Radiologic findings in seven patients with fibrodysplasia ossificans progressiva.
نویسندگان
چکیده
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 pediatrics wards of our centers between 1983 and 2002, and present the radiologic findings.
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 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...
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متن کاملFibrodysplasia ossificans progressiva.
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.
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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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ورودعنوان ژورنال:
- Archives of Iranian medicine
دوره 10 1 شماره
صفحات -
تاریخ انتشار 2007