Stratifying infants with cystic fibrosis for disease severity using intestinal organoid swelling as a biomarker of CFTR function

Stratifying infants with cystic fibrosis for disease severity using intestinal organoid swelling as a biomarker of CFTR function

Forskolin-induced swelling (FIS) of intestinal organoids from individuals with cystic fibrosis (CF) measures function of the cystic fibrosis transmembrane conductance regulator (CFTR), the protein mutated in CF.We investigated whether FIS corresponds with clinical outcome parameters and biomarkers o...

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Veröffentlicht in:The European respiratory journal 2018-09, Vol.52 (3), p.1702529
Hauptverfasser: de Winter-de Groot, Karin M, Janssens, Hettie M, van Uum, Rick T, Dekkers, Johanna F, Berkers, Gitte, Vonk, Annelotte, Kruisselbrink, Evelien, Oppelaar, Hugo, Vries, Robert, Clevers, Hans, Houwen, Roderick H J, Escher, Johanna C, Elias, Sjoerd G, de Jonge, Hugo R, de Rijke, Yolanda B, Tiddens, Harm A W M, van der Ent, Cornelis K, Beekman, Jeffrey M
Format: Artikel
Sprache:eng
Schlagworte:
Biomarkers - metabolism
Chlorides - metabolism
Cystic Fibrosis - complications
Cystic Fibrosis - diagnosis
Cystic Fibrosis Transmembrane Conductance Regulator - metabolism
Exocrine Pancreatic Insufficiency - diagnosis
Female
Humans
Infant
Ion Transport
Linear Models
Male
Organoids - pathology
Proof of Concept Study
Severity of Illness Index
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Zusammenfassung:Forskolin-induced swelling (FIS) of intestinal organoids from individuals with cystic fibrosis (CF) measures function of the cystic fibrosis transmembrane conductance regulator (CFTR), the protein mutated in CF.We investigated whether FIS corresponds with clinical outcome parameters and biomarkers of CFTR function in 34 infants diagnosed with CF. Relationships with FIS were studied for indicators of pulmonary and gastrointestinal disease.Children with low FIS had higher levels of immunoreactive trypsinogen (p=0.030) and pancreatitis-associated protein (p=0.039), more often had pancreatic insufficiency (p
ISSN:0903-1936
1399-3003
DOI:10.1183/13993003.02529-2017