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Stiff Person

Ewelina Bąk, Czesław Marcisz, Adriana Borodzicz, Danuta Sternal, Sylwia Krzemińska
Purpose: The main purpose of this study was to compare the level of health-related quality of life (HRQoL) using Nottingham Health Profile (NHP) in Polish patients with rheumatoid arthritis (RA) during therapy applying disease-modifying antirheumatic drugs (DMARDs) with conventional synthetics (csDMARDs) or with csDMARDs in combination with biological drugs (bDMARDs). The second purpose was to analyze the correlation between the domain values of NHP and the demographic and clinical parameters, functional efficiency, and mood...
2019: Patient Preference and Adherence
Hamid Naghibi, Valentina Mazzoli, Kaj Gijsbertse, Gerjon Hannink, Andre Sprengers, Dennis Janssen, Ton Van den Boogaard, Nico Verdonschot
Characterization of the main tibiofemoral ligaments is an essential step in developing patient-specific computational models of the knee joint for personalized surgery pre-planning. Tensile tests are commonly performed in-vitro to characterize the mechanical stiffness and rupture force of the knee ligaments which makes the technique unsuitable for in-vivo application. The time required for the limited noninvasive approaches for properties estimation based on knee laxity remained the main obstacle in clinical implementation...
February 7, 2019: Journal of the Mechanical Behavior of Biomedical Materials
Daniel G Di Luca, Sagari Bette, Carlos Singer, Corneliu Luca
No abstract text is available yet for this article.
February 12, 2019: Neurology
Janne Fuglsang Hansen, Karen Mølgaard Christiansen, Benjamin Staugaard, Belinda Klemmensen Moessner, Søren Lillevang, Aleksander Krag, Peer Brehm Christensen
BACKGROUND: Non-invasive methods are the first choice for liver fibrosis evaluation in chronic liver diseases, but few studies investigate the ability of combined methods to predict outcomes. METHODS: 591 chronic hepatitis C patients with baseline liver stiffness (LSM) by FibroScan and hyaluronic acid measurements were identified retrospectively. The patients were grouped by baseline LSM: < 10kPa, 10-16.9kPa, and 17-75kPa. Primary outcomes were all-cause mortality and liver-related mortality, analyzed using cox regression and competing risk regression models, respectively...
2019: PloS One
Michael H Radke, Christopher Polack, Mei Methawasin, Claudia Fink, Henk L Granzier, Michael Gotthardt
BACKGROUND: Titin is a giant elastic protein that spans the half-sarcomere from Z-disk to M-band. It acts as a molecular spring and mechanosensor and has been linked to striated muscle disease. The pathways that govern titin dependent cardiac growth and contribute to disease are diverse and difficult to dissect. METHODS: To study titin deficiency versus dysfunction, we generated and compared striated muscle specific knockouts with progressive postnatal loss of the complete titin protein by removing exon 2 (E2-KO) or an M-band truncation that eliminates proper sarcomeric integration but retains all other functional domains (M1/2-KO)...
January 31, 2019: Circulation
Carles Gaig, Yaroslau Compta
PURPOSE OF REVIEW: Anti-IgLON5 disease is a novel entity characterized by a distinctive sleep disorder associated with a variety of neurological symptoms, antibodies against IgLON5, and pathological findings of neuronal tauopathy. The characteristic sleep disorder occurs in most patients, but other neurological symptoms are also important because they can be the presenting and most disabling problem and mimic other conditions. This review focuses on nonsleep neurological symptoms and presentations of anti-IgLON5 disease...
January 28, 2019: Current Opinion in Neurology
Fabian Tomschi, Esther Rautenberg, Eduard Isenmann, Hannah Ottmann, Wilhelm Bloch, Marijke Grau
PURPOSE: Weight lifting training has gained much popularity in recent years and is frequently used in non-professional and professional settings. However, little is known about the acute effects of a highly intensive weight lifting exercise (clean and jerk) on the hemodynamic reaction. METHODS: 18 non-professional experienced weight lifters were recruited. Hemodynamic parameters were obtained and measured at baseline (T1), after warming up (T2), and after a highly intensive clean and jerk exercise (90-95% of personal best; T3)...
January 21, 2019: European Journal of Applied Physiology
Timm Dirrichs, Simone Schrading, Matthias Gatz, Markus Tingart, Christiane K Kuhl, Valentin Quack
RATIONALE AND OBJECTIVES: It has been shown that Shear Wave Elastography (SWE) is a useful tool to evaluate tendon stiffness, e.g. in diagnosing tendinopathies, as diseased or injured tendons are intra-individually softer than healthy ones. But reference values between different population groups are still missing. The purpose of this prospective clinical study was two-fold: First, to comparatively analyse Achilles tendon stiffness between asymptomatic semiprofessional athletes and an asymptomatic nonathletic control group...
January 14, 2019: Academic Radiology
Sarah Allegra, Jessica Cusato, Silvia De Francia, Filomena Longo, Elisa Pirro, Davide Massano, Valeria Avataneo, Amedeo De Nicolò, Antonio Piga, Antonio D'Avolio
Monitoring and treating iron overload is crucial in transfusion-dependent thalassaemia patients. Liver stiffness measurement by transient elastography and T2* magnetic resonance imaging represent non-invasive ways to evaluate the adequacy of the iron chelation treatment. We explored the role of single nucleotide polymorphisms involved in vitamin D metabolism, transport and activity, and in deferasirox metabolism on liver iron burden parameters. One-hundred and five beta-thalassaemia patients, treated with deferasirox, have been enrolled...
January 17, 2019: Pharmacogenomics Journal
Zhen-Yu Zhang, Esther Nkuipou-Kenfack, Jan A Staessen
Diastolic heart failure (DHF) is characterized by slow left ventricular (LV) relaxation, increased LV stiffness, interstitial deposition of collagen, and a modified extracellular matrix proteins. Among Europeans, the frequency of asymptomatic diastolic LV dysfunction (DD) is 25%. This constitutes a large pool of people at high risk of DHF. The goal of this review was to describe the discovery and the initial validation of new multidimensional urinary peptidomic biomarkers (UPB) indicative of DD, mainly consisting of collagen fragments, and to describe a roadmap for their introduction in to clinical practice...
January 11, 2019: Proteomics. Clinical Applications
Jurgen Damen, Rogier M van Rijn, Pieter J Emans, Willem K H A Hilberdink, Janet Wesseling, Edwin H G Oei, Sita M A Bierma-Zeinstra
BACKGROUND: We aimed to evaluate the prevalence of hip and knee osteoarthritis (HOA and KOA) according to American College of Rheumatology (ACR) criteria among participants with suspected early symptomatic osteoarthritis (OA) in the CHECK cohort. We also assessed whether participants not fulfilling ACR criteria at baseline develop ACR-defined OA at 2-year and/or 5-year follow up, and which baseline factors are associated with this development. METHODS: The CHECK cohort included 1002 subjects with first presentation of knee and/or hip complaints...
January 5, 2019: Arthritis Research & Therapy
Jonathan R Dillman, Suraj D Serai, Andrew T Trout, Ruchi Singh, Jean A Tkach, Amy E Taylor, Burns C Blaxall, Lin Fei, Alexander G Miethke
BACKGROUND: Primary sclerosing cholangitis, autoimmune hepatitis and autoimmune sclerosing cholangitis are forms of chronic, progressive autoimmune liver disease (AILD) that can affect the pediatric population. OBJECTIVE: To determine whether quantitative MRI- and laboratory-based biomarkers are associated with conventional imaging findings of portal hypertension (radiologic portal hypertension) in children and young adults with AILD. MATERIALS AND METHODS: Forty-four patients with AILD enrolled in an institutional registry underwent a research abdominal MRI examination at 1...
January 3, 2019: Pediatric Radiology
Goran Rakocevic, Harry Alexopoulos, Marinos C Dalakas
BACKGROUND: Stiff Person Syndrome (SPS) is an under-diagnosed disorder that affects mobility and the quality of life of affected patients. The aim of the study is to describe the natural history of SPS, the extent of accumulated disability and the associated clinical and immunological features in patients followed for up to 8 years in a single center. METHODS: Our collective cohort included 57 SPS patients. Additionally, 32 of these patients were examined every 6 months for a two-year period in a longitudinal study protocol, to assess disease progression using quantitative measures of stiffness and heightened sensitivity...
January 3, 2019: BMC Neurology
Arnold Y L Wong, Eric C Parent, Sukhvinder S Dhillon, Narasimha Prasad, Dino Samartzis, Gregory N Kawchuk
PURPOSE: Our prior study revealed that people with non-specific low back pain (LBP) who self-reported a > 30% improvement in disability after SMT demonstrated significant post-treatment improvements in spinal stiffness, dynamic muscle thickness and disc diffusion, while those not having self-reported improvement did not have these objective changes. The mechanism underlying this differential post-SMT response remains unknown. This exploratory secondary analysis aimed to determine whether persons with non-specific LBP who respond to spinal SMT have unique lumbar magnetic resonance imaging (MRI) findings compared to SMT non-responders...
January 2, 2019: European Spine Journal
L Susan Williams, Edward L W Green, William A Chernoff
The imperative to heed social environment and power of the situation, particularly as applied to prison settings, dates to the 1971 Stanford Prison Experiment; the widely cited study concluded that situational factors, not personality, created the damaging conditions observed. Despite critical need for research on prison climate, measurement has met stiff challenges, and little research on prison culture exists in the United States. This study applies a 17-item scale, primarily based on Essen Climate Evaluation Schema (EssenCES), an instrument validated in Australia, Germany, and the United Kingdom...
January 2, 2019: International Journal of Offender Therapy and Comparative Criminology
Taeko Osawa, Kazuya Fujihara, Mayuko Harada, Masahiko Yamamoto, Masahiro Ishizawa, Hiroshi Suzuki, Hajime Ishiguro, Yasuhiro Matsubayashi, Hiroyasu Seida, Nauta Yamanaka, Shiro Tanaka, Hitoshi Shimano, Satoru Kodama, Hirohito Sone
AIMS: To determine incidence and predictors of starting dialysis in patients with diabetes emphasizing blood pressure variables. METHODS: A nationwide database with claim data on 18,935 people (15,789 men and 3,146 women) with diabetes mellitus aged 19-72 y in Japan was used to elucidate predictors for starting dialysis. Initiation of dialysis was determined from claims using ICD-10 codes and medical procedures. Using multivariate Cox modeling, interactions between glycaemic and blood pressure values were determined...
December 22, 2018: Diabetes/metabolism Research and Reviews
Andrea Medrano Behlmann, Namita A Goyal, Xiaoyu Yang, Ping H Chen, Arunkanth Ankala
Phosphoglycerate kinase-1 (PGK1) deficiency is a rare X-linked disorder caused by pathogenic variants in the PGK1 gene. Complete loss-of-function variants have not been reported in this gene, indicating that residual enzyme function is critical for viability in males. Therefore, copy number variants (CNVs) that include single exon or multiple exon deletions or duplications are generally not expected in individuals with PGK1 deficiency. Here we describe a 64-year-old male presenting with a family history (three additional affected males) and a personal history of childhood-onset metabolic myopathy that involves episodes of muscle pain, stiffness after activity, exercise intolerance, and myoglobinuria after exertion...
December 21, 2018: JIMD Reports
P Kotruchin, S Hoshide, H Ueno, T Komori, K Kario
Background: The threshold of blood pressure (BP) reduction in cardiovascular patients is debatable due to the J-shaped curve phenomenon, which is particularly observed in patients with increased arterial stiffness. The renal resistive index (RRI) correlates well with systemic arterial stiffness; therefore, we aimed to demonstrate the role of RRI in guiding the choice of optimal BP. Methods: A retrospective analysis of prospectively collected data of the hospitalized cardiovascular patients at Jichi Medical University Hospital...
December 16, 2018: American Journal of Hypertension
Linxiao Wu, Adrián Magaz, Elizabeth Maughan, Nina Oliver, Arnold Darbyshire, Marilena Loizidou, Mark Emberton, Martin Birchall, Wenhui Song
Increasing evidence suggests the contribution of the dynamic mechanical properties of the extracellular matrix (ECM) to regulate tissue remodeling and regeneration. Following our recent study on a family of thermoresponsive 'stiffness memory' elastomeric nanohybrid scaffolds manufactured via an indirect 3D printing guided thermo-induced phase separation process (3D-TIPS), this work reports in vitro and in vivo cellular responses towards these scaffolds with different initial stiffness and hierarchical interconnected porous structure...
December 14, 2018: Acta Biomaterialia
Victor Grech
As an experienced writer, one is often asked the question "what can I publish" by younger and inexperienced colleagues who must publish. This paper will briefly review the reasons why academics publish and will then outline what kind of papers one may be able to publish with relative ease, using personal experience and citing first hand material as a practical guide. Potential authors must cultivate "a lean and hungry look" as competition from fellow writers is stiff, and editors are choosy...
December 10, 2018: Early Human Development
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