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Purpose: To investigate the natural history of ocular adnexal and orbital amyloidosis.Methods: In a retrospective, non-comparative case series, the clinical records of patients with biopsy-proven ocular, adnexal, and orbital amyloidosis managed at our institution between 1980 and 2016 were evaluated.Results: Forty-one patients (29 female; 71%) were identified. The mean interval from presentation to diagnosis was 24 months (median 12 months, range 1-84 months). Whilst most patients presented with a conjunctival mass (34/41; 83%) or ptosis (15/41; 37%), the diagnosis was not immediately evident in all - two patients had 3 ptosis operations prior to obtaining a tissue biopsy that revealed amyloid deposition. Three-quarters (31/41; 76%) of patients had localised primary ocular adnexal and orbital amyloidosis, 4 (10%) had associated systemic disease, and 6 (15%) were found to have underlying haematological malignancy on further investigation. During a mean follow-up of 8 years (median 7 years; range 6 months - 36 years), 2 (5%) patients lost vision, 21 (51%) had surgical intervention other than biopsy, and 2 (5%) had local radiotherapy for amyloid deposition secondary to lymphoproliferative disease.Conclusions: The varied presentations of ocular adnexal and orbital amyloidosis and the need for confirmatory biopsy often leads to a significant delay between first symptoms and diagnosis. While rarely sight-threatening, ocular adnexal and orbital amyloidosis carries significant morbidities and has a systemic association in a quarter of patients.
Assuntos
Amiloidose/diagnóstico , Doenças da Túnica Conjuntiva/diagnóstico , Doenças Palpebrais/diagnóstico , Doenças Orbitárias/diagnóstico , Adulto , Distribuição por Idade , Amiloidose/epidemiologia , Amiloidose/cirurgia , Biópsia por Agulha , Estudos de Coortes , Doenças da Túnica Conjuntiva/epidemiologia , Doenças da Túnica Conjuntiva/cirurgia , Doenças Palpebrais/cirurgia , Feminino , Finlândia , Humanos , Imuno-Histoquímica , Incidência , Masculino , Pessoa de Meia-Idade , Doenças Orbitárias/epidemiologia , Doenças Orbitárias/cirurgia , Prognóstico , Estudos Retrospectivos , Índice de Gravidade de Doença , Distribuição por Sexo , Tomografia Computadorizada por Raios X/métodos , Adulto JovemRESUMO
Glaucoma is a neurodegenerative disease with an estimated prevalence of 60 million people, and the most common cause of irreversible blindness worldwide. The mainstay of treatment has been aimed at lowering intraocular pressure, currently the only modifiable risk factor. Unfortunately, despite adequate pressure control, many patients go on to suffer irreversible visual loss. We first briefly examine currently established intraocular pressure lowering-treatments, with a discussion of their roles in neuroprotection as demonstrated by both animal and clinical studies. The review then examines currently available intraocular pressure independent agents that have shown promise for possessing neuroprotective effects in the management of glaucoma. Finally, we explore potential future treatments such as immune-modulation, stem cell therapy and neural regeneration as they may provide further protection against the neurodegenerative processes involved in glaucomatous optic neuropathy.
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Glaucoma/tratamento farmacológico , Doenças Neurodegenerativas/tratamento farmacológico , Fármacos Neuroprotetores/uso terapêutico , Animais , Humanos , Doenças Neurodegenerativas/terapia , Transplante de Células-TroncoRESUMO
AIM: To establish the efficacy and safety of bimatoprost 0.03% monotherapy in glaucoma and ocular hypertension (OHT) patients with inadequate intraocular pressure (IOP)on current therapy. METHODS: Pre- and post-switch IOPs were analyzed for 59 consecutive patients who were switched from current therapy to bimatoprost monotherapy between 2011-2015. Demographic information, diagnosis, and any adverse events were recorded. Change in IOP post-pre switch was analyzed using a 2-sided Student's paired t-test at the 5% significance level. RESULTS: There was a statistically significant mean reduction in IOP at the first follow up visit, which was maintained at subsequent follow up visits for patients regardless of diagnosis, or pre-switch treatment (P<0.001). Subgroup analysis also demonstrated a statistically significant mean reduction in IOP when looking at OHT patients only, as well as patients with any diagnosis switched from latanoprost monotherapy to bimatoprost monotherapy (P<0.001). CONCLUSION: This is the largest independent data set which supports switching glaucoma patients with poor response to current treatment onto bimatoprost monotherapy before considering other adjuvant medical or more invasive therapy.
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Retinal cell apoptosis occurs in many eye conditions, including glaucoma, diabetic retinopathy and Alzheimer's disease. Real-time detection of retinal cell apoptosis has potential clinical value in early disease detection, as well as evaluating disease progression and treatment efficacy. Here, we describe our novel imaging technology DARC (Detection of Apoptosing Retinal Cells), which can be used to visualize single retinal neurons undergoing apoptosis in real time, by using fluorescently labeled Annexin A5 and confocal scanning laser ophthalmoscopy (cSLO ). Clinical trials of DARC in glaucoma patients are due to start shortly, but in this chapter, we describe this technique in experimental animal models.
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Diagnóstico por Imagem , Oftalmoscopia/métodos , Retina/patologia , Células Ganglionares da Retina/patologia , Animais , Apoptose/genética , Glaucoma/diagnóstico , Glaucoma/patologia , Humanos , Microscopia Confocal , Biologia Molecular/métodos , Retina/ultraestrutura , Células Ganglionares da Retina/ultraestruturaRESUMO
Alzheimer's disease (AD) is neurodegenerative condition and most common cause of dementia worldwide. Current criteria for its diagnosis and monitoring rely on subjective, expensive or invasive methods that lack sufficient sensitivity, such that a concrete diagnosis of AD can only be made postmortem. Given the structural similarities of the neuro-retina and central nervous system, researchers have shown many manifestations of AD to be detectible in the retinae of humans and transgenic models of AD. Due to the eye's unique optical properties allowing noninvasive in vivo imaging, the retina could provide a window for the early diagnosis and monitoring of AD long before symptom manifestation.