POLYMORPHISM OF THE MANIFESTATIONS OF CONNECTIVE TISSUE DYSPLASIA IN CHILDREN
DOI:
https://doi.org/10.11603/24116-4944.2023.2.14337Keywords:
connective tissue dysplasia syndrome, joint hypermobility, strabismus, flat feetAbstract
The aim of the study – to pay the attention of practicing doctors to the multifaceted manifestations of connective tissue dysplasia, which is masked by various clinical manifestations and requires an interdisciplinary approach to timely diagnosis, treatment and prevention of these changes. Only comprehensive examination and treatment can guarantee positive results of therapy.
Materials and Methods. The clinical examination was carried out on the basis of the communal non-profit enterprise "Ternopil Regional Children's Clinical Hospital" of the Ternopil Regional Council. A boy with joint hypermobility syndrome was examined during long-term clinical observation (from 3 months to 14 years). The basic examination method was x-ray (standard x-ray equipment) and ultrasound – the "MEDISON" SA-800 EX device. Documentation was carefully studied – outpatient and inpatient charts. The obtained results were compared with the data of other scientists.
Results and Discussion. The study of connective tissue dysplasia occupies a prominent place in modern pediatrics. More and more scientists consider these changes to be serious deviations from the norm that are difficult to treat. The final cause of this disease has not been studied. Some believe that this is a sporadic disease, while others believe that it is hereditary. More and more scientists are paying attention to the important role of morphogenetic proteins, which indirectly or directly control the complex process of connective tissue morphogenesis, which is the basis and foundation of all body organs and systems. Our clinical observation showed all the complexity and prevalence of changes in the child's body, which were caused by changes in collagen fibers. The first bells of such disorders appeared at the age of 3 and were considered as one disease – dysplasia of the hip joints. X-ray of the hip joints revealed dysplastic changes in the hip joints, delayed enchondral calcification of the heads of both femurs. In the process of growth, the clinical manifestations of SDST gradually increased – flat feet, scoliotic posture, disorders of psychomotor development, anomalies of bite, eyes. This was especially clearly manifested in the period of puberty – obesity, hypothalamic syndrome of puberty, autonomic dysfunction, gallbladder dysfunction, the appearance of new behavioral reactions that were not characteristic of it before, because puberty is an extremely important stage of adolescent development and is equated with the second birthday.
Conclusions. Connective tissue dysplasia has various clinical manifestations. Hypermobility of joints is easily accessible and the main clinical criterion for these changes. The combination of hypermobility of the joints with other diseases, which are given in our clinical observation, give grounds for asserting the presence of a syndromic disease.
References
Cherkas, O.A., Kobeza, P.A., & Marchenko, D.G. (2023). Osnovni pryntsyhy dudovy ta organizatsiyi spolchnoyi tkanyny [Basic principles of the structure and organization of connective tissue]. Morfolohiya – Morphology, 1(17), 77-85 [in Ukrainian].
Pavlina, V., & Ross, M. (2021). Histolohiya: pidruchnyk i atlas. Z osnovamy klitunnoyi i molekulyarnoyi biolohiyid [Histology: textbook and atlas. With the basics of cellular and molecular biology]. Kyyiv: VSM. Meditsina [in Ukrainian].
Obernikhina, N.V., Mykhaylova, A.G., Pradiy, T.P., & Sanjur, T.S. (2020). Biokhimiya spoluchnoyі tkanyny [Biochemistry of connective tissue]. Biblioteka NMU. Retrieved from: htts://ir.librarynmu.com>bitstream: Biochemistry of connective tissue.pdf [in Ukrainian].
Rudenko, Yu.P., Zviryaka, O.M., & Prudence, A.M. (2018). Zastosuvannya zasobiv fizychnoyi terapiyi ditey iz patolohichnymy zminamy zorovoha ta oporno–rykhovoho aparativ na foni nedyferentsiovanoyi dysplaziei tkanyny [The use of physical therapy for children with pathological changes of the visual and locomotor apparatus against the background of undifferentiated connective tissue dysplasia]. Problemy zdorovya fizychnoyi reabilitatsiyi ta ergoterapiyi: materialy IV Vseukraynskoyi naukovo-praktychnoyi internet–konferentsiyi vidpovid. / Red. Ya. M. Kopytina: nauk. Red. M. O. Lyannoy. –– Health problems of physical rehabilitation and occupational therapy: materials of the IV All–Ukrainian remote scientific and practical Internet conference / response. ed. Y. M. Hoof; of science ed. M. O. Liannoy. Sumy: V–vo SuDPU іm. F.S. Makarenka [in Ukrainian].
Omelchenco, L.I., & Nikolaenko, V.D. (2004). Displaziya soyedinitelnoy tkani u detey [Connective tissue dysplasia in children]. Doctor–zhurnal dlya praktiuyushchikh vrachey – Doctor–Journal for Practicing Doctors, 1, 44-47 [in Russian].
Protsailo, M.D., Chornomydz, I.B., Horishniy, I.M., &. Vorontsova T.O. (2023). Syndrom dysplaziyi spoluchnoyi tkanyny v systemi dyspansernoho sposterezhennya simeynoho likarya [Connective tissue dysplasia syndrome in the dispensary observation system of a family doctor]. Aktualni pytannya pediatriyi, akusherstva ta hinecolohiyi – Current issues of pediatrics, obstetrics and gynecology, 1, 95-98 [in Ukrainian].
Churilina, A.V. (2003). Syndrom dysplazii spoluchnoi tkanyny u ditei [Syndrom of connective tissue dysplasia of the heart in children]. Pediatria, akusherstvo ta hinecolohia – Pediatrics, Obstetrics and Gynecology, 1, 20-22 [in Ukrainian].
Veselova, T.V. (2015). Metabolichni ta vegetatyvni rozlady u ditei pubertatnoho viku z nedyferentsiovanoiudysplaziieiu spoluchnoi tkanyny. Yikh diagnostyka ta Korektsiia [Metabolic and autonomic disorders in pubertal children with undifferentiated connective tissue dysplasia. Their diagnosis and correction]. Candidate’s thesis. Ministry of Health of Ukraine, National Medical University named after O. O. Bohomolets. Kyiv [in Ukrainian].
Marushko, Yu.V. (2012). Syndrom hipermobilnosti suhlobiv u ditey [Joint hypermobility syndrome in children]. Dytyachyy likar – Children’s doctor, 6(19), 40-44 [in Ukrainian].
Marushko, Yu.V. (2021). Dysplaziya spoluchnoyi nkanyny [Connective tissue dysplasia]. Zdorovya Ukrainy – Health of Ukraine, 3, 48-49 [in Ukrainian].
Roy, I.V., Zinchenko, V.V., Rusanova, T.V., & Bayandina, O.I. (2010). Osoblyvosti formuvannia kulshovykh suhlobiv z oznakamy dysplasizii apoluchnos tkanyny [Features of the formation of hip joints with signs of connective tissue dysplasia]. Zbirnyk naukovych prats KHU ziizdu ortopediv–travmatologiv Ukrainy–Collection of Scientific Papers of the Congress of Orthopedic Trauvatologists of Ukraine. Dnipropetrovsk [in Ukrainian].
Voloshyn, O.M., & Chumak, O.Yu. (2017). Nedyferentsiyovana dysplaziya spoluchnoyi tkanyny y respiratorni zakhvorni zakhovoryuvannya u ditey ta pidlitkiv (ohlyad literatury) [Undifferentiated connective tissue dysplasia and respiratory diseases in children and adolescents (literature review)]. Zdorovya dytyny – Child health, 6(12), 720-727 [in Ukrainian].
Veselova, T.V. (2017). Nedyferentsiyovana dysplaziya spoluchnoyi tkanyny – problemy ta shlyakny vyrishennya [Undifferentiated connective tissue dysplasia – problems and solutions]. Dytyachyy likar – Children's doctor, 3(54). 26-32 [in Ukrainian].
Sinyachenko, O.V., Kovalenko, V.M., & Bortkevich, O.P. (2020). Istoriya rozvytku systemnykh zakhvoryuvanspoluchnoyi tkanykn vaskylitiv u dzerkali numizmatyky [The history of the development of systemic connective tissue diseases and systemic vasculitis in the mirror of numismatics]. Ukr. Revmatolohichnyy zhurnal – Ukr. rheumatology journal, 3(81), 4-11 [in Ukrainian].
Umanets, M.M., Zborovska, O.V., Dorokhova, A.Ye., & Kogan, M.B. (2022). Vypadok likuvannya patsiyentky z syndromom Ellersa–Danlosa [The case of treatment of a patient with Ehlers-Danlos syndrome]. Oftalmon. zhurn – Ophthalmon. Journal, 1, 57-62. DOI: 10.31288/oftalmolzh202225762 [in Ukrainian].
Parkhomenko, L.K. (2007). Yuvenolohiya. Praktikum z pidkovoyi medycyny [Juvenology. Workshop on adolescent medicine]. Kharkiv: Fakt [in Ukrainian].
Stepanova-Kamysh, А. (2021). Dysleksiya ne mozhna poboroty, a lyshe vidkoryhuvaty. Yak [Dyslexia cannot be overcome, but only corrected. As]. Retrieved from: https://nus.org.ua>ctatti. June 23 [in Ukrainian].
Stepanova-Kamysh, А. (2021). Dysleksiya i yak dhopohty dytyni pokrashchyty uapishnist navchannya [Dyslexia: how to help a child improve academic performance]. Retrieved from: https://nus.org.ua/artikles/disleksiya-yak. November 16 [in Ukrainian].
Goncharova, S.A., Panteleev, G.V., & Tyrlovaya, E.I. (2013). Ambliopiya [Amblyopia]. Luhansk: Elton–2 [in Ukrainian].
Nemaltseva, O.M., & Korzh, Yu.M. (2019). Problemy zdorovya, fizychnoyi terapiyi, reabilitasiyi ta… [Problems of health, physical therapy, rehabilitation and...]. SumDPU. December 6. Retrieved from: htts://repository.sspu.edu.ua>bitstream>nem...[in Ukrainian].
Freh, Hefti (2007). Pediatric Orthopedics in Practice. Berlin Heidelberg: Springer–Verlad.
Golky, H.G., Buryanova, O.A., & Klymovytskoho, V.G. (2014). Travmatilohiya ta ortopediya: pidruchnyk dlya stud. vychchykh med. zakladiv [Traumatology and orthopedics: textbook for students. higher med. education institutions]. Vinnytsya: Nova knyha [in Ukrainian].
Markdante, K.D., & Kligman, R.M. (2019). Osnovy pediatriyi za Nelsonom u 2–kh tomakh [Fundamentals of pediatrics according to Nelson in 2 volumes]. Medytsyna: Pereclad 8–ho anhliyskoho vydannya [in Ukrainian].
Mavrushko, Yu.V., Kostynska, N.G., & Gishchak, T.V. (2023). Otsinka pokaznukiv yakosti zhtta v ditey shkilnogo viku, khvorykh na arterial’nu hipertenziyu ta ozhyrinnya [Assessment of quality of life indicators in school – age children with hypertension and obesity]. Suchasna peditriya. Ukraina – Modern pediatrics. Ukraine, 2 (130), 50-59 [in Ukrainian].
Serdyuk, V.V. (2010). Novyy vzhlyad na etiolohiyyu y hatohenez ydyyopatycheskoho skolyoza [New look at the etiology and pathogenesis of idiopathic scoliosis]. Zbirn. nauk. prakts XV zyizdu ortopediv–travmatologiv Ukrainy – Collection. of science Proceedings of the XV Congress of Orthopedic Traumatologists of Ukraine. Dnipropetrovsk [in Ukrainian].
Protsenko, V.N. (2010). K voprosu o roly funktsyonalnogo neravenstva dlyny nyzhnykh konechnosteyv formirovanyy deheneratyvno-dystrofycheskoy patolohyy poyasnychnoho otdela pozvonochnyka [To the question of the role of the functional inequality of the length of the lower limbs in the formation of degenerative-dystrophic pathology of the lumbar spine]. Zbirn. nauk. prakts XV zyizdu ortopediv–travmatologiv Ukrainy – Collection. of science Proceedings of the XV Congress of Orthopedic Traumatologists of Ukraine. Dnipropetrovsk [in Ukrainian].
Doroshenko, S., & Samoilik, S. (2020). Poshyrenist zuboshchelepnykh anomaliy u ditey vikom 4–17 rokiv [Prevalence of maxillofacial anomalies in children aged 4–17 years]. Suchasna stomatolohiya – Modern dentistry, 5, 70-73 [in Ukrainian].
Flis, P., Rashchenko, N., Filonenko, V., & Melnyk, A. (2018). Poshyrenist zuboshchelepnykh anomaliy ta movlenykh porushen’ sered ditey vikom 6–12 rokiv [Prevalence of maxillofacial anomalies and speech disorders among children aged 6-12 years]. Suchasna stomatolohiya – Modern dentistry, 4, 54-57 [in Ukrainian].
Vyshemirska, T.A. (2022). Klinika diahnosyka ta likuvannya sahitalnykh anomaliy prykusu u ditey z porushennyam nosovoho dykhannya [Clinic, diagnosis and treatment of sagittal anomalies of bite in children with nasal breathing disorders]. Candidate’s thesis [in Ukrainian].
Makhmenets, N.P., Ozhohan, Z.R., Pantus, A.V., & Yatsynovych, V.I. (2023) Shlakhy kompleksnoho likuvannya nabutykh zuboshchelepnykh deformatiy na foni shkidlyvykh zvychok [Ways of complex treatment of acquired maxillofacial deformities against the background of bad habits]. Visnyk Stomatolohiyi – Herald of dentistry, 2(123), 48, 29-34 [in Ukrainian].
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