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| 特发性青少年骨质疏松症 |
| The idiopathic juvenile osteoporosis |
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| DOI：10.3969/j.issn.1006.7108.2015.03.019 |
| 中文关键词: 骨质疏松症 特发性青少年骨质疏松症 成骨不全 |
| 英文关键词:Osteoporosis Idiopathic juvenile osteoporosis Osteogenesis imperfecta |
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| 中文摘要: |
| 特发性青少年骨质疏松症(idiopathic juvenile osteoporosis，IJO)是一种罕见的发生于先前身体健康的儿童身上的一种病因不明的主要表现为骨质疏松症的疾病。该病以反复发生的骨折为特征，包括脊椎压缩性骨折。它通常是一种自限性疾病，在青春期之后自然缓解。但在某些情况下可导致严重的畸形和功能障碍。IJO的病因和发病机制可能有下列几方面的原因：1.骨形成和骨吸收平衡被打破;2.青春期生长突增和骨量增加;3.骨代谢调节因素失常;4.胶原合成异常。IJO常急性起病，通常病程2-4年，在发病期间会出现生长停止和多发骨折，随后疾病自然缓解。中轴骨和四肢骨均可受累。严重的病例轻微外伤后即可出现脊椎骨和长骨的多发骨折，尤其是干骺端易发生骨折，常常导致背痛、足及髋部疼痛、四肢骨痛、肌力下降、自由运动受限、畸形和行走困难导致的独特步态。IJO的诊断需具备其自身的阳性特征并且同时排除其它任何可能引起骨质疏松症的疾病。IJO的诊断应基于其X线的特征，主要包括多发性的脊椎压缩性骨折和长骨骨折，尤其是在承重关节附近。IJO 的治疗十分复杂。最基本的治疗策略是保护脊柱、避免骨折和畸形发生直至疾病自然缓解。对于预料之中可以自发缓解的青春期发病的IJO患者应立即采取支持性疗法。 |
| 英文摘要: |
| Idiopathic juvenile osteoporosis (IJO) is a rare primary form of osteoporosis of unknown etiology that develops in a previously healthy child. IJO is characterized by repeated fractures，including vertebral compression fractures. It is usually a self- limiting disease that spontaneously resolves after puberty，but in some cases it may result in severe deformities and functional impairment. There are probably the following reasons about etiological factors and pathogenesis in IJO ：1) Imbalance between bone formation and bone resorption; 2) Adolescent growth burst and bone mass increase; 3) Abnormal metabolic regulation factors of bone; 4) Abnormal collagen synthesis. IJO runs an acute course，usually over a period of 2 to 4 years，during which time there is growth arrest and multiple fractures，and then the disease remits. Both the axial and the appendicular skeleton are affected. The severe cases may have fractures of the vertebrae and the long bones，particularly in the metaphyses，that lead to pain in the back， foot，hip and extremities，muscle weakness，limitation of free movement，deformity，and unique gait because of difficulty in walking. The diagnosis of IJO is based both on the exclusion of other diseases and on its own positive features. The diagnosis of IJO is best made from its X-ray features，which include multiple vertebral compression fractures and fractures of the long bones，especially around the weight-bearing joints. Treatment of IJO is extremely complicated. The basic strategy of treatment is to protect the spine and to avoid the occurring of fractures and deformity until remission occurs. Supportive care is instituted promptly in anticipation of spontaneous recovery with the onset of puberty. |
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