Abstract
Background: The primary treatment for inborn errors of metabolism (IEM) involves restricted intake of natural protein. Inadequate diets can lead to an increased risk of inflammation and susceptibility to infections. The Dietary Inflammatory Index (DII) is used to estimate whether a diet has anti-inflammatory or pro-inflammatory properties. This study aimed to investigate the relationship between the inflammatory index score of natural protein-restricted diets used in medical nutrition therapy for IEM intoxication, the anthropometric measurements and nutritional status of affected children. Method: The study included 20 patients (5 organic acidemia, 5 urea cycle disorders, 10 phenylketonuria) and 20 healthy children. Patients followed a natural protein-restricted diet, while the healthy control group maintained their usual dietary habits. Dietary records were collected for both groups, and the DII and macro-micronutrient intakes were calculated. Result: DII scores were similar between the patient and control groups. Anthropometric measurements did not differ significantly between the groups. However, carbohydrate and fat intakes were higher in the patient group compared to the control group (p < 0.05). Additionally, comparative analyses revealed that vitamin B1, C and E, iron, and magnesium intakes were higher in the patient group than in the control group. Conclusions: Children on a natural protein-restricted diet showed growth patterns comparable to their healthy peers. This study demonstrated that nutritional deficiencies can be prevented in amino acid metabolism disorders treated with a natural protein-restricted diet by carefully controlling nutrition with vitamin and mineral-fortified formulas.