Few articles have described the airway management of parenchymal lung hydatid cysts complicated by endobronchial or airway involvement, and none have described the use of flexible bronchoscopy (FB) therapeutically in children. We present a child with a ruptured pulmonary hydatid cyst who underwent repeated FB for removal of cystic material from an obstructed right upper lobe segmental bronchus. This child did not require thoracic surgery and made a full clinical recovery, demonstrating that in selected cases FB may offer curative treatment. Pediatr Pulmonol. 2012. 47:1140–1142. © 2012 Wiley Periodicals, Inc.

Objective: Our aim was to determine the safety of BAL in young children < 6 years with CF Methods: As part of a multi-center study of BAL-directed therapy, children with CF < 6 years had one or more BALs between September 1999 and December 2005. Adverse events were recorded intraoperatively and for 24 hr thereafter. Clinical characteristics before BAL, findings at bronchoscopy and BAL results were assessed as risk factors for adverse events. Results: 333 BALs were conducted in 107 (56 males) children, median age 23.5 (range 1.6-67.5) months, including 170 (51%) for pulmonary exacerbation. 29 BALs (8.7%) were followed by fever >= 38.5 degrees C and 10 (3%) had clinically significant episodes (five intraoperative hemoglobin desaturations to < 90% requiring intervention, one tachyarrhythmia, two needing post-operative supplemental oxygen, one hospitalization for stridor). Two contaminated bronchoscopes were detected. 180 minor adverse events were recorded in 174 (52%) BAL procedures (137 altered cough, 41 fever < 38.5 degrees C). Low percentage BAL return (P = 0.002) and focal bronchitis (P = 0.02) were associated with clinically significant deterioration. Multivariable analysis identified Streptococcus pneumoniae (OR 22.3; 95% confidence interval (CI); 6.9,72), Pseudomonas aeruginosa (OR 2.4; 95% CI 1.0, 5.8), respiratory signs (OR 5.0; 95% CI 1.7,14.6) and focal bronchitis (OR 5.9; 95% CI 1.2, 29.8) as independent risk factors for post-bronchoscopy fever > 38.5 degrees C. Conclusions: Adverse events are common with BAL in young CF children, but are usually transient and well tolerated. Parents should be counseled that signs of a pre-existing lower respiratory infection are associated with increased risk of post-BAL fever.

BACKGROUND Airway inflammation in cystic fibrosis (CF) is exaggerated and characterized by neutrophil-mediated tissue destruction, but its genesis and mechanisms remain poorly understood. To further define the pulmonary inflammatory response, we conducted a proteome-based screen of bronchoalveolar lavage fluid (BALF) collected from young children with and without CF experiencing endobronchial infection. METHODS We collected BALF samples from 45 children younger than 5 years and grouped them according to the presence of respiratory pathogens: > or = 1 x 10(5) colony-forming units (CFU)/mL BALF (18 and 12 samples with and without CF, respectively) and <1 x 10(5) CFU/mL (23 and 15 samples). BALF proteins were analyzed with SELDI-TOF mass spectrometry (MS) and H4 ProteinChips. Proteins were identified and characterized using trypsin digestion, tandem MS, Fourier transform ion cyclotron resonance MS, immunoblotting, and ELISA. RESULTS The SELDI-TOF MS BALF profiles contained 53 unique, reliably detected proteins. Peak intensities of 24 proteins differed significantly between the CF and non-CF samples. They included the neutrophil proteins, alpha-defensin 1 and 2, S100A8, S100A9, and S100A12, as well as novel forms of S100A8 and S100A12 with equivalent C-terminal deletions. Peak intensities of these neutrophil proteins and immunoreactive concentrations of selected examples were significantly higher in CF than non-CF samples. CONCLUSIONS Small neutrophil-derived BALF proteins, including novel C-terminal truncated forms of S100A proteins, are easily detected with SELDI-TOF MS. Concentrations of these molecules are abnormally high in early CF lung disease. The data provide new insights into CF lung disease and identify novel proteins strongly associated with CF airway inflammation.

Background: Serum vitamin A, normally depressed in inflammatory conditions, is frequently low in people with CF. Vitamin A is important in respiratory epithelial regeneration and repair. We hypothesised that serum vitamin A would be associated with inflammation and disease severity. Methods: Serum vitamin A (as retinol), C-reactive protein (CRP), vitamin E, 25-hydroxy vitamin D (25OHD), 1,25-dihydroxy vitamin D (1,25(OH)2D), weight, and lumbar spine bone mineral density (LSBMD) were measured in 138 subjects with CF (5–56 years) and 138 control subjects (5–48 years). FEV1, presence of CF liver disease (CFLD) and hospital admissions were recorded in those with CF. Results: Serum vitamin A level was lower in CF subjects than in controls (mean, 95% CI: 1.29, 1.0–1.37 vs. 1.80, 1.7–1.87 μmol/l, p<0.0001), and inversely correlated with CRP (rs=−0.37, p<0.0001). CF subjects with low vitamin A (45%) level had poorer FEV1, weight z-score, LSBMD z-score, and higher CRP compared with those with normal levels. In the CF group CRP, vitamin E, 1,25(OH)2D, presence of CFLD, admissions, and age were associated with vitamin A level. Conclusions: Serum vitamin A is negatively associated with CRP in subjects with CF, consistent with normal population studies. It is important to distinguish between low serum vitamin A associated with the inflammatory response and that due to poor nutritional stores. The role of vitamin A in CF warrants further study, in the contexts both of chronic recurrent inflammatory disease and acute pulmonary exacerbation.

Bioenergetics differ between males and females of many species. Human females apportion a substantial proportion of energy resources towards gynoid fat storage, to support the energetic burden of reproduction. Similarly, axial calcium accrual is favoured in females compared with males. Nutritional status is a prognostic indicator in cystic fibrosis (CF), but girls and young women are at greater risk of death despite equivalent nutritional status to males. The aim of this study was to compare fat (energy) and calcium stores (bone density) in males and females with CF over a spectrum of disease severity. Methods: Fat as % body weight (fat%) and lumbar spine (LS) and total body (TB) bone mineral density (BMD) were measured using dual absorption X-ray photometry in 127(59M) control and 101(54M) CF subjects, aged 9–25 years. An equation for predicted age at death had been determined using survival data and history of pulmonary function for the whole clinic, based on a trivariate normal model using maximum likelihood methods (1). For the CF group, a disease severity index (predicted age at death) was calculated from the derived equations according to each subjects history of pulmonary function, current age, and gender. Disease severity was classified according to percentile of predicted age at death (‘mild’ ≥75th, ‘moderate’ 25th–75th, ‘severe’ ≤25th percentile). Wt for age z-score was calculated. Serum testosterone and oestrogen were measured in males and females respectively. Fat% and LSBMD were compared between the groups using ANOVA. Results: There was an interaction between disease severity and gender: increasing disease severity was associated with greater deficits in TB (p=0.01), LSBMD (p<0.0001) and wt z-score (p=0.01) in males than females. These indices were preserved in females with severe disease compared with males (-0.36+/-0.9 vs -1.76+/-0.9, -0.88+/-1.1 vs - 3.0+/-0.9 and -0.31+/-0.91 vs -1.19+/-0.91 respectively). Fat% was higher in females as a group (p<0.0001), 25.3, 19.2, 20.9, 24.4 (SD 7.6)% for controls and those with mild, moderate and severe disease respectively, compared with 16.5, 13.6, 15.3, 15.9 (SD 7.6)% for corresponding groups in males. Testosterone was lower in males with CF (p=0.001 for comparison between the groups) but oestrogen similar to normal controls in females (p=0.75 for comparison between the groups). Conclusions: Girls and young women with CF had preserved BMD and wt z-score compared with males, and greater fat mass than males, independent of disease severity. We hypothesise that reproductive fitness favours diversion of energy resources in females to accrual of nutrient stores (fat and calcium), possibly at the expense of adaptation to the energy demands of CF disease. In contrast, males may be able to divert energy resources for support of cardiopulmonary and other functions, resulting in relatively improved prognosis. (1) Schluchter et al, Stat Med 2002;21:1271.

Adolescents and adults with CF have lower bone mineral density (BMD) than normal, but its relationship with phenotype is not well understood. Point FEV1% predicted (FEV) and rate of change of FEV are biased estimates of disease severity, because progressively older subjects represent a selected survivor population, with females at greater risk of death than males. To investigate the relationship between BMD and phenotype we used an index (predicted age at death) derived from Bayesian estimates of slope and intercept of FEV, age at last measurement and survival status. Predictive equations for the index were derived from 97 subjects (78 survivors) from the RCH CF clinic, and applied to a group of 102 comparable subjects who had BMD measured, classified as having‘mild’ ()75th), ‘moderate’ (25– 75th), or ‘severe’ (-25th centile) phenotype. Total body (TB) and lumbar spine (LS) BMD z-scores (Z) were compared, adjustingfor gender effects, using 2-way ANOVA. Annual mean change in FEV segregated, as expected, according to phenotype, ‘severe’ (ns25), ‘moderate’ (ns51) and ‘mild’ (ns25) y3.01(y3.73 to y2.30)%, y0.85(y1.36 to y0.35)%, 2.70(1.92 to 3.46)%, respectively, with no gender difference. LS and TB BMDZ were different in each phenotype (P-s 0.002), LS BMDZ for ‘severe’, ‘moderate’ and ‘mild’ y1.63(CI: y2.07 to y 1.19), y0.86(CI: y1.17 to y0.55), y0.06(CI: y0.54 to 0.41). Males had lower LS BMDZ than females overall (y1.22 (CI: y1.54 to y0.91) vs. y0.48(CI: y 0.84 to y0.12) Ps0.002). In the ‘severe’ group, males had lower TB BMDZ and LS BMDZ (PF0.002). Low BMD is associated with ‘moderate’ and ‘severe’ phenotypes, with relative preservation in females in the ‘severe’ group. Female biology (reproductive fitness) might promote resistance to bone resorption at a critical level of BMD loss.

Medical Journal of AustraliaVolume 167, Issue 4 p. 218-223 MJA Practice Essentials Respiratory Medicine 8. Asthma in children Claire Wainwright MB BS, FRACP, Claire Wainwright MB BS, FRACP Royal Children's Hospital Foundation Research Fellow Royal Children's Hospital, Brisbane, QLDSearch for more papers by this authorAlan F Isles MB BS(Hons), FRACP, Alan F Isles MB BS(Hons), FRACP Clinical Associate Professor and Senior Visiting Physician, Department of Respiratory Medicine Royal Children's Hospital, Brisbane, QLDSearch for more papers by this authorPaul W Francis MD, FRACP, Paul W Francis MD, FRACP Director, Department of Respiratory Medicine Royal Children's Hospital, Brisbane, QLDSearch for more papers by this author Claire Wainwright MB BS, FRACP, Claire Wainwright MB BS, FRACP Royal Children's Hospital Foundation Research Fellow Royal Children's Hospital, Brisbane, QLDSearch for more papers by this authorAlan F Isles MB BS(Hons), FRACP, Alan F Isles MB BS(Hons), FRACP Clinical Associate Professor and Senior Visiting Physician, Department of Respiratory Medicine Royal Children's Hospital, Brisbane, QLDSearch for more papers by this authorPaul W Francis MD, FRACP, Paul W Francis MD, FRACP Director, Department of Respiratory Medicine Royal Children's Hospital, Brisbane, QLDSearch for more papers by this author First published: 01 August 1997 https://doi.org/10.5694/j.1326-5377.1997.tb138856.xCitations: 17 Reprints will not be available from the authors. Correspondence: Associate Professor A F Isles, Department of Respiratory Medicine, Royal Children's Hospital, Herston, Brisbane, QLD 4029. AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat Abstract Children may wheeze or cough, but is it asthma and, if it is, are you overtreating, undertreating, or getting it right? Citing Literature Volume167, Issue4August 1997Pages 218-223 RelatedInformation

Journal of Paediatrics and Child HealthVolume 29, Issue 2 p. 101-103 Management of acute asthma R. L. HENRY, Corresponding Author R. L. HENRYAssoc. Prof. R. L. Henry, Department of Paediatrics, John Hunter Hospital, Locked Bag 1, Hunter Region Mail Centre, NSW 2310, Australia.Search for more papers by this authorC. F. ROBERTSON, C. F. ROBERTSONSearch for more papers by this authorI. ASHER, I. ASHERSearch for more papers by this authorD. M. COOPER, D. M. COOPERSearch for more papers by this authorP. COOPER, P. COOPERSearch for more papers by this authorK. P. DAWSON, K. P. DAWSONSearch for more papers by this authorP. FRANCIS, P. FRANCISSearch for more papers by this authorG. GEELHOED, G. GEELHOEDSearch for more papers by this authorJ. D. GILLIES, J. D. GILLIESSearch for more papers by this authorA. F. ISLES, A. F. ISLESSearch for more papers by this authorA. KEMP, A. KEMPSearch for more papers by this authorL. I. LANDAU, L. I. LANDAUSearch for more papers by this authorA. J. MARTIN, A. J. MARTINSearch for more papers by this authorB. MASTERS, B. MASTERSSearch for more papers by this authorC. M. MELLIS, C. M. MELLISSearch for more papers by this authorE. A. MITCHELL, E. A. MITCHELLSearch for more papers by this authorJ. MORTON, J. MORTONSearch for more papers by this authorA. OLINSKY, A. OLINSKYSearch for more papers by this authorP. D. PHELAN, P. D. PHELANSearch for more papers by this authorP. D. SLY, P. D. SLYSearch for more papers by this authorP. LE SOUEF, P. LE SOUEFSearch for more papers by this authorR. STAUGAS, R. STAUGASSearch for more papers by this authorP. P. VAN ASPEREN, P. P. VAN ASPERENSearch for more papers by this author R. L. HENRY, Corresponding Author R. L. HENRYAssoc. Prof. R. L. Henry, Department of Paediatrics, John Hunter Hospital, Locked Bag 1, Hunter Region Mail Centre, NSW 2310, Australia.Search for more papers by this authorC. F. ROBERTSON, C. F. ROBERTSONSearch for more papers by this authorI. ASHER, I. ASHERSearch for more papers by this authorD. M. COOPER, D. M. COOPERSearch for more papers by this authorP. COOPER, P. COOPERSearch for more papers by this authorK. P. DAWSON, K. P. DAWSONSearch for more papers by this authorP. FRANCIS, P. FRANCISSearch for more papers by this authorG. GEELHOED, G. GEELHOEDSearch for more papers by this authorJ. D. GILLIES, J. D. GILLIESSearch for more papers by this authorA. F. ISLES, A. F. ISLESSearch for more papers by this authorA. KEMP, A. KEMPSearch for more papers by this authorL. I. LANDAU, L. I. LANDAUSearch for more papers by this authorA. J. MARTIN, A. J. MARTINSearch for more papers by this authorB. MASTERS, B. MASTERSSearch for more papers by this authorC. M. MELLIS, C. M. MELLISSearch for more papers by this authorE. A. MITCHELL, E. A. MITCHELLSearch for more papers by this authorJ. MORTON, J. MORTONSearch for more papers by this authorA. OLINSKY, A. OLINSKYSearch for more papers by this authorP. D. PHELAN, P. D. PHELANSearch for more papers by this authorP. D. SLY, P. D. SLYSearch for more papers by this authorP. LE SOUEF, P. LE SOUEFSearch for more papers by this authorR. STAUGAS, R. STAUGASSearch for more papers by this authorP. P. VAN ASPEREN, P. P. VAN ASPERENSearch for more papers by this author First published: April 1993 https://doi.org/10.1111/j.1440-1754.1993.tb00459.xCitations: 21 Prepared by Australian and New Zealand respiratory paediatricians following a meeting in Perth, Western Australia in May 1991. AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat Citing Literature Volume29, Issue2April 1993Pages 101-103 RelatedInformation