Orphan diseases in children: experience in the use of oedematous enteral nutrition

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Introduction. Increased protein and energy requirements, as well as intoxication symptoms in patients with malignant neoplasms (MN), spinal muscular atrophy (SMA) and cystic fibrosis (CF) determine the need to use specialized protein detoxification nutrition in their diet.Aim. To evaluate the tolerability and clinical efficacy of the domestic specialized food product of dietary therapeutic and dietary preventive nutrition Protein detoxification cocktail NUTRIO (liquid mixture) in children with orphan diseases, over 2 years old. Materials and methods. We observed 18 children with rare diseases, aged from 2 years 11 months to 16 years. 6 children had a confirmed diagnosis of MN, 10 – SMA, 2 – CF. All children received the NUTRIO Detox Protein Cocktail twice a day: children aged 3 to 7 years old – ½ serving (100 ml), and aged 8 to 16 years – 1 serving (200 ml). The observation period was 1 month.Results. It was found that all patients (100% of patients) highly rated the taste of the product. There were no refusals from the product. No allergic, dyspeptic or other side effects were noted during the observation period. All patients showed positive dynamics of nutritional status, an increase in the level of total protein and albumin in the blood, a decrease in markers of intoxication and inflammation, an improvement in the quality of life, increased endurance, physical activity and an improvement in the psycho-emotional background.Discussion. The results we obtained correlate well with the authors’ data on the clinical efficacy, safety and significant economic benefits of using this diet.Conclusions. The use of the domestic specialized food product of dietary therapeutic and dietary preventive nutrition Protein detoxification cocktail NUTRIO in diet therapy of children with malignant neoplasms, SMA and CF is effective for correcting their nutritional status and improving the quality of life.