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: 78 relevant articles (0 outcomes, 3 trials/studies)

Disease Context: Research Results

Related Diseases

1.	Retinal Diseases
2.	Inborn Genetic Diseases (Disease, Hereditary)
3.	Cataract (Cataracts)
4.	Retinal Detachment (Retinal Detachments)
5.	Atrophy

Experts

1.	Grahn, Bruce H: 5 articles (11/2020 - 05/2006)
2.	Sandmeyer, Lynne S: 3 articles (11/2020 - 04/2010)
3.	Bauer, Bianca S: 3 articles (07/2016 - 04/2010)
4.	Forsyth, George W: 3 articles (04/2011 - 05/2006)
5.	Aguirre, Gustavo D: 2 articles (05/2022 - 03/2020)
6.	Beltran, William A: 2 articles (05/2022 - 03/2020)
7.	Cohen, Julie A: 2 articles (05/2022 - 03/2020)
8.	Holle, Dolores M: 2 articles (05/2022 - 03/2020)
9.	Nishina, Patsy M: 2 articles (01/2014 - 03/2008)
10.	Nishina, P M: 2 articles (08/2001 - 05/2000)

Drugs and Biologics

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

1.	Retinaldehyde (Retinal)IBA 05/01/2022 - "Retinal structural and microvascular abnormalities in retinal dysplasia imaged by OCT and OCT angiography." 11/01/2020 - "Circular plaques observed in the tapetal retina are predominantly formed by a thickening of inner retina, while retinal dysplasia has disorganization of both inner and outer retinal layers. " 03/01/2020 - "Retinal folds are the common feature of acquired focal/multifocal or geographic retinal dysplasia, are indistinguishable structurally from those associated with syndromic oculoskeletal dysplasia, and represent outer nuclear layer invaginations and rosettes visible by sdOCT. " 03/01/2020 - "To examine the in vivo microanatomy of retinal folds and geographic lesions in dogs with acquired or inherited retinal dysplasia. " 03/01/2020 - "Focal/multifocal and geographic retinal dysplasia in the dog-In vivo retinal microanatomy analyses."
2.	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. "
3.	Transcription Factors (Transcription Factor)IBA 01/01/2021 - "Our work indicated that inhibition of Sox2 could change cell fate by affecting transcription factors in the development of bipolar cells and amacrine cells, may provide new directions for the study and treatment of retinal genetic diseases and retinal dysplasia."
4.	Capsules (Microcapsules)IBA 12/01/2000 - "Reported ocular features include microphthalmus, "cloudy cornea", irregularities of Bowman's layer, thickened posterior lens capsule and retinal dysplasia. " 01/01/2018 - "Common ocular findings in the C57BL/6N strain included corneal deposits (3%), increased optical density of the anterior lens capsule (67%), punctate nuclear cataracts (98%), vitreous crystalline deposits (61%), hyaloid vascular remnant (6%), and retinal dysplasia attributed to the rd8 mutation (58%). " 10/01/2004 - "The mice had microphthalmia; fibrovascular, retrolental tissue containing retinal pigment epithelial cells and remnants of the hyaloid vascular system; posterior lens capsule destruction with lens degeneration and opacity; and severe retinal dysplasia and detachment. "
5.	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." 01/01/2017 - "Pathway analyses suggested a decrease in cellular respiration and increased expression of components of Hif1-α, VEGF, mTOR, NFκβ, and multiple interleukin pathways are associated with early retinal dysplasia. " 01/01/2017 - "Vascular Endothelial Growth Factor A and Leptin Expression Associated with Ectopic Proliferation and Retinal Dysplasia in Zebrafish Optic Pathway Tumors."
6.	Retinal Pigments (Pigments, Visual)IBA 10/01/2004 - "The mice had microphthalmia; fibrovascular, retrolental tissue containing retinal pigment epithelial cells and remnants of the hyaloid vascular system; posterior lens capsule destruction with lens degeneration and opacity; and severe retinal dysplasia and detachment. " 11/01/1994 - "Forty-four retinoblastoma and one retinal dysplasia specimens were analyzed by indirect immunohistochemistry, using specific antibodies against glial fibrillary acidic protein, S-100 protein, myelin basic protein, neuron-specific enolase, neurofilament, retinal S-antigen and retinal pigment epithelial antigen. "
7.	Proteins (Proteins, Gene)FDA Link 04/01/2001 - "This decrease of the amount of QN1 protein results in additional mitosis and in severe abnormalities such as retinal dysplasia. " 05/01/2009 - "In mammals, the effect of SkQs on aging was accompanied by inhibition of development of such age-related diseases and traits as cataract, retinopathy, glaucoma, balding, canities, osteoporosis, involution of the thymus, hypothermia, torpor, peroxidation of lipids and proteins, etc. SkQ1 manifested a strong therapeutic action on some already pronounced retinopathies, in particular, congenital retinal dysplasia. "
8.	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."
9.	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. "
10.	Microphthalmia-Associated Transcription FactorIBA 12/03/2018 - "Complete deficiency of microphthalmia transcription factor (MITF) in Mitfmi-vga9/mi-vga9 mice is associated with microphthalmia, retinal dysplasia, and albinism. "

Therapies and Procedures

1.	General Anesthesia 03/01/2020 - "Thirteen dogs had retinal microanatomy evaluation under general anesthesia using cSLO/sdOCT; two eyes had noninherited multifocal retinal folds, five had inherited multifocal retinal folds (drd1 or drd2), and 10 geographic retinal dysplasia. " 05/01/2022 - "Two dogs were diagnosed with unilateral multifocal or geographic retinal dysplasia and underwent advanced retinal imaging under general anesthesia at the Retinal Disease Studies Facility of the University of Pennsylvania. "
2.	Lasers (Laser) 05/01/2022 - "To describe the in vivo structural characteristics of multifocal and geographic retinal dysplasia visualized with advanced retinal imaging including confocal scanning laser ophthalmoscopy (cSLO), optical coherence tomography (OCT), en face OCT, and the novel vascular imaging technique OCT angiography (OCTA). " 11/01/2020 - "To compare the scanning laser ophthalmoscopy (SLO), optical coherence tomography (OCT), and fluorescein angiography (FA) findings in retrievers with a single unilateral circular retinal plaque to those of an English springer spaniel with bilateral retinal dysplasia. "
3.	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. "
4.	Therapeutics 06/01/1993 - "Radiation dose dependent cerebellar and retinal dysplasia and long bone growth retardation was observed when the therapy was initiated in newborn mice but not when the animals were transplanted as adults. "
5.	Sutures (Suture) 03/01/2017 - "The prevalence of congenital cataract (CC), distichiasis (DIST), hereditary cataract (HC), persistent pupillary membranes (PPMs), persistent hyperplastic tunica vasculosa lentis / persistent hyperplastic primary vitreous (PHTVL/PHPV), progressive retinal atrophy (PRA), retinal dysplasia (RD), and findings such as fiberglass-like cataract (FGC) and prominent suture lines (PSLs) was analyzed. "