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BACKGROUND: Familial amyloidosis polyneuropathy (FAP) is a rare, progressive, and life-threatening disease inherited in the autosomal dominant pattern. Liver transplantation is the only proven disease-modifying treatment to date. AIM: To study the long-term outcomes of patients transplanted for FAP under a multidisciplinary team care. METHODS: We included adult patients who were transplanted for FAP indication and were followed up in a relevant clinic or admitted in our department. RESULTS: Twelve patients (6 male) with a mean age of 43 years and mean follow-up post-transplant of 100 months were included. Three patients died in this period, 1 due to a disease-related cause. All patients had peripheral neuropathy (25% severe). Eighty-three percent had autonomic nervous system dysfunction; all men, except one, erectile dysfunction; and half of the patients several genitourinary manifestations. Gastrointestinal involvement was present in 75% of the patients. The severity of several complications related to FAP was found to be associated with waiting on the transplant list for more than 12 months. CONCLUSIONS: Patients transplanted for FAP have a long survival. Prolonged stay on the transplant waiting list is associated with frequency and severity of disease complications. These patients are best managed in the context of multidisciplinary team care.
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Neuropatías Amiloides Familiares/cirugía , Trasplante de Hígado/métodos , Adulto , Anciano , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Estudios Prospectivos , Resultado del TratamientoRESUMEN
PURPOSE: To report the incidence, microbial spectrum, and outcome of cataract operation related endophthalmitis cases from 2000 to 2008 in the Department of Ophthalmology, University Hospital of Heraklion, Crete. METHODS: Retrospective, observational case series collected from the files of the University Eye Clinic of Heraklion. RESULTS: From 2000 to 2008, 23 eyes of 23 patients with endophthalmitis after cataract surgery were reported. Vitreous specimens were obtained in all cases. Twelve were cultured positive, 8 with gram-positive bacteria, and 4 with gram-negative bacteria. Ten patients were treated medically, 13 surgically, and 2 finally underwent enucleation of the affected globe. Fourteen (60.8%) eyes of the total 23 managed in our hospital, achieved a final visual acuity of 1/10 or better and 9 lower than 1/10. Five eyes ended up with no light perception. CONCLUSIONS: We provide an overview of cataract operation related endophthalmitis cases managed in our clinic during the past 9 years.
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Extracción de Catarata/efectos adversos , Endoftalmitis/epidemiología , Endoftalmitis/terapia , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/terapia , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Grecia/epidemiología , Humanos , Incidencia , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
A 39-year-old hyperopic male was referred for laser refractive treatment. In the course of the pre-operative evaluation he complained of a recent deterioration of vision. The suspicion of unilateral central serous chorioretinopathy (CSCR) was confirmed by contrast sensitivity testing and by ocular fundus examination. Contrast sensitivity (CS) for six spatial frequencies (1, 2, 4, 8, 12 and 16 c/deg) was evaluated using Gabor patches of gratings projected on a high-resolution display by means of a stimulus generator card. Although VA remained unaltered, the pattern of contrast sensitivity function varied at different stages of CSCR: during the acute stage, performance at all spatial frequencies was depressed, while at two-month follow up, intermediate and high spatial frequencies were mainly affected. It is concluded that the level of visual deficit in CSCR cannot be evaluated by measuring visual acuity. History and contrast sensitivity can play a central role in setting the correct diagnosis and characterising its stage.
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Enfermedades de la Coroides/diagnóstico , Enfermedades de la Coroides/fisiopatología , Sensibilidad de Contraste , Enfermedades de la Retina/diagnóstico , Enfermedades de la Retina/fisiopatología , Adulto , Estudios de Seguimiento , Fondo de Ojo , Humanos , Masculino , Estimulación Luminosa/métodos , Agudeza VisualRESUMEN
PURPOSE: To study the surface of normal corneal endothelium by means of atomic force microscopy (AFM). METHODS: The central corneal endothelial posterior surface of New Zealand white rabbits was examined. Specimens were observed in Balanced Salt Solution using the contact mode of the AFM either fresh or after fixation in cacodylate-buffered glutaraldehyde solution. Removal of sialic acid residues and hyaluronic acid was achieved by means of enzymatic treatment with neuraminidase and hyaluronidase. RESULTS: Observation of the fresh specimens revealed the presence of an apical endothelial surface coating material (glycocalyx). Removal of sialic acid residues and hyaluronic acid after enzymatic treatment using neuraminidase and hyaluronidase, respectively, permitted the elucidation of the structure of the nondigested coating material. Fixation of the samples resulted in removal of the surface coating material. The imaging of the fixed endothelium surface revealed the mosaic of polygonal cells with the apical flaps of cell junctions emerging over the cell surface. The cell shape and the other characteristics of the posterior surface fixed endothelium were comparable to those described in the literature using scanning electron microscopy. The scanning of very small ranges has provided high-resolution images at the nanometer level in fixed and fresh corneal endothelial surfaces. CONCLUSION: The atomic force microscope represents a new powerful imaging tool permitting high-resolution observation of corneal endothelium surface in fresh and minimally prepared fixed specimens.
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Endotelio Corneal/citología , Microscopía de Fuerza Atómica , Animales , Endotelio Corneal/efectos de los fármacos , Femenino , Glicocálix/diagnóstico por imagen , Hialuronoglucosaminidasa/farmacología , Neuraminidasa/farmacología , Conejos , Fijación del Tejido , UltrasonografíaRESUMEN
PURPOSE: To investigate the morphologic and hypotensive effect of contact transscleral ciliary body PDT in pigmented rabbit eyes. METHODS: The right eyes of 33 pigmented rabbits were irradiated using chloraluminum sulfonated phthalocyanine as photosensitizer and a diode laser (670 nm) as the light source. Twenty-five animals received a single treatment. Eight animals received a second treatment 13 days after the first one. Photosensitizer was administered by means of continuous intravenous infusion. Ciliary body was irradiated transsclerally by means of an optic fiber applied on the corneoscleral limbus. In all cases 14-16 laser applications were performed to cover 360 degrees of the ciliary body. Animals were followed for a maximum of 30 days by means of tonometry and biomicroscopy. Retreatments were performed using the same irradiation protocol. At the end of the follow up time animals were sacrificed and their were eyes prepared for light and electron microscopy. RESULTS: Transscleral ciliary body PDT resulted in significant but temporary reduction of IOP in all cases. The effect lasted about two weeks. Retreatment led to a new significant drop of the IOP, which lasted about two weeks again. In histological examination the initial effect was vascular thrombosis, followed by edema and disintegration of the ciliary epithelial layers. In all cases the appearance of the ciliary body had returned to normal 15 days after irradiation. CONCLUSION: Contact transscleral PDT with the treatment parameters used in this study results in significant but temporary functional and morphological alteration in pigmented rabbits ciliary body.
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Cuerpo Ciliar/efectos de los fármacos , Fotoquimioterapia , Aluminio/farmacología , Animales , Cuerpo Ciliar/patología , Cuerpo Ciliar/fisiopatología , Indoles/farmacología , Presión Intraocular/efectos de los fármacos , Isoindoles , Conejos , Fármacos Sensibilizantes a Radiaciones/farmacología , Retratamiento , Factores de TiempoRESUMEN
PURPOSE: To evaluate the feasibility of imaging normal corneal epithelium by means of atomic force microscopy (AFM). METHODS: Twelve normal corneas from six albino rabbits were examined using a commercial atomic force microscope. Six corneas were examined in balanced salt solution after fixation in glutaraldehyde 2.5% and six without any fixation. Rectangular silicon nitride cantilevers with a spring constant of 10 to 20 mN/m were used. The measured forces after imaging were less than 100 pN. All reported images were made with 512x512-pixel definition with typical scan rates ranging from 1 to 5 Hz. RESULTS: High-quality images of corneal epithelium surface were obtained from fixed and unfixed specimens in magnifications ranging from x2000 to x2,000,000. Imaging of fixed specimens was always easier. In unfixed specimens fuzzy images were very common, probably because of the presence of the cell glycocalyx. AFM revealed the typical polygonal corneal epithelial cells. The cell surface was covered by microprojections; at cell borders the microprojections were arranged in two characteristic parallel rows. Craterlike formations were revealed in several specimens. The microprojections' morphology and their surface details were revealed using magnifications up to x2,000,000. Three-dimensional representation of the images facilitated better understanding of the surface topography. Measurements in horizontal and vertical plane were made using the section analysis tool. CONCLUSIONS: In this work the AFM parameters appropriate for corneal epithelium imaging in physiological medium were defined. AFM represents a new powerful tool for corneal epithelium imaging, and its application in this field warrants further investigation.
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Epitelio Corneal/citología , Microscopía de Fuerza Atómica/métodos , Animales , ConejosRESUMEN
OBJECTIVE: To evaluate the epidemiological correlation between age-related macular degeneration and pseudoexfoliation syndrome in the inhabitants of the island of Crete (Greece). SUBJECTS AND METHODS: A total of 777 persons (315 men and 462 women, aged 40-99 years), representing a randomized sample (1.43%) of the Cretan population, underwent slitlamp and fundus examinations according to protocol. The results were statistically analyzed. RESULTS: The prevalence of pseudoexfoliation was 16.1% (21.3% in men and 12.6% in women) and that of maculopathy, 7.9% (11.7% in men and 5.2% in women). The conditions were significantly correlated with each other (P = .002). Also, both displayed a significant direct correlation with age and altitude (for pseudoexfoliation, P<.001 and P = .002 for age and altitude, respectively; for age-related macular degeneration, P<.001 for age and for altitude) and an increase in bilateral incidence with progressing age. CONCLUSIONS: The observed prevalences of pseudoexfoliation and maculopathy were lower than those reported in the mainland of Greece and other Mediterranean regions. The correlation between age-related macular degeneration and pseudoexfoliation syndrome may be explained by the relationship of each disease with age and altitude.
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Síndrome de Exfoliación/epidemiología , Degeneración Macular/epidemiología , Adulto , Anciano , Anciano de 80 o más Años , Síndrome de Exfoliación/complicaciones , Femenino , Grecia/epidemiología , Humanos , Degeneración Macular/clasificación , Degeneración Macular/complicaciones , Masculino , Persona de Mediana Edad , PrevalenciaRESUMEN
Three patients are presented to whom an accidental needle entrance into the maxillary sinus occurred during retrobulbar anesthesia. In all cases air was aspirated during the aspiration check at the end of needle advancement, and the patients reported a bitter taste after the injection of a small quantity of anesthetic. After withdrawal and careful reinsertion of the needle, maxillary sinus entrance was avoided and a successful retrobulbar block was achieved. Two of the patients had no history of previous facial trauma or surgery, whereas the third had suffered a recent blowout fracture of the orbital floor. The authors suggest that air aspiration or anesthetic passage into the pharynx during retrobulbar anesthesia should raise the suspicion of maxillary sinus entrance, even in patients without any history of facial trauma or surgery. The early recognition of sinus entrance can prevent retrobulbar block failure and reinjection of a second anesthetic dose.
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Anestesia Local/efectos adversos , Seno Maxilar/lesiones , Agujas/efectos adversos , Lesiones por Pinchazo de Aguja/etiología , Anciano , Anestésicos Combinados/administración & dosificación , Anestésicos Locales/administración & dosificación , Extracción de Catarata , Humanos , Masculino , Seno Maxilar/diagnóstico por imagen , Lesiones por Pinchazo de Aguja/diagnóstico por imagen , Bloqueo Nervioso , Órbita , Tomografía Computarizada por Rayos XRESUMEN
BACKGROUND AND OBJECTIVES: To evaluate the morphologic and functional effects of ciliary body photodynamic therapy (PDT) using phthalocyanine and a diode laser. MATERIALS AND METHODS: The upper half of the left eye ciliary body of 16 albino rabbits was irradiated transsclerally using a 670-nm diode laser (400 mW/cm2) after intravenous injection of phthalocyanine (6 mg/kg). The animals were observed for a maximum of 2 months by means of tonometry, biomicroscopy, and fundus examination. At the end of the follow-up period, they were killed and their eyes were prepared for light and electron microscopy. RESULTS: Transscleral PDT resulted in intraocular pressure (IOP) reduction in the treated eye, which lasted about 2 weeks. During this time, the treated eye had IOP values that were significantly lower than its baseline IOP values and the IOP values of the untreated eye (P < .05). One month after the procedure, the IOP had returned to baseline values. Histologic examination revealed vascular endothelial cell damage causing vascular thrombosis in the treated areas. The architecture of the two ciliary epithelium layers showed a significant abnormality. Disappearance of epithelial apical junction complexes and loss of the normal b-cytomembrane enfolding were observed in the course of electron microscopic examination. Large intercellular spaces between epithelial cells were noticed. All of these changes had subsided by the end of the second postoperative month. CONCLUSION: Transscleral phthalocyanine-mediated PDT with the parameters used in this experiment results in significant but temporary functional and morphologic alterations in the ciliary bodies of albino rabbits.
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Cuerpo Ciliar/efectos de los fármacos , Indoles/uso terapéutico , Terapia por Láser , Fotoquimioterapia/métodos , Fármacos Sensibilizantes a Radiaciones/uso terapéutico , Animales , Segmento Anterior del Ojo/efectos de los fármacos , Segmento Anterior del Ojo/patología , Segmento Anterior del Ojo/efectos de la radiación , Velocidad del Flujo Sanguíneo , Cuerpo Ciliar/irrigación sanguínea , Cuerpo Ciliar/efectos de la radiación , Conjuntiva/irrigación sanguínea , Conjuntiva/efectos de los fármacos , Conjuntiva/efectos de la radiación , Modelos Animales de Enfermedad , Endotelio Vascular/efectos de los fármacos , Endotelio Vascular/ultraestructura , Estudios de Seguimiento , Indoles/administración & dosificación , Infusiones Intravenosas , Presión Intraocular , Isoindoles , Pigmentos Biológicos/administración & dosificación , Pigmentos Biológicos/uso terapéutico , Conejos , Fármacos Sensibilizantes a Radiaciones/administración & dosificación , Desprendimiento de Retina/patología , Desprendimiento de Retina/fisiopatología , Vasos Retinianos/efectos de los fármacos , Vasos Retinianos/fisiopatología , Vasos Retinianos/efectos de la radiación , Esclerótica/irrigación sanguínea , Esclerótica/efectos de los fármacos , Esclerótica/efectos de la radiación , Trombosis/tratamiento farmacológicoRESUMEN
PURPOSE: We evaluated the effective irradiation parameters for photodynamic thrombosis of experimental corneal neovascularization enhanced by simultaneous hyperbaric oxygenation. METHODS: Neovascularization was provoked in both eyes of each of 35 albino rabbit corneas using the intracorneal suture technique. The lasered animals were divided in 3 groups. Group 1 (10 rabbits) was treated under hyperbaric conditions (28 atm for 25 min.); group 2 (5 rabbits) was treated breathing pure oxygen delivered by a face mask; group 3 (10 rabbits) was treated breathing room air. The fourth group (10 rabbits) was used for control. Animals were anaesthetized, and irradiation of new corneal vessels was carried out 30 minutes after the injection of 5 mg/kg chloroaluminum sulfonated phthalocyanine. A 670 nm diode laser with a power 4 mW and a spot diameter 350 mm was used. Exposure times necessary for vascular occlusion were registered. Histological examination was carried out at the end of the follow-up time. RESULTS: Exposure times were significantly lower in groups 1 and 2 as compared to group 3 (1.75 +/- 0.15 min., 3.1 +/- 0.4 min., and 4.75 +/- 0.15 min. respectively). Total light dose averaged 490 J/cm,2 870 J/cm,2 and 1330 J/cm,2 respectively. Histological examination revealed thrombus formation in the targeted vessels of all three investigated groups. CONCLUSION: Combination of PDT with hyperbaric oxygenation results in an acceleration of the photodynamic process and provides for a possibility of significant reduction of photodynamic dose.
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Neovascularización de la Córnea/terapia , Oxigenoterapia Hiperbárica , Fotoquimioterapia , Animales , Capilares/patología , Neovascularización de la Córnea/patología , Indoles , Isoindoles , Conejos , Fármacos Sensibilizantes a RadiacionesRESUMEN
BACKGROUND AND OBJECTIVES: Photothrombosis is a relatively new photodynamic application leading to vascular occlusion. In the current work the effectiveness of phthalocyanine and a diode laser in photothrombosis of normal retinal and choroidal vessels was evaluated. STUDY DESIGN, MATERIALS AND METHODS: Big retinal vessels of temporal myelin wing were irradiated using a 670 nm diode laser (2 mW, 0.5 mm2) after the injection of chloroaluminum sulfonated phthalocyanine (5 mg/kg) in twenty albino rabbits. Animals were followed up to a maximum of 7 months using fundus photography, fluoroangiography, and histology. RESULTS: Photothrombosis of the irradiated retinal vessels and of underlying choroidal vessels resulted in all treated eyes after 13 to 17.5 min of irradiation. The retinal vessels were patent again by the 7th day after the procedure. Choroidal vessels remained closed during the whole follow-up period. Light and electron microscopy demonstrated occupation of irradiated choroidal and retinal vessels by platelet thrombi. Damage of endothelial cell structure of these vessels could be seen. Outer retinal and RPE damage localized at irradiation area was observed. CONCLUSION: The combination of phthalocyanine with a low power diode laser is a simple and effective way for the induction of photodynamic thrombosis in fundus vessels.
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Coroides/irrigación sanguínea , Indoles/uso terapéutico , Rayos Láser , Compuestos Organometálicos/uso terapéutico , Fotoquimioterapia , Fármacos Sensibilizantes a Radiaciones/uso terapéutico , Oclusión de la Arteria Retiniana/tratamiento farmacológico , Oclusión de la Vena Retiniana/tratamiento farmacológico , Vasos Retinianos/patología , Animales , Coroides/ultraestructura , Angiografía con Fluoresceína , Fondo de Ojo , Microscopía Electrónica , Neovascularización Patológica/tratamiento farmacológico , Conejos , Oclusión de la Arteria Retiniana/patología , Oclusión de la Vena Retiniana/patología , Vasos Retinianos/ultraestructuraRESUMEN
The aim of this study was to evaluate the possible influence of phthalocyanine dose and of time interval between phthalocyanine injection and irradiation commencement on the rate of experimental corneal neovascularization photodynamic thrombosis in albino rabbits. New corneal vessels were irradiated with a diode laser (670 nm, 2 mW) after the intravenous injection of chloroaluminum sulfonated phthalocyanine. Different animals were irradiated either 5 min after the injection of different phthalocyanine doses (3, 6, 8, 12, or 14 mg/kg), or at different times (5 min, 24 h, or 58 h) after a standard phthalocyanine dose (3 mg/kg) injection. Irradiation time necessary for vascular occlusion was recorded. Decrease of phthalocyanine dose as well as delay of irradiation onset resulted in a statistically significant increase of irradiation time. Electron and light histological examination revealed platelet thrombi inside irradiated corneal new vessels. Damage in the vascular endothelial cell membrane and in intercellular contact structure was noted, leading to disorganization of the endothelial cells layer and death of most endothelial cells. These results indicate that both early commencement of irradiation after phthalocyanine injection and phthalocyanine dose increase accelerate the rate of phthalocyanine mediated corneal neovascularization photodynamic thrombosis. Thrombosis seems to result from photochemically induced vascular endothelial cell damage.
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Neovascularización de la Córnea/tratamiento farmacológico , Indoles/uso terapéutico , Compuestos Organometálicos/uso terapéutico , Fotoquimioterapia , Fármacos Sensibilizantes a Radiaciones , Trombosis/tratamiento farmacológico , Aluminio , Animales , Córnea/irrigación sanguínea , Córnea/efectos de la radiación , Córnea/ultraestructura , Neovascularización de la Córnea/metabolismo , Neovascularización de la Córnea/patología , Modelos Animales de Enfermedad , Inyecciones Intravenosas , Terapia por Láser , Masculino , Conejos , Trombosis/metabolismo , Trombosis/patologíaRESUMEN
BACKGROUND: Achromobacter xylosoxidans is a bacterium not previously reported in a corneal infection. We present a case of infectious keratitis caused by this organism, occurring 1 month following penetrating keratoplasty. METHODS: Identification of the organism was accomplished by staining, culture, and sensitivity testing of the corneal scraping obtained from the involved area. The infection responded to subconjunctival ticarcillin, piperacillin eye drops, and I.V. azlocillin. RESULTS: Complete healing with a resultant corneal opacity involving mostly the anterior and middle stroma was achieved 1 month later. CONCLUSIONS: A. xylosoxidans should be considered as a potential pathogen in instances of early postkeratoplasty infectious keratitis.
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Alcaligenes , Infecciones Bacterianas del Ojo/microbiología , Infecciones por Bacterias Gramnegativas/microbiología , Queratitis/microbiología , Queratoplastia Penetrante/efectos adversos , Adulto , Humanos , MasculinoRESUMEN
We examined three patients who developed keratitis after myopic photorefractive keratectomy. All patients were treated on the same day and in all three cases paraformaldehyde tablets were used for disinfection of the excimer laser iris cone. All patients developed an intense postoperative corneal inflammation that resulted in corneal opacities. In the first patient, the opacities were central and persisted for at least 4 1/2 months postoperatively. He became more myopic than before the operation. His best spectacle-corrected visual acuity worsened by four Snellen lines. In the other two patients, the corneal opacities were paracentral and of less density. Six months postoperatively, these opacities were barely seen. In these two patients the postoperative best spectacle-corrected visual acuity remained unchanged or improved one Snellen line compared to the preoperative level. In all three patients, astigmatism increased postoperatively. These observations suggest that paraformaldehyde tablets are not safe for the disinfection of the excimer laser iris cone.
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Antiinfecciosos Locales/efectos adversos , Formaldehído/efectos adversos , Queratitis/inducido químicamente , Terapia por Láser/efectos adversos , Miopía/cirugía , Polímeros/efectos adversos , Adulto , Opacidad de la Córnea/inducido químicamente , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Complicaciones Posoperatorias , Agudeza VisualRESUMEN
Using a modified microkeratome, nasally based central corneal flaps were created on six human blind eyes at a depth of 300 microns. The flaps were allowed to heal with a bandage soft contact lens, using neither sutures nor bioadhesives. The optical quality of the corneas was evaluated with biomicroscopic examination, computer-assisted topography, and optical pachometry during a 3-month follow-up period. The maintenance of transparency and lack of distortion of the corneal surface 3 months after surgery support the concept that the flap technique may be useful in laser in situ keratomileusis.
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Trasplante de Córnea/métodos , Procedimientos Quirúrgicos Refractivos , Colgajos Quirúrgicos , Córnea/anatomía & histología , Trasplante de Córnea/instrumentación , Humanos , Terapia por Láser/instrumentación , Cuidados PosoperatoriosRESUMEN
We examined 92 normal eyes using Javal Ophthalmometer and Corneal Analysis System (EyeSys) intending to compare the keratometric readings obtained by these two instruments. Dioptric power and location at flatter and steeper meridians, keratometric astigmatism values, and average central keratometric power were measured in the central cornea of all eyes. Statistical analysis showed significant differences in flatter meridians' mean power values (mean difference = 0.13 D, P = .008), mean keratometric astigmatism values (mean difference = -0.20 D, P = .001) and steeper meridians' location (mean difference = 7 degrees, P = .0001). There was no significant difference in steeper meridians' mean power values (mean difference = -0.06 D, P = .38), flatter meridian location (mean difference = 5.9 degrees, P = .07), and mean average central keratometric power (mean difference = 0.03 D, P = .471). Clinically meaningful difference was observed only in keratometric astigmatism readings of eyes with keratometric astigmatism higher than 1.50 D (mean difference = -0.84 D). For all the other parameters measured, the differences between the two instruments readings were clinically acceptable.