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2.
Am J Case Rep ; 25: e944202, 2024 Sep 12.
Artigo em Inglês | MEDLINE | ID: mdl-39262095

RESUMO

BACKGROUND Polycythemia vera (PV) is a myeloproliferative neoplasm (MPNs) marked by elevated hemoglobin and hematocrit, which can lead to thromboembolic events and progress to myelofibrosis or acute myeloid leukemia (AML). MPNs, including PV, are relatively rare in Malaysia, and there is currently no recent published data reporting the demographics and outcomes of PV patients in the country. In Western countries, routine annual blood tests are standard, whereas this practice is less common in Malaysia, underscoring the need for improved awareness and accessibility to ensure timely diagnosis of PV. CASE REPORT This report presents a case of a 55-year-old Malaysian woman in a primary care setting, initially misdiagnosed with benign conditions due to atypical presentations of recurrent bilateral eye redness and dizziness. Persistent symptoms led to further evaluation by primary care and hematologist, which revealed elevated hemoglobin, hematocrit, leukocytosis, JAK2 V617F mutation, and low serum erythropoietin levels, confirming PV, even without proceeding with a bone marrow biopsy. Treatment with phlebotomy, hydroxyurea, and aspirin resulted in significant improvements in ocular symptoms and hematological parameters within 60 days. CONCLUSIONS This case underscores the critical role of primary care in the early detection of polycythemia vera. Timely identification and appropriate referral from primary care settings are essential to avoid diagnostic delays and ensure effective management, improving patient outcomes and preventing complications.


Assuntos
Policitemia Vera , Atenção Primária à Saúde , Humanos , Feminino , Pessoa de Meia-Idade , Policitemia Vera/diagnóstico , Malásia , Hidroxiureia/uso terapêutico , Erros de Diagnóstico , Diagnóstico Diferencial
3.
Rev Med Suisse ; 20(886): 1602-1606, 2024 Sep 11.
Artigo em Francês | MEDLINE | ID: mdl-39262186

RESUMO

The relationship between glaucoma and hypertension is complex. Intraocular pressure is the main modifiable risk factor for glaucoma. Literature describes hypertension as being linked to glaucomatous damage, although the mechanisms are not fully understood. Therefore, glaucoma screening is recommended for hypertensive individuals over 60 years old. The relationships between ocular, arterial pressure, and ocular perfusion pressure, and their impact on optic nerve perfusion, make coordinated management of these elements crucial. Hypertension, hypotension, extreme variability, nocturnal dipping, and overtreatment with antihypertensive drugs can increase the incidence and/or progression of glaucoma. This critical review discusses the causative, clinical, and therapeutic elements to consider in the management of these two pathologies.


La relation entre le glaucome et l'hypertension artérielle (HTA) est complexe. La pression intraoculaire est le principal facteur de risque modifiable du glaucome. Selon la littérature, l'HTA entraînerait un dommage glaucomateux, sans que les mécanismes soient entièrement compris. Le dépistage du glaucome est ainsi recommandé chez les hypertendus de plus 60 ans. La relation entre pression oculaire, artérielle et pression de perfusion oculaire, ainsi que leur impact sur la perfusion du nerf optique, rendent fondamentale la gestion coordonnée de ces deux pathologies. L'HTA, l'hypotension artérielle, la variabilité extrême ou le dipping nocturne, tout comme le surtraitement par antihypertenseurs, peuvent augmenter l'incidence et/ou la progression du glaucome.. Cette revue critique discute des éléments causatifs, cliniques et thérapeutiques à considérer dans la prise en charge de ces deux pathologies.


Assuntos
Glaucoma , Hipertensão , Humanos , Glaucoma/fisiopatologia , Glaucoma/diagnóstico , Hipertensão/diagnóstico , Hipertensão/fisiopatologia , Hipertensão/epidemiologia , Fatores de Risco , Pressão Intraocular/fisiologia , Anti-Hipertensivos/uso terapêutico , Pressão Sanguínea/fisiologia , Progressão da Doença , Pessoa de Meia-Idade
4.
Zhonghua Yan Ke Za Zhi ; 60(9): 746-756, 2024 Sep 11.
Artigo em Chinês | MEDLINE | ID: mdl-39267553

RESUMO

Objective: To evaluate the efficacy and safety of internal XEN gel stent implantation or combined with phacoemusification and intraocular lens (IOL) implantation in the treatment of glaucoma. Methods: It was a retrospective case series study. Forty-five patients (28 males and 17 females; 52 eyes) who had different types of glaucoma treated at the Shenzhen Eye Hospital from December 2021 to June 2023 were included. XEN gel stent implantation (the XEN group) or XEN implantation combined with phacoemusification and IOL implantation (the combined group) were performed in these patients. The postoperative follow-up was 18 months. The observational indexes included the best corrected visual acuity (recorded as the logarithm of the minimum angle of resolution), intraocular pressure (IOP), XEN implantation quadrant, number of IOP-lowering medications, corneal endothelial cell count, operation success rate and surgical complications. The one-way repeated measures of variance, LSD-t test, and rank sum test were used for statistical analysis. Results: There were 28 patients (33 eyes) in the XEN group and 17 patients (19 eyes) in the combined group. The postoperative best corrected visual acuity had no significant change in the XEN group, but improved significantly (0.21±0.30 at 6 months; P<0.05) in the combined group, compared with the preoperative value. The IOP of the two groups at 18 months after surgery [(12.2±3.1) and (11.9±3.9) mmHg (1 mmHg=0.133 kPa)] was significantly lower than that before surgery [(22.1±8.5) and (19.4±10.2) mmHg; P<0.05]. The average number of IOP-lowering medications in all patients was 3 (2, 3) before surgery and 0 (0, 0) at 18 months after surgery. The difference was statistically significant (P<0.05). In the XEN group, the corneal endothelial cell count was (2 387.37±478.22) cells/mm² preoperatively and (2 193.89±311.96) cells/mm² at 12 months. The decrease showed no statistical significance. The XEN gel stent was implanted in the supranasal quadrant in 8 eyes (15.4%), in the inferonasal quadrant in 36 eyes (69.2%), and in the infratemporal quadrant in 8 eyes (15.4%). The operation success rate [complete success rate, 86.5% (45/52); conditional success rate, 9.6% (5/52)] was 96.2% (50/52). The filtering bleb scarring occurred in 9.6% (5/52) of eyes. A small amount of hyphema, postoperative shallow anterior chamber, transient hypertension, and intraoperative drain breakage were found in 7.7% (4/52) of eyes, respectively. A small amount of subconjunctival bleeding was observed in 5.8%(3/52) of eyes. Re-implantation of an XEN gel stent was performed in 5.8% (3/52) of eyes. The intraoperative conjunctival tissue rupture, hypotony macular edema, and drain exposure were observed in 1.9% (1/52) of eyes, respectively. Conclusion: The XEN gel stent implantation or combined with phacoemusification and IOL implantation was effective and safe in treating different types of glaucoma, but its long-term clinical efficacy needs further follow-up observations in multi-center, larger-scale research.


Assuntos
Implantes para Drenagem de Glaucoma , Glaucoma , Implante de Lente Intraocular , Facoemulsificação , Humanos , Estudos Retrospectivos , Masculino , Feminino , Implante de Lente Intraocular/métodos , Glaucoma/cirurgia , Facoemulsificação/métodos , Stents , Pressão Intraocular , Resultado do Tratamento , Acuidade Visual , Pessoa de Meia-Idade
5.
Zhonghua Yan Ke Za Zhi ; 60(9): 736-745, 2024 Sep 11.
Artigo em Chinês | MEDLINE | ID: mdl-39267552

RESUMO

Objective: To investigate the factors affecting the progression of visual field defects in patients with myopia and primary open-angle glaucoma (POAG), and to clarify whether the factors vary in patients with different degrees of myopia. Method: An ambispective cohort study was conducted among patients diagnosed with myopia and POAG from the glaucoma outpatient department at the Zhongshan Ophthalmic Center of Sun Yat-sen University between January 2006 and January 2024. Based on the criteria of functional visual field progression, patients were divided into the progression group and non-progression group, and further divided into the low to moderate myopia subgroup and high myopia subgroup according to the degree of myopia. The patient age, gender, type of glaucoma (high tension glaucoma and normal tension glaucoma), spherical equivalent refraction, best corrected visual acuity (BCVA, recorded as the logarithm of the minimum angle of resolution), intraocular pressure (IOP), central corneal thickness, baseline visual field, history of ophthalmic surgery (corneal refractive surgery and glaucoma surgery), and number of anti-glaucoma medications were summarized. The generalized estimation equation was used for comparison between groups, and the Cox proportional hazards model was used to analyze the factors affecting the progression of visual field defects. Results: A total of 182 eyes from 106 patients were included in this study. There were 57 eyes in the progression group and 125 eyes in the non-progression group. Compared with the non-progression group, the progression group had the older age [43 (29, 53) years old], worse BCVA [0.05 (0.00, 0.17)], greater IOP fluctuation [1.8 (1.3, 2.9)mmHg(1 mmHg=0.133 kPa)], more common baseline central defects [52.6%(30/57)], higher visual field pattern standard deviations [8.92 (5.32, 12.00)dB], lower visual field index [77% (67%, 88%)], and more anti-glaucoma medications [35.1% (20/57) patients used three medications] (all P<0.05). The Cox proportional hazards models showed that the baseline moderate visual field defects [hazard ratio (HR)=2.33, 95% confidence interval (CI): 1.25 to 4.36, P=0.008], baseline central defects (HR=2.09, 95%CI: 1.11 to 3.93, P=0.022), older age (Model A, HR=1.03, 95%CI: 1.00 to 1.05, P=0.017; Model B, HR=1.02, 95%CI: 1.00 to 1.05, P=0.019), and greater IOP fluctuation (Model A, HR=1.54, 95%CI: 1.32 to 1.81, P<0.001; Model B, HR=1.49, 95%CI: 1.26 to 1.75, P<0.001) were risk factors for visual field progression. In the low to moderate myopia subgroup, the increased risk of progression was associated with baseline central defects (HR=5.74, 95%CI: 1.72 to 19.20, P=0.005), worse BCVA (Model A, HR=15.80, 95%CI: 2.07 to 121.00, P=0.008; Model B, HR=12.50, 95%CI: 2.65 to 58.70, P=0.001), and older age (Model A, HR=1.05, 95%CI: 1.02 to 1.08, P=0.002; Model B, HR=1.07, 95%CI: 1.03 to 1.11, P<0.001). In the high myopia subgroup, the increased risk of progression was associated with baseline moderate visual field defects (HR=2.35, 95%CI: 1.12 to 4.92, P=0.024) and greater IOP fluctuation (Model A, HR=1.50, 95%CI: 1.24 to 1.82, P<0.001; Model B, HR=1.52, 95%CI: 1.26 to 1.83, P<0.001). Conclusions: Age, IOP fluctuation, baseline moderate visual field defects, and baseline central defects were the factors affecting the progression of visual field defects in patients with myopia and POAG. There were differences in the influencing factors of visual field progression in patients with different degrees of myopia.


Assuntos
Progressão da Doença , Glaucoma de Ângulo Aberto , Pressão Intraocular , Miopia , Campos Visuais , Humanos , Glaucoma de Ângulo Aberto/fisiopatologia , Fatores de Risco , Estudos de Coortes , Transtornos da Visão , Feminino , Masculino , Pessoa de Meia-Idade
6.
BMC Ophthalmol ; 24(1): 296, 2024 Sep 15.
Artigo em Inglês | MEDLINE | ID: mdl-39277752

RESUMO

OBJECTIVE: To investigate the risk factors and prognostic factors that affect the long-term clinical outcomes of acute retinal necrosis (ARN). METHODS: A retrospective study of patients with ARN who underwent treatment and completed follow-up in our ophthalmology department from 2011 to 2021 was conducted. The incidence and risk factors of retinal detachment (RD) and prognostic factors affecting long-term clinical outcomes, such as late-onset RD and final vision loss (< 20/200), were analyzed. RESULTS: Totally 59 ARN patients (65 eyes) with an average follow-up of 48.9 months were enrolled. During the follow-up period, RD occurred in 34 eyes (52.3%). The risk factors for RD included quadrants of involved retinal necrosis (odds ratio [OR], 4.181; 95% confidence interval [CI], 1.950-10.834) and initial intraocular viral load (OR, 1.721; 95% CI, 1.071-3.083). Early intravitreal antiviral treatment (OR, 1.204; 95% CI, 1.040-1.480) was independently associated with a decreased risk of late-onset RD. The factors independently associated with an increased risk of final vision loss were worse initial visual acuity (OR, 3.895; 95% CI, 1.551-13.662) and late-onset RD (OR, 8.043; 95% CI, 1.380-67.216). In addition, we utilized the fluctuating magnitude of viral load to quantify the extent of its reduction in comparison to its original value following the initial intravitreal antiviral injection (IAI). This ratio was strongly related to initial intraocular IL-8 concentration (Spearman correlation coefficient=-0.741, P = 0.000) and moderately related to the initial degree of aqueous flare (Spearman correlation coefficient=-0.508, P = 0.010). CONCLUSION: RD is a common and severe complication of ARN with multiple risk factors, such as initial retinitis involvement area and initial intraocular viral load. Active local antiviral therapy may reduce the risk of late-onset RD. The antiviral medication should be adjusted according to the inflammatory state. Therefore, timely detection of causative viruses and intensive systemic and local antiviral therapy is crucial for preserving visual function in ARN patients.


Assuntos
Descolamento Retiniano , Síndrome de Necrose Retiniana Aguda , Acuidade Visual , Humanos , Síndrome de Necrose Retiniana Aguda/diagnóstico , Síndrome de Necrose Retiniana Aguda/virologia , Síndrome de Necrose Retiniana Aguda/tratamento farmacológico , Masculino , Fatores de Risco , Estudos Retrospectivos , Feminino , Descolamento Retiniano/epidemiologia , Descolamento Retiniano/diagnóstico , Acuidade Visual/fisiologia , Pessoa de Meia-Idade , Prognóstico , Adulto , Idoso , Seguimentos , Incidência , Antivirais/uso terapêutico , Infecções Oculares Virais/virologia , Infecções Oculares Virais/diagnóstico , Injeções Intravítreas , Adulto Jovem
7.
J Trop Pediatr ; 70(5)2024 Aug 10.
Artigo em Inglês | MEDLINE | ID: mdl-39277773

RESUMO

Ghosal hematodiaphyseal dysplasia (GHDD) is an autosomal recessive disorder characterized by diaphyseal dysplasia of long bones, bone marrow fibrosis, and steroid-responsive anemia. Patients with this disease have a mutation in the thromboxane-AS1 (TBXAS1) gene located on chromosome 7q33.34. They present with short stature, varying grades of myelofibrosis, and, hence cytopenias. Patients with the above presentation were evaluated through clinical presentation, X-ray of long bones, bone marrow examinations, and confirmed by genetic testing. In this article, we present two cases: The first case is a 3-year-old boy who presented with progressive pallor and ecchymotic patches for a year. On investigation, he had bicytopenia and bone marrow fibrosis. His anemia was steroid responsive and was finally diagnosed as GHDD. The second case is a 20-month-old girl who presented with blood in stools, developmental delay, anemia, and increased intensity of long bones on X-ray. Since other investigations were normal, suspicion of GHDD was raised, and a genetic workup was conducted which suggested mutation in TBXAS1 gene, confirming the diagnosis of GHDD. Children with refractory anemia and cortical thickening on skeletogram should always be evaluated for dysplasias. Timely treatment with steroids reduces transfusion requirements and halts bone damage, thus leading to better growth and improved quality of life.


Assuntos
Anemia , Humanos , Masculino , Pré-Escolar , Feminino , Anemia/etiologia , Anemia/tratamento farmacológico , Mutação , Lactente , Osteocondrodisplasias/genética , Osteocondrodisplasias/complicações , Osteocondrodisplasias/diagnóstico , Resultado do Tratamento , Radiografia , Esteroides/uso terapêutico , Anemia Refratária
8.
BMC Ophthalmol ; 24(1): 358, 2024 Sep 16.
Artigo em Inglês | MEDLINE | ID: mdl-39278928

RESUMO

BACKGROUND: To assess the efficacy and safety of virtual reality-based visual training (VRVT) in myopia control among children. METHODS: The randomized, parallel-group, single-blind clinical trial conducted at the Department of Ophthalmology of Shanghai Tenth People's Hospital enrolled 65 low-myopic children (aged 8 to 13 years) with cycloplegic spherical equivalent (SE) between - 0.50 and - 3.00 diopters (D), astigmatism less than - 1.00 D, anisometropia less than 1.50D, and best corrected visual acuity (BCVA) more than 0.0 logarithm (LogMAR) of the minimum angle of resolution. The participants were enrolled in December 2020, and the follow-up of this study concluded on August 2021. Children were assigned randomly to the intervention group (VRVT plus single-vision spectacle [SVS]) and the control group (only SVS without receiving VRVT). The intervention group was administered for 20 min per day with VRVT under parental supervision at home. The primary outcome was changes in axial length (AL) at 3 months. Macular choroidal thickness (mCT) was regarded as a key secondary outcome. RESULTS: Among 65 participants (mean age: 10.8 years, 52.3% male), 60 children (92.3%) who completed the 3-month intervention and 6-month follow-up were included in the analysis (30 in the intervention group and 30 in the control group). The changes of AL were 0.063 ± 0.060 mm (95% confidence interval [CI], 0.074 to 0.119 mm) in the intervention group and 0.129 ± 0.060 mm (95% CI, 0.107 to 0.152 mm) and in the control group at 3 months (t = - 2.135, P = 0.037), and the mean difference between the two groups was 0.066 mm. The change of mCT were 22.633 ± 36.171 µm (95% CI, 9.127 to 36.140 µm) in the intervention group and - 3.000 ± 31.056 µm (95% CI, - 14.597 to 8.597 µm) in the control group at 3 months (t = 2.945, P = 0.005). VR vertigo was the most common adverse event which was occurred in two children (2/30, 6.67%) in the intervention group. CONCLUSIONS: VRVT is a promising method for myopia control in children with good user acceptability. Among children aged 8 to 13 years with low-myopia, nightly use of VRVT resulted in slowing myopia progression. TRIAL REGISTRATION: This protocol was registered with ClinicalTrials.gov (NCT06250920), retrospectively registered on 01 February 2024.


Assuntos
Miopia , Refração Ocular , Realidade Virtual , Acuidade Visual , Humanos , Masculino , Criança , Feminino , Miopia/fisiopatologia , Miopia/terapia , Método Simples-Cego , Acuidade Visual/fisiologia , Adolescente , Refração Ocular/fisiologia , Seguimentos , Resultado do Tratamento , Óculos , Comprimento Axial do Olho
9.
Acta Pharm ; 74(3): 383-404, 2024 Sep 01.
Artigo em Inglês | MEDLINE | ID: mdl-39279530

RESUMO

Dry eye disease (DED) is an ocular condition characterized by altered tear film homeostasis, resulting in symptoms like tear film instability, hyperosmolarity, inflammation, and neurosensory abnormalities. It affects visual acuity and quality of life and is influenced by age, gender, and environmental factors. The first line of treatment consists of dynamically developing artificial tears, gels, and eyelid sprays, which can be supplemented with natural ingredients for enhanced efficacy. Other therapeutic steps include auto-logous serum tears, anti-inflammatory and immunosuppressive eyedrops, or oral tablets. Management also targets Meibomian gland dysfunction and the ocular surface micro-biome. This article explores various therapeutic approaches, including natural compounds and complementary strategies. Natural compounds, such as vitamins, and herbal substances (e.g., trehalose), offer promising benefits in enhancing tear film stability and ocular surface protection. Apitherapeutic products like manuka honey and propolis exhibit antibacterial and anti-inflammatory properties. Additionally, human tissue-derived solutions, such as auto-logous serum tears and amniotic membrane extracts, hold the potential for ocular surface regeneration. Other strategies, including polyherbal eye drops, liposomal eyelid sprays, and microbiome-supporting solutions offer alternative therapeutic avenues. Moreover, patient education, lifestyle modifications, and interdisciplinary collaboration play crucial roles in DED management, emphasizing the importance of holistic care approaches.


Assuntos
Produtos Biológicos , Síndromes do Olho Seco , Humanos , Síndromes do Olho Seco/tratamento farmacológico , Produtos Biológicos/farmacologia , Produtos Biológicos/uso terapêutico , Soluções Oftálmicas , Lágrimas/metabolismo , Anti-Inflamatórios/farmacologia , Animais , Lubrificantes Oftálmicos , Qualidade de Vida
10.
Lancet ; 404(10456): 962-970, 2024 Sep 07.
Artigo em Inglês | MEDLINE | ID: mdl-39244273

RESUMO

BACKGROUND: Leber congenital amaurosis 1 (LCA1), caused by mutations in GUCY2D, is a rare inherited retinal disease that typically causes blindness in early childhood. The aim of this study was to evaluate the safety and preliminary efficacy of ascending doses of ATSN-101, a subretinal AAV5 gene therapy for LCA1. METHODS: 15 patients with genetically confirmed biallelic mutations in GUCY2D were included in this phase 1/2 study. All patients received unilateral subretinal injections of ATSN-101. In the dose-escalation phase, three adult cohorts (n=3 each) were treated with three ascending doses: 1·0 × 1010 vg/eye (low dose), 3·0 × 1010 vg/eye (middle dose), and 1·0 × 1011 vg/eye (high dose). In the dose-expansion phase, one adult cohort (n=3) and one paediatric cohort (n=3) were treated at the high dose. The primary endpoint was the incidence of treatment-emergent adverse events (TEAEs), and secondary endpoints included full-field stimulus test (FST) and best-corrected visual acuity (BCVA). A multi-luminance mobility test (MLMT) was also done. Data through the 12-month main study period are reported. FINDINGS: Patients were enrolled between Sept 12, 2019, and May 5, 2022. A total of 68 TEAEs were observed, 56 of which were related to the surgical procedure. No serious TEAE was related to the study drug. Ocular inflammation was mild and reversible with steroid treatment. For patients who received the high dose, mean change in dark-adapted FST was 20·3 decibels (dB; 95% CI 6·6 to 34·0) for treated eyes and 1·1 dB (-3·7 to 5·9) for untreated eyes at month 12 (white stimulus); improvements were first observed at day 28 and persisted over 12 months (p=0·012). Modest improvements in BCVA were also observed (p=0·10). Three of six patients who received the high dose and did the MLMT achieved the maximum score in the treated eye. INTERPRETATION: ATSN-101 is well tolerated 12 months after treatment, with no drug-related serious adverse events. Clinically significant improvements in retinal sensitivity were sustained in patients receiving the high dose. FUNDING: Atsena Therapeutics.


Assuntos
Terapia Genética , Guanilato Ciclase , Amaurose Congênita de Leber , Receptores de Superfície Celular , Adolescente , Adulto , Criança , Humanos , Terapia Genética/métodos , Guanilato Ciclase/genética , Injeções Intraoculares , Amaurose Congênita de Leber/genética , Mutação , Receptores de Superfície Celular/genética , Resultado do Tratamento , Acuidade Visual
11.
Cont Lens Anterior Eye ; 47(5): 102302, 2024 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-39244400
12.
Int Ophthalmol ; 44(1): 376, 2024 Sep 10.
Artigo em Inglês | MEDLINE | ID: mdl-39254907

RESUMO

PURPOSE: Evaluate the response to adalimumab (ADA) in pediatric chronic anterior uveitis (pCAU). METHODS: Retrospective chart review of pCAU patients treated with ADA. Outcomes evaluated included the proportion of patients achieving zero ocular inflammation and discontinuation of topical corticosteroids, visual outcomes, and incidence of uveitis recurrences after ≥ 12 months of prescribing ADA. Incidence and risk factors for developing anti-adalimumab antibodies (AAAs) were also evaluated. RESULTS: Of 27 children aged 11 years, 16 (59%) were Caucasian and 6 (22%) African Americans. Thirteen (48%) patients had idiopathic pCAU, 12 (44%) had juvenile idiopathic arthritis (JIA) related pCAU, and 2 (7%) had tubulointerstitial nephritis and uveitis syndrome. At baseline, African American children had worse visual acuity (p = 0.026). At 1 year, 21 (78%) children achieved zero ocular inflammation (remission). Risk factors associated with non-remission were being African American (20% vs. 94%, p = 0.003) and experiencing ≥ 1 episode of uveitis recurrence (100% vs. 0%, p < 0.001). Six episodes of uveitis recurrence were documented in five children, four of whom were African American. Topical corticosteroids were discontinued in 83% of children, and visual acuity remained stable for 1 year. Twelve children were tested for AAAs due to arthritis or uveitis flare-ups, with five (42%) being positive. No significant factors were associated with the development of AAAs. CONCLUSIONS: We found that ADA is effective in controlling inflammation, reducing the need for topical corticosteroids, and maintaining visual acuity in pCAU. There appears to be racial differences in African American children who had worse baseline disease and poorer outcomes. Studies are necessary to understand better and address these disparities.


Assuntos
Adalimumab , Uveíte Anterior , Acuidade Visual , Humanos , Criança , Adalimumab/uso terapêutico , Masculino , Feminino , Estudos Retrospectivos , Uveíte Anterior/tratamento farmacológico , Uveíte Anterior/diagnóstico , Doença Crônica , Anti-Inflamatórios/uso terapêutico , Anti-Inflamatórios/administração & dosagem , Adolescente , Resultado do Tratamento , Seguimentos , Pré-Escolar
13.
Nat Commun ; 15(1): 7438, 2024 Sep 10.
Artigo em Inglês | MEDLINE | ID: mdl-39256350

RESUMO

Gene therapy holds promise for treatment of inherited retinal dystrophies, a group of rare genetic disorders characterized by severe loss of vision. Here, we report up to 3-year pre-specified interim safety and efficacy results of an open-label first-in-human dose-escalation phase 1/2 gene therapy clinical trial in 12 patients with retinal dystrophy caused by biallelic mutations in the retinaldehyde-binding protein 1 (RLBP1) gene of the visual cycle. The primary endpoints were systemic and ocular safety and recovery of dark adaptation. Secondary endpoints included microperimetry, visual field sensitivity, dominant eye test and patient-reported outcomes. Subretinal delivery of an adeno-associated viral vector (AAV8-RLBP1) was well tolerated with dose-dependent intraocular inflammation which responded to corticosteroid treatment, and focal atrophy of the retinal pigment epithelium as the dose limiting toxicity. Dark adaptation kinetics, the primary efficacy endpoint, improved significantly in all dose-cohorts. Treatment with AAV8-RLBP1 resulted in the resolution of disease-related retinal deposits, suggestive of successful restoration of the visual cycle. In conclusion, to date, AAV8-RLBP1 has shown preliminary safety and efficacy in patients with RLBP1-associated retinal dystrophy. Trial number: NCT03374657.


Assuntos
Dependovirus , Terapia Genética , Distrofias Retinianas , Humanos , Terapia Genética/métodos , Distrofias Retinianas/genética , Distrofias Retinianas/terapia , Masculino , Adulto , Feminino , Dependovirus/genética , Proteínas de Transporte/genética , Pessoa de Meia-Idade , Vetores Genéticos/administração & dosagem , Vetores Genéticos/genética , Adulto Jovem , Resultado do Tratamento , Mutação , Adolescente
14.
Front Public Health ; 12: 1421211, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-39257951

RESUMO

Objective: We investigated the risk factors associated with severe or critical Coronavirus disease 2019 (COVID-19) infection due to the Omicron variant in patients with myasthenia gravis (MG) and determined the potential effect of COVID-19 on myasthenic exacerbation during the Omicron pandemic. Methods: This retrospective study included 287 patients with MG in Tianjin, China. Clinical data of the patients were collected using electronic questionnaires, databases, and clinical records. Results: The overall infection rate was 84.7%. Advanced age, comorbidities, generalized phenotype, and MG instability were drivers of COVID-19 severity, and post-COVID-19 myasthenic exacerbation. The concurrent use of a steroid-sparing agent did not affect COVID-19 susceptibility or severity. It did lower the risk of myasthenic exacerbation after COVID-19 infection. Patients with severe COVID-19 experienced myasthenic exacerbation earlier than patients with non-severe infection (p < 0.001). The severity of COVID-19 (Hazards Ratio = 3.04, 95% CI: 1.41-6.54, p = 0.004) and the clinical phenotype (Hazards Ratio = 3.29, 95% CI: 1.63-6.63, p < 0.001) emerged as independent risk factors for early MG exacerbation. Conclusion: Generally, patients with MG appear to be susceptible to the Omicron strains. Immunotherapy for MG did not increase COVID-19 susceptibility or severity. We do not advocate an immediate cessation of ongoing immunosuppressive treatments once a COVID-19 infection is diagnosed. Instead, a judicious evaluation of the risks and benefits, tailored to each individual, is recommended.


Assuntos
COVID-19 , Miastenia Gravis , SARS-CoV-2 , Humanos , COVID-19/epidemiologia , COVID-19/complicações , Estudos Retrospectivos , Masculino , Feminino , Pessoa de Meia-Idade , China/epidemiologia , Adulto , Fatores de Risco , Idoso , Índice de Gravidade de Doença , Comorbidade
15.
BMJ ; 386: e080474, 2024 Sep 11.
Artigo em Inglês | MEDLINE | ID: mdl-39260878

RESUMO

OBJECTIVE: To assess efficacy and safety of laughter exercise in patients with symptomatic dry eye disease. DESIGN: Non-inferiority randomised controlled trial. SETTING: Recruitment was from clinics and community and the trial took place at Zhongshan Ophthalmic Center, Sun Yat-sen University, the largest ophthalmic centre in China, between 18 June 2020 to 8 January 2021. PARTICIPANTS: People with symptomatic dry eye disease aged 18-45 years with ocular surface disease index scores ranging from 18 to 80 and tear film break-up time of eight seconds or less. INTERVENTIONS: Participants were randomised 1:1 to receive laughter exercise or artificial tears (0.1% sodium hyaluronic acid eyedrop, control group) four times daily for eight weeks. The laughter exercise group viewed an instructional video and participants were requested to vocalise the phrases "Hee hee hee, hah hah hah, cheese cheese cheese, cheek cheek cheek, hah hah hah hah hah hah" 30 times per five minute session. Investigators assessing study outcomes were masked to group assignment but participants were unmasked for practical reasons. MAIN OUTCOME MEASURES: The primary outcome was the mean change in the ocular surface disease index (0-100, higher scores indicating worse ocular surface discomfort) from baseline to eight weeks in the per protocol population. The non-inferiority margin was 6 points of this index score. Main secondary outcomes included the proportion of patients with a decrease from baseline in ocular surface disease index score of at least 10 points and changes in dry eye disease signs, for example, non-invasive tear break up time at eight weeks. RESULTS: 299 participants (mean age 28.9 years; 74% female) were randomly assigned to receive laughter exercise (n=149) or 0.1% sodium hyaluronic acid (n=150). 283 (95%) completed the trial. The mean change in ocular surface disease index score at eight weeks was -10.5 points (95% confidence interval (CI) -13.1 to -7.82) in the laughter exercise group and -8.83 (-11.7 to -6.02) in the control group. The upper boundary of the CI for difference in change between groups was lower than the non-inferiority margin (mean difference -1.45 points (95% CI -5.08 to 2.19); P=0.43), supporting non-inferiority. Among secondary outcomes, the laughter exercise was better in improving non-invasive tear break up time (mean difference 2.30 seconds (95% CI 1.30 to 3.30), P<0.001); other secondary outcomes showed no significant difference. No adverse events were noted in either study group. CONCLUSIONS: The laughter exercise was non-inferior to 0.1% sodium hyaluronic acid in relieving subjective symptoms in patients with dry eye disease with limited corneal staining over eight weeks intervention. TRIAL REGISTRATION: ClinicalTrials.gov NCT04421300.


Assuntos
Síndromes do Olho Seco , Ácido Hialurônico , Humanos , Ácido Hialurônico/administração & dosagem , Ácido Hialurônico/uso terapêutico , Síndromes do Olho Seco/tratamento farmacológico , Feminino , Masculino , Adulto , Pessoa de Meia-Idade , Terapia do Riso/métodos , Adulto Jovem , Resultado do Tratamento , Adolescente , Lubrificantes Oftálmicos/administração & dosagem , Lubrificantes Oftálmicos/uso terapêutico , Soluções Oftálmicas
16.
Brasília; Fiocruz Brasília; 11 set 2024. 50 p.
Não convencional em Português | LILACS, Coleciona SUS, PIE | ID: biblio-1570461

RESUMO

DESTAQUES ● Este mapa de evidências apresenta resultados de efetividade de intervenções realizadas em escolas, recuperadas da literatura científica global. ● Foram incluídos 217 revisões sistemáticas (RS) e 3 estudos primários que avaliaram os efeitos de treze ações relativas à Promoção da Saúde em Escolas. ● Quanto à qualidade metodológica, as RS foram assim classificadas: 9 de confiança alta, 14 moderada, 16 baixa e 178 criticamente baixas. ● As intervenções foram categorizadas segundo as ações previstas no Programa Saúde na Escola (PSE): 1) Combate ao Aedes aegypti; 2) Promoção da atividade física; 3) Promoção da cidadania ; 4) Prevenção da Covid-19; 5) Promoção de direitos sexuais e reprodutivos; 6) Controle de doenças negligenciadas; 7) Ações de saúde auditiva; 8) Ações de saúde bucal; 9) Ações de saúde ocular; 10) Promoção da alimentação saudável; 11) Monitoramento da situação vacinal; 12) Prevenção e uso de substâncias e 13) Prevenção de violências e acidentes.


HIGHLIGHTS ● This evidence map presents results on the effectiveness of interventions carried out in schools, retrieved from the global scientific literature. ● A total of 217 systematic reviews (SRs) and 3 primary studies that evaluated the effects of thirteen actions related to Health Promotion in Schools were included. ● Regarding methodological quality, the SRs were classified as follows: 9 with high confidence, 14 moderate, 16 low and 178 critically low. ● The interventions were categorized according to the actions provided for in the Brazilian School Health Program (PSE): 1) Combating Aedes aegypti; 2) Promoting physical activity; 3) Promoting citizenship; 4) Preventing Covid-19; 5) Promoting sexual and reproductive rights; 6) Controlling neglected diseases; 7) Hearing health actions; 8) Oral health actions; 9) Eye health actions; 10) Promoting healthy eating; 11) Monitoring vaccination status; 12) Prevention and use of substances and 13) Prevention of violence and accidents.


Assuntos
Serviços de Saúde Escolar , Revisão
17.
J Cardiothorac Surg ; 19(1): 510, 2024 Sep 03.
Artigo em Inglês | MEDLINE | ID: mdl-39227955

RESUMO

BACKGROUND: Myasthenia gravis is an autoimmune disease with high prevalence of thymus disorders, in which, thymectomy is considered one of the therapeutic approaches in improving the patients' clinical outcomes. Today, thoracoscopic thymectomy has received significant attention than the classic transsternal approach due to fewer complication. Therefore, this study was designed with the aim of investigating the therapeutic outcomes of thymectomy in patients with myasthenia gravis in the Afzalipour Hospital of Kerman between 2011 and 2021. METHODS: The current study is a descriptive analytical study on patients with myasthenia gravis who underwent surgical thymectomy within 2011-2021. Demographic and clinical characteristics of patients from the time of operation to three years of follow-up were extracted and recorded from clinical records or by phone calls. Data were analyzed using SPSS software. RESULTS: The data of 70 patients who underwent surgical thymectomy were analyzed. Thymectomy caused a significant reduction in the severity of the disease according to the Osserman classification (P = 0.001). It also significantly reduced the use of corticosteroids (P = 0.001) and IVIG (P = 0.015) compared to the time before the surgery. Sixty-two patients (88.57%) needed to take less medicine than before surgery. Left VATS was associated with less post-operative severity of the disease (P = 0.023). There were only two deaths during the follow-up period. CONCLUSION: Overall, the findings of the present study demonstrated that thoracoscopic thymectomy is a useful surgical approach that leads to faster recovery, reducing the severity of the disease, need for medication, and complications in patients with myasthenia gravis, In comparison with the transsternal approach.


Assuntos
Miastenia Gravis , Timectomia , Humanos , Miastenia Gravis/cirurgia , Timectomia/métodos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Resultado do Tratamento , Estudos Retrospectivos , Adulto Jovem , Toracoscopia/métodos , Adolescente , Cirurgia Torácica Vídeoassistida/métodos , Seguimentos
18.
Bol Med Hosp Infant Mex ; 81(4): 217-224, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-39236669

RESUMO

BACKGROUND: Some cancer survivors experience difficulties with concentration, attention, and memory; however, there are no studies on neurodevelopment in patients under 5 years of age who are undergoing cancer treatment. Our aim was to evaluate neurodevelopment in cancer patients under 5 years of age using the Early Development Instrument (EDI) test, considering factors such as nutritional status, type of cancer, and treatment effect. METHODS: A cross-sectional study was conducted from February 2018 to March 2019. Patients with cancer diagnoses outside the central nervous system in any phase of cancer treatment were included. RESULTS: A total of 45 patients were included. Regarding fine motor skills, 28% of patients with retinoblastoma and 23% of patients with leukemia or lymphoma had a risk of developmental delay compared to 0% of patients with solid tumors (p = 0.025). The final results showed that 19 (42.2%) patients had normal neurodevelopment (gray), 7 (15.5%) had a delay in neurodevelopment (light gray), and 19 (42.2%) had a risk of developmental delay (black). Regarding developmental delay, 52% of patients in the leukemia and lymphoma group, 71% in the retinoblastoma group, and 23% in the solid tumor group presented developmental delay (p = 0.06). CONCLUSIONS: The risk of delay and lag in neurodevelopment is common in cancer patients under 5 years of age undergoing treatment. However, more studies are required to evaluate the effect of treatment on this group of patients as it may be affected by various factors.


INTRODUCCIÓN: En algunos pacientes supervivientes de cáncer se presentan dificultades de concentración, atención y memoria, sin embargo no hay estudios en relación al neurodesarrollo en pacientes menores de 5 años que se encuentran en tratamiento oncológico. Por lo que el objetivo fue valorar el neurodesarrollo en pacientes con cáncer durante el tratamiento oncológico mediante la prueba EDI tomando en cuenta diversos factores como su estado nutricional, tipo de cancer, y el efecto del tratamiento. MÉTODOS: Se realizó un estudio transversal, de febrero de 2018 a marzo de 2019. Se incluyeron pacientes mayores de 1 año y menores de 5 años con diagnóstico de cáncer fuera del sistema nervioso central, en tratamiento oncológico. RESULTADOS: Se incluyeron 45 pacientes. En el área motor fina el 28% de los pacientes con retinoblastoma y 23% con leucemias y linfomas se encontraron en rojo (retraso) en comparación con 0% de los pacientes con tumores sólidos (p = 0.025). En el resultado global se encontró que 19 (42.2%) pacientes tuvieron neurodesarrollo normal (gris), 7 (15.5%) rezago en el neurodesarrollo (gris claro) y 19 (42.2%) con riesgo de retraso en el desarrollo (negro). De los pacientes que presentaron riesgo de retraso el 52% fueron del grupo de leucemias y linfomas, el 71% en el grupo de retinoblastoma y el 23% del grupo de tumores sólidos (p = 0.06). CONCLUSIONES: La presencia de riesgo de retraso y rezago en el neurodesarrollo es frecuente en menores de 5 años con diagnóstico de cáncer. Se requieren más estudios, para evaluar el efecto del tratamiento en este grupo de pacientes, ya que pueden influir diversos factores.


Assuntos
Deficiências do Desenvolvimento , Neoplasias , Humanos , Estudos Transversais , Pré-Escolar , Masculino , Feminino , Lactente , Deficiências do Desenvolvimento/etiologia , Deficiências do Desenvolvimento/epidemiologia , Transtornos do Neurodesenvolvimento/epidemiologia , Transtornos do Neurodesenvolvimento/etiologia , Retinoblastoma , Estado Nutricional , Desenvolvimento Infantil/fisiologia , Sobreviventes de Câncer/estatística & dados numéricos , Fatores de Risco
19.
J Refract Surg ; 40(9): e625-e634, 2024 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-39254242

RESUMO

PURPOSE: To compare the postoperative outcomes following implantation of KERATACx ring segments (Imperial Medical Technologies Europe GmbH) in patients having eccentric keratoconus with three different topographic patterns. METHODS: This retrospective cohort study was conducted at Maadi Eye Subspeciality Center, Cairo, Egypt. The study included patients with keratoconus who had implantation of KERATACx ring segments. Three groups were segregated based on topographic keratoconus patterns using the Sirius CSO Topographer (CSO Italia): type I ectasia where the cone coincides with the corneal flat axis, type II ectasia in which the cone coincides with neither the steep nor the flat axis and lies between the two axes, and type II ectasia for cones coinciding with the corneal steep axis. The visual and topographic outcomes were compared preoperatively and postoperatively for the three enrolled groups. RESULTS: This study enrolled 92 eyes of 92 patients and had a mean ± standard deviation follow-up of 16.9 ± 9.2 months. The patients' medical records revealed that night vision complaints and halos around the light were experienced the most by the type III ectasia group (31.25%). For the type III ectasia group, four topographic indices and one visual parameter did not show statistically significant differences between the preoperative and postoperative data (inferior-superior difference at 2- and 4-mm diameter, coma aberration, higher order aberrations, and uncorrected distance visual acuity), contrary to the type I and II ectasia groups, which showed significant improvements in all evaluated parameters. CONCLUSIONS: The type III morphological pattern of ectasia is the least likely to benefit from KERATACx ring segments implantation. [J Refract Surg. 2024;40(9):e625-e634.].


Assuntos
Substância Própria , Topografia da Córnea , Ceratocone , Próteses e Implantes , Implantação de Prótese , Acuidade Visual , Humanos , Ceratocone/cirurgia , Ceratocone/fisiopatologia , Estudos Retrospectivos , Acuidade Visual/fisiologia , Masculino , Feminino , Substância Própria/cirurgia , Substância Própria/patologia , Adulto , Adulto Jovem , Seguimentos , Refração Ocular/fisiologia , Adolescente , Resultado do Tratamento , Período Pós-Operatório
20.
J Refract Surg ; 40(9): e595-e603, 2024 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-39254246

RESUMO

PURPOSE: To report 12-month visual and refractive outcomes following topography-guided femtosecond laser-assisted laser in situ keratomileusis (LASIK) for myopia and compound myopic astigmatism correction. METHODS: This prospective, single-center observational study was conducted in an outpatient clinical practice at the Stanford University Byers Eye Institute in Palo Alto, California. Uncorrected (UDVA) and corrected (CDVA) distance visual acuity, 5% and 25% contrast sensitivity CDVA, and manifest refraction following topography-guided femtosecond laser-assisted LASIK were assessed. Refractive measurements were used to perform a vector analysis. RESULTS: Sixty eyes of 30 patients (mean age: 32.8 ± 7.0 years; range: 23 to 52 years) undergoing topography-guided LASIK for the correction of myopia and compound myopic astigmatism were analyzed. Mean postoperative UDVA was -0.09 ± 0.10 logarithm of the minimum angle of resolution (logMAR) at 12 months. Mean preoperative CDVA was -0.09 ± 0.09 and -0.13 ± 0.08 logMAR at postoperative 12 months. At 12 months, 26.9% of eyes had gained one or more lines of postoperative UDVA compared to baseline CDVA. Mean pre-operative 5% contrast sensitivity CDVA was 0.68 ± 0.07 and 0.64 ± 0.12 logMAR at 12 months (P = .014) following LASIK. CONCLUSIONS: Topography-guided LASIK for myopia and myopic astigmatism correction provided excellent visual and refractive outcomes that were predictable, precise, and stable up to 12 months postoperatively. [J Refract Surg. 2024;40(9):e595-e603.].


Assuntos
Astigmatismo , Topografia da Córnea , Ceratomileuse Assistida por Excimer Laser In Situ , Lasers de Excimer , Miopia , Refração Ocular , Cirurgia Assistida por Computador , Acuidade Visual , Humanos , Ceratomileuse Assistida por Excimer Laser In Situ/métodos , Acuidade Visual/fisiologia , Astigmatismo/cirurgia , Astigmatismo/fisiopatologia , Estudos Prospectivos , Miopia/cirurgia , Miopia/fisiopatologia , Refração Ocular/fisiologia , Adulto , Masculino , Feminino , Adulto Jovem , Lasers de Excimer/uso terapêutico , Pessoa de Meia-Idade , Cirurgia Assistida por Computador/métodos , Sensibilidades de Contraste/fisiologia , Resultado do Tratamento , Seguimentos
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