Summary. Recombinant human deoxyribonuclease I (dornase alfa) is currently used as an inhaled mucoactive agent in the treatment of cystic fibrosis. In a randomized, placebo-controlled, double-blind clinical study in 100 infants, we investigated whether the therapeutic use of dornase alfa can be extended to ventilated, fluid-restricted children to reduce reintubation rate, ventilation duration, pediatric intensive care unit (PICU) stay, and ventilation complications. While reintubation rates were similar for dornase alfa 7% vs. placebo 9% (odds ratio, 0.77; confidence interval, 0.11-4.9), the incidence of atelectasis (6 vs. 17, respectively; P-value 0.051), median ventilation time (2.2 vs. 3.4 days, respectively; P-value 0.043), median length of PICU stay (7 vs. 8 days, respectively; P-value 0.051), and mean costs (s4,830 vs. s6,320, respectively) were lower in the dornase alfa group. No adverse effects were observed, even in critically ill patients. We found that dornase alfa was beneficial and safe. Our findings also indicate that dornase alfa is possibly of value from the first day of mechanical ventilation onward, particularly when longer ventilation (>3 days) is expected in fluid-restricted children after cardiac surgery.

Bridging bronchus (BB) is a rare, congenital bronchial anomaly that is frequently associated with congenital cardiac malformations, especially left pulmonary artery sling. It represents an anomalous bronchus to the right originating from the left main bronchus. Discrimination from other bronchial anomalies is important, since BB is frequently associated with bronchial stenoses due to abnormal cartilage rings.This case study describes the findings of bronchoscopy, bronchography and multidetector computed tomography (MDCT) in three patients. Bronchoscopy was helpful in the description of the severity and length of bronchial stenoses. However, it was not possible to establish a diagnosis of BB based on this method in two patients, since it is difficult or even impossible to differentiate the bifurcation from the pseudocarina. It was not possible to establish the correct diagnosis in all patients based on bronchography or MDCT. MDCT was able to depict the relationship of bronchial and vascular structures, which is particularly important in patients with pulmonary artery sling.Multidetector computed tomography is preferable to bronchography as it is less invasive and due to its short acquisition time it can be performed in children with severe respiratory disease. In the current authors' experience, detection of cartilage rings still requires flexible bronchoscopy.

Until puberty, boys and girls have the same indicators of exercise performance. Subsequent to puberty, uptake of oxygen and distance run differ, with males showing higher uptake of oxygen. There is still an age-dependent dynamic of peak uptake of oxygen related to body surface area. Using these new reference values, covering the whole range of age, it proves possible to compare performance during growth and aging of the individual. In this fashion, we have calculated centiles for all recorded variables. External calibration, validation and quality control ensures transferability of our data to other spiroergometry units.

Introduction: At present no evidence-based medical treatment for persistent atelectasis in pediatric non-cystic fibrosis (CF) patients is available. Method: To evaluate the use of intratracheally instilled recombinant human deoxyribonuclease (rhDNase) in intubated and ventilated pediatric patients, we performed a single-center observational study on 46 pediatric intensive care patients who had received intratracheal DNase. Patients were classified, according to radiologic findings of atelectasis (group 1) or infiltrates. As controls we examined a historical control group of 17 patients with atelectasis after cardiac surgery, who had been treated with NaCl 0.9% and matched for age and diagnosis with 21 patients from group 1 (subgroup 1a). Radiologic improvement and inflammatory markers in both serum and tracheal aspirates were measured. Results: In group 1, 35 patients had 51 atelectases/dystelectases episodes at baseline. 67 % of patients showed radiologic signs of improvement after 24h treatment with rhDNase. In subgroup 1a, 16 patients had complete resolution of atelectases and minimal change in dystelectases after a treatment of 24 hours rhDNase, compared with the control group of 17 patients, who had 7 atelectases and 10 dystelectases at baseline and an improvement in only 1 out of 17 (6 %) patients after 24h. Conclusion: Intratracheal instillation of rhDNase is an effective adjunct to conservative therapy of atelectases in children. Further randomized controlled prospective studies are necessary.

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BackgroundThe hemodynamically relevant patent ductus arteriosus in preterm infants is not well defined. Different clinical and echocardiographic parameters are used and the diagnostic accuracy is unknown because of the lack of a gold standard definition. Our study evaluates the inter-observer repeatability of echocardiographic and Doppler-ultrasound parameters.MethodsThis prospective observational study included 19 very low birth weight preterm infants (median [interquartile range]: gestational age 28.0 (28.0–29.0) weeks, birth weight 1130 (905–1321) g, postnatal age at measurement 8.7 (4.8–23.5) d) with a clinical suspicion of ductal patency in whom 27 repeated echocardiographic and Doppler-ultrasound examinations were performed within 30 min by 2 of 3 independent observers (54 measurements overall). The repeatability index (=2 times the standard deviation of the differences/mean of all measurements) according to Bland and Altman was used to assess repeatability of different parameters.ResultsThe repeatability indices of the echocardiographic parameters (left Atrium-to-Aortic root-ratio, diameter of the patent ductus arteriosus at its narrowest part, the left-ventricular-preejection-period-to-ejection-time-ratio and the ratio of the velocity time integrals in the large vessels were 16, 21, 23 and 26 % respectively. The repeatability indices of Doppler-ultrasound measurements (resistance index in celiac artery and anterior cerebral artery) were 11 and 14 %, respectively.ConclusionsThe inter-observer repeatability of all echocardiographic parameters was poor compared to that of resistance indices in peripheral vessels. Therefore, interventions for ductal patency should be indicated based on averaged repeated rather than single measurements, especially when measured values are close to their cut-off value - both in clinical routine and for study purposes.

Patients with alpha-1-antitrypsin deficiency (AATD) and a PI-ZZ genotype are at high risk to develop severe emphysema during adulthood. However, little is known about early stages of emphysema and disease manifestation in other PI-types. Spirometry is commonly used for monitoring although early manifestation of emphysema is suspected within the peripheral airways that are not accessible by forced expiratory manoeuvres. We hypothesized that the Lung Clearance Index (LCI) derived from multiple breath nitrogen-washout (N2-washout) is useful to bridge this diagnostic gap. Patients from age 4 years onward and different PI-types performed N2-washout and spirometry. Results were compared to controls. 193 patients (4-79 years, 75% PI-ZZ) and 33 controls (8-60 years) were included. Mean (SD) LCI in patients was 9.1 (3.1) and 6.3 (0.6) in controls (p ≤ 0.001). 47% of adult patients with other than PI-ZZ genotypes and 39% of all patients with normal spirometry had abnormal LCIs. The LCI measured by N2-washout discriminates between patients with AATD and controls, reflects AATD related lung disease in all stages and appears to identify early peripheral lung changes in younger age than spirometry. We conclude that a normal spirometry does not exclude presence of AATD related lung disease even in genotypes other than PI-ZZ.