Clinical validation of an evidence-based method to adjust Pancreatic Enzyme Replacement Therapy through a prospective interventional study in paediatric patients with Cystic Fibrosis.
Joaquim Calvo-LermaJessie HulstMieke BoonCarla ColomboEtna MasipMar RupertoVictoria Fornés-FerrerEls van der WielIne ClaesMaria GarrigaMaria RocaPaula Crespo-EscobarAnna BulfamanteSandra WoodcockSandra Martínez-BaronaAna AndrésKris de BoeckCarmen Ribes-Koninckxnull nullPublished in: PloS one (2019)
Strict adherence to the theoretical optimal dose of enzymatic supplement for a prescribed meal, led to median CFA levels at the clinical target of 90% with a low variability between patients. The proposed method can be considered as a first approach for an evidence-based method in PERT dosing based on food characteristics. Results have to be confirmed in free dietary settings.