Cardiopulmonary function in fibrodysplasia ossificans progressiva.

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Cardiopulmonary function in fibrodysplasia ossificans progressiva.

Cardiopulmonary function was evaluated in 21 patients with fibrodysplasia ossificans progressiva. Neither cardiac enlargement nor failure was observed, but six patients had abnormal electrocardiograms. All had marked restrictive spirometry because of chest wall fixation and depended upon diaphragmatic respiration. The severity of chest restriction was independent of sex, age, duration of diseas...

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Fibrodysplasia ossificans progressiva.

Fibrodysplasia ossificans progressiva (FOP) is a rare genetic disorder of the connective tissue characterized by progressive disability as a result of extensive extra skeletal enchondral bone formation and malformed big toes which are often monophalangic. Occasional features include short thumbs, fifth finger clinodactyly, malformed cervical vertebrae and mild mental retardation. Beginning duri...

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Fibrodysplasia 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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Fibrodysplasia Ossificans Progressiva.

Fibrodysplasia Ossificans Progressiva (FOP) is a rare autosomal dominant disorder characterized by postnatal progressive heterotopic ossification of connective tissue and congenital malformation of big toes. We report a 3-year male toddler with clinical and radiological features of FOP. He was born with bilateral hallux valgus and at the age of 3 years presented with hard swellings over back, s...

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[Fibrodysplasia ossificans progressiva: case report].

Fibrodysplasia ossificans progressiva (FOP) is a rare genetic disease characterized by widespread soft tissue ossification and congenital stigmata of the extremities. We report the case of a 33-year-old woman with clinical and radiological features of FOP. She was born with bilateral hallux valgus and at the age of 10 presented swelling and ossification of the left scapula. During the course of...

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ژورنال

عنوان ژورنال: Thorax

سال: 1981

ISSN: 0040-6376

DOI: 10.1136/thx.36.6.419