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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.
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Subluxação do Cristalino , Síndrome de Marfan , Humanos , Síndrome de Marfan/complicações , Síndrome de Marfan/diagnóstico , Pressão Intraocular , Fenômenos Biomecânicos , Córnea , Subluxação do Cristalino/diagnóstico , Subluxação do Cristalino/etiologia , Tonometria OcularRESUMO
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.
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Ectopia do Cristalino , Miopia , Erros de Refração , Humanos , Pré-Escolar , Criança , Refração Ocular , Ectopia do Cristalino/diagnóstico , Ectopia do Cristalino/cirurgia , Estudos Retrospectivos , Erros de Refração/diagnóstico , Miopia/diagnósticoRESUMO
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.
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Ectopia do Cristalino , Glaucoma , Síndrome de Weill-Marchesani , Humanos , Síndrome de Weill-Marchesani/genética , Mutação , Ectopia do Cristalino/genética , Estudos de Associação Genética , Proteínas ADAMTS/genética , Proteínas de Ligação a TGF-beta Latente/genéticaRESUMO
Accurate assessment of SARS-CoV-2 immunity is critical in evaluating vaccine efficacy and devising public health policies. Whilst the exact nature of effective immunity remains incompletely defined, SARS-CoV-2-specific T-cell responses are a critical feature that will likely form a key correlate of protection against COVID-19. Here, we developed and optimized a high-throughput whole blood-based assay to determine the T-cell response associated with prior SARS-CoV-2 infection and/or vaccination amongst 231 healthy donors and 68 cancer patients. Following overnight in vitro stimulation with SARS-CoV-2-specific peptides, blood plasma samples were analysed for TH 1-type cytokines. Highly significant differential IFN-γ+ /IL-2+ SARS-CoV-2-specific T-cell responses were seen amongst previously infected COVID-19-positive healthy donors in comparison with unknown / naïve individuals (p < 0·0001). IFN-γ production was more effective at identifying asymptomatic donors, demonstrating higher sensitivity (96·0% vs. 83·3%) but lower specificity (84·4% vs. 92·5%) than measurement of IL-2. A single COVID-19 vaccine dose induced IFN-γ and/or IL-2 SARS-CoV-2-specific T-cell responses in 116 of 128 (90·6%) healthy donors, reducing significantly to 27 of 56 (48·2%) when measured in cancer patients (p < 0·0001). A second dose was sufficient to boost T-cell responses in the majority (90·6%) of cancer patients, albeit IFN-γ+ responses were still significantly lower overall than those induced in healthy donors (p = 0·034). Three-month post-vaccination T-cell responses also declined at a faster rate in cancer patients. Overall, this cost-effective standardizable test ensures accurate and comparable assessments of SARS-CoV-2-specific T-cell responses amenable to widespread population immunity testing, and identifies individuals at greater need of booster vaccinations.
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Vacinas contra COVID-19/imunologia , COVID-19/imunologia , Portador Sadio/imunologia , Imunidade Celular , Imunogenicidade da Vacina , SARS-CoV-2/imunologia , Células Th1/imunologia , Vacinação , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , COVID-19/prevenção & controle , Feminino , Humanos , Interferon gama/imunologia , Masculino , Pessoa de Meia-IdadeRESUMO
OBJECTIVES: Intellectual disability (ID) and epilepsy are independent risk factors for osteoporosis. Diverse predisposing factors influence this, for example in ID, genetics and poor nutrition and in epilepsy, anti-seizure medication (ASM). Around 25% people with ID have epilepsy, majority treatment resistant. ASMs polypharmacy is common. However, little is known about the bone-related characteristics of this vulnerable group. A prospective observational cohort study of bone profile across a community ID Epilepsy service was undertaken to understand this. MATERIALS & METHODS: Participants were on minimum 2 years of ASMs. Baseline demographics, epilepsy data, bone metabolism biomarkers, bone mineral density (BMD) and vitamin D levels were collected. Doses needed to correct vitamin D insufficiency/deficiency were calculated. RESULTS: At baseline, of 104 participants, 92 (90.2%) were vitamin D insufficient/deficient. Seventy-six (73.1%) had a DEXA scan, 50 of whom-in the osteopaenic/osteoporotic range. DEXA scores between ambulant and non-ambulant patients were significantly different (p = .05) but not for ID severity. A high alkaline phosphatase (ALP) predicted lower vitamin D levels. Borderline significance (p = .06) in calcium levels between normal and high ALP was identified. There were no significant associations between parathyroid hormone, inorganic phosphate and magnesium levels, with vitamin D status or DEXA hip T-scores. Normalizing vitamin D levels (mean 101.4 nmol/L) required an average of 1951IU cholecalciferol daily. CONCLUSIONS: Vitamin D deficiency is highly prevalent in people with ID and epilepsy treated with ASMs impacting likely on their bone health. Screening with vitamin D levels, ALP and DEXA in this group should be pro-actively and routinely considered.
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Epilepsia , Deficiência Intelectual , Deficiência de Vitamina D , Densidade Óssea , Epilepsia/tratamento farmacológico , Epilepsia/epidemiologia , Humanos , Deficiência Intelectual/tratamento farmacológico , Deficiência Intelectual/epidemiologia , Estudos Prospectivos , Vitamina D/uso terapêutico , Deficiência de Vitamina D/complicações , Deficiência de Vitamina D/epidemiologia , Vitaminas/uso terapêuticoRESUMO
OBJECTIVE: The aim of this study was to evaluate which mesh type yields lower recurrence and complication rates after ventral hernia repair. SUMMARY BACKGROUND DATA: More than 400,000 ventral hernia repairs are performed annually in the United States. Although the most effective method for repairing ventral hernias involves using mesh, whether to use biologic mesh versus synthetic mesh is controversial. METHODS: Single-blind, randomized, controlled, pragmatic clinical trial conducted from March 2014 through October 2018; 165 patients enrolled with an average follow up of 26 months. Patients were randomized 1:1 to have their ventral hernias repaired using either a biologic (porcine) or synthetic (polypropylene) mesh. The primary study outcome measure was hernia recurrence at 2 years. RESULTS: A total of 165 patients (68 men), mean age 55 years, were included in the study with a mean follow-up of 26 months. An intention-to-treat analysis noted that hernias recurred in 25 patients (39.7%) assigned to biologic mesh and in 14 patients (21.9%) assigned to synthetic mesh (P = 0.035) at 2 years. Subgroup analysis identified an increased rate of hernia recurrence in the biologic versus the synthetic mesh group under contaminated wound conditions (50.0% vs 5.9%; P for interaction = 0.041). Postoperative complication rates were similar for the 2 mesh types. CONCLUSIONS: The risk of hernia recurrence was significantly higher for patients undergoing ventral hernia repair with biologic mesh compared to synthetic mesh, with similar rates of postoperative complications. These data indicate that the use of synthetic mesh over biologic mesh to repair ventral hernias is effective and can be endorsed, including under contaminated wound conditions. TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT02041494.
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Hérnia Ventral/cirurgia , Herniorrafia/métodos , Complicações Pós-Operatórias/prevenção & controle , Prevenção Secundária/métodos , Telas Cirúrgicas , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Desenho de Prótese , Recidiva , Estudos Retrospectivos , Método Simples-Cego , Resultado do TratamentoRESUMO
BACKGROUND: Around 25% of people with Intellectual Disability (PwID) have comorbid epilepsy with seizures in up to two-thirds being drug-resistant. Little is known of the general characteristics and prescribing practices to this population. AIM: Describe and compare characteristics of two cohorts of PwID and epilepsy in two different countries to inform clinical practice better. METHOD: An explorative, retrospective, case-note review in a specialist ID community service in England and in an expert center for PwID and epilepsy in the Netherlands was conducted. Information on ID severity, medical/behavioral/psychiatric/neurodevelopmental/genetic comorbidities, psychotropic, and antiepileptic drugs (AEDs) for each cohort was collected. FINDINGS: The English cohort consisted of 167 people (98 males; age range 18-73â¯years; mild/moderate ID- 35%) and the Dutch cohort of 189 people (111 males; age range 18-85â¯years; mild/moderate ID - 51%). The two cohorts were comparable in their baseline characteristics. The Dutch had higher rates of physical comorbidity, but less mental or behavioral disorders and were more likely to be on anti-psychotic medication. The mean dosages between three most common AEDs prescribed were similar. The most frequently prescribed drug in both centers is valproate. Three-quarters of the Dutch were on three or more AEDs compared to a third in the English cohort. CONCLUSIONS: Structured description of the characteristics, differences, and commonalities of PwID, treatment, and services of both countries is presented. This is the first real-world study to reveal unique characteristics of managing epilepsy for a complex ID population. In particular, it highlights the considerable comorbid psychiatric burden and psychotropic prescribing.
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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.
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Povo Asiático/etnologia , Degeneração Macular/epidemiologia , Idoso , Idoso de 80 Anos ou mais , China/epidemiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , PrevalênciaRESUMO
BACKGROUND: Sudden unexpected death in epilepsy (SUDEP) is a leading cause of death among people with chronic epilepsy. People with intellectual disability (ID) are overrepresented in this population. The SUDEP and Seizure Safety Checklist ("Checklist") is a tool to discuss risk factors influencing seizures and the risk of SUDEP. It includes questions about the availability of nocturnal monitoring. In Cornwall UK, people with epilepsy and ID and their relatives and carers are routinely advised to consider nocturnal surveillance to reduce harm from potential nocturnal seizures. We assessed the retention of advice provided on nocturnal monitoring and if there were differences between those in residential care and those living with their families. METHODS: A postal questionnaire was sent to carers of all people with epilepsy and ID in Cornwall followed by the adult specialist ID epilepsy service. All those who were contacted had received the same advice on SUDEP and nocturnal monitoring at least once in the past year. Each person was categorized into living in a residential setting or with their family group. Intergroup differences were compared using Fisher's exact test. RESULTS: Carers for 170 people were contacted and 121 responded (71%). The family group had statistically more nocturnal seizures than the residential group. While there was no difference in the awareness of SUDEP, the groups differed in their recollection of the person-centered discussion of risk with carers in residential setting being less aware. Where nocturnal monitoring advice was given, it was followed, and previously unknown seizures were identified in 75%. CONCLUSIONS: Carers in residential settings are less likely to recall specific person-centered discussion of risks to the individual they support as compared with those living with families although general awareness of SUDEP and implementing advice such as nocturnal monitoring is present equally in both groups. In improving detection of nocturnal seizures, audio monitoring may be a useful strategy to reduce risk of harm for people with ID.
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Cuidadores/normas , Lista de Checagem/normas , Comunicação , Morte Súbita/epidemiologia , Convulsões/mortalidade , Adulto , Cuidadores/psicologia , Lista de Checagem/métodos , Morte Súbita/etiologia , Morte Súbita/prevenção & controle , Feminino , Seguimentos , Humanos , Deficiência Intelectual/complicações , Deficiência Intelectual/mortalidade , Deficiência Intelectual/terapia , Masculino , Fatores de Risco , Convulsões/complicações , Convulsões/terapia , Inquéritos e Questionários , Reino Unido/epidemiologiaRESUMO
BACKGROUND/AIMS: Chronic renal failure (CRF) is often accompanied by increased oxidative stress and euthyroid sick syndrome (ESS). The cause of ESS is unknown, and it is unknown whether there exists a link between oxidant stress and ESS in CRF patients. Therefore, we aim to investigate oxidative stress and type 1 deiodinase (DIO1) expression, which plays the key role in the ESS in CRF patients. METHODS: In-patients with CRF were divided into the two group: Group 1 is ESS patients consisting of 60 patients with low free triiodothyronine (FT3) and Group 2 consisting of 60 patients with normal FT3. Group 3 consisted of 60 healthy volunteers recruited as controls. The baseline clinical parameters of patients were evaluated with standard routine methods in a clinical laboratory. Serum levels of 8-isoprostane and DIO1 were measured by enzyme-linked immunosorbent assay (ELISA). Multiple regression analysis was used to analyze the relationship between oxidative stress, DIO1 and FT3. RESULTS: The concentrations of serum 8-Isoprostane in Group 1 and Group 2 were substantially higher than that of Group 3 (p< 0.05), however there was no significant difference between Group 1 and Group 2 (p=0.516). The serum DIO1 level was higher in Group 2 than in Group 1 and Group 3 (p< 0.001). Multivariate linear regression analysis revealed that the DIO1 concentration and FT3 level were not associated with the concentration of serum 8-Isoprostane. CONCLUSIONS: CRF patients showed elevated oxidative stress. The CRF patients without ESS showed higher expression of DIO1 than patients with ESS and the control group. The concentration of serum 8-Isoprostane was not correlated with FT3 and DIO1 levels.
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Proteínas de Ligação a DNA/metabolismo , Síndromes do Eutireóideo Doente/etiologia , Falência Renal Crônica/complicações , Estresse Oxidativo , Idoso , Estudos de Casos e Controles , Dinoprosta/análogos & derivados , Dinoprosta/sangue , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Tri-IodotironinaRESUMO
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.
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Comprimento Axial do Olho/patologia , Ectopia do Cristalino/diagnóstico , Refração Ocular , Adolescente , Criança , Pré-Escolar , China/epidemiologia , Ectopia do Cristalino/epidemiologia , Ectopia do Cristalino/fisiopatologia , Feminino , Humanos , Incidência , Masculino , Estudos Retrospectivos , Acuidade VisualRESUMO
PURPOSE: Chronic obstructive pulmonary disease (COPD) is associated with substantial morbidity, including impaired health-related quality of life (HRQoL). Despite the prominent role of occupational factors in the aetiology of COPD, the relationship between these exposures and HRQoL has not been well elucidated. METHODS: A subpopulation from an epidemiological study, designed to assess the workplace contribution to COPD, was administered the EQ5D HRQoL tool. Demographics, an index of economic deprivation, health endpoints including the presence of COPD and lung function were also recorded. Workplace exposures were categorised using both self-reported exposures and also by the use of an established job exposure matrix (JEM). RESULTS: A total of 623 individuals participated (mean age 67.1 years). One hundred and forty-eight (24%) reported having received a physician diagnosis of COPD, 355 (57%) were male, and 386 (62%) were ever smokers. As anticipated, the presence of COPD was associated with a poorer HRQoL. Additionally, however, HRQoL was significantly lower in the presence of both self-reported vapours, gases, dusts and fumes exposure and JEM-based exposure irrespective of the presence of COPD. Regression analysis, adjusting for a variety of covariates including the presence of COPD, confirmed a persisting higher likelihood of occupational exposure categorised by JEM being associated with poorer HRQoL scores (ß estimate: -0.069; p < 0.05). CONCLUSIONS: Our findings suggest that work may have an important link to HRQoL and that this effect can persist even among those who have retired. In those with COPD, HRQoL is worse than among those without this condition, but the work-associated decrement appears to be similar across both groups.
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Doenças Profissionais/psicologia , Exposição Ocupacional/efeitos adversos , Doença Pulmonar Obstrutiva Crônica/psicologia , Qualidade de Vida , Idoso , Idoso de 80 Anos ou mais , Inglaterra , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Doenças Profissionais/etiologia , Doença Pulmonar Obstrutiva Crônica/etiologia , Aposentadoria/psicologia , Inquéritos e QuestionáriosRESUMO
Legionella bacteria can proliferate in poorly maintained water systems, posing risks to users. All Legionella species are potentially pathogenic, but Legionella pneumophila (L. pneumophila) is usually the primary focus of testing. However, Legionella anisa (L. anisa) also colonizes water distribution systems, is frequently found with L. pneumophila, and could be a good indicator for increased risk of nosocomial infection. Anonymized data from three commercial Legionella testing laboratories afforded an analysis of 565,750 water samples. The data covered July 2019 to August 2021, including the COVID-19 pandemic. The results confirmed that L. anisa commonly colonizes water distribution systems, being the most frequently identified non-L. pneumophila species. The proportions of L. anisa and L. pneumophila generally remained similar, but increases in L. pneumophila during COVID-19 lockdown suggest static water supplies might favor its growth. Disinfection of hospital water systems was effective, but re-colonization did occur, appearing to favor L. pneumophila; however, L. anisa colony numbers also increased as a proportion of the total. While L. pneumophila remains the main species of concern as a risk to human health, L. anisa's role should not be underestimated, either as a potential infection risk or as an indicator of the need to intervene to control Legionella's colonization of water supplies.
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Legionella , Microbiologia da Água , Legionella/isolamento & purificação , Humanos , COVID-19/transmissão , COVID-19/epidemiologia , Abastecimento de Água , Legionella pneumophila/isolamento & purificação , SARS-CoV-2RESUMO
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.
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Ectopia do Cristalino , Síndrome de Marfan , Miopia , Criança , Humanos , Masculino , Pré-Escolar , Ectopia do Cristalino/diagnóstico , Ectopia do Cristalino/cirurgia , Ectopia do Cristalino/complicações , Síndrome de Marfan/complicações , Estudos Retrospectivos , Acuidade Visual , Refração Ocular , Miopia/cirurgia , Miopia/complicaçõesRESUMO
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.
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Lentes Intraoculares , Facoemulsificação , Humanos , Metanálise em Rede , Estudos Prospectivos , Estudos Retrospectivos , Olho , Biometria , Óptica e Fotônica , Refração Ocular , Comprimento Axial do Olho , Estudos Multicêntricos como AssuntoRESUMO
BACKGROUND: Although the most durable method for ventral hernia repairs involves using mesh, whether to use biologic mesh versus synthetic mesh remains controversial. This study aimed to compare synthetic and biologic meshes with respect to patient-reported quality of life scores and costs after ventral hernia repair surgeries. METHODS: This study is part of the Preventing Recurrence in Clean and Contaminated Hernias (PRICE) pragmatic randomized control trial conducted from March 2014 through October 2018. Patients were randomized 1:1 to undergo ventral hernia repair using either a biologic or synthetic mesh. The coprimary outcomes were 2-year changes in Visual Analog Scale, Activities Assessment Scale, Hernia-Related Quality-of-Life Survey, and Short-Form 36 Health Survey (SF-36) quality-of-life scores from repair. The secondary outcome was the overall cost per patient. RESULTS: Among the 165 patients included in the study, 82 were randomized to biologic meshes and 83 to synthetic meshes. There were no significant differences in the performance between the 2 mesh types with regard to quality-of-life measures using a mixed model approach. This result was consistent even when performing subgroup analysis based on wound contamination. However, nonparametric tests comparing the differences in quality-of-life measures from preoperative to 24-month postoperative timepoints revealed that the synthetic mesh group showed a greater reduction in disability than biologic mesh for the SF-36 (median [interquartile range] of 20 [5-30] vs 6 [1-20], P = .025). This difference was due to reductions in the physical role limitations (62 [0-100] vs 0 [0-50], P = .018) and the pain (38 [12-50] vs 12 [0-25], P = .012) domains of the SF-36. Overall cost per patient was greater for biologic meshes (mean [95% confidence interval] of $80,420 [$66,485-$94,355] vs $61,036 [$48,946-$73,125], P = .038), regardless of insurance type. CONCLUSION: In this randomized clinical trial, there were no differences in changes in quality-of-life scores at the 2-year timepoint except for the SF-36, where the synthetic mesh may be associated with less pain and physical role limitations than the biologic mesh. Overall costs per patient were less for synthetic than biologic mesh.
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Produtos Biológicos , Hérnia Ventral , Humanos , Qualidade de Vida , Telas Cirúrgicas , Resultado do Tratamento , Recidiva Local de Neoplasia/cirurgia , Hérnia Ventral/prevenção & controle , Hérnia Ventral/cirurgia , Herniorrafia/efeitos adversos , Herniorrafia/métodos , Custos e Análise de Custo , Dor/cirurgia , Recidiva , Estudos RetrospectivosRESUMO
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.
RESUMO
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.
Assuntos
Ansiedade , Cuidadores , Depressão , Ectopia do Cristalino , Humanos , Estudos Transversais , Feminino , Masculino , Estudos Prospectivos , Cuidadores/psicologia , Ansiedade/etiologia , Ansiedade/epidemiologia , Depressão/etiologia , Depressão/epidemiologia , Criança , Adulto , Ectopia do Cristalino/psicologia , Pré-Escolar , Pessoa de Meia-Idade , Fatores de Risco , Adolescente , Inquéritos e Questionários , Índice de Gravidade de Doença , Escalas de Graduação PsiquiátricaRESUMO
Purpose: The purpose of this study was to investigate the association between retinal nerve fiber layer (RNFL) thickness and high-density lipoprotein cholesterol (HDL-C) in a healthy population. Methods: This cross-sectional study included 31,738 UK Biobank participants with high quality optical coherence tomography (OCT) images, excluding those with neurological or ocular diseases. The locally estimated scatterplot smoothing (LOESS) curve and multivariable piecewise linear regression models were applied to assess the association between HDL-C and RNFL thickness, and HDL-C subclasses were further analyzed using nuclear magnetic resonance (NMR) spectroscopy. Results: Multivariate piecewise linear regression revealed that high HDL-C levels (>1.7 mmol/L in women or > 1.5 mmol/L in men) were associated with thinner RNFL thickness (women: ß = -0.13, 95% confidence interval [CI] = -0.23 to -0.02, P = 0.017; male: ß = -0.23, 95% CI = -0.37 to -0.10, P = 0.001). Conversely, a significant positive association between HDL-C and RNFL thickness was observed when HDL-C was between 1.4 and 1.7 mmol/L for female participants (ß = 0.13, 95% CI = 0.02 to 0.24, P = 0.025). NMR analysis showed that these associations are potentially driven by distinct HDL-C subclasses. Conclusions: This study revealed an association between HDL-C levels and retinal markers of neurodegenerative diseases, suggesting that elevated HDL-C may serve as a new risk factor for neurodegenerative conditions. These findings may contribute to the implementation of preventive interventions and improved patient outcomes.
Assuntos
HDL-Colesterol , Fibras Nervosas , Células Ganglionares da Retina , Tomografia de Coerência Óptica , Humanos , Feminino , Masculino , Estudos Transversais , Tomografia de Coerência Óptica/métodos , Fibras Nervosas/patologia , Pessoa de Meia-Idade , Células Ganglionares da Retina/patologia , Reino Unido/epidemiologia , HDL-Colesterol/sangue , Idoso , Bancos de Espécimes Biológicos , Adulto , Biobanco do Reino UnidoRESUMO
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.