X-linked spondyloepimetaphyseal dysplasia

Orpha code: 93349OMIM code: 300106

Definicja

A rare, genetic primary bone dysplasia disorder characterized by disproportionate short stature with mesomelic short limbs, leg bowing, lumbar lordosis, brachydactyly, joint laxity and a waddling gait. Radiographs show platyspondyly with central protrusion of anterior vertebral bodies, kyphotic angulation and very short long bones with dysplastic epiphyses and flarred, irregular, cupped metaphyses.

Disease data
Klasyfikacja

Disease

Kod ORPHA
93349
Kod OMIM
300106
Kod ICD10
Q77.7
Kod ICD11
-

No additional description.

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