Homepage > Rare diseases > Search

Search for a rare disease

*
(*) mandatory field

Osteogenesis imperfecta type 3

Suggest an update
Your message has been sent Your message has not been sent. Please contact an administrator.
Disease definition

A severe type form osteogenesis imperfecta characterized by increased bone fragility and low bone mass clinically manifesting as susceptibility to bone fractures, severe short stature, a triangular face, moderate to severe scoliosis, blue or blue-grey sclera, and dentinogenesis imperfecta.

ORPHA:216812

Classification level: Subtype of disorder

Synonym(s):
  • OI type 3
  • Progressive deforming osteogenesis imperfecta
  • Severe osteogenesis imperfecta

Source: PubMed ID 36779427

Prevalence: Unknown

Inheritance: Autosomal dominant, Autosomal recessive, X-linked recessive

Age of onset: Infancy, Neonatal

ICD-10: Q78.0

ICD-11: LD24.K0

OMIM: 259420 259440 610682 610915 610968 613848 613982 617952 614856 615220 616229

UMLS: C0268362

MeSH: C536044

GARD: 8695

A summary on this disease is available in Français, Español, Deutsch, Italiano, Nederlands
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
Disease review articles
Clinical genetics review
English (2021) - GeneReviews
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
The documents contained in this website are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.