Moscow, Moscow, Russian Federation
Kazan, Kazan, Russian Federation
Kazan, Kazan, Russian Federation
Kazan, Kazan, Russian Federation
Relevance. Patients with hereditary ordental pathology of hereditary origin (genomic, chromosomal, gene mutations), as well as with multifactorial symptoms, constitute, albeit not a large, but stable group in the composition of human populations. Today, the issues of the diversity of the symptom complex of hereditary pathology are being actively discussed, while clinical observations of such patients expand the theoretical and practical knowledge of doctors of various specialties. Cruson's syndrome is classified as a hereditary monogenic disease (the carriage of a gene occurs without a change in the trait in all subsequent generations), it is distinguished by very bright orodental symptoms and requires an integrated approach to treatment, with the participation of both general practitioners and dentists. The aim of the study was to determine the main ordental symptoms of Cruson's syndrome in children and the peculiarities of supervision at the stages of dental care. Materials and methods: a comprehensive examination and treatment of a patient with a diagnosis of Cruson's syndrome was carried out; all clinical events were carried out on the basis of GAUZ DSP No. 5; GAUZ DRKB MH RT (Kazan). Results: a clinical case of a patient with Cruson's syndrome is presented, demonstrating the results of supervision at the stages of dental care. Taking into account the identified characteristics of the patient, it is important to correctly formulate medical-genetic and dental diagnoses, from which the duration and effectiveness of the chosen treatment tactics follow. A specific feature of the patient that affects the success of treatment is the presence of a psychologically motivated readiness for a large volume of dental reconstructive work, as well as the absence of phobias in front of the actions of a dentist. Conclusions: the treatment of patients with Cruson syndrome requires an integrated approach, depends on the correct diagnosis, the planning of complex treatment, its implementation and the motivation of the patient and his parents.
Cruson's syndrome, hereditary diseases, pediatric dentistry, medical genetics, anesthetic aid
1. Volkov E.M., Mamaeva E.V., Cinekker D.A., Valiev R.I., Ahmetova G.M., Salihova L.I. Stomatologicheskie aspekty medicinskoy genetiki. Kazan'. 2021:160. [E.M. Volkov, E.V. Mamaeva, D.A. Tsinekker, R.I. Valiev, G.M. Akhmetova, L.I. Salikhova. Dental aspects of medical genetics. Kazan. 2021:160. (In Russ.)]. https://elibrary.ru/item.asp?id=47290139
2. Zryachkina N.I. i dr. Sindrom Kruzona: klinicheskiy sluchay. Prakticheskaya farmakologiya. 2019;1(16):36-43. [N.I. Zryachkina et al. Crouzon syndrome: a clinical case. Practical pharmacology. 2019;1(16):36-43. (In Russ.)]. DOI:10.15690/pf.v16i1.2002
3. Topol'nickiy O.Z., Vasil'ev A.Yu. Atlas po detskoĭ hirurgicheskoĭ stomatologii chelyustno-licevoy hirurgii. Moskva : GEOTAR-Media. 2011:264. [O.Z. Topolnitsky, A.Yu. Vasiliev. Atlas of pediatric surgical dentistry of maxillofacial surgery. Moscow : GEOTAR-Media. 2011:264. (In Russ.)]. http://www.stomatkniga.ru/detskay/128-topolnickiy/
4. Lopatin A.V. i dr. Kraniometafizarnaya displaziya: klinicheskie proyavleniya, trudnosti differencial'noy diagnostiki i varianty lecheniya. Head and neck. Golova i sheya. Zhurnal obschestvennoy obscherossiyskoy obschestvennoy organizacii specialistov po lecheniyu zabolevaniy golovy i shei. 2020;2(8):22-32. [A.V. Lopatin et al. Craniometaphyseal dysplasia: clinical manifestations, difficulties of differential diagnosis and treatment options. and co. Head and neck. Head and neck. Journal of the public All-Russian public organization specialist in the treatment of head and neck diseases. 2020;2(8):22-32. (In Russ.)]. DOI: 10.25792/HN.2020.8.2.22-32
5. Kaushik A., Bhatia H., Sharma N. Crouzon’s syndrome: a rare genetic disorder // Int J Clin Pediatr Dent. – 2016;9(4):384-387. doi: 10.5005/jp-journals-10005-1395.
6. Koltunov D.E. Sindrom Kruzona: etiologiya i klinicheskie proyavleniya. Voprosy prakticheskoĭ pediatrii. 2011;5(6):49-52. [D.E. Koltunov. Kruzon syndrome: etiology and clinical manifestations. Questions of practical pediatrics. 2011;5(6):49-52. (In Russ.)]. https://elibrary.ru/item.asp?id=17010541
7. Kevin Flaherty, Nandini Singh, Joan T. Richtsmeier. Understanding craniosynostosis as a growth disorder // WIREs Developmental Biology. – 2016;5(4):429-459. doi: 10.1002/wdev.227.
8. Nikiforov A.S., Gusev E.I. Obschaya nevrologiya. Moskva : GEOTAR-Media. 2015:704. [A.S. Nikiforov, E.I. Gusev. General neurology. Moscow : GEOTAR-Media. 2015:704. (In Russ.)]. https://www.rosmedlib.ru/book/ISBN9785970433850.html
9. Cunningham M.L., Seto M.L., Ratisoontorn C., Heike C.L, Hing A.V. Syndromic craniosynostosis: from history to hydrogen bonds // Orthodontics & Craniofacial Research. – 2007;2(10):67-81. doi: 10.1111/j.1601-6343.2007.00389.x.
10. Rollnick B.R. Germinal mosaicism in Crouzon syndrome // Clin Genet. – 2008;33(3):145-150. DOI:10.1111/j.1399-0004.1988.tb03429.x
11. Kumar G.R., Jyothsna M., Ahmed S.B., Lakshmi K.S. Crouzon’s syndrome: a case report // Int J Clin Pediatr Dent. – 2013;6(1):33-37. DOI:10.5005/jp-journals-10005-1183
12. Koltunov D.E., Bel'chenko V.A. Harakteristika skeletnyh deformaciĭ u pacientov s sindromami Aperta, Kruzona, Pfaĭffera. Voprosy sovremennoĭ pediatrii. 2012;6(7):57-62. [D.E. Koltunov, V.A. Belchenko. Characteristics of skeletal deformities in patients with Apert, Kruzon, Pfeiffer syndromes. Issues of modern pediatrics. 2012;6(7):57-62. (In Russ.)]. https://elibrary.ru/item.asp?id=18758310
13. Conrady C.D., Patel B.C. Crouzon Syndrome. Stat Pearls. Treasure Island (FL) : StatPearls Publishing. 2018. PMID: 30085540
14. Nazarova O.F., Kvitkevich A.A. Opyt primeneniya obschego obezbolivaniya pri okazanii stomatologicheskoy pomoschi. Problemy stomatologii. 2013;6:46-50. [O.F. Nazarova, A.A. Kvitkevich. Experience in the use of general anesthesia in the provision of dental care. Actual problems in dentistry. 2013;6:46-50. (In Russ.)]. https://elibrary.ru/item.asp?id=21009704
