Epithelial-mesenchymal transdifferentiation in pediatric lens epithelial cells

PURPOSE. Posterior capsule opacification (PCO) is a complication after cataract surgery, particularly in children. Epithelial-mesenchymal transition (EMT) of lens epithelial cells, mediated by transforming growth factor beta (TGF beta), contributes to PCO. However, its pathogenesis in children is poorly understood. We correlated cell growth in culture with patient characteristics, studied gene expression of pediatric lens epithelial cells (pLEC), and examined the effects of TGF beta-2 on these cells in vitro. METHODS. Clinical characteristics of children with cataracts correlated with growth behavior of pLEC in vitro. mRNA expression of epithelial (alpha B-crystallin, connexin-43) and mesenchymal (alpha(V)-integrin, alpha-smooth muscle actin, collagen-I alpha 2, fibronectin-1) markers was quantified in pLEC and in cell line HLE-B3 in the presence and absence of TGF beta-2. RESULTS. Fifty-four anterior lens capsules from 40 children aged 1 to 180 months were obtained. Cell outgrowth occurred in 44% of the capsules from patients <= 12 months and in 33% of capsules from children aged 13 to 60 months, but in only 6% of capsules from children over 60 months. TGF beta-2 significantly upregulated expression of alpha B-crystallin (HLE-B3), alpha(V)-integrin (HLE-B3), collagen-I alpha 2, and fibronectin-1 (in pLEC and HLE-B3 cells). CONCLUSIONS. Patient characteristics correlated with growth behavior of pLEC in vitro, paralleling a higher clinical incidence of PCO in younger children. Gene expression profiles of pLEC and HLE-B3 suggest that upregulation of alpha(V)-integrin, collagen-I alpha 2, and fibronectin-1 are involved in EMT

Age-related distance esotropia--fusional amplitudes and clinical course.

BackgroundAge-related distance esotropia (ARDET) is a form of acquired strabismus that affects elderly patients and manifests as an esotropia greater with distance fixation. Limited information exists regarding fusional amplitudes and deficient divergence in this disorder.MethodsIn this retrospective study, patient characteristics, ocular alignment and motility, and clinical course of patients with ARDET were recorded. Fusional amplitudes were analyzed to assess whether deficient divergence was present in patients with ARDET.ResultsTwenty patients with ARDET were identified. Median age was 77.5 years (range, 59 to 89 years) and median best-corrected visual acuity was 20/25. Median esotropia angle with distance fixation was 5.5Δ (range, 2 to 18Δ); median angle with near fixation was esotropia 2.5Δ (range, exotropia 3Δ to esotropia 13Δ). Fusional divergence amplitudes were decreased in all but two patients. The median amplitude with distance fixation was 4.5Δ for break (range, 1 to 11Δ) and 2Δ for recovery (range, 0 to 9Δ). In 5 patients, the fusional divergence amplitude was as large or larger than the esotropia angle. Most patients remained stable throughout a mean follow-up of 18 months (range, 3 to 37 months). Nineteen patients were managed with prisms. Strabismus surgery was performed in one patient.ConclusionsFusional divergence amplitude was deficient in most but not all patients with ARDET. In this study, most patients with ARDET remained stable and free of diplopia with prism treatment

Biometric and structural ocular manifestations of Marfan syndrome.

To study biometric and structural ocular manifestations of Marfan syndrome (MFS).Observational, retrospective, comparative cohort study in a tertiary referral center on 285 MFS patients and 267 controls. Structural and biometric ocular characteristic were compared.MFS eyes were longer (axial length 24.25 ± 1.74 mm versus 23.89 ± 1.31 mm, p < 0.001) and had a flatter cornea than control eyes (mean keratometry 41.78 ± 1.80 diopters (D) versus 43.05 ± 1.51 D, p < 0.001). Corneal astigmatism was greater and the central cornea was thinner in MFS eyes (530.14 ± 41.31 μm versus 547.02 ± 39.18 μm, p < 0.001). MFS eyes were more myopic than control eyes (spherical equivalent -2.16 ± 3.75 D versus -1.17 ± 2.58 D, p < 0.001). Visual acuity was reduced (0.13 ± 0.25 logMAR versus 0.05 ± 0.18 logMAR, p < 0.001) and intraocular pressure was lower in MFS eyes (14.6 ± 3.4 mmHg versus 15.1 ± 3.2 mmHg, p = 0.01). Iris transillumination defects (ITD) were significantly more common in MFS eyes (odds ratio for MFS in the presence of ITD, 3.7). Ectopia lentis (EL) was only present in MFS eyes (33.4%). History of retinal detachment was significantly more common in MFS eyes. Glaucoma was equally common in both groups.ITD and EL are most characteristic findings in MFS. ITD and corneal curvature should be studied as diagnostic criteria for MFS. Visual acuity is reduced in MFS. MFS patients need regular eye exams to identify serious ocular complications

Absent optic chiasm presenting with horizontal nystagmus

A female infant with horizontal nystagmus and normal ophthalmic examination had isolated absence of the optic chiasm on magnetic resonance imaging. Eye movements were recorded on video and reviewed. Horizontal nystagmus without see-saw nystagmus was observed. Visual evoked potential showed inter-hemispheric asymmetry compatible with the absence of crossing chiasmal fibers. Systemic abnormalities in this patient included cleft lip, preauricular skin tags, broad thumbs, and an anteriorly positioned anus, suggestive of Townes - Brock syndrome. Copyright © SLACK Incorporated

Direction of lens subluxation in eyes of Marfan syndrome patients with ectopia lentis (n = 101 patients, 182 eyes).

<p>Direction of lens subluxation in eyes of Marfan syndrome patients with ectopia lentis (n = 101 patients, 182 eyes).</p

Biometry, dimensions and refractive error of eyes of Marfan patients and control eyes.

<p>Biometry, dimensions and refractive error of eyes of Marfan patients and control eyes.</p

Structural ocular findings in patients with Marfan syndrome (MFS) and controls.

<p>Total = number of patients with documented findings for the respective category, unilat. = unilateral, bilat. = bilateral.</p