Long-term steroid therapy is associated with improved peak cough flow and respiratory muscle strength in patients with Duchenne muscular dystrophy. Maximum voluntary ventilation may be a useful predictor of lung function in Duchenne muscular dystrophy.

Study Objectives: Polysomnography (PSG) is increasingly used in the assessment of infants. Newborn PSG reference values based on recent standardization are not available. This study provides reference values for PSG variables in healthy newborn infants. Methods: Cross-sectional study of normal term newborn infants using standardized PSG collection and American Academy of Sleep Medicine interpretation criteria. Results: Thirty infants born between 37 and 42 weeks gestation underwent PSG testing before 30 days of age (mean 19.6 days). The infants had a mean sleep efficiency of 71% with average proportions of transitional, NREM and REM sleep estimated at 16.1%, 43.3% and 40.6% respectively. Mean arousal index was 14.7 events/h with respiratory arousal index of 1.2 events/h. Mean apnea-hypopnea index (AHI) was 14.9 events/h. Central, obstructive, and mixed apnea indices were 5.4, 2.3, and 1.2 events/h respectively. Mean oxygen saturation in sleep was 97.9% with a nadir of 84.4%. Mean end tidal CO2 was 35.4 mmHg with an average of 6.2% of sleep time spent above end-tidal CO2 45 mmHg and 0.6% above 50 mmHg. Conclusions: The sleep efficiency was significantly lower and the AHI was significantly higher compared to healthy children older than 1 year. The AHI was also higher than reported in healthy infants older than 1 month. These findings suggest current severity classifications of sleep apnea may not apply to newborn infants.

Objective: To determine the incidence of congenital heart defects (CHD) in Aboriginal and non‐Aboriginal infants in Central Australia and to compare this with the incidence elsewhere in Australia. Design and setting: Data on cases were obtained from patient records of the Alice Springs Hospital, Central Australia, the sole referral centre for paediatric and initial cardiac diagnostic services for the region. Participants: Patients with CHD proven by echocardiography reported between 1 January 1993 and 30 June 2000. Main outcome measures: Incidence of CHD using all live births in Central Australia as the denominator. Results: 108 patients with CHD were detected among 6156 live births (incidence, 17.5 per 1000; 95% CI, 14.9–21.7 per 1000); 57 of 2991 were Aboriginal (19.0 per 1000; 95% CI, 14.4–24.6 per 1000) and 51 of 3165 were non‐Aboriginal (16.1 per 1000; 95% CI, 12.0–21.1 per 1000). The difference between the two groups was not statistically significant (relative risk, 1.18; 95% CI, 0.81–1.72). CHD incidence in Central Australia was significantly higher than that reported for other parts of Australia (4.3 per 1000 live births in New South Wales and the Australian Capital Territory, 1981–1984; 7.65 and 12 per 1000 total births in Western Australia, 1980–1989, and South Australia, 1993–2000, respectively). Conclusions: The high rates of CHD in Central Australia may partly reflect the high utilisation of echocardiography for assessing minor lesions. However, the incidence of both major and minor types of CHD was significantly higher than previously reported from other regions of Australia. The role of socioenvironmental factors in this high incidence should be explored.

Early detection and management of pancreatic insufficiency is essential to optimize health and outcomes in cystic fibrosis patients. The gold standard measures for assessment of pancreatic function are direct pancreatic stimulation tests, which have numerous limitations. Estimation of fecal elastase-1 level to determine pancreatic function is an attractive alternative as the test is simple, rapid, cost-effective and easy to perform even in children. This review summarizes the data from studies reflecting the validity, limitation and advantages of fecal elastase-1 in assessing pancreatic function in cystic fibrosis patients.