Browsing by Author "Torres, P."
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- Aqueous humor erythropoietin levels in open-angle glaucoma patients with and without TTR V30M familial amyloid polyneuropathyPublication . Beirão, J.; Moreira, L.; Oliveira, J.; Menéres, M.; Pessoa, B.; Matos, M.; Pinho-Costa, P.; Torres, P.; Beirão, I.Purpose: Glaucoma is the leading cause of irreversible blindness in familial amyloidotic polyneuropathy (FAP) patients. Erythropoietin (EPO) is a cytokine that has been shown to play a role in neuroprotection and is endogenously produced in the eye. EPO levels in the aqueous humor are increased in eyes with glaucoma. In this study, we evaluated the EPO concentration in the aqueous humor of FAP and non-FAP patients, with and without glaucoma. Methods: Undiluted aqueous humor samples were obtained from 42 eyes that underwent glaucoma surgery, phacoemulsification, or vitrectomy. EPO concentration in the aqueous humor and blood were measured using the Immulite 2000 Xpi using an automatic analyzer (Siemens Healthcare Diagnostics). Results: The mean EPO concentration in the aqueous humor of non-FAP glaucoma eyes group 2(75.73±13.25 mU/ml)was significantly higher than non-FAP cataract eyes (17.22±5.33 mU/ml; p<0.001), FAP glaucoma eyes (18.82±10.16 mU/ml; p<0.001), and FAP nonglaucoma eyes (20.62±6.22 mU/ml; p<0.001). There was no statistically significant difference between FAP nonglaucoma eyes versus non-FAP cataract eyes (p = 0.23) and FAP glaucoma eyes versus FAP nonglaucoma eyes (p = 0.29). In the glaucoma groups, there was no correlation between the aqueous humor EPO concentration and the ocular pressure (p = 0.95) and mean deviation (p = 0.41). There was no correlation between the EPO serum concentration and EPO aqueous humor concentration in our patients (p = 0.77). Conclusions: Unlike other glaucomatous patients, FAP patients with glaucoma do not show increased and potentially neuroprotective endocular EPO production in the aqueous humor and may need more aggressive glaucoma management
- Impact of liver transplantation on the natural history of oculopathy in Portuguese patients with transthyretin (V30M) amyloidosisPublication . Beirão, J.M.; Malheiro, J.; Lemos, C.; Matos, E.; Beirão, I.; Costa, P.P.; Torres, P.Purpose: Evaluation of the impact of liver transplantation in the natural history of ocular disorders in familial amyloidotic polyneuropathy (FAP) amyloidosis TTR V30M related (ATTR V30M) patients. Design: A clinical, retrospective and cross-sectional study of 64 Portuguese FAP ATTR V30M patients was carried out between January 2005 and December 2011. Methods: Thirty-two liver transplanted patients (both eyes) aged 39.6–53.8 years old, 32/32 male/female, were paired with an equal number of non-transplanted patients, matching for age, gender, age at onset, disease duration and gender of transmitting parent. Intervention or observation procedure: Routine ophthalmological observation. Main outcome measures: Slit-lamp observation for abnormal conjunctival vessels (ACV), tears break-up time, iris, lens; fundus observation for vitreous, retina and optic disc; Schirmer test. Results: Liver transplantation had no influence on tears break-up time, deposition of amyloid on the iris and retinal amyloid angiopathy. Slight, non-statistically significant protective effects of liver transplantation were noted in the first years for some ocular manifestations (ACV and scalloped iris), except for the abnormal Schirmer test, which was significantly more prevalent in non-transplanted patients’ eyes (81% versus 56%, p = 0.002). On the other hand, deposition of amyloid on the lens, vitreous amyloidosis and glaucoma were apparently more common in transplanted patients. Those differences tended to disappear with time. Conclusions: Ocular manifestations of FAP were not influenced by liver transplantation in a meaningful way. Both transplanted and non-transplanted FAP patients need similar regular follow-up due to long-term risk of serious ocular disease.
- No ocular involvement in familial amyloidotic polyneuropathy ATTR V30M domino liver recipientsPublication . Melo Beirão, J.; Matos, E.; Beirão, I.; Costa, P.; Torres, P.In many transplantation centers domino liver transplantation is an established procedure, increasing the number of available liver grafts. Increasingly, grafts from familial amyloidotic polyneuropathy (FAP) patients are used. Ocular involvement is a well known manifestation of FAP, and can be vision-threatening. The aim of this study was to evaluate the risk of development of familial amyloidotic polyneuropathy ocular manifestations in domino liver recipients. Forty-four cirrhotic patients submitted to liver transplantation were studied, with an average of 6 years of follow up after the procedure. Twenty two patients had received a liver from a FAP donor (Group 1) and 22 had received a liver from a non-FAP cadaveric donor (Group 2). Both groups were similar for mean age and gender. Routine ophthalmological examinations with particular attention to amyloid deposition in the anterior segment and vitreous, peripheral retina state, lacrimal functions tests (Schirmer and tear break-up time) and pupillometry (dynamic and static) were performed. No statistically significant differences were observed in all studied ophthalmic parameters between the two groups. No FAP related ophthalmic manifestations were detected after 6 years of domino liver transplantation, but further prospective regular ophthalmological examinations are necessary to detect the eventual development of late ocular manifestations.
- The use of intravitreal ranibizumab to treat neovascular glaucoma because of retinal amyloid angiopathy in familial amyloidosis transthyretin V30M relatedPublication . Beirão, N.M.; Miranda, V.; Beirão, I.; Costa, P.P.; Torres, P.Purpose: The purpose of this study to report a patient with amyloidotic angiopathy and neovascular glaucoma who was treated with intravitreal injection of ranibizumab followed by laser photocoagulation. Methods: A 52-year-old liver-transplanted woman with familial amyloidotic polyneuropathy presented with unilateral rubeosis iridis and neovascular glaucoma. A complete ocular examination and fluorescein and indocyanine green angiography were performed. Results: Best-corrected visual acuity before injection was 0.05 (Snellen) in the left eye, and intraocular pressure was 42 mmHg. Fluorescein angiography showed vascular occlusion in the retinal periphery, focal staining of vessels, and microaneurysms. Indocyanine green angiography showed hyperfluorescent spots alongside the choroidal veins. Two days after receiving intravitreal injection of ranibizumab, the clinical picture regressed. The diagnosis of retinal amyloid angiopathy was made, and a peripheral retinal laser photocoagulation was done. The final best-corrected visual acuity after 2 years of follow-up was 0.4 (Snellen) in the left eye. Conclusion: Intravitreal injections of ranibizumab should be evaluated for a potential role on the treatment of amyloid angiopathy neovascular glaucoma. Careful retinal periphery examination should be included in the ophthalmologic examination of all familial amyloidotic polyneuropathy patients.