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.

Renal granulomatous sarcoidosis in childhood: a report of 11 cases and a review of the literature.

UNLABELLED: We analysed retrospectively 11 children with renal granulomatous sarcoidosis confirmed by renal histology in order to describe the course and prognosis of the disease. Symptomatic sarcoidosis was diagnosed at a mean age of 10.1 years. Nine children had renal involvement at the time of diagnosis. In the course of the disease, nine patients developed renal failure and mild proteinuria, seven had transient sterile leukocyturia, four showed microscopic haematuria, seven had a urinary concentrating defect, and enlarged kidneys were seen in three patients. One child had hypercalcaemia and hypercalciuria, none had hypertension. Light microscopy of the kidney showed interstitial infiltration by mononuclear cells in all children, interstitial fibrosis in nine patients, epithelioid granulomas in seven, tubular involvement in eight, and mild glomerular involvement in seven patients. Renal immunofluorescence was negative. Ten children received prednisone for 1-11 years. After a mean follow up of 5.5 years, three patients had entered end-stage renal failure and one had chronic insufficiency after interruption of medical supervision and prednisone therapy. CONCLUSION: Renal failure, proteinuria, leukocyturia, haematuria, and concentration defect are the prominent features of renal granulomatous sarcoidosis in children. Steroid therapy, adjusted according to disease activity, may prevent end-stage renal failure.

[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.

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.

[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.

[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)

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.

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.

[Mechanical ventilation by nasal mask in children with cystic fibrosis. Initial results of a non-invasive method]. / Ventilation mécanique par masque nasal en pression positive intermittente chez l'enfant atteint de mucoviscidose. Résultats préliminaires d'une méthode non invasive.

BACKGROUND: Chronic respiratory failure (CRF) with hypoxia and hypercapnia is the last ineluctable phase in cystic fibrosis (CF). Nasal positive pressure ventilation (NPPV), a non-invasive method, may be given to CF children with CRF, especially to patients accepted for transplantation (T). This method improves ventilatory function by resting the chronically exhausted respiratory muscles, facilitates bronchial drainage by physiotherapy, prevents the exacerbations of the illness and prepares patients for T. METHODS AND PATIENTS: NPPV was used in 6 CF patients (mean age 13 years 6 months). One of them was transplanted 15 days later, two of them were accepted for T. All had hypoxia. Five of them had hypercapnia. RESULTS: NPPV was given to four patients for 3 to 14 months. The preliminary results were positive. One patient gained weight, two had more fluid sputum. One patient showed an increase in functional respiratory tests (FRT: PaO2, vital capacity, FEV-1) while these tests were stabilized in the others. CONCLUSIONS: NPPV in an effective non-invasive method for use with CF children. It is indicated for CF patients accepted for T and also earlier, for CF patients with CRF in order to prevent acute exacerbations and functional respiratory deterioration.

[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.

[Mediastino-pulmonary sarcoidosis in children. Clinical study, analysis of data of bronchoalveolar fluid lavage and respiratory function tests, therapeutic trends]. / Sarcoïdose médiastino-pulmonaire de l'enfant. Etude clinique, analyse des données du lavage broncho-alvéolaire et des épreuves fonctionnelles respiratoires, orientations thérapeutiques.

A series of 27 children (mean age: 12 yrs, 5 mos.) presenting with thoracic sarcoidosis is reported. This series, collected from 1961 to 1988 shows the rarity of the disease at that age. However the low rate of asymptomatic forms (22%) suggests that the frequency of the disease is underestimated, as it is not diagnosed. The histological proof is necessary for the diagnosis. When peripheral lesions available for biopsy are lacking, a liver needle biopsy is helpful (93% of positivity). This study shows the frequency of multivisceral types, the intensity of the macrophagic and lymphocytic alveolitis. The therapeutic indications depend on the comparison of the radiological stage, the results of pulmonary function tests (PFT), those of the bronchoalveolar lavages (BAL) and of the serum granulomatous activity markers, especially concerning angiotensin converting enzyme (ACE). When present at the beginning of evolution, several risk factors lead to use a corticosteroid treatment: age of onset before 4 years, multivisceral involvement, presence of functional pulmonary signs, delayed diagnosis and onset of treatment, impaired respiratory function (especially concerning the alveolo-capillary diffusion), PMN cells greater than or equal to 2% in the initial BAL, and IgG proteins greater than 4 SD. Thus sarcoidosis in children differs from that seen in adults as it has a more marked evolutive tendency and leaves severe sequelae in one third of patients.

[Pleuropulmonary colibacillus infection in a 9-year-old boy leading to the diagnosis of Bruton agammaglobulinemia]. / Infection pleuro-pulmonaire à colibacille chez un garçon de neuf ans, conduisant au diagnostic d'agammaglobulinémie de Bruton.

A nine-year-old boy was hospitalized for pneumonia of the left lower lobe. A left pleural effusion developed 48 hours later. The same E. coli strain was recovered from five blood cultures, pleural fluid, and middle ear fluid. Recovery was achieved after two months. Pleural lavage was performed twice daily for the first 15 days and parenteral antimicrobial therapy was given for 45 days. Because E. coli is not usually responsible for ENT or lower respiratory tract infections, an immune deficiency was sought for. The index patient had a twin in whom a history of osteomyelitis at the age of 2 and pneumonia at the age of 8 was found. Recurrent otitis had been a problem in both twins from the age of 14 months. In both twins, immunoglobulin assays led to the diagnosis of Bruton agammaglobulinemia. These two children are now receiving intravenous infusions of human immunoglobulins every three weeks. Bruton agammaglobulinemia is infrequently diagnosed at so late an age.

[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.