Baccetti T, Pierleoni L, Filippi L, Donati M A, Tollaro I, Zammarchi E
Department of Orthodontics, University of Florence, Italy.
J Clin Pediatr Dent. 1994 Fall;19(1):55-60.
This paper reports on previously undescribed dentofacial features a child suffering from Glycogen-Storage Disease type III with hepatomegaly and hypertransaminasemia with muscular involvement. Deficient craniofacial development, reduced width of the upper jaw resulting in posterior cross-bite, and taurodontism of the primary dentition were found. Pathogenetic basis for craniofacial abnormalities is discussed. Cooperation between pediatricians and pediatric dentists is strongly recommended for an early diagnosis and treatment of the dentofacial defects of the syndrome.
本文报道了一名患有III型糖原贮积病并伴有肝肿大、高转氨酶血症及肌肉受累的儿童，其具有此前未被描述的牙颌面特征。发现该患儿存在颅面发育不足、上颌宽度减小导致后牙反合以及乳牙列牛牙样牙畸形。文中讨论了颅面异常的发病机制。强烈建议儿科医生和儿童牙医合作，以便早期诊断和治疗该综合征的牙颌面缺陷。
