RESUMEN
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.
Asunto(s)
Neuropatías Amiloides Familiares/complicaciones , Neuropatías Amiloides Familiares/metabolismo , Humor Acuoso/metabolismo , Eritropoyetina/metabolismo , Glaucoma de Ángulo Abierto/complicaciones , Glaucoma de Ángulo Abierto/metabolismo , Proteínas Mutantes/metabolismo , Neuropatías Amiloides Familiares/sangre , Neuropatías Amiloides Familiares/fisiopatología , Demografía , Femenino , Glaucoma de Ángulo Abierto/sangre , Glaucoma de Ángulo Abierto/fisiopatología , Humanos , Presión Intraocular , Masculino , Persona de Mediana EdadRESUMEN
UNLABELLED: PURPOSE. Evaluation of the use of topical cyclosporine eyedrops in the treatment of severe dry eye disease in liver transplanted patients with familial amyloidotic polyneuropathy (FAP) unresponsive to therapy with artificial tears and lacrimal plugs. METHODS: A prospective clinical study of 5 patients (10 eyes) admitted to the Ophthalmology Department of the Centro Hospitalar do Porto with severe dry eye disease refractory to artificial tears and lacrimal plug treatments. Evaluation of the patients included best-corrected visual acuity, corneal punctuate fluorescein staining, tear break-up time, Schirmer test without anesthesia, and Ocular Surface Disease Index. Patients were observed at time 0, and at 3, 7, and 11 months. RESULTS: Treatment with topical cyclosporine improved all studied parameters from baseline, and in all the patients (p<0.001). The safety profile was excellent, without topical or systemic adverse events. CONCLUSIONS: Topical cyclosporine was beneficial in the treatment of severe dry eye disease in liver transplanted patients with FAP.