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B.G.M. van Engelen

Bio: B.G.M. van Engelen is an academic researcher from Radboud University Nijmegen Medical Centre. The author has contributed to research in topics: Myopathy & Ehlers–Danlos syndrome. The author has an hindex of 22, co-authored 37 publications receiving 1205 citations.

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Journal ArticleDOI
TL;DR: The reliability of the psychological and clinical neurophysiological assessment techniques available today allows a multidisciplinary approach to fatigue in neurological patients, which may contribute to the elucidation of the pathophysiological mechanisms of chronic fatigue, with the ultimate goal to develop tailored treatments for fatigue in Neurological patients.

207 citations

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TL;DR: Clinicians and researchers dealing with myopathies and inherited connective tissue disorders should be aware of the clinical and molecular overlap between the groups of disorders, which has important implications for scientific research, diagnosis, and for the treatment of these disorders.

77 citations

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TL;DR: Distinguishing between normal and deficient balance control due to VL or PL required pitch and roll pelvis sway measures, and pelvis measures yielded better discrimination than shoulder measures.

70 citations

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TL;DR: It is concluded that primarily distal but also proximal muscle weakness leads to significant postural instability and this observation, together with the retained ability of patients to use compensatory arm movements, provides targets that may be amenable to improvement with therapeutic intervention.

67 citations


Cited by
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Journal ArticleDOI
TL;DR: In this paper, the authors review issues of epidemiology and clinical manifestations, focusing on the scientific status of chronic fatigue syndrome (CFS) and suggest multidisciplinary prospective studies of CFS in the general population.

921 citations

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TL;DR: A case is made for a unified definition of fatigue to facilitate its management in health and disease and the proposed framework provides a foundation to address the many gaps in knowledge of how laboratory measures of fatigue and fatigability affect real-world performance.
Abstract: Despite flourishing interest in the topic of fatigue-as indicated by the many presentations on fatigue at the 2015 Annual Meeting of the American College of Sports Medicine-surprisingly little is known about its effect on human performance. There are two main reasons for this dilemma: 1) the inability of current terminology to accommodate the scope of the conditions ascribed to fatigue, and 2) a paucity of validated experimental models. In contrast to current practice, a case is made for a unified definition of fatigue to facilitate its management in health and disease. On the basis of the classic two-domain concept of Mosso, fatigue is defined as a disabling symptom in which physical and cognitive function is limited by interactions between performance fatigability and perceived fatigability. As a symptom, fatigue can only be measured by self-report, quantified as either a trait characteristic or a state variable. One consequence of such a definition is that the word fatigue should not be preceded by an adjective (e.g., central, mental, muscle, peripheral, and supraspinal) to suggest the locus of the changes responsible for an observed level of fatigue. Rather, mechanistic studies should be performed with validated experimental models to identify the changes responsible for the reported fatigue. As indicated by three examples (walking endurance in old adults, time trials by endurance athletes, and fatigue in persons with multiple sclerosis) discussed in the review, however, it has proven challenging to develop valid experimental models of fatigue. The proposed framework provides a foundation to address the many gaps in knowledge of how laboratory measures of fatigue and fatigability affect real-world performance.

513 citations

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TL;DR: The extracellular matrix (ECM) consists of numerous macromolecules classified traditionally into collagens, elastin, and microfibrillar proteins, proteoglycans including hyaluronan, and noncollagenous glycoproteins, and this activity may in part be beneficial to the drugs' disease-modifying properties.
Abstract: The extracellular matrix (ECM) consists of numerous macromolecules classified traditionally into collagens, elastin, and microfibrillar proteins, proteoglycans including hyaluronan, and noncollagenous glycoproteins. In addition to being necessary structural components, ECM molecules exhibit important functional roles in the control of key cellular events such as adhesion, migration, proliferation, differentiation, and survival. Any structural inherited or acquired defect and/or metabolic disturbance in the ECM may cause cellular and tissue alterations that can lead to the development or progression of disease. Consequently, ECM molecules are important targets for pharmacotherapy. Specific agents that prevent theexcess accumulation of ECM molecules in the vascular system, liver, kidney, skin, and lung; alternatively, agents that inhibit the degradation of the ECM in degenerative diseases such as osteoarthritis would be clinically beneficial. Unfortunately, until recently, the ECM in drug discovery has been largely ignored. However, several of today's drugs that act on various primary targets affect the ECM as a byproduct of the drugs' actions, and this activity may in part be beneficial to the drugs' disease-modifying properties. In the future, agents and compounds targeting directly the ECM will significantly advance the treatment of various human diseases, even those for which efficient therapies are not yet available.

492 citations

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TL;DR: A diagnostic algorithm for rhabdomyolysis is proposed, which is aimed at preserving renal function, resolving compartment syndrome, restoring metabolic derangements, and volume replacement in patients with acute renal failure.

467 citations