Characteristics of infections associated with external ventricular drains of cerebrospinal fluid.

OBJECTIVES: Manifestations of external ventricular drain (EVD) - associated infections overlap with those of the underlying neurosurgical conditions. We analyzed characteristics of EVD-associated infections. METHODS: We included patients aged ≥18 years with EVD-associated infections from 1997 to 2008, using modified CDC criteria for nosocomial infections. Hospital charts were reviewed retrospectively and the in-hospital outcome was evaluated. RESULTS: Forty-eight patients with EVD-associated infections were included (median age, 52 years, range 20-74 years). The median EVD-indwelling time was 7 days (range, 1-39 days) and EVD-associated infection occurred 6 days after insertion (range, 1-17 days). In 23% of patients, meningitis occurred 1-10 days after EVD removal. Fever >38 °C was present in 79% of patients, but Glasgow Coma Scale (GCS) scores were reduced in only 29%, and headache, vomiting and/or neck stiffness were present in only 31%. The median cerebrospinal fluid (CSF) leukocyte count was higher at onset of EVD-associated infection than at EVD insertion (175 × 10(6)/l versus 46 × 10(6)/l, p = 0.021), but other CSF parameters did not differ significantly. The most commonly implicated organisms were coagulase-negative staphylococci (63%) and Propionibacterium acnes (15%). CONCLUSIONS: Fever and increased CSF leukocytes should raise the suspicion of EVD-associated infection, which may occur up to 10 days after removal of EVD.

Perry Syndrome

Disease characteristics. Perry syndrome is characterized by parkinsonism, hypoventilation, depression, and weight loss. The mean age at onset is 48 years; the mean disease duration is five years. Parkinsonism and psychiatric changes (depression, apathy, character changes, and withdrawal) tend to occur early; severe weight loss and hypoventilation manifest later. Diagnosis/testing. The diagnosis is based on clinical findings and molecular genetic testing of DCTN1, the only gene known to be associated with Perry syndrome. Management. Treatment of manifestations: Dopaminergic therapy (particularly levodopa/carbidopa) should be considered in all individuals with significant parkinsonism. Although response to levodopa is often poor, some individuals may have long-term benefit. Noninvasive or invasive ventilation support may improve quality of life and prolong life expectancy. Those patients with psychiatric manifestations may benefit from antidepressants and psychiatric care. Weight loss is managed with appropriate dietary changes. Surveillance: routine evaluation of weight and calorie intake, respiratory function (particularly at night or during sleep), strength; and mood. Agents/circumstances to avoid: Central respiratory depressants (e.g., benzodiazepines, alcohol). Genetic counseling. Perry syndrome is inherited in an autosomal dominant manner. The proportion of cases attributed to de novo mutations is unknown. Each child of an individual with Perry syndrome has a 50% chance of inheriting the mutation. No laboratories offering molecular genetic testing for prenatal diagnosis are listed in the GeneTests Laboratory Directory; however, prenatal testing may be available through laboratories offering custom prenatal testing for families in which the disease-causing mutation has been identified.

Des personnages érigés en narrateurs : les voix over chez Joseph L. Mankiewicz (A Letter to Three Wives, All about Eve et The Barefoot Contessa). Pour une approche énonciative du récit filmique

L'auteur analyse dans une perspective narratologique trois films de Joseph L. Mankiewicz réalisés entre 1949 et 1954 (A Letter to Three Wives, All about Eve et The Barefoot Contessa), afin d'observer les particularités qui résultent du recours à un ou plusieurs narrateurs (ou narratrices) s'exprimant en voix over. L'accent est mis sur les implications de l'organisation énonciative complexe de ces productions cinématographiques en termes de relation du film au spectateur et de représentation des rapports de genre. Ces études de cas permettent de nuancer certains acquis issus du champ des théories de l'énonciation. Dans ces films où les voix over sont proférées par des acteurs qui incarnent par ailleurs un personnage visualisé, certaines interactions sont observables - interprétées notamment comme des relations de pouvoir - entre le statut des narrateurs et le niveau diégétique. La matérialité sonore est prise en compte dans le cas particulier de Letter to Three Wives, où la voix se transforme en bruit. Cet « effet spécial » est l'occasion de discuter un modèle « impersonnel » de l'énonciation filmique qui intègre, tout en tenant compte de la dimension technologique de l'enregistrement sonore, le pouvoir fondamentalement humanisant des manifestations vocales.

Predictors of temporary and permanent work disability in patients with inflammatory bowel disease: results of the swiss inflammatory bowel disease cohort study.

BACKGROUND: Inflammatory bowel disease can decrease the quality of life and induce work disability. We sought to (1) identify and quantify the predictors of disease-specific work disability in patients with inflammatory bowel disease and (2) assess the suitability of using cross-sectional data to predict future outcomes, using the Swiss Inflammatory Bowel Disease Cohort Study data. METHODS: A total of 1187 patients were enrolled and followed up for an average of 13 months. Predictors included patient and disease characteristics and drug utilization. Potential predictors were identified through an expert panel and published literature. We estimated adjusted effect estimates with 95% confidence intervals using logistic and zero-inflated Poisson regression. RESULTS: Overall, 699 (58.9%) experienced Crohn's disease and 488 (41.1%) had ulcerative colitis. Most important predictors for temporary work disability in patients with Crohn's disease included gender, disease duration, disease activity, C-reactive protein level, smoking, depressive symptoms, fistulas, extraintestinal manifestations, and the use of immunosuppressants/steroids. Temporary work disability in patients with ulcerative colitis was associated with age, disease duration, disease activity, and the use of steroids/antibiotics. In all patients, disease activity emerged as the only predictor of permanent work disability. Comparing data at enrollment versus follow-up yielded substantial differences regarding disability and predictors, with follow-up data showing greater predictor effects. CONCLUSIONS: We identified predictors of work disability in patients with Crohn's disease and ulcerative colitis. Our findings can help in forecasting these disease courses and guide the choice of appropriate measures to prevent adverse outcomes. Comparing cross-sectional and longitudinal data showed that the conduction of cohort studies is inevitable for the examination of disability.

Reversible behavioural autistic-like regression: A manifestation of a special (new?) epileptic syndrome in a 28-month-old child. A 2-year longitudinal study .

A 28-month-old boy was referred for acute onset of abnormal head movements. History revealed an insidious progressive regression in behaviour and communication over several months. Head and shoulder 'spasms' with alteration of consciousness and on one occasion ictal laughter were seen. The electroencephalograph (EEG) showed repeated bursts of brief generalized polyspikes and spike-wave during the 'spasms', followed by flattening, a special pattern which never recurred after treatment. Review of family videos showed a single 'minor' identical seizure 6 months previously. Magnetic resonance imaging was normal. Clonazepam brought immediate cessation of seizures, normalization of the EEG and a parallel spectacular improvement in communication, mood and language. Follow-up over the next 10 months showed a new regression unaccompained by recognized seizures, although numerous seizures were discovered during the videotaped neuropsychological examination, when stereotyped subtle brief paroxysmal changes in posture and behaviour could be studied in slow motion and compared with the 'prototypical' initial ones. The EEG showed predominant rare left-sided fronto-temporal discharges. Clonazepam was changed to carbamazepin with marked improvement in behaviour, language and cognition which has been sustained up to the last control at 51 months. Videotaped home observations allowed the documentation of striking qualitative and quantitative variations in social interaction and play of autistic type in relation to the epileptic activity. We conclude that this child has a special characteristic epileptic syndrome with subtle motor and vegetative symptomatology associated with an insidious catastrophic 'autistic-like' regression which could be overlooked. The methods used to document such fluctuating epileptic behavioural manifestations are discussed.

Brittle cornea syndrome: recognition, molecular diagnosis and management.

Brittle cornea syndrome (BCS) is an autosomal recessive disorder characterised by extreme corneal thinning and fragility. Corneal rupture can therefore occur either spontaneously or following minimal trauma in affected patients. Two genes, ZNF469 and PRDM5, have now been identified, in which causative pathogenic mutations collectively account for the condition in nearly all patients with BCS ascertained to date. Therefore, effective molecular diagnosis is now available for affected patients, and those at risk of being heterozygous carriers for BCS. We have previously identified mutations in ZNF469 in 14 families (in addition to 6 reported by others in the literature), and in PRDM5 in 8 families (with 1 further family now published by others). Clinical features include extreme corneal thinning with rupture, high myopia, blue sclerae, deafness of mixed aetiology with hypercompliant tympanic membranes, and variable skeletal manifestations. Corneal rupture may be the presenting feature of BCS, and it is possible that this may be incorrectly attributed to non-accidental injury. Mainstays of management include the prevention of ocular rupture by provision of protective polycarbonate spectacles, careful monitoring of visual and auditory function, and assessment for skeletal complications such as developmental dysplasia of the hip. Effective management depends upon appropriate identification of affected individuals, which may be challenging given the phenotypic overlap of BCS with other connective tissue disorders.

Manifestació dels canvis en la interacció entre el medi natural i la societat: la influència del despoblament a Alins i Farrera

L’objectiu principal d’aquest projecte és analitzar les manifestacions dels canvis produïts en els medis natural i social influenciats pel despoblament (a la segona meitat del s.XX), als municipis d’Alins i Farrera, situats al Pallars Sobirà, i fer-ne una comparació, ja que es tracta de municipis de característiques diferents. Per dur a terme l’estudi, es fa un anàlisi de l’evolució del poblament, un anàlisi dels canvis físics, mitjançant l’avaluació dels canvis en les cobertes del sòl i de la transformació del paisatge amb les fotografies, i un anàlisi dels canvis en les activitats i comportaments socials mitjançant la realització d’entrevistes per elaborar un retrat social de cada època, distingint entre les aportacions de la població autòctona respecte de la nouvinguda. Amb tot, es pretén la realització d’una diagnosi transversal que permeti integrar les diferents temàtiques, i definir unes conclusions generals per mitjà d’uns patrons detectats. Finalment, la conclusió que millor permet recollir tota la informació és la que en resulta d’acceptar el fenomen de despoblament generalitzat, combinat amb el d’un canvi de l’estil de vida global, no només local, que és eina de justificació de moltes de les dinàmiques de canvi observades.

The value of ultrasonography in assessment of portal hypertension in hepatosplenic schistosomiasis

Ultrasonography can reveal most of the manifestations of portal hypertension complicating hepatosplenic, schistosomiasis. However, direct demonstration of gastroesophageal varices by ultrasonography is still very difficult. An attempt was done to correlate sonographic features of portal hypertension with the degree of fibrosis to screen patients having varices and predicting their chance of bleeding. The results obtained were found to be consistent with the esophagogastric endoscopy and with history of hematemesis. Four parameters were used, size of spleen, degree of periportal fibrosis, presence of collaterals and portal vein diameter. A pilot field survey was also done adopting the same principle.

L'enfant terrible du christianisme

(Résumé du numéro) Que sait-on de Paul aujourd'hui ? Les Actes des Apôtres, le seul récit de la vie de Paul en notre possession, racontent la lapidation d'Étienne sous les yeux de Saül, puis les voyages missionnaires de Saül, devenu Paul sur la route de Damas. On ne connaît de lui ni son nom complet, ni sa date de naissance, ni les circonstances exactes de sa mort, à Rome. Dans la Bible, on trouve treize lettres de Paul écrites à des amis et à des communautés chrétiennes, en Méditerranée. Mais elles ne sont pas forcément de lui. Le personnage, son itinéraire et son oeuvre, fascinent encore et divisent. Juif radical pour certains, fils de la grande culture grecque, ou enfant terrible du christianisme pour d'autres... Paul le converti est un passeur de frontières, un visionnaire. Le monde de la Bible a mené l'enquête auprès des meilleurs spécialistes contemporains.

La relation ambiguë des protestantismes à la sociologie

(Résumé de l'ouvrage) What are the relations between sociology and the different religions - Christianity with its various branches, Judaism, Islam, Oriental religions, sects and New Religious Movements ? That is the question which this work, - conceived on the occasion of the XXVth Conference of the International Society for the Sociology of Religion/Société Internationale de Sociologie des Religions (SISR) - wishes to clarify. The book retraces the varied and troubled history of these relations and also reveals how in opening up its research to other religions besides the Christan, sociology is forced to redefine the very object of its field of study. What is the religious? This question, which until recently was considered impertinent, informs this book throughout. If confronts the necessity of rethinking theories and methodological appoaches which, constructed in the context of 19th and early 20th century Western Europe, prove to be rather inadequate for encompassing contemporary religious phenomena and religious manifestations in other contexts. To these new theoretical and methodological demands is added, for the sociologist, a deontological imperative, which takes on all the more importance today as the religious provokes passionate social debate.

Generation and phenotypic analysis of protein S-deficient mice.

Protein S (PS) is an important natural anticoagulant with potentially multiple biologic functions. To investigate further the role of PS in vivo, we generated Pros(+/-) heterozygous mice. In the null (-) allele, the Pros exons 3 to 7 have been excised through conditional gene targeting. Pros(+/-) mice did not present any signs of spontaneous thrombosis and had reduced PS plasma levels and activated protein C cofactor activity in plasma coagulation and thrombin generation assays. Tissue factor pathway inhibitor cofactor activity of PS could not be demonstrated. Heterozygous Pros(+/-) mice exhibited a notable thrombotic phenotype in vivo when challenged in a tissue factor-induced thromboembolism model. No viable Pros(-/-) mice were obtained through mating of Pros(+/-) parents. Most E17.5 Pros(-/-) embryos were found dead with severe intracranial hemorrhages and most likely presented consumptive coagulopathy, as demonstrated by intravascular and interstitial fibrin deposition and an increased number of megakaryocytes in the liver, suggesting peripheral thrombocytopenia. A few E17.5 Pros(-/-) embryos had less severe phenotype, indicating that life-threatening manifestations might occur between E17.5 and the full term. Thus, similar to human phenotypes, mild heterozygous PS deficiency in mice was associated with a thrombotic phenotype, whereas total homozygous deficiency in PS was incompatible with life.

New approaches for the control and eradication of schistosomiasis in Venezuela

Schistosomiasis in Americawith the exception of Brazil, behaves as a chronic mild disease with few clinical manifestations due to low parasite burden. These features restrict the clinical and parasitological diagnosis. The most commonly used stool examination method, Kato-Katz, becomes intensitive when the majority of individuals excrete less than 100 eggs/g of feces. In view that antigen-detecting techniques have not been able to reveal light infections, the antibody detecting assays remain as a very valuable diagnostic tool for epidemiological surveillance. The Venezuelan Schistosomiasis Research group (CECOICE) has designed a mass chemotherapy strategy based on sero-diagnosis. Since blood sampling is one of the important limitating factors for large seroepidemiological trials we developed a simple capillary technique that sucessfully overcomed most of the limitations of blood drawing. In this sense, ELISA seems to be the most adecuate test for epidemiological studies. Soluble egg Schistosoma mansoni antigen (SEA) has been largely used in Venezuela. The sensitivity ELISA-SEA in our hands is 90% moreover its specific reach 92% when populations from non-endemic areas but heavily infected with other intestinal parasites are analyzed. The Schistosomiasis Control Program is currently carrying out the surveillance of endemic areas using ELISA-SEA as the first screening method, followed by the Circumoval Precipitin test for validation assay. The results with these two serological techniques allowed us to defined the criteria of chemotherapy in populations of the endemic areas. On the search of better diagnostic technique, Alkaline Phosphatase Immunoenzyme Assay (APIA) is being evaluated in field surveys.

Forma aguda ou toxêmica da esquistossomose mansoni

The acute toxemic form of schistosomiasis mansoni is studied under anatomic and clinical point of view, according to classification made by Neves, Raso and Bagliolo in 1975. The first phase is characterized by the following facts: cutaneous (immediate and late) manifestations; high fever or in progressive elevation; intense diaphoresis abdominal disconfort; intense acquous diarrhea; dehidratation; loss of weight, dry cough; painful hepatosplenomegaly; discreet lymphademegaly, progressive increase of blood leucocytes and eosinophisles; radiological pulmonary alterations; absence of alterations in serum protein and hepatic functional tests; the hepatic function byopsy shows focus of acute hepatitis. The second stage or properly named toxemic period was clinically characterized by the neat aggravation of the previously observed phenomena. At last, the evolutive course of the disease has implication derived not only of the worm's presence, but from the intense dissemination of eggs in the tissue. In the pre-laying phase one studied the forms of cercarian dermatitis, prodromic and innapparent. In the post laying phase, the properly named acute toxemic form, with its types: pseudocholeraic, pseudotyphous, pseudodysenteric-bacillary, pseudonophritic, pseudoenterovirotic, the reactivated, the ischemic enterocolitis and others; whenever possible clinical and anatomic correlation will be made.

Pathogenesis of Schistosoma mansoni infection

In this paper a discussin is made on the pathogenesis of schistosomiasis mansoni in mice, presented from the perspectives of "processes", "mediators", "strategies for study" and vasculitis are discussed. The role of mediators, including cells, antibodies and immune complexes, cytokines and distal mediators is commented as related to the pathological processes occuring in schistosomiasis. Finally, strategies for study are presented, followed by a discussion on the etiopathogenesis of the different clinical stages and pathologic manifestations of schistosomiasis mansoni.