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https://www.readbyqxmd.com/read/28549901/degradation-of-proteoglycan-4-lubricin-by-cathepsin-s-potential-mechanism-for-diminished-ocular-surface-lubrication-in-sj%C3%A3-gren-s-syndrome
#1
Suresh C Regmi, Michael L Samsom, Miriam Heynen, Gregory D Jay, Benjamin D Sullivan, Sruthi Srinivasan, Barbara Caffery, Lyndon Jones, Tannin A Schmidt
Sjögren's syndrome (SS) is an autoimmune disease affecting the lacrimal and salivary glands with hallmark clinical symptoms of dry eye and dry mouth. Recently, markedly increased cathepsin S (CTSS) activity has been observed in the tears of SS patients. Proteoglycan 4 (PRG4), also known as lubricin, is an effective boundary lubricant that is naturally present on the ocular surface. While PRG4 is susceptible to proteolytic digestion, the potential effect of CTSS on PRG4 remains unknown. The objective of this study was to assess the ability of CTSS to enzymatically degrade purified PRG4, and PRG4 naturally present in human tears, and alter ocular surface boundary lubricating properties...
May 23, 2017: Experimental Eye Research
https://www.readbyqxmd.com/read/28546925/comparison-of-corneal-biomechanics-in-sj%C3%A3-gren-s-syndrome-and-non-sj%C3%A3-gren-s-syndrome-dry-eyes-by-scheimpflug-based-device
#2
Qin Long, Jing-Yi Wang, Dong Xu, Ying Li
AIM: To compare the corneal biomechanics of Sjögren's syndrome (SS) and non-SS dry eyes with Corneal Visualization Scheimpflug Technology (CorVis ST). METHODS: Corneal biomechanics and tear film parameters, namely the Schirmer I test value, tear film break-up time (TBUT) and corneal staining score (CSS) were detected in 34 eyes of 34 dry eye patients with SS (SSDE group) and 34 dry eye subjects without SS (NSSDE group) using CorVis ST. The differences of the above parameters between the two groups were examined, and the relationship between corneal biomechanics and tear film parameters were observed...
2017: International Journal of Ophthalmology
https://www.readbyqxmd.com/read/28546881/haematological-malignancies-in-systemic-sclerosis-patients-case-reports-and-review-of-the-world-literature
#3
M Colaci, D Giuggioli, C Vacchi, C Ferri
Background. The association of systemic sclerosis (SSc) and haematological cancers was reported in a large number of case reports and cohort studies, describing SSc patients with highly heterogeneous clinical pictures. Objective. We reviewed the literature to better describe SSc patients with haematological malignancies. Methods. SSc cases complicated by haematological malignancies described in the world literature were collected; other 2 cases referred to our centre were reported. Results. One hundred-thirty SSc subjects were collected from 1954 up to date...
2017: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/28546741/sj%C3%A3-gren-s-syndrome-is-associated-with-negatively-variable-impacts-on-domains-of-health-related-quality-of-life-evidence-from-short-form-36-questionnaire-and-a-meta-analysis
#4
REVIEW
Qiuxiang Zhang, Xulin Wang, Haoyang Chen, Biyu Shen
PURPOSE: The purpose of this article was to systematically review the literature to identify the impact of primary Sjögren's syndrome (pSS) on specific health-related quality of life (HRQoL) domains. METHODS: A meta-analysis was performed, and the related articles were searched in Medline, Embase, Cochrane Library, China National Knowledge Infrastructure, China Biology Medicine, and Web of Science databases and in reference lists of articles and systematic reviews...
2017: Patient Preference and Adherence
https://www.readbyqxmd.com/read/28543186/clinical-biochemical-and-genetic-aspects-of-sj%C3%A3-gren-larsson-syndrome
#5
REVIEW
Kye Hee Cho, Sung Han Shim, MinYoung Kim
Sjögren-Larsson syndrome (SLS) is caused by an autosomal recessive mutation in ALDH3A2, which encodes the fatty aldehyde dehydrogenase responsible for the metabolism of long-chain aliphatic aldehydes and alcohols. The pathophysiologic accumulation of aldehydes in various organs, including the skin, brain, and eyes, leads to characteristic features of ichthyosis, intellectual disability, spastic di-/quadriplegia, and low visual acuity with photophobia. The severity of the clinical manifestations thereof can vary greatly, although most patients are bound to a wheelchair due to contractures...
May 23, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/28542241/signification-of-distal-urinary-acidification-defects-in-hypocitraturic-patients
#6
Valentina Forni Ogna, Anne Blanchard, Rosa Vargas-Poussou, Adam Ogna, Stéphanie Baron, Jean-Philippe Bertocchio, Caroline Prot-Bertoye, Jérôme Nevoux, Julie Dubourg, Gérard Maruani, Margarida Mendes, Alejandro Garcia-Castaño, Cyrielle Treard, Nelly Lepottier, Pascal Houillier, Marie Courbebaisse
BACKGROUND AND OBJECTIVES: Hypocitraturia has been associated with metabolic acidosis and mineral disorders. The aim of this study was to investigate the occurrence of urinary acidification defects underlying hypocitraturia. MATERIALS AND METHODS: This retrospective observational study included 67 patients (32 men), aged 40.7±15.1 years with hypocitraturia (<1.67 mmol/24-h) and nephrolithiasis, nephrocalcinosis, and/or bone demineralization, referred to our center from 2000 to 2015...
2017: PloS One
https://www.readbyqxmd.com/read/28542139/regulator-of-g-protein-signaling-2-is-a-key-regulator-of-pancreatic-%C3%AE-cell-mass-and-function
#7
H Dong, Y Zhang, J Wang, D S Kim, H Wu, B Sjögren, W Gao, L Luttrell, H Wang
Pancreatic β-cell death and dysfunction contributes to the pathogenesis of both type 1 and type 2 diabetes. We aimed to examine whether the regulator of G protein signaling protein 2 (RGS2), a multifunctional inhibitor of G protein-coupled receptor (GPCR) signaling, impacts β-cell death and function. Metabolic phenotypes, β-cell secretory function, and glucose and insulin tolerance were measured in RGS2 knockout (RGS2(-/-)) mice and their wild-type (RGS2(+/+)) littermate controls. β-Cell death was evaluated in RGS2-knockdown and -overexpressing β cells and RGS2(-/-) islets by flow cytometry, western blot, ELISA, TUNEL staining, and apoptosis RT(2) profiler PCR array analysis...
May 25, 2017: Cell Death & Disease
https://www.readbyqxmd.com/read/28541303/corrigendum-the-core-pattern-analysis-on-chinese-herbal-medicine-for-sj%C3%A3-gren-s-syndrome-a-nationwide-population-based-study
#8
Ching-Mao Chang, Hsueh-Ting Chu, Yau-Huei Wei, Fang-Pey Chen, Shengwen Wang, Po-Chang Wu, Hung-Rong Yen, Tzeng-Ji Chen, Hen-Hong Chang
This corrects the article DOI: 10.1038/srep09541.
May 25, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28541294/connective-tissue-diseases-mycobacterial-infection-and-sj%C3%A3-gren-syndrome
#9
Dario Ummarino
No abstract text is available yet for this article.
May 25, 2017: Nature Reviews. Rheumatology
https://www.readbyqxmd.com/read/28541269/immediate-loading-of-tantalum-based-implants-in-fresh-extraction-sockets-in-patient-with-sjogren-syndrome-a-case-report-and-literature-review
#10
Cristian Peron, Fawad Javed, Georgios E Romanos
PURPOSE: The objective of this article is to demonstrate treatment of a clinical case using implants in conjunction with immediate loading in a patient with Sjogren syndrome (SS) and to present the current status of knowledge about this type of patients with dental implants. MATERIALS AND METHODS: This article describes a 62-year-old woman patient with SS and partially edentulous maxilla who was rehabilitated with 5 immediately loaded tantalum-based dental implants (TBDIs) placed in fresh extraction sockets...
May 22, 2017: Implant Dentistry
https://www.readbyqxmd.com/read/28538612/scleral-contact-lenses-in-an-academic-oculoplastics-clinic-epidemiology-and-emerging-considerations
#11
Harinder S Chahal, Marcela Estrada, Christine W Sindt, Jacob A Boehme, Mark A Greiner, Jeffrey A Nerad, Keith D Carter, Richard C Allen, Erin M Shriver
PURPOSE: To describe the role and efficacy of scleral contact lenses (SCLs) in the treatment of progressive keratopathy in patients who have undergone periocular surgical procedures, to investigate the financial impact of these surgical interventions, and to demonstrate the role of oculoplastic surgery in improving scleral contact lens fit. METHODS: A retrospective medical record review was performed to identify patients who both received SCLs and were examined by the oculoplastics service at the University of Iowa between January 1990 and December 2015...
May 22, 2017: Ophthalmic Plastic and Reconstructive Surgery
https://www.readbyqxmd.com/read/28537471/characteristics-of-labial-gland-mesenchymal-stem-cells-of-healthy-individuals-and-patients-with-sj%C3%A3-gren-syndrome-a-preliminary-study
#12
Shiqin Wang, Hong Hua, Yixiang Wang
Sjögren's Syndrome (SS) is a systemic autoimmune disease characterized by focal lymphocytic infiltration into exocrine organs such as salivary and lacrimal glands, resulting in dry mouth and eyes, and other systemic injuries. The mesenchymal stem cells (MSCs) in the salivary glands of healthy individuals and patients with SS have not been extensively studied. The aim of this study was to eluciadate the characteristics of MSCs from the labial glands of healthy controls and of those from patients with SS in order to elucidate the related pathogenesis and uncover potential avenues for novel clinical interventions...
May 24, 2017: Stem Cells and Development
https://www.readbyqxmd.com/read/28535892/are-the-women-with-sj%C3%A3-gren-s-syndrome-satisfied-with-their-sexual-activity
#13
Hatice Isik, Metin Isik, Oner Aynioglu, Deniz Karcaaltincaba, Ahmet Sahbaz, Tugba Beyazcicek, Mehmet Ibrahim Harma, Nejat Demircan
OBJECTIVE: Females with Sjögren's Syndrome (SS) often experience vaginal dryness and dyspareunia, along with glandular and extraglandular symptoms. We aimed to evaluate sexual function and life quality in women with SS. METHODS: Forty-six premenopausal women with SS and 47 age-matched controls were studied. Age, duration of the disease, medications, and comorbid diseases were noted. Participants completed 36-Item Short Form Health Survey (SF-36) and Female Sexual Function Index (FSFI)...
May 2017: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/28534947/research-and-progress-on-clc%C3%A2-2-review
#14
Hongwei Wang, Minghui Xu, Qingjie Kong, Peng Sun, Fengyun Yan, Wenying Tian, Xin Wang
Chloride channel 2 (ClC-2) is one of the nine mammalian members of the ClC family. The present review discusses the molecular properties of ClC‑2, including CLCN2, ClC‑2 promoter and the structural properties of ClC‑2 protein; physiological properties; functional properties, including the regulation of cell volume. The effects of ClC‑2 on the digestive, respiratory, circulatory, nervous and optical systems are also discussed, in addition to the mechanisms involved in the regulation of ClC‑2. The review then discusses the diseases associated with ClC‑2, including degeneration of the retina, Sjögren's syndrome, age‑related cataracts, degeneration of the testes, azoospermia, lung cancer, constipation, repair of impaired intestinal mucosa barrier, leukemia, cystic fibrosis, leukoencephalopathy, epilepsy and diabetes mellitus...
May 18, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28533099/surgical-aortic-valve-replacement-following-early-sapien-xt-valve-failure-a-first
#15
Tanveer Ahmad, Prakash Ludhani, Ronen Gurvitch, John Goldblatt, James Tatoulis
BACKGROUND: Early degeneration of prosthetic aortic valve in transcatheter aortic valve replacement (TAVR) is a rare complication. METHOD: We report the case of a 75-year-old woman who presented with severe calcific stenosis of Edwards SAPIEN-XT valve implanted only four years previously. She is a Jehovah's Witness and has background of Sjogren's syndrome with secondary cryoglobulinaemic vasculitis. She was not a suitable candidate for valve-in-valve TAVR in view of early prosthetic valve failure by calcification...
May 9, 2017: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/28532640/ajkd-atlas-of-renal-pathology-kidney-disease-in-primary-sj%C3%A3-gren-syndrome
#16
Agnes B Fogo, Mark A Lusco, Behzad Najafian, Charles E Alpers
No abstract text is available yet for this article.
June 2017: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/28532233/updates-on-sj%C3%A3-gren-s-syndrome-from-proteomics-to-protein-biomarkers
#17
Chiara Baldini, Antonella Cecchettini, Alessia Gallo, Stefano Bombardieri
Primary Sjögren's syndrome (pSS) is a complex heterogeneous autoimmune disorder, typically affecting exocrine glands. Recently, a great interest has arisen in searching for novel biomarkers able to improve the diagnostic work-up of the disease as well as the general assessment and the prognostic stratification of pSS patients. From this perspective, salivary proteomics has appeared as a promising tool considering that salivary proteins may closely reflect the underlying disease processes in the salivary glands...
May 26, 2017: Expert Review of Proteomics
https://www.readbyqxmd.com/read/28532196/fetal-and-neonatal-involvement-in-maternal-rheumatologic-disease
#18
Shigeo Iijima
A pregnancy complicated with rheumatologic diseases can have various influences on the fetus and/or neonate. Maternal systemic lupus erythematosus (SLE) may cause preterm and/or small for gestational age (SGA) delivery and neonatal lupus (NL). Some neonates with NL have congenital heart block (CHB) with increased morbidity and mortality, even requiring pacemakers. Antiphospholipid syndrome may occur with SLE and affect fetal and/or neonatal outcomes. Pregnancy involving primary Sjögren syndrome (pSS) tends to result in preterm delivery and low birthweight infants...
May 22, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28528521/myeloid-populations-in-systemic-autoimmune-diseases
#19
REVIEW
María Morell, Nieves Varela, Concepción Marañón
Systemic autoimmune diseases (SADs) encompass a wide spectrum of clinical signs as a reflection of their complex physiopathology. A variety of mechanisms related with the innate immune system are in the origin of the loss of self-tolerance in these diseases, and for most of them, the myeloid leukocytes are key actors. Monocytes, macrophages, dendritic cells, and neutrophils are first-line immune effectors located in the interface between innate and adaptive immunity. They are crucial in the organization of the local and systemic responses to damage-associated molecular patterns (DAMPs) and determine the intensity, orientation, and duration of the local immune response through the expression of chemokines, costimulatory or protolerogenic factors...
May 20, 2017: Clinical Reviews in Allergy & Immunology
https://www.readbyqxmd.com/read/28528377/correlation-between-corneal-innervation-and-inflammation-evaluated-with-confocal-microscopy-and-symptomatology-in-patients-with-dry-eye-syndromes-a-preliminary-study
#20
Tudor C Tepelus, Gloria B Chiu, Jianyan Huang, Ping Huang, SriniVas R Sadda, John Irvine, Olivia L Lee
PURPOSE: To evaluate corneal innervation and inflammatory cell infiltration using in vivo confocal microscopy (IVCM) and to correlate these findings with subjective symptoms of dry eye, as measured by the Ocular Surface Disease Index (OSDI) in patients with non-Sjögren's (NSDE) and Sjögren's syndrome dry eyes (SSDE). METHODS: Central corneal images were prospectively captured from 10 age-matched healthy control eyes, 24 eyes with clinically diagnosed NSDE and 44 eyes with clinically diagnosed SSDE, using IVCM (HRT III RCM)...
May 20, 2017: Graefe's Archive for Clinical and Experimental Ophthalmology
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