Exercise with incorporated expiratory manoeuvres was as effective as breathing techniques for airway clearance in children with cystic fibrosis: a randomised crossover trial.

QUESTION: : Can a session of exercise with incorporated expiratory manoeuvres substitute for a session of breathing techniques for airway clearance in children with cystic fibrosis? Are children with cystic fibrosis as co-operative and satisfied with the exercise regimen as with the breathing techniques?. DESIGN: Randomised, cross-over trial with concealed allocation and intention-to-treat analysis. PARTICIPANTS: 34 children with cystic fibrosis in a stable clinical state. INTERVENTIONS: Participants underwent two 20-min airway clearance interventions on two scheduled clinic days: one involving three bouts of various whole-body exercise modalities each followed by independent expiratory manoeuvres, and the other involving breathing control, thoracic expansions with manual expiratory compressions, and the forced expiratory technique. OUTCOME MEASURES: Wet weight of expectorated sputum, change in lung function, co-operation with treatment, perceived treatment quality, and satisfaction with treatment were all assessed after each intervention. RESULTS: The wet weight of sputum after exercise was 0.6g higher after the exercise intervention, which was not statistically or clinically significant (95% CI -0.2 to 1.4). However, lung function and participant satisfaction with the treatment were both significantly better after the exercise intervention. Co-operation with treatment and perceived treatment quality were equally high for each intervention. CONCLUSION: A session of various whole-body exercises interspersed with independent expiratory manoeuvres could be an acceptable substitute for a session of breathing control, thoracic expansions with manual expiratory compressions, and the forced expiratory technique in children with mild cystic fibrosis lung disease.

Growth hormone to improve short bowel syndrome intestinal autonomy: a pediatric randomized open-label clinical trial.

BACKGROUND: The ability of growth hormone (GH) to promote the weaning-off of parenteral nutrition (PN) in short bowel syndrome (SBS) is unclear. No randomized controlled study is available in children. This study was undertaken to determine if GH could enhance the weaning off of PN in PN-dependent children with SBS. METHODS: A prospective randomized open-label multicenter study was performed in 14 patients (mean age, 9 ± 1.4 years) with SBS (average small bowel length, 33 cm) and long-term PN dependency (8 years) on an unrestricted diet. A standardized PN decrease with and without GH (0.14 mg/kg/d) was conducted. The patients were randomized to either a GH group (4 months of GH) or a control (CTR) group (4 months without GH, followed by 4 months with GH). Blood tests and a nutrition assessment of enteral and parenteral intakes were performed. Groups were compared with the Wilcoxon test. RESULTS: Treatment with GH did not improve the weaning off of PN (decrease in PN caloric intake of 32.5% ± 9.6% in the GH group vs 35.2% ± 8.7% in the CTR group, nonsignificant). In the CTR group, GH treatment induced an additional but not statistically significant decrease of 8.8% ± 12.4% in daily calories. Parenteral needs returned to near basal rates 6 months after GH discontinuation (GH: 77.6% ± 10.6% vs CTR: 73.2% ± 7.4%). Weight decreased slightly in both groups. No biological parameters varied significantly. CONCLUSIONS: GH did not improve the weaning off of PN in PN-dependent children with SBS.

Hyperpolarized 3He MR for sensitive imaging of ventilation function and treatment efficiency in young cystic fibrosis patients with normal lung function.

PURPOSE: To assess the sensitivity of hyperpolarized helium 3 ((3)He) magnetic resonance (MR) imaging for the detection of peripheral airway obstruction in younger cystic fibrosis (CF) patients showing normal spirometric results (mean forced expiratory volume in 1 second [FEV(1)], 112% +/- 14.5 [standard deviation]) and to observe the immediate effects of a single chest physical therapy (CPT) session, thereby comparing two image quantification techniques. MATERIALS AND METHODS: Ten pediatric CF patients (age range, 8-16 years) with normal spirometric results were included in this study after approval from the local research ethics committee. Spirometry followed by proton and hyperpolarized (3)He three-dimensional lung imaging were performed with a 1.5-T MR unit before and after 20 minutes of CPT. The number of ventilation defects per image (VDI) and the ventilated lung fraction (VF), defined as the ratio of ventilated lung volume divided by total lung volume, were quantified. RESULTS: Ventilation defects were found in all patients (mean VDI, 5.1 +/- 1.9; mean global VF, 78.5% +/- 12.3; and mean peripheral VF, 75.5% +/- 17.1) despite normal spirometric results. After CPT, disparate changes in the distribution of ventilation defects were observed but the average VDI and VF did not change significantly (mean VDI, 5.1 +/- 1.1; mean global VF, 83.5% +/- 12.2; and mean peripheral VF, 80.3% +/- 12.2). There was no correlation between FEV(1) and VDI (rho = -0.041, P = .863) or global VF (rho = -0.196, P = .408) values but peripheral VF and VDI were correlated (rho = -0.563, P = .011). CONCLUSION: Although spirometric results indicate normal lung function, the mean VDI in patients (5.1) found in this study is well above the VDI in healthy subjects (1.6) reported in the literature. A single CPT session induces disparate changes in the distribution and extent of ventilation defects.

A positioning pillow to improve lumbar puncture success rate in paediatric haematology-oncology patients: a randomized controlled trial.

BACKGROUND: Lumbar punctures (LPs) are common in children with cancer. Although pain management during the lumbar puncture has been well standardized, dealing with stress and anxiety is not well addressed yet. Our objective was to evaluate the potential improvement of the LP success rate using a positioning pillow, to ensure maximum lumbar flexion, and allow paravertebral muscles to relax, in children who are awake, with either conscious sedation or no sedation. METHODS: Children aged 2-18 years undergoing LP were randomly assigned to a positioning pillow or no intervention. The primary outcome was the rate of success, i.e. achieving the LP (sampling or injection) at the first attempt, without bleeding (RBC < 50/mm3). The secondary outcomes included: the child's pain, assessed by a self-administered visual analogical scales (VAS) for children over 6 years of age; the parents' and caregivers' perception of the child's pain; the satisfaction of the children, the parents, the caregivers and the physician. The child's cooperation and the occurrence of post-LP syndrome were also evaluated. RESULTS: 124 children (62 in each group) were included. The LP pillow tended to increase the success rate of LPs (67% vs. 57%, p = 0.23), and decreased the post-LP syndromes (15% vs. 24%, p = 0.17) but the differences were not statistically significant. In children over 6-year of age (n = 72), the rate of success was significantly higher in the pillow group (58.5% vs. 41.5%, p = 0.031), with a tendency to feel less pain (median VAS 25 vs. 15 mm, p = 0.39) and being more satisfied (84.4% vs. 75.0%, p = 0.34). CONCLUSION: Overall results do not demonstrate a benefit in using this pillow for lumbar punctures. This study results also suggest a benefit in the sub group of children over 6-year of age; this result needs confirmation.

Successful use of covered stent to treat superior systemic baffle obstruction and leak after atrial switch procedure.

Progressive dyspnea and cyanosis occurred in a 41-year-old patient status after Mustard atrial switch repair for transposition of great arteries. Cardiac catheterization and magnetic resonance imaging revealed the association of superior limb systemic venous baffle obstruction and leaks with right-to-left shunting. He underwent successful dilation of the venous channel and obstruction of baffle leaks by using a covered stent.

Complete correction of interrupted right aortic arch with isolation of left subclavian artery.

Isolation of the left subclavian artery is a rare aortic arch anomaly. Association with a right interrupted aortic arch and a double patent ductus arteriosus is exceptional. We report a case of such an association with successful surgical correction.

Transcatheter closure of traumatic ventricular septal defect: an alternative to surgical repair?

A 19-year-old man with multiple-system injuries including a serious head injury and two poorly tolerated traumatic ventricular septal defects, was admitted to our hospital. Transcatheter closure of the cardiac defects was attempted instead of surgical repair because the required anticoagulation for cardiopulmonary bypass could precipitate intracranial bleeding. The two ventricular septal defects were successfully closed with Amplatzer devices, but the patient remained in hemodynamically unstable condition and subsequently died. Transcatheter closure of traumatic ventricular septal defect is an alternative to surgical repair, although it remains a hazardous procedure and requires experienced anesthesia management.