Nutrition Studies in Long-Chain Fatty Acid Oxidation Disorders: Diet Composition and Monitoring

Gillingham, Melanie B.

doi:10.1007/978-3-319-14621-8_23

Cited by 1 publication

(4 citation statements)

References 39 publications

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“…Supplementation with specific oils such as walnut or flaxseed oil may be necessary to meet essential fatty acid requirements (Table 5). Patients are at higher risk for becoming deficient in fat soluble vitamins and may require supplementation (62).…”

Section: Categorymentioning

confidence: 99%

“…Linoleic acid and α-linolenic acid are precursors for the endogenous synthesis of longer fatty acids such as eicosapentaenoic acid (EPA; C20:5n3) and docosahexaenoic acid (DHA; C22:6n3), which are crucial for brain, visual, and immune functions (62). It is often difficult to increase plasma EPA and DHA concentrations from dietary oils alone so patients require supplementation with preformed DHA (60 mg/day for infants <20 kg; 100 mg/day for children >20 kg; and 100-200 mg/day for adults) to achieve normal levels (62). All patients with LCFAODs should be given DHA, as DHA supplementation may stabilize, but not prevent, the retinopathy seen in LCHADD (63).…”

Section: Categorymentioning

confidence: 99%

“…With a fat restricted diet, patients with LCFAODs are potentially at risk for inadvertently lowering their protein intake unless they specifically consume low fat, high protein foods (e.g., nonfat dairy, lean meats). An investigation of a higher protein diet in LCHADD and TFPD patients showed a higher protein diet with less carbohydrates does not improve metabolic control, but may be beneficial for body composition and liver lipid content (62).…”

Section: Categorymentioning

confidence: 99%

“…Patients with LCFAODs are at high risk of exercise-induced rhabdomyolysis. To prevent rhabdomyolysis and increase exercise tolerance, patients require additional MCT supplementation (0.15-0.2 g/kg weight) mixed with a glucose solution (e.g., Gatorade ® ) prior to exercise and a 3:1 ratio of carbohydrate to protein snack after exercise (62).…”

Section: Categorymentioning

confidence: 99%

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Fatty acid oxidation disorders

Merritt¹,

Norris²,

Kanungo³

2018

Ann. Transl. Med

149

116

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Fatty acid oxidation disorders (FAODs) are inborn errors of metabolism due to disruption of either mitochondrial β-oxidation or the fatty acid transport using the carnitine transport pathway. The presentation of a FAOD will depend upon the specific disorder, but common elements may be seen, and ultimately require a similar treatment. Initial presentations of the FAODs in the neonatal period with severe symptoms include cardiomyopathy, while during infancy and childhood liver dysfunction and hypoketotic hypoglycemia are common. Episodic rhabdomyolysis is frequently the initial presentation during or after adolescence; although, these symptoms may develop at any age for most of the FAODs The treatment of all FAOD's include avoidance of fasting, aggressive treatment during illness, and supplementation of carnitine, if necessary. The long-chain FAODs differ by requiring a fat-restricted diet and supplementation of medium chain triglyceride oil and often docosahexaenoic acid (DHA)-an essential fatty acid, crucial for brain, visual, and immune functions and prevention of fat soluble vitamin deficiencies. The FAOD are a group of autosomal recessive disorders associated with significant morbidity and mortality, but early diagnosis on newborn screening (NBS) and early initiation of treatment are improving outcomes. There is a need for clinical studies including randomized, controlled, therapeutic trials to continue to evaluate current understanding and to implement future therapies.

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Section: Categorymentioning

confidence: 99%