Acromesomelic dysplasia, Grebe type

Orpha code: 2098OMIM code: 200700

Definition

A rare autosomal recessive acromesomelic dysplasia characterized by severe dwarfism at birth, abnormalities confined to limbs, severe shortening and deformity of long bones, fusion or absence of carpal and tarsal bones, ball shaped fingers and, occasionally, polydactyly and absent joints. As seen in acromesomelic dysplasia, Hunter-Thomson type and acromesomelic dysplasia, Maroteaux Type, facial features and intelligence are normal.

Disease data
Classification

Malformation syndrome

Synonyms
Chondrodysplasia, Grebe type
Chondrodysplazja, typu Grebe
ORPHA code
2098
OMIM code
200700
ICD10 code
Q78.8
ICD11 code
LD24.9

No additional description.

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