Cardiovascular Abnormalities in Osteogenesis Imperfecta

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Cardiovascular Abnormalities in Osteogenesis Imperfecta.

THE GROUP of diseases characterized by McKusick as generalized "heritable disorders of connective tissue" includes the Marfan syndrome, the Ehlers-Danlos syndrome, osteogenesis imperfecta, the Hurler syndrome, and pseudoxanthoma elasticum. With the exception of osteogenesis imperfecta, all of these have been known to involve significant cardiovascular abnormalities.' The Marfan syndrome is freq...

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Muscle abnormalities in osteogenesis imperfecta

Osteogenesis imperfecta (OI) is mainly characterized by bone fragility but muscle abnormalities have been reported both in OI mouse models and in children with OI. Muscle mass is decreased in OI, even when short stature is taken into account. Dynamic muscle tests aiming at maximal eccentric force production reveal functional deficits that can not be explained by low muscle mass alone. However, ...

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Cardiovascular Abnormalities in Osteogenesis

THE GROUP of diseases characterized by McKusick as generalized "heritable disorders of connective tissue" includes the Marfan syndrome, the Ehlers-Danlos syndrome, osteogenesis imperfecta, the Hurler syndrome, and pseudoxanthoma elasticum. With the exception of osteogenesis imperfecta, all of these have been known to involve significant cardiovascular abnormalities.' The Marfan syndrome is freq...

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Cardiovascular Involvement in Children with Osteogenesis Imperfecta

OBJECTIVE Osteogenesis imperfecta is a hereditary disease resulting from mutation in type I procollagen genes. One of the extra skeletal manifestations of this disease is cardiac involvement. The prevalence of cardiac involvement is still unknown in the children with osteogenesis imperfecta. The present study aimed to investigate the prevalence of cardiovascular abnormalities in these patients....

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Abnormalities in the cerebral arterial system in osteogenesis imperfecta.

A 9-year-old girl with osteogenesis imperfecta (OI) type 4 was admitted to the hospital because of choreatic movement. Cerebral angiography demonstrated bilateral symmetrical stenosis of the proximal cavernous segment of internal carotid artery, prethrombotic occlusion of the M1 segment of left middle cerebral artery, and moyamoya-like collateral vascular structure arising from the right lentic...

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ژورنال

عنوان ژورنال: Circulation

سال: 1965

ISSN: 0009-7322,1524-4539

DOI: 10.1161/01.cir.31.2.255