ISSN:1395-3907    

DOI:10.1111/j.1600-0420.1996.tb00671.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

ABSTRACTSixty‐two patients from three large Danish families with autosomal dominant optic atrophy were clinically examined, and retrospective follow‐up was made on 30 patients. We found great inter‐ and intrafamiliar variation in visual acuity and visual decline. Onehundred and seventy‐five chromosomal markers were analyzed in 118 family members. Linkage was demonstrated between the disease gene (OPA1) and the microsatellite markers D3S1314, D3S1262, D3S1265 and D3S1601, with the highest Lod score to D3S1601 Z = 11.75. All markers are located on chromosome 3q in the telomeric area, the most probable location for the OPA1 gene being D3S1601‐OPA1‐D3S1265. Using data from the Danish Family Register of Hereditary Eye Diseases, the minimum prevalence rate was estimated to 1:12.301, making DOA the most common hereditary op

ISSN:1395-3907    

DOI:10.1111/j.1600-0420.1996.tb00672.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

ABSTRACTMaximal 0.5‐Hz and cone 30‐Hz ERG responses were recorded from 19 patients showing a Laurence‐Moon‐Bardet‐Biedl (LMBB) phenotype. Off‐line averaging of 80 to 100 iterations was routinely performed. When needed, our previously described low‐noise techniques and off‐line fast Fourier transform procedures were used. The maximal ERG response was non‐detectable in 52.6% of cases. About half of the recordable signals were below 5% of the lower normal amplitudes. Cone 30‐Hz ERGs were measurable in 64.7% of cases. Of these, 63% of tracings were below 5% of the lower normal range. In most cases no dystrophic pattern was definable, due to severe reduction of both signals. Statistical analyses showed no correlation between ERG amplitudes and residual visual field areas. Clinical and electroretinographic observations suggest that retinopathy in most LMBB patients is a widespread form of degeneration, initially affecting rods but rapidly involving cones as well. However, there are also cases with a clear‐cut cone‐rod pattern, with fairly well preserved maximal ERG responses. The lack of correlation between maximal ERG responses and visual field residual areas, different from non‐syndromic retinitis pigmentosa (RP) patients, could be related either to a low reliability of visual field testing in LMBB patients or to mechanisms accounting for the ongoing retinal degeneration in LMBB syndrome that are different from those of pure RP. Variable findings are in line with the documented genetic hete

ISSN:1395-3907    

DOI:10.1111/j.1600-0420.1996.tb00673.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

ABSTRACTA method is described for measuring internal ocular surface (posterior cornea, anterior and posterior crystalline lens) astigmatism. This involves the use of videokeratography, A‐scan ultrasonography, and autorefractometry along with multi‐meridional phakometric measurements of Purkinje images I (anterior corneal surface) II (posterior corneal surface) and IV (posterior lens surface). Data was collected from both eyes of 66 subjects. Right and left eyes exhibited similar mean levels of astigmatism from the posterior corneal surface (R + 0.21 DC axis 82°; L + 0.22 DC axis 80°), anterior lens surface (R + 0.52 DC axis 8°; L + 0.49 DC axis 165°) and posterior lens surface (R + 1.48 DC axis 99°; L + 1.16 DC axis 90°). It was generally found that astigmatism arising from the anterior corneal and lens surfaces in conjunction with intraocular distance effectivity are almost completely compensated for by the posterior corneal and lens surfaces. Repeatability was assessed on 20 subjects. Although the methods is prone to accumulated experimental errors, these are random in nature so that the difference between repeat group averaged data never exceeded ± 0.27 DC cylindrical component and ± 6° c

ISSN:1395-3907    

DOI:10.1111/j.1600-0420.1996.tb00674.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

ABSTRACTIntegrin complex α6β4is a component of the hemidesmosome. In the unwounded cornea both the integrin subunits face the laminin‐containing basement membrane, but the α6subunit is also located between the basal cells. While the migrating epithelium is known to be without hemidesmosomes, we investigated the distribution of α6β4during epithelial healing. Epithelial abrasion 7.5 mm in diameter was mechanically created. The rabbits were killed 1–24 h or 2, 3, or 7 days later. Monoclonal antibodies against α6, β4, and laminin A were used to detect their distributions by immunohistochemistry. Positive immunostaining for laminin A on the surface of the unepithelialized stroma indicated that basement membrane was intact after the epithelial abrasion. Three hours after corneal wounding, α6was detectable around the entire cell up to the leading edge of the migrating epithelium. In the peripheral wound, α6was also prominently present around the basal and suprabasal cells with only the superficial cell layers being negative. The β4subunit showed a dissimilar distribution; it was not detectable subjacent to the leading edge. After 1 h the immunoreaction for the β4subunit had faded 15–20 μm peripheral to the wound margin. Thereafter the subepithelial band was segmentally reassembled, starting from the periphery and progressing toward the central area of the wound. One week after epithelial wounding, immunolabeling for both integrin subunits was indistinguishable from that of the control cornea. Our results indicate that in addition to the β4in the rabbit cornea, the α6subunit is also complexed with another β subunit during the epithelial healing phase. The results also suggest that basal cells 15–20 μm peripheral to the wound margin disassemble their HDs prior t

ISSN:1395-3907    

DOI:10.1111/j.1600-0420.1996.tb00675.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

ABSTRACTSubretinal ingrowth of choroidal fibroblasts is of importance in several clinical settings such as age‐related macular degeneration or after traumas such as laser treatment and infection. In the present investigation we describe a method for isolation and propagation of human choroidal fibroblasts in vitro. Outgrowth from the outseeded choroid was studied by means of phase contrast microscopy, scanning electron microscopy and by immunohistochemical methods. The secondary cultures were found to contain almost only fibroblasts (more than 95% of the cells), some contaminating retinal pigment cells were also found. Choroidal fibroblasts contribute to the fibrovascular subretinal scarring process, and our cell culturing system seems to be a suitable tool for studying factors regulating the behaviour of these cells in vitr

ISSN:1395-3907    

DOI:10.1111/j.1600-0420.1996.tb00676.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

ABSTRACTThis study examines the effect of transforming growth factor beta 1 and 2 (TGF‐beta 1 and TGF‐beta 2) on the proliferation of human Tenon's capsule fibroblasts in tissue culture. Both TGF‐beta 1 and TGF‐beta 2 were shown to stimulate proliferation at 1000 and 100 pg/ml concentrations. We discuss the significance of these findings with respect to wound healing following trabeculectomy, and postulate why some eyes are more at risk of failure than

ISSN:1395-3907    

DOI:10.1111/j.1600-0420.1996.tb00677.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

ABSTRACTThe retinal vessels from seven diabetic patients and from six age‐matched normal controls were studied qualitatively and quantitatively using various histological staining techniques. In diabetic patients the walls of retinal arterioles and capillaries showed significantly more staining than normals for periodic acid Schiff (neutral glycoproteins), Sirius red (connective tissue), and for Alcian blue at pH 2.6, pH 5.8 and at pH 5.8 combined with MgCl2in concentrations less than 0.9 M (acid mucopolysaccharides). In the retina from diabetic patients there was no difference between the number of capillaries staining with these dyes in areas of vascular occlusion and in adjacent control areas. Furthermore, in areas of vascular occlusion, the material accumulated centrally to occlude the lumen of ghost vessels did not stain with any of the dyes used. A homogenous material, accumulated in the outer retina in areas of vascular occlusion in the retina from diabetic patients, only stained with Alcian blue at pH 5.8 combined with MgCl2in concentrations less than 0.4 M, suggesting a different molecular composition from the Alcian blue material accumulated in the retinal vascular walls. The findings are in accordance with the knowledge that basement membranes of retinal vessels are thickened in diabetes mellitus. However, the findings also indicate that basement membrane thickening cannot fully account for vascular occlusion in diabetic retinopath

ISSN:1395-3907    

DOI:10.1111/j.1600-0420.1996.tb00678.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

ABSTRACTGlutathione peroxidase activities and malondialdehyde levels were measured in the homogenated anterior segment of rat eyes with endotoxin induced acute anterior uveitis in euthyroid, hyperthyroid and hypothyroid rats. Malondialdehyde concentrations were found to be significantly increased (p<0.0005) and glutathione peroxidase activities significantly decreased (p<0.01) in the hyperthyroid group when compared with controls. Malondialdehyde concentrations of the hypothyroid rat eyes were higher than the control group (p0.05). These results suggest that excess or deficiency of the thyroid hormones cause alterations in the malondialdehyde levels and glutathione peroxidase activities of the rat eyes in endotoxin induced uveitis, and hyperthyroidism may increase the oxidative stress in endotoxin induced acute anterior uveitis.

ISSN:1395-3907    

DOI:10.1111/j.1600-0420.1996.tb00679.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

ABSTRACTThe blepharophimosis‐ptosis‐epicanthus inversus syndrome is characterized by shortening of the horizontal orbital fissure (blepharophimosis), congenital ptosis and epicanthus inversus. The condition may occur either as an autosomal dominant trait (blepharophimosis‐ptosis‐epicanthus inversus syndrome types 1 and 2), or sporadically. Blepharophimosis‐ptosis‐epicanthus inversus syndrome type 1 is associated with female infertility. Mental subnormality may occur, especially in the sporadic cases. Chromosome analysis from a few patients suggests that the genetic defect causing the syndrome is localized to chro

ISSN:1395-3907    

DOI:10.1111/j.1600-0420.1996.tb00680.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY