BACKGROUND: Nocturnal desaturation in cystic fibrosis (CF) may have prognostic implications because a significant and maintained nocturnal desaturation can contribute to the development and progression of pulmonary hypertension with cor pulmonale. Its relation with the desaturation in exercise has not been sufficiently studied. We aimed to determine whether desaturation during 6MWT can be an indicator of nocturnal desaturation in adult subjects with CF. METHODS: 57 subjects were included: 50.9% male, 27.5 ؎ 7.7 y old, mean FEV 1 ؍ 2.37 ؎ 0.74 L, and %FEV 1 67 ؎ 18.1%. Desaturation during 6MWT was defined as oxygen saturation (S pO 2) < 90% or a decline of > 4 points in S pO 2 from baseline, and nocturnal desaturation as a desaturation index > 4 or > 5% of sleep time with S pO 2 < 90%. RESULTS: Desaturation observed during 6MWT in adult subjects with CF did not correlate with nocturnal desaturation (P ؍ .27). Subjects with %FEV 1 < 55% and diffusion capacity of carbon monoxide (D LCO) < 50 mmol/min/mm Hg were at higher risk of 6MWT desaturation. Nocturnal desaturation was more frequent in males, with P aO 2 < 71 mm Hg in blood gas analysis. CONCLUSIONS: Desaturation observed in 6MWT cannot predict desaturation at night in adults with CF. Other parameters were identified as predictors of desaturation.
Achromobacter xylosoxidans is a gram negative non-fermentative rod that infects adult cystic fibrosis patients with moderate to severe lung compromise. Its clinical implications are still to be determined. The aim of this study was to analyze the impact of inhaled imipenem on forced expired volume in 1 sec (FEV 1 ) and number of exacerbations in adult cystic fibrosis patients. Patients colonized by A. xylosoxidans with more than two intravenous antibiotic cycles and/or ≥ 5% FEV 1 impairment during the last year were included.We included six patients with a mean age of 24.8 years. At the beginning of treatment FEV 1 was 1.18 l (52%) with a mean of 3.4 exacerbations in the previous 12 months and 2.24 (p=0.04) the following 12 months. There was a significant difference between the initial FEV 1 value and three months after treatment (p=0.005).
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