Secondary muscular carnitine deficiency following immunosuppressive treatment

Carnitine deficiency syndromes can be classified into two groups: primary carnitine deficiency and secondary carnitine deficiency syndromes. A lipid storage myopathy with carnitine deficiency following an immunosuppressive therapy is described in a young man suffering from a possible polymyositis. After treatment with L-carnitine both biochemical and morphological features recovered. A secondary carnitine deficiency syndrome due to an immunosuppressive therapy is supposed.     

Assessing mobility in an aging society: Personal and built environment factors associated with older people’s subjective transportation deficiency in the US

This study analyzed subjective transportation deficiency, in terms of missing activities due to lack of transportation, of the older population aged 65 and over in the United States. This study found that those who have lower personal automobile accessibility and minority females are more likely to experience transportation deficiency. Older people who have lower income, have lived for a relatively short period (3–10 years) in their communities, and live with minors are also more likely to experience lack of transportation. Older people who live in suburban communities have greater transportation deficiency, but the placement of activity locations within walking distance can improve transportation deficiency among older people who do not drive. The availability of public transit services within walking distance and knowledge about transportation alternatives do not significantly affect the transportation deficiency of the older population. The results indicate that a substantive focus should be given to how to help the older population keep driving as long as they can, and aid communities with high concentrations of older minority females. Also, developing activity clusters containing commercial and social service facilities in suburban areas with high concentrations of older populations, has the potential to alleviate transportation deficiency among this widely observed group of people who age in place.     

Approaching Emptiness: Subjective,Objective and Existential Dimensions

Clinicians have lacked a coherent approach to emptiness, which is both a pervasive metaphor for loss, deficiency, or alienation and a frequently cited spiritual goal. We suggest a framework for approaching emptiness that distinguishes among its subjective, objective, and existential dimensions. Clinicians can use psychodynamic and cognitive behavioral approaches to clarify schemas that distort patients’ perceptions of others and of themselves, behavioral and relational approaches to help them deal with real deficiency and loss, and spiritually oriented approaches to put these into a larger context.     

Deficiency arguments against empiricism and the question of empirical indefeasibility

I give a brief overview of Albert Casullo’s Essays on A Priori Knowledge and Justification (2012), followed by a summary of his diagnostic framework for evaluating accounts of a priori knowledge and a priori justification. I then discuss Casullo’s strategy for countering deficiency arguments against empiricism. A deficiency argument against empiricism can be countered by mounting a parallel argument against moderate rationalism that shows moderate rationalism to be defective in a similar way. I argue that a particular deficiency argument put forth by George Bealer in “The Incoherence of Empiricism” (1992) can withstand a parallel challenge mounted by Casullo (2012, Ch.6).     

The cognitive and social-emotional correlates of color deficiency in children: a literature review and analysis.

Although color deficiency is a prevalent problem in childhood, little is known about the cognitive and social-emotional variables linked to this visual deficit. A review of the literature revealed just seven empirical articles designed to explore the psychological functioning of color-deficient children, with the results yielding contradictory findings. An analysis of the literature indicated that the equivocal nature of past research may be due to an inadequate rationale explaining what outcome variables should be tied to color deficiency. This weakness can be remedied, and as a means toward that end, specific investigative recommendations are made to provide a more valid theoretical link between color deficiency and selected cognitive and social-emotional variables.     

Alcohol-related amnesia and dementia: animal models have revealed the contributions of different etiological factors on neuropathology, neurochemical dysfunction and cognitive impairment

Chronic alcoholism is associated with impaired cognitive functioning. Over 75% of autopsied chronic alcoholics have significant brain damage and over 50% of detoxified alcoholics display some degree of learning and memory impairment. However, the relative contributions of different etiological factors to the development of alcohol-related neuropathology and cognitive impairment are questioned. One reason for this quandary is that both alcohol toxicity and thiamine deficiency result in brain damage and cognitive problems. Two alcohol-related neurological disorders, alcohol-associated dementia and Wernicke-Korsakoff syndrome have been modeled in rodents. These pre-clinical models have elucidated the relative contributions of ethanol toxicity and thiamine deficiency to the development of dementia and amnesia. What is observed in these models--from repeated and chronic ethanol exposure to thiamine deficiency--is a progression of both neural and cognitive dysregulation. Repeated binge exposure to ethanol leads to changes in neural plasticity by reducing GABAergic inhibition and facilitating glutamatergic excitation, long-term chronic ethanol exposure results in hippocampal and cortical cell loss as well as reduced hippocampal neurotrophin protein content critical for neural survival, and thiamine deficiency results in gross pathological lesions in the diencephalon, reduced neurotrophic protein levels, and neurotransmitters levels in the hippocampus and cortex. Behaviorally, after recovery from repeated or chronic ethanol exposure there is impairment in working or episodic memory that can recover with prolonged abstinence. In contrast, after thiamine deficiency there is severe and persistent spatial memory impairments and increased perseverative behavior. The interaction between ethanol and thiamine deficiency does not produce more behavioral or neural pathology, with the exception of reduction of white matter, than long-term thiamine deficiency alone.     

The Impact of Time‐Keeping Direction on Compensatory Consumption: The Role of Perceived Resource Deficiency

Time is a scarce resource, and time‐keeping has become a common part of our lives. This research investigates the impact of exposure to a specific direction of time‐keeping on resource deficiency perception and on compensatory consumption of calories. Results from four studies show that exposure to downward time‐keeping (e.g., 60→1 s), compared to upward time‐keeping (e.g., 1→60 s), results in a higher perceived resource deficiency, leading to a higher preference, consumption, and choice of caloric foods. The effect is attenuated for evaluation of calorie‐lean foods, or by recalling instances of resourcefulness, or when time is not a resource (as in the case of waiting). Related alternate explanations are addressed. This research is the first to identify a novel antecedent of resource deficiency (i.e., time‐keeping direction), and to elicit an unexplored aspect of temporal cognition, that exposure to direction of time‐keeping can influence compensatory consumption in an unrelated domain. These findings contribute to the literature on temporal cognition and resource deficiency and may inspire further research in these domains.     

The Evolution and Treatment of Korsakoff's Syndrome

Wernicke's Encephalopathy is an acute neuro-psychiatric condition caused by an insufficient supply of thiamine (Vitamin B1) to the brain. If undiagnosed or inadequately treated, it is likely to proceed to Korsakoff's Syndrome. Wernicke's Encephalopathy can result from dietary deficiency alone and this form is usually successfully treated, with little chance of Korsakoff's Syndrome supervening. On the other hand, thiamine deficiency associated with alcohol misuse/dependence may require up to 1 gram of thiamine IV in the first 24 hours to be treated successfully. The reasons for this difference in treatment will be discussed. Thiamine diphosphate acts as a co-factor for a number of thiamine-dependent enzymes. Thiamine deficiency leads to a reduction in the activity of these enzymes, and this leads to alterations in mitochondrial activity, impairment of oxidative metabolism, decreased energy status and eventually selective neuronal death. The damage caused by the combination of thiamine deficiency and alcohol metabolism probably interferes with adequate thiamine transport at a number of sites in the body, including the blood-brain barrier, as well as causing damage to the apoenzymes which then require higher concentrations of thiamine to work normally. The accumulated damage is likely to render the use of oral thiamine therapeutically inadequate since the body is unable to produce high enough concentrations of thiamine in the blood to traverse the blood-brain barrier. Some individuals are probably genetically predisposed to develop Wernicke's. Long before individuals with alcohol misuse or dependence develop Wernicke's Encephalopathy the neurons and other cells of the body are functioning sub-optimally because of the inadequate supply of thiamine and the neurotoxic effect of alcohol. This relative deficiency initiates a series of pathological changes which accumulate and further interfere with the supply of thiamine and its utilisation at a time when the requirements are increased. The best treatment for Korsakoff's Syndrome is timely recognition of Wernicke's Encephalopathy and appropriate intervention and prevention.     

Libido and hormones

Libido is the drive to have a sexual activity. Gonadal hormones play a major role in activating and maintaining libido in both men and women. Other hormones, though, interact with them in influencing sexuality, such as prolactin and also brain neurotransmitters. The role of hormones declines with age and sexuality becomes more mind-induced. Nevertheless, some aspects of sexuality remain linked to hormones. For example, a reduction of central arousability is typical of hypogonadal state. However, it is not clear at what level of androgen deficiency the loss of libido begins and if adequate external stimuli can overcome a partial deficiency.     

Judging health status: effects of perceived prevalence and personal relevance

In this article, we show that people's evaluations of the seriousness of a health disorder are influenced by the perceived prevalence and personal relevance of that disorder. As part of a study ostensibly concerned with college students' health characteristics, 60 undergraduates were "tested" for the presence of a fictitious enzyme deficiency. The subjects discovered either that they had the deficiency (deficiency-present subjects) or that they did not have it (deficiency-absent subjects), and were led to believe either that 1 of the 5 people in the laboratory had the deficiency (low-prevalence subjects) or that 4 of them had it (high-prevalence subjects). As predicted, the low-prevalence subjects evaluated the deficiency as more serious than did the high-prevalence subjects. In addition, consistent with the view that personal relevance affects perceptions of health disorders, the deficiency-present subjects evaluated the deficiency as less serious than did the deficiency-absent subjects. The deficiency-present subjects also derogated the validity of the test ostensibly used to diagnose the deficiency compared with other subjects. Finally, the deficiency-present subjects requested more information about the deficiency than did the deficiency-absent subjects.     

Suppose Yalcin is wrong about epistemic modals

In “Epistemic Modals,” Seth Yalcin argues that what explains the deficiency of sentences containing epistemic modals of the form ‘p and it might be that not-p’ is that sentences of this sort are strictly contradictory, and thus are not instances of a Moore-paradox as has been previous suggested. Benjamin Schnieder, however, argues in his Yalcin’s explanation of these sentences’ deficiency turns out to be insufficiently general, as it cannot account for less complex but still defective sentences, such as ‘Suppose it might be raining.’ Consequently, Schnieder proposes his own, expressivist treatment of epistemic modals which he thinks can explain the deficiency of both the original sentence type as well as more complex cases of embedded sentences containing epistemic modals. In this study, I argue that although Schnieder is right to draw our attention to the explanatory failure of Yalcin’s account, we aren’t forced to adopt Schnieder’s expressivist account of epistemic modals. I defend instead a contextualist-friendly alternative which explains the deficiencies of all the relevant sentence types, while avoiding both the defects of Yalcin’s account and the intuitive costs of expressivism.     

安乐死是何种权利?——关于安乐死的法伦理学解读

安乐死是能为人们带来人格利益的道德权利。我们没有合理的理由对其做出限制 ,但它却很难成为一种法定的权利。主要的阻碍是立法的客观条件不具备 ,以及法律天生的对人性的忧虑。这意味着 ,安乐死作为一种应有的权利要转化为法定权利 ,尽管不是没有可能 ,但要有十分的耐心和谨慎。     

中医药对牙周病的研究和治疗思路

中医药对牙周病的研究和治疗已有悠久的历史,它与牙周基础治疗结合可以获得良好的治疗效果,可弥补单纯西医在治疗牙周病时偏重局部的缺陷。对中医药对牙周病研究和治疗的历史和进展情况作了简要综述,并就中医药对牙周病的治疗思路和中西医结合治疗牙周病的科学合理性进行了探讨。     

孤独症碎镜理论述评

孤独症是泛指一大类在社会交往、沟通方面存在功能缺陷的疾病, 至今病因尚未阐明。“碎镜理论”作为孤独症研究领域的新兴理论, 在神经—认知层面较先前理论更为全面地解释了孤独症的各种临床行为表现, 并为这些异常行为间的相互关系提供了一种统一性的研究视角。文章首先对镜像神经元的发现进行了简介, 在此基础上对碎镜理论的由来及其与孤独症相关功能(包括动作识别与模仿、心理理论、共情以及语言理解)缺陷的关系进行了综述, 最后通过区分主动模仿和自动模仿, 就镜像神经元系统及其调控系统与孤独症关系中存在的问题进行了反思与展望。     

Wernicke’s Encephalopathy: Expanding the Diagnostic Toolbox

Wernicke's encephalopathy (WE) is a life threatening neurological disorder that results from thiamine (Vitamin B1) deficiency. Clinical signs include mental status changes, ataxia, occulomotor changes and nutritional deficiency. The conundrum is that the clinical presentation is highly variable. WE clinical signs, brain imaging, and thiamine blood levels, are reviewed in 53 published case reports from 2001 to 2011; 81 % (43/53) were non-alcohol related. Korsakoff Syndrome or long-term cognitive neurological changes occurred in 28 % (15/53). Seven WE cases (13 %) had a normal magnetic resonance image (MRI). Four WE cases (8 %) had normal or high thiamine blood levels. Neither diagnostic tool can be relied upon exclusively to confirm a diagnosis of WE.     

A Case of Partial Biotinidase Deficiency Associated With Autism

We report the case of a child with partial biotinidase deficiency and autistic developmental disorder. We arrived at the diagnosis of biotinidase deficiency when the child was almost 4 years of age. Consequently, he began cofactor biotin treatment (10 mg daily) which did not resolve his autistic behavior. His younger brother was affected by partial biotinidase deficiency diagnosed at birth through our neonatal screening program. He was precociously treated with cofactor biotin therapy (10 mg daily) and did not show any behavioral abnormality or developmental delay. Since the brain is quite vulnerable to biotin deficiency, delayed biotin therapy could result in neurological damage. Our patient is the first case of partial biotinidase deficiency associated with autism. We hypothesize that the low biotinidase activity could have caused biotin deficiency in his brain and cerebrospinal fluids and consequently serious neurological problems, such as stereotyped and autistic behaviors, which were irreversible in spite of biotin supplementation.     

A case of partial biotinidase deficiency associated with autism.

We report the case of a child with partial biotinidase deficiency and autistic developmental disorder. We arrived at the diagnosis of biotinidase deficiency when the child was almost 4 years of age. Consequently, he began cofactor biotin treatment (10 mg daily) which did not resolve his autistic behavior. His younger brother was affected by partial biotinidase deficiency diagnosed at birth through our neonatal screening program. He was precociously treated with cofactor biotin therapy (10 mg daily) and did not show any behavioral abnormality or developmental delay. Since the brain is quite vulnerable to biotin deficiency, delayed biotin therapy could result in neurological damage. Our patient is the first case of partial biotinidase deficiency associated with autism. We hypothesize that the low biotinidase activity could have caused biotin deficiency in his brain and cerebrospinal fluids and consequently serious neurological problems, such as stereotyped and autistic behaviors, which were irreversible in spite of biotin supplementation.     

NUCLEAR VIOLENCE: A PHILOSOPHICAL FRAMEWORK FOR THE PROBLEM

This has been more a programmatic than a substantive contribution to the moral problem of nuclear violence. Substantive issues of technology and tactics, of conventional and deterrent strategy, of political and social norms, of critical and theoretical foundations must all be addressed before there can be an adequate solution to this problem, not to speak of the revision of ethics it entails. These are, however, other questions. For lack of a comprehensive framework they have not always been properly addressed. The framework I have outlined may supply for that deficiency.     

Denial and Confirmatory Search: Paradoxical Consequences of Medical Diagnosis1

An experiment was conducted to examine the effects of medical diagnostic-test results on illness appraisal and the recall of symptoms and behavior. Subjects were tested for a fictitious enzyme deficiency and were told either that the test result was positive (deficiency present) or that it was negative (deficiency absent). In addition, some of the subjects were told the test was accurate 95% of the time and others were told it was accurate 75% of the time. As predicted, subjects judged the enzyme deficiency as less serious and more prevalent when presented with positive test results. Subjects with positive test results also recalled more behaviors that had been labeled as risk factors. Although positive test subjects tended to report more deficiency-related symptoms, diagnosis did not affect the free recall of general symptoms. In addition, information concerning the reliability of the diagnostic test had no effect on judgments or recall but did affect information seeking. Subjects with positive test results were less likely to request a definitive follow-up test when their results were unreliable. The results are interpreted as evidence for independent confirmatory search and denial processes following medical diagnosis.