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Aneurysm-osteoarthritis syndrome

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Disease definition

A rare, genetic, systemic disease characterized by the presence of arterial aneurysms, tortuosity and dissection throughout the arterial tree, associated with early-onset osteoarthritis (predominantly affecting the spine, hands and/or wrists, and knees) and mild craniofacial dysmorphism (incl. long face, high forehead, flat supraorbital ridges, hypertelorism, malar hypoplasia and, anomalies of the palate and uvula), as well as mild skeletal and cutaneous anomalies. Joint abnormalities, such as osteochondritis dissecans and intervertebral disc degeneration, are frequently associated. Additional cardiovascular anomalies may include mitral valve defects, congenital heart malformations, ventricular hypertrophy and atrial fibrillation.

ORPHA:284984

Classification level: Disorder

Prevalence: <1 / 1 000 000

Inheritance: Autosomal dominant

Age of onset: All ages

ICD-10: Q87.8

ICD-11: LD28.0Y

OMIM: 613795

UMLS: C4760764

GARD: 10997

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
Français (2019.pdf) - Fondation Groupama
Guidelines
Emergency guidelines
Français (2017.pdf) - Orphanet Urgences
Polski (2007.pdf) - Orphanet Urgences
Deutsch (2014.pdf) - Orphanet Urgences
English (2007.pdf) - Orphanet Urgences
Español (2007.pdf) - Orphanet Urgences
Italiano (2007.pdf) - Orphanet Urgences
Português (2007.pdf) - Orphanet Urgences
Anesthesia guidelines
English (2013) - Orphananesthesia
Español (2013) - Orphananesthesia
Čeština (2013) - Orphananesthesia
Clinical practice guidelines
English (2024) - Eur Heart J Logo ERN
English (2023) - Eur J Med Genet Logo ERN
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.