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STUDY OBJECTIVES: To analyze the physiologic effects and tolerance of mechanical insufflation-exsufflation (MI-E) by means of mechanical cough assistance (Cough Assist; JH Emerson Company; Cambridge, MA) for children with neuromuscular disease. DESIGN: Prospective clinical trial. SETTING: Physiology laboratory of a pediatric pulmonary department of a university hospital. PATIENTS: Seventeen children with Duchenne muscular dystrophy (n = 4), spinal muscular atrophy (n = 4), or other congenital myopathy (n = 9) who were in a stable state. INTERVENTIONS: Pressures of 15, 30, and 40 cm H(2)O were cycled to each patient, with 2 s for insufflation and 3 s for exsufflation. One application consisted of six cycles at each pressure for a total of three applications. MEASUREMENTS AND RESULTS: Airway pressure and airflow were measured during every application. Breathing pattern, vital capacity (VC), sniff nasal inspiratory pressure (SNIP), peak expiratory flow (PEF), and respiratory comfort were evaluated at baseline and after each application. The tolerance of the patients was excellent, with a significant increase in the respiratory comfort score in all of the patients (p = 0.02). Expired volume during the MI-E application increased significantly to reach twice the VC at 40 cm H(2)O. Mean and maximal inspiratory and expiratory flows increased in a pressure-dependent manner. Breathing pattern did not change after the MI-E applications and pulse oximetric saturation remained stable within normal values, but the mean end-tidal carbon dioxide pressure decreased significantly. VC did not change, but the mean SNIP and PEF improved significantly after MI-E applications. CONCLUSIONS: Our results confirm the good tolerance and physiologic short-term benefit of the MI-E in children with neuromuscular disease who were in a stable state. [ABSTRACT FROM AUTHOR]

Study Objective: s: The aim of the study was to evaluate the efficacy and safety of premixed 50% nitrous oxide and oxygen on the quality of sedation and pain control during fiberoptic bronchoscopy (FB) in children.Design: A prospective, randomized, double-blind study.Setting: Pediatric pulmonary department in a pediatric tertiary university hospital.Patients: One hundred five children aged 1 month to 18 years.Interventions: Patients inhaled after sedation and local anesthesia either premixed 50% nitrous oxide and oxygen (nitrous oxide group) or premixed 50% nitrogen and oxygen (control group) during FB.Measurement and Results: The rate of failure was significantly greater in the control group (62%) than in the nitrous oxide group (21%, p = 0.00003). The efficacy of premixed 50% nitrous oxide and oxygen was also demonstrated with higher satisfaction scores (p = 0.000001), lower Children's Hospital of Eastern Ontario Pain Scores (p = 0.002), better visual analog scale ratings (p = 0.03), and improved behavior scores. Side effects were minor and similar in both groups.Conclusions: This study demonstrates the improved efficacy of sedation, pain control, and safety of premixed 50% nitrous oxide and oxygen for FB in children. [ABSTRACT FROM AUTHOR]

The article discusses the optimal time to perform polysomnography in patients with neuromuscular disease. Non-invasive ventilation (NIV) is recognized as an efficient therapeutic option in patients with chronic respiratory insufficiency due to neuromuscular disorders. If NIV is proposed earlier in the course of ventilatory failure, it should be accompanied by a significant improvement in measurable symptoms attributable to nocturnal hypoventilation, disruption of sleep architecture, and poor quality of sleep.

This document is the outcome of a group of experts brought together at the request of the French Society of Sleep Research and Medicine to provide recommendations for the management of obstructive sleep apnea syndrome type 1 (OSA 1) in children. The recommendations are based on shared experience and published literature. OSA 1 is suspected when several nighttime respiratory symptoms related to upper airway obstruction are identified on clinical history taking. A specialist otolaryngologist examination, including nasofibroscopy, is essential during diagnosis. A sleep study for OSA 1 is not mandatory when at least two nighttime symptoms (including snoring) are noted. Therapeutic management must be individualized according to the location of the obstruction. Ear, nose, and throat (ENT) surgery is often required, as hypertrophy of the lymphoid tissues is the main cause of OSA 1 in children. According to clinical findings, orthodontic treatment generally associated with specialized orofacial–myofunctional therapy might also be indicated. Whatever treatment is chosen, follow-up must be continuous and multidisciplinary, in a network of trained specialists. [ABSTRACT FROM AUTHOR]

The aim of the study was to analyze the weaning success, the type of weaning procedures, and weaning duration in consecutive infants hospitalized in a pediatric intensive care unit over a winter season. A retrospective observational study was conducted in a pediatric intensive care unit in a tertiary center. Infants hospitalized for severe bronchiolitis were included and the weaning procedure from continuous positive airway pressure (CPAP), noninvasive ventilation (NIV), or high-flow nasal cannula (HFNC) was analyzed. Data from 95 infants (median age, 47 days) were analyzed. On admission, 26 (27%), 46 (49%), and 23 (24%) infants were supported with CPAP, NIV, and HFNC, respectively. Weaning failed in one (4%), nine (20%), and one (4%) infants while supported with CPAP, NIV, or HFNC, respectively (p = 0.1). In infants supported with CPAP, CPAP was stopped directly in five patients (19%) while HFNC was used as an intermediate ventilatory support in 21 (81%). The duration of weaning was shorter for HFNC (17 h, [IQR: 0–26]) than for CPAP (24 h, [14–40]) and NIV (28 h, [19–49]) (p < 0.01). The weaning phase corresponds to a large proportion of noninvasive ventilatory support duration in infants with bronchiolitis. The weaning procedure following a "step-down" strategy may lead to an increase in the duration of weaning. [ABSTRACT FROM AUTHOR]

Summary: Background: Infants treated have been followed for one year in order to assess conditions of use of palivizumab, safety, tolerability, and its impact on respiratory syncytial virus (RSV) hospitalisation rate. Patients and methods: Patients who received palivizumab during the epidemic season 2005–2006 were eligible. Follow-up was carried out 12 months after initiation of prophylaxis. Results: Sixty-four neonatal level II and III centers, pulmonary and cardiology units enrolled 1420 children. Mean follow-up was 10.9±0.2 months, mean gestational age (GA) 30±4 weeks and mean age at the start of prophylaxis was 5 months. Median number of injections was 5 and mean time interval between 2 consecutive injections was 30±6 days. Treatment was prescribed in accordance with the marketing authorisation indications (MA) for 84% of patients. For preterm infants born before 35 SA and less than 6 months of age, 60% was born before 33 SA and without BDP. The global readmission rate (for more than 24h) for documented RSV infection during the period of protection by palivizumab was 2.7% (37 in 1371) for all treated children: respectively 2% [IC95% =1.3–3.2], 2.7% [IC95% =0.7–4.7] and 3.7% [IC95% =0.8–6.6] for preterm infants less than 6 months of age, preterm from 6 to 24 months of age and for children with congenital cardiopathy. Palivizumab safety and tolerability were good. Conclusion: Evaluation of palivizumab prophylaxis in clinical practice confirms the clinical characteristics of treated infants, outlines their evolution and confirms safety of treatment. MA were generally well observed and a registry could be usefull to track the impact of the treatment out of MA. [Copyright &y& Elsevier]

Background and purpose: To describe a large series of patients with α, β, and γ sarcoglycanopathies (LGMD‐R3, R4, and R5) and study phenotypic correlations and disease progression. Methods: A multicentric retrospective study in four centers in the Paris area collecting neuromuscular, respiratory, cardiac, histologic, and genetic data. The primary outcome of progression was age of loss of ambulation (LoA); disease severity was established according to LoA before or after 18 years of age. Time‐to‐event analysis was performed. Results: One hundred patients (54 γ‐SG; 41 α‐SG; 5 β‐SG) from 80 families were included. The γ‐SG patients had earlier disease onset than α‐SG patients (5.5 vs. 8 years; p = 0.022) and β‐SG patients (24.4 years). Axial muscle weakness and joint contractures were frequent and exercise intolerance was observed. At mean follow‐up of 22.9 years, 65.3% of patients were wheelchair‐bound (66.7% α‐SG, 67.3% γ‐SG, 40% β‐SG). Dilated cardiomyopathy occurred in all sarcoglycanopathy subtypes, especially in γ‐SG patients (p = 0.01). Thirty patients were ventilated and six died. Absent sarcoglycan protein expression on muscle biopsy and younger age at onset were associated with earlier time to LoA (p = 0.021 and p = 0.002). Age at onset was an independent predictor of both severity and time to LoA (p = 0.0004 and p = 0.009). The α‐SG patients showed genetic heterogeneity, whereas >90% of γ‐SG patients carried the homozygous c.525delT frameshift variant. Five new mutations were identified. Conclusions: This large multicentric series delineates the clinical spectrum of patients with sarcoglycanopathies. Age at disease onset is an independent predictor of severity of disease and LoA, and should be taken into account in future clinical trials. [ABSTRACT FROM AUTHOR]

Background: Inspiratory muscle strength in patients with neuromuscular disorders can be assessed using sniff inspiratory nasal pressure (Pn(sn)) and maximum inspiratory mouth pressure (PI(max)). However, the relative merits of Pn(sn) against PI(max) are not known in patients with severe neuromuscular disease.Objective: To investigate whether severity of disease modifies the relation between Pn(sn) and PI(max).Methods: Vital capacity (VC), Pn(sn), and PI(max) were measured in 258 patients with neuromuscular disorders.Results: Data were analysed from 241 patients, 17 being unable to perform PI(max) or Pn(sn) manoeuvres. The correlation between Pn(sn) and PI(max) was +0.94 (p<0.0001), with a mean (SD) difference between Pn(sn) and PI(max) of -4.8 (21.2) cm H(2)O (the limits of agreement were 37.6 and -47.2 cm H(2)O). VC (% predicted) was positively correlated with Pn(sn)/PI(max) (r = +0.86; p<0.0001), with a lower Pn(sn)/PI(max) value in patients with a VC <40% of predicted than in those with a VC >40% (0.80 (0.35) v 1.04 (0.41); p<0.0001).Conclusions: PI(max) is greater than Pn(sn) in patients with a severe restrictive ventilatory defect caused by neuromuscular disease. Pn(sn) may not accurately reflect inspiratory muscle strength in such patients and it is thus advisable to use both tests. [ABSTRACT FROM AUTHOR]

La prise en charge secondaire des craniosténoses simples consiste surtout en des retouches esthétiques, et beaucoup plus rarement en des reprises fonctionnelles (cranioplasties ou insuffisances de correction). La prise en charge chirurgicale des faciocraniosténoses nécessite dans la plupart des cas de nombreuses interventions dont la séquence est variable selon la mutation. Habituellement, il est souhaitable de débuter la prise en charge par une expansion crânienne postérieure avant l'âge de 6 mois, le traitement d'une descente des amygdales cérébelleuses avant un an, et un monobloc fronto-facial à partir de l'âge de 18–24 mois. La prise en charge peut varier, mais il est préférable de retarder ou d'éviter la dérivation ventriculaire. Si le protocole initial ne suit pas ces règles, la prise en charge secondaire devient beaucoup plus complexe. La prise en charge primaire par avancement fronto-orbitaire compromet généralement le rattrapage par avancement fronto-facial monobloc, de même si un avancement facial précoce a été réalisé. Néanmoins, en chirurgie secondaire, on peut être amené à réaliser un monobloc de rattrapage, malgré les ostéotomies précédentes antérieures, mais avec un risque de morbidité accrue. Secondary surgeries for single craniosynostosis surgeries are mainly esthetic refinements rather than functional indications. However, cranioplasties for bone defects correction or insufficient corrections may be undertaken. Management of syndromic craniosynostoses usually requires multiple surgical interventions, the sequence of which might vary per the genetic mutation. It is commonplace to start with posterior vault expansion before age 6 months, then treat cerebellar tonsillar herniation by the age of twelve months, and delay fronto-facial monobloc advancement until at least 18–24 months of age. Ventricular shunting is preferably avoided or delayed. Failure to respect these guidelines can significantly complicate the subsequent management. Primary fronto-orbital advancement or early facial osteotomy type Le Fort3, may compromise the subsequent fronto-facial monobloc advancement. However, this salvage secondary monobloc may be undertaken in some instances despite previous anterior osteotomies with a higher morbidity. [ABSTRACT FROM AUTHOR]

Résumé La ventilation non invasive (VNI) permet de s’affranchir de certains effets indésirables de la ventilation invasive, de diminuer la durée d’hospitalisation et améliore la survie de certains patients. L’importance de l’utilisation de la VNI en pratique courante contraste avec le manque d’études prospectives ou d’études randomisées contrôlées pédiatriques dans ce domaine. L’objectif de cette revue est de recenser les principales indications de VNI de courte durée en réanimation pédiatrique : bronchiolite, détresse respiratoire après extubation, syndrome de détresse respiratoire aiguë, pneumopathie aiguë, asthme aigu grave, syndrome thoracique aigu, décompensation cardiaque gauche, décompensation d’une insuffisance respiratoire chronique, obstruction des voies aériennes supérieures et soins de support. La plupart de ces indications reposent sur des données descriptives et des opinions d’experts, peu étant issues d’essais randomisés. Alors que le bénéfice de la VNI est évident dans certaines indications, comme la bronchiolite, il est plus discutable dans d’autres. La surveillance des patients et de marqueurs d’échec est donc capitale. Summary Respiratory failure is the leading cause of hospital admissions in the pediatric intensive care unit (PICU) and is associated with significant morbidity and mortality. Mechanical ventilation, preferentially delivered by a non-invasive route (NIV), is currently the first-line treatment for respiratory failure since it is associated with a reduction in the intubation rate. This ventilatory support is increasingly used in the PICU, but its wider use contrasts with the paucity of studies in this field. This review aims to describe the main indications of NIV in acute settings: (i) bronchiolitis; (ii) postextubation respiratory failure; (iii) acute respiratory distress syndrome; (iv) pneumonia; (v) status asthmaticus; (vi) acute chest syndrome; (vii) left heart failure; (viii) exacerbation of chronic respiratory failure; (ix) upper airway obstruction and (x) end-of-life care. Most of these data are based on descriptive studies and expert opinions, and few are from randomized trials. While the benefit of NIV is significant in some indications, such as bronchiolitis, it is more questionable in others. Monitoring these patients for the occurrence of NIV failure markers is crucial. [ABSTRACT FROM AUTHOR]

Résumé La complexité de la prise en charge des faciocraniosténoses justifie sa prise en charge dans un centre de référence maladies rares (CRMR). Le trouble de la croissance crânienne et faciale étant variable selon la mutation du FGFr (Crouzon, Pfeiffer et Apert), la stratégie est adaptée au phénotype selon les principes suivants : expansion postérieure première sans ou avec distraction vers l’âge de 6 mois pour limiter la descente des amygdales cérébelleuses et pour prévenir la turricéphalie ; monobloc fronto-facial précoce avec distraction à partir de l’âge de 18 mois, surtout si l’exorbitisme ou le problème respiratoire sont importants. La stratégie dissociée avec expansion frontale précédent un avancement facial type Le Fort 3 reste une option possible, après le premier temps postérieur. L’évolution ultérieure dicte la séquence des interventions complémentaires selon un monitoring annuel de l’évaluation de la PIC par examen ophtalmologique et de la respiration par polysomnographie. Le Fort 3 et la disjonction maxillaire transversale peuvent être répétés. Une chirurgie orthognathique est quasi constamment nécessaire à partir de 14 ans, avant les interventions de raffinement esthétique (rhinoplastie, génioplastie, canthopexies, lipofilling…). Summary The complexity of treatment of faciocraniosynostosis justifies the treatment in a reference center for rare diseases. The growth disturbances in the skull and face being variable according to the type of mutation in the FGFr (Crouzon, Pfeiffer, Apert), the strategy is adapted to the phenotype according to the following principles: posterior expansion with or without distraction around 6 months to limit the descent of the cerebellum tonsils and to prevent the turricephalic development; fronto-facial monobloc advancement with internal distraction around the age of 18 months in case of severe exorbitism or breathing impairment. The dissociated strategy (fronto-orbital advancement first, followed by facial osteotomy of Le Fort 3 type). The growing evolution dictates the sequence of subsequent surgeries according to the monitoring of intracranial pressure by fundus examination and of the respiration by polysomnography. Le Fort 3 and transversal maxillary distraction may be repeated if necessary. Orthognathic surgery is almost always compulsory after the age of 14, before the aesthetic refinements which can be undertaken ultimately (rhinoplasty, genioplasty, canthopexies, fat grafting…). [ABSTRACT FROM AUTHOR]

Abstract: Collagen VI-related myopathies are hereditary disorders causing progressive restrictive respiratory insufficiency. Specific diaphragm involvement has been suggested by a drop in supine volumes. This pilot study aimed at characterizing the respiratory muscle phenotype in patients with COL6A1-3 genes mutations. Lung function, blood gases, muscle strength and respiratory mechanics were measured in 7 patients between 2002 and 2012. Patients were classified as Early-Severe (n =3), Moderate-Progressive (n =2) and Mild (n =2) according to clinical disease presentation. Seven patients (aged 6–28) were evaluated. Forced vital capacity distinguished the Mild group (>60% predicted) from the two other groups (<50% predicted). This distinction was also possible using the motor function measure scale. Diaphragmatic dysfunction at rest was observed in all the Early-Severe and Moderate-Progressive patients. During a voluntary sniff maneuver diaphragmatic dysfunction was observed in all patients, as assessed by a negative gastric pressure. All patients had diaphragmatic fatigue assessed by a tension-time index over the threshold of 0.15. Diaphragmatic dysfunction during a maximal voluntary maneuver and diaphragmatic fatigue are constant features in Collagen VI myopathies. These observations can assist the diagnosis and should be taken in account for the clinical management, with the early detection of sleep-disordered breathing. [Copyright &y& Elsevier]

Summary: Sleep-disordered breathing (SDB) in children comprises a wide spectrum of symptoms ranging from primary snoring to obstructive sleep apnea (OSA). Twelve percent of children present primary snoring and 1–2% OSA. Polysomnography is the gold standard for diagnosis of SDB allowing the analysis of sleep stages, respiratory movements, airflow, and gas exchange. However, this test remains highly technical, expensive, and difficult to conduct; other simpler diagnostic methods are under evaluation. Recent studies highlight the frequency and importance of cognitive and behavioral disorders in children with SDB; both the age and the severity of the SDB seem to modulate in the expression of neurocognitive consequences. Local and systemic inflammation plays a key role in the physiopathology of SDB and its complications: OSA is a cardiovascular risk factor in childhood that could favor atheromatous complications later in life. Adenoidotonsillectomy is the treatment of choice, but anti-inflammatory therapies such as leukotriene receptor antagonists or nasal corticoids may be beneficial in mild SDB or in residual OSA after adenotonsillectomy. In case of failure, noninvasive ventilation by means of nasal continuous positive pressure will be necessary, aided by specialists. SDB and OSA are a public health problem, underlining the pivotal role of the pediatrician in preventing, diagnosing, and treating these frequent disorders. [Copyright &y& Elsevier]

Summary: 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. [Copyright &y& Elsevier]

Abstract: Aims of the study: Respiratory-related evoked potentials (RREPs) are a method of recording brain activities in response to respiratory stimuli. Although data in childhood are scarce, the absence of the early P1 component of RREPs has been reported in children with a history of life-threatening asthma. This study was focused on the presence, latencies, and amplitudes of the P1, N1, P2, and N2 components of the RREPs in a paediatric series of asthmatic patients. Patients and methods: RREPs were recorded in 21 patients with stable asthma, age range 8–17years, 11 healthy children, age range 6–16 years, and 24 healthy adults, age range 20–28 years. The signals from left (C3–Cz) and right (C4–Cz) central (rolandic) location were recorded separately, using surface electrodes. Evoked responses to two series of 80 consecutive mid-inspiratory occlusions were averaged. Recordings were analysed manually. Results: All 4 RREPs components were significantly more often absent in asthmatic children than in healthy children and adults (P1, p =0.01; N1, p =0.008; P2, p =0.008, N2, p =0.01). The latencies and amplitudes of the four components were similar in patients and healthy subjects. Conclusion: RREPs components were less frequently present in children with asthma than in healthy subjects. This finding should promote the recording of RREPs in other acute and chronic respiratory diseases in children in order to search for possible electroclinical correlations. [Copyright &y& Elsevier]

Background: Macrolides display immunomodulatory effects that may be beneficial in chronic inflammatory pulmonary diseases. The aim of the study was to document whether long term use of azithromycin may be associated with respiratory benefits in young patients with cystic fibrosis. Methods: A multicentre, randomised, double blind, placebo controlled trial was conducted from October 2001 to June 2003. The criteria for enrolment were age older than 6 years and forced expiratory volume in 1 second (FEV1) of 40% or more. The active group received either 250 mg or 500 mg (body weight < or ~40 kg) of oral azithromycin three times a week for 12 months. The primary end point was change in FEV1. Results: Eighty two patients of mean (SD) age 11.0 (3.3) years and mean (SD) FEV1 85 (22)% predicted were randomised: 40 in the azithromycin group and 42 in the placebo group. Nineteen patients were infected with Pseudomonas aeruginosa. The relative change in FEV1 at month 12 did not differ significantly between the two groups. The number of pulmonary exacerbations (count ratio 0.50 (95% CI 0.32 to 0.79), p<0.005), the time elapsed before the first pulmonary exacerbation (hazard ratio 0.37 (95% CI 0.22 to 0.63), p<0.0001), and the number of additional courses of oral antibiotics were significantly reduced in the azithromycin group regardless of the infectious status (count ratio 0.55 (95% CI 0.36 to 0.85), p<0.01). No severe adverse events were reported. Conclusion: Long term use of low dose azithromycin in young patients with cystic fibrosis has a beneficial effect on lung disease expression, even before infection with Pseudomonas aeruginosa. [ABSTRACT FROM AUTHOR]

Abstract: Lower respiratory tract infections are a common cause of morbidity among children. The child''s age is an important help for the etiological diagnosis. Viruses are the most common cause of pneumonia in preschool children and Streptococcus pneumoniae is the most common bacterial pathogen at all ages. Mycoplasma pneumoniae is frequently isolated in children older than 2 years but can also infect preschool children. Pneumonia diagnosis is based on the patient''s history and physical examination combined to appropriate use of chest radiograph and laboratory tests as needed. Hospitalization and intravenous antibiotics are recommended in infants, patients with pre-existing lung disease, or when oral treatment is not efficient. In preschool children, antibiotic is not systematic and is recommended in case of severe pneumonia or suspicion of bacterial co-infection. At all ages, amoxicillin is the drug of choice when Streptococcus pneumoniae is suspected. Children older than 2 years old with atypical pneumonia must been given empiric treatment with macrolid. In preschool children, immunization with anti-haemophilus B vaccine has helped decrease the incidence of invasive Haemophilus influenzae type B infection, and the newly introduced heptavalent pneumococcal vaccine translates into a similar benefit for Streptococcus pneumoniae infection. [Copyright &y& Elsevier]

The aim of this work was to evaluate the effect of a more conservative use of chest-tube insertion on the short-term and long-term outcome of pleural infection. Sixty-five patients with pleural infection, aged 1 month to 16 years were each treated according to one of the two protocols: classical management with chest-tube insertion (classical group, n = 33), or conservative use of chest-tube insertion (conservative group, n = 32), with drainage indicated only in the case of voluminous pleural effusion defined by a mediastinal shift and respiratory distress and/or an uncontrolled septic situation. The two groups were comparable with regard to age, baseline C-reactive protein (CRP) value and white blood cell counts, pleural thickness, identified bacteria, and antibiotic treatment. Chest-tube insertion was performed in 17 patients (52%) of the classical group compared to eight patients (25%) of the conservative group (P = 0.03). Duration of temperature above 39 degrees C was shorter in the conservative group (10 +/- 1 vs. 14 +/- 1 days, P = 0.01), as was the normalization of CRP (13 +/- 1 vs. 17 +/- 1 days, P = 0.03). Duration of hospitalization and intravenous (IV) antibiotherapy as well as the delay of chest-radiograph normalization was not significantly different between the two groups. A more conservative use of chest-tube insertion did not change short- and long-term outcome of the pleural infection in children. Drainage could be restricted to the most severely affected patients with pleural empyema causing a mediastinal shift and respiratory distress and/or presenting with an uncontrolled septic situation. [ABSTRACT FROM AUTHOR]

Aims: To determine the frequency of and the risk factors for readmissions for any lower respiratory tract illness (LRTI) and for respiratory syncytial virus (RSV) documented LRTI in children born very prematurely who had or had not received RSV prophylaxis. Methods: Multicentre prospective longitudinal cohort study of 2813 infants, born between April 2000 and December 2000 at less than 33 weeks of gestational age, and followed until the end of the epidemic season. Results: Among the 2256 children who had no bronchopulmonary dysplasia at 36 weeks of postmenstrual age and were not submitted to RSV prophylaxis, 27.4% were readmitted at least once for any reason during the epidemic season; 15.1% and 7.2% were readmitted at least once for any LRII and RSV related LRTI, respectively. Children born at less than 31 weeks' gestation, having an intrauterine growth restriction, or living in a single mother family were at a significantly higher risk of readmission for LRTI in general as well as for RSV related LRTI. Of the 376 children submitted to prophylaxis, 28.2% were readmitted at least once for any LRTI and 6.1% for RSV related LRTI. Conclusion: One out of four children who had received no prophylaxis, was born very prematurely, and was without bronchopulmonary dysplasia at 36 weeks of postmenstrual age, was readmitted at least once for any reason. Roughly 50% and 20% of these readmissions were related to a LRTI and an RSV infection, respectively. Further epidemiological studies are warranted to assess the aetiology and impact of other respiratory pathogens on post-discharge readmission and respiratory morbidity in this population. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background: The physiological and symptomatic effects of proportional assist ventilation (PAV) and pressure support ventilation (PSV) were compared in stable awake patients with neuromuscular and chest wall deformity (NMCWD).Methods: Oxygen saturation (SaO(2)), transcutaneous carbon dioxide (TcCO(2)), minute ventilation (VE), tidal volume (VT), respiratory rate (RR), and diaphragm electromyography (EMGdi) were measured in 15 patients during both modes. Subjective effort of breathing and synchrony with the ventilator were assessed using visual analogue scales.Results: Three of 15 patients failed to trigger the ventilator in either mode and were excluded. In the 12 remaining patients there were similar improvements in SaO(2), TcCO(2), VE, VT, and RR during both modes. The mean (SD) percentage fall in EMGdi was greater during PSV (-80.5 (10.7)%) than during PAV (-41.3 (35.2)%; p= 0.01). Effort of breathing (p=0.004) and synchrony with the ventilator (p=0.004) were enhanced more with PSV than with PAV.Conclusion: Both PSV and PAV produced similar improvements in physiological parameters. However, greater diaphragm unloading was observed with PSV than with PAV, associated with greater symptomatic benefit. These findings suggest that tolerance to PAV may be compromised in patients with NMCWD. [ABSTRACT FROM AUTHOR]

Résumé L’obésité est avec l’hypertrophie des végétations et des amygdales un des facteurs de risque principaux du syndrome d’apnées obstructives du sommeil (SAOS) de l’enfant. Elle est associée à une fréquence et une sévérité accrue du SAOS, et constitue également un facteur prédictif de la persistance et de l’aggravation du SAOS dans le temps. Le SAOS de l’enfant est associé à une morbidité d’organe qui touche avant tout le développement neurocognitif et comportemental. Les conséquences cardiovasculaires et métaboliques sont moins importantes chez l’enfant que chez l’adulte. Des interactions réciproques entre le sommeil en général, le SAOS, l’obésité et des dysrégulations de l’homéostasie métabolique ont été mises en évidence, ce qui rend très difficile l’établissement du rôle exact de l’obésité dans la morbidité d’organe liée au SAOS. Aucune étude n’a jusqu’à présent réussi à démontrer que l’obésité était associée à une « sur-morbidité d’organe » dans le SAOS de l’enfant. Elle est toutefois associée à une efficacité moindre des traitements du SAOS et, en particulier, de l’adéno-amygdalectomie. Les complications per- et postopératoires sont plus fréquentes et plus sévères chez l’enfant obèse. Le recours à un traitement par pression positive continue (PPC) est donc plus fréquent chez l’enfant obèse mais la compliance de celui-ci à la PPC est moins bonne que chez l’enfant non obèse. En conclusion, l’obésité de l’enfant constituant un problème de santé publique à l’échelle mondiale, sa prévention constitue une des mesures les plus efficaces pour diminuer l’incidence et la morbidité associée au SAOS de l’enfant. Summary Obesity, along with hypertrophy of the adenoids and the tonsils, represents one of the major risk factors for obstructive sleep apnea (OSA) in children. Obesity is associated with an increase in the prevalence and the severity of OSA and is a major factor in the persistence and aggravation of OSA over time. Neurocognitive dysfunction and abnormal behavior are the most important and frequent end-organ morbidities associated with OSA in children. Other deleterious consequences such as cardiovascular stress and metabolic syndrome are less common in children than in adults with OSA. Defining the exact role of obesity in OSA-associated end-organ morbidity in children is difficult because of the complex and multidimensional interactions between sleep in general, OSA, obesity, and metabolic dysregulation. This may explain why obesity itself has not been shown to be associated with a significant increase in OSA-associated end-organ morbidity. Obesity is linked to a decreased treatment efficacy and, in particular, of adenotonsillectomy. Peri- and postoperative complications are more common and more severe in obese children as compared with normal-weight controls. Continuous positive airway pressure (CPAP) is frequently needed, but compliance with CPAP is less optimal in obese children than in non-obese children. In conclusion, obesity represents a major public health problem worldwide; its prevention is one of the most efficient tools for decreasing the incidence and the morbidity associated with OSA in children. [ABSTRACT FROM AUTHOR]

Interstitial lung disease (ILD) in infants and children comprises a large spectrum of rare respiratory disorders that are mostly chronic and associated with high morbidity and mortality. These disorders are characterized by inflammatory and fibrotic changes that affect alveolar walls. Typical features of ILD include dyspnea, diffuse infiltrates on chest radiographs, and abnormal pulmonary function tests with restrictive ventilatory defect and/or impaired gas exchange. Many pathological situations can impair gas exchange and, therefore, may contribute to progressive lung damage and ILD. Consequently, diagnosis approach needs to be structured with a clinical evaluation requiring a careful history paying attention to exposures and systemic diseases. Several classifications for ILD have been proposed but none is entirely satisfactory especially in children. The present article reviews current concepts of pathophysiological mechanisms, etiology and diagnostic approaches, as well as therapeutic strategies. The following diagnostic grouping is used to discuss the various causes of pediatric ILD: 1) exposure-related ILD; 2) systemic disease-associated ILD; 3) alveolar structure disorder-associated ILD; and 4) ILD specific to infancy. Therapeutic options include mainly anti-inflammatory, immunosuppressive, and/or anti-fibrotic drugs. The outcome is highly variable with a mortality rate around 15%. An overall favorable response to corticosteroid therapy is observed in around 50% of cases, often associated with sequelae such as limited exercise tolerance or the need for long-term oxygen therapy. [ABSTRACT FROM AUTHOR]

La maladie de Gorham se présente comme une ostéolyse au sein de laquelle se développe un tissu vasculaire et lymphatique anormal, touchant de préférence les enfants et les adultes jeunes. En 1955, L.W.Gorham en a défini les principales caractéristiques. Plus de 300 cas ont été déjà publiés, mais les critères diagnostics restent mal définis et la physiopathologie peu élucidée. Dans l’objectif de mieux la caractériser, nous avons étudié rétrospectivement les patients pris en charge pour une maladie de Gorham en France sur ces 20 dernières années. Nous en rapportons les différentes caractéristiques épidémiologiques,cliniques, histologiques et radiologiques. Méthodes: Les correspondants de la Société Française des cancers de l’enfant ont été contactés par mailing, ainsi que les auteurs de cas français rapportés dans la littérature. Une fois un cas identifié, les différents médecins référents du patient ont été recontactés et les dossiers des patients ont été colligés sur chaque site. Résultat: Nous avons identifié 17 cas en France. Aucun regroupement de cas familiaux n’est observé et la maladie apparaît sporadique. La moyenne d’âge à la découverte de la maladie est de 7 ans et demi [1–16 ans], avec un sex-ratio de 2,2. La découverte de la maladie se fait le plus souvent à l’occasion d’une complication : fracture osseuse ou tableau de détresse respiratoire en rapport avec des lymphangiectasies ou un chylothorax. 63 % des cas présentent une atteinte osseuse multifocale et 81 % au moins une localisation extra osseuse. Les complications pulmonaires et rachidiennes conditionnent le pronostic, et la mortalité globale est de 25 %. Après une durée de phase active de plusieurs années [1–13 ans], les lésions ne semblent plus évoluer. Mais, les atteintes osseuses constituées pendant la phase active laissent de lourdes séquelles avec l’absence de reconstruction osseuse secondaire complète. Les bisphosphonates, l’interféron et la radiothérapie ont été utilisés pour enrailler cette phase active, et pour certains cas une réponse favorable à court terme a été observée. Conclusion: La maladie de Gorham est une maladie extrêmement rare, le plus souvent multiviscérale et très sévère. L’analyse des données rétrospectives doit servir à proposer des recommandations pour sa prise en charge. [Copyright &y& Elsevier]