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Retinitis pigmentosa 51(RP51)

MedGen UID:
462065
Concept ID:
C3150715
Disease or Syndrome
Synonym: RP51
 
Gene (location): TTC8 (14q31.3)
 
Monarch Initiative: MONDO:0013274
OMIM®: 613464

Definition

Any retinitis pigmentosa in which the cause of the disease is a mutation in the TTC8 gene. [from MONDO]

Clinical features

From HPO
Abnormality of the kidney
MedGen UID:
78593
Concept ID:
C0266292
Congenital Abnormality
An abnormality of the kidney.
Polydactyly
MedGen UID:
57774
Concept ID:
C0152427
Congenital Abnormality
A congenital anomaly characterized by the presence of supernumerary fingers or toes.
Obesity
MedGen UID:
18127
Concept ID:
C0028754
Disease or Syndrome
Accumulation of substantial excess body fat.
Photophobia
MedGen UID:
43220
Concept ID:
C0085636
Sign or Symptom
Excessive sensitivity to light with the sensation of discomfort or pain in the eyes due to exposure to bright light.
Global developmental delay
MedGen UID:
107838
Concept ID:
C0557874
Finding
A delay in the achievement of motor or mental milestones in the domains of development of a child, including motor skills, speech and language, cognitive skills, and social and emotional skills. This term should only be used to describe children younger than five years of age.
Pallor
MedGen UID:
10547
Concept ID:
C0030232
Finding
Abnormally pale skin.
Macular degeneration
MedGen UID:
7434
Concept ID:
C0024437
Disease or Syndrome
A nonspecific term denoting degeneration of the retinal pigment epithelium and/or retinal photoreceptor cells of the macula lutea.
Night blindness
MedGen UID:
10349
Concept ID:
C0028077
Disease or Syndrome
Inability to see well at night or in poor light.
Reduced visual acuity
MedGen UID:
65889
Concept ID:
C0234632
Finding
Diminished clarity of vision.
High myopia
MedGen UID:
78759
Concept ID:
C0271183
Disease or Syndrome
A severe form of myopia with greater than -6.00 diopters.
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).
Abnormal light- and dark-adapted electroretinogram
MedGen UID:
462461
Concept ID:
C3151111
Finding
An abnormality of the combined rod-and-cone response on electroretinogram.
Attenuation of retinal blood vessels
MedGen UID:
480605
Concept ID:
C3278975
Finding
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.
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  

Professional guidelines

PubMed

Erger F, Brüchle NO, Gembruch U, Zerres K
Arch Gynecol Obstet 2017 Apr;295(4):897-906. Epub 2017 Mar 10 doi: 10.1007/s00404-017-4336-6. PMID: 28283827
Berkowitz BA, Bissig D, Roberts R
Prog Retin Eye Res 2016 Mar;51:90-106. Epub 2015 Sep 4 doi: 10.1016/j.preteyeres.2015.09.001. PMID: 26344734Free PMC Article
Barisic I, Boban L, Loane M, Garne E, Wellesley D, Calzolari E, Dolk H, Addor MC, Bergman JE, Braz P, Draper ES, Haeusler M, Khoshnood B, Klungsoyr K, Pierini A, Queisser-Luft A, Rankin J, Rissmann A, Verellen-Dumoulin C
Eur J Hum Genet 2015 Jun;23(6):746-52. Epub 2014 Sep 3 doi: 10.1038/ejhg.2014.174. PMID: 25182137Free PMC Article

Recent clinical studies

Etiology

Eshel YM, Abaev O, Yahalom C
Eur J Ophthalmol 2024 Jul;34(4):986-991. Epub 2023 Nov 3 doi: 10.1177/11206721231212768. PMID: 37920903Free PMC Article
Azab B, Barham R, Ali D, Dardas Z, Rashdan L, Bijawi M, Maswadi R, Awidi A, Jafar H, Abu-Ameerh M, Al-Bdour M, Amr S, Awidi A
Can J Ophthalmol 2019 Feb;54(1):51-59. Epub 2018 Apr 10 doi: 10.1016/j.jcjo.2018.02.018. PMID: 30851774
McMurtrey JJ, Tso MOM
Surv Ophthalmol 2018 Nov-Dec;63(6):769-781. Epub 2018 Mar 16 doi: 10.1016/j.survophthal.2018.03.002. PMID: 29551596
Horsager A, Greenberg RJ, Fine I
Invest Ophthalmol Vis Sci 2010 Feb;51(2):1223-33. Epub 2009 Sep 9 doi: 10.1167/iovs.09-3746. PMID: 19741248Free PMC Article
Michaelides M, Hardcastle AJ, Hunt DM, Moore AT
Surv Ophthalmol 2006 May-Jun;51(3):232-58. doi: 10.1016/j.survophthal.2006.02.007. PMID: 16644365

Diagnosis

Eshel YM, Abaev O, Yahalom C
Eur J Ophthalmol 2024 Jul;34(4):986-991. Epub 2023 Nov 3 doi: 10.1177/11206721231212768. PMID: 37920903Free PMC Article
Busskamp V
J Perinat Med 2023 Jul 26;51(6):759-762. Epub 2022 Dec 7 doi: 10.1515/jpm-2022-0510. PMID: 36474335
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
Azab B, Barham R, Ali D, Dardas Z, Rashdan L, Bijawi M, Maswadi R, Awidi A, Jafar H, Abu-Ameerh M, Al-Bdour M, Amr S, Awidi A
Can J Ophthalmol 2019 Feb;54(1):51-59. Epub 2018 Apr 10 doi: 10.1016/j.jcjo.2018.02.018. PMID: 30851774
Bird AC
Trans Ophthalmol Soc N Z 1977;29:51-8. PMID: 269555

Therapy

Haqq AM, Chung WK, Dollfus H, Haws RM, Martos-Moreno GÁ, Poitou C, Yanovski JA, Mittleman RS, Yuan G, Forsythe E, Clément K, Argente J
Lancet Diabetes Endocrinol 2022 Dec;10(12):859-868. Epub 2022 Nov 7 doi: 10.1016/S2213-8587(22)00277-7. PMID: 36356613Free PMC Article
Berkowitz BA, Bissig D, Roberts R
Prog Retin Eye Res 2016 Mar;51:90-106. Epub 2015 Sep 4 doi: 10.1016/j.preteyeres.2015.09.001. PMID: 26344734Free PMC Article
Hirata A, Okinami S, Hayashi K
Graefes Arch Clin Exp Ophthalmol 2011 Sep;249(9):1409-15. Epub 2011 Jan 6 doi: 10.1007/s00417-010-1605-5. PMID: 21210140
Salom D, Diaz-Llopis M, Quijada A, García-Delpech S, Udaondo P, Romero FJ, Millan JM, Arevalo JF
Invest Ophthalmol Vis Sci 2010 Jun;51(6):3157-61. Epub 2010 Jan 6 doi: 10.1167/iovs.09-4390. PMID: 20053975
Clarke CE, Alger S, Preece MA, Burdon MA, Chavda S, Denis S, Ferdinandusse S, Wanders RJ
Neurology 2004 Jul 13;63(1):188-9. doi: 10.1212/01.wnl.0000132841.81250.b7. PMID: 15249642

Prognosis

Eshel YM, Abaev O, Yahalom C
Eur J Ophthalmol 2024 Jul;34(4):986-991. Epub 2023 Nov 3 doi: 10.1177/11206721231212768. PMID: 37920903Free PMC Article
Yamashita K, Sundaram P, Uchida T, Matsuo T, Wong W
J Neural Eng 2021 May 5;18(4) doi: 10.1088/1741-2552/abf892. PMID: 33857924
Ma DJ, Lee HS, Kim K, Choi S, Jang I, Cho SH, Yoon CK, Lee EK, Yu HG
BMC Med Genomics 2021 Mar 10;14(1):74. doi: 10.1186/s12920-021-00874-6. PMID: 33691693Free PMC Article
Mary L, Chennen K, Stoetzel C, Antin M, Leuvrey A, Nourisson E, Alanio-Detton E, Antal MC, Attié-Bitach T, Bouvagnet P, Bouvier R, Buenerd A, Clémenson A, Devisme L, Gasser B, Gilbert-Dussardier B, Guimiot F, Khau Van Kien P, Leroy B, Loget P, Martinovic J, Pelluard F, Perez MJ, Petit F, Pinson L, Rooryck-Thambo C, Poch O, Dollfus H, Schaefer E, Muller J
Clin Genet 2019 Mar;95(3):384-397. doi: 10.1111/cge.13500. PMID: 30614526
Lee S, Inglis H, Boylan B, Grigg J
J Pediatr Ophthalmol Strabismus 2014 Mar 4;51 Online:e13-5. doi: 10.3928/01913913-20140225-02. PMID: 25314308

Clinical prediction guides

Yamashita K, Sundaram P, Uchida T, Matsuo T, Wong W
J Neural Eng 2021 May 5;18(4) doi: 10.1088/1741-2552/abf892. PMID: 33857924
Ma DJ, Lee HS, Kim K, Choi S, Jang I, Cho SH, Yoon CK, Lee EK, Yu HG
BMC Med Genomics 2021 Mar 10;14(1):74. doi: 10.1186/s12920-021-00874-6. PMID: 33691693Free PMC Article
McMurtrey JJ, Tso MOM
Surv Ophthalmol 2018 Nov-Dec;63(6):769-781. Epub 2018 Mar 16 doi: 10.1016/j.survophthal.2018.03.002. PMID: 29551596
Vecino E, Rodriguez FD, Ruzafa N, Pereiro X, Sharma SC
Prog Retin Eye Res 2016 Mar;51:1-40. Epub 2015 Jun 23 doi: 10.1016/j.preteyeres.2015.06.003. PMID: 26113209
Greenwald DL, Cashman SM, Kumar-Singh R
Invest Ophthalmol Vis Sci 2010 Dec;51(12):6374-80. Epub 2010 Jul 29 doi: 10.1167/iovs.10-5781. PMID: 20671268Free PMC Article

Recent systematic reviews

Sobh M, Lagali PS, Ghiasi M, Montroy J, Dollin M, Hurley B, Leonard BC, Dimopoulos I, Lafreniere M, Fergusson DA, Lalu MM, Tsilfidis C
Transl Vis Sci Technol 2023 Nov 1;12(11):24. doi: 10.1167/tvst.12.11.24. PMID: 37982768Free PMC Article
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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