-Assessing the performance of doctors while they are engaged in clinical work is a challenging concept. The introduction of objective-based curricula provides the stimulus and opportunity for the Royal Colleges of Physicians to develop relevant and reliable methods of in-service assessment. We propose to pilot a study investigating the validity, reliability and feasibility of three assessment methodsdirect observation of the clinical encounter using an adapted mini-CEX, direct observation of the performance of practical procedures (DOPS), and the doctor's ability to perform effectively as part of a team using 360°assessment. The methods will be studied in the setting of routine clinical care. Whilst demanding of time from both trainees and trainers, they will represent a significant advance on the current system which is characterised by a lack of evidence in the assessment process.KEY WORDS: assessment, competence, curriculum, performance, specialist registrar, standards IntroductionAssessment of the performance of doctors has recently become an important issue both publicly, as a result of high profile cases such as the Bristol Heart case, and within the profession, following the redesign of higher specialist training. The assessment of doctors in an honest and objective manner is laid out as a fundamental part of practice in the General Medical Council's guidance, Good Medical Practice. 1 At present, the assessment of specialist registrars (SpRs) in medicine is a subjective process. It is based on an educational supervisor 'signing up' a trainee as competent in a specific task in the Record of Training Book. This usually occurs after an arbitrary period of time has been spent by the SpR working in that area and often having completed an arbitrary number of procedures in the case of practical skills. There is no process by which the educational supervisor collects objective evidence to inform judgements about a trainee's competence. It has been acknowledged that there needs to be a greater emphasis on performance-based assessment. There are still no robust mechanisms for formal assessment, and poor performance is not reliably recognised or addressed. There is a real need to develop and implement reliable objective methods of assessment to form an integral part of training of SpRs. The role of the new curriculaThe Joint Committee of Higher Medical Training (JCHMT), which represents all three medical Royal Colleges in the UK, has for some time recognised that there needs to be a review of the approach to specialist physician training. As a result, the JCHMT and specialist advisory committees (SACs) of the medical specialities have rewritten the specialty curricula in line with current educational thinking. The new curricula, which were launched in December 2002, are now objective-based and encompass modern educational principles.Although the content of the new curricula has not changed dramatically, the emphasis of it has. The curricula lay out clear objectives that trainees will have to achieve during ...
Fifty four patients with peripheral nerve syndromes were seen during a 15 month period in a population of about 1500 HIV infected patients at all stages of the disease. Distal symmetrical peripheral neuropathies were seen in 38 of the 54 patients, (11.5% of AIDS patients) and could be distinguished into two forms. The most common (n = 25) was a painful peripheral neuropathy during AIDS, which is distinct clinically and pathologically, having axonal atrophy, and is associated with cytomegalovirus infection at other sites. The 13 non-painful neuropathies seen were more heterogenous. Lumbosacral polyradiculopathy associated with cytomegalovirus and lymphomatous mononeuritis multiplex occurred in fewer than 1% of AIDS patients. Mononeuropathies were seen in 3% of AIDS patients. No patients with acute or chronic inflammatory demyelinating polyradiculoneuropathies were seen. The annual incidence of neuropathies during the AIDS related complex stage was less than 1%; none were seen in asymptomatic HIV seropositive patients.
Spinocerebellar ataxia type 14 (SCA14) is a subtype of the autosomal dominant cerebellar ataxias that is characterized by slowly progressive cerebellar dysfunction and neurodegeneration. SCA14 is caused by mutations in the PRKCG gene, encoding protein kinase C gamma (PKCγ). Despite the identification of 40 distinct disease-causing mutations in PRKCG, the pathological mechanisms underlying SCA14 remain poorly understood. Here we report the molecular neuropathology of SCA14 in post-mortem cerebellum and in human patient-derived induced pluripotent stem cells (iPSCs) carrying two distinct SCA14 mutations in the C1 domain of PKCγ, H36R and H101Q. We show that endogenous expression of these mutations results in the cytoplasmic mislocalization and aggregation of PKCγ in both patient iPSCs and cerebellum. PKCγ aggregates were not efficiently targeted for degradation. Moreover, mutant PKCγ was found to be hyper-activated, resulting in increased substrate phosphorylation. Together, our findings demonstrate that a combination of both, loss-of-function and gain-of-function mechanisms are likely to underlie the pathogenesis of SCA14, caused by mutations in the C1 domain of PKCγ. Importantly, SCA14 patient iPSCs were found to accurately recapitulate pathological features observed in post-mortem SCA14 cerebellum, underscoring their potential as relevant disease models and their promise as future drug discovery tools.Electronic supplementary materialThe online version of this article (10.1186/s40478-018-0600-7) contains supplementary material, which is available to authorized users.
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