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Nanophthalmia

MedGen UID:
901455
Concept ID:
C4274282
Congenital Abnormality
Synonym: Nanophthalmos
SNOMED CT: Nanophthalmia (716775009)
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).
Autosomal dominant inheritance
MedGen UID:
141047
Concept ID:
C0443147
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 heterozygotes. In the context of medical genetics, an autosomal dominant disorder is caused when a single copy of the mutant allele is present. Males and females are affected equally, and can both transmit the disorder with a risk of 50% for each child of inheriting the mutant allele.
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0005514
OMIM® Phenotypic series: PS600165
Orphanet: ORPHA35612

Definition

A severe form of microphthalmia with characteristics of a small eye with a short axial length, severe hyperopia, an elevated lens/eye ratio, and a high incidence of angle-closure glaucoma. Nanophthalmia is generally bilateral. Strabism is present in most patients. Mutations in the MFRP gene (11q23.1) have been found to be responsible for the hereditary form with recessive transmission. Chromosomal anomalies involving chromosome 11p and 2q11-14 have been identified for autosomal dominant forms of nanophthalmia. It may be inherited as an autosomal dominant or recessive trait, or may occur sporadically. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVNanophthalmia

Professional guidelines

PubMed

Lang E, Koller S, Atac D, Pfäffli OA, Hanson JVM, Feil S, Bähr L, Bahr A, Kottke R, Joset P, Fasler K, Barthelmes D, Steindl K, Konrad D, Wille DA, Berger W, Gerth-Kahlert C
Acta Ophthalmol 2021 Jun;99(4):e594-e607. Epub 2020 Sep 30 doi: 10.1111/aos.14615. PMID: 32996714

Recent clinical studies

Etiology

Gonzalez-Rodriguez J, Pelcastre EL, Tovilla-Canales JL, Garcia-Ortiz JE, Amato-Almanza M, Villanueva-Mendoza C, Espinosa-Mattar Z, Zenteno JC
Br J Ophthalmol 2010 Aug;94(8):1100-4. Epub 2010 May 21 doi: 10.1136/bjo.2009.173500. PMID: 20494911
Dietlein TS, Mietz H, Jacobi PC, Krieglstein GK
Graefes Arch Clin Exp Ophthalmol 1996 Aug;234 Suppl 1:S187-92. doi: 10.1007/BF02343070. PMID: 8871172

Diagnosis

Acón D, Hussain RM, Quan AV, Mendoza-Santiesteban C, Berrocal AM
Ophthalmic Surg Lasers Imaging Retina 2021 Aug;52(8):444-446. Epub 2021 Aug 1 doi: 10.3928/23258160-20210727-06. PMID: 34410185
Terubayashi Y, Morishita S, Kohmoto R, Mimura M, Fukumoto M, Sato T, Kobayashi T, Kida T, Ikeda T
Medicine (Baltimore) 2020 Jul 31;99(31):e21441. doi: 10.1097/MD.0000000000021441. PMID: 32756157Free PMC Article
Said MB, Chouchène E, Salem SB, Daoud K, Largueche L, Bouassida W, Benzina Z, Ayadi H, Söderkvist P, Matri L, Hmani-Aifa M
Gene 2013 Oct 10;528(2):288-94. Epub 2013 Jun 29 doi: 10.1016/j.gene.2013.06.045. PMID: 23820083

Therapy

Terubayashi Y, Morishita S, Kohmoto R, Mimura M, Fukumoto M, Sato T, Kobayashi T, Kida T, Ikeda T
Medicine (Baltimore) 2020 Jul 31;99(31):e21441. doi: 10.1097/MD.0000000000021441. PMID: 32756157Free PMC Article

Prognosis

Said MB, Chouchène E, Salem SB, Daoud K, Largueche L, Bouassida W, Benzina Z, Ayadi H, Söderkvist P, Matri L, Hmani-Aifa M
Gene 2013 Oct 10;528(2):288-94. Epub 2013 Jun 29 doi: 10.1016/j.gene.2013.06.045. PMID: 23820083

Clinical prediction guides

Lang E, Koller S, Atac D, Pfäffli OA, Hanson JVM, Feil S, Bähr L, Bahr A, Kottke R, Joset P, Fasler K, Barthelmes D, Steindl K, Konrad D, Wille DA, Berger W, Gerth-Kahlert C
Acta Ophthalmol 2021 Jun;99(4):e594-e607. Epub 2020 Sep 30 doi: 10.1111/aos.14615. PMID: 32996714
Terubayashi Y, Morishita S, Kohmoto R, Mimura M, Fukumoto M, Sato T, Kobayashi T, Kida T, Ikeda T
Medicine (Baltimore) 2020 Jul 31;99(31):e21441. doi: 10.1097/MD.0000000000021441. PMID: 32756157Free PMC Article
Collery RF, Volberding PJ, Bostrom JR, Link BA, Besharse JC
Invest Ophthalmol Vis Sci 2016 Dec 1;57(15):6805-6814. doi: 10.1167/iovs.16-19593. PMID: 28002843Free PMC Article
Li H, Wang JX, Wang CY, Yu P, Zhou Q, Chen YG, Zhao LH, Zhang YP
Hum Genet 2008 Jan;122(6):589-93. Epub 2007 Oct 9 doi: 10.1007/s00439-007-0435-y. PMID: 17924146
Yeyati PL, Bancewicz RM, Maule J, van Heyningen V
PLoS Genet 2007 Mar 30;3(3):e43. Epub 2007 Feb 8 doi: 10.1371/journal.pgen.0030043. PMID: 17397257Free PMC Article

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