vspВопросы современной педиатрииCurrent Pediatrics1682-55271682-5535Издательство «ПедиатрЪ»10.15690/vsp.v12i1.574vsp-385Research ArticleВ ПОМОЩЬ ВРАЧУA DOCTOR’S AIDНЕКОТОРЫЕ АСПЕКТЫ ПОРАЖЕНИЯ ОРГАНОВ ДЫХАНИЯ И ПИЩЕВАРЕНИЯ ПРИ МУКОВИСЦИДОЗЕ У ДЕТЕЙCERTAIN ASPECTS OF RESPIRATORY AND DIGESTIVE TRACT INVOLVEMENT IN CYSTIC FIBROSIS IN CHILDRENВасильеваТ. Г.Vasil’evaT. G.

Vasilyeva Tatyana Gennagyevna, PhD, MD, head of Regional Children’s Consultative Outpatient Clinic SAHI “Regional Clinical Center of Specialized Types of Healthcare” Address: 690091, Vladivostok, Uborevicha str., 30/37, Tel.: (4232) 40-61-24, e-mail: kkcsvmp@moybox.ru; vladmedic@mail.primorye.ru

vladmedic@mail.primorye.ruШишацкаяС. Н.ShishatskayaS. N.vladmedic@mail.primorye.ruНиА. Н.NiA. N.vladmedic@mail.primorye.ruКраевой клинический центр специализированных видов медицинской помощи», Владивосток; Дальневосточный филиал ФГБУ «НЦ ПЗСРЧ» СО РАМН, ВладивостокРоссияTerritorial Centre of Specialized Medical Care, Vladivostok; Far Eastern Branch of the FSFI “Scientific Center of Family Health and Human Reproduction Problems” of the Siberian Department of RAMS, VladivostokRussian FederationВладивостокский государственный медицинский университетРоссияVladivostok State Medical University, Russian FederationRussian FederationВладивостокский государственный медицинский университетРоссияVladivostok State Medical UniversityRussian Federation201303022013121Вопросы современной педиатрии162165Copyright © Васильева Т.Г., Шишацкая С.Н., Ни А.Н., 20132013Васильева Т.Г., Шишацкая С.Н., Ни А.Н.Vasil’eva T.G., Shishatskaya S.N., Ni A.N.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://vsp.spr-journal.ru/jour/article/view/385

Муковисцидоз относится к тяжелым формам патологии детского возраста с ранней манифестацией клинической картины полиорганного поражения. Наиболее тяжело при этой патологии страдают органы дыхания и пищеварения, степень повреждения которых определяет прогноз болезни. В статье приведены результаты анализа заболеваемости муковисцидозом в Приморском крае по данным определения неонатального иммунореактивного трипсина у 102 251 новорожденного. Изучена структура муковисцидоза по возрасту, полу, клиническим формам и степеням тяжести заболевания у 71 ребенка. Определена распространенность 13 мутаций гена трансмембранного регулятора проводимости у 41 больного. Выявлены корреляции между сроком манифестации легочных осложнений, инфицированием P. aeruginosa, степенью хронической гипоксемии, нутритивной недостаточности, генетическим статусом и тяжестью течения заболевания.

Ключевые слова: муковисцидоз, дети, органы дыхания, пищеварения.

Cystic fibrosis is one of the severe childhood disorders with early manifestation of multiple organ involvement. The respiratory and digestive tracts are suffered the most, the prognosis of disease course depends on the degree of the impairment of these systems.   The results of the analysis of cystic fibrosis prevalence in Primorsky Krai according to the neonatal immunoreactive trypsin screening in 102 251 newborn are shown in the article. The age, sex, clinical and severity structure of cystic fibrosis was studied in 71 children. The prevalence of 13 mutations of transmembrane regulator conductance gene in 41 patients was determined. Correlations between time of pulmonary complications manifestation, P. Aeruginosa infection development, chronic hypoxemia degree, malnutrition, genetic status and severity of the disease were found.

муковисцидоздетиорганы дыханияпищеваренияcystic fibrosischildrenrespiratory tractdigestive tractReferencesKrasovskiy S. A., Chernyak A. V., Amelina Ye. L., V. S. Nikonova, A.Yu. Voron kova, N.Yu. Kashirskaya, N. I. Kapranov. Survival rate of patients with cystic fibrosis in Moscow and Moscow Region during 2000–2010. Cystic fibrosis in children and adolescents: collection of articles and theses, X Anniversary National Congress. Yaroslavl. 2011. 49 pp.Krasovskiy S. A., Chernyak A. V., Amelina Ye. L., V. S. Nikonova, A.Yu. Voron kova, N.Yu. Kashirskaya, N. I. Kapranov. Survival rate of patients with cystic fibrosis in Moscow and Moscow Region during 2000–2010. Cystic fibrosis in children and adolescents: collection of articles and theses, X Anniversary National Congress. Yaroslavl. 2011. 49 pp.Vasilyeva T. G., Shishatskaya S. N. Risk factors of unfavorable outcome of cystic fibrosis in children. Collection of materials of 15th Congress of Pediatricians of Russia with International Participation “Actual problems of pediatrics”. Moscow. 2011. 117 pp.Vasilyeva T. G., Shishatskaya S. N. Risk factors of unfavorable outcome of cystic fibrosis in children. Collection of materials of 15th Congress of Pediatricians of Russia with International Participation “Actual problems of pediatrics”. Moscow. 2011. 117 pp.Kapranov N. I., Kashirskaya N.Yu, Tolstova V. D. Cystic fibrosis. Early diagnostics and treatment. Moscow: GEOTAR-Media. 2008.Kapranov N. I., Kashirskaya N.Yu, Tolstova V. D. Cystic fibrosis. Early diagnostics and treatment. Moscow: GEOTAR-Media. 2008.Modern classification of clinical forms of bronchopulmonary diseases in children. Pediatriya. Zhurnal imeni G. N. Speranskogo — Pediatrics. 2010; 89 (4): 6–15.Modern classification of clinical forms of bronchopulmonary diseases in children. Pediatriya. Zhurnal imeni G. N. Speranskogo — Pediatrics. 2010; 89 (4): 6–15.Rebrova O.Yu. Statistical analysis of medical data. Use of a package of applied programs STATISTICA. Moscow: MediaSphera. 2006.Rebrova O.Yu. Statistical analysis of medical data. Use of a package of applied programs STATISTICA. Moscow: MediaSphera. 2006.Radionovich A. M., Kashirskaya N.Yu., Kapranov N. I. Clinical significance of prolonged treatment with sub therapeutic doses of macrolides in patients with cystic fibrosis and chronic Pseudomonas aeruginosa infection. Pulmonology: appendix on cystic fibrosis. 2006; 40–46.Radionovich A. M., Kashirskaya N.Yu., Kapranov N. I. Clinical significance of prolonged treatment with sub therapeutic doses of macrolides in patients with cystic fibrosis and chronic Pseudomonas aeruginosa infection. Pulmonology: appendix on cystic fibrosis. 2006; 40–46.Tsyvkina G. I., Vasilyeva T. G., Grishchenko D.Zh. Immunotropic therapy in patients with cystic fibrosis. Collection of materials of XVI Russian National Congress “Human and Medication”. Moscow. 2009. Pp. 466–467.Tsyvkina G. I., Vasilyeva T. G., Grishchenko D.Zh. Immunotropic therapy in patients with cystic fibrosis. Collection of materials of XVI Russian National Congress “Human and Medication”. Moscow. 2009. Pp. 466–467.Pavlova Ya.Ye. Endoscopic and functional evaluation of mucous tunic of gastrointestinal tract in syndrome of intestinal malabsorption in children. Author’s abstract of a doctoral dissertation. Irkutsk. 2005. Pp. 52–58. 9. Gastroenterology of children’s health. Edited by Belmer S. V., Khavkin A. I. Moscow: Medpractice. 2003.Pavlova Ya.Ye. Endoscopic and functional evaluation of mucous tunic of gastrointestinal tract in syndrome of intestinal malabsorption in children. Author’s abstract of a doctoral dissertation. Irkutsk. 2005. Pp. 52–58. 9. Gastroenterology of children’s health. Edited by Belmer S. V., Khavkin A. I. Moscow: Medpractice. 2003.

The authors declare that there are no conflicts of interest present.