Fibrodysplasia ossificans progressiva : Report of two cases

Fibrodysplasia Ossificans Progressiva (FOP) is a rare hereditary connective tissue disease, genetically inherited as an autosomal dominant trait with complete penetrance but variable expressivity. Onset is typically in childhood and progressive involvement of the spine and proximal extremities leads...

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Bibliographic Details
Published in:Clinical and experimental rheumatology 2000-11, Vol.18 (6), p.749-752
Main Authors: FONSECA, J. E, BRANCO, J. C, REIS, J, EVANGELISTA, T, TAVARES, V, RODRIGUES GOMES, A, VIANA QUEIROZ, M
Format: Article
Language:English
Subjects:
Adolescent
Biological and medical sciences
Diseases of striated muscles. Neuromuscular diseases
Hip Joint - diagnostic imaging
Humans
Male
Medical sciences
Myositis Ossificans - diagnostic imaging
Myositis Ossificans - etiology
Myositis Ossificans - physiopathology
Myositis Ossificans - therapy
Neurology
Radiography
Scoliosis - complications
Scoliosis - diagnostic imaging
Spine - diagnostic imaging
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Summary:Fibrodysplasia Ossificans Progressiva (FOP) is a rare hereditary connective tissue disease, genetically inherited as an autosomal dominant trait with complete penetrance but variable expressivity. Onset is typically in childhood and progressive involvement of the spine and proximal extremities leads to immobility and articular dysfunction. We present two cases with the typical features of FOP and a review of the pathogenesis, clinical manifestations and treatment options of this rare disease.
ISSN:0392-856X
1593-098X