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BACKGROUND: Glycogen storage disease type Ia (GSD-Ia) is one of the most common hepatic GSD. Its treatment mainly consists of a diet including a high intake of slow-digestion carbohydrates such as raw cornstarch and the restriction of simple sugars. This enables the maintenance of euglycemia and prevents secondary metabolic disorders. Starch is a glucose polymer formed by amylose and amylopectin, which can be obtained from distinct sources. Although uncooked cornstarch has been successfully used in the treatment of GSD-Ia, it can lead to hyperglycemia and weight gain. in vitro andin vivo tests indicated that sweet manioc starch can be potentially used in the treatment of GSD-Ia. RESULTS: The moisture analysis revealed a variation from 10.3 to 12.8% in the sweet manioc starch samples, whereas the moisture content of uncooked cornstarch ranged from 7.3 to 11.1%. Quantifiable sugar was detected in 3/5 samples of sweet manioc starch and 1/3 samples of uncooked cornstarch. Notably, this uncooked cornstarch brand is widely employed in GSD-Ia treatment in Brazil. Products B and E had higher values of amylopectin and undetectable levels of sugars. A clinical trial is warranted to compare samples F and G and determine the impact of sugar trace in the same dietary source of starch. CONCLUSIONS: Collectively, the results demonstrated possible therapeutic alternatives for GSD-Ia in addition to traditional uncooked cornstarch.

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BACKGROUND: Glycogen storage disease type 1a (GSD Ia) is characterized by severe fasting hypoglycemia. The clinical management includes the administration of uncooked cornstarch (UCCS). Although such a diet approach is effective in achieving euglycemia, its impact on the quality of life of patients should be considered. In vitro analyses suggest a longer release of glucose when using sweet manioc starch (SMS). METHODS: We compared the efficacy and safety of the administration of SMS and UCCS during a short-fasting challenge in patients with GSD Ia in a randomized, triple-blind, phase I/II, cross-over study. GSD Ia patients aged ≥ 16 years and treated with UCCS were enrolled. Participants were hospitalized for two consecutive nights, receiving UCCS or SMS in each night. After the administration of the starches, glucose, lactate and insulin levels were measured in 1-h interval throughout the hospitalization period. The procedures were interrupted after 10 h of fasting or in a hypoglycemic episode (< 3.88 mmol/L). RESULTS: Eleven individuals (mean age: 21.6 ± 4.3 years; all presenting body mass index > 25 kg/m2) participated in the study. The average fasting period was 8.2 ± 2.0 h for SMS and 7.7 ± 2.3 h for UCCS (p = 0.04). SMS maintained euglycemia for a greater period over UCCS. Increased lactate concentrations were detected even in absence of hypoglycemia, not being influenced by the different starches investigated (p = 0.17). No significant difference was found in total cholesterol, HDL, triglycerides and uric acid levels in both arms. None of the patients showed severe adverse events. CONCLUSIONS: SMS appears to be non-inferior to UCCS in the maintenance of euglycemia, thus emerging as a promising alternative to the treatment of GSD Ia.

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The aim of the present study was to characterize the physical-chemical and sensory attributes of two papaya varieties (Aliança and Golden THB) (Carica papaya L.) coated with different solutions of manioc starch and clove essential oil (Syzygium aromaticum L. Merr. et Perry). Four different treatments were studied in papaya fruits at ripening stage 1: T0 (control); T1 (fruits coated with a solution of manioc starch); T2 (fruits coated with clove essential oil at 0.175 mL L-1); T3 (solution of manioc starch with clove essential oil-0.175 mL L-1). The physical-chemical and sensory analyses were conducted in the fruits at ripening stage 5. The edible coatings did not influence the physical-chemical characteristics of the two varieties. Those fruits coated with manioc starch reported a good sensory acceptance not differing statistically from the control fruits in the majority of the attributes studied. By evaluating the purchase intention regarding the variety Aliança, all the treatments reported good levels of acceptance, with higher scores in the option "would certainly buy". After the sensory evaluation, treatment T3 reported a purchase intention of 80%. It was concluded that the edible coatings did not influence the physical-chemical characteristics of the fruits, however, they influenced the sensory acceptance of the tasters. For the variety Aliança, the application of treatment T3 is recommended, while for Golden THB, treatments T1 and T2 are recommended due to their similarity to the control.

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Objetivando reduzir os impactos ambientais decorrentes do acúmulo de plásticos sintéticos, estão sendo desenvolvidas embalagens derivadas de polímeros renováveis, os chamados filmes biodegradáveis. Empregados na cobertura de produtos desidratados e frescos são capazes de conservar suas características sensoriais, valor nutritivo e evitar o desenvolvimento de micro-organismos (MO). Com este intuito, foram produzidos filmes com amido acetilado, gelatina e plastificante impregnados com óleos essenciais de Tetradenia riparia (Hochst.) Codd e Rosmarinus officinalis nas concentrações de 0,25; 0,5; 1; 2 e 3% (em relação à massa total do filme). Os parâmetros avaliados foram a espessura, solubilidade, opacidade e a capacidade de inibição microbiana frente aos micro-organismos Escherichia coli, Staphylococcus aureus e Candida albicans. Após a adição do óleo, observou-se redução na espessura nos filmes contendo R. officinalis quando comparados ao controle negativo (CN). Já nos filmes com T. riparia, os valores foram semelhantes ao CN, indicando melhor compatibilidade entre reagentes dos filmes a esse óleo. As solubilidades desses filmes aumentaram ao incorporar os óleos, exceto nos filmes com 0,25 e 1% do óleo de T. riparia, em que os valores ficaram próximos ao CN. A opacidade dos filmes com óleo de R. officinalis aumentou com o aumento da concentração deste óleo na composição do filme, porém nos filmes com T. Riparia, ocorreu o contrário a opacidade diminuiu com o acréscimo deste óleo nos filmes. Quanto ao filme incorporado com cloranfenicol, nos três parâmetros não houve diferenças significativas ao compará-lo com o CN. No teste microbiológico, para todos os filmes analisados, os MO demonstraram resistência comparados aos halos formados pelo controle positivo. Portanto, novos estudos deverão ser realizados, objetivando aumentar a compatibilidade dos filmes com os óleos, bem como novos óleos poderão ser analisados.

In order to reduce the environmental impacts resulting from the accumulation of synthetic plastic, packaging derived from renewable polymers are being developed, which are known as biodegradable films. Used in dried and fresh products, they are able to retain their sensory characteristics and nutritional value, as well as preventing the development of microorganisms (MO). For this purpose, films with acetylated starch, gelatin and plasticizer impregnated with Tetradenia riparia (Hochst.) Codd and Rosmarinus officinalis essential oils at concentrations of 0.25, 0.5, 1, 2 and 3% (relative to the total weight of the film) have been developed. This paper assessed the thickness, solubility, opacity and the capacity to inhibit Escherichia coli, Staphylococcus aureus and Candida albicans in these films. After the addition of oil, a decrease in the thickness of film incorporated with R. officinalis could be observed when compared to the negative control (NC). However, the film incorporated with T. riparia, the values were similar to the NC, indicating better compatibility between the film reagents and this oil. The solubility of these films increased when the oils were incorporated, except for the films with concentration of 0.25 and 1% of T. riparia oil, in which the values were close to the NC. The opacity of the films with R. officinalis oil increased with the increase in the concentration of oil in the composition of the film. However, in the films with T. riparia, the opposite could be noticed, that is, the opacity decreased with the addition of this oil in the films. For the films with chloramphenicol, there were no significant differences for the three parameters measured when compared to the NC. In the microbiological test, for all films analyzed, the MO showed resistance compared to the inhibition formed by the positive control. Therefore, further studies should be conducted in order to increase the compatibility of the films with the oils, as well as analyzing new oils;

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Objetivando reduzir os impactos ambientais decorrentes do acúmulo de plásticos sintéticos, estão sendo desenvolvidas embalagens derivadas de polímeros renováveis, os chamados filmes biodegradáveis. Empregados na cobertura de produtos desidratados e frescos são capazes de conservar suas características sensoriais, valor nutritivo e evitar o desenvolvimento de micro-organismos (MO). Com este intuito, foram produzidos filmes com amido acetilado, gelatina e plastificante impregnados com óleos essenciais de Tetradenia riparia (Hochst.) Codd e Rosmarinus officinalis nas concentrações de 0,25; 0,5; 1; 2 e 3% (em relação à massa total do filme). Os parâmetros avaliados foram a espessura, solubilidade, opacidade e a capacidade de inibição microbiana frente aos micro-organismos Escherichia coli, Staphylococcus aureus e Candida albicans. Após a adição do óleo, observou-se redução na espessura nos filmes contendo R. officinalis quando comparados ao controle negativo (CN). Já nos filmes com T. riparia, os valores foram semelhantes ao CN, indicando melhor compatibilidade entre reagentes dos filmes a esse óleo. As solubilidades desses filmes aumentaram ao incorporar os óleos, exceto nos filmes com 0,25 e 1% do óleo de T. riparia, em que os valores ficaram próximos ao CN. A opacidade dos filmes com óleo de R. officinalis aumentou com o aumento da concentração deste óleo na composição do filme, porém nos filmes com T. Riparia, ocorreu o contrário a opacidade diminuiu com o acréscimo deste óleo nos filmes. Quanto ao filme incorporado com cloranfenicol, nos três parâmetros não houve diferenças significativas ao compará-lo com o CN. No teste microbiológico, para todos os filmes analisados, os MO demonstraram resistência comparados aos halos formados pelo controle positivo. Portanto, novos estudos deverão ser realizados, objetivando aumentar a compatibilidade dos filmes com os óleos, bem como novos óleos poderão ser analisados.(AU)

In order to reduce the environmental impacts resulting from the accumulation of synthetic plastic, packaging derived from renewable polymers are being developed, which are known as biodegradable films. Used in dried and fresh products, they are able to retain their sensory characteristics and nutritional value, as well as preventing the development of microorganisms (MO). For this purpose, films with acetylated starch, gelatin and plasticizer impregnated with Tetradenia riparia (Hochst.) Codd and Rosmarinus officinalis essential oils at concentrations of 0.25, 0.5, 1, 2 and 3% (relative to the total weight of the film) have been developed. This paper assessed the thickness, solubility, opacity and the capacity to inhibit Escherichia coli, Staphylococcus aureus and Candida albicans in these films. After the addition of oil, a decrease in the thickness of film incorporated with R. officinalis could be observed when compared to the negative control (NC). However, the film incorporated with T. riparia, the values were similar to the NC, indicating better compatibility between the film reagents and this oil. The solubility of these films increased when the oils were incorporated, except for the films with concentration of 0.25 and 1% of T. riparia oil, in which the values were close to the NC. The opacity of the films with R. officinalis oil increased with the increase in the concentration of oil in the composition of the film. However, in the films with T. riparia, the opposite could be noticed, that is, the opacity decreased with the addition of this oil in the films. For the films with chloramphenicol, there were no significant differences for the three parameters measured when compared to the NC. In the microbiological test, for all films analyzed, the MO showed resistance compared to the inhibition formed by the positive control. Therefore, further studies should be conducted in order to increase the compatibility of the films with the oils, as well as analyzing new oils;(AU)

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