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Article

The Impact of Nutritional Management on Fat-Soluble Nutrient Status in Patients with Fatty Acid Oxidation Disorders: A Cross-Sectional Study

by
Maria Wasiewicz-Gajdzis
1,
Małgorzata Jamka
1,
Łukasz Kałużny
1,
Natalia Wichłacz-Trojanowska
1,
Anna Blask-Osipa
1,
Monika Duś-Żuchowska
1,
Joanna Jagłowska
2,
Szymon Kurek
1,
Anna Miśkiewicz-Chotnicka
1 and
Jarosław Walkowiak
1,*
1
Poznan University of Medical Sciences, Department of Pediatric Gastroenterology and Metabolic Diseases, Szpitalna 27/33, 60-572 Poznan, Poland
2
Department of Pediatrics, Hematology and Oncology, Medical University of Gdansk, Dębinki Str. 7, 80-211 Gdansk, Poland
*
Author to whom correspondence should be addressed.
Metabolites 2026, 16(2), 124; https://doi.org/10.3390/metabo16020124
Submission received: 21 November 2025 / Revised: 22 January 2026 / Accepted: 5 February 2026 / Published: 11 February 2026
(This article belongs to the Section Nutrition and Metabolism)
Browse Figure
Versions Notes

Abstract

Background: Fatty acid oxidation disorders (FAOD) are rare inborn errors of metabolism that impair mitochondrial β-oxidation and energy production. Management includes fasting avoidance for all FAOD types. Patients with long-chain FAOD are advised to restrict long-chain triglycerides (LCTs) to 10% of total energy intake and supplement medium-chain triglycerides (MCTs). The impact of such dietary modification on fat-soluble vitamin status has not yet been studied. Methods: In this cross-sectional study, serum concentrations of vitamins A, 25(OH)D, E, and β-carotene were measured in 36 FAOD patients and 36 healthy controls matched for age and sex. Vitamins A, E, and β-carotene were quantified using high-performance liquid chromatography and vitamin 25(OH)D through an immunoassay. FAOD patients were further divided into fat-modified (LCT-restricted) and standard-fat diet subgroups based on dietary management. Results: FAOD patients had significantly higher vitamin A concentrations than controls (p < 0.05), while there was no difference in vitamins 25(OH)D, E, and β-carotene. Within the FAOD cohort, the fat-modified group had higher levels of vitamins A and 25(OH)D but lower levels of vitamin E and β-carotene than the standard-fat group (all p < 0.05). Vitamin 25(OH)D deficiency (<20 ng/mL) was more frequent in the standard-fat group (p = 0.03). Conclusions: Fat-modified diets influence fat-soluble vitamin status in FAOD, emphasising the importance of ongoing monitoring and tailored supplementation. Future work should focus on optimising nutritional management, including modifications to formula composition, and on addressing the currently limited evidence on nutritional status and vitamin deficiencies in patients with FAOD.

1. Introduction

Fatty acid oxidation is a complex mitochondrial pathway that converts fatty acids released from adipose tissue into acetyl-CoA and reducing equivalents, which are utilised for energy production, especially under conditions of limited glucose availability, during fasting, exercise, or febrile illness [1,2]. Fatty acids are also a preferred energy source for the myocardium in the neonatal period, for skeletal muscle during sub-maximal exercise, and for the liver [1,3]. Mutations in genes encoding fatty acid transport proteins and enzymes involved in fatty acid oxidation cause fatty acid oxidation disorders (FAOD), which are inherited in an autosomal recessive manner [1]. FAODs result in defective energy metabolism and accumulation of toxic acylocarnitines [1,2,4,5]. There are at least 20 known FAODs, with a combined incidence of 0.9–15.2 per 100,000, with medium-chain acyl-CoA dehydrogenase deficiency (MCAD) being the most common (1 per 20,000 in Northern European/Caucasian populations) [6,7]. Among long-chain fatty acid oxidation disorders (LC-FAOD), very long-chain acyl-CoA dehydrogenase deficiency (VLCAD) has the highest incidence (1 per 30,000 to 100,000 births) [8,9], followed by long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHAD)—1 per 50,000 to 170,000 births [7,10]. The incidence of FAODs is believed to be increasing due to the wide implementation of newborn screening programmes (NBS) [7,11,12].
The clinical presentation of FAOD varies depending on the type of defect, but some common features can be identified. Because fatty acid oxidation is an important energy source for organs with high metabolic demands, clinical symptoms predominantly affect the liver, heart, skeletal muscle, and central nervous system [9,13,14,15,16]. During acute episodes of metabolic stress, the patient may present with hypoglycemia without ketones, elevation of transaminases, and elevated ammonia [5]. Progression of symptoms to hepatic insufficiency, brain oedema, and death is possible. Additionally, LC-FAODs, such as LCHAD and VLCAD, as well as certain diseases of carnitine metabolism, including carnitine acylcarnitine translocase deficiency (CACT) and carnitine palmitoyltransferase I deficiency (CPT1), may present with cardiomyopathy and rhabdomyolysis. Neuropathy and retinopathy occur exclusively in patients with LCHAD [1,5,17].
Management depends on the type of FAOD. In cases of LC-FAODs, fasting should be avoided, and dietary management typically includes restricting long-chain triglycerides (LCTs) to approximately 10% of total energy intake [18,19] while supplementing with medium-chain triglycerides (MCT oil or MCT-enhanced formula) at about 20% of total energy intake [9]. A newer treatment option for LC-FAOD is triheptanoin. This synthetic medium-chain odd-chain triglyceride was approved for patient use in the United States in 2020 but is only available through compassionate programmes in other countries [14,20]. Patients with MCAD and short-chain acyl-CoA dehydrogenase deficiency (SCAD) are advised to avoid fasting [21], and dietary fat restrictions are not recommended [9]. Patients with carnitine uptake defect (CUD) require carnitine supplementation and fasting avoidance [12].
The dietary modifications required in FAOD may place patients at risk of nutritional disturbances. It is well established that exclusion or modified diets used to treat certain diseases predispose patients to micro- and macronutrient deficiencies and disturbances in nutritional state [22,23,24]. Patients with LC-FAOD, who must follow significant restrictions in fat intake, are unable to consume many natural sources of fat-soluble nutrients such as plant oils, nuts, fish, full-fat dairy, and eggs [25]. Poor intake of nutrients, including vitamins K and E and essential fatty acids (linoleic and linolenic acid), has previously been documented in children with LCHAD [18]. Some guidelines [26,27] and reviews [28] highlight the possibility of deficiencies in fat-soluble micronutrients; however, robust scientific evidence is lacking.
We hypothesised that FAOD patients are more likely to experience disturbances in the status of vitamins A, D, E, and β-carotene. Therefore, we aimed to assess fat-soluble vitamin concentrations in FAOD patients compared with healthy subjects and to examine the differences within the FAOD group according to dietary management strategy (fat-modified diet versus standard-fat diet).

2. Materials and Methods

2.1. Ethical Approval

This cross-sectional study [29] was conducted between 2020 and 2025. The study protocol was approved by the Poznań University of Medical Sciences Ethical Committee (approval no. 803/20), and all procedures were performed in accordance with the Helsinki Declaration of 1975 [30].

2.2. Study Population

The FAOD patients were recruited at the Department of Pediatric Gastroenterology and Metabolic Diseases at the University of Medical Sciences in Poznań, Greater Poland Voivodeship, Poland, between November 2020 and February 2025. Blood acylcarnitine assays, urinary organic acid profiles, and genetic testing confirmed the diagnosis of FAOD in all patients [16,31,32]. Patients born after December 2013 were diagnosed through NBS [33]. Inclusion criteria included age (1–20 years) and willingness to participate. Exclusion criteria included current metabolic decompensation, acute illness, or pregnancy. Due to the exploratory nature of the study, all candidates who expressed willingness to participate were enrolled.
Sex- and age-matched control subjects (aged 1 to 20 years) were recruited through a family medicine practice clinic in Wronki, Greater Poland Voivodeship, Poland, between September 2024 and February 2025. Exclusion criteria included the presence of acute or chronic disease and mental or physical disability. Information regarding the use of vitamins and dietary supplements was obtained from all participants. Written and oral consent was obtained from all study participants and their legal guardians.

2.3. Subgroup Analysis

To assess whether there were differences between FAOD patients treated with a diet restricted in LCTs and those receiving a standard-fat diet, the patients were divided into two subgroups:
  • Fat-modified diet group—patients treated with LCTs restriction: 9 patients with LCHAD and 5 patients with VLCAD.
  • Standard-fat diet group—patients managed with fasting avoidance (16 patients with MCAD and 1 patient with SCAD) and carnitine (5 patients with CUD).

2.4. Anthropometric Measurements

Measurements were taken in the morning by study physicians, in light clothing and without shoes, using Seca 703 scales (Hamburg, Germany) for participants > 2 years of age and Seca 376 scales for participants < 2 years of age. Weight was measured with an accuracy of 0.1 kg and height with an accuracy of 0.1 cm. Based on these measurements, BMI was calculated with a precision of 0.1 kg/m2.
To determine the percentiles of body weight, height, and BMI in study participants under 18, WHO growth charts were used for children < 3 years of age [34] and OLA/OLAF growth charts for children aged 3–18 [35].

2.5. Blood Sampling

Venous blood was drawn after a fasting period of 10 h from the vein of the antecubital fossa. The length of fasting time did not exceed the recommended safe periods for FAOD patients [26,27,36,37]. The blood samples were protected from light with aluminum foil, centrifuged, aliquoted, and stored at −80 °C until analysis.

2.6. Biochemical Analysis

Vitamins A, E, and β-carotene were measured at the Laboratory of the Department of Pediatric Gastroenterology and Metabolic Diseases using high-performance liquid chromatography (HPLC; Hewlett-Packard 1100 Series HPLC System, Wladbronn, Germany) as previously described [38,39]. The reference ranges were 300–750 ng/mL for vitamin A [40,41], 5.7–19.920 µg/mL for vitamin E [42], and 30–910 µg/mL for β-carotene [43,44]. Vitamin 25(OH)D concentrations were measured in a commercial laboratory (Diagnostyka S.A., Poznan, Poland) using an immunoassay on the Alinity i analyser (Abbott, Chicago, IL, USA). Vitamin 25(OH)D status was assessed per the Polish Guidelines for Preventing and Treating vitamin D Deficiency, according to which 25(OH)D < 20 ng/mL indicates deficiency; 20–30 ng/mL suboptimal status; 30–50 ng/mL adequate to optimal; 50–100 ng/mL high supply; and >100 ng/mL increased intoxication risk [45]

2.7. Data Quality and Bias Control

We applied several strategies to control for bias, including a prospective study design, clear and consistent inclusion and exclusion criteria, standardised measurement protocols and validated instruments operated by experienced, well-trained staff, predefined outcomes and endpoints, a pre-specified statistical analysis plan, and reporting in accordance with the STROBE guidelines. There were no missing data, as all eligible FAOD patients who attended our metabolic centre during the study period were consecutively enrolled. All assessed markers were available for both patients and healthy controls. Population-based controls were recruited from the same geographic area as the FAOD group (Greater Poland Voivodeship). Furthermore, propensity score matching for age and sex was used to select the control group.

2.8. Sample Size Calculation

Sample size estimation was performed using G*Power 3.1 (Heinrich Heine University Düsseldorf, Düsseldorf, Germany). The calculation was based on anticipated differences in serum vitamin 25(OH)D concentrations between the study groups and assumed a two-sided significance level of 0.05, a statistical power of 80%, a mean difference of 20% between groups, a standard deviation estimated at 30% of the mean, and equal allocation of participants to each group (1:1). Based on these assumptions, a minimum of 30 participants per group were required. To account for a potential dropout rate of up to 10%, the target recruitment was increased to 34 participants per group to maintain sufficient statistical power.

2.9. Statistical Analysis

Continuous data are presented as means (M) with standard deviations (SD), medians (Me), and interquartile ranges (Q1–Q3). Categorical variables are presented as absolute and relative frequencies.
Controls were selected using propensity score matching, adjusting for age and sex. The Shapiro–Wilk test was used to assess the normality of the parameters. When the parameters followed a Gaussian distribution, the equality of variances was tested using Levene’s test. If the assumption of equal variances was met, Student’s t-test was used to test for differences between the two groups; otherwise, Welch’s t-test was used. When a non-normal distribution of data was observed, the Mann–Whitney U test with continuity correction was used to compare the groups. A Chi2 test was used to check for differences in sex distribution. p < 0.05 was considered statistically significant.
To analyse the associations between categorical variables, the likelihood-ratio Chi2, Pearson’s Chi2, and Fisher’s exact test (two-sided) were used. Statistica Software v.14 (TIBCO Software Inc., Palo Alto, CA, USA) was used for all calculations.

3. Results

3.1. Clinical Characteristics

The sample selection flowchart (Figure 1) outlines the recruitment process. We have included 36 out of 37 FAOD patients (one patient declined to participate). A total of 122 individuals were initially screened for inclusion in the control group. Of these, 20 were excluded: 5 declined to participate, and 15 did not meet the inclusion criteria. The reasons for exclusion due to lack of eligibility included age (n = 9), chronic disease (n = 3), acute infection (n = 2), and mental disability (n = 1). As a result, 102 participants were available for the control group. Propensity score matching was subsequently performed, resulting in a final matched control group of 36 participants. Table 1 summarises population characteristics. The FAOD group consisted of 16 patients with MCAD (44%), 9 with LCHAD (25%), 5 with VLCAD (14%), 5 with CUD (14%), and 1 with SCAD (3%). Four patients (12%) were born before the introduction of NBS in Poland. There were no statistically significant differences in any of the recorded anthropometric parameters between the FAOD group and the control group.

3.2. Vitamin Concentrations—FAOD vs. Control Group

The findings are summarised in Table 1. No significant differences in serum concentrations of vitamin 25(OH)D, vitamin E, or β-carotene were observed between the FAOD group and the control group. The FAOD group exhibited a significantly higher vitamin A concentration.
At the individual level, 11.1% of the FAOD group had vitamin 25(OH)D deficiency (<20 ng/mL), compared with 5.6% of controls. Suboptimal vitamin 25(OH)D levels (20–30 ng/mL) were observed in 30.6% and 36.1% of subjects, respectively. No participant had a potentially toxic vitamin 25(OH)D concentration (>100 ng/mL). Vitamin A levels were below the reference range in 8.3% of the FAOD group compared with 19.4% of controls; one control participant (2.8%) exceeded 750 ng/mL. β-carotene concentrations were below the reference range in 22.2% of the FAOD group and 5.6% of controls. All but one FAOD patient (2.7%) had vitamin E concentrations within the reference range. Overall, no statistically significant differences in the prevalence of excessive, normal, or deficient levels of vitamin A (p = 0.18), D (p = 0.65), E (p = 1.00), and β-carotene (p = 0.08) were observed between FAOD patients and controls.

3.3. Vitamin Concentrations—Fat-Modified Group vs. Standard-Fat Group

The comparison of vitamin concentrations between the fat-modified group and the standard-fat group is presented in Table 2. There were no statistically significant differences in sex structure or anthropometric parameters between the groups. Statistically significant differences in the serum concentrations of all fat-soluble vitamins were observed. Vitamin 25(OH)D and vitamin A concentrations were higher in the fat-modified group. In contrast, the concentrations of vitamin E and β-carotene were significantly higher in the standard-fat diet group.
No patients on a fat-restricted diet had vitamin 25(OH)D deficiency (<20 ng/mL) compared with 18.2% on a non-fat-restricted diet. Suboptimal 25(OH)D levels (20–30 ng/mL) were observed in 14.3% vs. 40.9% subjects, respectively. No participant in either group exhibited a toxic level of vitamin 25(OH)D; however, 13.6% of patients in the non-fat-restricted group had vitamin A deficiency, and the remaining subjects with FAOD had normal vitamin A levels. β-carotene deficiency was common, 36.7% in the fat-restricted group and 13.6% in the non-fat-restricted group. Vitamin E values were normal in all but one FAOD patient (7.14%). In summary, vitamin 25(OH)D deficiency was statistically more prevalent in the standard-fat diet group (p = 0.03). No statistically significant differences were found between the fat-modified group and the standard-fat group in the prevalence of excessive or deficient levels of vitamins A (p = 0.27), E (p = 0.39) and β-carotene (p = 1.0).

4. Discussion

Our study investigated the serum concentrations of fat-soluble vitamins in patients with FOAD and compared them to those of healthy control subjects. While no statistically significant differences were observed in the concentrations of vitamin 25(OH)D, E, and β-carotene, the FAOD group exhibited significantly higher vitamin A levels.
Interestingly, patients on the fat-modified diet had significantly higher concentrations of vitamins A and 25(OH)D but lower concentrations of vitamin E and β-carotene than those on the standard-fat diet. We observed a higher percentage of deficient and suboptimal vitamin 25(OH)D levels in the standard-fat diet group compared with the fat-restricted diet group (59.1% vs. 14.3%). There was also a high percentage of decreased β-carotene concentrations in the fat-modified group compared to the standard-fat diet group (50% vs. 22.7%, respectively). In the standard fat group, three patients (13.6%) had decreased vitamin A concentrations; in both groups, vitamin A and E were within reference ranges.
To date, micronutrient deficiencies in FAOD have been poorly studied in the scientific literature. One study by Gillingham et al. [18] revealed deficient intake of fat-soluble vitamins E and K, assessed by a three-day diet record. VLCAD treatment guidelines recommend assessing fat-soluble vitamin levels in patients due to the risk of deficiency [27].
The higher concentrations of vitamins A and 25(OH)D observed in the fat-modified diet group are likely due to the regular use of formulas (Table 3) and supplements. Fortified medical foods (such as Modulen or Lipistart) contain substantial amounts of vitamins A and D. Supplements used by some patients, particularly fish liver oil, are a natural source of both vitamins [46,47]. In the fat-modified group, 9 (64.3%) patients used formula, including 4 (28.6%) who additionally supplemented with vitamin D and 3 (21.4%) who used fish oil preparations. One (7.1%) patient took multiple vitamin preparation and one (7.1%) a vitamin D supplement. Considering vitamin use, 10 (71.4%) patients received supplementary doses of vitamin A, D, and E, and 1 (7.1%) subject received vitamin D. In the standard-fat diet group, 12 (54.5%) patients were supplemented with vitamin D preparations and 2 (9.1%) with fish oil. Considering vitamin use, 2 patients (9.1%) received vitamins A, D, and E, whereas 12 patients (54.5%) received only vitamin D. In the control group, 21 (61.8%) subjects were supplemented with vitamin D. The frequency of vitamin D supplementation was not significantly higher in the fat-modified group (78.5% vs. 63.6% vs. 61.8%). However, considering formula intake, the amount consumed from additional sources was definitely higher. Vitamin A and E supplementary intake was significantly more frequent in the fat-modified group than in the standard-fat diet (71.4% vs. 16.7%, p = 0.0002).
Several factors may have contributed to lower vitamin E concentrations in the fat-modified group compared to the standard-fat group. Firstly, patients are restricted to provide only 10% of their total energy intake from LCTs [36], which limits their ability to consume foods naturally rich in vitamin E, such as vegetable oils and nuts. The bioavailability of tocopherol is enhanced by the fat content of the meal [48,49]. Consistently, tocopherol acetate (a vitamin E precursor) showed greater bioaccessibility in long-chain than in medium-chain triglyceride emulsions [50]. Given that patients in the fat-modified group use 100% MCT oil for cooking, the intake and absorption of vitamin E may be reduced. Furthermore, the bioavailability of vitamin E is influenced by the food matrix of the meal. Research has demonstrated that vitamin E bioaccessibility varies depending on the type of produce, being very low in apples but almost complete in bananas, bread, or lettuce [51], and differing between pasta types depending on the egg content [52]. Similarly, Cilla et al. [53] reported greater α-tocopherol bioaccessibility in cow milk-based fruit beverages than in soy-based drinks. A study by Jeanes et al. [48] found that both the amount of fat and the meal matrix significantly influenced α-tocopherol absorption.
Lower concentrations of β-carotene in the fat-modified diet group could be explained by both decreased bioavailability from meals and lack of supplementation. Medical foods consumed by patients on the fat-modified diet do not contain β-carotene. Furthermore, the bioaccessibility of β-carotene increases significantly (from 14% to 86%) with increasing LCT fraction (from 0% to 100%) [54]. It was documented that the bioaccessibility of β-carotene from carrots [55] and peppers [56] was significantly higher when co-digested with emulsions containing long-chain fatty acids than with emulsions based on medium- or short-chain fatty acids. Therefore, as in the case of vitamin E, the absorption of β-carotene may be affected negatively by the LCT restriction [57]. The food matrix and its processing influence β-carotene bioavailability. Disruption of plant cell walls enhances carotenoid release and micellarization, thereby improving bioavailability. In vitro studies have shown that the homogenization and cooking of carrots increased β-carotene accessibility from 3% in raw samples to 27–39% when cooked and combined with oil [58]. Furthermore, the ingestion of carrot juice caused a two times the increase in plasma β-carotene compared with raw carrots [59]. In addition, the patients did not receive β-carotene supplementation.
Our study’s strengths include a robust sample size (compared with other case-control studies of patients with IEM) and a well-matched control group. The limitations of this study include the lack of a detailed, long-term quantitative assessment of dietary intake and supplementation, which could be correlated with serum vitamin concentrations. The evaluation of meal patterns and preparation techniques that affect the bioaccessibility of fat-soluble nutrients in patients with FOAD would allow for a more robust analysis. Interpretation of the results is hampered by the lack of widely accepted reference ranges and by the limited number of studies establishing normative vitamin A and β-carotene concentrations in healthy children. Increasing the sample size of LC-FAOD patients could improve subgroup analysis.

5. Conclusions

Generally, FAOD patients have higher vitamin A concentrations than healthy controls. Interestingly, fat-modified diets were associated with higher vitamin A and 25(OH)D concentrations and lower vitamin E and β-carotene levels compared to a standard-fat diet, reflecting the combined impact of supplementation, food fortification, and reduced bioavailability under LCT restriction. These findings emphasise the importance of tailored nutritional and supplementation monitoring in FAOD and warrant larger studies with detailed dietary assessments. In particular, future work should focus on optimising nutritional management, including modifications to formula composition, and on addressing the currently limited evidence on nutritional status and vitamin deficiencies in patients with FAOD.

Author Contributions

Conceptualization, J.W. and M.J.; methodology, J.W., M.W.-G., Ł.K. and M.J.; validation, J.W. and M.W.-G.; formal analysis, M.J.; investigation, M.W.-G.; resources, N.W.-T., M.W.-G., A.B.-O., M.D.-Ż., J.J. and Ł.K.; data curation, M.W.-G., N.W.-T., S.K., A.M.-C. and M.J.; writing—original draft preparation, M.W.-G.; writing—review and editing, J.W., M.W.-G. and M.J.; visualisation, M.W.-G. and N.W.-T.; supervision, J.W.; project administration, J.W. All authors have read and agreed to the published version of the manuscript.

Funding

This research received no external funding.

Institutional Review Board Statement

The study was conducted in accordance with the Declaration of Helsinki and approved by the Ethics Committee) of Poznan University of Medical Sciences (No. of approval 803/20, approval date 4 November 2020).

Informed Consent Statement

Informed consent was obtained from all subjects involved in the study.

Data Availability Statement

The original contributions presented in this study are included in the article. Further inquiries can be directed to the corresponding author.

Conflicts of Interest

The authors declare no conflicts of interest.

Abbreviations

BMIbody mass index
CACTcarnitine–acylcarnitine translocase
CPT1carnitine palmitoyltransferase I deficiency
CUDcarnitine uptake defect
25(OH)Dvitamin 25(OH)D
FAODfatty acid oxidation disorders
HPLChigh-performance liquid chromatography
LCHADlong-chain 3-hydroxyacyl-CoA dehydrogenase deficiency
LCTlong-chain triglycerides
MCADmedium-chain acyl-CoA dehydrogenase
MCTmedium-chain triglycerides
Memedian
NBSnewborn screening
OLA/OLAFgrowth charts for the Polish population aged 3–18
Q1–Q3quartile 1–quartile 3
SCADshort-chain acyl-CoA dehydrogenase deficiency
SDstandard deviation
VLCADvery long-chain acyl-CoA dehydrogenase deficiency
WHOWorld Health Organization

References

  1. Knottnerus, S.J.G.; Bleeker, J.C.; Wüst, R.C.I.; Ferdinandusse, S.; IJlst, L.; Wijburg, F.A.; Wanders, R.J.A.; Visser, G.; Houtkooper, R.H. Disorders of Mitochondrial Long-Chain Fatty Acid Oxidation and the Carnitine Shuttle. Rev. Endocr. Metab. Disord. 2018, 19, 93–106. [Google Scholar] [CrossRef]
  2. Guerra, I.M.S.; Ferreira, H.B.; Melo, T.; Rocha, H.; Moreira, S.; Diogo, L.; Domingues, M.R.; Moreira, A.S.P. Mitochondrial Fatty Acid β-Oxidation Disorders: From Disease to Lipidomic Studies—A Critical Review. Int. J. Mol. Sci. 2022, 23, 13933. [Google Scholar] [CrossRef]
  3. Peña-Quintana, L.; Correcher-Medina, P. Nutritional Management of Patients with Fatty Acid Oxidation Disorders. Nutrients 2024, 16, 2707. [Google Scholar] [CrossRef]
  4. Sebaa, R.; AlMalki, R.H.; Alseraty, W.; Abdel Rahman, A.M. A Distinctive Metabolomics Profile and Potential Biomarkers for Very Long Acylcarnitine Dehydrogenase Deficiency (VLCADD) Diagnosis in Newborns. Metabolites 2023, 13, 725. [Google Scholar] [CrossRef]
  5. Merritt, J.L., II; Norris, M.; Kanungo, S. Fatty Acid Oxidation Disorders. Ann. Transl. Med. 2018, 6, 473. [Google Scholar] [CrossRef]
  6. Vianey-Saban, C.; Guffon, N.; Fouilhoux, A.; Acquaviva, C. Fifty Years of Research on Mitochondrial Fatty Acid Oxidation Disorders: The Remaining Challenges. J. Inherit. Metab. Dis. 2023, 46, 848–873. [Google Scholar] [CrossRef]
  7. Marsden, D.; Bedrosian, C.L.; Vockley, J. Impact of Newborn Screening on the Reported Incidence and Clinical Outcomes Associated with Medium- and Long-Chain Fatty Acid Oxidation Disorders. Genet. Med. 2021, 23, 816–829. [Google Scholar] [CrossRef] [PubMed]
  8. Al Bandari, M.; Nagy, L.; Cruz, V.; Hewson, S.; Hossain, A.; Inbar-Feigenberg, M. Management and Outcomes of Very Long-Chain Acyl-CoA Dehydrogenase Deficiency (VLCAD Deficiency): A Retrospective Chart Review. Int. J. Neonatal Screen. 2024, 10, 29. [Google Scholar] [CrossRef] [PubMed]
  9. Merritt, J.L.; MacLeod, E.; Jurecka, A.; Hainline, B. Clinical Manifestations and Management of Fatty Acid Oxidation Disorders. Rev. Endocr. Metab. Disord. 2020, 21, 479–493. [Google Scholar] [CrossRef]
  10. Kruger, E.; McNiven, P.; Marsden, D. Estimating the Prevalence of Rare Diseases: Long-Chain Fatty Acid Oxidation Disorders as an Illustrative Example. Adv. Ther. 2022, 39, 3361–3377. [Google Scholar] [CrossRef] [PubMed]
  11. Spiekerkoetter, U. Mitochondrial Fatty Acid Oxidation Disorders: Clinical Presentation of Long-Chain Fatty Acid Oxidation Defects before and after Newborn Screening. J. Inherit. Metab. Dis. 2010, 33, 527–532. [Google Scholar] [CrossRef]
  12. Maguolo, A.; Rodella, G.; Dianin, A.; Nurti, R.; Monge, I.; Rigotti, E.; Cantalupo, G.; Salviati, L.; Tucci, S.; Pellegrini, F.; et al. Diagnosis, Genetic Characterization and Clinical Follow up of Mitochondrial Fatty Acid Oxidation Disorders in the New Era of Expanded Newborn Screening: A Single Centre Experience. Mol. Genet. Metab. Rep. 2020, 24, 100632. [Google Scholar] [CrossRef]
  13. Yoo, H.-W. Inborn Errors of Mitochondrial Fatty Acid Oxidation: Overview from a Clinical Perspective. J. Lipid Atheroscler. 2021, 10, 1. [Google Scholar] [CrossRef]
  14. Vockley, J.; Burton, B.; Berry, G.; Longo, N.; Phillips, J.; Sanchez-Valle, A.; Chapman, K.; Tanpaiboon, P.; Grunewald, S.; Murphy, E.; et al. Effects of Triheptanoin (UX007) in Patients with Long-chain Fatty Acid Oxidation Disorders: Results from an Open-label, Long-term Extension Study. J. Inherit. Metab. Dis. 2021, 44, 253–263. [Google Scholar] [CrossRef]
  15. Akan, G.; Balcı, M.C.; Tuncel, G.; Karaca, M.; Kazan, H.H.; Özketen, A.Ç.; Özgen, Ö.; Gökçay, G.F.; Atalar, F. Development and Validation of a Carnitine Cycle and Transport Disorders (CCD) Panel: An ONT-Compatible Multi-Gene Diagnostic Kit for Newborn and Selective Screening. Orphanet J. Rare Dis. 2025, 20, 250. [Google Scholar] [CrossRef] [PubMed]
  16. Vianey-Saban, C.; Fouilhoux, A.; Vockley, J.; Acquaviva-Bourdain, C.; Guffon, N. Improving Diagnosis of Mitochondrial Fatty-Acid Oxidation Disorders. Eur. J. Hum. Genet. 2023, 31, 265–272. [Google Scholar] [CrossRef]
  17. Baker, J.J.; Burton, B.K. Diagnosis and Clinical Management of Long-Chain Fatty-Acid Oxidation Disorders: A Review. TouchREVIEWS Endocrinol. 2021, 17, 108–111. [Google Scholar] [CrossRef]
  18. Gillingham, M.B.; Connor, W.E.; Matern, D.; Rinaldo, P.; Burlingame, T.; Meeuws, K.; Harding, C.O. Optimal Dietary Therapy of Long-Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency. Mol. Genet. Metab. 2003, 79, 114–123. [Google Scholar] [CrossRef]
  19. Jones, P.M.; Butt, Y.; Bennett, M.J. Accumulation of 3-Hydroxy-Fatty Acids in the Culture Medium of Long-Chain L-3-Hydroxyacyl CoA Dehydrogenase (LCHAD) and Mitochondrial Trifunctional Protein-Deficient Skin Fibroblasts: Implications for Medium Chain Triglyceride Dietary Treatment of LCHAD Deficiency. Pediatr. Res. 2003, 53, 783–787. [Google Scholar] [CrossRef] [PubMed]
  20. Porta, F.; Maiorana, A.; Gragnaniello, V.; Procopio, E.; Gasperini, S.; Taurisano, R.; Spada, M.; Dionisi-Vici, C.; Burlina, A. Triheptanoin in Patients with Long-Chain Fatty Acid Oxidation Disorders: Clinical Experience in Italy. Ital. J. Pediatr. 2024, 50, 204. [Google Scholar] [CrossRef] [PubMed]
  21. Mason, E.; Hindmarch, C.C.T.; Dunham-Snary, K.J. Medium-Chain Acyl-COA Dehydrogenase Deficiency: Pathogenesis, Diagnosis, and Treatment. Endocrinol. Diabetes Metab. 2023, 6, e385. [Google Scholar] [CrossRef]
  22. Kreutz, J.M.; Heynen, L.; Vreugdenhil, A.C.E. Nutrient Deficiencies in Children with Celiac Disease during Long Term Follow-Up. Clin. Nutr. Edinb. Scotl. 2023, 42, 1175–1180. [Google Scholar] [CrossRef]
  23. Mehta, H.; Groetch, M.; Wang, J. Growth and Nutritional Concerns in Children with Food Allergy. Curr. Opin. Allergy Clin. Immunol. 2013, 13, 275–279. [Google Scholar] [CrossRef]
  24. Black, R.E.; Williams, S.M.; Jones, I.E.; Goulding, A. Children Who Avoid Drinking Cow Milk Have Low Dietary Calcium Intakes and Poor Bone Health123. Am. J. Clin. Nutr. 2002, 76, 675–680. [Google Scholar] [CrossRef]
  25. Reddy, P.; Jialal, I. Biochemistry, Fat Soluble Vitamins. In StatPearls; StatPearls Publishing: Treasure Island, FL, USA, 2025. [Google Scholar]
  26. Spiekerkoetter, U.; Lindner, M.; Santer, R.; Grotzke, M.; Baumgartner, M.R.; Boehles, H.; Das, A.; Haase, C.; Hennermann, J.B.; Karall, D.; et al. Treatment Recommendations in Long-Chain Fatty Acid Oxidation Defects: Consensus from a Workshop. J. Inherit. Metab. Dis. 2009, 32, 498–505. [Google Scholar] [CrossRef]
  27. Van Calcar, S.C.; Sowa, M.; Rohr, F.; Beazer, J.; Setlock, T.; Weihe, T.U.; Pendyal, S.; Wallace, L.S.; Hansen, J.G.; Stembridge, A.; et al. Nutrition Management Guideline for Very-Long Chain Acyl-CoA Dehydrogenase Deficiency (VLCAD): An Evidence- and Consensus-Based Approach. Mol. Genet. Metab. 2020, 131, 23–37. [Google Scholar] [CrossRef]
  28. Tummolo, A.; Carella, R.; De Giovanni, D.; Paterno, G.; Simonetti, S.; Tolomeo, M.; Leone, P.; Barile, M. Micronutrient Deficiency in Inherited Metabolic Disorders Requiring Diet Regimen: A Brief Critical Review. Int. J. Mol. Sci. 2023, 24, 17024. [Google Scholar] [CrossRef] [PubMed]
  29. Nowak, J.K.; Walkowiak, J. Study Designs in Medical Research and Their Key Characteristics. J. Med. Sci. 2023, 92, e928. [Google Scholar] [CrossRef]
  30. Sawicka-Gutaj, N.; Gruszczyński, D.; Guzik, P.; Mostowska, A.; Walkowiak, J. Publication Ethics of Human Studies in the Light of the Declaration of Helsinki—A Mini-Review. J. Med. Sci. 2022, 91, e700. [Google Scholar] [CrossRef]
  31. Leslie, N.D.; Saenz-Ayala, S. Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency. In GeneReviews®; Adam, M.P., Feldman, J., Mirzaa, G.M., Pagon, R.A., Wallace, S.E., Amemiya, A., Eds.; University of Washington: Seattle, WA, USA, 1993. [Google Scholar]
  32. Prasun, P.; LoPiccolo, M.K.; Ginevic, I. Long-Chain Hydroxyacyl-CoA Dehydrogenase Deficiency/Trifunctional Protein Deficiency. In GeneReviews®; Adam, M.P., Feldman, J., Mirzaa, G.M., Pagon, R.A., Wallace, S.E., Amemiya, A., Eds.; University of Washington: Seattle, WA, USA, 1993. [Google Scholar]
  33. Wszystkie Noworodki w Polsce Zostały Objęte Badaniem Przesiewowym w Kierunku Rzadkich Wad Metabolizmu—Institute of Mother and Child. Available online: https://imid.med.pl/en/409/wszystkie-noworodki-w-polsce-zostaly-objete-badaniem-przesiewowym-w-kierunku-rzadkich-wad-metabolizmu (accessed on 28 August 2022).
  34. The WHO Child Growth Standards. Available online: https://www.who.int/tools/child-growth-standards/standards (accessed on 15 August 2022).
  35. Kulaga, Z.; Litwin, M.; Tkaczyk, M.; Różdżyńska, A.; Barwicka, K.; Grajda, A.; Świąder, A.; Gurzkowska, B.; Napieralska, E.; Pan, H. The Height-, Weight-, and BMI-for-Age of Polish School-Aged Children and Adolescents Relative to International and Local Growth References. BMC Public Health 2010, 10, 109. [Google Scholar] [CrossRef]
  36. VLCAD Nutrition Management Guidelines. Available online: https://managementguidelines.net/guidelines.php/146/nr/32/summary/VLCAD%20Nutrition%20Guidelines/Version%201.4/#sd (accessed on 12 October 2025).
  37. Derks, T.G.J.; van Spronsen, F.J.; Rake, J.P.; van der Hilst, C.S.; Span, M.M.; Smit, G.P.A. Safe and Unsafe Duration of Fasting for Children with MCAD Deficiency. Eur. J. Pediatr. 2007, 166, 5–11. [Google Scholar] [CrossRef]
  38. Sapiejka, E.; Krzyżanowska, P.; Walkowiak, D.; Wenska-Chyży, E.; Szczepanik, M.; Cofta, S.; Pogorzelski, A.; Skorupa, W.; Walkowiak, J. Vitamin A Status and Its Determinants in Patients with Cystic Fibrosis. Acta Sci. Pol. Technol. Aliment. 2017, 16, 345–354. [Google Scholar] [CrossRef] [PubMed]
  39. Sapiejka, E.; Krzyżanowska-Jankowska, P.; Wenska-Chyży, E.; Szczepanik, M.; Walkowiak, D.; Cofta, S.; Pogorzelski, A.; Skorupa, W.; Walkowiak, J. Vitamin E Status and Its Determinants in Patients with Cystic Fibrosis. Adv. Med. Sci. 2018, 63, 341–346. [Google Scholar] [CrossRef]
  40. Raizman, J.E.; Cohen, A.H.; Teodoro-Morrison, T.; Wan, B.; Khun-Chen, M.; Wilkenson, C.; Bevilaqua, V.; Adeli, K. Pediatric Reference Value Distributions for Vitamins A and E in the CALIPER Cohort and Establishment of Age-Stratified Reference Intervals. Clin. Biochem. 2014, 47, 812–815. [Google Scholar] [CrossRef]
  41. Johnson-Davis, K.L.; Moore, S.J.; Owen, W.E.; Cutler, J.M.; Frank, E.L. A Rapid HPLC Method Used to Establish Pediatric Reference Intervals for Vitamins A and E. Clin. Chim. Acta Int. J. Clin. Chem. 2009, 405, 35–38. [Google Scholar] [CrossRef]
  42. Le, N.K.; Kesayan, T.; Chang, J.Y.; Rose, D.Z. Cryptogenic Intracranial Hemorrhagic Strokes Associated with Hypervitaminosis E and Acutely Elevated α-Tocopherol Levels. J. Stroke Cerebrovasc. Dis. 2020, 29, 104747. [Google Scholar] [CrossRef]
  43. FCATB—Overview: Carotene, Beta. Available online: https://www.mayocliniclabs.com/test-catalog/overview/75927#Clinical-and-Interpretive (accessed on 2 October 2025).
  44. 001529: Carotene, β. Available online: https://www.labcorp.com/content/labcorp/us/en/test-menu/search/test-details (accessed on 2 October 2025).
  45. Płudowski, P.; Kos-Kudła, B.; Walczak, M.; Fal, A.; Zozulińska-Ziółkiewicz, D.; Sieroszewski, P.; Peregud-Pogorzelski, J.; Lauterbach, R.; Targowski, T.; Lewiński, A.; et al. Guidelines for Preventing and Treating Vitamin D Deficiency: A 2023 Update in Poland. Nutrients 2023, 15, 695. [Google Scholar] [CrossRef] [PubMed]
  46. Noreen, S.; Hashmi, B.; Aja, P.M.; Atoki, A.V. Health Benefits of Fish and Fish By-Products—A Nutritional and Functional Perspective. Front. Nutr. 2025, 12, 1564315. [Google Scholar] [CrossRef] [PubMed]
  47. Andrès, E.; Lorenzo-Villalba, N.; Terrade, J.-E.; Méndez-Bailon, M. Fat-Soluble Vitamins A, D, E, and K: Review of the Literature and Points of Interest for the Clinician. J. Clin. Med. 2024, 13, 3641. [Google Scholar] [CrossRef]
  48. Jeanes, Y.M.; Hall, W.L.; Ellard, S.; Lee, E.; Lodge, J.K. The Absorption of Vitamin E Is Influenced by the Amount of Fat in a Meal and the Food Matrix. Br. J. Nutr. 2004, 92, 575–579. [Google Scholar] [CrossRef]
  49. Traber, M.G.; Leonard, S.W.; Ebenuwa, I.; Violet, P.-C.; Wang, Y.; Niyyati, M.; Padayatty, S.; Tu, H.; Courville, A.; Bernstein, S.; et al. Vitamin E Absorption and Kinetics in Healthy Women, as Modulated by Food and by Fat, Studied Using 2 Deuterium-Labeled α-Tocopherols in a 3-Phase Crossover Design. Am. J. Clin. Nutr. 2019, 110, 1148–1167. [Google Scholar] [CrossRef]
  50. Reboul, E. Vitamin E Bioavailability: Mechanisms of Intestinal Absorption in the Spotlight. Antioxid. Basel Switz. 2017, 6, 95. [Google Scholar] [CrossRef] [PubMed]
  51. Reboul, E.; Richelle, M.; Perrot, E.; Desmoulins-Malezet, C.; Pirisi, V.; Borel, P. Bioaccessibility of Carotenoids and Vitamin E from Their Main Dietary Sources. J. Agric. Food Chem. 2006, 54, 8749–8755. [Google Scholar] [CrossRef]
  52. Werner, S.; Böhm, V. Bioaccessibility of Carotenoids and Vitamin e from Pasta: Evaluation of an in Vitro Digestion Model. J. Agric. Food Chem. 2011, 59, 1163–1170. [Google Scholar] [CrossRef]
  53. Cilla, A.; Alegría, A.; de Ancos, B.; Sánchez-Moreno, C.; Cano, M.P.; Plaza, L.; Clemente, G.; Lagarda, M.J.; Barberá, R. Bioaccessibility of Tocopherols, Carotenoids, and Ascorbic Acid from Milk- and Soy-Based Fruit Beverages: Influence of Food Matrix and Processing. J. Agric. Food Chem. 2012, 60, 7282–7290. [Google Scholar] [CrossRef] [PubMed]
  54. Salvia-Trujillo, L.; Qian, C.; Martín-Belloso, O.; McClements, D.J. Modulating β-Carotene Bioaccessibility by Controlling Oil Composition and Concentration in Edible Nanoemulsions. Food Chem. 2013, 139, 878–884. [Google Scholar] [CrossRef] [PubMed]
  55. Zhang, R.; Zhang, Z.; Zou, L.; Xiao, H.; Zhang, G.; Decker, E.A.; McClements, D.J. Enhancing Nutraceutical Bioavailability from Raw and Cooked Vegetables Using Excipient Emulsions: Influence of Lipid Type on Carotenoid Bioaccessibility from Carrots. J. Agric. Food Chem. 2015, 63, 10508–10517. [Google Scholar] [CrossRef]
  56. Liu, X.; Bi, J.; Xiao, H.; McClements, D.J. Increasing Carotenoid Bioaccessibility from Yellow Peppers Using Excipient Emulsions: Impact of Lipid Type and Thermal Processing. J. Agric. Food Chem. 2015, 63, 8534–8543. [Google Scholar] [CrossRef]
  57. Yan, X.; Huang, J.; Huang, L.; Luo, C.; Li, Z.; Xu, P.; Tan, K.; Cheong, K.-L.; Tan, K. Effects of Dietary Lipids on Bioaccessibility and Bioavailability of Natural Carotenoids. LWT 2024, 200, 116171. [Google Scholar] [CrossRef]
  58. Hedrén, E.; Mulokozi, G.; Svanberg, U. In Vitro Accessibility of Carotenes from Green Leafy Vegetables Cooked with Sunflower Oil or Red Palm Oil—PubMed. Int. J. Food Sci. Nutr. 2002, 53, 445–453. [Google Scholar]
  59. Choi, M.; Baek, J.; Park, E. Comparative Bioavailability of β-Carotene from Raw Carrots and Fresh Carrot Juice in Humans: A Crossover Study. Nutr. Res. Pract. 2025, 19, 215–224. [Google Scholar] [CrossRef] [PubMed]
Metabolites 16 00124 g001
Figure 1. Sample selection flowchart. FAOD—fatty acid oxidation disorders.
Figure 1. Sample selection flowchart. FAOD—fatty acid oxidation disorders.
Metabolites 16 00124 g001
Table 1. Characteristics of the study population.
Table 1. Characteristics of the study population.
FAOD
n = 36		Control
n = 36		p
Age (years) 0.84 *
Mean ± SD7.9 ± 4.58.1 ± 4.6
Median7.27.8
Q1–Q3(4.6–11.6)(4.6–11.7)
Sex (n (%)) 0.81 #
Females20 (55.6%)18 (50.0%)
Males16 (44.4%)18 (50.0%)
Weight (kg) 0.95 #
Mean ± SD35.0 ± 22.133.3 ± 18.6
Median25.127.1
Q1–Q3(18.8–52.8)(18.8–44.5)
Height (cm) 0.87 *
Mean ± SD129.1 ± 29.5130.2 ± 30.2
Median123.0126.0
Q1–Q3(113.0–152.0)(107.0–155.5)
BMI (kg/m2) 0.86 #
Mean ± SD18.5 ± 4.617.9 ± 3.0
median18.017.8
Q1–Q3(14.7–21.5)(16.0–19.7)
Weight Percentile 0.65 #
Median77.561.2
Q1–Q3(33.0–93.5)(37.0–88.0)
Height Percentile 0.21 #
Median75.560
Q1–Q3(53.5–92.0)(26.0–95.0)
BMI Percentile 0.60 #
Median68.069.0
Q1–Q3(20.0–91.0)(40.0–88.0)
Vitamin 25(OH)D (ng/mL) 0.87 #
Mean ± SD33.98 ± 15.7633.41 ± 9.84
Median33.0032.20
Q1–Q3(22.45–40.15)(26.60–42.25)
Vitamin A (Retinol, ng/mL) 0.03 #
Mean ± SD426.21 ± 99.87390.97 ± 113.88
Median430.27364.08
Q1–Q3(365.29–474.75)(320.50–420.50)
Vitamin E (Tocopherol, µg/mL) 0.25 *
Mean ± SD8.40 ± 1.698.84 ± 1.55
Median8.249.05
Q1–Q3(7.17–9.43)(7.70–10.00)
β-carotene (ng/mL) 0.58 #
Mean ± SD102.53 ± 107.7496.50 ± 62.31
Median84.6874.50
Q1–Q3(36.21–11.76)(53.65–131.40)
BMI—body mass index, FAOD—fatty acid oxidation disorders, SD—standard deviation, Q1–Q3—interquartile range; * Student t-Test; # U Mann–Whitney Test.
Table 2. Comparison of the fat-modified diet group and the standard-fat group.
Table 2. Comparison of the fat-modified diet group and the standard-fat group.
Fat-Modified Diet Group
n = 14		Standard-Fat Diet Group
n = 22		p
Age (years) 0.65 *
Mean ± SD8.3 ± 5.37.6 ± 4.1
Median7.36.9
Q1–Q3(4.7–11.6)(4.6–11.6)
Sex (n (%)) 0.85 #
Females7 (50%)13 (59%
Males7 (50%)9 (41%)
Weight (kg) 0.76 #
Mean ± SD37.7 ± 26.833.3 ± 19.0
Median24.026.5
Q1–Q3(21.0–54.0)(17.0–51.6)
Height (cm) 0.69 *
Mean ± SD131.6 ± 32.4127.6 ± 28.2
Median126.2123.0
Q1–Q3(112.0–152.0)(114.0–152.0)
BMI (kg/m2) 0.90 #
Mean ± SD18.7 ± 5.318.4 ± 4.2
median16.318.4
Q1–Q3(14.6–22.1)(14.8–21.2)
Weight Percentile 0.45 #
Median81.573.0
Q1–Q3(34–96)(32.0–92.0)
Height Percentile 0.56 #
Median79.567.8
Q1–Q3(57.0–92.0)(49.0–92.0)
BMI Percentile 0.72 #
Median63.568.0
Q1–Q3(22.0–94.0)(20.0–90.0)
Vitamin 25(OH)D (ng/mL) 0.02 #
mean ± SD41.19 ± 16.4129.40 ± 13.81
median35.727.35
Q1–Q3(32.60–43.60)(21.50–35.70)
Vitamin A (Retinol, ng/mL) 0.03 *
Mean ± SD470.02 ± 74.43398.33 ± 105.35
Median471.56398.92
Q1–Q3(430.18–528.21)(341.40–444.22)
Vitamin E (Tocopherol, µg/mL) 0.01 #
Mean ± SD7.49 ± 1.788.98 ± 1.37
Median7.149.02
Q1–Q3(6.30–8.09)(8.09–9.61)
β-carotene (ng/mL) 0.04 #
Mean ± SD59.17 ± 40.95130.13 ± 127.50
Median54.7899.68
Q1–Q3(22.88–104.18)(50.90–147.29)
BMI—body mass index, FAOD—fatty acid oxidation disorders, SD—standard deviation, Q1–Q3—interquartile range; * Student t-test; # U Mann–Whitney Test.
Table 3. Vitamin contents of several types of MCT-based products.
Table 3. Vitamin contents of several types of MCT-based products.
Monogen
(Nutricia)		Lipistart
(Vitaflo)		MCTprocal
(Vitaflo)		MCTOil
(Nutricia)
per 100 gper 100 mLper 100 gper 100 mL per 100 gper 100 mL
Vitamin A (µg)32554.64666900
Vitamin D3 (µg)11.92.09.21.800
Vitamin E (mg)4.90.82111.500
β-carotene 0 *0 *0 *0 *00
* Information not provided on the nutritional label.
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Wasiewicz-Gajdzis, M.; Jamka, M.; Kałużny, Ł.; Wichłacz-Trojanowska, N.; Blask-Osipa, A.; Duś-Żuchowska, M.; Jagłowska, J.; Kurek, S.; Miśkiewicz-Chotnicka, A.; Walkowiak, J. The Impact of Nutritional Management on Fat-Soluble Nutrient Status in Patients with Fatty Acid Oxidation Disorders: A Cross-Sectional Study. Metabolites 2026, 16, 124. https://doi.org/10.3390/metabo16020124

AMA Style

Wasiewicz-Gajdzis M, Jamka M, Kałużny Ł, Wichłacz-Trojanowska N, Blask-Osipa A, Duś-Żuchowska M, Jagłowska J, Kurek S, Miśkiewicz-Chotnicka A, Walkowiak J. The Impact of Nutritional Management on Fat-Soluble Nutrient Status in Patients with Fatty Acid Oxidation Disorders: A Cross-Sectional Study. Metabolites. 2026; 16(2):124. https://doi.org/10.3390/metabo16020124

Chicago/Turabian Style

Wasiewicz-Gajdzis, Maria, Małgorzata Jamka, Łukasz Kałużny, Natalia Wichłacz-Trojanowska, Anna Blask-Osipa, Monika Duś-Żuchowska, Joanna Jagłowska, Szymon Kurek, Anna Miśkiewicz-Chotnicka, and Jarosław Walkowiak. 2026. "The Impact of Nutritional Management on Fat-Soluble Nutrient Status in Patients with Fatty Acid Oxidation Disorders: A Cross-Sectional Study" Metabolites 16, no. 2: 124. https://doi.org/10.3390/metabo16020124

APA Style

Wasiewicz-Gajdzis, M., Jamka, M., Kałużny, Ł., Wichłacz-Trojanowska, N., Blask-Osipa, A., Duś-Żuchowska, M., Jagłowska, J., Kurek, S., Miśkiewicz-Chotnicka, A., & Walkowiak, J. (2026). The Impact of Nutritional Management on Fat-Soluble Nutrient Status in Patients with Fatty Acid Oxidation Disorders: A Cross-Sectional Study. Metabolites, 16(2), 124. https://doi.org/10.3390/metabo16020124

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