Dietary recommendations could influence the progression of necrotizing enterocolitis in patients with long chain fatty acid oxidation disorder (LCFAOD), according to a study recently published in Frontiers in Pediatrics.
“It is important to consider skimmed human milk to minimize exposure to formula in the NEC risk period during feeding advancement, which may be longer in neonates with LC-FAOD than in otherwise healthy premature neonates,” the authors wrote.
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This case report describes a male newborn who was born at 33 weeks and 2 days to a 36-year-old female by cesarian section due to Hemolysis, Elevated Liver enzymes and Low Platelets syndrome. The pregnancy was complicated by gestational diabetes and gestational hypertension that required medical treatment. Prenatal routine screening for infectious diseases was negative, except for Group B Streptococcus, which was unknown. The patient’s mother had a family history positive for a sibling with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD).
The patient received neonatal resuscitation and obtained Appearance, Pulse, Grimace, Activity, and Respiration (APGAR) scores of 8 and 9 points at the 1st and 5th minute, respectively. He had normal measurements for the gestational age.
The patient remained in the neonatal intensive care unit (NICU) and underwent blood tests for LCFAOD. Elevated C16-OH and C18-OH indicated a diagnosis of LCHADD. The healthcare team gave human milk from a donor, beginning at 12 hours of life, with up to 23 ml/kg/day, and administered 0.5 g/kg/day of triheptanoin at 14 hours of life, with a subsequent dose adjustment to 0.5 g/kg every 5 to 7 days. The remaining diet consisted of formulas, achieving a fortified mix of 24 kcal/oz on day 6.
On the 7th day of life, he presented with bradycardia, desaturation, and 2 low-volume emeses. The abdominal physical examination was normal. On the 9th day, another episode of emesis occurred, along with hematochezia, and an abdominal x-ray revealed pneumatosis intestinalis, yielding a diagnosis of necrotizing enterocolitis.
The patient received ampicillin-sulbactam and gentamicin for 7 days, along with bowel decompression and total parenteral nutrition. Enteral nutrition was resumed with skimmed and full-fat human milk, both from the mother and donor, formulas and protein fortifier, and MCT oil instead of triheptanoin. Recovery was optimal, leading to a discharge on day 33.
Although the authors were not able to fully attribute the development of necrotizing enterocolitis to triheptanoin or full-fat human milk, these results provide important insight regarding the management of this rare but dangerous complication among patients with LCFAOD.
“Given the challenge of studying an uncommon complication (NEC) of a rare group of disorders (LC-FAODs) and the relatively limited data on triheptanoin (FDA approved in 2020), case reports such as this are likely to comprise a significant portion of the evidence basis for assessing a possible link between triheptanoin and NEC,” the authors explained.
Reference
Metzler M, Burns W, Mitchell C, Napolitano S, Chaudhari B. A case report of necrotizing enterocolitis in a moderately preterm neonate with LCHADD—a call to focus on the basics while utilizing advanced new therapies. Front Pediatr. Published online February 13, 2013. doi:10.3389/fped.2023.1081802
