Spondylometaphyseal dysplasia, Golden type

Orpha code: 168544OMIM code: 313420

Definition

Spondylometaphyseal dysplasia, Golden type is a rare primary bone dysplasia disorder characterized by severe short stature, coarse facies, thoracolumbar kyphoscoliosis and enlarged joints with contractures. Psychomotor delay and intellectual disability may also be associated. Radiographic features include flat vertebral bodies, lacy ossification of the metaphyses of long bones and iliac crests, and marked sclerosis of the skull base.

Disease data
Classification

Disease

Synonyms
X-linked spondylometaphyseal dysplasia
Dysplazja kręgowo-przynasadowa sprzężona z chromosomem X
ORPHA code
168544
OMIM code
313420
ICD10 code
Q77.8
ICD11 code
LD24.4

No additional description.

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