Publications

2018

Newbegin K, Pilkington K, Shanthikumar S, Ranganathan S. Clinical utility of surveillance computed tomography scans in infants with cystic fibrosis. Pediatr Pulmonol. 2018 Jul 8.

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Clinical utility of surveillance computed tomography scans in infants with cystic fibrosis.   

• Martinovich KM, Shaw NC, Kicic A, Schultz A, Fletcher S, Wilton SD, et al. The potential of antisense oligonucleotide therapies for inherited childhood lung diseases. Mol Cell Pediatr. 2018 Feb 6;5(1):3.

Sutanto EN, Scaffidi A, Garratt LW, Looi K, Foo CJ, Tessari MA, et al. Assessment of p.Phe508del-CFTR functional restoration in pediatric primary cystic fibrosis airway epithelial cells. PLoS One. 2018 Jan 23;13(1):e0191618.

Muhlebach MS, Zorn BT, Esther CR, Hatch JE, Murray CP, Turkovic L, et al. Initial acquisition and succession of the cystic fibrosis lung microbiome is associated with disease progression in infants and preschool children. PLoS Pathog. 2018 Jan 18;14(1):e1006798.

Breuer O, Caudri D, Stick S, Schultz A, Akesson L. The clinical significance of oropharyngeal cultures in young children with cystic fibrosis. Eur Respir J. 2018 Apr 20.

Caudri D, Turkovic L, de Klerk N, Rosenow T, Hall G, Ranganathan S, et al. The association between Staphylococcus aureus and subsequent bronchiectasis in children with cystic fibrosis. J Cyst Fibros 2018 Jul; (4):462-469.

Foong R, Harper A, Skoric B, King L, Turkovic L, Ranganathan S, et al. The clinical utility of lung clearance index in early cystic fibrosis lung disease is not impacted by the number of multiple-breath washout trials. ERJ Open Res. 2018 Feb 16;4(1). pii: 00094-2017.

Montgomery S, Kicic A, Stick S, Garratt L, Turkovic L, Mall M, et al. The role of interleukin-1 in the pathology of early inflammation and structural lung disease in cystic fibrosis. Journal of Cystic Fibrosis – 28 February 2017.

Montgomery S, Kicic A, Stick S. Hypoxia and Inflammation in Cystic Fibrosis Airways: Identifying Pathway Mechanisms and Potential Therapeutic Targets. ERJ.

Rosenow T. Quantification of CT bronchiectasis and its relationship to ventilation in cystic fibrosis. Thorax. 2018 Jan;73(1):4.

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2017

The association between Staphylococcus aureus and subsequent bronchiectasis in children with cystic fibrosis

We aimed to study the effects of the bacteria Staphylococcus aureus on patients with cystic fibrosis (CF). Specifically, we wanted to find out if infections with this bacterium resulted in a more rapid progression of lung disease in young children with CF.

In healthy individuals, Staphylococcus aureus can be cultured from the nose and skin, but will not be found in the lungs. In children with CF however, Staphylococcus aureus is present in the lungs, with a prevalence of 1 in 5 patients. Globally, Staphylococcus aureus is treated differently, as it is unclear if the bacteria really causes damage. Our study will clarify the effects that Staphylococcus aureus has on the lungs and guide doctors on how aggressively they should treat these infections, in an effort to minimise lung damage as well as medication side effects.

In an Australian study children with CF underwent a bronchoscopy every year for the first 4 years of life. During the bronchoscopy some fluid was flushed through the lungs and cultured for Staphylococcus aureus. When the children were 5 to 7 years old we performed a CT-scan of the lungs and lung function tests to determine the severity of lung disease. We analysed if children that were infected with Staphylococcus aureus before the age of 4 had worse lung disease when they approached 5-7 years.

In our analyses, we found that children who were infected with Staphylococcus aureus for the first time at the age of 3 years, sustained more lung damage, visible on CT-scans and performed more poorly on lung function tests when they reached 5-7 years. We wanted to exclude that Staphylococcus aureus is a benign bacterium in children who are exhibiting more severe disease progression. We adjusted for disease severity when comparing participants, of children with and without Staphylococcus aureus. To note, the relationships between Staphylococcus aureus and more severe lung disease remained the same in those adjusted analyses.

Our results suggest that when a Staphylococcus aureus infection is present at 3 years of age, there is a significant association with faster progression of lung disease, regardless of previous disease severity. It implies that doctors should try to prevent and treat Staphylococcus aureus lung infections in the young CF population. We found the association only for first time infections at 3 years of age, so it should be considered that this may have just been a chance finding. Secondly, there is a risk that we were not able to fully account for all lung damage that occurred prior to the first Staphylococcus aureus infection.

Our study shows that Staphylococcus aureus lung infections are common in children with CF, furthermore, these infections are associated with more advanced lung damage. To prove that Staphylococcus aureus directly causes lung damage, a placebo-controlled randomised clinical trial should be performed using CT-scans of the lungs as outcome measure.

Reference: Caudri D, Turkovic L, Ng J, de Klerk NH, Rosenow T, Hall GL, Ranganathan SC, Sly PD, Stick SM; AREST CF.

Access the PDF version here:
The association between Staphylococcus aureus and subsequent bronchiectasis in children with cystic fibrosis

Effect of Posture on Lung Ventilation distribution and Associations with Structure in Children with Cystic Fibrosis

We investigated whether sitting or lying down changes how well air mixes in the lung during normal breathing using the multiple breath washout test in healthy children or children with cystic fibrosis. We also studied whether posture (sitting or lying down) impacts on the relationships between lung function (normally measured with the child seated) and lung structure (normally measured with the child lying down) in children with cystic fibrosis. Specifically, we studied whether lung function better reflects the structural lung damage on chest CT when both the tests are performed lying down.

The multiple breath washout test was performed in 25 healthy children and 21 children with cystic fibrosis both seated and lying down. Children with cystic fibrosis also underwent a chest CT scan (lying down).

We found that lying down increased the amount of uneven gas mixing in the lung, indicated by an increase in a measure called lung clearance index. We found a larger increase in the lung clearance index in children with cystic fibrosis compared with healthy children. In children with cystic fibrosis, the measure of lung function more closely corresponded to the measure of lung structure (the CT scan) when both tests were performed lying down.

Our data show that the multiple breath washout test gives different results depending on whether the child is sitting up or lying down during the test. When the child is lying down, gas in the lung mixes unevenly. This could be caused by smaller lung volume, narrowing and closure of airways, and trapped air in the lung. Our results suggest that performing the multiple breath washout test lying down may give a more accurate estimate of structural lung damage than performing the same test sitting up. Further work is needed to confirm these findings in a larger population of individuals, including individuals with more severe cystic fibrosis lung disease.

Reference: Ramsey KA, McGirr C, Stick SM, Hall GL, Simpson SJ; AREST CF. Effect of Posture on Lung Ventilation distribution and Associations with Structure in Children with Cystic Fibrosis. J Cyst Fibros. 2017.

 

Multiple Breath Washout Outcomes Are Sensitive to Inflammation and Infection in Children with Cystic Fibrosis

The multiple breath washout technique is a lung function test that tells us how evenly gas mixes in the lung. In diseased lungs, regions of the lungs can be either partially or completely closed resulting in less gas mixing in these regions and uneven gas mixing in the lung. The lung clearance index is a measure of how evenly gas mixes in the lung, with higher values indicating uneven mixing and worse lung function. 

We assessed whether the lung clearance index would be higher in preschool children with cystic fibrosis compared with healthy children. We also assessed whether the lung clearance index would be higher in children with cystic fibrosis who had detectable inflammation and infection in the lungs compared to those without.

We performed the multiple breath washout test in 82 visits with 58 children with cystic fibrosis and 38 visits with 31 healthy children aged three to six years. Children with cystic fibrosis also underwent bronchoalveolar lavage collection for detection of lower respiratory tract inflammation and infection.

The lung clearance index was higher in children with cystic fibrosis compared with healthy controls. In children with cystic fibrosis, the lung clearance index was higher in those with lower respiratory tract infections and inflammation detected in the lung.

Our data indicates that the lung clearance index is sensitive to the presence and extent of inflammation and respiratory pathogens in bronchoalveolar lavage fluid in preschool children with cystic fibrosis. The lung clearance index may be an important tool for monitoring clinical status and response to therapeutic interventions in preschool children with cystic fibrosis. 

Reference: Ramsey KA, Foong RE, Grdosic J, Harper A, Skoric B, Clem C, Davis M, Turkovic L, Stick SM, Davis S, Ranganathan SC, Hall GL; AREST CF. Multiple Breath Washout Outcomes Are Sensitive to Inflammation and Infection in Children with Cystic Fibrosis. Ann Am Thorac Soc. 2017.

 

Does 100% oxygen change the way children breathe?

The multiple breath washout lung function test relies on normal breathing of 100% oxygen to measure if gas exchange occurring in the lungs is normal. It is known that breathing 100% oxygen changes the way babies breath, but this does not occur in school-aged children and adults. We wanted to understand if there were changes in breathing pattern in preschool-aged children as this may impact the results of the lung function test.

We used data from children in AREST CF aged 3 to 6 years who performed the multiple breath washout lung function test and found very small changes in breathing pattern. These small changes are not likely to affect the results from the multiple breath washout lung function test.

Reference: Foong RE, Harper AJ, Hall GL, Ramsey KA; AREST CF. The Effect of 100% Oxygen on Tidal Breathing Parameters in Preschool Children. Eur Respir J. 2017.

The lack of the presence of Small colony variants of S.aureus in lower respiratory tract.

Lung infection with the bacterium Staphylococcus aureus (S.aureus) is a frequent problem for patients with Cystic fibrosis (CF) and is commonly treated with antibiotics. However, S.aureus may evolve and adapt to their environment in the lung by slowing down their growth and appearing smaller in size, aptly named “small-colony variants of Staphylococcus aureus” or SCVs of S.aureus.

Studies have found that SCVs of S.aureus are associated with worse lung disease in children with CF and have a higher resistance to antibiotics compared to the “normal” strain of S.aureus.

It was important to find out if these SCVs of S.aureus were also present in young children with CF in our clinics as they are not tested for in routine hospital laboratories and their presence is unknown.

We studied 277 lower airway samples collected from 124 patients with CF undergoing routine tests, during 2014-2015 at the RCH in Melbourne for microbiology analysis.

Sixty-one (22%) of these samples were infected with a “normal” strain of S. aureus, butno SCVs of S.aureuswere found.

We concluded that SCVs of S.aureus were not commonly found and does not appear to pose a problem in young children with CF in our centre

Reference: Carzino R, Hart E, Sutton P, King L, Ranganathan S; AREST CF. Lack of Small Colony Variants of Staphylococcus Aureus from Lower Respiratory Tract Specimens. Pediatr Pulmonol. 2017.

2017 cont. 

Rosenow T, Ramsey K, Turkovic L, Mok C, Murray CP, Hall G, Stick S; AREST CF. Air Trapping in Early Cystic Fibrosis Lung Disease - Does CT Tell a Full Story? Pediatr Pulmonol. 2017. 

Kuo W, Soffers T, Andrinopoulou ER, Rosenow T, Ranganathan S, Turkovic L, Stick SM, Tiddens H; AREST CF. Quantitative Assessment of Airway Dimensions in Young Children with Cystic Fibrosis Lung Disease using CT. Pediatr Pulmonol. 2017.

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2016

Garratt LW, Sutanto EN, Ling KM, Looi K, Isofidis T, Martinovich KM, Shaw NC, Buckley AG, Kicic-Starcevich E, Lannigan FJ, Knight DA, Stick SM, Kicic A; AREST CF. Alpha-1 Antitrypsin Mitigates the Inhibition of Airway Epithelial Cell Repair by Neutrophil Elastase. Am J Respir Cell Mol Biol. 2016. 

Ramsey KA, Rosenow T, Turkovic L, Skoric B, Banton G, Adams AM, Simpson SJ, Murray C, Ranganathan SC, Stick SM, Hall GL; AREST CF. Lung Clearance Index and Structural Lung Disease on Computed Tomography in Early Cystic Fibrosis. Am J Respir Crit Care Med. 2016.

Douglas T, Green J, Park J, Turkovic L, Massie J, Shields L. Psychosocial characteristics and predictors of health-care use in families of young children with cystic fibrosis in Western Australia. J Paediatr Child Health. 2016.

Jessup M, Douglas T, Priddis L, Branch-Smith C, Shields L; AREST CF. Parental Experience of Information and Education Processes Following Diagnosis of Their Infant With Cystic Fibrosis Via Newborn Screening. J Pediatr Nurs. 2016.

Ulluwishewa D, Wang L, Pereira C, Flynn S, Cain E, Stick S, Reen FJ, Ramsay JP, O'Gara F. Dissecting the regulation of bile-induced biofilm formation in Staphylococcus aureus. Microbiology. 2016. 

Sly PD, Wainwright CE. Diagnosis and Early Life Risk Factors for Bronchiectasis in Cystic Fibrosis: A Review. Expert Rev Respir Med. 2016.

Ramsey KA, Hart E, Turkovic L, Padros-Goossens M, Stick SM, Ranganathan SC. Respiratory Infection Rates Differ Between Geographically Distant Paediatric Cystic Fibrosis Cohorts. ERJ Open Res. 2016.

Esther CR Jr, Turkovic L, Rosenow T, Muhlebach MS, Boucher RC, Ranganathan S, Stick SM; AREST CF. Metabolomic Biomarkers Predictive of Early Structural Lung Disease in Cystic Fibrosis. Eur Respir J. 2016.

Ranganathan SC, Hall GL, Sly PD, Stick SM, Douglas TA; Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST-CF). Early Lung Disease in Infants and Preschool Children with Cystic Fibrosis. What Have We Learned and What Should We Do about It? Am J Respir Crit Care Med. 2017.

Foong RE, Rosenow T, Garratt LW, Hall GL. Early Lung Surveillance of Cystic Fibrosis: what have we learnt? Expert Rev Respir Med. 2017.

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2015

Simpson SJ, Ranganathan S, Park J, Turkovic L, Robins-Browne RM, Skoric B, et al. Progressive ventilation inhomogeneity in infants with cystic fibrosis after pulmonary infection. Eur Respir J. 2015 Dec;46(6):1680-90.

Garratt LW, Sutanto EN, Ling KM, Looi K, Iosifidis T, Martinovich KM, et al. Matrix metalloproteinase activation by free neutrophil elastase contributes to bronchiectasis progression in early cystic fibrosis. Eur Respir J. 2015 Aug;46(2):384-94.

Rosenow T, Oudraad MC, Murray CP, Turkovic L, Kuo W, de Bruijne M, et al. Reply: Excess Risk of Cancer from Computed Tomography Scan Is Small but Not So Low as to Be Incalculable. Am J Respir Crit Care Med. 2015 Dec 1;192(11):1397-9.

Ramsey KA, Schultz A, Stick SM. Biomarkers in Paediatric Cystic Fibrosis Lung Disease. Paediatr Respir Rev. 2015 Sep;16(4):213-8.

Ramsey KA, Ranganathan SC, Gangell CL, Turkovic L, Park J, Skoric B, et al. Impact of lung disease on respiratory impedance in young children with cystic fibrosis. Eur Respir J. 2015 Dec;46(6):1672-9.

Douglas T, Jordan B, Priddis L, Anderson V, Sheehan J, Kane RT, et al. Protocol for a study of the psychosocial determinants of health in early childhood among children with cystic fibrosis. J Adv Nurs. 2015 Jul;71(7):1704-16.

Schultz A, Stick S. Early pulmonary inflammation and lung damage in children with cystic fibrosis. Respirology. 2015;20(4):569-78.

Rosenow T, Oudraad MC, Murray CP, Turkovic L, Kuo W, de Bruijne M, et al. PRAGMA-CF: a Quantitative Structural Lung Disease CT Outcome in Young Children with Cystic Fibrosis. Am  J Respir Crit Care Med. 2015 May 15;191(10):1158-65.

Tiddens HA, Stick SM, Wild JM, Ciet P, Parker GJ, Koch A, et al. Respiratory tract exacerbations revisited: ventilation, inflammation, perfusion, and structure (VIPS) monitoring to redefine treatment. Pediatr Pulmonol. 2015 Oct;50 Suppl 40:S57-65.

Banton GL, Hall GL, Tan M, Skoric B, Ranganathan SC, Franklin PJ, et al. Multiple breath washout cannot be used for tidal breath parameter analysis in infants. Pediatr Pulmonol. 2016 May;51(5):531-40.

Salamon ER, Gain KR, Hall GL. Defining the appropriate waiting time between multiple-breath nitrogen washout measurements. Eur Respir J. 2015 May;45(5):1489-91.

Magon NJ, Turner R, Gearry RB, Hampton MB, Sly PD, Kettle AJ. Oxidation of calprotectin by hypochlorous acid prevents chelation of essential metal ions and allows bacterial growth: Relevance to infections in cystic fibrosis. Free Radic Biol Med. 2015 Sep;86:133-44.

Loeve M, Rosenow T, Gorbunova V, Hop WC, Tiddens HA, de Bruijne M. Reversibility of trapped air on chest computed tomography in cystic fibrosis patients. Eur J Radiol. 2015 Jun;84(6):1184-90.

Ledder O, Oliver MR, Heine RG, Graham J, Volders E, Robinson PJ. Clinical audit results in earlier nutritional intervention in malnourished children with cystic fibrosis with improved outcome. J Paediatr Child Health. 2015 Oct;51(10):988-93.

Irvin CG, Hall GL. An epilogue to lung function and lung disease: state-of-the-art 2015. Respirology. 2015 Oct;20(7):1008-9.

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2014

Ramsey KA, Ranganathan S, Park J, et al. Early respiratory infection is associated with reduced spirometry in children with cystic fibrosis. American journal of respiratory and critical care medicine 2014;190:1111-6.

Kettle AJ, Turner R, Gangell CL, et al. Oxidation contributes to low glutathione in the airways of children with cystic fibrosis. The European respiratory journal 2014;44:122-9.

Garratt LW, Sutanto EN, Foo CJ, et al. Determinants of culture success in an airway epithelium sampling program of young children with cystic fibrosis. Experimental lung research 2014;40:447-59.

Fantino E, Gangell CL, Hartl D, Sly PD, Arest CF. Airway, but not serum or urinary, levels of YKL-40 reflect inflammation in early cystic fibrosis lung disease. BMC pulmonary medicine 2014;14(6):28.

Gangell CL, Shackleton C, Poreddy S, Kappers J, Gaydon JE, Sloots TP, Stick SM, Ranganathan SC, Sly PD. Feasibility of parental collected nasal swabs for virus detection in young children with cystic fibrosis. Journal of Cystic Fibrosis 2014;13:661-66.

Taccetti G, Sly PD.  Early detection of infection with Pseudomonas aeruginosa in cystic fibrosis: The Holy Grail or an achievable goal? J Cyst Fibros. 2014;13:491-493.

Zemanick ET, Wagner BD, Robertson CE, et al. Assessment of Airway Microbiota and Inflammation in Cystic Fibrosis Using Multiple Sampling Methods. Annals of the American Thoracic Society 2015;12(2):221-29.

Welsh L, Robertson CF, Ranganathan SC. Increased rate of lung function decline in Australian adolescents with cystic fibrosis. Pediatric pulmonology 2014;49:873-7.

Welsh L, Nesci C, Tran H, Tomai M, Ranganathan S. Lung clearance index during hospital admission in school-age children with cystic fibrosis. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2014;13:687-91.

Tiddens HA, Stick SM, Davis S. Multi-modality monitoring of cystic fibrosis lung disease: the role of chest computed tomography. Paediatric respiratory reviews 2014;15:92-7.

Sheehan J, Hiscock H, Massie J, Jaffe A, Hay M. Caregiver coping, mental health and child problem behaviours in cystic fibrosis: a cross-sectional study. International journal of behavioral medicine 2014;21:211-20.

Ramsey KA, Ranganathan S. Interpretation of lung function in infants and young children with cystic fibrosis. Respirology 2014;19:792-9.

Massie J, Ioannou L, Delatycki M. Prenatal and preconception population carrier screening for cystic fibrosis in Australia: where are we up to? The Australian & New Zealand journal of obstetrics & gynaecology 2014;54:503-9.

Massie J, Gillam L. Uncertain diagnosis after newborn screening for cystic fibrosis: An ethics-based approach to a clinical dilemma. Pediatric pulmonology 2014;49:1-7.

Massie J, Castellani C, Grody WW. Carrier screening for cystic fibrosis in the new era of medications that restore CFTR function. Lancet 2014;383:923-5.

Ioannou L, McClaren BJ, Massie J, et al. Population-based carrier screening for cystic fibrosis: a systematic review of 23 years of research. Genetics in medicine: official journal of the American College of Medical Genetics 2014;16:207-16.

Ioannou L, Massie J, Lewis S, McClaren B, Collins V, Delatycki MB. 'No thanks'-reasons why pregnant women declined an offer of cystic fibrosis carrier screening. Journal of community genetics 2014;5:109-17.

Ioannou L, Massie J, Lewis S, Collins V, McClaren B, Delatycki MB. Attitudes and opinions of pregnant women who are not offered cystic fibrosis carrier screening. European journal of human genetics: EJHG 2014;22:859-65.

Gu Y, Garcia-Perez S, Massie J, van Gool K. Cost of care for cystic fibrosis: an investigation of cost determinants using national registry data. The European journal of health economics: HEPAC : health economics in prevention and care 2014.

Elkins MR, Robinson P, Anderson SD, Perry CP, Daviskas E, Charlton B. Inspiratory flows and volumes in subjects with cystic fibrosis using a new dry powder inhaler device. The open respiratory medicine journal 2014;8:1-7.

Delatycki MB, Burke J, Christie L, et al. Human genetics society of australasia position statement: population-based carrier screening for cystic fibrosis. Twin research and human genetics: the official journal of the International Society for Twin Studies 2014;17:578-83.

Cunningham F, Lewis S, Curnow L, Glazner J, Massie J. Respiratory physicians and clinic coordinators' attitudes to population-based cystic fibrosis carrier screening. Journal of cystic fibrosis: official journal of the European Cystic Fibrosis Society 2014;13:99-105.

Chmiel JF, Aksamit TR, Chotirmall SH, et al. Antibiotic management of lung infections in cystic fibrosis. II. Nontuberculous mycobacteria, anaerobic bacteria, and fungi. Annals of the American Thoracic Society 2014;11:1298-306.

Castellani C, Massie J. Newborn screening and carrier screening for cystic fibrosis: alternative or complementary? The European respiratory journal 2014;43:20-3.

Blau H, Linnane B, Carzino R, et al. Induced sputum compared to bronchoalveolar lavage in young, non-expectorating cystic fibrosis children. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2014;13:106-10.

Archibald AD, Massie J, Smith MJ, Dalton DG, du Sart D, Amor DJ. Population-based genetic carrier screening for cystic fibrosis in Victoria. The Medical journal of Australia 2014;200:205-6.

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2013

Wong JK, Ranganathan SC, Hart E, Australian Respiratory Early Surveillance Team for Cystic F. Staphylococcus aureus in early cystic fibrosis lung disease. Pediatric pulmonology 2013;48:1151-9.

Ranganathan SC, Skoric B, Ramsay KA, et al. Geographical differences in first acquisition of Pseudomonas aeruginosa in cystic fibrosis. Annals of the American Thoracic Society 2013;10:108-14.

Mott LS, Park J, Gangell CL, et al. Distribution of early structural lung changes due to cystic fibrosis detected with chest computed tomography. The Journal of pediatrics 2013;163:243-8 e1-3.

Mott LS, Graniel KG, Park J, et al. Assessment of early bronchiectasis in young children with cystic fibrosis is dependent on lung volume. Chest 2013;144:1193-8.

Mott LS, Park J, Murray CP, Gangell CL, de Klerk NH, Robinson PJ, Robertson CF, Ranganathan SC, Sly PD, Stick SM, AREST CF. (2013) Authors' response. Thorax 68:106

Simpson SJ, Mott LS, Esther CR, Jr., Stick SM, Hall GL. Novel end points for clinical trials in young children with cystic fibrosis. Expert review of respiratory medicine 2013;7:231-43.

Shields L, Munns A, Taylor M, Priddis L, Park J, Douglas T, AREST-CF. Scoping review of the literature about family-centered care with caregivers of children with cystic fibrosis. Neonatal, Paediatric and Child Health Nursing, 2013; 16(3): 21-5

Stick S, Tiddens H, Aurora P, Gustafsson P, Ranganathan S, Robinson P, Rosenfeld M, Sly P, Ratjen F. Early intervention studies in infants and preschool children with cystic fibrosis: are we ready? Eur Respir J. 2013;42:527-38.

Sly PD, Gangell CL, Chen L, Ware RS, Ranganathan S, Mott LS, Murrap CP, Stick SM, on behalf of AREST CF. Risk Factors for Bronchiectasis in Children with Cystic Fibrosis. N Engl J Med. 2013;368:1963-70.

Zemanick ET, Harris JK, Wagner BD, et al. Inflammation and airway microbiota during cystic fibrosis pulmonary exacerbations. PloS one 2013;8:e62917.

Wong R, Wong M, Robinson PD, Fitzgerald DA. Omalizumab in the management of steroid dependent allergic bronchopulmonary aspergillosis (ABPA) complicating cystic fibrosis. Paediatric respiratory reviews 2013;14:22-4.

Vandeleur M, Massie J, Oliver M. Gastrostomy in children with cystic fibrosis and portal hypertension. Journal of pediatric gastroenterology and nutrition 2013;57:245-7.

van Gool K, Norman R, Delatycki MB, Hall J, Massie J. Understanding the costs of care for cystic fibrosis: an analysis by age and health state. Value in health : the journal of the International Society for Pharmacoeconomics and Outcomes Research 2013;16:345-55.

Rosenfeld M, Allen J, Arets BH, et al. An official American Thoracic Society workshop report: optimal lung function tests for monitoring cystic fibrosis, bronchopulmonary dysplasia, and recurrent wheezing in children less than 6 years of age. Annals of the American Thoracic Society 2013;10:S1-S11.

McClaren BJ, Aitken M, Massie J, Amor D, Ukoumunne OC, Metcalfe SA. Cascade carrier testing after a child is diagnosed with cystic fibrosis through newborn screening: investigating why most relatives do not have testing. Genetics in medicine : official journal of the American College of Medical Genetics 2013;15:533-40.

Massie J, Delatycki MB. Cystic fibrosis carrier screening. Paediatric respiratory reviews 2013;14:270-5.

Loeve M, Krestin GP, Rosenfeld M, de Bruijne M, Stick SM, Tiddens HA. Chest computed tomography: a validated surrogate endpoint of cystic fibrosis lung disease? The European respiratory journal 2013;42:844-57.

Kidd TJ, Ramsay KA, Hu H, et al. Shared Pseudomonas aeruginosa genotypes are common in Australian cystic fibrosis centres. The European respiratory journal 2013;41:1091-100.

Beydon N, Robinson PD. Early intervention for newborns screened for cystic fibrosis. American journal of respiratory and critical care medicine 2013;188:409-10.

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2012

Garratt LW, Wright AK, Ranganathan SC, Grigg J, Sly PD, AREST CF. (2012) Small macrophages are present in early childhood respiratory disease. J Cyst Fibros 11:201-8

Mott LS, Park J, Murray CP, et al. Progression of early structural lung disease in young children with cystic fibrosis assessed using CT. Thorax 2012;67:509-16.

Martin B, Schechter MS, Jaffe A, Cooper P, Bell SC, Ranganathan S. Comparison of the US and Australian cystic fibrosis registries: the impact of newborn screening. Pediatrics 2012;129:e348-55.

Robinson PD, Latzin P, Verbanck S, Hall GL, Horsley A, Gappa M, Thamrin C, Arets HGM, Aurora P, Fuchs S, King GG, Lum S, Macleod K, Paiva M, Pillow J, Ranganathan S, Ratjen F, Singer F, Sonnappa S, Stocks J, Subbarao P, Thompson B and Gustafsson P. (2012) Consensus statement for inert gas washout measurement using multiple- and single-breath tests. Eur Respir J 41:507-522

Smith C, Winn A, Seddon P, Ranganathan S. A fat lot of good: balance and trends in fat intake in children with cystic fibrosis. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2012;11:154-7.

Sheehan J, Massie J, Hay M, et al. The natural history and predictors of persistent problem behaviours in cystic fibrosis: a multicentre, prospective study. Archives of disease in childhood 2012;97:625-31.

Robinson P, Schechter MS, Sly PD, et al. Clarithromycin therapy for patients with cystic fibrosis: a randomized controlled trial. Pediatric pulmonology 2012;47:551-7.

Norman R, van Gool K, Hall J, Delatycki M, Massie J. Cost-effectiveness of carrier screening for cystic fibrosis in Australia. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2012;11:281-7.

Loeve M, Hop WC, de Bruijne M, et al. Chest computed tomography scores are predictive of survival in patients with cystic fibrosis awaiting lung transplantation. American journal of respiratory and critical care medicine 2012;185:1096-103.

Griffiths AL, Wurzel DF, Robinson PJ, Carzino R, Massie J. Australian epidemic strain pseudomonas (AES-1) declines further in a cohort segregated cystic fibrosis clinic. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2012;11:49-52.

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2011

Sutanto EN, Kicic A, Foo CJ, et al. Innate inflammatory responses of pediatric cystic fibrosis airway epithelial cells: effects of nonviral and viral stimulation. American journal of respiratory cell and molecular biology 2011;44:761-7.

Stutz MD, Gangell CL, Berry LJ, et al. Cyanide in bronchoalveolar lavage is not diagnostic for Pseudomonas aeruginosa in children with cystic fibrosis. The European respiratory journal 2011;37:553-8.

Ranganathan SC, Parsons F, Gangell C, et al. Evolution of pulmonary inflammation and nutritional status in infants and young children with cystic fibrosis. Thorax 2011;66:408-13.

Pillarisetti N, Williamson E, Linnane B, et al. Infection, inflammation, and lung function decline in infants with cystic fibrosis. American journal of respiratory and critical care medicine 2011;184:75-81.

Hall GL, Logie KM, Parsons F, et al. Air trapping on chest CT is associated with worse ventilation distribution in infants with cystic fibrosis diagnosed following newborn screening. PloS one 2011;6:e23932.

Gangell C, Gard S, Douglas T, et al. Inflammatory responses to individual microorganisms in the lungs of children with cystic fibrosis. Clinical infectious diseases: an official publication of the Infectious Diseases Society of America 2011;53:425-32.

Gangell C, Hall GL, AREST CF. (2011) Early detection of lung function abnormalities in young children with Cystic Fibrosis. Annal Respir Med 2:22-27

Stick SM, Sly PD. Exciting new clinical trials in cystic fibrosis: infants need not apply. American journal of respiratory and critical care medicine 2011;183:1577-8.

Sly PD, Ware RS, de Klerk N, Stick SM. Randomised controlled trials in cystic fibrosis: what, when and how? The European respiratory journal 2011;37:991-3.

McClaren BJ, Metcalfe SA, Amor DJ, Aitken M, Massie J. A case for cystic fibrosis carrier testing in the general population. The Medical journal of Australia 2011;194:208-9.

Hall GL, Stocks J. Intervention trials and ventilation distribution in mild cystic fibrosis lung disease: will it all come out in the wash? The European respiratory journal 2011;37:757-9.

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2010

Thomson E, Brennan S, Senthilmohan R, et al. Identifying peroxidases and their oxidants in the early pathology of cystic fibrosis. Free radical biology & medicine 2010;49:1354-60.

Sturges NC, Wikstrom ME, Winfield KR, et al. Monocytes from children with clinically stable cystic fibrosis show enhanced expression of Toll-like receptor 4. Pediatric pulmonology 2010;45:883-9.

Pillarisetti N, Linnane B, Ranganathan S, Arest CF. Early bronchiectasis in cystic fibrosis detected by surveillance CT. Respirology 2010;15:1009-11.

Gangell CL, Hall GL, Stick SM, Sly PD, Arest CF. Lung function testing in preschool-aged children with cystic fibrosis in the clinical setting. Pediatric pulmonology 2010;45:419-33.

Douglas TA, Brennan S, Berry L, et al. Value of serology in predicting Pseudomonas aeruginosa infection in young children with cystic fibrosis. Thorax 2010;65:985-90.

Berry LJ, Shiel B, Garratt L, Sly PD, AREST CF. (2010) Stability of interleukin 8 and neutrophil elastase in bronchoalveolar lavage fluid following long-term storage. J Cyst Fibros 9:346-50

Garratt LW, Mistry V, Singh R, Kaur Sandhu J, Sheil B, Cooke MS, Sly PD, AREST CF. (2010) Interpretation of urinary 8-oxo-7,8-dihydro-2'-deoxyguanosine is adversely affected by methodological inaccuracies when using a commercial ELISA. Free Radic Biol Med 48:1460-4

Massie J, Curnow L, Gaffney L, Carlin J, Francis I. Declining prevalence of cystic fibrosis since the introduction of newborn screening. Archives of disease in childhood 2010;95:531-3.

Massie J. Persistent elevated tissue-transglutaminase in cystic fibrosis. Journal of paediatrics and child health 2010;46:210.

Ioannou L, Massie J, Collins V, McClaren B, Delatycki MB. Population-based genetic screening for cystic fibrosis: attitudes and outcomes. Public health genomics 2010;13:449-56.

Cugley K, Crawford N, Royle J, Elia S, Massie J. Immunisation rates of children with cystic fibrosis using the Australian Childhood Immunisation Register. Journal of paediatrics and child health 2010;46:768-71.

Castellani C, Massie J. Emerging issues in cystic fibrosis newborn screening. Current opinion in pulmonary medicine 2010;16:584-90.

Castellani C, Macek M, Jr., Cassiman JJ, et al. Benchmarks for cystic fibrosis carrier screening: a European consensus document. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2010;9:165-78.

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2009

Stick SM, Brennan S, Murray C, et al. Bronchiectasis in infants and preschool children diagnosed with cystic fibrosis after newborn screening. The Journal of pediatrics 2009;155:623-8 e1.

Sly PD, Brennan S, Gangell C, et al. Lung disease at diagnosis in infants with cystic fibrosis detected by newborn screening. American journal of respiratory and critical care medicine 2009;180:146-52.

Mott LS, Gangell CL, Murray CP, Stick SM, Sly PD, Arest CF. Bronchiectasis in an asymptomatic infant with cystic fibrosis diagnosed following newborn screening. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2009;8:285-7.

Brennan S, Sly PD, Gangell CL, et al. Alveolar macrophages and CC chemokines are increased in children with cystic fibrosis. The European respiratory journal 2009;34:655-61.

Harwood DT, Kettle AJ, Brennan S, Winterbourn CC. (2009) Simultaneous determination of reduced glutathione, glutathione disulphide and glutathione sulphonamide in cells and physiological fluids by isotope dilution liquid chromotagraphy-tandem mass spectrometry. J Chromatogr B Analyt Technol Biomed Life Sci 877:3393-9.

Douglas TA, Brennan S, Gard S, et al. Acquisition and eradication of P. aeruginosa in young children with cystic fibrosis. The European respiratory journal 2009;33:305-11.

Ward C, Massie J, Glazner J, et al. Problem behaviours and parenting in preschool children with cystic fibrosis. Archives of disease in childhood 2009;94:341-7.

Robinson PD, Cooper P, Van Asperen P, Fitzgerald D, Selvadurai H. Using index of ventilation to assess response to treatment for acute pulmonary exacerbation in children with cystic fibrosis. Pediatric pulmonology 2009;44:733-42.

Perera E, Massie J, Phillips RJ. Treatment of acne with oral isotretinoin in patients with cystic fibrosis. Archives of disease in childhood 2009;94:583-6.

O'Riordan SM, Robinson PD, Donaghue KC, Moran A. Management of cystic fibrosis-related diabetes in children and adolescents. Pediatric diabetes 2009;10 Suppl 12:43-50.

Neville LA, Ranganathan SC. Vitamin D in infants with cystic fibrosis diagnosed by newborn screening. Journal of paediatrics and child health 2009;45:36-41.

Massie J, Petrou V, Forbes R, et al. Population-based carrier screening for cystic fibrosis in Victoria: the first three years experience. The Australian & New Zealand journal of obstetrics & gynaecology 2009;49:484-9.

Massie J. Carrier screening for cystic fibrosis. Lancet 2009;374:978; author reply.

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2008

Linnane BM, Hall GL, Nolan G, et al. Lung function in infants with cystic fibrosis diagnosed by newborn screening. American journal of respiratory and critical care medicine 2008;178:1238-44.

Stick SM. (2008) Clinical trials in infants with cystic fibrosis detected following newborn screening. Paediatr Respir Rev 9:176-80.

Sly PD. (2008) Early detection of lung disease in CF: do we have the necessary techniques? Paediatr Respir Rev 9:149-50.

Ranganathan S, Linnane B, Nolan G, Gangell C, Hall G. Early detection of lung disease in children with cystic fibrosis using lung function. Paediatric respiratory reviews 2008;9:160-7.

Linnane B, Robinson P, Ranganathan S, Stick S, Murray C. Role of high-resolution computed tomography in the detection of early cystic fibrosis lung disease. Paediatric respiratory reviews 2008;9:168-74; quiz 74-5.

Brennan S, Gangell C, Wainwright C, Sly PD. (2008) Disease surveillance using bronchoalveolar lavage. Paediatr Respir Rev 9:151-9.

Radhakrishnan M, van Gool K, Hall J, Delatycki M, Massie J. Economic evaluation of cystic fibrosis screening: a review of the literature. Health policy 2008;85:133-47.

Mishra A, Greaves R, Smith K, et al. Diagnosis of cystic fibrosis by sweat testing: age-specific reference intervals. The Journal of pediatrics 2008;153:758-63.

Hafen GM, Hurst C, Yearwood J, Smith J, Dzalilov Z, Robinson PJ. A new scoring system in Cystic Fibrosis: statistical tools for database analysis - a preliminary report. BMC medical informatics and decision making 2008;8:44.

Farrell PM, Rosenstein BJ, White TB, et al. Guidelines for diagnosis of cystic fibrosis in newborns through older adults: Cystic Fibrosis Foundation consensus report. The Journal of pediatrics 2008;153:S4-S14.

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2007

Thamrin C, Gangell CL, Udomittipong K, Kusel MMH, Patterson H, Fukushima T, Schultz A, Hall GL, Stick SM, Sly PD. (2007) Assessment of bronchodilator responsiveness in preschool children using forced oscillations. Thorax 62:814-9.

Gangell CL, Horak F, Jr., Patterson HJ, Sly PD, Stick SM, Hall GL. Respiratory impedance in children with cystic fibrosis using forced oscillations in clinic. The European respiratory journal 2007;30:892-7.

Mishra A, Greaves R, Massie J. The limitations of sweat electrolyte reference intervals for the diagnosis of cystic fibrosis: a systematic review. The Clinical biochemist Reviews / Australian Association of Clinical Biochemists 2007;28:60-76.

Massie J, Forbes R, Dusart D, Bankier A, Delatycki MB. Community-wide screening for cystic fibrosis carriers could replace newborn screening for the diagnosis of cystic fibrosis. Journal of paediatrics and child health 2007;43:721-3.

Horak F, Jr., Moeller A, Singer F, et al. Longitudinal monitoring of pediatric cystic fibrosis lung disease using nitrite in exhaled breath condensate. Pediatric pulmonology 2007;42:1198-206.

Comeau AM, Accurso FJ, White TB, et al. Guidelines for implementation of cystic fibrosis newborn screening programs: Cystic Fibrosis Foundation workshop report. Pediatrics 2007;119:e495-518.

Cipolli M, Castellani C, Wilcken B, et al. Pancreatic phenotype in infants with cystic fibrosis identified by mutation screening. Archives of disease in childhood 2007;92:842-6.

Bell SC, Robinson PJ. Exacerbations in cystic fibrosis: 2 . prevention. Thorax 2007;62:723-32.

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2006

Winfield KR, Gard S, Kent GN, Sly PD, Brennan S. (2006) Assay for urinary desmosines in a healthy pre-pubertal population using an improved extraction technique. Ann Clin Biochem 43:146-52.

Moeller A, Horak F, Jr., Lane C, et al. Inducible NO synthase expression is low in airway epithelium from young children with cystic fibrosis. Thorax 2006;61:514-20.

Franklin PJ, Hall GL, Moeller A, Horak F, Jr., Brennan S, Stick SM. Exhaled nitric oxide is not reduced in infants with cystic fibrosis. The European respiratory journal 2006;27:350-3.

Sawyer SM, Cerritelli B, Carter LS, Cooke M, Glazner JA, Massie J. Changing their minds with time: a comparison of hypothetical and actual reproductive behaviors in parents of children with cystic fibrosis. Pediatrics 2006;118:e649-56.

Massie J, Curnow L, Tzanakos N, Francis I, Robertson CF. Markedly elevated neonatal immunoreactive trypsinogen levels in the absence of cystic fibrosis gene mutations is not an indication for further testing. Archives of disease in childhood 2006;91:222-5.

Massie J, Cranswick N. Pharmacokinetic profile of once daily intravenous tobramycin in children with cystic fibrosis. Journal of paediatrics and child health 2006;42:601-5.

Massie J. Sweat testing for cystic fibrosis: How good is your laboratory? Journal of paediatrics and child health 2006;42:153-4.

Lewis S, Curnow L, Ross M, Massie J. Parental attitudes to the identification of their infants as carriers of cystic fibrosis by newborn screening. Journal of paediatrics and child health 2006;42:533-7.

Hafen GM, Ranganathan SC, Robertson CF, Robinson PJ. Clinical scoring systems in cystic fibrosis. Pediatric pulmonology 2006;41:602-17.

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2005

Pitrez PM, Brennan S, Turner S, Sly PD. Nasal wash as an alternative to bronchoalveolar lavage in detecting early pulmonary inflammation in children with cystic fibrosis. Respirology 2005;10:177-82.

Martin AC, Laing IA, Zhang G, et al. CD14 C-159T and early infection with Pseudomonas aeruginosa in children with cystic fibrosis. Respiratory research 2005;6:63.

Brennan S, Hall GL, Horak F, et al. Correlation of forced oscillation technique in preschool children with cystic fibrosis with pulmonary inflammation. Thorax 2005;60:159-63.

Ranganathan SC, Robertson CF. Ventilation inhomogeneities in patients with cystic fibrosis: inappropriate reference data and errors? American journal of respiratory and critical care medicine 2005;172:645; author reply -6.

Mishra A, Greaves R, Massie J. The relevance of sweat testing for the diagnosis of cystic fibrosis in the genomic era. The Clinical biochemist Reviews / Australian Association of Clinical Biochemists 2005;26:135-53.

Massie J, Clements B, Australian Paediatric Respiratory G. Diagnosis of cystic fibrosis after newborn screening: the Australasian experience--twenty years and five million babies later: a consensus statement from the Australasian Paediatric Respiratory Group. Pediatric pulmonology 2005;39:440-6.

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1996-2004

Sly PD, Brennan S. Detecting early lung disease in cystic fibrosis: are current techniques sufficient? Thorax 2004;59:1008-10.

Stapleton D, Kerr D, Gurrin L, Sherriff J, Sly P. Height and weight fail to detect early signs of malnutrition in children with cystic fibrosis. Journal of pediatric gastroenterology and nutrition 2001;33:319-25.

Brennan S, Cooper D, Sly PD. Directed neutrophil migration to IL-8 is increased in cystic fibrosis: a study of the effect of erythromycin. Thorax 2001;56:62-4.

Stapleton DR, Gurrin LC, Zubrick SR, Silburn SR, Sherriff JL, Sly PD. What do children with cystic fibrosis and their parents know about nutrition and pancreatic enzymes? Journal of the American Dietetic Association 2000;100:1494-500.

Everard ML, Sly P, Brenan S, Ryan G. Macrolide antibiotics in diffuse panbronchiolitis and in cystic fibrosis. The European respiratory journal 1997;10:2926.