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
Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD) is an autosomal recessive condition of impaired beta-oxidation. Traditionally, treatment included restriction of dietary long-chain fatty acids via a low-fat diet and supplementation of medium chain triglycerides. In 2020, triheptanoin r...
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2023-02-01
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| Series: | Frontiers in Pediatrics |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fped.2023.1081802/full |
| _version_ | 1811165266808143872 |
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| author | Marina Metzler William Burns Carly Mitchell Stephanie Napolitano Stephanie Napolitano Bimal P. Chaudhari Bimal P. Chaudhari Bimal P. Chaudhari Bimal P. Chaudhari |
| author_facet | Marina Metzler William Burns Carly Mitchell Stephanie Napolitano Stephanie Napolitano Bimal P. Chaudhari Bimal P. Chaudhari Bimal P. Chaudhari Bimal P. Chaudhari |
| author_sort | Marina Metzler |
| collection | DOAJ |
| description | Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD) is an autosomal recessive condition of impaired beta-oxidation. Traditionally, treatment included restriction of dietary long-chain fatty acids via a low-fat diet and supplementation of medium chain triglycerides. In 2020, triheptanoin received FDA approval as an alternative source of medium chain fatty acids for individuals with long-chain fatty acid oxidation disorders (LC-FAOD). We present a case of a moderately preterm neonate born at 33 2/7 weeks gestational age with LCHADD who received triheptanoin and developed necrotizing enterocolitis (NEC). Prematurity is known as a major risk factor for NEC, with risk increasing with decreasing gestational age. To our knowledge, NEC has not previously been reported in patients with LCHADD or with triheptanoin use. While metabolic formula is part of the standard of care for LC-FAOD in early life, preterm neonates may benefit from more aggressive attempts to use skimmed human milk to minimize exposure to formula during the risk period for NEC during feed advancement. This risk period may be longer in neonates with LC-FAOD compared to otherwise healthy premature neonates. |
| first_indexed | 2024-04-10T15:34:26Z |
| format | Article |
| id | doaj.art-5d90822830a346a0828912e249cccec2 |
| institution | Directory Open Access Journal |
| issn | 2296-2360 |
| language | English |
| last_indexed | 2024-04-10T15:34:26Z |
| publishDate | 2023-02-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Pediatrics |
| spelling | doaj.art-5d90822830a346a0828912e249cccec22023-02-13T08:02:11ZengFrontiers Media S.A.Frontiers in Pediatrics2296-23602023-02-011110.3389/fped.2023.10818021081802A case report of necrotizing enterocolitis in a moderately preterm neonate with LCHADD—A call to focus on the basics while utilizing advanced new therapiesMarina Metzler0William Burns1Carly Mitchell2Stephanie Napolitano3Stephanie Napolitano4Bimal P. Chaudhari5Bimal P. Chaudhari6Bimal P. Chaudhari7Bimal P. Chaudhari8Pediatric Residency, Nationwide Children’s Hospital, Columbus, OH, United StatesDivision of Genetic and Genomic Medicine, Nationwide Children’s Hospital, Columbus, OH, United StatesDivision of Genetic and Genomic Medicine, Nationwide Children’s Hospital, Columbus, OH, United StatesDivision of Neonatology, Nationwide Children’s Hospital, Columbus, OH, United StatesDepartment of Pediatrics, College of Medicine, Ohio State University, Columbus, OH, United StatesDivision of Genetic and Genomic Medicine, Nationwide Children’s Hospital, Columbus, OH, United StatesDivision of Neonatology, Nationwide Children’s Hospital, Columbus, OH, United StatesDepartment of Pediatrics, College of Medicine, Ohio State University, Columbus, OH, United StatesSteve and Cindy Rasmussen Institute for Genomic Medicine, Nationwide Children’s Hospital, Columbus, OH, United StatesLong-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD) is an autosomal recessive condition of impaired beta-oxidation. Traditionally, treatment included restriction of dietary long-chain fatty acids via a low-fat diet and supplementation of medium chain triglycerides. In 2020, triheptanoin received FDA approval as an alternative source of medium chain fatty acids for individuals with long-chain fatty acid oxidation disorders (LC-FAOD). We present a case of a moderately preterm neonate born at 33 2/7 weeks gestational age with LCHADD who received triheptanoin and developed necrotizing enterocolitis (NEC). Prematurity is known as a major risk factor for NEC, with risk increasing with decreasing gestational age. To our knowledge, NEC has not previously been reported in patients with LCHADD or with triheptanoin use. While metabolic formula is part of the standard of care for LC-FAOD in early life, preterm neonates may benefit from more aggressive attempts to use skimmed human milk to minimize exposure to formula during the risk period for NEC during feed advancement. This risk period may be longer in neonates with LC-FAOD compared to otherwise healthy premature neonates.https://www.frontiersin.org/articles/10.3389/fped.2023.1081802/fullnecrotizing enterocolitistriheptanoinpretermhuman milkLCHADD |
| spellingShingle | Marina Metzler William Burns Carly Mitchell Stephanie Napolitano Stephanie Napolitano Bimal P. Chaudhari Bimal P. Chaudhari Bimal P. Chaudhari Bimal P. Chaudhari 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 Frontiers in Pediatrics necrotizing enterocolitis triheptanoin preterm human milk LCHADD |
| title | 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 |
| title_full | 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 |
| title_fullStr | 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 |
| title_full_unstemmed | 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 |
| title_short | 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 |
| title_sort | case report of necrotizing enterocolitis in a moderately preterm neonate with lchadd a call to focus on the basics while utilizing advanced new therapies |
| topic | necrotizing enterocolitis triheptanoin preterm human milk LCHADD |
| url | https://www.frontiersin.org/articles/10.3389/fped.2023.1081802/full |
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