Pediatric familial type III hyperlipoproteinemia.

Familial type III hyperlipoproteinemia is almost always first diagnosed in adults; the two 16-yr-old probands with type III in this report represent only the fifth and sixth children reported with the disorder. S.E., a 16-yr-old female, and C.H., a 21-yr-old male, (with palmar xanthomas at age 16) had beta-migrating abnormal very low density lipoproteins (beta-VLDL), density less than 1.006. S.E.'s brother (age 21) and mother (age 57) had type III; her grandmother had beta-VLDL and elevated triglycerides, and a maternal uncle had type IV hyperlipoproteinemia. C.H.'s father had type III; a sister and paternal aunt had type IV hyperlipoproteinemia. It is important to consider the diagnosis of type III hyperlipoproteinemia in the pediatric age group, particularly in children from families with type III hyperlipoproteinemia.     

癫痫外科研究生临床教学的体会

通过对神经外科研究生的临床教学,做好癫痫临床教学工作必须做到：临床教学前的准备工作,让研究生对癫痫外科观念有直观的了解;在教学中教师应正确指导学生的问诊、体格检查;培养学生癫痫外科定位诊断的临床思维;同时要做好教学后病例总结工作。     

225例结直肠锯齿状腺瘤的临床、内镜及病理学特征分析

目的探讨结直肠锯齿状腺瘤(serrated adenoma,SA)的临床、内镜及病理学特征。方法回顾性分析北京军区总医院消化内镜中心2009年1月-2013年10月检出的225例结直肠SA的临床、内镜及病理学资料。结果全部患者中男148例,女77例,年龄19~89岁,平均年龄(53.5±14.3)岁。以单发型多见(87.1%)。在同期结肠镜中的检出率为2.1%。内镜下形态以扁平型和广基型居多(64.3%、20.0%),多数病变直径在10 mm以下(83.1%);左半结肠和右半结肠的病变大小和形态分布的差异均有统计学意义(χ2=14.2662、12.2168,P0.05)。全部病例中广基锯齿状腺瘤息肉(SSA/P)30例(13.3%),非广基锯齿状腺瘤(包括增生性息肉、传统锯齿状腺瘤)195例(86.7%)。SSA/P中有20例位于右半结肠(66.7%),平均直径13.3 mm。其中6例SSA/P呈侧向发育型息肉,均位于升结肠及回盲部。伴腺上皮异型增生12例,另有3例腺瘤癌变。结论结直肠SA临床相对少见,内镜下形态及分布部位有其自身特点,尤其是SSA/P。有必要对结直肠SA进行明确分类,并对其发展、转归做长期随访研究。     

A case series of LMWH use in pregnancy: Should trough anti-Xa levels guide dosing?

Introduction

Pregnancy is a thrombogenic state, increasing the risk for venous thromboembolism (VTE), and the risk of valve thrombosis amongst women with mechanical heart valves (MHV). While low molecular weight heparins (LMWH) are generally dosed based on weight (i.e., enoxaparin 1 mg/kg every 12 hours), data in pregnant women have shown that weight-based dosing does not consistently achieve target anti-Xa levels. In women with MHV, our practice includes titrating LMWH doses to target both trough and peak anti-Xa levels, while for those with VTE peak anti-Xa levels guide dosing.

Materials/Methods

This retrospective case series included pregnant women requiring LMWH treatment doses with at least 3 peak (+/− trough) anti-Xa levels. Our primary objective was to describe the actual LMWH dose required to achieve targeted anti-Xa levels relative to weight-based dosing in patients with MHV. Secondarily, we compared the same for VTE patients; compared actual dosing between those with MHV and VTE; and examined maternal and fetal outcomes.

Results/Conclusion

Women with MHV (N = 4) required greater than weight-based dosing of enoxaparin (1.35 mg/kg Q12H) to achieve targeted anti-Xa levels. Importantly, achieving target peak anti-Xa levels did not always ensure maintenance of minimum trough levels. VTE patients (N = 12) did not require more enoxaparin (0.96 mg/kg Q12H) than weight based dosing. MHV patients received more enoxaparin compared to VTE patients (P < 0.001). No bleeding or clotting complications were associated with LMWH administration. In pregnant women with MHV at high risk of thromboembolism, LMWH dosing guided by trough and peak anti-Xa levels should be considered.     

老年发热患者的临床特点及病因分析研究

目的分析老年人发热性疾病的病因与临床特征。方法收集中关村医院2009-01—2010-11收治的以发热为首发临床表现的符合发热诊断标准的患者60例,进行回顾性分析。结果 60例患者中最终明确诊断者55例,其中感染性疾病30例、恶性肿瘤19倒、风湿性疾病3例、其他疾病3例。结论老年人发热性疾病由于症状不典型,诊断较困难。感染性疾病仍然是老年人主要的发热原因。     

Primary sleep disorders seen at a Neurology service-based sleep clinic in India: Patterns over an 8-year period

There is an increasing awareness for recognition of sleep disorders in India; however, there is still a huge gap in the number of people suffering from various sleep disorders, in the community versus those visiting hospital clinics for the same. Ours is a neurology services-based sleep disorders clinic, which has evolved successfully over the last decade. In this study, we aimed to evaluate the changes in referral patterns and distribution of various sleep disorders in the patients presenting to the clinic.

Materials and Methods:

This is a retrospective chart review-based study on all patients seen over an 8-year period, divided into 2 groups comprising of patients seen during the first 4 years versus those seen over the next 4 years. Only those patients who had the sleep disorder as their presenting manifestation and those who had been formally interviewed with a pre-structured questionnaire detailing about the main features of the common sleep disorders according to the ICSD-R were included. Patients, in whom the sleep disorder could be clearly attributable to another neurological or systemic disorder, were excluded. Statistical analysis was carried out to identify the differences between the two groups as regards the distribution of various sleep disorders and other clinical data.

Results:

Among 710 patients registered in the clinic, 469 were included for analysis and 222 patients formed group 1 while 247 formed group 2. The main differences observed were in the form of a clear increase in the percentage of patients with sleep-related breathing disorders, sleep-related movement disorder, and the hypersomnias on comparison of distribution over the first 4 years versus the last 4 years; while a clear decline was seen in the number of patients with insomnia and parasomnias. A 3-fold increase was observed in the number of patients in whom polysomnography was obtained.

Conclusion:

The distribution of various sleep disorders as seen in a neurology service-based sleep clinic is demonstrated in this study. Increasing referrals for sleep-disordered breathing, restless legs syndrome, and fewer referrals for insomnia and parasomnias might reflect on changing physician and patient awareness in our community.     

Jean Garrabé et l’histoire de la psychiatrie dans les Annales Médico-Psychologiques

Jean Garrabé has published about 40 historical articles in the Annales Médico-Psychologiques. He has written on the history of the classifications, on the evolution of clinical entities (bipolar disorders) and of theoretical matters (psychopathology, politics, mondialization, antipsychiatry), on the relationships of psychiatry with artistic, literary and musical creation. But he has above all written many biographical notices of practitioners of mental medicine in France from the beginning of the 17th century (Montalto) to the end of the 20^th (Ey, Lacan). The personage of Pinel often appears in his papers like a central figure of the psychiatry, analyzed far from both hagiography and demolition. Garrabé can be considered like a “psychiatrist-historian”, erudite as well as critical.     

Vers une psychiatrie énactive et computationnelle

IntroductionPsychiatry is challenged by a plurality of complementary approaches. These challenges stem from the existence of multiple levels of understanding, i.e. systems of representations, tools, methodologies and objectives in psychiatry–ranging from computational approaches and systems dynamics to the multiplicity of emerging nosographies, such as the NIMH Research Domain Criteria project or staging models. In this plurality, a significant number of clinicians have adopted the biopsychosocial model. However, such a model has been widely criticized for more than twenty years. In parallel, science has declined a set of different pluralistic frameworks. Thus, through the challenges of computational modeling in psychiatry, we will see how the enactive approach of psychiatry could respond to this multiplicity. Indeed, such an enactive approach considers that perception is a (predictive) activity, which gives sense to the environment (i.e., sense making). Perception and, by extension, cognitive processes are not internal representations of the outside world, but they are deployed according to the 5E approach, i.e., an embodied, embedded, enacted, emotive and extended approach.MethodsIn this article, we first study the pluralist framework in psychiatry, in order to show its contributions in the clinical practice. Secondly, we analyze the contributions of the enactive approach for clinical practice in psychiatry.ResultsTwo forms of pluralisms can be described: a non-integrative pluralism and an integrative pluralism. The first examines the coexistence of different potentially incompatible or untranslatable systems in the scientific or clinical landscape. The second proposes the development of a general framework, bringing together the different levels of understanding and systems of representations. However, pluralism has many pitfalls and limitations. Especially by allowing computational modeling, the enactive framework, anchored both in cognitive sciences, theory of dynamic systems, systems biology and phenomenology, has recently been proposed as an answer to the challenge of integrative psychiatry.ConclusionsA significant number of mental health professionals are already working accepting such a variety of clinical and scientific approaches. We show that the enactive approach allows psychiatry: (1) to consider the subjectivity and the patient's experience, (2) to articulate different “granularities” within the clinical consultation, (3) to explain the benefits the creation of meaning for the patient, (4) to provide concrete models, (5) to support pedagogy in psychiatry. The enactive approach provides a conception for understanding psychiatric disorders as embodied, embedded, enacted, emotional and extended. In that way, the manifestations experienced by the patients are sense making experiences and can be conceived according to various levels of granularity.     

2016年滦南县放射工作人员外照射个人剂量及放射诊疗设备现状分析

目的 了解滦南县放射工作人员外照射个人剂量水平及放射诊疗设备现状,为评价放射工作场所防护现状提供依据。方法 按照《职业性外照射个人监测规范》（GBZ 128-2016）的要求,采用热释光剂量法对滦南县所有医疗卫生机构放射工作人员外照射进行监测。结果 滦南县共有放射诊疗单位30家,放射工作人员146人,实际监测人员131人,监测率89.73%。131人的集体年剂量当量为51.97 mSv人,人均年剂量当量为0.40 mSv。其中128人年剂量当量低于2 mSv,3人年剂量当量在2~5 mSv之间,占2.29%,年剂量当量值均无超过5 mSv。县级医院人均年剂量当量为0.36 mSv,乡镇级医院人均年剂量当量为0.44 mSv,牙科门诊人均年剂量当量为0.34 mSv,个体诊所人均年剂量当量为0.31 mSv。放射工作人员中以普通放射工作人员为主,共计97人,占74.05%,人均年剂量当量0.38 mSv;从事CT人员19人,人均年剂量当量0.50 mSv;从事介入人员9人,人均年剂量当量0.24 mSv;专职牙科人员4人,人均年剂量当量0.34 mSv;放射治疗2人,人均年剂量当量1.04 mSv。经比较,放射治疗与其他放射工种的人均年剂量当量差异有统计学意义（P<0.05）。结论 全县放射工作人员人均年有效剂量均远远低于国家标准限值,表明放射职业环境相对安全。     

小儿肺炎235例病原学临床分析

目的：对小儿肺炎进行病原学分析．以指导临床选用抗生素。方法采用无菌吸痰管连接负压吸引器吸取848例小儿肺炎鼻咽深部的痰液，在2h内进行痰液涂片与培养。结果阳性标本为235份．阳性率为27．71％。检出细菌10余种，其中流感嗜血杆菌65例、肠杆菌35例、肺炎克雷伯氏菌30例、表皮葡萄球菌28例、金黄色葡萄球菌25例、不动杆菌13例．其它包括链球菌、绿脓假单胞菌、白色念珠菌、副流感杆菌等共39例。结论细菌感染是小儿肺炎的重要原因，流感嗜血杆菌、肠杆菌、肺炎克雷伯氏菌、表皮葡萄球菌、金黄色葡萄球菌是主要的致病菌；季节不同各种细菌所占比例也不同。