Intake Of Long-chain Fatty Acids Research Articles

Very-Long-Chain Acyl-CoA Dehydrogenase Deficiency: Family Impact and Perspectives.

Very-Long-Chain Acyl-CoA Dehydrogenase Deficiency (VLCADD) is a fatty acid oxidation disorder characterized by the decreased ability of the enzyme very-long-chain acyl-CoA dehydrogenase to break down fatty acids with 14 to 20-long carbon chains. The resulting clinical manifestations are variable in severity and include hypoketotic hypoglycemia, rhabdomyolysis, and cardiomyopathy. Treatment can consist of limiting the dietary intake of long-chain fatty acids, the prevention of fasting, and the supplementation of medium-chain fats. This study, conducted in the context of a 5-year long-term follow-up on VLCADD, evaluates how the diagnosis of this fatty acid disorder impacts the family, specifically as it relates to the medical diet and barriers to care. Caregivers (n = 10) of individuals with VLCADD responded to a survey about how VLCADD potentially impacts their family. The review included the clinical outcomes of the patients (n = 11), covering instances of rhabdomyolysis, cardiomyopathy, and hospitalizations related to VLCADD. Families affected by VLCADD experience barriers to care, including difficulties with finances, ability to work, and access to nutrition.

Risk of Disease Recurrence and Mortality Varies by Type of Fat Consumed before Cancer Treatment in a Longitudinal Cohort of Head and Neck Squamous Cell Carcinoma Patients

BackgroundThe associations between specific types of fat and head and neck squamous cell carcinoma (HNSCC) recurrence and mortality rates have not yet been examined. ObjectivesThe purpose of this study was to determine how intakes of various fat subtypes before cancer treatment are associated with recurrence and mortality in adults diagnosed with HNSCC. MethodsThis was a secondary analysis longitudinal cohort study of data collected from 476 newly diagnosed patients with HNSCC. Patients completed baseline FFQs and epidemiologic health surveys. Recurrence and mortality events were collected annually. Fat intakes examined included long-chain fatty acids (LCFAs), unsaturated fatty acids (FAs), PUFAs, ω-3 (n–3) PUFAs, ω-6 (n–6) PUFAs, MUFAs, animal fats, vegetable fats, saturated FAs, and trans fats. Associations between fat intake (categorized into tertiles) and time to event were tested using multivariable Cox proportional hazards models, adjusting for age, sex, smoking status, human papillomavirus status, tumor site, cancer stage, and total caloric intake. Intake of fats was compared with the lowest tertile. ResultsDuring the study period, there were 115 recurrent and 211 death events. High LCFA intake was associated with a reduced all-cause mortality risk (HR: 0.55; 95% CI: 0.34, 0.91; P-trend = 0.02). High unsaturated FA intake was associated with a reduced all-cause mortality risk (HR: 0.62; 95% CI: 0.40, 0.97; P-trend = 0.04) and HNSCC-specific mortality risk (HR: 0.51; 95% CI: 0.29, 0.90; P-trend = 0.02). High intakes of ω-3 PUFAs (HR: 0.56; 95% CI: 0.35, 0.91; P-trend = 0.02) and ω-6 PUFAs (HR: 0.57; 95% CI: 0.34, 0.94; P-trend = 0.02) were significantly associated with a reduced all-cause mortality risk. There were no significant associations between other fat types and recurrence or mortality risk. ConclusionsIn this prospective survival cohort of 476 newly diagnosed patients with HNSCC, our data suggest that HNSCC prognosis may vary depending on the fat types consumed before cancer treatment. Clinical intervention trials should test these associations.

Establishing age-stratified red blood cell fatty acid reference ranges using model-based clustering and iterative application of the harris-boyd method

BackgroundThe current assessment of nutritional status and diagnosis of essential fatty acids deficiency (EFAD) utilizes the analysis of long-chain fatty acids (LCFAs) in serum or plasma; however, these concentrations do not represent habitual LCFA intake. LCFAs in red blood cells (RBCs) are less prone to intra-individual variability and exclude the need for fasting, which is unrealistic in pediatric populations. Our study objective was to characterize the RBC LCFA profiles in pediatric and adult reference populations and establish age-specific reference intervals (RIs). MethodsTwenty-one LCFAs in RBCs were measured in 523 pediatric and adult controls by gas chromatography-mass spectrometry. Model-based clustering was used to identify possible age subgroups. After removing outliers by the Tukey method, initial age subgroups were then compared using the Harris-Boyd method in an iterative manner. RIs (95%), with confidence intervals (90%), in the final age groups were established using parametric or non-parametric statistics. ResultsOur data showed heterogeneous changes in the concentrations of most LCFAs and the EFAD biomarkers (mead acid, Triene/Tetraene ratio) during infancy. Model-based clustering identified six initial age subgroups per fatty acid, on average. Our application of the iterative Harris-Boyd method decreased the average number of age groups to three per fatty acid, with 13 total unique age cut-offs. Finally, using these age groups, we established age-specific RIs for 21 fatty acids, six group totals, and the Triene/Tetraene ratio. ConclusionOur study revealed significant age-dependent changes in RBC fatty acid profiles warranting separate pediatric and adults RIs. Model-based clustering and the iterative application of the Harris-Boyd method were successfully used to establish RBC fatty acid RIs for an objective assessment of long-term nutritional status in pediatric and adult populations.

Dietary fatty acids and risk of Alzheimer's disease and related dementias: Observations from the Washington Heights-Hamilton Heights-Inwood Columbia Aging Project (WHICAP).

Introduction:High dietary intake of long chain, polyunsaturated fatty acids is associated with lower Alzheimer’s disease (AD) risk.Methods:Washington Heights-Hamilton Heights-Inwood Columbia Aging Project is a multiethnic, prospective observational study of aging and dementia among elderly (≥ 65 years). Dietary intake was measured using a food frequency questionnaire. Dietary short-, medium-, and long-chain fatty acid intakes were categorized by number of carbons and double bonds. Consensus AD diagnoses were made. Associations between AD risk and dietary fatty acid and cholesterol intakes were estimated using multivariable Cox proportional hazards regression models.Results:Of 2612 multiethnic women (67%) and men (baseline age 76.3 [6.4] years), 380 developed AD over an average 4.5 years follow-up. Lower risk of AD was associated with increasing intakes of docosahexaenoic acid (DHA; hazard ratio [HR] = 0.73, 95% confidence interval [CI]: 0.57 to 0.95, P = 0.018) and eicosapentaenoic acid (EPA; HR = 0.74, 95% CI: 0.57 to 0.95, P = 0.021), and longer AD-free survival (P < 0.05).Discussion:Higher intake of DHA and EPA are protective for AD.

Higher dietary protein intake preserves lean body mass, lowers liver lipid deposition, and maintains metabolic control in participants with long-chain fatty acid oxidation disorders.

Medical nutrition therapy for long-chain fatty acid oxidation disorders (LC-FAODs) currently emphasizes fasting avoidance, restricted dietary long-chain fatty acid intake, supplementation with medium chain triglycerides, and increased carbohydrate intake. We hypothesize that increasing dietary protein intake relative to carbohydrate intake would preserve metabolic control yet induce physical benefits including reduced hepatic lipogenesis. Therefore, we compared two dietary approaches with similar fat intake but different carbohydrate to protein ratios in participants diagnosed with LC-FAODs. Thirteen participants were enrolled and randomized into either a high-protein (PRO) or a high-carbohydrate (CHO) diet for 4 months. Baseline and 4-month assessments included body composition, ectopic lipid deposition, and resting energy expenditure. End of study assessments also included total energy expenditure, metabolic responses to oral feedings, and whole-body fatty acid oxidation capacity. At the end of the dietary intervention, both groups had similar energy expenditure, fat and glucose oxidation rates, and glucolipid responses to mixed meal and oral glucose loads. Neither dietary group experienced worsening symptoms related to their LC-FAOD. Compared to the CHO group, the PRO group exhibited increased blood levels of short-chain acylcarnitines, reduced intrahepatic lipid content, and maintained lean body mass while the CHO group lost lean mass. In patients with LC-FAODs, increasing protein intake maintained metabolic control, reduced liver fat without risk of metabolic decompensation, and helped preserve lean body mass. We propose that a modest increase in dietary protein along with fasting avoidance and fat restriction may improve body composition and energy expenditure in patients with LC-FAODs.

CD36 mediates lipid accumulation in pancreatic beta cells under the duress of glucolipotoxic conditions: Novel roles of lysine deacetylases

The cluster of differentiation 36 (CD36) is implicated in the intake of long-chain fatty acids and fat storage in various cell types including the pancreatic beta cell, thus contributing to the pathogenesis of metabolic stress and diabetes. Recent evidence indicates that CD36 undergoes post-translational modifications such as acetylation-deacetylation. However, putative roles of such modifications in its functional activation and onset of beta cell dysregulation under the duress of glucolipotoxicity (GLT) remain largely unknown. Using pharmacological approaches, we validated, herein, the hypothesis that acetylation-deacetylation signaling steps are involved in CD36-mediated lipid accumulation and downstream apoptotic signaling in pancreatic beta (INS-1832/13) cells under GLT. Exposure of these cells to GLT resulted in significant lipid accumulation without affecting the CD36 expression. Sulfo-n-succinimidyl oleate (SSO), an irreversible inhibitor of CD36, significantly attenuated lipid accumulation under GLT conditions, thus implicating CD36 in this metabolic step. Furthermore, trichostatin A (TSA) or valproic acid (VPA), known inhibitors of lysine deacetylases, markedly suppressed GLT-associated lipid accumulation with no discernible effects on CD36 expression. Lastly, SSO or TSA prevented caspase 3 activation in INS-1832/13 cells exposed to GLT conditions. Based on these findings, we conclude that an acetylation-deacetylation signaling step might regulate CD36 functional activity and subsequent lipid accumulation and caspase 3 activation in pancreatic beta cells exposed to GLT conditions. Identification of specific lysine deacetylases that control CD36 function should provide novel clues for the prevention of beta-cell dysfunction under GLT.

The composition of dietary fat alters the transcriptional profile of pathways associated with lipid metabolism in the liver and adipose tissue in the pig.

The objective was to investigate the of effect chemical composition of dietary fat on transcription of genes involved in lipid metabolism in adipose tissue and the liver via transcriptional profiling in growing pigs. A total of 48 Genetiporc 6.0 × Genetiporc F25 (PIC, Inc., Hendersonville, TN) barrows (initial BW of 44.1 ± 1.2 kg) were randomly allotted to 1 of 6 dietary treatments. Each experimental diet included 95% of a corn-soybean meal basal diet and 5% cornstarch (control; CNTR), animal-vegetable blend (AV), coconut oil (COCO), corn oil (COIL), fish oil (FO), or tallow (TAL). Pigs were sacrificed on d 10 (final BW of 51.2 ± 1.7 kg) to collect tissues. Expression normalization across samples was performed by calculating a delta cycle threshold (ΔCt) value using . Delta delta cycle threshold (ΔΔCt) values were expressed relative to the CNTR treatment. In adipose tissue, adding dietary fat, regardless of the source, decreased the mRNA abundance of compared with the CNTR ( = 0.014). Pigs fed a COIL-based diet tended to have greater adipose tissue expression of ( = 0.071) than pigs fed the other dietary fat sources tested. Abundance of mRNA was greater in adipose tissue of barrows a fed COIL-based diet than barrows fed CNTR or FO-based diets ( = 0.047). In the liver, adding dietary fat, regardless of source, increased the mRNA abundance of , , , , , and ( ≤ 0.020) and tended to increase the abundance of ( = 0.071) and ( = 0.086) compared with the CNTR. Pigs fed a TAL-based diet had greater hepatic transcription of than pigs fed CNTR-, COCO-, or FO-based diets ( = 0.013). Hepatic transcription of tended to be greater in pigs fed COCO than in pigs fed other dietary fat sources ( = 0.074). Dietary omega-3 fatty acid content tended to negatively correlate with mRNA abundance of ( = 0.065) in adipose tissue and ( = 0.063) in the liver. Dietary fat SFA content was negatively correlated with in the liver ( ≤ 0.039). Dietary fat MUFA content tended to be positively correlated with , , and mRNA abundance in the liver ( ≤ 0.100). To conclude, the intake of omega-3 fatty acids suppressed the mRNA abundance of genes involved in lipolysis in both adipose tissue and the liver. Dietary SFA are greater inhibitors of lipogenesis in adipose tissue than omega-6 fatty acids. Intake of medium-chain fatty acids alters hepatic lipid metabolism differently than intake of long-chain fatty acids.

Effect of dietary fatty acid and micronutrient intake/energy ratio on serum diamine oxidase activity in healthy women.

Serum diamine oxidase (DAO) activity varies to a greater extent in women than in men. DAO activity during the luteal phase was higher than that during the follicular phase in healthy women. Recent reports have indicated that duodenal lipid infusion increased DAO activity in the intestinal lymph in rats. The aim of this study was to elucidate the effect of dietary nutrient intake on serum DAO activity in healthy women. Thirty-four healthy Japanese women were recruited. Food surveys were performed using dietary records for 3d during both the follicular and luteal phases. Nutrient intake was calculated and expressed as the energy intake ratio. The correlation between DAO activity and nutrient intake was analyzed. Serum DAO activity in both phases was positively correlated with intake of long-chain fatty acids, saturated fatty acids, and monounsaturated fatty acids (P<0.05). Intake of phosphorus, calcium, zinc, magnesium, iron, and vitamin B12 during the luteal phase was positively correlated with serum DAO activity (P<0.05). In healthy women, serum DAO activity was influenced by dietary fatty acid and micronutrient intake.

Contrasting metabolic effects of medium- versus long-chain fatty acids in skeletal muscle

Dietary intake of long-chain fatty acids (LCFAs) plays a causative role in insulin resistance and risk of diabetes. Whereas LCFAs promote lipid accumulation and insulin resistance, diets rich in medium-chain fatty acids (MCFAs) have been associated with increased oxidative metabolism and reduced adiposity, with few deleterious effects on insulin action. The molecular mechanisms underlying these differences between dietary fat subtypes are poorly understood. To investigate this further, we treated C2C12 myotubes with various LCFAs (16:0, 18:1n9, and 18:2n6) and MCFAs (10:0 and 12:0), as well as fed mice diets rich in LCFAs or MCFAs, and investigated fatty acid-induced changes in mitochondrial metabolism and oxidative stress. MCFA-treated cells displayed less lipid accumulation, increased mitochondrial oxidative capacity, and less oxidative stress than LCFA-treated cells. These changes were associated with improved insulin action in MCFA-treated myotubes. MCFA-fed mice exhibited increased energy expenditure, reduced adiposity, and better glucose tolerance compared with LCFA-fed mice. Dietary MCFAs increased respiration in isolated mitochondria, with a simultaneous reduction in reactive oxygen species generation, and subsequently low oxidative damage. Collectively our findings indicate that in contrast to LCFAs, MCFAs increase the intrinsic respiratory capacity of mitochondria without increasing oxidative stress. These effects potentially contribute to the beneficial metabolic actions of dietary MCFAs.

Can nutritional biomarkers help to provide the ‘missing link’ in diet–diabetes associations?

93 ISSN 1758-1907 10.2217/DMT.12.83 © 2013 Future Medicine Ltd Diabetes Manage. (2013) 3(2), 93–96 There are two well-known facts regarding diabetes prevention. First, the prevention of diabetes is important, nay urgent, given its increasing burden, with an estimated 366 million individuals worldwide with diabetes in 2011, projected to rise to 552 million by 2030 [1], and it is a leading cause of death [2]. Second, there is evidence from randomized trials that the primary prevention, or at least the delay of onset, of Type 2 diabetes is possible with lifestyle interventions, including diet [3]. What is not well known, however, is which specific dietary factors relate to diabetes risk in what specific ways, and what dietary advice is optimal for populations and individuals for the prevention of diabetes. This is despite the fact that the potential contribution of dietary intake to the prevention of chronic diseases, including diabetes, has been prominently highlighted by many international and national agencies such as WHO [4]. In reality, the scientific literature and the media are full of examples of inconsistent and contradictory reports of associations between dietary factors and the risk of diabetes, or indeed other medical conditions, often leaving the readers confused. Let us consider one example. It has been reported that eating one or more portions per week of fish versus less than one portion per week was associated with a 25% lower risk of incident Type 2 diabetes among men and women in the EPIC Norfolk study, which included approximately 22,000 participants (odds ratio: 0.75; 95% CI: 0.58–0.96) [5]. In the same year (2009), there were reports from the USA [6] and The Netherlands [7] that fish intake is associated with an increased risk of diabetes, and in the US study an increased risk of diabetes was also associated with greater dietary long-chain fatty acid intake, derived from the food frequency questionnaire [6]. Not surprisingly, the public are confused: should they or should they not eat fish? The exact reasons for the lack of consensus regarding the associations for fish intake observed between the different studies are unclear, but it has been discussed in a systematic review that they could include geographical or cultural differences, including different cooking methods or levels of contamination of fish, such as by persistent organic pollutants or mercury [8]. What is not known, however, is to what extent these different findings are a result of measurement error introduced by the self-report methods that are currently available to researchers to assess dietary intake. While a validated food frequency questionnaire was

Obesity generates metabolic alterations in the myocardium leading to changes in mitochondrial membrane permeability

Dietary intake of long-chain fatty acids (LCFA, CN16) plays a causative role in insulin resistance and risk of diabetes. Whereas LCFA promote lipid accumulation and have detrimental effects on metabolic health, diets rich in medium-chain fatty acids (MCFA; C8– C14) have been associated with increased oxidative metabolism and reduced adiposity, with little effect on insulin action in vivo. The molecular mechanisms underlying these differences are poorly understood. To shed light into this question, we have treated L6 myotubes with specific MCFA (capric acid, 10:0 and lauric acid, 12:0) and LCFA (palmitic acid, 16:0 and oleic acid, 18:1) and determined the effect of fatty acid treatment on metabolic parameters including triglyceride accumulation and insulin-stimulated glycogen synthesis as a measure of insulin sensitivity. A potential mechanism linking lipid accumulation and insulin resistance is increased oxidative stress within the insulinsensitive tissue. Therefore, these studies were extended by measuring reactive oxygen species (ROS) production and oxidative damage in muscle cells treated with different fatty acids. Incubation of myotubes with LCFA led to lipid accumulation (+90% with palmitic acid vs. control BSA-treated cells, pb0.01), impaired glycogen synthesis (−40%, pb0.05) and decreased succinate dehydrogenase activity (−40–55% vs. control, pb0.05), however these deleterious effects were not observed in the MCFA-treated myotubes. Furthermore, ROS generation (both superoxide and hydrogen peroxide), measured using HPLC and spectrophotometry, was not significantly altered with MCFA, but was significantly greater in the LCFA-treated cells (+500% vs. control, pb0.001), suggesting that MCFA might prevent the induction of oxidative stress. Increased oxidative stress with LCFA, but not MCFA, was confirmed by measuring a marker of lipoxidative damage, lipid hydroperoxides, which were increased by LCFA (+40% vs. control, pb0.01), but remained unchanged in the MCFA-treated cells. These results show that treatment of muscle cells with MCFA does not produce the detrimental metabolic effects observed when cells are exposed to LCFA. A potential mechanism is the differential effects observed in ROS production and oxidative damage in the MCFAand LCFA-treated L6 myotubes.

Omega-3 fatty acids status in human subjects estimated using a food frequency questionnaire and plasma phospholipids levels

BackgroundIntakes of omega-3 (n-3) fatty acids (FA) are associated with several health benefits. The aim of this study was to verify whether intakes of n-3 FA estimated from a food frequency questionnaire (FFQ) correlate with n-3 FA levels measured in plasma phospholipids (PL).MethodsThe study sample consisted of 200 French-Canadians men and women aged between 18 to 55 years. Dietary data were collected using a validated FFQ. Fasting blood samples were collected and the plasma PL FA profile was measured by gas chromatography.ResultsLow intakes of n-3 long-chain FA together with low percentages of n-3 long-chain FA in plasma PL were found in French-Canadian population. Daily intakes of eicosapentaenoic acid (EPA), docosapentaenoic acid (DPA) and docosahexaenoic acid (DHA) were similar between men and women. Yet, alpha-linolenic acid (ALA) and total n-3 FA intakes were significantly higher in men compared to women (ALA: 2.28 g and 1.69 g, p < 0.0001, total n-3 FA: 2.57 g and 1.99 g, p < 0.0001; respectively). In plasma PL, DPA and DHA percentages were significantly different between men and women (DPA: 1.03% and 0.88%, p < 0.0001, DHA: 3.00% and 3.43%, p = 0.0005; respectively). Moreover, DHA (men: r = 0.52, p < 0.0001; women: r = 0.57, p < 0.0001) and total n-3 FA (men: r = 0.47, p < 0.0001; women: r = 0.52, p < 0.0001) intakes were positively correlated to their respective plasma PL FA levels. In women, EPA (r = 0.44, p < 0.0001) and DPA (r = 0.23, p = 0.02) intakes were also correlated respectively with EPA and DPA plasma PL FA percentages.ConclusionEstimated n-3 long-chain FA intake among this young and well-educated French-Canadian population is lower than the recommendations. Further, FFQ data is comparable to plasma PL results to estimate DHA and total n-3 FA status in healthy individuals as well as to evaluate the EPA and DPA status in women. Overall, this FFQ could be used as a simple, low-cost tool in future studies to rank n-3 FA status of individuals.

Effects of nutrient supply, plasma metabolites, and nutritional status of sows during transition on performance in the next lactation1

The aim of the present study was to evaluate the effects of nutrient supply, plasma metabolites, and nutritional status of sows during the transition from gestation to lactation on performance of piglets during the colostral period and throughout lactation. Forty second-parity sows were fed 1 of 4 gestation diets containing a different quantity of dietary fiber (171 to 404 g/kg of DM) from mating until d 108 of gestation. From d 108 of gestation until weaning (d 28 of lactation), sows were fed 1 of 5 lactation diets with a different quantity of dietary fat [3 or 8% with different proportions of medium- (MCFA) and long-chain fatty acids (LCFA)]. Blood was obtained by jugular venipuncture on d 108 and 112 of gestation and on d 1 of lactation, and concentrations of plasma glucose, NEFA, lactate, acetate, propionate, butyrate, and fatty acids were analyzed. Piglet growth and mortality were noted throughout lactation. Piglet mortality during the colostral period (0 to 24 h) was affected by the lactation diets and was positively related to sow backfat (d 108) and plasma lactate (d 112) and negatively related to mean piglet birth weight (P < 0.05). Mean piglet live BW gain (LWG) was recorded in the periods 0 to 24 h, 7 to 10 d, 14 to 17 d, and 17 to 28 d relative to parturition as indirect measures of colostrum yield (0 to 24 h), milk yield in early lactation (d 7 to 10), and at peak lactation (d 14 to 17 and d 17 to 28). Effects of gestation and lactation diets on studied sow traits were tested on selected days during the transition period and the next lactation, and tested statistically on separate days. The LWG in the colostral period was positively correlated with mean piglet birth weight (P < 0.001), plasma concentrations of propionate and MCFA (P < 0.05), and plasma acetate and butyrate (P < 0.1) on d 1 of lactation. The LWG in early lactation was inversely correlated with plasma lactate on d 108 (P < 0.05), plasma glucose on d 112, and backfat thickness on d 108 (P < 0.10). The LWG at peak lactation was positively correlated with MCFA intake of the sow on d 113 to 115 and backfat thickness on d 108 during the transition, and negatively correlated with intake of LCFA and ME intake on d 108 to 112 (P < 0.05). In conclusion, feeding and body condition of sows during the transition from gestation to lactation is important for neonatal piglet survival, lactation performance of sows, and piglet growth during the next lactation.

Dietary fatty acids and insulin resistance

High-fat diets have been associated with insulin resistance, a risk factor for both Type II diabetes and heart disease. The effect of dietary fat on insulin varies depending on the type of fatty acid consumed. Saturated fatty acids have been consistently associated with insulin resistance. On the other hand, medium and long-chain fatty acid intakes are associated with insulin sensitivity, as are high intakes of w3 fatty acids. Trans fatty acids appear to potentiate insulin secretion, at least in the short-term, to a greater degree than cis fatty acids. This may reflect chronic alterations in insulin sensitivity, although this remains to be tested. In summary, although it must be emphasized that all diets high in fat cause insulin resistance relative to high-carbohydrate diets, it appears that dietary saturated, short-chain, and w6 fatty acids have the most deleterious effects on insulin action.

Recognition and management of fatty acid oxidation defects: a series of 107 patients.

In a personal series of 107 patients, we describe clinical presentations, methods of recognition and therapeutic management of inherited fatty acid oxidation (FAO) defects. As a whole, FAO disorders appear very severe: among the 107 patients, only 57 are still living. Including 47 siblings who died early in infancy, in total 97 patients died, of whom 30% died within the first week of life and 69% before 1 year. Twenty-eight patients presented in the neonatal period with sudden death, heart beat disorders, or neurological distress with various metabolic disturbances. Hepatic presentations were observed in 73% of patients (steatosis, hypoketotic hypoglycaemia, hepatomegaly, Reye syndrome). True hepatic failure was rare (10%); cholestasis was observed in one patient with LCHAD deficiency. Cardiac presentations were observed in 51% of patients: 67% patients presented with cardiomyopathy, mostly hypertrophic, and 47% of patients had heart beat disorders with various conduction abnormalities and arrhythmias responsible for collapse, near-miss and sudden unexpected death. All enzymatic blocks affecting FAO except CPT I and MCAD were found associated with cardiac signs. Muscular signs were observed in 51% of patients (of whom 64% had myalgias or paroxysmal myoglobinuria, and 29% had progressive proximal myopathy). Chronic neurologic presentation was rare, except in LCHAD deficiency (retinitis pigmentosa and peripheral neuropathy). Renal presentation (tubulopathy) and transient renal failure were observed in 27% of patients. The diagnosis of FAO disorders is generally based on the plasma acylcarnitine profile determined by FAB-MS/MS from simple blood spots collected on a Guthrie card. Urinary organic acid profile and total and free plasma carnitine can also be very helpful, mostly in acute attacks. If there is no significant disturbance between attacks, the diagnosis is based upon a long-chain fatty acid loading test, fasting test, and in vitro studies of fatty acid oxidation on fresh lymphocytes or cultured fibroblasts. Treatment includes avoiding fasting or catabolism, suppressing lipolysis, and carnitine supplementation. The long-term dietary therapy aims to prevent periods of fasting and restrict long-chain fatty acid intake with supplementation of medium-chain triglycerides. Despite these therapeutic measures, the long-term prognosis remains uncertain.

Net Energy for Lactation of Calcium Salts of Long-Chain Fatty Acids for Cows Fed Silage-Based Diets1,2

The NEL of calcium salts of long-chain fatty acids from palm oil was determined in mature Holstein cows. Twelve lactating (fed for ad libitum intake) and six nonlactating (restricted to near maintenance intake) Holstein cows were fed 0 or 2.95% fat supplement in diets formulated to contain 16 or 20% CP in a 2 × 2 factorial arrangement of treatments in a single reversal design within protein level. The fat supplement was substituted for ground corn and minerals. Two 6-d total collection balance trials were conducted during which cows were in open circuit respiration chambers. Intake of OM was lower for lactating cows fed the fat supplement (18.1 vs. 19.1 kg/d), but energy intake did not differ (93.2 Mcal/d). Total long-chain fatty acid intake was increased from 477 to 820 g/d with fat feeding. Apparent digestibility of long-chain fatty acids was increased 11.1 percentage units with increased dietary CP for lactating cows with no difference in fatty acid digestibility for the dry cows. Milk yield was higher (34.3 vs. 32.0 kg/d) with fat feeding, but milk energy yield did not differ (22.6 Mcal/d). The NEL of the fat supplement was estimated from the incremental differences in energy values within cows, assuming NEL of corn replaced by fat to be 1.96 Mcal/kg DM, and was determined to be 6.52 Mcal/kg DM (SE = 1.74). The efficiency of the use of metabolizable energy for lactation from dietary fat was 77.2%. The energy in calcium salts of long-chain fatty acids is utilized efficiently for lactation in mature cows.

The effect of a blend of dietary unesterified and saturated long-chain fatty acids on the performance of dairy cows in mid-lactation

This experiment studied the effects of feeding a supplement of a blend of unesterified and saturated long-chain fatty acids on the productivity of dairy cows in mid-lactation. Twenty-three cows in their fourth month of lactation were individually fed ad libitum, a mixed balanced ration based on maize silage, lucerne hay and rolled grain. Varying quantities, up to 1020 g cow-1 day-1 of the fatty acid supplement, were mixed into the ration. Yields of milk and milk products were linearly related to total long-chain fatty acid intake. Milk fat content increased linearly while milk protein content averaged 3.59 (s.d. � 0.15)%. The marginal returns from feeding 1 kg of the supplement were 3.3 kg milk, 0.33 kg fat and 0.07 kg protein. The proportions of C 10:0, C12:0 and C 14:0 fatty acids in milk were decreased, while those of C 18:0 and C18:1 were increased as the result of feeding long-chain fatty acids. The concentration of lipid in plasma was increased, but acetate and D-(3)-hydroxybutyrate levels in blood remained unchanged with increased levels of dietary long-chain fatty acid. Efficiency of milk production was increased by 11% from feeding 1 kg of the supplement. In vivo digestibilities of dry matter, neutral and acid detergent fibres, and dietary long-chain fatty acids were unaffected by supplement.

Effect of Fat and Lactose Supplementation on Digestion in Dairy Cows. 2. Long-Chain Fatty Acids

Dairy cows fitted with rumen and proximal duodenal cannulae were given diets of 60% hay, 7% soybean and rapeseed meal, and either 33% concentrate (control diet) or 33% milk (lipid-supplemented diet: “milk” diet). Amounts of total long-chain fatty acids consumed, entering the duodenum and excreted in the feces were examined. Long-chain fatty acid intake was 192 and 764 g/d with the control and milk diets, respectively. The duodenal flow of long-chain fatty acids was greater (17.3%) than the amount consumed when the control diet was fed; with the milk diet, there was a net loss (−22.2%), mainly due to a decrease in total C16 and C18 acids. The extent of C18:2 hydrogenation in the rumen was reduced by the high fat ration, but for C18:3, hydrogenation was very high and unchanged. Apparent intestinal digestibility of fatty acids was high, especially on the milk diet (86.1%), although the amount of fatty acids absorbed (60.6 g/kg dry matter intake/d) was three times greater than with the control.