O aleitamento materno no tratamento de crianças com fenilcetonúria

Abstract

Objective: To evaluate the effect of breastmilk as a source of phenylalanine (phe) on levels of this amino acid and on growth in phenylketonuric infants. Methods:The study recruited 35 breastfed phenylketonuric infants and compared their results with those of 35 infants fed on commercial, milk-based formula. The groups were paired for sex and age at weaning from breastfeeding.Data were analyzed up until cessation of breastmilk or for 12 months' follow-up. The breastfed group were given a "special formula" free of phe, by bottle every 3 hours, and breastmilk at will during the intervals. Levels of phe in the blood, collected weekly up to 6 months and fortnightly up to 1 year de age, were analyzed while breastfeeding continued.The two groups were compared in terms of the time taken for the levels of phe in blood to return to normal after treatment was started, using the Wilcoxon test. Anthropometric data were compared with Student's t paired test in the form of z scores. The phe assays were analyzed throughout breastfeeding. Results:The median time taken for phe levels to return to normal was 8 days for the breastfed group and 7 days for the control group. The phe assay results were normal in 87% of tests for the breastfed group and in 74.4% for the control group. The majority of children in both groups exhibited a z score > -2 on anthropometric examination. Conclusions:Continuation of breastfeeding, during the treatment, proved adequate for metabolic control and growth in children with phenylketonuria.J Pediatr (Rio J). 2007;83(5):447-452: Breastfeeding, phenylketonuria, phenylalanine.

Breast-feeding Success Among Infants With Phenylketonuria

“…In addition, combining breast milk and Phe-free formula in managing the diet of infants with PKU did not adversely affect Phe control (Greve, Wheeler, Green-Burgeson, & Zorn, 1994; McCabe et al, 1989). In more recent studies, breast-fed infants with PKU had no significant differences in weight gain, daily Phe intake, and the mean plasma Phe concentrations compared to formula-fed infants with PKU (Cornejo et al, 2003; Demirkol et al, 2001; Huner, Baykal, Demir, & Demirkol, 2005; Kanufre et al, 2007; McCabe et al, 1989; Motzfeldt et al, 1999; van Rijn et al, 2003). Although based on relatively small samples (usually < 50 participants) reflecting the rarity of PKU in the population, the results from these studies consistently supported the conclusion that breast milk supplemented with Phe-free formula is an acceptable dietary treatment for infants with PKU.…”

“…For example, an Italian study identified one-year breastfeeding rates were significantly lower in infants with PKU than in the general population (Agonstoni, Verduci, Fiori, Riva, & Giovannini, 2000). Most recently, only 19 of 35 Brazilian mothers were found to be breastfeeding at 6 months and only 11 of these mothers continued to breastfeed at 12 months (Kanufre et al, 2007). …”

Recommendations for the nutrition management of phenylalanine hydroxylase deficiency

“…59 Although there is little consensus about the best way to incorporate breast milk into the diet of an infant with PAH deficiency, there is agreement that feeding at the breast, as well as feeding expressed breast milk by bottle, results in good metabolic control. 60 Later in the first year, breast milk or infant formula is slowly removed in exchange for limited amounts of intact protein from solid foods containing an equivalent amount of PHE.…”

Consumo alimentar de lactentes com fenilcetonúria em uso de aleitamento materno

“…A partir da década de 80, entretanto, a utilização do leite materno associado a uma fórmula especial isenta em phe demonstrou ser efetiva na manutenção de níveis sanguíneos de phe e na promoção do crescimento e desenvolvimento adequados de lactentes com PKU em uso de leite materno 4,[6][7][8][9][10][11] .…”

“…O SEG-HC-UFMG introduziu o aleitamento materno no tratamento da PKU em 2000, e atualmente a maioria dos lactentes admitidos no Serviço faz uso do mesmo 11 .…”