Dietary Management of a Patient with Both Maple Syrup Urine Disease and Type I Diabetes
Maple Syrup Urine disease (MSUD) is caused by the deficiency of the branched chain 2-ketoacid dehydrogenase complex. Type I diabetes mellitus (TIDM) is a chronic illness characterized by the body’s inability to produce insulin due to the autoimmune destruction of the beta cells in the pancreas. A ca...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Galenos Yayinevi
2018-03-01
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| Series: | Journal of Pediatric Research |
| Subjects: | |
| Online Access: |
http://jpedres.org/archives/archive-detail/article-preview/dietary-management-of-a-patient-with-both-maple-sy/18774
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| _version_ | 1797905688615190528 |
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| author | Mehmet Gündüz Nevra Koç Özlem Ünal Seyit Ahmet Uçaktürk |
| author_facet | Mehmet Gündüz Nevra Koç Özlem Ünal Seyit Ahmet Uçaktürk |
| author_sort | Mehmet Gündüz |
| collection | DOAJ |
| description | Maple Syrup Urine disease (MSUD) is caused by the deficiency of the branched chain 2-ketoacid dehydrogenase complex. Type I diabetes mellitus (TIDM) is a chronic illness characterized by the body’s inability to produce insulin due to the autoimmune destruction of the beta cells in the pancreas. A case with both MSUD and TIDM has not been reported previously. In this study, we presented a patient with both MSUD and TIDM, dietary management and the follow up period. Dietary management could be difficult in the presence of two disorders affecting more than one macronutrient component such as both carbohydrate and protein. Our patient had good metabolic control in the follow-up period. Treatment was successful with intensive insulin, frequent feeding and a leucine restricted diet. |
| first_indexed | 2024-04-10T10:09:19Z |
| format | Article |
| id | doaj.art-b9e6745bc2a0417b9d7da23cf84c804a |
| institution | Directory Open Access Journal |
| issn | 2147-9445 2587-2478 |
| language | English |
| last_indexed | 2024-04-10T10:09:19Z |
| publishDate | 2018-03-01 |
| publisher | Galenos Yayinevi |
| record_format | Article |
| series | Journal of Pediatric Research |
| spelling | doaj.art-b9e6745bc2a0417b9d7da23cf84c804a2023-02-15T16:22:20ZengGalenos YayineviJournal of Pediatric Research2147-94452587-24782018-03-0151444610.4274/jpr.8942113049054Dietary Management of a Patient with Both Maple Syrup Urine Disease and Type I DiabetesMehmet Gündüz0Nevra Koç1Özlem Ünal2Seyit Ahmet Uçaktürk3 University of Health Sciences, Ankara Children Health and Diseases Hematology Oncology Training and Research Hospital, Clinic of Pediatric Nutrition and Metabolism, Ankara, Turkey University of Health Sciences, Ankara Children Health and Diseases Hematology Oncology Training and Research Hospital, Clinic of Pediatric Nutrition and Metabolism, Ankara, Turkey University of Health Sciences, Ankara Children Health and Diseases Hematology Oncology Training and Research Hospital, Clinic of Pediatric Nutrition and Metabolism, Ankara, Turkey University of Health Sciences, Ankara Children Health and Diseases Hematology Oncology Training and Research Hospital, Clinic of Pediatric Endocrinology and Metabolism, Ankara, Turkey Maple Syrup Urine disease (MSUD) is caused by the deficiency of the branched chain 2-ketoacid dehydrogenase complex. Type I diabetes mellitus (TIDM) is a chronic illness characterized by the body’s inability to produce insulin due to the autoimmune destruction of the beta cells in the pancreas. A case with both MSUD and TIDM has not been reported previously. In this study, we presented a patient with both MSUD and TIDM, dietary management and the follow up period. Dietary management could be difficult in the presence of two disorders affecting more than one macronutrient component such as both carbohydrate and protein. Our patient had good metabolic control in the follow-up period. Treatment was successful with intensive insulin, frequent feeding and a leucine restricted diet. http://jpedres.org/archives/archive-detail/article-preview/dietary-management-of-a-patient-with-both-maple-sy/18774 Maple Syrup Urine diseaseType I diabetes mellitusdietchildrenmetabolic control |
| spellingShingle | Mehmet Gündüz Nevra Koç Özlem Ünal Seyit Ahmet Uçaktürk Dietary Management of a Patient with Both Maple Syrup Urine Disease and Type I Diabetes Journal of Pediatric Research Maple Syrup Urine disease Type I diabetes mellitus diet children metabolic control |
| title | Dietary Management of a Patient with Both Maple Syrup Urine Disease and Type I Diabetes |
| title_full | Dietary Management of a Patient with Both Maple Syrup Urine Disease and Type I Diabetes |
| title_fullStr | Dietary Management of a Patient with Both Maple Syrup Urine Disease and Type I Diabetes |
| title_full_unstemmed | Dietary Management of a Patient with Both Maple Syrup Urine Disease and Type I Diabetes |
| title_short | Dietary Management of a Patient with Both Maple Syrup Urine Disease and Type I Diabetes |
| title_sort | dietary management of a patient with both maple syrup urine disease and type i diabetes |
| topic | Maple Syrup Urine disease Type I diabetes mellitus diet children metabolic control |
| url |
http://jpedres.org/archives/archive-detail/article-preview/dietary-management-of-a-patient-with-both-maple-sy/18774
|
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