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PubMed2003

Can true monocular keratoconus occur?

Evidence increasingly supports keratoconus as an inherited, genetic disorder. If this is the case, it would be expected to express itself as a binocular condition even though variable expression of the gene may show differing levels in the two eyes. It is a common occurrence in specialist contact lens practices to see pseudo-monocular keratoconus, that is, an overt clinical level of the condition in one eye and forme fruste keratoconus in the other. The advent of videokeratoscopy has shown almost every case of apparent monocular keratoconus to be different expressions of the condition in the two eyes. Nevertheless, rare cases of the condition are seen that appear to be truly monocular. Such a case is presented in which the condition has been present for more than 23 years. The age of the subject makes the future onset of keratoconus highly unlikely and videokeratoscopy does not show the presence of forme fruste in the 'good' eye. There is no history of monocular eye rubbing. Pachymetry shows the normal eye to be well within the range of normal corneal thickness, while the keratoconus in the other eye is sufficiently advanced to warrant corneal grafting. Thus, it is a case of true monocular keratoconus and represents an extreme example of variable gene expression.

Corneal Topography↗
PubMed1982

Atopy and HLA antigens in patients with keratoconus.

Thirty unrelated Caucasian patients with keratoconus and 30 patients with regular astigmatism which served as a control group were typed for histocompatibility (HLA) antigens. Their serum levels of immunoglobulins were determined and past and present history of atopic disease of their own as well as among their relatives were examined. No association between keratoconus and any specific HLA antigen was noted. Neither was any statistically significant increase of atopic disease in the keratoconus population observed. Nor was any correlation between elevated IgE level and keratoconus found. However, a statistically significant increase of atopic disease among the relatives of the keratoconus group was found. In conclusion, no evidence for HLA association with keratoconus disease was found. The results suggest that there may be a association between keratoconus and atopic disease.

Adolescent↗
PubMed1998

Altered organization of collagen in the apex of keratoconus corneas.

PURPOSE: In 15 keratoconus corneas, the three-dimensional arrangement of collagen lamellae was investigated by means of scanning electron microscopy. METHODS: Keratoconus corneas without visible scars were obtained during perforating keratoplasty. The noncollagenous matrix of the stroma was removed with sodium hydroxide. Descemet's membrane was removed mechanically and deeper layers of the stroma were exposed by cutting the tissue tangentially to the corneal surface with an ultramicrotome. The apical and the para-apical regions of keratoconus were compared the central regions of normal corneas. RESULTS: In the apical regions of 11 out of the 15 keratoconus corneas (73%), the arrangement of the collagen lamellae differs from those of the para-apical regions and normal corneas. Their collagen fibrils from uniform layers and no delimited collagen lamellae can be differentiated. Interlacing between adjacent layers in extremely decreased or even absent. In the para-apical region of keratoconus corneas the three-dimensional arrangement of collagen lamellae does not differ from that in normal corneas. CONCLUSION: Stromal thinning and conical ectasia in the apex of keratoconus corneas alters the organization of collagen. This will certainly affect the biomechanical properties of the cornea and further lead to a progression of keratoconus irrespective of its primary pathogenesis.

Adolescent↗
PubMed2005

Increased levels of catalase and cathepsin V/L2 but decreased TIMP-1 in keratoconus corneas: evidence that oxidative stress plays a role in this disorder.

PURPOSE: The mRNA levels of antioxidant enzymes, matrix metalloproteinases, cathepsin V/L2, and tissue inhibitor of matrix metalloproteinases (TIMPs) were determined in keratoconus and normal corneas. Protein levels or enzyme activities were analyzed when RNA levels were different. METHODS: A total of 25 physiologic (normal) and 32 keratoconus corneas were studied. mRNAs were analyzed by semiquantitative reverse transcription-polymerase chain reaction and Southern blot analysis. Proteins were assessed by immunohistochemistry and/or Western blot analysis. Catalase activity was measured in corneal extracts. Antioxidant enzymes examined were catalase, superoxide dismutase (SOD)-1, SOD3, glutathione reductase, glutathione S-transferase and aldehyde dehydrogenase 3A1. Degradative enzymes examined were cathepsin V/L2 and matrix metalloproteinase (MMP)-1, -2, -7, -9, and -14. Tissue inhibitor of matrix metalloproteinase (TIMP)-1, -2, and -3 were also examined. RESULTS: Keratoconus corneas exhibited a 2.2-fold increase of catalase mRNA level (P < 0.01) and 1.8-fold of enzyme activity (P < 0.03); a 1.5-fold increase of cathepsin V/L2 mRNA (P < 0.03) and abnormal protein distribution; and a 1.8-fold decrease of TIMP-1 mRNA (P < 0.05) and 2.8-fold decrease of protein (P < 0.0001) compared with normal (physiologic) corneas. RNA levels for other antioxidant and degradative enzymes were similar between normal and keratoconus corneas. CONCLUSIONS: Keratoconus corneas have elevated levels of cathepsins V/L2, -B, and -G, which can stimulate hydrogen peroxide production, which, in turn, can upregulate catalase, an antioxidant enzyme. In addition, decreased TIMP-1 and increased cathepsin V/L2 levels may play a role in the matrix degradation that is a hallmark of keratoconus corneas. The findings support the hypothesis that keratoconus corneas undergo oxidative stress and tissue degradation.

Aldehyde Dehydrogenase↗
PubMed1979

The synthesis of glycosaminoglycans by cultures of corneal stromal cells from patients with keratoconus.

Keratoconus is a disease that results in thinning and ectasia of the central cornea. Cultures of corneal stromal cells from patients with keratoconus were established and the synthesis of glycosaminoglycans compared with the synthesis of glycosaminoglycans by normal human corneal stromal cells in culture. Keratoconus and normal control cell cultures were incubated with sodium [(35)S]sulfate and [(3)H]glucosamine for 4 h. After incubation, the labeled glycosaminoglycans were isolated from the medium fractions and cells. Keratoconus and normal control cultures synthesized similar amounts of sulfated glycosaminoglycans independent of the age of donors and(or) the number of subcultures. In contrast to normal control cultures, most of the newly synthesized glycosaminoglycans produced by keratoconus cells were found in the growth medium and much less were in the cell layer. Treatment with glycosaminoglycan-degrading enzymes followed by paper chromatography showed that keratoconus cells, as normal control cells, produced hyaluronic acid and various sulfated glycosaminoglycans. The production of cell layer-related heparan sulfate was markedly reduced in keratoconus cultures. Because heparan sulfate has been shown to be associated with cell surfaces, the decreased heparan sulfate content could reflect changes at this location.

Adolescent↗
PubMed1985

RNA metabolism in cultures of corneal stromal cells from patients with keratoconus.

Total cellular RNA was extracted from cultured keratoconus and normal human corneal stromal cells. The translational activity of these RNAs was examined in a cell-free translation system derived from reticulocyte lysate. Results indicated that keratoconus cells can be separated into two groups, as has been shown previously. Group I keratoconus cells contained the same amount of total RNA as normal cells. RNA activity and the rate of mRNA synthesis in this group of keratoconus cells were also normal. By these criteria it seems that the protein synthesizing system is functioning properly, and group I keratoconus cells should have a normal rate of protein synthesis. These results correlate well with previous findings. Group II keratoconus cells, in contrast, contained more RNA than normal cells. The translational efficiency of RNA was so markedly reduced that the elevation in RNA content did not compensate for the decrease in translational efficiency. It is likely that the reduced protein and collagen synthesis in this group of cells is related to the reduction in the RNA activity. An inhibitory component was present in the keratoconus RNA which affected synthesis of all proteins and suppressed translation of normal RNA.

Adolescent↗
PubMed1999

Expression of betaig-h3 is lower than normal in keratoconus corneas but increases with scarring.

PURPOSE: Keratoconus is a progressive ectatic disease of the cornea. Despite extensive clinical and laboratory investigations, its pathogenesis remains unclear. In this study, we examined the localization of betaig-h3, a recently described extracellular matrix protein in keratoconus corneas both in the absence and presence of subepithelial scarring. METHODS: Two normal corneas and central corneal buttons of 10 patients with keratoconus were excised during perforating keratoplasty and examined, including one case with acute corneal hydrops. In one case, keratoconus was associated with Down syndrome. Immunodetection was done with an antipeptide antibody reacting with the N-terminal part of betaig-h3. RESULTS: We found decreased betaig-h3 levels in the basal epithelial layer and keratocytes of keratoconus corneas. In the scarred corneas, however, betaig-h3 levels were increased in the basal epithelial layers and in activated keratocytes at the places of scarring. In the cornea of the patient with Down syndrome, we found an additional betaig-h3-positive zone in the anterior stroma. CONCLUSIONS: The decreased levels of betaig-h3 corneas seem to be specific for keratoconus. Considering the putative role of betaig-h3 as a cellular-attachment protein, paucity of betaig-h3 in the corneal stroma may lead to decreased mechanical stability and contribute to the development of keratoconus.

Adolescent↗
PubMed2002

Androgen-dependent hereditary mouse keratoconus: linkage to an MHC region.

PURPOSE: To better understand the pathogenesis of hereditary keratoconus, an inbred line of spontaneous mutant mice with keratoconus-affected corneas (SKC mice) was established and studied with a multidisciplinary approach. METHODS: Using a mutant mouse with corneas having a keratoconical appearance as the progenitor, an inbred line of SKC mouse was established by repeated sibling mating. Morphology, cell growth, apoptosis and protein expression of SKC mouse corneas were examined. Castration of males and androgen treatment for females were conducted to determine any androgen dependency of the phenotype. Linkage analysis was conducted to reveal the responsible or predisposing gene of SKC mouse keratoconus. RESULTS: Corneas of the SKC mouse resemble those of human eyes with keratoconus. Both are conical and show similar corneal changes, including apoptosis of keratocytes and increased expression of c-fos protein. The SKC mouse phenotype was transmitted in an autosomal recessive manner, although it was observed almost exclusively in males. Intriguingly, female mice showed the phenotype when injected with testosterone, whereas male incidence of the phenotype diminished drastically when mice were castrated. Linkage analysis localized a predisposition locus to an MHC region on mouse chromosome 17, which includes a locus for the gene for sex-limited protein (Slp). CONCLUSIONS: SKC mouse keratoconus is a potential model for a subset of human keratoconus, which is a disease entity with heterogeneous pathogeneses. Alternatively, SKC mouse keratoconus could be a model for other human or mouse-specific keratopathies.

Animals↗
PubMed2001

[Immunohistochemistry research on keratoconus].

PURPOSE: Keratoconus is a noninflammatory disease characterized by thining and ectasia of the central cornea. The etiology and pathogenesis of keratoconus remain unclear. We studied the composition of basement membranes (BM) in normal, scarred and keratconus corneas to find out possible changes specific for keratoconus. METHODS: Paraffin-embeded sections of normal, scarred and keratoconus corneas were immunostained with antibodys against type IV collagen, Fibronectin (Fn) and Laminin (Ln). RESULTS: Keratoconus had increased staining of the epithelial BM for alpha 1-alpha 2 chain of type IV collagen and Fn, especially in scarred corneal regions. A similar enhancement was also observed in scarred corneas, and there was no significant difference between keratoconus and scarred cornea. CONCLUSION: A process similar to wound healing would largely contribute to the differences seen in the keratoconus.

Adolescent↗
PubMed1989

Protein-related abnormalities in keratoconus.

Two-dimensional electrophoretic maps of extracts from eleven normal and eleven keratoconus corneas were compared. Of the eleven corneas analyzed, eight were pooled and the remaining three were analyzed individually. Several differences were demonstrated between electrophoretic patterns of normal and keratoconus corneas. In keratoconus corneas, 1) two abnormal components (MW 54kD and 26kD) were observed; 2) three normal corneal components (MW 12kD, 14kD, and 39kD) were present in significantly higher amounts; and 3) three normal corneal proteins (MW 66kD, 55kD, and 13kD) were present in reduced amounts. The molecular weight and isoelectric point of one of the normal corneal proteins that we found to be reduced in keratoconus corneas were close to that of a subunit of prolyl-4-hydroxylase, an enzyme required for hydroxylation of proline residues of collagen. The possibility the abnormal proteins detected in the keratoconus corneas were derived from those normal corneal proteins which were absent or were present in reduced amounts in the keratoconus corneas remains to be established. This study may provide protein markers for elucidation of the biochemical abnormality in keratoconus.

Adult↗
PubMed1978

Keratoconus and atopic diseases.

In a survey of 162 keratoconus patients, we set out to investigate individual conditions, such as asthma, hay fever, connective tissue syndromes, and eye rubbing, and to determine their relationship of keratoconus. It was found that the prevalence of asthma rises from 0.4% to 1% in the control group to 17.9% in the keratoconus group, or an eighteenfold increase. In addition, the incidence of hay fever in keratoconus patients was found to be 35.7%. As multifactorial disorders, keratoconus and asthma may share some of the multiple genes necessary for the expression of the disorder. Previous findings of excessive eye rubbing in keratoconus patients were confirmed in this study. Finally, we found that the incidence of connective tissue disorders associated with keratoconus was negligible.

Asthma↗
PubMed1981

Detection of specific collagen types in normal and keratoconus corneas.

Keratoconus is a corneal disease of unknown cause that involves a progressive thinning and scarring of the corneal connective tissue. We examined normal human and keratoconus corneas, including one healed penetrating keratoplasty specimen. Organ cell cultures of normal and keratoconus corneal specimens were labeled with radioactive proline and analyzed by CM-cellulose chromatography and slab gel electrophoresis to determine collagen biosynthesis. Collagen types I and III were synthesized in similar amounts by normal and keratoconus stromacytes in culture. Specifically purified antibodies were used to determine the distribution of collagen types in tissue sections by immunofluorescence. The distribution of collagen types I, III, and IV in keratoconus was also similar to that in normal corneas, except that scarred regions in keratoconus and at the host-graft juncture were largely type III. Immunofluorescent reaction of the anti-type IV collagen antibodies with Bowman's layer, in particular, and Descemet's membrane in keratoconus specimens indicated extensive destruction. Basement membrane destruction may play an important role in this disease.

Adult↗
PubMed1996

Influence of contact lens wear on the corneal topography of keratoconus.

PURPOSE: Corneal videokeratography is frequently used to evaluate keratoconus patients, as it is believed to allow for a more thorough appreciation of the complex curvature changes associated with the anterior corneal surface. The goal of this study was to determine the qualitative and quantitative influence of soft and rigid contact lens wear on the corneal topography of keratoconus. METHODS: 205 keratoconus patients were analyzed by computerized videokeratography for both qualitative corneal topographic patterns and quantitative indices. Fifty-six patients were non-contact lens wearers, 130 wore polymethylmethacrylate (PMMA) or rigid gas permeable (RGP) contact lenses, and 19 wore soft contact lenses. Data from the keratoconus patients were also compared to a control group comprised of normal patients with no history of contact lens wear. RESULTS: All three keratoconus groups had a significantly increased frequency of an asymmetric bowtie/skewed radial axes (AB/SRAX) pattern compared with normals. Differences among the videokeratography patterns for the kertoconus patients included a significant shift from the AB/SRAX videokeratographic pattern to the irregular videokeratographic pattern in the rigid (PMMA or RGP) lens subgroup as well as an increased frequency of the irregular pattern in the soft contact lens group versus the no contact lens group (P < 0.0001). Additional differences between the rigid contact lens and no contact lens keratoconus groups included increased values for the quantitative indices of SAI, SRI, SIM-K, and central K in the rigid contact lens group (P < 0.05). CONCLUSIONS: The increased frequency of qualitative and quantitative corneal irregularity in the rigid contact lens keratoconus group may reflect a mechanical effect of contact lens wear or an advanced disease state in these patients limiting them to rigid lens wear. The advanced disease is evidenced by steeper SIM-K and Central K indices in the rigid contact lens group.

Contact Lenses↗
PubMed1997

Computerized videokeratography of keratoconus kindreds.

OBJECTIVE: To assess the role of heredity in the development of keratoconus. DESIGN: Prospective study. SETTING: Eye clinic providing secondary and tertiary ophthalmic care in Toronto. PATIENTS: Thirty-nine patients with keratoconus (57 eyes) and 48 relatives of 11 patients with keratoconus. The corneal topography of the family members was compared with that of a group of 68 volunteer control subjects (136 eyes) without clinical evidence or a family history of keratoconus. OUTCOME MEASURES: Three quantitative measures derived from computerized videokeratography: the relative steepness of the inferior cornea versus the superior cornea, central corneal power and the difference in central corneal power between the two eyes. All the data were statistically analysed with the use of nonparametric discriminant analysis. RESULTS: Fifteen family members who were believed to be clinically normal on the basis of refraction, keratometry and slit-lamp examination has statistically significant topographic abnormalities suggestive of early or mild keratoconus. CONCLUSIONS: The presence of these findings in family members of patients with keratoconus may represent the incomplete expression of a gene contributing to the development of the condition. Pedigree analysis suggested an autosomal dominant inheritance pattern in 9 of the 11 families. Our results underline the value of videokeratography for accurate family pedigree analysis and the diagnosis of keratoconus.

Adolescent↗
PubMed1998

Comparison of axial and instantaneous videokeratographic data in keratoconus and utility in contact lens curvature prediction.

PURPOSE: Instantaneous radius of curvature data in corneal videokeratoscopy better represents corneal shape and local curvature changes in keratoconus than axial maps, especially in the periphery. Knowledge of instantaneous radius is especially important in keratoconus for analysis of the cone apex, which is often decentered. In this study, we quantitatively compared axial and instantaneous displays of videokeratographic data in keratoconus, with emphasis on apex curvature and position. We assessed the utility of instantaneous data for rigid gas permeable (RGP) contact lens curvature selection and compared it with data derived from the original axial displays in keratoconus. METHODS: We performed modern placido-based videokeratography on 35 eyes with clinically diagnosed keratoconus. We derived the apex curvature, the position of the apex relative to the center of the map, and the steep simulated keratometry (SIM-K) reading from axial and instantaneous videokeratographic displays. We also collected the RGP base curves for 28 eyes that were fit with a minimal apical bearing or clearance RGP contact lens. RESULTS: The instantaneous apex was consistently located closer to the center of the map than the axial apex. Means (and standard deviations) for the best fit RGP base curve, axial steep SIM-K, axial apex curvature, instantaneous steep SIM-K, and instantaneous apex curvature were 52.00 D +/- 5.92 D, 50.93 D +/- 5.94 D, 54.81 D +/- 7.14 D, 49.19 D +/- 5.54 D, and 56.86 D +/- 7.89 D, respectively. Statistical comparisons revealed axial steep SIM-K to best represent the RGP base curve for the entire group. Similarly, in the subset of patients with apex decentrations within 1 mm of the map center, the axial steep SIM-K best represented the RGP base curve. In the remaining decentered cone subset, the axial apex curvature best represented the base curve. CONCLUSIONS: Axial and instantaneous maps differ significantly in apical position and apex curvature in keratoconus. Although instantaneous radius may better represent corneal shape, axial curvatures better predict the final RGP base curve in keratoconus patients.

Algorithms↗
PubMed1998

Baseline findings in the Collaborative Longitudinal Evaluation of Keratoconus (CLEK) Study.

PURPOSE: To describe the baseline findings in patients enrolled in the Collaborative Longitudinal Evaluation of Keratoconus (CLEK) Study. METHODS: This is a longitudinal observational study of 1209 patients with keratoconus enrolled at 16 clinical centers. Its main outcome measures are corneal scarring, visual acuity, keratometry, and quality of life. RESULTS: The CLEK Study patients had a mean age of 39.29+/-10.90 years with moderate to severe disease, assessed by a keratometric-based criterion (95.4% of patients had steep keratometric readings of at least 45 D) and relatively good visual acuity (77.9% had best corrected visual acuity of at least 20/40 in both eyes). Sixty-five percent of the patients wore rigid gas-permeable contact lens, and most of those (73%) reported that their lenses were comfortable. Only 13.5% of patients reported a family history of keratoconus. None reported serious systemic diseases that had been previously reported to be associated with keratoconus. Many (53%) reported a history of atopy. Fifty-three percent had corneal scarring in one or both eyes. CONCLUSIONS: Baseline findings suggest that keratoconus is not associated with increased risk of connective tissue disease and that most patients in the CLEK Study sample represent mild to moderate keratoconus. Additional follow-up of at least 3 years will provide new information about the progression of keratoconus, identify factors associated with progression, and assess its impact on quality of life.

Adult↗
PubMed1998

Three-dimensional scanning electron microscopic study of keratoconus corneas.

OBJECTIVE: To examine the 3-dimensional collagen fibril organization in the Bowman layer of keratoconus corneas. METHODS: Eight keratoconus corneas, 8 corneas with other diseases, and 5 normal human corneas were studied. A cell maceration method in combination with scanning electron microscopy was used to examine the collagen network in the Bowman layer. RESULTS: In normal corneas, the surface of the Bowman layer was smooth and collagen fibrils were regularly arranged. By contrast, sharply edged defects in the Bowman layer were found in keratoconus corneas. Lattice-like configurations of the ruptured Bowman layer and collagenous scar tissue were observed, to varying degrees, in all keratoconus corneas examined. None of the other diseased corneas exhibited the ruptures. CONCLUSIONS: Scanning electron microscopy demonstrated alterations in the Bowman layer specific to keratoconus. Fragmentation of the Bowman layer may be an early change leading to keratoconus conditions.

Adolescent↗
PubMed1984

Keratoconus with pellucid marginal corneal degeneration.

Examination of 1,625 Japanese patients with keratoconus and 20 patients with pellucid marginal corneal degeneration showed that 17 patients with pellucid degeneration also had keratoconus in the central portion of the cornea. Three patients with pellucid degeneration without keratoconus showed bilateral involvement. Among the 17 cases of pellucid degeneration with keratoconus, eight patients showed bilateral involvement and the others showed unilateral involvement. Pellucid marginal corneal degeneration with or without keratoconus may be a variant of keratoconus or a different manifestation of the same etiologic factor.

Adult↗