[Malnutrition in children with chronic bronchitis]. / Prévalence et conséquences de la dénutrition au cours de la bronchite chronique de l'enfant.

OBJECTIVE: To study nutritional status in children with chronic bronchitis (CB) in relation with lung function. METHODS: In this cohort of study, 46 patients aged 6.0 to 17.5 years (mean: 11.9 years) with chronic bronchitis were recruited. None had cystic fibrosis. Body weight, height, skinfold thicknesses, percentage of ideal body weight-for-height (percentage of IBW), body mass index (BMI), BMI Z-score, fat mass and fat-free mass were used to evaluate nutritional status. Arterial blood gases, vital capacity (VC), forced expiratory volume in one s (FEV1), functional residual capacity (FRC) and maximum inspiratory (Pi(max)) and expiratory (Pe(max)) pressures at the mouth were used to evaluate respiratory function. RESULTS: Thirteen children (28%) had malnutrition defined as percentage of IBW lower than 90%, with a predominant fat mass depletion. VC (65+/-13% versus 79+/-15%; p=0.006) and FEV1 (59+/-16% versus 69+/-14%; p=0.03) were significantly lower in children with malnutrition than in children without malnutrition, but no significant differences were observed with regard to the FEV1/VC ratio and blood gases. Pi(max) (56+/-11% versus 88+/-37%, p=0,02) and Pe(max) (46+/-12% versus 58+/-19%, p=0,3) were also lower in children with malnutrition as compared to than without malnutrition. CONCLUSION: Malnutrition can be observed in children with CB and is associated with significant lower lung function parameters. This could be explained by decrease in respiratory muscle strength.

Genetic resistance/susceptibility to mycobacteria: phenotypic expression in bone marrow derived macrophage lines.

Congenic strains of mice susceptible (B10A.Bcgs) or resistant (B10A.Bcgr) to BCG were established. Here we describe the model system which has been established to analyze the functional activities of macrophages in the two strains. We have immortalized bone marrow macrophages from B10A.Bcgs and B10A.Bcgr congenic strains of mice and derived cloned macrophage lines designated B10S and B10R, respectively. B10R and B10S cell lines exhibited surface markers and morphology typical of macrophages. B10S and B10R were similar in their phagocytic activity, in their level of c-fms, in their transforming growth factor beta (TGF beta) mRNAs expression, and in their expression of tumoricidal activity in response to interferon-gamma (IFN gamma) plus lipopolysaccharides (LPS). However, B10R macrophages expressed a higher level of la mRNA when activated with IFN gamma compared with B10S macrophages. Analysis of the bacteriostatic activity of the two cell lines revealed that B10R macrophages were much more active in inhibiting Mycobacterium smegmatis replication than B10S. To measure the intracellular destruction of bacilli, a bactericidal assay based on hybridization with an oligonucleotide probe specific for mycobacterial ribosomal RNA was designed. The results demonstrated that B10R macrophages were endowed with enhanced constitutive bactericidal activity as compared with B10S. In conclusion we have obtained macrophage lines from bone marrow of B10A.Bcgs and B10A.Bcgr mice that express to a similar extent functional and phenotypic characteristics of macrophages. However, we demonstrate that relative to B10S macrophages, the B10R macrophages have higher expression of la mRNA and that they are constitutively more active in expressing mycobactericidal activity.

[Methacholine challenge in young children: measurement of resistance by interruption]. / Test a la métacholine chez le jeune enfant: Mesure de la résistance par interruption.

The aims of this study were 1. To evaluate the measurement of resistance by interruption (Rint) of bronchoconstriction induced by inhalation of methacholine and 2. To determine a threshold of increase of resistance in young children to differentiate responders from non-responders. Forty-six children (mean age 5 [4.3-6.1] years) referred for methacholine challenge were tested by measurement of Rint and transcutaneous oxygen tension. A fall of 20% or more in oxygen tension from the baseline was used to define the responders. The children studied had a baseline Rint significantly higher than normal (0.84 [0.68-1.01] vs. 0.76 [0.60-0.90] kPa L(-1)s; p < 0.03). Forty-one children were responders and had an increase in Rint significantly different from the non-responders (p < 0/04). An increase in Rint of 35% distinguished responders from non-responders in young children with chronic cough. Interrupter resistance increases significantly during bronchial provocation in responding young children and may be used to measure the degree of bronchoconstriction.

Lung mechanics and breathing pattern during wakefulness and sleep in children with enlarged tonsils.

Thirteen children (mean age, 45 months) with nocturnal symptoms of upper airway obstruction, the result of enlarged tonsils, were tested during wakefulness (W) and sleep (S) induced by chloral hydrate (less than or equal to 50 mg/kg). During W, lung mechanics, blood gas, breathing pattern, and airflows during tidal breathing were in the normal range. During S, total lung resistance increased significantly, and dynamic lung compliance and transcutaneous PO2 decreased significantly. During S, the tidal volume (VT) and the mean inspiratory flow, normalized for body weight (BW), decreased whereas the ratio of the inspiratory time (TI) over the total duration of the respiratory cycle (TTOT) rose, indicating a longer contraction time of the respiratory muscles. The time to reach peak inspiratory flow, measured as a percentage of TI (dTI/TI), increased in seven children, with no change in the ratio of the expiratory flow over the inspiratory flow, both measured at 50% of VT (EF50/IF50). In three other patients dTI/TI decreased with an increase in EF50/IF50. We conclude that in children with enlarged tonsils, S modified lung mechanics, gas exchange, and the inspiratory components of the breathing pattern and airflow.

Resting lung function in children after repair of tetralogy of Fallot.

Children with tetralogy of Fallot had pulmonary function tests (PFT) after intracardiac repair (ICR). According to the age at time of ICR and to the existence or not of palliative surgery preceding ICR, they were divided into three groups. Group 1 had ICR at a mean of one year one month of age (range four months to one year seven months), that is, during the active period of postnatal lung growth. Groups 2 and 3 had ICR later in childhood, that is, respectively, at four years seven months (range two years nine months to 11 years 9 months) and at five years four months (range two years seven months to 11 years five months). In group 3, ICR was preceded by palliative surgery. The PFT at rest included measurement of lung volumes (functional residual capacity), vital capacity (VC), dynamic or static compliance (CL), total pulmonary resistance, lung transfer factor for CO (TLCO) and blood gases. Group 1 had normal lung function suggesting that early repair of TOF saves lung development. In groups 2 and 3, significant decrease in VC(p less than 0.01) and CL (p less than 0.01) were found suggesting impaired alveolar growth. Additional defect in TLCO (p less than 0.01) in group 3 suggested that palliative surgery induces abnormal vascular growth. Thus, the present functional results suggest repair of TOF during the two first years of life, ie, the active period of postnatal lung growth.

Comparison of transcutaneous and alveolar partial pressure of carbon dioxide during carbon dioxide breathing in healthy children.

In 18 healthy children three to 13 years of age, the transcutaneous partial pressure of carbon dioxide (PtcCO2) (Radiometer electrode) and the alveolar partial pressure of carbon dioxide (PACO2) (Beckman analyzer) were measured simultaneously during the breathing of room air and 5 percent carbon dioxide. The PtcCO2 electrode was placed on the anterior thorax and heated to 42 degrees C. The PACO2 was calculated on the 4/5 part of the carbon dioxide expired trace. Minute ventilation (VE) was measured in 11 cases. There was a significant correlation between PtcCO2 (in millimeters of mercury) and PACO2 (in millimeters of mercury) while breathing room air (PtcCO2 = 0.82 PACO2 + 19.7; r = 0.55; p less than 0.02) and while breathing 5 percent carbon dioxide (PtcCO2 = 0.77 PACO2 + 22.5; r = 0.61; p less than 0.01); however, the ratio of PtcCO2 over PACO2 was significantly lower while breathing 5 percent carbon dioxide (p less than 0.01) than while breathing room air. When considering the relationship between the increase in VE (delta VE while breathing 5 percent carbon dioxide and the changes in PACO2 (delta PACO2) or in PtcCO2 (delta PtcCO2), a significant correlation was found only between delta VE and delta PACO2, ie, delta VE = 0.41 delta PACO2 + 0.44 (r = 0.63; p less than 0.01). These results suggest that breathing carbon dioxide modified the factors acting on PtcCO2, possibly by changes in the vasomotor tone of cutaneous blood vessels. These modifications appeared to be variable from subject to subject. Therefore, we conclude that PtcCO2 does not appear to be an accurate quantitative index to assess ventilatory response to carbon dioxide.

Genotype analysis and phenotypic manifestations of children with intermediate sweat chloride test results.

STUDY OBJECTIVES: Cystic fibrosis (CF) is one of the most common inherited diseases among whites. Since the cloning of the CF transmembrane conductance regulator (CFTR) gene, a number of studies have focused on associations between the genotype and phenotype in CF. This had led to the progressive identification of new groups of patients, including those who have mild lung disease and those who have normal sweat chloride values (< 60 mEq/L). The aim of the present work was to provide information on the genotype and the phenotypic characteristics of children with intermediate-range sweat chloride test results. PATIENTS AND RESULTS: We focused on children referred to the pulmonary department for various types of pulmonary disease and who had several sweat chloride test results with median values in the range of 40 to 60 mEq/L. Twenty-four patients over a 10-year period were enrolled (mean age, 4.8 years). Respiratory manifestations at initial evaluation included recurrent bronchitis, wheezing, chronic cough, and pneumonia. The duration of the follow-up ranged from 0.5 to 10.5 years. Sputum cultures revealed the presence of Haemophilus influenzae (10 children), Staphylococcus aureus (4 children), and Pseudomonas aeruginosa (3 children). Pancreatic insufficiency was found in two patients. Analysis of the entire coding sequence allowed identification of 16 known mutations in CFTR gene. Fifteen chromosomes (31.2%) carried a mutation in CFTR gene and one allele carried two mutations. Three patients were homozygous or double heterozygous (DeltaF508/DeltaF508, DeltaF508/3849 + 10 kb C-->T, S1235R/G551D). The 5-thymidine allele was identified in four children. CONCLUSION: These results indicate an higher frequency of CFTR gene mutations in patients with borderline sweat chloride test results, compared to data reported in the general population. They lead to the recommendations for complete pulmonary and GI investigations in this group of patients, as well as assiduous care and medical follow-up.

Effects of hypercapnia and hypoxia on inspiratory and expiratory diaphragmatic activity in conscious cats.

The influence of steady-state changes in chemical stimuli on ventilation and electromyographic activity of the diaphragm during both inspiration (total DI) and expiration (total DE) was studied in unanesthetized intact adult cats before and after carotid denervation. In intact animals, during hypercapnia (2 4, and 6% CO2), tidal volume (VT) and total DI increase, whereas total DE did not consistently change. During ambient hypocapnic hypoxia (14, 12, and 10% O2), VT increased only at 10% O2, whereas total DI increased at all levels studied. Total DE increased substantially at 14% O2, persisting up to the end of expiration with 12 and 10% O2. This effect was markedly attenuated during normocapnic hypoxia. During CO hypoxemia (1,700 ppm in air), VT as well as total DI and total DE decreased because of a large reduction in inspiratory and expiratory time elicited by tachypneic breathing. The effects of hypercapnia and hypoxia persisted after carotid denervation. Therefore, 1) in contrast to hypercapnia, hypoxia markedly enhances the expiratory diaphragmatic activity, 1) this expiratory braking mechanism depends on the severity of hypoxia and is partly due to hypocapnia secondary to hypoxia; and 3) because this effect was observed after carotid denervation and during CO hypoxemia, it may arise in the central nervous system, possibly in bulbopontine structures.

Diaphragmatic and ventilatory responses to alveolar hypoxia and hypercapnia in conscious kittens.

Ventilation and electromyographic (EMG) activity of the diaphragm were recorded in unanesthetized kittens 2 and 10 wk of age during normoxia, hypercapnia (2 and 4% CO2), and hypoxia (12 and 10% O2). We measured integrated diaphragmatic EMG activity at end inspiration (DIAI) and end expiration (DIAE); the difference (DIAI-E), which represents the phasic change of the diaphragmatic activity, was considered responsible for a given tidal volume (VT). During hypercapnia, the 2-wk-old kittens increased minute ventilation (V) by increases in both VT and respiratory frequency (f), whereas the 10-wk-old kittens increased V primarily by an increase in VT. At both ages, DIAI and DIAI-E increased during hypercapnia, whereas DIAE did not change significantly. During hypoxia, in the young kittens, V and VT decreased while f increased markedly; in the older kittens, V, VT, and f did not change significantly. In kittens of both ages, DIAI increased during hypoxia; because diaphragmatic activity persisted into expiration, DIAE also increased. DIAI-E, as well as VT, was decreased in the young kittens, whereas in the older ones DIAI-E was slightly increased despite an unchanged VT. Finally, the ventilatory and diaphragmatic response to hypoxia changes with maturation in contrast to the response to hypercapnia. It is concluded that 1) the hypoxia-induced reduction of VT may result from prolongation of diaphragmatic activity into expiration, inasmuch as it induces a reduction of the phasic change of the diaphragmatic activity, and 2) because DIAI-E indirectly reflects central inspiratory output, a central mechanism should be involved in the reduced VT and V in response to hypoxia in newborns.

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.

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.

Pulmonary function in infancy and in childhood following mechanical ventilation in the neonatal period.

Pulmonary function was evaluated in both infancy and childhood in the same 19 prematurely born infants, who required mechanical ventilation (MV) during the neonatal period. Results of our patients were compared with those of control subjects. Upon first evaluation, we found that lung resistance (RL) was significantly elevated (24.85 +/- 6.06 vs. 17.77 +/- 2.39 cmH2O/L/s; P less than 0.01). The mean value of dynamic lung compliance (CLdyn) was low, but the difference compared to controls did not reach significance. From infancy to childhood, elevated RL persisted (9.33 +/- 2.51 vs. 6.52 +/- 1.52 cm H2O/L/s; P less than 0.01), and the decrease of CLdyn became significant (46.86 +/- 12.84 vs. 59.34 +/- 15.68 mL/cmH2O; P less than 0.05). In addition, maximum flow at functional residual capacity was significantly decreased (0.824 +/- 0.284 vs. 1.215 +/- 0.358 L/s; P less than 0.01); whereas pulmonary diffusing capacity for carbon monoxide was similar in the patients (7.62 +/- 2.16 mL/min/mm Hg) and in the controls (8.38 +/- 1.6). Pulmonary dysfunction following premature birth, respiratory distress, and prolonged MV may not resolve from infancy to childhood.

Pulmonary function at 1 year of age in survivors of neonatal respiratory distress: a multivariate analysis of factors associated with sequelae.

We studied pulmonary function 1 year after neonatal respiratory distress (RD) in 54 infants who had been treated at birth in the same neonatal intensive care unit. RD was related to hyaline membrane disease in 36 cases (group I) and to other causes in 18 cases (group II). Compared with predicted values, dynamic lung compliance (CL) was lower (less than -2 SD) and total pulmonary resistance (RL) was higher (+2 SD) in 18 (33%) and 12 (22%) infants, respectively. The relationships between these functional abnormalities at 1 year of age and the characteristics of the neonatal respiratory disease were assessed using a multifactorial analysis (multiple correspondences analysis). We found that elevated RL (greater than +2 SD) at 1 year of age was very significantly related with hyaline membrane disease, involving both high rate of positive pressure ventilation and prolonged intubation, and with the presence of both tachypnea and abnormal chest X rays at the time of discharge. Compared with elevated RL, the relationships between low CL (less than -2 SD) and the same neonatal characteristics were less significant; in particular, there was no strong link between low CL and hyaline membrane disease. Finally, birth before 30 weeks gestation was an index of severity.

Decrease in the rate of protein synthesis by polysomes from cultured fibroblasts of patients and carriers with Duchenne muscular dystrophy.

Polysomes extracted from cultured fibroblast cells isolated from patients with Duchenne muscular dystrophy (DMD), carriers of the disease, and normal controls were used for in vitro measurement of protein synthesis in a wheat germ extract system. It was observed that polysomes from patients and carriers (seven of each aged 17 years or older) exhibited a 3-fold and a 1.5-fold decrease in the rate of protein synthesis, respectively, as compared with controls. These results are discussed with a view to developing a sensitive and easily available assay for the detection of DMD carriers.

[Functional respiratory disorders in children with left-to-right shunt]. / Perturbations fonctionnelles respiratoires chez les enfants proteurs de cardiopathies avec shunt gauche-droite.

The respiratory function of 19 children aged from 2 months to 9 years old with a cardiac lesion giving a left-to-right shunt was studied and analysed with respect to the hemodynamic disturbance. Respiratory function tests (RFTs) included measurement of functional residual capacity (FRC), the partial pressure in oxygen (Pa 02) on arterialised blood taken from the ear lobe, dynamic pulmonary compliance (CI dyn.) and total pulmonary resistance (RI). FRC and RI were not significantly modified. On the other hand, CI. dyn. was low (-31.2 +/- 22.9%, p less than 0,001) as were the CI. dyn. CFR ratio (-25.5 +/- 20.9%, p less than 0.001) and the Pa 02 (-7.6 +/- 12.7% p less than 0.02). There was a significant correlation between the reduction of CI. dyn. and mean pulmonary arterial pressure (PAP) and the increase in the pulmonary blood flow. In addition, a significant correlation was also found between the reduction in Pa 02 and the PAP and increase in pulmonary blood flow. These results show that cardiac lesions with left-to-right shunts give rise to diffuse changes in respiratory function and are discussed in the light of the abnormalities in pulmonary development previously described in pulmonary biopsy of patients with left-to-right shunts. The value of RFTs in the surveillance of patients with left-to-right shunts is underlined.

[Clinical and respiratory function follow-up of 39 infants treated with neonatal respiratory extracorporeal assistance]. / Suivi clinique et fonctionnel respiratoire de 39 nourrissons traités par assistance respiratoire extracorporelle néonatale.

UNLABELLED: The aim of this prospective study was to evaluate the consequences of neonatal treatment with a venovenous extracorporeal respiratory assistance. POPULATION AND METHODS: Thirty nine neonates (28 boys) with acute respiratory failure were included. Extracorporeal respiratory assistance consisted of an apnoeic oxygenation with low frequency positive pressure ventilation and extracorporeal membrane CO2 removal through a venous single canula perfusion circuit. The causes of respiratory distress were: 15 meconium aspiration syndrome, 12 respiratory distress syndrome, six hyaline membrane disease, three sepsis, two diaphragmatic hernia, and one post-surgery Mendelson syndrome. The mean duration of mechanical ventilation was 18 days, including 5 days of extracorporeal respiratory assistance. The prospective follow-up included physical examination, chest radiographs, scintigraphy and pulmonary function tests. These tests studied the following parameters: functional residual capacity by helium dilution technique, lung resistance and dynamic lung compliance by the esophageal balloon technique and blood gases with arterialized blood samples. RESULTS: The mean duration of the follow-up was 21.3 months (6 months to 5 years). Results showed in the first year 33% of children with chronic obstructive pulmonary disease and chest (X-ray abnormalities, such as bronchopulmonary dysplasia in 23% of children. Data of pulmonary function test at the end of the first year: lung resistance and functional residual capacity were within limits of predicted values for height, and dynamic lung compliance was slightly decreased; according to the analysis of the functional profile: 31% without abnormality, 41% of obstructive syndrome and 26% with restrictive pattern. Blood gases were normal in 37 children. At the end of the second year, we noticed normal functional residual capacity, an increase of lung resistance while lung compliance was normalized; functional profile was quite different: with a decrease of the number of patients without abnormality (22%) and increase of those with obstructive syndrome (56%). CONCLUSION: The percentage of abnormalities is high but these are moderate in most cases, especially if we compare with the initial seriousness of the pulmonary pathology. We suggest a regular follow-up to study the respective incidence of pulmonary disease and/or extracorporeal respiratory assistance over these abnormalities.

[Isocapnic hyperventilation test adjusted to child's resting ventilation rate]. / Un test d'hyperventilation isocapnique adapté à la ventilation de repos de l'enfant.

In order to validate an Isocapnic Voluntary Hyperventilation (IVH) test applicable to daily practice and to adapt the stimulus to height, 9 healthy and 15 asthmatic children performed a Resting Ventilation Rate (RVR)-corrected IVH. They performed a three-minute IVH with room temperature dry air achieving twice (IVH2) and three times (IVH3) their RVR. Mean Maximal Expiratory Flow (MEF) in the middle half of Forced Vital Capacity (FVC) (MEF25-75%) and mean MEF at 25% of FVC (MEF25%) are decreased in the asthmatic group 10 minutes IVH3 (p = 0.02 and < 0.002) compared to healthy group. Mean FEV1 of both group are not different. Comparing Forced Expiratory Flows variation after IVH to baseline intrasubject coefficient of variation, sensitivity of the test is 80% and specificity 100% when variations of MEF25-75% and MEF25% together with FEV1 variations are considered. This suggests an easy way to adapt an hyperventilation stimulus to size and emphasizes the utility of taking account of MEF25-75% and MEF25% in detecting non specific bronchial hyperreactivity in asthmatic children.

[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.

[Diseases of the pulmonary lymphatic system in children]. / Pathologies des lymphatiques pulmonaires chez l'enfant.

Diseases of the lymphatic system in children include a group of exceptional conditions difficult to manage. The anatomy of lymphatic system is complex in the lung. Variable from one subject to another, its complex physiology plays an important role in air-blood exchanges occurring in the lung. In the pulmonary interstitium and in the pleura, the lymphatic system acts like an overflow valve capable of regulating variations in interstitial fluid. The presence or development of dysplasic lymphatics causes leakage, dilatation, and reflux of the lymph through incontinent valves leading to chylothorax and/or fluid overload in the pulmonary interstitium. Symptomatic care is usually proposed, based on a fat-free diet supplemented with light-chain triglycerides and liposoluble vitamins. Other therapeutic options can be proposed. Medical options include cytotoxic agents, somatostatin, and interferon-alpha. Surgery may also be useful, but an assessment of therapeutic efficacy is very difficult due to partial effects and the small number of cases studied.