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Connective tissue disease

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https://www.readbyqxmd.com/read/28544856/recent-advances-in-connective-tissue-disease-related-interstitial-lung-disease
#1
Atsushi Suzuki, Yasuhiro Kondoh, Aryeh Fischer
Interstitial lung disease (ILD) is a common manifestation of connective tissue disease (CTD). Although the majority of patients with CTD-ILD are stable or slowly progressive, a significant group exhibits a more severe and progressive decline. Interstitial pneumonia with autoimmune features (IPAF) describes the subset of patients with interstitial pneumonia who have features suggesting underlying autoimmunity, but whose features fall short of a clear diagnosis of CTD. Areas covered: In this focused review, we discuss recent advances in early detection, prognostic evaluation, and management of autoimmune forms of ILD...
May 25, 2017: Expert Review of Respiratory Medicine
https://www.readbyqxmd.com/read/28541294/connective-tissue-diseases-mycobacterial-infection-and-sj%C3%A3-gren-syndrome
#2
Dario Ummarino
No abstract text is available yet for this article.
May 25, 2017: Nature Reviews. Rheumatology
https://www.readbyqxmd.com/read/28540432/central-overweight-and-obesity-in-polish-schoolchildren-aged-7-18%C3%A2-years-secular-changes-of-waist-circumference-between-1966-and-2012
#3
Agnieszka Suder, Aleksandra Gomula, Slawomir Koziel
We investigated secular trends of body mass index (BMI) and waist circumference (WC) in Polish schoolchildren examined through a period of almost 50 years. Data on height, weight and WC came from four cross-sectional surveys conducted in Poland between 1966 and 2012, covering 34,005 boys and 34,008 girls. Raw data of BMI and WC were standardized for age classes. Statistical analyses included the Kruskal-Willis test and Pearson Chi-square test. BMI and WC increased during the studied period; however, the growth was higher for WC (increase by 0...
May 24, 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/28540270/fgf-23-klotho-and-vitamin-d-levels-in-scleroderma
#4
Ravan Ahmadi, Mehrzad Hajialilo, Amir Ghorbanihaghjo, Ali Mota, Sina Raeisi, Nasrin Bargahi, Mohammad Valilo, Farahnaz Askarian
BACKGROUND: Scleroderma is a chronic connective tissue disease of unknown etiology. Vitamin D and parathyroid hormone (PTH) that play particular functions in calcium and phosphate homeostasis may be involved in the etiology of this disorder. Klotho, the co-receptor of the fibroblast growth factor 23 (FGF-23), can interfere with calcium and phosphate metabolism. The purpose of this study was to evaluate serum Klotho, FGF-23, intact PTH (iPTH) and vitamin D levels in scleroderma patients compared with the healthy controls...
April 2017: Iranian Journal of Public Health
https://www.readbyqxmd.com/read/28539832/recapitulating-and-correcting-marfan-syndrome-in-a-cellular-model
#5
Jung Woo Park, Li Yan, Chris Stoddard, Xiaofang Wang, Zhichao Yue, Leann Crandall, Tiwanna Robinson, Yuxiao Chang, Kyle Denton, Enqin Li, Bin Jiang, Zhenwu Zhang, Kristen Martins-Taylor, Siu-Pok Yee, Hong Nie, Feng Gu, Wei Si, Ting Xie, Lixia Yue, Ren-He Xu
Marfan syndrome (MFS) is a connective tissue disorder caused by mutations in FBN1 gene, which encodes a key extracellular matrix protein FIBRILLIN-1. The haplosufficiency of FBN1 has been implicated in pathogenesis of MFS with manifestations primarily in cardiovascular, muscular, and ocular tissues. Due to limitations in animal models to study the late-onset diseases, human pluripotent stem cells (PSCs) offer a homogeneic tool for dissection of cellular and molecular pathogenic mechanism for MFS in vitro. Here, we first derived induced PSCs (iPSCs) from a MFS patient with a FBN1 mutation and corrected the mutation, thereby generating an isogenic "gain-of-function" control cells for the parental MFS iPSCs...
2017: International Journal of Biological Sciences
https://www.readbyqxmd.com/read/28539683/stubborn-rectal-prolapse-in-systemic-sclerosis
#6
Sven Petersen, Alexander Tobisch, Gero Puhl, Ina Kötter, Uwe Wollina
Systemic sclerosis (SSc) is an autoimmune connective tissue disorder. Anorectal involvement might typically cause fecal incontinence and rarely rectal prolapse. Here we report three female patients, who were admitted with a mean history of 10 years suffering from SSc. All patients presented with the initial symptom of anal incontinence, in all cases this was associated with rectal intussusception or rectal prolapse. The three women faced prolapse recurrence, independent of the initial procedure. After surgical removal of the prolapse, the incontinence remained...
2017: Reumatologia
https://www.readbyqxmd.com/read/28539677/fatigue-and-sleep-quality-in-rheumatoid-arthritis-patients-during-hospital-admission
#7
Paulina Szady, Grażyna Bączyk, Katarzyna Kozłowska
OBJECTIVES: Rheumatoid arthritis (RA) is a systemic disease of connective tissue characterised by chronic course with periods of exacerbation and remission. Even in the early stages of the disease patients report the occurrence of fatigue and sleep disorders. Reduced sleep quality and chronic fatigue are common among patients with rheumatoid arthritis. The aim of the research was to evaluate the severity of fatigue and sleep quality assessment among patients hospitalised with rheumatoid arthritis and to determine the relation between the level of symptoms of fatigue and sleep quality and variables such as: age, gender, disease duration, marital status, applied pharmacological treatment, and pain intensity...
2017: Reumatologia
https://www.readbyqxmd.com/read/28539414/moderately-elevated-homocysteine-does-not-contribute-to-thoracic-aortic-aneurysm-in-mice
#8
Jasmin Roohi, Benjamin Kang, David Bernard, Djahida Bedja, Harry C Dietz, Lawrence C Brody
Background: Moderate hyperhomocysteinemia is an attractive target for intervention because it is present in 5-7% of the population and can be reversed by diet. This approach presupposes that hyperhomocysteinemia is directly involved in the disease process. Epidemiologic studies have indicated that moderately elevated homocysteine may contribute to thoracic aortic aneurysm (TAA) dilatation and dissection in humans. In vitro, elevated homocysteine disrupts the structure and function of extracellular matrix components, suggesting that moderate hyperhomocysteinemia may contribute to the development and/or progression of TAA...
May 24, 2017: Journal of Nutrition
https://www.readbyqxmd.com/read/28537690/effect-of-mitomycin-c-and-triamcinolone-on-preventing-urethral-strictures
#9
Omer Kurt, Fethullah Gevher, Cenk Murat Yazici, Mustafa Erboga, Mucahit Dogru, Cevat Aktas
Urethral stricture is a common disease with high recurrence rate. Several manipulations were defined to prevent the recurrence but the results were disappointing. This study aimed to evaluate the efficacy of triamcinolone and mitomycin-C on urethral stricture formation and their effect on inhibition of urethral fibrosis. A total of 24 New Zealand rabbits were divided into 3 groups. Urethras of rabbits were traumatized with pediatric resectoscope. Resection area was irrigated with 10mL saline, swapped with a cotton wool soaked with 0...
May 23, 2017: International Braz J Urol: Official Journal of the Brazilian Society of Urology
https://www.readbyqxmd.com/read/28537563/anastomotic-leaks-in-gastrointestinal-surgery-and-their-prevention
#10
Tomasz Banasiewicz, Adam Dziki, Paweł Lampe, Zbigniew Lorenc, Marek Szczepkowski, Jacek Zieliński, Grzegorz Wallner
Anastomotic leak in the gastrointestinal tract is one of the most important complications of resection. They are the main cause of reoperation, their occurrence worsens the prognosis of the patient, increasing the proportion of direct mortality, as well as being a significant risk factor for recurrence of cancer. The risk of leaks within the gastrointestinal tract is greatly varied, depending on the location and extent of the resection, but also on patient, disease or a surgical procedure, including surgeon...
April 30, 2017: Polski Przeglad Chirurgiczny
https://www.readbyqxmd.com/read/28535941/-anesthetic-management-of-two-patients-with-alkaptonuric-ochronosis-for-total-knee-arthroplasty
#11
Betul Kozanhan
The current case report describes two cases of alkaptonuric ochronosis for anesthetic management. Alkaptonuria is a rare genetic orphan disease of tyrosine metabolism characterized by an accumulation of homogentisic acid in cartilage and connective tissues. Patients present most commonly for orthopedic joint surgery due to progressive arthropathy that can be misdiagnosed many a times. However respiratory, airway, cardiovascular and genitourinary systems complications can occur with age progressing. Restricted range of motion of cervical spine may lead to difficulty with airway management...
May 20, 2017: Revista Brasileira de Anestesiologia
https://www.readbyqxmd.com/read/28535619/prevalence-and-incidence-of-osteoporotic-fractures-in-patients-on-long-term-glucocorticoid-treatment-for-rheumatic-diseases-the-glucocorticoid-induced-osteoporosis-tool-giotto-study
#12
M Rossini, O Viapiana, M Vitiello, N Malavolta, G La Montagna, S Maddali Bongi, O Di Munno, R Nuti, C U Manzini, C Ferri, L Bogliolo, A Mathieu, F Cantatore, A Del Puente, M Muratore, W Grassi, B Frediani, G Saviola, P Delvino, L Mirone, G Ferraccioli, G Tripi, I Piazza, D Gatti
Osteoporosis and fractures are common and invalidating consequences of chronic glucorticoid (GC) treatment. Reliable information regarding the epidemiology of GC induced osteoporosis (GIOP) comes exclusively from the placebo group of randomized clinical trials while observational studies are generally lacking data on the real prevalence of vertebral fractures, GC dosage and primary diagnosis. The objective of this study was to evaluate the prevalence and incidence of osteoporotic fractures and to identify their major determinants (primary disease, GC dosage, bone mineral density, risk factors, specific treatment for GIOP) in a large cohort of consecutive patients aged >21 years, on chronic treatment with GC (≥5 mg prednisone - PN - equivalent) and attending rheumatology centers located all over Italy...
May 22, 2017: Reumatismo
https://www.readbyqxmd.com/read/28534442/genotype-and-haplotype-analysis-of-abcb1-at-1236-2677-and-3435-among-systemic-sclerosis-patients
#13
Małgorzata Barańska, Mariola Rychlik-Sych, Jadwiga Skrętkowicz, Michał Dudarewicz, Bożena Dziankowska-Bartkowiak, Jacek Owczarek, Elżbieta Waszczykowska
Systemic sclerosis (SSc) belongs to the group of systemic diseases of the connective tissue, which are characterized by a chronic autoimmune inflammatory process. P-glycoprotein, initially associated with the drug resistance in patients with cancer, becomes more and more often a subject of considerations in terms of its significance in the development of illnesses, including autoimmune diseases. The aim of the study was an attempt to answer the question whether there was a relationship between ABCB1 polymorphisms and morbidity of systemic sclerosis in a Polish population...
May 23, 2017: Autoimmunity
https://www.readbyqxmd.com/read/28534076/serum-prolidase-activity-in-systemic-sclerosis
#14
Ahmet Celik, Muhammed Nur Birer, Metin Kilinc
Systemic sclerosis, also known as scleroderma, is a complex systemic inflammatory autoimmune disease that targets the vasculature and connective tissue-producing cells and components of the innate and adaptive immune systems. The disease is characterized by a hardening of the skin and an increased synthesis of collagen . Prolidase is a specific imidodipeptidase involved in collagen degradation. The aim of this study was to search the serum prolidase activity (SPA) in the two subtypes of systemic sclerosis: diffuse and limited cutaneous systemic sclerosis...
May 22, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28532352/understanding-glaucomatous-optic-neuropathy-the-synergy-between-clinical-observation-and-investigation
#15
Harry A Quigley
Glaucoma is a complex disorder of aging defined by the death of retinal ganglion cells and remodeling of connective tissues at the optic nerve head. Intraocular pressure-induced axonal injury at the optic nerve head leads to apoptosis. Loss of retinal ganglion cells follows a slowly progressive sequence. Clinical features of the disease have suggested and corroborated pathological events. The death of retinal ganglion cells causes secondary loss of neurons in the brain, but only as a by-product of injury to the retinal ganglion cells...
October 14, 2016: Annual Review of Vision Science
https://www.readbyqxmd.com/read/28530745/self-assembling-peptide-and-protein-amyloids-from-structure-to-tailored-function-in-nanotechnology
#16
REVIEW
Gang Wei, Zhiqiang Su, Nicholas P Reynolds, Paolo Arosio, Ian W Hamley, Ehud Gazit, Raffaele Mezzenga
Self-assembled peptide and protein amyloid nanostructures have traditionally been considered only as pathological aggregates implicated in human neurodegenerative diseases. In more recent times, these nanostructures have found interesting applications as advanced materials in biomedicine, tissue engineering, renewable energy, environmental science, nanotechnology and material science, to name only a few fields. In all these applications, the final function depends on: (i) the specific mechanisms of protein aggregation, (ii) the hierarchical structure of the protein and peptide amyloids from the atomistic to mesoscopic length scales and (iii) the physical properties of the amyloids in the context of their surrounding environment (biological or artificial)...
May 22, 2017: Chemical Society Reviews
https://www.readbyqxmd.com/read/28529927/staphylococcus-aureus-manipulates-innate-immunity-through-own-and-host-expressed-proteases
#17
REVIEW
Giampiero Pietrocola, Giulia Nobile, Simonetta Rindi, Pietro Speziale
Neutrophils, complement system and skin collectively represent the main elements of the innate immune system, the first line of defense of the host against many common microorganisms. Bacterial pathogens have evolved strategies to counteract all these defense activities. Specifically, Staphylococcus aureus, a major human pathogen, secretes a variety of immune evasion molecules including proteases, which cleave components of the innate immune system or disrupt the integrity of extracellular matrix and intercellular connections of tissues...
2017: Frontiers in Cellular and Infection Microbiology
https://www.readbyqxmd.com/read/28529812/diabetic-muscle-infarction-masquerading-as-necrotizing-fasciitis
#18
Kalyana C Janga, Ankur Sinha, Perry Wengrofsky, Phone Oo, Sheldon Greenberg, Regina Tarkovsky, Kavita Sharma
A 43-year-old male patient with past medical history of diabetes mellitus (DM), end stage renal disease (ESRD) on hemodialysis (HD), congestive heart failure (CHF), obstructive sleep apnea (OSA), and chronic anemia presented with complaints of left thigh pain. A computerized tomogram (CT) of the thigh revealed evidence of edema with no evidence of a focal collection or gas formation noted. The patient's clinical symptoms persisted and he underwent magnetic resonance imaging (MRI) of his thigh which was reported to show small areas of muscle necrosis with fluid collection...
2017: Case Reports in Nephrology
https://www.readbyqxmd.com/read/28529328/perivascular-adipose-tissue-epiphenomenon-or-local-risk-factor
#19
REVIEW
K Schäfer, I Drosos, S Konstantinides
Obesity is associated with an increased cardiovascular risk, but the mechanisms underlying the link between increased body weight and vascular disease are incompletely understood. Over the past 15 years, perivascular adipose tissue has emerged as active component of the vessel wall involved in vascular homeostasis. However, perivascular adipose tissue can adopt detrimental properties under the influence of obesity and other factors and contribute actively to the pathophysiology of cardiovascular disease. Conversely, changes of the vessel wall may negatively affect perivascular adipose tissue qualities...
May 22, 2017: International Journal of Obesity: Journal of the International Association for the Study of Obesity
https://www.readbyqxmd.com/read/28528779/atypical-articular-presentations-in-indian-children-with-rheumatic-fever
#20
Euden Bhutia, Dinesh Kumar, Mohan Kundal, Sunil Kishore, Atul Juneja
BACKGROUND: The objective of the study was to describe the clinical characteristics of atypical articular presentations during the initial outbreak and recurrence in patients with acute rheumatic fever (ARF) in the paediatric age group. METHODS: This was a retrospective, observational study conducted between January 2012 and December 2014 on all suspected cases of acute rheumatic fever (ARF) fulfilling either WHO 2004 or Australian guidelines with atypical articular manifestations ie, presence of at least one of the following features: duration of symptoms more than three weeks; monoarthritis/arthralgia; involvement of small joints of hand and feet and/or cervical spine and/or hip joint; and, not responding to salicylates in one week...
April 24, 2017: Heart, Lung & Circulation
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