-
Je něco špatně v tomto záznamu ?
Standards for the care of people with cystic fibrosis (CF); recognising and addressing CF health issues
PR. Burgel, KW. Southern, C. Addy, A. Battezzati, C. Berry, JP. Bouchara, E. Brokaar, W. Brown, P. Azevedo, I. Durieu, M. Ekkelenkamp, F. Finlayson, J. Forton, J. Gardecki, P. Hodkova, G. Hong, J. Lowdon, S. Madge, C. Martin, E. McKone, A. Munck,...
Jazyk angličtina Země Nizozemsko
Typ dokumentu časopisecké články, přehledy
- MeSH
- cystická fibróza * terapie MeSH
- lidé MeSH
- společnosti lékařské MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
- přehledy MeSH
- Geografické názvy
- Evropa MeSH
This is the third in a series of four papers updating the European Cystic Fibrosis Society (ECFS) standards for the care of people with CF. This paper focuses on recognising and addressing CF health issues. The guidance was produced with wide stakeholder engagement, including people from the CF community, using an evidence-based framework. Authors contributed sections, and summary statements which were reviewed by a Delphi consultation. Monitoring and treating airway infection, inflammation and pulmonary exacerbations remains important, despite the widespread availability of CFTR modulators and their accompanying health improvements. Extrapulmonary CF-specific health issues persist, such as diabetes, liver disease, bone disease, stones and other renal issues, and intestinal obstruction. These health issues require multidisciplinary care with input from the relevant specialists. Cancer is more common in people with CF compared to the general population, and requires regular screening. The CF life journey requires mental and emotional adaptation to psychosocial and physical challenges, with support from the CF team and the CF psychologist. This is particularly important when life gets challenging, with disease progression requiring increased treatments, breathing support and potentially transplantation. Planning for end of life remains a necessary aspect of care and should be discussed openly, honestly, with sensitivity and compassion for the person with CF and their family. CF teams should proactively recognise and address CF-specific health issues, and support mental and emotional wellbeing while accompanying people with CF and their families on their life journey.
Central Clinical School Faculty of Medicine University of Sydney Sydney Australia
CF Center at University Hospital Motol Prague Czech Republic
CF Centre at Goethe University Frankfurt Frankfurt am Main Germany
Clinical Specialist Paediatric Cystic Fibrosis Dietitian Leeds Children's Hospital UK
Cork Centre for Cystic Fibrosis Cork University Hospital University College Cork Ireland
Cystic Fibrosis Europe Brussels Belgium and the Belgian CF Association Brussels Belgium
Cystic Fibrosis Foundation Inova Fairfax Hospital Bethesda Maryland USA Falls Church VA USA
Cystic Fibrosis Reference Centre Centro Hospitalar Universitário Lisboa Norte Portugal
Cystic Fibrosis Unit and Pediatric Pulmonology Hospital Universitari Vall d'Hebron Barcelona Spain
Department of Medical Microbiology University Medical Center Utrecht Utrecht the Netherlands
Department of Medicine Johns Hopkins University Baltimore MD USA
Department of Medicine University of Pennsylvania Perelman School of Medicine Philadelphia PA USA
Department of Nutrition and Dietetics Alder Hey Children's NHS Trust Liverpool UK
Department of Pediatrics Indiana University School of Medicine Indianapolis IN USA
Department of Pharmacy Haga Teaching Hospital The Hague the Netherlands
Department of Respiratory Medicine Children's Health Ireland Dublin Ireland
Department of Respiratory Medicine The Alfred Hospital Melbourne Australia
ERN Lung Cystic Fibrosis Network Frankfurt Germany
Hospital Necker Enfants Malades AP HP CF Centre Université Paris Descartes Paris France
IRCCS Istituto Giannina Gaslini Via Gerolamo Gaslini 5 Genova 16147 Italy
Meyer Children's Hospital IRCCS Cystic Fibrosis Regional Reference Centre Italy
Pediatric Gastroenterology Unit CF Center Hadassah Medical Center Jerusalem Israel
Royal Brompton Hospital Part of Guys and StThomas's Hospital London UK
St Vincent's University Hospital and University College Dublin School of Medicine Dublin Ireland
The Children's Hospital for Wales Cardiff UK
The Graub CF Center Pulmonary Institute Schneider Children's Medical Center Petah Tikva Israel
University Hospital Southampton NHS Foundation Trust Southampton United Kingdom
Citace poskytuje Crossref.org
- 000
- 00000naa a2200000 a 4500
- 001
- bmc24014563
- 003
- CZ-PrNML
- 005
- 20240905133848.0
- 007
- ta
- 008
- 240725s2024 ne f 000 0|eng||
- 009
- AR
- 024 7_
- $a 10.1016/j.jcf.2024.01.005 $2 doi
- 035 __
- $a (PubMed)38233247
- 040 __
- $a ABA008 $b cze $d ABA008 $e AACR2
- 041 0_
- $a eng
- 044 __
- $a ne
- 100 1_
- $a Burgel, Pierre-Régis $u Respiratory Medicine and Cystic Fibrosis National Reference Center, Cochin Hospital, Assistance Publique Hôpitaux de Paris (AP-HP), Institut Cochin, Inserm U1016, Université Paris-Cité, Paris, France
- 245 10
- $a Standards for the care of people with cystic fibrosis (CF); recognising and addressing CF health issues / $c PR. Burgel, KW. Southern, C. Addy, A. Battezzati, C. Berry, JP. Bouchara, E. Brokaar, W. Brown, P. Azevedo, I. Durieu, M. Ekkelenkamp, F. Finlayson, J. Forton, J. Gardecki, P. Hodkova, G. Hong, J. Lowdon, S. Madge, C. Martin, E. McKone, A. Munck, CY. Ooi, L. Perrem, A. Piper, A. Prayle, F. Ratjen, M. Rosenfeld, DB. Sanders, C. Schwarz, G. Taccetti, C. Wainwright, NE. West, M. Wilschanski, A. Bevan, C. Castellani, P. Drevinek, S. Gartner, A. Gramegna, E. Lammertyn, EEC. Landau, BJ. Plant, AR. Smyth, S. van Koningsbruggen-Rietschel, PG. Middleton
- 520 9_
- $a This is the third in a series of four papers updating the European Cystic Fibrosis Society (ECFS) standards for the care of people with CF. This paper focuses on recognising and addressing CF health issues. The guidance was produced with wide stakeholder engagement, including people from the CF community, using an evidence-based framework. Authors contributed sections, and summary statements which were reviewed by a Delphi consultation. Monitoring and treating airway infection, inflammation and pulmonary exacerbations remains important, despite the widespread availability of CFTR modulators and their accompanying health improvements. Extrapulmonary CF-specific health issues persist, such as diabetes, liver disease, bone disease, stones and other renal issues, and intestinal obstruction. These health issues require multidisciplinary care with input from the relevant specialists. Cancer is more common in people with CF compared to the general population, and requires regular screening. The CF life journey requires mental and emotional adaptation to psychosocial and physical challenges, with support from the CF team and the CF psychologist. This is particularly important when life gets challenging, with disease progression requiring increased treatments, breathing support and potentially transplantation. Planning for end of life remains a necessary aspect of care and should be discussed openly, honestly, with sensitivity and compassion for the person with CF and their family. CF teams should proactively recognise and address CF-specific health issues, and support mental and emotional wellbeing while accompanying people with CF and their families on their life journey.
- 650 12
- $a cystická fibróza $x terapie $7 D003550
- 650 _2
- $a lidé $7 D006801
- 650 _2
- $a společnosti lékařské $7 D012955
- 651 _2
- $a Evropa $7 D005060
- 655 _2
- $a časopisecké články $7 D016428
- 655 _2
- $a přehledy $7 D016454
- 700 1_
- $a Southern, Kevin W $u Department of Women's and Children's Health, Institute in the Park, Alder Hey Children's Hospital, University of Liverpool, Eaton Road, Liverpool L12 2AP, UK. Electronic address: kwsouth@liv.ac.uk
- 700 1_
- $a Addy, Charlotte $u All Wales Adult Cystic Fibrosis Centre, University Hospital Llandough, Cardiff and Vale University Health Board, Cardiff, UK
- 700 1_
- $a Battezzati, Alberto $u Clinical Nutrition Unit, Department of Endocrine and Metabolic Medicine, IRCCS Istituto Auxologico Italiano, and ICANS-DIS, Department of Food Environmental and Nutritional Sciences, University of Milan, Milan, Italy
- 700 1_
- $a Berry, Claire $u Department of Nutrition and Dietetics, Alder Hey Children's NHS Trust, Liverpool, UK
- 700 1_
- $a Bouchara, Jean-Philippe $u University of Brest, Fungal Respiratory Infections Research Unit, SFR ICAT, University of Angers, Angers, France
- 700 1_
- $a Brokaar, Edwin $u Department of Pharmacy, Haga Teaching Hospital, The Hague, the Netherlands
- 700 1_
- $a Brown, Whitney $u Cystic Fibrosis Foundation, Inova Fairfax Hospital, Bethesda, Maryland, USA, Falls Church, VA, USA
- 700 1_
- $a Azevedo, Pilar $u Cystic Fibrosis Reference Centre-Centro, Hospitalar Universitário Lisboa Norte, Portugal
- 700 1_
- $a Durieu, Isabelle $u Cystic Fibrosis Reference Center (Constitutif), Service de médecine interne et de pathologie vasculaire, Hospices Civils de Lyon, Hôpital Lyon Sud, RESearch on HealthcAre PErformance (RESHAPE), INSERM U1290, Université Claude Bernard Lyon 1, 8 avenue Rockefeller, 69373 Lyon Cedex 08, France; ERN-Lung Cystic Fibrosis Network, Frankfurt, Germany
- 700 1_
- $a Ekkelenkamp, Miquel $u Department of Medical Microbiology, University Medical Center Utrecht, Utrecht, the Netherlands
- 700 1_
- $a Finlayson, Felicity $u Department of Respiratory Medicine, The Alfred Hospital, Melbourne, Australia
- 700 1_
- $a Forton, Julian $u The Children's Hospital for Wales, Cardiff, UK
- 700 1_
- $a Gardecki, Johanna $u CF Centre at Goethe University Frankfurt, Frankfurt am Main, Germany
- 700 1_
- $a Hodkova, Pavla $u CF Center at University Hospital Motol, Prague, Czech Republic
- 700 1_
- $a Hong, Gina $u Department of Medicine, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, USA
- 700 1_
- $a Lowdon, Jacqueline $u Clinical Specialist Paediatric Cystic Fibrosis Dietitian, Leeds Children's Hospital, UK
- 700 1_
- $a Madge, Su $u Royal Brompton Hospital, Part of Guys and StThomas's Hospital, London, UK
- 700 1_
- $a Martin, Clémence $u Institut Cochin, Inserm U1016, Université Paris-Cité and National Reference Center for Cystic Fibrosis, Hôpital Cochin AP-HP, ERN-Lung CF Network, Paris 75014, France
- 700 1_
- $a McKone, Edward $u St.Vincent's University Hospital and University College Dublin School of Medicine, Dublin, Ireland
- 700 1_
- $a Munck, Anne $u Hospital Necker Enfants-Malades, AP-HP, CF Centre, Université Paris Descartes, Paris, France
- 700 1_
- $a Ooi, Chee Y $u School of Clinical Medicine, Discipline of Paediatrics and Child Health, Faculty of Medicine & Health, Department of Gastroenterology, Sydney Children's Hospital, University of New South Wales, Sydney, NSW, Australia
- 700 1_
- $a Perrem, Lucy $u Department of Respiratory Medicine, Children's Health Ireland, Dublin, Ireland
- 700 1_
- $a Piper, Amanda $u Central Clinical School, Faculty of Medicine, University of Sydney, Sydney, Australia
- 700 1_
- $a Prayle, Andrew $u Child Health, Lifespan and Population Health & Nottingham Biomedical Research Centre, School of Medicine, University of Nottingham, Nottingham, UK
- 700 1_
- $a Ratjen, Felix $u Division of Respiratory Medicine, Department of Pediatrics and Translational Medicine, Research Institute, Hospital for Sick Children, Toronto, Canada
- 700 1_
- $a Rosenfeld, Margaret $u Department of Pediatrics, Seattle Children's Research Institute, University of Washington School of Medicine, Seattle, WA, USA
- 700 1_
- $a Sanders, Don B $u Department of Pediatrics, Indiana University School of Medicine, Indianapolis, IN, USA
- 700 1_
- $a Schwarz, Carsten $u Division Cystic Fibrosis, CF Center, Clinic Westbrandenburg, HMU-Health and Medical University, Potsdam, Germany
- 700 1_
- $a Taccetti, Giovanni $u Meyer Children's Hospital IRCCS, Cystic Fibrosis Regional Reference Centre, Italy
- 700 1_
- $a Wainwright, Claire $u University of Queensland, South Brisbane, QLD, Australia
- 700 1_
- $a West, Natalie E $u Department of Medicine, Johns Hopkins University, Baltimore, MD, USA
- 700 1_
- $a Wilschanski, Michael $u Pediatric Gastroenterology Unit, CF Center, Hadassah Medical Center, Jerusalem, Israel
- 700 1_
- $a Bevan, Amanda $u University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom
- 700 1_
- $a Castellani, Carlo $u IRCCS Istituto Giannina Gaslini, Via Gerolamo Gaslini 5, Genova 16147, Italy
- 700 1_
- $a Drevinek, Pavel $u Department of Medical Microbiology, Second Faculty of Medicine, Motol University Hospital, Charles University, Prague, Czech Republic
- 700 1_
- $a Gartner, Silvia $u Cystic Fibrosis Unit and Pediatric Pulmonology, Hospital Universitari Vall d'Hebron, Barcelona, Spain
- 700 1_
- $a Gramegna, Andrea $u Department of Pathophysiology and Transplantation, Respiratory Unit and Adult Cystic Fibrosis Center, Università degli Studi di Milano, Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Milano, Italy
- 700 1_
- $a Lammertyn, Elise $u Cystic Fibrosis Europe, Brussels, Belgium and the Belgian CF Association, Brussels, Belgium
- 700 1_
- $a Landau, Eddie Edwina C $u The Graub CF Center, Pulmonary Institute, Schneider Children's Medical Center, Petah Tikva, Israel
- 700 1_
- $a Plant, Barry J $u Cork Centre for Cystic Fibrosis (3CF), Cork University Hospital, University College Cork, Ireland
- 700 1_
- $a Smyth, Alan R $u School of Medicine, Dentistry and Biomedical Sciences, Belfast and NIHR Nottingham Biomedical Research Centre, Queens University Belfast, Nottingham, UK
- 700 1_
- $a van Koningsbruggen-Rietschel, Silke $u CF Center Cologne, Children ́s Hospital, Faculty of Medicine, University Hospital Cologne, Cologne, Germany
- 700 1_
- $a Middleton, Peter G $u Westmead Clinical School, Department Respiratory & Sleep Medicine, Westmead Hospital, University of Sydney and CITRICA, Westmead, Australia
- 773 0_
- $w MED00006892 $t Journal of cystic fibrosis $x 1873-5010 $g Roč. 23, č. 2 (2024), s. 187-202
- 856 41
- $u https://pubmed.ncbi.nlm.nih.gov/38233247 $y Pubmed
- 910 __
- $a ABA008 $b sig $c sign $y - $z 0
- 990 __
- $a 20240725 $b ABA008
- 991 __
- $a 20240905133841 $b ABA008
- 999 __
- $a ok $b bmc $g 2143995 $s 1226429
- BAS __
- $a 3
- BAS __
- $a PreBMC-MEDLINE
- BMC __
- $a 2024 $b 23 $c 2 $d 187-202 $e 20240116 $i 1873-5010 $m Journal of cystic fibrosis $n J Cyst Fibros $x MED00006892
- LZP __
- $a Pubmed-20240725
