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https://www.readbyqxmd.com/read/28534947/research-and-progress-on-clc%C3%A2-2-review
#1
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
#2
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
#3
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
#4
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 23, 2017: Expert Review of Proteomics
https://www.readbyqxmd.com/read/28532196/fetal-and-neonatal-involvement-in-maternal-rheumatologic-disease
#5
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
#6
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
#7
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
https://www.readbyqxmd.com/read/28528128/comparison-of-the-diagnostic-value-of-four-scoring-systems-in-primary-sj%C3%A3-gren-s-syndrome-patients
#8
Xuan Qi, Chao Sun, Yu Tian, Yuxiang Han, Chenxing Peng, Hongtao Jin, Lixia Gao
PURPOSE: We attempted to evaluate the diagnostic value of different salivary gland ultrasonography (SGUS) scoring systems for primary sjögren's syndrome (pSS). METHODS: Total 134 patients with pSS and 109 non-SS sicca controls were included in our study. All the cases were evaluated by four scoring systems (such as 0-16 SGUS, 0-3 SGUS, Parotid glands and Submandibular salivary glands scoring system). The receiver operating characteristic (ROC) analysis was performed for the four scoring systems...
May 17, 2017: Immunology Letters
https://www.readbyqxmd.com/read/28526333/systemic-manifestations-of-primary-sj%C3%A3-gren-s-syndrome-in-the-nod-b10sn-h2-b-j-mouse-model
#9
Jeremy Kiripolsky, Long Shen, Yichen Liang, Alisa Li, Lakshmanan Suresh, Yun Lian, Quan-Zhen Li, Daniel P Gaile, Jill M Kramer
Animal models that recapitulate human disease are crucial for the study of Sjögren's Syndrome (SS). While several SS mouse models exist, there are few primary SS (pSS) models that mimic systemic disease manifestations seen in humans. Similar to pSS patients, NOD.B10Sn-H2(b)/J (NOD.B10) mice develop exocrine gland disease and anti-nuclear autoantibodies. However, the disease kinetics and spectrum of extra-glandular disease remain poorly characterized in this model. Our objective was to characterize local and systemic SS manifestations in depth in NOD...
May 16, 2017: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/28522958/coexistence-of-ulcerative-colitis-and-sj%C3%A3-gren-s-syndrome-in-a-patient-with-takayasu-s-arteritis-and-hashimoto-s-thyroiditis
#10
Hyun Woo Park, Hyun Seok Lee, Sejin Hwang, Han Sol Lee, Han-Ik Bae, Ghilsuk Yoon
A 31-year-old woman with a 15-year history of Takayasu's arteritis (TA) and a 13-year history of Hashimoto's thyroiditis presented with hematochezia. She received a diagnosis of Sjögren's syndrome at 1 month before her visit to Kyungpook National University Medical Center. Her colonoscopic findings were compatible with a diagnosis of ulcerative colitis (UC). She was treated with oral mesalazine, and her hematochezia symptoms subsequently disappeared. The coexistence of UC and TA has been reported; however, reports on the coexistence of UC and Sjögren's syndrome, or of UC and Hashimoto's thyroiditis are rare...
April 2017: Intestinal Research
https://www.readbyqxmd.com/read/28522454/multiomic-disease-signatures-converge-to-cytotoxic-cd8-t-cells-in-primary-sj%C3%A3-gren-s-syndrome
#11
Shinya Tasaki, Katsuya Suzuki, Ayumi Nishikawa, Yoshiaki Kassai, Maiko Takiguchi, Rina Kurisu, Yuumi Okuzono, Takahiro Miyazaki, Masaru Takeshita, Keiko Yoshimoto, Hidekata Yasuoka, Kunihiro Yamaoka, Kazuhiro Ikeura, Kazuyuki Tsunoda, Rimpei Morita, Akihiko Yoshimura, Hiroyoshi Toyoshiba, Tsutomu Takeuchi
OBJECTIVES: Multiomics study was conducted to elucidate the crucial molecular mechanisms of primary Sjögren's syndrome (SS) pathology. METHODS: We generated multiple data set from well-defined patients with SS, which includes whole-blood transcriptomes, serum proteomes and peripheral immunophenotyping. Based on our newly generated data, we performed an extensive bioinformatic investigation. RESULTS: Our integrative analysis identified SS gene signatures (SGS) dysregulated in widespread omics layers, including epigenomes, mRNAs and proteins...
May 18, 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/28522453/classification-and-diagnostic-criteria-in-sj%C3%A3-gren-s-syndrome-a-long-standing-and-still-open-controversy
#12
EDITORIAL
Claudio Vitali, Nicoletta Del Papa
No abstract text is available yet for this article.
May 18, 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/28516978/sialendoscopic-management-of-autoimmune-sialadenitis-a-review-of-literature
#13
A Gallo, S Martellucci, M Fusconi, G Pagliuca, A Greco, A De Virgilio, M De Vincentiis
Autoimmune diseases of major salivary glands include Sjögren's syndrome and a complex of disorders classified as immunoglobulin G4-related diseases. These pathologies are characterised by an autoimmune reaction mediated by T-helper lymphocytes that targets the ducts of exocrine glands in Sjögren's syndrome and glandular parenchyma in immunoglobulin G4-related diseases. Immunoglobulin G4-related diseases represent recently introduced multi-organ diseases that also involve the salivary glands. However, the morbid conditions once known as Mikulicz's disease and Kuttner's tumour were recently considered as two variants of immunoglobulin G4-related diseases affecting the major salivary glands ( immunoglobulin G4-related sialadenitis)...
April 2017: Acta Otorhinolaryngologica Italica
https://www.readbyqxmd.com/read/28516971/salivary-biomarkers-and-proteomics-future-diagnostic-and-clinical-utilities
#14
M Castagnola, E Scarano, G C Passali, I Messana, T Cabras, F Iavarone, G Di Cintio, A Fiorita, E De Corso, G Paludetti
Saliva testing is a non-invasive and inexpensive test that can serve as a source of information useful for diagnosis of disease. As we enter the era of genomic technologies and -omic research, collection of saliva has increased. Recent proteomic platforms have analysed the human salivary proteome and characterised about 3000 differentially expressed proteins and peptides: in saliva, more than 90% of proteins in weight are derived from the secretion of three couples of "major" glands; all the other components are derived from minor glands, gingival crevicular fluid, mucosal exudates and oral microflora...
April 2017: Acta Otorhinolaryngologica Italica
https://www.readbyqxmd.com/read/28516885/rapid3-correlates-with-esspri-and-other-patient-reported-outcomes-in-patients-with-primary-sj%C3%A3-gren-s-syndrome
#15
Marja Pertovaara, Markku Korpela
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May 15, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28515613/secondary-sjogren-s-syndrome-presenting-with-distal-tubular-acidosis-and-quadriparesis
#16
Kartik Munta, Manimala Rao Surath, K Seshikiran
A 52-year-old female patient was admitted to Intensive Care Unit with complaints of quadriparesis. Investigations revealed distal renal tubular acidosis (DRTA) secondary to Sjogren's syndrome with involvement of the parotid and thyroid glands. Laboratory investigations showed hyperchloremic metabolic acidosis and an alkaline urine pH with clinical signs of sicca syndrome. Sjogren's syndrome is associated with DRTA and occurrences of quadriparetic hypokalemia, nephrolithiasis, and osteomalacia can be prevented with early diagnosis and lifelong treatment with potassium and alkali replacement...
April 2017: Indian Journal of Critical Care Medicine
https://www.readbyqxmd.com/read/28508950/malignancy-as-a-comorbidity-in-rheumatic-diseases-a-retrospective-hospital-based-study
#17
Hai-Long Wang, Yong-Ming Zhou, Guang-Zhao Zhu, Zhu Yang, Bao-Jin Hua
Patients with Rheumatic diseases (RDs) are at an increased risk of malignancies compared with the general population. The aim of this study was to examine the relative frequency of several cancers in a single homogeneous cohort of patients with different RDs. Patients diagnosed with rheumatoid arthritis (RA), Ankylosing spondylitis (AS), Sjögren's syndrome (SS), systemic lupus erythematosus (SLE), systemic sclerosis (SSc), dermatomyositis (DM), or polymyositis were included. Out of 3982 adult residents admitted to the division of rheumatology, 61 malignancies were observed...
May 16, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28507729/difference-in-clinical-presentation-between-women-and-men-in-incident-primary-sj%C3%A3-gren-s-syndrome
#18
Jorge I Ramírez Sepúlveda, Marika Kvarnström, Susanna Brauner, Chiara Baldini, Marie Wahren-Herlenius
BACKGROUND: A more severe disease phenotype has been reported in men compared to women in several rheumatic diseases. However, studies have not conclusively established sex-related clinical features in primary Sjögren's syndrome (pSS). In this study, we therefore investigated the clinical presentation of pSS in women and men at diagnosis. METHODS: Incident, treatment naïve patients (n = 199) during a 5-year period in a specified area were prospectively included and examined for items of classification criteria for pSS as well as extraglandular manifestations (EGM)...
2017: Biology of Sex Differences
https://www.readbyqxmd.com/read/28507188/cryoglobulinemia-in-sj%C3%A3-gren-syndrome-a-disease-subset-that-links-higher-systemic-disease-activity-autoimmunity-and-local-b-cell-proliferation-in-mucosa-associated-lymphoid-tissue
#19
Luca Quartuccio, Chiara Baldini, Roberta Priori, Elena Bartoloni, Francesco Carubbi, Alessia Alunno, Saviana Gandolfo, Serena Colafrancesco, Roberto Giacomelli, Roberto Gerli, Guido Valesini, Stefano Bombardieri, Salvatore De Vita
OBJECTIVE: To compare systemic disease activity by validated tools, i.e., the European League Against Rheumatism Sjögren Syndrome Disease Activity Index (ESSDAI) and the Clinical ESSDAI (ClinESSDAI) scores, between primary Sjögren syndrome (pSS) with positive serum cryoglobulins and pSS without serum cryoglobulins. METHODS: There were 825 consecutive patients with pSS who were retrospectively evaluated. RESULTS: The ESSDAI and the ClinESSDAI scores were significantly higher in cryoglobulin-positive patients (p < 0...
May 15, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28503820/the-p2x7-receptor-nlrp3-inflammasome-complex-predicts-the-development-of-non-hodgkin-s-lymphoma-in-sjogren-s-syndrome-a-prospective-observational-single-center-study
#20
Chiara Baldini, Eleonora Santini, Chiara Rossi, Valentina Donati, Anna Solini
BACKGROUND: P2X7 receptor (P2X7R), trigger of acute inflammatory responses via the NLRP3 inflammasome, is hyperfunctioning in patients with Sjogren Syndrome (SS), where it stimulates IL-18 production. Some patients with SS develop a mucosa-associated lymphoid tissue non-Hodgkin's lymphoma (MALT-NHL). OBJECTIVES: To prospectively evaluate the involvement and the putative prognostic role of this inflammatory pathway in the development of MALT-NHL. METHODS: 147 women with SS have been prospectively followed for a mean of 52 months, relating the expression and function of the P2X7R-inflammasome axis in salivary glands and circulating lymphomonocytes to the prognosis and the degree of the disease...
May 15, 2017: Journal of Internal Medicine
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