Abstract Phenylketonuria (PKU) is an autosomal recessive defect affecting the metabolic pathway of phenylalanine (Phe), causing hyperphenylalaninemia and neurotoxicity. Diagnosis must occur in the neonatal period and treatment should begin as early as possible. Evidence implies that treatment adherence declines as age advances. The aim was to describe the diet of a subgroup of Chilean adults with PKU currently in follow-up. Fifty-three subjects (49% women) followed up between January 2021 to April 2023 were considered. The concentration of Phe (PheC) in dried blood spots measured by fluorometry and 24-hour dietary recalls were analyzed. The median PheC of the sample was 438µmol/L (interquartile range(IQR):351-585µmol/L). A protein intake of 1.35±0.3 gr/Kg/d was observed of which 87% came from the protein substitute without Phe. Participants had a median Phe intake of 459mg/d (IQR:327-976) and 13.1g/d of fiber intake. Most participants, 51% and 92% reported consuming fruits and vegetables, respectively, and 32% consumed Low-Protein foods. Regarding micronutrients, all participants exceeded 90% adequacy according to recommendations. For vitamin-D and vitamin-B12, 100% is provided by the protein substitute. According to our results, it is mandatory to establish transition programs toward adulthood, to constantly maintain good metabolic control, and to adapt diet therapy to their new lifestyle. (PKU Phe, , (Phe) neurotoxicity possible advances followup. followup follow up. follow-up Fiftythree Fifty three 49% 49 (49 women 202 considered (PheC 24hour hour 24 analyzed 438µmolL µmolL 438µmol L µmol interquartile rangeIQR351585µmol/L. rangeIQR351585µmolL rangeIQRµmolL range IQR 351 585µmol/L . 585µmol range(IQR):351-585µmol/L) 13503 1 35 0 3 1.35±0. grKgd gr Kg d 87 459mgd mgd 459mg mg IQR327976 327 976 (IQR:327-976 131gd gd 13 1g g 51 92 vegetables respectively 32 LowProtein Low Protein foods micronutrients 90 recommendations vitaminD vitamin D vitaminB12, vitaminB12 vitaminB B12, B12 B vitamin-B12 100 results adulthood control lifestyle (Phe 4 (4 20 2 rangeIQR351585µmol rangeIQR rangeIQR351585µmol/L 585µmolL range(IQR):351-585µmol/L 1350 1.35±0 8 IQR32797 97 (IQR:327-97 5 9 vitaminB1 B1 vitamin-B1 10 ( rangeIQRµmol 135 1.35± IQR3279 (IQR:327-9 vitamin-B 1.35 IQR327 (IQR:327- 1.3 IQR32 (IQR:327 1. IQR3 (IQR:32 (IQR:3 (IQR: (IQR

Abstract Since 1992, Chile has had a Newborn Screening Program for Phenylketonuria (PKU), which currently has an incidence of 1:18,916 newborns. The objective of the current study was to describe the 2020 follow up of the Chilean PKU cohort. The variables analyzed were: nutritional status, dietary compliance and neuropsychological functioning. We conducted a descriptive cross-sectional statistical analysis. The 271 subjects with PKU had an average age of diagnosis of 17±8 days and a phenylalanine (Phe) level of 1122±546 umol/L. Approximately 80% of protein requirement came from a protein substitute. For those <18 years of age, 80% had good dietary compliance with Phe level between 120-360 umol/L and those >18 years had a median of 522 umol/L (95%CI 468 - 636). Forty-four percent of the active PKU cohort had overweight/obesity. Eighty-five percent of the cohort >4 years of age had a normal intelligence quotient (IQ) (score 80-120). We observed a negative correlation (p <0.001; 95% CI: - 0.5, -0.2) between IQ score and Phe level. The Chilean protocol and protein substitute subsidy for life, together with the follow-up and continuous education carried out by the clinical team has encouraged compliance.

RESUMO Objetivou-se avaliar o efeito da qualidade de luz e da disponibilidade de nitrogênio na produção de fitomassa, rendimento e composição do óleo essencial de erva cidreira (Lippia alba). Estudaram-se cinco doses de N (0; 40; 80; 120 e 160 kg ha-1), tendo como fonte a ureia e três condições de qualidade de luz, obtidas com o uso de malhas fotoconversoras coloridas (azul e vermelha) com 50% de irradiância e sob pleno sol. O arranjo experimental foi em esquema fatorial 5x3, em delineamento em parcelas subdivididas, com sete repetições. Após 60 dias de cultivo sob as malhas, observou-se maior fitomassa seca das folhas (4,83 g per planta) nas plantas submetidas ao cultivo sob malha azul na dose de 102 kg ha-1 de N. As plantas cultivadas sob pleno sol apresentaram maior quantidade de neral e geranial, componentes majoritários do óleo essencial da erva cidreira, que atribuem valor comercial à espécie. O uso de malhas não exerce influência na qualidade do óleo essencial, mas aumenta a quantidade de compostos encontrados no mesmo. O nitrogênio não afeta a qualidade do óleo essencial, mas proporciona maior (0,27g per planta) rendimento de óleo, sob dose 65 kg ha-1 de N.

ABSTRACT The aim of this study was to evaluate the effect of light quality and nitrogen availability on the biomass production, yield and composition of essential oil of lemon balm (Lippia alba). Five doses of N (0; 40; 80; 120 and 160 kg ha-1) were studied, using urea and three light quality conditions obtained with the use of colored shading nets (blue and red), 50% irradiance and under full sun. The experiment was arranged in a 5x3 factorial scheme, in split plots, with seven replicates. After 60 days of cultivation under the shading nets, a higher dry leaf biomass (4.83 g per plant) was observed in the plants under blue net cultivation at a dose of 102 kg ha-1 of N. The plants grown under full sun presented greater amount of neral and geranial, major components of the essential oil of the lemon balm, which give commercial value to the species. The use of colored shading nets does not influence the composition of the essential oil of lemon balm, but increases the concentration of the compounds found in it. Nitrogen does not affect the quality of the essential oil of lemon balm, but it provides higher oil yield (0.27 g per plant) at the dose of 65 kg ha-1 of N.

Plantas de orégano foram cultivadas em diferentes ambientes de luz, obtidos com o uso de malhas coloridas, com o objetivo de avaliar o teor e a composição de óleo essencial e características anatômicas nas folhas destas plantas. Para isto, foi realizado o cultivo em vasos com capacidade para 3 dm³ submetidas aos seguintes tratamentos: cultivo sob pleno sol (testemunha absoluta), malha azul, malha vermelha e malha cinza. Foi adotado o DIC com seis repetições. Foram avaliadas características anatômicas de pecíolo e folhas, teor e composição do óleo essencial. Observa-se que a modificação no ambiente de luz possibilita modificações no aumento do teor de óleo essencial, assim como influencia na composição química deste metabólito secundário.

Oregano's plants were grown in different light environments, obtained with the use of colorful fabrics, in order to evaluate the content and composition of essential oil and anatomical characteristics in leaves of these plants. For this, the plants were grown in pots with capacity for 3 dm³ submitted to the following treatments: cultivation under full sun (absolute witness), shading with blue, red and gray colors. It was adopted the DIC with six repetitions. Anatomical features were evaluated of petiole and leaf, content and composition of essential oil. It is observed that modification in the light environment allows increasing the content of essential oil and it influences in the chemical composition of this secondary metabolite.

Introduction: There is wide availability of products containing sweeteners but there is no regulation on its consumption. Objective: To establish if adults and children with normal weight or obesity from three socioeconomic levels, and a group of adults and children with diabetes; do not exceed ADI levels for some sweeteners. Methods: Group 1 (477 adults, Group 2 (516 children) from socioeconomic levels: ABC1, C2 and C3, normal nutritional status and obesity, and group 3 (218) adults and children with diabetes. The daily intake of sweeteners was recorded, including: aspartame (ASP), acesulfame K (AK), cyclamate (CICL), saccharin (SAC), sucralose (SUC) and stevia (STV). Results: 85% adults and 75%% of children consumed products with sweeteners, and of these 50% were instant powdered beverages, soft drinks or diet yogurts. When comparing the consumption between groups 1 and 2, group 1 consumed a larger amount of sweeteners (p<0.05). Group 1 ABC1 ate more AK, ASP and SUC than C2 and C3 (p<0.05). Group 3 did not surpass the acceptable daily intake of AK, ASP, SUC and STE, but 5.8% of adults and 25% of diabetic children exceeded the ADI for SAC. Conclusions: The 97.5% and the 98.8% had a safe consumption of artificial sweeteners. It should be emphasized that 5.8% of adults and 25% of diabetic children exceeded the maximum ADI for SAC, finding that suggests to be continued long-term studies to elucidate whether this has implications for health.

Introducción: Existe gran disponibilidad de productos con edulcorantes pero no existe regulación sobre su consumo. Objetivo: determinar si individuos adultos y niños con estado nutricional normal u obesidad de tres niveles socioeconómicos y un grupo de adultos y niños con diabetes, no excedían la ingesta diaria admisible de los edulcorantes permitidos. Metodología: Grupo 1 (477 adultos) y grupo 2 (516 niños) de niveles socioeconómicos (NSE): ABC1, C2 y C3, estado nutricional normal y obesos, y grupo 3 (218) adultos y niños diabéticos. Se registró la ingesta diaria de edulcorantes incluyéndose: aspartame (ASP), acesulfamo K (AK), ciclamato (CICL), sacarina (SAC), sucralosa (SUC) y estevia (STV). Resultados: El 85 % adultos y 75 % de niños consumían productos con edulcorantes y de estos el 50% eran bebidas instantáneas en polvo, bebidas gaseosas o yogurts dietéticos. Al comparar la ingesta de edulcorantes entre los grupos 1 y 2, el grupo 1 tuvo una mayor ingesta (p<0.05) que el grupo 2. El grupo 1 del NSE ABC1, consumió mas AK, ASP y SUC que NSE C2 y C3 (p<0.05). En el grupo 3, el 5.8% de adultos y el 25% de niños diabéticos sobrepasaron el IDA sólo para SAC. Conclusiones: El 97.5% adultos y el 98.8% niños tuvieron ingesta dentro del nivel seguro en cada edulcorante. Se debe enfatizar que el 5,8% de adultos y 25% de niños diabéticos excedieron el IDA máximo para SAC, hallazgo que sugiere continuar con estudios a largo plazo que permitan dilucidar si esto tiene repercusión para la salud.

Phenylketonuria (PKU) is caused by a deficiency of the enzyme Phenylalanine Hydroxylase, resulting in increased plasma Phenylalanine (Phe). The aim of this study was to assess the intake of Selenium (Se), Zinc (Zn) and vitamin E in PKU children, who were on a diet restricted in Phenylalanine (Phe) and receiving a special formula without Phe. Method: the study included 50 PKU children between 0 and 13 years controled at INTA, University of Chile. We analyzed intake of vitamin E, Se andZn, measured Phe plasma levels and assessed nutritional status. Results: The daily recommendations ofSe and Zn were 100% covered with the usual PKU diet. By excluding the formula without Phe, the coverage of both nutrients decreased to 45% and 20% respectively. The intake of vitamin E was covered by the consumption of vegetable oils and when the formula without Phe was included, the coverage was increased 5 times over the recommended levels. Blood Phe level remained on average at 5.4 mgldL, considered a good metabolic control. Conclusions: We conclude that the diet of PKU children covered the recommended levels ofZn, Se and vitamin E. However, it should be emphasized the importance of the formula without Phe to meet nutritional recommendations, particularly of micronutrients.

La Fenilquetonuria (PKU) se produces por la deficiencia de la enzima Fenilalanina Hidroxilasa, causando un aumento plasmático de fenilalanina (FA). El objetivo del presente estudio fue evaluar la ingesta de Selenio (Se), Zinc (Zn) y vitamina E en niños PKU menores de 13 años de edad, que estaban en dieta restringida en FA y recibiendo fórmula especial sin FA. Método: Se incluyeron 50 PKU entre los 0 y 13 años y en control en el INTA, Universidad de Chile. Se analizó ingesta de vitamina E, Se y Zn, se midió nivel de FA en plasma y se evaluó estado nutritional. Resultados: Las recomendaciones diarias de Se y Zn se cubren en un 100% con la dieta habitual de PKU. Pero al excluir la formula sin FA, la cobertura de ambos nutriente disminuye a 45% y 20 % respectivamente. La ingesta de vitamina E se cubre en forma natural por el consumo de aceites vegetales y al incluir la vitamina de la fórmula sin FA, la cobertura se incrementaba 5 veces sobre su recomendación. El nivel de FA en la sangre fue en promedio de 5.4 mg/dL, considerado un buen control metabólico. El 64% tenía un estado nutritional normal, el 30% estaba sobrepeso u obeso y un 6% riesgo de desnutrición. Conclusiones: Se concluye que la dieta de niños PKU cubre las recomendaciones de los micronutrientes: Zn, Se y vitamina E. No obstante se debe enfatizar la importancia que tiene la fórmula sin FA para cumplir con las recomendaciones nutricionales, especialmente de micronutrientes.