Experience of correction of enzyme therapy in children with cystic fibrosis using computer systems and means of network communication
Authors: Maksimycheva T. Yu. , Kondratyeva E. I. , Tlif A. I. , Basova A. V. 
Published: March 2024

Justification. In recent years, great progress in the treatment of cystic fibrosis has been associated with the use of pathogenetic therapy (since 2012 - in world practice, since 2020 in the Russian Federation). Against the background of the use of which the nutritional status, the functional state of the lungs improves, but there is no complete restoration of the impaired functions of the organs, in particular, the external secretory function of the pancreas is restored only in young children with the timely appointment of targeted therapy The purpose of the study: to increase the effectiveness of correction of enzyme replacement therapy by an individualized approach. Methods. 140 children (boys - 81, girls - 59) with MV aged from 1 year to 18 years (average age 12.9±4.8 years) were examined. The study was conducted in 2022 in the Department of Cystic Fibrosis Scientific and «Research Clinical Institute of Childhood of the Ministry of Health of the Moscow Region» (clinical base of the scientific and clinical Department of Cystic fibrosis Research Center for Medical Genetic). Inclusion criteria - diagnosis of cystic fibrosis, chronic pancreatic insufficiency, severe. Exclusion criteria - short bowel syndrome, exacerbation of the disease. The doses of pancreatin per day were evaluated using the calculation method: units/g of fat in food and intestinal syndrome before and after correction using an algorithm of an individualized approach to calculating enzyme therapy. The observation period is 3 months Design: single-center, prospective, comparative Results. The study showed that the majority of 82 patients (59%) in the general group received less than 2000 U/g of fat, which is less than the reference values (2000-4000 U/g). The median daily dose was 1500 U / g of fat, which can be attributed to the peculiarities of dosing and the need for pancreatin in the Russian pediatric population of patients with cystic fibrosis. The results are consistent with the data of recent studies indicating an acceptable dosage range of pancreatin 1000-4000 units / g of fat in food. In the course of the study, an algorithm for individualized correction of enzyme replacement therapy was developed and the effectiveness of this approach was shown. Conclusion. When calculating pancreatin for the fat content in food, the dose was below the recommended values of 2000-4000 units / g and amounted to 1500 units / g, which indicated that it did not meet the needs of children in enzymes and required individual correction. This served as the basis for the development of an algorithm for individualized correction of enzyme replacement therapy. The effectiveness of this approach using computer technology (inpatient program for doctors and mobile version for patients), in the form of reduction / relief of intestinal syndrome, is shown. To increase compliance and to train parents to control diet and enzyme replacement therapy, it is advisable to use computer programs and mobile applications to calculate the diet and dosage of pancreatin for each meal.