Retinal Dysplasia

Congenital, often bilateral, retinal abnormality characterized by the arrangement of outer nuclear retinal cells in a palisading or radiating pattern surrounding a central ocular space. This disorder is sometimes hereditary.

Also Known As:

Networked: 77 relevant articles (0 outcomes, 1 trials/studies)

Disease Context: Research Results

Related Diseases

1.	Cataract (Cataracts)
2.	Retinal Detachment (Retinal Detachments)
3.	Microphthalmos
4.	Retinal Degeneration
5.	Cysts

Experts

1.	Forsyth, George W: 3 articles (04/2011 - 05/2006)
2.	Grahn, Bruce H: 3 articles (04/2011 - 05/2006)
3.	Nishina, Patsy M: 2 articles (01/2014 - 03/2008)
4.	Bauer, Bianca S: 2 articles (04/2011 - 04/2010)
5.	Sandmeyer, Lynne S: 2 articles (04/2011 - 04/2010)
6.	Nishina, P M: 2 articles (08/2001 - 05/2000)
7.	Capasso, Jenina E: 1 article (06/2015)
8.	Hope, William C: 1 article (06/2015)
9.	Levin, Alex V: 1 article (06/2015)
10.	Lall-Trail, Joel: 1 article (06/2015)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Retinal Dysplasia:

1.	short-wavelength opsinIBA 12/01/2005 - "Earlier studies have shown that rd7 mice have a distinctive pattern of retinal dysplasia with an increased number of cone cells, particularly those with S (short wavelength)-opsin immunoreactivity. " 03/01/2008 - "The retinal degeneration 7 (rd7) mouse, lacking expression of the Nr2e3 gene, exhibits retinal dysplasia and a slow, progressive degeneration due to an abnormal production of blue opsin-expressing cone cells. "
2.	Retinaldehyde (Retinal)IBA 01/01/2014 - "Unlike C57BL/6NJ mice, which exhibited external limiting membrane fragmentation and regional retinal dysplasia, heterozygous Crb1(em1Mvw)/Crb1(rd8) mice showed a normal retinal phenotype. " 01/01/2012 - "Raman microscopy, a rapid nondestructive technique that profiles the composition of biological samples, was used to characterize retinal biochemistry in the retinal dysplasia and degeneration (rdd) and wild-type (wt) chick retina during retinogenesis and at hatching. " 01/01/2012 - "Profiling retinal biochemistry in the MPDZ mutant retinal dysplasia and degeneration chick: a model of human RP and LCA." 01/01/2011 - "Juvenile ptc2-/- mutants exhibited peripheral retinal dysplasias that included the presence of ectopic neuronal clusters in the inner nuclear layer (INL) and regions of disrupted retinal lamination. " 01/01/2008 - "Before the onset of severe retinal dysplasia, Pax6 overexpression causes defects of retinal axons, preventing their normal growth and navigation through the optic chiasm."
3.	Norrie diseaseIBA 08/01/1993 - "Our results map the gene responsible for the disorder between the MAOB and DXS426, m27 beta and p58-1 loci, on the short arm of the X chromosome at Xp11.3, which suggest the possibility that the same gene is responsible for both primary retinal dysplasia and Norrie disease." 08/01/1993 - "X-linked recessive primary retinal dysplasia is linked to the Norrie disease locus." 09/01/1985 - "The differential diagnosis of leukocoria (pseudoglioma) in the neonate includes multiple conditions, including malformations with retinal dysplasia as a component. " 11/01/1969 - "[Hyaloid-retinal dysplasia (pseudoglioma) due to recessive autosomal heredity]." 01/01/1997 - "Due to ophthalmological and audio-otological findings, including bilateral retinal dysplasia and detachment, progressive bilateral sensorineural hearing loss, and an X-linked pattern of inheritance, a tentative diagnosis of Norrie disease was considered. "
4.	Patau syndromeIBA 01/01/2007 - "Here, we describe the ocular findings in a case of trisomy 13 to better understand the histogenesis of the rosettes, or tubules, characteristic of the retinal dysplasia associated with this condition. " 01/01/2007 - "Histogenesis of retinal dysplasia in trisomy 13." 01/01/1981 - "In Trisomy 13 there is a severe retinal dysplasia, and in Bloom's syndrome excrescences on Bruch's membrane have been described. " 01/01/1991 - "Pathologic examination of the eyes revealed typical features of trisomy 13, including microphthalmos, extensive colobomata, and retinal dysplasia. " 10/01/1977 - "The patient in this report had many of the classic neuropathologic stigmata of trisomy 13, including retinal dysplasia, arrhinencephaly, holoprosencephaly, single external nare and granular cell heterotopias in the cerebellum and microthalmia. "
5.	Peters anomalyIBA 06/01/2015 - "We report the first child with cri-du-chat and the findings of unilateral corneal staphyloma due to Peters anomaly and retinal dysplasia. " 07/15/1992 - "Both patients have additional ocular anomalies, including microphthalmia, retinal dysplasia, and Peters anomaly. " 06/01/2004 - "All parts of the eye may be affected and anomalies such as microphthalmus, microcornea, Peters' anomaly, cataract, persistent hyperplastic primary hyaloid vitreous body, coloboma of the iris and choroid, retinal dysplasia and, most commonly, optic nerve hypoplasia and tortuosity of the retinal vessels, have been reported. "
6.	Mitochondrial DNA (mtDNA)IBA 04/01/2010 - "No significant differences were detected in relative amounts of WBC mtDNA or the size, number, or morphology of lymphocyte mitochondria in English Springer Spaniels affected with retinal dysplasia, compared with results for unaffected control dogs." 04/01/2010 - "To compare relative amounts of WBC mitochondrial DNA (mtDNA; assessed via real-time PCR assay) and morphology of lymphocyte mitochondria (assessed via transmission electron microscopy [TEM]) in blood samples collected from English Springer Spaniels with and without retinal dysplasia. " 04/01/2010 - "Relative quantification of white blood cell mitochondrial DNA and assessment of mitochondria by use of transmission electron microscopy in English Springer Spaniels with and without retinal dysplasia." 05/01/2006 - "Differential mitochondrial DNA and gene expression in inherited retinal dysplasia in miniature Schnauzer dogs."
7.	Tumor Viral Antigens (Large T Antigen)IBA 01/01/1991 - "Our results indicate that large T antigen seems to play an important role in cataract formation but not in the pathogenesis of retinal dysplasia." 07/01/1990 - "Transgenic mice expressing the large T-antigen of the simian virus 40 (SV 40) under the control of 1) the enhancer of Moloney murine sarcoma virus (MSV) and 2) the SV 40 promoter develop undifferentiated neuroectodermal tumors located in the midline of the dorsal brain surface, abnormalities in lens fiber differentiation and retinal dysplasia. "
8.	5-HT2B Serotonin ReceptorIBA 12/09/2008 - "Overexpression of 5-HT2B receptor results in retinal dysplasia and defective ocular morphogenesis in Xenopus embryos."
9.	Vascular Endothelial Growth Factor A (Vascular Endothelial Growth Factor)IBA 12/01/2011 - "Unilateral sporadic retinal dysplasia: results of histopathologic, immunohistochemical, chromosomal, genetic, and VEGF-A analyses."
10.	Activated-Leukocyte Cell Adhesion Molecule (Neurolin)IBA 09/01/2004 - "Axon fasciculation defects and retinal dysplasias in mice lacking the immunoglobulin superfamily adhesion molecule BEN/ALCAM/SC1."

Therapies and Procedures

1.	Traction 03/01/1994 - "Clinical and/or histologic abnormalities were found in all seven eyes exposed to light in the presence of rose bengal, as follows: vitreous hemorrhage in four eyes, incomplete peripheral retinal vascularization in four eyes, fibrovascular/fibrocellular proliferation with traction on the retina in two eyes (including preretinal neovascularization in one eye), total or partial retinal detachment in two eyes, dilated peripheral vessels compatible with shunt vessels in one eye, retinal dysplasia with loss of normal architecture and formation of rosettes in five eyes. "
2.	Lenses 11/01/2006 - "The ocular lesions were pathologically similar to each other, except for the left eye of cow No. 1. The ocular lesions of 5 globes were characterized by microphthalmia, hypoplasia, and/or dysplasia of the lenses; persistence of the primary vitreous; and retinal dysplasia with total nonattachment. "