[Oxygen therapy in children].
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Age Factors↗
Biomedical subjects
B Fauroux
Publications and source records attributed to B Fauroux.
Results of tympanoplasty in children with primary ciliary dyskinesia.
OBJECTIVE: To assess the results of tympanoplasty in children with primary ciliary dyskinesia complicated by tympanic perforation or cholesteatoma with hearing loss and/or recurrent otorrhea. DESIGN: Retrospective study. Postoperative follow-up of 26.3 months in the type 1 tympanoplasty group and 46 months in the child with cholesteatoma. SETTING: Hospitalized care, referral center. PATIENTS: Seven children with primary ciliary dyskinesia, complicated in 6 children by 9 tympanic perforations (3 bilateral perforations) and in 1 child by an attical cholesteatoma. RESULTS: After 9 type 1 tympanoplasties, the grafts were intact in 9 ears, with no recurrence of otorrhea, but serous otitis media was present in 6 of the 9 ears. Auditory improvement was significant, with an average gain of 17-dB hearing level in speech frequencies. After a canal wall-down tympanoplasty with mastoidectomy for attic cholesteatoma in 1 ear, the cavity that was operated on showed no signs of otorrhea or residual cholesteatoma after a follow-up of 46 months. CONCLUSION: In children with primary ciliary dyskinesia, tympanoplasty has a high probability of graft success and auditory improvement, despite the frequent recurrence of serous otitis media.
Adolescent↗
Prevalence of asthma or respiratory symptoms among children attending primary schools in Paris.
The objectives of this study were to determine the prevalence of chronic respiratory symptoms and asthma in 8- to 9-year-old children in Paris, and to analyze their medical management. This cross-sectional study was carried out in 1994 on a randomized sample of 3,756 pupils attending Paris public primary schools. The response rate by parents to an initial standardized self-administered questionnaire was 94.8%. This questionnaire identified 601 children (17%) as having recurrent respiratory symptoms. Of these children, 555 (92.3%) agreed to participate in a follow-up survey that evaluated their medical management; these children were examined by 73 school doctors of the city of Paris. Prevalence of parent-reported doctor-diagnosed asthma was 6.1%. In addition to these 211 children with asthma, 344 other children had recurrent respiratory symptoms: 120 children were "wheezers," and the remaining 224 children were "coughers." Among "chesty" pupils not identified as asthmatics, physical education teachers reported exercise-induced cough or respiratory discomfort in 13.7%, and nearly 14% had a peak expiratory flow 20% lower than the predicted values for age and height. In children identified as asthmatic, 25.3% were not under medical supervision, 55.5% had never performed lung function tests, 63.7% did not receive any prophylactic treatment, and 59.7% were receiving no treatment. Bronchodilator prophylactic medication before exercise was used by only 7% of asthmatics, although physical training teachers noted chest discomfort on exercise in 30.4% of these pupils. These results confirm that children with asthma and participating in this study were less than optimally investigated, were underdiagnosed and undertreated, and their medical management was not optimal. In addition to its epidemiologic value, the study has helped Paris school doctors to advise parents to refer their children to their general practitioner when asthma was suspected or undertreated.
Asthma↗
Chronic interstitial lung disease in children: response to high-dose intravenous methylprednisolone pulses.
The prognosis for children with chronic interstitial lung disease is poor and the mortality rate is high, especially in infants. This explains the many therapeutical protocols which have been proposed and investigated by several authors. In the present work, we evaluated the response of three infants with idiopathic pulmonary fibrosis to high-dose intravenous prednisolone pulses. The patients were referred to the department at the age of 4, 17, and 3 months, respectively. The diagnosis was confirmed by open lung biopsy and intravenous pulse methyl prednisolone therapy was started with the following protocol: 300 mg/m2 methylprednisolone daily for 3 days, repeated every 4 to 6 weeks. Because of the extreme severity of the respiratory distress at the time of diagnosis, the intravenous pulse treatments were initially complemented by oral prednisone. Clinical improvement was noticed within 6 months with progressive correction of hypoxemia. After follow-up for 3.5 to 4 years, with a total number of pulses of 37, 26, and 32, respectively, the children are symptom-free and do not require oxygen supplementation. During this period, no side effects and no adrenal insufficiency could be documented. Based on current knowledge of steroid action, it can be speculated that the response to intermittent high-dose intravenous methylprednisolone may explain the ability of this mode of hormone administration to maintain an adequate level of glucocorticoid receptor expression. More information and trials through multicenter collaborations are needed to assess therapeutical protocols of repeated high-dose intravenous steroid treatment.
Adolescent↗
[Good clinical practice in nebulization].
A meeting on nebulization held in April 1997 defined good clinical practices. Guidelines that were proposed pertained to the following: pneumatic and ultrasonic nebulizers; delivering circuit, occluded or not, the choice of the tip being done according to the disease to treat and to the drugs to be delivered; various functions, depending on the type of nebulizer; the particle size, as it will indicate which disease may be treated: between 2 and 6 microns for bronchi, between 0.5 et 3 microns for lung, > 5 microns for ear, nose and throat diseases; compatibility between the type of nebulizer and drugs. Ten drugs are currently registered in France. Nebulization has multiple clinical indications, such as asthma, cystic fibrosis, chronic obstructive pulmonary disease, Pneumocystis carinii pneumonia, acute laryngitis, and in infants, acute bronchiolitis. The prescription must be detailed, and the physician should make sure that the medical staff put it into application.
Administration, Inhalation↗
[Mucoviscidosis: therapeutic strategies are multiplying].
Since the cloning of the defective gene in cystic fibrosis, much has been learned on the function of CFTR and on the mechanisms regulating its expression. Based on the current understanding of the processes involved in lung disease progression, a number of approaches have been developed using gene therapy and pharmacological agents. Several of these agents have been reported to restitute a function to CFTR with specific mutations. Other molecules act on channels other than CFTR, and may be effective by bypassing CFTR itself. In the present review the various therapeutical strategies currently investigated are discussed.
Child↗
Nonchemical influence of inspiratory pressure support on inspiratory activity in humans.
To determine whether nonchemical inhibition of respiratory activity occurs during inspiratory pressure support (IPS) ventilation (IPSV), respiratory motor output (in 9 subjects), obtained by calculating transdiaphragmatic pressure-time products, and central respiratory output (in 5 subjects), obtained by integrating the electromyographic activity of the diaphragm (EMGdi) during mechanical inspiratory time, EMGdi per minute, and electrical inspiratory time, as determined from onset to peak EMGdi, were compared during spontaneous ventilation (control) and IPSV with (IPS+CO2) and without (IPS) correction of hypocapnia. Both IPS and IPS+CO2 induced significant decreases in transdiaphragmatic pressure-time products (46 +/- 31 and 53 +/- 23%, respectively), EMGdi during mechanical inspiratory time (49 +/- 12 and 57 +/- 14%, respectively), EMGdi per minute (65 +/- 22 and 69 +/- 15%, respectively), and electrical inspiratory time (73 +/- 8 and 65 +/- 6%, respectively). Because correction of hypocapnia failed to eliminate the marked inhibition of both respiratory and central motor output seen with IPS, we conclude that nonchemical inhibition of respiratory activity occurs during IPSV.
Adult↗
Expression of insulin-like growth factors and their binding proteins by bronchoalveolar cells from children with and without interstitial lung disease.
The involvement of the insulin-like growth factor (IGF) system in lung growth and repair following injury is sustained by a number of studies. Based on this knowledge, the aim of the present work was to document the expression of the IGFs and their binding proteins by alveolar cells obtained by bronchoalveolar lavage (BAL). Two groups were investigated: a control group of five children and a group of 11 children referred to the department for exploration of interstitial lung disease (ILD). Components of the IGF system studied included IGF-I, IGF-II and IGF-binding proteins (IGFBP). Expression of these factors was analysed at the level of messenger ribonucleic acid (mRNA) (by semi-quantitative reverse transcription polymerase chain reaction techniques), and of protein for the IGFBPs. In addition, expression of two major cytokines associated with the inflammatory process, tumour necrosis factor-alpha (TNF-alpha) and transforming growth factor-beta (TGF-beta), was also documented. In children without parenchymal disease, the growth factor expressed was IGF-I, in association with the presence of mRNA for IGFBP-2 in all cases. In children with ILD, expression of IGF-I was observed in nine patients and of IGF-II in three patients, and the presence of IGFBP-2 was found in all extracts analysed (mRNA and proteins). Evaluation of IGFBP-2 expression indicated an increase in the group of children with ILD. Interestingly, a significant association was observed between the increase in IGFBP-2 expression and TGF-beta expression. The present data emphasize the presence on insulin-like growth factor-binding protein-2 in the BAL of all patients, and suggest that this protein may be an important factor of the injury/repair processes during the progression of alveolar inflammation.
Adolescent↗
Short-term effect of winter air pollution on respiratory health of asthmatic children in Paris.
There is controversy as to whether low levels of air pollution affect the symptoms and lung function in asthma. We addressed this by examining the short-term effects of winter air pollution on childhood asthma in Paris. We performed a 6 month follow-up of 84 medically diagnosed asthmatic children classified into two groups of severity. The outcomes included incidence and prevalence of asthma attacks, symptoms and use of supplementary beta2-agonists, peak expiratory flow (PEF) value and its variability. The statistical analysis controlled the lack of independence between daily health outcomes, trends and meteorology. Air pollution was associated with an increase in reports and duration of asthma attacks and asthma-like symptoms in mild asthmatic children. The strongest association was the risk of asthma attack for an increase of 50 microg x m(-3) of sulphur dioxide (SO2) on the same day (odds ratio (OR)=2.86). Maximum reduction in morning peak expiratory flow (PEF) (5%) and maximum increase in PEF variability (2%) were observed at a lag of 3 days for an increase of 50 microg x m(-3) of SO2 in the subgroup of mild asthmatics receiving no regular inhaled medication. In moderate asthmatic children, the duration of supplementary beta2-agonist use was strongly associated with air pollution. The general pattern of our results provides evidence of the effect of the low levels of air pollution encountered in Western Europe on symptoms and lung function in childhood asthma.
Administration, Inhalation↗
[The sickle cell anemia lung from childhood to adulthood].
The pulmonary complications remain the prime cause of morbidity and mortality in sickle cell disease. The pathogenetic mechanisms consists both of an alteration of the rheological properties of the blood, the existence of a hypercoagulability state and above all specific interactions between the abnormal sickle cells and the vascular endothelium and a dysregulation of the vascular reactivity in which nitrous oxide intervenes. The acute chest syndrome (ACS) is characterised by chest pain with dyspnoea and recent radiological abnormalities and it is an acute lung complication whose problem is one of aetiology. The infectious pneumonias are rarely documented. On the other hand, alveolar hypoventilation linked to infarcts of the thoracic ribs, thoracoabdominal trauma, subdiaphragmatic pain, the administration of analgesics causing respiratory depression, obesity or sleep disturbance are frequent causes of ACS. Bronchoalveolar lavage has revealed a frequency of fat emboli following infarcts in the long bones. Pulmonary emboli is rarely a cause. Pulmonary thrombosis is a serious complication, the diagnosis is difficult and is seen in a predisposed clinical setting. The treatment of ACS rests on controlled hydration and antibiotic therapy, oxygen therapy and controlled analgesic therapy. The indications for blood transfusion and for exchange transfusion merits a better evaluation. In the long term patients with sickle cell disease present with a failure of normal thoracopulmonary growth with a restrictive ventilatory defect and progressive diminution in the transfer factor of carbon monoxide with age. A history of ACS favours chronic lung disease. Pulmonary arterial hypertension is less frequent.
Acute Disease↗
Idiopathic pulmonary fibrosis in infants.
Idiopathic pulmonary fibrosis is a poorly characterized disease in infants. In the present report, we reviewed our experience with 10 infants during a 10-year period. In 9 patients, onset of symptoms occurred before the age of 2 months and included tachypnea, cough, and inadequate weight gain. However, despite the presence of these symptoms, diagnosis was delayed for 3 months at which time the infants were referred to the pediatric pulmonary department, when the diagnosis was confirmed by open lung biopsy. At the time of admission, bronchoscopy with alveolar lavage was performed in 9 children and showed severe alveolitis with an increase in the neutrophil count. Nine infants were treated with prednisone alone or in combination with chloroquine, colchicine, or cyclophosphamide; all these patients died despite treatment. One infant was treated with pulses of methylprednisolone because of a failure in response to oral prednisone. This girl who displayed similar clinical, radiological, and histological abnormalities as the other children at the time of diagnosis is the only child still alive after 3 years of follow-up. She is now free of respiratory symptoms and has a normal growth curve. The present report raised two important points: (1) a thorough evaluation of characteristic symptoms should lead to an early diagnosis of pulmonary fibrosis in infants; and (2) administration of pulse therapy using corticosteroids has been helpful and needs to be evaluated further.
Biopsy↗
Mycobacterial lung disease in cystic fibrosis: a prospective study.
BACKGROUND: Patients with cystic fibrosis (CF) may be predisposed to airway infections with unusual organisms, such as mycobacteria. The aim of the study was to determine the incidence and clinical picture of mycobacterial infection in CF children. METHODS: At least 2 acid-fast bacillus (AFB) smears and mycobacterial cultures were performed on a prospective basis on 682 sputum specimens from 106 patients during a 1-year period. RESULTS: Thirty-three percent of the cultures were contaminated with other bacteria. Seven children had at least one sputum culture positive for one mycobacterium. Five children had only one positive AFB culture. Their clinical status and lung function remained stable during follow-up. Two teenagers with severe lung disease had several positive AFB smears and cultures for Mycobacterium chelonae and Mycobacterium abscessus. The isolation of M. chelonae and M. abscessus was associated with a clinical and functional decline. Clarithromycin treatment resulted in temporary improvement with the disappearance of the mycobacteria after 6 months of treatment. This prospective study shows an incidence of 2.3% for positive cultures. The prevalence was 6.6% for mycobacterial colonization but only 1.9% for mycobacterial lung disease in our pediatric population. CONCLUSIONS: We recommend performing AFB smears and cultures in CF children with severe lung disease and/or during a lung exacerbation. In these patients persistence of M. chelonae or M. abscessus in sputum should lead to consideration of treatment with clarithromycin.
Adolescent↗
Predictors of survival in patients receiving domiciliary oxygen therapy or mechanical ventilation. A 10-year analysis of ANTADIR Observatory.
STUDY OBJECTIVE: To analyze predictors of survival for patients receiving home long-term oxygen therapy (LTOT) or prolonged mechanical ventilation (PMV) according to the cause of chronic respiratory insufficiency (CRI) and the patients physiologic data. DESIGN: Analysis of a nationwide database (ANTADIR Observatory). SETTING: The national nonprofit network for home treatment of patients with CRI Association Nationale pour le Traitement a Domicile de lInsuffisance Respiratoire Chronique (ANTADIR); founded in France in the 1980s. PATIENTS: There were 26,140 patients receiving LTOT or PMV (noninvasive or via tracheostomy) between January 1, 1984 and January 1, 1993 (chronic bronchitis, 12,043; asthma, 1,755; bronchiectasis, 1,556; emphysema, 551; tuberculosis sequelae, 4,147; kyphoscoliosis, 1,574; neuromuscular diseases, 1,097; pneumoconiosis, 919; and fibrosis, 2,498. MEASUREMENTS AND RESULTS: Survival analysis was performed using the actuarial and the Cox's semiparametric model. The mean survival for patients with chronic bronchitis is 3 years. Survival is slightly better for patients with bronchiectasis and asthma and worse for those with emphysema. Patients with kyphoscoliosis and a neuromuscular disease have the longest survival (8 and 6.5 years, respectively). Patients with CRI due to tuberculosis sequelae experience the same survival as COPD patients (3 years). Prognosis is the worst in patients with pneumoconiosis or fibrosis: 50% of these patients die during the year following the beginning of home treatment. The association of an obstructive lung disease worsens the prognosis of patients with kyphoscoliosis or neuromuscular disease and tends to bring the survival rate of the patients with pneumoconiosis or fibrosis closer to that of COPD patients. In COPD, male sex, older age, lower body mass index (BMI),FEV1 percent predicted,PaO2,and PaCO2 are independent negative prognostic factors. For tuberculous sequelae and kyphoscoliosis, female sex, younger age, a high BMI, PaO2 and PaCO2 (and for kyphoscoliosis a higher FEV1/vital capacity [VC] ratio) are all independent favorable prognostic factors. In pulmonary fibrosis, a lower PaO2 and PaCO2 values, a lower VC percent predicted, and a higher FEV1/VC ratio are negative prognostic factors. CONCLUSIONS: The ANTADIR Observatory allows a unique opportunity to analyze long-term survival of a large population with CRI treated at home.
Aged↗
[Pulmonary toxicity of drugs and thoracic irradiation in children].
The pathology of drug-induced pulmonary toxicity in children is poorly understood and probably under-estimated, in the absence of any prospective studies evaluating in a systematic fashion the side effect of medication on the respiratory apparatus. The pulmonary toxicity of thoracic irradiation has markedly receded with more restricted indications for this sort of treatment. Three clinical patterns are most commonly encountered in drug induced lung disease in children: interstitial lung disease, hypersensitivity lung disease and non-cardiogenic pulmonary oedema. The diagnosis is a diagnosis of exclusion and rests on a group of clinical arguments and also on the progress of the disease. Broncho-alveolar lavage rules out infectious disease. Respiratory function tests show non-specific anomalies. A lung biopsy may be indicated. The mechanism of the pulmonary toxicity are associated with disequilibrium of the oxidant/antioxidant and protease/antiprotease system as well as disturbance of the immune response or alteration of the pulmonary matrix by disease of the collagen system. Increased toxicity may be seen in children because of a very significant cumulative dose. The cytotoxic drugs which are most often implicated in causing this are bleomycin, methotrexate, cyclophosphamide and busulfan. Other drugs which are responsible for toxic lung disease are nitrofurantoin, sulfasalazine, D-penicillamine, betalactams, Diphenyl-hydantoin and carbamazepine. Acute post-radiation lung disease is rare. Post-radiation fibrosis is found six months after irradiation and hinders thoraco-pulmonary growth in the child. It is important to assess lung function in all children before any chemotherapy or thoracic irradiation. Cytotoxic drugs are the most common cause of toxic lung disease. This iatrogenic disease requires a multi-discipline approach to ensure the quality of care for these children.
Anti-Bacterial Agents↗
Azathioprine-induced pulmonary haemorrhage in a child after renal transplantation.
We report a case of azathioprine-induced haemorrhagic alveolitis, in a 14-year-old boy, after renal transplantation. On day 25 the patient developed haemoptysis, fever and hypoxaemia. Chest X-ray showed diffuse reticulo-nodular shadows in both lung fields. Bronchoalveolar lavage samples were haemorrhagic and demonstrated a relative neutrophilia and a mild lymphocytosis, with a normal CD4/CD8 ratio. Azathioprine was discontinued on day 26. The patient required mechanical ventilation for 4 days. A positive leucocyte migration inhibition test and the recurrence of the symptoms after a second short course of azathioprine therapy suggested a cell-mediated mechanism. This patient is, to our knowledge, the first child to suffer from azathioprine-induced pulmonary haemorrhage.
Acute Disease↗
Home treatment for chronic respiratory failure in children: a prospective study.
Home treatment for children with chronic respiratory failure (CRF) is increasing. However, the causes of CRF in children and the details of their home treatment are not well-known. The aim of this study was to describe the causes of CRF in the paediatric population and the treatments that the patients received at home. We surveyed all children (aged < or = 18 yrs) entering the Association Nationale pour le Traitement à Domicile de l'Insuffisance Respiratoire chronique (ANTADIR) for home treatment of CRF between March 1992 and March 1993. Two hundred and eighty seven children (178 boys, 62%) started home treatment for CRF during the year. One hundred and eleven patients had obstructive respiratory disease: cystic fibrosis (CF) (n = 24); bronchopulmonary dysplasia (BPD) (n = 79); other obstructive respiratory disease (n = 8). One hundred and seventy six patients had restrictive lung disease: neuromuscular disease (n = 87); kyphoscoliosis (n = 21); pulmonary fibrosis (n = 6); cardiac disease (n = 14); stomatological disease (n = 10); other restrictive respiratory disease (n = 9); and 29 miscellaneous causes. One hundred and thirteen patients received oxygen therapy, with a mean daily use of 17.7 h (20 h.day-1 for BPD patients and 12.3 h.day-1 for CF patients). Oxygen was delivered by a concentrator in 88% of cases. One hundred and fifty eight children received mechanical ventilation (MV). Five children received nasal continuous positive airway pressure ventilation for sleep apnoea, four had pneumatic belt ventilation, and 12 had a tracheostomy without MV. Treatment was stopped in 21 children, because of death in nine and improvement in the other 12. Home treatment for children with CRF is well developed in France via the ANTADIR network. Causes of CRF in children are heterogeneous, with a relatively good prognosis.
Adolescent↗
[Noninvasive ventilation using nasal mask in mucoviscidosis].
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Adult↗
Cytotoxic drug-induced pulmonary disease in infants and children.
The increased survival rate of malignant diseases due to more aggressive treatments contributes to the occurrence of drug-induced pulmonary diseases (DIPD). We reviewed, retrospectively over a 10-year period, 15 children (8 girls) who presented a DIPD. Their mean age was 9 years (range, 1 to 17 years), with an underlying malignant disease in 14 (9 leukemias). Three typical patterns have emerged from this analysis: (1) acute hypersensitivity lung disease caused by methotrexate (in 6 patients) or azathioprine (in 1 patient). This acute syndrome consisted of alveolar-interstitial infiltrate with a hypercellularity on bronchoalveolar lavage (BAL) (mean, 714,286 cells/mL; range, 180,000-2,940,000 cells/mL) and an increase of lymphocyte counts (mean, 39%; range 11-64%) with predominantly CD8-suppressor/cytotoxic lymphocytes. Inhibition of leukocyte migration or leukocyte aggregation in the presence of low drug concentrations was positive in the 5 cases tested. Lung function tests showed a restrictive pattern and the outcome of DIPD was always favorable. (2) Chronic pneumonitis/fibrosis was seen in 6 patients who received a variable association of cyclophosphamide (3 patients), bleomycin (2 patients), BCNU (2 patients), and melphalan (1 patient). Symptoms of an alveolar-interstitial pneumonitis developed progressively. BAL showed a moderate increase of total cell numbers (mean, 495,000 cells/mL; range, 150,000-900,000 cells/mL). Lung function tests showed a restrictive pattern. Despite corticosteroid treatment in 4 children, one died after bleomycin lung injury and 2 had functional lung impairment. (3) Noncardiogenic pulmonary edema occurred in 2 patients with leukemia treated with recombinant interleukin II. BAL showed hypercellularity and outcome was rapidly favorable.(ABSTRACT TRUNCATED AT 250 WORDS)
Acute Disease↗