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Cataract 1 multiple types(CZP1; CZP; CTRCT1; CAE1)

MedGen UID:
349374
Concept ID:
C1861828
Disease or Syndrome
Synonyms: Cataract 1; CATARACT 1 WITH MICROCORNEA; CATARACT 1, MULTIPLE TYPES, WITH OR WITHOUT MICROCORNEA; CATARACT 1, NUCLEAR PROGRESSIVE; CATARACT 1, POSTERIOR SUBCAPSULAR, WITH MICROCORNEA; CATARACT 1, STELLATE NUCLEAR, WITH MICROCORNEA; Cataract microcornea syndrome; CATARACT, DUFFY-LINKED
 
Gene (location): GJA8 (1q21.2)
 
Monarch Initiative: MONDO:0007285
OMIM®: 116200

Definition

Mutations in the GJA8 gene have been found to cause several types of autosomal dominant cataract, which have been described as congenital, zonular pulverulent, nuclear progressive, nuclear pulverulent, stellate nuclear, nuclear total, total, and posterior subcapsular. Cataract associated with microcornea, sometimes called the cataract-microcornea syndrome, is also caused by mutation in the GJA8 gene. Before it was known that mutation in the GJB8 gene caused multiple types of cataract, this entry was titled 'Cataract, zonular pulverulent, 1,' with the symbols CZP1, CZP, and CAE1. [from OMIM]

Clinical features

From HPO
Microcornea
MedGen UID:
78610
Concept ID:
C0266544
Congenital Abnormality
A congenital abnormality of the cornea in which the cornea and the anterior segment of the eye are smaller than normal. The horizontal diameter of the cornea does not reach 10 mm even in adulthood.
Nuclear cataract
MedGen UID:
140274
Concept ID:
C0392557
Finding
A nuclear cataract is an opacity or clouding that develops in the lens nucleus. That is, a nuclear cataract is one that is located in the center of the lens. The nucleus tends to darken changing from clear to yellow and sometimes brown.
Posterior subcapsular cataract
MedGen UID:
163646
Concept ID:
C0858617
Acquired Abnormality
A type of cataract affecting the posterior pole of lens immediately adjacent to ('beneath') the Lens capsule.
Pulverulent cataract
MedGen UID:
318793
Concept ID:
C1833118
Congenital Abnormality
A kind of congenital cataract that is characterized by a hollow sphere of punctate opacities involving the fetal nucleus and that usually occurs bilaterally.

Professional guidelines

PubMed

Wykrota AA, Abdin AD, Munteanu C, Löw U, Seitz B
Graefes Arch Clin Exp Ophthalmol 2023 Dec;261(12):3569-3579. Epub 2023 Jul 11 doi: 10.1007/s00417-023-06163-5. PMID: 37432451Free PMC Article
Leonardi A, Tavolato M, Curnow SJ, Fregona IA, Violato D, Alió JL
J Cataract Refract Surg 2009 Feb;35(2):240-7. doi: 10.1016/j.jcrs.2008.10.030. PMID: 19185237
Pasquale LR, Dolgitser M, Wentzloff JN, Stern LS, Doyle JJ, Chiang TH, Walt JG
Ophthalmology 2008 Apr;115(4):633-638.e4. Epub 2007 Aug 22 doi: 10.1016/j.ophtha.2007.04.059. PMID: 17716736

Recent clinical studies

Etiology

Fan H, Han X, Shang X, Zhu Z, He M, Xu G, Chen Z, Deng R
Eye (Lond) 2023 Oct;37(15):3234-3242. Epub 2023 Mar 27 doi: 10.1038/s41433-023-02498-9. PMID: 36973404Free PMC Article
Wenninger S, Stahl K, Montagnese F, Schoser B
Eur Neurol 2020;83(5):523-533. Epub 2020 Oct 29 doi: 10.1159/000511237. PMID: 33120389
Theodoropoulou S, Samoli E, Theodossiadis PG, Papathanassiou M, Lagiou A, Lagiou P, Tzonou A
Int Ophthalmol 2014 Feb;34(1):59-68. Epub 2013 May 29 doi: 10.1007/s10792-013-9795-6. PMID: 23715849
Lu-Emerson C, Plotkin SR
Rev Neurol Dis 2009 Summer;6(3):E81-6. PMID: 19898272
Lee MD, King LS, Agre P
Medicine (Baltimore) 1997 May;76(3):141-56. doi: 10.1097/00005792-199705000-00001. PMID: 9193450

Diagnosis

Hashemi H, Asharlous A, Yekta A, Aghamirsalim M, Nabovati P, Sadoughi MM, Khabazkhoob M
Jpn J Ophthalmol 2022 Sep;66(5):461-473. Epub 2022 Aug 10 doi: 10.1007/s10384-022-00936-x. PMID: 35947225
Wenninger S, Stahl K, Montagnese F, Schoser B
Eur Neurol 2020;83(5):523-533. Epub 2020 Oct 29 doi: 10.1159/000511237. PMID: 33120389
Chee SP, Chan NS, Yang Y, Ti SE
Br J Ophthalmol 2019 Apr;103(4):544-550. Epub 2018 Jun 15 doi: 10.1136/bjophthalmol-2018-312289. PMID: 29907631
Calladine D, Evans JR, Shah S, Leyland M
Cochrane Database Syst Rev 2012 Sep 12;(9):CD003169. doi: 10.1002/14651858.CD003169.pub3. PMID: 22972061
Lu-Emerson C, Plotkin SR
Rev Neurol Dis 2009 Summer;6(3):E81-6. PMID: 19898272

Therapy

Wykrota AA, Abdin AD, Munteanu C, Löw U, Seitz B
Graefes Arch Clin Exp Ophthalmol 2023 Dec;261(12):3569-3579. Epub 2023 Jul 11 doi: 10.1007/s00417-023-06163-5. PMID: 37432451Free PMC Article
Hashemi H, Asharlous A, Yekta A, Aghamirsalim M, Nabovati P, Sadoughi MM, Khabazkhoob M
Jpn J Ophthalmol 2022 Sep;66(5):461-473. Epub 2022 Aug 10 doi: 10.1007/s10384-022-00936-x. PMID: 35947225
Chou PH, Chu CS, Chen YH, Hsu MY, Huang MW, Lan TH, Lin CH
J Affect Disord 2017 Jun;215:237-244. Epub 2017 Mar 18 doi: 10.1016/j.jad.2017.03.044. PMID: 28342338
Theodoropoulou S, Samoli E, Theodossiadis PG, Papathanassiou M, Lagiou A, Lagiou P, Tzonou A
Int Ophthalmol 2014 Feb;34(1):59-68. Epub 2013 May 29 doi: 10.1007/s10792-013-9795-6. PMID: 23715849
Calladine D, Evans JR, Shah S, Leyland M
Cochrane Database Syst Rev 2012 Sep 12;(9):CD003169. doi: 10.1002/14651858.CD003169.pub3. PMID: 22972061

Prognosis

Wykrota AA, Abdin AD, Munteanu C, Löw U, Seitz B
Graefes Arch Clin Exp Ophthalmol 2023 Dec;261(12):3569-3579. Epub 2023 Jul 11 doi: 10.1007/s00417-023-06163-5. PMID: 37432451Free PMC Article
Wenninger S, Stahl K, Montagnese F, Schoser B
Eur Neurol 2020;83(5):523-533. Epub 2020 Oct 29 doi: 10.1159/000511237. PMID: 33120389
Chee SP, Chan NS, Yang Y, Ti SE
Br J Ophthalmol 2019 Apr;103(4):544-550. Epub 2018 Jun 15 doi: 10.1136/bjophthalmol-2018-312289. PMID: 29907631
Khan S, Finger PT, Yu GP, Razzaq L, Jager MJ, de Keizer RJ, Sandkull P, Seregard S, Gologorsky D, Schefler AC, Murray TG, Kivelä T, Giuliari GP, McGowan H, Simpson ER, Corriveau C, Coupland SE, Damato BE
Arch Ophthalmol 2012 Jan;130(1):57-64. Epub 2011 Sep 12 doi: 10.1001/archophthalmol.2011.286. PMID: 21911649
Yang LL, Lambert SR
Ophthalmol Clin North Am 2001 Sep;14(3):467-77. doi: 10.1016/s0896-1549(05)70245-5. PMID: 11705147

Clinical prediction guides

Yotsukura E, Ayaki M, Nezu N, Torii H, Arai H, Sakatani K, Tsubota K, Negishi K
Sci Rep 2020 Oct 14;10(1):17273. doi: 10.1038/s41598-020-72846-2. PMID: 33057036Free PMC Article
Chee SP, Chan NS, Yang Y, Ti SE
Br J Ophthalmol 2019 Apr;103(4):544-550. Epub 2018 Jun 15 doi: 10.1136/bjophthalmol-2018-312289. PMID: 29907631
Iwase T, Yamamoto K, Yanagida K, Kobayashi M, Ra E, Murotani K, Terasaki H
Medicine (Baltimore) 2016 Aug;95(32):e4317. doi: 10.1097/MD.0000000000004317. PMID: 27512843Free PMC Article
Ni W, Li X, Ao M, Zhang H, Hou Z, Si S, Wang W
Eye (Lond) 2012 Nov;26(11):1402-11. Epub 2012 Aug 10 doi: 10.1038/eye.2012.168. PMID: 22878441Free PMC Article
Moser AB, Steinberg SJ, Watkins PA, Moser HW, Ramaswamy K, Siegmund KD, Lee DR, Ely JJ, Ryder OA, Hacia JG
Lipids Health Dis 2011 Jun 17;10:101. doi: 10.1186/1476-511X-10-101. PMID: 21679470Free PMC Article

Recent systematic reviews

Calladine D, Evans JR, Shah S, Leyland M
Cochrane Database Syst Rev 2012 Sep 12;(9):CD003169. doi: 10.1002/14651858.CD003169.pub3. PMID: 22972061

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