Search results for: Kevin W. Southern

Items from 1 to 20 out of 29 results

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Aerosolized agents for airway clearance in cystic fibrosis

Kevin W. Southern, John P. Clancy, Sarath Ranganathan

Pediatric Pulmonology > 54 > 6 > 858 - 864

The outlook for people with cystic fibrosis (CF) has improved considerably as a result of conventional therapies including aerosolized agents for airway clearance. These will continue to play a significant role in maintaining well‐being and improving survival, even as newer agents emerge that correct the underlying CF defect. In this review, we explore the evidence supporting the use of dornase alfa,...

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A formative study exploring perceptions of physical activity and physical activity monitoring among children and young people with cystic fibrosis and health care professionals

James Shelley, Stuart J Fairclough, Zoe R Knowles, Kevin W Southern, more

BMC Pediatrics > 2018 > 18 > 1 > 1-16

Background Physical activity (PA) is associated with reduced hospitalisations and maintenance of lung function in patients with Cystic Fibrosis (CF). PA is therefore recommended as part of standard care. Despite this, there is no consensus for monitoring of PA and little is known about perceptions of PA monitoring among children and young people with CF. Therefore, the research aimed to explore patients’...

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A mixed methods study of the administration of flucloxacillin oral liquid; identifying strategies to overcome administration issues of medicines with poor palatability

Claudia Rouse, Punam Mistry, Oli Rayner, Jess Nickless, more

International Journal of Pharmacy Practice > 25 > 5 > 326 - 334

Background The palatability of flucloxacillin oral liquid is poor. Parents/carers use strategies to aid the administration of poorly palatable medicines. Aim To assess views on the palatability of flucloxacillin oral liquid and identify factors associated with successful administration. Methods A mixed methods study which included a structured review of online forums and a survey of parent/carers...

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Ataluren and similar compounds (specific therapies for premature termination codon class I mutations) for cystic fibrosis

Aisha Aslam, Nikki Jahnke, Tracey Remmington, Kevin W. Southern

Paediatric Respiratory Reviews > 2017 > 24 > C > 32-34

article

The expansion and performance of national newborn screening programmes for cystic fibrosis in Europe

Jürg Barben, Carlo Castellani, Jeannette Dankert-Roelse, Silvia Gartner, more

Journal of Cystic Fibrosis > 2017 > 16 > 2 > 207-213

Newborn screening (NBS) for cystic fibrosis (CF) is a well-established public health strategy with international standards. The aim of this study was to provide an update on NBS for CF in Europe and assess performance against the standards.Questionnaires were sent to key workers in each European country.In 2016, there were 17 national programmes, 4 countries with regional programmes and 25 countries...

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Cystic Fibrosis Transmembrane Conductance Regulator-Related Metabolic Syndrome and Cystic Fibrosis Screen Positive, Inconclusive Diagnosis

Clement L. Ren, Drucy S. Borowitz, Tanja Gonska, Michelle S. Howenstine, more

The Journal of Pediatrics > 2017 > 181 > S > S45-S51.e1

An unintended consequence of cystic fibrosis (CF) newborn screening (NBS) is the identification of infants with a positive NBS test but inconclusive diagnostic testing. These infants are classified as CF transmembrane conductance regulator-related metabolic syndrome (CRMS) in the US and CF screen positive, inconclusive diagnosis (CFSPID) in other countries. Diagnostic and management decisions of these...

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Diagnosis of Cystic Fibrosis: Consensus Guidelines from the Cystic Fibrosis Foundation

Philip M. Farrell, Terry B. White, Clement L. Ren, Sarah E. Hempstead, more

The Journal of Pediatrics > 2017 > 181 > S > S4-S15.e1

Cystic fibrosis (CF), caused by mutations in the CF transmembrane conductance regulator (CFTR) gene, continues to present diagnostic challenges. Newborn screening and an evolving understanding of CF genetics have prompted a reconsideration of the diagnosis criteria.To improve diagnosis and achieve standardized definitions worldwide, the CF Foundation convened a committee of 32 experts in CF diagnosis...

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Standardy opieki Europejskiego Towarzystwa Mukowiscydozy: wytyczne i najlepsze praktyki

Alan R. Smyth, Scott C. Bell, Snezana Bojcin, Mandy Bryon, more

Pediatria Polska > 2016 > 91 > S1 > S30-S53

Specjalistyczna opieka nad chorymi na mukowiscydozę doprowadziła do znaczącej poprawy przeżycia: w ciągu ostatnich czterech dekad było ono istotnie wyższe niż wśród ogólnej populacji pacjentów w analogicznym wcześniejszym okresie. Wprowadzenie badań przesiewowych noworodków w wielu krajach Europy sprawiło, że w coraz większej liczbie ośrodków prowadzeni są pacjenci z minimalnymi zmianami płucnymi...

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Newborn screening for cystic fibrosis

Carlo Castellani, John Massie, Marci Sontag, Kevin W Southern

The Lancet Respiratory Medicine > 2016 > 4 > 8 > 653-661

Since the late 1970s when the potential of the immunoreactive trypsinogen assay for early identification of infants with cystic fibrosis was first recognised, the performance of newborn blood spot screening (NBS) has been continually assessed and its use has gradually expanded. NBS for cystic fibrosis is a cost-effective strategy and, if standards of care are fully implemented and robust management...

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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

Jane C Davies, Steve Cunningham, William T Harris, Allen Lapey, more

The Lancet Respiratory Medicine > 2016 > 4 > 2 > 107-115

Ivacaftor has been shown to be a safe, effective treatment for cystic fibrosis in patients aged 6 years or older with a CFTR gating mutation. We aimed to assess the safety, pharmacokinetics, and pharmacodynamics of ivacaftor in children aged 2–5 years. In the two-part KIWI study, we enrolled children aged 2–5 years weighing 8 kg or more with a confirmed diagnosis of cystic fibrosis and a CFTR gating...

article

Social network analysis of Pseudomonas aeruginosa in cystic fibrosis

David C Taylor-Robinson, Kevin W Southern, Craig Winstanley

The Lancet Respiratory Medicine > 2015 > 3 > 8 > 595-596

article

Potentiators for cystic fibrosis – targeting the underlying molecular defect

Sanjay Patel, Ian P. Sinha, Kerry Dwan, Carlos Echevarria, more

Paediatric Respiratory Reviews > 2015 > 16 > 3 > 162-164

article

Turnover of strains and intraclonal variation amongst Pseudomonas aeruginosa isolates from paediatric CF patients

Amanda J. Hall, Joanne L. Fothergill, Paul S. McNamara, Kevin W. Southern, more

Diagnostic Microbiology & Infectious Disease > 2014 > 80 > 4 > 324-326

Transmissible strains of Pseudomonas aeruginosa in cystic fibrosis (CF) have been well documented. Our longitudinal survey of P. aeruginosa isolates from 45 paediatric CF patients indicated strain persistence and intraclonal diversity, but not cross infection. This study demonstrates the need for regular P. aeruginosa strain surveillance using genotyping.

article

The challenge of improving outcomes for patients with CF sinonasal disease

Donald R. VanDevanter, Kevin W. Southern

Journal of Cystic Fibrosis > 2014 > 13 > 4 > 361-362

article

European Cystic Fibrosis Society Standards of Care: Best Practice guidelines

Alan R. Smyth, Scott C. Bell, Snezana Bojcin, Mandy Bryon, more

Journal of Cystic Fibrosis > 2014 > 13 > Supplement 1 > S23-S42

Specialised CF care has led to a dramatic improvement in survival in CF: in the last four decades, well above what was seen in the general population over the same period. With the implementation of newborn screening in many European countries, centres are increasingly caring for a cohort of patients who have minimal lung disease at diagnosis and therefore have the potential to enjoy an excellent...

article

Gastrointestinal surgery in cystic fibrosis: A 20-year review

Paul J. Farrelly, Caroline Charlesworth, Sophie Lee, Kevin W. Southern, more

Journal of Pediatric Surgery > 2014 > 49 > 2 > 280-283

The purpose of this study was to evaluate outcomes of the surgical management for meconium ileus (MI) and Distal Intestinal Obstruction Syndrome (DIOS) in Cystic Fibrosis (CF).Children born between 1990 and 2010 were identified using a regional CF database. Retrospective case note analysis was performed. Outcome measures for MI were mortality, relaparotomy rate, length of stay (LOS), time on parental...

article

Considering consent: a structural equation modelling analysis of factors influencing decisional quality when accepting newborn screening

Stuart G. Nicholls, Kevin W. Southern

Journal of Inherited Metabolic Disease > 2014 > 37 > 2 > 197-205

Introduction Newborn bloodspot screening (NBS) programs generate an ethical tension between promoting the uptake of effective public health measures and facilitating informed consent from individuals. Aim To explore the factors that affect parental perceptions of decision quality when accepting NBS Methods Survey of parents with children screened in 2008 (n = 154, 32 % response rate). Questions...

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Adherence to nebulised therapies in adolescents with cystic fibrosis is best on week-days during school term-time

Rosemary Ball, Kevin W. Southern, Pamela McCormack, Alistair J.A. Duff, more

Journal of Cystic Fibrosis > 2013 > 12 > 5 > 440-444

Treatment regimen for families of children with cystic fibrosis (CF) is considerable, particularly when nebulised therapies for chronic Pseudomonas aeruginosa airway infection are prescribed. Adherence to these regimens is variable, particularly in adolescence. Previously, we reported children to be more adherent in evenings compared to mornings, suggesting an association with time-pressure. The aim...

article

Macrolide antibiotics for cystic fibrosis

Kevin W. Southern

Paediatric Respiratory Reviews > 2012 > 13 > 4 > 228-229

article

A randomised controlled trial of breathing modes for adaptive aerosol delivery in children with cystic fibrosis

Pamela McCormack, Paul S. McNamara, Kevin W. Southern

Journal of Cystic Fibrosis > 2011 > 10 > 5 > 343-349

Aerosol delivery is a cornerstone of CF airways disease management. New nebulisers have reduced treatment times by utilising mesh technology for aerosol production. We have evaluated a further modification (target inhalation mode (TIM)) that may reduce treatment delivery times further.Following a baseline period on tidal breathing mode (TBM), children with CF on long-term aerosol therapy were randomly...

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Search results for: Kevin W. Southern

Aerosolized agents for airway clearance in cystic fibrosis

A formative study exploring perceptions of physical activity and physical activity monitoring among children and young people with cystic fibrosis and health care professionals

A mixed methods study of the administration of flucloxacillin oral liquid; identifying strategies to overcome administration issues of medicines with poor palatability

Ataluren and similar compounds (specific therapies for premature termination codon class I mutations) for cystic fibrosis

The expansion and performance of national newborn screening programmes for cystic fibrosis in Europe

Cystic Fibrosis Transmembrane Conductance Regulator-Related Metabolic Syndrome and Cystic Fibrosis Screen Positive, Inconclusive Diagnosis

Diagnosis of Cystic Fibrosis: Consensus Guidelines from the Cystic Fibrosis Foundation

Standardy opieki Europejskiego Towarzystwa Mukowiscydozy: wytyczne i najlepsze praktyki

Newborn screening for cystic fibrosis

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

Social network analysis of Pseudomonas aeruginosa in cystic fibrosis

Potentiators for cystic fibrosis – targeting the underlying molecular defect

Turnover of strains and intraclonal variation amongst Pseudomonas aeruginosa isolates from paediatric CF patients

The challenge of improving outcomes for patients with CF sinonasal disease

European Cystic Fibrosis Society Standards of Care: Best Practice guidelines

Gastrointestinal surgery in cystic fibrosis: A 20-year review

Considering consent: a structural equation modelling analysis of factors influencing decisional quality when accepting newborn screening

Adherence to nebulised therapies in adolescents with cystic fibrosis is best on week-days during school term-time

Macrolide antibiotics for cystic fibrosis

A randomised controlled trial of breathing modes for adaptive aerosol delivery in children with cystic fibrosis

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