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Adult onset stills

Holger Kudela, Susanne Drynda, Anke Lux, Gerd Horneff, Joern Kekow
Background: Signs and symptoms establish the diagnosis of adult onset Still's disease (AOSD) as well as of systemic onset juvenile idiopathic arthritis (sJIA). The published data regarding the importance of IL-18 as a marker for diagnosis and disease activity so far are conflicting. The aim of this study was to clarify the role of IL-18 as a diagnostic and disease activity marker in AOSD and sJIA. Methods: Thirty adult patients diagnosed with AOSD and twenty children diagnosed with sJIA were included in the study...
2019: BMC rheumatology
P Ahumada-Pascual, Daniel García Gañán, Yasmina El Berdei Montero, Ana Velasco
Multiple sclerosis is a high-frequency neurological disorder in young adults. Although there are some genetic and environmental factors that have been related to the onset of the disease, these are not still completely understood and nowadays multiple sclerosis can neither be prevented, nor its symptom effectively treated due to disease heterogeneity. For this reason, the search of prognostic factors and new therapeutic compounds for MS has long aroused among clinicians and researchers. Among these therapeutic compounds, GEMSP, which consists of a mixture of functional constituents as fatty acids, antioxidants, free radical scavengers and amino acids linked individually to poly-L-Lysine (PL), is emerging as a promising drug for MS treatment...
March 11, 2019: Current Pharmaceutical Design
Mile Bosilkovski, Marija Dimzova, Marija Cvetkova, Kostadin Poposki, Katerina Spasovska, Ivan Vidinic
INTRODUCTION: The study aimed to compare the etiologic spectrum of diseases causing fever of unknown origin (FUO) and methods for definitive diagnosis in a tertiary care hospital in Republic of North Macedonia during two different time periods. PATIENTS AND METHODS: retrospectively were analysed the causes for FUO and final diagnostic approaches in 185 patients with classic FUO that were treated at the University Hospital for Infectious diseases in Skopje during two time periods...
March 11, 2019: Romanian Journal of Internal Medicine
Qunxi Dong, Wen Zhang, Jianfeng Wu, Bolun Li, Emily H Schron, Travis McMahon, Jie Shi, Boris A Gutman, Kewei Chen, Leslie C Baxter, Paul M Thompson, Eric M Reiman, Richard J Caselli, Yalin Wang
Apolipoprotein E (APOE) e4 is the major genetic risk factor for late-onset Alzheimer's disease (AD). The dose-dependent impact of this allele on hippocampal volumes has been documented, but its influence on general hippocampal morphology in cognitively unimpaired individuals is still elusive. Capitalizing on the study of a large number of cognitively unimpaired late middle aged and older adults with two, one and no APOE-e4 alleles, the current study aims to characterize the ability of our automated surface-based hippocampal morphometry algorithm to distinguish between these three levels of genetic risk for AD and demonstrate its superiority to a commonly used hippocampal volume measurement...
March 4, 2019: NeuroImage: Clinical
Elena Biagi, Daniele Zama, Simone Rampelli, Silvia Turroni, Patrizia Brigidi, Clarissa Consolandi, Marco Severgnini, Eleonora Picotti, Pietro Gasperini, Pietro Merli, Nunzia Decembrino, Marco Zecca, Simone Cesaro, Maura Faraci, Arcangelo Prete, Franco Locatelli, Andrea Pession, Marco Candela, Riccardo Masetti
BACKGROUND: The onset of acute Graft-versus-Host Disease (aGvHD) has been correlated with the gut microbiota (GM) composition, but experimental observations are still few, mainly involving cohorts of adult patients. In the current scenario where fecal microbiota transplantation has been used as a pioneer therapeutic approach to treat steroid-refractory aGvHD, there is an urgent need to expand existing observational studies of the GM dynamics in Hematopoietic Stem Cell Transplantation (HSCT)...
March 7, 2019: BMC Medical Genomics
Allan Mf Kwok, Andrew B Still, Kimberly Hart
BACKGROUND: Hypoganglionosis is a rare condition that most often presents with abnormal gastrointestinal transit and usually arises in early childhood or adolescence. Two types have been described (Type I and Type II). The adult-onset form (acquired hypoganglionosis) is extremely uncommon and is thought to arise due to cellular remodelling as a result of chronic inflammation. It differs from Hirschprung's disease in that there is a reduction in ganglion cells in the colonic neural plexuses as opposed to being completely absent...
February 27, 2019: World Journal of Gastrointestinal Surgery
Ben C Creelan, M Usman Ahmad, Frank J Kaszuba, Farah K Khalil, Allison W Welsh, Metin Ozdemirli, Nazaneen N Grant, Deepa S Subramaniam
BACKGROUND: Juvenile-onset recurrent respiratory papillomatosis (JO-RRP) is a human papilloma virus-mediated progressive benign neoplasm that affects children and young adults. Primary management consists of regular surgical debulking to maintain airway patency and vocal function. Like condyloma acuminata, JO-RRP is associated with immune dysregulation, and T cells isolated from papillomas express an anergic phenotype. Therefore, we hypothesized that programmed death protein 1 axis inhibition could stabilize tumor growth...
March 6, 2019: Oncologist
Mahmood Y Hachim, Noha M Elemam, Azzam A Maghazachi
Multiple sclerosis (MS), a chronic inflammatory disease of the central nervous system is common amongst young adults, leading to major personal and socioeconomic burdens. However, it is still considered complex and challenging to understand and treat, in spite of the efforts made to explain its etiopathology. Despite the discovery of many genetic and environmental factors that might be related to its etiology, no clear answer was found about the causes of the illness and neither about the detailed mechanism of these environmental triggers that make individuals susceptible to MS...
March 5, 2019: Toxins
Giulia Menchinelli, Flora Marzia Liotti, Barbara Fiori, Giulia De Angelis, Tiziana D'Inzeo, Liliana Giordano, Brunella Posteraro, Michela Sabatucci, Maurizio Sanguinetti, Teresa Spanu
Blood culture (BC) is still the standard for diagnosing bloodstream infections (BSIs), especially those caused by bacteria and fungi. Infection-complicating sepsis or septic shock often occurs at BSI onset, making necessary to improve the diagnostic yield of positive BCs. Among the BC systems currently available, the BACT/ALERT® VIRTUO® (VIRTUO) system has been developed to shorten time to detection (TTD) of positive BCs. In this study, we assessed TTD for 330 clinically relevant species including 14 Gram-positive, 14 Gram-negative, and 5 yeast isolates in spiked human blood samples that were tested in parallel with VIRTUO BACT/ALERT® 3D (BTA3D) and BACTEC™ FX (BACTEC) systems...
2019: Frontiers in Microbiology
Luqi Shen, Li Wang, Ying Hu, Tingting Liu, Jinzhen Guo, Ye Shen, Ruiyuan Zhang, Toni Miles, Changwei Li
We evaluated the associations of the ages at menarche and menopause with blood pressure (BP) and hypertension using the baseline data of 7893 women from the China Health and Retirement Longitudinal Study, a nationally representative survey among Chinese adults aged ≥45 years. Multivariate linear and logistic regression analyses were performed to evaluate the associations of the ages at menarche and men`opause with BP and hypertension, respectively. Nonlinear associations were evaluated using spline analyses...
February 28, 2019: Hypertension Research: Official Journal of the Japanese Society of Hypertension
Giulio Cavalli, Alessandro Tomelleri, Giacomo De Luca, Corrado Campochiaro, Charles A Dinarello, Elena Baldissera, Lorenzo Dagna
Following publication of the original article [1], the authors reported an error in the spelling of Lorenzo Dagna's name.
February 25, 2019: Arthritis Research & Therapy
Carolina Melero-Jerez, Margarita Suardíaz, Rafael Lebrón-Galán, Carmen Marín-Bañasco, Begoña Oliver-Martos, Isabel Machín-Díaz, Óscar Fernández, Fernando de Castro, Diego Clemente
Multiple sclerosis (MS) is an autoimmune demyelinating disease of the human central nervous system (CNS), mainly affecting young adults. Among the immunomodulatory disease modifying treatments approved up to date to treat MS, IFN-β remains to be one of the most widely prescribed for the Relapsing-Remitting (RR) variant of the disease, although its mechanism of action is still partially understood. RR-MS variant is characterized by phases with increasing neurological symptoms (relapses) followed by periods of total or partial recovery (remissions), which implies the existence of immunomodulatory agents to promote the relapsing-to-remitting transition...
February 21, 2019: Neurobiology of Disease
Ayoung Jeong, Medea Imboden, Akram Ghantous, Alexei Novoloaca, Anne-Elie Carsin, Manolis Kogevinas, Christian Schindler, Gianfranco Lovison, Zdenko Herceg, Cyrille Cuenin, Roel Vermeulen, Deborah Jarvis, André F S Amaral, Florian Kronenberg, Paolo Vineis, Nicole Probst-Hensch
A high body mass (BMI) index has repeatedly been associated with non-atopic asthma, but the biological mechanism linking obesity to asthma is still poorly understood. We aimed to test the hypothesis that inflammation and/or innate immunity plays a role in the obesity-asthma link. DNA methylome was measured in blood samples of 61 non-atopic participants with asthma and 146 non-atopic participants without asthma (non-smokers for at least 10 years) taking part in the Swiss Cohort Study on Air Pollution and Lung and Heart Diseases in Adults (SAPALDIA) study...
February 19, 2019: International Journal of Environmental Research and Public Health
Yue Leng, Erik S Musiek, Kun Hu, Francesco P Cappuccio, Kristine Yaffe
Dysfunction in 24-h circadian rhythms is a common occurrence in ageing adults; however, circadian rhythm disruptions are more severe in people with age-related neurodegenerative diseases, including Alzheimer's disease and related dementias, and Parkinson's disease. Manifestations of circadian rhythm disruptions differ according to the type and severity of neurodegenerative disease and, for some patients, occur before the onset of typical clinical symptoms of neurodegeneration. Evidence from preliminary studies suggest that circadian rhythm disruptions, in addition to being a symptom of neurodegeneration, might also be a potential risk factor for developing Alzheimer's disease and related dementias, and Parkinson's disease, although large, longitudinal studies are needed to confirm this relationship...
March 2019: Lancet Neurology
Emre Bilgin, Mutlu Hayran, Abdulsamet Erden, Berkan Armağan, Alper Sarı, Levent Kılıç, Ali Akdoğan, Ömer Karadağ, Şule Apraş Bilgen, Sedat Kiraz, İhsan Ertenli, Umut Kalyoncu
The author regrets that the original version of this article contained error. Figure 1 was shown in the wrong version, thus corrected figure is shown in this article.
February 19, 2019: Clinical Rheumatology
Yasuhiro Shimojima, Dai Kishida, Ken-Ichi Ueno, Satoru Ushiyama, Takanori Ichikawa, Yoshiki Sekijima
OBJECTIVE: To investigate the characteristics of circulating NK cells and their IFN-γ-producing ability in adult-onset Still's disease (AOSD). METHODS: Peripheral blood mononuclear cells were obtained from 22 patients in the acute phase of AOSD (acute AOSD); 7 of the 22 patients after treatment (remission AOSD), and 11 healthy controls (HC). NK cells and their IFN-γ expression levels were analyzed by flow cytometry. Additionally, the cytokine receptors of interleukin (IL)-12, IL-15, and IL-18 on NK cells were also evaluated...
February 15, 2019: Journal of Rheumatology
Yukiko Takakuwa, Hironari Hanaoka, Tomofumi Kiyokawa, Harunobu Iida, Kana Ishimori, Toshimasa Uekusa, Hidehiro Yamada, Kimito Kawahata
OBJECTIVES: Adult-onset Still's disease (AOSD) is an inflammatory disorder characterised by sustained fevers, arthritis, and skin involvement. Interstitial lung disease (ILD) is a rare manifestation, and its clinical characteristics have yet to be determined. METHODS: We sought to examine the clinical characteristics of AOSD-associated ILD. We retrospectively investigated 78 patients diagnosed as AOSD. ILD was diagnosed based on chest high-resolution computed tomography (HRCT)...
February 11, 2019: Clinical and Experimental Rheumatology
Albina Moreira, Miguel Borges Silva, Ana Afonso, Cristina Rodrigues
We report a clinical case of adult-onset Still's disease. In addition to the imaging features of the case, the following aspects are also briefly described: clinical presentation (prolonged fever associated with migratory polyarthritis and skin rash), treatment performed (resort to anti-inflammatory and corticoid), and clinical evolution of the patient after having performed the adult-onset. The treatment was found to be appropriate as the patient presented a favourable clinical evolution. LEARNING POINTS: Skin manifestations are associated with systemic diseases...
2019: European Journal of Case Reports in Internal Medicine
François Vercruysse, Thomas Barnetche, Estibaliz Lazaro, Emilie Shipley, François Lifermann, Alexandre Balageas, Xavier Delbrel, Bruno Fautrel, Christophe Richez, Thierry Schaeverbeke, Marie-Elise Truchetet
OBJECTIVES: Adult-onset Still's disease (AOSD) phenotype appears to be dichotomized in systemic or chronic articular forms. As biologicals and particularly interleukin (IL)-1 and IL-6 blockers play a more and more prominent role in the treatment, their place requires clarification. This study aimed to identify factors predictive of treatment response to anakinra or tocilizumab and investigate whether the choice of biotherapy and delays in the initiation of biotherapy influenced the likelihood of steroid discontinuation...
February 12, 2019: Arthritis Research & Therapy
Veronika C Stark, Katrin Doering, Yskert von Kodolitsch, Rainer Kozlik-Feldmann, Götz C Mueller, Jakob Olfe, Meike Rybczynski, Helke Schueler, Thomas S Mir
Background: Care for patients with Marfan syndrome (MFS) has improved substantially in recent decades. Increasing clinical knowledge and genetic analysis allow early diagnosis of the disease in childhood. Because of the earlier initiation to preventive and medical treatment, patients' life expectancy has risen. To ensure optimal care, pediatric patients require a safe follow-up regime, multidisciplinary care, and a safe transition to adult care. Methods: We collected a sample of 149 pediatric Marfan patients, of whom 34 patients had already been transferred to adult care or who were currently transitioning...
December 2018: Cardiovascular Diagnosis and Therapy
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