MCID: IRD003
MIFTS: 27

Iridocorneal Endothelial Syndrome

Categories: Rare diseases, Eye diseases, Fetal diseases

Aliases & Classifications for Iridocorneal Endothelial Syndrome

MalaCards integrated aliases for Iridocorneal Endothelial Syndrome:

Name: Iridocorneal Endothelial Syndrome 53 59
Ice Syndrome 53 59
Ichthyosis--Cheek--Eyebrow Syndrome 73

Characteristics:

Orphanet epidemiological data:

59
iridocorneal endothelial syndrome
Inheritance: Not applicable; Age of onset: Adult;

Classifications:



External Ids:

Orphanet 59 ORPHA64734
MESH via Orphanet 45 D057129
UMLS via Orphanet 74 C0339285 C1096100
ICD10 via Orphanet 34 H21.1
UMLS 73 C1840283

Summaries for Iridocorneal Endothelial Syndrome

NIH Rare Diseases : 53 Iridocorneal endothelial (ICE) syndrome describes a group of eye diseases that are characterized by three main features:  Visible changes in the iris (the colored part of the eye that regulates the amount of light entering the eye)  Swelling of the cornea, and  The development of glaucoma (a disease that can cause severe vision loss when normal fluid inside the eye cannot drain properly) ICE syndrome, is more common in women than men, most commonly diagnosed in middle age, and is usually present in only one eye. The condition is actually a grouping of three closely linked conditions: Cogan-Reese syndrome; Chandler's syndrome; and essential (progressive) iris atrophy. The cause of ICE syndrome is unknown, however there is a theory that it is triggered by a virus that leads to swelling of the cornea. While there is no way to stop the progression of the condition, treatment of the symptoms may include medication for glaucoma and corneal transplant for corneal swelling.

MalaCards based summary : Iridocorneal Endothelial Syndrome, also known as ice syndrome, is related to corneal endothelial dystrophy and endotheliitis. An important gene associated with Iridocorneal Endothelial Syndrome is KRT3 (Keratin 3). The drugs Ophthalmic Solutions and Pharmaceutical Solutions have been mentioned in the context of this disorder. Affiliated tissues include endothelial and eye.

Wikipedia : 76 Iridocorneal Endothelial (ICE) syndromes are a spectrum of diseases characteriezed by slowly progressive... more...

Related Diseases for Iridocorneal Endothelial Syndrome

Diseases related to Iridocorneal Endothelial Syndrome via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 14)
# Related Disease Score Top Affiliating Genes
1 corneal endothelial dystrophy 11.2
2 endotheliitis 10.9
3 melanoma 10.0
4 macular retinal edema 10.0
5 herpes simplex 10.0
6 macular dystrophy, dominant cystoid 9.9
7 corneal dystrophy, band-shaped 9.9
8 keratoconus 9.9
9 keratopathy 9.9
10 corneal edema 9.9
11 axenfeld-rieger syndrome 9.9
12 retinitis 9.9
13 glaucomatocyclitic crisis 9.9
14 posner-schlossman syndrome 9.9

Graphical network of the top 20 diseases related to Iridocorneal Endothelial Syndrome:



Diseases related to Iridocorneal Endothelial Syndrome

Symptoms & Phenotypes for Iridocorneal Endothelial Syndrome

Drugs & Therapeutics for Iridocorneal Endothelial Syndrome

Drugs for Iridocorneal Endothelial Syndrome (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):


# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Ophthalmic Solutions Phase 1, Phase 2
2 Pharmaceutical Solutions Phase 1, Phase 2

Interventional clinical trials:

(show all 12)
# Name Status NCT ID Phase Drugs
1 Evaluating Results of Neusidl Corneal Inserter in Descemet's Stripping Automated Endothelial Keratoplasty (DSAEK) Unknown status NCT01357122 Phase 4
2 Ripasudil 0.4% Eye Drops in Fuchs Endothelial Corneal Dystrophy Recruiting NCT03575130 Phase 2 Ripasudil eye drops;Optive eye drops
3 A Study Investigating the Safety, Tolerability, and Efficacy of Elamipretide Topical Ophthalmic Solution for the Treatment of Fuchs' Corneal Endothelial Dystrophy (FCED) Active, not recruiting NCT02653391 Phase 1, Phase 2 Elamipretide 1.0% Ophthalmic Solution;Elamipretide 3.0% Ophthalmic Solution
4 Outcome After Descemet Membrane Endothelial Keratoplasty (DMEK) and Ultra-thin Descemet Stripping Automated Endothelial Keratoplasty (DSAEK) Unknown status NCT02020044 Not Applicable
5 Early Experience With Descemet's Stripping Automated Endothelial Keratoplasty Unknown status NCT00744796 Not Applicable
6 Abnormalities of the Eye's Anterior Chamber, Iris, Cornea and Lens Completed NCT00001161
7 Fuchs' Endothelial Dystrophy: Clinical Characteristics, Treatment Outcome, and Pathology Completed NCT01979250 Not Applicable
8 The Molecular Pathogenesis of Late-onset Fuchs' Endothelial Corneal Dystrophy Completed NCT01795001
9 Zhongshan Iridocorneal Endothelial Syndrome Study (ICEs) Recruiting NCT03270761
10 Study of Endothelial Keratoplasty Outcomes Recruiting NCT00800111 Not Applicable
11 Assessment of Corneal Graft Attachment in Patients With Fuchs Endothelial Corneal Dystrophy Following DMEK Using Ultra-high Resolution OCT Recruiting NCT02542644 Not Applicable
12 Specular Microscopy Study Withdrawn NCT02478970 Not Applicable

Search NIH Clinical Center for Iridocorneal Endothelial Syndrome

Genetic Tests for Iridocorneal Endothelial Syndrome

Anatomical Context for Iridocorneal Endothelial Syndrome

MalaCards organs/tissues related to Iridocorneal Endothelial Syndrome:

41
Endothelial, Eye

Publications for Iridocorneal Endothelial Syndrome

Articles related to Iridocorneal Endothelial Syndrome:

(show top 50) (show all 95)
# Title Authors Year
1
Atypical Presentation of Iridocorneal Endothelial Syndrome With Band Keratopathy but No Corneal Edema Managed With Descemet Membrane Endothelial Keratoplasty. ( 29668572 )
2018
2
Iridocorneal endothelial syndrome: clinical perspectives. ( 29670326 )
2018
3
The Iridocorneal Endothelial Syndrome. ( 29331589 )
2018
4
Clinical outcome of Descemet stripping automated endothelial keratoplasty in 18 cases with iridocorneal endothelial syndrome. ( 29243737 )
2017
5
Glaucoma associated with iridocorneal endothelial syndrome in 203 Indian subjects. ( 28282413 )
2017
6
Unique variations and characteristics of iridocorneal endothelial syndrome in China: a case series of 58 patients. ( 28993974 )
2017
7
Trabeculectomy with Mitomycin-C in Patients with Iridocorneal Endothelial Syndrome: A Case Series. ( 27437260 )
2016
8
Iridocorneal Endothelial Syndrome Presenting With Large Diurnal Intraocular Pressure Fluctuation. ( 27977477 )
2016
9
Post Filtering Surgery Globe Massage-induced Keratoconus in an Eye with Iridocorneal Endothelial Syndrome: A Case Report and Literature Brief Review. ( 27621792 )
2016
10
Cystoid macular edema associated with iridocorneal endothelial syndrome: a case report. ( 27585567 )
2016
11
Surgical outcomes of patients with iridocorneal endothelial syndrome: a case series. ( 27495952 )
2016
12
Concurrent Acute Retinal Necrosis in a Patient With Iridocorneal Endothelial Syndrome. ( 27362881 )
2016
13
Iridocorneal Endothelial Syndrome in a 14-Year-Old Male. ( 26035422 )
2015
14
Graft Failure and Intraocular Pressure Control After Keratoplasty in Iridocorneal Endothelial Syndrome. ( 26525380 )
2015
15
Descemet Stripping Endothelial Keratoplasty in Iridocorneal Endothelial Syndrome: Postoperative Complications and Long-Term Outcomes. ( 26203744 )
2015
16
Diagnosis and Management of Iridocorneal Endothelial Syndrome. ( 26451377 )
2015
17
Graft failure and intraocular pressure control after keratoplasty in iridocorneal endothelial syndrome. ( 26032193 )
2015
18
Graft Failure and Intraocular Pressure Control After Keratoplasty in Iridocorneal Endothelial Syndrome. ( 26514395 )
2015
19
Outcomes of Primary Trabeculectomy With Mitomycin-C in Glaucoma Secondary to Iridocorneal Endothelial Syndrome. ( 25943731 )
2015
20
Iridocorneal endothelial syndrome. ( 24906259 )
2014
21
Concurrence of iridocorneal endothelial syndrome in a patient with glaucomatocyclitic crisis. ( 24790889 )
2014
22
Iridocorneal endothelial syndrome. ( 24407828 )
2014
23
Phenotypic heterogeneity of corneal endothelium in iridocorneal endothelial syndrome by in vivo confocal microscopy. ( 24727634 )
2014
24
Intracameral injection of tissue plasminogen activator to treat severe postoperative fibrinous reaction in iridocorneal endothelial syndrome. ( 24109245 )
2013
25
Retrocorneal membrane formation after Baerveldt shunt implantation for iridocorneal endothelial syndrome. ( 23538633 )
2013
26
Iridocorneal endothelial syndrome in a Chinese child. ( 24579558 )
2013
27
Management of detached graft donor by SFa88 injection following Descemet stripping automated endothelial keratoplasty of an eye with iridocorneal endothelial syndrome and Ahmed glaucoma drainage tube. ( 22752650 )
2012
28
Clinicopathologic findings in iridocorneal endothelial syndrome and posterior polymorphous membranous dystrophy after Descemet stripping automated endothelial keratoplasty. ( 22333668 )
2012
29
Iridocorneal endothelial syndrome in a 16-year-old. ( 20616751 )
2011
30
Iridocorneal endothelial syndrome masquerading as iris melanoma in 71 cases. ( 21482858 )
2011
31
Bilateral iridocorneal endothelial syndrome in a young girl with Down's syndrome. ( 19075416 )
2009
32
[Ultrasound biomicroscopy in the monitoring of iridocorneal endothelial syndrome]. ( 19566045 )
2009
33
Deep lamellar endothelial keratoplasty for iridocorneal endothelial syndrome in phakic eyes. ( 19139335 )
2009
34
In-vivo confocal microscopy of iridocorneal endothelial syndrome. ( 18297250 )
2009
35
[Morphological changes of cornea in iridocorneal endothelial syndrome under the confocal microscopy]. ( 19176093 )
2008
36
Descemet's stripping with endothelial keratoplasty in iridocorneal endothelial syndrome. ( 18254352 )
2008
37
Coexistence of unilateral iridocorneal endothelial syndrome and sensorineural deafness. ( 17641715 )
2007
38
Descemet stripping with endothelial keratoplasty for treatment of iridocorneal endothelial syndrome. ( 17457204 )
2007
39
Iridocorneal endothelial syndrome in a child. ( 17022165 )
2006
40
Ultrasound biomicroscopy of Chinese eyes with iridocorneal endothelial syndrome. ( 16361670 )
2006
41
Reduced corneal swelling and deswelling response in the iridocorneal endothelial syndrome. ( 17041320 )
2006
42
Macular edema and iridocorneal endothelial syndrome: a case report. ( 15725892 )
2005
43
Features of the iridocorneal endothelial syndrome on confocal microscopy. ( 15778613 )
2005
44
Iris reconstruction with a multipiece endocapsular prosthesis in iridocorneal endothelial syndrome. ( 16412914 )
2005
45
In vivo confocal microscopic characteristics of iridocorneal endothelial syndrome. ( 15180840 )
2004
46
Bilaminar interepithelial bodies within fingerprint dystrophy-like changes in bilateral iridocorneal endothelial syndrome. ( 12642328 )
2003
47
Penetrating keratoplasty in iridocorneal endothelial syndrome. ( 11248814 )
2001
48
Clinical and specular microscopic manifestations of iridocorneal endothelial syndrome. ( 11369379 )
2001
49
Posterior polymorphous membranous dystrophy with overlapping features of iridocorneal endothelial syndrome. ( 11296040 )
2001
50
[Iridocorneal endothelial syndrome. Series of 7 cases]. ( 11460060 )
2001

Variations for Iridocorneal Endothelial Syndrome

Expression for Iridocorneal Endothelial Syndrome

Search GEO for disease gene expression data for Iridocorneal Endothelial Syndrome.

Pathways for Iridocorneal Endothelial Syndrome

GO Terms for Iridocorneal Endothelial Syndrome

Sources for Iridocorneal Endothelial Syndrome

3 CDC
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11 DGIdb
17 ExPASy
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28 GO
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32 HPO
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45 MESH via Orphanet
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54 NINDS
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62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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