Carbamyl phosphate synthase deficiency: Diagnosed during pregnancy in a 41-year-old

Carbamyl phosphate synthase deficiency (CPS) is a rare urea cycle defect. We present a case of a 41-year-old woman diagnosed with CPS deficiency during pregnancy. She is the oldest CPS-deficient patient, at diagnosis, reported to date and the first to be diagnosed during pregnancy. This case highlights the need for consideration of inborn errors of metabolism in adults presenting with unusual neurological and psychiatric conditions. Crown Copyright (c) 2006 Published by Elsevier Ltd. All rights reserved.

Clinical tests of musculoskeletal dysfunction in the diagnosis of cervicogenic headache

Persistent intermittent headache is a common disorder and is often accompanied by neck aching or stiffness, which could infer a cervical contribution to headache. However, the incidence of cervicogenic headache is estimated to be 14-18% of all chronic headaches, highlighting the need for clear criterion of cervical musculoskeletal impairment to identify cervicogenic headache sufferers who may benefit from treatments such as manual therapy. This study examined the presence of cervical musculoskeletal impairment in 77 subjects, 27 with cervicogenic headache, 25 with migraine with aura and 25 control subjects. Assessments included a photographic measure of posture, range of movement, cervical manual examination, pressure pain thresholds, muscle length, performance in the cranio-cervical flexion test and cervical kinaesthetic sense. The results indicated that when compared to the migraine with aura and control groups who scored similarly in the tests, the cervicogenic headache group had less range of cervical flexion/extension (P = 0.048) and significantly higher incidences of painful upper cervical joint dysfunction assessed by manual examination (all P < 0.05) and muscle tightness (P < 0.05). Sternocleidomastoid normalized EMG values were higher in the latter three stages of the cranio-cervical flexion test although they failed to reach significance. There were no between group differences for other measures. A discriminant analysis revealed that manual examination could discriminate the cervicogenic headache group from the other subjects (migraine with aura and control subjects combined) with an 80% sensitivity. (C) 2005 Elsevier Ltd. All rights reserved.

The human genome and gene expression profiling

The mapping and sequencing of the human genome has generated a large resource for answering questions about human disease. This achievement is akin in scientific importance to developing the periodic table of elements. Plastic surgery has always been at the frontier medical research. This resource will help us to improve our understanding on the many unknown physiological and pathogical conditions we deal with daily, such as wound heating keloid scar formation, Dupuytren's disease, rheumatoid arthritis, vascular malformation and carcinogenesis. We are primed in obtaining both disease and normal tissues to use this resource and applying it to clinical use. This review is about the human genome, the basis of gene expression profiling and how it will affect our clinical and research practices in the future and for those embarking on the use of this new technology as a research tool, we provide a brief insight on its limitations and pitfalls. (C) 2006 The British Association of Plastic Surgeons. Published by Elsevier Ltd. All rights reserved.

Circadian blood pressure variation after acute stroke

We aimed to characterise the patterns of circadian blood pressure (BP) variation after acute stroke and determine whether any relationship exists between these patterns and stroke outcome. BP was recorded manually every 4 h for 48 h following acute stroke. Patients were classified according to the percentage fall in mean systolic BP (SBP) at night compared to during the day as: dippers (fall >= 10-= 20%); non-dippers (>= 0-

Prismatic dentine in the Australian lungfish, Neoceratodus forsteri (Osteichthyes : Dipnoi)

The Australian lungfish, Neoceratodus forsteri, has a dentition consisting of enamel, mantle dentine and bone, enclosing circumdenteonal, core and interdenteonal dentines. Branching processes from cells that produce interdenteonal dentine leave the cell surface at different angles, with collagen fibrils aligned parallel to the long axis of each process. In the interdenteonal dentine, crystals of calcium hydroxyapatite, form within fibrils of collagen, and grow within a matrix of non-collagenous protein. Crystals are aligned parallel to the cell process, as are the original collagen fibrils. Because the processes are angled to the cell surface, the crystals within the core or interdenteonal dentine are arranged in bundles set at angles to each other. Apatite crystals in circumdenteonal dentine are finer and denser than those of the interdenteonal dentine, and form outside the fibrils of collagen. In mature circumdenteonal dentine the crystals of circumdenteonal dentine form a dense tangled mass, linked to interdenteonal dentine by isolated crystals. The functional lungfish tooth plate contains prisms of large apatite crystals in the interdenteonal dentine and masses of fine tangled crystals around each denteon. This confers mechanical strength on a structure with little enamel that is subjected to heavy wear. (c) 2006 Elsevier Ltd. All rights reserved.

A pathology of the animal spirits - the clinical neurology of Thomas Willis (1621-1675) Part I - Background, and disorders of intrinsically normal animal spirits

Thomas Willis (1621-1675), author of the classical work Cerebri Anatome (1664), was arguably the father of the modem era of neurology. As compared with his neuroanatomy, relatively little attention has been paid to Willis' clinical neurology, as described in his Pathologiae Cerebri (1667) and Do Anima Brutorum (1672), where he gave a structured account of disease of the nervous system as it was known in his day. His account was largely derived from personal observations and not from traditional authorities and was based around his concept of the animal spirits, a fictitious entity in many ways analogous to the present day idea of the nerve impulse. This concept allowed him to develop a pathology of the animal spirits which embraced the whole content of the clinical neurology and psychiatry of his times. The anatomical and physiological background to Willis! concepts of animal spirit dysfunction, and those disorders he regarded as due to disturbed function of intrinsically normal animal spirits (mainly headache, disorders of consciousness, apoplexy and palsy) are dealt with in the present paper. The disorders he attributed to inherently abnormal animal spirits are considered in a second part of the paper. (C) 2002 Elsevier Science Ltd. All eights reserved.

ANÁLISE DAS MUDANÇAS DOS FATORES DETERMINANTES DA ESTRUTURA DE CAPITAL EM FUNÇÃO DO CICLO DE VIDA DAS EMPRESAS BRASILEIRAS SÃO BERNARDO DO CAMPO

As várias teorias acerca da estrutura de capital despertam interesse motivando diversos estudos sobre o assunto sem, no entanto, ter um consenso. Outro tema aparentemente pouco explorado refere-se ao ciclo de vida das empresas e como ele pode influenciar a estrutura de capital. Este estudo teve como objetivo verificar quais determinantes possuem maior relevância no endividamento das empresas e se estes determinantes alteram-se dependendo do ciclo de vida da empresa apoiada pelas teorias Trade Off, Pecking Order e Teoria da Agência. Para alcançar o objetivo deste trabalho foi utilizado análise em painel de efeito fixo sendo a amostra composta por empresas brasileiras de capital aberto, com dados secundários disponíveis na Economática® no período de 2005 a 2013, utilizando-se os setores da BM&FBOVESPA. Como resultado principal destaca-se o mesmo comportamento entre a amostra geral, alto e baixo crescimento pelo endividamento contábil para o determinante Lucratividade apresentando uma relação negativa, e para os determinantes Oportunidade de Crescimento e Tamanho, estes com uma relação positiva. Para os grupos de alto e baixo crescimento alguns determinantes apresentaram resultados diferentes, como a singularidade que resultou significância nestes dois grupos, sendo positiva no baixo crescimento e negativa no alto crescimento, para o valor colateral dos ativos e benefício fiscal não dívida apresentaram significância apenas no grupo de baixo crescimento. Para o endividamento a valor de mercado foi observado significância para o Benefício fiscal não dívida e Singularidade. Este resultado reforça o argumento de que o ciclo de vida influência a estrutura de capital

Determinants of sales performance in insurance industry:a cross-cultural comparison between the UK and Malaysia

This thesis is concerned with an empirical investigation of the factors that predict a successful salesperson, using a cross-cultural comparison of two countries: the UK and Malaysia. Besides collecting quantitative data, qualitative data on organisational, environmental and cultural factors were also collected through interviews, personal and case observations. The quantitative data consist of sixteen independent factors and three dependent factors. The independent variables include self-efficacy, self-esteem, locus of control, self-monitoring, extrinsic motivation, intrinsic motivation, experience, training perception, role ambiguity, role conflict, role inaccuracy, gender, age, education, race and religion. The dependent variables are performance target achieved, performance earnings and performance ratings. Questionnaires were distributed to about 500 salespersons in each country, from three insurance companies in the UK and two insurance companies in Malaysia. Response rates were 75 and 50 percent from the UK and Malaysia respectively. The survey results indicated that a salesperson's performance in the UK is predicted by self-efficacy, internal locus of control, self-esteem, extrinsic motivation, experience, training perceptions, role conflict and gender. In Malaysia, a salesperson's performance is predicted by self-efficacy, self-monitoring, experience, role conflict, role ambiguity, education, gender, race and religion. Self-efficacy, experience, role conflict and gender are common predictors of salespersons' performance in both cultures. The likely explanation for these results is culture differences, i.e. UK has a homogeneous culture, while Malaysia has a heterogeneous one. Results from the case observations, such as organisational and environmental factors, give supporting evidence in explaining the empirical results. Implications from the findings are discussed from two aspects: (1) theoretical implications for divergence/convergence theory, Hofstede's model, Churchill's model, and (2) managerial implications for selection, training, motivation and appraisal.

The variance in sales performance explained by the knowledge structures of salespeople

The last major study of sales performance variance explained by salespeople attributes was by Churchill et al. (1985). They examined the effect of role, skills, motivation, personal factors, aptitude, and organizational/environmental factors on sales performance—factors that have dominated the sales performance area. About the same time, Weitz, Sujan, and Sujan (1986) introduced the concepts of salespeople's knowledge structures. Considerable work on the relationship of the elements of knowledge structures and performance can be found in the literature. In this research note, we determine the degree to which sales performance can be explained by knowledge structure variables, a heretofore unexplored area. If knowledge structure variables explain more variance than traditional variables, then this paper would be a call to further research in this area. In examining this research question in a retail context, we find that knowledge structure variables explain 50.2 percent of the variance in sales performance. We also find that variance explained by knowledge structures is significantly different based on gender. The impact of knowledge structures on performance was higher for men than for women. The models using education demonstrated smaller differences.

Stochasticity and coherent structures in ultra-long mode-locked lasers

Ultra-long mode-locked lasers are known to be strongly influenced by nonlinear interactions in long cavities that results in noise-like stochastic pulses. Here, by using an advanced technique of real-time measurements of both temporal and spatial (over round-trips) intensity evolution, we reveal an existence of wide range of generation regimes. Different kinds of coherent structures including dark and grey solitons and rogue-like bright coherent structures are observed as well as interaction between them are revealed.