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Retinitis pigmentosa 12(RP12)

MedGen UID:
374019
Concept ID:
C1838647
Disease or Syndrome
Synonyms: RETINITIS PIGMENTOSA WITH OR WITHOUT PARAARTERIOLAR PRESERVATION OF RETINAL PIGMENT EPITHELIUM; RP 12; RP WITH OR WITHOUT PPRPE; RP WITH OR WITHOUT PRESERVED PARAARTERIOLE RETINAL PIGMENT EPITHELIUM; RP12
 
Gene (location): CRB1 (1q31.3)
 
Monarch Initiative: MONDO:0010818
OMIM®: 600105

Definition

Any retinitis pigmentosa in which the cause of the disease is a mutation in the CRB1 gene. [from MONDO]

Clinical features

From HPO
Exotropia
MedGen UID:
4613
Concept ID:
C0015310
Disease or Syndrome
A form of strabismus with one or both eyes deviated outward.
Night blindness
MedGen UID:
10349
Concept ID:
C0028077
Disease or Syndrome
Inability to see well at night or in poor light.
Nystagmus
MedGen UID:
45166
Concept ID:
C0028738
Disease or Syndrome
Rhythmic, involuntary oscillations of one or both eyes related to abnormality in fixation, conjugate gaze, or vestibular mechanisms.
Reduced visual acuity
MedGen UID:
65889
Concept ID:
C0234632
Finding
Diminished clarity of vision.
Optic disc pallor
MedGen UID:
108218
Concept ID:
C0554970
Finding
A pale yellow discoloration of the optic disc (the area of the optic nerve head in the retina). The optic disc normally has a pinkish hue with a central yellowish depression.
Bone spicule pigmentation of the retina
MedGen UID:
323029
Concept ID:
C1836926
Finding
Pigment migration into the retina in a bone-spicule configuration (resembling the nucleated cells within the lacuna of bone).
Undetectable electroretinogram
MedGen UID:
383742
Concept ID:
C1855685
Finding
Lack of any response to stimulation upon electroretinography.
Hyperopia, high
MedGen UID:
341009
Concept ID:
C1855925
Finding
A severe form of hypermetropia with over +5.00 diopters.
Attenuation of retinal blood vessels
MedGen UID:
480605
Concept ID:
C3278975
Finding
Rod-cone dystrophy
MedGen UID:
1632921
Concept ID:
C4551714
Disease or Syndrome
An inherited retinal disease subtype in which the rod photoreceptors appear to be more severely affected than the cone photoreceptors. Typical presentation is with nyctalopia (due to rod dysfunction) followed by loss of mid-peripheral field of vision, which gradually extends and leaves many patients with a small central island of vision due to the preservation of macular cones.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  

Recent clinical studies

Etiology

Ikesugi K, Ichio T, Tsukitome H, Kondo M
Jpn J Ophthalmol 2017 Jul;61(4):293-298. Epub 2017 Apr 26 doi: 10.1007/s10384-017-0517-x. PMID: 28447270
Mathijssen IB, Florijn RJ, van den Born LI, Zekveld-Vroon RC, Ten Brink JB, Plomp AS, Baas F, Meijers-Heijboer H, Bergen AA, van Schooneveld MJ
Retina 2017 Jan;37(1):161-172. doi: 10.1097/IAE.0000000000001127. PMID: 27380427

Diagnosis

Nakazawa M, Hara A, Ishiguro SI
Biomed Res Int 2019;2019:8276140. Epub 2019 Oct 30 doi: 10.1155/2019/8276140. PMID: 31781647Free PMC Article

Prognosis

Ikesugi K, Ichio T, Tsukitome H, Kondo M
Jpn J Ophthalmol 2017 Jul;61(4):293-298. Epub 2017 Apr 26 doi: 10.1007/s10384-017-0517-x. PMID: 28447270
Mathijssen IB, Florijn RJ, van den Born LI, Zekveld-Vroon RC, Ten Brink JB, Plomp AS, Baas F, Meijers-Heijboer H, Bergen AA, van Schooneveld MJ
Retina 2017 Jan;37(1):161-172. doi: 10.1097/IAE.0000000000001127. PMID: 27380427

Clinical prediction guides

Nakazawa M, Hara A, Ishiguro SI
Biomed Res Int 2019;2019:8276140. Epub 2019 Oct 30 doi: 10.1155/2019/8276140. PMID: 31781647Free PMC Article
Mathijssen IB, Florijn RJ, van den Born LI, Zekveld-Vroon RC, Ten Brink JB, Plomp AS, Baas F, Meijers-Heijboer H, Bergen AA, van Schooneveld MJ
Retina 2017 Jan;37(1):161-172. doi: 10.1097/IAE.0000000000001127. PMID: 27380427

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