Pediatric long-term noninvasive respiratory support in children with central nervous system disorders.

Rationale: The use of long-term noninvasive respiratory support is increasing in children along with an extension of indications, in particular in children with central nervous system (CNS) disorders.

Objective: The aim of this study was to describe the characteristics of children with CNS disorders treated with long-term noninvasive respiratory support in France.

Methods: Data were collected from 27 French pediatric university centers through an anonymous questionnaire filled for every child treated with noninvasive ventilatory support ≥3 months on 1st June 2019.

Long term noninvasive ventilation and continuous positive airway pressure in children with neuromuscular diseases in France.

The aim of the study was to describe the characteristics of children with neuromuscular diseases treated with long term noninvasive ventilation or continuous positive airway pressure in France. On June 1st 2019, 387 patients (63% boys, mean age 11.2 ± 5.

Paediatric long term continuous positive airway pressure and noninvasive ventilation in France: A cross-sectional study.

Objective: To describe the characteristics of children treated with long term continuous positive airway pressure (CPAP) or noninvasive ventilation (NIV) in France.

Design: Cross-sectional national survey.

Setting: Paediatric CPAP/NIV teams of 28 tertiary university hospitals in France.

Respiratory management of children with spinal muscular atrophy (SMA).

Spinal muscular atrophy (SMA) causes a predominantly bilateral proximal muscle weakness and atrophy. The respiratory muscles are also involved with a weakness of the intercostal muscles and a relatively spared diaphragm. This respiratory muscle weakness translates into a cough impairment, resulting in poor clearance of airway secretions and recurrent pulmonary infections, restrictive lung disease due to a poor or insufficient chest wall and lung growth, nocturnal hypoventilation and, finally, respiratory failure.

Severe adenovirus pneumonia with hemophagocytic syndrome and respiratory failure.

We report the case of an 18-month-old infant with severe serotype 3 adenovirus pneumonia, exceptionally associated with hemophagocytic syndrome. Treatment included cidofovir and mechanical ventilation for 13 days. The child developed chronic respiratory insufficiency due to bronchiectasis and bronchiolitis obliterans.

Cystic fibrosis in the modern therapeutic era: Give the shower a thought!

Fifty-two meshes of e-flow rapid® were characterized for tobramycin delivery with a laser diffractometer after 6 months of home use by cystic fibrosis patients treated with various nebulized drugs. Three meshes were out of order and 30 considered to be defective for tobramycin delivery. The use of the specific mesh cleaning shower system permitted 14 defective meshes to be in the expected range of nebulized volume.

When Christmas decoration goes hand in hand with bronchial aspiration ….

We report the case of a 14-month-old girl suffering from cough and wheeze around Christmas. She was treated with anti-asthmatic drugs with no success, and 3 weeks later a chest X-ray revealed a LED bulb in the left main bronchus. This LED bulb came from a Christmas light garland decorating the Christmas tree.

Multivariate Analysis As a Support for Diagnostic Flowcharts in Allergic Bronchopulmonary Aspergillosis: A Proof-of-Concept Study.

Molecular-based allergy diagnosis yields multiple biomarker datasets. The classical diagnostic score for allergic bronchopulmonary aspergillosis (ABPA), a severe disease usually occurring in asthmatic patients and people with cystic fibrosis, comprises succinct immunological criteria formulated in 1977: total IgE, anti- () IgE, anti- "precipitins," and anti- IgG. Progress achieved over the last four decades led to multiple IgE and IgG(4) biomarkers available with quantitative, standardized, molecular-level reports.

Vibrating-mesh nebulizer maintenance by CF patients: Results from a French survey.

Purpose Of The Study: Vibrating-mesh nebulizers are widely used at home for cystic fibrosis (CF) treatment, with a therapeutic efficiency closely linked to the mesh performance. This national study looks at the maintenance at home by CF patients and their families of the mesh of the eFlowrapid nebulizer. Ninety-two patients from 34 French CF centers, treated at home with inhaled drugs delivered with a vibrating-mesh nebulizer, answered to a phone standardized questionnaire specifying the different techniques of maintenance of the nebulizer.

Combined Effects of in Utero and Adolescent Tobacco Smoke Exposure on Lung Function in C57Bl/6J Mice.

Background: Fetal determinants of airway function, such as exposure to maternal cigarette smoke (CS), may create a predisposition to adult airflow obstruction and chronic obstructive pulmonary disease (COPD) in adulthood. It has been suggested that active smoking in adolescence and preexisting airflow obstruction have synergistic deleterious effects.

Objective: We used a mouse model to investigate whether there is a synergistic effect of exposure to CS and during adolescence on lung function.

[Not Available].

Use of inhaled treatments in acute viral bronchiolitis in infants. Inhaled therapies are widely used by practitioners for treating acute viral bronchiolitis. Therefore, their efficacy has a low level of proof that does not sustain their use.

Aspergillus fumigatus components distinguish IgE but not IgG4 profiles between fungal sensitization and allergic broncho-pulmonary aspergillosis.

Aspergillus fumigatus is the causative agent of allergic broncho-pulmonary aspergillosis. Prompt and accurate diagnosis may be difficult to achieve with current clinical and laboratory scores, which do not include immune responses to recombinant A. fumigatus allergens.

[Fat overload syndrome].

We report the case of a 21-month-old child suffering from pulmonary fibrosis, who presented with acute respiratory distress and liver damage, due to an accidental overdose of intravenous lipid emulsion. This poisoning is a rare entity, whose potential severity and almost exclusive iatrogenic effect deserve to be remembered.