成骨不全(OT)是结缔组织代谢性遗传疾病,特征为骨脆性,患者均有增加骨折趋势,有些患者体重减轻,蓝色巩膜,牙质形成差,早期失聪和/或马凡样(Marf-an like)骨折。研究包括:(1)测定出生时点发生率,包括有永久性住址母亲所生下从1970年1月1日至1983年12月31日所有OT新生儿(活产和妊娠第28周后的死产);(2)所有在1983年12月31日活着有永久住址OT的人群发生率。从1973年4月1日至1983年12月31日中央计算机登记确定的患者,1973年4 Osteogenesis imperfecta (OT) is a metabolic genetic disease of the connective tissue characterized by bone fragility, with an increased fracture tendency in some patients, weight loss in some patients, blue sclera, poor dentition, early deafness and / or Marfan -an like) fracture. The study included: (1) Determination of the incidence at birth, including all OT newborns born alive from January 1, 1970 to December 31, 1983 (live births and weeks 28 after gestation Stillbirth); (2) The prevalence of all those with a permanent address OT as of December 31, 1983. From April 1, 1973 to December 31, 1983 the central computer registration identified patients, 1973 4