AICA-ribosiduria

Orpha code: 250977OMIM code: 608688

Definition

A rare and severe inborn metabolic disease characterized clinically by the association of severe-to-profound neurodevelopmental impairment, severe visual impairment, ante-postnatal growth impairment, severe scoliosis and, frequently, early-onset epilepsy.

Disease data
Classification

Disease

Synonyms
5-amino-4-imidazole carboxamide ribosiduria
5-amino-4-imidazole carboxamide ribosiduria
Niedobór ATIC
AICA-ribosiduria due to ATIC deficiency
AICAR transformylase/IMP cyclohydrolase deficiency
ATIC deficiency
ORPHA code
250977
OMIM code
608688
ICD10 code
E79.8
ICD11 code
5C55.0Y

No additional description.

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