Hip dysplasia, Beukes type

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Synonyms: BFHD | Beukes familial hip dysplasia | Cilliers-Beighton syndrome | Premature degenerative osteoarthropathy of the hip

A primary bone dysplasia characterized by premature degenerative arthropathy of the hip. The disease presents with hip joint discomfort/pain and gait disturbances that usually develop in childhood and that progress to severe functional disability and limited mobility by early adulthood. Involvement of the vertebral bodies and other joints is minimal height is not significantly reduced and general health is unimpaired. Radiographically the femoral heads are flattened and irregular and degenerative osteoarthritis develops in the hip joints as evidenced by the presence of periarticular cysts sclerosis and joint space narrowing.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview. Reference: Access aggregated data from Orphanet at Orphadata. Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version June 2024

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