As afecções que acometem a via óptica anterior podem acarretar perda de fibras das células ganglionares retinianas, gerando alterações na camada de fibras nervosas da retina (CFNR). O objetivo deste trabalho foi o de comparar a capacidade da tomografia de coerência óptica (OCT-1), do OCT 3 (Stratus OCT) e do polarímetro de varredura a laser (GDx) em diferenciar olhos normais de olhos com atrofia em banda do nervo óptico e hemianopsia temporal, assim como, comparar o desempenho de tais tecnologias entre si. Foram estudados 37 indivíduos num total de 46 olhos divididos igualmente em dois grupos. O Stratus OCT, o OCT 1 e o GDx se mostraram capazes de identificar corretamente a redução global (average) da espessura da CFNR dos indivíduos com AB comparados com uma população normal. Observamos uma melhora progressiva das sensibilidades/especificidades e das AROC, com o desempenho melhor do OCT1 sobre o GDx e do Stratus OCT sobre o OCT 1 / Most diseases that affect the optic pathways present clinically with loss of one or more visual functions.When optic pathway injuries involve the anterior visual pathways they may cause loss of the retinal nerve fiber layer (RNFL) and "band atrophy" (BA). The purpose of this study was therefore to compare the ability of the optical coherence tomography (OCT) 1, the OCT 3 (Stratus OCT) and the scanning laser polarimetry (GDx) to differentiate normal eyes from eyes with BA of the optic nerve and temporal hemianopsia. Thirty-seven individuals in a total of 46 eyes divided equally in two groups were studied. Each one of the parameters, except the values from the nasal quadrant measured by the GDx, showed statistically significant difference when we compare normal patients with the BA group. The study also showed a gradual improvement of the sensibilities/specificities and of the AROC, with a better performance of the OCT1 compared to GDx and of the Stratus OCT compared to the OCT 1

O albinismo e uma alteracao genetica rara que compromete a producao de melanina. As alteracoes clinicas consistem na falta de pigmentacao da pele, cabelo e pelos. Apresenta alteracoes oftalmologicas importantes, que comprometem a acuidade visual de forma severa, na grande maioria dos casos. As alteracoes oftalmologicas sao, ametropias, nistagmo, rarefacao do epitelio pigmentado da iris e da retina, hipoplasia foveal e decussacao anomala do nervo optico. Este estudo foi dividido em tres diferentes partes com os seguintes objetivos principais: Parte 1: Analise comparativa entre o grau de transparencia da iris (GTI), o grau de transparencia da retina (GTR) e a espessura da regiao macular com a acuidade visual nos pacientes com albinismo. Parte 2: Teste de sensibilidade ao contraste espacial de luminancia e ofuscamento com lentes de contato filtrantes em ambientes claros e escuros. Parte 3: Avaliacao da visao de cores, utilizando\\se o teste de Pranchas de Ishihara e o Cambridge Color Test (CCT). Material\' e\' Métodos: Participaram do estudo 121 individuos albinos, com idade media de 31,18 (} 15,47) anos, o que totalizou 242 olhos. Os participantes foram divididos em diferentes grupos nas tres partes do estudo, alguns participaram de uma ou mais partes. Na primeira parte os participantes foram submetidos a consulta oftalmologica, classificacao do grau de transparencia da iris e da retina em uma nova classificacao baseada em quatro diferentes graus de transparencia, alem de avaliacao foveal pelo exame de Tomografia de Coerencia Optica (OCT). Na segunda parte, os participantes foram submetidos ao teste de contraste computadorizado, utilizando lentes de contato filtrante e transparentes, em ambientes claros e escuros. Nesta etapa foi avaliada a influencia da lente filtrante na sensibilidade de contraste visual dos individuos com albinismo. Na terceira parte, os participantes foram submetidos aos testes de Pranchas de Ishihara e de Cambridge Colour Test (CCT), para avaliacao da visao de cores nos pacientes albinos. Resultados: Os resultados foram os seguintes: Parte\'1: A correlacao entre AV logMAR e GTI foi positiva (+0,569) e significativa (p<0,001). A correlacao entre AV logMAR e GTR foi positiva (+0,531) e significativa (p<0,001). A correlacao entre AV logMAR e a espessura macular nao foi significativa (p=0,105). A correlacao entre GTI e GTR foi positiva (+ 0,627) e significativa (p<0,001). A correlacao entre espessura macular e GTI nao foi significativa (p=0,397). A correlacao entre espessura macular e GTR nao foi significativa (p=0,458). Parte 2: Houve melhora estatisticamente significante da sensibilidade ao contraste com as lentes filtrantes em relacao as lentes transparentes no ambiente de ofuscamento (claro), na frequencia de 0,3 cpg, 0,6 cpg e 1,0 cpg (ciclos por grau). Nao houve melhora significante nas frequencia 2,0 cpg e 4,0 cpg. Nao houve melhora estatisticamente significante da sensibilidade ao contraste de lentes filtrantes em relacao as lentes transparentes no ambiente escuro (penumbra) em nenhuma das frequencias espaciais estudadas. Parte\' 3: Todos os individuos com albinismo nao apresentaram nenhuma alteracao de visao de cores no teste de Pranchas de Ishihara. O CCT demonstrou piora no limiar de deteccao de cores significativa nos eixos protan (p=0,021) e deutan (p=0,017), mas nao houve diferenca no eixo tritan (p=0,212). Os testes estatisticos sugerem que uma amostra maior seja estudada para validacao dos resultados / Albinism is a rare genetic alteration that compromises the production of melanine in all body tissues. The clinical alterations are the lack of pigment in the skin and hair. It presents important ophthalmological changes that compromise the visual acuity, in most cases. The ophthalmological changes are: ametropia, nystagmus, iris pigmented epithelium rarefaction, retina pigmented epithelium rarefaction, foveal hipoplasia and abnormal optic nerve decussation. This study has been divided in three different parts and had the following main objectives: Part 1, comparative analysis of the iris transparency degree (ITD), the retina transparency degree (RTD) and macular thickness with the visual acuity in albino patients. Part 2: Spatial contrast sensitivity test of luminance and outshine wearing contact lenses with filters in light and dark environments. Part 3: color vision evaluation using the Ishihara Test and the Cambridge Color Test (CCT). For the study 121 individuals with albinism were included with an average of 31,18 years old } 15,47, a total of 242 eyes. The participants were divided in different groups in the three parts of the study, some participated in one or more parts. In the first part the participants have been through an ophthalmological exam, classification of the iris transparency degree (ITD) and the retinal transparency degree (RTD) by a new classification based in 4 different degrees of transparency. Foveal evaluation by the Optic Coherence Tomography exam (OCT) was made in the first part of this study. In the second part, the participants have been submitted to the computerized contrast test, using filtered and transparent contact lenses in light and dark environments. In this part the influence of the filtering lenses in the visual contrast sensitivity in the albinism patients has been evaluated. In the third part, the participants have been submitted to Ishihara Test and Cambridge Color Test in order to evaluate the color vision in albino patients. The results are as follows: Part\' 1: the correlation between visual acuity in Logarithm of the Minimum Angle of Resolution (logMAR) and ITD was positive (+0,569) and significant (p<0,001). The correlation between visual acuity in logMAR and RTD was positive (+0,531) and significant (p<0,001). The correlation between visual acuity in logMAR and the macular thickness was not significant (p=0,105). The correlation between ITD and RTD was positive (+ 0,627) and significant (p<0,001). The correlation between the macular thickness and the ITD was not significant (p=0,397). The correlation between the macular thickness and the RTD was not significant (p=0,458). Part\'2: There has been a statistically significant improvement of the contrast sensitivity wearing the filtering lenses in relation to the transparent ones in bright environment, in the frequencies of 0,3 cycles per degree (cpd), 0,6 cpd and 1,0 cpd. There has been no significant improvement in the frequencies 2,0 cpd and 4,0 cpd. There has been no statistically significant improvement in contrast sensitivity wearing the filtering lenses in relation to the transparent one in the dark environment (shadow) in any of the spatial frequencies studied. Part\' 3: all albinism patients did not present any color vision change in the Ishihara Test. The CCT has shown a worsening in the color detection threshold in the protan axis (p=0,021) and deutan axis (p=0,017), but there has been no difference in the tritan axis (p=0,212). The statistic tests show that a bigger sample is recommended to confirm part 3 results

O decorim e o biglicam são membros da família dos pequenos proteoglicanos ricos em leucina e possuem importantes funções no controle da proliferação, migração e invasão do citotrofoblasto extraviloso (TEV). O objetivo deste trabalho foi de caracterizar a expressão diferencial e a imunolocalização de decorim e biglicam em placentas humanas normais a termo (PNT), na placenta acreta (PA), na mola invasora (MI) e no coriocarcinoma (CO). Na PNT, as células deciduais apresentaram positividade para o decorim, enquanto o TEV foi negativo. O decorim foi fracamente expresso na matriz endometrial, mas negativo no fibrinoide do tipo matriz, enquanto foi positivo para biglicam. Na PA e na MI, o TEV mostrou positividade para decorim e biglicam. No CO, somente o citotrofoblasto foi positivo para ambos proteoglicanos. Portanto, o decorim e o biglicam são expressos diferencialmente em placentas normais e patológicas, sugerindo que os padrões de expressão desses proteoglicanos nas patologias estudadas indicam um papel na modulação da migração e da invasão do trofoblasto. / Decorin and biglycan are family members of the small leucine-rich proteoglycans family, and they have many functions as controlling proliferation, migration and invasion of extravillous trophoblast cells (EVT). The aim of this study was to characterize decorin and biglycan differential expression and immunolocalization in human normal term placenta (NTP), in placenta accreta (PA), in invasive mole (IM), and in choriocarcinoma (CH) samples. In PNT, deciduas cells were positive to decorin whereas EVT was negative. Decorin was faintly stained at endometrial matrix, but negative at matrix-type fibrinoid, although it was positive for biglycan. In PA and IM, the EVT was positive for decorin and biglycan. In CH, only cytotrophoblast cells were positive for both proteoglycans. Therefore, decorin and biglycan are differentially expressed in normal placenta and in placenta pathologies, suggesting that the expression patterns of the proteoglycans in studied pathologies indicate a role in modulating trophoblast migration and invasion.

As Oclusões Venosas da Retina são complicações do sistema vascular retiniano com grande potencial de impacto no sentido da visão. As oclusões Venosas são classificadas de acordo com a anatomia da região acometida e podem ser divididas em oclusão de ramo e da veia central da retina. O edema macular é a principal causa de baixa acuidade visual na Oclusão de Ramo da Veia Central da Retina (OVR). Sua detecção, localização e classificação são cruciais para a prevenção e tratamento da perda visual na OVR. A Biomicrospia Macular e a Angiofluoresceinografia são os métodos tradicionais de avaliação do edema macular na OVR, a Tomografia de Coerência Óptica (TCO) é uma tecnologia nova, que permite uma avaliação objetiva da morfologia macular. O objetivo primário do presente estudo foi avaliar, em uma mesma população, a acurácia dos métodos de diagnóstico do edema macular na OVR através da análise de três quesitos, aumento da espessura macular e da sua localização, presença de cistos intra-retinianos e a presença de descolamento seroso macular (DSM). Indivíduos com OVR foram selecionados com base nos achados fundoscópicos da doença e com tempo de história da doença entre três e vinte quatro meses. Todos os sujeitos foram submetidos a exames com o Stratus OCT (TCO), Angiofluoresceinografia digital (Angio) e o exame da biomicroscopia macular com lente de Volk de 78 D (BM) dentro do período de uma semana, além de exame oftalmológico completo. As medidas utilizadas para avaliação da acurácia diagnóstica foram o índice de concordância Kappa e a sensibilidade. Dos 32 indivíduos inicialmente avaliados, 8 foram posteriormente excluídos por não cumprirem os critérios de inclusão ou de exclusão, restando 24 indivíduos para análise. Na avaliação da espessura macular, a concordância entre BM e a TCO foi substancial e significante para a detecção do aumento da espessura macular com Kappa = 0,778 (p < 0,001) e a sensibilidade foi 95,5%. Na comparação entre a BM e a TCO para a detecção de cistos intra-retinianos, a concordância foi fraca com Kappa = 0,250 (p = 0,066) e a sensibilidade foi 57,9 %. A concordância entre a Angio e a TCO para os cistos foi pobre e não significante com Kappa = 0,124 (p = 0,237) e a sensibilidade foi 58,7 %. Houve uma correlação significante entre a presença de extravasamento na angiofluoresceinografia e a presença de cistos intra-retinianos na TCO (p=0,042). Na avaliação do DSM a concordância entre a BM e a TCO foi fraca com Kappa = 0,314 (p = 0,062) e a sensibilidade foi 60%, não houve concordância entre a Angio e a TCO para a detecção do DSM com Kappa = 0 e sensibilidade de 0%, e também não houve correlação significante entre a presença de descolamento seroso na TCO e a presença de extravasamento na angiofluoresceinografia (p=0,615). Os dados obtidos no presente estudo sugeriram que o Stratus OCT têm acurácia superior para a detecção dos cistos intra-retinianos e do descolamento seroso macular, se comparado à biomicroscopia macular e à angiofluoresceinografia / Retinal Vein Occlusions are complications of the retinal vascular system that can cause a great impact on vision. The Vein Occlusions are classified according to the anatomy of the affected region and can be categorized as: branch retinal vein occlusion and central retinal vein occlusion. Macular edema is the main cause for low visual acuity in Branch Retinal Vein Occlusion (BRVO). Its detection, location and classification are crucial for the prevention and treatment of vision loss in BRVO. Both Macular Biomicroscopy and Angiofluoresceinography are traditional methods for the evaluation of the macular edema in BRVO; the Optical Coherence Tomography (OCT) is a new technology that allows for an objective evaluation of macular morphology. The main objective of the present study was to evaluate, among the same population, the accuracy of the diagnostic methods of macular edema in BRVO through the analysis of three items: the increase of macular thickness and its location, the presence of intraretinal cysts, and the presence of serous macular detachment. Patients with BRVO were selected according to the fundoscopic findings of the disease and a disease history of three to twenty-four months. All of the individuals underwent exams with the Stratus OCT, digital Angiofluoresceinography (Angio) and a macular biomicroscopy with a 78D Volk lens (MB), within a period of one week, as well as a complete ophthalmologic exam. The parameters used in the evaluation of the diagnostic accuracy were sensitivity and the kappa coefficient. Of the 32 subjects who were initially evaluated, 8 were later excluded since they did not meet inclusion or exclusion criteria. The remaining 24 subjects were analyzed. In the evaluation of macular thickness, the concordance between the MB and the OCT was substantial and significant in the detection of the increase in macular thickness, with Kappa = 0.778 (p<0.001) and a sensitivity of 95.5%. When comparing the MB and the OCT in the detection of intraretinal cysts, the concordance was fair, with Kappa = 0.250 (p = 0.066) and a sensitivity of 57.9%. The concordance between the Angio and the OCT for cysts detection was poor and non significant, with Kappa = 0.124 (p = 0.237) and a sensitivity of 58.7%. There was a significant correlation between the presence of leakage in the angiofluoresceinography and the presence of intraretinal cysts in the OCT (p = 0.042). In the evaluation of the SMD, the concordance between the MB and the OCT was fair, with Kappa = 0.314 (p = 0.062) and a sensitivity of 60% an there was no concordance between the Angio and the OCT in the detection of SMD, with Kappa = 0 and sensitivity of 0%. There was also no significant correlation between the presence of serous detachment in the OCT and the presence of leakage in the angiofluoresceinography (p = 0.615). The results obtained in the present study suggest that Stratus OCT is more accurate to the detection of intraretinal cysts and serous macular detachment than macular biomicroscopy with a 78D Volk lens, and by angiofluoresceinography

No description available.

Photoreceptors are of prime importance for humans, since vision is one of the most important senses for us. In our daily life, where nearly every action is dependent on visual input, an impairment or a loss of eyesight leads to severe disability. With a non-syndromic prevalence of 1:4000, retinitis pigmentosa, a collective term for a group of inherited retinal eye diseases, represents, together with age-related macula degeneration, one of the main causes for visual impairment and blindness in industrialized countries. The dominant reason for vision loss is, in both cases, the irreversible loss of photoreceptor cells located in the outer nuclear layer of the retina. To date, no effective treatment is available to preserve or regain visual function in affected patients. Recent promising strategies for new retinal therapeutical approaches focus on one hand on the development of gene therapies, where an introduced wild-type allele compensates a mutated gene, and on the other hand on cell therapies, where stem or photoreceptor precursor cells (PPCs) are transplanted to the sub-retinal space to replace degenerated host photoreceptors. The current study is subdivided into three parts, addressing the issue of non-reversible photoreceptor cell loss due to retinal degenerative diseases by investigating in the first two parts new qualitative as well as quantitative approaches in the field of retinal cell therapy, while in the third part an ocular gene therapeutical approach targeting prominin-1, a gene involved in retinal degenerative disorders, was investigated. Briefly, this study shows in the first part, a significant enhancement of the integration rate of PPCs in wild-type host retinas, achieved by pre-transplantational sorting, using the recently discovered PPC - specific cell surface marker CD73. This sets another step further towards retinal cell therapy by increasing the effectiveness of such treatment. Next to this quantitative approach, it is also shown that the quality of transplanted photoreceptor precursor cells is comparable to native photoreceptors by demonstrating, that an indispensable prerequisite of every photoreceptor cell, the outer segment, is developed by transplanted PPCs after proper integration. Importantly, transplanted PPCs develop native outer segments even when not integrated in the host tissue but located in the sub-retinal space, as it is predominantly observed after transplantation into severely degenerated retinas. These results substantiate the feasibility of cell therapeutical treatment of severely degenerated retinas. At the end of this part, it is demonstrated, that outer segments are not formed properly by PPCs transplanted to the vitreal side of the retina. This suggests an influence of signaling molecules, presumably secreted by retinal pigment epithelial cells into the sub-retinal space, on transplanted PPC final differentiation. Since intensive research is done to differentiate stem cells into PPCs for cell therapeutical transplantation, these results may contribute significantly to this research by demonstrating, that factors secreted by the retinal pigment epithelium might play a crucial role for successful stem cell to PPC differentiation. The last part of my work investigates a gene therapeutical approach to cure inherited retinal degenerative diseases. One gene, where reported mutations cause retinal degeneration in humans is prominin-1, a protein expressed at cell membrane evaginations in a variety of cell types. Interestingly, the prominin-1 knock-out mouse is characterized exclusively by disorganized photoreceptor outer segment formation and progressive retinal degeneration. Successful delivery of a wild-type form of mouse prominin-1 using adeno-associated viral vector transfer, into the photoreceptors of prominin-1 - deficient mice is demonstrated. The divergent results show on one hand a rescue of the thickness of the photoreceptor outer nuclear layer on a short time period (3 weeks post treatment), and on the other hand long-term data (8-10 weeks post treatment) suggests histologically as well as functionally a negative effect on treated photoreceptors. This might be due to effects caused by an over-expression of prominin-1 and will be investigated in future studies. In conclusion, distinct and important investigations were made which contribute significant puzzle pieces to new cell- as well as gene therapeutical approaches for the treatment of retinal degenerative disorders.

As afecções que acometem a via óptica anterior podem acarretar perda de fibras das células ganglionares retinianas, gerando alterações na camada de fibras nervosas da retina (CFNR). O objetivo deste trabalho foi o de comparar a capacidade da tomografia de coerência óptica (OCT-1), do OCT 3 (Stratus OCT) e do polarímetro de varredura a laser (GDx) em diferenciar olhos normais de olhos com atrofia em banda do nervo óptico e hemianopsia temporal, assim como, comparar o desempenho de tais tecnologias entre si. Foram estudados 37 indivíduos num total de 46 olhos divididos igualmente em dois grupos. O Stratus OCT, o OCT 1 e o GDx se mostraram capazes de identificar corretamente a redução global (average) da espessura da CFNR dos indivíduos com AB comparados com uma população normal. Observamos uma melhora progressiva das sensibilidades/especificidades e das AROC, com o desempenho melhor do OCT1 sobre o GDx e do Stratus OCT sobre o OCT 1 / Most diseases that affect the optic pathways present clinically with loss of one or more visual functions.When optic pathway injuries involve the anterior visual pathways they may cause loss of the retinal nerve fiber layer (RNFL) and "band atrophy" (BA). The purpose of this study was therefore to compare the ability of the optical coherence tomography (OCT) 1, the OCT 3 (Stratus OCT) and the scanning laser polarimetry (GDx) to differentiate normal eyes from eyes with BA of the optic nerve and temporal hemianopsia. Thirty-seven individuals in a total of 46 eyes divided equally in two groups were studied. Each one of the parameters, except the values from the nasal quadrant measured by the GDx, showed statistically significant difference when we compare normal patients with the BA group. The study also showed a gradual improvement of the sensibilities/specificities and of the AROC, with a better performance of the OCT1 compared to GDx and of the Stratus OCT compared to the OCT 1

As Oclusões Venosas da Retina são complicações do sistema vascular retiniano com grande potencial de impacto no sentido da visão. As oclusões Venosas são classificadas de acordo com a anatomia da região acometida e podem ser divididas em oclusão de ramo e da veia central da retina. O edema macular é a principal causa de baixa acuidade visual na Oclusão de Ramo da Veia Central da Retina (OVR). Sua detecção, localização e classificação são cruciais para a prevenção e tratamento da perda visual na OVR. A Biomicrospia Macular e a Angiofluoresceinografia são os métodos tradicionais de avaliação do edema macular na OVR, a Tomografia de Coerência Óptica (TCO) é uma tecnologia nova, que permite uma avaliação objetiva da morfologia macular. O objetivo primário do presente estudo foi avaliar, em uma mesma população, a acurácia dos métodos de diagnóstico do edema macular na OVR através da análise de três quesitos, aumento da espessura macular e da sua localização, presença de cistos intra-retinianos e a presença de descolamento seroso macular (DSM). Indivíduos com OVR foram selecionados com base nos achados fundoscópicos da doença e com tempo de história da doença entre três e vinte quatro meses. Todos os sujeitos foram submetidos a exames com o Stratus OCT (TCO), Angiofluoresceinografia digital (Angio) e o exame da biomicroscopia macular com lente de Volk de 78 D (BM) dentro do período de uma semana, além de exame oftalmológico completo. As medidas utilizadas para avaliação da acurácia diagnóstica foram o índice de concordância Kappa e a sensibilidade. Dos 32 indivíduos inicialmente avaliados, 8 foram posteriormente excluídos por não cumprirem os critérios de inclusão ou de exclusão, restando 24 indivíduos para análise. Na avaliação da espessura macular, a concordância entre BM e a TCO foi substancial e significante para a detecção do aumento da espessura macular com Kappa = 0,778 (p < 0,001) e a sensibilidade foi 95,5%. Na comparação entre a BM e a TCO para a detecção de cistos intra-retinianos, a concordância foi fraca com Kappa = 0,250 (p = 0,066) e a sensibilidade foi 57,9 %. A concordância entre a Angio e a TCO para os cistos foi pobre e não significante com Kappa = 0,124 (p = 0,237) e a sensibilidade foi 58,7 %. Houve uma correlação significante entre a presença de extravasamento na angiofluoresceinografia e a presença de cistos intra-retinianos na TCO (p=0,042). Na avaliação do DSM a concordância entre a BM e a TCO foi fraca com Kappa = 0,314 (p = 0,062) e a sensibilidade foi 60%, não houve concordância entre a Angio e a TCO para a detecção do DSM com Kappa = 0 e sensibilidade de 0%, e também não houve correlação significante entre a presença de descolamento seroso na TCO e a presença de extravasamento na angiofluoresceinografia (p=0,615). Os dados obtidos no presente estudo sugeriram que o Stratus OCT têm acurácia superior para a detecção dos cistos intra-retinianos e do descolamento seroso macular, se comparado à biomicroscopia macular e à angiofluoresceinografia / Retinal Vein Occlusions are complications of the retinal vascular system that can cause a great impact on vision. The Vein Occlusions are classified according to the anatomy of the affected region and can be categorized as: branch retinal vein occlusion and central retinal vein occlusion. Macular edema is the main cause for low visual acuity in Branch Retinal Vein Occlusion (BRVO). Its detection, location and classification are crucial for the prevention and treatment of vision loss in BRVO. Both Macular Biomicroscopy and Angiofluoresceinography are traditional methods for the evaluation of the macular edema in BRVO; the Optical Coherence Tomography (OCT) is a new technology that allows for an objective evaluation of macular morphology. The main objective of the present study was to evaluate, among the same population, the accuracy of the diagnostic methods of macular edema in BRVO through the analysis of three items: the increase of macular thickness and its location, the presence of intraretinal cysts, and the presence of serous macular detachment. Patients with BRVO were selected according to the fundoscopic findings of the disease and a disease history of three to twenty-four months. All of the individuals underwent exams with the Stratus OCT, digital Angiofluoresceinography (Angio) and a macular biomicroscopy with a 78D Volk lens (MB), within a period of one week, as well as a complete ophthalmologic exam. The parameters used in the evaluation of the diagnostic accuracy were sensitivity and the kappa coefficient. Of the 32 subjects who were initially evaluated, 8 were later excluded since they did not meet inclusion or exclusion criteria. The remaining 24 subjects were analyzed. In the evaluation of macular thickness, the concordance between the MB and the OCT was substantial and significant in the detection of the increase in macular thickness, with Kappa = 0.778 (p<0.001) and a sensitivity of 95.5%. When comparing the MB and the OCT in the detection of intraretinal cysts, the concordance was fair, with Kappa = 0.250 (p = 0.066) and a sensitivity of 57.9%. The concordance between the Angio and the OCT for cysts detection was poor and non significant, with Kappa = 0.124 (p = 0.237) and a sensitivity of 58.7%. There was a significant correlation between the presence of leakage in the angiofluoresceinography and the presence of intraretinal cysts in the OCT (p = 0.042). In the evaluation of the SMD, the concordance between the MB and the OCT was fair, with Kappa = 0.314 (p = 0.062) and a sensitivity of 60% an there was no concordance between the Angio and the OCT in the detection of SMD, with Kappa = 0 and sensitivity of 0%. There was also no significant correlation between the presence of serous detachment in the OCT and the presence of leakage in the angiofluoresceinography (p = 0.615). The results obtained in the present study suggest that Stratus OCT is more accurate to the detection of intraretinal cysts and serous macular detachment than macular biomicroscopy with a 78D Volk lens, and by angiofluoresceinography

O albinismo e uma alteracao genetica rara que compromete a producao de melanina. As alteracoes clinicas consistem na falta de pigmentacao da pele, cabelo e pelos. Apresenta alteracoes oftalmologicas importantes, que comprometem a acuidade visual de forma severa, na grande maioria dos casos. As alteracoes oftalmologicas sao, ametropias, nistagmo, rarefacao do epitelio pigmentado da iris e da retina, hipoplasia foveal e decussacao anomala do nervo optico. Este estudo foi dividido em tres diferentes partes com os seguintes objetivos principais: Parte 1: Analise comparativa entre o grau de transparencia da iris (GTI), o grau de transparencia da retina (GTR) e a espessura da regiao macular com a acuidade visual nos pacientes com albinismo. Parte 2: Teste de sensibilidade ao contraste espacial de luminancia e ofuscamento com lentes de contato filtrantes em ambientes claros e escuros. Parte 3: Avaliacao da visao de cores, utilizando\\se o teste de Pranchas de Ishihara e o Cambridge Color Test (CCT). Material\' e\' Métodos: Participaram do estudo 121 individuos albinos, com idade media de 31,18 (} 15,47) anos, o que totalizou 242 olhos. Os participantes foram divididos em diferentes grupos nas tres partes do estudo, alguns participaram de uma ou mais partes. Na primeira parte os participantes foram submetidos a consulta oftalmologica, classificacao do grau de transparencia da iris e da retina em uma nova classificacao baseada em quatro diferentes graus de transparencia, alem de avaliacao foveal pelo exame de Tomografia de Coerencia Optica (OCT). Na segunda parte, os participantes foram submetidos ao teste de contraste computadorizado, utilizando lentes de contato filtrante e transparentes, em ambientes claros e escuros. Nesta etapa foi avaliada a influencia da lente filtrante na sensibilidade de contraste visual dos individuos com albinismo. Na terceira parte, os participantes foram submetidos aos testes de Pranchas de Ishihara e de Cambridge Colour Test (CCT), para avaliacao da visao de cores nos pacientes albinos. Resultados: Os resultados foram os seguintes: Parte\'1: A correlacao entre AV logMAR e GTI foi positiva (+0,569) e significativa (p<0,001). A correlacao entre AV logMAR e GTR foi positiva (+0,531) e significativa (p<0,001). A correlacao entre AV logMAR e a espessura macular nao foi significativa (p=0,105). A correlacao entre GTI e GTR foi positiva (+ 0,627) e significativa (p<0,001). A correlacao entre espessura macular e GTI nao foi significativa (p=0,397). A correlacao entre espessura macular e GTR nao foi significativa (p=0,458). Parte 2: Houve melhora estatisticamente significante da sensibilidade ao contraste com as lentes filtrantes em relacao as lentes transparentes no ambiente de ofuscamento (claro), na frequencia de 0,3 cpg, 0,6 cpg e 1,0 cpg (ciclos por grau). Nao houve melhora significante nas frequencia 2,0 cpg e 4,0 cpg. Nao houve melhora estatisticamente significante da sensibilidade ao contraste de lentes filtrantes em relacao as lentes transparentes no ambiente escuro (penumbra) em nenhuma das frequencias espaciais estudadas. Parte\' 3: Todos os individuos com albinismo nao apresentaram nenhuma alteracao de visao de cores no teste de Pranchas de Ishihara. O CCT demonstrou piora no limiar de deteccao de cores significativa nos eixos protan (p=0,021) e deutan (p=0,017), mas nao houve diferenca no eixo tritan (p=0,212). Os testes estatisticos sugerem que uma amostra maior seja estudada para validacao dos resultados / Albinism is a rare genetic alteration that compromises the production of melanine in all body tissues. The clinical alterations are the lack of pigment in the skin and hair. It presents important ophthalmological changes that compromise the visual acuity, in most cases. The ophthalmological changes are: ametropia, nystagmus, iris pigmented epithelium rarefaction, retina pigmented epithelium rarefaction, foveal hipoplasia and abnormal optic nerve decussation. This study has been divided in three different parts and had the following main objectives: Part 1, comparative analysis of the iris transparency degree (ITD), the retina transparency degree (RTD) and macular thickness with the visual acuity in albino patients. Part 2: Spatial contrast sensitivity test of luminance and outshine wearing contact lenses with filters in light and dark environments. Part 3: color vision evaluation using the Ishihara Test and the Cambridge Color Test (CCT). For the study 121 individuals with albinism were included with an average of 31,18 years old } 15,47, a total of 242 eyes. The participants were divided in different groups in the three parts of the study, some participated in one or more parts. In the first part the participants have been through an ophthalmological exam, classification of the iris transparency degree (ITD) and the retinal transparency degree (RTD) by a new classification based in 4 different degrees of transparency. Foveal evaluation by the Optic Coherence Tomography exam (OCT) was made in the first part of this study. In the second part, the participants have been submitted to the computerized contrast test, using filtered and transparent contact lenses in light and dark environments. In this part the influence of the filtering lenses in the visual contrast sensitivity in the albinism patients has been evaluated. In the third part, the participants have been submitted to Ishihara Test and Cambridge Color Test in order to evaluate the color vision in albino patients. The results are as follows: Part\' 1: the correlation between visual acuity in Logarithm of the Minimum Angle of Resolution (logMAR) and ITD was positive (+0,569) and significant (p<0,001). The correlation between visual acuity in logMAR and RTD was positive (+0,531) and significant (p<0,001). The correlation between visual acuity in logMAR and the macular thickness was not significant (p=0,105). The correlation between ITD and RTD was positive (+ 0,627) and significant (p<0,001). The correlation between the macular thickness and the ITD was not significant (p=0,397). The correlation between the macular thickness and the RTD was not significant (p=0,458). Part\'2: There has been a statistically significant improvement of the contrast sensitivity wearing the filtering lenses in relation to the transparent ones in bright environment, in the frequencies of 0,3 cycles per degree (cpd), 0,6 cpd and 1,0 cpd. There has been no significant improvement in the frequencies 2,0 cpd and 4,0 cpd. There has been no statistically significant improvement in contrast sensitivity wearing the filtering lenses in relation to the transparent one in the dark environment (shadow) in any of the spatial frequencies studied. Part\' 3: all albinism patients did not present any color vision change in the Ishihara Test. The CCT has shown a worsening in the color detection threshold in the protan axis (p=0,021) and deutan axis (p=0,017), but there has been no difference in the tritan axis (p=0,212). The statistic tests show that a bigger sample is recommended to confirm part 3 results

O decorim e o biglicam são membros da família dos pequenos proteoglicanos ricos em leucina e possuem importantes funções no controle da proliferação, migração e invasão do citotrofoblasto extraviloso (TEV). O objetivo deste trabalho foi de caracterizar a expressão diferencial e a imunolocalização de decorim e biglicam em placentas humanas normais a termo (PNT), na placenta acreta (PA), na mola invasora (MI) e no coriocarcinoma (CO). Na PNT, as células deciduais apresentaram positividade para o decorim, enquanto o TEV foi negativo. O decorim foi fracamente expresso na matriz endometrial, mas negativo no fibrinoide do tipo matriz, enquanto foi positivo para biglicam. Na PA e na MI, o TEV mostrou positividade para decorim e biglicam. No CO, somente o citotrofoblasto foi positivo para ambos proteoglicanos. Portanto, o decorim e o biglicam são expressos diferencialmente em placentas normais e patológicas, sugerindo que os padrões de expressão desses proteoglicanos nas patologias estudadas indicam um papel na modulação da migração e da invasão do trofoblasto. / Decorin and biglycan are family members of the small leucine-rich proteoglycans family, and they have many functions as controlling proliferation, migration and invasion of extravillous trophoblast cells (EVT). The aim of this study was to characterize decorin and biglycan differential expression and immunolocalization in human normal term placenta (NTP), in placenta accreta (PA), in invasive mole (IM), and in choriocarcinoma (CH) samples. In PNT, deciduas cells were positive to decorin whereas EVT was negative. Decorin was faintly stained at endometrial matrix, but negative at matrix-type fibrinoid, although it was positive for biglycan. In PA and IM, the EVT was positive for decorin and biglycan. In CH, only cytotrophoblast cells were positive for both proteoglycans. Therefore, decorin and biglycan are differentially expressed in normal placenta and in placenta pathologies, suggesting that the expression patterns of the proteoglycans in studied pathologies indicate a role in modulating trophoblast migration and invasion.