Ko Su-Jin, Seo Ji Young, Kwon Yong-Dae, Cheon Kyounga, Park Jae Hyun
J Clin Pediatr Dent. 2017;41(5):392-397. doi: 10.17796/1053-4628-41.5.392.
Silver-Russell syndrome (SRS) is a very rare genetic disorder characterized by intrauterine growth retardation, short stature, and typical craniofacial abnormalities including micrognathia. While growth hormone (GH) therapy in children with SRS significantly improves somatic growth, functional orthopedic treatment can also be effective in adolescents with mandibular deficiency. We report the effects of Phase 1 functional orthopedic treatment of a twin-block appliance in conjunction with GH administration in a 9-year-old boy with GH deficiency and SRS, and the result of the subsequent Phase 2 orthodontic treatment.
Silver-Russell综合征(SRS)是一种非常罕见的遗传性疾病,其特征为宫内生长迟缓、身材矮小以及包括小颌畸形在内的典型颅面部异常。虽然生长激素(GH)治疗可显著改善SRS患儿的躯体生长,但功能性正畸治疗对下颌骨发育不全的青少年也可能有效。我们报告了一名9岁患有生长激素缺乏症和SRS的男孩在接受第一阶段双阻板矫治器功能性正畸治疗联合生长激素给药的效果,以及随后第二阶段正畸治疗的结果。