Intermediate osteopetrosis

Orpha code: 210110OMIM code: 611497

Definicja

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
Klasyfikacja

Malformation syndrome

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

No additional description.

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