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2012, Número 6

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Acta Pediatr Mex 2012; 33 (6)


Los ácidos grasos poliinsaturados de cadena larga en hiperfenilalaninemias

Guillén-López S, Rodríguez- Schmidt R, Vela-Amieva M

Idioma: Español
Referencias bibliográficas: 39
Paginas: 335-339
Archivo PDF: 86.70 Kb.


RESUMEN

Los pacientes con hiperfenilalaninemia (HFA) deben restringir el consumo de alimentos con ácidos grasos poliinsaturados (AGPI-CL) como los de origen animal, lo cual puede propiciar deficiencias causantes de trastornos neurológicos, que no se explican con las variaciones en las concentraciones de fenilalanina en sangre. Se ha demostrado que los pacientes con HFA se benefician con suplemento de ácido docosahexaenóico (DHA), ácido graso poliinsaturado de cadena larga (AGPI-CL) esencial que cumple diversas funciones en el cerebro y la retina. Sin embargo, son necesarios más estudios para determinar la dosis y las fuentes de DHA, en las distintas etapas de la vida.


REFERENCIAS (EN ESTE ARTÍCULO)

  1. Scriver CR, Kaufman S, Eisensmith RC, Woo SLC. The hyperphenylalaninemias. In: Scriver CR, Beaudet AL, Sly WS, Valle D, The Metabolic and Molecular Bases of Inherited Disease, 7th ed. New York: McGraw-Hill; 2001 p.1015–75.

  2. Ramírez-Farías C, Pérez-Andrade ME, Ibarra-González I, Vela-Amieva M. Controversias en la clasificación de las hiperfenilalaninemias. Acta Pediatr Mex 2007;28(6):261-9.

  3. Scriver C, Kaufmann S. Hyperphenylalaninemia: Phenylalanine Hidroxylase Deficiency, In: Scriver CR, Beaudet AL, Sly WS, Valle D, The Metabolic and Molecular Bases of Inherited Disease, 8th ed. New York: McGraw-Hill; 2001. p.1667-724.

  4. Cornejo V, Raimann E. Errores innatos del metabolismo de los aminoácidos: Hiperfenilalaninemias. En: Colombo M, Cornejo V, Raimann E. Errores innatos en el metabolismo del niño. 1ª edición, Santiago de Chile: 2009. p. 59-65.

  5. Smith I, Phillip L. The hyperphenylalaninemias, In: Fernandes J, Saudubray JM, van den Berghe G ed. Inborn Metabolic Diseases, diagnosis and treatment. 3rd edition. Germany: Springer-Verlag; 2000. p.172-83.

  6. Acosta P, Michals Matalon K. Nutrition management of patients with inherited disorders of aromatic aminoacid metabolism. In: Acosta P. Nutrition Management of Patients with inherited metabolic disorders. Sudbury MA: Jones and Barlett Publishers; 2010: p.119-174.

  7. van Spronsen FJ. Phenylketonuria: a 21st century perspective. Nat Rev Endocrinol 2010; 6:509-14.

  8. Neuringer M, Anderson GJ, Connor WE. The essentiality of n-3 fatty acids for the development and function of the retina and brain. Annu Rev Nutr 1988;8:517-41.

  9. Undurti N. Das. Essential fatty acids: biochemistry, physiology and pathology. Biotechnol J 2006;1:420-39.

  10. Koletzko B, Beblo S, Demmelmair H, Hannebutt FL.Omega-3 LC-PUFA Supply and Neurological Outcomes in Children with Phenylketonuria (PKU). J Pediatr Gastroenterol Nutr 2009;48(1):S2-7.

  11. Uauy R, Dangour AD. Fat and Fatty Acid Requirements and Recommendations for Infants of 0–2 Years and Children of 2–18 Years. Ann Nutr Metab 2009;55:76-96.

  12. Koletzko B, Decsi T, Demmelmair H. Arachidonic acid supply and metabolism in human infants born at full term. Lipids 1996;31:79-83.

  13. Uauy R, Hoffman DR. Essential fatty acid requirements for normal eye and brain development. Semin Perinatol1991; 15:449-55.

  14. Salem N Jr, Wegher B, Mena P, Uauy R. Arachidonic and docoahexaenoic acids are biosynthesized from their 18-carbon precursors in human infants. Proc Natl Acad Sci USA 1996;93:49-54.

  15. Burdge GC, Calder PC. Conversion of alpha-linolenic acid to longer-chain polyunsaturated fatty acids in human adults. Reprod Nutr Dev 2005;45:581-97.

  16. Innis SM. Essential fatty acid transfer and fetal development. Placenta 2005;26:S70-5.

  17. Cornejo V, Concha M, Cabello JF, Raimann E. Composición Lipídica de la Dieta de Niños Fenilquetonúricos Diagnosticados Precozmente. ALAN 2005;55(4):332-5.

  18. Goyens P, Spilker ME, Zock PL, Katan MB, Mensink RP. Conversion of linolenic acid in humans is influenced by the absolute amounts of linolenic acid and linoleic acid in the diet and not by their ratio. Am J Clin Nutr 2006;84:44-53.

  19. Rose HJ, White F, MacDonald A, Rutherford PJ, Favre E. Fat intakes of children with PKU on low phenylalanine diets. J Hum Nutr Dietet 2005;18:395-400.

  20. Infante JP, Huszagh V. Impaired arachidonic (20:4n-6) and docosahexaenoic (22:6n-3) acid synthesis by phenylalanine metabolites as etiological factors in the neuropathology of phenylketonuria. Molecular Genetics and Metabolism 2001;72:185-98.

  21. Infante JP. Vitamin E and selenium participation in fatty acid desaturation: a proposal for an enzymatic function of these nutrients. Mol Cell Biochem 1986;69(2):93-108.

  22. Weigel C, Kiener C, Meier N, Schmid P, Rauh M, Rascher W, Knerr I. Carnitine status in early-treated children, adolescents and young adults with phenylketonuria on low phenylalanine diets. Ann Nutr Metab 2008;53(2):91-5.

  23. Vilaseca MA, Briones P, Ferrer I, Campistol J, Riverola A, Castillo P, Ramon F. Controlled diet in phenylketonuria may cause serum carnitine deficiency. J Inherit Metab Dis 1993;16(1):101-4.

  24. Galli C, Agostoni C, Mosconi C, Riva E, Salari, P, Giovannini M. Reduced plasma C-20 and C-22 polyunsaturated fatty acids in children with phenylketonuria during dietary intervention. J Pediatr 1991;119:562-7.

  25. Sanjurjo P, Perteagudo L, Rodriguez Soriano J, Vilaseca A, Campistol J. Polyunsaturated fatty acid status in patients with phenylketonuria. J Inher Metab Dis 1994;17:704-9.

  26. Moseley K, Koch R, Moser B. Lipid status and long-chain polyunsaturated fatty acid concentrations in adults and adolescents with phenylketonuria on phenylalanine-restricted diet. J Inherit Dis 2002; 25:54-64.

  27. Lage S, Bueno M, Andrade F, Prieto JA, Delgado C, Legarda M, et al. Fatty acid profile in patients with phenylketonuria and its relationship with bone mineral density. J Inherit Metab Dis 2010. DOI 10.1007/s10545-010-9189-0.

  28. Cleary MA, Feillet F, White FJ, Vidailhet M, Macdonald A, Grimsley A, Maurin N, et al. Randomised controlled trial of essential fatty acid supplementation in phenylketonuria. Eur J Clin Nutr 2006;60(7):915-20.

  29. Koletzko B, Sauerwald T, Demmelmair H, Herzog M, von Schenck U, Böhles H, et al. Dietary long-chain polyunsaturated fatty acid supplementation in infants with phenylketonuria: a randomized controlled trial. J Inherit Metab Dis 2006;30(3):326-32.

  30. Pöge A, Bäumann K, Müller E, Leichsenring M, Schmidt H, Bremer H. Long-chain polyunsaturated fatty acids in plasma and erythrocyte membrane lipids of children with phenylketonuria alter controlled linoleic acid intake. J Inher Metab Dis 1998,21:373-81.

  31. Agostoni C, Massetto N, Biasucci G, Rottoli A, Bonvissuto M, Bruzzese MG, et al. Effects of long-chain polyunsaturated fatty acid supplementation on fatty acid status and visual function in treated children with hyperphenylalaninemia. J Pediatr 2000;137(4):504-9.

  32. Agostoni C, Verduci E, Massetto N, Fiori L, Radaelli G, Riva E, et al. Long term effects of long chain polyunsaturated fats in hyperphenylalaninemic children. Arch Dis Child 2003;88(7):582-3.

  33. Beblo S, Reinhardt H, Demmelmair H, Muntau AC, Koletzko B. Effect of fish oil supplementation on fatty acid status, coordination, and fine motor skills in children with phenylketonuria. J Pediatr 2007;150(5):479-84.

  34. Yi SH, Kable JA, Evatt ML, Singh RH. A cross-sectional study of docosahexaenoic acid status and cognitive outcomes in females of reproductive age with phenylketonuria. J Inherit Metab Dis 2011;34(2):455-63.

  35. Acosta PB, Yannicelli S, Singh R, Eisas II LJ, Kennedy MJ, Bernstein L, Rohr F, et al. Intake and Blood Levels of Fatty Acids in Treated Patients With Phenylketonuria. J Pediatr Gastroenterol Nutr 2001;33(3):253-9.

  36. LaVoie SM, Harding CO, Gillingham MB. Normal fatty acid concentrations in young children with phenylketonuria (PKU). Top Clin Nutr 2009;24(4):333-40.

  37. Giovannini M, Verduci E, Radaelli G, Lammardo A, Minghetti D, Cagnoli G, Salvatici E, Riva E. Long chain polyunsaturated fatty acids profile in plasma phospholipids of hyperphenylalaninemic children on unrestricted diet, Prostaglandins Leukot Essent Fatty Acids 2011;84(1-2):39-42.

  38. Burgard P, Schmidt E, Rupp A, Schneider W, Bremer HJ. Intellectual development of the patients of the German Collaborative Study of children treated for phenylketonuria. Eur J Pediatr 1996;155(Suppl 1): S33-38.

  39. Stemerdink BA, Kalverboer AF, van der Meere JJ, van der Molen MW, Huisman J, de Jong LW, Sliiper FM, Verker PH, van Spronsen FJ. Behaviour and school achievement in patients with early and continuosly treated phenylketonuria. J Inherit Metab Dis 2000;23:548-562.




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