[An enteral modular formula in dibasic amino aciduria].
Lysinuric protein intolerance (LPI) or dibasic amino acid aminoaciduria is an unusual metabolic illness which, in countries where it is most common, affects one individual for every 60,000-80,000 births, and which is characterised by the inability to transport dibasic amino acids to the interior of the different cells of the organism. This paper sets out the design for an enteral modular formula for a pediatric patient very probably suffering from LPI. The formula has a very limited protein content, and incorporates L-citruline, a non-proteinogenic amino acid which intervenes in the urea cycle.
Servicio de Farmacia, Hospital Materno-Infantil, Málaga, España.
S A
SA
Serna Juan
C
C
Sánchez Barroso
M
M
Damas Fernández-Figares
E
E
Valverde Molina
D000592:Amino Acid Metabolism, Inborn Errors / Q000628:therapy* / Q000652:urine; D000599:Amino Acids, Diamino / Q000652:urine*; D004750:Enteral Nutrition / Q000379:methods*; D006801:Humans; D007231:Infant, Newborn; D008239:Lysine / Q000652:urine; D012074:Remission Induction; D000608:Renal Aminoacidurias / Q000652:urine*
D000599:Amino Acids, Diamino; D008239:Lysine
Fórmula modular enteral en aminoaciduria dibásica.