Vincristine and intestinal pseudo-obstruction in children: report of 5 cases, literature review, and suggested management.

Summary: Intestinal pseudo-obstruction is a rare complication resulting from a variety of disorders. Symptoms include abdominal pain, nausea, vomiting, diarrhea, constipation, and malnutrition. Vincristine-related pseudo-obstruction has been reported in the literature, but its description in children and recommendations for management are lacking. A review of the literature revealed 21 reported pediatric cases of vincristine-related pseudo-obstruction. Most have, however, been attributed to a drug interaction with itraconazole, accidental vincristine overdose, or liver failure. Potential genetic causes are rarely addressed. We present here 5 cases of pseudo-obstruction related to vincristine without any identifiable predisposing factors, and a suggested algorithm for management

Le Protévangile de Jacques latin dans l'homélie Inquirendum est pour la fête de la Nativité de Marie

L'article est le fruit d'une recherche sur la survie du Protévangile de Jacques en latin. Il contient l'édition critique et la traduction d'une homélie pour la fête de la Nativité de Marie, désignée par son incipit, Inquirendum est, et conservant les ch. 1-8 du Protévangile de Jacques (PJ). Dans trois des six manuscrits utilisés pour l'édition, l'homélie fait partie d'un recueil de sermons de l'époque carolingienne, l'« Homéliaire de Saint-Père de Chartres ». Elle a été composée en même temps que cet homéliaire, entre 820 et 950, dans un milieu marqué par des échanges entre l'Angleterre et la France. L'auteur de l'homélie a inséré dans un cadre homilétique les ch. 1-8 du PJ. Il a utilisé une version latine amplifiée du Protévangile (traduction II), dont dépendent également plusieurs autres témoins: le manuscrit de Paris, Sainte-Geneviève 2787 (PJlatG); les Latin Infancy Gospels édités par M. R. James (JAr et JHer, formes Arundel et Hereford de la « compilation J »); le récit irlandais de l'enfance du Liber Flavus Fergusiorum (InfLFF). Certaines amplifications du récit primitif sont présentes dans l'ensemble de ces témoins, comme l'épisode de la révélation céleste du nom de Marie (traduction IIa). D'autres sont communes à l'homélie, à JAr-JHer et/ou à InfLFF, comme l'ordre supplémentaire donné par Joachim à ses bergers (traduction IIb). A côté de ces éléments traditionnels, l'article met en évidence une série de particularités rédactionnelles (omissions, retouches, additions). L'auteur de l'homélie tient notamment à souligner le caractère naturel de la conception de Marie.

Do Legal Immigrants and Natives Compete in the Labour Market? Evidence from Catalonia

The precondition for labour-market competition between immigrants and natives is that both are willing to accept jobs that do not differ in quality. To test this hypothesis, in this paper we compare the working conditions between immigrants and natives in Catalonia. Comparing immigrants’ working conditions in relation to their native counterparts is not only a useful analysis for studying the extent to which immigrants and low-skilled native workers are direct competitors in the labour market, but also allows us to contribute to the literature on this issue by moving away from the conventional approach used in previous studies. Our results indicate that: i) natives and immigrants display a different taste for job (dis)amenities; ii) Catalan-born workers might be in direct competition with EU15 immigrants, while non-Catalan Spanish workers might be competing with Latin American immigrants, and; iii) African-born immigrants are the group in the Catalan workforce that by far face the worst working conditions.

Consensus summary statement of the International Multidisciplinary Consensus Conference on Multimodality Monitoring in Neurocritical Care : A statement for healthcare professionals from the Neurocritical Care Society and the European Society of Intensive Care Medicine.

Neurocritical care depends, in part, on careful patient monitoring but as yet there are little data on what processes are the most important to monitor, how these should be monitored, and whether monitoring these processes is cost-effective and impacts outcome. At the same time, bioinformatics is a rapidly emerging field in critical care but as yet there is little agreement or standardization on what information is important and how it should be displayed and analyzed. The Neurocritical Care Society in collaboration with the European Society of Intensive Care Medicine, the Society for Critical Care Medicine, and the Latin America Brain Injury Consortium organized an international, multidisciplinary consensus conference to begin to address these needs. International experts from neurosurgery, neurocritical care, neurology, critical care, neuroanesthesiology, nursing, pharmacy, and informatics were recruited on the basis of their research, publication record, and expertise. They undertook a systematic literature review to develop recommendations about specific topics on physiologic processes important to the care of patients with disorders that require neurocritical care. This review does not make recommendations about treatment, imaging, and intraoperative monitoring. A multidisciplinary jury, selected for their expertise in clinical investigation and development of practice guidelines, guided this process. The GRADE system was used to develop recommendations based on literature review, discussion, integrating the literature with the participants' collective experience, and critical review by an impartial jury. Emphasis was placed on the principle that recommendations should be based on both data quality and on trade-offs and translation into clinical practice. Strong consideration was given to providing pragmatic guidance and recommendations for bedside neuromonitoring, even in the absence of high quality data.

Reversible congestive heart failure associated with primary carnitin deficiency: report of a case and review of the literature.

A 5-year-old previously healthy boy was admitted for abdominal pain and vomiting. Physical examination showed tachypnoe (32/min), hepatomegaly and painful palpation of the upper right abdominal quadrant. Laboratory tests were normal except for elevated ammonium (202mcmol/l). Chest X-ray was performed, showing cardiomegaly and interstitial edema. Transthoracic echocardiography revealed dilated left cavities and LV hypertrophy together with a diffuse hypokinesia and LVEF of 30-40%. Diuretics and ACE-inhibitors were introduced. At that time, the differential diagnosis for the DCM included myocarditis, congenital or genetic, metabolic or autoimmune disease. The next day, the boy underwent cardiac magnetic resonance (CMR) examination, showing a severe dilatation of the LV with an end-diastolic diameter of 50mm and a volume of 150ml. LVEF was 20% with diffuse LV hypokinesia (Fig. 1). No late enhancement was present after Gadolinium injection, ruling out myocarditis. Further laboratory metabolic analysis indicated severely decreased total and free carnitin levels and low renal carnitin reabsorption, corroborating the diagnosis of primary carnitin deficiency (PCD). Carnitin substitution was initiated. The clinical condition rapidly improved. No symptoms of heart failure were present anymore. A follow-up CMR performed 9 months later confirmed the recovery. LV end-diastolic volume decreased from 150ml to 66ml, LVEF increased from 20% to 55% (Fig. 2). Late enhancement was absent after Gadolinum injection (Fig. 3).Carnitin is required for the transport of fatty acids from the cytosol into mitochondria during lipid breakdown. 75% of carnitin is obtained from food, 25% is endogenously synthesized. PCD is an autosomal recessive disorder resulting from impairment of a transporter activity, caused by mutation of the SLC22A5 gene. Incidence is about 1 in 40'000 newborns. Diagnosis is usually made at age 1 to 7. Three forms of PCD are described. In the form associated with cardiomyopathy, the disease is progressive and patient die from heart failure if not treated. Substitution of L-Carnitin leads to a dramatic improvement of disease course.This case underlines the crucial role of etiologic diagnostics in this reversible form of DCM. Early diagnostics and therapy are critical for the prognosis of the patient. This is furthermore an example of a role played by CMR in the diagnostic work-up of heart failure and its follow-up under therapy.

Expérimentation générique et dialogisme intertextuel. Perrault, La Fontaine, Apulée, Straparola, Basile

EXPERIMENTATION GENERIQUE ET DIALOGISME INTERTEXTUEL: PERRAULT, LA FONTAINE, APULEIUS, STRAPAROLA AND BASILE. Les contes en vers et en prose de Perrault relèvent, selon l'hypothèse présentée dans cette étude, d'un dialogisme intertextuel très complexe avec les formes génériques du conte déjà existantes dans les littératures européennes. L'académicien s'adonne à une véritable «expérimentation générique » au cours de laquelle il crée de nouvelles formes génériques, de nouvelles intrigues et de nouvelles figures à partir de la fabella de Psyché enchâssée dans les Métamorphoses d'Apulée, et de sa récriture galante par La Fontaine dans Les Amours de Psiché et de Cupidon en 1669. Ce double dialogue intertextuel est encore sous-tendu par le recours aux favole de Straparola (Le Piacevole notti) et aux cunti (Lo cunto de li cunti) de Basile, célèbres narrateurs italiens, qui avaient déjà reconfiguré de façon originale certains épisodes et personnages de la fabella de Psyché. Ce processus dialogique complexe est ici mis en évidence par l'analyse (inter)textuelle successive des trois premiers contes en prose, La Belle au bois dormant, Le Petit Chaperon rouge et La Barbe bleue. L'analyse comparative montre que Perrault les invente à partir des trois moments successifs de l'épreuve la plus difficile que Vénus inflige à Psyché, celle de la descente aux Enfers. En introduisant des différences significatives par rapport aux textes latins, italiens et français, l'académicien parvient à créer une nouvelle variation générique : le conte pseudo-naïf doté d'un sens crypté qui se découvre « plus ou moins selon le degré de pénétration de ceux qui les lisent ».GENERIC EXPERIMENTATION AND INTERTEXTUAL DIALOGISM: PERRAULT, LA FONTAINE, APULEIUS, STRAPAROLA AND BASILEAccording to Ute Heidmann, Perrault's tales have very complex inter- textual relations with other generic forms which already existed in other European literatures. Heidmann demonstrates here how Perrault experi- ments "generically" with the fairy tale and how he creates new generic forms from other tales by Apuleus, Straparola, Basile, or La Fontaine. This dialogic process is here underlined by the analysis of three particular fairy tales, Sleeping Beauty in the Woods, Little Red Riding Hood and Blue Beard. Heidmann shows how, by introducing key differences with the Latin, Italian and French models, Perrault succeeds in creating a new generic variation of the fairy tale: "the pseudo-naïve fairy tale".

Prevention of transfusional Trypanosoma cruzi infection in Latin America

Trypanosoma cruzi is a protozoan infection widely spread in Latin America, from Mexico in the north to Argentina and Chile in the south. The second most important way of acquiring the infection is by blood transfusion. Even if most countries of Latin America have law/decree/norms, that make mandatory the screening of blood donors for infectious diseases, including T. cruzi (El Salvador and Nicaragua do not have laws on the subject), there is usually no enforcement or it is very lax. Analysis of published serologic surveys of T. cruzi antibodies in blood donors done in 1993, indicating the number of donors and screening coverage for T. cruzi in ten countries of Central and South America indicated that the probability of receiving a potentially infected transfusion unit in each country varied from 1,096 per 10,000 transfusions in Bolivia, the highest, to 13.02 or 13.86 per 10,000 transfusions in Honduras and Venezuela respectively, where screening coverage was 100%. On the other hand the probability of transmitting a T. cruzi infected unit was 219/10,000 in Bolivia, 24/10,000 in Colombia, 17/10,000 in El Salvador, and around 2-12/10,000 for the seven other countries. Infectivity risks defined as the likelihood of being infected when receiving an infected transfusion unit were assumed to be 20% for T. cruzi. Based on this, estimates of the absolute number of infections induced by transfusion indicated that they were 832, 236, and 875 in Bolivia, Chile and Colombia respectively. In all the other countries varied from seven in Honduras to 85 in El Salvador. Since 1993, the situation has improved. At that time only Honduras and Venezuela screened 100% of donors, while seven countries, Argentina, Colombia, El Salvador, Honduras, Paraguay, Uruguay and Venezuela, did the same in 1996. In Central America, without information from Guatemala, the screening of donors for T. cruzi prevented the transfusion of 1,481 infected units and the potential infection of 300 individuals in 1996. In the same year, in seven countries of South America, the screening prevented the transfusion of 36,017 infected units and 7, 201 potential cases of transfusional infection.

Partnerships: a literature review

The Research and Development Office for Health and Personal Social Services in Northern Ireland funded the Institute of Public Health in Ireland (IPH) to undertake research into partnerships between 2003 and 2006, as part of their New Targeting Social Need programme.The aim of the research was to identify the impacts of multisectoral partnerships, how they can be measured, and what contribution they make to tackling inequalities in health. This document is one of a suite of three produced as a result of this work

The role of ceramide trihexoside (globotriaosylceramide) in the diagnosis and follow-up of the efficacy of treatment of Fabry disease: a review of the literature.

Fabry disease is caused by a deficiency of a-galactosidase A which leads to the progressive intra-lysosomal accumulation of ceramide trihexoside (CTH), also known as globotriaosylceramide (Gb3), in different cell types and body fluids. The clinical manifestations are multisystemic and predominantly affect the heart, kidney and central nervous system. The role of CTH in the pathophysiological process of Fabry disease is not established, and the link between the degree of accumulation and disease manifestations is not systematic. The use of CTH as a diagnostic tool has been proposed for several decades. The recent introduction of a specific treatment for Fabry disease in the form of enzyme replacement therapy (ERT) has led to the need for a biological marker, in place of a clinical sign, for evaluating the efficacy of treatment and also as a tool for following the long term effects of treatment. The ideal biomarker must adhere to strict criteria, and there should be a correlation between the degree of clinical efficacy of treatment and a change in its concentration. This review of the literature assesses the utility of CTH as a diagnostic tool and as a marker of the efficacy of ERT in patients with Fabry disease. Several techniques have been developed for measuring CTH; the principles and the sensitivity thresholds of these methods and the units used to express the results should be taken into consideration when interpreting data. The use of CTH measurement in Fabry disease should be re-evaluated in light of recent published data.

Posterior cortical atrophy: review of the recent literature.

Posterior cortical atrophy (PCA) is a group of neurodegenerative dementing disorders characterized by initial predominant visual complaints followed by progressive decline in cognitive functions. The visuospatial and visuoperceptual defects arise from the dysfunction of, respectively, the dorsal (occipito-parietal) and the ventral (occipito-temporal) streams. Clinical symptoms, results of neuropsychological examination, and findings of posterior cerebral atrophy and/or posterior hypoperfusion/hypometabolism contribute to the diagnosis. However, owing to the insidious onset of PCA and the non-specificity of initial symptoms, the diagnosis is often delayed. Specific etiologies include Alzheimer's disease, dementia with Lewy bodies, subcortical gliosis, corticobasal degeneration, and prion-associated diseases. Alzheimer's disease accounts for at least 80 % of PCA cases. Recent research has concentrated on better defining the clinical presentation of PCA, improving neuroimaging analysis, testing new neuroimaging techniques, and developing biological measurements. Selected recent papers on PCA are reviewed in this article.

Chagas infection transmission control: situation of transfusional transmission in Brazil and other countries of Latin America

The transmission of the transfusion-associated Chagas disease is an important mechanism of its dissemination in several Latin American countries. The transmission risk depends on five factors: prevalence of infection in blood donors, degree of serological coverage, sensibility of used tests, safety of obtained results and infection risk. The Southern Cone Iniciative set off by the Pan-American Health Organization, in 1991, is contributing to the implementation of blood law in each endemic country, and to reduce the risk of transfusional transmission of this horrible disease. Despite the clear improvement of Brasilian hemotherapy after 1980 (with the creation of the Blood National Program - Pró-Sangue) and the significant reduction of the chagasic infection among its blood donors; socio-economic, politic and cultural unlevels, prevent it from reaching the necessary universality and security. In order to assure both, the Brazilian Ministry of Health decided to restructure its blood system. In May, 1998, a great program was launched, to reach a specific goal: Blood - 100% with quality safety in all its process until 2003. It was divided in 12 projects, intends to guarantee the quality and self sufficiency in blood and hemoderivates.

Resolution of foveal detachment in dome-shaped macula after treatment by spironolactone: report of two cases and mini-review of the literature.

Dome-shaped macula (DSM) was recently described in myopic patients as a convex protrusion of the macula within a posterior pole staphyloma. The pathogenesis of DSM and the development of associated serous foveal detachment (SFD) remain unclear. The obstruction of choroidal outflow and compressive changes of choroidal capillaries have been proposed as causative factors. In this paper, we report two cases of patients with chronic SFD associated with DSM treated with oral spironolactone. After treatment, there was a complete resolution of SFD in both patients. To the best of our knowledge, this is the first report of successful treatment of SFD in DSM by a mineralocorticoid receptor antagonist.