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Still onset adult disease

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https://www.readbyqxmd.com/read/28424565/invasive-pneumococcal-disease-still-lots-to-learn-and-a-need-for-standardized-data-collection-instruments
#1
T J Marrie, G J Tyrrell, Sumit R Majumdar, Dean T Eurich
Background. Large studies of invasive pneumococcal disease (IPD) are frequently lacking detailed clinical information. Methods. A population-based 15-year study of IPD in Northern Alberta. Results. 2435 patients with a mean age of 54.2 years formed the study group. Males outnumbered females and Aboriginal and homeless persons were overrepresented. High rates of smoking, excessive alcohol use, and illicit drug use were seen. Almost all (87%) had a major comorbidity and 15% had functional limitations prior to admission...
2017: Canadian Respiratory Journal: Journal of the Canadian Thoracic Society
https://www.readbyqxmd.com/read/28421535/microrna-metabolism-and-dysregulation-in-amyotrophic-lateral-sclerosis
#2
REVIEW
Paola Rinchetti, Mafalda Rizzuti, Irene Faravelli, Stefania Corti
MicroRNAs (miRNAs) are a subset of endogenous, small, non-coding RNA molecules involved in the post-transcriptional regulation of eukaryotic gene expression. Dysregulation in miRNA-related pathways in the central nervous system (CNS) is associated with severe neuronal injury and cell death, which can lead to the development of neurodegenerative disorders, such as amyotrophic lateral sclerosis (ALS). ALS is a fatal adult onset disease characterized by the selective loss of upper and lower motor neurons. While the pathogenesis of ALS is still largely unknown, familial ALS forms linked to TAR DNA-binding protein 43 (TDP-43) and fused in sarcoma (FUS) gene mutations, as well as sporadic forms, display changes in several steps of RNA metabolism, including miRNA processing...
April 18, 2017: Molecular Neurobiology
https://www.readbyqxmd.com/read/28421028/adult-onset-niemann-pick-disease-type-c-rapid-treatment-initiation-advised-but-early-diagnosis-remains-difficult
#3
Tobias Piroth, Kai Boelmans, Florian Amtage, Michel Rijntjes, Anna Wierciochin, Thomas Musacchio, Cornelius Weiller, Jens Volkmann, Stephan Klebe
Niemann-Pick type C disease (NP-C) presents with heterogeneous neurological and psychiatric symptoms. Adult onset is rare and possibly underdiagnosed due to frequent lack of specific and obvious key symptoms. For both early and adolescent/adult onset, the available data from studies and case reports describe a positive effect of Miglustat (symptom relief or stabilization). However, due to the low frequency of NP-C, experience with this therapy is still limited. We describe two adult-onset cases of NP-C. In both cases, vertical supranuclear gaze palsy was not recognized at symptom onset...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28412707/application-of-the-2016-eular-acr-printo-classification-criteria-for-macrophage-activation-syndrome-in-patients-with-adult-onset-still-disease
#4
Sung Soo Ahn, Byung-Woo Yoo, Seung Min Jung, Sang-Won Lee, Yong-Beom Park, Jason Jungsik Song
OBJECTIVE: To evaluate the clinical significance of the 2016 European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR)/Pediatric Rheumatology International Trials Organization (PRINTO) classification criteria for macrophage activation syndrome (MAS) in patients with adult-onset Still disease (AOSD). METHODS: We performed a retrospective analysis of patients with AOSD with fever who were admitted to Severance Hospital between 2005 and 2016...
April 15, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28411118/regulation-of-motor-proteins-axonal-transport-deficits-and-adult-onset-neurodegenerative-diseases
#5
REVIEW
Scott T Brady, Gerardo A Morfini
Neurons affected in a wide variety of unrelated adult-onset neurodegenerative diseases (AONDs) typically exhibit a "dying back" pattern of degeneration, which is characterized by early deficits in synaptic function and neuritic pathology long before neuronal cell death. Consistent with this observation, multiple unrelated AONDs including Alzheimer's disease, Parkinson's disease, Huntington's disease, and several motor neuron diseases feature early alterations in kinase-based signaling pathways associated with deficits in axonal transport (AT), a complex cellular process involving multiple intracellular trafficking events powered by microtubule-based motor proteins...
April 11, 2017: Neurobiology of Disease
https://www.readbyqxmd.com/read/28397955/unusual-manifestations-and-unusual-mimics-of-adult-onset-still-s-disease
#6
Marwan H Adwan, Faris G Bakri, Ahmad Z Qureshi
[No Abstract Available].
April 2017: Saudi Medical Journal
https://www.readbyqxmd.com/read/28365573/serum-levels-of-interleukin-33-and-soluble-st2-are-associated-with-the-extent-of-disease-activity-and-cutaneous-manifestations-in-patients-with-active-adult-onset-still-s-disease
#7
Jae Ho Han, Chang-Hee Suh, Ju-Yang Jung, Mi-Hyun Ahn, Ji Eun Kwon, Hyunee Yim, Hyoun-Ah Kim
OBJECTIVE: Interleukin 33 (IL-33), a member of the IL-1 family and a ligand of the orphan receptor ST2, plays key roles in innate and adaptive immunity. We examined the associations between IL-33/ST2 levels and clinical manifestations of patients with active adult-onset Still's disease (AOSD). METHODS: Blood samples were collected from 40 patients with active AOSD, 28 patients with rheumatoid arthritis (RA), and 27 healthy controls (HC). The serum levels of IL-33 and soluble ST2 were determined using ELISA...
April 1, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28364296/juvenile-onset-myasthenia-gravis-autoantibody-status-clinical-characteristics-and-genetic-polymorphisms
#8
Yu Hong, Geir Olve Skeie, Paraskevi Zisimopoulou, Katerina Karagiorgou, Socrates J Tzartos, Xiang Gao, Yao-Xian Yue, Fredrik Romi, Xu Zhang, Hai-Feng Li, Nils Erik Gilhus
Myasthenia gravis (MG) is an autoimmune disorder mediated by antibodies against proteins at the neuromuscular junction. Juvenile-onset MG (JMG) has been reported to have special characteristics. It is still unclear whether there are any pathogenic and genetic differences between juvenile and adult MG. In this study, we evaluated the clinical characteristics, autoantibody status (antibodies against AChR, MuSK, LRP4, titin and RyR) and genetic susceptibility (CHRNA1, CTLA4 and AIRE) in 114 Chinese JMG patients, and compared with 207 young adult MG patients (onset age 18-40 years)...
March 31, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28342882/functional-neuroimaging-findings-in-healthy-middle-aged-adults-at-risk-of-alzheimer-s-disease
#9
REVIEW
Mirette Habib, Elijah Mak, Silvy Gabel, Li Su, Guy Williams, Adam Waldman, Katie Wells, Karen Ritchie, Craig Ritchie, John T O'Brien
It is well established that the neurodegenerative process of Alzheimer's disease (AD) begins many years before symptom onset. This preclinical phase provides a crucial time-window for therapeutic intervention, though this requires biomarkers that could evaluate the efficacy of future disease-modification treatments in asymptomatic individuals. The last decade has witnessed a proliferation of studies characterizing the temporal sequence of the earliest functional and structural brain imaging changes in AD. These efforts have focused on studying individuals who are highly vulnerable to develop AD, such as those with familial genetic mutations, susceptibility genes (i...
March 22, 2017: Ageing Research Reviews
https://www.readbyqxmd.com/read/28342280/clinical-and-histopathological-features-of-cutaneous-manifestations-of-adult-onset-still-disease
#10
Erin Santa, Jeanne M McFalls, Joya Sahu, Jason B Lee
Adult-onset Still disease (AOSD) is a rare autoinflammatory syndrome characterized by recurring fevers, arthralgia, and consistent laboratory abnormalities that include leukocytosis and hyperferritinemia. Skin findings accompany the disease in nearly 90% of the cases. Early reports described evanescent, pruritic, salmon-pink or urticarial lesions, referred to as the typical eruption of AOSD. Histopathologic findings consist of superficial perivascular dermatitis with varying number of interstitial neutrophils...
March 25, 2017: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/28340859/heterogeneity-of-obesity-asthma-association-disentangled-by-latent-class-analysis-the-sapaldia-cohort
#11
Ayoung Jeong, Medea Imboden, Sofie Hansen, Elisabeth Zemp, Pierre-Olivier Bridevaux, Gianfranco Lovison, Christian Schindler, Nicole Probst-Hensch
Although evidence for the heterogeneity of asthma accumulated, consensus for definitions of asthma phenotypes is still lacking. Obesity may have heterogeneous effects on various asthma phenotypes. We aimed to distinguish asthma phenotypes by latent class analysis and to investigate their associations with different obesity parameters in adults using a population-based Swiss cohort (SAPALDIA). We applied latent class analysis to 959 self-reported asthmatics using information on disease activity, atopy, and age of onset...
April 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/28332257/exomic-variants-of-an-elderly-cohort-of-brazilians-in-the-abraom%C3%A2-database
#12
Michel Satya Naslavsky, Guilherme Lopes Yamamoto, Tatiana Ferreira de Almeida, Suzana A M Ezquina, Daniele Yumi Sunaga, Nam Pho, Daniel Bozoklian, Tatiana Orli Milkewitz Sandberg, Luciano Abreu Brito, Monize Lazar, Danilo Vicensotto Bernardo, Edson Amaro, Yeda A O Duarte, Maria Lúcia Lebrão, Maria Rita Passos-Bueno, Mayana Zatz
Brazilians are highly admixed with ancestry from Europe, Africa, America, and Asia yet still underrepresented in genomic databanks. We hereby present a collection of exomic variants from 609 elderly Brazilians in a census-based cohort (SABE609) with comprehensive phenotyping. Variants were deposited in ABraOM (Online Archive of Brazilian Mutations), a web-based public database. Population representative phenotype and genotype repositories are essential for variant interpretation through allele frequency filtering; since elderly individuals are less likely to harbor pathogenic mutations for early and adult-onset diseases, such variant databases are of great interest...
March 23, 2017: Human Mutation
https://www.readbyqxmd.com/read/28296747/adult-onset-still-s-disease-with-atypical-cutaneous-manifestations
#13
Francisco Javier Narváez Garcia, María Pascual, Mercè López de Recalde, Pablo Juarez, Isabel Morales-Ivorra, Jaime Notario, Anna Jucglà, Joan M Nolla
The diagnosis of adult-onset Still's disease (AOSD) can be very difficult. There are no specific tests available, and diagnosis is usually based on a symptom complex and the well-described typical evanescent rash seen in the majority of patients. However, in recent years, other atypical cutaneous manifestations of AOSD have been reported. These atypical skin eruptions often present in addition to the typical evanescent rash but may also be the only skin manifestation, resulting in delayed diagnosis because of under-recognition...
March 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28284341/mog-antibody-associated-demyelinating-disease-of-the-cns-a-clinical-and-pathological-study-in-chinese-han-patients
#14
Lei Zhou, Yongheng Huang, Haiqing Li, Jie Fan, Jingzi Zhangbao, Hai Yu, Yuxin Li, Jiahong Lu, Chongbo Zhao, Chuanzhen Lu, Min Wang, Chao Quan
We aim to evaluate the clinical relevance of MOG-ab in a cohort of Chinese Han adults with CNS inflammatory demyelinating diseases (IDDs). MOG-ab and AQP4-ab were examined through a fixed cell based indirect immune-fluorescence assay in 86 patients with CNS-IDDs. MOG-ab was positive in 12 patients, while AQP4-ab was positive in 31 patients; none double positives. Optic neuritis (ON) was the most frequent symptom at onset (75.0%) or during the whole disease course (83.3%) of MOG-ab associated IDDs (MOG-IDDs); 79...
April 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28222533/type-1-diabetes-mellitus-and-cognitive-impairments-a-systematic-review
#15
Wei Li, Edgar Huang, Sujuan Gao
Type 1 diabetes mellitus (T1DM) is a major subtype of diabetes and is usually diagnosed at a young age with insulin deficiency. The life expectancy of T1DM patients has increased substantially in comparison with that three decades ago due to the availability of exogenous insulin, though it is still shorter than that of healthy people. However, the relation remains unclear between T1DM and dementia as an aging-related disease. We conducted a systematic review of existing literature on T1DM and cognition impairments by carrying out searches in electronic databases Medline, EMBASE, and Google Scholar...
2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28215902/childhood-abuse-and-the-two-year-course-of-late-life-depression
#16
Ilse Wielaard, Hannie C Comijs, Max L Stek, Didi Rhebergen
OBJECTIVES: Late-life depression often has a chronic course, with debilitating effects on functioning and quality of life; there is still no consensus on important risk factors explaining this chronicity. Cross-sectional studies have shown that childhood abuse is associated with late-life depression, and in longitudinal studies with chronicity of depression in younger adults. We aim to investigate the impact of childhood abuse on the course of late-life depression. DESIGN: Two-year longitudinal cohort study...
January 24, 2017: American Journal of Geriatric Psychiatry
https://www.readbyqxmd.com/read/28215901/physical-health-medication-and-healthcare-utilization-among-70-year-old-people-with-schizophrenia-a-nationwide-danish-register-study
#17
Maria Brink, Anders Green, Anders Bo Bojesen, J Steven Lamberti, Yeates Conwell, Kjeld Andersen
OBJECTIVES: In light of the excess early mortality in schizophrenia, mainly due to physical illnesses, we investigated medical comorbidity, use of medication, and healthcare utilization among individuals with schizophrenia who survived into older ages to uncover potential factors contributing to their longevity. DESIGN: A nationwide register-based case-control study comparing 70-year-olds with and without schizophrenia. SETTING: Cases were drawn from the Danish Psychiatric Central Register...
January 9, 2017: American Journal of Geriatric Psychiatry
https://www.readbyqxmd.com/read/28204878/unusual-onset-of-adult-still-s-disease-due-to-a%C3%A2-systemic-reaction-to-artificial-breast-implants
#18
A Wehr, G Grieb, C Trautwein, K Streetz
In this manuscript, we report an exceptional observation study of a young woman suffering from an autoimmune syndrome induced by adjuvants (ASIA). Until today only seven cases of adult-onset Still's disease (AOSD) induced by breast implants have been published. This may be due to the fact that this illness itself is very rare; however, the reason might also be that the community is not sensitized to the case-specific symptoms. Within this article, we show for the first time highly detailed diagnostic test procedures such as PET-CT scans and specific histological staining of the breast tissue, displaying proinflammatory macrophages that are a well-known activator and booster of autoimmune diseases...
February 15, 2017: Zeitschrift Für Rheumatologie
https://www.readbyqxmd.com/read/28191382/inadvertent-skipping-of-steroids-in-septic-shock-leads-to-a-diagnosis-of-adult-onset-still-s-disease
#19
Vinoth K Sethuraman, Kavitha Balasubramanian, Stalin Viswanathan, Rajeswari Aghoram
Adult onset Still's disease is uncommon in middle-aged and elderly individuals and can rarely present with shock; shock is usually associated with disseminated intravascular coagulation, multiorgan dysfunction syndrome or acute respiratory distress syndrome. We report a post-menopausal woman with arthritis, fever, pneumonitis and hypotension which was managed as septic shock. Steroids were inadvertently missed during the second day of hospitalization in the intensive care unit. Persistence of hypotension on inotropes, with normal renal, hepatic and neurological function and recurrence of fever when steroids were skipped, led to suspicion of an inflammatory disorder...
January 14, 2017: Curēus
https://www.readbyqxmd.com/read/28154368/adult-onset-still-s-disease-still-a-serious-health-problem-a-case-report-and-literature-review
#20
REVIEW
Mojgan Agha-Abbaslou, Ana Maria Bensaci, Oluchi Dike, Mark C Poznansky, Arooj Hyat
BACKGROUND Adult-onset Still's Disease (AOSD) is a rare systemic inflammatory disease accompanied by a triad of spiking fever, maculopapular exanthema, and arthralgia. To date, there is no definite laboratory or imaging test available for diagnosing AOSD, and the diagnosis is one of exclusion, which can be very challenging. CASE REPORT We report on the case of a 53-year-old female who presented with fever, arthralgia, and abdominal pain. Her initial laboratory tests showed elevated AST and ALT, and normal leukocytes with bandemia...
February 3, 2017: American Journal of Case Reports
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