Limbal stem cell deficiency

disease
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Summary

Limbal stem cell deficiency (MONDO:0025667) is a disease and 30 clinical trials. A subtype of corneal disorder — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

  • Prevalence: 1-5 / 10 000 (Europe) [Orphanet-validated]
  • Phenotypes (HPO): 15
  • Clinical trials: 30

Clinical features

Epidemiology

Prevalence records

1 prevalence record(s), Orphanet:

TypeClassValueGeographyValidation
Point prevalence1-5 / 10 00020EuropeValidated

Signs & symptoms

Clinical features (HPO)

15 HPO clinical features (Orphanet curated; top 15 by frequency):

HPO IDTermFrequency
HP:0000613PhotophobiaFrequent (30-79%)
HP:0000632Lacrimation abnormalityFrequent (30-79%)
HP:0000643BlepharospasmFrequent (30-79%)
HP:0007663Reduced visual acuityFrequent (30-79%)
HP:0008000Decreased corneal reflexFrequent (30-79%)
HP:0009926EpiphoraFrequent (30-79%)
HP:0030953Conjunctival hyperemiaFrequent (30-79%)
HP:0200026Ocular painFrequent (30-79%)
HP:0000491KeratitisOccasional (5-29%)
HP:0000559Corneal scarringOccasional (5-29%)
HP:0007727Opacification of the corneal epitheliumOccasional (5-29%)
HP:0011494Generalized opacification of the corneaOccasional (5-29%)
HP:0011496Corneal neovascularizationOccasional (5-29%)
HP:0100583Corneal perforationVery rare (<1-4%)
HP:0500008Cornea verticillataVery rare (<1-4%)

Identifiers

Disease identifiers

FieldValue
Canonical namelimbal stem cell deficiency
Mondo IDMONDO:0025667
Orphanet171673
UMLSC1561989
MedGen295775
GARD0020123
Is cancer (heuristic)no

Disease family

This is a subtype of corneal disorder. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease › human disease › disease by body system or component › disorder of orbital regioneye disordercorneal disorderlimbal stem cell deficiency

Related subtypes (23): cornea plana, pseudopterygium, corneal deposit, Bowman’s membrane folds or rupture, corneal degeneration, corneal staphyloma, corneal argyrosis, corneal ectasia, keratopathy, keratitis, corneal edema, brittle cornea syndrome, megalocornea, X-linked corneal dermoid, Peters anomaly, pellucid marginal degeneration, keratoconus, corneal dystrophy, sclerocornea, cornea neoplasm, Arnold stickler bourne syndrome, thygeson superficial punctate keratopathy, Terrien marginal degeneration

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

No druggable-target or therapeutic data for this disease’s cohort.

Clinical trials & evidence

Clinical trials

Clinical trials: 30.

Phase distribution (across all retrieved trials)

PhaseTrials
Not specified17
PHASE1/PHASE26
PHASE15
PHASE31
PHASE21

Top trials by phase / activity

NCTPhaseStatusTitle
NCT01489501PHASE3WITHDRAWNMulticenter Study of CAOMECS Transplantation to Patients With Total Limbal Stem Cell Deficiency
NCT00736307PHASE1/PHASE2COMPLETEDAutologous Transplantation of Cultivated Limbal Stem Cells on Amniotic Membrane in Limbal Stem Cell Deficiency (LSD) Patients
NCT00845117PHASE1/PHASE2UNKNOWNCultivated Stem Cell Transplantation for the Treatment of Limbal Stem Cell Deficiency
NCT02318485PHASE2COMPLETEDLimbal Epithelial Stem Cell Transplantation: a Phase II Multicenter Trial
NCT02415218PHASE1/PHASE2COMPLETEDTransplantation of Autologous Oral Mucosal Epithelial Sheets for Limbal Stem-cell Deficiency
NCT02592330PHASE1/PHASE2COMPLETEDLimbal Stem Cell Deficiency (LSCD) Treatment With Cultivated Stem Cell (CALEC) Graft
NCT03015779PHASE1/PHASE2UNKNOWNTransplantation of Autologous Oral Mucosal Epithelial Stem Cell Sheet for Treating Limbal Stem Cell Deficiency Disease
NCT03549299PHASE1/PHASE2UNKNOWNAllogeneic ABCB5-positive Limbal Stem Cells for Treatment of LSCD
NCT03957954PHASE1ACTIVE_NOT_RECRUITINGStem Cell Therapy for Limbal Stem Cell Deficiency
NCT06452316PHASE1RECRUITINGStudy to Evaluate the Safety and Efficacy of CSB-001 Ophthalmic Solution 0.1% in Subjects With Limbal Stem Cell Deficiency
NCT02649621PHASE1COMPLETEDThe Improvement of Limbal Stem Cell Deficiency (LSCD) in Unilateral Stem Cell Damage by Amniotic Membrane Extract Eye Drop (AMEED)
NCT03943797PHASE1UNKNOWNCultivated Autologous Oral Mucosal Epithelial Transplantation
NCT05909735PHASE1COMPLETEDTreatment of LSCD With DM
NCT01756365Not specifiedRECRUITINGAutologous Cultured Corneal Epithelium (CECA) for the Treatment of Limbal Stem Cell Deficiency
NCT02149732Not specifiedAVAILABLEClinical Trial on the Effect of Autologous Oral Mucosal Epithelial Sheet Transplantation
NCT03884569Not specifiedNOT_YET_RECRUITINGCultivated Limbal Epithelial Transplantation (CLET) for Limbal Stem Cell Deficiency (LSCD)
NCT06265298Not specifiedRECRUITINGImplementation of a Protocol for the Transdifferentiation of Buccal Mucosal Epithelium Into Corneal Epithelium
NCT07119580Not specifiedRECRUITINGAssociation Between Limbal Function and Tear Proteomics in Chronic Ocular Diseases: Focusing on Glaucoma
NCT07422389Not specifiedAVAILABLEBRM421 Ophthalmic Solution in Patients With Limbal Stem Cell Deficiency
NCT02568527Not specifiedCOMPLETEDBiodegradable Synthetic Scaffold as a Substitute for hAM in Limbal Epithelial Cells Transplant in LSCD Patients
NCT02579993Not specifiedTERMINATEDABCB5 as a Prognostic Marker in Survival of Cultivated Limbal Stem Cell Transplantation
NCT03217435Not specifiedCOMPLETEDCorneal Epithelial Allograft From Living-related Donor for LSCD
NCT03217487Not specifiedUNKNOWNCorneal Epithelial Autograft for LSCD
NCT03226015Not specifiedCOMPLETEDAutologous Oral Mucosa Transplantation for Limbal Stem Cell Deficiency
NCT03594370Not specifiedCOMPLETEDMultiple Noninvasive Examination Modality to Evaluate the Severity of Ocular Surface Disorders
NCT04021134Not specifiedUNKNOWNThe Effects of Allogeneic SLET
NCT04021875Not specifiedUNKNOWNThe Effects of Autologous SLET
NCT04995926Not specifiedUNKNOWNLabial Mucosal Epithelium Grafting for Corneal Limbus Substitution
NCT05461469Not specifiedCOMPLETEDCDL Validation Study
NCT06412718Not specifiedUNKNOWNValidation of Human Drugs Target of Repurposed Drugs and Novel Therapies

No linked Atlas pages yet — the cross-entity mesh grows as the corpus expands.