Dynamic vascular changes following intravenous antibiotics in patients with cystic fibrosis.

BACKGROUND: Adults with cystic fibrosis (CF) have altered large artery haemodynamics which is associated with a persisting systemic inflammatory state. We hypothesized that a short-term intervention favorably influencing the inflammatory status may modify their haemodynamic state. METHODS: Adult patients with CF were studied immediately preceding and following 2 weeks of intravenous antibiotics. Large artery haemodynamics, principally heart rate-adjusted augmentation index (AIx) were obtained. Blood pressure (BP), spirometry and CRP were also measured. RESULTS: Complete data was available for 15 patients; mean (SD) age 28 (6)years. CRP was reduced while FEV1% predicted improved. Following treatment AIx was lower: 10.9 (10.9)% to 8.1 (10.9)% (p<0.05) while BP was similar and a trend toward lower heart rate (p=0.06). Change in AIx was related to baseline FEV1% predicted (r=0.77) and BMI (r=0.71) (both P<0.01). CONCLUSION: The abnormal central haemodynamics evident in adults with CF is modulated with a short intervention of intravenous antibiotics.

The effect of exercise on large artery haemodynamics in cystic fibrosis.

BACKGROUND: Adult patients with cystic fibrosis (CF) have resting abnormal large artery haemodynamics. Here, we obtain further insight in patients with CF by evaluating haemodynamic response to physiological stress. METHODS: Thirty-six stable CF patients mean (SD) age 28.9 (9.0)years and 25 controls matched for age, gender and body mass index were studied. Central haemodynamic parameters; including augmentation index (AIx) and wasted left ventricular pressure energy (∆E(W)) were determined pre, during and post light intensity cycle ergometry. RESULTS: During exercise, despite a similar heart rate and blood pressure, patients had comparatively greater ∆E(W) (P=0.03) and trend towards greater AIx (P=0.07) than controls. Exercise ∆E(W) was greatest in patients with CF related diabetes (n=11). In all subjects, exercise ∆E(W) was related to age (r=0.54, P<0.001) and FEV(1)% predicted (r=-0.32, P=0.01). CONCLUSIONS: Adults with CF have an abnormal haemodynamic response to exercise. This finding has deleterious implications for myocardial performance.

Nebulised DNase in the treatment of life threatening asthma.

A 25-year-old pregnant patient developed life-threatening asthma refractory to all standard treatment including anaesthetic agents. Ventilatory pressures continued to rise with impending cardio-respiratory arrest. The introduction of nebulised endotracheal DNase however resulted in a rapid and dramatic improvement with good clinical outcome.

An unusual case of abdominal pain in a patient with cystic fibrosis.

We report the case of a 19 year old man with cystic fibrosis (CF) who presented with atypical abdominal pain precipitated by pressing his ribs. This was subsequently discovered to be referred pain from an intercostal schwannoma. Surgical resection led to a resolution of his symptoms.