Assessing Nutrient Composition of Skimmed Human Milk for Treatment of Chylothorax and Very Long-Chain Acyl-CoA Dehydrogenase Deficiency Patients

Very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency is an inherited autosomal recessive metabolic disorder of fatty acid oxidation, and chylothorax is a condition where chyle accumulates in the chest cavity; both conditions require fat restriction as a treatment. Human milk feeding is encouraged for newborns but may be discontinued since it is a contraindication in severe forms of both disorders. The long-term goal of this study is to inform clinical practice guidelines for newborns with chylothorax or symptomatic VLCAD deficiency. The objective and specific aim is to evaluate the full nutrient content of human milk across four methods of fat separation: (1) refrigerated centrifuge, (2) nonrefrigerated centrifuge, (3) cream separation, and (4) manual separation, as compared to the control. It is hypothesized that all fat separation methods will yield lower amounts of total fat and fat-soluble vitamins (A, D, E, and K) and decrease choline while water-soluble vitamins and minerals will be unaffected. The proposed research is innovative because researchers have not yet examined complete micronutrient profiles of skimmed human milk to inform co-interventions. The rationale for exploring skimmed human milk over an MCT-based formula is the ability to provide the beneficial bioactive compounds (e.g., growth factors, immunoglobulins, oligosaccharides) in human milk that support infant growth and development. This research addresses the 2023 NIFA Programs Area Priority Code A1344a Diet, Nutrition, and the Prevention of Chronic Diseases program and is significant because it will inform human milk skimming methods and nutrient fortification practices for newly diagnosed VLCAD deficiency or chylothorax patients so that they may still receive the benefits of human milk nutrition.