Abstract
Background
Neonatal presentation of congenital lipodystrophy is rare. The nutritional management of high triglyceride (TG) levels in neonates is mostly derived from familial hypertriglyceridemia conditions. We describe a neonatal presentation of familial congenital lipodystrophy and the challenges we faced in optimizing nutritional management.
Case Description
A neonate presented with phenotypical features of lipodystrophy, including sub-optimal weight gain, prominent musculature, and lack of subcutaneous fat. TG levels were high. The unit and the family made a shared decision to continue breastfeeds. The risks of acute pancreatitis, stroke, and lipemia retinalis were concerns associated with continuing the “high-fat” diet of breastmilk. Fortunately, with close follow-up and monitoring, the infant’s TG values serially reduced, and growth is adequate with normal development.
Conclusion
Nutritional management of neonatal hypertriglyceridemia requires a case to case approach, especially if the condition is not due to familial hyperlipidemia states. These should focus on informed decisions based on the benefits of breastfeeding over fat-free formula.
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