Larsen-like osseous dysplasia-short stature syndrome

Orpha code: 2370OMIM code: 608545

Definition

Larsen-like osseous dysplasia-short stature syndrome is a rare primary bone dysplasia characterized by a Larsen-like phenotype including multiple, congenital, large joint dislocations, craniofacial abnormalities (i.e. macrocephaly, flat occiput, prominent forehead, hypertelorism, low-set, malformed ears, flat nose, cleft palate), spinal abnormalities, cylindrical fingers, and talipes equinovarus, as well as growth retardation (resulting in short stature) and delayed bone age. Other reported clinical manifestations include severe developmental delay, hypotonia, clinodactyly, congenital heart defect and renal dysplasia.

Disease data
Classification

Malformation syndrome

ORPHA code
2370
OMIM code
608545
ICD10 code
Q74.8
ICD11 code
LD24.E

No additional description.

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