Revista Portuguesa de Pneumologia (English Edition)最新文献

Background

An outbreak of Legionella pneumophila serogroup 1, with 403 cases was identified on the 7th November 2014 in Vila Franca de Xira, Portugal. Outbreak source was the wet cooling system of a local factory. Hospital Pulido Valente was one of the hospitals receiving patients with Legionnaires’ disease (LD).

Methods

We describe the clinical findings and diagnostic methods used among the 43 confirmed or probable cases admitted to our department.

Results

60.5% were male, mean age was 56.1 ± 13.5 years and tobacco smoking was the most frequent risk factor (76.7%). All patients had fever, 62.8% ≥39.5 °C, 72.1% had chills and myalgia/arthralgia and 62.8% had dry cough. Extra pulmonary symptoms were frequent: confusion and headache occurred in 34.9% and gastrointestinal symptoms in 20.9%.

High C-Reactive Protein (55.8% ≥30 mg/dL) and hyponatremia (62.8%) were the laboratorial abnormalities most commonly found. Hypoxemia occurred in 55.8% and hypocapnia in 93%. Urinary Antigen Test (UAT) was positive in 83.7% of the cases.

Conclusions

Although not specific, a combination of risk factors, symptoms and laboratory findings can be highly suggestive of LD, even in an outbreak. This should prompt diagnosis confirmation. Routine use of UAT in less severe cases of community acquired pneumonia might contribute to earlier diagnosis.

Background

Pulmonary arterial hypertension (PAH) is a progressive, fatal disease. Long-term outcomes data are scarce in Portugal. We aimed to estimate survival of newly diagnosed PAH at a Portuguese referral center in the modern management era.

Methods

Between January 2009 and November 2015 all incident PAH cases were consecutively enrolled in a prospective cohort study. Sixty-five patients were followed up for a median of 3.1 [interquartile range 1.7–5.4] years. Kaplan–Meier survival analysis was used to estimate 1-, 3-, and 5-year survival and to compare it with a historical PAH survival estimated from the NIH cohort.

Results

Mean age was 48 ± 19 years with female preponderance (68%). The most common PAH subgroup was congenital heart disease (PAH-CHD) (n = 31; 48%), followed by connective tissue disease (PAH-CTD) (n = 16; 25%), idiopathic (IPAH) (n = 8; 12%) and hereditary (HPAP) (n = 1; 1.5%). BNP values (hazard ratio [HR] 2.07; 95%CI 1.34–3.22; P = 0.001) and male gender [HR 4.34 (1.44–13.09); P = 0.009] were predictors of death. Survival rates at 1-, 3- and 5-years were 95%, 77% and 71%. Survival was not statistically different between PAH etiologies (Log-rank P = 0.7). However, PAH-CHD was associated with a decreased risk of the combined endpoint of all-cause mortality and admission for decompensated heart failure [HR 0.36 (0.15–0.85); P = 0.02]. We found a non-significant numerically higher survival of incident IPAH, HPAH and DPAH patients in comparison with the historical NIH cohort.

Conclusions

In this cohort of incident PAH patients, PAH-CHD patients had better overall prognosis. Higher BNP values and male gender were associated with higher mortality.

A 57-year old woman underwent lung transplantation for non-specific interstitial pneumonia. Primary graft dysfunction was diagnosed requiring continued use of extracorporeal membrane oxygenation (ECMO). Within three days she developed recurring hemothoraces requiring two surgical evacuations. After ECMO removal a series of complications occurred within four months: femoral thrombosis, persisting tachycardic atrial fibrillation, pneumopericardium with an esophagopericardial fistula and purulent pericarditis, septic shock, multiorgan failure and intracerebral hemorrhage with ventricular involvement requiring external ventricular drainage. Interdisciplinary management coordinated by the intensive care specialist, transplant surgeon and pulmonologist with various interventions by the respective specialists followed by intensive physical rehabilitation allowed for discharge home on day 235 post transplant. Subsequently quality of life was considered good by the patient and family.