[Specialized products for nutrition of children with mucoviscidosis]

Mucoviscidosis is serious inherited disease with lesions of almost all secretory cells of mucosa and exocrine glands, following by disturbances of metabolism, digestion and nutritional status. Using of modern high effective pancreatic enzymes does not allow to reach a normalization of protein metabolism and digestion especially of fats. For success treatment of this disease the effective dietetic methods should be used including dietary intake of special food products which can supply enough energy and nutrients in small volume. The nutrient contents of a few foreign substitution of breast milk and food product for older children are given. The results of treatment of 86 children suffering from mucoviscidosis are presented. Milk proteins (casein, whey proteins), hydrolysates of milk protein and soy protein isolates are main resources of protein component in special food. Fat components are produced from middle-chain triglycerides (60-70%) and vegetable oils. Carbohydrates in special food are made from glucose polymers, glucose, fructose and sucrose. Positive results of clinical trials of foreign special food have been an example for creation of two home-produced food for treatment of children with mucoviscidosis: 1) adapted version "A" for children in age under 1 year, and 2) version "B" for older children. The products were created on basis of native milk protein with ratio casein to whey protein as 60:40. Middle-chain triglycerides compose 70-75% of fats. Carbohydrates are presented by dextrin-maltose (85%), glucose and sucrose. In present time these special products are under clinical trial in scientific medico-genetic center of Russia' Academy of Medical Science.