Background: Pancreatic insufficiency is common in patients with cystic fibrosis (CF) and leads to malabsorption of fat-soluble vitamins. Multivitamins, including vitamins A, D, E, and K, are routinely prescribed to patients with CF to prevent vitamin deficiencies. Our objective was to examine the relationship between fat-soluble vitamin supplements and their impact on blood concentrations. Methods: This was a retrospective chart review of patients with CF who were treated at Emory Clinic and Emory University Hospital during 2008–2012. The amount of fat-soluble vitamin supplementation, serum markers of fat-soluble vitamin concentrations, CF transmembrane conductance regulator genotype, and other demographic information were recorded from electronic medical records. Mixed-effects models were used to investigate the trends over time of fat-soluble vitamin supplements and serum vitamin concentrations. Results: In total, 177 charts were eligible. Mean (SD) age was 26.1 (10.2) years. Ninety-two percent of patients had pancreatic insufficiency and 52% had the homozygous ΔF508 mutation. Recorded fat-soluble vitamin supplementation increased in the past 5 years (P < .001 for all). Serum 25-hydroxyvitamin D increased slightly (3% increase; P < .01); however, there were no changes in the blood concentrations of vitamins A, E, and K (P = .26–.96). Conclusions: Despite a near doubling of recorded fat-soluble vitamin supplementation over the past 5 years, there was no parallel increase in blood concentrations of these vitamins. Potential reasons include suboptimal dosages, low adherence, or ongoing issues with malabsorption.
KeremEConwaySElbornSHeijermanHConsensusC. Standards of care for patients with cystic fibrosis: a European consensus. J Cyst Fibros. 2005;4(1):7-26.
2.
LiouTGAdlerFRFitzsimmonsSCCahillBCHibbsJRMarshallBC. Predictive 5-year survivorship model of cystic fibrosis. Am J Epidemiol. 2001;153(4):345-352.
3.
GozdzikJCoftaSPiorunekTBatura-GabryelHKosickiJ. Relationship between nutritional status and pulmonary function in adult cystic fibrosis patients. J Physiol Pharmacol. 2008;59(suppl 6):253-260.
4.
DodgeJATurckD. Cystic fibrosis: nutritional consequences and management. Best Pract Res Clin Gastroenterol. 2006;20(3):531-546.
5.
WalkowiakJLisowskaABlaszczynskiM. The changing face of the exocrine pancreas in cystic fibrosis: pancreatic sufficiency, pancreatitis and genotype. Eur J Gastroenterol Hepatol. 2008;20(3):157-160.
6.
CouperRTCoreyMMooreDJFisherLJForstnerGGDuriePR. Decline of exocrine pancreatic function in cystic fibrosis patients with pancreatic sufficiency. Pediatr Res. 1992;32(2):179-182.
7.
Wouthuyzen-BakkerMBodewesFAVerkadeHJ. Persistent fat malabsorption in cystic fibrosis: lessons from patients and mice. J Cyst Fibros. 2011;10(3):150-158.
8.
KalivianakisMMinichDMBijleveldCM. Fat malabsorption in cystic fibrosis patients receiving enzyme replacement therapy is due to impaired intestinal uptake of long-chain fatty acids. Am J Clin Nutr. 1999;69(1):127-134.
9.
FeranchakAPSontagMKWagenerJSHammondKBAccursoFJSokolRJ. Prospective, long-term study of fat-soluble vitamin status in children with cystic fibrosis identified by newborn screen. J Pediatr. 1999;135(5):601-610.
10.
SokolRJReardonMCAccursoFJ. Fat-soluble-vitamin status during the first year of life in infants with cystic fibrosis identified by screening of newborns. Am J Clin Nutr. 1989;50(5):1064-1071.
11.
RaynerRJ. Fat-soluble vitamins in cystic fibrosis. Proc Nutr Soc. 1992;51(2):245-250.
12.
WilsonDCPencharzPB. Nutrition and cystic fibrosis. Nutrition. 1998;14(10):792-795.
13.
PencharzPBDuriePR. Nutritional management of cystic fibrosis. Annu Rev Nutr. 1993;13:111-136.
14.
SolomonsNWWagonfeldJBRiegerC. Some biochemical indices of nutrition in treated cystic fibrosis patients. Am J Clin Nutr. 1981;34(4):462-474.
15.
RovnerAJStallingsVASchallJILeonardMBZemelBS. Vitamin D insufficiency in children, adolescents, and young adults with cystic fibrosis despite routine oral supplementation. Am J Clin Nutr. 2007;86(6):1694-1699.
16.
HallWBSparksAAArisRM. Vitamin D deficiency in cystic fibrosis. Int J Endocrinol. 2010;2010:1-9.
17.
BrodlieMOrchardWAReeksGA. Vitamin D in children with cystic fibrosis. Arch Dis Child. 2012;97(11):982-984.
18.
BinesJETrubyHDArmstrongDSCarzinoRGrimwoodK. Vitamin A and E deficiency and lung disease in infants with cystic fibrosis. J Paediatr Child Health. 2005;41(12):663-668.
19.
ConwaySP. Vitamin K in cystic fibrosis. J R Soc Med. 2004;97(suppl 44):48-51.
20.
ConwaySPWolfeSPBrownleeKG. Vitamin K status among children with cystic fibrosis and its relationship to bone mineral density and bone turnover. Pediatrics. 2005;115(5):1325-1331.
21.
DuriePR. Vitamin K and the management of patients with cystic fibrosis. CMAJ. 1994;151(7):933-936.
22.
SinaasappelMSternMLittlewoodJ. Nutrition in patients with cystic fibrosis: a European consensus. J Cyst Fibros. 2002;1(2):51-75.
23.
RamseyBWFarrellPMPencharzP. Nutritional assessment and management in cystic fibrosis: a consensus report. The Consensus Committee. Am J Clin Nutr. 1992;55(1):108-116.
24.
JavierRMJacquotJ. Bone disease in cystic fibrosis: what’s new?Joint Bone Spine. 2011;78(5):445-450.
25.
KalninsDWilschanskiM. Maintenance of nutritional status in patients with cystic fibrosis: new and emerging therapies. Drug Des Devel Ther. 2012;6:151-161.
26.
TangprichaVKellyAStephensonA. Cystic Fibrosis Foundation Vitamin DE-BRC: an update on the screening, diagnosis, management, and treatment of vitamin D deficiency in individuals with cystic fibrosis: evidence-based recommendations from the Cystic Fibrosis Foundation. J Clin Endocrinol Metab. 2012;97(4):1082-1093.
27.
CongdenPJBruceGRothburnMM. Vitamin status in treated patients with cystic fibrosis. Arch Dis Child. 1981;56(9):708-714.
28.
RoddyMFGreallyPClancyGLeenGFeehanSElnazirB. Night blindness in a teenager with cystic fibrosis. Nutr Clin Pract. 2011(6);26:718-721.
29.
ObeidMPriceJSunL. Facial palsy and idiopathic intracranial hypertension in twins with cystic fibrosis and hypovitaminosis A. Pediatr Neurol. 2011;44(2):150-152.
30.
Graham-MaarRCSchallJIStettlerNZemelBSStallingsVA. Elevated vitamin A intake and serum retinol in preadolescent children with cystic fibrosis. Am J Clin Nutr. 2006;84(1):174-182.
31.
MaqboolAGraham-MaarRCSchallJIZemelBSStallingsVA. Vitamin A intake and elevated serum retinol levels in children and young adults with cystic fibrosis. J Cyst Fibros. 2008;7(2):137-141.
32.
MaqboolAStallingsVA. Update on fat-soluble vitamins in cystic fibrosis. Curr Opin Pulm Med. 2008;14(6):574-581.
33.
HuangSHSchallJIZemelBSStallingsVA. Vitamin E status in children with cystic fibrosis and pancreatic insufficiency. J Pediatr. 2006;148(4):556-559.
34.
HakimFKeremERivlinJ. Vitamins A and E and pulmonary exacerbations in patients with cystic fibrosis. J Pediatr Gastroenterol Nutr. 2007;45(3):347-353.
35.
HaworthCSSelbyPLWebbAKMawerEBAdamsJEFreemontTJ. Severe bone pain after intravenous pamidronate in adult patients with cystic fibrosis. Lancet. 1998;352(9142):1753-1754.
36.
BoyleMPNoscheseMLWattsSLDavisMEStennerSELechtzinN. Failure of high-dose ergocalciferol to correct vitamin D deficiency in adults with cystic fibrosis. Am J Respir Crit Care Med. 2005;172(2):212-217.
37.
StephensonABrotherwoodMRobertRAtenafuECoreyMTullisE. Cholecalciferol significantly increases 25-hydroxyvitamin D concentrations in adults with cystic fibrosis. Am J Clin Nutr. 2007;85(5):1307-1311.
38.
DoughertyKASchallJIStallingsVA. Suboptimal vitamin K status despite supplementation in children and young adults with cystic fibrosis. Am J Clin Nutr. 2010;92(3):660-667.
39.
NicolaidouPStavrinadisILoukouI. The effect of vitamin K supplementation on biochemical markers of bone formation in children and adolescents with cystic fibrosis. Eur J Pediatr. 2006;165(8):540-545.
40.
RossACCaballeroBCousinsRJTuckerKLZieglerTR. Modern Nutrition in Health and Disease. Philadelphia, PA: Lippincott Williams & Wilkins; 2014:260-315.
41.
StallingsVAStarkLJRobinsonKAFeranchakAPQuintonH. Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency: results of a systematic review. J Am Diet Assoc. 2008;108(5):832-839.
42.
VerbekeGMolenberghsG. Linear Mixed Models for Longitudinal Data. New York, NY: Springer Verlag; 2009;1-132.
43.
CarrSBMcBratneyJ. The role of vitamins in cystic fibrosis. J R Soc Med. 2000;93(suppl 38):14-19.
44.
SembaRD. Vitamin A, immunity, and infection. Clin Infect Dis. 1994;19(3):489-499.
45.
HuetFSemamaDMaingueneauCCharavelANivelonJL. Vitamin A deficiency and nocturnal vision in teenagers with cystic fibrosis. Eur J Pediatr. 1997;156(12):949-951.
46.
GreerRMBuntainHMLewindonPJ. Vitamin A levels in patients with CF are influenced by the inflammatory response. J Cyst Fibros. 2004;3(3):143-149.
47.
AirdFKGreeneSAOgstonSAMacdonaldTMMukhopadhyayS. Vitamin A and lung function in CF. J Cyst Fibros. 2006;5(2):129-131.
48.
KoscikRLFarrellPMKosorokMR. Cognitive function of children with cystic fibrosis: deleterious effect of early malnutrition. Pediatrics. 2004;113(6):1549-1558.
49.
SitrinMDLiebermanFJensenWENoronhaAMilburnCAddingtonW. Vitamin E deficiency and neurologic disease in adults with cystic fibrosis. Ann Intern Med. 1987;107(1):51-54.
50.
ArisRMMerkelPABachrachLK. Guide to bone health and disease in cystic fibrosis. J Clin Endocrinol Metab. 2005;90(3):1888-1896.
51.
WolfendenLLJuddSEShahRSanyalRZieglerTRTangprichaV. Vitamin D and bone health in adults with cystic fibrosis. Clin Endocrinol (Oxf). 2008;69(3):374-381.
52.
GrossmannREZughaierSMLiuSLylesRHTangprichaV. Impact of vitamin D supplementation on markers of inflammation in adults with cystic fibrosis hospitalized for a pulmonary exacerbation. Eur J Clin Nutr. 2012;66(9):1072-1074.
53.
van HoornJHHendriksJJVermeerCForgetPP. Vitamin K supplementation in cystic fibrosis. Arch Dis Child. 2003;88(11):974-975.
54.
VergheseTBeverleyD. Vitamin K deficient bleeding in cystic fibrosis. Arch Dis Child. 2003;88(6):553.
55.
CliftonIJMortonAMPeckhamDGConwaySP. An unusual cause of hypercalcaemia in a patient with cystic fibrosis. J Cyst Fibros. 2007;6(5):369-370.
56.
CialdellaPCarellaF. Hypervitaminosis D: case report of pediatric osteoporosis secondary to cystic fibrosis. Clin Cases Miner Bone Metab. 2011;8(3):66-68.
57.
McKoneEFEmersonSSEdwardsKLAitkenML. Effect of genotype on phenotype and mortality in cystic fibrosis: a retrospective cohort study. Lancet. 2003;361(9370):1671-1676.
58.
KeremECoreyMKeremBS. The relation between genotype and phenotype in cystic fibrosis—analysis of the most common mutation (delta F508). N Engl J Med. 1990;323(22):1517-1522.
59.
KeremEReismanJCoreyMCannyGJLevisonH. Prediction of mortality in patients with cystic fibrosis. N Engl J Med. 1992;326(18):1187-1191.
60.
KristidisPBozonDCoreyM. Genetic determination of exocrine pancreatic function in cystic fibrosis. Am J Hum Genet. 1992;50(6):1178-1184.
61.
ZindaniGNStreetmanDDStreetmanDSNasrSZ. Adherence to treatment in children and adolescent patients with cystic fibrosis. J Adolesc Health. 2006;38(1):13-17.
62.
BorowitzDWegmanTHarrisM. Preventive care for patients with chronic illness: multivitamin use in patients with cystic fibrosis. Clin Pediatr (Phila). 1994;33(12):720-725.
