V. N. Kotov, N. S. Grachev, A. Lopatin, A. Kugushev, A.A. Korneev
{"title":"Cherubism.书目回顾","authors":"V. N. Kotov, N. S. Grachev, A. Lopatin, A. Kugushev, A.A. Korneev","doi":"10.24110/0031-403x-2023-102-6-81-88","DOIUrl":null,"url":null,"abstract":"This bibliographical review represents current data on a rare hereditary disease, cherubism in children and adolescents, methods of its diagnosis and staging. The causes for and possible mechanisms of development of this condition: SH3BP2 gene mutations, the role of the RANKL-OPG system and the TNF-alpha in the pathogenesis of cherubism, are considered. Clinical manifestations of the disease are described as well. Various treatment methods (medical and surgical) are presented and analyzed. Special attention is paid to the Denosumab drug as to the most effective one in the treatment of cherubism.","PeriodicalId":503254,"journal":{"name":"Pediatria. Journal named after G.N. Speransky","volume":"226 1","pages":""},"PeriodicalIF":0.0000,"publicationDate":"2023-12-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Cherubism. A bibliographical review\",\"authors\":\"V. N. Kotov, N. S. Grachev, A. Lopatin, A. Kugushev, A.A. Korneev\",\"doi\":\"10.24110/0031-403x-2023-102-6-81-88\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"This bibliographical review represents current data on a rare hereditary disease, cherubism in children and adolescents, methods of its diagnosis and staging. The causes for and possible mechanisms of development of this condition: SH3BP2 gene mutations, the role of the RANKL-OPG system and the TNF-alpha in the pathogenesis of cherubism, are considered. Clinical manifestations of the disease are described as well. Various treatment methods (medical and surgical) are presented and analyzed. Special attention is paid to the Denosumab drug as to the most effective one in the treatment of cherubism.\",\"PeriodicalId\":503254,\"journal\":{\"name\":\"Pediatria. Journal named after G.N. Speransky\",\"volume\":\"226 1\",\"pages\":\"\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2023-12-15\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Pediatria. Journal named after G.N. Speransky\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.24110/0031-403x-2023-102-6-81-88\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Pediatria. Journal named after G.N. Speransky","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.24110/0031-403x-2023-102-6-81-88","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
This bibliographical review represents current data on a rare hereditary disease, cherubism in children and adolescents, methods of its diagnosis and staging. The causes for and possible mechanisms of development of this condition: SH3BP2 gene mutations, the role of the RANKL-OPG system and the TNF-alpha in the pathogenesis of cherubism, are considered. Clinical manifestations of the disease are described as well. Various treatment methods (medical and surgical) are presented and analyzed. Special attention is paid to the Denosumab drug as to the most effective one in the treatment of cherubism.
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