Aumento acentuado da leucina como fator de mau prognóstico na doença da urina de xarope de bordo - relato de caso

Aumento acentuado da leucina como fator de mau prognóstico na doença da urina de xarope de bordo - relato de caso

INTRODUCTION: Leucinosis or Maple Syrup Disease (MSD) is an innate metabolism error caused by a deficiency in the activity of the alpha-keto acid-dehydrogenase enzyme complex, which leads to the accumulation of essential branched-chain amino acids (leucine, valine, and isoleucine), leucine is partic...

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Bibliographic Details
Main Authors: Bruna Duque de Almeida Braga, Gessianni Claire Alves de Souza, Filipe Marinho Pinheiro da Camara, Ana Cecília Menezes de Siqueira, Lucas Victor Alves
Format: Article
Language:English
Published: Sociedade Brasileira de Pediatria 2023-06-01
Series:Residência Pediátrica
Subjects:
maple syrup urine disease
leucine
amino acid metabolism
inborn errors
Online Access:https://residenciapediatrica.com.br/detalhes/1340/aumento%20acentuado%20da%20leucina%20como%20fator%20de%20mau%20prognostico%20na%20doenca%20da%20urina%20de%20xarope%20de%20bordo%20-%20relato%20de%20caso

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https://residenciapediatrica.com.br/detalhes/1340/aumento%20acentuado%20da%20leucina%20como%20fator%20de%20mau%20prognostico%20na%20doenca%20da%20urina%20de%20xarope%20de%20bordo%20-%20relato%20de%20caso

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