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Radio-ulnar synostosis-amegakaryocytic thrombocytopenia syndrome

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Disease definition

Radio-ulnar synostosis-amegakaryocytic thrombocytopenia syndrome is characterised by the association of proximal fusion of the radius and ulna with congenital amegakaryocytic thrombocytopaenia. Less than 10 cases have been reported in the literature so far. The syndrome is transmitted as an autosomal dominant trait and is caused by mutations in the HOXA11 gene (7p15).

ORPHA:71289

Classification level: Disorder

Synonym(s):
  • ATRUS syndrome

Prevalence: <1 / 1 000 000

Inheritance: Autosomal dominant

Age of onset: Neonatal

ICD-10: Q87.2

ICD-11: 3B64.01

OMIM: 605432 616738

UMLS: C4303670

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

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

Guidelines
Emergency guidelines
Français (2022.pdf) - Orphanet Urgences
Clinical practice guidelines
Genetic testing
Guidance for genetic testing
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