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

TL;DR

A late preterm infant with a metabolic disorder was successfully fed skimmed breast milk supplemented with MCT oil, avoiding formula and maintaining normal growth and development.

Contribution

Demonstrates that defatted breast milk is a safe and feasible alternative to formula for managing LCHADD in infants.

Findings

Skimmed breast milk met the dietary requirement of <1.0 g/dL fat content in both hospital and home settings.

The infant showed normal growth and development with an improved serum acylcarnitine profile over 5 months.

Defatting breast milk using a centrifuge is a practical method for managing LCHADD during hospital and home care.

Abstract

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
Escherichia coli
(
E. coli
) 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.

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