Systemic multiple fibrous dysplasia of bone?

summary

Multifibrotic dysplasia is a disease of bone growth and development, which is characterized by bone damage, precocious puberty and skin pigmentation. A few patients also had other endocrine dysfunction. Systemic multiple fibrous dysplasia of bone? Next, I'd like to share my views with you.

Systemic multiple fibrous dysplasia of bone?

The etiology of multifibrotic dysplasia has not been fully elucidated. But in recent years, there has been a breakthrough. The excitability of G protein is widespread in patients with idiopathic epilepsy α GS gene mutation, which leads to the proliferation of proosteoblasts and fibrous dysplasia of bone. GS mutations were mostly located in the raolc or raolh region.

Bone lesions were mainly focal lesions. The focal lesion was composed of fibrous connective tissue and scattered immature intertwined bone and cartilage tissue nodules. Bone damage is usually caused by the expansion of the bone marrow cavity to the bone cortex, resulting in the thinning of the bone cortex. Some of the local lesions of multiple fibrous dysplasia may have liquefaction, cystic degeneration, hemorrhage and ossification in cartilage nodules, which may form focal deformity. The involvement of bone bearing parts may lead to claudication or even pathological fracture.

According to the nature of the lesions, they can be divided into single type and multiple type. Femur, tibia and rib are the most common cases, while spine and pelvis are relatively rare. 30% of them involved craniofacial bone, but mainly involved maxilla, mandible and skull top. Hyperosteosclerosis of skull base forces cranial nerve under normal pressure, leading to atrophy of optic nerve. Excessive hyperplasia of the facial bone makes the face asymmetric and the nasal sinuses occluded. Long bone damage of spine, pelvis and limbs leads to bone deformity, pathological fracture and bone pain. Multiple cases mainly involved bilateral or one side of the body, lower limbs, femur, tibia and pelvis are more common, less involved parts are ribs and skull invasion, can involve the skull base.

matters needing attention

Usually, you should take your uncle out to bask in the sun, so as to promote calcium absorption. You should eat more high protein foods, such as milk and shrimp, and do more physical exercise to prevent fracture caused by illness again.