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1.
Vestn Oftalmol ; 139(6): 69-76, 2023.
Artigo em Russo | MEDLINE | ID: mdl-38235632

RESUMO

Secondary glaucoma is one of the main problems of rehabilitation of patients with traumatic damage of the iris. PURPOSE: This study analyzes the long-term results of rehabilitation of patients with posttraumatic aniridia and glaucoma. MATERIAL AND METHODS: The study included 310 patients (310 eyes) with posttraumatic aniridia who had artificial iris-lens diaphragm (ILD) MIOL-Raduzhka implanted in 2002-2022. Before ILD implantation, 61 patients (22.8%) had secondary glaucoma. Among them 35 patients (11.3%) underwent various modifications of glaucoma surgery. In 26 patients (8.6%), intraocular pressure (IOP) was compensated medically before ILD implantation. RESULTS: Until the third month after ILD implantation, there was a trend for IOP increase in some patients. Decompensation was noted in 8 (22.9%) out of 35 patients who had underwent glaucoma surgery. IOP decompensation was observed in 21 (80.8%) of 26 cases in patients with glaucoma compensated by drugs before ILD implantation. After ILD implantation, glaucoma appeared for the first time in 21 patients (6.8%) out of 310. In order to compensate IOP after ILD implantation, Ahmed valve implantation was performed most often - in 35 cases (70%) out of 50, deep sclerectomy - in 5 cases (10%), non-penetrating deep sclerectomy - in 4 cases (8%), micropulse transscleral laser cyclophotocoagulation (MP-TSCPC) - in 5 cases (10%), endoscopic cyclophotocoagulation (ECP) - in 1 case (2%). CONCLUSION: ILD implantation in patients with posttraumatic aniridia and secondary glaucoma should be performed with IOP compensated without hypotensive therapy and not earlier than 6-12 months after glaucoma surgery. The most optimal glaucoma surgery types in this group of patients are Ahmed valve implantation and MP-TSCPC.


Assuntos
Aniridia , Glaucoma , Humanos , Implante de Lente Intraocular/métodos , Acuidade Visual , Aniridia/complicações , Aniridia/diagnóstico , Iris/cirurgia , Glaucoma/diagnóstico , Glaucoma/etiologia , Glaucoma/cirurgia , Pressão Intraocular , Estudos Retrospectivos , Resultado do Tratamento , Fotocoagulação a Laser
2.
Clin Genet ; 92(6): 639-644, 2017 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-28321846

RESUMO

Congenital aniridia is a severe autosomal dominant congenital panocular disorder, mainly associated with pathogenic variants in the PAX6 gene. The objective of the study was to investigate the mutational and clinical spectra of congenital aniridia in a cohort of 117 patients from Russia. Each patient underwent detailed ophthalmological examination. From 91 unrelated families, 110 patients were diagnosed with congenital aniridia and 7 with WAGR syndrome (Wilms tumor, Aniridia, Genitourinary anomalies, and mental Retardation syndrome). The clinical presentation in aniridia patients varied from the complete bilateral absence of the iris (75.5%) to partial aniridia or iris hypoplasia (24.5%). Additional ocular abnormalities were consistent with previous reports. In our cohort, we saw a previously not described high percentage of patients (45%) who showed non-ocular phenotypes. Prevalence of deletions coherent with WAGR syndrome appeared to be 19.4% out of sporadic patients. Among the other aniridia cases, PAX6 deletions were identified in 18 probands, and small intragenic changes were detected in 58 probands with 27 of these mutations being novel and 21 previously reported. In 3 families mosaic mutation was transmitted from a subtly affected parent. Therefore, PAX6 mutations explained 96.7% of aniridia phenotypes in this study with only 3 of 91 probands lacking pathogenic variants in the gene.


Assuntos
Aniridia/genética , Predisposição Genética para Doença , Mutação , Fator de Transcrição PAX6/genética , Síndrome WAGR/genética , Adulto , Alelos , Aniridia/diagnóstico , Aniridia/patologia , Estudos de Coortes , Éxons , Feminino , Expressão Gênica , Humanos , Lactente , Padrões de Herança , Íntrons , Masculino , Fenótipo , Federação Russa , Índice de Gravidade de Doença , Síndrome WAGR/diagnóstico , Síndrome WAGR/patologia
3.
Vestn Oftalmol ; 133(1): 60-69, 2017.
Artigo em Russo | MEDLINE | ID: mdl-28291202

RESUMO

AIM: to conduct a comparative analysis of the value of information provided by anterior segment optical coherence tomography (AS-OCT) and in vivo confocal laser scanning microscopy (CLSM) for identifying the palisades of Vogt (POV) in normal limbus. MATERIAL AND METHODS: POV were studied in 15 healthy participants (30 eyes) without any anterior segment disease. Two examination methods were used: AS-OCT En Face imaging (RTVue XR Avanti, 3D Cornea protocol) and CLSM (HRT3). Concordance of the results was then analyzed. RESULTS: Structural features of POV were distinguishable by both methods. CLSM was able to visualize POV in both the superior and inferior limbi of all the 15 patients (30 eyes). Within the inferior corneal hemisphere, POV had the appearance of well-differentiated hyperreflective double-contoured structures organized in parallel lines at the level of basal epithelium. The structure of the superior limbus, as judged from CLSM data, varied significantly: «classic¼ palisades were seen in only 13 eyes (43%), while in the other 17 cases (57%) POV were atypical. On OCT, POV structure was seen in detail in 29 eyes. By comparing the abilities of CLSM and OCT to visualize POV, a strong positive correlation (rS=0.99, p<0.05) was revealed between the results obtained in both the superior and inferior limbi. CONCLUSION: OCT data provide a detailed understanding of POV structure and strongly correlate with CLSM results. Taking into consideration the noninvasiveness, demonstrativeness, and repeatability of AS-OCT En Face imaging as well as the high value of provided information, the method cay be recommended as an alternative in POV diagnostics.


Assuntos
Limbo da Córnea/diagnóstico por imagem , Microscopia Confocal/métodos , Adulto , Pesquisa Comparativa da Efetividade , Feminino , Voluntários Saudáveis , Humanos , Masculino , Pessoa de Meia-Idade , Tomografia de Coerência Óptica/métodos
4.
Vestn Oftalmol ; 133(6): 30-44, 2017.
Artigo em Russo | MEDLINE | ID: mdl-29319667

RESUMO

AIM: to investigate the possible use of anterior segment optical coherence tomography (AS-OCT) and laser scanning confocal microscopy (LSCM) for visualization of limbal progenitor structures and epithelial changes at different stages of aniridia-associated keratopathy (AAK) and to analyze genotype-phenotype correlations of corneal damage. MATERIAL AND METHODS: Thirty-four patients (63 eyes) with congenital aniridia (CA) were subjected to epithelial cell density measurement in the central cornea as well as epithelial surface assessment with limbal palisades of Vogt (POV) detection in the corresponding sites of the two corneas. For that, LSCM (HRT3) and AS-OCT (RTVue XR Avanti) were performed. Central corneal and epithelial thicknesses were measured using the Pachymetry protocol. RESULTS: There has been found an increase in the central corneal thickness (CCT) of CA patients, which correlated with the stage of AAK, and a decrease in the central epithelial thickness as compared with healthy subjects (p<0.05). The difference between the basal and wing epithelial cells density in eyes with stages I and II AAK and normal cells density at stage 0 AAK was statistically significant (p<0.05). Intact or disturbed POV were detected in all patients with PAX6 3' deletion. At that, AS-OCT findings highly agreed with LSCM images for both the inferii (rS=0.85, p<0.05) and superior limbi (rS=0.53, p<0.05). A negative correlation was established between the stage of AAK and in vivo morphology of POV (rS=-0.5, p<0.05). However, no correlation was found between the stage of AAK and patient's age (rS=0.169, p=0.174). CONCLUSION: AS-OCT and LSCM are both important diagnostic tools for corneal surface monitoring in patients with limbal stem cells deficiency.


Assuntos
Aniridia , Córnea/diagnóstico por imagem , Doenças da Córnea , Microscopia Confocal/métodos , Tomografia de Coerência Óptica/métodos , Adolescente , Adulto , Aniridia/complicações , Aniridia/diagnóstico , Aniridia/genética , Córnea/patologia , Doenças da Córnea/diagnóstico , Doenças da Córnea/etiologia , Paquimetria Corneana/métodos , Feminino , Estudos de Associação Genética , Humanos , Masculino , Pessoa de Meia-Idade
5.
Int J Mol Med ; 26(1): 85-94, 2010 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-20514426

RESUMO

Novel amphiphilic poly-N-vinylpyrrolidone derivatives with different molecular weight of hydrophilic PVP fragment and one secondary di-n-alkyl terminal hydrophobic fragment of different length were synthesized to compare their inclination for formation of nano-scaled micelle-like aggregates in aqueous media with previously studied primary n-alkyl terminated poly-N-vinylpyrrolidones. The behavior of amphiphilies in water solutions was studied and critical aggregation concentration values for prepared polymer samples were determined by fluorescence spectroscopy and compared with those for primary n-alkyl derivatives. Polymeric micelle-like particles with or without encapsulated drug were prepared using dialysis or solvent evaporation techniques. Indomethacin was incorporated into hydrophobic inner core of these nanoparticles as a typical model drug. Dynamic light-scattering studies determined that the average size of particles formed was from 90 nm up to 600 nm with monodisperse size distribution and the nanoparticle size slightly increased with the amount of indomethacin encapsulated into inner core of the particles. In vitro release experiments carried out at different medium pH values using indomethacin-loaded nanoparticles exhibited slow and steady drug release into the medium.


Assuntos
Portadores de Fármacos/química , Indometacina/farmacocinética , Nanopartículas/química , Pirrolidinonas/química , Anti-Inflamatórios não Esteroides/química , Anti-Inflamatórios não Esteroides/farmacocinética , Composição de Medicamentos , Concentração de Íons de Hidrogênio , Interações Hidrofóbicas e Hidrofílicas , Indometacina/química , Cinética , Lipídeos/química , Microscopia Eletrônica de Transmissão , Nanopartículas/ultraestrutura , Tamanho da Partícula , Temperatura , Água/química
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