Your browser doesn't support javascript.
loading
Show: 20 | 50 | 100
Results 1 - 10 de 10
Filter
1.
Cornea ; 2024 Jul 05.
Article in English | MEDLINE | ID: mdl-38967497

ABSTRACT

PURPOSE: Mpox is a rare infectious disease. Lack of knowledge among eye care professionals regarding mpox keratitis greatly reduces the likelihood of diagnosis and effective management. This report and review seek to increase the knowledge of mpox keratitis among eye care professionals. METHODS: We report a patient with mpox keratitis who underwent successful penetrating keratoplasty, with 20 years of follow-up. A systematic literature search and review of cases of mpox keratitis from 1970 to 2024 was performed. RESULTS: A total of 24 articles and 2 abstracts reporting 35 cases of mpox keratitis were identified. A frequency of 0.5% to 1.0% may be the lower range of mpox keratitis among symptomatic patients with a confirmed mpox diagnosis. Mpox keratitis occurred with and without systemic mpox. Initial misdiagnoses were common (40%). Polymerase chain reaction results aided clinical diagnosis. Corneal disease ranged from mild epitheliopathy to fulminant ulcerative keratitis. Outcomes ranged from 20/20 acuity to no light perception. In the absence of fulminant systemic disease, tecovirimat was associated with clinical improvement of mpox keratitis in almost all cases. Our case is the only known report of successful penetrating keratoplasty for mpox keratitis and the only case whereby monkeypox virus was cultured from the corneal surface. CONCLUSIONS: Mpox keratitis is rare but can result in severe vision loss and blindness. Systemic tecovirimat seems to be effective in treating mpox keratitis, although the low frequency of keratitis precludes clinical trials. Topical steroids may extend virus survival in the cornea. Polymerase chain reaction may help confirm mpox corneal involvement.

2.
Nat Commun ; 8: 14898, 2017 03 30.
Article in English | MEDLINE | ID: mdl-28358029

ABSTRACT

The structure of the cornea is vital to its transparency, and dystrophies that disrupt corneal organization are highly heritable. To understand the genetic aetiology of Fuchs endothelial corneal dystrophy (FECD), the most prevalent corneal disorder requiring transplantation, we conducted a genome-wide association study (GWAS) on 1,404 FECD cases and 2,564 controls of European ancestry, followed by replication and meta-analysis, for a total of 2,075 cases and 3,342 controls. We identify three novel loci meeting genome-wide significance (P<5 × 10-8): KANK4 rs79742895, LAMC1 rs3768617 and LINC00970/ATP1B1 rs1200114. We also observe an overwhelming effect of the established TCF4 locus. Interestingly, we detect differential sex-specific association at LAMC1, with greater risk in women, and TCF4, with greater risk in men. Combining GWAS results with biological evidence we expand the knowledge of common FECD loci from one to four, and provide a deeper understanding of the underlying pathogenic basis of FECD.


Subject(s)
Fuchs' Endothelial Dystrophy/genetics , Genetic Loci , Genome-Wide Association Study , Humans , ROC Curve , Reproducibility of Results , Risk Factors
3.
JAMA Ophthalmol ; 133(3): 246-54, 2015 Mar.
Article in English | MEDLINE | ID: mdl-25322173

ABSTRACT

IMPORTANCE: The Cornea Donor Study (CDS) showed that donor age is not a factor in survival of most penetrating keratoplasties for endothelial disease. Secondary analyses confirm the importance of surgical indication and presence of glaucoma in outcomes at 10 years. OBJECTIVE: To assess the relationship between donor and recipient factors and corneal graft survival in the CDS. DESIGN, SETTING, AND PARTICIPANTS: Multicenter prospective, double-masked, controlled clinical trial conducted at 80 clinical sites. One hundred five surgeons enrolled 1090 participants undergoing corneal transplant for a moderate-risk condition, principally Fuchs dystrophy or pseudophakic or aphakic corneal edema (PACE). Forty-three eye banks provided corneas. INTERVENTIONS: Corneas from donors younger than 66 years and donors 66 years or older were assigned, masked to donor age. Surgery and postoperative care were performed according to the surgeons' usual routines. Participants were followed up for as long as 12 years. MAIN OUTCOMES AND MEASURES: Graft failure, defined as a regrafting procedure or a cloudy cornea for 3 consecutive months. RESULTS: The 10-year cumulative probability of graft failure was higher in participants with PACE than in those with Fuchs dystrophy (37% vs 20%; hazard ratio [HR], 2.1 [99% CI, 1.4-3.0]; P < .001) and in participants with a history of glaucoma before penetrating keratoplasty, particularly with prior glaucoma surgery (58% with prior glaucoma surgery and use of medications to lower intraocular pressure at the time of surgery vs 22% with no history of glaucoma surgery or medication use; HR, 4.1 [99% CI, 2.2-7.5]; P < .001). We found trends toward increased graft failure in recipients who were 70 years or older compared with those younger than 60 years (29% vs 19%; HR, 1.2 [99% CI, 0.7-2.1]; P = .04) or were African American (HR, 1.5; P = .11) or who had a history of smoking (35% vs 24%; HR, 1.6 [99% CI, 0.9-2.8]; P = .02). Lower endothelial cell density (ECD) and higher corneal thickness (CT) at 6 months (6% vs 41% for ECD ≥2700 vs <1700 cells/mm2 [P < .001]; 14% vs 36% for CT <500 vs ≥600 µm [P = .001]), 1 year (4% vs 39% for ECD ≥2700 vs <1700 cells/mm2 [P < .001]; 18% vs 28% for CT <500 vs ≥600 µm [P = .04]), and 5 years (2% vs 29% for ECD ≥1500 vs <500 cells/mm2 [P < .001]; 7% vs 34% for CT <550 vs ≥650 µm [P < .001]) were associated with subsequent graft failure. CONCLUSIONS AND RELEVANCE: Most penetrating corneal grafts for Fuchs dystrophy or PACE remain clear at 10 years. The risk for failure is greater for graft recipients with PACE and those with a history of glaucoma. Measurements of ECD and CT during the course of postkeratoplasty follow-up are associated with a risk for failure. However, even with very low ECD and high CT at 5 years, most corneas remain clear at 10 years.


Subject(s)
Cornea , Graft Survival/physiology , Keratoplasty, Penetrating , Tissue Donors , Age Factors , Aged , Cell Count , Corneal Edema/surgery , Corneal Endothelial Cell Loss/diagnosis , Double-Blind Method , Endothelium, Corneal/pathology , Eye Banks/statistics & numerical data , Female , Follow-Up Studies , Fuchs' Endothelial Dystrophy/surgery , Glaucoma/complications , Graft Rejection/epidemiology , Humans , Male , Middle Aged , Prospective Studies , Pseudophakia/complications , Risk Factors , Time Factors
4.
Cornea ; 32(3): 280-4, 2013 Mar.
Article in English | MEDLINE | ID: mdl-22820607

ABSTRACT

PURPOSE: To determine rates of tissue use for corneal transplants via endothelial keratoplasty (EK) relative to penetrating keratoplasty (PK). METHODS: Retrospective chart review of all cornea tissues (n = 3669) distributed from the Lions Eye Bank of Wisconsin for EK or PK from August 1, 2004 through July 31, 2009 (60 months). Rates of tissue use for EK relative to PK were determined both on a yearly basis and for the overall study period. Replacement frequency and time to subsequent surgery were established for each group. Donor tissue and recipient characteristics were compared between groups. RESULTS: Donor characteristics did not differ between the 2 groups; 11.9% of EK tissues failed and were replaced during the study period compared with 5.1% of PK tissues (P < 0.0001). Additional tissue for the same eye came at a mean of 174 days after an EK surgery compared with 558 days after a PK (P < 0.0001). Surgeons requesting tissue for EK increased each year, whereas the number of repeat tissue requests decreased over time. CONCLUSIONS: Additional tissues were required for recipients of EK more than twice as often as for recipients of PK, and replacement of EK grafts occurred at a mean of more than 1 year before replacement of PK grafts. This pattern of tissue utilization during the first 5 years of distribution for EK did not negatively affect the Lions Eye Bank of Wisconsin from meeting the surgeon demand for tissue in its service area. Eye banks may wish to monitor tissue utilization as part of their quality assurance program.


Subject(s)
Cornea , Descemet Stripping Endothelial Keratoplasty/statistics & numerical data , Endothelium, Corneal/transplantation , Eye Banks/statistics & numerical data , Keratoplasty, Penetrating/statistics & numerical data , Tissue and Organ Procurement/statistics & numerical data , Eye Banks/supply & distribution , Female , Graft Rejection , Humans , Male , Quality Assurance, Health Care , Reoperation , Retrospective Studies , Tissue Donors
5.
PLoS One ; 7(10): e46742, 2012.
Article in English | MEDLINE | ID: mdl-23110055

ABSTRACT

Fuchs endothelial corneal dystrophy (FECD) is the most common late-onset, vision-threatening corneal dystrophy in the United States, affecting about 4% of the population. Advanced FECD involves a thickening of the cornea from stromal edema and changes in Descemet membrane. To understand the relationship between FECD and central corneal thickness (CCT), we characterized common genetic variation in COL8A2 and TCF4, genes previously implicated in CCT and/or FECD. Other genes previously associated with FECD (PITX2, ZEB1, SLC4A11), and genes only known to affect CCT (COL5A1, FOXO1, AVGR8, ZNF469) were also interrogated. FECD probands, relatives and controls were recruited from 32 clinical sites; a total of 532 cases and 204 controls were genotyped and tested for association of FECD case/control status, a 7-step FECD severity scale and CCT, adjusting for age and sex. Association of FECD grade with TCF4 was highly significant (OR= 6.01 at rs613872; p = 4.8×10(-25)), and remained significant when adjusted for changes in CCT (OR= 4.84; p = 2.2×10(-16)). Association of CCT with TCF4 was also significant (p = 6.1×10(-7)), but was abolished with adjustment for FECD grade (p = 0.92). After adjusting for FECD grade, markers in other genes examined were modestly associated (p ∼ 0.001) with FECD and/or CCT. Thus, common variants in TCF4 appear to influence FECD directly, and CCT secondarily via FECD. Additionally, changes in corneal thickness due to the effect of other loci may modify disease severity, age-at-onset, or other biomechanical characteristics.


Subject(s)
Basic Helix-Loop-Helix Leucine Zipper Transcription Factors/genetics , Collagen Type VIII/genetics , Cornea/metabolism , Cornea/pathology , Fuchs' Endothelial Dystrophy/genetics , Fuchs' Endothelial Dystrophy/pathology , Transcription Factors/genetics , Aged , Female , Genotype , Haplotypes , Humans , Linkage Disequilibrium/genetics , Male , Middle Aged , Polymorphism, Single Nucleotide/genetics , Transcription Factor 4
6.
Micron ; 43(12): 1293-8, 2012 Dec.
Article in English | MEDLINE | ID: mdl-22421334

ABSTRACT

The ability to accurately determine the elastic modulus of each layer of the human cornea is a crucial step in the design of better corneal prosthetics. In addition, knowledge of the elastic modulus will allow design of substrates with relevant mechanical properties for in vitro investigations of cellular behavior. Previously, we have reported elastic modulus values for the anterior basement membrane and Descemet's membrane of the human cornea, the surfaces in contact with the epithelial and endothelial cells, respectively. We have completed the compliance profile of the stromal elements of the human cornea by obtaining elastic modulus values for Bowman's layer and the anterior stroma. Atomic force microscopy (AFM) was used to determine the elastic modulus, which is a measure of the tissue stiffness and is inversely proportional to the compliance. The elastic response of the tissue allows analysis with the Hertz equation, a model that provides a relationship between the indentation force and depth and is a function of the tip radius and the modulus of the substrate. The elastic modulus values for each layer of the cornea are: 7.5±4.2 kPa (anterior basement membrane), 109.8±13.2 kPa (Bowman's layer), 33.1±6.1 kPa (anterior stroma), and 50±17.8 kPa (Descemet's membrane). These results indicate that the biophysical properties, including elastic modulus, of each layer of the human cornea are unique and may play a role in the maintenance of homeostasis as well as in the response to therapeutic agents and disease states. The data will also inform the design and fabrication of improved corneal prosthetics.


Subject(s)
Biomechanical Phenomena , Compliance , Cornea/physiology , Microscopy, Atomic Force/methods , Elastic Modulus , Humans
7.
Arch Ophthalmol ; 130(1): 33-8, 2012 Jan.
Article in English | MEDLINE | ID: mdl-22232473

ABSTRACT

OBJECTIVE: To investigate a correlation between the severity of histologic changes of the Descemet membrane in patients with Fuchs endothelial dystrophy and the best-corrected visual acuity (VA) after Descemet membrane-stripping automated endothelial keratoplasty (DSAEK). METHODS: In a retrospective study design, we created a histologic grading system based on common characteristics observed histologically among 92 DSAEK specimens sent to the University of Wisconsin Eye Pathology Laboratory with a clinical diagnosis of Fuchs dystrophy from 3 separate corneal surgeons. Cases were graded as mild, moderate, or severe on the basis of guttae dispersion, presence of a laminated Descemet membrane, presence of embedded guttae, and density of guttae. Regression models were built to study the relationship among preoperative VA, histologic findings, and best-corrected VA 6 months and 1 and 2 years after DSAEK. RESULTS: No correlation was found between the severity of histologic changes of Descemet membrane and preoperative VA. However, a correlation was noted between the preoperative and final VA. Cases with a laminated Descemet membrane but no embedded guttae (n = 8) appeared to be less responsive to DSAEK. Otherwise, the severity of histologic changes of Descemet membrane observed in patients with Fuchs corneal dystrophy after DSAEK did not show a statistically significant correlation with final VA. CONCLUSIONS: Our analysis fails to show an inverse relationship between the severity of histologic changes of the Descemet membrane and the best-corrected VA of at least 20/40 after DSAEK for Fuchs endothelial dystrophy. However, in a subset of patients with Fuchs dystrophy who develop a laminated Descemet membrane without embedded guttae, the visual recovery after DSAEK is less than expected. The laminated architecture of Descemet membrane without embedded guttae may facilitate separation between the membrane layers and, thus, incomplete removal of the recipient's Descemet membrane during DSAEK, which may then limit the postoperative visual outcome.


Subject(s)
Descemet Stripping Endothelial Keratoplasty , Fuchs' Endothelial Dystrophy/surgery , Visual Acuity/physiology , Corneal Stroma/physiopathology , Fuchs' Endothelial Dystrophy/classification , Fuchs' Endothelial Dystrophy/physiopathology , Humans , Postoperative Period , Retrospective Studies
8.
Cornea ; 31(1): 26-35, 2012 Jan.
Article in English | MEDLINE | ID: mdl-22045388

ABSTRACT

PURPOSE: To describe the methods for family and case-control recruitment for a multicenter genetic and associated heritability analyses of Fuchs endothelial corneal dystrophy (FECD). METHODS: Twenty-nine enrolling sites with 62 trained investigators and coordinators gathered individual and family information, graded the phenotype, and collected blood and/or saliva for genetic analysis on all individuals with and without FECD. The degree of FECD was assessed in a 0 to 6 semiquantitative scale using standardized clinical methods with pathological verification of FECD on at least 1 member of each family. Central corneal thickness was measured by ultrasonic pachymetry. RESULTS: Three hundred twenty-two families with 330 affected sibling pairs with FECD were enrolled and included a total of 650 sibling pairs of all disease grades. Using the entire 7-step FECD grading scale or a dichotomous definition of severe disease, heritability was assessed in families via sib-sib correlations. Both binary indicators of severe disease and semiquantitative measures of disease severity were significantly heritable, with heritability estimates of 30% for severe disease, 37% to 39% for FECD score, and 47% for central corneal thickness. CONCLUSIONS: Genetic risk factors have a strong role in the severity of the FECD phenotype and corneal thickness. Genotyping this cohort with high-density genetic markers followed by appropriate statistical analyses should lead to novel loci for disease susceptibility.


Subject(s)
Chromosome Mapping , Fuchs' Endothelial Dystrophy/genetics , Aged , Case-Control Studies , Cohort Studies , Cornea/pathology , Female , Fuchs' Endothelial Dystrophy/pathology , Genetic Predisposition to Disease , Genotype , Humans , Male , Middle Aged , Phenotype
SELECTION OF CITATIONS
SEARCH DETAIL
...