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Osteogenesis imperfecta type 5

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Disease definition

A moderate form of osteogenesis imperfecta characterized by increased bone fragility and low bone mass that clinically manifests with susceptibility to bone fractures of variable severity, metaphyseal changes at birth, short stature, dislocation of the radial head, mineralized interosseous membranes, hyperplasic callus (occurring more often during periods of more rapid growth), white sclera and absence of dentinogenesis imperfecta.

ORPHA:216828

Classification level: Subtype of disorder

Synonym(s):
  • OI type 5

Prevalence: <1 / 1 000 000

Inheritance: Autosomal dominant, Autosomal recessive

Age of onset: Infancy, Neonatal

ICD-10: Q78.0

ICD-11: LD24.K0

OMIM: 610967

UMLS: C2931093

MeSH: C567042

GARD: 8699

A summary on this disease is available in Français, Español, Deutsch, Italiano, Nederlands Polski
Detailed information

Logo ERN: produced/endorsed by ERN(s) Logo FSMR: produced/endorsed by FSMR(s)

General public
Article for general public
Svenska (2023) - Socialstyrelsen
Guidelines
Emergency guidelines
Français (2018.pdf) - Orphanet Urgences
Polski (2008.pdf) - Orphanet Urgences
Deutsch (2008.pdf) - Orphanet Urgences
English (2008.pdf) - Orphanet Urgences
Español (2008.pdf) - Orphanet Urgences
Italiano (2008.pdf) - Orphanet Urgences
Português (2008.pdf) - Orphanet Urgences
Anesthesia guidelines
Deutsch (2019) - Orphananesthesia
English (2019) - Orphananesthesia
Čeština (2019) - Orphananesthesia
Clinical practice guidelines
Español (2020.pdf) - Asoc Española de Pediatría
Genetic testing
Guidance for genetic testing
English (2012) - Eur J Hum Genet
Diagnostic Keys
Français (2024) - Les clés du diagnostic Logo FSMR
Français (2024) - Les clés du diagnostic Logo FSMR
Français (2024) - Les clés du diagnostic Logo FSMR
Français (2024) - Les clés du diagnostic Logo FSMR
Français (2024) - Les clés du diagnostic Logo FSMR
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