In paediatrics, the pulmonary function test (PFT) is most often performed to support the diagnosis or in follow-up of asthma patients. Whatever the pathology responsible for respiratory symptoms and/or functional impairment, repeated PFTs make it possible to establish a prognosis (pulmonary function trajectories…) and to orient preventive interventions. PFT can be performed routinely from the age of three years, provided that the following requirements are met: suitable techniques and equipment, staff trained to apply the techniques and to receive young children, reference values for each technique indicating the limits of normal values and of between-test significant variation. From the age of three, children can be subjected to tidal breathing measurement of: resistance of the respiratory system (oscillometry, Rrs; airflow interruption, Rint) or of airways specific resistance (sRaw) and functional residual capacity (by applying a dilution technique). With maturity, the child will become capable of mobilizing his or her slow vital capacity to measure total lung capacity (TLC), once again by applying a dilution technique, then later by breathing against a closed shutter (plethysmography TLC and Raw). Finally, the child will be able to carry out forced expiration (forced spirometry) along with all of the other PFTs. It is important to take into account the paediatric adaptations specified in the international recommendations regarding the performance, reproducibility and quality of PFTs targeting this population.

Ehlers-Danlos syndromes (EDS) represent a heterogeneous group of heritable connective tissue disorders characterized by the clinical \"triad\" consisting in joint hypermobility, skin hyperextensibility and tissue fragility. Respiratory manifestations associated with EDS are frequent and variable. They vary mainly according to the type of EDS. In hypermobile and classical EDS, the most frequent non-vascular types, dyspnea is a common symptom. Its etiologies are wide-ranging and can coexist in the same patient: asthma, respiratory muscle weakness, chest wall abnormalities, upper and lower airway collapse. The prevalence of obstructive sleep apnea syndrome in nvEDS is high. Identification of the relevant dyspnea mechanism is essential to providing appropriate therapeutic measures. In vascular EDS (vEDS), the main pulmonary complications are pneumothorax, hemothorax and hemoptysis. As they frequently precede the diagnosis of vEDS by several years, it is imperative to raise the possibility of vEDS in a young patient with spontaneous pneumothorax or hemothorax. The presence of suggestive computed tomography parenchymal abnormalities (emphysema, clusters of calcified nodules, cavitated nodule) can be an aid to diagnosis. Treatment is based on the usual approaches, which must be carried out with caution by an experienced operator fully informed of the diagnosis. Better knowledge of respiratory manifestations of EDS by the pneumological community would improve patient care and pave the way for further research.

BACKGROUND: Allergic asthma has variable characteristics depending on the region of occurrence. Management is often organized without easy access to a specialized pneumo-allergological evaluation.
OBJECTIVE: PRIMARY: To characterize allergic asthma in patients followed at the Nancy University Hospital.
UNASSIGNED: To estimate the impact at 12 months of the proposed specialized management.
METHODS: Retrospective study including allergic asthmatic adults conducted between 01/01/2016 and 31/12/2018. Parameters studied (aside from descriptive parameters): asthma control score ACQ-7 (Asthma Control Questionnaire) and lung function at inclusion and at one year.
RESULTS: One hundred and fifteen patients were included (age 42±16 years, women 63%, non-smokers 70%). Even though asthma symptoms arose at 19±18 years, the specialized consultation was performed 21±17.5 years later. Frequent sensitizations involved house dust mites (49%), cat dander (43%), grasses (43%). Patients tended to underestimate the frequency of their allergies (mites 36% P=0.011, pollens 36% P=0.001, pet dander 42% P=0.003, molds 80% P=0.007). At 1-year follow-up both the ACQ-7 score (1.3±1.3 vs. 0.8±0.9; P=0.007) and the forced expiratory volume per second (81.3±20.8% vs. 86.2±21.9%; P=0.002) had improved.
CONCLUSIONS: The specificities of allergic asthmatics followed up in Nancy: late age of asthma onset of asthma and specialized consultation; predominant sensitization to cat dander and Betulaceae pollens; underestimation of allergies by the patient. Specialized care seems to important in the management of allergic asthma. While access to specialized care remains problematic, it could be facilitated by the creation of dedicated networks.

BACKGROUND: Pectus excavatum (PE) is the most common congenital chest wall deformity, whose cardiopulmonary consequences are controversial. PE surgery is in our experience usually performed for aesthetic reasons.
OBJECTIVE: The aim of this study was to evaluate the impact of PE on respiratory function and exercise capacity in patients with PE before patient-specific silicone implant correction.
METHODS: This monocentric prospective study conducted at Toulouse University Hospital included sixty patients scheduled for custom-made silicone implants correction. Respiratory function (pulmonary function tests (FPTs)) and exercise capacity (VO2 max) were measured before surgery.
RESULTS: Before surgery, no (0/60) restrictive lung disease was detected, with a mean total lung capacity (TLC) of 98.5% of predicted value (IC 95%; 80.4-137). Median VO2 max (n=56) was normal (89% predicted), with no cardiac limitation.
CONCLUSIONS: In this cohort, PE had no impact on respiratory function nor exercise capacity. In patients without cardiac or respiratory effects of PE, silicone implants should be considered the preferred approach as it adequately addressed patients\' main complaint of low self-esteem.

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The intervention of physiotherapists in the intensive care unit allows the optimisation of the care project through the assessment and management of the motor, respiratory and swallowing functions of patients. If COVID-19 has only slightly modified the practice of these professionals, the impact of the pandemic on intensive care units has reinforced the added value of their care.

BACKGROUND: The aim of this study was to evaluate the impact of the transition period from childhood to adulthood in patients with cystic fibrosis (CF) being followed up in our reference center.
METHODS: The clinical, functional, inflammatory and microbiological parameters of all transition patients were compared two years before (T-2) and two years after the transfer (T+2) from paediatric to adult centers and further analysed according to whether the transition conditions were optimal or suboptimal.
RESULTS: Twenty-eight patients were included. The mean age at the transfer visit was 19.5 years (±3.5). There were no deaths during the study period. Consultations were more numerous at T-2 [14.5 (±5.9) vs. 12.0 (±5.1), P<0.004]. Chronic colonization with Pseudomonas aeruginosa was more frequent at T+2 (46.4% vs. 17.9%, P=0.021). A progressive decrease in FEV1 and FVC was observed between T-2 and T+2. The number of pulmonary exacerbations was lower in the optimal transition group.
CONCLUSIONS: The period of transition from childhood to adulthood in patients with CF appears to be associated with functional and microbiological changes.

Chronic obstructive pulmonary disease (COPD) most often results from the inhalation of toxic agents. Cigarette smoking still remains the principal cause but the pertinence of occupational COPD is now clearly established. After a brief overview of the epidemiology of this \"other COPD\", the clinical and functional characteristics are summarized, taking into account recent advances in this field. The combined effects of occupational exposure and tobacco are also considered, providing evidence of the need to continuously reinforce campaigns of education and prevention in occupational COPD.

BACKGROUND: Beneficial effects of physical exercise have been previously demonstrated in patients with chronic obstructive pulmonary disease. The aim of this systematic review was to summarize the evidence supporting physical exercise to improve on lung function, exercise capacity and quality of life in cystic fibrosis patients.
METHODS: Medline database was used to search clinical studies from 2000 to 2015. We also analyzed the bibliographic section of the included studies, in order to identify additional references.
RESULTS: A total of 17 studies were identified. A great disparity was found in the results of the different studies. No systematic benefit was found on lung function, exercise capacity or quality of life. No relationship between the type of program and the benefits achieved was observed.
CONCLUSIONS: Evidence that physical exercise benefits lung function, exercise capacity and quality of life in cystic fibrosis patient is inconsistent and evidence does not support a particular standardized program for all patients.

The lungs of infants with cystic fibrosis (CF) have been considered to be normal at birth. However, recent data indicates that this is unlikely to be true in most cases. Animal CF-models developed in the early 2000s have shown that constitutional airway narrowing may be present at birth, and is associated with both functional and structural abnormalities. Longitudinal birth cohort studies have shown that 25 % of CF infants followed in specialized centers, while being asymptomatic, showed decreased lung function at 3months of age. Air trapping was present in 68 % and bronchiectasis in 28 % of patients at the same age. The presence of neutrophil elastase in the bronchoalveolar lavage at 3months of age tripled the risk of bronchiectasis at the age of 3years. Currently available tools such as infant pulmonary function tests (both the jacket and multiple breath washout) as well as high-resolution volume controlled chest-computed tomography or functional magnetic resonance imaging will facilitate early intervention trials in the very near future. The role of such tools for the routine follow-up of patients, and the ability of early therapeutic interventions to alter the natural history of CF-lung disease should soon be established.