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We report the isolation of Staphylococcus aureus with decreased susceptibility to glycopeptides in five CF patients and review the clinical and microbiological features of these cases. Three patients presented with pulmonary exacerbation that may be attributed to these strains and two of them were successfully treated using linezolid therapy. Glycopeptide-intermediate S. aureus (GISA) strains isolated in two patients were susceptible to methicillin, while the three other patients harbored methicillin-resistant GISA. Rarely reported in CF, GISA may remain underestimated due to the difficulty of detection, and both clinicians and microbiologists should be aware of the GISA emergence in CF patients' population., (Copyright © 2011 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.)

NKX2-1 (NK2 homeobox 1) is a critical regulator of transcription for the surfactant protein (SP)-B and -C genes (SFTPB and SFTPC, respectively). We identified and functionally characterized two new de novo NKX2-1 mutations c.493C>T (p.R165W) and c.786_787del2 (p.L263fs) in infants with closely similar severe interstitial lung disease (ILD), hypotonia, and congenital hypothyroidism. Functional analyses using A549 and HeLa cells revealed that NKX2-1-p.L263fs induced neither SFTPB nor SFTPC promoter activation and had a dominant negative effect on wild-type (WT) NKX2-1. In contrast,NKX2-1-p.R165W activated SFTPC, to a significantly greater extent than did WTNKX2-1, while SFTPB activation was only significantly reduced in HeLa cells. In accordance with our in vitro data, we found decreased amounts of SP-B and SP-C by western blot in bronchoalveolar lavage fluid (patient with p.L263fs) and features of altered surfactant protein metabolism on lung histology (patient with NKX2-1-p.R165W). In conclusion, ILD in patients with NKX2-1 mutations was associated with altered surfactant protein metabolism, and both gain and loss of function of the mutated NKX2-1 genes on surfactant protein promoters were associated with ILD in "Brain-Lung-Thyroid syndrome"., ((c) 2009 Wiley-Liss, Inc.)

The present document is being produced on behalf of the French Society of the Physiology Task Force on standards for Infant Respiratory Function Testing whose aim is to provide guidelines for good laboratory practices according to the latest international recommendations. Application of such recommendations could be of particular value when attempting to develop standardized protocols in the scope of multi-centre trials. The first part resume these recommendations about apparatus, acquisition system and software for Infant Respiratory Function Testing. The second part focuses on physiological principles and practical considerations: calibration procedure, infant conditioning, tidal breathing measurements, and occlusion techniques for assessing passive respiratory mechanics, plethysmographic measurements of lung volume and airway resistance and forced expiratory flows measurements. The major problem when collecting lung function data is that of predicted values. Valid reference data, set up according to these recommendations, are, to date, still to be established. The last part of the document provides a review of the literature concerning infant respiratory function reference data and a resume of the most used of them. This document will clearly need to be updated regularly in response to advances in knowledge in this field.

Introduction: Neonatal screening for cystic fibrosis (CF) leads to early dedicated specialist care for all patients., Background: Pulmonary function tests (PFT) are mandatory for routine monitoring of CF patients. The aim of this article is to review the current guidelines for PFTs in CF, particularly the type of test, the age and the clinical status of the patient., Viewpoint: The regular use of spirometry is generally accepted. Many other tests are used but their clinical value in the routine follow-up of CF patients remains to be established., Conclusion: Further efforts should be made to evaluate the value of PFTs in CF, particularly in very young children.

Purpose: The indications for thoracoscopy remain imprecise in cases of pleural empyema. This study aimed to identify preoperative prognostic factors to help in the surgical decision., Methods: From 1996 to 2004, 50 children with parapneumonic pleural empyema underwent thoracoscopy either as the initial procedure (n = 26) or after failure of medical treatment (n = 24). Using multivariate analysis, we tested the prognostic value of clinical and bacteriological data, the ultrasonographic staging of empyema, and the delay before surgery. Outcome measures were technical difficulties, postoperative complications, time to apyrexia, duration of drainage, and length of hospitalization., Results: The clinical and bacterial data did not significantly predict the postoperative course. Echogenicity and the presence of pleural loculations at ultrasonography were not independent significant prognostic factors. A delay between diagnosis and surgery of more than 4 days was significantly correlated (P < .05) with more frequent surgical difficulties, longer operative time, more postoperative fever, longer drainage time, longer hospitalization, and more postoperative complications, such as bronchopleural fistula, empyema relapse, and persistent atelectasia., Conclusion: The main prognostic factor for thoracoscopic treatment of pleural empyema is the interval between diagnosis and surgery. A 4-day limit, corresponding to the natural process of empyema organization, is significant. The assessment of loculations by ultrasonography alone is not sufficient to predict the postoperative course.

Objective: To assess the effect of heliox, a helium-oxygen mixture, on respiratory distress symptoms in young infants., Design: Prospective, randomized, double-blind study., Setting: Pediatric ICU (PICU) of a university hospital., Patients: Twenty infants, all < 3 months old, admitted to the PICU with moderate-to-severe acute respiratory syncytial virus bronchiolitis., Interventions: All infants were randomly and blindly assigned to inhale either heliox or an air-oxygen mixture (airox) for 1 h under an oxyhood., Measurements and Results: After 1 h, the respiratory distress score was significantly lower in the heliox group compared with the airox group (3.05 vs 5.5, p < 0.01), with a significant reduction in accessory muscles use (p < 0.05) and expiratory wheezing (p < 0.01). In contrast, inspiratory breath sounds and cyanosis did not significantly differ between groups. The ex-premature infants of the heliox group had a higher respiratory distress score at baseline compared with the term infants of this group (5.8 vs 5.2, p < 0.05) and a comparable decrease in the score at 60 min., Conclusions: In young infants, even those born prematurely, heliox breathing induced a rapid reduction in accessory muscles use and expiratory wheezing. Further studies are needed to confirm the decreased respiratory muscle work of breathing during heliox inhalation in this population.

Patients with cystic fibrosis (CF) may be colonized with unusual gram-negative bacilli whose identification is difficult and clinical impact unclear. We describe the clinical and microbiological features of five colonizations with organisms belonging to the recently described genus Inquilinus in CF patients. Isolates were identified from Burkholderia cepacia selective medium by means of 16S rRNA analysis. All of them were resistant to colistin, penicillins, cephalosporins, and monobactams but exhibited a remarkable susceptibility to imipenem. One of the five patients was transiently colonized with a nonmucoid isolate, whereas the four other patients were persistently colonized over the period of follow-up (8 to 21 months) with mucoid isolates. Pulsed-field gel electrophoresis of SpeI-digested genomic DNA was powerful for strain genotyping and demonstrated the clonality of Inquilinus sp. colonization for the two patients tested. Clinical evolution after the onset of Inquilinus was heterogeneous, but for at least one patient the lung function worsened and eradication of Inquilinus sp. was unsuccessful despite several imipenem courses. Finally, Inquilinus spp. may represent a new threat for CF patients due to their mucoid characteristic, their multiresistant pattern to antibiotics, and their ability to persist in the respiratory tract.

Introduction: Fibreoptic bronchoscopy (FB) is an important diagnostic examination in paediatric pulmonology. In 2002 the Paediatric Pulmonology and Allergy Club undertook a retrospective study to establish the current status of fibreoptic bronchoscopy among its members., Methods: In 2001 sixty five paediatric pulmonologists carried out an average of 116 examinations (+/- 111) in 35 paediatric centres. FB was performed either in an operating theatre (15 centres), a dedicated bronchoscopy suite (6 centres) or an endoscopy suite shared with gastro-enterologists (7 centres). Other examinations were performed in areas dedicated to, or associated with intensive care. General anaesthesia was routinely used in 18 centres. The others used sedation including an equimolar mixture of oxygen and nitrous oxide in 14 centres. Ten centres performed less than 50 examinations, 12 between 51 and 100, 4 between 101 and 200 and 8 centres more than 200 in the year. Seventy two per cent of the children were less than 6 years old. The washing and disinfection procedures were manual in 20 centres and automatic in 15., Results: Three principal indications were reported: persistent wheezing, suspicion of a foreign body and ventilatory difficulties. Cough, desaturation and fever were the most frequently reported side effects., Conclusions: This is the first survey in paediatric pulmonology in France. It shows a wide variation in the practice of fibreoptic bronchoscopy in children.

An acute wheezing episode is the most common feature of severe lower respiratory tract infection during infancy. Respiratory syncytial virus (RSV) is the major causative agent. In order to study inflammation during acute wheezing episodes in infants, we wanted to assess the feasibility and contribution of induction of pharyngeal secretions. We therefore compared inflammatory markers in the pharyngeal secretions of 27 infants suffering from acute wheezing episodes with an RSV infection (RSV+) and 18 infants suffering with acute wheezing episodes without RSV infection (RSV-). Pharyngeal secretions were recovered by physiotherapy using isotonic saline. The safety of the procedure was carefully checked. Pharyngeal secretions were homogenized with dithiothreitol. Total cells and eosinophils were counted and levels of eosinophil cationic protein (ECP) and histamine were measured. Induction of pharyngeal secretion was always well tolerated. Eosinophils were present in five RSV+ and seven RSV- patients. ECP levels were not significantly different between the groups. Histamine levels after protein adjustment were significantly increased in RSV+ patients (p<0.01) in comparison to RSV- patients. In this study, we have shown, that pharyngeal secretion can be safely recovered from infants suffering from acute wheezing episodes, and that it can be analysed for enumeration of inflammatory cells and measurement of inflammatory mediators.

The aim of this study was to assess cardiac adaptation to muscular exercise in children operated for tetralogy of Fallot. Eight children with a history of tetralogy of Fallot were studied and compared with ten control children. The basal evaluation consisted of an electrocardiogram, spirometry in all cases. Chest X-ray and echocardiography in all operated children. A muscular exercise stress test with incremental load on a bicycle ergometer was carried out with measurement of the cardiac output by CO2 rebreathing (experimental method). Under resting conditions, the two groups were comparable with respect to anthropometrical parameters and respiratory function. The cardiovascular data confirmed the good postoperative results of the children with a history of tetralogy of Fallot; cardiothoracic index of 0.49 to 0.55; sinus rhythm on the electrocardiogram; right ventricular pressures within normal limits; residual instantaneous right ventricular-pulmonary artery pressure gradient less than 25 mmH. On exercise, there was no significant difference with respect to VO2max, maximal heart rate, maximal cardiac output and maximal ventilation. The relationship between cardiac output and oxygen consumption was linear in two groups: y = 8.17x + 1.95 in the control group, y = 8.57x + 2.82 in the operated children. The change in cardiac output on exercise was comparable in the two groups. These observations seemed to be related to the good postoperative haemodynamic result: absence of pulmonary sequellae and right ventricular dysfunction. Despite the normality of the results obtained in this series, exercise stress testing with analysis of cardiac and respiratory adaptation would seem to be necessary in the follow-up of children operated for tetralogy of Fallot to exclude a ventilatory or circulatory limitation.

A retrospective study carried out from January 1981 through August 1988 identified nine cases of materno-fetal infections (Staphylococcus aureus, 1 case; Klebsiella pneumoniae, 3 cases; enterococcus, 3 cases; Hemophilus influenzae, 1 case; and Pneumococcus, 1 case) in six girls and three boys; four premature infants developed respiratory distress requiring mechanical ventilation. In four cases, maternal history found risk factors for infection. All patients developed circulatory failure and one had purulent meningitis. Diagnosis was confirmed upon recovery of the microorganism from a central sample in six cases; in three cases, only the peripheral cultures were positive but diagnosis was supported by the positive maternal history and by clinical findings. Bacterial species were not associated with specific clinical or biological features, but infections due to Hemophilus influenzae, Klebsiella pneumoniae and pneumococcus were particularly severe (two deaths and two infants with neurologic sequelae). In some studies, unusual microorganisms account for 46% of septicemias and 36% of purulent meningitis. Group D streptococci are the most common bacteria in this category and may account for 10% to 15% of early neonatal septicemias. Organism-specific features are analyzed on the basis of data from the literature. Concerning therapy, this epidemiologic aspect of materno-fetal infections complicates the choice of the initial antimicrobial agents; because third-generation cephalosporins are not effective on listeria and group D streptococci, we advocate more widespread use of the ureidopenicillins instead of ampicillin.