Fibrodysplasia ossificans progressiva.
نویسندگان
چکیده
Fibrodysplasia (myositis) ossificans progressiva (FOP) is a rare autosomal dominant disorder in which there is a progressive ectopic ossification and skeletal malformation, mainly in the connective tissue of muscle. The diagnosis is based on the clinical findings and radiological demonstration of the skeletal malformations. We report a 5 year-old female case with FOP.
منابع مشابه
Fibrodysplasia ossificans progressiva - radiological findings: a case report.
UNLABELLED Fibrodysplasia ossificans progressiva formerly known as Myositis ossificans progressiva is a rare hereditary mesodermal disorder. It is characterized by congenital skeletal anomalies and progressive ectopic bone formation in connective tissue, resulting in mature ossification within soft tissues and bridging between osseous structures. It is extremely rare and has an incidence of one...
متن کاملRadiologic findings in seven patients with fibrodysplasia ossificans progressiva.
Fibrodysplasia ossificans progressiva is a rare and disabling syndrome, which is characterized by heterotopic ossifications and skeletal deformities. So far, around 200 patients with fibrodysplasia ossificans progressiva have been reported in the world literature. Herein, we analyze the clinical records of 7 known cases of fibrodysplasia ossificans progressiva from Iran who were admitted to the...
متن کاملInfluenza-like viral illnesses and flare-ups of fibrodysplasia ossificans progressiva.
Flare-ups of fibrodysplasia ossificans progressiva are most commonly triggered by soft tissue trauma. After observing severe flare-ups of fibrodysplasia ossificans progressiva in two half-sisters with culture-confirmed influenza B infections, we hypothesized that influenza-like viral illnesses also can trigger fibrodysplasia ossificans progressiva flare-ups. To address this hypothesis, we desig...
متن کاملEarly mortality and cardiorespiratory failure in patients with fibrodysplasia ossificans progressiva.
BACKGROUND Fibrodysplasia ossificans progressiva, a rare genetic disorder of progressive extraskeletal ossification, is the most disabling form of heterotopic ossification in humans. However, little is known about the lifespan or causes of mortality in these patients. We undertook this study to determine the lifespan and causes of mortality in individuals who had fibrodysplasia ossificans progr...
متن کاملA three generation family with fibrodysplasia ossificans progressiva.
A family with five persons affected with fibrodysplasia ossificans progressiva (myositis ossificans progressiva) in three generations is described. This is the first well documented three generation family with this condition and provides further evidence for autosomal dominant inheritance. A wide range of phenotypic severity is apparent, from disabling ectopic bone formation and premature deat...
متن کاملOverexpression of an osteogenic morphogen in fibrodysplasia ossificans progressiva.
BACKGROUND Fibrodysplasia ossificans progressiva is a heritable disorder of connective tissue characterized by congenital malformation of the great toes and postnatal formation of ectopic bone. Although the disorder was first described more than 300 years ago, the genetic defect and pathophysiology remain unknown. Bone morphogenetic proteins are potent bone-inducing morphogens that participate ...
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عنوان ژورنال:
- Arquivos de neuro-psiquiatria
دوره 52 1 شماره
صفحات -
تاریخ انتشار 1994