scholarly journals Longitudinal Study on Ocular Manifestations in a Cohort of Patients with Fabry Disease

2019 ◽  
Author(s):  
Langis Michaud
Keyword(s):  
Anterior Segment ◽  
Published Data ◽  
Retinal Vessels ◽  
Enzyme Replacement ◽  
Corneal Haze ◽  
Ocular Manifestations ◽  
Ocular Pressure ◽  
Upper Lid ◽  
Group 2 ◽  
Conjunctival Vessels

ABSTRACTPurposeThis study aims to assess the evolution of ocular manifestations in a cohort of Fabry patients.METHODSThis is a prospective observational study conducted from 2013 to 2017 (5 consecutive exams). All subjects underwent a comprehensive ocular examination including oriented case history, refraction, corneal topography, biomechanical corneal properties and pachometry assessments, aberrometry, anterior segment evaluation, double-frequency visual field (FDT), intra-ocular pressure, and ocular fundus. At baseline, 41 subjects enrolled but 9 dropped-out and 4 files were not kept for analysis (missing data). Remaining 28 subjects were classified into: Group 1 -hemizygotes (HMZ), all on enzyme replacement therapy (ERT) (N=10); Group 2 -heterozygotes (HTZ) actively ERT-treated (N=8), and Group 3 -HTZ not treated (N=10).RESULTSThere is a high intra and inter-subjects variability. At baseline, prevalence of the ocular manifestations found is similar to published data: cornea verticillata (89.2%), conjunctival vessels tortuosity (85.7%), corneal haze (67.8%), retinal vessels tortuosity (64.2%), anterior cataract (39.2%) and posterior cataract (28.5%). Prevalence for new elements are found: upper lid vessels toricity (96.4%) and micro-aneurysms (42.8%). At the end, micro-aneurysms (+82%), posterior cataract (+75%) corneal haze (+21%) anterior cataract (+17%) and retinal vessels tortuosities (+4%) evolved in prevalence and severity despite the fact that 68% of the patients were on ERT. Treated heterozygotes evolved more than other groups (p>0.05)CONCLUSIONERT does not halt the clinical evolution of several ocular manifestations. Longer observational time may be required to fully confirm these findings.

scholarly journals “Old wine in a new bottle” - post COVID-19 infection, central serous chorioretinopathy and the steroids

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Srinivasan Sanjay ◽  
Poornachandra B. Gowda ◽  
Bhimasena Rao ◽  
Deepashri Mutalik ◽  
Padmamalini Mahendradas ◽  
...  
Keyword(s):  
Virus Disease ◽  
Anterior Segment ◽  
Central Serous Retinopathy ◽  
Oral Antibiotics ◽  
Rt Pcr ◽  
Case Presentation ◽  
Ocular Manifestations ◽  
Corrected Distance Visual Acuity ◽  
History Of ◽  
The Right

Abstract Introduction Corona virus disease (COVID-19) pandemic can cause myriad of ocular manifestations. We report a case of unilateral multi focal central serous retinopathy, post COVID-19 infection in an Asian Indian female. Case presentation A 42-year-old female presented to us with unilateral blurring, in the right eye (OD), 12 days after COVID-19 infection. She had fever, chills, shortness of breath and cough with tiredness and was COVID- RT PCR positive. She was administered intravenous and oral antibiotics with injection heparin/remdesivir, during her 7 day stay at the hospital. She was also on steroid inhalers. She had no systemic history of note. On ocular evaluation, her corrected distance visual acuity was 20/40 in OD and 20/20 in left eye (OS). Anterior segment was normal. Anterior vitreous was clear. Fundus examination of the OD showed central serous retinopathy (CSCR) with OS being normal. Conclusion CSCR can occur post COVID-19 due to steroid administration and physicians administering it should be aware of this and refer the patients to an ophthalmologist earlier.

Ocular manifestations in patients who underwent COVID-19 in the early stages after Phaco surgery with implantation of IOL

2021 ◽  
pp. 80-85
Author(s):  
T.N. Savranova ◽  
◽  
V.U. Rozukulov ◽  
A.F. Yusupov ◽  
◽  
...  
Keyword(s):  
Dry Eye ◽  
Anterior Part ◽  
Anterior Segment ◽  
Early Postoperative Period ◽  
Posterior Segment ◽  
Vascular Pathology ◽  
Ocular Manifestations ◽  
Uveal Tract ◽  
Ocular Symptoms ◽  
Rehabilitation Period

Purpose. To study the ocular manifestations in patients with pseudophakia who underwent COVID-19 during the rehabilitation period after surgery. Material and methods. 46 patients with Phaco with implantation of IOL who underwent COVID-19 in the period from 1 week to 2 months after surgery. Of the examined patients, there were 28 men (61%), 18 women (391%). The average age of the patients was 63±1,2 years. Results. In 78% of cases, vascular pathology of the anterior and posterior segments of the eyeball was observed in patients who underwent COVID-19 in the early postoperative period after Phaco with implantation of IOL. Conclusions. The main ocular symptoms from the anterior segment of the eyeball in patients in 17% of cases were the occurrence or intensification of previously existing manifestations of the «dry eye» syndrome, as well as inflammatory phenomena from the anterior part of the uveal tract. From the posterior segment of the eye, in 70% of cases, there was a progression of vascular disorders, as well as the appearance of complications associated with manifestations of hypercoagulation syndrome and systemic vasculopathy. Key words: Covid-19, cataract phacoemulsification, pseudophakia.

A Comparative Study Between Preoperative Subconjunctival Bevacizumab Injection And Postoperative Topical Application In Prevention Of Recurrence After Excision Of Primary Pterygium In Egyptian Patients

QJM ◽  
2021 ◽  
Vol 114 (Supplement_1) ◽  
Author(s):  
Basma Helal Mohamed ◽  
Othman Ali Othman Ziko ◽  
Hisham M Khairy Abd El Dayem ◽  
Nancy Ezzelregal Khamis Ahmed
Keyword(s):  
Corneal Thickness ◽  
Anterior Segment ◽  
P Value ◽  
Eye Drops ◽  
Subconjunctival Injection ◽  
Fibrovascular Tissue ◽  
Significant Difference ◽  
Group 2 ◽  
Group 1 ◽  
Primary Pterygium

Abstract Purpose to compare between recurrence incidence after primary pterygium excision when using preoperative subconjunctival injection of Bevacizumab (Avastin) and using it as a postoperative eye drops. Methods thirty two eyes of thirty patients (two patients had bilateral pterygium) with primary pterygia were clinically examined, classified into 3 groups and operated by simple excision with bare sclera technique. Group 1 included 10 patients received Bevacizumab (Avastin) in the form of eye drops (10 mg/ml) 3 times daily for 6 days postoperative. Group 2 included 10 patients received preoperative Bevacizumab in the form of subconjunctival injection (1.25 mg/0.05ml) single dose 1 week preoperative. Group 3 included 10 patients (12 eyes) 2 patients with bilateral Pterygium didn’t receive any form of Bevacizumab. Postoperative follow up was done clinically and by serial photography at 1 week, 1 month, 3 months and 6 months searching for signs of recurrence and/or complications. Results The results showed different grades of recurrence in 18 eyes of 32.True recurrence was seen in 7 patients of 18 (1 patient in group 1, 2 in group 2 and 4 in group3).Recurrence grades in group 1and 2 who used the Bevacizumab (20%grade II, 50% grade III, and 30% grade IV). Recurrence could be predicted by 100% depending on fibrovascular tissue appearing in the surgical bed at 3 months postoperative (P value 0.038).Preoperative fleshy pterygium has high statistical significance in realation to recurrence(P value = 0.006).Patient’s sex, residence and occupation had no statistically significant value in the process of recurrence (P value > 0.05). Patients with recurrent Pterygia (in group 1&2) had statistically significant changes in the corneal K- readings at 3 months and 6 months.No significant difference in the limbal or central corneal thickness in the operated eye and the other eye (Pvalue > 0.05). Conclusion Bevacizumab (Avastin) is a well tolerated drug with multiple drug delivery methods.The eye drops give better results than the subconjunctival injection.Appearance of fibrovascular tissue in the surgical bed at 3 months predict the recurrence by 100%. Preoperative fleshy pterygia will mostly recur again whatever Bevacizumab form was used .The corneal thickness by anterior segment OCT has no role in prediction or detection of early pterygium recurrence.

scholarly journals Whip: Communicate and Test What to Expect from Data

2018 ◽  
Vol 2 ◽  
pp. e25317
Author(s):  
Stijn Van Hoey ◽  
Peter Desmet
Keyword(s):  
Data Quality ◽  
Controlled Vocabulary ◽  
Published Data ◽  
Data Set ◽  
Data Publication ◽  
Use Of Data ◽  
Maximum Utility ◽  
Group 2 ◽  
Publication Guidelines ◽  
Available Information

The ability to communicate and assess the quality and fitness for use of data is crucial to ensure maximum utility and re-use. Data consumers have certain requirements for the data they seek and need to be able to check if a data set conforms with these requirements. Data publishers aim to provide data with the highest possible quality and need to be able to identify potential errors that can be addressed with the available information at hand. The development and adoption of data publication guidelines is one approach to define and meet those requirements. However, the use of a guideline, the mapping decisions, and the requirements a dataset is expected to meet, are generally not communicated with the provided data. Moreover, these guidelines are typically intended for humans only. In this talk, we will present 'whip': a proposed syntax for data specifications. With whip, one can define column-based constraints for tabular (tidy) data using a number of rules, e.g. how data is structured following Darwin Core, how a term uses controlled vocabulary values, or what the expected minimum and maximum values are. These rules are human- and machine-readable, which communicates the specifications, and allows to automatically validate those in pipelines for data publication and quality assessment, such as Kurator. Whip can be formatted as a (yaml) text file that can be provided with the published data, communicating the specifications a dataset is expected to meet. The scope of these specifications can be specific to a dataset, but can also be used to express expected data quality and fitness for use of a publisher, consumer or community, allowing bottom-up and top-down adoption. As such, these specifications are complementary to the core set of data quality tests as currently under development by the TDWG Biodiversity Data Quality Task 2 Group 2. Whip rules are currently generic, but more specific ones can be defined to address requirements for biodiversity information.

Rifting and subduction records of the Paleo–Tethys in North Laos: Constraints from Late Paleozoic mafic and plagiogranitic magmatism along the Song Ma tectonic zone

2020 ◽  
Vol 133 (1-2) ◽  
pp. 212-232 ◽  
Author(s):  
Yuzhi Zhang ◽  
Xue Yang ◽  
Yuejun Wang ◽  
Xin Qian ◽  
Yukun Wang ◽  
...  
Keyword(s):  
South China ◽  
Southwest China ◽  
Hainan Island ◽  
Suture Zone ◽  
Published Data ◽  
Tectonic Zone ◽  
Group 3 ◽  
Group 2 ◽  
Subduction Setting ◽  
Group 1

Abstract In Southeast Asia, the Jinshajiang–Ailaoshan suture in Southwest China and the Song Ma zone in Vietnam were generally accepted as the amalgamation boundary of the South China and Simao/Indochina blocks. However, the tectonic location of the Paleotethyan suture in North Laos and its tectonic affinity remain ambiguous. To address this issue, we present the geochemical and geochronological data of a set of mafic and plagiogranitic rocks along the Song Ma suture zone in North Laos. Three groups of rocks with distinctive petrological, geochronological, and geochemical signatures were identified. The ca. 370 Ma Group 1 plagioclase amphibolite was preserved as xenoliths in the ca. 260 Ma granitoids. It exhibits a normal mid–oceanic–ridge basalt (N–MORB)-like, multi-elemental pattern with highly positive εNd(t) (+3.3 to +10.7) and εHf(t) (+6.68 to +7.41) values and low δ18O values (5.3‰−5.5‰), that are interpreted as products of the Late Devonian − earliest Carboniferous continental rift setting. The ca. 270 Ma Group 2 gabbro/diabase–diorite exhibits arc-like geochemical affinity with the εNd(t), εHf(t), and δ18O values of –5.85 to –3.76, –3.8 to +3.2, and 6.6‰–7.6‰, respectively. It is derived from the metasomatized wedge. The ca. 260 Ma Group 3 plagiogranite shows Nb-Ta and Ti negative anomalies and positive εNd(t) (+3.21 to +4.21), εHf(t) (+8.4 to +13.6), and δ18O (5.9‰−6.8‰) values, which are indicative of its derivation from mafic oceanic crust in a subduction setting. In combination with the published data, these results support the development of the Carboniferous–Permian Paleotethyan branch in North Laos, which represents the tectonic location of the Song Ma suture zone that spatially connects the Jinshajiang–Ailaoshan suture zone in Southwest China and the Chenxing–Bangxi suture zone in Hainan Island, South China.

Long-Term Follow-Up of Monoclonal Gammopathies in Gaucher Disease Patients on Enzyme Replacement Therapy.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 3336-3336
Author(s):  
Jae H. Park ◽  
Allan F. Moore ◽  
David J. Kuter
Keyword(s):  
Enzyme Replacement Therapy ◽  
Replacement Therapy ◽  
Gaucher Disease ◽  
Monoclonal Gammopathies ◽  
Enzyme Replacement ◽  
Group 3 ◽  
Group 2 ◽  
Initiation Of Therapy ◽  
Group 1

Abstract Gaucher Disease is a lysosomal storage disease in which glucocerebroside accumulates in macrophages because of a deficiency of the lysosomal enzyme, β-glucocerebrosidase. Polyclonal and monoclonal gammopathies (MG) have been frequently associated with long-standing Gaucher Disease, and there have been reports suggesting an increased incidence of multiple myeloma (MM) in these patients. Chronic stimulation of the immune system secondary to progressive glucocerebroside storage and increased expression of cytokines such as Interleukin 6 have all been postulated to be the mechanism underlying this association. The purpose of this study was to ascertain the incidence and natural history of MG in Type 1 Gaucher Disease patients on enzyme replacement therapy (ERT) followed for up to 15 years. Twenty-eight patients with Type I Gaucher Disease on ERT seen at a single medical center were selected for the analysis. Of the twenty-eight patients, 17 (60.7%) were male; 11 (39.3%) were female; and the median age at the initiation of therapy was 42 years (range 25–85 years). Serum protein electrophresis (SPEP), immunofixation, and immunoglobulin quantitation were performed in all of these patients before initiating ERT and at intervals during follow-up. The patients were categorized into three groups based on their initial SPEP results: Group 1: those with normal SPEP pattern; Group 2: those with polyclonal gammopathy; and Group 3: those with MG. At the time of initiation of ERT, Group 1 contained 12 patients (42.8%) while Groups 2 and 3 each had 8 patients (28.6%). During follow up for a median of 8.5 years (range 1–15 years), 2 patients from the Group 1 (16.7%) and 4 patients from Group 2 (50%) developed MG. Of the patients in Group 3, 3 patients (21.4%) reverted to a normal SPEP pattern. Overall, 14 patients (50%) in all three groups had a MG identified at the time of initiation of therapy or during follow-up: 3 (all in Group 3) reverted to a normal SPEP pattern while the other 11 patients showed no change in M-spike levels after a median of 8.5 years of follow up. All 14 patients with MG had a monoclonal IgG immunoglobulin, and 3 of them also had a monoclonal IgM immunoglobulin. There was no association between the occurance or type of MG with any particular Gaucher Disease genotype, patient age, or other clinical variable. Only one of the patients developed a malignancy: an elderly man with MG developed Hodgkin’s Disease. None of our patients developed MM. In conclusion, these data confirm the high rate of MG in patients with Gaucher Disease but a low rate of progression to any other plasmacytic or lymphocytic disorder.

scholarly journals Circulating Forms of B-Type Natriuretic Peptide in Very Preterm Infants

2020 ◽  
Vol 5 (3) ◽  
pp. 506-515
Author(s):  
Sarah L Harris ◽  
Richard Troughton ◽  
Lynley Lewis ◽  
Nicola Austin ◽  
Chris Pemberton
Keyword(s):  
Natriuretic Peptide ◽  
Preterm Infants ◽  
Premature Infants ◽  
High Performance ◽  
Ductus Arteriosus ◽  
Clinical Status ◽  
Volume Overload ◽  
Published Data ◽  
Very Preterm Infants ◽  
Group 2

Abstract Background B-type natriuretic peptide (BNP) is a cardiac hormone released with an N-terminal fragment (NTproBNP) under conditions of ventricular pressure or volume overload. BNP has been proposed for use as a biomarker of cardiac dysfunction in premature infants in the setting of hemodynamically significant patent ductus arteriosus (HsPDA) and bronchopulmonary dysplasia (BPD). In adult settings the presence of proBNP and glycosylated isoforms may affect assay interpretation. However, there are limited data on how immature preterm physiology may affect BNP or NTproBNP levels and no published data on post-translational BNP processing in premature infants. Methods Pooled serial plasma samples from preterm infants born at less than 30 weeks gestation were analyzed for BNP congeners using Luminex® assay and high performance liquid chromatography. Samples were grouped according to clinical status: Group 1, no HsPDA and no BPD, Group 2 HsPDA and no/mild BPD, Group 3 HsPDA and moderate/severe BPD. Results Plasma from 15 infants was analyzed, and across all three groups NTproBNP predominated with minimal amounts of other isoforms; no glycosylation was detected. Conclusions NTproBNP appears to be the predominant isoform across each of our clinical groups in our pooled sample analysis with no evidence of significant glycosylation. This suggests NTproBNP is likely to be a robust marker in this clinical setting.

scholarly journals Lens-induced hypopyon uveitis as the presenting manifestation of posterior lens nucleus dislocation following pars-plana vitrectomy: case report

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Ksiaa Imen ◽  
Ben Hadj Tahar Meriam ◽  
Sellem Ilhem ◽  
Attia Sonia ◽  
Abroug Nesrine ◽  
...  
Keyword(s):  
Pars Plana Vitrectomy ◽  
Oral Corticosteroid ◽  
Anterior Segment ◽  
Final Diagnosis ◽  
Anterior Capsule ◽  
Ocular Pressure ◽  
Pars Plana ◽  
Bullous Retinal Detachment ◽  
Lens Material

AbstractA 57-year-old otherwise healthy male presented to our department seven days following uneventful pars-plana vitrectomy with gas tamponade for a superior bullous retinal detachment in the left eye. Ophthalmic examination revealed anterior segment inflammation with hypopyon and fibrinous exudate. Intra-ocular pressure was 28 mmHg. Posterior segment evaluation was difficult to assess due to the presence of anterior capsule opacification and gas bubble. A Toxic Anterior Segment Syndrome was suspected, and the patient was treated with topical and oral corticosteroid medication in combination with anti-glaucomatous therapy. On follow-up, anterior segment inflammation and ocular hypertension improved. On day ten post-operatively, ocular ultrasonography demonstrated lens material inferiorly with attached retina. The final diagnosis of posterior lens nucleus dislocation with lens-induced uveitis was retained. The patient underwent an uneventful second vitrectomy with aspiration of the dislocated lens nucleus and sulcus three piece-lens implantation. On last follow-up, visual acuity was 20/50 with no relapsing of ocular inflammation and the retina remained reattached.

scholarly journals The effect of conjunctivochalasis detected by anterior segment optical coherence tomography on tear function in an elderly population

2020 ◽  
Vol 12 ◽  
pp. 251584142093087
Author(s):  
Dilay Ozek ◽  
Emine Esra Karaca ◽  
Ozlem Evren Kemer
Keyword(s):  
Ocular Surface ◽  
Elderly Population ◽  
Anterior Segment ◽  
Optic Coherence Tomography ◽  
Index Test ◽  
Test Results ◽  
Surface Staining ◽  
Tear Meniscus ◽  
Group 2 ◽  
Group 1

Introduction: The aim of this study was to evaluate conjunctivochalasis (CCH) and its relationship with tear meniscus and tear function in an elderly population. Materials and methods: This prospective, observational study included 144 eyes of 144 patients aged >65 years who were referred to our clinic for various reasons. The patients were separated into group 1 including 64 eyes of 64 patients with CCH and group 2 including 80 eyes of 80 patients without CCH. All patients in both groups underwent a full ophthalmological examination, and the presence of CCH, fluorescein tear break-up time (FTBUT) test, Schirmer test, ocular surface staining (Oxford grading score) and OSDI (Ocular Surface Disease Index) test results were recorded. Measurements of the conjunctivochalasis area (CCHA), tear meniscus height (TMH) and tear meniscus area (TMA) were taken using anterior segment optic coherence tomography (AS-OCT). Results: Group 1 comprised 34 females and 30 males with a mean age of 71.15 ± 12.34 years. Group 2 comprised 43 females and 37 males with a mean age of 68.16 ± 6.05 years ( p = 0.122). The CCH rate was 44.4% in all of the examined patients. The OSDI score and the ocular surface staining test were significantly higher ( p < 0.05), and the FTBUT, TMH and TMA were significantly lower ( p < 0.05) in group 1 than in group 2. The Schirmer I test results were not significantly different between the two groups. Conclusion: The prevalence of CCH is quite high in elderly individuals and may disrupt tear function in these patients.

scholarly journals A clinical case of central retinal artery occlusion after pneumonia caused by SARS-CoV-2 (COVID-19)

2021 ◽  
Vol 6 (6-1) ◽  
pp. 41-47
Author(s):  
K. L. Latigan ◽  
D. A. Latigan ◽  
K. E. Dubnov ◽  
E. V. Bykova ◽  
D. A. Nefedov
Keyword(s):  
High Risk ◽  
Clinical Case ◽  
Vision Loss ◽  
Central Retinal Artery Occlusion ◽  
Anterior Segment ◽  
Retinal Artery Occlusion ◽  
Neovascular Glaucoma ◽  
Artery Occlusion ◽  
Retinal Vessels ◽  
Coronavirus Infection

In domestic and foreign medical periodical literature, highlighting the problem of ophthalmopathology against the background of coronavirus infection, the lesion of the anterior segment of the eye is most often described. It is extremely hard to find reports about pathology of the retina, optic nerve or central parts of the visual analyzer. However, it is widely acknowledged that there is a high risk of developing coagulopathy against the background of COVID-19 infection, which leads to occlusion and thrombosis of retinal vessels, ischemic neuropathies. The problem of irreversible loss of vision due to circulatory disorders of the retinal vessels was urgent even before the wide spread of coronavirus infection due to the high prevalence of atherosclerosis, hypertension and type 1 and 2 diabetes in the population. Also, it is widely known that occlusions and thrombosis of retinal vessels can be formidable harbingers of the developing life-threatening conditions. Knowledge about the high risk of vascular ophthalmopatology against the background of a previous COVID-19 infection by the primary echelon ophthalmologists, early diagnostics and treatment of ischemic conditions of the posterior segment of the eye will reduce the frequency of irreversible vision loss due to these diseases, secondary neovascular glaucoma cases, and will also help to send patients to the multidisciplinary hospitals in a timely manner for the prevention of fatal complications of coagulopathy. The article provides a brief overview of foreign literary sources regarding the history of outbreaks of coronavirus infection in the world, as well as possible ways of damage to the organ of vision by the coronavirus. A clinical case of damage to the vascular bed of the retina in both eyes due to coagulopathy against the background of pneumonia caused by COVID-19 is presented, which is actual due to the low illumination of similarly cases.

Sign in / Sign up

Export Citation Format

Share Document