Riley-Day syndrome

ICD-10 Code G90.1

Riley-Day syndrome

Definition

A congenital disorder caused by mutations in the IKBKAP gene. It is characterized by damage of the sympathetic and parasympathetic and sensory nervous system.

Also known as Dysautonomia, Familial, familial dysautonomia, hereditary sensory and autonomic neuropathy 3, hereditary sensory and autonomic neuropathy type 3, hereditary sensory and autonomic neuropathy type III, hereditary sensory neuropathy type 3, HSAN 3, HSAN III, HSAN3, HSN 3, neuropathy, hereditary sensory and autonomic, type 3, neuropathy, hereditary sensory and autonomic, type III, Riley Day syndrome, Riley-Day syndrome — per MONDO

Also identified as

Affected Organs

Organ Involvement Source
Nervous system Disease Has Associated Anatomic Site NCIT · CC BY 4.0
Nervous system Disease Has Primary Anatomic Site NCIT · CC BY 4.0
Peripheral nervous system Disease Has Associated Anatomic Site NCIT · CC BY 4.0
Peripheral nervous system Disease Has Primary Anatomic Site NCIT · CC BY 4.0