P284 Food Characteristics As Determinants of PERT Requirements: Summary of the in Vitro Digestion Experiments of MyCyFAPP Project
Journal of cystic fibrosis(2019)
摘要
spontaneous increase of FE1 up to normal values (>200 mcg/g), without signs or symptoms of PI at temporary suspension of PERT.At a mean age of 4,3 years (range 1-8) all patients were without PERT, maintaining a normal growth with a mean FE1 of 348,5 mcg/g (range 208-500 mcg/g). Conclusion:We observed a transitory PI in 20% of infants with mild CFTR mutation, leading to an erroneous definition of CF with PI.PI in the first year of life was probably related to a delay in postnatal development of PF.Our experience underlines that it is essential to monitor closely FE1 levels in infants with a diagnosis of PI in the first months of life when their genotype should confer PS, in order to avoid useless therapy.
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