Safety and efficacy of low-dose PI3K inhibitor taselisib in adult patients with CLOVES and Klippel-Trenaunay syndrome (KTS): the TOTEM trial, a phase 1/2 multicenter, open-label, single-arm study.

PURPOSE: PIK3CA pathogenic variants in the PIK3CA-related overgrowth spectrum (PROS) activate phosphoinositide 3-kinase signaling, providing a rationale for targeted therapy, but no drug has proven efficacy and safety in this population. Our aim was to establish the six-month tolerability and efficacy of low-dose taselisib, a selective class I PI3K inhibitor, in PROS patients. METHODS: Patients over 16 years with PROS and PIK3CA pathogenic variants were included in a phase IB/IIA multicenter, open-label single-arm trial (six patients at 1 mg/day of taselisib, then 24 at 2 mg/day). The primary outcome was the occurrence of dose limiting toxicity (DLT). Efficacy outcomes were the relative changes after treatment of (1) tissue volume at affected and unaffected sites, both clinically and on imaging; (2) cutaneous vascular outcomes when relevant; (3) biologic parameters; (4) quality of life; and (5) patient-reported outcomes. RESULTS: Among 19 enrolled patients, 2 experienced a DLT (enteritis and pachymeningitis) leading to early trial termination (17 treated, 10 completed the study). No serious adverse reaction occurred in the 1 mg cohort (n = 6). No significant reduction in affected tissue volume was observed (mean -4.2%; p = 0.81; SD 14.01). Thirteen (76.4%) participants reported clinical improvement (pain reduction, chronic bleeding resolution, functional improvement). CONCLUSION: Despite functional improvement, the safety profile of low-dose taselisib precludes its long-term use.

Identification of a Novel Elastin-Degrading Enzyme from the Fish Pathogen Flavobacterium psychrophilum.

Hydrolytic extracellular enzymes degrading host tissues potentially play a role in bacterial pathogenesis. Flavobacterium psychrophilum is an important bacterial pathogen of salmonid fish reared in freshwater throughout the world. Diversity among isolates has been described at the phenotypic, serological, and genomic levels, but the links between these various traits remain poorly understood. Using a genome-wide association study, we identified a gene encoding a novel elastinolytic enzyme in F. psychrophilum To formally demonstrate enzymatic activity, this gene (FP0506 from strain JIP 02/86) was expressed in the elastinolysis-deficient strain OSU THCO2-90, resulting in proficient elastin-degrading cells. The encoded protein is predicted to be a cell-surface-exposed lipoprotein with no homology to previously reported elastases. FP0506 might belong to the zincin tribe and gluzincin clan of metalloproteases, and this new elastase-encoding gene seems to be present only in some members of the family FlavobacteriaceaeIMPORTANCE Elastin is an important proteinaceous component of vertebrate connective tissues (e.g., blood vessels, lung, and skin), to which it confers elasticity. Elastases have been identified in a number of pathogenic bacteria. They are thought to be required for tissue penetration and dissemination, acting as "spreading factors." Flavobacterium psychrophilum is a devastating bacterial pathogen of salmonid fish (salmon and trout) that is responsible for severe economic losses worldwide. This pathogen displays strong proteolytic activities. Using a variety of techniques, including genome comparisons, we identified a gene encoding a novel elastase in F. psychrophilum The encoded protein is predicted to be a cell-surface-exposed lipoprotein with no homology to previously reported elastases. In addition, this elastase likely belongs to a new family of proteases that seems to be present only in some members of this important group of bacteria.

Tabla Air Stand-Up Paddle de rescate acuático: ¿Cómo puede ayudar al socorrista? / Air Table Stand-Up Paddle water rescue: How can you help the lifeguard?

Las tablas tradicionales y rígidas en socorrismo acuático han sido rechazadas en muchos servicios de socorrismo por los problemas que generaban. La experiencia con tablas hinchables (AIRSUPRA) ha demostrado muchas ventajas y las objeciones sobre este material están desapareciendo y ya comienzan a utilizarse en servicios de socorrismo, que antes eran reticentes. El objetivo de este estudio fue analizar el efecto del uso de la tabla AIRSUPRA en el tiempo de aproximación en una distancia de 100 metros, con una muestra de 16 socorristas profesionales (13 hombres y 3 mujeres), certificados y actualizados en técnicas de rescate, pero sin experiencia con tablas AIRSUPRA. Los resultados demuestran que cuando se realiza la aproximación con la tabla AIRSUPRA (TR1) se obtiene un tiempo de 54.13 + 8.58, mientras que cuando se realiza la aproximación a nado el tiempo es 93.19 + 25.52, con una diferencia de 39.06 segundos, estadísticamente significativa (p <0.001). Este primer estudio y sus resultados apuntan a la necesidad de profundizar en la investigación con este nuevo material para el rescate (AU)

The use of traditional and rigid boards in aquatic lifesaving cause more problems than advantages, for this reason was frequently rejected in many lifesaving services. However, based on knowledge and experimentation with inflatable boards many advantages have been proven, doubts and objections on this material are disappearing and, in fact, are beginning to be used in lifesaving services that were previously reluctant to use them. The aim of this study was to analyze the effect of using the table AIRSUPRA in the time of approaching for a distance of 100 meters. The sample was integrated by 16 professionals (13 men and 3 women), all of them with rescue techniques certification and updated training, but with no experience managing boards AIRSUPRA. The results show that when the approximation with AIRSUPRA table (TR1) is performed the time was of 54.13 + 8.58, whereas when the approaching is performed swmming the time was of 93.19 + 25.52, with a difference of 39.06 seconds, statistically significant (p <0.001). This first study and its results point to the need for further research with this new material for rescue (AU)

Australian Cattle Dogs with Neuronal Ceroid Lipofuscinosis are Homozygous for a CLN5 Nonsense Mutation Previously Identified in Border Collies.

BACKGROUND: Neuronal ceroid lipofuscinosis (NCL), a fatal neurodegenerative disease, has been diagnosed in young adult Australian Cattle Dogs. OBJECTIVE: Characterize the Australian Cattle Dog form of NCL and determine its molecular genetic cause. ANIMALS: Tissues from 4 Australian Cattle Dogs with NCL-like signs and buccal swabs from both parents of a fifth affected breed member. Archived DNA samples from 712 individual dogs were genotyped. METHODS: Tissues were examined by fluorescence, electron, and immunohistochemical microscopy. A whole-genome sequence was generated for 1 affected dog. A TaqMan allelic discrimination assay was used for genotyping. RESULTS: The accumulation of autofluorescent cytoplasmic storage material with characteristic ultrastructure in tissues from the 4 affected dogs supported a diagnosis of NCL. The whole-genome sequence contained a homozygous nonsense mutation: CLN5:c.619C>T. All 4 DNA samples from clinically affected dogs tested homozygous for the variant allele. Both parents of the fifth affected dog were heterozygotes. Archived DNA samples from 346 Australian Cattle Dogs, 188 Border Collies, and 177 dogs of other breeds were homozygous for the reference allele. One archived Australian Cattle Dog sample was from a heterozygote. CONCLUSIONS AND CLINICAL IMPORTANCE: The homozygous CLN5 nonsense is almost certainly causal because the same mutation previously had been reported to cause a similar form of NCL in Border Collies. Identification of the molecular genetic cause of Australian Cattle Dog NCL will allow the use of DNA tests to confirm the diagnosis of NCL in this breed.

[Partial cystectomy for bladder endometriosis: Robotic assisted laparoscopy versus standard laparoscopy]. / Étude comparative : laparoscopie robot assistée versus cœlioscopie chez les patientes avec une endométriose vésicale.

OBJECTIVES: To compare robot-assisted laparoscopy (RL) and conventional laparoscopy (CL) in surgery for bladder endometriosis. METHODS: A retrospective study was conducted between January 2007 and December 2013, including patients with bladder endometriosis receiving at least a partial cystectomy by RL or CL. The primary endpoint was the presence of a radiological recurrence at bladder level. RESULTS: We included 15 patients in the RL group and 22 in the CL group. The median age was 29 years±7 years. The symptoms were similar in the 2 groups. Pre-surgical mapping of the lesions was carried out with MRI. Sixty percent of patients in the RL group vs 91% in the CL group had other associated endometriosis lesions, P=0.04. The median size of the bladder lesion was 30±8mm in the RL group vs 23±7mm in the CL group, P=0.03. The median operative time was 210 vs 225min, P=0.8. We did not find any significant difference in intraoperative and early and late postoperative complications between the 2 groups. The median length of stay was 5 days vs 6 days. The proportion of relapse was 20 vs 23%, P>0.05. Clinical improvement was similar between the groups, i.e. 93 vs 86%, P=0.6 and the pregnancy rate was 93 vs 86%, P=0.6. CONCLUSIONS: Robot-assisted laparoscopy in the surgical treatment of bladder endometriosis as compared to traditional laparoscopy does not seem to have an adverse effect neither on the risk of recurrence nor on the occurrence of intra- and postoperative complications.

A nephrotic syndrome of tropical origin: case report and short review of the aetiology.

We present a case of nephrotic syndrome in a 38-year-old man of Ivorian origin. In the search of the cause of his illness an infection with Plasmodium malariae (P. malariae) was diagnosed by serology and by microscopy of a Giemsa thin blood smear which revealed rare gametocytes of P. malariae. Proteinuria significantly diminished within three months after antimalarial treatment. Antibodies against Schistosoma were detected as well. Examination of kidney biopsy revealed a discrete mesangioproliferative glomerulonephritis. This case highlights that a thorough history-taking may be essential and that infectious diseases should be included in the differential diagnostic thinking process when a nephrotic syndrome is diagnosed.

Accidental out-of-hospital deliveries: a case-control study.

AIM: To determine risk factors for accidental out-of-hospital deliveries (OHDs), which represent 0.5% of live births in France and are associated with poor neonatal outcomes. METHODS: This retrospective case-control study assessed accidental OHDs that occurred in the Finistère District (Brittany, France) between January 2007 and December 2009. For each OHD case, two controls were randomly selected. Outcome measures included maternal demographics, obstetric characteristics and neonatal outcomes. RESULTS: During the study period, accidental OHDs accounted for 0.42% of all births; 76 accidental OHDs were included in the analysis. Multivariate analysis found four independent risk factors for accidental OHD: multiparity [OR: 8.84 (3.22-24.29)], unemployment [OR: 4.99 (1.85-13.47)], lack of or poor antenatal care [OR: 9.00 (2.41-33.72)] and a travel time >45 min from home to the delivery unit [OR: 6.18 (1.33-28.65) versus < 15 min]. Significantly more newborns from the OHD group required admission to the neonatal unit (p = 0.04), but accidental OHD was not significantly associated with prematurity or low birth weight. CONCLUSION: Four risk factors for accidental OHD were identified. Setting up an anonymous registry of OHD cases could improve our knowledge and screening of women at risk.

Outbreak of extended spectrum beta-lactamase-producing Klebsiella pneumoniae in an intensive care unit (Brest).

INTRODUCTION: We had for aim to describe control and investigation of an outbreak caused by a strain of Extended spectrum beta-lactamase producing Klebsiella pneumoniae in intensive care units of the Brest teaching hospital. PATIENTS AND METHOD: The case definition was a patient infected by or carrying the epidemic strain. Control measures and investigations are presented. A case-control study was conducted in the surgical intensive care unit. Each case was matched with two controls based on admission times in the unit. The study focused on diagnostic and therapeutic procedures, and potential contacts with healthcare workers, in this context of cross transmission. RESULTS: Between February and May 2011, nine cases were reported in the surgical ICU and two in the medical ICU. Eighteen controls were matched with the nine surgical ICU cases. Several factors were found to be statistically associated with infection or colonization by the epidemic strain: the surgical block in which patients had been operated and the ward of first hospitalization; the number of trans-esophageal and trans-thoracic echocardiographies, of central venous catheter insertions, and of surgical operations; intubation. The total number of invasive procedures was also found to be statistically higher among cases. CONCLUSION: This study identified factors associated with colonization or infection by the epidemic strain. These factors might have been involved in the transmission tree, and be vulnerable elements for the prevention of nosocomial infections and colonisations, and their epidemic spread.

10th survey of antimicrobial resistance in noninvasive clinical isolates of Streptococcus pneumoniae collected in Belgium during winter 2007-2008.

OBJECTIVES: The aim of the study was to evaluate the antibiotic resistance in noninvasive clinical isolates of Streptococcus pneumoniae collected in Belgium during winter 2008-2007. METHOD: Four hundred and forty eight unduplicated isolates collected by 15 laboratories were tested by microdilution following CLSI. RESULTS: Insusceptibility rates (I+R) were as follows: penicillin G (PEN) 11.6% (4.0% R), ampicillin 11.4% (4.0% R), amoxicillin+/-clavulanic acid 0, cefaclor 10.3% (9.6% R), cefuroxime 9.2% (8.7% R), cefuroxime-axetil 8.7% (7.8% R), cefotaxime, ceftazidime and cefepime 2.0% (0% R), imipenem 2.5% (0% R), ciprofloxacin and ofloxacin 5.1% (0.4% R), levofloxacin 0.7% (0.4% R), moxifloxacin 0.4% (0.2% R), erythromycin (ERY) 29.7% (29.2% R), azithromycin 29.7% (28.8% R), telithromycin 0%, clindamycin 26.3% (25.4% R) and tetracycline (TET) 21.9% (16.5% R). From 2001 to 2008, a significant decrease in penicillin-insusceptibility (21.0% to 11.6%), penicillin-resistance (9.7% to 4.0%) and ciprofloxacin-insusceptibility (11.2% to 5.1%) was found. Cross-resistance between penicillin and other betalactams in penicillin-insusceptible isolates was incomplete: all these isolates remained fully susceptible to amoxicillin. Erythromycin-insusceptibility was significantly higher in children than in adults (43.9%/27.4%), while penicillin-insusceptibility significantly higher in Brussels than in the Flanders (22.9%/8.1%). The commonest resistance phenotype was ERY-TET (12.7%) followed by ERY (7.4%) and PEN-ERY-TET (5.8%). Capsular types 19 (25%), 14 (19.3%), 23 (15.4%) and 15 (13.5%) were the most important in penicillin-insusceptible. CONCLUSION: We noted a decrease in resistance to the majority of the compounds. Insusceptibility rates were higher in children than in adults and the difference between the north and the south of Belgium became less marked.

Fluorescence characterization of the hydrophobic pocket of cyclophilin B.

Human cyclophilin B is a monomeric protein that contains two tryptophan residues, Trp104 and 128. Trp128-residue belongs to the binding site of cyclosporin A and is the homologous of Trp 121 in CyPA, while Trp104 residue belongs to the hydrophobic pocket. In the present work, we studied the dynamics of Trp residue(s) of cyclophilin B and of the CyPB(w128A) mutant and of TNS-mutant complex. Our results showed that Trp-104 and TNS show restricted motions within their environments and that energy transfer between the two fluorophores is occurring.

[DRESS syndrome, a drug reaction which remains bad known from paediatricians]. / Le DRESS syndrome, une réaction d'hypersensibilité aux médicaments, qui reste mal connue des pédiatres.

The DRESS syndrome (Rash with Eosinophilia and Systemic Symptoms) is a drug hypersensitivity reaction poorly known by paediatricians. It occurs within 1 to 8 weeks of treatment. Clinical features associate in variable patterns, fever, rash, lymphadenopathies, arthritis and potentially life-threatening damage (hepatitis, nephritis, pneumonitis), hyperleucocytosis and eosinophilia. This condition must be early recognized in order to immediately stop suspect drugs. A 6.5 year old girl had a febrile rash, hyperleucocytosis, lymph nodes and cytolitic hepatitis probably due to phenobarbital. Diagnosis of DRESS syndrome was performed only 13 days after the beginning of the eruption. Evolution was favorable but characterized by the recurrence of the febrile eruption with pleuritis. DRESS syndrome is a well described disease that occurs during treatment with a number drugs, particularly anti-epileptic drugs. Steroid therapy and immunoglobulins are proposed for treatment but have not been evaluated.

[Evaluation of the bacteriologic quality of breast milk in a neonatology service in Belgium]. / Evaluation de la qualité bactériologique du lait maternel dans un service de néonatologie.

Many studies demonstrated that human milk is the recommended source of enteral nutrition in preterm infants providing several benefits with regards to feeding tolerance, immunity and cognitive development However, neurological immaturity and associated clinical conditions prevent them from suckling effectively. Therefore, mother's milk must be expressed, stored and transported to the neonatal unit and could be contaminated. The microbiological quality of human milk was evaluated on each donation to the neonatal intensive care unit of the University of Liege, Belgium from November 1, 2003 to January 31, 2005. In all, 5842 samples from 176 mothers were included in the study. Samples were classified according to the exclusive presence of coagulase negative Staphylococcus and their number (less or more than 104 germs per ml) or to contamination with pathogens. More than 50% of analyzed milks had to be pasteurized (46%; >104 coagulase negative Staphylococcus per ml) or to be discarded (7% pathogen contamination). The incidence of pasteurisation tends to increase during the summer, suggesting a seasonal influence. Maternal profiles were established longitudinally. Among the 60 mothers whose at least one sample had pathogen contamination, 27% had a contamination occurring only during a few days, but 73% had more than 50% of their samples discarded. This study suggest the need to promote the use and the financial support of intrahospital human milk bank units to support the safe use of raw and pasteurised human milk in preterm infants.

Surveillance of antibiotic resistance in non invasive clinical isolates of Streptococcus pneumoniae collected in Belgium during winters 2003 and 2004.

A total of 391 and 424 non-invasive isolates of Streptococcus pneumoniae collected by 15 laboratories during the 2003 and 2004 survey were tested for their susceptibility by a microdilution technique following NCCLS recommendations. Insusceptibility rates (IR) in the two surveys (2003/2004) were as follows: penicillin 15.0/14.7% [8.4/6.4% Resistance (R)], ampicillin 17.4/14.6% (R 9.0/7.1%), amoxicillin +/- clavulanic acid 2.6/1.2 % (R 0/0%), cefaclor 14.3/14.1% (R 11.5/13.4%), cefuroxime 13.6/12.7% (R 10.5/11.8%), cefuroxime-axetil 10.5/11.8% (R 10.0/9.2%) (breakpoints based on 250 mg), cefotaxime 4.9/6.2% (R 1.3/2.4%), ceftazidime NotTested (NT)/6.4 (R NT/2.6%), cefepime NT/6.4 (R NT/2.6%), imipenem 7.7/8.9 % (R 1.8/1.4%), ertapenem 0.8/NT% (R O/NT%), ciprofloxacin 13.8/9.0% (R 4.3/2.4%), levofloxacin 3.3/2.8% (R 1.5/0.2%), moxifloxacin 0.6/0.2% (R 0.3/0%), ofloxacin 13.5/9.0% (R 4.3/2.4%), erythromycin 26.1/24.7% (R 25.3/24.5%), azithromycin 25.4/24.7% (R 24.6/24.5%), telithromycin 0.8/0.2% (R 0.5/0%), clindamycin 21.2/18.4% (R 19.2/17.7%) and tetracycline 32.3/22.1% (R 29.2/19.3%). There were only minor differences in resistance rates according to age, sample site, admission type (i.e. ambulatory, hospitalized or long-term care facility patients), gender and geographic origin. Overall, telithromycin (MIC50, MIC90 in 2003/2004: 0.015 microg/ml, 0.12 microg/ml/ 0.008,0.06 respectively), ertapenem (0.03; 0.25/NT), moxifloxacin (0.06; 0.25/0.06, 0.12), and amoxicillin +/- clavulanic acid (0.03; 0.25/0.015, 0.5) were the most active compounds in both surveys. In 2003, the most common resistance phenotype was isolated insusceptibility to tetracycline (10.5%) followed by combined insusceptibility to erythromycin and tetracycline (9.3%). Erythromycin-tetracycline resistance (10.4%) was the most common in 2004. Isolates showing resistance to an antibiotic were significantly more present in 2003 than in 2004 (50.4% versus 40.8%). In penicillin-insusceptible isolates, MICs of all beta-lactams were increased but cross-resistance between penicillin and other beta-lactams in the penicillin-insusceptible isolates was not complete. In the 2003 survey, most of these isolates remained fully susceptible to ertapenem (94.9%) and amoxicillin +/- clavulanic acid (83.1%). In the 2004 survey, 91.9% of the penicillin insusceptible isolates remained susceptible to amoxicillin +/- clavulanic acid. In both surveys, the most common serotypes in penicillin insusceptible isolates were 14, 23,19 and 9 (20.0%, 20.0%, 16.4% and 10.9% respectively in 2003; 41.6%, 11.7%, 15.0% and 18.3% respectively in 2004).

Lactoferrin: a modulator of immune and inflammatory responses.

Lactoferrin is an iron-binding glycoprotein of the transferrin family. Abundant expression and secretion of lactoferrin, in particular in milk and fluids of the digestive tract, are related to its implication in the first line of host defense. Lactoferrin is also a prominent component of the secondary granules of neutrophils (PMNs) and is released in infected tissues and blood during the inflammatory process. In addition to its direct antimicrobial properties, the abilities of lactoferrin to regulate the immune response and to protect against infection and septic shock have been described in numerous in vitro and in vivo studies. Although the cellular and molecular mechanisms that account for the modulation of the inflammatory and immune responses by lactoferrin are not yet totally elucidated, many are now established. At the cellular level, lactoferrin modulates the migration, maturation and function of immune cells. At the molecular level and in addition to iron binding, interactions of lactoferrin with a plethora of compounds, either soluble or membrane molecules, account for its modulatory properties. This paper reviews our current understanding of the cellular and molecular mechanisms that explain the regulatory properties of lactoferrin in host defence.

[Group B streptococcus in the antenatal clinic and in the delivery room: a matter of systematic attitude]. / Le streptocoque du groupe B en clinique anténatale et en salle de travail: un problème d'attitude systématique.

OBJECTIVES: We wanted to evaluate the compliance to the local recommendations, similar to the CDC (Centers for Disease Control and prevention) recommendations launched in 1996, for the prevention of perinatal group B streptococcal (GBS) disease in the clinical practice of a academic maternity and to identify the causes of missed screening and antibiotic prophylaxis. MATERIALS AND METHODS: Retrospective study of 1249 consecutive pregnancies between 1st January and 31th August 2002. The screening methods for GBS colonisation were the culture of rectovaginal swabs collected between 35 and 37 weeks and/or a rapid antigenic screening performed on a vaginal swab collected at the patient's admission for labor. RESULTS: Rate of global screening was very high (97.8%): 28.8% of antenatal screening versus 90.3% during labor. An appropriate antibiotic prophylaxis was administered to only one-third of positive women when the screening was performed at admission to the labor room, whereas two-thirds of GBS-positive women screened between 35 and 37 weeks received their antibiotic prophylaxis. 2.4%o of the newborns were infected and 2.9% were colonized. Among the different risk factors, intrapartum fever was more often associated with maternal GBS colonisation. The observed sensitivity of the rapide antigenic test was 20.4%. CONCLUSION: Compliance to guidelines is sometimes difficult in the clinical practice of an academic maternity. In our hands the rapid test for GBS screening had low sensitivity. The analysis of these data led to introducing a computerized algorithm in our maternity to improve the prevention of perinatal group B streptococcal disease.

RPBS: a web resource for structural bioinformatics.

RPBS (Ressource Parisienne en Bioinformatique Structurale) is a resource dedicated primarily to structural bioinformatics. It is the result of a joint effort by several teams to set up an interface that offers original and powerful methods in the field. As an illustration, we focus here on three such methods uniquely available at RPBS: AUTOMAT for sequence databank scanning, YAKUSA for structure databank scanning and WLOOP for homology loop modelling. The RPBS server can be accessed at http://bioserv.rpbs.jussieu.fr/ and the specific services at http://bioserv.rpbs.jussieu.fr/SpecificServices.html.

[Vulvovaginal candidiasis: prevalence of different Candida species in the Liege region]. / Prevalence des différentes espéces de Candida au niveau vaginal dans la région Liégeoise.

We calculated the prevalences of different yeast species isolated from more than 20,000 vulvovaginal specimens carried out at the CHR hospital in Liege. To assess the value of the observed relative frequencies, the culture results of 149 samples were confronted with those of a real-time PCR technique of fungal identification. With a prevalence close to 90%, Candida albicans remains the largely dominant species. In contrast with other teams, we observed no increase of the prevalences of Candida non-albicans species.

[The nursing labour market in Canada (1985-1999)]. / Le marché du travail en soins infirmiers au Canada (1985-1999).

This literature review analysed both published and unpublished scientific and professional studies on the nursing labour market in Canada within the period of 1985 to 1999. The goal was to conduct a situational analysis utilising statistical data and canvassing all concerned parties to extract their points of view. The analysis revealed significant cyclical variations in the evolution of the workforce, particularly with respect to auxiliary nurses, such as the perceived existence of major problems in recruiting new professionals in the field and retaining existing professionals in their organisations, the lack of homogeneity in educational training programmes, and the co-existence of several operational structures for organising nursing care, of which there is a lack of evaluation on their effectiveness. The results of the literature review identify the necessity to further develop the knowledge base on such a relevant dimension of the nursing labour market.

[Isolated familial corpus callosum agenesis prognosis]. / Les agénésies isolées et familiales du corps calleux sont-elles de bon pronostic?

Agenesia of corpus callosum belongs to a group of cerebral malformations whose prognosis is uncertain. In such cases, assessment of prognosis may benefit from eventual associated fetal, obstetrical or familial features. We report a patient with an isolated corpus callosum agenesia that led to the discovery of a similar malformation in her father. This observation demonstrates that some forms of isolated and familial corpus callosum agenesia could have a favorable outcome. However, the difficulty of the assessment of prognosis in isolated corpus callosum agenesia is emphasized and the question of parental RMI exploration in such a peculiar context is raised.