Diagnosis and management of α1-antitrypsin deficiency in Europe: an expert survey.

Despite recent improvements, α1-antitrypsin deficiency (AATD) remains a rarely diagnosed and treated condition. To assess the variability of AATD diagnosis/treatment in Europe, and to evaluate clinicians' views on methods to optimise management, specialist AATD clinicians were invited to complete a web-based survey. Surveys were completed by 15 physicians from 14 centres in 13 European countries. All respondents perceived the AATD diagnosis rate to be low in their country; 77% of physicians believed that ∼15% of cases were diagnosed. Low awareness was perceived as the greatest barrier to diagnosis. Spirometry was considered more practical than quantitative computed tomography (QCT) for monitoring AATD patients in clinical practice; QCT was considered more useful in trials. AAT therapy provision was reported to be highly variable: France and Germany were reported to treat the highest proportion (∼60%) of diagnosed patients, in contrast to the UK and Hungary, where virtually no patients receive AAT therapy. Most clinicians supported self-administration and extended dosing intervals to improve convenience of AAT therapy. This survey indicates that AATD diagnosis and management are highly heterogeneous in Europe; European cooperation is essential to generate data to support access to AAT therapy. Improving convenience of AAT therapy is an ongoing objective.

[Transbronchial needle aspiration]. / Punção aspirativa transbrônquica.

Transbronchial needle aspiration was initially invented in 1949 by Schieppati. After its adaptation to the flexible bronchoscope in 1983 by Wang this technique has gain firm indications in the diagnosis and staging of lung cancer, in peripheral pulmonary nodules and masses; in the evaluation of endobronchial masses; in the disease of submucosal, in benign diseases, i.e. sarcoidoses and mediastinal cysts and abscesses. The yield of this technique published in the literature makes it more than useful. The material available has different indications and usefulness in different clinical settings. Despite the almost absence of complications this procedure is yet underutilized, in spite of its twenty years of results which may be due to the established routines and the lack of training.

[Idiophatic mediastinal fibrosis: a case report]. / Fibrose idiopática do mediastino: A propósito de um caso clínico.

We herein report a case of a male patient, who presented a mediastinal mass that had a slow growth over the years. After surgery, that made the diagnosis of idiopathic mediastinal fibrosis, the growth stabilized. Four years latter it was observed a substantial increase which lead to the inevitable compression of vital structures which, in turn, led to pulmonary hypertension. Idiopathic mediastinal fibrosis is an extremely rare pathology. There are same cases in which it is associated with other pathologies but has always a fatal prognosis when surgery is not an option.