Pediatrician (St. Petersburg)Pediatrician (St. Petersburg)2079-78502587-6252Eco-Vector11995610.17816/PED13523-32Research ArticleConnective tissue dysplasia in children with broncho-obstructive syndromeTsymbalAnastasia A.<p>Pediatrician of the Reception Department, Children’s City Medical Center of High Medical Technologies named after K.A. Rauhfus; Postgraduate Student of the Department of Children's Diseases, Kirov Military Medical Academy</p>tsymbal.anastasi@gmail.comArsentevVadim G.<p>MD, PhD, Dr. Sci. (Med.), Professor, Head, Department of Children's Diseases</p>rainman63@mail.ruShabalovNikolai P.<p>MD, PhD, Dr. Sci. (Med.), Professor, Department of Childhood Illness</p>npshabalov@yandex.ruPahomovaMariya A.<p>Senior Research Associate</p>mariya.pahomova@mail.ruRauhfus St. Petersburg Children’s Municipal Multi-Specialty Clinical Center of High Medical TechnologyKirov Military Medical AcademySt. Petersburg State Pediatric Medical University2612202213523322512202225122022Copyright © 2022, Tsymbal A.A., Arsentev V.G., Shabalov N.P., Pahomova M.A.2022<p><strong>BACKGROUND: </strong>Changes in the respiratory system in children with connective tissue dysplasia (CTD) have not been studied enough.</p>
<p><strong>AIM: </strong>To study the features of CTD in patients of the pediatric pulmonology department with broncho-obstructive syndrome and chronic inflammation of the bronchi, to assess complaints and the nature of the disease.</p>
<p><strong>MATERIALS AND METHODS: </strong>Patients admitted to the department were evaluated according to the table of diagnostic signs of CTD. 380 children were examined, aged 2 to 17 years 11 months.</p>
<p><strong>RESULTS: </strong>Out of 380 children, the following were identified: with bronchial asthma 309, with obliterating bronchiolitis 18, with bronchiectasis 11, with recurrent bronchitis 42. Three groups were distinguished: 1<sup>st</sup> patients with CTD (172; 45%), 2<sup>nd</sup> with increased dysplastic stigmatization (111; 29%), 3<sup>rd</sup> children without signs of CTD (97; 26%). Most patients admitted to the pulmonology department have signs of CTD or dysplastic stigmatization (74%). Children with CTD often complain of feeling unwell, asthenia, joint pain, they are more likely to have orthopedic pathology. Patients of the 1st group often complain of articular syndrome 19.2%, group 2 13.5%, children without CTD 4.1 % (<em>p</em> 0.001). Complaints about feeling short of breath were presented by 32.6% of patients with CTD, 21.6% with dysplastic stigmatization, and 7.2% in the comparison group (<em>p</em> 0.001). Children with CTD and dysplastic stigmatization more often had chest deformities.</p>
<p><strong>CONCLUSIONS: </strong>Patients with CTD are characterized by asthenic physique, changes in the axial skeleton chest deformities of II and III degrees, scoliotic deformity of the spine of II and III degrees, which may contribute to the development of the pathology of the respiratory system. CTD is a comorbid condition that significantly affects all organs and systems. Timely diagnosis and an integrated approach to these patients are required. Diseases proceed with severe clinical symptoms, require more active examination and aggressive treatment.</p>connective tissue dysplasiachildrenchildhoodscoliosisbronchial asthmabronchiolitis obliteransbronchitisдисплазия соединительной тканидетидетский возрастсколиозбронхиальная астмаоблитерирующий бронхиолитбронхит[Abbakumova LN, Arsentev VG, Gnusaev SF, et al. Multifactorial and hereditary connective tissue disorders in children. Diagnostic algorithms. Management tactics. Russian guidelines. Pediatrician (St. Petersburg). 2016;7(2):5–39. (In Russ.) DOI: 10.17816/PED725-39][Arsent’ev VG, Baranov VS, Shabalov NP. Nasledstvennye narusheniya soedinitel’noi tkani kak konstitutsional’naya osnova poliorgannoi patologii u detei. 2-e edition. N.N. Atamanenko, editor. Saint Petersburg: SpetsLit, 2019. 239 p. (In Russ.)][Kalmykova AA, Fedko NA, Kalmykova VS, et al. Clinical and functional peculiarities of bronche-obstructive syndrome in children with connective tissue dysplasia. Medical news of the North Caucasus. 2019;14(1–1): 88–90. (In Russ.) DOI: 10.14300/mnnc.2019.14057][Konyushevskaya AA, Franchuk MA. Syndrome of undifferentiated connective tissue dysplasia. Pulmonological aspects. Zdorov’e rebenka. 2012;(7):147–152. (In Russ.) DOI: 10.22141/2224-0551.0.7.42.2012.193653][Moskalyuk ON, Chalaya LF. Dysplastic dependent changes of the vertebral column and thoracic cage in children. University Clinic. 2018;(3):63–67. (In Russ.) DOI: 10.26435/uc.v013(28).150][Nechaeva GI, Viktorova IA, Druk IV, Vershinina MV. Displastia of connective tissue: pulmonological aspects. Pulmonologiya. 2004;(2):116–120. (In Russ.)][Nikolaev KYu, Oteva EhA, Nikolaeva AA, et al. Displaziya soedinitel’noi tkani i poliorgannaya patologiya u detei shkol’nogo vozrasta. Pediatrics. Journal named after G.N. Speransky. 2006;85(2):89–91. (In Russ.)][Orlova NA, Etkina EI, Gurjeva LL, et al. The interconnection of connective tissue dysplasia and structural-functional state of endothelium in children with bronchial asthma. Practical medicine. 2011;(5):115–119. (In Russ.)][Poliorgannye narusheniya pri displaziyakh soedinitel’noi tkani u detei. Algoritmy diagnostiki. Taktika vedeniya. Rossiiskie rekomendatsii. Chast’ 2. Pediatric nutrition. 2017;15(3):53–79. (In Russ.) DOI: 10.20953/1727-5784-2017-3-53-79][Fedko NA, Kalmykova AS, Lagodina NA, et al. Connective tissue dysplasia syndrome as a risk factor of severe course of bronchial obstruction syndrome in children. Medical news of the North Caucasus. 2016;11(4): 540–543. (In Russ.) DOI: 10.14300/mnnc.2016.11128][Khalmatova BT, Abdullaeva DT, Sadykova LG. Osobennosti techeniya bronkhial’noi astmy, assotsiirovannoi s displaziei soedinitel’noi tkani u detei. The Unity of Science: International Scientific Periodical Journal. 2017;1:77–79. (In Russ.)][Chemodanov VV, Krasnova EE, Kopilova EB, Slatina MYu. Evaluation of respiratory function in infants with acute bronchitis on connective tissue dysplasia. Mezhdunarodnyi zhurnal prikladnykh i fundamental’nykh issledovanii. 2013;(5):48–51. (In Russ.)][Chernov IA, Malysheva EM, Timofeev SE, et al. Pulmonary fibrosis as a manifestation of systemic pathology of connective tissue. Medical science and education of Ural. 2019;22(2):66–69. (In Russ.)][Yur’eva EhA, Sukhorukov VS, Vozdvizhenskaya ES, et al. Assotsiirovannaya patologiya u detei s displaziei soedinitel’noi tkani. Praktika pediatra. 2019;(3):32–38. (In Russ.)][Sobhani-Eraghi A, Motalebi M, Sarreshtehdari S, et al. Prevalence of joint hypermobility in children and adolescents: A systematic review and meta-analysis. J Res Med Sci. 2020;25:104. DOI: 10.4103/jrms.JRMS_983_19][Strelkova M, Senatorova G. Clinical and anamnestic features of the course of acute bronchitis in children. Inter Collegas. 2018;(3):143–147. DOI: 10.35339/ic.5.3.143-147][rosstat.gov.ru [Internet]. Federal’naya sluzhba gosudarstvennoi statistiki. Zdravookhranenie [cited 2022 Sept 16]. Available at: https://rosstat.gov.ru/folder/13721 (In Russ.)]