Developments in managing CF continue to drive dramatic improvements in survival. As newborn screening rolls-out across Europe, CF centres are increasingly caring for cohorts of patients who have minimal lung disease on diagnosis. With the introduction of mutation-specific therapies and the prospect of truly personalised medicine, patients have the potential to enjoy good quality of life in adulthood with ever-increasing life expectancy. The landmark Standards of Care published in 2005 set out what high quality CF care is and how it can be delivered throughout Europe. This underwent a fundamental re-write in 2014, resulting in three documents; center framework, quality management and best practice guidelines. This document is a revision of the latter, updating standards for best practice in key aspects of CF care, in the context of a fast-moving and dynamic field. In continuing to give a broad overview of the standards expected for newborn screening, diagnosis, preventative treatment of lung disease, nutrition, complications, transplant/end of life care and psychological support, this consensus on best practice is expected to prove useful to clinical teams both in countries where CF care is developing and those with established CF centres. The document is an ECFS product and endorsed by the CF Network in ERN LUNG and CF Europe.

The influence of various variables on the rate of pneumothorax and intrapulmonal hemorrhage associated with computed tomography (CT)-guided transthoracic needle biopsy of the lung were evaluated retrospectively. One hundred and thirty-three patients underwent CT guided biopsy of a pulmonary lesion. Two patients were biopsied twice. Variables analyzed were lesion size, lesion location, number of pleural needle passes, lesion margin, length of intrapulmonal biopsy path and puncture time. Eighteen-gauge (18G) cutting needles (Trucut, Somatex, Teltow, Germany) were used for biopsy. Pneumothorax occurred in 23 of 135 biopsies (17%). Chest tube placement was required in three out of 23 cases of pneumothorax (2% of all biopsies). Pneumothorax rate was significantly higher when the lesions were located in the lung parenchyma compared with locations at the pleura or chest wall (P < 0.05), but all pneumothorax cases which required chest tube treatment occurred in lesions located less than 2 cm from the pleura. Longer puncture time led to an increase in pneumothorax rate (P < 0.05). Thirty-seven (27%) out of 135 biopsies showed perifocal hemorrhage. Intrapulmonal biopsy paths longer than 4 cm showed significantly higher numbers of perifocal hemorrhage and pneumothorax (P < 0.05). Significantly more hemorrhage occurred when the pleura was penetrated twice during the puncture (P < 0.05). Lesion size <4 cm is strongly correlated with higher occurrence of perifocal hemorrhage (P < 0.05). Lesion margination showed no significant effect on complication rate. CT-guided biopsy of smaller lesions correlates with a higher bleeding rate. Puncture time should be minimized to reduce pneumothorax rate. Passing the pleura twice significantly increases the risk of hemorrhage. Intrapulmonal biopsy paths longer than 4 cm showed significantly higher numbers of perifocal hemorrhage as well as pneumothorax.

Emotional stress is often followed by increased susceptibility to infections. One major role in the immediate immune response to infection is played by natural killer (NK) cells. This study was designed to establish whether acute psychological stress influences cellular immune functions and to elucidate the role of endocrine parameters as potent mediators of stress induced alterations of the immune system. Forty-five first-time tandem parachutists were examined continuously for their plasma concentrations of cortisol and catecholamines from 120 min before to 60 min after jumping. Lymphocyte subsets, NK activity, and ADCC were determined 2 hr before, immediately after, and 1 hr after jumping. There was a significant increase in sympathetic-adrenal hormones during (adrenaline, noradrenaline) and shortly after jumping (cortisol). Lymphocyte subsets and the functional capacity of NK cells revealed an increase immediately after jumping followed by a decrease significantly below starting values 1 hr later. These changes were significantly correlated to plasma concentrations of noradrenaline. Thus, quick mobilization of NK cells is suggested as one major mechanism for this effective adaptation of the immune system to stress situations.