Orphanet: Vici syndrome

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Vici syndrome

Disease definition

Vici syndrome is a very rare and severe congenital multisystem disorder characterized by the principal features of agenesis of the corpus callosum, cataracts, oculocutaneous hypopigmentation, cardiomyopathy and combined immunodeficiency.


Classification level: Disorder
  • Synonym(s):
    • Corpus callosum agenesis-cataract-immunodeficiency syndrome
    • Dionisi-Vici-Sabetta-Gambarara syndrome
  • Prevalence: <1 / 1 000 000
  • Inheritance: Autosomal recessive 
  • Age of onset: Neonatal, Antenatal
  • ICD-10: Q87.8
  • OMIM: 242840
  • UMLS: C1855772
  • MeSH: -
  • GARD: 448
  • MedDRA: -

Detailed information


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