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Connective tissue disorder

Hai-Bo Ding, Kai-Xiong Liu, Jie-Feng Huang, Da-Wen Wu, Jun-Ying Chen, Qing-Shi Chen
BACKGROUND: Chronic obstructive pulmonary disease (COPD) is a multicomponent disorder characterized by inflammation, representing a significant leading cause of chronic morbidity and mortality. Reports have implicated hydrogen sulfide (H2 S) in both the pathology and treatment of COPD. The present study aimed to explore the effects involved with exogenous H2 S on endoplasmic reticulum stress (ERS) and pulmonary artery endothelial cells (PAECs) in a rat model of COPD. METHODS: Rat models of COPD were successfully established by means of passive smoke exposure and intratracheal injection with lipopolysaccharide (LPS)...
June 13, 2018: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
Pio Conti, Alessandro Caraffa, Gianpaolo Ronconi, Chiara M Conti, Spiros K Kritas, Filiberto Mastrangelo, Lucia Tettamanti, Theoharis C Theoharides
The purpose of this article is to study the involvement of inflammatory mast cells (MCs) in depression which may be inhibited by IL-37. We evaluate mast cells in depression on the basis of our previous experimental data, and using the most relevant studies reported in the literature. Dysfunction of mood, feelings, and thoughts is a major risk factor for several metabolic diseases and may influence the physiology of the body leading to depression. Depression, present in mastocytosis, is an important endogenous process that promotes the activation of meningeal cell receptors through a low-grade neurogenic chronic inflammation, and MCs...
June 16, 2018: Immunologic Research
Aafke Engwerda, Barbara Frentz, A Lya den Ouden, Boudien C T Flapper, Morris A Swertz, Erica H Gerkes, Mirjam Plantinga, Trijnie Dijkhuizen, Conny M A van Ravenswaaij-Arts
Proximal 6q (6q11-q15) deletions are extremely rare and little is known about their phenotypic consequences. Since parents and caregivers now use social media to seek information on rare disorders, the Chromosome 6 Project has successfully collaborated with a Facebook group to collect data on individuals worldwide. Here we describe a cohort of 20 newly identified individuals and 25 literature cases with a proximal 6q deletion. Microarray results and phenotype data were reported directly by parents via a multilingual online questionnaire...
June 8, 2018: European Journal of Human Genetics: EJHG
Yong-Zhi Zhai, Xin Chen, Xin Liu, Zhi-Qiang Zhang, Hong-Ju Xiao, Gang Liu
Fever of unknown origin (FUO) is a frequently observed phenomenon in clinical practice. The present study was aimed to investigate potential causes of FUO, thereby improving clinical diagnosis of this disorder.In this retrospective study, clinical data were collected from 215 patients who were diagnosed with FUO between January 2009 and December 2010, and an 18 to 36 months follow-up visit was also performed for these patients.Among these FUO cases, the most common causes of the disease were infectious diseases (IDs) (42...
June 2018: Medicine (Baltimore)
Gehan Botrus, Omar Baker, Erica Borrego, Kyari Sumayin Ngamdu, Mohamed Teleb, Jose L Gonzales Martinez, Gerardo Maldonado, Ahmed M Hussein, Richard McCallum
Joint hypermobility is a common, primarily benign finding in the general population. However, in a subset of individuals joint hypermobility causes a range of clinical problems mainly affecting the musculoskeletal system and, to a lesser extent, extra-articular disorders. Joint hypermobility often appears as a familial trait and is shared by several inherited connective tissue disorders, including the hypermobility subtype of Ehlers-Danlos syndrome (hEDS) and benign joint hypermobility syndrome (BJHS/JHS). Although joint hypermobility has primarily been thought of as a rheumatological disorder, increasing evidence shows significant associations between both hEDS and BJHS with specific extra-articular disorders...
June 2018: American Journal of the Medical Sciences
Katrien Van Raemdonck, Sadiq Umar, Zoltán Szekanecz, Ryan K Zomorrodi, Shiva Shahrara
Obesity can instigate and sustain a systemic low-grade inflammatory environment that can amplify autoimmune disorders and their associated comorbidities. Metabolic changes and inflammatory factors produced by the adipose tissue have been reported to aggravate autoimmunity and predispose the patient to cardiovascular disease (CVD) and metabolic comorbidities. Rheumatoid arthritis (RA) and psoriatic arthritis (PsA) are autoimmune arthritic diseases, often linked with altered body mass index (BMI). Severe joint inflammation and bone destruction have a debilitating impact on the patient's life; there is also a staggering risk of cardiovascular morbidity and mortality...
June 6, 2018: Autoimmunity Reviews
Tong Zou, Mei Zhu, Yi-Cheng Ma, Fei Xiao, Xue Yu, Li Xu, Lan-Qing Ma, Jiefu Yang, Jian-Zeng Dong
Metabolic disorders, such as obesity and type 2 diabetes, are associated with an increased risk of cardiomyopathy. To date, microRNA (miRNAs) functions in cardiac remodeling induced by obesity remain to be elucidated. We found that rats fed a high fat diet (HFD) manifested cardiac fibrosis and LV dysfunction. In the heart of rats fed HFD, the phosphorylation levels of Smad 2 and the expression of fibrotic genes, such as connective tissue growth factor, collagen-1α1 (Col1α1), Col3α1, and Col4α1, were up-regulated, which accompanied by an increase in Smad 7 protein levels, but not its mRNA levels...
June 8, 2018: Scientific Reports
Matthias C Wurm, Tessa K Behrends, Wolfgang Wüst, Marco Wiesmüller, Andre Wilkerling, Friedrich W Neukam, Tilo Schlittenbauer
INTRODUCTION: Magnetic resonance imaging has been established as the gold standard for assessment of the temporomandibular joint. Apart from an excellent assessment of the soft tissues it has the advantage not to expose the patient to ionizing radiation. There is a lack of literature concerning the correlation between pain intensity and radiological findings of the temporomandibular joint. Moreover there is the question of whether a progressive degeneration of the cartilaginous components is accompanied by an increasing degeneration of the osseous parts of the mandibular joint and vice versa...
January 4, 2018: Journal of Cranio-maxillo-facial Surgery
Karin A Jansen, Albert J Licup, Abhinav Sharma, Robbie Rens, Fred C MacKintosh, Gijsje H Koenderink
Collagen forms fibrous networks that reinforce tissues and provide an extracellular matrix for cells. These networks exhibit remarkable strain-stiffening properties that tailor the mechanical functions of tissues and regulate cell behavior. Recent models explain this nonlinear behavior as an intrinsic feature of disordered networks of stiff fibers. Here, we experimentally validate this theoretical framework by measuring the elastic properties of collagen networks over a wide range of self-assembly conditions...
June 5, 2018: Biophysical Journal
Mark D P Davis, Jeroen C H van der Hilst
A wide differential diagnosis must be considered in a patient presenting with urticarial plaques. Although acute and chronic urticaria are the commonest diagnoses, other differential diagnoses include polymorphous eruption of pregnancy, mast cell disorders, hypereosinophilic syndrome, urticarial vasculitis, pemphigoid, systemic lupus erythematosus, and autoinflammatory disease. This review will specifically address urticarial vasculitis and autoinflammatory syndromes. These entities represent contrasting examples of urticarial-like lesions resulting from either an adaptive immune complex-mediated mechanism (urticarial vasculitis) or an innate immune-mediated mechanism (autoinflammatory disorders), with differing therapeutic implications...
June 2, 2018: Journal of Allergy and Clinical Immunology in Practice
Cortney A Turner, Vikram Sharma, Megan H Hagenauer, Sraboni Chaudhury, Angela M O'Connor, Elaine K Hebda-Bauer, Robert C Thompson, Richard M Myers, William E Bunney, Jack D Barchas, Francis S Lee, Alan F Schatzberg, Stanley J Watson, Huda Akil
BACKGROUND: While downregulation of several growth factors in major depressive disorder is well established, less attention has been paid to the upregulation of other growth factors. Yet, upregulated growth factors may offer better therapeutic targets. We show that connective tissue growth factor (CTGF) represents a target based on its upregulation in major depressive disorder and studies in animal models implicating it in negative affect. METHODS: CTGF gene expression was first evaluated in the postmortem human amygdala...
May 3, 2018: Biological Psychiatry
Fabio Da Silva, Filippo Massa, Fariba Jian Motamedi, Valerie Vidal, Ana Sofia Rocha, Elodie P Gregoire, Chen-Leng Cai, Kay Dietrich Wagner, Andreas Schedl
Coronary artery anomalies are common congenital disorders with serious consequences in adult life. Coronary circulation begins when the coronary stems form connections between the aorta and the developing vascular plexus. We recently identified the WNT signaling modulator R-spondin 3 (Rspo3), as a crucial regulator of coronary stem proliferation. Using expression analysis and tissue-specific deletion we now demonstrate that Rspo3 is primarily produced by cardiomyocytes. Moreover, we have employed CRISPR/Cas9 technology to generate novel Lgr4-null alleles that showed a significant decrease in coronary stem proliferation and thus phenocopied the coronary artery defects seen in Rspo3 mutants...
May 31, 2018: Developmental Biology
Niele D Mendes, Artur Fernandes, Glaucia M Almeida, Luis E Santos, Maria Clara Selles, Natalia Lyra-Silva, Carla M Machado, José A C Horta-Júnior, Paulo R Louzada, Fernanda G De Felice, Soniza Alvez-Leon, Jorge Marcondes, João Alberto Assirati, Caio M Matias, William L Klein, Norberto Garcia-Cairasco, Sergio T Ferreira, Luciano Neder, Adriano Sebollela
BACKGROUND: Slice cultures have been prepared from several organs. With respect to the brain, advantages of slice cultures over dissociated cell cultures include maintenance of the cytoarchitecture and neuronal connectivity. Slice cultures from adult human brain have been reported and constitute a promising method to study neurological diseases. Despite this potential, few studies have characterized in detail cell survival and function along time in short-term, free-floating cultures...
May 31, 2018: Journal of Neuroscience Methods
Daniel Pinggera, Ondra Petr, Gabriel Putzer, Claudius Thomé
Multimodal neuromonitoring has become a fundamental part of management for many neurosurgical disorders such as subarachnoid hemorrhage and severe traumatic brain injury. Obviously, brain tissue oxygen tension monitoring requires insertion of a probe into the brain parenchyma through a single multiple lumen bolt, or in a subcutaneously tunneled fashion. As those patients often require early magnetic resonance imaging(MRI), typically used bolts are disadvantageous due to massive metal artifact. Similarly, subcutaneous tunneling is often problematic as suture fixation can loosen over time...
May 30, 2018: World Neurosurgery
Clotilde Chatre, François Roubille, Hélène Vernhet, Christian Jorgensen, Yves-Marie Pers
INTRODUCTION: Chloroquine and hydroxychloroquine are widely used in the long-term treatment of connective tissue disease and usually considered safe. However, chloroquine- or hydroxychloroquine-related cardiac disorder is a rare but severe adverse event, which can lead to death. This systematic review investigates cardiac complications attributed to chloroquine and hydroxychloroquine. METHODS: PubMED, EMBASE, and Cochrane database searches were conducted using keywords derived from MeSH terms...
June 1, 2018: Drug Safety: An International Journal of Medical Toxicology and Drug Experience
Yueli Wang, Xiaoyan Li, Rongjuan Li, Ya Yang, Jie Du
Marfan syndrome (MFS) is an autosomal dominant genetic disorder of the connective tissue, typically characteristic of cardiovascular manifestations, valve prolapse, left ventricle enlargement, and cardiac failure. Fibrillin-1 (FBN1 ) is the causative gene in the pathogenesis of MFS. Patients with different FBN1 mutations often present more considerable phenotypic variation. In the present study, three affected MFS pedigrees were collected for genetic analysis. Using next-generation sequencing (NGS) technologies, 3 novel frameshift pathogenic mutations which are cosegregated with affected subjects in 3 pedigrees were identified...
2018: International Journal of Genomics
Evan J Zucker
A variety of syndromes are associated with thoracoabdominal aortic pathologies. While these diseases are collectively rare, the presence of advanced or unusual aortic disease at a young age should raise suspicion of an underlying syndrome. Similarly, patients with a known syndrome require close monitoring in anticipation of future aortic disease. In this article, the syndromes most commonly encountered in clinical practice are reviewed, including Marfan syndrome (MFS) and other connective tissue disorders, Turner syndrome (TS), autosomal dominant polycystic kidney disease (ADPKD), neurofibromatosis (NF), Williams syndrome (WS), Alagille syndrome (AGS), and DiGeorge syndrome (DGS)...
April 2018: Cardiovascular Diagnosis and Therapy
Malgorzata Pihut, Piotr Ceranowicz, Andrzej Gala
Temporomandibular joint dysfunction is a functional disorder concerned with the abnormal functioning of the muscles of the stomatognathic system and temporomandibular joints involved in the dynamic movements of the jaw and surrounding structures. The aim of the study was to compare the level of C-reactive protein in patients with pain and painless forms of temporomandibular joint dysfunction. Materials and methods . The study group consisted of 72 patients who reported to the prosthetic treatment because of temporomandibular joint dysfunction...
2018: Pain Research & Management: the Journal of the Canadian Pain Society
Jonathan T Lau, John S Hunt, David I Bruner, Andrea L Austin
Cervical artery dissection is a common cause of stroke in young adults. This may result from head and neck trauma; it can also occur spontaneously or secondary to genetic connective tissue or vascular disorders. Neurologic symptoms arise as a result of thromboembolism and hypoperfusion causing cerebral ischemia. We present a case of a previously healthy male who was found to have a cervical internal carotid artery dissection and the decision to use antiplatelet therapy instead of anticoagulation to prevent stroke...
August 2017: Clinical practice and cases in emergency medicine
Jelena Todic, Valérie Schweizer, Igor Leuchter
OBJECTIVE: Bamboo nodes are vocal fold lesions, mostly associated with autoimmune diseases. PATIENTS AND METHODS: This is a retrospective clinical study including 10 patients with bamboo nodes. Data were collected regarding associated autoimmune disorder and type of treatment. A systematic review of the literature was conducted. RESULTS: All patients were women, with hoarseness as the most frequent symptom. There was in most cases an associated autoimmune disease: 3 patients with systemic lupus erythematosus; 3 with rheumatoid arthritis; 1 with Sjögren syndrome; 1 with Hashimoto disease; and 1 with mixed connective tissue disease...
May 30, 2018: Folia Phoniatrica et Logopaedica
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