Hip dysplasia, Beukes type

Also known as: BFHD, Beukes familial hip dysplasia, Cilliers-Beighton syndrome, Premature degenerative osteoarthropathy of the hip

Definition

Orphanet

Beukes familial hip dysplasia (BFHD) is 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.

Go To Source: Orphanet

Symptoms

Medical Term Other Names Description
Arthropathy
Hip dysplasia Congenital hip dysplasia The presence of developmental dysplasia of the hip.
Osteoarthritis Degenerative joint disease Degeneration (wear and tear) of articular cartilage, i.e., of the joint surface. Joint degeneration may be accompanied by osteophytes (bone overgrowth), narrowing of the joint space, regions of sclerosis at the joint surface, or joint deformity.
Gait disturbance Abnormal gait, Abnormal walk, Impaired gait, Gait difficulties [more] The term gait disturbance can refer to any disruption of the ability to walk. In general, this can refer to neurological diseases but also fractures or other sources of pain that is triggered upon walking. However, in the current context gait disturbance refers to difficulty walking on the basis of a neurological or muscular disease.

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