Fitness to fly in the paediatric population, how to assess and advice.

The number of children on commercial aircrafts is rising steeply and poses a need for their treating physicians to be aware of the physiologic effects and risks of air travel. The most important risk factors while flying are a decrease in partial oxygen pressure, expansion of trapped air volume, low cabin humidity, immobility, recirculation of air and limited options for medical emergencies. Because on-board medical emergencies mostly concern exacerbations of chronic disease, the medical history, stability of current disease and previous flight experience should be assessed before flight. If necessary, hypoxia altitude simulation testing can be performed to simulate the effects of in-flight hypoxia. Although the literature on paediatric safety of air travel is sparse, recommendations for many different situations can be given. CONCLUSION: We present an overview of the most up to date recommendations to ensure the safety of children during flight. What is Known: • Around 65% of on-board medical emergencies are complications of underlying disease. • In children, the three most common emergencies during flight concern respiratory, neurological and infectious disease. What is New: • Although studies are scarce, some advices to ensure safe air travel can be given for most underlying medical conditions in children, based on physiology, studies in adults and expert opinions. • In former preterm infants without chronic lung disease, hypoxia altitude simulation testing to rule out in-flight desaturation is not recommended.

Spontaneous pneumothorax as indicator for Birt-Hogg-Dubé syndrome in paediatric patients.

BACKGROUND: Birt-Hogg-Dubé syndrome (BHD) is a rare autosomal dominantly inherited disorder caused by germline mutations in the folliculin (FLCN) gene. Clinical manifestations of BHD include skin fibrofolliculomas, renal cell cancer, lung cysts and (recurrent) spontaneous pneumothorax (SP). All clinical manifestations usually present in adults > 20 years of age. CASE PRESENTATIONS: Two non-related patients with (recurrent) pneumothorax starting at age 14 accompanied by multiple basal lung cysts on thoracic CT underwent FLCN germline mutation analysis. A pathogenic FLCN mutation was found in both patients confirming suspected BHD. The family history was negative for spontaneous pneumothorax in both families. CONCLUSION: Although childhood occurrence of SP in BHD is rare, these two cases illustrate that BHD should be considered as cause of SP in children.

Controlled trial of cycled antibiotic prophylaxis to prevent initial Pseudomonas aeruginosa infection in children with cystic fibrosis.

BACKGROUND: Initial pulmonary Pseudomonas aeruginosa infection in patients with cystic fibrosis (CF) is currently treated with intensive antibiotic therapy. At this stage, inflammation and tissue injury might have already occurred. Moreover, bacterial eradication is not always achieved. Prophylactic treatment against P aeruginosa seemed to have a preventive effect in retrospective studies. A study was undertaken to establish prospectively the effect of cycled prophylactic treatment on prevention of initial P aeruginosa infection in children with CF. METHODS: This 3-year triple-blind randomised controlled trial included 65 children with CF without P aeruginosa infection. Intervention existed of 3-monthly 3-week treatments with oral ciprofloxacin and inhaled colistin or both placebo controls. The primary outcome was P aeruginosa infection. Secondary outcomes were serum anti-Pseudomonas antibodies, pulmonary function, exacerbations, chest x-ray scores, inflammation parameters, respiratory pathogens and antimicrobial resistance. RESULTS: There was no difference in acquisition of P aeruginosa infection between the control and treatment groups (annual incidence 14% vs 11%; HR 0.738, 95% CI 0.299 to 1.822). Anti-Pseudomonas antibodies emerged earlier in the control group, but this difference had disappeared after 3 years. Chronic infection was observed in 19% of controls and 12% of treated patients. Decline in pulmonary function and other clinical outcomes did not differ between the two groups. In the treatment group, significantly fewer Gram-positive bacteria and Enterobacteriaceae were observed but there were more non-P aeruginosa non-fermentative Gram-negative bacteria. Conclusions Three-monthly cycled anti-P aeruginosa prophylaxis does not reduce the risk of initial and chronic infection in P aeruginosa-negative children with CF of all ages. Shifts in bacterial colonisation demand caution. Trial Registration Number ISRCTN 11604593.

What general practitioners and paediatricians think about their patients' asthma.

The Asthma Insight and Reality in Europe (AIRE) study showed that the current management and treatment of asthma in Europe falls short of the goals set in the GINA guidelines. Patient care may be negatively influenced by the physicians' underestimation of their patients' disease state, and overestimation of their patients' knowledge of asthma management. We interviewed 118 paediatricians and 152 general practitioners (response rate 70 and 86%, respectively) in order to get an insight into the physicians view on his patients' asthma management. A questionnaire containing similar items to those used in the AIRE study was used. Dutch physicians believe that the asthma of the majority of their patients is well controlled and underestimate the prevalence of daytime symptoms. They believe that their patients are aware of the differences between reliever medication and maintenance medication and overestimate the number of patients in possession of a written action plan. Dutch paediatricians and general practitioners underestimate the severity of their patients' disease state and overestimate their patients' knowledge of disease management.

Predicting adherence to prophylactic medication in adolescents with asthma: an application of the ASE-model.

An explanatory framework, referred to as the attitude/social influence/self-efficacy-model (ASE-model), was utilised to explain future self-reported adherence of adolescents to daily inhaled prophylactic asthma medication. The objective was to investigate the long-term influence of these earlier reported cognitive variables and other psychological and medical determinants on self-reported adherence 1 year later. Data were collected, via a questionnaire, from 86 adolescents with asthma (aged 11 through 18 years) recruited from outpatient clinics. Adherence was assessed by asking the patients to give themselves a report mark for adherence. The results of the multiple regression analyses showed that the three major ASE-variables were predictors of self-reported adherence to a moderate degree (R(2)=0.21). Previous self-reported adherence was found to be the best predictor of self-reported adherence to prophylactic asthma medication 1 year later (R(2)=0.45). The results of this study could be useful in the development of interventions to enhance adherence to asthma medication. In future, such interventions should focus on feelings of shame about having asthma and promoting healthy habits, such as adherence to medication.