Osteopetrosis

Osteopetrosis Overview:
Osteopetrosis, also called marble bone, primary brittle bone sclerosis, is a rare dysostosis diseases. Osteopetrosis Was first found by Albers-Schonberg (1904), also known as Albers-schonberg disease. The disease is characterized by the persistence of calcified cartilage, bone sclerosis cause widespread, heavy bearing on the overall situation of the medullary cavity were closed, resulting in severe anemia. The disease often familial, the vast majority of cases of recessive inheritance. According to clinical manifestations are divided into malignant (child type) and benign (adult type), the former often stillborn or died after birth, anemia, and poor prognosis.

Osteopetrosis Causes:
Causes of Osteopetrosis is related to genetic factors. Because the normal osteoclast function is lack or defects, the main change over osteoid tissue calcification and ossification of the lack of real, so that calcification of the cartilage matrix and the original slow bone reabsorption, resulting in a lack of bone in bone lamellar and osteogenic cells, the loss of this flexibility, bone dysplasia, so easy to cut brittle bone. A large number of calcified cartilage as a result of the existence of matrix so that the bone marrow cavity significantly reduced, or even occlusion, cortical and cancellous bone sclerosis, can not distinguish between the two. Cortical hyperplasia, dense cancellous bone and systemic characteristics of bone X-ray changes.