MCID: IRD003
MIFTS: 26

Iridocorneal Endothelial Syndrome

Categories: Eye diseases, Fetal diseases, Rare diseases

Aliases & Classifications for Iridocorneal Endothelial Syndrome

MalaCards integrated aliases for Iridocorneal Endothelial Syndrome:

Name: Iridocorneal Endothelial Syndrome 52 58
Ice Syndrome 52 58
Ichthyosis--Cheek--Eyebrow Syndrome 71

Characteristics:

Orphanet epidemiological data:

58
iridocorneal endothelial syndrome
Inheritance: Not applicable; Age of onset: Adult;

Classifications:

Orphanet: 58  
Rare eye diseases
Developmental anomalies during embryogenesis


External Ids:

MESH via Orphanet 44 D057129
ICD10 via Orphanet 33 H21.1
UMLS via Orphanet 72 C0339285 C1096100
Orphanet 58 ORPHA64734
UMLS 71 C1840283

Summaries for Iridocorneal Endothelial Syndrome

NIH Rare Diseases : 52 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 corneal edema. An important gene associated with Iridocorneal Endothelial Syndrome is KRT3 (Keratin 3). The drugs Pharmaceutical Solutions and Ophthalmic Solutions have been mentioned in the context of this disorder. Affiliated tissues include endothelial, eye and retina.

Wikipedia : 74 Iridocorneal Endothelial (ICE) syndromes are a spectrum of diseases characterized 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 49)
# Related Disease Score Top Affiliating Genes
1 corneal endothelial dystrophy 11.8
2 corneal edema 10.7
3 essential iris atrophy 10.7
4 cogan-reese syndrome 10.6
5 ectropion 10.4
6 intraocular pressure quantitative trait locus 10.4
7 keratopathy 10.4
8 bullous keratopathy 10.4
9 hydrops, lactic acidosis, and sideroblastic anemia 10.3
10 corneal disease 10.3
11 keratoconus 10.3
12 herpes simplex 10.3
13 corneal dystrophy, posterior polymorphous, 1 10.2
14 glaucoma, primary open angle 10.2
15 astigmatism 10.2
16 open-angle glaucoma 10.2
17 axenfeld-rieger syndrome 10.2
18 melanoma 10.2
19 macular retinal edema 10.2
20 cataract 10.2
21 ocular hypertension 10.2
22 scotoma 10.2
23 ichthyosis--cheek--eyebrow syndrome 10.0
24 keratitis, hereditary 10.0
25 corneal dystrophy, band-shaped 10.0
26 3-methylglutaconic aciduria, type iii 10.0
27 branchiootic syndrome 1 10.0
28 pars planitis 10.0
29 secondary corneal edema 10.0
30 fuchs' endothelial dystrophy 10.0
31 uveitis 10.0
32 primary angle-closure glaucoma 10.0
33 iritis 10.0
34 vascular disease 10.0
35 optic nerve disease 10.0
36 corneal dystrophy 10.0
37 malignant iris melanoma 10.0
38 hyperthyroidism 10.0
39 herpes zoster 10.0
40 measles 10.0
41 glaucomatocyclitic crisis 10.0
42 47,xyy 10.0
43 adamtsl4-related eye disorders 10.0
44 farsightedness 10.0
45 overgrowth syndrome 10.0
46 ichthyosis 9.8
47 aniridia 1 9.7
48 ichthyosis vulgaris 9.7
49 juvenile glaucoma 9.7

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 Pharmaceutical Solutions Phase 1, Phase 2
2 Ophthalmic Solutions Phase 1, Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 PART A: A PROSPECTIVE, RANDOMIZED, DOUBLE-MASKED, VEHICLE CONTROLLED, PAIRED-EYE PHASE 1/2 CLINICAL STUDY TO EVALUATE THE SAFETY, TOLERABILITY AND EFFICACY OF ELAMIPRETIDE TOPICAL OPHTHALMIC SOLUTION IN SUBJECTS WITH FCED PRESENTING WITH MILD TO MODERATE CORNEAL EDEMA PART B: A PROSPECTIVE, RANDOMIZED, DOUBLE-MASKED, VEHICLE CONTROLLED, PHASE 1/2 CLINICAL STUDY TO EVALUATE THE SAFETY, TOLERABILITY, AND EFFICACY OF ELAMIPRETIDE TOPICAL OPHTHALMIC SOLUTION IN SUBJECTS WITH FCED PRESENTING WITH MILD TO MODERATE CORNEAL EDEMA Completed NCT02653391 Phase 1, Phase 2 Elamipretide 1.0% Ophthalmic Solution;Elamipretide 3.0% Ophthalmic Solution
2 Prospective Clinical Study on Postoperative Outcome After Descemet Membrane Endothelial Keratoplasty (DMEK) and Ultra-thin Descemet Stripping Automated Endothelial Keratoplasty (DSAEK) Unknown status NCT02020044
3 Anomalies of the Anterior Chamber, Angle, Iris, Cornea and Lens With or Without Glaucoma or Ocular Hypertension Completed NCT00001161
4 Open-enrollment, Prospective Study of Endothelial Keratoplasty Outcomes Completed NCT00800111
5 A Multi-center Study on Iridocorneal Endothelial (ICE) Syndrome in China Recruiting NCT03270761
6 Heidelberg Retina Tomograph (HRT) in Vivo Confocal Microscopy to Evaluate the Ocular Surface Disorders of Healthy and Diseased Individuals Not yet recruiting NCT04025801

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:

40
Endothelial, Eye, Retina, Testes

Publications for Iridocorneal Endothelial Syndrome

Articles related to Iridocorneal Endothelial Syndrome:

(show top 50) (show all 203)
# Title Authors PMID Year
1
Graft Survival After Penetrating and Endothelial Keratoplasty in Iridocorneal Endothelial Syndrome. 61
31335531 2020
2
Ex-PRESS mini-shunt implanted in a pregnant patient with iridocorneal endothelial syndrome. 61
30618278 2020
3
XEN Gel Stent to Treat ICE Syndrome: 4 Cases. 61
31425336 2019
4
Iridocorneal endothelial syndrome in a patient with keratoconus - a case report. 61
31711443 2019
5
Iridocorneal endothelial syndrome: Evaluation of patient demographics and endothelial morphology by in vivo confocal microscopy in an Indian cohort. 61
31007217 2019
6
Commentary: In vivo confocal microscopy in iridocorneal endothelial syndrome. 61
31007218 2019
7
Case Report: Iridocorneal Endothelial Syndrome Progression Documented by Anterior Segment Optical Coherence Tomography. 61
30907858 2019
8
Descemet membrane endothelial keratoplasty in iridocorneal endothelial syndrome and posterior polymorphous corneal dystrophy. 61
30975342 2019
9
Cogan-Reese syndrome: image analysis with specular microscopy, optical coherence tomography, and ultrasound biomicroscopy. 61
31327934 2019
10
Primary implantation of non-valved glaucoma-drainage-device in sulcus in iridocorneal endothelial syndrome. 61
31741874 2019
11
Descemet membrane endothelial keratoplasty for treatment of iridocorneal endothelial syndrome. 61
30502999 2018
12
Unique variations and characteristics of iridocorneal endothelial syndrome in China: a case series of 58 patients. 61
28993974 2018
13
The iridocorneal endothelial syndrome. 61
29331589 2018
14
Atypical Presentation of Iridocorneal Endothelial Syndrome With Band Keratopathy but No Corneal Edema Managed With Descemet Membrane Endothelial Keratoplasty. 61
29668572 2018
15
Clinical outcome of Descemet stripping automated endothelial keratoplasty in 18 cases with iridocorneal endothelial syndrome. 61
29243737 2018
16
[Differential diagnosis and treatment of Chandler iridocorneal endothelial syndrome]. 61
29543203 2018
17
Unclassified Axenfeld-Rieger Syndrome: A CASE SERIES and Review of Literature. 61
27929720 2018
18
Iridocorneal endothelial syndrome: clinical perspectives. 61
29670326 2018
19
Etiological mechanism of iridocorneal endothelial (ICE) syndrome may involve infection of herpes simplex virus (HSV) and integration of viral genes into human genome. 61
29317068 2018
20
Retinoinvasive Uveal Melanoma: Report of 2 Cases and Review of the Literature. 61
29344483 2017
21
Surgical outcomes of patients with iridocorneal endothelial syndrome: a case series. 61
27495952 2017
22
ICE-Syndrome: A Case Report of Implantation of a Microbypass Xen Gel Stent After DMEK Transplantation. 61
27841796 2017
23
Iridocorneal Endothelial Syndrome Presenting With Large Diurnal Intraocular Pressure Fluctuation. 61
27977477 2017
24
Glaucoma associated with iridocorneal endothelial syndrome in 203 Indian subjects. 61
28282413 2017
25
[Diagnostic methods in the clinical evaluation of iridocorneal endothelial syndrome]. 61
27023272 2016
26
Concurrent Acute Retinal Necrosis in a Patient With Iridocorneal Endothelial Syndrome. 61
27362881 2016
27
Cystoid macular edema associated with iridocorneal endothelial syndrome: a case report. 61
27585567 2016
28
Post Filtering Surgery Globe Massage-induced Keratoconus in an Eye with Iridocorneal Endothelial Syndrome: A Case Report and Literature Brief Review. 61
27621792 2016
29
Outcomes of Primary Trabeculectomy With Mitomycin-C in Glaucoma Secondary to Iridocorneal Endothelial Syndrome. 61
25943731 2016
30
Trabeculectomy with Mitomycin-C in Patients with Iridocorneal Endothelial Syndrome: A Case Series. 61
27437260 2016
31
Iridocorneal Endothelial Syndrome in a 14-Year-Old Male. 61
26035422 2016
32
A review of the role of ultrasound biomicroscopy in glaucoma associated with rare diseases of the anterior segment. 61
27536058 2016
33
Graft Failure and Intraocular Pressure Control After Keratoplasty in Iridocorneal Endothelial Syndrome. 61
26514395 2016
34
Boston Type 1 Keratoprosthesis for Iridocorneal Endothelial Syndromes. 61
26398156 2015
35
Descemet Stripping Endothelial Keratoplasty in Iridocorneal Endothelial Syndrome: Postoperative Complications and Long-Term Outcomes. 61
26203744 2015
36
Graft failure and intraocular pressure control after keratoplasty in iridocorneal endothelial syndrome. 61
26032193 2015
37
ICE SYNDROME--CASE REPORT. 61
26978875 2015
38
Retinitis pigmentosa with concomitant essential iris atrophy and glaucoma - case report. 61
26648683 2015
39
Diagnosis and Management of Iridocorneal Endothelial Syndrome. 61
26451377 2015
40
Evolving indications for Descemet's stripping automated endothelial keratoplasty. 61
24871355 2014
41
Endothelial Keratoplasty: A Review of Indications at a Tertiary Eye Care Centre in South India. 61
26107758 2014
42
Phenotypic heterogeneity of corneal endothelium in iridocorneal endothelial syndrome by in vivo confocal microscopy. 61
24727634 2014
43
Combined endothelial keratoplasty and clear lens extraction for corneal decompensation in irido-corneal endothelial syndrome. 61
24088632 2014
44
Iridocorneal endothelial syndrome. 61
24906259 2014
45
[Post operation complications analysis and their treatment of Descemet's stripping automated endothelium keratoplasty]. 61
24931150 2014
46
Concurrence of iridocorneal endothelial syndrome in a patient with glaucomatocyclitic crisis. 61
24790889 2014
47
Iridocorneal endothelial syndrome. 61
24407828 2014
48
Persistent leak after glaucoma aqueous shunt implantation. 61
22576169 2013
49
Iridocorneal endothelial syndrome in a Chinese child. 61
24579558 2013
50
Coincidence of Varicella-Zoster Virus Anterior Uveitis in a Patient with Chandler's Syndrome. 61
24474927 2013

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
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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