BAL in children: a controlled study of differential cytology and cytokine expression profiles by alveolar cells in pediatric sarcoidosis.

STUDY OBJECTIVE: The development of BAL in children for both research and clinical purposes has been limited so far by the difficulty in establishing reference values. The aim of the study was (1) to define composition of BAL cellular components in control children and to evaluate the ability of these cells to express various cytokines, and (2) to study modifications of differential cytology and BAL cell cytokine responses in children with interstitial lung disorders. POPULATIONS AND METHODS: Two groups were investigated: a control group of 16 children who were concluded to be free of parenchymal lung disease after complete pulmonary investigation, and a group of 11 children with pulmonary sarcoidosis. Differential cytology was evaluated by standard techniques. BAL cell cytokine expression was studied at the level of messenger RNA (mRNA) by reverse transcription-polymerase chain reaction (RT-PCR) methods. RESULTS: In the control group, differential cell counts appeared to be similar to values reported in adult populations with normal distribution of the data and no influence of age. In this group, no transcripts for interleukin-1beta (IL-1beta), tumor necrosis factor-alpha (TNF-alpha), IL-6, and transforming (correction of tranforming) growth factor-beta (TGF-beta) could be detected. In children with sarcoidosis, different profiles of IL-1beta, TNF-alpha, IL-6, and TGF-beta expression were individualized which seemed to be related to the activity and/or severity of the disease, IL-6 and TGF-beta mRNA being observed only in the more severe forms. CONCLUSION: These data provide information on BAL cell number and function in children. Characterization of BAL cytokine expression patterns during the course of interstitial lung diseases in children may be of great interest for evaluation of disease activity and/or severity and therefore for planning of therapy.

Expandable prosthesis in right postpneumonectomy syndrome in childhood and adolescence.

Right pneumonectomy can lead to severe respiratory impairment due to stenosis of the left main bronchus. This syndrome is usually treated by inserting a fixed-volume prosthesis but, in children, expandable prostheses have the advantage of being adaptable to growth and permit progressive recentering of the mediastinum. We report 3 such cases, with the results of pulmonary function tests. The patients were aged 11, 17, and 22 years at the time of implantation and had undergone pneumonectomy during childhood for either bronchiectasis or complete pulmonary sequestration. All 3 patients are doing well, with a follow-up of 1 to 3 1/2 years. Pulmonary function tests have shown a substantial improvement in the obstructive syndrome in 2 patients whereas, in the third patient, in whom the contralateral lung was not perfectly healthy, the functional improvement was only moderate.

Pulmonary sarcoidosis in children: serial evaluation of bronchoalveolar lavage cells during corticosteroid treatment.

The clinical course of sarcoidosis in children has not been well defined. Eight children with symptomatic sarcoidosis included in this study underwent pulmonary function tests and bronchoscopy with bronchoalveolar lavage (BAL) before treatment and during steroid therapy. At the start of therapy, functional parameters, mostly dynamic lung compliance and lung transfer factor for CO, were impaired. This was associated with abnormalities of BAL cell populations: increased total cell number with a high proportion of lymphocytes, modifications of lymphocyte subpopulation with an elevated CD4+/CD8+ ratio, and enhanced ability of alveolar macrophages to release hydrogen peroxide. Although respiratory abnormalities seemed to be similar at the initial stage of sarcoidosis in children and adults, the course of the disease appeared to be different. Despite the absence of respiratory symptoms and disappearance of chest radiographic abnormalities on prolonged steroid treatment, we found slow improvement of pulmonary functions associated with persistence of BAL lymphocytosis and elevated CD4+/CD8+ ratios. However, the ability of alveolar macrophages to release hydrogen peroxide was significantly reduced after 6 months of steroid treatment, and it remained identical to the control group. Therefore, the evaluation of disease activity appears to be critical for therapy in pediatrics, and for this purpose studies of alveolar macrophage activation may be of particular interest.

Sarcoidosis of the upper respiratory tract in children.

Sarcoidosis is a multisystemic granulomatosis of unknown etiology which mainly affects young adults. It is characterized primarily by bilateral hilar adenopathies, a pulmonary infiltrate and cutaneous and ocular lesions. It rarely occurs in children under the age of 16. Localization in the upper respiratory tract (URT) is infrequent and sarcoidosis of the URT in children is exceptional, with only 13 cases reported in the literature. In the present report we describe the clinical, diagnostic explorations, histological and therapeutic aspects of 2 new cases in children.

[Role of Bronchodual in the long-term treatment of asthma in children]. / Place du Bronchodual dans le traitement de fond de l'asthme de l'enfant.

Bronchodual is a combination of two bronchodilators, a beta-2 adrenergic substance (fenoterol, 50 micrograms per dose), and an anticholinergic substance (ipratropium bromide, 20 micrograms per dose), administered by metered aerosol. According to different studies carried out with adults and children, the bronchodilating action of this combination is greatly superior to that obtained with each substance individually; the beta-2 adrenergic substance can be spared; its action is longer and involves a decrease in the number of drug intake and lastly its tolerance is excellent. A French multicentre study was performed with 74 children (7 to 15 years old, mean: 11.8 +/- 2.6 years), 53 boys and 21 girls with allergic or non-allergic asthma: 41 children presented a moderate asthma (one crisis per month) and 33 children moderately severe asthma (one crisis per week). These children had been given no corticosteroid therapy (per os or inhaled) for at least 15 days, their usual long-term treatment (cromoglycate, anti H1, theophylline LP, antibiotics) was given as normal. Their FVC was > or = 80% of predicted values and they presented an intercritical airway obstruction. Hypoxemia was noted in 41.2% of the children. A reversibility test was performed with Bronchodual: 68 of 74 children (i.e. 91.9%) were responders, ie after two doses of Bronchodual at least one of the FEFs had increased by at least 15% when compared with the initial values. The responders were given a 2 month treatment with two inhalations 3 times/day with a spacer. Fifty-seven patients were considered for the final evaluation.(ABSTRACT TRUNCATED AT 250 WORDS)

[Treatment of childhood asthma]. / Traitement de l'asthme infantile.

The treatment of childhood asthma should be considered with respect to age, severity and aetiology. Treatment should be instituted early from the first crisis in order to avoid progression to a more severe form. It consists of two aspects: the treatment of the acute episode and the chronic treatment. The treatment of the acute episode consists of using bronchodilators (BD) (rapid release Theophylline and/or beta agonists) to which one might add corticosteroids if the crisis lasts for more than a few hours or seems severe at the outset, an antibiotic should also be used as infection is often a trigger factor in infants. Maintenance treatment is necessary in asthmatics with frequent exacerbations. It should be tailored to the symptomatology and aetiology. The symptomatic treatment consists of a bronchodilator (slow release Theophylline or an atropine-like pharmacological derivative) to which one may add, in severe cases, corticosteroids which may be in the form of aerosol, or as rarely as possible by mouth. The second aspect of treatment relating to the aetiology is the most difficult to apply as childhood asthma is often multi-factorial: in allergic asthma the avoidance of allergens, disodium cromoglycate, ketotifen, and if necessary specific desensitization. In non-allergic asthma, physiotherapy, treatment of infectious foci, particularly ENT (ORL), and attention to psychosomatic features. When asthma is diagnosed and treated early the prognosis is transformed and progress towards chronicity is avoided.

[Bronchogenic cysts in the carina]. / Kystes bronchogéniques de la carène.

Between 1977 and 1990, 11 children with carinal bronchogenic cysts were operated in our institution: 8 girls and 3 boys, ranging in age from 1 month to 5 years. All were symptomatic (acute respiratory distress and recurrent bronchiolitis). Chest X-ray showed an unilateral over distension in 10/11 cases. Barium oesophagogram showed a compression in 6/10 cases. Bronchoscopy noticed an extrinsic compression in 10/11 cases and a tracheal and/or bronchial diskinesia in 5/11 cases. The computed tomography showed a low density mass in 4/4 cases. 9 cysts were left-sided and 2 right-sided. Both children underwent a second surgery for a second cyst. 2 pneumonectomies for complete parenchyma destruction were realised. 1 left pulmonary hypoplasia was noticed. A tracheal and/or bronchial diskinesia in post-operative was noticed in 5/6 cases. The clinical and functional respiratory following was good in 10/11 cases. An early surgery treatment is necessary before definitive sequelae.

[Bronchopulmonary aspergillosis and mucoviscidosis]. / Aspergillose bronchopulmonaire et mucoviscidose.

Patients with cystic fibrosis often suffer from aspergillosis infection (basically due to Aspergillus fumigatus) which frequently colonizes their respiratory tract, but its role in the respiratory insufficiency are poorly understood. Several clinical situations occur. Allergic bronchopulmonary aspergillosis rarely occurs in a typical form, and is usually difficult to diagnose from the atypical manifestations. Finally indications and treatment modalities are still subject to debate.

[New pharmacological approaches: rhDNase]. / Nouvelles approches pharmacologiques: la rhDNase.

rhDNase (Pulmozyme) is a new agent in the therapeutic strategy for patients with cystic fibrosis. It is one of the first specific treatments aimed at the respiratory tract. It affects the extracellular DNA which is present in abundant quantities in the bronchial secretions of these patients. rhDNase significantly reduces the incidence of infections and improves respiratory function. It should be used as a major treatment in combination with all other treatments in patients over 5 years of age with a vital capacity of at least 40% the theoretical value. It is important to schedule the respiratory exercises as a function of rhDNase intake. The long-term therapeutic benefit remains to be evaluated.

[Bronchopulmonary infections caused by Mycoplasma pneumoniae in children]. / Infections bronchopulmonaires à Mycoplasma pneumoniae de l'enfant.

Mycoplasma pneumoniae, a gram-negative bacteria, is an important cause of lower respiratory tract infection in children (20% of cases). The infection tends to be endemic and is punctuated by epidemic episodes every 4 to 7 years. Its frequency seems to be higher in children between 5 and 9 years of age, but is probably underestimated before 5 years. M.pneumoniae may cause multisystem infection. Diagnosis is established upon clinical data and laboratory findings. Usually, the infection is associated with leucocyte count under 15,000/mL and C-reactive protein under 50 m/L. Detection of M. pneumoniae DNA in clinical samples appears to have advantages over serological tests. Severe infections have been described in patients with humoral and cellular immunodeficiencies, sickle cell disease, cystic fibrosis. Treatment with macrolids and tetracyclines (after 8 years of age) is indicated. Respiratory functional sequelae are possible.

[Lipoma of the posterior mediastinum. 2 cases in children]. / Lipome du médiastin postérieur. Deux cas chez l'enfant.

The authors report the cases of 2 children, 12 and 4 years old respectively, presenting with a lipoma of the postero-superior mediastinum, that were investigated within a 6 year-period. Costal erosions were present in both cases with intraspinal extension in one child. Standard X-ray films should have led to the diagnosis, because of the relative low density of the mass considering its volume. In one case, CT scan confirmed the homogeneous fatty nature of the mass.

[Recurrent respiratory disease and IgA deficiency in infants and children (author's transl)]. / Broncho-pneumopathies récidivantes et déficit en IgA chez l'enfant.

Among nearly 1 300 infants and children studied for chronic or recurrent respiratory diseases 36 of them had plasma and/or salivary IgA deficiency. They were 1 to 13 years of age with 20 between 3 to 6 years; 11 had total, lack or traces of salivary IgA, 9 had very low or nul plasma IgA titer and 6 had both. The deficiency was transitory in 10. Associated abnormalities with potential liability for the disease: allergy, gastroesophageal reflux, alpha-1-antitrypsin deficiency and other immunologic deficiency were encountered in 26. The often multifactorial pattern of respiratory diseases in pediatrics is emphasized. The pathogenic role of IgA deficiency is discussed. Even though its responsibility is demonstrated, its real magnitude and extent requires further studies.

[Hypersensitivity pneumonitis in children. A study of 5 cases (author's transl)]. / Pneumopathies par hypersensibilité chez l'enfant. A propos de cinq cas.

Five cases of hypersensitivity pneumonitis in children are reported. Four had the pattern of bird breeder's disease and one of aspergillus lung hypersensitivity. All were clearly defined by clinical, radiological, functional and immunologic features. The possible difficulties in diagnosis are emphasized. The disease might be frequently overlooked and its frequency underestimated in children. The diagnostic value of precipitating antibodies is discussed. Although some immunologic studies suggest immune complex mechanisms, recent experimental and clinical data are in favor of a role for cell-mediated immunity. In addition whether or not immunological damage is to occur and result into disease depends not merely upon the characteristics of the antigen exposure. Genetic and/or acquired individual differences in immune reactivity can play a definite role as well. The risk of secondary pulmonary fibrosis and chronic respiratory failure in untreated patients is emphasized. Immunologic and functional tests for identification of the provoking antigen and ceasing of contamination exposure are urgent : they are also useful for monitoring steroid therapy.