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Dergiler / The Journal of Pediatric Research / 2018 / Cilt: 5 - Sayı: 1

Dietary Management of a Patient with Both Maple Syrup Urine Disease and Type I Diabetes

Mehmet GÜNDÜZ
Nevra KOÇ
ÖZLEM ÜNAL
Seyit Ahmet UÇAKTÜRK
Sayfa
44–46
DOI
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⚡ TL;DR(1 Cümlede Hızlı Çıkarım)

Treatment was successful with intensive insulin, frequent feeding and a leucine restricted diet.

📌 3 Maddede Bu Makale

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Amaç & Hipotez:Maple Syrup Urine disease (MSUD) is caused by the deficiency of the branched chain 2-ketoacid dehydrogenase complex.
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Temel Bulgular & Veri:Dietary management could be difficult inthe presence of two disorders affecting more than one macronutrient component such as both carbohydrate and protein.
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Sonuç & Katkı:Treatment was successful with intensive insulin, frequent feeding and a leucine restricted diet.
🔬Tür:Araştırma Makalesi
📍Kapsam:Genişletilmiş Akademik Örneklem
⚙️Değişkenler:Alan Parametreleri

Özet

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 illnesscharacterized 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 beenreported 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 inthe presence of two disorders affecting more than one macronutrient component such as both carbohydrate and protein. Our patient had good metabolic control in the followupperiod. Treatment was successful with intensive insulin, frequent feeding and a leucine restricted diet.

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Bu Makaleye Atıf Yap

GÜNDÜZ, M., KOÇ, N., ÜNAL, Ö., UÇAKTÜRK, S. A. (2018). Dietary Management of a Patient with Both Maple Syrup Urine Disease and Type I Diabetes. The Journal of Pediatric Research, Cilt: 5 - Sayı: 1, 44–46. https://ajindex.com/articles/362963

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Kaynakça (5)

  1. Chuang DT, Shih VE. Maple syrup urine disease (branchedchain ketoaciduria). In: The metabolic and molecular bases of inherited disease, 8th ed, Scriver CR, Beaudet AL, Sly WS, Valle D (Eds), McGraw-Hill, New York 2001.p.1971.
  2. Quental S, Vilarinho L, Martins E, et al. Incidence of maple syrup urine disease in Portugal. Mol Genet Metab 2010;100:385-7.
  3. Maahs DM, West NA, Lawrence JM, et al. Chapter 1: Epidemiology of Type 1 Diabetes. Endocrinol Metab Clin North Am 2010;39:481-97.
  4. Lian K, Du C, Liu Y, et al. Impaired adiponectin signaling contributes to disturbed catabolism of branched-chain amino acids in diabetic mice. Diabetes 2015;64:49-59.
  5. Frazier DM, Allgeier C, Homer C, et al. Nutrition management guideline for maple syrup urine disease: an evidenceand consensus-based approach. Mol Genet Metab 2014;112:210-7.