dyschondrosteosis


Also found in: Acronyms.

dys·chon·dros·te·o·sis

(dis'kon-dros'tē-ō'sis), [MIM*127300]
A skeletal dysplasia, more severe in females and with a female preponderance, characterized by bowing of radius, dorsal dislocation of the distal ulna with limited movement of the elbow and wrist (wrist deformity is called Madelung deformity), and mesomelic dwarfism; dominant inheritance, caused by mutation in the short stature homeobox gene (SHOX) on the pseudoautosomal region of Xp. Langer mesomelic dysplasia, the homozygous form of dyschondrosteosis, is also caused by homozygous mutations in the SHOX gene.
[dys- + G. chondros, cartilage, + osteon, bone, + -osis, condition]

Leri,

André, French orthopedic surgeon, 1875-1930.
Leri pleonosteosis - Synonym(s): dyschondrosteosis
Leri sign - voluntary flexion of the elbow is impossible in a case of hemiplegia when the wrist on that side is passively flexed.
Leri-Weill disease - Synonym(s): dyschondrosteosis
Leri-Weill syndrome - Synonym(s): dyschondrosteosis
Marie-Leri syndrome - see under Marie, Pierre

Weill,

Jean A., French physician, 1903–.
Leri-Weill disease - Synonym(s): dyschondrosteosis
Leri-Weill syndrome - Synonym(s): dyschondrosteosis
References in periodicals archive ?
The prominent Madelung deformity (shortening and bowing of the radii and triangulation of the distal radial epiphysis producing an ulnar slant to the articular surface) of the distal radius and ulna is a well-described feature of dyschondrosteosis (Leri-Weill disease).
Madelung deformity is diagnostic of Leri-Weill dyschondrosteosis.