Targeted breast milk modification: A low-cost feeding option in young infants with citrullinemia
Background: The management of urea cycle disorders (UCDs) needs use of nitrogen scavenger drugs and protein restricted special formulas. The latter is not easily accessible and expensive. Continued breastfeeding is poorly tolerated by most babies. In these circumstances, targeted breast milk modific...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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Wolters Kluwer Medknow Publications
2021-01-01
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| Series: | Indian Pediatrics Case Reports |
| Subjects: | |
| Online Access: | http://www.ipcares.org/article.asp?issn=2772-5170;year=2021;volume=1;issue=4;spage=266;epage=268;aulast=Patra |
| _version_ | 1797245406706401280 |
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| author | Saikat Patra Swapnil Bhisikar Pavan Kalamdani Thaslima Kalathingal |
| author_facet | Saikat Patra Swapnil Bhisikar Pavan Kalamdani Thaslima Kalathingal |
| author_sort | Saikat Patra |
| collection | DOAJ |
| description | Background: The management of urea cycle disorders (UCDs) needs use of nitrogen scavenger drugs and protein restricted special formulas. The latter is not easily accessible and expensive. Continued breastfeeding is poorly tolerated by most babies. In these circumstances, targeted breast milk modification can be lifesaving by reducing ammonia and preventing a catabolic state. Clinical Description: A term baby born by normal delivery was discharged at 48 h after successfully initiating breastfeeding. He developed lethargy, poor feeding, and seizures on the 4th day. Vitals were stable. Sepsis, hypoglycemia, hypoxic-ischemic encephalopathy, dyselectrolytemia, hepatic/renal derangement, structural brain anomalies, cerebral edema, and hemorrhage were ruled out. A UCD was suspected due to hyperammonemia without acidosis. Citrullinemia was established by elevated citrulline and no orotic acid. Management: Breastfeeding was stopped, nitrogen scavenger drugs started, and peritoneal dialysis performed. As specialized milk formula was unavailable, we started diluted breast milk mixed with corn starch and coconut oil to achieve protein restriction and provide appropriate carbohydrates, lipids, and energy. Dilution was gradually decreased. There was gradual improvement in sensorium with normalization of ammonia. The baby was well till 8 weeks but developed symptomatic hyperammonemia due to noncompliance with therapy. Conclusion: Targeted modification of breast milk may be the only viable option for feeding infants with UCD in extraordinary circumstances. However, its short- and long-term consequences need to be researched thoroughly. |
| first_indexed | 2024-04-24T19:26:24Z |
| format | Article |
| id | doaj.art-53c33f9fa9884a08807b8aad69749f6e |
| institution | Directory Open Access Journal |
| issn | 2772-5170 2772-5189 |
| language | English |
| last_indexed | 2024-04-24T19:26:24Z |
| publishDate | 2021-01-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Indian Pediatrics Case Reports |
| spelling | doaj.art-53c33f9fa9884a08807b8aad69749f6e2024-03-25T15:37:46ZengWolters Kluwer Medknow PublicationsIndian Pediatrics Case Reports2772-51702772-51892021-01-011426626810.4103/ipcares.ipcares_263_21Targeted breast milk modification: A low-cost feeding option in young infants with citrullinemiaSaikat PatraSwapnil BhisikarPavan KalamdaniThaslima KalathingalBackground: The management of urea cycle disorders (UCDs) needs use of nitrogen scavenger drugs and protein restricted special formulas. The latter is not easily accessible and expensive. Continued breastfeeding is poorly tolerated by most babies. In these circumstances, targeted breast milk modification can be lifesaving by reducing ammonia and preventing a catabolic state. Clinical Description: A term baby born by normal delivery was discharged at 48 h after successfully initiating breastfeeding. He developed lethargy, poor feeding, and seizures on the 4th day. Vitals were stable. Sepsis, hypoglycemia, hypoxic-ischemic encephalopathy, dyselectrolytemia, hepatic/renal derangement, structural brain anomalies, cerebral edema, and hemorrhage were ruled out. A UCD was suspected due to hyperammonemia without acidosis. Citrullinemia was established by elevated citrulline and no orotic acid. Management: Breastfeeding was stopped, nitrogen scavenger drugs started, and peritoneal dialysis performed. As specialized milk formula was unavailable, we started diluted breast milk mixed with corn starch and coconut oil to achieve protein restriction and provide appropriate carbohydrates, lipids, and energy. Dilution was gradually decreased. There was gradual improvement in sensorium with normalization of ammonia. The baby was well till 8 weeks but developed symptomatic hyperammonemia due to noncompliance with therapy. Conclusion: Targeted modification of breast milk may be the only viable option for feeding infants with UCD in extraordinary circumstances. However, its short- and long-term consequences need to be researched thoroughly.http://www.ipcares.org/article.asp?issn=2772-5170;year=2021;volume=1;issue=4;spage=266;epage=268;aulast=Patrabreast milkcitrullinemianeonate |
| spellingShingle | Saikat Patra Swapnil Bhisikar Pavan Kalamdani Thaslima Kalathingal Targeted breast milk modification: A low-cost feeding option in young infants with citrullinemia Indian Pediatrics Case Reports breast milk citrullinemia neonate |
| title | Targeted breast milk modification: A low-cost feeding option in young infants with citrullinemia |
| title_full | Targeted breast milk modification: A low-cost feeding option in young infants with citrullinemia |
| title_fullStr | Targeted breast milk modification: A low-cost feeding option in young infants with citrullinemia |
| title_full_unstemmed | Targeted breast milk modification: A low-cost feeding option in young infants with citrullinemia |
| title_short | Targeted breast milk modification: A low-cost feeding option in young infants with citrullinemia |
| title_sort | targeted breast milk modification a low cost feeding option in young infants with citrullinemia |
| topic | breast milk citrullinemia neonate |
| url | http://www.ipcares.org/article.asp?issn=2772-5170;year=2021;volume=1;issue=4;spage=266;epage=268;aulast=Patra |
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