2015–2016

23 September 2015

Choosing your spirometer: the answer is blowing in the wind?

Posted in 2015–2016

Jellien Makonga-Braaksma

In this article we are offering a decision matrix that will help you to choose and buy the spirometer that will give the right service in your specific situation (figure 1). Everyone working in respiratory healthcare knows that there is a very wide range of spirometers offered by different manufacturers. They vary from handheld devices to ingenious high-tech computers. It’s complicated to choose the best spirometer in a specific situation. This article aims to give you an overview of what should be crucial points in the purchasing process of a diagnostic spirometer.

23 September 2015

Update on genetics of idiopathic pulmonary fibrosis

Posted in 2015–2016

Raphael Borie, Caroline Kannengiesser and Bruno Crestani

The occurrence of pulmonary fibrosis in numerous individuals from the same family suggests a genetic cause for the disease. During the past 10 years, germline mutations involving proteins from the telomerase complex and from the surfactant system have been identified in association with pulmonary fibrosis. Mutations of TERT, the coding gene for the telomerase reverse transcriptase, are the most frequently identified mutations and are present in 15% of cases of familial pulmonary fibrosis. More recently mutations in RTEL1 and PARN have been described in 5–10% of the family, respectively. Other mutations (TERC, surfactant proteins genes) are only rarely evidenced in adults. Patients with mutations involving the telomerase complex may present with pulmonary fibrosis, haematological, cutaneous or hepatic diseases. Evidence for mutations associated with the development of pulmonary fibrosis raises numerous clinical questions from establishing a diagnosis, providing counselling to deciding on therapy, and requires specific studies. From a pathophysiological point of view, the function of the genes highlights the central role of alveolar epithelium and ageing in fibrogenesis.