Structural Characteristics of the Lens in Presenile Cataract

The purpose of this work is to examine the structure of the anterior lens epithelial cells (aLECs) of presenile idiopathic cortical cataract to investigate the possible structural reasons for its development. The anterior lens capsules (aLCs: basement membrane and associated lens epithelial cells) were obtained from routine uneventful cataract surgery of 5 presenile cataract patients (16 and 41 years old women and 29, 39, and 45 years old men). None of the patients had family history of cataract, medication, or trauma and they were otherwise healthy. In addition, the patients did not have any other abnormal features in the ocular status except cataract. The aLCs were prepared for scanning electron microscopy (SEM) and transmission electron microscopy (TEM). The most prominent abnormal features observed by SEM for all 5 studied presenile cataract patients were the changes of the aLECs structure with the dents, the selective concavity of some LECs, at their apical side centrally toward the nucleus. In addition, TEM showed the thinning of the lens epithelium with the segmentally concave cells and the compressed and elongated nuclei. Abnormal and distinguishable structural features were observed in the anterior lens epithelium aLECs in all 5 patients with presenile cataract. Disturbed structure of aLECs, regularly present in presenile cataract type is shown that might be associated with water accumulation in the presenile idiopathic cortical cataract lens.

Correlation of Sunlight Exposure and Different Morphological Types of Age-Related Cataract

Purpose. The previous lab and clinical studies of the correlation between the ultraviolet B and age-related cataract (ARC) did not reach in the universal agreement, especially in different morphological types of ARC. It is important to systemically summarize those previous data of epidemiological studies, which might penetrate the relevance between three morphological types of ARC, cortical, nuclear, and posterior capsular (PSC), with sunlight exposure. Methods. PubMed, Web of Science, CNKI, Embase, and Cochrane were searched online. Data were extracted and recalculated, and quality check was performed by hand. Review Manager was used to perform the fixed effects meta-analysis on ARC and its morphological types. The highest exposed dose group was defined as the exposed group, and the lowest dose group as the control group as possible. Results. Finally, the number of analyzed studies was 31: 20 for ARC and twelve, eleven, and nine for the morphological types cortical, nuclear, and PSC, respectively. The pooled OR for ARC was 1.15 (range 1.00~43.78, 95% CI 1.09 to 1.21). The cortical cataract revealed a slightly higher risk, and pooled OR was 1.03 (range 0.67~2.91, 95% CI 1.02 to 1.03). But the pooled OR for nuclear and PSC were 1.00 (range 0.50~5.35, 95% CI 1.00 to 1.00) and 0.99 (range 0.57~1.87, 95% CI 0.95 to 1.01), respectively. Conclusions. The systemic analysis of epidemiological articles reported till now reveals a significantly increased risk of ARC for those exposed with more sunlight, especially the morphological type of cortical cataract.

A monoklonális gammopathia szemészeti jelei és szövődményei.

Összefoglaló. Célkitűzés: A monoklonális gammopathia szemészeti jeleinek és szövődményeinek vizsgálata. Betegek és módszerek: Két nagy budapesti hematológiai ellátóhely 1999 és 2020 között diagnosztizált és/vagy kezelt, monoklonális gammopathiát mutató betegeit vizsgáltuk (42 beteg 84 szeme, 42,86% férfi; átlagéletkor 63,83 ± 10,76 év). A hematológiai diagnózis 3 esetben bizonytalan jelentőségű monoklonális gammopathia, 34 esetben myeloma multiplex, 3 esetben parázsló myeloma, 1-1 esetben Waldenström-macroglobulinaemia és amyloidosis voltak. Kontrollcsoportként véletlenszerűen kiválasztott, hasonló korcsoportú, hematológiai betegség nélküli egyéneket vizsgáltunk (43 beteg 86 szeme, 32,56% férfi; átlagéletkor 62,44 ± 11,89 év). A szemészeti vizsgálat előtt minden személy kitöltötte a Szemfelszíni Betegség Index (OSDI-) kérdőívet. A szemészeti vizsgálat során a látóélesség vizsgálata mellett pupillatágítást követően réslámpás vizsgálatot végeztünk. Eredmények: Monoklonális gammopathiában az OSDI-érték szignifikánsan magasabb volt, mint a kontrollokban (p = 0,002). Gammopathiában 3 beteg 5 szeménél (5,95%) találtunk potenciális szaruhártya-immunglobulinlerakódást. Gammopathiában szárazszem-betegség 66,67%-ban, szürke hályog 55,95%-ban, Meibom-mirigy-diszfunkció 20,24%-ban, hátsó kérgi szürke hályog 19,05%-ban, egyéb szaruhártyahegek és -homályok 17,86%-ban, krónikus szemhéjgyulladás 14,29%-ban, szemészeti eltérés hiánya 11,90%-ban, macula- és/vagy retinadrusen 9,52%-ban, szaruhártya-immunglobulinlerakódás 5,95%-ban, epiretinalis membrán 5,95%-ban, korábbi szürkehályog-műtét 5,95%-ban, glaucoma 4,76%-ban, Fuchs-dystrophia 2,38%-ban, perifériás retinadegeneráció 2,38%-ban, chorioidea naevus 2,38%-ban, diabeteses retinopathia 1,19%-ban, arteria centralis retinae elzáródás 1,19%-ban, vena centralis retinae ágelzáródás 1,19%-ban, amblyopia 1,19%-ban volt kimutatható. A szárazszem-betegség (p = 0,002), a hátsó kérgi szürke hályog (p = 0,001), a szürke hályog (p<0,00001) és az egyéb szaruhártyahegek és -homályok (p = 0,01) szignifikánsan magasabb arányban fordultak elő a monoklonális gammopathiát mutató betegekben, mint a kontrollokban. Következtetés : Monoklonális gammopathiában a szárazszem-betegség és a szürke hályog a leggyakoribb szemészeti eltérés. A monoklonális gammopathia potenciális szemészeti jelei és szövődményei miatt javasoljuk a betegek évenkénti szemészeti ellenőrzését, életminőségük javítása érdekében. Orv Hetil. 2021; 162(38): 1533–1540. Summary. Objective: To examine ocular signs and ocular comorbidities in monoclonal gammopathy. Patients and methods: We analyzed patients from two large referral hematology centers in Budapest, who were diagnosed and/or treated with monoclonal gammopathy between 1997 and 2020 (84 eyes of 42 patients, 42.86% male, mean age 63.83 ± 10.76 years). Before the ophthalmic examination, the subjects filled in the Ocular Surface Disease Index (OSDI) questionnaire. Ophthalmic examination included visual acuity test and slit-lamp examination following dilation of the pupil. Results: OSDI scores were significantly higher in subjects with monoclonal gammopathy than in controls (p = 0.002). Among gammopathy subjects, we observed potential corneal immunoglobulin deposition in 5 eyes of 3 patients (5.95%). In gammopathy subjects, there was dry eye disease (66.67%), cataract (55.95%), Meibomian gland dysfunction (20.24%), posterior cortical cataract (19.05%), corneal scars and degenerations (17.86%), chronic blepharitis (14.29%), absence of ocular complaint (11.90%), macular or retinal drusen (9.52%), corneal immunoglobulin deposition (5.95%), epiretinal membrane (5.95%), previous cataract surgery (5.95%), glaucoma (4.76%), Fuchs dystrophy (2.38%), peripheral retinal degeneration (2.38%), chorioideal naevus (2.38%), diabetic retinopathy (1.19%), central retinal artery occlusion (1.19%), central retinal vein branch occlusion (1.19%) and amblyopia (1.19%). The proportion of dry eye disease (p = 0.002), posterior cortical cataract (p = 0.001), cataract (p<0.00001), and corneal scars and degenerations (p = 0.01) were significantly higher in gammopathy subjects than in controls. Conclusion: Dry eye disease and cataracts are the most common ocular comorbidities in patients with monoclonal gammopathy. Therefore, due to the potential ocular signs and comorbidities of monoclonal gammopathy, we suggest a regular, yearly ophthalmic checkup of these patients to improve their quality of life. Orv Hetil. 2021; 162(38): 1533–1540.

Ocular signs in Fabry Disease

Fabry disease is a rare, hereditary disease characterized by a deficiency of an enzyme, α galactosidase A (α gal A), responsible for progressive damage to many organs, leading to various symptomsn, Ocular damage, particularly to the cornea, is sometimes a precious element helping the positive diagnosis of the disease.We report the case of a 40-year-old patient diagnosed with Fabry disease, with bilateral conjunctival vascular toruosities , a "cornea verticillata and a peripheral cortical cataract. Better knowledge of ophthalmological signs, allows better screening and can participate in the evaluation of the effectiveness of substitute therapy. Keywords: α galactosidase A, cornea verticillata, hereditary, Farby disease