Revue des maladies respiratoires
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Review
[Patients'illness perceptions and adherence to treatment with inhaled corticosteroids in asthma].
Regular use of inhaled corticosteroids as preventive treatment of asthma is an integral part of management but remains inadequate among adults. Studying the perceptions of illness and treatment beliefs is one way to understand the patient's adherence to medication. ⋯ Patients' perceptions of asthma and inhaled corticosteroids are predictors of adherence to treatment. The identification and discussion of these issues is an essential part of building a therapeutic relationship that facilitates adherence.
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Endobronchial hamartoma is a benign tumour derived from peribronchial mesenchymal tissue. It is a form of intrapulmonary hamartoma and can cause irreversible pulmonary destruction due to bronchial obstruction. Early diagnosis and treatment is very important and endoscopic treatment is usually the first choice. In cases in which prolonged bronchial obstruction has produced irreversible lung destruction surgical, resection of the tumour is necessary. The aim of this study is to describe the clinicopathological characteristics of resected endobronchial hamartoma. ⋯ Endoscopic techniques are effective for the diagnosis and treatment of endobronchial hamartochondroma. Treatment should be prompt to prevent irreversible lung damage due to chronic obstruction and suppuration, in which case, pulmonary resection may be necessary.
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Acute exacerbation of idiopathic pulmonary fibrosis (IPF) is defined by an acute worsening of the respiratory status without any identified cause. ⋯ Acute exacerbation of IPF is a severe complication of IPF, but its optimal management is not yet clearly defined.
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Multicenter Study Comparative Study
[Prognostic value of clinical exercise testing in idiopathic pulmonary fibrosis].
Current guidelines for referring patients with idiopathic pulmonary fibrosis (IPF) for lung transplantation, based on resting parameters, are insufficient to predict 3-year mortality. The aim of this study was to determine the prognostic value of cardio-pulmonary exercise testing (CPET) in patients with IPF. A multicentre retrospective study of 3-year outcome was made on 63 adult patients with IPF who underwent CPET with blood gas analysis. ⋯ The multivariate logistic regression analysis showed that CPT (<65%) and VE/VO(2) at VT (>45) were independently associated with a lower survival at 3 years. Restriction and hyperventilation at ventilatory threshold are major prognostic factors in the course of IPF. CPET with blood gas analysis may have a prognostic value in these patients and initial evaluation of these parameters can help to predict disease progression.
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The Birt-Hogg-Dubé (BHD) syndrome is associated with cutaneous disorders including fibrofolliculomas and trichodiscomas, and also lung pneumatocysts and kidney tumours. The BHD syndrome occurs as a consequence of an autosomal dominantly inherited genodermatosis, linked to multiple germline mutations in the 14 exons of the BHD gene, mapped on 17p11.2 and encoding for folliculin (FLCN). The size and number of lung pneumatocysts are extremely variable and the cysts are surrounded by normal pulmonary tissue. ⋯ However, they must be systematically evaluated since kidney tumours occur in one third of patients. We report a case of classical BHD syndrome with specific cutaneous involvement, recurrent pneumothoraces complicating lung cysts, an exon 12 germline mutation on BHD gene and a familial history suggesting other related cases. This observation allows us to update this orphan disease, to consider BHD in the differential diagnosis of lung cysts and, above all, to highlight the high frequency and the prognostic significance of associated kidney tumours.