Newborn babies with long chain fatty acid oxidation disorder (LCFAOD) may be more susceptible to necrotizing enterocolitis, a complication that often occurs in premature babies, according to a new study published in Molecular Genetics and Metabolism and being presented at the Society for Inherited Metabolic Disorders 43rd Annual Meeting.

A team of researchers led by Bimal P. Chaudhari MD, MPH, from Nationwide Children’s Hospital in Columbus, Ohio presented the case of a male baby born at 33 2/7 weeks. He was suspected of having long chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHAD), a type of LCFAOD based on the medical history of his mother and brother.

He started being fed enterally with donor human milk when he was 12 hours old. He was then given triheptanoin 2 hours later at a dose of 0.5 g/kg/day and advanced 0.5 g/kg every 5 to 7 days. 


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The baby developed necrotizing enterocolitis on his 9th day of life. “To our knowledge, [necrotizing enterocolitis] has not previously been reported with triheptanoin use,” the researchers wrote. They suggested that long-chain fatty acids that are expressed in the gut epithelium may make newborn babies with LCFAOD more susceptible to this complication.

The metabolic formula is part of the care to manage LCFAOD in early life. However, based on this case study, the researchers propose that preterm babies with the disease may benefit from “more aggressive attempts to use skimmed human milk and minimize exposure to formula during the window of risk” for the development of necrotizing enterocolitis. This may be larger in newborn babies with LCFAOD compared to babies born prematurely but without the disease.

Necrotizing enterocolitis is characterized by mucosal or deeper intestinal necrosis and is associated with enteral feeding causing the proliferation of luminal bacteria. These bacteria can penetrate the intestinal wall and cause causing intestinal perforation.

Reference

Metzler M, Burns W, Loar C, Napolitano S, Chaudhari BP. Necrotizing enterocolitis following triheptanoin treatment in a neonate with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHAD). Mol Genet Metab. 2022;135(4):287-288. doi:10.1016/j.ymgme.2022.01.067