Larsen-like osseous dysplasia-short stature syndrome

Orpha code: 2370OMIM code: 608545

Definicja

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
Klasyfikacja

Malformation syndrome

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

No additional description.

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