The future of cystic fibrosis care: a global perspective

…, KS Raraigh, SM Rowe, KW Southern… - The Lancet …, 2020 - thelancet.com
The past six decades have seen remarkable improvements in health outcomes for people
with cystic fibrosis, which was once a fatal disease of infants and young children. However, …

[HTML][HTML] European cystic fibrosis society standards of care: best practice guidelines

…, I Sermet-Gaudelus, KW Southern… - Journal of cystic …, 2014 - Elsevier
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 …

[HTML][HTML] ECFS best practice guidelines: the 2018 revision

…, F Ratjen, I Sermet-Gaudelus, KW Southern… - Journal of cystic …, 2018 - Elsevier
Developments in managing CF continue to drive dramatic improvements in survival. As
newborn screening rolls-out across Europe, CF centres are increasingly caring for cohorts of …

[HTML][HTML] Diagnosis of cystic fibrosis: consensus guidelines from the cystic fibrosis foundation

…, M Rosenfeld, I Sermet-Gaudelus, KW Southern… - The Journal of …, 2017 - Elsevier
Objective Cystic fibrosis (CF), caused by mutations in the CF transmembrane conductance
regulator (CFTR) gene, continues to present diagnostic challenges. Newborn screening and …

[HTML][HTML] Recommendations for the classification of diseases as CFTR-related disorders

…, M Seia, DN Sheppard, KW Southern… - Journal of Cystic …, 2011 - Elsevier
Several diseases have been clinically or genetically related to cystic fibrosis (CF), but a
consensus definition is lacking. Here, we present a proposal for consensus guidelines on cystic …

Macrolide antibiotics for cystic fibrosis

KW Southern, PM Barker… - … of systematic reviews, 2004 - cochranelibrary.com
Macrolide antibiotics for cystic fibrosis - Southern, KW - 2004 | Cochrane Library … Kevin W
Southern … The meta‐analysis of these trials in an earlier version of this review supported …

[HTML][HTML] European best practice guidelines for cystic fibrosis neonatal screening

C Castellani, KW Southern, K Brownlee… - Journal of Cystic …, 2009 - Elsevier
… Vitamin K supplementation Vitamin K supplementation is recommended at all ages, but
there is still no standard dosage validated for vitamin K therapy in infants. …

COVID-19 infection in children

…, R Viner, SL Hewer, KW Southern - The Lancet …, 2020 - thelancet.com
With rates of hospitalisation and mortality from novel coronavirus disease 2019 (COVID-19)
increasing, there is understandable concern across the UK for medical professionals and the …

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 …

…, WE Regelmann, GS Sawicki, KW Southern… - The Lancet …, 2016 - thelancet.com
Background 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, …

Repeat administration of DNA/liposomes to the nasal epithelium of patients with cystic fibrosis

SC Hyde, KW Southern, U Gileadi, EM Fitzjohn… - Gene therapy, 2000 - nature.com
The major cause of mortality in patients with cystic fibrosis (CF) is lung disease. Expression
of the cystic fibrosis transmembrane conductance regulator (CFTR) gene product in the …