Biomedical subjects
Publications and source records attributed to G Tournier.
Gorham's disease usually manifests as diffuse osteolysis but may be complicated with pleural effusion. We describe the case of a 12 year-old boy who had repeated pleural effusions. Radiographs show a mediastinal widening and an interstitial syndrome related to hemolymphangiomatous involvement.
Positive diagnosis of acute bronchopneumopathy in the child is relatively easy, but defining its aetiology is another matter. Criteria to distinguish between pneumopathy of viral or of bacterial origin should be known but are often on the wrong track. Some clinical and radiological pictures, associated with epidemiological data, can also orient toward an infectious agent and should be taken into account. When clinical severity or the background requires precise diagnosis in order to better adapt the treatment, a choice will need to be made among the numerous techniques now available to detect the responsible infectious agent.
Burkholderia cepacia, Stenotrophomonas maltophilia, and Alcaligenes xylosoxidans have been isolated with increasing frequency from the sputum of patients with cystic fibrosis in a pediatric hospital. In 1994-95, 27 of 120 patients were persistently colonized, 17 with Burkholderia cepacia, eight with Alcaligenes xylosoxidans, and five with Stenotrophomonas maltophilia. Genotyping of 220 clinical isolates revealed that most of the Burkholderia cepacia strains were clonally related, suggesting either cross-infection or a common source of exposure. In contrast, neither cross-infection nor a common source of exposure appear to have occurred in the cases of Alcaligenes xylosoxidans or Stenotrophomonas maltophilia.
BACKGROUND: Mucoepidermoïd carcinomas (MEC) are very rare (less than 20 cases reported in the literature) and potentially malignant bronchial gland carcinomas. PATIENTS: Three children, two boys (11 and 7 years old) and one girl (5 years old) suffered from respiratory symptoms such as cough, recurrent pneumonia and/or hemoptysis for 2 to 12 months. Bronchial endoscopy showed a mass into the left (two cases), or the right main bronchus (one case). Chest CT scan identified local extension, and lung-associated lesions. Histopathological study concluded to MEC in the three cases. The patients were treated by segmental bronchial resection, completed with left upper lobectomy (two cases), bronchotomy (one case). All the tumor could be removed; there was no metastasis. The outcome was uneventful with a 8 to 24 months follow-up. CONCLUSION: Bronchial tumors of children must be considered in patients with chronic cough, recurrent pneumonia and/or hemoptysis and require bronchial endoscopy for their diagnosis.
Since 1987, Pseudomonas cepacia has been isolated with an increasing frequency in the expectorants of children with cystic fibrosis followed at the Hôpital d'enfants Armand Trousseau (Paris, France). Colonization by P. cepacia may be responsible for serious secondary infections and rapid deterioration in respiratory function in these patients. Among the 130 children attending our centre, 14 (8 girls and 6 boys) aged 3 to 18, exhibited chronic colonization. 132 isolates, originating from sputum obtained between 1992 and 1994 were studied. Nine biochemical patterns and 6 antibiotic susceptibility patterns at least were defined, therefore exhibiting great polymorphism. Chromosome restriction patterns with Xba I after pulsed field gel electrophoresis enabled 4 pulsotypes to be identified: A, B, C and D. Thirteen patients harboured pulsotypes A, C and D, and 1 patient pulsotype B, the last being quite distinct from the first three. Pulsotypes A, C and D were almost similar, suggesting that closely related strains, probably the same strain, was harboured by 13 of the 14 patients. The origin could be contamination from a single source, or stem from patient-to-patient crossed transmission.
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.
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.
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.
Explore the source record for details and available documents.
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)
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.
To evaluate the contribution of energy expenditure to the energy imbalance seen in cystic fibrosis patients, resting energy expenditure was measured using open-circuit indirect calorimetry in eight infants with cystic fibrosis, aged 2-7 months (mean, 4), without overt lung disease and in 10 healthy age-matched controls. In both groups, we found close, significant, linear correlations between resting energy expenditure and body weight and between resting energy expenditure and fat-free mass as measured by anthropometry. Cystic fibrosis patients had a 26% increase in resting energy expenditure per kilogram of fat-free mass as compared with controls and a 32% increase in resting energy expenditure as compared with predicted values for fat-free mass. These data from young infants free of clinical symptoms suggest a constitutional metabolic disorder in cystic fibrosis and support the need for early nutritional therapy in cystic fibrosis patients.
In developed countries like France, the incidence of tuberculosis is low, 5 to 6% in patients under 15 years of age. At the present time, tuberculosis has been irradicated in 11 French regions. Inversely, tuberculosis is still found in the Paris area. Half of the children with tuberculosis are under 5 years of age, underscoring the high risk at this age. Lung and lymph node infection is most often encountered in children with typical complications of endobronchial fistulization and segmentary or lobular atelectasy. Direct examination does not reveal germs in more than 80% of the cases. Other forms are more rarely found in children although congenital, meningeal, serofibrinous pleural, and cervical lymph node tuberculosis are sometimes observed. Latent primary infection, by far the most frequent, can only be recognized by the intradferal tuberculin test. Systematic treatment modalities would now center on the use of combined chemotherapy using two bacteriocidal antibiotics for 2 or 3 years.
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.
Among all organ transplantations, those of heart or heart-lung carry the greatest risk of toxoplasmosis. The disease is observed mainly when the donor is seropositive and the recipient seronegative. In these mismatched couples the risk may be as high as 57 percent. Cardiac tissue transplants are responsible for most contaminations. A subclinical serological reactivation can be observed in seropositive recipients. Patent forms are associated with seroconversion in seronegative subjects. Toxoplasmosis is often severe with multivisceral foci; interstitial pneumonia is possible. The serological diagnosis is easy in cases with significant antibody movements, but it may be difficult if the titre is low or stable. The parasitological diagnosis rests on the isolation of toxoplasma in blood, cerebrospinal fluid, bronchoalveolar lavage fluid and cardiac or cerebral biopsy. Immune defence against toxoplasmosis is primarily cellular, with lymphocytosis and inversion of the CD4/CD8 ratio. Macrophages play a crucial role. Interferon-gamma is the major mediator of cellular resistance. In spite of its immunosuppressive action, cyclosporin clearly has an antiparasitic action in vitro and in vivo. A cytomegalovirus infection might facilitate toxoplasma reactivation. Prevention of toxoplasmosis in transplant recipients includes systematic serology of the recipient and, if possible, the donor, detection of mismatched couples and systematic treatment with pyrimethamine of recipients at risk (in seronegative recipients, this drug has reduced the risk from 57 to 14 percent). Cyclosporin should be used as immunosuppressant in preference to other drugs of this kind. Corticosteroids administered in rejections increase the risk of toxoplasmosis.
Explore the source record for details and available documents.
A 14-year-old girl with high fever, dyspnea and bilateral miliary nodules on chest X-ray, developed a rapidly progressive respiratory failure associated with histiocytic hemophagocytosis. Histologic examination of bone marrow biopsy revealed tuberculous granulomas with caseating necrosis. We report a pediatric case in which treatment with extracorporeal lung support and epipodophyllotoxin VP 16-213 was successful.
Peak-flow measurement increasingly seems to be very useful for routine monitoring of asthma in children. The purpose of this study was to evaluate the reliability and reproductibility of three different peak-flow meters: Miniwright, Vitalograph and Assess. This prospective study was conducted in 100 primary school children aged from 6 to 11 years. In each child, 9 measurements of expiratory peak-flow rate (EPF) were performed with these 3 peak flow meters in a random order. Reproductibility was assessed for each instrument by the greatest divergence observed between the 3 EPF values measured in the same session. Reliability was assessed by comparison between the values obtained with each instrument and theoretical EPF values calculated from a formula which included age, sex and size. Our study validated the reliability and reproductibility of the 3 peak flow meters studied. EPF values were more scattered and less reproductible with Assess, but the difference was not significant.