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Biomedical subjects

B Fauroux

Publications and source records attributed to B Fauroux.

At least 37 records · Page 2Linked to original sources

[Chronic respiratory insufficiency in the child].

Chronic respiratory insufficiency in childhood can be caused by a heterogeneous group of diseases whose management and prognosis differ. Bronchopulmonary dysplasia in infants, and cystic fibrosis and neuromuscular diseases in older children, represent the most common causes of chronic respiratory insufficiency. The improvements in medical treatments, with the development of long term oxygen therapy and non invasive mechanical ventilation, have contributed to the increased survival and the better quality of life of these children. An objective evaluation of treatments, such as long term oxygen therapy and non invasive mechanical ventilation, are warranted to improve the indications and benefits of these therapies.

Bronchopulmonary Dysplasia↗

In vivo physiologic comparison of two ventilators used for domiciliary ventilation in children with cystic fibrosis.

OBJECTIVE: Home noninvasive mechanical ventilation (NIMV) is used with increasing frequency for the treatment of patients with respiratory failure caused by cystic fibrosis, yet the optimal mode of ventilation in such children is unknown. We compared the physiologic short-term effects of two ventilators with different modes (one pressure support and the other assist control/volume-targeted [AC/VT]) commonly used for domiciliary ventilation. DESIGN: Prospective, randomized, crossover comparison of two ventilators with different modes. SETTING: Tertiary pediatric university hospital. PATIENTS: Eight children with cystic fibrosis (age, 11-17 yrs) and chronic respiratory failure (pH 7.4 +/- 0.0; PaO2, 57.5 +/- 7.5 torr; PaCO2, 46.1 +/- 2.5 torr), naive to NIMV. INTERVENTIONS: Two 20-min runs of pressure support and AC/VT ventilation were performed in random order, each run being preceded and followed by 20 mins of spontaneous breathing. MEASUREMENTS: Flow and airway pressure and esophageal and gastric pressures were measured to calculate esophageal (PTPes) and diaphragmatic pressure-time product (PTPdi) and the work of breathing. RESULTS: The two NIMV sessions significantly improved blood gas variables and increased tidal volume with no change in respiratory rate. Indexes of respiratory effort decreased significantly during the two modes of NIMV compared with spontaneous breathing, with PTPdi/min decreasing from 497.8 +/- 115.4 cm H2O x sec x min(-1) during spontaneous breathing to 127.8 +/- 98.3 cm H2O x sec x min(-1) and 184.3 +/- 79.8 cm H2O x sec x min(-1), during AC/VT and pressure support, respectively (p <.0001), and the work of breathing decreasing from 1.83 +/- 0.12 J.L-1 during spontaneous breathing to 0.48 +/- 0.32 J.L-1 and 0.75 +/- 0.30 J.L-1, during AC/VT and pressure support, respectively (p <.0001). In addition, the effect of AC/VT ventilation was significantly superior to pressure support judged by PTPes and the work of breathing, but this result was explained by three patients who adapted extremely well to the AC/VT ventilation, with the disappearance of ventilator triggering, in effect adopting a controlled mode. There was a correlation between the improvement in PTPdi/min or the work of breathing and patient's subjective impression of comfort during the AC/VT ventilation. CONCLUSIONS: In awake, stable children with cystic fibrosis, both AC/VT and pressure support unloaded the respiratory muscles. The disappearance of ventilator triggering occurred in a subgroup of patients during AC/VT ventilation, and this explained the good tolerance and the superiority of this mode in the present study.

Adolescent↗

Pulmonary sarcoidosis in children: a follow-up study.

Progression of pulmonary sarcoidosis in children remains poorly documented. The aim of this work was to gather follow-up information on pulmonary outcomes in children with sarcoidosis and to obtain data of relevance to a discussion of the optimal length and regimen of glucocorticoid therapy. In the present study, the authors experience of pulmonary sarcoidosis in 21 children referred to the paediatric pulmonary department over a 10-yr period is reported with a documented follow-up of at least 4 yr. Evaluation of the disease during the follow-up included analysis of clinical manifestations, chest radiographs, pulmonary function tests with measurements of the vital capacity (VC), dynamic lung compliance (CL,dyn), lung transfer for CO (TL,CO), and arterial blood gases, as well as bronchoalveolar lavage (BAL) with determination of total and differential cell counts. After initial evaluation the decision was a careful observation of four children without therapy. Corticosteroid treatment was initiated in 17 children. Analysis of results indicated that after 6-12 months of treatment most clinical manifestations of the disease and chest radiograph abnormalities disappeared, and beneficial effects on VC and TL,CO were apparent. After 18 months of steroids no benefit on pulmonary function tests could be noticed, with mainly persistence of alterations of CL,dyn. Results of BAL studies documented the presence of an alveolitis with increased lymphocyte populations throughout the follow-up. Relapses were observed in four children during tapering of prednisone; they were not reported after discontinuation of steroid therapy. Taken together data obtained in the presented population can lead to the following suggestions for the management of pulmonary sarcoidosis in children. BAL should be performed at the initial evaluation to document alveolitis; however, nothing seems to be gained from repeating this investigation during follow-up in the absence of specific reasons. Once the decision to initiate glucocorticoid therapy is made, 18 months may be a reasonable treatment duration. Discontinuation of therapy can be decided even if the pulmonary function tests remain abnormal, but the child should then be carefully monitored for a relapse.

Adolescent↗

[Chronic respiratory failure in children. Evaluation and management].

Chronic respiratory insufficiency in childhood can be caused by a heterogeneous group of diseases of which the management and prognosis differ. Bronchopulmonary dysplasia in infants and cystic fibrosis and neuromuscular diseases in older children, represent the most common causes of chronic respiratory insufficiency. The improvements in medical treatments, with the development of long term oxygen therapy and non invasive mechanical ventilatory assistance, have contributed to the increased survival and the better quality of life of these children. An objective evaluation of treatments, such as long term oxygen therapy and non invasive mechanical ventilation, are warranted to improve the indications and benefits of these therapies.

Child↗

Ozone: a trigger for hospital pediatric asthma emergency room visits.

A time-series study was carried out in Paris from January 1 to December 31, 1988 with the aim of investigating the association between urban air pollution and daily emergency room visits for asthma in a pediatric hospital. Levels of black smoke, sulfur dioxide, nitrogen dioxide, and ozone were monitored throughout the study area, and meteorological data were collected. Influenza epidemics and pollen periods were identified. Health data were collected from a pediatric hospital emergency room. Case definition of asthma attacks was based on clinical diagnosis. Children were included in the study if: 1) they were 1 to 15 years old; 2) they had doctor-diagnosed asthma and were followed in our asthma outpatient clinic; and 3) they were residents in the Paris region. The relation between daily asthma visit counts and air pollution levels was assessed, using a multiple linear regression model and taking into account temporal variations and autocorrelation in the data. A thousand and twenty visits for asthma were observed during the study period. A positive statistical association was found between daily asthma visits and daily variations of ozone levels (1 day after exposure, relative risk = 1.52 [95% confidence interval: 1. 06-2.19]) after controlling for monthly and weekly variations, influenza epidemics, periods of pollen exposure, and daily mean temperature (2 days' lag). This study underlines the significant role of ozone as a trigger for asthma attacks in children.

Adolescent↗

[Respiratory distress and drepanocytosis].

Acute chest syndrome (ACS) is characterized by chest pain with dyspnea and recent radiologic abnormalities, and is an acute lung complication whose problem is one of etiology. Alveolar hypoventilation linked to infarcts of the thoracic ribs, thoracoabdominal trauma, subdiaphragmatic pain, the administration of analgesics causing respiratory depression, or sleep disturbance, is a frequent cause of ACS. Bronchoalveolar lavage has revealed the frequency of fat embolism following infarcts in the long bones. Pulmonary vascular occlusion, due to thrombosis or emboli, is rare, as are the infectious pneumonia and pulmonary edema. The pathogenetic mechanisms consist of an alteration of the rheological properties of the blood, the existence of an hypercoagulability state, specific interactions between the abnormal sickle cells and the vascular endothelium, and a dysregulation of the vascular reactivity. Research centered around NO biology has led to an expanded understanding of the critical interdependence of NO, hemoglobin, and the microvasculature. An anemic patient with ACS suffers from loss of pulmonary scavenging and hypoxic pulmonary vasoconstriction and loss of peripheral NO delivery. Interruption of this cycle by transfusing normal (hemoglobin A-containing) erythrocytes might improve all the abnormalities.

Acute Disease↗

Long-term outcome of idiopathic pulmonary hemosiderosis in children.

We retrospectively analyzed the long-term outcome of idiopathic pulmonary hemosiderosis (IPH) in 15 children. IPH started at a mean age of 5 years, and the mean duration of follow-up was 17.2 years (range, 10-36 yr). Four patients developed immune disorders, 3 cases of rheumatoid polyarthritis or rheumatoid polyarthritis-like diseases and 1 case of celiac disease. Respiratory outcome showed that 3 patients had severe symptoms: 2 patients developed severe pulmonary fibrosis resulting in major chronic respiratory insufficiency, and 1 patient had severe asthma. Twelve patients (80%) had mild or no respiratory problems and were able to lead a normal life. According to chest X-ray and pulmonary function test data, 4 patients had normal chest X-ray and no evidence of restrictive syndrome, 6 patients had an interstitial pattern on chest X-ray and evidence of restrictive pattern, 1 patient had an interstitial pattern but normal lung function, and 1 patient had a normal chest X-ray but evidence of mixed obstructive and restrictive pattern. Our results show that long-term survival is possible in patients with IPH. Factors of poor prognosis seem to be the presence of antineutrophil cytoplasm antibodies (ANCA) or other autoantibodies. No other clinical or biological predictive factors for prolonged survival were found.

Adolescent↗

Optimization of aerosol deposition by pressure support in children with cystic fibrosis: an experimental and clinical study.

Nebulized aerosols are commonly used to deliver drugs into the lungs of patients with cystic fibrosis (CF). The aim of this study was to assess the effectiveness of pressure-support (PS) ventilation in increasing aerosol deposition within the lungs of children with CF. An in vitro study demonstrated the feasibility of coupling a breath-actuated nebulizer to a PS device. An in vivo study was done with 18 children (ages 6 to 21 yr) with clinically stable CF, each of whom underwent both a standard and a PS-driven ventilation scan (control session and PS session, respectively). In addition, a perfusion scan was used to determine lung outlines and to construct a geometric model for quantifying aerosol deposition by radioactivity counting in MBq. Homogeneity of nebulization was evaluated from the four first-order moments of aerosol distribution in the peripheral and central lung regions. The time-activity nebulization curve was linear in all patients, with higher slopes during the PS than during the control session (0.43 +/- 0.07 [mean +/- SD] MBq/min and 0.32 +/- 0.23 MBq/min, respectively; p < 0.018). Quantitatively, aerosol deposition was about 30% greater after the PS session (4.4 +/- 2.7 MBq) than after the control session (3.4 +/- 2.1 MBq; p < 0.05). Similarly, deposition efficacy (as a percentage of nebulizer output) was significantly better during the PS session than during the control session (15.3 +/- 8.3% versus 11.5 +/- 5.7%, p < 0.05). No differences in the regional deposition pattern or in homogeneity of uptake were observed. In conclusion, our data show that driving the delivery of a nebulized aerosol by noninvasive PS ventilation enhances total lung aerosol deposition without increasing particle impaction in the proximal airways.

Adolescent↗

Genotype analysis and phenotypic manifestations of children with intermediate sweat chloride test results.

STUDY OBJECTIVES: Cystic fibrosis (CF) is one of the most common inherited diseases among whites. Since the cloning of the CF transmembrane conductance regulator (CFTR) gene, a number of studies have focused on associations between the genotype and phenotype in CF. This had led to the progressive identification of new groups of patients, including those who have mild lung disease and those who have normal sweat chloride values (< 60 mEq/L). The aim of the present work was to provide information on the genotype and the phenotypic characteristics of children with intermediate-range sweat chloride test results. PATIENTS AND RESULTS: We focused on children referred to the pulmonary department for various types of pulmonary disease and who had several sweat chloride test results with median values in the range of 40 to 60 mEq/L. Twenty-four patients over a 10-year period were enrolled (mean age, 4.8 years). Respiratory manifestations at initial evaluation included recurrent bronchitis, wheezing, chronic cough, and pneumonia. The duration of the follow-up ranged from 0.5 to 10.5 years. Sputum cultures revealed the presence of Haemophilus influenzae (10 children), Staphylococcus aureus (4 children), and Pseudomonas aeruginosa (3 children). Pancreatic insufficiency was found in two patients. Analysis of the entire coding sequence allowed identification of 16 known mutations in CFTR gene. Fifteen chromosomes (31.2%) carried a mutation in CFTR gene and one allele carried two mutations. Three patients were homozygous or double heterozygous (DeltaF508/DeltaF508, DeltaF508/3849 + 10 kb C-->T, S1235R/G551D). The 5-thymidine allele was identified in four children. CONCLUSION: These results indicate an higher frequency of CFTR gene mutations in patients with borderline sweat chloride test results, compared to data reported in the general population. They lead to the recommendations for complete pulmonary and GI investigations in this group of patients, as well as assiduous care and medical follow-up.

Adolescent↗

[Diagnosis and management of cystic fibrosis in children].

Cystic fibrosis is a genetic disease occurring more frequently in Caucasians. The cystic fibrosis gene, cloned in 1999, codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. Dysfunction of this protein leads to the clinical manifestations of cystic fibrosis, mainly lung disease and exocrine pancreas disorders. The pathophysiology of the respiratory component is quite complex, basically related to major and excessive inflammatory processes and to early microbial colonization. Respiratory physical therapy is a key element to management of the respiratory disorder. Antibiotic treatments should be adapted to the bacterial ecology, mainly using antistaphylococcal and antihaemophilus drugs initially, then directed against Pseudomonas aeruginosa. Other drugs including inhaled antiinflammatory drugs are currently in the evaluation stage. In addition, nutritional care and correction of pancreas insufficiency are necessary. The diagnosis of this disease must be made early although systematic neonatal screening is not proposed. Early diagnosis is necessary for improved care and prognosis. Currently, median survival is 29 years. This survival time should probably improve with better understanding of the pathophysiological mechanisms and new therapeutic perspectives.

Adult↗

Comparison of randomly amplified polymorphic DNA analysis and pulsed-field gel electrophoresis for typing of Moraxella catarrhalis strains.

Randomly amplified polymorphic DNA (RAPD) and pulsed-field gel electrophoresis (PFGE) for the analysis of 13 Moraxella catarrhalis isolates, 11 successive strains isolated from sputa of five children and 2 isolates obtained the same day from twins, were compared. RAPD and PFGE both yielded nine types from the 13 isolates, showing a chronic colonization with one strain in three patients and a successive colonization with different strains in two patients. The promising results obtained with RAPD should be confirmed with a larger number of strains, but RAPD seems as suitable as PFGE for the typing of M. catarrhalis.

Adolescent↗

Chest physiotherapy in cystic fibrosis: improved tolerance with nasal pressure support ventilation.

OBJECTIVE: Chest physiotherapy (CPT) is an integral part of the treatment of patients with cystic fibrosis (CF). CPT imposes additional respiratory work that may carry a risk of respiratory muscle fatigue. Inspiratory pressure support ventilation (PSV) is a new mode of ventilatory assistance designed to maintain a constant preset positive airway pressure during spontaneous inspiration with the goal of decreasing the patient's inspiratory work. The aim of our study was 1) to evaluate respiratory muscle fatigue and oxygen desaturation during CPT and 2) to determine whether noninvasive PSV can relieve these potential adverse effects of CPT. METHODS: Sixteen CF patients in stable condition with a mean age of 13 +/- 4 years participated to the study. For CPT, we used the forced expiratory technique (FET), which consisted of one or more slow active expirations starting near the total lung capacity (TLC) and ending near the residual volume. After each expiration, the child was asked to perform a slow, nonmaximal, diaphragmatic inspiration. After one to four forced breathing cycles, the child was asked to cough and to expectorate. A typical 20-minute CPT session consisted of 10 to 15 FET maneuvers separated by rest periods of 10 to 20 breathing cycles each. During the study, each patient received two CPT sessions in random order on two different days, at the same time of day, with the same physiotherapist. During one of these two sessions, PSV was provided throughout the session (PSV session) via a nasal mask using the pressure support generator ARM25 designed for acute patients (TAEMA, Antony, France). The control session was performed with no nasal mask or PSV. Both CPT sessions were performed without supplemental oxygen. Lung function and maximal inspiratory pressures (PImax) and expiratory pressures (PEmax) were recorded before and after each CPT session. RESULTS: Mean lung function parameters were comparable before the PSV and the control sessions. Baseline pulse oximetry (SpO2) was significantly correlated with the baseline vital capacity (% predicted) and forced expiratory volume in 1 second (FEV1) (% predicted). PSV was associated with an increase in tidal volume (Vt) from 0.42 +/- 0.01 liters to 1.0 +/- 0.02 liters. Respiratory rate was significantly lower during PSV. SpO2 between the FET maneuvers was significantly higher during PSV as compared with the control session. SpO2 decreases after FET were significantly larger during the control session (nadir: 91.8 +/- 0. 7%) than during the PSV session (93.8 +/- 0.6%). Maximal pressures decreased during the control session (from 71.9 +/- 6.1 to 60.9 +/- 5.3 cmH2O, and from 85.3 +/- 7.9 to 77.5 +/- 4.8 cmH2O, for PImax and PEmax, respectively) and increased during the PSV session (from 71.6 +/- 8.6 to 83.9 +/- 8.7 cmH2O, and from 80.4 +/- 7.8 to 88.0 +/- 7.4 cmH2O, for PImax and PEmax, respectively). The decrease in PEmax was significantly correlated with the severity of bronchial obstruction as evaluated based on baseline FEV1 (% predicted). Forced expiratory flows did not change after either CPT session. The amount of sputum expectorated was similar for the two CPT sessions (5.3 +/- 5.3 g vs 4.6 +/- 4.8 g after the control and PSV session, respectively; NS). Fifteen patients felt less tired after the PSV session. Ten patients reported that expectoration was easier with PSV, whereas 4 did not note any difference; 2 patients did not expectorate. Nine patients expressed a marked and 5 a small preference for PSV, and 2 patients had no preference. The physiotherapists found it easier to perform CPT with PSV in 14 patients and did not perceive any difference in 2 patients. DISCUSSION: Our study in CF children shows that respiratory muscle performance, as evaluated based on various parameters, decreased after CPT and that significant falls in oxygen saturation occurred after the FET maneuvers despite the quiet breathing periods between each FET cycle. These unwanted effects of CPT were

Adolescent↗

[Pathophysiology of acute thoracic syndrome].

Pulmonary complications are the leading cause of morbidity and mortality in sickle cell disease patients. Acute chest syndrome (ACS), in which chest pain and dyspnea, occurs in combination with a recent chest radiograph abnormality, raises both diagnostic and therapeutic challenges. The pathogenesis of ACS involves alterations in blood rheology, increased coagulability, and, above all, increased adhesion of sickle cells to the vascular endothelium and nitric oxide-mediated dysregulation of vascular reactivity. Sickle cell disease thus impacts all the cells in the vascular environment. Recently gained insights into pathophysiology offer hope that new treatments for preventing and treating acute and chronic pulmonary complications will soon become available.

Acute Disease↗