Essential Fatty Acid Deficiency In Patients Research Articles

Composite lipid emulsion use and essential fatty acid deficiency in pediatric patients with intestinal failure with high parenteral nutrition dependence: A retrospective cohort study.

Reports of essential fatty acid deficiency (EFAD) in patients receiving parenteral nutrition (PN) and a composite lipid (mixed oil intravenous lipid emulsion [MO ILE]) are predominantly when managed by lipid restriction. The objective of this study was to determine the prevalence of EFAD in patients with intestinal failure (IF) who are PN dependent without lipid restriction. We retrospectively evaluated patients, ages 0-17 years, followed by our intestinal rehabilitation program between November 2020 and June 2021 with PN dependency index (PNDI) of >80% on a MO ILE. Demographic data, PN composition, PN days, growth, and plasma fatty acid profile were collected. A plasma triene-tetraene (T:T) ratio >0.2 indicated EFAD. Summary statistics and Wilcoxon rank sum test evaluated to compare between PNDI category and ILE administration (grams/kilograms/day). P < 0.05 was considered significant. Twenty-six patients (median age, 4.1 years [interquartile range (IQR) = 2.4-9.6]) were included. The median duration of PN was 1367 days (IQR = 824-3195). Sixteen patients had a PNDI of 80%-120% (61.5%). Fat intake for the group was 1.7 g/kg/day (IQR = 1.3-2.0). The median T:T ratio was 0.1 (IQR = 0.1-0.2) with no values >0.2. Linoleic and arachidonic acid were low in 85% and 19% of patients, respectively; however, Mead acid was normal in all patients. This report is the largest to date on the EFA status of patients with IF on PN. These results suggest that, in the absence of lipid restriction, EFAD is not a concern when using MO ILEs in children receiving PN for IF.

Normal Growth and Essential Fatty Acid Status in Children With Intestinal Failure on Lipid Limitation.

Lipid limitation, that is, ≤1 g · kg⁻¹ · day⁻¹ of soy oil lipid emulsion (SOLE), has been suggested as a method to reduce the risk of intestinal failure (IF)-associated liver disease (IFALD). There are limited data as to the effects of this strategy on growth and essential fatty acid (EFA) status. The aim of the study was to assess growth, prevalence of cholestasis, and EFA deficiency in patients with IF who were provided daily SOLE at a dose ≤1 g · kg⁻¹ · day⁻¹. Medical records were retrospectively reviewed from 9 patients age 16 months to 8 years who had IF requiring parenteral nutrition support for >12 months. Parenteral nutrition supplied a mean of 53% of total energy (range 24%-86%). Mean SOLE dose was 0.61 g · kg⁻¹ · day⁻¹ (range 0.4-0.81 g · kg⁻¹ · day⁻¹). After 1 month of lipid limitation between 2011 and 2014, no patient developed IFALD as defined by a direct bilirubin >2 mg/dL. The median direct bilirubin was 0.1 mg/dL (range 0.075-0.85 mg/dL). No patient developed EFA deficiency as defined by a triene-to-tetraene ratio >0.2 (median 0.026, range 0.017-0.076). Height z scores increased from mean of -2.568 (range -10.8 to 0.878) to -0.484 (range -3.546 to 0.822). Weight z scores increased from mean of -1.412 (range -5.871 to 0.906) to -0.595 (range -2.178 to 0.926). In this case series, lipid limitation allowed normal growth while preventing the development of cholestasis and EFA deficiency.

Intake and blood levels of fatty acids in treated patients with phenylketonuria.

Investigators in Italy and Spain have suggested that therapy for patients with phenylketonuria (PKU) may result in essential fatty acid (EFA) deficiency. Objectives of this study were to determine if the diets of patients with PKU in the United States provided adequate EFA intakes and whether patients could form long-chain polyunsaturated fatty acids. Patients (1-13 years of age) with classic PKU undergoing therapy and their non-PKU sibling closest in age were compared. Nutrient intakes were calculated from 3-day diet diaries. Fatty acids in plasma and erythrocytes were identified and quantified. Paired t tests compared results for the patients and their non-PKU siblings. Twenty-eight patients and 26 siblings were studied. Mean fat intake was greatest by siblings (34.8 +/- 1.3% of energy) and lowest by Phenyl-Free-fed patients (19.5 +/- 1.2% of energy; P < 0.05). Fat intake (30.4 +/- 1.8% of energy) by Phenex-fed patients did not differ from that of siblings. Percentage of energy ingested as C18:2n-6 and C18:3n-3 did not differ significantly between patients and siblings. No clinically significant, consistent differences were found in fatty acid levels (wt%) in plasma or erythrocytes between patients with PKU and siblings. No patient in this study exhibited a Holman index of EFA deficiency. Siblings ingested animal protein containing C20:5n-3 and C22:6n-3 fatty acids, and this may account for their greater wt% of these plasma and erythrocyte fatty acids. Because patients with PKU do not ingest fatty acids >C18 but C20:4n-6, C20:5n-3, and C22:6n-3 were found in their plasma and erythrocytes, in vivo synthesis from C18:2n-6 and C18:3n-3 appears to occur. Lack of EFA deficiency in patients in this study may be the result of the use of canola and soy oils containing C18:2n-6 and C18:3n-3 rather than olive oil in the diets.

Intake and Blood Levels of Fatty Acids in Treated Patients With Phenylketonuria

ABSTRACTBackgroundInvestigators in Italy and Spain have suggested that therapy for patients with phenylketonuria (PKU) may result in essential fatty acid (EFA) deficiency. Objectives of this study were to determine if the diets of patients with PKU in the United States provided adequate EFA intakes and whether patients could form long‐chain polyunsaturated fatty acids.MethodsPatients (1–13 years of age) with classic PKU undergoing therapy and their non‐PKU sibling closest in age were compared. Nutrient intakes were calculated from 3‐day diet diaries. Fatty acids in plasma and erythrocytes were identified and quantified. Paired t tests compared results for the patients and their non‐PKU siblings.ResultsTwenty‐eight patients and 26 siblings were studied. Mean fat intake was greatest by siblings (34.8 ± 1.3% of energy) and lowest by Phenyl‐Free–fed patients (19.5 ± 1.2% of energy;P &lt; 0.05). Fat intake (30.4 ± 1.8% of energy) by Phenex‐fed patients did not differ from that of siblings. Percentage of energy ingested as C18:2n‐6 and C18:3n‐3 did not differ significantly between patients and siblings. No clinically significant, consistent differences were found in fatty acid levels (wt%) in plasma or erythrocytes between patients with PKU and siblings.ConclusionsNo patient in this study exhibited a Holman index of EFA deficiency. Siblings ingested animal protein containing C20:5n‐3 and C22:6n‐3 fatty acids, and this may account for their greater wt% of these plasma and erythrocyte fatty acids. Because patients with PKU do not ingest fatty acids &gt;C18 but C20:4n‐6, C20:5n‐3, and C22:6n‐3 were found in their plasma and erythrocytes, in vivo synthesis from C18:2n‐6 and C18:3n‐3 appears to occur. Lack of EFA deficiency in patients in this study may be the result of the use of canola and soy oils containing C18:2n‐6 and C18:3n‐3 rather than olive oil in the diets.

Topical corn oil in the management of essential fatty acid deficiency

Conflicting anecdotal reports about the efficacy of topical linoleate in managing essential fatty acid deficiency prompted this prospective study of 10 critically ill surgical patients receiving continuous total parenteral nutrition (TPN). Ten ml of corn oil (4800 mg of linoleate) were massaged into the skin daily commencing after 7.7 +/- 3.8 (mean +/- SD) days of fat-free intake. Plasma samples were obtained weekly. Total lipids were extracted and methylated, and fatty acids were quantitated by gas-liquid chromatography. The triene:tetraene ratio (20:3 omega 9/20:4 omega 6) increased progressively in patients despite corn oil therapy. In 89% of patients the ratio exceeded 0.2, which is diagnostic of essential fatty acid deficiency (EFAD). Topical application of corn oil does not prevent or treat EFAD in patients on TPN.