Les formes psychiatriques de la maladie de Niemann-Pick de type C

Niemann-Pick type C disease diagnosed in adult neurology departments may be infantile or juvenile forms with prolongated life spans or forms starting at adolescence or adulthood. Psychomotor retardation is practically constant. In some cases, a psychosis may be the only manifestation for several years. The diagnosis is often made when extrapyramidal symptoms appear as well as cerebellar ataxia and ophtalmoplegia in the vertical gaze. The treatment with psychotropic drugs raises the question of a superimposition of a drug induced lipidosis. Hepatosplenomegaly is often discrete contrary to classical infantile cases. Foam cells as well as sea blue histiocytes are features of the disease. The filipin test evidences the lysosomal storage of cholesterol.     

Le suicide par saut et les stratégies de prévention

The topic of this article is to probe into the specificities of suicide by jumping. This means of suicide hasn't been studied much and isn't well known yet.We reviewed the international scientific literature so as to take an in-depth look at this type of auto-aggressive action and highlight the characteristics of the subjects likely to commit suicide by jumping. We thus focus on the available epidemiological data and risk factors associated to suicide jumping. We also describe the prevention strategies most often evoked to deal with this public health issue, which are respectively about improving safety on the identified suicide sites and providing medical and psychological care to individuals likely to commit suicide by jumping.This meta-analysis presents our critical analysis of the methodological limits in the studies exposed. We provide further hints for researching this field of study; they're based on both the taking into account of variables that could be risk factors for this particular means of suicide and the evaluation of the efficiency for the preventive measures used.     

La fonctionnalité alimentaire : illusion aujourd’hui, réalité demain

Foods have always been considered by man as a means of ensuring his physiological needs, allowing the growth, development and preservation of the body and its tissues (nutritional value of food). To this can be added the feelings of satisfaction and well-being that food gives to its consumer, thus constituting an element that is fundamental for our physiological and mental balance (the sensorial value of food).Nevertheless, recent scientific studies have shown that over and above the ensuring of nutritional needs, eating habits can also adjust certain functions of the human organism and thus play a beneficial or harmful role on one's health (the functional value of food).The whole concept of nutrition has been enriched by the notion that eating is not only a survival reflex (satisfaction derived through eating and the avoidance of harmful effects due to eating deficiencies or excesses): eating aims to improve one's health and well-being and to reduce the risk of developing various pathologies. These new data open interesting new horizons in today's context where health is increasingly expensive and people are increasingly concerned to improve their quality of live.     

Sinoatrial Node Electrogram Studies in Patients with Sick Sinus Syndrome

Sinoatrial node electrogram (SNE) was recorded successfully in recent years, using transvenous electrode catheter. Via SNE and intratrialelectrogram (IEG), one could measure sinoatrial conduction time(SACT) directly, observe sinoatrial node (SN) potential changes in cadiac cycles, diagnose some sinus arrhythmias which couldn't be confirmed by surface ECG. All these could offer accurate evidence for differential diagnosis of sick sinus syndrome (SSS). The authors recommend a modified method for the location of the electrode catheter, by which one can increase success rate of obtaining stable SNE. Using this method, the authors recorded SNE in 10 cases with SSS successfully.     

Metabolic and hormonal blood flow modeling in patients with coronary heart disease: In vitro and clinical study

The blood flow pattern investigations are of great importance in coronary blood flow destabilization pathogenesis understanding, and consequently in acute coronary event (ACE) risk stratification in patients with coronary heart disease. The aim of the study was to research the principal hormonal and metabolic blood flow regulative aspects and its structure in patients with ischemic heart disease and the contribution to cumulative ACE risk.A total of 182 patients with stable angina pectoris were included in the prospective study (2000–2006). Complex clinical examination, biochemical tests and blood tests were performed. Whole-blood (WB) viscosity, erythrocyte aggregation and deformation ability, WB suspension stability, and initial erythrocytes disaggregation parameter were studied. Dynamic characteristics of blood flow were obtained in the experiment.Received results allow marking the principle components of blood flow pattern with proven high prognostic value of ACE in patients with ischemic heart disease. ACE risk stratification program was developed.     

肝癌化疗栓塞术后综合征临床分析

本文分析报告192例各型肝癌化疗栓塞术后综合征发生情况:总发生率为89.1%,转氨酶升高发生率为77.2%,肝区疼痛64.1%,发热54.2%,恶心呕吐35.4%,腹胀38.0%,腹水形成19.8%,黄疸18. 8%,呃逆5.7%.结合我们的研究和经验讨论了肝癌化疗栓塞的病理生理改变,化疗栓塞术后综合征发生机理及临床对症处理方法.     

An evaluation of Tourette Syndrome and medication use in Canada

Self-report data were gathered from a national sample of over 200 Canadian Tourette Syndrome (TS) patients. Information regarding symptom severity both on and off medication was gathered along with an analysis of different medications in use, and patient ratings of effectiveness of those medications. Patients also rated their own mental health. Results indicated that approximately 60% of TS patients take some form of medication for relief from their symptoms. Of these, over 80% reported that symptoms are less severe when medicated. The most commonly prescribed medications in order of popularity are haloperidol, pimozide, clonidine and benztropine mesylate (Cogentin). Patient ratings of effectiveness of these medications places haloperidol first, pimozide second and clonidine third although all were found to be "somewhat" to "very" effective. Of those TS patients on medications, 50% rated their own mental health as good to excellent and 50% rated it as fair to poor.     

Intestinal microvillous atrophy in a patient with Down syndrome and aganglionic megacolon

Intestinal microvillous disorders are an uncommon cause of severe diarrhea, with very poor prognosis. The authors report the case of a female infant with Down syndrome, aganglionic megacolon, severe diarrhea, and jejunal biopsy with ultrastructural changes consistent with microvillous atrophy. The patient condition improved after a colostomy performed in the setting of the treatment of Hirschprung disease.     

Complex Ph1 translocation in chronic myeloid leukemia

This report describes a new case of chronic myeloid leukemia with an unusual Philadelphia chromosome translocation involving chromosomes No. 4,9, and 22; t(4,9,22) (q31;q34;q11).     

Antibodies to squalene in recipients of anthrax vaccine

We previously reported that antibodies to squalene, an experimental vaccine adjuvant, are present in persons with symptoms consistent with Gulf War Syndrome (GWS) (P. B. Asa et al., Exp. Mol. Pathol 68, 196-197, 2000). The United States Department of Defense initiated the Anthrax Vaccine Immunization Program (AVIP) in 1997 to immunize 2.4 million military personnel. Because adverse reactions in vaccinated personnel were similar to symptoms of GWS, we tested AVIP participants for anti-squalene antibodies (ASA). In a pilot study, 6 of 6 vaccine recipients with GWS-like symptoms were positive for ASA. In a larger blinded study, only 32% (8/25) of AVIP personnel compared to 15.7% (3/19) of controls were positive (P > 0.05). Further analysis revealed that ASA were associated with specific lots of vaccine. The incidence of ASA in personnel in the blinded study receiving these lots was 47% (8/17) compared to an incidence of 0% (0/8; P < 0.025) of the AVIP participants receiving other lots of vaccine. Analysis of additional personnel revealed that in all but one case (19/20; 95%), ASA were restricted to personnel immunized with lots of vaccine known to contain squalene. Except for one symptomatic individual, positive clinical findings in 17 ASA-negative personnel were restricted to 4 individuals receiving vaccine from lots containing squalene. ASA were not present prior to vaccination in preimmunization sera available from 4 AVIP personnel. Three of these individuals became ASA positive after vaccination. These results suggest that the production of ASA in GWS patients is linked to the presence of squalene in certain lots of anthrax vaccine.