Infantile Refsum disease

Orpha code: 772OMIM code: 617370

Definition

Infantile Refsum disease (IRD) is the mildest variant of the peroxisome biogenesis disorders, Zellweger syndrome spectrum (PBD- ZSS; see this term), characterized by hypotonia, retinitis pigmentosa, developmental delay, sensorineural hearing loss and liver dysfunction. Phenotypic overlap is seen between IRD and neonatal adrenoleukodystrophy (NALD) (see this term).

Disease data
Classification

Disease

Synonyms
IRD
IRD
Mild PBD-ZSD
Mild peroxisome biogenesis disorder-Zellweger spectrum disorder
ORPHA code
772
OMIM code
617370
ICD10 code
G60.1
ICD11 code
-

No additional description.

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