Feasibility, classification and potential clinical impact of non-invasive delineation of abdominal lymphatic vessels in patients following TCPC with T2 weighted MRI.

Recent research in patients with functionally univentricular hearts (UVH) is focusing on pathologies of the lymphatic vessels. Morphology of the abdominal lymphatic vessels was analyzed by MRI in patients with UVH following total cavopulmonary connection (TCPC) and it was examined, if clinical and laboratory parameters correlate with changes after TCPC. We prospectively examined 33 patients at the age of 19.

Randomized controlled phase 2 trial of hydroxychloroquine in childhood interstitial lung disease.

Background: No results of controlled trials are available for any of the few treatments offered to children with interstitial lung diseases (chILD). We evaluated hydroxychloroquine (HCQ) in a phase 2, prospective, multicentre, 1:1-randomized, double-blind, placebo-controlled, parallel-group/crossover trial. HCQ (START arm) or placebo were given for 4 weeks.

Interventional closure of a bronchopleural fistula in a 2 year old child with detachable coils.

Background: Bronchopleural fistula (BPF) is a severe complication following pneumonia or pulmonary surgery, resulting in persistent air leakage (PAL) and pneumothorax. Surgical options include resection, coverage of the fistula by video-assisted thoracoscopic surgery (VATS), or pleurodesis. Interventional bronchoscopy is preferred in complex cases and involves the use of sclerosants, sealants and occlusive valve devices.

Acute exacerbations in children's interstitial lung disease.

Introduction: Acute exacerbations (AEs) increase morbidity and mortality of patients with chronic pulmonary diseases. Little is known about the characteristics and impact of AEs on children's interstitial lung disease (chILD).

Methods: The Kids Lung Register collected data on AEs, the clinical course and quality of life (patient-reported outcomes - PRO) of rare paediatric lung diseases.

Lymphocytic interstitial pneumonia and follicular bronchiolitis in children: A registry-based case series.

Objectives: Pediatric lymphocytic interstitial pneumonia (LIP) and follicular bronchiolitis (FB) are poorly characterized lymphoproliferative disorders. We present and quantify demographics, radiological and histopathologic patterns, treatments and their responses, and outcomes in non-HIV-infected children with LIP and FB.

Methods: This structured registry-based study included a retrospective chart review, blinded analysis of imaging studies and lung biopsies, genetic testing, and evaluation of treatments and outcomes.

New severity assessment in cystic fibrosis: signal intensity and lung volume compared to LCI and FEV: preliminary results.

Objectives: Magnetic resonance imaging (MRI) aids diagnosis in cystic fibrosis (CF) but its use in quantitative severity assessment is under research. This study aims to assess changes in signal intensity (SI) and lung volumes (Vol) during functional MRI and their use as a severity assessment tool in CF patients.

Methods: The CF intra-hospital standard chest 1.

Lung clearance index for monitoring early lung disease in alpha-1-antitrypsin deficiency.

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.

Increased Risk of Interstitial Lung Disease in Children with a Single R288K Variant of ABCA3.

The gene encodes a lipid transporter in type II pneumocytes critical for survival and normal respiratory function. The frequent ABCA3 variant R288K increases the risk for neonatal respiratory distress syndrome among term and late preterm neonates, but its role in children's interstitial lung disease has not been studied in detail. In a retrospective cohort study of 228 children with interstitial lung disease related to the alveolar surfactant system, the frequency of R288K was assessed and the phenotype of patients carrying a single R288K variant further characterized by clinical course, lung histology, computed tomography and bronchoalveolar lavage phosphatidylcholine PC 32:0.

Repeatability of echocardiographic parameters to evaluate the hemodynamic relevance of patent ductus arteriosus in preterm infants: a prospective observational study.

Background: The 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.

Experiences and challenges of thorcoscopic lung surgery in the pediatric age group.

Background: We report a single-center study of minimally invasive thoracoscopic lung surgery with pediatric patients.

Methods: We performed a retrospective analysis of patients who underwent thoracoscopic lung surgery between 2004 and 2013. The procedures were divided into anatomic and non-anatomical lung resections.

Surfactant proteins in pediatric interstitial lung disease.

Background: Children's interstitial lung diseases (chILD) comprise a broad spectrum of diseases. Besides the genetically defined surfactant dysfunction disorders, most entities pathologically involve the alveolar surfactant region, possibly affecting the surfactant proteins SP-B and SP-C. Therefore, our objective was to determine the value of quantitation of SP-B and SP-C levels in bronchoalveolar lavage fluid (BALF) for the diagnosis of chILD.

[Thrombosis of the pelvic and leg--conservative procedure or thrombolytic therapy?].

History And Admission Findings: We report on a young female patient with flank pain and a swelling of the lower limb of the left side.

Investigations: In magnetic resonance angiography as well as Doppler-duplex ultrasound an iliofemoral phlebothrombosis of the left side due to vena cava inferior hypoplasia with collateral circulation was diagnosed. Oral contraception could play a role as a trigger.

Refractory congestive heart failure following delayed pericardectomy in a 12-year-old child with Mulibrey nanism due to a novel mutation in TRIM37.

Unlabelled: Mulibrey nanism (MUL) is a rare autosomal recessive disorder with severe primordial growth retardation and multiorgan involvement, caused by mutations in TRIM37. Early clinical detection is important since more than 50 % of the patients develop congestive heart failure. We report a 12-year-old patient who presented in infancy with severe growth retardation, dysmorphic features, and cleft palate.

Critical coarctation in an extremely low birth weight pre-term infant.

Neonatal interventions for critical aortic coarctation may be associated with considerable morbidity and mortality if the patient is extremely premature. We report the successful treatment of critical coarctation in a 25-week, 740-gram infant using initial clipping of the duct until continued prostaglandin E1 infusion delayed end-to-end anastomosis 7 weeks later.

Noninvasive cardiac output determination for children by the inert gas-rebreathing method.

Standard methods for determination of cardiac output (CO) are either invasive or technically demanding. Measurement of CO by the inert gas-rebreathing (IGR) method, applied successfully in adults, uses a low-concentration mixture of an inert and a blood-soluble gas, respectively. This study tested the feasibility of this method for determining CO during exercise for pediatric patients with complete congenital atrioventricular block (CCAVB) stimulated with a VVI pacemaker.

Clinical and in vitro effect of dornase alfa in mechanically ventilated pediatric non-cystic fibrosis patients with atelectases.

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.

A practical and transferable new protocol for treadmill testing of children and adults.

Background: It is just as vital to have an exact overview of the physical fitness of young and growing people as it is for adults. The currently used exercise protocols have limitations in healthy small children, and in senior citizens. In particular with chronically ill patients, regardless of their age, there is a need for an exercise protocol that permits observations over the long term.

Recombinant human deoxyribonuclease shortens ventilation time in young, mechanically ventilated children.

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.