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Retinitis pigmentosa 47(RP47)

MedGen UID:
462411
Concept ID:
C3151061
Disease or Syndrome
Synonym: RP47
 
Gene (location): SAG (2q37.1)
 
Monarch Initiative: MONDO:0013407
OMIM®: 613758

Definition

Retinitis pigmentosa-47 (RP47) is characterized by relatively late-onset visual decline, although most patients experience night blindness in childhood. A characteristic golden sheen, considered to be pathognomonic for Oguchi disease (258100), may be observed in the periphery on ultra-widefield fundus images (Nishiguchi et al., 2019). For a phenotypic description and a discussion of genetic heterogeneity of retinitis pigmentosa, see 268000. [from OMIM]

Clinical features

From HPO
Night blindness
MedGen UID:
10349
Concept ID:
C0028077
Disease or Syndrome
Inability to see well at night or in poor light.
Decreased light- and dark-adapted electroretinogram amplitude
MedGen UID:
326793
Concept ID:
C1839025
Finding
Decreased amplitude of eletrical response upon electroretinography.
Visual impairment
MedGen UID:
777085
Concept ID:
C3665347
Finding
Visual impairment (or vision impairment) is vision loss (of a person) to such a degree as to qualify as an additional support need through a significant limitation of visual capability resulting from either disease, trauma, or congenital or degenerative conditions that cannot be corrected by conventional means, such as refractive correction, medication, or surgery.
Chorioretinal atrophy
MedGen UID:
884881
Concept ID:
C4048273
Disease or Syndrome
Atrophy of the choroid and retinal layers of the fundus.
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.
Pigmentary retinopathy
MedGen UID:
1643295
Concept ID:
C4551715
Disease or Syndrome
An abnormality of the retina characterized by pigment deposition. It is typically associated with migration and proliferation of macrophages or retinal pigment epithelial cells into the retina; melanin from these cells causes the pigmentary changes. Pigmentary retinopathy is a common final pathway of many retinal conditions and is often associated with visual loss.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  

Professional guidelines

PubMed

Stephenson KAJ, Whelan L, Zhu J, Dockery A, Wynne NC, Cairns RM, Kirk C, Turner J, Duignan ES, O'Byrne JJ, Silvestri G, Kenna PF, Farrar GJ, Keegan DJ
Invest Ophthalmol Vis Sci 2023 Jul 3;64(10):23. doi: 10.1167/iovs.64.10.23. PMID: 37466950Free PMC Article
Hufnagel RB, Liang W, Duncan JL, Brewer CC, Audo I, Ayala AR, Branham K, Cheetham JK, Daiger SP, Durham TA, Guan B, Heon E, Hoyng CB, Iannaccone A, Kay CN, Michaelides M, Pennesi ME, Singh MS, Ullah E; Foundation Fighting Blindness Consortium Investigator Group
Hum Mutat 2022 May;43(5):613-624. Epub 2022 Mar 21 doi: 10.1002/humu.24365. PMID: 35266249Free PMC Article
Birtel J, Gliem M, Oishi A, Müller PL, Herrmann P, Holz FG, Mangold E, Knapp M, Bolz HJ, Charbel Issa P
Clin Exp Ophthalmol 2019 Aug;47(6):779-786. Epub 2019 May 8 doi: 10.1111/ceo.13516. PMID: 30977268

Recent clinical studies

Etiology

Jun JH, Kim JS, Palomera LF, Jo DG
Arch Pharm Res 2024 Jan;47(1):20-39. Epub 2023 Dec 27 doi: 10.1007/s12272-023-01482-x. PMID: 38151648
Schuerch K, Woods RL, Lee W, Duncker T, Delori FC, Allikmets R, Tsang SH, Sparrow JR
Invest Ophthalmol Vis Sci 2017 Mar 1;58(3):1843-1855. doi: 10.1167/iovs.16-21302. PMID: 28358950Free PMC Article
Jiang F, Pan Z, Xu K, Tian L, Xie Y, Zhang X, Chen J, Dong B, Li Y
Invest Ophthalmol Vis Sci 2016 Jan 1;57(1):145-52. doi: 10.1167/iovs.15-18190. PMID: 26780318
Yoshida N, Ikeda Y, Murakami Y, Nakatake S, Tachibana T, Notomi S, Hisatomi T, Ishibashi T
Jpn J Ophthalmol 2015 Nov;59(6):373-7. Epub 2015 Aug 29 doi: 10.1007/s10384-015-0405-1. PMID: 26314744
Sahu JK, Jain V
JNMA J Nepal Med Assoc 2008 Oct-Dec;47(172):235-7. PMID: 19079403

Diagnosis

Kim YJ, Kim YN, Yoon YH, Seo EJ, Seo GH, Keum C, Lee BH, Lee JY
Genes (Basel) 2021 Apr 30;12(5) doi: 10.3390/genes12050675. PMID: 33946315Free PMC Article
Nguyen XT, Talib M, van Schooneveld MJ, Brinks J, Ten Brink J, Florijn RJ, Wijnholds J, Verdijk RM, Bergen AA, Boon CJF
Int J Mol Sci 2020 Jan 28;21(3) doi: 10.3390/ijms21030835. PMID: 32012938Free PMC Article
Schuerch K, Woods RL, Lee W, Duncker T, Delori FC, Allikmets R, Tsang SH, Sparrow JR
Invest Ophthalmol Vis Sci 2017 Mar 1;58(3):1843-1855. doi: 10.1167/iovs.16-21302. PMID: 28358950Free PMC Article
Jiang F, Pan Z, Xu K, Tian L, Xie Y, Zhang X, Chen J, Dong B, Li Y
Invest Ophthalmol Vis Sci 2016 Jan 1;57(1):145-52. doi: 10.1167/iovs.15-18190. PMID: 26780318
Skeik N, Jabr FI
Intern Med 2008;47(8):807-8. Epub 2008 Apr 16 doi: 10.2169/internalmedicine.47.0888. PMID: 18421205

Therapy

Jun JH, Kim JS, Palomera LF, Jo DG
Arch Pharm Res 2024 Jan;47(1):20-39. Epub 2023 Dec 27 doi: 10.1007/s12272-023-01482-x. PMID: 38151648
Schwartz SG, Wang X, Chavis P, Kuriyan AE, Abariga SA
Cochrane Database Syst Rev 2020 Jun 18;6(6):CD008428. doi: 10.1002/14651858.CD008428.pub3. PMID: 32573764Free PMC Article
Daschner R, Greppmaier U, Kokelmann M, Rudorf S, Rudorf R, Schleehauf S, Wrobel WG
Biomed Microdevices 2017 Mar;19(1):7. doi: 10.1007/s10544-017-0147-6. PMID: 28124761Free PMC Article
Sahel J, Bonnel S, Mrejen S, Paques M
Dev Ophthalmol 2010;47:160-167. Epub 2010 Aug 10 doi: 10.1159/000320079. PMID: 20703049
Graffin B, Genty I, Crétel E, Jean R, Durand JM
Dig Dis Sci 2002 Apr;47(4):793. doi: 10.1023/a:1014744101321. PMID: 11991611

Prognosis

Schuerch K, Woods RL, Lee W, Duncker T, Delori FC, Allikmets R, Tsang SH, Sparrow JR
Invest Ophthalmol Vis Sci 2017 Mar 1;58(3):1843-1855. doi: 10.1167/iovs.16-21302. PMID: 28358950Free PMC Article
Jiang F, Pan Z, Xu K, Tian L, Xie Y, Zhang X, Chen J, Dong B, Li Y
Invest Ophthalmol Vis Sci 2016 Jan 1;57(1):145-52. doi: 10.1167/iovs.15-18190. PMID: 26780318
Potsidis E, Berson EL, Sandberg MA
Invest Ophthalmol Vis Sci 2011 Nov 29;52(12):9244-9. doi: 10.1167/iovs.11-7892. PMID: 21989720Free PMC Article
Abu Safieh L, Aldahmesh MA, Shamseldin H, Hashem M, Shaheen R, Alkuraya H, Al Hazzaa SA, Al-Rajhi A, Alkuraya FS
J Med Genet 2010 Apr;47(4):236-41. Epub 2009 Oct 26 doi: 10.1136/jmg.2009.070755. PMID: 19858128
Sahu JK, Jain V
JNMA J Nepal Med Assoc 2008 Oct-Dec;47(172):235-7. PMID: 19079403

Clinical prediction guides

Li X, Xiao H, Su Y, Xiao X, Li S, Lin S, Fang L, Sun W, Wang P, Hejtmancik JF, Yu M, Chen L, Zhang Q, Liu X
Br J Ophthalmol 2024 Nov 22;108(12):1679-1687. doi: 10.1136/bjo-2023-324931. PMID: 38749530
Rodriguez-Martinez AC, Higgins BE, Tailor-Hamblin V, Malka S, Cheloni R, Collins AM, Bladen J, Henderson R, Moosajee M
Int J Mol Sci 2023 Sep 11;24(18) doi: 10.3390/ijms241813932. PMID: 37762234Free PMC Article
Yoshida N, Ikeda Y, Murakami Y, Nakatake S, Tachibana T, Notomi S, Hisatomi T, Ishibashi T
Jpn J Ophthalmol 2015 Nov;59(6):373-7. Epub 2015 Aug 29 doi: 10.1007/s10384-015-0405-1. PMID: 26314744
Potsidis E, Berson EL, Sandberg MA
Invest Ophthalmol Vis Sci 2011 Nov 29;52(12):9244-9. doi: 10.1167/iovs.11-7892. PMID: 21989720Free PMC Article
Kar B, John S, Kumaramanickavel G
Clin Genet 1995 Feb;47(2):75-9. doi: 10.1111/j.1399-0004.1995.tb03927.x. PMID: 7606847

Recent systematic reviews

Zada M, Cornish EE, Fraser CL, Jamieson RV, Grigg JR
Acta Ophthalmol 2021 Aug;99(5):499-510. Epub 2020 Nov 30 doi: 10.1111/aos.14662. PMID: 33258268
Schwartz SG, Wang X, Chavis P, Kuriyan AE, Abariga SA
Cochrane Database Syst Rev 2020 Jun 18;6(6):CD008428. doi: 10.1002/14651858.CD008428.pub3. PMID: 32573764Free PMC Article

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