Intermediate osteopetrosis

Orpha code: 210110OMIM code: 611497

Definition

A rare, genetic primary bone dysplasia with increased bone density characterized by susceptibility to fractures after minor trauma, anemia, and characteristic skeletal radiographic changes, such as sandwich vertebra, bone-within-bone appearance, Erlenmeyer-shaped femoral metaphysis, and mild osteosclerosis of the skull base. Dental anomalies and visual impairment secondary to optic nerve compression have been rarely described.

Disease data
Classification

Malformation syndrome

Synonyms
Autosomal recessive intermediate osteopetrosis
Autosomalna recesywna osteopetroza pośrednia
ORPHA code
210110
OMIM code
611497
ICD10 code
Q78.2
ICD11 code
LD24.10

No additional description.

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