Vici syndrome

Orpha code: 1493OMIM code: 242840

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.

Disease data
Classification

Malformation syndrome

Synonyms
Corpus callosum agenesis-cataract-immunodeficiency syndrome
Zespół Dionisi, Viciego, Sabetta i Gambarara
Dionisi-Vici-Sabetta-Gambarara syndrome
ORPHA code
1493
OMIM code
242840
ICD10 code
Q87.8
ICD11 code
4A01.1Y

No additional description.

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