Overview

Respiratory

Over the last 15 years I have developed a research interest into non-CF Bronchiectasis. This focused primarily on primary ciliary dyskinesia. I developed a new method for the evaluation of function of the microscopic hairs (cilia) which line the airways of the lung. This has been standardized and now used as an accepted method for the diagnosis of primary ciliary dyskinesia and research platform.

Cystic Fibrosis

Cystic Fibrosis (CF) research is another significant research area. I am the PI on several clinical trials looking at new medications in CF patients.

I coordinate the CF clinic multidisciplinary research program and this is promoting research into nursing, physiotherapy, pharmacy and dietetic issues within CF. Current themes are transitional care to adulthood; physiotherapy interventions; location of CF care and outcome in adolescence and adherence to medication. We work closely with the adult CF clinic at St. Paul's on several joint projects.

Publications

High-quality read-based phasing of cystic fibrosis cohort informs genetic understanding of disease modification
Human Genetics and Genomics Advances
Mastromatteo, S. and Chen, A. and Gong, J. and Lin, F. and Thiruvahindrapuram, B. and Sung, W.W.L. and Whitney, J. and Wang, Z. and Patel, R.V. and Keenan, K. and Halevy, A. and Panjwani, N. and Avolio, J. and Wang, C. and C{\^o}t{\'e}-Maurais, G. and B{\'e}gin, S. and Adam, D. and Brochiero, E. and Bjornson, C. and Chilvers, M. and Price, A. and Parkins, M. and van Wylick, R. and Mateos-Corral, D. and Hughes, D. and Smith, M.J. and Morrison, N. and Tullis, E. and Stephenson, A.L. and Wilcox, P. and Quon, B.S. and Leung, W.M. and Solomon, M. and Sun, L. and Ratjen, F. and Strug, L.J.
DOI: 10.1016/j.xhgg.2022.100156
2023

A germline heterozygous dominant negative IKZF2 variant causing syndromic primary immune regulatory disorder and ICHAD
medRxiv
DOI: 10.1101/2023.09.09.23295301
2023

Genetic evidence supports the development of SLC26A9 targeting therapies for the treatment of lung disease
npj Genomic Medicine
Gong, J. and He, G. and Wang, C. and Bartlett, C. and Panjwani, N. and Mastromatteo, S. and Lin, F. and Keenan, K. and Avolio, J. and Halevy, A. and Shaw, M. and Esmaeili, M. and Côté-Maurais, G. and Adam, D. and Bégin, S. and Bjornson, C. and Chilvers, M. and Reisman, J. and Price, A. and Parkins, M. and van Wylick, R. and Berthiaume, Y. and Bilodeau, L. and Mateos-Corral, D. and Hughes, D. and Smith, M.J. and Morrison, N. and Brusky, J. and Tullis, E. and Stephenson, A.L. and Quon, B.S. and Wilcox, P. and Leung, W.M. and Solomon, M. and Sun, L. and Brochiero, E. and Moraes, T.J. and Gonska, T. and Ratjen, F. and Rommens, J.M. and Strug, L.J.
DOI: 10.1038/s41525-022-00299-9
2022

Long-term respiratory outcomes following solid organ transplantation in children: A retrospective cohort study
Pediatric Pulmonology
Wright, M.F.A. and Blydt-Hansen, T. and Chilvers, M.A.
DOI: 10.1002/ppul.25968
2022

A Phase 3, open-label, 96-week trial to study the safety, tolerability, and efficacy of tezacaftor/ivacaftor in children = 6 years of age homozygous for F508del or heterozygous for F508del and a residual function CFTR variant
Journal of Cystic Fibrosis
Sawicki, G.S. and Chilvers, M. and McNamara, J. and Naehrlich, L. and Saunders, C. and Sermet-Gaudelus, I. and Wainwright, C.E. and Ahluwalia, N. and Campbell, D. and Harris, R.S. and Paz-Diaz, H. and Shih, J.L. and Davies, J.C.
DOI: 10.1016/j.jcf.2022.02.003
2022

High Quality Phasing Using Linked-Read Whole Genome Sequencing of Patient Cohorts Informs Genetic Understanding of Complex Traits
bioRxiv
Mastromatteo, S. and Chen, A. and Gong, J. and Lin, F. and Thiruvahindrapuram, B. and Sung, W.W.L. and Whitney, J. and Wang, Z. and Patel, R.V. and Keenan, K. and Halevy, A. and Panjwani, N. and Avolio, J. and Wang, C. and Côté-Maurais, G. and Bégin, S. and Adam, D. and Brochiero, E. and Bjornson, C. and Chilvers, M. and Price, A. and Parkins, M. and van Wylick, R. and Mateos-Corral, D. and Hughes, D. and Smith, M.J. and Morrison, N. and Tullis, E. and Stephenson, A.L. and Wilcox, P. and Quon, B.S. and Leung, W.M. and Solomon, M. and Sun, L. and Ratjen, F. and Strug, L.J.
DOI: 10.1101/2022.03.28.486092
2022

Long-term safety and efficacy of lumacaftor-ivacaftor therapy in children aged 6-11 years with cystic fibrosis homozygous for the F508del-CFTR mutation: a phase 3, open-label, extension study.
The Lancet. Respiratory medicine
Chilvers MA and Davies JC and Milla C and Tian S and Han Z and Cornell AG and Owen CA and Ratjen F
DOI: 10.1016/s2213-2600(20)30517-8
PubMed: 33516285
01/2021

Cystic fibrosis-related diabetes onset can be predicted using biomarkers measured at birth.
Genetics in medicine : official journal of the American College of Medical Genetics
Lin YC and Keenan K and Gong J and Panjwani N and Avolio J and Lin F and Adam D and Barrett P and Bégin S and Berthiaume Y and Bilodeau L and Bjornson C and Brusky J and Burgess C and Strug LJ
DOI: 10.1038/s41436-020-01073-x
PubMed: 33500570
01/2021

Long-term safety and efficacy of lumacaftor–ivacaftor therapy in children aged 6–11 years with cystic fibrosis homozygous for the F508del-CFTR mutation: a phase 3, open-label, extension study
The Lancet Respiratory Medicine
Chilvers, M.A. and Davies, J.C. and Milla, C. and Tian, S. and Han, Z. and Cornell, A.G. and Owen, C.A. and Ratjen, F.
DOI: 10.1016/S2213-2600(20)30517-8
2021

Genetic evidence supports the development of SLC26A9 targeting therapies for the treatment of lung disease
medRxiv
Gong, J. and He, G. and Wang, C. and Bartlett, C. and Panjwani, N. and Mastromatteo, S. and Lin, F. and Keenan, K. and Avolio, J. and Halevy, A. and Shaw, M. and Esmaeili, M. and C{\^o}t{\'e}-Maurais, G. and Adam, D. and B{\'e}gin, S. and Bjornson, C. and Chilvers, M. and Reisman, J. and Price, A. and Parkins, M. and Van Wylick, R. and Berthiaume, Y. and Bilodeau, L. and Mateos-Corral, D. and Hughes, D. and Smith, M.J. and Morrison, N. and Brusky, J. and Tullis, E. and Stephenson, A.L. and Quon, B.S. and Wilcox, P. and Leung, W.M. and Solomon, M. and Sun, L. and Brochiero, E. and Moraes, T.J. and Gonska, T. and Ratjen, F. and Rommens, J.M. and Strug, L.J.
DOI: 10.1101/2021.10.07.21264392
2021

Outcomes of Cystic Fibrosis Screening–Positive Infants With Inconclusive Diagnosis at School Age
Pediatrics
Gonska, T. and Keenan, K. and Au, J. and Dupuis, A. and Chilvers, M.A. and Burgess, C. and Bjornson, C. and Fairservice, L. and Brusky, J. and Kherani, T. and Jober, A. and Kosteniuk, L. and Price, A. and Itterman, J. and Morgan, L. and Mateos-Corral, D. and Hughes, D. and Donnelly, C. and Smith, M.J. and Iqbal, S. and Arpin, J. and Reisman, J. and Hammel, J. and van Wylick, R. and Derynck, M. and Henderson, N. and Solomon, M. and Ratjen, F.
DOI: 10.1542/peds.2021-051740
2021

Role of transient elastography and apri in the assessment of pediatric cystic fibrosis liver disease
Canadian Liver Journal
Woolfson, J.P. and Schreiber, R.A. and Raveendran, S. and Chilvers, M. and Barker, C. and Guttman, O.R.
DOI: 10.3138/canlivj-2020-0008
2021

Factors influencing clinical trial participation for adult and pediatric patients with cystic fibrosis
Journal of Cystic Fibrosis
Lee, M. and Hu, X.Y. and Desai, S. and Kwong, E. and Fu, J. and Flores, E. and Lazosky, L. and Wilcox, P.G. and Mcllwaine, M. and Chilvers, M. and Yang, C. and Rayment, J.H. and Quon, B.S.
DOI: 10.1016/j.jcf.2020.08.019
2021

Correction to: Cystic fibrosis–related diabetes onset can be predicted using biomarkers measured at birth (Genetics in Medicine, (2021), 23, 5, (927-933), 10.1038/s41436-020-01073-x)
Genetics in Medicine
Lin, Y.-C. and Keenan, K. and Gong, J. and Panjwani, N. and Avolio, J. and Lin, F. and Adam, D. and Barrett, P. and Bégin, S. and Berthiaume, Y. and Bilodeau, L. and Bjornson, C. and Brusky, J. and Burgess, C. and Chilvers, M. and Consunji-Araneta, R. and Côté-Maurais, G. and Dale, A. and Donnelly, C. and Fairservice, L. and Griffin, K. and Henderson, N. and Hillaby, A. and Hughes, D. and Iqbal, S. and Itterman, J. and Jackson, M. and Karlsen, E. and Kosteniuk, L. and Lazosky, L. and Leung, W. and Levesque, V. and Maille, ?. and Mateos-Corral, D. and McMahon, V. and Merjaneh, M. and Morrison, N. and Parkins, M. and Pike, J. and Price, A. and Quon, B.S. and Reisman, J. and Smith, C. and Smith, M.J. and Vadeboncoeur, N. and Veniott, D. and Viczko, T. and Wilcox, P. and van Wylick, R. and Cutting, G. and Tullis, E. and Ratjen, F. and Rommens, J.M. and Sun, L. and Solomon, M. and Stephenson, A.L. and Brochiero, E. and Blackman, S. and Corvol, H. and Strug, L.J.
DOI: 10.1038/s41436-021-01281-z
2021

Long-term safety of lumacaftor–ivacaftor in children aged 2–5 years with cystic fibrosis homozygous for the F508del-CFTR mutation: a multicentre, phase 3, open-label, extension study
The Lancet Respiratory Medicine
Hoppe, J.E. and Chilvers, M. and Ratjen, F. and McNamara, J.J. and Owen, C.A. and Tian, S. and Zahigian, R. and Cornell, A.G. and McColley, S.A.
DOI: 10.1016/S2213-2600(21)00069-2
2021

Nasal Brushing Sampling and Processing using Digital High Speed Ciliary Videomicroscopy - Adaptation for the COVID-19 Pandemic.
Journal of visualized experiments : JoVE
Bricmont N and Benchimol L and Poirrier AL and Grignet C and Seaton C and Chilvers MA and Seghaye MC and Louis R and Lefebvre P and Kempeneers C
DOI: 10.3791/61949
PubMed: 33226018
11/2020

Matrix-assisted laser desorption/ionization time-of-flight MS for the accurate identification of Burkholderia cepacia complex and Burkholderia gladioli in the clinical microbiology laboratory
Journal of Medical Microbiology
Kendrew S. K. Wong and Suk Dhaliwal and Jennifer Bilawka and Jocelyn A. Srigley and Sylvie Champagne and Marc G. Romney and Peter Tilley and Manish Sadarangani and James E. A. Zlosnik and Mark A. Chilvers
DOI: 10.1099/jmm.0.001223
08/2020

Performance of a Three-Tier (IRT-DNA-IRT) Cystic Fibrosis Screening Algorithm in British Columbia
International Journal of Neonatal Screening
Graham Sinclair and Vanessa McMahon and Amy Schellenberg and Tanya N. Nelson and Mark Chilvers and Hilary Vallance
DOI: 10.3390/ijns6020046
06/2020

Epidemiology of burkholderia infections in people with cystic fibrosis in Canada between 2000 and 2017
Annals of the American Thoracic Society
Zlosnik, J.E.A. and Henry, D.A. and Hird, T.J. and Hickman, R. and Campbell, M. and Cabrera, A. and Chiavegatti, G.L. and Chilvers, M.A. and Sadarangani, M.
DOI: 10.1513/AnnalsATS.201906-443OC
2020

Immunoreactive trypsinogen levels in newborn screened infants with an inconclusive diagnosis of cystic fibrosis.
BMC pediatrics
Ooi CY and Sutherland R and Castellani C and Keenan K and Boland M and Reisman J and Bjornson C and Chilvers MA and van Wylick R and Kent S and Price A and Mateos-Corral D and Hughes D and Gonska T
DOI: 10.1186/s12887-019-1756-4
PubMed: 31640630
10/2019

Inhaled hypertonic saline in preschool children with cystic fibrosis (SHIP): a multicentre, randomised, double-blind, placebo-controlled trial
The Lancet Respiratory Medicine
DOI: 10.1016/s2213-2600(19)30187-0
09/2019

Ciliary functional analysis: Beating a path towards standardization.
Pediatric pulmonology
Kempeneers C and Seaton C and Garcia Espinosa B and Chilvers MA
DOI: 10.1002/ppul.24439
PubMed: 31313529
07/2019

An investigation into biomarkers for the diagnosis of ABPA and aspergillus disease in cystic fibrosis.
Pediatric pulmonology
Keown K and Abbott S and Kuzeljevic B and Rayment JH and Chilvers MA and Yang CL
DOI: 10.1002/ppul.24465
PubMed: 31359612
07/2019

A phase 3 study of tezacaftor in combination with ivacaftor in children aged 6 through 11¿years with cystic fibrosis.
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
Walker S and Flume P and McNamara J and Solomon M and Chilvers M and Chmiel J and Harris RS and Haseltine E and Stiles D and Li C and Ahluwalia N and Zhou H and Owen CA and Sawicki G and VX15-661-113 Investigator Group
DOI: 10.1016/j.jcf.2019.06.009
PubMed: 31253540
06/2019

An open-label extension study of ivacaftor in children with CF and a CFTR gating mutation initiating treatment at age 2-5¿years (KLIMB).
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
Rosenfeld M and Cunningham S and Harris WT and Lapey A and Regelmann WE and Sawicki GS and Southern KW and Chilvers M and Higgins M and Tian S and Cooke J and Davies JC and KLIMB study group
DOI: 10.1016/j.jcf.2019.03.009
PubMed: 31053538
04/2019

Inhaled hypertonic saline in preschool children with cystic fibrosis (SHIP): a multicentre, randomised, double-blind, placebo-controlled trial
The Lancet Respiratory Medicine
Ratjen, F. and Davis, S.D. and Stanojevic, S. and Kronmal, R.A. and Hinckley Stukovsky, K.D. and Jorgensen, N. and Rosenfeld, M. and Kerby, G. and Kopecky, C. and Anthony, M. and Mogayzel, P. and Walker, D. and Zeglin, B. and Hoover, W. and Hathorne, H. and Slaten, K. and Dorkin, H.H. and Fowler, R. and Fenton, C.N. and Ulles, M. and Goetz, D. and Caci, N. and Cahill, B. and Roach, C. and Retsch-Bogart, G. and Johnson, R. and Cunnion, R. and McColley, S. and Ward, S. and Bell, E. and McPhail, G. and Keller, K. and Thornton, K. and Parsons, A. and Chmiel, J. and Schaefer, C. and Tribout, M. and Consiglio, B. and Tribout, H. and McCoy, K. and Johnson, T. and Olson, P. and Raterman, L. and Hiatt, P. and Walker, B. and Schaap, N. and Davis, M. and Davis, S. and Clem, C. and Bendy, L. and Starner, T. and Lux, C. and Carver, T. and Thompson, R. and Williams, A. and Schmoll, C. and Hastings, P.M. and Noe, J. and Roth, L. and Kump, T. and McNamara, J. and Franck Thompson, E. and Yousef, S. and Wezel, G.G. and Oquendo, O. and Darling, A. and Valencia, W. and Milla, C. and Zirbes, J. and Rubenstein, R. and Donnelly, E. and Malpass, J. and Weiner, D. and Agostini, B. and Hartigan, E. and Cornell, A. and Klein, B. and Bucher, J. and Nusbaum, P. and McNamara, S. and Genatossio, A. and Pittman, J. and Hicks, T. and Bauer, I. and Siegel, M. and Isaac, S. and Jensen, R. and Au, J. and McDonald, N. and Prentice, C. and Chilvers, M. and Richmond, M.
DOI: 10.1016/S2213-2600(19)30187-0
2019

Epidemiology of Clonal Pseudomonas aeruginosa Infection in a Canadian Cystic Fibrosis Population.
Annals of the American Thoracic Society
Middleton MA and Layeghifard M and Klingel M and Stanojevic S and Yau YCW and Zlosnik JEA and Coriati A and Ratjen FA and Tullis ED and Stephenson A and Wilcox P and Freitag A and Waters VJ
DOI: 10.1513/annalsats.201801-007oc
PubMed: 29911888
07/2018

To beat, or not to beat, that is question! The spectrum of ciliopathies
Pediatric Pulmonology
Céline Kempeneers and Mark A. Chilvers
DOI: 10.1002/ppul.24078
06/2018

Diagnosis of Primary Ciliary Dyskinesia. An Official American Thoracic Society Clinical Practice Guideline.
American journal of respiratory and critical care medicine
Shapiro AJ and Davis SD and Polineni D and Manion M and Rosenfeld M and Dell SD and Chilvers MA and Ferkol TW and Zariwala MA and Sagel SD and Josephson M and Morgan L and Yilmaz O and American Thoracic Society Assembly on Pediatrics
DOI: 10.1164/rccm.201805-0819st
PubMed: 29905515
06/2018

Diagnosis of primary ciliary dyskinesia: An official American thoracic society clinical practice guideline
American Journal of Respiratory and Critical Care Medicine
Shapiro, A.J. and Davis, S.D. and Polineni, D. and Manion, M. and Rosenfeld, M. and Dell, S.D. and Chilvers, M.A. and Ferkol, T.W. and Zariwala, M.A. and Sagel, S.D. and Josephson, M. and Morgan, L. and Yilmaz, O. and Olivier, K.N. and Milla, C. and Pittman, J.E. and Leigh Anne Daniels, M. and Jones, M.H. and Janahi, I.A. and Ware, S.M. and Daniel, S.J. and Cooper, M.L. and Nogee, L.M. and Anton, B. and Eastvold, T. and Ehrne, L. and Guadagno, E. and Knowles, M.R. and Leigh, M.W. and Lavergne, V.
DOI: 10.1164/rccm.201805-0819ST
2018

Epidemiology of clonal pseudomonas aeruginosa infection in a canadian cystic fibrosis population
Annals of the American Thoracic Society
Middleton, M.A. and Layeghifard, M. and Klingel, M. and Stanojevic, S. and Yau, Y.C.W. and Zlosnik, J.E.A. and Coriati, A. and Ratjen, F.A. and Tullis, E.D. and Stephenson, A. and Wilcox, P. and Freitag, A. and Chilvers, M. and McKinney, M. and Lavoie, A. and Wang, P.W. and Guttman, D.S. and Waters, V.J.
DOI: 10.1513/AnnalsATS.201801-007OC
2018

Efficacy and safety of lumacaftor and ivacaftor in patients aged 6-11 years with cystic fibrosis homozygous for F508del-CFTR: a randomised, placebo-controlled phase 3 trial.
The Lancet. Respiratory medicine
Ratjen F and Hug C and Marigowda G and Tian S and Huang X and Stanojevic S and Milla CE and Robinson PD and Waltz D and Davies JC and VX14-809-109 investigator group
DOI: 10.1016/s2213-2600(17)30215-1
PubMed: 28606620
06/2017

Efficacy and safety of lumacaftor and ivacaftor in patients aged 6–11 years with cystic fibrosis homozygous for F508del-CFTR: a randomised, placebo-controlled phase 3 trial
The Lancet Respiratory Medicine
Ratjen, F. and Hug, C. and Marigowda, G. and Tian, S. and Huang, X. and Stanojevic, S. and Milla, C.E. and Robinson, P.D. and Waltz, D. and Davies, J.C. and Rosenfeld, M. and Starner, T. and Retsch-Bogart, G. and Chmiel, J. and Orenstein, D. and Milla, C. and Rubenstein, R. and Walker, S. and Cornell, A. and Asfour, F. and Black, P. and Colombo, J. and Froh, D. and McColley, S. and Ruiz, F. and Quintero, D. and Casey, A. and Mueller, G. and Flume, P. and Livingston, F. and Rock, M. and O'Sullivan, B. and Schmidt, H. and Lahiri, T. and McNamara, J. and Chidekel, A. and Sass, L. and Keens, T. and Schaeffer, D. and Solomon, M. and Chilvers, M. and Lands, L. and Junge, S. and Griese, M. and Staab, D. and Pressler, T. and van Koningsburggen-Rietschel, S. and Naehrlich, L. and Reid, A. and Balfour-Lynn, I. and Urquhart, D. and Lee, T. and Munck, A. and Gaudelus, I.S. and De Boeck, C. and Reix, P. and Malfroot, A. and Bui, S. and Selvadurai, H. and Robinson, P. and Wainwright, C. and Clements, B. and Hilton, J. and Hjelte, L.
DOI: 10.1016/S2213-2600(17)30215-1
2017

Adverse events following live-attenuated intranasal influenza vaccination of children with cystic fibrosis: Results from two influenza seasons
Vaccine
Boikos, C. and Joseph, L. and Scheifele, D. and Lands, L.C. and De Serres, G. and Papenburg, J. and Winters, N. and Chilvers, M. and Quach, C.
DOI: 10.1016/j.vaccine.2017.07.068
2017

Dornase alfa for cystic fibrosis
Paediatric Respiratory Reviews
Yang, C.L. and Chilvers, M. and Montgomery, M. and Nolan, S.J.
DOI: 10.1016/j.prrv.2016.09.001
2017

Response
Chest
Kempeneers, C. and Seaton, C. and Chilvers, M.A.
DOI: 10.1016/j.chest.2017.08.1177
2017

Variation of Ciliary Beat Pattern in Three Different Beating Planes in Healthy Subjects
Chest
Kempeneers, C. and Seaton, C. and Chilvers, M.A.
DOI: 10.1016/j.chest.2016.09.015
2017

Viral interference and the live-attenuated intranasal influenza vaccine: Results from a pediatric cohort with cystic fibrosis
Human Vaccines and Immunotherapeutics
Boikos, C. and Papenburg, J. and Martineau, C. and Joseph, L. and Scheifele, D. and Chilvers, M. and Lands, L.C. and De Serres, G. and Quach, C.
DOI: 10.1080/21645515.2017.1287641
2017

Safety, pharmacokinetics, and pharmacodynamics of ivacaftor in patients aged 2-5 years with cystic fibrosis and a CFTR gating mutation (KIWI): an open-label, single-arm study.
The Lancet. Respiratory medicine
Davies JC and Cunningham S and Harris WT and Lapey A and Regelmann WE and Sawicki GS and Southern KW and Robertson S and Green Y and Cooke J and Rosenfeld M and KIWI Study Group
DOI: 10.1016/s2213-2600(15)00545-7
PubMed: 26803277
01/2016

Safety, pharmacokinetics, and pharmacodynamics of ivacaftor in patients aged 2-years with cystic fibrosis and a CFTR gating mutation (KIWI): An open-label, single-arm study
The Lancet Respiratory Medicine
Davies, J.C. and Cunningham, S. and Harris, W.T. and Lapey, A. and Regelmann, W.E. and Sawicki, G.S. and Southern, K.W. and Robertson, S. and Green, Y. and Cooke, J. and Rosenfeld, M. and Bufi, J. and Sharon McNamara and Reeves, G. and Hathorne, H. and Brand, K. and Harris, W. and Spahr, J. and Hartigan, E. and Noren, B. and Grover, P. and Regelmann, W. and Hunter, E. and Walker, S. and Chatfield, B. and Vroom, J. and Williams, A. and Schmoll, C. and Black, P. and Telfer, R. and Colombo, J. and Bendy, L. and Montgomery, G. and Doolittle, C. and Symington, T. and Gile, C. and Millard, S. and Greenberg, J. and Correia, C. and Sawicki, G. and Ward, M. and Schmidt, H. and Accurso, F. and Faut, S. and Chilvers, M. and Maggie McIlwaine and Richmond, M. and Hayers, L. and Scott, S. and Davies, J. and Fall, A. and Miller, D. and Southern, K. and Doolan, K.
DOI: 10.1016/S2213-2600(15)00545-7
2016

Dornase alfa for cystic fibrosis
Cochrane Database of Systematic Reviews
Yang, C. and Chilvers, M. and Montgomery, M. and Nolan, S.J.
DOI: 10.1002/14651858.CD001127.pub3
2016

Influenza virus detection following administration of live-attenuated intranasal influenza vaccine in children with cystic fibrosis and their healthy siblings
Open Forum Infectious Diseases
Boikos, C. and Joseph, L. and Martineau, C. and Papenburg, J. and Scheifele, D. and Lands, L.C. and De Serres, G. and Chilvers, M. and Quach, C.
DOI: 10.1093/ofid/ofw187
2016

Inconclusive Diagnosis of Cystic Fibrosis After Newborn Screening
PEDIATRICS
DOI: 10.1542/peds.2014-2081
06/2015

Evaluation of a multidimensional cystic fibrosis transition program: A quality improvement initiative
Journal of Pediatric Nursing
Gravelle, A.M. and Paone, M. and Davidson, A.G.F. and Chilvers, M.A.
DOI: 10.1016/j.pedn.2014.06.011
2015

Surgical masks reduce airborne spread of Pseudomonas aeruginosa in colonized patients with cystic fibrosis
American Journal of Respiratory and Critical Care Medicine
Driessche, K.V. and Hens, N. and Tilley, P. and Quon, B.S. and Chilvers, M.A. and De Groot, R. and Cotton, M.F. and Marais, B.J. and Speert, D.P. and Zlosnik, J.E.A.
DOI: 10.1164/rccm.201503-0481LE
2015

Factors associated with response to treatment of pulmonary exacerbations in cystic fibrosis patients
Journal of Cystic Fibrosis
Waters, V.J. and Stanojevic, S. and Sonneveld, N. and Klingel, M. and Grasemann, H. and Yau, Y.C.W. and Tullis, E. and Wilcox, P. and Freitag, A. and Chilvers, M. and Ratjen, F.A.
DOI: 10.1016/j.jcf.2015.01.007
2015

Burkholderia species infections in patients with cystic fibrosis in British Columbia, Canada: 30 years' experience
Annals of the American Thoracic Society
Zlosnik, J.E.A. and Zhou, G. and Brant, R. and Henry, D.A. and Hird, T.J. and Mahenthiralingam, E. and Chilvers, M.A. and Wilcox, P. and Speert, D.P.
DOI: 10.1513/AnnalsATS.201408-395OC
2015

Randomized controlled trial of biofilm antimicrobial susceptibility testing in cystic fibrosis patients
Journal of Cystic Fibrosis
Yau, Y.C.W. and Ratjen, F. and Tullis, E. and Wilcox, P. and Freitag, A. and Chilvers, M. and Grasemann, H. and Zlosnik, J. and Speert, D. and Corey, M. and Stanojevic, S. and Matukas, L. and Leahy, T.R. and Shih, S. and Waters, V.
DOI: 10.1016/j.jcf.2014.09.013
2015

Casting a look at pediatric plastic bronchitis
International Journal of Pediatric Otorhinolaryngology
Jasinovic, T. and Kozak, F.K. and Moxham, J.P. and Chilvers, M. and Wensley, D. and Seear, M. and Campbell, A. and Ludemann, J.P.
DOI: 10.1016/j.ijporl.2015.07.011
2015

Use of the SNOT-22 and UPSIT to appropriately select pediatric patients with cystic fibrosis who should be referred to an otolaryngologist: Cross-sectional study
JAMA Otolaryngology - Head and Neck Surgery
Thamboo, A. and Dar Santos, R.C. and Naidoo, L. and Rahmanian, R. and Chilvers, M.A. and Chadha, N.K.
DOI: 10.1001/jamaoto.2014.1650
2014

In need of a patch UP: Recurrent congenital diaphragmatic hernia presenting with a large pleural effusion
Journal of Pediatric Surgery Case Reports
Shariff, F. and McDougall, C.M. and Chilvers, M.A. and Butterworth, S.A.
DOI: 10.1016/j.epsc.2014.09.008
2014

Embolization of a central venous catheter due to pinch-off syndrome
Acta Paediatrica, International Journal of Paediatrics
Gowraiah, V. and Culham, G. and Chilvers, M.A. and Yang, C.L.
DOI: 10.1111/apa.12085
2013

Efficacy and safety of ivacaftor in patients aged 6 to 11 years with cystic fibrosis with a G551D mutation
American Journal of Respiratory and Critical Care Medicine
Davies, J.C. and Wainwright, C.E. and Canny, G.J. and Chilvers, M.A. and Howenstine, M.S. and Munck, A. and Mainz, J.G. and Rodriguez, S. and Li, H. and Yen, K. and Ordoñez, C.L. and Ahrens, R.
DOI: 10.1164/rccm.201301-0153OC
2013

Superior herniation of the mediastinum presenting as an anterior neck mass on straining
Pediatric Pulmonology
McDougall, C.M. and Culham, G. and Seear, M.D. and Chilvers, M.A.
DOI: 10.1002/ppul.21618
2012

Inflammasome-mediated IL-1ß production in humans with cystic fibrosis
PLoS ONE
Tang, A. and Sharma, A. and Jen, R. and Hirschfeld, A.F. and Chilvers, M.A. and Lavoie, P.M. and Turvey, S.E.
DOI: 10.1371/journal.pone.0037689
2012

Cystic fibrosis adolescent transition care in Canada: A snapshot of current practice
Paediatrics and Child Health (Canada)
Gravelle, A. and Davidson, G. and Chilvers, M.
DOI: 10.1093/pch/17.10.553
2012

Atypical activation of the unfolded protein response in cystic fibrosis airway cells contributes to p38 MAPK-mediated innate immune responses
Journal of Immunology
Blohmke, C.J. and Mayer, M.L. and Tang, A.C. and Hirschfeld, A.F. and Fjell, C.D. and Sze, M.A. and Falsafi, R. and Wang, S. and Hsu, K. and Chilvers, M.A. and Hogg, J.C. and Hancock, R.E.W. and Turvey, S.E.
DOI: 10.4049/jimmunol.1103661
2012

Analysis of ependymal ciliary beat pattern and beat frequency using high speed imaging: Comparison with the photomultiplier and photodiode methods
Cilia
O'Callaghan, C. and Sikand, K. and Chilvers, M.A.
DOI: 10.1186/2046-2530-1-8
2012

Effects of a Pseudomonas aeruginosa eradication policy in a cystic fibrosis clinic
Current Opinion in Pulmonary Medicine
Davidson, A.G.F. and Chilvers, M.A. and Lillquist, Y.P.
DOI: 10.1097/MCP.0b013e328358f5a2
2012

Renal function in pediatric cystic fibrosis patients in the first decade of life
Pediatric Nephrology
Prestidge, C. and Chilvers, M.A. and Davidson, A.G.F. and Cho, E. and McMahon, V. and White, C.T.
DOI: 10.1007/s00467-010-1737-1
2011

Case 1: Chronic cough in a Vietnamese adolescent: Should we be sweating?
Paediatrics and Child Health
Luu, K. and Chilvers, M.
DOI: 10.1093/pch/16.8.465a
2011

TLR5 as an anti-inflammatory target and modifier gene in cystic fibrosis
Journal of Immunology
Blohmke, C.J. and Park, J. and Hirschfeld, A.F. and Victor, R.E. and Schneiderman, J. and Stefanowicz, D. and Chilvers, M.A. and Durie, P.R. and Corey, M. and Zielenski, J. and Dorfman, R. and Sandford, A.J. and Daley, D. and Turvey, S.E.
DOI: 10.4049/jimmunol.1001513
2010

Long-term comparative trial of two different physiotherapy techniques; postural drainage with percussion and autogenic drainage, in the treatment of cystic fibrosis
Pediatric Pulmonology
Mcilwaine, M. and Wong, L.T. and Chilvers, M. and Davidson, G.F.
DOI: 10.1002/ppul.21247
2010

Diagnostic testing of patients suspected of primary ciliary dyskinesia
American Journal of Respiratory and Critical Care Medicine
Stannard, W.A. and Chilvers, M.A. and Rutman, A.R. and Williams, C.D. and O'Callaghan, C.
DOI: 10.1164/rccm.200903-0459OC
2010

Acidification-dependent activation of CD1d-restricted natural killer T cells is intact in cystic fibrosis
Immunology
Rzemieniak, S.E. and Hirschfeld, A.F. and Victor, R.E. and Chilvers, M.A. and Zheng, D. and Van Den Elzen, P. and Turvey, S.E.
DOI: 10.1111/j.1365-2567.2009.03234.x
2010

Pleuropulmonary complications of PVL-positive Staphylococcus aureus infection in children
Acta Paediatrica, International Journal of Paediatrics
Thomas, B. and Pugalenthi, A. and Chilvers, M.
DOI: 10.1111/j.1651-2227.2009.01293.x
2009

Primary ciliary dyskinesia
Paediatrics and Child Health
Chilvers, M.A. and O'Callaghan, C.
DOI: 10.1016/j.paed.2007.02.003
2007

Diagnosing primary ciliary dyskinesia
Thorax
O'Callaghan, C. and Chilvers, M. and Hogg, C. and Bush, A. and Lucas, J.
DOI: 10.1136/thx.2007.083147
2007

Functional analysis of cilia and ciliated epithelial ultrastructure in healthy children and young adults
Thorax
Chilvers, M.A. and Rutman, A. and O'Callaghan, C.
DOI: 10.1136/thorax.58.4.333
2003

Ciliary beat pattern is associated with specific ultrastructural defects in primary ciliary dyskinesia
Journal of Allergy and Clinical Immunology
Chilvers, M.A. and Rutman, A. and O'Callaghan, C.
DOI: 10.1016/S0091-6749(03)01799-8
2003

The effects of coronavirus on human nasal ciliated respiratory epithelium
European Respiratory Journal
Chilvers, M.A. and McKean, M. and Rutman, A. and Myint, B.S. and Silverman, M. and O'Callaghan, C.
DOI: 10.1183/09031936.01.00093001
2001

Case 1: Assessment: chronic wet cough.
Paediatric respiratory reviews
Chilvers, M.A. and O'Callaghan, C.
2000

Local mucociliary defence mechanisms
Paediatric Respiratory Reviews
Chilvers, M.A. and O'Callaghan, C.
DOI: 10.1053/prrv.2000.0009
2000

Analysis of ciliary beat pattern and beat frequency using digital high speed imaging: Comparison with the photomultiplier and photodiode methods
Thorax
Chilvers, M.A. and O'Callaghan, C.
DOI: 10.1136/thorax.55.4.314
2000

Research

Current Projects
Evalaution of a transitional pathway in CF care
This is a rewtrospective analysis of an quality improvement project to inpmprove health care in CF youth.

Current Projects
Adherence to therapy in CF patients
Co PI:
Self funded.
Evalaution of adherance therapy in CF patients. ldentifying bariers to adherance wuithin BC. This is a joint project with St Pauls.

Current Projects
Establishment of Digitial high speed video in the diagnosis of primary ciliary dyskinesia in BC.
Principal investigator for this trial
Funded: Telethon

Grants

Telethon Award 2009

Research Group Members

Jessica Friesen
Adelia Jacobs, Registered Dietitian
Alam Lakhani, Research Manager
Nicole Lee Son, Physiotherapist
Nazifaa Vasaya, Research Coordinator
James Zlosnik, Research Associate - Chronic Bacterial Infections in Cystic Fibrosis. Primary Scientist and Manager - Canadian Burkholderia Cepacia Complex research Referral Repository. Project Manager - Centre for Understanding and Preventing Infection in Children. , Research Associate - Chronic Bacterial Infections in Cystic Fibrosis. Primary Scientist and Manager - Canadian Burkholderia Cepacia Complex research Referral Repository. Project Manager - Centre for Understanding and Preventing Infection in Children.