Revue des maladies respiratoires
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Several etiologies are involved in the pathogenesis of cavitating pulmonary disease including neoplastic, infectious or inflammatory processes. Another is pulmonary infarction associated with venous thromboembolism. ⋯ CTEPH is sometimes diagnosed during an episode of recurrent pulmonary embolism following previously unnoticed lesions. Thrombophilias such as isolated elevated factor VIII are risk factors for CTEPH.
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Congenital lung lesions comprise a broad spectrum of various malformations including congenital cystic adenomatoid malformation (CCAM), bronchopulmonary sequestration (BPS), congenital lobar emphysema, bronchial atresia and bronchogenic cyst. This review aims at the description of their natural history, and of the underlying pathophysiological mechanisms. ⋯ Prospective follow-up of operated and unoperated children would complete our knowledge about the natural history of these lesions. The contribution of experimental models has led to advances in the understanding of pathogenic mechanisms. Further studies are needed to identify the factors initiating the malformative process.
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The aim of mineralogical analysis of lung tissue, bronchoalveolar lavage (BAL) and sputum is to characterize individuals' exposure to asbestos fibres by identifying markers of this; asbestos bodies (AB) and uncoated fibres. The techniques of mineralogical analysis, habitually used to identify AB and uncoated fibres, are respectively optical microscopy (OM) and analytical electronic microscopy (EM). Correlations between levels of retention of AB in lung tissue, BAL and sputum have been established and validated threshold values indicating a high probability of significant exposure exist. ⋯ Mineralogical analysis is not suitable for use in routine medical screening but it can be considered when a source of exposure is not evident from the questionnaire since a positive analysis of BAL or of sputum is highly specific and thus useful to confirm an important retention of asbestos in the lung, which justifies medical follow-up. A negative result does not exclude previous significant asbestos exposure (frequent false negatives occur especially in sputum and biopersistence of chrysotile is lower than for amphiboles). Thus it can be a complementary tool for the assessment of asbestos exposure but its use imposes conditions for the collection and handling of samples.
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Drug hypersensitivity (DRESS syndrome) is a rare disorder with diverse systemic and visceral manifestations. Pulmonary involvement is uncommon and is mainly characterized by eosinophilic infiltration. We report a case of DRESS syndrome induced by clomipramine with predominant pulmonary involvement.
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The congenital central hypoventilation syndrome (CCHS) or Ondine's curse is a rare autosomal dominant disease, characterized by disorders of the autonomic nervous system, with abnormal ventilatory responses to hypercapnia and hypoxia. PHOX2B has been identified as the major gene causing CCHS. It results from polyalanine repeat expansion mutations. It typically presents in the newborn period but some cases have been described in adults (late onset CCHS) reflecting the variable penetrance of PHOX2B mutations. ⋯ The diagnosis of late onset CCHS should be considered in patients with unexplained hypoventilation, and physiological evaluation should be undertaken to document the abnormal ventilatory responses. The presence of a PHOX2B mutation confirms the diagnosis.