Phenylketonuria: Nutritional survey with quantification of phenylalanine intake in childhood: assessing the need of nutritional guidance in patients with Hyperphenylalaninemia. Preliminary report

C FRAGA ;; G. VALLE.; A CHIESA.; R ENACAN,

Rio de Janeiro

Congreso; 13th International Congress of Inborn Errors of Metabolism .; 2017

SOCIEDAD LATINOAMERICANA DE ERRORES CONGENITOS Y PESQUISA NEONATAL (SLEIMPN)

Food habits differ worldwide from one culture to another. This is important when recommending a normal diet to patients with mild forms of metabolic disorders.OBJECTIVE: Estimate the average intake of nutrients, especially phenylalanine (phe), in children without metabolic or nutritional pathology of our country, to determine the need of nutritional guidance in patients with hyperphenylalaninemia (HFA) and to indicate the protein and phenylalanine intake during pregnancy in the HFA mother.METHODOLOGY:A specially designed survey was administered to 61 children attending the Endocrinology Division of the R. Gutierrez Children´s Hospital, Buenos Aires, Argentina. Those who had previous nutritional intervention or health condition who could have modified their diet, were excluded. They were divided in three age groups: 6-23 months (n:12), 2-5 years (n:21), 6 -10 years (n:28) A 24-hour food record of the consumption of everything ingested the day prior to the survey was built. Visual models (Nasco Food) were used to specify portions, in raw and cooked weight. Preparation´s composition was standardized prior to the survey. Nutritional information was analyzed with SARA system (Food composition analyzer). Own constructed tables with phe values, and information provided by food processing companies in cases in which the other sources lacked information were used. Average protein intake (API), exceeding percentage from RDAs (E),%High biological value proteins (HBVP), Phe intake (PHI) ,% Phe covered from food >0.75mg or beverages containing aspartame (HPF)..RESULTS expressed as Median (M) and Range (R)6-23 months: API: M: 30.9 g/day (12,45 - 49,6);.E: M 159.6 %(13.2-281.5);HBVP: 87 % (19-98);PHI: M:1348.7 mg/day (469 -2424).HPF: M:95.4% (83-100)2-5 years: API: M:46.1 g (17-73).E: M: 202.7 (30.8-461.5);HBVP: M:65% (20-84);PHI: M: 2264 mg/day (913mg - 3308 mg); HPF: M:98% (61-100)6-11 years: API: M:49.2 g (18.9-102);HBVP: M: 66 % (16-86);E: M: 112.8 (0-436.8)PHI: M: 2350.5 mg/day (900- 4228);HPF: M: 99% (28-100)CONCLUSION:Our preliminary results show that the average intake of protein and phenylalanine in Argentina, exceeds the protein recommendations issued by the RDAs in the groups assessed by the survey. If confirmed with a larger number of observations, our data support the need out of nutrition education in patients with HFA to ensure an adequate evolution of the pathology.