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Aravind Haripriya, Pradeep Y Ramulu, Shivkumar Chandrashekharan, Rengaraj Venkatesh, Kalpana Narendran, Madhu Shekhar, Rengappa Ramakrishnan, Ravilla D Ravindran, Alan L Robin
PURPOSE: To compare: (1) intraoperative complication rates, (2) one-year visual outcomes, and (3) post-operative complication rates over the first post-operative year in eyes with and without pseudoexfoliation (PEX) undergoing cataract surgery. DESIGN: Prospective comparative interventional study PARTICIPANTS: Nine-hundred thirty eyes with cataract and uncomplicated PEX (without phacodonesis, clinically shallow anterior chambers, or pupil size < 4mm ) and 476 controls with cataract but without PEX recruited from four centers of the Aravind Eye Care System in Southern India...
October 16, 2018: Ophthalmology
Ramazan Yağcı, Serdar Dervişoğulları, Emre Güler, Yüksel Totan, İbrahim Feyzi Hepşen
PURPOSE: The purpose of the present study was to evaluate the aqueous humor Endocan and Endostatin levels in patients with pseudoexfoliation syndrome (PEX) and to compare the results with healthy individuals. METHODS: 29 cataract patients with PEX syndrome (PEX group) and 32 cataract patients without PEX syndrome (control group) were enrolled in the study. Endocan and Endostatin were measured in the aqueous humor of the PEX and control groups by enzyme-linked immunosorbent assay...
October 18, 2018: Journal of Glaucoma
Stéphanie Romeo Villadóniga, Elena Rodríguez García, Olatz Sagastagoia Epelde, M Dolores Álvarez Díaz, Joan Carles Domingo Pedrol
Purpose: To assess the effects of antioxidant oral supplementation based on docosahexaenoic acid (DHA) in pseudoexfoliative (PEX) glaucoma. Patients and Methods: A prospective 6-month open-label randomized controlled trial was conducted in patients with PEX glaucoma and adequate intraocular pressure (IOP) control. Patients in the DHA group received a high-rich DHA (1 g) nutraceutical formulation. Ophthalmological examination, DHA erythrocyte membrane content (% total fatty acids), plasma total antioxidant capacity (TAC), plasma malondialdehyde (MDA), and plasma IL-6 levels were assessed...
2018: Journal of Ophthalmology
Yukichi Fujikawa, Marina Suekawa, Satoshi Endo, Youjirou Fukami, Shoji Mano, Mikio Nishimura, Muneharu Esaka
We evaluated the import of Arabidopsis catalase to peroxisomes under homogenous transient expression. The amino acids at -11 to -4 from the C-terminus are necessary for catalase import. The results are in agreement with the previous work under stable expression. We first demonstrate that heme-binding sites are important for peroxisomal import, suggesting the importance of catalase folding. ABBREVIATIONS: AtCat: Arabidopsis catalase; PTS: peroxisomal targeting signal; PEX: Peroxin.
October 8, 2018: Bioscience, Biotechnology, and Biochemistry
J E Cote-Orozco, O Y Echeverri-Pena, J M Guevara-Morales, E Espinosa
INTRODUCTION: Peroxisomal biogenesis disorders are due to mutations in the PEX genes, which code for peroxins that are required for peroxisomal biogenesis. Clinically, they are expressed as a Zellweger syndrome spectrum, and there is a wide phenotypic variety. They are diagnosed biochemically, and confirmation is molecular. The aim of this illustrative case is to highlight the importance of the clinical features and biochemical testing in the management of a peroxisomal disease. CASE REPORT: A 3-year-old boy with neonatal hypotonia, overall developmental delay and failure to thrive and a pattern of hypomyelinating leukodystrophy in brain resonance...
October 16, 2018: Revista de Neurologia
Curt Ekström, Sophia Wilger, Anders Wanhainen
PURPOSE: To study the relationship between pseudoexfoliation (PEX) and aortic aneurysm in a population with a high prevalence of PEX. METHODS: Survival analyses were performed in a cohort of 735 residents aged 65-74 years, examined in a population survey in the municipality of Tierp, Sweden, 1984-1986. To expand the sample size, 1040 people were recruited by means of glaucoma case records established at the Eye Department in Tierp in 1978-2007. In this way, the cohort comprised 1775 subjects, representing more than 25 400 person-years at risk...
September 20, 2018: Acta Ophthalmologica
Tanja Falter, Stephanie Herold, Veronika Weyer-Elberich, Carina Scheiner, Veronique Schmitt, Charis von Auer, Xavier Messmer, Philipp Wild, Karl J Lackner, Bernhard Lämmle, Inge Scharrer
BACKGROUND:  Autoimmune thrombotic thrombocytopenic purpura (iTTP) is caused by autoantibody-mediated severe a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13 (ADAMTS13) deficiency leading to micro-angiopathic haemolytic anaemia (MAHA) and thrombocytopenia with organ damage. Patients survive with plasma exchange (PEX), fresh frozen plasma replacement and corticosteroid treatment. Anti-CD20 monoclonal antibody rituximab is increasingly used in patients resistant to conventional PEX or relapsing after an acute bout...
October 2018: Thrombosis and Haemostasis
Alberto Galvez-Ruiz, Alicia Galindo-Ferreiro, Hind Alkatan
Peroxisomal biogenesis disorders (PBDs) are autosomal recessive diseases caused by mutations in one of the 14 PEX genes described in the scientific literature. All of these syndromes may be associated with different mutations in the PEX genes, the most frequent being PEX1 for patients with Zellweger syndrome (ZS). In this paper, we present the case of a patient with a peculiar clinical history: evisceration of the left eye (LE) at 4 years of age because of a benign ocular teratoid medulloepithelioma and a progressive loss of visual acuity (VA) in the right eye (RE) beginning at 9 years of age, leading to the diagnosis of ZS...
July 2018: Saudi Journal of Ophthalmology: Official Journal of the Saudi Ophthalmological Society
C Lehoux Dubois, E Labrèche, V Boudreau, J Colomba, M Mailhot, A Lavoie, R Rabasa-Lhoret, A Coriati
BACKGROUND: Due to lack of vitamin D absorption in patients with cystic fibrosis (CF), vitamin D supplementation becomes necessary. Our aim was to study the association between serum vitamin D levels and key clinical factors, such as nutritional status, pulmonary function and pulmonary exacerbations (PEx) frequency, in an adult CF population. METHODS: Prospective analysis of a published vitamin D (VitD3 ) supplementation protocol (N = 200 adult patients) over a follow-up period of 5 years...
August 25, 2018: Clinical Nutrition: Official Journal of the European Society of Parenteral and Enteral Nutrition
Steven P Campbell, Erin R Zylstra, Catherine R Darst, Roy C Averill-Murray, Robert J Steidl
Many of the processes that govern the viability of animal populations vary spatially, yet population viability analyses (PVAs) that account explicitly for spatial variation are rare. We develop a PVA model that incorporates autocorrelation into the analysis of local demographic information to produce spatially explicit estimates of demography and viability at relatively fine spatial scales across a large spatial extent. We use a hierarchical, spatial autoregressive model for capture-recapture data from multiple locations to obtain spatially explicit estimates of adult survival (ϕad ), juvenile survival (ϕjuv ), and juvenile-to-adult transition rates (ψ), and a spatial autoregressive model for recruitment data from multiple locations to obtain spatially explicit estimates of recruitment (R)...
September 5, 2018: Ecological Applications: a Publication of the Ecological Society of America
Laura S Gold, Donald L Patrick, Ryan N Hansen, Valeria Beckett, Christopher H Goss, Larry Kessler
BACKGROUND: Pulmonary exacerbations (PEx) in cystic fibrosis (CF) patients decrease lung function, increase symptoms and reduce health-related quality of life (HRQoL). We evaluated associations between 8 symptom-based questions from the Cystic Fibrosis Respiratory Symptom Diary - Chronic Respiratory Infection Symptom Score (CFRSD-CRISS) and the 5-level EuroQOL-5 Dimensions (EQ-5D-5 L) summary score and hypothesized the CFRSD-CRISS would be well-correlated with quality-of-life measures among CF patients with PEx...
August 28, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
Alison J Mbekeani, Will A Stanley, Vishal C Kalel, Noa Dahan, Einat Zalckvar, Lilach Sheiner, Wolfgang Schliebs, Ralf Erdmann, Ehmke Pohl, Paul W Denny
Peroxisomes are central to eukaryotic metabolism, including the oxidation of fatty acids-which subsequently provide an important source of metabolic energy-and in the biosynthesis of cholesterol and plasmalogens. However, the presence and nature of peroxisomes in the parasitic apicomplexan protozoa remains controversial. A survey of the available genomes revealed that genes encoding peroxisome biogenesis factors, so-called peroxins (Pex), are only present in a subset of these parasites, the coccidia. The basic principle of peroxisomal protein import is evolutionarily conserved, proteins harbouring a peroxisomal-targeting signal 1 (PTS1) interact in the cytosol with the shuttling receptor Pex5 and are then imported into the peroxisome via the membrane-bound protein complex formed by Pex13 and Pex14...
August 29, 2018: Genes
Anthoula Chatzikyriakidou, Panayiota Founti, Angeliki Melidou, Fani Minti, Emmanouil Bouras, Eleftherios Anastasopoulos, Theofanis Pappas, Anna-Bettina Haidich, Alexandros Lambropoulos, Fotis Topouzis
BACKGROUND: Pseudoexfoliation syndrome (PEX) and glaucoma (pseudoexfoliative glaucoma; PEXG, primary open-angle glaucoma; POAG) have mainly been studied for their associations with genes' polymorphisms. The purpose of this exploratory study was to investigate the role of polymorphisms in genes encoding for micro RNAs (miRNAs) and in genes related to miRNA biogenesis. MATERIAL AND METHODS: In the present genetic association study, ninety-two polymorphisms were investigated for their contribution to PEX (n = 203), PEXG (n = 38), and POAG (n = 40) pathogenesis compared to a control group (n = 188)...
October 2018: Ophthalmic Genetics
Susanna A McColley, Michael W Konstan, Bonnie W Ramsey, J Stuart Elborn, Michael P Boyle, Claire E Wainwright, David Waltz, Montserrat Vera-Llonch, Gautham Marigowda, John G Jiang, Jaime L Rubin
BACKGROUND: Improved lung function and fewer pulmonary exacerbations (PEx) were observed with lumacaftor/ivacaftor (LUM/IVA) in patients with cystic fibrosis homozygous for F508del. It is unknown whether PEx reduction extends to patients without early lung function improvement. METHODS: Post hoc analyses of pooled phase 3 data (NCT01807923, NCT01807949) categorized LUM/IVA-treated patients by percent predicted forced expiratory volume in 1 s (ppFEV1 ) change from baseline to day 15 into threshold categories (absolute change ≤0 vs >0; relative change <5% vs ≥5%) and compared PEx rates vs placebo...
August 23, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
Dorota Urbaniak, Małgorzata Seredyka-Burduk, Waldemar Błoch, Grażyna Malukiewicz, Bartłomiej J Kałużny
BACKGROUND The present study evaluated the optical density of particular layers of the cornea and anterior lens capsule in patients with pseudoexfoliation syndrome (PEX). MATERIAL AND METHODS Sixty patients with cataract and PEX (mean age 76.6±5.6 years, range 62-88 years) were compared to 55 controls with cataract without PEX syndrome (mean age 76.3±6.09 years, range 62-90 years; P>0.05). The anterior segment of one eye was examined in each patient using Pentacam HR by Oculus before the planned cataract surgery...
August 21, 2018: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
Lei Qin, Zhihua Jia, Dawei Xie, Zhongyuan Liu
BACKGROUND: Long noncoding RNA urothelial carcinoma-associated 1 (UCA1) has been reported to be a vital mediator in various cancers. But, in terms of gastric cancer (GC), the effects of UCA1 on GC cell proliferation, migration, invasion, and apoptosis remain unclear. This study aimed to uncover the potential regulatory mechanism of UCA1 in GC cells. METHODS: The expression level of UCA1 was first examined in the five different cell lines of HEK293, CCL-153, HUVEC, SUN-216, and SGC-7901 using a reverse-transcriptase quantitative polymerase chain reaction...
August 20, 2018: Journal of Cellular Biochemistry
Helen Chung, Sourabh Arora, Karim F Damji, Ezekiel Weis
OBJECTIVE: Pseudoexfoliation syndrome (PEX) is a systemic disease, but evidence of its association with cardiovascular disease (CVD) and cerebrovascular disease (CVA) is controversial. A quantitative systematic review will provide an accurate summary of the current body of the literature. DESIGN: Meta-analysis. PARTICIPANTS: Not applicable. METHODS: A comprehensive literature search of published and unpublished English-language studies was performed...
August 2018: Canadian Journal of Ophthalmology. Journal Canadien D'ophtalmologie
Yavuz Oruc, Sinem Keser, Elif Yusufoglu, Fatih Celik, İbrahim Sahin, Meltem Yardim, Suleyman Aydin
Purpose: Pseudoexfoliation syndrome (PEX) is an eye disease that develops under the influence of regional population differences, genetic factors, age, and environmental factors and is characterized by visualization of a gray-white fibrogranular substance in the lens anterior capsule and/or pupil margin during anterior segment examination. The underlying biochemical mechanisms of the disease have not yet been fully elucidated. Therefore, this study was designed to show the changes in aqueous humor and blood serum levels of matrix metalloproteinases (decorin and tenascin C), total antioxidants (TAS), and total oxidants (TOS) in both cataract patients who have unilateral PEX material and cataract patients who do not have unilateral PEX material...
2018: Journal of Ophthalmology
Marcia Soares, Ekaterina Mirgorodskaya, Hatice Koca, Emilia Viklund, Matthew Richardson, Per Gustafsson, Anna-Carin Olin, Salman Siddiqui
RATIONALE: Asthma is often characterised by inflammation, damage and dysfunction of the small airways, but no standardised biomarkers are available. OBJECTIVES: Using a novel approach-particles in exhaled air (PExA)-we sought to (a) sample and analyse abundant protein biomarkers: surfactant protein A (SPA) and albumin in adult asthmatic and healthy patients and (b) relate protein concentrations with physiological markers using phenotyping. METHODS: 83 adult asthmatics and 21 healthy volunteers were recruited from a discovery cohort in Leicester, UK, and 32 adult asthmatics as replication cohort from Sweden...
September 14, 2018: Journal of Breath Research
Mariam Hassan, Machaon M Bonafede, Brendan L Limone, Paul Hodgkins, Gregory S Sawicki
Purpose: To conduct an analysis describing clinical characteristics, pulmonary exacerbation (PEx) events, and health care resource utilization among Medicaid-insured patients with cystic fibrosis (CF). Patients and methods: A retrospective analysis of the Truven Health MarketScan® Medicaid Multi-State administrative claims database (2010-2014) was undertaken. Patients aged ≥6 years with a CF diagnosis, continuously enrolled for 12 months, were identified. Demographics, comorbidities, PEx events, and health care resource utilization and costs over a 12-month enrollment period were analyzed for all patients and by age groups...
2018: ClinicoEconomics and Outcomes Research: CEOR
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