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[Isolated isoleucine deficiency in diet therapy of a case of maple syrup urine disease].
An Esp Pediatr. 1983 Nov; 19(5):389-92.AE

Abstract

A case of maple syrup urine disease is described presenting during diet therapy a clinical picture with predominant cutaneous symptoms attributed to a dietary deficiency. It is believed that probable cause was the coexistence of low serum levels of isoleucine with still elevated levels of leucine. Given the metabolic interrelationship existing between different branch-chained amino acids, an independent dietary correction of each amino acid is recommended.

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Pub Type(s)

Case Reports
English Abstract
Journal Article

Language

spa

PubMed ID

6660656

Citation

Sanjurjo, P, et al. "[Isolated Isoleucine Deficiency in Diet Therapy of a Case of Maple Syrup Urine Disease]." Anales Espanoles De Pediatria, vol. 19, no. 5, 1983, pp. 389-92.
Sanjurjo P, Centeno C, Vallo A, et al. [Isolated isoleucine deficiency in diet therapy of a case of maple syrup urine disease]. An Esp Pediatr. 1983;19(5):389-92.
Sanjurjo, P., Centeno, C., Vallo, A., Ojembarrena, E., & Rodríguez Soriano, J. (1983). [Isolated isoleucine deficiency in diet therapy of a case of maple syrup urine disease]. Anales Espanoles De Pediatria, 19(5), 389-92.
Sanjurjo P, et al. [Isolated Isoleucine Deficiency in Diet Therapy of a Case of Maple Syrup Urine Disease]. An Esp Pediatr. 1983;19(5):389-92. PubMed PMID: 6660656.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Isolated isoleucine deficiency in diet therapy of a case of maple syrup urine disease]. AU - Sanjurjo,P, AU - Centeno,C, AU - Vallo,A, AU - Ojembarrena,E, AU - Rodríguez Soriano,J, PY - 1983/11/1/pubmed PY - 1983/11/1/medline PY - 1983/11/1/entrez SP - 389 EP - 92 JF - Anales espanoles de pediatria JO - An Esp Pediatr VL - 19 IS - 5 N2 - A case of maple syrup urine disease is described presenting during diet therapy a clinical picture with predominant cutaneous symptoms attributed to a dietary deficiency. It is believed that probable cause was the coexistence of low serum levels of isoleucine with still elevated levels of leucine. Given the metabolic interrelationship existing between different branch-chained amino acids, an independent dietary correction of each amino acid is recommended. SN - 0302-4342 UR - https://www.unboundmedicine.com/medline/citation/6660656/[Isolated_isoleucine_deficiency_in_diet_therapy_of_a_case_of_maple_syrup_urine_disease]_ L2 - http://babysfirsttest.org/newborn-screening/conditions/maple-syrup-urine-disease-msud DB - PRIME DP - Unbound Medicine ER -