Berkovitz B K, Grigson C, Dean M C
Biomedical Sciences Division, Anatomy and Human Biology Group, King's College London, United Kingdom.
Am J Med Genet. 1998 Apr 1;76(4):343-8.
Caroline Crachami (C.C.) considered to have had the Seckel syndrome was one of the most extreme cases of dwarfism ever recorded. Reputedly born in Sicily in 1815, she attracted much attention when exhibited in England before her death on 3 June 1824. Although she is said to have been 9 years old at death, published descriptions give her a dental age varying from 2 to 7 years. Examination of her skull in the Royal College of Surgeons of England demonstrated a more or less erupted complete deciduous dentition, with no erupted permanent teeth. Radiographs showed agenesis of several permanent teeth. It was concluded that the dental age of C.C. was 3 years (+/- 6 months). Perikymata were evident in the surface enamel encircling the crown of the partially exposed maxillary left first permanent molar. Their distribution and spacing were normal, with no evidence of developmental retardation. The distance between the periradicular bands in the root near the cement-enamel junction was also normal. We conclude that the dental age of C.C. was similar to her chronological age and that at death she was about 3 and not 9 years old. The reason that she was said to be nearly 9 when exhibited in England we believe was related to financial considerations, as people would be generally less impressed with a dwarf only 3 years old. The new age we give C.C. has implications on the diagnosis of her medical condition.
卡罗琳·克拉查米（C.C.）被认为患有塞克尔综合征，是有记录以来最极端的侏儒症病例之一。据说她于1815年出生在西西里岛，1824年6月3日去世前在英国展出时引起了广泛关注。尽管据说她去世时9岁，但已发表的描述显示她的牙齿年龄在2至7岁之间。对她保存在英国皇家外科医学院的头骨进行检查发现，她的乳牙基本全部萌出，恒牙未萌出。X光片显示几颗恒牙先天缺失。结论是C.C.的牙齿年龄为3岁（±6个月）。在上颌左侧部分暴露的第一恒磨牙牙冠周围的釉质表面，釉质横纹清晰可见。它们的分布和间距正常，没有发育迟缓的迹象。在靠近牙骨质-釉质界的牙根处，牙根周围带之间的距离也正常。我们得出结论，C.C.的牙齿年龄与她的实际年龄相近，她去世时约3岁而非9岁。我们认为，她在英国展出时被说成将近9岁，原因与经济因素有关，因为人们通常对一个只有3岁的侏儒印象不深。我们为C.C.确定的新年龄对她病情的诊断有影响。
