214: Improvement in fat-soluble vitamin levels following highly effective CFTR modulator use in children with CF

Kuffel, H.; Hoppe, Jordana E.; Meier, Michelle; Mark, Jerome; Wagner, Bernard M.; Towler, E.; Zemanick, Edith T.

doi:10.1016/s1569-1993(21)01639-8

Cited by 2 publications

(2 citation statements)

References 0 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In contrast, earlier studies of lumacaftor/ivacaftor found reduction in total and LDL cholesterol 16,17 . Most existing studies find either no change or higher vitamins A and D levels after ETI, while changes in vitamin E are variable 13,18‐23 . Case reports of vitamin A toxicity with ETI suggest that changes in absorption vary between individuals 24 .…”

Section: Introductionmentioning

confidence: 91%

“…16,17 Most existing studies find either no change or higher vitamins A and D levels after ETI, while changes in vitamin E are variable. 13,[18][19][20][21][22][23] Case reports of vitamin A toxicity with ETI suggest that changes in absorption vary between individuals. 24 Elevation of liver enzymes occurs with ETI therapy in many cases, though improvement in CF-associated liver disease, particularly hepatic steatosis, has been reported with ivacaftor and lumacaftor/ivacaftor, possibly due to improvement in essential fatty acid deficiency.…”

mentioning

confidence: 99%

See 1 more Smart Citation

Impact of elexacaftor/tezacaftor/ivacaftor on lipid and fat‐soluble vitamin levels and association with body mass index

Patel,

McBennett,

Sankararaman

et al. 2024

Pediatric Pulmonology

View full text Add to dashboard Cite

IntroductionCystic fibrosis transmembrane conductance regulator (CFTR) modulators improve gastrointestinal absorption of nutrients and may result in changes in body mass index (BMI), serum lipids, and fat‐soluble vitamin levels. We hypothesized that serum lipids and vitamin levels would increase with CFTR modulator therapy and that greater increase in lipids and vitamin levels would be related to greater increase in BMI.MethodsA retrospective study was performed to evaluate the impact of elexacaftor/tezacaftor/ivacaftor (ETI) on nutritional parameters, serum lipids, and fat‐soluble vitamin levels. Pre‐ETI values (<2 years prior) and post‐ETI values (>1 month after) were compared. Linear regression was used to evaluate whether change in BMI is associated with the change in lipid and/or vitamin levels and whether modulator duration is associated with the degree of rise in lipid and/or vitamin levels.ResultsAdults and adolescents with CF (n = 137) were evaluated before and 31–300 days after starting ETI. Median BMI (adults 21.9 vs. 23.5 kg/m2; adolescents 48 vs. 63 percentile) increased after initiation of ETI. Total cholesterol (126 vs. 154 mg/dL), low‐density lipoprotein cholesterol (63 vs. 78 mg/dL), non‐high‐density lipoprotein cholesterol (84 vs. 102 mg/dL), and high density lipoprotein cholesterol (43 vs. 49 mg/dL) increased after ETI, while triglycerides and very low density lipoprotein did not change. Median values for vitamin D (34.5 vs. 38.0 ng/mL) and vitamin A (40.1 vs. 47.9 µg/dL) increased, while vitamin E did not change significantly. There was no significant correlation between BMI change or duration of modulator therapy with vitamin levels or lipid changes.ConclusionAfter initiation of ETI therapy, serum lipids increased in our population, but most values remained within the normal range. Vitamins A and D levels increased post‐ETI and no changes were noted in vitamin E. No significant correlation between the degree of BMI change and the magnitude of increase in lipids or vitamin levels was found.

show abstract

Section: Introductionmentioning

confidence: 91%