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What is Enteral Formulas for Chylothorax Long Chain Fatty Acid Oxidation Disorders or Fat Malabsorption
Enteral formulas for chylothorax, long chain fatty acid oxidation disorders (i.e., LCHAD deficiency), fat malabsorption syndromes, and other lymphatic disorders are formulas that contain a high percentage of medium chain triglyceride (MCT) oil for easier fat absorption compared to standard formulas.
Approximately 80—87% of fat in these formulas is derived from MCT.
Conditions, such as chylothorax and primary intestinal lymphangiectasia, are characterized by a disruption of the lymphatic drainage, resulting in excessive losses of triglycerides, proteins, and lymphocytes into the lymphatic system, which can lead to malnutrition due to excessive losses.
The primary dietary management for these conditions is an MCT-enriched diet.
MCTs, which have a 6- to 12-carbon backbone, are the only fats absorbed directly via the portal system bypassing the lymphatics.
This is in contrast to long chain triglycerides (LCTs) (12 or more carbons), which are absorbed via the intestinal lymphatic ducts and transported in chylomicrons through the thoracic duct into the systemic circulation.
An MCT-enriched diet, will therefore, decrease the amount of lymphatic lymph flow. In clinical studies of patients with chylothorax, conservative treatment with MCT-enriched diets was effective as initial treatment in approximately 70—85% of patients.
For patients with LCHAD deficiency, dietary management also consists of MCT supplementation and limiting long-chain fatty acids (LCFAs). Reduction in LCFA intake along with an MCT-enriched diet likely suppresses long-chain fatty acid B-oxidation and prevents the accumulation of potentially toxic long-chain 3-hydroxy fatty acids and/or acylcarnitines.
Exclusion of LCT reduces lymphatic flow and pressure and thus prevents rupture of malformed lymphatics. MCT oil should not completely replace all dietary fats, as essential fatty acid deficiency may develop.
Infant formulas for chylothorax, fat metabolism disorders, and fat malabsorption syndromes are nutritionally complete. The standard caloric concentration for infant formulas designed for the needs of infants with chylothorax or LCHAD deficiency is 30 kcal/oz; however, lower caloric strengths can be prepared.
Formulas for children and adults are not intended as the sole source of nutrition; supplementation with additional nutrients, such as essential fatty acids and micronutrients, is recommended if long-term use is necessary.
Indications
- chylothorax
- fat malabsorption diagnosis
- long chain 3-hydroxyacyl-CoA dehydrogenase deficiency
- long chain fatty acid oxidation disorders
- nutritional supplementation
Monitoring Parameters
- serum electrolytes
- serum lipid profile
- weight
Contraindications
- breast-feeding
- dehydration
- essential fatty acid deficiency
- feeding intolerance
- galactosemia
- immunosuppression
- intravenous administration
- milk protein hypersensitivity
- nutritionally incomplete
- phenylketonuria
- pregnancy
- premature neonates
- severe combined immunodeficiency (SCID)
- soya lecithin hypersensitivity
Interactions
No information is available regarding drug interactions associated with Enteral Formulas for Chylothorax, Long-Chain Fatty Acid Oxidation Disorders, or Fat Malabsorption
