Managutti Sunita, Managutti Anil, Pragasm Michael
Department of Oral and Maxillofacial Pathology, Modern Dental College and Research Centre, Indore, 453112 Madhya Pradesh India.
J Maxillofac Oral Surg. 2012 Mar;11(1):109-14. doi: 10.1007/s12663-010-0115-7. Epub 2011 Mar 25.
Osteopetrosis (OP) is a rare congenital disorder (present at birth) in which the bones become over dense. This results from an imbalance between the formation of bone and the resorption of the bone. Thickening of the bones which become abnormally dense due to an inherited defect in bone resorption, the process in which old bone is broken down and removed so that new bone can be added to the skeleton. Osteoclasts are the cells responsible for bone resorption. In osteopetrosis the osteoclasts do not perform normally. This flaw in bone resorption results in bones that are abnormally dense, yet are fragile and easily broken. Osteopetrosis is also known as Albers-Schonberg disease, generalized congenital osteosclerosis, ivory bones, marble bones, osteosclerosis fragilis generalisata. In this article, we have described about the diagnosis and medical and surgical management of osteopetrosis reported case to our hospital.
骨质石化症（OP）是一种罕见的先天性疾病（出生时即存在），其中骨骼变得过度致密。这是由于骨形成与骨吸收之间的不平衡所致。由于骨吸收的遗传缺陷，骨骼会增厚并变得异常致密，骨吸收是旧骨被分解和去除以便新骨可以添加到骨骼中的过程。破骨细胞是负责骨吸收的细胞。在骨质石化症中，破骨细胞功能不正常。这种骨吸收缺陷导致骨骼异常致密，但却脆弱且容易骨折。骨质石化症也被称为阿尔伯斯-尚伯格病、全身性先天性骨硬化症、象牙骨、大理石骨、全身性脆弱性骨硬化症。在本文中，我们描述了我院报告病例的骨质石化症的诊断以及药物和手术治疗。
