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The 2 rare entities, pigmentary dispersion syndrome and pigmented pattern dystrophy of the retinal pigment epithelium, were found in a young male patient. Visual function was undisturbed.
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Five patients with retinitis pigmentosa (RP) with probable autosomal recessive inheritance have been identified in whom there is relative preservation of retinal pigment epithelium adjacent to and under retinal arterioles despite a panretinal degenerative process. All the patients were hypermetropic, though patients with RP tend to be myopic. This implies that there is a factor associated with retinal arterioles which locally retards the RP process in these patients. It may be appropriate to look for the PPRPE pattern in hypermetropic RP patients.
BACKGROUND: A peripheral retinal pigment epithelial tear and an extensive exudative retinal detachment caused by choroidal leakage from the denuded Bruch's membrane are extremely rare. A peripheral retinal pigment epithelial tear has not been reported in an eye with retinochoroidal folds after blunt ocular trauma. METHODS: Case report. RESULTS: The course of a large nasal peripheral retinal pigment epithelial tear that occurred after blunt ocular trauma in a patient with retinochoroidal folds was followed. The inferior retinal detachment caused by leakage from the denuded Bruch's membrane following the development of the tear gradually worsened. Initial treatment with cryotherapy was ineffective, but the retinal detachment eventually resolved after the patient underwent sclerectomy and sclerostomy. CONCLUSION: A large peripheral retinal pigment epithelial tear can occur in patients with retinochoroidal folds following blunt ocular trauma, and extensive retinal detachment can be induced. Sclerectomy and sclerostomy can be beneficial in patients with an extensive exudative retinal detachment caused by choroidal leakage from the denuded Bruch's membrane.
Retinal pigment epithelial cells carry out phagocytosis and digestion of material shed from the photoreceptor outer segments. In this process, the integrity of lysosomal enzymes is of major importance. In the present study the effects of tamoxifen, toremifene and chloroquine on the activity of two lysosomal enzymes (cathepsin D and N-acetyl-beta-D-glucosaminidase) in the retinal pigment epithelial cells were studied. Retinal pigment epithelial cells from pig eyes were cultured for two weeks in Dulbecco's Modified Eagle Medium, after which the cells were exposed to 1-40 microM concentrations of tamoxifen citrate, toremifene citrate and chloroquine diphosphate. To eliminate possible medium-borne oestrogenic mechanisms, the test was repeated using phenol red-free medium with charcoal-stripped fetal calf serum. The exposure time was one week, after which the lysosomal enzymes cathepsin D and N-acetyl-beta-glucosaminidase were determined. Cellular injuries were assessed by quantifying the leakage of lactate dehydrogenase into the culture medium. Cathepsin D and N-acetyl-beta-D-glucosaminidase showed different sensitivities to tamoxifen, toremifene and chloroquine. The main lysosomal protease cathepsin D was more sensitive than N-acetyl-beta-D-glucosaminidase to the effects of tamoxifen and toremifene, possibly due to their antioestrogenic properties. The phenol red-free medium with charcoal-stripped serum seemed to make the drugs more effective than the reference medium. Chloroquine had only a minor effect on the lysosomal protease cathepsin D, but a clearer effect could be seen on N-acetyl-beta-glucosaminidase.
OBJECTIVE: To investigate mRNA expression of vascular endothelial growth factor (VEGF) and its receptor FLK-l in iris pigment epithelium (IPE) cells and retinal pigment epithelium (RPE) cells, and the feasibility of transplantation of autologous IPE cells to substitute RPE in the treatment of age-related macular degeneration (AKMD) and diseases of RPE dysfunction. METHODS: IPE and RPE cells were isolated from pigmented rabbits. The expression of mRNA for VEGF and FLK-l were detected by reverse transcription polymerase chain reaction (RT-PCR). RESULTS: Rabbit IPE and RPE cells express VEGF mRNA and FLK-l; the express of VEGF and FLK-1 mRNA in IPE cells was relatively low compared with the expression in RPE cells. The amount of VEGF mRNA in IPE cells was 44.62% of RPE cells, and amount of FLK-l mRNA was 52.36% of RPE cells- The results of the differences of expression of VEGF and FLK-l mRNA between IPE and RPE cells was statistically significant (t-test, P < 0.001). CONCLUSIONS: VEGF and FLK-l mRNA in IPE cells express is relatively low compared with the expression in RPE cells, and the results suggest that IPE cells could be more beneficial than RPE cells in the transplantation into the subretinal space.
PURPOSE: To determine whether hypothermia of 8 degrees C can protect cultured human retinal pigment epithelial (ARPE-19) cells and rat retinal ganglion cells (RGC-5) against trypan blue (TB) toxicity. DESIGN: Laboratory investigation. METHODS: ARPE-19 cells and RGC-5 were exposed to balanced salt solution as controls, and 0.05% and 0.5% TB at 37 degrees C, and at 8 degrees C for one minute. The percentage of surviving cells was determined by the resazurin test. RESULTS: TB induced a statistically significant decrease in the percentage of ARPE-19 cells surviving at 0.5% TB at 37 degrees C (P < .01). Conversely, TB induced a statistically significant decrease in the percentage of RGC-5 surviving at all conditions except for 0.05% TB at 8 degrees C (0.05% 37 degrees C; P < .05, 0.5% 37 degrees C and 8 degrees C; P < .01). CONCLUSIONS: These results indicate that reducing the temperature to 8 degrees C has a protective effect against the TB toxicity for ARPE-19 cells and RGC-5 in culture.
Retinal pigment cells were dislodged from normal monkey eyes and incubated in glass-slide chambers. All viable pigment cells adhered strongly to glass. They demonstrated surface receptors for the Fc portion of immunoglobulin G and for the third component of complement by selectively binding and phagocytizing antibody or complement-coated erythrocytes. These phagocytic cells with receptors were identified as retinal pigment cells by characteristic ultrastructural features. Thus, retinal pigment cells, which are generally believed to be derived from neural tissue, are not only scavengers of photoreceptor cell debris, but also have surface receptors and phagocytic functions that may be important in ocular defense.
Acute retinal pigment epitheliitis is an infrequently described macular disorder of unknown cause that affects healthy young adults. Acute symptoms include either reduced visual acuity or central scotoma. Ophthalmoscopy discloses discrete pigment clumps with surrounding hypopigmented halos in the center of the macula. Inflammatory signs are mild or absent. The hypopigmented areas show transmission hyperfluorescence without leakage on fluorescein angiography. Symptoms subside spontaneously although retinal pigment epithelial (RPE) changes are usually permanent. Central serous choroidopathy, macular edema, or permanently decreased vision have not developed in any of the eight patients we have followed for up to 8 years. Accurate identification of retinal pigment epitheliitis as a specific clinical entity is important because of the good prognosis without treatment.
PURPOSE: To evaluate the toxicity of trypan blue on retinal cells in vitro. METHODS: Human retinal pigment epithelial cells (ARPE-19) and rat neurosensory retinal cells (R28) were grown in tissue culture and treated with four different concentrations of trypan blue (0.1%, 0.05%, 0.025%, and 0.0125%), in combination with surgical light exposure (0, 5, or 10 minutes). Cell viability, mitochondrial function, and DNA synthesis were measured by trypan blue dye-exclusion assay, mitochondrial dehydrogenase assay, and tritiated [3H] thymidine incorporation, respectively. RESULTS: ARPE-19 and R28 cells exposed to trypan blue with or without illumination did not show any significant decrease, either in cell viability by the dye-exclusion assay or in [3H] thymidine incorporation. R28 cells exposed to 0.1% trypan blue with and without light showed a significant reduction of mitochondrial dehydrogenase activity (P <0.05). ARPE-19 cells exposed to trypan blue, with or without light, did not show any significant decrease in mitochondrial dehydrogenase activity. CONCLUSIONS: This study suggests that rat neurosensory retina (R28) cells are more sensitive than human RPE (ARPE-19) cells to trypan blue. ARPE-19 cells showed no evidence of toxicity with any of the three assays, but R28 cells showed evidence of toxicity with the mitochondrial dehydrogenase assay at the higher doses and light-exposure times studied. Clinical studies must be conducted to determine the safety and efficacy of staining of the inner limiting membrane with trypan blue.
PURPOSE: This study was designed to examine whether retinal function can be rescued by allogeneic normal retinal pigment epithelial (RPE) grafts in Royal College of Surgeons (RCS) with retinal degeneration and, if so, whether this rescued function can be measured and followed by recording the corneal electroretinogram (ERG). METHODS: RPE donors were RCS-Long Evans crossbred F1 rats with phenotypically normal retinas. Half an RPE sheet was implanted in the subretinal space of RCS rats at postnatal day 20. The fundi of the recipients' eyes were examined, and the corneal ERGs were recorded. The eyes were also examined histologically. RESULTS: The RPE grafts were identified by fundus examination in all 21 recipients. No clinical or histologic evidence of inflammation was detected in the media or the retina of the host eye. Eighteen of 21 (86%) recipients showed rescued corneal ERG function. In nine recipients, the PIII response in the grafted eye was significantly greater than in the nongrafted eye. In the other nine recipients, the ERG in the grafted eye showed a b-wave and an a-wave, whereas no b-wave was detected in the nongrafted eye. Recipients of the sham operation (n = 13) revealed no ERG function rescue. To determine long-term corneal ERG function in RPE recipients, 8 of 18 animals in which ERG function was rescued were randomly selected for continued observation. These recipients sustained rescued ERG function for 16 to 17 weeks, at which time the experiment ended. CONCLUSION: Results indicate that retinal function of degenerative RCS rats, as measured by corneal ERG, can be rescued by implantation of allogeneic normal RPE into the subretinal space of the eye. Furthermore, this rescued function can be followed up over a relatively long period of time, thus providing a useful model for studying the functional changes of RPE allografts resulting from either immunologic or neurobiologic influences.
Acute retinal pigment epitheliitis appears to be a specific discrete process of unknown etiology. It is generally unilateral but can be recurrent and can affect both eyes. Eyes in which the disease has run its course exhibit little or no unusual ophthalmoscopic or visual function abnormalities. The actue phase of the disease is localized to the macular area. The lesions are localized in the retinal pigment epithelium as shown by slit-lamp and contact-lens examination, the small lesions more or less surrounding the fovea. They vary from tiny grayish to black spots, which may become surrounded by a lighter halo. Fluroescein angiography is normal in acute phases but the halo-like areas may represent "window defects" with hyperfluorescence in later stages.
Congenital hypertrophy of the retinal pigment epithelium (CHRPE) is known to occur in patients with familial adenomatous polyposis. Its relation to the course of the retinal blood vessels is emphasized in this publication. A 15-year-old girl with familial polyposis coli showed a longitudinal strip of whitish change following the course of a superior nasal artery of the left eye, falsely appearing to enclose the blood vessel like a sheath. A 34-year-old woman from another family with polyposis coli also showed a longitudinally orientated area of hypo- and hyperpigmentation close to temporal retinal veins. Two further patients revealed hyperpigmentation of the RPE under retinal vessels. It is hypothesized that these changes indicate an effect of the hypo- and hyperpigmentations of the RPE on the development of retinal vessels. In four patients, multiple dotlike hyperpigmentations were found in the extreme periphery of the retina; however, the bigger patchy hyperpigmentations were predominantly located in the midperiphery of the retina.
Two patients with bilateral chronic retinal pigment epitheliopathy are presented. Both patients had large areas of pigment epithelial decompensation and small pigment epithelial detachments at the posterior pole. In the macula subretinal fluid was scanty, but the first patient developed an extensive bullous retinal detachment with shifting of subretinal fluid with changes in posture in both eyes, the second patient had similar detachment in one eye. The etiology of the pigment epithelial disorder remained unknown. No inflammatory cells were found in the vitreous specimen or subretinal fluid in the first patient. Treatment with peroral corticosteroids alone or in combination with azathioprine and cyclosporin A, as well as surgery for retinal detachment in one eye, proved unsuccessful. Argon laser coagulation of the decompensated areas in the macula resulted in resorption of subretinal fluid and reattachment of the exudative detachment.
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PURPOSE: The accumulation of age pigment, or lipofuscin, in postmitotic cells appears to be a universal feature of the aging process in animals. In mammals, the lipofuscin content of the retinal pigment epithelium (RPE) increases progressively during senescence. Dietary restriction has been shown to slow the rate at which many biologic parameters change during aging. Experiments were conducted to determine if dietary restriction alters the rate of age pigment accumulation in the RPE. METHODS: Male Wistar rats were placed on one of three dietary regimens starting at weaning. One group was fed a nutritionally complete diet ad libitum. Another group was fed the same diet but was only allowed to consume 60% as much food daily as the ad libitum group ate. The final group was fed ad libitum a nutritionally complete diet that had a lower caloric density per gram than the diets fed to the other animals primarily because of the replacement of carbohydrate with oat fiber. Ultrastructural morphometric analysis was used to determine the RPE age pigment content in the first group at 6 months of age, and in all of the groups at 18 months of age. RESULTS: Dietary restriction, achieved either by reducing total food intake or by reducing the caloric content of the diet, resulted in significant decreases in RPE lipofuscin accumulation. CONCLUSIONS: Dietary restriction provides a relatively simple means by which RPE age pigment content can be modulated. This should prove useful in assessing the role of RPE lipofuscin accumulation in age-related retinal disorders. That the oat fiber diet fed ad libitum was almost as effective as restriction of total food intake in slowing RPE age pigment accumulation indicates that the effect of restricted caloric intake is not mediated by almost constant hunger.
The beta-glucuronidase-deficient mucopolysaccharidosis type VII (MPS VII) mouse accumulates partially degraded glycosaminoglycans in many cell types, including retinal pigmented epithelial (RPE) cells in the eye. This lysosomal storage in RPE cells leads to progressive retinal degeneration and reduced function as measured by flash electroretinography (ERG). The impact of AAV-mediated intraocular gene therapy on pathology and retinal function was examined in normal and MPS VII mice treated at 4 weeks of age, when lysosomal storage is evident but functional impairment is minimal in affected animals. At 16 weeks, an age at which untreated MPS VII mice have advanced histologic lesions and significantly reduced ERG amplitudes, treated eyes had nearly normal levels of beta-glucuronidase activity, preservation of cells in the outer nuclear layer of the retina, and decreased lysosomal storage within the RPE. The AAV-treated MPS VII mice also had significantly increased dark-adapted ERG amplitudes compared to untreated MPS VII mice. Although retinal function was improved, the efficacy of the treatment depended heavily on parameters related to the injection procedure, such as the injection volume, injection site, and vector dose. These data suggest that intraocular AAV-mediated therapy may be efficacious for treating the retinal disease associated with certain lysosomal storage diseases.
OBJECTIVE: To determine the gene expression profiles of primary retinal pigment epithelium (RPE) and iris pigment epithelium (IPE) using microarrays. METHODS: Primary RPE and IPE from 6 human donor eyes were collected, and total RNA was isolated. Differences in gene expression were determined using a human genechip (human U95Av2 [12 600 probes]; Affymetrix Inc, Santa Clara, Calif). RESULTS: Hierarchical cluster analysis differentiated the gene expression profiles of RPE and IPE clusters into 2 distinct groups. A mean +/- SD of 5308 +/- 416 gene probes were expressed in RPE vs 6130 +/- 205 in IPE. Sixty-eight genes were expressed only in RPE; 154 genes were expressed only in IPE. Twenty-two additional genes had greater than 3-fold increased expression in RPE vs IPE, and 147 genes had greater than 3-fold decreased expression in RPE vs IPE. CONCLUSION: There are major differences in the gene expression profiles of primary RPE vs IPE. Clinical Relevance The different gene expression profiles of primary RPE vs IPE harvested from the same donor eyes infer that it may be difficult for IPE to replace all aspects of damaged RPE function in transplantation studies.