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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 andTL,COwere apparent. After 18 months of steroids no benefit on pulmonary function tests could be noticed, with mainly persistence of alterations ofCL,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.

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.

The colonization over time of cystic fibrosis patients by Aspergillus fumigatus was investigated using a DNA fingerprinting method. Aspergillus fumigatus isolates collected sequentially for more than one year from six patients with cystic fibrosis were typed by Southern blot hybridization with a repetitive DNA sequence. Each cystic fibrosis patient harbored several strains of Aspergillus fumigatus that were isolated recurrently over time. Isolates collected from a cystic fibrosis patient with aspergilloma displayed the same genotype, suggesting that the infection was due to a single strain. Continuous isolation of the same genotype in another cystic fibrosis patient, however, was not correlated clinically with an Aspergillus infection.

The dominant role of inflammation in airways disease progression in cystic fibrosis (CF) is now well established and, based on recent findings, the possibility of an inappropriate inflammatory response in the lung of patients with CF has emerged. In order to characterize this response, the aim of the present work was to evaluate the levels of a number of pro- and anti-inflammatory cytokines in the sputum of CF children and to compare these levels to those observed in the sputum from non-CF children with diffuse bronchiectasis (DB). Three groups of patients were investigated: a group of 25 CF children (mean age: 12.2 yrs), a group of 10 non-CF children with DB (mean age 11.5 yrs), and a group of five healthy young adults (mean age 24 yrs). Elevated concentrations of pro-inflammatory cytokines, tumour necrosis factor (TNF)-alpha, interleukin (IL)-1beta and IL-8 were found in children with CF and in non-CF children with DB, with significantly higher concentrations of IL-1beta in CF children. Analysis of the natural anti-inflammatory cytokine IL-1 receptor antagonist (IL-1ra) and type II TNF soluble receptor (sTNFRII) concentrations showed distinct patterns, with elevated levels of both inhibitors in CF patients, whereas only sTNFRII was found to be increased in non-CF children with DB. IL-10 data indicated low concentrations in the CF group. In all CF children, the concentrations of IL-6 in the airways were extremely low, independent of the clinical, bacteriological or functional status. By contrast, significantly increased IL-6 levels were found in non-CF children with DB. These results document distinct cytokine profiles in cystic fibrosis patients and noncystic fibrosis patients. They also suggest that impairment of interleukin-6 expression may represent an important component of the excessive inflammatory response observed in cystic fibrosis.

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.

Cystic fibrosis is characterized by the accumulation of thick viscous purulent secretions. Recombinant human deoxyribonuclease I (rhDNase) breaks down extracellular DNA, which contributes to the increased viscosity of sputum. A multinational, open-label study was conducted in 974 cystic fibrosis patients with moderate lung disease [forced vital capacity (FVC) 40-70% of predicted values] to examine the safety and efficacy of aerosolized rhDNase, 2.5 mg, once daily over a period of at least 12 weeks. Patients were assessed under conditions reflecting routine clinical practice. During rhDNase therapy, at least one respiratory tract infection (RTI) requiring intravenous antibiotics was experienced by 29.5% of patients. Forced expiratory volume in 1 second (FEV1) and FVC were significantly improved from baseline by a mean of 10.5% and 7.2%, respectively. Voice alteration and pharyngitis were the most frequent rhDNase-related adverse events, but only 2% of all patients discontinued treatment due to adverse events. The results obtained were similar to a subanalysis of data from the first 3 months of a placebo-controlled U.S. study. The patients in the present study had a similar frequency of RTIs and improvement in pulmonary function, and reported fewer rhDNase-related and cystic fibrosis-related adverse events than patients in the U.S. study. We conclude that administration of rhDNase is safe, well tolerated, and effective under conditions reflecting routine clinical practice in patients with cystic fibrosis and moderate lung disease.

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.

More than 600 mutations have been identified in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene and are known to cause cystic fibrosis (CF). This large number of mutations makes the search of the molecular defects in CF patients difficult. We have used the techniques of denaturing gradient gel electrophoresis (DGGE) and direct DNA sequencing to detect the mutations in 334 CF chromosomes mostly of French origin. The whole coding sequence of the CFTR gene corresponding to the 27 exons and their exon-intron boundaries was studied. 45 different mutations were identified. This method allowed us to detect the molecular defect in 90.5% of the mutant alleles and to report a novel mutation D979V.

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.

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.

Mucoepidermoïd carcinomas (MEC) are very rare (less than 20 cases reported in the literature) and potentially malignant bronchial gland carcinomas.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.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.

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.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.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.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.

The value (in terms of decreased numbers of attacks and of hospitalizations for attacks, decreased need for asthma medications, and improved tolerance to allergens) of rush immunotherapy to a mixture of allergens was studied in children with multiple sensitizations and severe asthma (as evaluated on the number of attacks, number of hospitalizations, and dependence on corticosteroids) already receiving optimal medical therapy. Because syndromic reactions are common, rush immunotherapy should be performed in the hospital and premedication with corticosteroids may be warranted in the most severe cases.

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.

Compliance with treatment is a crucial factor in the management of asthmatic children which depends on the understanding of the disease and its treatment. The understanding of, and compliance with treatment were evaluated by means of a questionnaire in 50 parents of asthmatic children. 50% of these parents used anti-histamines as maintenance treatment and 30% also used these drugs during attacks. Half the parents knew about the bronchodilator effects of theophylline and B2-agonists. 42% and 30% respectively of the parents thought that the side-effects of theophylline or corticosteroids were few or inexistent; 86% claimed regular attendance to out-patient clinics, but 30% confessed that they had forgotten such drugs as theophylline and antihistamines. 50% took the appropriate therapeutic measures when confronted with a moderate or severe attack of asthma. Each child received 2.3 drugs on average for his or her asthma. This study shows that parents have an insufficient knowledge of asthma treatments, and this may partially explain the poor compliance with therapy as well as the morbidity and mortality associated with childhood asthma.

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.

The only available method for quantitative exploration of skin allergy to tuberculin is intradermal injection, since there is no other satisfactory method to evaluate the induration observed. Below 5 mm the induration has no significance. Between 5 and 10 mm it corresponds to an allergic reaction induced by BCG vaccine or by usually inapparent infections due to atypical mycobacteria. A diameter of 10 mm or more suggests either M. tuberculosis infection or allergy to BCG. The intensity of allergic reaction varies under the influence of numerous factors which may be genetic or nutritional, or associated with viral infections, antiviral vaccines and medicinal treatments. Exploring this allergy is of interest on the scores: (1) for epidemiological purposes, to obtain information on the annual risk tuberculosis infection or on the quality of the protection conferred by the vaccine in a given population; (2) for diagnostic purposes, to detect tuberculosis, and especially primary tuberculosis in children; (3) for vaccinal purposes, to determine the limitation of BCG. In subjects who have been vaccinated tuberculosis is rare but remains possible, and in such cases reactions to tuberculin may be difficult or even impossible to interpret.

ENT involvement is very frequent in mucoviscidosis, particularly rhinitis and sinusitis. This prospective study, which spanned from September 1st, 1988 to August 31st, 1989 anc included 27 children with cystic fibrosis of pancreas was carried out with the intent of determining the incidence of the various associated diseases, their bacteriologic profile, and the optimal therapeutic procedure. Less than a child in 5 is symptomless when the nose and sinuses are affected. Polyposis is found to occur in 6/27 cases, and all children have sinus radio-opacities. Ear disease is rare (1/4 of cases) and is manifested by asymptomatic tubal dysfunction with minimal audiometric repercussions. Bacteriologic specimens of sputum and sinus purulent discharge show simultaneous germ positivity (10 times out of 11). Our therapeutic recommendations are based on our own experience as well as literature data, and are, in our opinion, only relevant for children with clinical signs of disease, as opposed to advocating systematic treatment of sinus foci found on X-ray.

The short term prognosis of the esophageal atresia has been improved by the progress of diagnosis and surgical techniques. The long term prognosis still depends upon the occurrence of bronchopulmonary complications. Having investigated fourteen cases and a review of the literature, the authors studied the cause of these respiratory complication. Two factors seem to play an important role: the gastroesophageal reflux and the tracheomalacia. This has important therapeutical implications: treatment of the gastro-esophageal reflux and long term respiratory kinesitherapy. The authors suggest for these patients a management plan over several years.

The authors report a new case of partial immune deficiency of cellular immunity, associated with chronic mucocutaneous candidiasis in a 12 Years-old boy. The disease began very early during the first few weeks of life, with thrush in the mouth. This candidiasis then evolved intermittently and was still present. Numerous cutaneous, pulmonary and ear infections occured throughout this child's life. This morbid association led to a search for an immune deficiency. Humoral immunity was normal. Abnormalities of cellular immunity were as follows: apart from candidine skin anergy, there was a deficiency in the factor which inhibits leukocyte migration, secretion of a factor favouring this migration (MEF). It was also noted the presence of the patient's serum, of a factor inhibiting lymphocyte transformation in the presence of candidine. In spite of treatment with intravenous route, amphotericin B, followed by transfer factor, the oral candidiasis persisted together with the skin anergy to candidine. On the other hand, the serum inhibitory factor disappeared. Pulmonary cryptococcosis probably favoured by corticosteroid treatment, developed on this background of immune deficiency; as usual it spread to the meninges. Treatment associating intraveinous amphotericin B and 5 fluorocytosine oral and later intravenous, total duration 6 months, grave a recovery maintained on a 8 months follow up.

The authors discuss the etiological features of interstitial pneumopathies, with particular emphasis on their characteristics in the newborn and in immunodeficient children, and review the diagnostic findings : clinical and radiological signs, functional disturbances, and results of pulmonary biopsy. The results of 154 needle biopsies in 124 children aged 1 month to 16 years are described. In 103 cases a diffuse pneumopathy was present and 80 % of the biopsies were successful. Precise diagnosis could be made from the histology specimen in 22 cases out of the 82 examined, while in 60 of these cases the specimen could be identified.

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.

Chlamydia trachomatis now has an important place in infantile respiratory pathology. In neonates, it is transmitted in utero, which explains one quarter of the cases of interstitial pneumonia seen during the first months of life. In children, the number of carriers of antibodies increases with age and may be more than 40 p. cent by the age of 7 years. The mode of contamination and the pathological significance remain uncertain in this age group. Chlamydia trachomatis can be a cause of pneumonia in immuno-depressed patients. A survey conducted in a Paediatric Respiratory Medicine Unit showed that 22 p. cent of patients were carriers of the antibody and at least 7.8 p. cent were infected.

The present study relates to 39 children, 24 boys and 15 girls, aged 1 to 17 (mean age, 6 1/2 years) suffering from chronic airflow obstruction without muco-viscidosis. The search for a ciliary anomaly was motivated either by the coexistence of situs inversus (group I: 12 cases) or by the negative or scanty aetiological history (group II: 27 cases). The investigation of the cilia consists of a study of ciliary motility and an ultrastructural study of biopsy specimens using the electron microscope. Groups I and II were similar as regards severity of disease, assessed by the incidence of bronchiectasis and chronic hypoxia at rest; a higher incidence of neonatal respiratory distress was noted in group I (7 cases against 4 in group II). Ciliary immotility was particularly noted in group I (7 cases) and one case of weak motility in group II. The major ultrastructural anomalies of the axonemal complex were seen as well in both groups I and II; it was noted that all the structures may be totally or partially missing with the exception of the peripheral microtubules. The discussion centred on three points: 1. In the first analysis, the discordance between the existance of major ultrastructural anomalies of the axonemal complex, a priori incompatible with the conservation of ciliary motility on light microscopy; the link seems to lie in the percentage of cilia affected, the existence of ciliary dyskinesia and finally the disorientation of the basal corpuscles. 2. The immunologic abnormalities sometimes associated with a syndrome of ciliary immotility: that is the anomalies of leucokyte migration under the control of microtubular structures in the peri-centriolar region. 3. The practical consequences. Current treatment rests on daily respiratory physiotherapy and antibiotics adapted for cases of superinfection; also there are drugs stimulating ciliary activity perhaps by the effect on their residual motility; some substances carrying ATP and ATP-ase may re-establish ciliary motility anulled by the absence of dynein arms; also the observations of major ultrastructural abnormalities ought to lead to better genetic counselling than at present, where the mode or modes of transmission are uncertain.

Three cases of cholesterol interstitial pneumonia in patients 3, 9 and 10 years of age respectively are reported. All three were born in Island of Reunion. Two were sisters. All had failure to thrive, dyspnea on rest and clubbing. Respiratory symptoms had appeared early in infancy. Open pulmonary biopsy was diagnostic. Prognosis was poor the boy dying at 4 years of age and severe respiratory insufficiency at 9 or 10 years in the two girls. Current etiological investigations were non contributory. However a profile of chronic infection with Epstein Barr virus (EBV) was found in each case while serological profiles ruled out infection with a virus of the herpes group virus (cytomegalovirus, herpes simplex). The possible role of EBV as an etiological agent of cholesterol pneumonia is discussed and genetic or environmental factors as well.

127 cases of tracheal dyskinesia were seen in infants and children out of which 87 were 1 to 12 months of age. The diagnosis was based on the existence of a collapse reducing the tracheal diameter of more than 50% on endoscopy. Endoscopic examination was performed without general anesthesia. This material represents 5,8% of the patients submitted to this procedure. 85 patients had "primitive" dyskinesia and 42 had major associated abnormalities. Uni or bilateral bronchial dyskinesia was associated in 43% of the cases. The four commonest presenting symptoms were a stridulous or wheezing respiration, recurrent bronchitis, chronic cough, cyanosis. The frequency of associated digestive troubles: gastroesophageal reflux aspiration was noteworthy. Several functional consequences were encountered: hypoxemia, hypercapnia, abnormalities of FRC, increased RL, lowering of dynamic compliance, alterations of perfusion and ventilation on scintiscans. The prognosis was good in primitive cases. Two deaths occurred, in the group with associated abnormalities. The pattern of the patient with primitive dyskinesia and that of the patient with dyskinesia and associated abnormalities are outlined. Some features remarkable in this series of patients are pointed out in a discussion of the pathophysiology of the syndrome. Increased transmural pressure is not a common cause of tracheal dyskinesia and infection as well. The possibility of a temporary intrinsic anomaly of the tracheal wall is suggested. Even if its exact mechanism remains unknown, tracheal dyskinesia is a distinct entity observed in infants and children. It appears as a common cause of recurrent bronchopulmonary disease in the young.

The authors describe 7 cases of idiopathic pulmonary hemosiderosis occurring in children, and emphasize the diagnostic value of the association of pulmonary and anemic manifestations, one of which could appear to be an isolated finding and thus delay diagnosis. Complementary investigations are discussed and the relative inocuity of a lung biopsy with a Gerbeaux needle, and the greater value of bronchoscopic samples over those obtained by gastric intubation for evaluating the presence of siderophagic activity emphasized. Functional pulmonary tests, especially for blood gases and ventilatory function, were also conducted. The difficulties in classifying the various types of pulmonary hemosiderosis, especially the "idiopathic" form in children, are outlined. Immunological disorders must be systematically searched for in order to choose the most effective therapy: corticoids or immunodepressants.

The purpose of this study was to evaluate inspiratory muscle force reserve in children with chronic obstructive pulmonary disease (COPD). In 15 hyperinflated (FRC/TLC, 65 +/- 0.7%) children, maximal mouth inspiratory static pressure (PImax) at FRC, mouth occlusion pressure (P0.1), tidal volume (VT), inspiratory time (TI), and total duration of the respiratory cycle (Ttot) were all measured. It was found that PImax at FRC was reduced compared with predicted values. However, after lung volume correction, PImax was in the normal range, and P0.1 was higher, TI was shorter, and Ti/Ttot was lower than predicted. The estimated mean inspiratory pressure for breathing at rest (PI) was significantly higher than predicted and was related to total pulmonary resistance (r = 0.74, p less than 0.001). The fraction of PImax developed by the respiratory muscles for breathing at rest (PI/PImax) significantly increased. The higher the PI/PImax ratio, the more the TI/Ttot ratio decreased (r = -0.64, p = 0.01). At rest, our subjects had to develop a mean inspiratory power (W) of as much as 48% (range, 30 to 76%) of the critical W above which fatigue occurs. Thus, even minimal increases in breathing load might expose children with COPD to respiratory muscle fatigue and to respiratory failure.