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 20 58
Ice Syndrome 20 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 45 D057129
ICD10 via Orphanet 33 H21.1
UMLS via Orphanet 72 C0339285 C1096100
Orphanet 58 ORPHA64734
UMLS 71 C1840283

Summaries for Iridocorneal Endothelial Syndrome

GARD : 20 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 Acetylcysteine and Cysteine have been mentioned in the context of this disorder. Affiliated tissues include endothelial and eye.

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 44)
# Related Disease Score Top Affiliating Genes
1 corneal endothelial dystrophy 11.3
2 corneal edema 10.8
3 essential iris atrophy 10.7
4 cogan-reese syndrome 10.6
5 intraocular pressure quantitative trait locus 10.5
6 ectropion 10.4
7 keratopathy 10.4
8 bullous keratopathy 10.3
9 corneal dystrophy, fuchs endothelial, 1 10.2
10 corneal disease 10.2
11 keratoconus 10.2
12 fuchs' endothelial dystrophy 10.2
13 herpes simplex 10.2
14 corneal dystrophy, posterior polymorphous, 1 10.2
15 glaucoma, primary open angle 10.2
16 astigmatism 10.2
17 open-angle glaucoma 10.2
18 axenfeld-rieger syndrome 10.2
19 macular retinal edema 10.2
20 ocular hypertension 10.2
21 scotoma 10.2
22 ichthyosis--cheek--eyebrow syndrome 10.0
23 keratitis, hereditary 10.0
24 corneal dystrophy, band-shaped 10.0
25 multinucleated neurons, anhydramnios, renal dysplasia, cerebellar hypoplasia, and hydranencephaly 10.0
26 3-methylglutaconic aciduria, type iii 10.0
27 branchiootic syndrome 1 10.0
28 secondary corneal edema 10.0
29 uveitis 10.0
30 primary angle-closure glaucoma 10.0
31 iritis 10.0
32 melanoma 10.0
33 corneal dystrophy 10.0
34 malignant iris melanoma 10.0
35 hyperthyroidism 10.0
36 cataract 10.0
37 herpes zoster 10.0
38 measles 10.0
39 glaucomatocyclitic crisis 10.0
40 adamtsl4-related eye disorders 10.0
41 farsightedness 10.0
42 overgrowth syndrome 10.0
43 ichthyosis 9.9
44 ichthyosis vulgaris 9.8

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):

(show all 13)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Acetylcysteine Approved, Investigational Phase 2 616-91-1 12035
2
Cysteine Approved, Nutraceutical Phase 2 52-90-4 5862
3 Ophthalmic Solutions Phase 2
4 Pharmaceutical Solutions Phase 2
5 Protective Agents Phase 2
6 Respiratory System Agents Phase 2
7 Antioxidants Phase 2
8 Anti-Infective Agents Phase 2
9 Antiviral Agents Phase 2
10 Expectorants Phase 2
11 N-monoacetylcystine Phase 2
12 Lubricant Eye Drops Phase 2
13 Antidotes 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 A Double-Masked, Randomised, Placebo-Controlled, Parallel-Group, 12 Week, Phase 2 Study to Investigate the Safety and Efficacy of K-321 Eye Drops After Descemetorhexis in Patients With Fuchs Endothelial Corneal Dystrophy Recruiting NCT04250207 Phase 2 K-321 Solution;Placebo Solution
3 Targeting Reactive Oxygen Species Production as a Novel Therapeutic in Fuch's Endothelial Corneal Dystrophy Recruiting NCT04440280 Phase 2 N-acetyl cysteine (NAC) 10% solution;N-acetyl cysteine (NAC) 20% solution;Visine Dry Eye Relief eye drops
4 A Phase 1/ Phase 2 Study Evaluating the Safety and Efficacy of the Investigational New Drug TTHX1114(NM141) on the Regeneration of Corneal Endothelial Cells in Patients With Corneal Endothelial Dystrophies Following Intracameral Delivery Recruiting NCT04520321 Phase 1, Phase 2 TTHX1114(NM141)
5 Prospective Clinical Study on Postoperative Outcome After Descemet Membrane Endothelial Keratoplasty (DMEK) and Ultra-thin Descemet Stripping Automated Endothelial Keratoplasty (DSAEK) Unknown status NCT02020044
6 Open-enrollment, Prospective Study of Endothelial Keratoplasty Outcomes Completed NCT00800111
7 A Multi-center Study on Iridocorneal Endothelial (ICE) Syndrome in China Recruiting NCT03270761

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

Publications for Iridocorneal Endothelial Syndrome

Articles related to Iridocorneal Endothelial Syndrome:

(show top 50) (show all 224)
# Title Authors PMID Year
1
Comparison of Descemet membrane endothelial keratoplasty for iridocorneal endothelial syndrome and Fuchs endothelial dystrophy. 61
33556383 2021
2
Diseases of the corneal endothelium. 61
33596440 2021
3
Surgical outcomes of Ahmed glaucoma valve implantation in patients with glaucoma secondary to iridocorneal endothelial syndrome. 61
32367005 2021
4
Two Rare Causes of Anterior Pseudo-Uveitis Affecting the Iris: Sequelae of Bilateral Acute Iris Transillumination (BAIT) Syndrome and Iridocorneal Endothelial (ICE) Syndrome. 61
33607683 2021
5
Outcomes of Descemet stripping endothelial keratoplasty combined with near total iridectomy in iridocorneal endothelial syndrome. 61
33563680 2021
6
Case Series: Novel Utilization of Rho-Kinase Inhibitor for the Treatment of Corneal Edema. 61
33269892 2021
7
Long-term clinical outcomes after Descemet Membrane Endothelial Keratoplasty (DMEK) in Irido-Corneal Endothelial Syndrome. 61
32923740 2020
8
Atypical Case of Bilateral Chandler Syndrome With Recurrent Band Keratopathy. 61
33264150 2020
9
Two-year clinical outcome after Descemet membrane endothelial keratoplasty using a standardized protocol. 61
33120629 2020
10
Metagenomic Deep Sequencing to Investigate for an Infectious Etiology of Iridocorneal Endothelial Syndrome. 61
32398422 2020
11
Atypical variant of Fuchs' endothelial dystrophy mimicking iridocorneal endothelial syndrome: A case report. 61
32971690 2020
12
Combined cataract surgery and lamellar endothelial keratoplasty in iridocorneal endothelial syndrome. 61
32595005 2020
13
Cornea thinning in two cases of ICE syndrome. 61
32988977 2020
14
Clinical description of a Cogan-Reese type iridocorneal endothelial syndrome using anterior segment optical coherence tomography and specular microscopy. 61
32576400 2020
15
Reply to "Metagenomic Deep Sequencing to Investigate for an Infectious Etiology of Iridocorneal Endothelial Syndrome". 61
32769681 2020
16
Outcomes of Ahmed Glaucoma Drainage Implant in Eyes With Glaucoma Secondary to Iridocorneal Endothelial Syndrome. 61
32251072 2020
17
Letter: XEN Gel Stent to Treat ICE Syndrome. 61
31977542 2020
18
XEN Gel Stent to Treat ICE Syndrome: 4 Cases. 61
32229787 2020
19
Bilateral Chandler's syndrome: Uncommon entity diagnosed by ultrasound biomicroscopy and confocal microscopy. 61
32057025 2020
20
Erratum: Iridocorneal endothelial syndrome: Evaluation of patient demographics and endothelial morphology by in vivo confocal microscopy in an Indian cohort. 61
32057033 2020
21
Microscope Integrated Intraoperative Optical Coherence Tomography-Guided DMEK in Corneas with Poor Visualization. 61
32184553 2020
22
Ex-PRESS mini-shunt implanted in a pregnant patient with iridocorneal endothelial syndrome. 61
30618278 2020
23
Graft Survival After Penetrating and Endothelial Keratoplasty in Iridocorneal Endothelial Syndrome. 61
31335531 2020
24
XEN Gel Stent to Treat ICE Syndrome: 4 Cases. 61
31425336 2019
25
Iridocorneal endothelial syndrome in a patient with keratoconus - a case report. 61
31711443 2019
26
Iridocorneal endothelial syndrome: Evaluation of patient demographics and endothelial morphology by in vivo confocal microscopy in an Indian cohort. 61
31007217 2019
27
Commentary: In vivo confocal microscopy in iridocorneal endothelial syndrome. 61
31007218 2019
28
Case Report: Iridocorneal Endothelial Syndrome Progression Documented by Anterior Segment Optical Coherence Tomography. 61
30907858 2019
29
Descemet membrane endothelial keratoplasty in iridocorneal endothelial syndrome and posterior polymorphous corneal dystrophy. 61
30975342 2019
30
Primary implantation of non-valved glaucoma-drainage-device in sulcus in iridocorneal endothelial syndrome. 61
31741874 2019
31
Cogan-Reese syndrome: image analysis with specular microscopy, optical coherence tomography, and ultrasound biomicroscopy. 61
31327934 2019
32
Descemet membrane endothelial keratoplasty for treatment of iridocorneal endothelial syndrome. 61
30502999 2018
33
Unique variations and characteristics of iridocorneal endothelial syndrome in China: a case series of 58 patients. 61
28993974 2018
34
The iridocorneal endothelial syndrome. 61
29331589 2018
35
Atypical Presentation of Iridocorneal Endothelial Syndrome With Band Keratopathy but No Corneal Edema Managed With Descemet Membrane Endothelial Keratoplasty. 61
29668572 2018
36
Clinical outcome of Descemet stripping automated endothelial keratoplasty in 18 cases with iridocorneal endothelial syndrome. 61
29243737 2018
37
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
38
Unclassified Axenfeld-Rieger Syndrome: A CASE SERIES and Review of Literature. 61
27929720 2018
39
Iridocorneal endothelial syndrome: clinical perspectives. 61
29670326 2018
40
[Differential diagnosis and treatment of Chandler iridocorneal endothelial syndrome]. 61
29543203 2018
41
Retinoinvasive Uveal Melanoma: Report of 2 Cases and Review of the Literature. 61
29344483 2017
42
Surgical outcomes of patients with iridocorneal endothelial syndrome: a case series. 61
27495952 2017
43
ICE-Syndrome: A Case Report of Implantation of a Microbypass Xen Gel Stent After DMEK Transplantation. 61
27841796 2017
44
Iridocorneal Endothelial Syndrome Presenting With Large Diurnal Intraocular Pressure Fluctuation. 61
27977477 2017
45
Glaucoma associated with iridocorneal endothelial syndrome in 203 Indian subjects. 61
28282413 2017
46
[Diagnostic methods in the clinical evaluation of iridocorneal endothelial syndrome]. 61
27023272 2016
47
Concurrent Acute Retinal Necrosis in a Patient With Iridocorneal Endothelial Syndrome. 61
27362881 2016
48
Cystoid macular edema associated with iridocorneal endothelial syndrome: a case report. 61
27585567 2016
49
Outcomes of Primary Trabeculectomy With Mitomycin-C in Glaucoma Secondary to Iridocorneal Endothelial Syndrome. 61
25943731 2016
50
Post Filtering Surgery Globe Massage-induced Keratoconus in an Eye with Iridocorneal Endothelial Syndrome: A Case Report and Literature Brief Review. 61
27621792 2016

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
20 GARD
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
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Mar-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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