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Status: Bibliographieeintrag

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Verfasst von:Barben, Jürg [VerfasserIn]   i
 Castellani, Carlo [VerfasserIn]   i
 Munck, Anne [VerfasserIn]   i
 Davies, Jane C. [VerfasserIn]   i
 de Winter-de Groot, Karin M. [VerfasserIn]   i
 Gartner, Silvia [VerfasserIn]   i
 Kashirskaya, Nataliya [VerfasserIn]   i
 Linnane, Barry [VerfasserIn]   i
 Mayell, Sarah J [VerfasserIn]   i
 McColley, Susanna [VerfasserIn]   i
 Ooi, Chee Y. [VerfasserIn]   i
 Proesmans, Marijke [VerfasserIn]   i
 Ren, Clement L. [VerfasserIn]   i
 Salinas, Danieli [VerfasserIn]   i
 Sands, Dorota [VerfasserIn]   i
 Sermet-Gaudelus, Isabelle [VerfasserIn]   i
 Sommerburg, Olaf [VerfasserIn]   i
 Southern, Kevin W [VerfasserIn]   i
Titel:Updated guidance on the management of children with cystic fibrosis transmembrane conductance regulator-related metabolic syndrome/cystic fibrosis screen positive, inconclusive diagnosis (CRMS/CFSPID)
Verf.angabe:Jürg Barben, Carlo Castellani, Anne Munck, Jane C. Davies, Karin M. de Winter-de Groot, Silvia Gartner, Nataliya Kashirskaya, Barry Linnane, Sarah J Mayell, Susanna McColley, Chee Y. Ooi, Marijke Proesmans, Clement L. Ren, Danieli Salinas, Dorota Sands, Isabelle Sermet-Gaudelus, Olaf Sommerburg, Kevin W. Southern, for the European CF Society Neonatal Screening Working Group (ECFS NSWG)
Jahr:2021
Umfang:10 S.
Fussnoten:Available online 27 November 2020 ; Gesehen am 31.05.2022
Titel Quelle:Enthalten in: Journal of cystic fibrosis
Ort Quelle:Amsterdam [u.a.] : Elsevier Science, 2002
Jahr Quelle:2021
Band/Heft Quelle:20(2021), 5, Seite 810-819
ISSN Quelle:1873-5010
Abstract:Over the past two decades there has been considerable progress with the evaluation and management of infants with an inconclusive diagnosis following Newborn Screening (NBS) for cystic Fibrosis (CF). In addition, we have an increasing amount of evidence on which to base guidance on the management of these infants and, importantly, we have a consistent designation being used across the globe of CRMS/CFSPID. There is still work to be undertaken and research questions to answer, but these infants now receive more consistent and appropriate care pathways than previously. It is clear that the majority of these infants remain healthy, do not convert to a diagnosis of CF in childhood, and advice on management should reflect this. However, it is also clear that some will convert to a CF diagnosis and monitoring of these infants should facilitate their early recognition. Those infants that do not convert to a CF diagnosis have some potential of developing a CFTR-RD later in life. At present, it is not possible to quantify this risk, but families need to be provided with clear information of what to look out for. This paper contains a number of changes from previous guidance in light of developing evidence, but the major change is the recommendation of a detailed assessment of the child with CRMS/CFSPID in the sixth year of age, including respiratory function assessment and imaging. With these data, the CF team can discuss future care arrangements with the family and come to a shared decision on the best way forward, which may include discharge to primary care with appropriate information. Information is key for these families, and we recommend consideration of a further appointment when the individual is a young adult to directly communicate the implications of the CRMS/CFSPID designation.
DOI:doi:10.1016/j.jcf.2020.11.006
URL:Bitte beachten Sie: Dies ist ein Bibliographieeintrag. Ein Volltextzugriff für Mitglieder der Universität besteht hier nur, falls für die entsprechende Zeitschrift/den entsprechenden Sammelband ein Abonnement besteht oder es sich um einen OpenAccess-Titel handelt.

Volltext ; Verlag: https://doi.org/10.1016/j.jcf.2020.11.006
 Volltext: https://www.sciencedirect.com/science/article/pii/S1569199320309097
 DOI: https://doi.org/10.1016/j.jcf.2020.11.006
Datenträger:Online-Ressource
Sprache:eng
Sach-SW:CFSPID
 CRMS
 Cystic fibrosis
 Management
 Newborn screening
K10plus-PPN:1805370448
Verknüpfungen:→ Zeitschrift

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