Enteral formulas for nutritional support of patients with cystic fibrosis

Abstract

Cystic fibrosis (CF) is a hereditary disease characterized by impaired functioning of the glands of external secretion, the phenomena of maldigestion, malabsorption, chronic inflammation in the bronchopulmonary system, which negatively affects the nutritional status. Among patients with CF, regardless of age, there is a high prevalence of malnutrition, which has a multifactorial nature and leads to somatic and psychological consequences, significantly worsens the course and prognosis of the disease, and reduces the quality of life. The high-risk group consists of adolescent children due to the increased energy needs of the body during this period and the progression of the severity of the disease with age. A good nutritional status (NS) is an important parameter for successful control of the CF flow. General dietary recommendations for CF include an increased energy value of food (up to 200% of the target values of kca /day for healthy individuals) while taking pancreatic enzymes. An increased intake of protein, fat, salt, and fat-soluble vitamins is recommended. Important nutritional factors are polyunsaturated fatty acids, fiber, pre-probiotics, trace elements, and antioxidants. It is necessary to take preventive measures to prevent the development of malnutrition and timely correct existing deficiencies, taking into account the individual characteristics of the patient. For this purpose, specialized therapeutic nutrition is used. A wide range of such products in the form of ready-made (drinks, yoghurts, creamy products) or dry mixes to increase the energy and biological value of the actual diet is the best option for correcting the nutritional needs of patients with CF. They have proven clinical efficacy and safety, are well absorbed and tolerated, convenient and easy to use for patients, which significantly increases compliance and improves the result of treatment. The available assortment allows you to choose a personalized approach to each patient, taking into account the general condition, age, existing complications, and associated diseases. The review highlights the causes of nutritional deficiency, changes in approaches to diet therapy against the background of targeted therapy, enteral nutrition products recommended for patients with MV in the territory of the Russian Federation and indications for their appointment.