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Biomedical subjects

C M Mooy

Publications and source records attributed to C M Mooy.

At least 55 records · Page 3Linked to original sources

Increased prevalence of disciform macular degeneration after cataract extraction with implantation of an intraocular lens.

After cataract extraction with implantation of an intraocular lens the increased transmission of ultraviolet and blue light may accelerate the development of age-related macular degeneration by producing free radicals in the retina. The maculae of 82 randomly selected postmortem human pseudophakic eyes and 16 fellow phakic eyes were examined by light microscopy. The presence of a basal laminar deposit, hard and soft drusen, thickening and calcification of Bruch's membrane, geographic atrophy, subretinal neovascularisation, and disciform scars was assessed in a standardised way. An age-matched series of 126 postmortem phakic eyes was used as control group. There was no difference between the two groups, except for a higher prevalence of hard drusen (exact trend test, p = 0.038) and disciform scars for the pseudophakic eyes (Fisher's exact test, p = 0.007). There was no significant correlation between either age-related changes in the macula or disciform degeneration and the length of time between cataract surgery and death. No significant difference was found between pseudophakic eyes with or without ultraviolet filter. These findings do not confirm that disciform scar formation is caused by an increase in ultraviolet or blue light.

Age Distribution↗

Morphometric analysis of Bruch's membrane, the choriocapillaris, and the choroid in aging.

PURPOSE: To quantify changes in choriocapillary density and in thickness of Bruch's membrane, the choriocapillaris, and the choroid in 95 unpaired, histologically normal human maculae aged 6 to 100 years and in 25 maculae with advanced age-related macular degeneration. METHODS: Light microscopic, computer-aided, morphometric quantitative analysis. RESULTS: In ten decades, Bruch's membrane thickness increased by 135%, from 2.0 to 4.7 microns; the choriocapillary density decreased by 45%; the diameter of the choriocapillaris decreased by 34%, from 9.8 to 6.5 microns; and the choroidal thickness decreased by 57%, from 193.5 to 84 microns in normal maculae. In maculae with basal laminar deposit, geographic atrophy, or disciform scarring, the density of the choriocapillaris was 63%, 54%, and 43% of normal and the choriocapillary diameter was 81%, 73%, and 75% of normal, respectively. Choroidal thickness remained unchanged. CONCLUSIONS: Thickness of Bruch's membrane was only related to age (rs = 0.63) and not to age-related atrophy of the choriocapillaris. Age was also the strongest factor related to choriocapillary density (rs = -0.58). In advanced stages of age-related macular degeneration, the decrease in choriocapillary density and diameter was significantly larger than in normal maculae, but the thickness of the choroid and Bruch's membrane was the same. The latter was significantly thinner (81% of normal) in disciform scarring.

Adolescent↗

Analysis of carbohydrate structures in basal laminar deposit in aging human maculae.

PURPOSE: To analyze carbohydrate structures in basal laminar deposit (BLD), an extracellular material that accumulates between the retinal pigment epithelium (RPE) and Bruch's membrane. BLD has been shown to correlate positively with visual loss in age-related macular degeneration. METHODS: Thirteen postmortem human maculae with BLD were histochemically examined by light microscopy using the monoclonal antibody HNK-1 and seven lectins; canavalia ensiformis (ConA), soybean agglutinin (SBA), wheat germ agglutinin (WGA), dolichos bifloris (DBA), ulex europaeus (UEA-I), ricinius communis agglutinin I (RCA-I), and peanut agglutinin (PNA). Three maculae were stained with polyclonal antibodies against laminin and collagen type IV. RESULTS: BLD was exclusively stained by DBA and SBA, whereas Con A, WGA, UEA-I, RCA-I, and HNK-1 stained various other structures in the human macula as well. The main part of the BLD adjacent to Bruch's membrane stained with these lectins and the monoclonal antibody HNK-1, whereas only a small part of the BLD adjoining the RPE stained with antibodies against laminin and collagen type IV. Drusen stained neither with any lectin nor with any antibody. CONCLUSIONS: DBA and SBA, which bind specifically to an alpha-D-GalNAc moiety, are specific markers for the light-microscopic detection of BLD in human macular tissue. Furthermore, the authors conclude that BLD contains several carbohydrate structures other than the carbohydrate moieties on laminin and collagen type IV. If drusen contain carbohydrate structures, these must be different from those in BLD.

Aged↗

A chicken embryo model to study the growth of human uveal melanoma.

In vitro cultured human uveal and skin melanoma cells were injected into the chicken embryonal eye at a stage when the immune system was not yet mature. The melanoma cells were accepted as part of the organism by the host. Even single melanoma cells could be traced by morphological methods as well as by immunohistochemical markers, such as S100, HMB-45, NKI/C3 and HNK-1. We found tumors in 20 and 40 percent of the embryos injected with uveal melanoma and skin melanoma, respectively. The embryos did not exhibit abnormal development of the eye as a result of the microinjection and had a high survival rate (90 and 60%, respectively) during embryogenesis. With this model for uveal melanoma the growth and possibly the metastatic behavior of human uveal melanoma cells can be studied.

Animals↗

Ocular findings in cerebro-ocular-myopathy syndrome (COMS). A possible role of growth factors?

The clinical and histopathological findings are described in a case of cerebral and ocular abnormalities associated with (congenital) muscular dystrophy. Histopathological examination of the eyes revealed a fetal configuration of the anterior chamber angle, elongated ciliary processes, cataract, persistence of primary hyperplastic vitreous and total retinal detachment with retinal dysplasia. The similarity of ocular findings in Walker-Warburg syndrome, muscle-eye-brain disease and Fukuyama's congenital muscular dystrophy, and the role of growth factors as a possible unifying (foetal) cause are discussed. This case has been presented at the meeting of the EOPS Verhoeff Society, Nürnberg 1991.

Abnormalities, Multiple↗

Basal laminar deposit in the aging peripheral human retina.

A basal laminar deposit (BLD) in the human macula has been described as an early sign of age-related macular degeneration. In some eyes with a BLD in the macula, light microscopic sections of the peripheral retina revealed almost similar deposits between the retinal pigment epithelium and Bruch's membrane. Because the exact pathogenesis of age-related macular degeneration and the origin of the BLD are unknown, we studied the ultrastructure of these peripheral sub-RPE deposits. Parts of the equatorial and peripheral regions of the retina of ten human eyes, with BLD-like deposits between the retinal pigment epithelium and Bruch's membrane, were examined by electron microscopy. In eight of these ten eyes the ultrastructure of these deposits was amorphous and finely granular. Five of the eight deposits also contained small amounts of long-spacing collagen. Ultrastructurally, the deposits were similar to an early type BLD in the macula. In the remaining two eyes, the deposits appeared to consist of flat, elongated drusen. Our findings indicate that a BLD can develop not only in the macula but also in the peripheral region of the retina.

Aged↗

Early stages of age-related macular degeneration: an immunofluorescence and electron microscopy study.

In subretinal neovascularisation capillaries originating from the choriocapillaris must cross Bruch's membrane to reach the subretinal pigment epithelial space. Thus gaps in Bruch's membrane have to be formed before subretinal neovascularisation. Histological examination of eyes with subretinal neovascularisation or disciform scars has shown macrophages adjacent to thin areas and ruptures in Bruch's membrane. This has been interpreted as phagocytosis of Bruch's membrane. The purpose of this study was to investigate whether immune complex depositions can be detected in maculae with early stages of age-related macular degeneration and to explain the macrophage reaction before the disciform reaction. A series of 20 human maculae were examined by direct immunofluorescence light microscopy to detect the presence of immune complexes with antibodies directed against immunoglobulins, fibrinogen, and complement factors. Transmission electron microscopy on several maculae was performed to identify the macrophages. Macrophages were observed in close relation to the readily recognisable long spacing collagen, which suggested that long spacing collagen was selectively internalised by these cells. Definite immune complex depositions were not found in basal laminar deposits or drusen. Linear deposits of fibrinogen and complement were frequently found in the outer collagenous zone of Bruch's membrane. However, because of the absence of immunoglobulins, it seems unlikely that these non-specific deposits might cause chemoattraction of macrophages and play a role in the initial phase of the development of subretinal neovascularisation and disciform macular degeneration.

Adult↗

Immune deposits in iris biopsy specimens from patients with Fuchs' heterochromic iridocyclitis.

To investigate whether Fuchs' heterochromic iridocyclitis may be an immune complex vasculitis, we used an immunofluorescence technique to detect immunoglobulins and complement in iris biopsy specimens from nine patients with Fuchs' heterochromic iridocyclitis, 12 patients with other types of uveitis, and nine patients with glaucoma but without uveitis. No specific immune deposits were observed in the irises of the patients with Fuchs' heterochromic iridocyclitis. Immunoglobulin G, IgA, IgM, and complement were detected in patients with Fuchs' heterochromic iridocyclitis and patients with uveitis, and these results differed significantly (P less than .05) from the group without uveitis. The immune deposits were found only in the iris vessel walls. No light-microscopic evidence of an inflammatory vascular process could be detected. Further studies are necessary to investigate whether the immune reactants originate from the circulation or result from local formation.

Adult↗

Element analysis of the early stages of age-related macular degeneration.

The accumulation of basal laminar deposit (BLD) in the macula is considered to be a precursor of age-related macular degeneration. To learn more about the composition of BLD and the role of zinc in age-related macular degeneration, we investigated the elements in BLD, as well as in surrounding structures in 38 postmortem human maculae by electron-probe x-ray microanalysis. Basal laminar deposit and capillary vessel walls of the choriocapillaris appeared to contain no typical elements. Calcium, phosphorus, sulfur, zinc, and chlorine were detected in the lipofuscin granules in retinal pigment epithelium. Pigment granules of the retinal pigment epithelium and choroidal melanocytes contained predominantly sulfur and copper and, to a lesser degree, zinc, calcium, and iron. Local calcifications in Bruch's membrane were composed of large amounts of calcium and phosphorus and smaller amounts of zinc, iron, and chlorine. Metal-mirror fixation of the maculae, followed by freeze-drying and vapor fixation, showed additional amounts of sodium and potassium. From these experiments, no conclusions could be drawn about the origin of BLD. No relationship was found between the detection of zinc and the presence of BLD or drusen in the macula.

Adolescent↗

Histologic features of the early stages of age-related macular degeneration. A statistical analysis.

The age distribution, frequency, and correlation among histologic macular changes, including formation of a basal laminar deposit, drusen, and thickening and calcification of Bruch's membrane, were studied by light microscopy. The authors studied 182 unpaired postmortem human maculae from patients between 8 and 100 years of age. In addition, 45 maculae of contralateral eyes and the peripheral retina of 50 eyes were studied. In 92%, Bruch's membrane was thickened starting at age 19, and calcifications in this membrane were found in 59% starting at age 33. In 37% of the maculae, hard drusen were found starting at age 34. Soft drusen were found in 10% beginning at age 54. Basal laminar deposit was found in 39% of the maculae starting at age 40. All changes correlated strongly with age (P less than 0.0001). No sex differences were found. Fellow eyes showed similar aging changes (P less than 0.001). The presence of basal laminar deposit in the macula correlated with basal laminar deposit-like material in the peripheral retina (correlation coefficient, 0.39; P less than 0.003), whereas drusen in the macula correlated with drusen in the peripheral retina (correlation coefficient, 0.42; P = 0.001). Geographic atrophy was found in 6.6% of the eyes from subjects older than 70 years and subretinal neovascularization in 3.8%, especially in the maculae with basal laminar deposit.

Adolescent↗

Congenital glaucoma in a child with partial 1q duplication and 9p deletion.

A case of partial duplication of chromosome 1 (1q41-qter) and partial deletion of chromosome 9 (9p24-pter) with infantile congenital glaucoma is reported. The histopathology of the eyes is described. The clinical findings ascribed to trisomy 1q and partial monosomy 9p are summarized and compared to this case. As this is the second report of a patient with monosomy 9p24-pter and congenital glaucoma, it may indicate localization of a gene involved in congenital glaucoma in this region of the human genome.

Chromosome Deletion↗

Is basal laminar deposit unique for age-related macular degeneration?

The ultrastructural nature and distribution of basal laminar deposit, considered to be a precursor of age-related macular degeneration, were studied in 42 human maculae. Basal laminar deposit was found from age 19 years on, not only between the retinal pigment epithelial cells and their basement membrane but also more often on the choriocapillary side of Bruch's membrane. No direct relationship was found with other aging changes, such as calcifications in Bruch's membrane, accumulation of lipofuscin granules, or drusen in the macular area. Material similar to basal laminar deposit can be found in the trabecular system, in the cornea, and also in many other organs and tissues. On a structural and morphometrical basis, we think that basal laminar deposit is similar to fibrous long-spacing collagen and thus does not seem to be a purely ocular abnormality.

Adolescent↗

Neuropathological findings in muscle-eye-brain disease (MEB-D). Neuropathological delineation of MEB-D from congenital muscular dystrophy of the Fukuyama type.

Congenital muscular dystrophy (CMD) associated with cerebro-ocular dysplasia named muscle-eye-brain disease (MEB-D) is described in two sisters. Progressive hypotonia, mental retardation and severe visual failure appeared immediately after birth. Pathological examination demonstrated muscular dystrophy, hydrocephalus, type II lissencephaly and defective eye development of foetal origin. The great similarity of the clinical and neuropathological picture of both sisters is in agreement with an autosomal recessive inheritance. Neuropathological distinction between Fukuyama-CMD and MEB-D is a more severe and earlier cerebral developmental defect and the association with ocular dysplasia in MEB-D.

Brain↗

Peters' anomaly: an unusual case.

The authors report on a case of congenital bilateral protruding corneal opacities. Clinical and histological examination was consistent with a diagnosis of Peters' anomaly with a defect in the endothelium and Descemet's membrane but without abnormalities of iris and lens. Corneal grafting was performed on both eyes. During a follow-up period of one year the corneas have remained clear. However, surgical intervention for glaucoma has been necessary.

Consanguinity↗

Immunohistochemical analysis of iris biopsy specimens from patients with Fuchs' heterochromic cyclitis.

Using immunohistochemical techniques, we analyzed iris biopsy specimens from eight patients with Fuchs' heterochromic cyclitis, seven patients with various other types of uveitis, and eight glaucoma patients without uveitis. No specific abnormalities related to Fuchs' heterochromic cyclitis could be detected. Four of the patients with Fuchs' heterochromic cyclitis and four of the patients with uveitis showed evidence of an inflammatory cell infiltrate, which was a mixture of interleukin-2 receptor-negative T helper and suppressor cells, B lymphocytes, and plasma cells. Only an occasional T lymphocyte could be seen in two of the patients without uveitis. The class II antigen HLA-DR was expressed on iris stromal cells in every patient in the Fuchs' heterochromic cyclitis group and uveitis group and in six of the patients in the nonuveitis group. In six of the Fuchs' heterochromic cyclitis patients, including two without immunohistochemical evidence of inflammatory cell infiltrate, histologic abnormalities were present on hematoxylin and eosin sections.

Adult↗

Choroidal metastasis of oesophageal squamous cell carcinoma.

A case report of a choroidal metastasis from an oesophageal squamous cell carcinoma is described. The computerised tomographic, ultrasonographic, histologic and immunohistochemic findings are presented with a review of the literature.

Carcinoma, Squamous Cell↗