Objective: To perform anthropometric and dietary evaluation of patients with glycogenosis type Ia and Ib. Methods: This cross-sectional study is composed of a sample of 11 patients with glycogenosis divided into two subgroups according to the classification of glycogenosis (type Ia=5 and type Ib=6), aged between 4 and 20 years. The analyzed anthropometric variables were weight, height, body mass index, and measures of lean and fat body mass, which were compared with reference values. For dietary assessment, a food frequency questionnaire was used to calculate energy and macronutrients intake as well as the amount of raw cornstarch consumed. Mann-Whitney U test and Fisher’s exact test were performed, considering a significance level of 5%. Results: Patients ingested raw cornstarch in the amount of 0.49 to 1.34 g/kg/dose at a frequency of six times a day, which is lower than recommended (1.75-2.50 g/kg/dose, four times a day). The amount of energy intake was, on average, 50% higher than energy requirements; however, carbohydrate intake was below the adequacy percentage in 5/11 patients. Short stature was found in 4/10 patients; obesity, in 3/11; and muscle mass deficit, in 7/11. There were no statistical differences between the subgroups. Conclusions: In patients with glycogenosis type I, there was deficit in growth and muscle mass, but no differences were found between the subgroups (Ia and Ib). Although the diet did not exceed the adequacy of carbohydrates, about 1/3 of the patients presented obesity, probably due to higher energy intake.

The determination of iodine in iodized mineral dietary supplements is considered a challenge, especially in view of the variety in the sample composition and the analyte concentration. Thus, in this work, microwave-induced combustion (MIC) was combined with ion chromatography (IC) and ion-selective electrode potentiometry (ISE) for iodine determination and dose uniformity assays in mineral dietary supplements. Sample masses up to 800 mg were efficiently digested and only a diluted alkaline solution (200 mmol L-1 NH4OH) was necessary to absorb the analyte for further determination step. The final digest was fully compatible with multi-technique detection usually available in routine analysis laboratories. Recoveries ranging from 94% to 106% was achieved and relative standard deviations for repeatability and intermediate precision were always lower than 8%. Limits of quantification were 4 µg g-1 and 10 µg g-1, respectively, by using IC and ISE. The analytical method was applied for iodine determination in mineral dietary supplements from four brands with different iodine dosages (from 100 to 1250 μg g-1, according to the manufacturers) and for uniformity assay evaluation using individual tablets/capsules of mineral dietary supplements. Non-compliance regarding label information for some samples was reported, drawing the attention of supervisory institutions. The analytical strategies presented in the present study can be successfully used in routine analysis of the quality control of mineral dietary supplements.

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