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Connective tissue disorder

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https://www.readbyqxmd.com/read/29045979/-changes-of-serum-krebs-von-den-lungen-6-levels-in-interstitial-lung-disease-associated-with-dermatomyositis-and-secondary-sj%C3%A3-gren-s-syndrome-a-case-report
#1
J F Yu, Y B Jin, J He, Y An, Z G Li
Interstitial lung diseases (ILDs) are a diverse group of pulmonary disorders characterized by various patterns of inflammation and fibrosis in the interstitium of the lung. The underlying pathogenesis of ILDs is complex and associated with multiple rheumatologic conditions, such as systemic sclerosis, rheumatoid arthritis, pollymyositis and dermatomyositis, Sjögren's syndrome, and systemic lupus erythematosus. As the disease progresses, excessive pulmonary fibrosis impairs alveolar gas exchange and damages pulmonary function...
October 18, 2017: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
https://www.readbyqxmd.com/read/29035287/cardio-pulmonary-involvement-in-systemic-sclerosis-a-study-at-a-tertiary-care-center
#2
Geetakiran Arakkal, Sudha Rani Chintagunta, Vasavilatha Chandika, Sudha Vani Damarla, Srinivas Manchala, B Udaya Kumar
BACKGROUND: Systemic sclerosis is a multisystem disorder characterized by microangiopathy, dysregulation of the immune system and massive deposition of collagen in the connective tissue of the skin, blood vessels and various internal organs. Although the mortality from renal crises has dropped significantly due to the use of angiotensin-converting enzyme inhibitors, cardiac and pulmonary involvement accounts for significant morbidity and mortality. We studied 28 patients with systemic sclerosis at Gandhi Medical College and Hospital, Hyderabad, over a period of two years for cardiopulmonary involvement...
November 2017: Indian Journal of Dermatology, Venereology and Leprology
https://www.readbyqxmd.com/read/29032927/mortality-from-systemic-lupus-erythematosus-in-brazil-evaluation-of-causes-according-to-the-government-health-database
#3
Luisa Ribeiro Costi, Hatsumi Miyashiro Iwamoto, Dilma Costa de Oliveira Neves, Cezar Augusto Muniz Caldas
OBJECTIVE: To characterize the causes of mortality in patients with systemic lupus erythematosus (SLE) in Brazil between 2002 and 2011. METHODS: An exploratory ecological study of a time series using data from the Mortality Information System of DATASUS, the Department of the Unified Health System (Brazil's National Health System). RESULTS: Brazil's SLE mortality rate was 4.76 deaths/10(5) inhabitants. The mortality rate was higher in the Midwest, North and Southeast regions than in the country as a whole...
October 9, 2017: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/29032848/-ehlers-danlos-syndromes
#4
D-P Germain
Ehlers-Danlos syndromes (EDS) are a heterogeneous group of inheritable connective tissue disorders characterized by skin hyperextensibility, joint hypermobility and cutaneous fragility with delayed wound healing. Over and above these common features, they differ in the presence or absence of various organ and tissue abnormalities, and differences in genetic causal mechanisms and degree of severity. They are complex and multisystem diseases, with the majority being highly disabling because of major joint problems and neurosensory deficiencies, and in some cases, they may be life-threatening due to associated complications, especially vascular disorders...
October 9, 2017: Annales de Dermatologie et de Vénéréologie
https://www.readbyqxmd.com/read/29031005/quantitative-high-content-high-throughput-microscopy-analysis-of-lipid-droplets-in-subject-specific-adipogenesis-models
#5
Maxime Bombrun, Hui Gao, Petter Ranefall, Niklas Mejhert, Peter Arner, Carolina Wählby
Neutral lipids packed in lipid droplets (LDs) are essential as a source of fuel for organisms, and specialized storing cells, the adipocytes, provide a buffer for energy variations. Many modern-society-disorders are connected with excess accumulation or deficiency of LDs in adipose tissue. Intracellular LD number and size distribution reflect the tissue conditions, while the associated mechanisms and genes rs are still poorly understood. Large-scale genetic screens using human in vitro differentiated primary adipocytes require cell samples donated from many patients...
October 14, 2017: Cytometry. Part A: the Journal of the International Society for Analytical Cytology
https://www.readbyqxmd.com/read/29025543/pericardial-effusion
#6
REVIEW
Amir Azarbal, Martin M LeWinter
The normal pericardial sac contains up to 50 mL of fluid, which consists of a plasma ultrafiltrate. Anything greater constitutes a pathologic effusion. The curvilinear pressure-volume relationship of the pericardial sac dictates hemodynamic consequences of a pericardial effusion and is responsible for rapidly accumulating fluid that causes cardiac tamponade. A variety of diseases and complications cause pericardial effusion. The most common are idiopathic pericarditis, cancer, connective tissue disorders, and hemorrhage...
November 2017: Cardiology Clinics
https://www.readbyqxmd.com/read/29024514/therapeutic-potential-of-haecs-for-early-achilles-tendon-defect-repair-through-regeneration
#7
Barbara Barboni, Valentina Russo, Valentina Gatta, Nicola Bernabò, Paolo Berardinelli, Annunziata Mauro, Alessandra Martelli, Luca Valbonetti, Aurelio Muttini, Oriana Di Giacinto, Maura Turriani, Antonietta Silini, Giuseppe Calabrese, Michele Abate, Ornella Parolini, Liborio Stuppia, Mauro Mattioli
Cell-based therapy hold great promise for tendon disorders, a widespread debilitating musculoskeletal condition. Even if the cell line remains to be defined, preliminary evidences have proven that amniotic-derived cells possess in vitro and in vivo a great tenogenic potential. The present study investigated the efficacy of transplanted human amniotic epithelial cells (hAECs) by testing their early regenerative properties and mechanisms involved on a validated ovine Achilles tendon partial defect performed on 29 animals...
October 11, 2017: Journal of Tissue Engineering and Regenerative Medicine
https://www.readbyqxmd.com/read/29022822/a-late-presentation-of-loeys-dietz-syndrome-associated-with-an-aortic-root-aneurysm
#8
A Harky, M Garner, N Roberts
Loeys-Dietz syndrome (LDS) is characterised by a mutation in the transforming growth factor beta receptor, and is strongly associated with aortic aneurysms and rupture. Most cases of LDS present in the second decade of life, with the average life expectancy being 37 years. We report a case of suspected LDS (owing to significant family history of the disorder) that presented in the seventh decade of life. Our patient had aortic root dilation and was initially believed to have Marfan syndrome. However, subsequent tests were negative...
February 13, 2017: Annals of the Royal College of Surgeons of England
https://www.readbyqxmd.com/read/29022136/aortic-carotid-intima-media-thickness-and-flow-mediated-dilation-as-markers-of-early-atherosclerosis-in-a-cohort-of-pediatric-patients-with-rheumatic-diseases
#9
Emanuela Del Giudice, Anna Dilillo, Luciana Tromba, Giuseppe La Torre, Sara Blasi, Fabrizio Conti, Franca Viola, Salvatore Cucchiara, Marzia Duse
The aims of this study were to identify the presence of endothelial dysfunction as a marker of early atherosclerosis by measuring aortic and carotid intimal-medial thickness (aIMT and cIMT) and flow-mediated dilation (FMD) and their correlation with traditional and no traditional risk factors for atherosclerosis in children with rheumatic diseases. Thirty-nine patients (mean age 15.3 ± 5.7 years), 23 juvenile idiopathic arthritis, 9 juvenile spondyloarthropathies, 7 connective tissue diseases (mean disease duration and onset respectively 5 ± 3...
October 12, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28994317/isolated-cervical-anterior-spinal-artery-aneurysm-case-report
#10
Yam Bahadur Roka
Isolated cervical anterior spinal artery (CASA) aneurysms are extremely rare with most of them mostly associated with arteriovenous malformation. The underlying pathology is not known but some factors leading to the aneurysm are inflammatory, infection and connective tissue disorders. Trauma with formation of pseudoaneurysm has also been reported. We report a case which presented with fourth ventricle bleed and was managed successfully with conservative treatment.
October 10, 2017: British Journal of Neurosurgery
https://www.readbyqxmd.com/read/28991210/a-central-bioactive-region-of-ltbp-2-stimulates-the-expression-of-tgf-%C3%AE-1-in-fibroblasts-via-akt-and-p38-signalling-pathways
#11
Mohamed A Sideek, Joshua Smith, Clementine Menz, Julian R J Adams, Allison J Cowin, Mark A Gibson
Latent transforming growth factor-β-1 binding protein-2 (LTBP-2) belongs to the LTBP-fibrillin superfamily of extracellular proteins. Unlike other LTBPs, LTBP-2 does not covalently bind transforming growth factor-β1 (TGF-β1) but appears to be implicated in the regulation of TGF-β1 bioactivity, although the mechanisms are largely unknown. In experiments originally designed to study the displacement of latent TGF-β1 complexes from matrix storage, we found that the addition of exogenous LTBP-2 to cultured human MSU-1...
October 9, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28990742/autoimmune-endocrine-diseases
#12
Rosaria M Ruggeri, Giuseppe Giuffrida, Alfredo Campennì
The endocrine system is interested by several autoimmune diseases, characterized by different impact and severity, according to the organs involved. Autoimmune thyroid disorders (i.e. Hashimoto's thyroiditis and Graves' disease) and type 1 diabetes mellitus are the most common autoimmune endocrine disorders, while hypophysitis, adrenalitis (90% of cases of primary hypocortisolism or Addison's disease), premature ovarian failure and hypoparathyroidism represent quite rare conditions. Autoimmune endocrine diseases can also coexist in the same individuals and cluster in families...
October 9, 2017: Minerva Endocrinologica
https://www.readbyqxmd.com/read/28990520/collagenolytic-enzymes-and-their-applications-in-biomedicine
#13
Anatoly Borisovich Shekhter, Anastasia V Balakireva, Natalia V Kuznetsova, Marina N Vukolova, Petr F Litvitsky, Andrey A Zamyatnin
Nowadays, enzymatic therapy is a very promising line of treatment for many different diseases. There is a group of disorders and conditions, caused by fibrotic and scar processes and associated with the excessive accumulation of collagen that needs to be catabolized to normalize the connective tissue content. The human body normally synthesizes special extracellular enzymes, matrix metalloproteases (MMPs) by itself. These enzymes can cleave components of extracellular matrix (ECM) and different types of collagen and thus maintain the balance of the connective tissue components...
October 6, 2017: Current Medicinal Chemistry
https://www.readbyqxmd.com/read/28990148/athletes-and-the-aorta-normal-adaptations-and-the-diagnosis-and-management-of-pathology
#14
REVIEW
J Stephen Hedley, Dermot Phelan
Over a hundred years ago, physicians first recognized that participation in regular, vigorous training resulted in enlargement of the heart. Since that time, the term "athlete's heart" has entered the medical lexicon as a global expression encompassing the electrical, functional, and morphological adaptations that develop in response to physical training. Exercise-induced adaptations of the aorta, which is also exposed to large hemodynamic stresses during prolonged endurance exercise or resistance training, are less well recognized...
October 9, 2017: Current Treatment Options in Cardiovascular Medicine
https://www.readbyqxmd.com/read/28987432/abdominal-aortic-emergencies
#15
REVIEW
Christie Lech, Anand Swaminathan
This article discusses abdominal aortic emergencies. There is a common thread of risk factors and causes of these diseases, including age, male gender, hypertension, dyslipidemia, and connective tissue disorders. The most common presenting symptom of these disorders is pain, usually in the chest, flank, abdomen, or back. Computed tomography scan is the gold standard for diagnosis of pathologic conditions of the aorta in the hemodynamically stable patient. Treatment consists of a combination of blood pressure and heart rate control and, in many cases, emergent surgical intervention...
November 2017: Emergency Medicine Clinics of North America
https://www.readbyqxmd.com/read/28986986/depletion-of-mast-cells-and-macrophages-impairs-heterotopic-ossification-in-an-acvr1-r206h-mouse-model-of-fibrodysplasia-ossificans-progressiva
#16
Michael R Convente, Salin A Chakkalakal, EnJun Yang, Robert J Caron, Deyu Zhang, Taku Kambayashi, Frederick S Kaplan, Eileen M Shore
Heterotopic ossification (HO) is a clinical condition that often reduces mobility and diminishes quality of life for affected individuals. The most severe form of progressive HO occurs in those with fibrodysplasia ossificans progressiva (FOP; OMIM #135100), a genetic disorder caused by a recurrent heterozygous gain-of-function mutation (R206H) in the bone morphogenetic protein (BMP) type I receptor ACVR1/ALK2. In individuals with FOP, episodes of HO frequently follow injury. The first sign of active disease is commonly an inflammatory "flare-up" that precedes connective tissue degradation, progenitor cell recruitment, and endochondral HO...
October 7, 2017: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
https://www.readbyqxmd.com/read/28984114/a-novel-de-novo-col6a1-mutation-emphasizes-the-role-of-intron-14-donor-splice-site-defects-as-a-cause-of-moderate-progressive-form-of-colvi-myopathy-a-case-report-and-review-of-the-genotype-phenotype-correlation
#17
Agnieszka A Koppolu, Agnieszka Madej-Pilarczyk, Małgorzata Rydzanicz, Joanna Kosińska, Piotr Gasperowicz, Jolanta Dorszewska, Wojciech Kozubski, Barbara Steinborn, Andrzej M Kochański, Rafał Płoski
Collagen VI-related myopathy is a group of disorders affecting skeletal muscles and connective tissue. The most common symptoms are muscle weakness and joint deformities which limit the movement and progress over time. Several forms of collagen VI-related myopathies have been described: Bethlem myopathy, an intermediate form and Ullrich congenital muscular dystrophy, which is the most severe. Here we report a novel de novo c.1056+3A>C substitution in intron 14 of the COL6A1 gene encoding alpha-chains of collagen VI in a 13-year-old girl suffering from collagen VI (ColVI) myopathy...
2017: Folia Neuropathologica
https://www.readbyqxmd.com/read/28983407/novel-and-recurrent-col11a1-and-col2a1-mutations-in-the-marshall-stickler-syndrome-spectrum
#18
Long Guo, Nursel H Elcioglu, Zheng Wang, Yasemin K Demirkol, Pinar Isguven, Naomichi Matsumoto, Gen Nishimura, Noriko Miyake, Shiro Ikegawa
Marshall-Stickler syndrome represents a spectrum of inherited connective tissue disorders affecting the ocular, auditory, and skeletal systems. The syndrome is caused by mutations in the COL2A1, COL11A1, COL11A2, COL9A1, and COL9A2 genes. In this study, we examined four Turkish families with Marshall-Stickler syndrome using whole-exome sequencing and identified one COL2A1 mutation and three COL11A1 mutations. Two of the COL11A1 mutations were novel. Our findings expand our knowledge of the COL11A1 mutational spectrum that causes Marshall-Stickler syndrome...
2017: Human Genome Variation
https://www.readbyqxmd.com/read/28980852/mast-cell-mediators-inhibit-osteoblastic-differentiation-and-extracellular-matrix-mineralization
#19
William Marcatti Amarú Maximiano, Elaine Zayas Marcelino da Silva, Ana Carolina Santana, Paulo Tambasco de Oliveira, Maria Célia Jamur, Constance Oliver
Mast cells are multifunctional immune cells that participate in many important processes such as defense against pathogens, allergic reactions, and tissue repair. These cells perform their functions through the release of a wide variety of mediators. This release occurs mainly through cross-linking IgE (immunoglobulin E) bound to high affinity IgE receptors by multivalent antigens. The abundance of mast cells in connective tissue, surrounding blood vessels, and their involvement in the early stages of bone repair support the possibility of physiological and pathological interactions between mast cells and osteoblasts...
October 1, 2017: Journal of Histochemistry and Cytochemistry: Official Journal of the Histochemistry Society
https://www.readbyqxmd.com/read/28979858/using-humeral-nail-for-surgical-reconstruction-of-femur-in-adolescents-with-osteogenesis-imperfecta
#20
Paphon Sa-Ngasoongsong, Tanyawat Saisongcroh, Chanika Angsanuntsukh, Patarawan Woratanarat, Pornchai Mulpruek
Osteogenesis imperfecta (OI) is a rare inherited connective tissue disorder caused by mutation of collagen which results in a wide spectrum of clinical manifestations including long bone fragility fractures and deformities. While the treatment for these fractures was recommended as using intramedullary fixation for minimizing stress concentration, the selection of the best implant in the adolescent OI patients for the surgical reconstruction of femur was still problematic, due to anatomy distortion and implant availability...
September 18, 2017: World Journal of Orthopedics
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