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Osteogenesis imperfecta type 1

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Disease definition

A mild form of osteogenesis imperfecta (OI) characterized by increased bone fragility and low bone mass that clinically manifests with increased susceptibility to bone fractures (including vertebral crush fractures), normal height or short stature (typically between 0 and -2.0 SD scores), mild (Cobb angle <30 degrees) or no scoliosis, blue sclera, and in dentinogenesis imperfecta, and mild long bone bowing bone deformities.

ORPHA:216796

Classification level: Subtype of disorder

Synonym(s):
  • Adair-Dighton syndrome
  • Mild osteogenesis imperfecta
  • Non-deforming osteogenesis imperfecta
  • OI type 1
  • Van der Hoeve syndrome

Prevalence: Unknown

Inheritance: Autosomal dominant

Age of onset: Childhood

ICD-10: Q78.0

ICD-11: LD24.K0

OMIM: 166200 166230

UMLS: C0023931

GARD: 8694

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
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.