Osteogenesis imperfecta: pathophysiology and treatment.
Wien Med Wochenschr
; 165(13-14): 278-84, 2015 Jul.
Article
en En
| MEDLINE
| ID: mdl-26055811
ABSTRACT
Osteogenesis imperfecta is a rare hereditary disease mostly caused by mutations impairing collagen synthesis and modification. Recently recessive forms have been described influencing differentiation and activity of osteoblasts and osteoclasts. Most prominent signs are fractures due to low traumata and deformities of long bones and vertebrae. Additional patients can be affected by dwarfism, scoliosis Dentinogenesis imperfecta, deafness and a blueish discoloration of the sclera. During childhood state of the art medical treatment are i.v. bisphosphonates to increase bone mass and to reduce fracture rate. Surgical interventions are needed to treat fractures, to correct deformities and should always be accompanied by physiotherapeutic and rehabilitative interventions.
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Osteogénesis Imperfecta
Límite:
Female
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Humans
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Infant
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Male
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Newborn
Idioma:
En
Revista:
Wien Med Wochenschr
Asunto de la revista:
MEDICINA
Año:
2015
Tipo del documento:
Article
Pais de publicación:
AT
/
AUSTRIA
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ÁUSTRIA