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Aplastic anemia-intellectual disability-dwarfism syndrome

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Disease definition

A rare constitutional aplastic anemia characterized by aplastic anemia, intellectual disability, short stature and microcephaly. Skin pigmentation or cafe au lait spots are often present. Majority of the patients present global developmental delay with impaired motor skills, learning disabilities, speech delay whereas some patients also may have behavioral problems including autistic features. Patients often develop premalignant myelodysplastic syndromes or leukemia.

ORPHA:611216

Classification level: Disorder

Synonym(s):
  • AMeD syndrome

Source: PubMed ID 33355142 33147438

Prevalence: <1 / 1 000 000

Inheritance: -

Age of onset: Infancy, Neonatal

ICD-10: D61.0

ICD-11: 3A70.0

OMIM: 619151

UMLS: C5436906

A summary on this disease is available in Français, Español, Deutsch, Português, Nederlands, Polski
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
English (2016) - Br J Haematol Logo ERN
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