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 (r50.33) and forced vital capacity (r5 -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.

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Plerixafor augments PBSC collection, but the optimal approach for incorporating it into mobilization is uncertain. Forty-nine consecutive patients mobilized with G-CSF alone were analyzed, and a day 4 peripheral blood CD34 þ cell count of 0.015/ml was found to predict for a day 5 apheresis yield of 2 Â 10 6 CD34 þ progenitors/kg, our institutional minimum necessary for a single autologous transplant. On the basis of this relationship, a clinical guideline was developed which recommended pre-emptive use of plerixafor if the day 4 peripheral blood CD34 þ cell count was between 0.005 and 0.015/ml. A total of 166 consecutive subjects with lymphoma or plasma cell dyscrasias underwent G-CSF mobilization after adoption of this care pathway, and the mobilization failure rate was only 7% in patients managed per guideline. The median PBSC yield was 6.3 Â 10 6 CD34 þ progenitors/kg with G-CSF (day 4 peripheral blood CD34 þ cell40.015/ml) and 4.9 Â 10 6 CD34 þ progenitors/kg with G-CSF þ plerixafor (day 4 peripheral blood CD34 þ cell 0.005-0.015/ml). The median number of days of apheresis was 2 in both groups. This clinical guideline is an effective mobilization algorithm that minimizes mobilization failures, reduces poor apheresis yields, does not require risk factor identification and is simple to implement.

The histories of 243 adults with cystic fibrosis were reviewed; 46 had experienced one or more spontaneous pneumothoraces (18.9%), and seven had died of this complication. There was a high incidence of recurrence on the same side after conservative management (50%) or intercostal drainage (55.2%). Prolonged intercostal drainage was associated with a high mortality. One-third of patients required surgical treatment. Twenty thoracotomies were performed. Three of these patients died but only two of the 17 survivors had recurrences. Severe airflow obstruction was not a contraindication to surgery. A plan of management for future cases is outlined.

Adults with CF have an abnormal haemodynamic response to exercise. This finding has deleterious implications for myocardial performance.