Effect of Posterior Keratometry on the Accuracy of 10 Intraocular Lens Calculation Formulas: Standard Keratometry versus Total Keratometry.

PURPOSE: To investigate the effect of posterior keratometry (PK) on the accuracy of 10 intraocular lens (IOL) power calculation formulas using standard keratometry (K) and total keratometry (TK). METHODS: This is a retrospective consecutive case-series study. The IOL power was calculated using K and TK measured by IOLMaster 700 in 6 new-generation formulas (Barrett Universal II, Emmetropia Verifying Optical (EVO) 2.0, RBF Calculator 3.0, Hoffer QST, Kane, and Ladas Super Formula) and 4 traditional formulas (Haigis, Hoffer Q, Holladay 1, and SRK/T). The arithmetic prediction error (PE) and mean absolute PE (MAE) were evaluated. The locally-weighted scatterplot smoothing was performed to assess the relationship between PE and PK. RESULTS: A total of 576 patients (576 eyes) who underwent cataract surgery were included. Compared with using K, all formulas using TK showed a hyperopic shift in the whole group. Specifically, for eyes with PK exceeding -5.90 D, all formulas using TK exhibited a hyperopic shift (all P < 0.001), while eyes with PK less than -5.90 D showed a myopic shift (all P < 0.001). The MAE of new-generation formulas calculated with TK and K showed no statistical differences, while the MAE of traditional formulas with TK was larger (TK: 0.34 ~ 0.43 D; K: 0.33 ~ 0.42 D, all P < 0.05). CONCLUSIONS: The prediction bias of formulas with TK increased as PK deviated from -5.90 D. TK did not improve the prediction accuracy of new-generation formulas, and even performed worse in traditional formulas.

Autosomal Dominant Weill-Marchesani-Like Syndrome in a Chinese Family due to Novel Haplotypic Mutations in LTBP2.

INTRODUCTION: Weill-Marchesani syndrome (WMS) is a hereditary connective tissue disorder with substantial heterogeneity in clinical features and genetic etiology, so it is essential to define the full mutation spectrum for earlier diagnosis. In this study, we report Weill-Marchesani-like syndrome (WMS-like) change to autosomal dominance inheritance caused by novel haplotypic mutations in latent transforming growth factor beta-binding protein 2 (LTBP2). METHODS: Twenty-five members from a 4-generation Chinese family were recruited from Guangzhou, of whom nine were diagnosed with WMS-like disease, nine were healthy, and seven were of "uncertain" clinical status because of their young age. All members received detailed physical and ocular examinations. Whole-exome sequencing, Sanger sequencing, and real-time PCR were used to identify and verify the causative mutations in family members. RESULTS: Genetic sequencing revealed novel haplotypic mutations on the same LTBP2 chromosome associated with WMS-like, c. 2657C>A/p.T886K in exon 16 and deletion of exons 25-36. Real-time PCR and Sanger sequencing verified both mutations in patients with clinically diagnosed WMS-like, and in one "uncertain" child. In these patients, the haplotypic mutations led to ectopia lentis, short stature, and obesity. CONCLUSION: Our study revealed that WMS-like may be associated with haplotypic LTBP2 mutations with autosomal dominant inheritance.

Corneal biomechanics and their association with severity of lens dislocation in Marfan syndrome.

PURPOSE: To investigate corneal biomechanical properties and its associations with the severity of lens dislocation in patients with Marfan syndrome. METHODS: A total of 30 patients with Marfan syndrome and 30 age-, sex- and axial length (AL)-matched controls were recruited. Corneal biomechanical parameters of both groups were measured by CorVis ST and were compared between groups. Potential associations between corneal biomechanical parameters and severity of lens dislocation were also investigated. RESULTS: Lower applanation 1 velocity (A1V) (0.13 ± 0.004 vs. 0.15 ± 0.003, P = 0.016), shorter applanation 2 time (A2T)(22.64 ± 0.11 vs. 22.94 ± 0.11, P = 0.013), longer peak distance (PD) (5.03 ± 0.07 vs. 4.81 ± 0.05, P = 0.008), longer radius (R) of highest concavity (7.44 ± 0.16 vs. 6.93 ± 0.14, P = 0.012), greater Ambrosio relational thickness horizontal (ARTh) (603 ± 20 vs. 498 ± 12, P < 0.001), and integrated radius (IR) (8.32 ± 0.25 vs. 8.95 ± 0.21, P = 0.033) were detected among Marfan eyes compared with controls (all P < 0.05). Marfan individuals with more severe lens dislocation tended to have increased stiffness parameter as longer A1T, slower A1V, shorter A2T, slower application 2 velocity (A2V), smaller PD and smaller Distance Amplitude (DA) (P < 0.05). CONCLUSION: Marfan patients were detected to have increased corneal stiffness compared with normal subjects. Corneal biomechanical parameters were significantly associated with the severity of lens dislocation in Marfan patients.

Longitudinal changes of refractive error in preschool children with congenital ectopia lentis.

BACKGROUND: Congenital ectopia lentis (CEL) is a hereditary eye disease which severely impacts preschool children's visual function and development. This study aimed to evaluate the longitudinal changes in spherical equivalent (SE) refractive error in preschool children with CEL. METHODS: A retrospective cohort study was conducted at Zhongshan Ophthalmic Center, Guangzhou, China. Medical records of CEL patients under 6-year-old who were diagnosed with Marfan syndrome at the initial visit from January 2014 to March 2022 were collected and were divided into surgery and non-surgery groups. Mean change rate of SE in the two groups was evaluated, and the potential associated factors of SE change rate were investigated by mixed-effect regression model. RESULTS: A total of 94 preschool patients from 14 provinces of China were included. Among the 42 children of the surgery group, the mean age with standard deviation (SD) was 5.02 ± 0.81 years and patients experienced a myopic shift of -0.05 ± 0.09 D/month in average. The mean age with SD of the 52 children of the non-surgery group was 4.34 ± 1.02 years, and the mean myopic shift was -0.09 ± 0.14 D/month. The mixed-effect regression model identified that higher degree of myopia at baseline was associated with slower myopic shift both in surgery (ß = 0.901, 95% CI: 0.822 ~ 0.980, P < 0.001) and in non-surgery group (ß = 1.006, 95% CI: 0.977 ~ 1.034, P < 0.001) in CEL patients. Surgical treatment (ß = 2.635, 95% CI: 1.376 ~ 3.894, P < 0.001) was associated with slower myopic shift in all participants CEL patients. CONCLUSIONS: Myopic progression was slower in the surgery group than in the non-surgery group of CEL. Preschool CEL patients who met the surgical indication are suggested being performed with timely surgery to slow down the myopic progression.

Identification and phenotypic analysis of novel LTBP2 mutations in a Chinese cohort with congenital ectopia lentis.

Purpose: To evaluate the frequency of LTBP2 mutations and to elaborate on LTBP2-related clinical phenotypes in a Chinese congenital ectopia lentis (CEL) cohort. Methods: In total, 145 Chinese probands with CEL were recruited for this study and underwent ocular and systemic examinations. Whole-exome sequencing was used to identify mutations, and Sanger sequencing and bioinformatics analysis were further performed to verify pathogenic mutations. Results: Overall, biallelic mutations in LTBP2 involving eight novel mutations (c.4370-7_4370-9delTCT, c.4370-5C>G, c.3452G>A, c.2253delG, c.4114T>C, c.1251G>A, c.4760G>A, and c.620G>A) were identified in four CEL probands (4/145, 2.76%). Patients with LTBP2 mutations were characterized by a megalocornea, spherophakia, high myopia, and glaucoma instead of a flat cornea, high corneal astigmatism, cardiovascular and skeletal abnormalities that were reported in other gene mutations. A novel homozygous frameshift mutation was detected, and this type of mutation was found to cause more complicated ocular symptoms than others, ranging from the anterior segment to the fundus. Conclusion: This study reported the mutation frequency of the LTBP2 gene in a Chinese CEL cohort and provided novel insight into LTBP2-related genotype-phenotype associations in CEL.

Characteristics and genotype-phenotype correlations in ADAMTS17 mutation-related Weill-Marchesani syndrome.

Weill-Marchesani syndrome (WMS) manifests as ectopia lentis (EL), microspherophakia and short stature, which is caused by ADAMTS10, LTBP2, or ADAMTS17 gene defects. This study aims to investigate the characteristics and genotype-phenotype correlations of WMS with ADAMTS17 mutations. WMS patients with ADAMTS17 variants were identified by whole-exome sequencing from 185 patients with EL. All the included patients underwent comprehensive ocular and systemic examinations. ADAMTS17 variants were reviewed from included patients, published literature, and public databases. Bioinformatics analysis, co-segregation analysis, species sequence analysis, and protein silico modeling were used to verify the pathogenic mutations. A total of six novel ADAMTS17 mutations (c.1297C > T, c.2948C > T, c.1322+2T > C, c.1716C > G, c.1630G > A, and c.1669C > T) were identified in four WMS probands in our EL cohort (4/185, 2.16%). All probands and their biological parents presented with apparent short stature compared with the standard value. In particular, one child was detected with valvular heart disease, which has not previously been reported in patients with ADAMTS17 mutations. Conserved residues were greatly affected by the substitution of amino acids caused by these six mutations. Short stature could be considered a clue for EL patients with ADAMTS17 mutations, and much more attention needs to be paid to heart disorders among these patients. This study not only reported the characteristics of ADAMTS17 mutation-related WMS but also helped to recognize the genotype-phenotype correlations in these patients.

Ocular, cardiovascular, and genetic characteristics and their associations in children with Marfan syndrome and related fibrillinopathies.

PURPOSE: Congenital ectopia lentis (CEL) and heart abnormalities are common clinical symptoms in patients with Marfan syndrome (MFS) and related fibrillinopathies, which is caused by mutations in fibrillin-1 (FBN1) gene. This study aims to explore the ocular and cardiovascular characteristics and their association with genotype in children with MFS and related fibrillinopathies. METHODS: Seventy-nine children diagnosed with CEL and with FBN1 mutations confirmed via whole-exome sequencing were included for genotypes and phenotypes analysis. The axial length (AL), corneal curvature, and refractive status were included for ocular phenotypes analysis. The cardiovascular examination was performed by echocardiography, and aortic root Z score was calculated to evaluate the severity of aortic dilatation. The heart disorders were classified as aortic root dilatation, valvular disorders, and others. Both the ocular and cardiac manifestations were collected for comprehensive analysis and compared among patients with different genotypes, including the mutation involving cysteine substitution or mutation in different regions. RESULTS: In CEL children with FBN1 mutations, 77.2% patients could be diagnosed as MFS. It was observed that children with mutations in exons 22-42 had significant higher aortic root Z score (P = 0.003) and higher incidence of cardiovascular disorders (P = 0.004). Additionally, children with cysteine substitution mutations had significant higher aortic root Z score (P = 0.011), and the aortic root Z score was positively associated with axial length (AL) in children under 6 years old (P = 0.035). Those with long AL (≥ 26 mm) had significant higher incidence of valve disorders (P = 0.023). In addition, nearly half the children with CEL (46.8%) were diagnosed with cardiovascular disease for the first time. CONCLUSIONS: CEL children with FBN1 mutations involving cysteine substitution or mutations in exons 22-42 or with long AL had higher risks of severe cardiovascular complications. Knowing the phenotype may help in anticipating severe cardiovascular disease in CEL patients.

Performance enhancement of solar cells based on high photoelectric conversion efficiency of h-BN and metal nanoparticles.

In this article, we propose a new type of CdTe thin-film solar cell based on a CdTe/CdS heterojunction. We used the finite difference time domain method to simulate the propagation of electromagnetic waves in the time domain under certain boundary conditions and the change in the absorption rate of cells when optimising the structure. The simulation shows that the light absorption rate of the cell is significantly enhanced after adding h-BN and metal particles to the proposed structure. Under the irradiation of standard light AM1.5 with the wavelength range of 300 nm to 1000 nm, presenting a 90% absorption bandwidth over 700 nm, and the average absorption rate is as high as 92.9%. The short-circuit current and open-circuit voltage are 30.98 mA/cm2 and 1.155 V, respectively, and the photoelectric conversion efficiency (PCE) increases to 30.76%, which is an increase of 27.58% compared to the original PCE. The result shows that, after metal nanoparticles are embedded in the absorption layer of the cell, the free electrons on the surface of the metal particles oscillate under the action of light. The electromagnetic field is confined to a small area on the surface of the particles and is enhanced, which is beneficial for the absorption of light by the cells. This study provides a basis for theoretical research and feasible solutions for the manufacture of thin-film solar cells with a high absorption rate and high efficiency.

CHARACTERISTICS OF THE FOVEAL MICROVASCULATURE IN CHILDREN WITH MARFAN SYNDROME: An Optical Coherence Tomography Angiography Study.

PURPOSE: To investigate the characteristics of foveal microvasculature in children with Marfan syndrome (MFS). METHODS: Ninety eyes from 45 MFS patients and 76 eyes from 38 healthy individuals of age-matched, sex-matched, and axial length-matched were enrolled. Characteristics of the superficial capillary plexus including the vessel density, perfusion density, and foveal avascular zone were analyzed by optical coherence tomography angiography. RESULTS: The vessel density and the circularity index of the foveal avascular zone were significantly decreased in the MFS group compared with the controls (P = 0.017 and P = 0.004 respectively). In MFS group, the central vessel density (P = 0.003) and perfusion density (P = 0.001) were negatively correlated with the best-corrected visual acuity. The foveal avascular zone area was correlated with the aortic diameters (P = 0.001) and the paratemporal perfusion density was correlated with the ejection fraction (P = 0.003). Moreover, the paratemporal perfusion density and the circularity index of foveal avascular zone were found to be correlated with the aortic Z-score (P < 0.001 and P = 0.003 respectively). CONCLUSION: Retinal microvascular decrease and its correlation with best-corrected visual acuity and cardiac functions were observed in the MFS group. The optical coherence tomography angiography may help to characterize the underlying pathophysiology features of MFS and enable early detection and prevention of vascular changes in MFS.

Mutation spectrum and genotype-phenotype correlations in Chinese congenital ectopia lentis patients.

PURPOSE: To identify the spectrum and frequency of mutations in congenital ectopia lentis (CEL) and to investigate the correlations between genotype and clinical phenotype in Chinese CEL patients. METHODS: Ninety-three participants with CEL were enrolled from March 2017 to April 2020. Ocular and systemic examinations were performed for each included patient. Genomic DNA from the included patients was analysed by whole-exome sequencing to detect mutations. Clinical manifestations were compared for different mutation subgroups. RESULTS: Gene mutations were detected in 79 patients. Sixty-five were FBN1-associated, and most were related to Marfan syndrome (MFS). The FBN1 mutations mainly consisted of missense mutations (49/65) and were concentrated in the 5' region. Probands with missense mutations tend to show high corneal astigmatism (χ2 = 3.98, P = 0.046) and severe lens dislocation (t = 2.90, P = 0.006) compared to premature termination codon (PTC) mutations. CONCLUSIONS: Most Chinese CEL patients were identified as having FBN1 mutations. Those with missense mutations commonly showed severe ocular phenotypes; therefore, reinforced follow-up and long-term observation are required. These correlations implicated the crucial role of missense and cysteine-involving mutations in ocular phenotypes, which might be explained by dominant-negative and nonsense-mediated mRNA decay (NMD).

Global disease burden of uncorrected refractive error among adolescents from 1990 to 2019.

BACKGROUND: To estimate the global disease burden of uncorrected refractive error (URE) among adolescents and assess the contributions of various risk factors to disability-adjusted life-years (DALYs) due to URE. METHODS: Global, regional and country-level DALY numbers and rates due to URE among adolescents were acquired from the Global Burden of Disease Study 2019 database. Human Development Index (HDI), Socio-Demographic Index (SDI) and other country-level data were obtained from other open databases as potential indicators. Regression analysis was used to evaluate associations between DALY rates among adolescents and potential predictors. RESULTS: Global DALYs due to URE among adolescents rose by 8% between 1990 and 2019 but moderately decreased by 4.8% during this period after adjusting for population size. Female adolescents showed higher DALY rates. DALY rates sharply increased from 5 to 9 years of age, then rose more slowly, reaching a plateau before 20 years of age. Country-level DALY rates in 2019 were positively associated with HDI, SDI, and urbanization rates but negatively correlated with primary school dropout rates. Higher disease burden of adolescents visually impaired from URE was associated with lower primary school dropout rates (ß = - 0.257, 95% CI - 0.376 to - 0.138, P < 0.001) and higher urbanization rates (ß = 0.257, 95% CI 0.067 to 0.256, P = 0.001). CONCLUSIONS: Higher socioeconomic status, urbanization rates and education levels are associated with a heavier disease burden of URE among adolescents. The findings of this study can provide a reference for policy making on resource allocation for URE prevention and control in teenagers.

Prevalence of age-related macular degeneration in Chinese populations worldwide: A systematic review and meta-analysis.

IMPORTANCE: Age-related macular degeneration (AMD) is a leading cause of irreversible blindness, but the magnitude of AMD among Chinese populations worldwide is still unknown. BACKGROUND: To investigate the prevalence of AMD in Chinese populations worldwide. DESIGN: Meta-analysis. PARTICIPANTS: Nine studies with 29 344 subjects in total. METHODS: All population-based studies on AMD prevalence in Chinese populations worldwide were identified and only studies using standardized AMD grading systems (Wisconsin Age-Related Maculopathy Grading System, Age-Related Eye Disease Study System of Classifying AMD, the International Classification and Grading System for AMD or the Clinical Classification of Age-Related Macular Degeneration) were included. We used meta-analysis to estimate the pooled prevalence and its 95% confidence interval (95% CI) of AMD, and to explore the racial differences and regional differences. MAIN OUTCOME MEASURES: Age-specific prevalence, gender-specific prevalence and pooled prevalence of early and late AMD among Chinese population worldwide. RESULTS: Altogether, 9 studies with 29 344 individuals were included and analysed. The crude pooled prevalence of early and late AMD among Chinese populations worldwide aged 50 years and above is 4.9% (95% CI: 3.1%-7.7%) and 0.7% (95% CI: 0.5%-1.1%), respectively. Corresponding crude prevalence among Caucasian populations are 10.1% (95% CI: 5.7%-17.2%) and 1.6% (95% CI: 1.0%-2.4%). There are statistically significant differences within age and gender subgroups. CONCLUSIONS AND RELEVANCE: Among persons aged 50+ years, both early AMD and late AMD in Chinese populations worldwide were less common compared with that reported from Caucasian populations. Considering the significant racial or ethnic differences in AMD prevalence between Chinese and Caucasian people, further studies are needed to explore the possible mechanism behind this discrepancy.

Distribution of axial length in Chinese congenital ectopia lentis patients: a retrospective study.

BACKGROUND: Congenital ectopia lentis (CEL) usually leads to refractive error and may influence the axial length development. But few investigations have reported patient demographics and the distribution of axial length (AL) before surgery in Chinese pediatric patients with CEL. To describe the distribution of AL before surgery in CEL patients and its relationship with patients' demographics, such as age, Marfan syndrome, sex, and laterality. METHODS: This retrospective study reviewed 306 CEL patients from January 1, 2006 to December 31, 2015. One eye was randomly selected from each patient if both eyes were EL. The influences of Marfan syndrome, sex, and laterality to AL in different age subgroups were evaluated and compared. The differences of the AL between groups were assessed using the student t test or paired t-test. P-values less than 0.05 were considered statistically significant. RESULTS: Two hundred forty-seven eyes were enrolled. 58.3% of all the patients had binoculus EL, 70% of all the patients were male and 36% of all the patients were diagnosed with Marfan syndrome. The mean AL of EL patients was 25.1 ± 2.5 mm. There was no statistical difference in the AL between patients with and without Marfan syndrome, and in the AL between male and female patients. There was statistical difference in AL between the EL-affected eye and the unaffected eye in monocular EL patients younger than 12 years old. CONCLUSIONS: This study suggests that AL can be influenced by CEL, but the influence of CEL may be reduced after the age of 12 years old, which will likely provide a useful reference when considering the most appropriate time of surgery.

A case of alveolar soft tissue sarcoma detected by ultrasound examination.

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Postoperative longitudinal refractive changes in children younger than 8 years with ectopia lentis and Marfan syndrome.

PURPOSE: To evaluate the postoperative longitudinal refractive changes in children younger than 8 years with ectopia lentis and Marfan syndrome (MFS). SETTING: Zhongshan ophthalmic center, Guangzhou, China. DESIGN: Retrospective cohort study. METHODS: Medical data of patients diagnosed with ectopia lentis and MFS that underwent surgery younger than 8 years were collected. Refractive errors and ocular biometric parameters were collected preoperatively and at each follow-up visit. Patients were stratified into groups according to age at surgery, and only the eye operated on first was selected. Multivariate analysis was performed to determine the association between refractive shift and potential risk factors. RESULTS: In total, 54 eyes of 54 patients were enrolled. The median age at surgery was 6.21 years (interquartile range [IQR], 5.25 to 6.85), and the median follow-up was 2.0 years (IQR, 1.2 to 2.8 years). At age 8 years, patients demonstrated a median myopic shift ranged from -1.75 diopters (D) (IQR, -2.75 to -1.00 D) for the 4-year-old group to -0.13 D (IQR, -0.50 to -0.06 D) for the 7-year-old group. Multivariate analysis showed that greater myopic shift was associated with younger age at surgery ( P = .004), male sex ( P = .026), and shorter preoperative axis length ( P = .005). CONCLUSIONS: A tendency toward increasing postoperative myopic was demonstrated in children with ectopia lentis and MFS, with the greatest myopic shift in the younger age groups. If the goal is to reach emmetropia by age 8 years, the immediate postoperative hypermetropic targets should be 1.75 D for age 4 years, 1 D for age 5 years, 0.5 D for age 6 years, and 0 to 0.25 D for age 7 years.

Network Meta-analysis of Intraocular Lens Power Calculation Formula Accuracy in 1016 Eyes With Long Axial Length.

PURPOSE: To systematically review the literature and quantitatively synthesize the currently available evidence to compare the accuracy of different intraocular lens calculation formulas in eyes with long axial length (AL). DESIGN: Network meta-analysis. METHODS: PubMed, Embase, Web of Science, and the Cochrane Library were systematically searched for studies published between January 2000 and June 2022. Included were prospective or retrospective clinical studies reporting the following outcomes in cataract patients with long AL (ie, ≥26 mm): percentage of eyes with a prediction error (PE) within ±0.25, ±0.50, and ±1.00 diopters (D). Network meta-analysis was conducted using R software (version 4.2.1). RESULTS: Ten prospective or retrospective clinical studies, including 1016 eyes and 11 calculation formulas, were identified. A traditional meta-analysis showed that for the percentage of eyes with PE within ±0.25 and ±0.50 D, the Olsen, Kane, and Emmetropia Verifying Optical (EVO) all had insignificantly higher percentages compared with others. Considering the percentage of eyes with PE within ±1.00 D, the original and modified Wang-Koch adjustment formulas for Holladay 1 (H1-WK and H1-MWK) and EVO formulas showed superiority, but the difference was insignificant. This network meta-analysis revealed that compared with the widely used Barrett Universal II (BUII) formula, the Olsen, Kane, and EVO formulas had higher percentages of eyes with PE within ±0.25, ±0.50, and ±1.00 D (all odds ratios >1 but P >.05). Based on the surface under the cumulative ranking area (SUCRA) values for the percentage of eyes with PE within ±0.25 D, the Olsen (96.4%), Kane (77.5%), and EVO (75.9%) formulas had the highest probability of being in the top 3 of the 11 formulas. CONCLUSIONS: The Olsen, Kane, and EVO formulas may perform better than others in calculating IOL power in eyes with long AL. Nevertheless, there is still considerable uncertainty in this regard and the accuracy of these formulas in highly myopic eyes should be confirmed in studies based on large multicenter registries.

Depressive and anxiety symptoms in caregivers of children with congenital ectopia lentis: a prospective cross-sectional study.

OBJECTIVES: To evaluate the frequency and severity of depressive and anxiety symptoms and explore possible risk factors among caregivers of children with congenital ectopia lentis (CEL). DESIGN: A prospective cross-sectional study was conducted. PARTICIPANTS: 108 patients and 108 informal caregivers (mainly parents) were included. Participants were grouped based on whether patients had systemic abnormalities: group 1 were children without systemic abnormalities and group 2 were children with systemic abnormalities. OUTCOME MEASURES: The 9-item Patient Health Questionnaire (PHQ-9) and the 7-item Generalized Anxiety Disorder Scale (GAD-7) were used to assess depressive and anxiety symptoms, respectively. RESULTS: More than half of caregivers (51.9%) have depressive or anxiety symptoms of some degree. 38.0% of caregivers suffered from both depressive and anxiety symptoms. 19.4% of caregivers had moderate to severe depressive symptoms (PHQ-9 score ≥10) while 16.7% reported moderate to severe anxiety symptoms (GAD-7 score ≥10). Between the two groups, the mean PHQ-9 and GAD-7 scores significantly differed (p=0.026 in PHQ-9; p=0.018 in GAD-7). The proportion of caregivers with moderate to severe symptoms was greater in group 2 than in group 1. In addition, there was a significant positive correlation between PHQ-9 and GAD-7 scores (r=0.827; p<0.001). Furthermore, best corrected visual acuity in the better eye of patients was positively correlated with both the PHQ-9 and GAD-7 scores (r=0.314, p<0.05 in PHQ-9; r=0.325, p<0.05 in GAD-7). CONCLUSIONS: Depressive and anxiety symptoms were common in caregivers of children with CEL, especially among those whose children had other systemic disease manifestations or low vision. This study illustrates the importance of depressive and anxiety symptom screening for these caregivers to implement effective psychological interventions and support strategies.

Changes in corneal endothelial cells after scleral-sutured fixation of intraocular lens in children with congenital ectopia lentis: a 2-year follow-up.

PURPOSE: To investigate the changing patterns of corneal endothelial cells and the associated factors in children with congenital ectopia lentis (CEL) after scleral-sutured fixation of intraocular lens (SSFIOL). SETTING: Zhongshan ophthalmic center, Guangzhou, China. DESIGN: Retrospective study. METHODS: Patients were divided into the surgery group and the control group. Central endothelial cell density (ECD), coefficient of variation in cell size (CV), the percentage of hexagonal cells (hexagonality, HEX), average cell size (AVG) and central corneal thickness (CCT) were analyzed for both group at baseline and each follow-up visit. Clinic characteristic, ocular parameters, IOL decentration and IOL tilt of patients in the surgery group were collected. Multiple linear regression was performed to assess the potential associated factors for the postoperative changes in corneal endothelial cells in the surgery group. RESULTS: After 2-year follow-up, the decline of ECD was 17.8% (95%CI: -21.8 to -13.9) in the surgery group and -3.1% (95%CI: -5.2 to -1.0) in the control group(P<0.001), while the increase of AVG was 24.3%(17.1to 31.6) in the surgery group and 2.7%(1.0 to 4.5) in the control group (P<0.001). Multivariate analysis showed that AL≥24mm and WTW<12.2mm were significantly associated with greater loss of ECD (ß=-241.41, 95% CI: -457.91 to -24.91, P=0.030 and ß=251.63, 95% CI: 42.10 to 461.17,P=0.020, respectively) and AL≥ 24mm was significantly positively associated with the increase of AVG (ß=34.81, 95% CI: 0.90 to 68.71, P=0.044). CONCLUSIONS: SSFIOL has a significant impact on corneal endothelium in children with CEL. More attention should be paid to monitor postoperative corneal endothelium change during long-term follow-up in CEL children, especially for those with longer AL and smaller WTW.

The burden, causes, and determinants of blindness and vision impairment in Asia: An analysis of the Global Burden of Disease Study.

Background: Asia accounts for more than half of the world's population and carries a substantial proportion of the global burden of blindness and vision impairment. Characterising this burden, as well as its causes and determinants, could help with devising targeted interventions for reducing the occurrence of blindness and visual impairment. Methods: Using the Global Burden of Disease Study 2019 database, we retrieved data on the number of disability-adjusted life years (DALYs); crude and age-standardised rates; and the prevalence (with 95% uncertainty intervals (95%UIs)) of blindness and vision loss due to six causes (age-related macular degeneration, cataracts, glaucoma, near-vision impairment, refractive error, and other vision loss) for Asian countries for the period between 1990 and 2019. We defined DALYs as the sum of the years lost due to disability and years of life lost, and calculated age-standardised figures for the number of DALYs and prevalence by adjusting for population size and age structure. We then evaluated the time trend of the disease burden and conducted subgroup analyses by gender, age, geographic locations, and socio-demographic index (SDI). Results: In 2019, the DALYs and prevalence of blindness and vision loss had risen by 90.1% and 116% compared with 1990, reaching 15.84 million DALYs (95% UI = 15.83, 15.85) and 506.71 million cases (95% UI = 506.68, 506.74). Meanwhile, the age-standardised rate of DALYs decreased from 1990 to 2019. Cataracts, refractive error, and near vision impairment were the three most common causes. South Asia had the heaviest regional disease burden (age-standardised rate of DALYs = 517 per 100 000 population; 95% UI = 512, 521). Moreover, the burden due to cataracts ranked high in most Asian populations. Being a woman; being older; and having a lower national SDI were factors associated with a greater vision loss burden. Conclusions: The burden due to vision loss remains high in Asian populations. Cataracts, refractive error, and near vision loss were the primary causes of blindness and vision loss. Greater investment in ocular disease prevention and care by countries with lower socioeconomic status is needed, as well as specific strategies targeting cataract management, women and the elderly.

Predicting Marfan Syndrome in Children With Congenital Ectopia Lentis: Development and Validation of a Nomogram.

Purpose: To derive an effective nomogram for predicting Marfan syndrome (MFS) in children with congenital ectopia lentis (CEL) using regularly collected data. Methods: Diagnostic standards (Ghent nosology) and genetic test were applied in all patients with CEL to determine the presence or absence of MFS. Three potential MFS predictors were tested and chosen to build a prediction model using logistic regression. The predictive performance of the nomogram was validated internally through time-dependent receiver operating characteristic curves, calibration curves, and decision curve analysis. Results: Eyes from 103 patients under 20 years old and with CEL were enrolled in this study. Z score of body mass index (odds ratio [OR] = 0.659; 95% confidence interval [CI], 0.453-0.958), corneal curvature radius (OR = 3.397; 95% CI, 1.829-6.307), and aortic root diameter (OR = 2.342; 95% CI, 1.403-3.911) were identified as predictors of MFS. The combination of the above predictors shows good predictive ability, as indicated by area under the curve of 0.889 (95% CI, 0.826-0.953). The calibration curves showed good agreement between the prediction of the nomogram and the actual observations. In addition, decision curve analysis showed that the nomogram was clinically useful and had better discriminatory power in identifying patients with MFS. For better individual prediction, an online MFS calculator was created. Conclusions: The nomogram provides accurate and individualized prediction of MFS in children with CEL who cannot be identified with the Ghent criteria, enabling clinicians to personalize treatment plans and improve MFS outcomes. Translational Relevance: The prediction model may help clinicians identify MFS in its early stages, which could reduce the likelihood of developing severe symptoms and improve MFS outcomes.