Increased augmentation index in patients with cystic fibrosis.

Increased large artery stiffness occurs in a range of inflammatory conditions indicating an ageing of the vasculature and additionally being an independent risk factor for cardiovascular events. We determined large artery parameters in adults with cystic fibrosis (CF). 50 clinically stable adult patients with CF (mean+/-sd age 28.0+/-8.2 yrs) and 26 controls matched for age, sex and body mass index were studied. Central aortic blood pressure, augmentation index (AIx) and aortic pulse wave velocity (PWV) were determined using applanation tonometry. Lung function, diabetic status and C-reactive protein (CRP) were also determined. Mean+/-sd AIx was greater in patients than controls, 8.5+/-11.1% and -1.8+/-13.1%, respectively (p<0.001), while PWV was similar. Although AIx was greatest in the sub-group with CF-related diabetes (CFRD), it was also increased in the non-CFRD sub-group when compared with controls. In patients, AIx was related to log(10) CRP (r = 0.33) and forced vital capacity (r = -0.34; both p<0.05), and CRP remained predictive in multiple regression. AIx is increased in adults with CF, in the presence of a normal blood pressure and independent of diabetic status. AIx was related to the systemic inflammatory status. These findings have implications for management and require further exploration so that cardiovascular health can be maintained.

A novel approach to central venous catheter thrombosis in a patient with cystic fibrosis.

Catheter directed thrombolysis has been described as a treatment for large pulmonary emboli resistant to systemic therapy [Kelly P, Carroll N, Grant C, Barrett C, Kocka V. Successful treatment of massive pulmonary embolism with prolonged catheter-directed thrombolysis. Heart Vessels 2006;21:124?6]. We now describe a case in which local catheter directed thrombolysis, via a peripherally inserted central catheter (PICC), was used to treat a large thrombus surrounding the tip of an indwelling central venous line that was causing superior vena cava obstruction (SVCO), in a patient with cystic fibrosis.

Novel approach to management of a posterior tracheal tear complicating percutaneous tracheostomy.

We treated a patient who developed a posterior tracheal wall perforation and severe respiratory compromise following percutaneous tracheostomy, using a covered expandable metallic stent. The stent was deployed under direct vision using rigid and fibreoptic bronchoscopy. The defect was sealed and the right lung, which had been collapsed, was re-expanded. The patient was subsequently weaned from mechanical ventilation. Late complications included halitosis, which was treated with nebulized colistin sulphate, and the development of intratracheal granulation tissue, which was cleared using low power (10 W) Nd:YAG laser.