Nutrition With Skimmed Breast Milk in an Infant With Long Chain 3‐Hydroxyacyl‐coA Dehydrogenase Deficiency
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, withhold...
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| Format: | Article |
| Language: | English |
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Wiley
2025-05-01
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| Series: | JIMD Reports |
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| Online Access: | https://doi.org/10.1002/jmd2.70018 |
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| author | Clara Alonso‐Diaz Diana Escuder‐Vieco Pilar Quijada‐Fraile Delia Barrio‐Carreras Patricia Pérez‐Mohand Elena Martín‐Hernández Carmen Rosa Pallas‐Alonso Nadia Raquel García‐Lara |
| author_facet | Clara Alonso‐Diaz Diana Escuder‐Vieco Pilar Quijada‐Fraile Delia Barrio‐Carreras Patricia Pérez‐Mohand Elena Martín‐Hernández Carmen Rosa Pallas‐Alonso Nadia Raquel García‐Lara |
| author_sort | Clara Alonso‐Diaz |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-dfee923dffdf4f8d87de84b5c7544b0c |
| institution | Kabale University |
| issn | 2192-8312 |
| language | English |
| publishDate | 2025-05-01 |
| publisher | Wiley |
| record_format | Article |
| series | JIMD Reports |
| spelling | doaj-art-dfee923dffdf4f8d87de84b5c7544b0c2025-08-20T03:48:14ZengWileyJIMD Reports2192-83122025-05-01663n/an/a10.1002/jmd2.70018Nutrition With Skimmed Breast Milk in an Infant With Long Chain 3‐Hydroxyacyl‐coA Dehydrogenase DeficiencyClara Alonso‐Diaz0Diana Escuder‐Vieco1Pilar Quijada‐Fraile2Delia Barrio‐Carreras3Patricia Pérez‐Mohand4Elena Martín‐Hernández5Carmen Rosa Pallas‐Alonso6Nadia Raquel García‐Lara7Neonatology Department Research Institut i+12. 12 de Octubre Hospital. Universidad Complutense Madrid SpainAladina‐MGU Regional Mother's Milk Bank Research Institut i+12. 12 de Octubre Hospital Madrid SpainEnfermedades Mitocondriales‐Metabólicas Hereditarias Research Institut i+12. 12 de Octubre Hospital Madrid SpainEnfermedades Mitocondriales‐Metabólicas Hereditarias Research Institut i+12. 12 de Octubre Hospital Madrid SpainEnfermedades Mitocondriales‐Metabólicas Hereditarias Research Institut i+12. 12 de Octubre Hospital Madrid SpainEnfermedades Mitocondriales‐Metabólicas Hereditarias Research Institut i+12. 12 de Octubre Hospital Madrid SpainNeonatology Department Research Institut i+12. 12 de Octubre Hospital. Universidad Complutense Madrid SpainNeonatology Department Research Institut i+12. 12 de Octubre Hospital. Universidad Complutense Madrid SpainABSTRACT 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.https://doi.org/10.1002/jmd2.70018(skimmed) breast milk feedingenteral nutritionhuman milkLCHADDlong‐chain fatty acid oxidation defects (LC‐FAODs)necrotizing enterocolitis |
| spellingShingle | Clara Alonso‐Diaz Diana Escuder‐Vieco Pilar Quijada‐Fraile Delia Barrio‐Carreras Patricia Pérez‐Mohand Elena Martín‐Hernández Carmen Rosa Pallas‐Alonso Nadia Raquel García‐Lara Nutrition With Skimmed Breast Milk in an Infant With Long Chain 3‐Hydroxyacyl‐coA Dehydrogenase Deficiency JIMD Reports (skimmed) breast milk feeding enteral nutrition human milk LCHADD long‐chain fatty acid oxidation defects (LC‐FAODs) necrotizing enterocolitis |
| title | Nutrition With Skimmed Breast Milk in an Infant With Long Chain 3‐Hydroxyacyl‐coA Dehydrogenase Deficiency |
| title_full | Nutrition With Skimmed Breast Milk in an Infant With Long Chain 3‐Hydroxyacyl‐coA Dehydrogenase Deficiency |
| title_fullStr | Nutrition With Skimmed Breast Milk in an Infant With Long Chain 3‐Hydroxyacyl‐coA Dehydrogenase Deficiency |
| title_full_unstemmed | Nutrition With Skimmed Breast Milk in an Infant With Long Chain 3‐Hydroxyacyl‐coA Dehydrogenase Deficiency |
| title_short | Nutrition With Skimmed Breast Milk in an Infant With Long Chain 3‐Hydroxyacyl‐coA Dehydrogenase Deficiency |
| title_sort | nutrition with skimmed breast milk in an infant with long chain 3 hydroxyacyl coa dehydrogenase deficiency |
| topic | (skimmed) breast milk feeding enteral nutrition human milk LCHADD long‐chain fatty acid oxidation defects (LC‐FAODs) necrotizing enterocolitis |
| url | https://doi.org/10.1002/jmd2.70018 |
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