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Biomedical subjects

Brigitte Fauroux

Publications and source records attributed to Brigitte Fauroux.

At least 19 recordsLinked to original sources
PubMed2006

Respiratory muscle testing: a valuable tool for children with neuromuscular disorders.

RATIONALE: Data on respiratory muscle performance in children with neuromuscular disorders are limited. OBJECTIVES: The aim of this study was to assess respiratory muscle strength by volitional and nonvolitional tests and to compare these tests with forced vital capacity. METHODS: Inspiratory muscle strength was assessed by measuring transdiaphragmatic and esophageal pressures generated during volitional and nonvolitional maneuvers, whereas expiratory muscle strength was assessed by measuring the gastric pressure generated during a cough maneuver. Lung volumes were assessed by measuring forced vital capacity. MEASUREMENTS AND MAIN RESULTS: Forty-one patients with Duchenne muscular dystrophy (n = 20), spinal amyotrophy (n = 8), and congenital myopathy (n = 13) were included, aged 2 to 18 yr. All the patients were able to perform the sniff and the cough maneuver. Sniff transdiaphragmatic pressure decreased with age in Duchenne patients, whereas it increased with age in patients with spinal amyotrophy and congenital myopathy. Magnetic stimulation of the phrenic nerves was obtained in all patients. Twenty-five (61%) patients were able to perform forced vital capacity. In the three groups of patients, a positive correlation was observed between volitional, assessed by the sniff maneuver, and nonvolitional respiratory muscle tests, assessed by the magnetic stimulation of the phrenic nerves. Also, forced vital capacity correlated with sniff transdiaphragmatic pressure and cough gastric pressure. CONCLUSIONS: Volitional respiratory muscle tests correlated with nonvolitional tests and with forced vital capacity. Simple volitional respiratory muscle tests constitute a valuable tool for the assessment of respiratory muscle strength in young patients with neuromuscular disorders.

Adolescent↗
PubMed2006

Noninvasive positive pressure ventilation: five years of experience in a pediatric intensive care unit.

OBJECTIVES: To evaluate the feasibility and outcome of noninvasive positive pressure ventilation (NPPV) in daily clinical practice. DESIGN: Observational retrospective cohort study. SETTING: Pediatric intensive care unit in a university hospital. PATIENTS: : Patients treated by NPPV, regardless of the indication, during five consecutive years (2000-2004). MEASUREMENTS AND RESULTS: A total of 114 patients were included, and 83 of the 114 patients (77%) were successfully treated by NPPV without intubation (NPPV success group). The success rate of NPPV was significantly lower (22%) in the patients with acute respiratory distress syndrome (p < .05) than in the other patients. The Pediatric Risk of Mortality II (p = .003) and Pediatric Logistic Organ Dysfunction scores (p = .002) at admission were significantly higher in patients who were unsuccessfully treated with NPPV (NPPV failure group). Baseline values of Pco2, pulse oximetry, and respiratory rate did not differ between the two groups. A significant decrease in Pco2 and respiratory rate within the first 2 hrs of NPPV was observed in the NPPV success group. Multivariate analysis showed that a diagnosis of acute respiratory distress syndrome (odds ratio, 76.8; 95% confidence interval, 4.4-1342; p = .003) and a high Pediatric Logistic Organ Dysfunction score (odds ratio, 1.09; 95% confidence interval, 1.01-1.17; p = .01) were independent predictive factors for NPPV failure. A total of 11 patients (9.6%), all belonging to the NPPV failure group, died during the study. CONCLUSIONS: This study demonstrates the feasibility and efficacy of NPPV in the daily practice of a pediatric intensive care unit. This ventilatory support could be proposed as a first-line treatment in children with acute respiratory distress, except in those with a diagnosis of acute respiratory distress syndrome.

Adolescent↗
PubMed2006

Value of the chlorhexidine decontamination method for recovery of nontuberculous mycobacteria from sputum samples of patients with cystic fibrosis.

The chlorhexidine method was compared to the N-acetyl-l-cysteine-NaOH-oxalic acid decontamination method currently recommended for the recovery of nontuberculous mycobacteria (NTM) from patients with cystic fibrosis. Sputum samples (n = 827) treated with chlorhexidine yielded twice as many NTM-positive cultures as those treated by the reference method (54 [6.50%] versus 27 [3.25%]; P < 0.0001) despite a higher contamination rate (20% versus 14.2%; P = 0.0017).

Acetylcysteine↗
PubMed2005

Mechanical limitation during CO2 rebreathing in young patients with cystic fibrosis.

The aim of the study was to determine whether a decrease in the ventilatory response to carbon dioxide (CO2) in children with cystic fibrosis (CF) is related to a mechanical limitation of the respiratory muscle capacity. The ventilatory response during CO2 rebreathing was performed in 15 patients (mean forced expiratory volume in 1 s (FEV1): 37 +/- 21% predicted, mean arterial CO2: 41+/- 5 mmHg). The slope of the minute ventilation normalised for weight per mmHg CO2 increment correlated negatively with respiratory muscle output, assessed by the oesophageal (p = 0.002), the diaphragmatic pressure time product (p = 0.01), and the tension time index (p = 0.005). In addition, this slope was correlated with dynamic lung compliance (p < 0.0001) and FEV1 (p = 0.03) but not with airway resistance and maximal transdiaphragmatic pressure. Therefore, an excessive load imposed on the respiratory muscles explains the blunting of the ventilatory response to CO2 in young patients with CF.

Adolescent↗
PubMed2005

Carcinoid and mucoepidermoid bronchial tumours in children.

UNLABELLED: The aim of the study was to determine the characteristic features and outcome of carcinoid or mucoepidermoid tumours in children. A retrospective analysis of all patients treated for a carcinoid or mucoepidermoid tumour in France between 1984 and 2001 was performed. There were 11 cases of carcinoid tumour and 6 cases of mucoepidermoid tumour. The mean age of the patients was 10.5+/-3.0 years, with a range of 5 to 15 years. Twelve and 6 patients presented with evidence of bronchial obstruction and haemoptysis, respectively. Fibre optic bronchoscopy confirmed the presence of a bronchial tumour in all cases and endobronchial biopsies were diagnostic in 11 of 12 cases. A chest CT scan revealed the presence of a hypervascular tumour in 8 of 12 patients. The distribution of the location of the tumours was equal between the right and the left lung, and, in 9 cases, the airways were totally occluded by the tumour. Complete surgical resection (lobectomy in 15 patients and pneumonectomy in 2 patients) was performed in all cases without pre-operative chemotherapy or radiotherapy. The mean duration of follow-up was 4.0+/-3.0 years. In 2 patients, auscultation assymetry and an episode of haemoptysis revealed the recurrence of a mucoepidermoid tumour, successfully cured by removal of the tumour and chemotherapy and radiotherapy in one child. No death was observed. CONCLUSION: Pulmonary carcinoid and mucoepidermoid tumours are rare in children. Bronchoscopic removal should not be performed. With aggressive surgical therapy, the prognosis is excellent. Fibre optic bronchoscopy confirms the presence of an endobronchial mass. A biopsy is needed for diagnosis and complete surgical removal is the treatment of choice. Long-term results are excellent but a clinical follow-up is recommended.

Adolescent↗
PubMed2005

Facial side effects during noninvasive positive pressure ventilation in children.

OBJECTIVE: The study quantified the side effects of nasal masks use for noninvasive positive pressure ventilation (NPPV) in children. DESIGN AND SETTING: Cross-sectional retrospective study in a tertiary pediatric university hospital. PATIENTS: Patients with obstructive sleep apnea (n=16), neuromuscular disorders (n=14), and cystic fibrosis (n=10). INTERVENTIONS: Clinical evaluation of facial tolerance. MEASUREMENTS AND RESULTS: A skin injury was observed in 19 patients (48%), with a transient erythema in 7 (18%), prolonged erythema in 9 (23%), and skin necrosis in 3 (8%). Skin injury was associated with age over 10 years (OR=16) and use of a commercial mask (OR=15) and was less frequent in patients with obstructive sleep apnea. The change of a commercial mask for a custom-made mask was associated with reduction in the skin injury score. Global facial flattening was present in 68% of the patients. No correlation was observed with age, daily or cumulative use of NPPV, or the type of mask. A maxillary retrusion was present in 37% of patients. No correlation was observed with age or the type of mask or the underlying disease, but an association was found with a longer daily use of NPPV (OR=6.3). CONCLUSIONS: The prevalence of facial side effects is clinically significant in children using NPPV. Systematic maxillofacial follow-up enables these effects to be identified. Remedial measures could include the change of the interface or reducing the daily use of NPPV.

Adolescent↗
PubMed2005

Influence of interleukin-10 on Aspergillus fumigatus infection in patients with cystic fibrosis.

Recent evidence suggests that genetic polymorphisms that affect the production of interleukin (IL)-10 may play a role in the response to pathogens in cystic fibrosis (CF). The present study was designed to investigate a possible association between alleles carried at position -1082 in the promoter region of the IL-10 gene and clinical data on 378 patients with CF. After adjustment for potential confounding variables, a significant relationship was found between the -1082GG genotype and both colonization with Aspergillus fumigatus and allergic bronchopulmonary aspergillosis. In addition, higher serum levels of IL-10 were observed in patients colonized with A. fumigatus. These results suggest that polymorphisms in the promoter region of the IL-10 gene may influence the host response to A. fumigatus in the context of CF.

Adolescent↗
PubMed2005

Noninvasive positive pressure ventilation in infants with upper airway obstruction: comparison of continuous and bilevel positive pressure.

OBJECTIVE: This study evaluated the efficacy of noninvasive continuous positive pressure (CPAP) ventilation in infants with severe upper airway obstruction and compared CPAP to bilevel positive airway pressure (BIPAP) ventilation. DESIGN AND SETTING: Prospective, randomized, controlled study in the pulmonary pediatric department of a university hospital. PATIENTS: Ten infants (median age 9.5 months, range 3-18) with laryngomalacia (n=5), tracheomalacia (n=3), tracheal hypoplasia (n=1), and Pierre Robin syndrome (n=1). INTERVENTIONS: Breathing pattern and respiratory effort were measured by esophageal and transdiaphragmatic pressure monitoring during spontaneous breathing, with or without CPAP and BIPAP ventilation. MEASUREMENTS AND RESULTS: Median respiratory rate decreased from 45 breaths/min (range 24-84) during spontaneous breathing to 29 (range 18-60) during CPAP ventilation. All indices of respiratory effort decreased significantly during CPAP ventilation compared to unassisted spontaneous breathing (median, range): esophageal pressure swing from 28 to 10 cmH(2)O (13-76 to 7-28), esophageal pressure time product from 695 to 143 cmH(2)O/s per minute (264-1417 to 98-469), diaphragmatic pressure time product from 845 to 195 cmH(2)O/s per minute (264-1417 to 159-1183) During BIPAP ventilation a similar decrease in respiratory effort was observed but with patient-ventilator asynchrony in all patients. CONCLUSIONS: This short-term study shows that noninvasive CPAP and BIPAP ventilation are associated with a significant and comparable decrease in respiratory effort in infants with upper airway obstruction. However, BIPAP ventilation was associated with patient-ventilator asynchrony.

Airway Obstruction↗
PubMed2005

Paediatric sarcoidosis.

Sarcoidosis is a multisystem granulomatous disease of unknown aetiology, most commonly affecting young adults. Divergent prevalence rates and clinical appearances in different races indicate the existence of predisposing genes, with probably a major effect of the major histocompatibility complex. The diagnosis is relatively rare in children. Lesions can occur in almost any tissue or organ but the lungs, lymph nodes, eyes, skin and liver are the most commonly involved. Clinical symptoms are non-specific and often minor. The diagnosis can only be supported by typical histopathological findings with non-caseating epithelioid-cell granulomas. The prognosis seems to be more severe in younger children and in cases of multi-organ involvement. Corticosteroids are the therapeutic agents most commonly used and are indicated in cases of significant lung or eye lesions, cardiac, neurological, or multiorgan involvement. Close monitoring is mandatory during and after treatment because relapses are common.

Arthritis↗
PubMed2005

Age-related prevalence and distribution of nontuberculous mycobacterial species among patients with cystic fibrosis.

We studied the prevalence and species distribution of nontuberculous mycobacteria (NTM) in relation to age in 385 patients with cystic fibrosis (CF) (mean age +/- standard deviation [range], 12.0 +/- 6.1 [1 to 24] years; sex ratio, 0.53) attending three Parisian centers. The overall prevalence of NTM in sputum was 8.1% (31 out of 385). The following NTM were isolated (n = 33): Mycobacterium abscessus (n = 13, 39.4%), Mycobacterium avium complex (MAC) (n = 7, 21.2%), Mycobacterium gordonae (n = 6, 18.2%), and other (n = 7, 21.2%). Sixteen patients met the American Thoracic Society microbiological criteria for NTM infection, including 11 patients positive for M. abscessus, 4 for MAC, and 1 for MAC and Mycobacterium kansasii. The overall prevalence of NTM was significantly lower in patients under 15 years old than for patients equal to or more than 15 years old (4.8 versus 14.9%, respectively; P = 0.001). M. abscessus was isolated at all ages, while MAC was not recovered before 15 years (prevalence of 0.0 and 5.2% in patients aged 1 to 14 and 15 to 24, respectively; P = 0.001).

Adolescent↗
PubMed2004

The effect of back-up rate during non-invasive ventilation in young patients with cystic fibrosis.

OBJECTIVE: The aim of the study was to evaluate the effect of the back-up rate on respiratory effort during non-invasive mechanical ventilation. DESIGN: An in vitro study evaluated the inspiratory trigger in seven domiciliary ventilators. Then, a prospective, randomized, crossover trial compared the effect on respiratory effort of three different back-up rates during pressure support (PS) and assist-control/volume-targeted (AC/VT) ventilation. SETTING: A research unit and a tertiary referral pediatric center. PATIENTS: Ten patients with cystic fibrosis (CF). INTERVENTIONS: During the in vivo study, the back-up rate was progressively increased to the maximum that patients could tolerate (Fmax) and respiratory effort, as judged by pressure/time product of the diaphragm (PTPdi/min), was compared between the two ventilatory modes. RESULTS: Differences were observed between trigger pressure, trigger time delay, trigger pressure/time product and the slope between flow and pressure in the seven ventilators. PS and AC/VT ventilation were associated with a decrease in respiratory effort (PTPdi/min was 518+/-172, 271+/-119 and 291+/-138 cmH(2)O. s(-1). min(-1), for spontaneous breathing, PS and AC/VT ventilation, respectively, p=0.05). During the two modes, increasing the back-up rate to Fmax resulted in a greater reduction in PTPdi/min (p=0.001), which was more pronounced during AC/VT ventilation, due to the automatic adjustment of the inspiratory/expiratory time ratio. CONCLUSIONS: Increasing the back-up rate during PS and AC/VT ventilation decreases respiratory effort in young patients with CF, but this effect was more marked with AC/VT ventilation.

Adolescent↗
PubMed2004

The pathogenesis of interstitial lung diseases in children.

The pathological changes observed in interstitial lung disease (ILD) are characterised by derangements of the alveolar walls. For a long time, the prevailing hypothesis has emphasised the key role of a persistent alveolitis that injures the lung and modulates fibrogenesis, regardless of initiating agents. The current concept on ILD pathogenesis relies on an epithelial/fibroblastic pathway with epithelial injury and activation, formation of subepithelial fibroblast/myofibroblast foci and excessive accumulation of extracellular matrix. An essential step in the restoration of alveolar integrity is the rapid re-epithelialisation of the altered surface, mainly through epithelial proliferation and migration. In the context of lung growth and development in paediatric ILD, it is suggested that the programmed production of mitogenic factors may promote the process of re-epithelialisation and may help to counteract the altered secretion of mediators involved in migration and proliferation of fibroblasts and differentiation into myofibroblasts. This is supported by clinical observations indicating that paediatric ILD is more responsive to therapeutic strategies than adult ILD.

Child↗
PubMed2004

Clinical presentation of interstitial lung disease in children.

Interstitial lung disease (ILD) in children represents a heterogeneous group of rare lung disorders characterised by an inflammatory process of the alveolar wall and the pulmonary interstitium, which share common clinical, radiological and histological findings. Clinical presentation of paediatric ILD is non-specific and variable. Respiratory signs and symptoms include tachypnoea, rapid shallow breathing, retractions, cyanosis and crackles. Failure to thrive and respiratory distress are common in infants. Dyspnoea on exertion is a symptom encountered in older children. Non-respiratory symptoms can be present when ILD is part of a systemic disorder. Although non-specific and variable, the clinical presentation at the time of diagnosis has some prognostic value.

Child↗
PubMed2004

Residual gravimetric method to measure nebulizer output.

The aim of this study was to assess a residual gravimetric method based on weighing dry filters to measure the aerosol output of nebulizers. This residual gravimetric method was compared to assay methods based on spectrophotometric measurement of terbutaline (Bricanyl, Astra Zeneca, France), high-performance liquid chromatography (HPLC) measurement of tobramycin (Tobi, Chiron, U.S.A.), and electrochemical measurements of NaF (as defined by the European standard). Two breath-enhanced jet nebulizers, one standard jet nebulizer, and one ultrasonic nebulizer were tested. Output produced by the residual gravimetric method was calculated by weighing the filters both before and after aerosol collection and by filter drying corrected by the proportion of drug contained in total solute mass. Output produced by the electrochemical, spectrophotometric, and HPLC methods was determined after assaying the drug extraction filter. The results demonstrated a strong correlation between the residual gravimetric method (x axis) and assay methods (y axis) in terms of drug mass output (y = 1.00 x -0.02, r(2) = 0.99, n = 27). We conclude that a residual gravimetric method based on dry filters, when validated for a particular agent, is an accurate way of measuring aerosol output.

Aerosols↗
PubMed2004

Nutritional status is an important predictor of diaphragm strength in young patients with cystic fibrosis.

BACKGROUND: The effect of nutritional status and lung disease progression on diaphragm strength in young patients with cystic fibrosis remains unclear. OBJECTIVE: The aim of this study was to investigate the effect of nutritional status and airway obstruction on diaphragm strength. DESIGN: Twitch transdiaphragmatic pressure (Tw Pdi) obtained by bilateral anterior magnetic phrenic nerve stimulation, body mass index (BMI) z score, fat mass, fat-free mass (FFM), arm muscle circumference (AMC), forced expiratory volume in 1 s (FEV(1)), and functional residual capacity (FRC) were measured in 20 patients aged 15.1 +/- 2.8 y (x +/- SD). Values were expressed as a percentage of predicted values. RESULTS: Mean (+/-SD) Tw Pdi was 24.3 +/- 5.5 cm H(2)O. Univariate regression analysis showed positive correlations between Tw Pdi and nutrition scores (BMI z score: r = 0.63, P = 0.003; FFM: r = 0.47, P = 0.04; AMC: r = 0.45, P = 0.04), airway obstruction (FEV(1): r = 0.68, P = 0.001), and arterial oxygen partial pressure (r = 0.68, P = 0.001). Negative correlations were observed between Tw Pdi and dynamic hyperinflation (FRC: r = -0.65, P = 0.005) and arterial carbon dioxide pressure (r = -0.50, P = 0.03). Furthermore, stepwise regression analysis showed that Tw Pdi correlated with BMI z score (r = 0.75, P = 0.0002) and FEV(1) (r = 0.69, P = 0.001). CONCLUSIONS: Diaphragm strength is relatively well preserved in young patients with cystic fibrosis. However, the strength of the diaphragm decreases with the progression of malnutrition and airway obstruction.

Adolescent↗
PubMed2004

Quantitative lung perfusion scan as a predictor of aerosol distribution heterogeneity and disease severity in children with cystic fibrosis.

BACKGROUND: The assessment of lung ventilation by radionuclide imaging has proved useful for the optimization of aerosol therapy in children with cystic fibrosis. Further analysis of lung perfusion may provide additional information. METHODS: Quantitative analysis of regional lung aerosol distribution (Tc phytates) and perfusion (Tc macroaggregates) homogeneity was performed in 18 children with cystic fibrosis, using the third and fourth spatial moments (skew and Kurtosis) of count distribution. Patients were chosen from a prospective study whose goal was to compare the efficacy of two nebulization methods of a radiolabelled aerosol: one session involved a nebulizer activated by patient inspiratory flow (control session), whereas the other involved a nebulizer powered by a pressure support device (PS session). RESULTS: Quantitative regional distribution of perfusion was similar to aerosol distribution, although skew and Kurtosis were lower, indicating better homogeneity. Perfusion skew was inversely correlated with pulmonary volumes and Shwachman score, even more significantly than ventilation skew. Using receiver operating characteristic curve analysis, a perfusion skew threshold of 0.67 was predictive of disease severity (FEV1 > or =60% or FEV1 <60%) with 86% sensitivity and 91% specificity. Furthermore, same skew threshold allowed the identification of patients who were 'PS responders' (greater amount of radioactivity deposited after the PS session) or 'PS non-responders' with 80% sensitivity and 77% specificity. CONCLUSION: Quantification of regional lung perfusion is easy to perform and heterogeneity of the distribution is closely correlated to disease severity. Moreover, perfusion skew can identify patients who are likely to benefit from pressure support (to optimize aerosol therapy) and may be helpful for orienting potential non-responders towards alternative therapies.

Adolescent↗