Nutrition With Skimmed Breast Milk in an Infant With Long Chain 3-Hydroxyacyl-coA Dehydrogenase Deficiency.

The current standard diet for long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD) in the first months of life includes a special formula low in long-chain triglycerides (LCT) and enriched in medium-chain triglycerides (MCT). It involves the interruption of breastfeeding, withholding its nutritional and nonnutritional benefits. We describe the clinical case of a late preterm with 36 weeks gestational age diagnosed with LCHADD through newborn screening (NBS) who developed necrotizing enterocolitis (NEC) and sepsis due to () at 7 days of life. During hospital admission, the patient was fed skimmed breast milk supplemented with MCT oil and a low-fat MCT-enriched formula. Because the family wished to continue pumping milk after discharge, they were trained to defat milk using a non-refrigerated benchtop centrifuge. At home, a similar feeding regime was followed for 4 months. Hospital and home-produced skimmed breast milk met the dietary treatment requirement of < 1.0 g/dL of fat content. Growth and development during the first 5 months of life were normal, with an improved serum acylcarnitine profile and no decompensation. In this report, we demonstrated that breast milk defatting is a safe and feasible option for patients with LCHADD during hospital admission and at home, providing the benefits of human milk in these patients. This approach could influence dietary management guidelines for metabolic disorders or expand breast milk feeding options for medically complex infants.