Recombinant human deoxyribonuclease (rhDNase) has been demonstrated to reduce the viscosity of purulent cystic fibrosis (CF) respiratory mucus, to improve pulmonary function and to reduce the risk of respiratory tract infectious exacerbations, but its effect on mucus transportability has not so far been investigated. The dose-dependent effect of rhDNase was analysed in vitro on mucus transport rate (tr) by ciliary activity and by simulated cough (cough transport (ct)), as well as on mucus viscosity and surface properties. Purulent CF sputa (n = 15) were incubated for 30 min at 37 degrees C with either rhDNase at three different concentrations (final concentrations 0.2, 2 or 20 micrograms.ml-1 of mucus) or placebo. No significant dose-dependent effect of rhDNase on the mucociliary transport rate was observed when the samples wer statistically analysed together. However, in the larger group of mucus samples (n = 11) with a low initial mucociliary transport rate, the latter was improved at each rhDNase concentration (tr0.2 = 0.69, tr2 = 0.88 and tr20 = 0.87) as compared to placebo (trp = 0.58). In the smaller group of mucus samples (n = 4) with high initial transport rate, a decrease in mucociliary transport rate was observed, particularly at the highest concentration rhDNase assayed, i.e. 20 micrograms.ml-1 of mucus (tr20 = 0.58) as compared to placebo (trp = 0.86). The mucus cough transport was increased by rhDNase (ct0.2 = 25 mm, ct2 = 27.5 mm and ct20 = 31 mm) as compared to placebo (ctp = 23.5 mm).(ABSTRACT TRUNCATED AT 250 WORDS)

PICC lines are a future solution in pediatrics. This technique is reliable and has a similar complication rate to studies carried out in adults, most of which can be prevented by careful catheter maintenance and informing the care staff.

Pelvic ring injuries carry a high mortality rate, the main cause of which, in the first 24hours, is exsanguination. Injured patients are managed by a multidisciplinary damage-control strategy. Unstable patients should have instrumentalized hemostasis without delay. Arterial embolization is an effective way of achieving this and justifies this approach being permanently available in level 1 trauma-centers. After CT assessment of injuries, stable patients can undergo arterial embolization if active arterial bleeding or vascular damage is present. The embolization methods (selective or unselective) and agents used depend on the patient's hemodynamic stage and assessment of the injury whenever possible.

In women with metrorrhagia due to AVM, arterial embolization with Onyx is effective and safe. Additional research is needed to confirm the possibility of future pregnancy after Onyx embolization.

Our aim was to collect a large number of cases to characterize clinical presentation, outcome, and prognosis of chronic intestinal pseusoobstruction in children. We conducted a retrospective multicenter study that included children treated for chronic intestinal pseusoobstruction defined as recurrent episodes of intestinal obstruction with no mechanical obstruction, excluding Hirschsprung's disease. In all, 105 children, 57 boys and 48 girls, were studied, including five familial forms. Prenatal diagnosis was made in 18 patients. Eighty patients were less than 12 months old at onset; the disease began at birth for 37 patients. The most frequent signs were abdominal distension, vomiting, and constipation. Megacystis was noted in myopathies (7 cases), neuropathies (10 cases) and unclassified forms (13 cases). For all but three cases (two patients with CMV infection, one with Munchhausen-by-proxy syndrome), the associated diseases and disorders could not account for chronic intestinal pseusoobstruction as a secondary disorder. At least one full-thickness biopsy from the digestive tract was studied for 99 patients. The diagnosis recorded was visceral neuropathy in 58 cases, visceral myopathy in 17 cases, and uncertain or normal biopsy results in 24 cases. Seventy-eight children were fed intravenously, and only 18 were able to be fed orally throughout their illness. Seventy-one patients underwent surgery during their illness, and 217 surgical procedures, a mean of 3 per patient, were performed. Ostomy was the most performed procedure. Follow-up continued in 89 patients for 3 months to 16 years (mean 85 months). Forty-two patients were still fed by parenteral (39 patients) or enteral nutrition (3 patients) at the time of the study. Eleven patients died between the age of 1 month and 14 years 7 months.

If ultrasound examination proves technically challenging, fetal MRI can be used to obtain the same diagnostic information in 91% of cases and can help surgeons and interdisciplinary teams provide appropriate antenatal counseling.

Aortic coarctation is a local narrowing of the aortic lumen, which is located at the level of the isthmus in 95% of patients. Aortic coarctation accounts for 5 to 8% of all congenital heart diseases. It may have an acute presentation in the form of heart failure in the neonate or may be discovered incidentally in adult because of severe treatment-resistant hypertension. Ultrasound may reveal the presence of aortic coarctation during the antenatal period. In this situation, associated abnormalities should be investigated (including karyotype), because they influence prognosis and indicates whether or not the birth should occur in a center with pediatric cardiology expertise. Postnatally, ultrasound and chest radiography are the basic imaging work-up. Computed tomography is often the second line imaging investigation in infants and young children for whom magnetic resonance imaging fails to confirm the diagnosis. Magnetic resonance imaging with cardiac synchronization is the preferred imaging tool in the post-treatment period. Aortic coarctation may be treated surgically or by endovascular techniques. Potential complications should be searched for using ultrasound and magnetic resonance imaging.

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