Nutrient intake of breastfed infants with phenylketonuria
Keywords:
Breast feeding, Phenylketonurias, Risk groups nutrition, Infant nutritionAbstract
Objective
This study aimed to assess energy, phenylalanine, tyrosine and protein intakes of breastfed infants with phenylketonuria (breastfed group).
Methods
A retrospective/prospective cohort study was used to compare a group of 39 breastfed infants aged 6 months or less (breastfed group) with a control group being fed a special low-phenylalanine formula. The groups were paired by gender and breastfeeding duration. A total of 719 dietary recalls of the breastfed group and 628 of the control group was reviewed. Anthropometric assessment was done at baseline and end of study. Nutrient intakes were calculated by the software Minitab and LogXact 4.0 and anthropometric assessment was done by the software Epi Info 6.0.
Results
The breastfed group presented adequate intake of phenylalanine and tyrosine and more adequate protein and energy intakes than the other group. Most infants, regardless of group, presented z-scores within the normal range (Z u-2), with good progression of the studied indicators (weight-for-age, height-for-age, weight-for-height and head circumference).
Conclusion
Breastfeeding of infants with phenylketonuria provided adequate energy, phenylalanine, tyrosine and protein intakes. The likelihood of a child in the breastfeed group to present adequate energy intake was 10.64 times higher than that of a child in the control group. In relation to protein intake, the chance was 5.34 times higher. Both groups presented similar growth.
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References
Scriver CR, Kaufman S. Hiperphenylalaninemia: phenylalanine hydroxilase deficiency. In: Scriver CR, Beauted AL, Sly WS, Valle D, editors. The metabolic and molecular bases of inherited disease. 8th ed. New York: McGraw-Hill; 2001. p.1667-724.
Aguiar MJB. Genetic services and research in the State of Minas Gerais-Brazil. Comm Genet. 2004; 7(2-3):117-20.
Shaw V, Lawson M. Disorders of amino acid metabolism, organic acidaemias and urea cycles defects. In: Shaw V, Lawson M, editors. Clinical paediatric dietetics. London: Blackwell Science; 1994. p.177-209.
Cornejo VE, Raimann B. Errores innatos del metabolismo de los aminoácidos. In: Colombo MC Cornejo VE, Raimann B, editors. Errores innatos en el metabolismo del niño. Santiago do Chile: Universitária; 1999. p.59-66
Acosta PB, Yannicelli S. Phenylketonuria (PKU). In: Acosta PB, Yannicelli S, editors. The Ross metabolic formula system, nutrition support protocols. 4th ed. Columbus: Ross Laboratories; 2001. p.1-49.
McCabe ERB, McCabe L. Issues in the dietary management of phenylketonuria: breast-feeding and trace-metal nutriture. Ann N Y Acad Sci. 1986; 477:215-22.
Greve LC, Wheeler MD, Green-Burgeson DK, Zorn EM. Breast-feeding in the management of the newborn with phenylketonuria: a practical approach to dietary therapy. J Am Diet Assoc. 1994; 94(3):305-9.
Motzfeldt K, Lilje R, Nylander G. Breastfeeding in phenylketonuria. Acta Paediatr. 1999; 88(12):25-7.
Cornejo VE, Manríquez VE, Colombo MC, Mabe OS. Fenilquetonuria de diagnóstico neonatal y lactancia materna. Rev Méd Chile. 2003; 131(11): 1280-87.
Rijn MV, Bekhof J, Dijkstra T, Smit PG, Moddermam P, Spronsen FJV. A different approach to breastfeeding of the infant with phenylketonuria. Eur J Pediatr. 2003; 162(5):323-6
Kanufre VC, Starling ALP, Leão E, Aguiar MJB, Santos JS, Soares RDL, et al. O aleitamento materno no tratamento da fenilcetonúria. J Pediatr. 2007; 83(6):447-52.
Butte NF, Lopez-Alarcon MG, Garza C. Nutrient adequacy of exclusive breastfeeding for the term infant during the first six months of life. Geneve: WHO; 2002.
Wappner R, Cho S, Kronmal RA, Schuett V, Seashore MR. Management of phenylketonuria for optimal outcome: a review of guidelines for phenylketonuria management and report of surveys of parents, patients, and clinic directors. Peditrics [Internet].1999 [cited 2006 Mar 5]; 104)6): e68. Available from: <http://www.peditrics.org.br>.
Food and Nutrition Board. National Research Council. Recommended dietary allowances. Washington (DC): National Academy Press; 1989 [cited 2006 Feb 25]. Available from: <http://www.nap.edu/catalog/1349.html>.
Diet PRO 3 tecnologia para nutrição. Versão 4.0 [CD-Rom]. Viçosa: A.S. Sistemas; 2002
National Center of Health Statistics. Growth curves for children, birth -18 years. Hyattsville: National Center of Health Statistics; 1977. Series 11, Number 165, DHS Publication.
Dean AG, Dean JA, Coulombier D, Brendel KA, Smith DC, Burton AH, et al. Epi Info, Version 6. Atlanta (Georgia): Centers of Disease Control and Prevention; 1996.
Minitab Statistical Software. Minitab StatGuide: release 14 for Windows. State College (PA): State College of Pennsylvania; ©2000-2003.
Cytel Software. Logxact-4.0 Version 4.1. Cambridge (MA): Cytel Statistical Software & Services; ©2000
Euclydes MP. Aleitamento materno. In: Euclydes MP. Nutrição do lactente: bases científicas para uma alimentação adequada. Viçosa: Universidade Federal de Viçosa; 1997. p.245-335.
Dewey KG. Growth characteristics of breast feeding compared to formula-fed infants. Biol Neonate. 1998; 74(2):94-105
Fisberg RM, Silva-Fernandes ME, Schmidt BSJ. Nutritional evaluation of children with phenilketonuria. Rev Paul Med. 1999; 117(5):185-91.
British and European guidelines for the treatment of PKU. British guidelines for PKU management: report of Medical Research Council Working Party on PKU and recommendations of the European Society for PKU on dietary management of PKU. Seattle: National PKU News; 1998.
Acosta PB, Yannicelli S, Marriage B, Steiner R, Gaffield B, Arnold G, et al. Protein status of infants with phenylketonuria undergoing nutrition management. J Am Coll Nutr. 1999; 18(2):102-7.
Spronsen FJV, Rijn MV, Bekhof J, Koch R, Smit PG. Phenylketonuria: tyrosine supplementation in phenylalanine restrict diets. Am J Clin Nutr. 2001; 73(2):153-7.
Bross RO, Ball R, Clarke JTR, Pencharz PB. Tyrosine requirements in children with classical PKU determined by indicator amino acid oxidation. Am J Physiol Endocrinol Metab. 2000; 278(2):195-201.
Acosta PB, Wenz E, Williamson M. Nutrient intake of treated infants with phenylketonuria. Am J Clin Nutr. 1977; 30(2):198-208.
Acosta PB, Yannicelli S, Marriage B, Mantia C, Gaffield B, Porterfield M, et al. Nutrient intake and growth of infants with phenylketonuria undergoing therapy. J Pediatr Gastroenterol Nutr. 1998; 27(3): 287-91.
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Copyright (c) 2023 Jacqueline Siqueira SANTOS, Marcos José Burle de AGUIAR, Ana Lúcia Pimenta STARLING, Viviane de Cássia KANUFRE, Jacqueline Domingues TIBÚRCIO, Marília Oliveira Barbosa LIMA

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