Spondyloepimetaphyseal dysplasia, Shohat type

Orpha code: 93352OMIM code: 602557

Definition

Spondyloepimetaphyseal dysplasia congenita, Shohat type is characterized by severely disproportionate short stature, short limbs, small chest, short neck, thin lips, severe lumbar lordosis, marked genu varum, joint laxity, distended abdomen, mild hepatomegaly and splenomegaly.

Disease data
Classification

Disease

Synonyms
SEMD, Shohat type
SEMD, typ Shohat
ORPHA code
93352
OMIM code
602557
ICD10 code
Q77.7
ICD11 code
-

No additional description.

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