Anales espanoles de pediatria · 1998
[Amino acid changes in blood of children with severe liver disease. The evaluation of differences according to distinct physiopathology].
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Delgado Domínguez M A, Ruza Tarrío F J, Hernanz Macías A, Madero Jarabo R, González Ojeda V, García García S, de la Oliva Senovilla P
Аннотация
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.
PATIENTS AND 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.
PATIENTS AND 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.