Congenital Stromal Corneal Dystrophy

Also Known As:

Corneal Dystrophy, Congenital Stromal; Congenital Hereditary Stromal Dystrophy of the Cornea; Congenital Stromal Dystrophy of the Cornea; Decorin-Associated Congenital Stromal Corneal Dystrophy; Dystrophia Corneae Parenchymatosa Congenita

Networked: 12 relevant articles (0 outcomes, 1 trials/studies)

Disease Context: Research Results

Eye Diseases: 3522
- Corneal Diseases: 916
  - Hereditary Corneal Dystrophies: 820
    - Congenital Stromal Corneal Dystrophy: 12
- Hereditary Eye Diseases: 18
  - Hereditary Corneal Dystrophies: 820
    - Congenital Stromal Corneal Dystrophy: 12
Congenital, Hereditary, and Neonatal Diseases and Abnormalities: 933
- Inborn Genetic Diseases: 11939
  - Hereditary Eye Diseases: 18
    - Hereditary Corneal Dystrophies: 820
      - Congenital Stromal Corneal Dystrophy: 12

Related Diseases

1.	Ehlers-Danlos Syndrome (Syndrome, Ehlers-Danlos)

Experts

1.	Bredrup, Cecilie: 5 articles (01/2016 - 02/2005)
2.	Rødahl, Eyvind: 5 articles (01/2016 - 02/2005)
3.	Knappskog, Per M: 3 articles (11/2010 - 02/2005)
4.	Young, Robert D: 2 articles (01/2016 - 11/2010)
5.	Bruland, Ove: 2 articles (05/2015 - 11/2010)
6.	Birk, David E: 2 articles (07/2013 - 11/2011)
7.	Chen, Shoujun: 2 articles (07/2013 - 11/2011)
8.	Iozzo, Renato V: 2 articles (07/2013 - 11/2011)
9.	Kao, Winston W-Y: 2 articles (07/2013 - 11/2011)
10.	Sun, Mei: 2 articles (07/2013 - 11/2011)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Congenital Stromal Corneal Dystrophy:

1.	DecorinIBA 03/01/2014 - "The aim of this study was to characterize the congenital stromal corneal dystrophy (CSCD) pathological and clinical phenotype in a Chinese family with a novel mutation of decorin and its possible molecular pathogenesis. " 11/01/2023 - "We herein report 4 cases of congenital stromal corneal dystrophy in 2 families, highlighting the previously undescribed histopathologic features, the possible differential diagnosis of this entity and the key role played by decorin staining in its diagnosis." 04/01/2023 - "Congenital stromal corneal dystrophy (CSCD) is a rare congenital, dominantly inherited disorder characterized by diffuse stromal opacification associated with mutations in the decorin gene ( DCN ). " 01/01/2016 - "The role of Decorin in organising the extracellular matrix was examined in normal human corneas and in corneas from patients with Congenital Stromal Corneal Dystrophy (CSCD). " 05/01/2015 - "Development of congenital stromal corneal dystrophy is dependent on export and extracellular deposition of truncated decorin."
2.	Proteins (Proteins, Gene)FDA Link 03/27/2017 - "Defects in the core proteins of DS-PGs such as decorin and biglycan cause congenital stromal dystrophy of the cornea, spondyloepimetaphyseal dysplasia, and Meester-Loeys syndrome. " 01/01/2016 - "Role of Decorin Core Protein in Collagen Organisation in Congenital Stromal Corneal Dystrophy (CSCD)."
3.	Small Leucine-Rich ProteoglycansIBA 07/01/2013 - "Decorin, a small leucine-rich proteoglycan (SLRP), is involved in the pathophysiology of human congenital stromal corneal dystrophy (CSCD). " 11/01/2011 - "Pathophysiological mechanisms of autosomal dominant congenital stromal corneal dystrophy: C-terminal-truncated decorin results in abnormal matrix assembly and altered expression of small leucine-rich proteoglycans."
4.	BiglycanIBA 03/27/2017 - "Defects in the core proteins of DS-PGs such as decorin and biglycan cause congenital stromal dystrophy of the cornea, spondyloepimetaphyseal dysplasia, and Meester-Loeys syndrome. "
5.	GlycosaminoglycansIBA 10/01/2012 - "Mutations in decorin or alterations in the post-translational modifications of the glycosaminoglycan (GAG) chain lead to connective tissue disorders such as the congenital stromal corneal dystrophy and the Ehlers-Danlos syndrome. "
6.	CollagenIBA 01/01/2016 - "Role of Decorin Core Protein in Collagen Organisation in Congenital Stromal Corneal Dystrophy (CSCD)."