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Ehlers-Danlos/osteogenesis imperfecta syndrome

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Disease definition

A rare systemic disease characterized by the association of the features of Ehlers-Danlos syndrome with those of osteogenesis imperfecta. Predominant clinical manifestations include generalized joint hypermobility and dislocations, skin hyperextensibility and/or translucency, easy bruising, and invariable association with mild signs of osteogenesis imperfecta, including short stature, blue sclera, and osteopenia or fractures.

ORPHA:230857

Classification level: Disorder

Synonym(s):
  • EDS/OI syndrome

Source: PubMed ID 23692737

Prevalence: <1 / 1 000 000

Inheritance: Autosomal dominant

Age of onset: Infancy, Neonatal

ICD-10: Q79.6

ICD-11: LD28.1Y

OMIM: 619115 619120

UMLS: C4518787

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)

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
Español (2019) - Orphananesthesia
Čeština (2019) - Orphananesthesia
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.