What disease is infantile osteogenesis incomplete?

summary

Many parents don't know much about the disease of osteogenesis imperfecta in children, so when they know that their children suffer from this disease, they are often confused and don't know what kind of situation it is. Experts pointed out: children with osteogenesis imperfecta, strictly speaking, belongs to a kind of reproductive development malformation. Children with serious illness may lead to lifelong disability or even death.

What disease is infantile osteogenesis incomplete?

The etiology of this disease is unknown, and most of them have family genetic history. It is a congenital genetic disease. According to gene mutation, it can be divided into 4-11 types, but it is controversial. The main pathological changes were insufficient collagen fibers, abnormal structure and systemic connective tissue diseases. The lesions are not only limited to bones, but also involve other connective tissues such as eyes, ears, skin and teeth.

The X-ray findings of different patients with osteogenesis imperfecta are quite different, depending on the type of osteogenesis imperfecta. Usually, the long bone shaft is slender, which makes the bone end of the joint relatively thick, bone trabeculae are rare, the shaft is curved, the cortex on the convex side is thin, the concave side is relatively thick, the medullary cavity is relatively large, and there may be cystic changes. There are many old or fresh fracture lines.

There is no special treatment for this disease. It is mainly to prevent fractures, to strictly protect children, until the fracture trend is reduced, but also to prevent the complications of long-term bedridden. The treatment of fracture is the same as that of normal people. Due to the rapid healing of fracture, the fixation period can be short. If the deformity is serious, measures can be taken to correct the deformity and improve the load line. Drug therapy includes bisphosphonates, estrogen, calcitonin and vitamin D3, but the curative effect is uncertain. Stem cell therapy and gene therapy need to be further studied and identified, and can not be used in clinic in a short time.

matters needing attention

For the introduction of osteogenesis imperfecta in children, Xiao Bian will first describe it here. Although the incidence rate of osteogenesis incomplete is not very high in clinic, the harm caused by this disease is great. Therefore, parents need to take corresponding preventive measures for children's osteogenesis imperfecta for their children's healthy growth.