Exploring disease perception in Behçet's syndrome: combining a quantitative and a qualitative study based on a narrative medicine approach.

BACKGROUND: Behçet Syndrome (BS) has a significant psychological and social impact on patients, caregivers and families. The present study aims at exploring disease perception in BS patients, using both a co-designed survey and the narrative medicine (NM) approach. METHODS: An ad-hoc questionnaire was co-designed by clinicians expert in BS, BS patients and caregivers and BS adult patients were invited to answer the online questionnaires. Cluster analysis was used to analyse data from the survey and to identify groups of patients with diverse disease perception. To further explore real-life perspectives, the stories of illness of a smaller group of adult BS patients were anonymously collected online and analysed by means of text, sentiment and qualitative analysis. RESULTS: Two hundred and seven patients answered the survey and forty-three stories were collected. The cluster analysis highlighted that accepting or not the disease has a strong impact on the daily life, on how BS patients perceive themselves and in terms of hope for the future. The stories revealed that patients often address common issues, such as the long and complex journey faced from the disease onset until the BS diagnosis, which was strongly connected to the concept of time and perceived as an exhausting period of their lives. CONCLUSION: To our knowledge, this is the first study that addressed disease perception also applying the NM principles in BS. The current perception that BS patients have of their disease should encourage the BS scientific and patient community in joining forces in order to improve the journey of BS patients.

Exhaled nitric oxide in systemic sclerosis: relationships with lung involvement and pulmonary hypertension.

OBJECTIVE: To measure nitric oxide (NO) concentration in exhaled air of patients with systemic sclerosis (SSc) and to investigate its relationships with lung involvement, complicated or not by pulmonary hypertension (PH). METHODS: Exhaled NO was measured by chemiluminescence in 47 patients with SSc (16 with PH) and in 30 controls. All the patients underwent Doppler echocardiography to assess pulmonary artery pressure (PAP), lung function tests, and thin section computed tomographic scans of the lung to quantify the extent of fibrosing alveolitis. RESULTS: Exhaled NO levels were higher in patients with SSc (16.6 +/- 9.1 ppb), particularly those with interstitial lung disease (ILD) (18.3 +/- 10.4 ppb), compared to controls (9.9 +/- 2.9 ppb; p < 0.0001). In patients with PH, exhaled NO was less than in patients without PH (10.7 +/- 5.9 vs 19.6 +/- 9 ppb, respectively; p < 0.001), and patients with PH without ILD had even lower exhaled NO than patients with PH and ILD (6.6 +/- 1.1 vs 12.6 +/- 6.3 ppb; p = 0.004). There was an inverse correlation between PAP and exhaled NO (r = 04).53, p = 0.004). Exhaled NO was not correlated to age, disease duration, current therapy, or form of disease (limited or diffuse). CONCLUSION: The increased concentration of exhaled NO in patients with SSc may reflect respiratory tract inflammation. The relatively low value of exhaled NO in patients with PH and the negative correlation between PAP and exhaled NO suggest the important role of NO in regulating pulmonary vascular resistance in patients with SSc.

Augmentation of blood platelet levels by intratracheal administration of an adenovirus vector encoding human thrombopoietin cDNA.

This study was designed to evaluate the hypothesis that administration of a replication-deficient, recombinant adenovirus vector to the epithelial surface of the respiratory tract can be used to deliver a recombinant protein to the systemic circulation in sufficient quantities to evoke a systemic response appropriate to the recombinant protein. We administered AdCMV.TPO-an adenovirus vector containing an expression cassette coding for the human thrombopoietin (TPO) cDNA-to the respiratory epithelium of immunocompetent Balb/c mice. Over the following week, serum human TPO levels were elevated, platelet levels increased more than sixfold, and megakaryocytosis was evident in bone marrow. This strategy may be a useful approach to the nonparenteral administration of a variety of therapeutic recombinant proteins, such as those relevant to clotting, endocrine function, and bone-marrow function.

Respiratory function during oxygen administration in chronic obstructive pulmonary disease.

Patient with severe chronic obstructive pulmonary disease (COPD) can develop respiratory muscle weakness and fatigue. Arterial hypoxemia can predispose to this condition. To assess whether O2 administration improved respiratory muscle function and respiratory pattern in COPD 11 patients with chronic hypoxemia were examined. Each patient was first submitted to respiratory function studies, including lung volumes, in normal and recumbent position, respiratory frequency, Ti/Te, Ti/Tot, maximal inspiratory and expiratory pressures (Pimax, Pemax) and arterial gas analysis breathing room air. All these tests were repeated during oxygen Administration through a nasal cannula. The arterial PO2 improved from 50 +/- 7 mmHg to 81 +/- 28 mmHg but the breathing pattern, Pimax and Pemax as well as lung volumes remained unchanged. It is concluded that oxygen, in short term administration, doesn't influence respiratory pattern and muscle function.

[pH measurement in the diagnosis of pleural exudates]. / La determinazione del pH nella diagnosi dei versamenti pleurici.

The diagnostic accuracy of pH measurement of various types of pleural exudate is examined. Despite the limitations of this examination it is easy and quick to perform and offers an undeniable diagnostic validity especially in the case of pleural transudates or malignant effusions.