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Metadata
ID DOID:0110122
PURL http://purl.obolibrary.org/obo/DOID_0110122 Copy
Name Axenfeld-Rieger syndrome type 3
Definition An Axenfeld-Rieger characterized by an anteriorly displaced Schwalbe line, the presence of another ocular anomaly (hypoplasia of iris stroma, corectopia, or iridocorneal adhesions), and nonocular anomalies including maxillary hypoplasia, hypodontia, microdontia, protuberant periumbilical skin, sensorineural hearing loss, and congenital cardiac or renal anomalies syndrome that has_material_basis_in heterozygous mutation in the FOXC1 gene on chromosome 6p25.
https://www.ncbi.nlm.nih.gov/pubmed/9792859
Xrefs

ICD10CM:Q13.8

MIM:602482

Synonyms

anterior chamber cleavage syndrome [EXACT]

anterior segment mesenchymal dysgenesis [EXACT]

Axenfeld-Rieger anomaly with or without cardiac defects and/or sensorineural hearing loss [EXACT]

RIEG3 [EXACT]

Rieger syndrome type 3 [EXACT]

Parent Relationships

is_a Axenfeld-Rieger syndrome

Subclass Logical Relationships

has material basis in some autosomal dominant inheritance

disease has basis in some structural_variant

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