title: [Amino acid changes in blood of children with severe liver disease. The evaluation of differences according to distinct physiopathology].
abstract: OBJECTIVE
  The aim of this study was to know the serum amino acid profiles in children with terminal hepatic diseases and to assess the differences between the two main physiopathological groups of hepatic damage: cholestasis and cellular necrosis.
  
  
  METHODS
  We studied twenty-six pediatric patients with severe hepatic diseases admitted to the Pediatric Intensive Care Unit. Patients were divided into two groups according to the predominant hepatic lesion: cellular damage (fourteen children) and cholestasis damage (twelve cases).
  
  
  RESULTS
  Overall, there is a significant increase in the aromatic amino acids (AAA) phenylalanine (p < 0.04) and tyrosine (p < 0.0003) and a decrease in the branched-chain amino acids (BCAA) leucine, isoleucine and valine (p < 0.00001), with a reduction in the BCAA/AAA ratio (p < 0.00001). However, we found a significant decrease in glutamine, cysteine, taurine, serine, threonine, tryptophan, total amino acids and essential amino acids, together with higher levels of glutamic acid, ornithine and citrulline, which reflects a more complex metabolic disturbance. The group with cholestatic damage shows very low taurine levels (p < 0.0003). Patients with predominantly cellular damage have higher increases in tyrosine (p < 0.01), phenylalanine and hydroxyproline (p < 0.01).
  
  
  CONCLUSIONS
  These findings may help us to better understand the complex physiopathology of amino acid metabolism in different liver diseases. Moreover, the extremely low levels of taurine found prompted us to recommend additional dietary support particularly in children with cholestatic hepatopathy.
authors.affiliation: Cuidados Intensivos Pediátricos, Hospital Infantil La Paz, Universidad Autónoma, Madrid.
authors.forename: M A
authors.initials: MA
authors.lastname: Delgado Domínguez
authors.forename: F J
authors.initials: FJ
authors.lastname: Ruza Tarrío
authors.forename: A
authors.initials: A
authors.lastname: Hernanz Macías
authors.forename: R
authors.initials: R
authors.lastname: Madero Jarabo
authors.forename: V
authors.initials: V
authors.lastname: González Ojeda
authors.forename: S
authors.initials: S
authors.lastname: García García
authors.forename: P
authors.initials: P
authors.lastname: de la Oliva Senovilla
mesh_terms: D000293:Adolescent; D000596:Amino Acids / Q000097:blood*; D002648:Child; D005260:Female; D006801:Humans; D008107:Liver Diseases / Q000097:blood* / Q000175:diagnosis / Q000503:physiopathology*; D008297:Male; D012720:Severity of Illness Index
chemical_list: D000596:Amino Acids
vernacular_title: Alteraciones de los aminoácidos en sangre en niños con enfermedad hepática severa. Valoración de las diferencias en relación a la distinta fisiopatología.