Background Domiciliary assisted ven-

The authors present the case of an 18-year-old girl with cystic fi brosis. Her genotype was F508/N1303K. She had sputum colonisation by Pseudomonas aeruginosa and Staphylococcus aureus and since 2006, she also had Burkholderia cepacia in sputum, which was typed as genomovar II (Burkholderia multivorans). Her lung function had remained stable but low over the last few years despite complex psychosocial problems and issues with treatment compliance. She deteriorated at the end of May 2010 and was admitted with right upper lobe cavitating pneumonia. After 6 weeks of

We report a cystic fibrosis (CF) subject with extensive, central venous catheter-associated thrombosis and sustained elevation of factor VIII to levels normally associated with significantly increased risks of deep venous thrombosis. To determine the potential significance of this finding, the prevalence of elevated factor VIII levels in 22 adults with CF was investigated. Mean (S.D.) factor VIII level was 177 (43) U/dl, with 77% of subjects having levels >150 U/dl. The high prevalence of elevated factor VIII levels questions the significance of this finding in CF subjects with catheter-related thrombosis.

Ten patients (two male) suffering from acute exacerbations of long-standing chronic obstructive pulmonary disease and admitted in hypoxic, hypercapnic respiratory failure were treated with Nasal Intermittent Positive Pressure Ventilation (NIPPV) plus supplemental oxygen, on a general medical ward. The median (range) pH on admission was 7.30 (7.20-7.35), the median age was 67 years (47-77) with an FEV1 (percent of predicted) of 30 (17-39). On admission the median arterial oxygen tension (PaO2) was 4.71 kPa (3.45-6.26) on air, and the carbon dioxide tension (PaCO2) was 7.68 kPa (6.85-9.83). With controlled oxygen therapy there was no significant improvement in PaO2, but the median PaCO2 increased significantly to 9.75 kPa (7.04-11.70) (P < 0.05). By using NIPPV with supplemental oxygen it was possible to significantly improve the median PaO2 to 11.25 kPa (6.70-26.90) (P < 0.01) without worsening PaCO2 levels (8.96 kPa; 6.85-13.10). NIPPV was applied by a senior, respiratory physiotherapist and used intermittently depending on patient tolerance and clinical response. The median total time on NIPPV was 27 h, delivered over 1-5 days. One patient found the mask difficult to tolerate beyond a short period of time. NIPPV was well accepted on a general ward by nursing staff. Three patients later died with progressive hypercapnia, despite an initial response; with one of these patients also receiving intubation and mechanical ventilation. A further patient also received intubation and mechanical ventilation and was eventually discharged. NIPPV plus supplemental oxygen offers a method to correct hypoxaemia on a general medical ward without worsening hypercapnia for acute on chronic, hypoxic, hypercapnic respiratory failure, and warrants further investigation.

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