Роль компетентностного подхода при управлении профессиональными рисками

В статье рассмотрены соответствия между опасностью, вредом здоровью, профессиональным риском и компетентностью работников, которые могут послужить основой для системы выявления и управления профессиональными рисками, обусловленными человеческим фактором.In this article compliances between danger, harm to health, occupational risk and competence of employees are considered. They can serve as a basis for the system for the detection and management of occupational risks caused by human factors

Clinical and morphological phenotype of the filamin myopathy: a study of 31 German patients

Mutations in the filamin C gene (FLNC) cause a myofibrillar myopathy (MFM), morphologically characterized by focal myofibrillar destruction and abnormal accumulation of several proteins within skeletal muscle fibres. We studied 31 patients from four German families to evaluate the phenotype of filaminopathy. All patients harboured the same p.W2710X mutation in FLNC. Haplotype analysis suggested a founder mutation in these German filaminopathy families. The mean age at onset of clinical symptoms was 44 +/− 6 years (range, 24-57 years). Slowly progressive muscle weakness was mostly pronounced proximally, initially affecting the lower extremities and involving the upper extremities in the course of disease progression, similar to the distribution of weakness seen in limb-girdle muscular dystrophies (LGMD). Patients frequently developed respiratory muscle weakness. About one-third of the patients showed cardiac abnormalities comprising conduction blocks, tachycardia, diastolic dysfunction and left ventricular hypertrophy indicating a cardiac involvement in filaminopathy. Serum creatine kinase levels varied from normal up to 10-fold of the upper limit. Magnetic resonance imaging studies showed a rather homogenous pattern of muscle involvement in the lower extremities differing from that in other types of MFM. Myopathological features included perturbation of myofibrillar alignment, accumulation of granulofilamentous material similar to that seen in primary desminopathies and abnormal intracellular protein deposits typical of MFM. Decreased activities of oxidative enzymes and fibre hypertrophy seem to be early features, whereas dystrophic changes were present in advanced stages of filaminopathy. Rimmed vacuoles were detected in only a few cases. The intracellular aggregates were composed of a variety of proteins including filamin C, desmin, myotilin, Xin, dystrophin and sarcoglycans. Therapy is so far limited to symptomatic treatment. The German filaminopathy cohort, the largest group of patients studied so far, shares phenotypic features with LGMD and presents with characteristic histopathological findings of MF

Challenging the Western stereotype : do Chinese international foundation students learn by rote?

The dissonance between Eastern and Western learning approaches is regarded as an obstacle for Chinese students in adjusting to Western education environments, and one of the reasons is the lack of an understanding of Chinese learning approaches, that is, Chinese learners are uncritically perceived as rote learners. This paper investigates Chinese international foundation students’ learning approaches when they were in China and here in the UK. Their experiences indicate that, similarly to UK students, Chinese students learn with the intention of understanding, they use memorisation only when they fail to understand or have examination pressure. Consequently, Chinese students adjust well to Western teaching styles. Some difficulties they experienced in classrooms are due to language problems and a lack of understanding of Western cultural backgrounds and expectations. Moreover, similarly to UK students who leave home for the first time, the greatest challenge is to develop self-regulated learning. The paper argues that, although Eastern and Western teaching styles are significantly different, students’ underlying learning approaches can be similar in China and the West