Abstract

Objective: Abnormal CFTR function in the pancreas causes pancreatic ductular obstruction related to subsequent pancreatic insufficiency and intestinal malabsorption of both fat-soluble vitamins and nutrients. Malnutrition is an important problem during adolescence and commonly persists into early adult life. In the present study, the nutritional status of the patients were evaluated and the prevelance of fat-soluble vitamin deficiencies were determined in patients with cystic fibrosis who attended to our center.

Material  and  Methods:  The  data  of  fat-soluble vitamin  levels,  microbiological  status,  lung  function tests  and  nutritional  status were retrospectively analysed in patients with CF who attended to Hacettepe University Department of Pediatric Pulmonology and Gastroenterology.

Results: 122 of 700 CF patients, who attended to hospital in one year period were included in our cohort; the mean age of the patients was 11.9 ± 6.7 years and the mean duration of following time was 8.7 years. Of these 122 patients, 104 of them were equal and/or below the age of 18 while 22 of them above the age of 18. According to antrophometric measurements, 15.5% (n=19) of patients had malnutrition in all age groups, therefore, the incidence of malnutrition was 9.5% (n=10) in patients under ≤ 18 years of age while itwas 40.9% (n=9) in patients above the age of 18. The difference between patients who had malnutrition and who had normal nutritional status, according to FEV1 and FVC values, was statistically significant and these values remarkably lower in malnutrated patient group (p=0.001 and p=0.001).Staphylococcus aureus (44.6%) was the most and Pseudomonas aeruginosa (29.2%) was the second common microorganism isolated from sputum culture. Pancreatic insufficiency was detected in 99% of patients. Multivitamin supplement including vitamin A, D, E, K was given to 28% of patients. Therefore, multivitamin supplement including vitamin A, D and E were given to 72% of patients. Additional vitamin A and E were given 20.5% and 23% of patients whom also receiving multivitamin supplement including A, D and E. Vitamin D (32.3%) and A (26.9%) deficiencies were the most common findings in our cohort. There was also a moderate uphill (positive) correlation between FEV1-FVC values and vitamin A levels (p<0.001).

Conclusion: This is one of few studies of fat-soluble vitamin deficiency and malnutrition assessment of patients with CF. A well balanced nutritional status is a key factor for patients to maintain their respiratory functions in CF. Monitoring of fat-soluble vitamins is also essential to identify deficiency in pancreatic-insufficient children who may be non-compliant to supplementation, or require a higher supplement dose.

Keywords: Cystic Fibrosis, Deficiency, Fat-soluble vitamin, Malnutrition

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Copyright and license

Copyright © 2019 The Author(s). This is an open access article distributed under the Creative Commons Attribution License (CC BY), which permits unrestricted use, distribution, and reproduction in any medium or format, provided the original work is properly cited.

How to cite

1.
Karhan AN. Evaluation of the Prevelance of Malnutrition and Fat Soluble Vitamin Deficiencies and the Assessment of the Relationship Between Pulmonary Functions Among Children and Adult Cystic Fibrosis Patient. Turk J Pediatr Dis [Internet]. 2019 Dec. 23 [cited 2025 Aug. 23];13(6):463-7. Available from: https://turkjpediatrdis.org/article/view/558