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MARFAN’S AND OBSTRUCTIVE SLEEP APNOEA?

There have been previous isolated reports of the association of Marfan’s syndrome and obstructive sleep apnoea (OSA), and in this month’s Thorax, Kohler and colleagues report on a case control study of the prevalence of OSA in Marfan’s syndrome. The results show that OSA is highly prevalent in patients with Marfan’s. Interestingly they also found that the apnoea–hypopnoea index was correlated with the aortic root diameter, with the aortic root diameter being significantly greater in the presence of OSA than in patients without OSA. Thus OSA may be a risk factor for aortic root dilatation in Marfan’s syndrome, and the effect of CPAP in this patient group now needs to be evaluated. See page 162

In patients with Marfan’s syndrome, the apnoea–hypopnoea index showed a strong relationship with the diameter of the aortic root (r = 0.50, 95% CI 0.26 to 0.69, p = 0.0003). The solid line represents the regression line according to the function: aortic root diameter  =  3.66 + 0.36 × apnoea/hypopnoea index.

ASTHMA DURING PREGNANCY AND CAESAREAN SECTIONS

In this issue of Thorax we are publishing two papers on the relationship between pregnancy and asthma. Roduit and colleagues show that children born by caesarean section, especially from allergic parents, have a higher risk of asthma than those born by vaginal delivery. The reasons for this intriguing observation are discussed but may be related to the hygiene hypothesis, in that there are different microbial exposures for children born by caesarean section that will affect the immune system. Breton and colleagues report on the risk of perinatal mortality and asthma during pregnancy and show that women with asthma had a higher rate of low birthweight babies and preterm delivery than those without asthma. In the accompanying editorial, Schatz concludes that we need to follow and manage pregnant asthmatic patients with the control required of a “life and death situation”. See pages 93, 101 and 107

MBL AND CHILDHOOD INFECTION

Mannose-binding lectin (MBL) deficiency has been associated with respiratory infection, though it is not clear whether MBL acts locally or systemically. In this month’s Thorax, Fidler and colleagues study MBL in children with various infective and non-infective conditions. They show that MBL is not detectable in non-infected airways but is found in the presence of airway infection. MBL levels also correlated with neutrophil elastase. MBL is related to surfactant proteins (SP A and D) and may also contribute to host defence in the lung. See page 150

Chest CT scan showing a wide lesion with irregular margins in the right inferior pulmonary lobe. Multiple bibasilar smaller lesions are also visible. See Pulmonary puzzle page 100.

OPTIMISTIC OUTCOME OF INTUBATED AIRWAYS PATIENTS

Despite the widespread availability of non-invasive ventilation, endotracheal intubation may still be required. In this issue of Thorax, Wildman and colleagues report on outcomes from a large cohort of 832 patients with airways disease who were admitted to intensive care or high dependency units in the UK. The results show that 62% of the patients survived to 180 days and 73% reported that their quality of life was the same or better than in a stable phase before admission. Most patients would choose similar intensive care treatment. This study emphasises the importance of considering intensive care admission for any patient who fails or is not suitable for non-invasive ventilation and should encourage a more optimistic approach to the critical care management of these patients. See page 128

(A) Chest x ray showed a small and non-specific opacity at the left upper lobe (LUL). His chest x ray prior to commencement of infliximab was unremarkable. (B) Chest CT revealed a cavity at the posterolateral aspect of LUL with several opacities within, suggestive of mycetoma. Inflammatory changes were also noted at the right upper lobe. See Images in Thorax page 184.
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