonged exposure to sodium hypochlorite. The patient was treated with multiple courses of antibiotics with no pathogen isolation. Chest CT revealed condensation foci of all pulmonary lobes. His clinical history, laboratory results, and CT images led to the diagnosis of OP, which was confirmed with a transbronchial lung biopsy. The patient was treated with oral prednisolone with clinical improvement and discharge one week after the beginning of the corticosteroid treatment. OP diagnosis can be challenging and, if not considered, may lead to a delay in providing appropriate treatment to the patients, which can often lead to a prolonged hospital stay and poor outcomes.

[Stroke in HIV-infected patients]. / Acidente vascular cerebral em doentes infectados com o vírus da imunodeficiência humana.

The spectrum of human immunodeficiency virus infection (HIV) is changing. New drug treatments have reduced morbidity and mortality of this disease, therefore it is necessary to start treating the HIV infection as a chronical disease. The association of the stroke with the HIV infection was inicially thought to be a result of other opportunistic infeccions and tumors. However, the vascular disease associated with HIV infection has been a subject of research and debate. New evidence shows that the vascular diseases could be a threat for the pacients doing highly active antirretroviral therapy (HAART). In this paper, we review the association between the HIV infection and stroke. Furthermore, we have done an analysis of the risk for the stroke on pacients with HIV infection considering the changes of the infection spectrum by the introduction of HAART.