Spondyloepimetaphyseal dysplasia, matrilin-3 type

Orpha code: 156728OMIM code: 608728

Definition

A rare primary bone dysplasia due to matrilin-3 varaints and characterized by disproportionate early-onset dwarfism, bowing of the lower limbs, short, wide and stocky long bones with severe epiphyseal and metaphyseal changes, lumbar lordosis, hypoplastic iliac bones, flat ovoid vertebral bodies and normal hands.

Disease data
Classification

Disease

Synonyms
SEMD, MATN3-related
SEMD, typ matryliny-3
SEMD, związana z MATN3
SEMD, matrilin-3 type
ORPHA code
156728
OMIM code
608728
ICD10 code
Q77.7
ICD11 code
LD24.3

No additional description.

Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl