Несовершенный остеогенез-группа редких наследственных заболеваний соединительной ткани, в основе которых лежит дефект коллагенообразования. Главным направлением медикаментозной терапии несовершенного остеогенеза является циклическое применение бисфосфонатов (памидроновой кислоты). В статье представлен порядок организации медицинской помощи и лекарственного обеспечения детей с данным заболеванием, включая разработку нормативно-правовой базы.
Juvenile arthritis (JA) is a disease of unknown etiology that begins before the age of 16 years and lasts for at least 6 weeks. The main objective of most existing international registers of children with JA is assessment various aspects of the efficacy and safety of biologics in comparison with methotrexate. The results of the analysis of the effectiveness of registers as a tool for long-term monitoring of the disease and medical care for children with JA are not presented in the available literature. The aim of the study is to develop methodological approaches to maintaining the Register of children with JA and algorithms of actions of medical personnel on the basis of the experience of the Republican Children's Clinical Hospital (Ufa). This work is a retro- and prospective observational study. The Register included children under the age of 18 years with an established diagnosis of JA, who were admitted to the cardio-rheumatologic department of Republican Children's Clinical Hospital (Ufa). In this paper, we used the All-Russian register of children with JA, developed by «Aston Consulting» and «Norbit» (official partner of Microsoft) on the basis of the technological platform Microsoft Dynamics CRM 5.0. In total, the Register included information about 426 children with JA. The average age of children during the analysis of the register data was 10.9±4.3 years, and in the onset of the disease – 4.7±3.7 years. According to the results of the analysis, the tasks, areas of responsibility and scope of action for each subject of medical care (rheumatologist of rheumatology hospital and office, head of department, chief freelance specialist of the Ministry of Health) and the corresponding tools of the Register were determined. A clear algorithm of actions of specialists depending on the task will optimize the work with the register of patients, reduce the time required to enter and receive information and, at the same time, provide the possibility of the most complete and effective use of the Register in optimizing medical care for children with JA.
INTRODUCTION: Intensive care does not always require only a large amount of resources. An important aspect is the formation of a multidisciplinary team involved in the treatment of patients in critical condition. OBJECTIVE: To demonstrate the need for the staff of intensive care units to use protocols for the diagnosis and treatment of critical conditions on the example of sepsis in children. MATERIALS AND METHODS: The analysis of publications devoted to the study of the impact of the implementation of the clinical recommendations of the Surviving Sepsis Campaign for the treatment of sepsis in children on the outcomes of the disease. The articles were searched in the abstract databases PubMed, Embase, Cochrane Central Register of Controlled Trials, Web of Science Core Collection and Google Scholar for the period from 2011 to July 2022. Keywords were used: “pediatric sepsis”, “implementation”, “protocolized treatment”, “adherence”. RESULTS: During the search, only thirteen observational studies were found, nine of which were presented retrospectively, which can be attributed to the design flaws of the submitted works. It was revealed that the use of the entire complex of diagnostic and therapeutic measures presented in clinical recommendations and protocols for intensive therapy of sepsis in children can significantly improve the results of treatment, but the commitment of doctors to their use in routine clinical practice remains low and does not exceed 40 %. CONCLUSIONS: The main reason for the lack of commitment to the implementation of recommendations and standards for the treatment of sepsis in children is not only in the presence of organizational obstacles, but also in the absence of reasonable algorithms for its implementation.
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