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Amaurosis-hypertrichosis syndrome

MedGen UID:
341805
Concept ID:
C1857588
Disease or Syndrome
Synonym: Amaurosis congenita cone-rod type with congenital hypertrichosis
Modes of inheritance:
Autosomal recessive inheritance
MedGen UID:
141025
Concept ID:
C0441748
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in individuals with two pathogenic alleles, either homozygotes (two copies of the same mutant allele) or compound heterozygotes (whereby each copy of a gene has a distinct mutant allele).
 
Monarch Initiative: MONDO:0008766
OMIM®: 204110
Orphanet: ORPHA1021

Definition

A rare, syndromic, inherited retinal disorder characterized by cone-rod type congenital amaurosis, severe retinal dystrophy leading to visual impairment and profound photophobia (without night blindness), and trichomegaly (bushy eyebrows with synophrys, excessive facial and body hair (including marked circumaleolar hypertrichosis). There have been no further descriptions in the literature since 1989. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAmaurosis-hypertrichosis syndrome
Follow this link to review classifications for Amaurosis-hypertrichosis syndrome in Orphanet.

Recent clinical studies

Diagnosis

Luder HU, Gerth-Kahlert C, Ostertag-Benzinger S, Schorderet DF
PLoS One 2013;8(10):e78529. Epub 2013 Oct 23 doi: 10.1371/journal.pone.0078529. PMID: 24194943Free PMC Article
Zobor D, Kaufmann DH, Weckerle P, Sauer A, Wissinger B, Wilhelm H, Kohl S
Ophthalmic Genet 2012 Mar;33(1):34-8. Epub 2011 Jul 5 doi: 10.3109/13816810.2011.592178. PMID: 21728811
Jalili IK
Eye (Lond) 2010 Nov;24(11):1659-68. Epub 2010 Aug 13 doi: 10.1038/eye.2010.103. PMID: 20706282

Therapy

Zobor D, Kaufmann DH, Weckerle P, Sauer A, Wissinger B, Wilhelm H, Kohl S
Ophthalmic Genet 2012 Mar;33(1):34-8. Epub 2011 Jul 5 doi: 10.3109/13816810.2011.592178. PMID: 21728811

Clinical prediction guides

Luder HU, Gerth-Kahlert C, Ostertag-Benzinger S, Schorderet DF
PLoS One 2013;8(10):e78529. Epub 2013 Oct 23 doi: 10.1371/journal.pone.0078529. PMID: 24194943Free PMC Article
Zobor D, Kaufmann DH, Weckerle P, Sauer A, Wissinger B, Wilhelm H, Kohl S
Ophthalmic Genet 2012 Mar;33(1):34-8. Epub 2011 Jul 5 doi: 10.3109/13816810.2011.592178. PMID: 21728811
Jalili IK
Eye (Lond) 2010 Nov;24(11):1659-68. Epub 2010 Aug 13 doi: 10.1038/eye.2010.103. PMID: 20706282

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