BK virus infection in hematopoietic stem cell transplant recipients: frequency, risk factors, and association with postengraftment hemorrhagic cystitis.

Blood samples from 132 consecutive hematopoietic stem cell transplant recipients were obtained and tested weekly for BK virus DNA by use of quantitative real-time PCR. Forty-four patients (33%) developed BK viremia at a median of 41 days (range, 9-91 days) after transplantation. Patients with hemorrhagic cystitis that occurred after platelet engraftment had higher levels of viremia than did patients without hemorrhagic cystitis (median, 9.7x10(3) vs. 0 copies/mL; P=.008) and patients with hemorrhagic cystitis that occurred before platelet engraftment (median, 9.7x10(3) vs. 0 copies/mL; P=.0006). BK viremia also was strongly associated with postengraftment hemorrhagic cystitis in a time-dependent analysis (P=.004).

Les professions de la santé: guide des formations

[Table des matières] I. Formations de base : Aide-soignant - Ambulancier - Diététicien - Ergothérapeute - Hygiéniste dentaire - Infirmier niveau I; niveau II - Laborantin medical - Pédicure ; podologue - Physiothérapeute - Sage-femme - Technicien en radiologie médicale - Technicien en salle d'opération - Assistant social. II. Formations continues : Ambulancier - Ergothérapeutes - Centre romand d'éducation permanente, Association suisse des infirmières - Laborantin médical - Formation continue en physiothérapie- Technicien en radiologie médicale, Contrôle de qualité en radiodiagnostic - Technicien en radiologie médicale, Formation continue en radioprotection et technique radiologique - Laborantin médical - Programme du service de formation continue du CHUV - Activités de formation continue du centre de formation H+ - Introduction à l'éthique en psychiatrie. III. Formations complémentaires : ESEI - Les activités de formation complémentaire du centre de formation H+ - Infirmier, Formation complémentaire de clinicien niveau I - Infirmier, Formation complémentaire en anesthésie - Infirmier, Formation complémentaire en soins intensifs - Infirmier, Formation complémentaire en salle d'opération - Laborantin médical, Formation supérieure et de cadres - Laborantin médical, Formation complémentaire spécialisée cytotechnicien - Assistant social - Formation de physiothérapeute enseignant. IV. Formations universitaires : Séminaire de gestion hospitalière - Bioéthique - Diplôme en économie et administration de la santé - Management des Institutions de santé - Ingéniérie biomédicale - Certificat en nutrition humaine - Cours postgrade de santé au travail.

International periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis syndrome cohort: description of distinct phenotypes in 301 patients.

OBJECTIVES: The aims of this study were to describe the clinical features of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) and identify distinct phenotypes in a large cohort of patients from different countries. METHODS: We established a web-based multicentre cohort through an international collaboration within the periodic fevers working party of the Pediatric Rheumatology European Society (PReS). The inclusion criterion was a diagnosis of PFAPA given by an experienced paediatric rheumatologist participating in an international working group on periodic fever syndromes. RESULTS: Of the 301 patients included from the 15 centres, 271 had pharyngitis, 236 cervical adenitis, 171 oral aphthosis and 132 with all three clinical features. A total of 228 patients presented with additional symptoms (131 gastrointestinal symptoms, 86 arthralgias and/or myalgias, 36 skin rashes, 8 neurological symptoms). Thirty-one patients had disease onset after 5 years and they reported more additional symptoms. A positive family history for recurrent fever or recurrent tonsillitis was found in 81 patients (26.9%). Genetic testing for monogenic periodic fever syndromes was performed on 111 patients, who reported fewer occurrences of oral aphthosis or additional symptoms. Twenty-four patients reported symptoms (oral aphthosis and malaise) outside the flares. The CRP was >50 mg/l in the majority (131/190) of the patients tested during the fever. CONCLUSION: We describe the largest cohort of PFAPA patients presented so far. We confirm that PFAPA may present with varied clinical manifestations and we show the limitations of the commonly used diagnostic criteria. Based on detailed analysis of this cohort, a consensus definition of PFAPA with better-defined criteria should be proposed.

SOMATIC MUTATIONS IN THE KREBS CYCLE ENZYME ISOCITRATE DEHYDROGENASE 1 (IDH1) CAUSE METAPHYSEAL ENCHONDROMATOSIS WITH D-2-HYDROXYGLUTARIC ACIDURIA

Metaphyseal chondromatosis with hydroxyglutaric aciduria (MC-HGA) is a generalized skeletal dysplasia, accompanied by urinary excretion of D-2- hydroxyglutarate (HGA), and variable cerebral involvement. By wholeexome sequencing 2 unrelated patients with MC-HGA, we have found mutations in isocitrate dehydrogenase 1 (IDH1) at codon 132, as apparent somatic mosaicism. IDH1 is a key enzyme of the Krebs cycle, which converts isocitrate into alpha-ketoglutarate (a-KG). Mutations at IDH1 Arg132 residue have originally been identified in different tumour types (isolated gliomas, leukemias, and chondrosarcomas). These mutations trans-specify the enzyme activity resulting in HGA accumulation and a-KG depletion. This induces activation of hypoxia-inducible factor 1-alpha (HIF-1a), an important regulator of chondrocyte proliferation at the growth plate. Differently from Arg132 somatic mutations found in isolated tumours, themutation in our patientsmust have occurred very early in embryogenesis to cause a generalized dysplasia with involvement of all long bones metaphyses and mutation detectability in blood. Identical mutations have subsequently been identified in chondromas excised from patients with multiple chondromatosis (Ollier disease). Tissue distribution of themutationmay explain variable cerebral involvement and the susceptibility to develop tumours in other organs. The postulated pathophysiology ofMC-HGA points out the link between Krebs cycle, hypoxia sensing and bone growth.

Video analysis software increases the interrater reliability of video gait assessments in children with cerebral palsy.

The aim of this study was to determine the effect of using video analysis software on the interrater reliability of visual assessments of gait videos in children with cerebral palsy. Two clinicians viewed the same random selection of 20 sagittal and frontal video recordings of 12 children with cerebral palsy routinely acquired during outpatient rehabilitation clinics. Both observers rated these videos in a random sequence for each lower limb using the Observational Gait Scale, once with standard video software and another with video analysis software (Dartfish(®)) which can perform angle and timing measurements. The video analysis software improved interrater agreement, measured by weighted Cohen's kappas, for the total score (κ 0.778→0.809) and all of the items that required angle and/or timing measurements (knee position mid-stance κ 0.344→0.591; hindfoot position mid-stance κ 0.160→0.346; foot contact mid-stance κ 0.700→0.854; timing of heel rise κ 0.769→0.835). The use of video analysis software is an efficient approach to improve the reliability of visual video assessments.

Type I IFNs at the interface between cutaneous immunity and epidermal remodeling.

Type I IFNs are key cytokines in antiviral host defense. Preferentially expressed by plasmacytoid dendritic cells, type I IFNs are induced by viral infection and in common skin wounds. In this issue, Tohyama et al. identify a new link between type I IFNs and epidermal remodeling, by showing that type I IFNs specifically upregulate IL-22R expression on keratinocytes and, thereby, IL-22-mediated Stat3 phosphorylation in keratinocytes. The findings suggest that type I IFNs play dual roles in human skin: first, they induce immune activation with the induction of IL-22-producing T cells; second, they provide the interface between immune activation and epidermal remodeling by increasing keratinocyte responsiveness to IL-22.

Quantification of human adenoviruses in European recreational waters

The presence of human adenoviruses in recreational water might cause disease in the population upon exposure. Human adenoviruses detected by PCR could also serve as indicators of the virological water quality. In order to assess the applicability of human adenoviruses to the evaluation of the faecal contamination in European bathing waters, a real-time quantitative PCR assay was developed for the quantification of human adenoviruses in 132 samples collected from 24 different recreational marine and freshwater sites in nine European countries.Selected samples presenting positive nested-PCR results for human adenoviruses were analyzed using quantitative PCR and 80 samples from a total of 132 produced quantitative results with mean values of 3.2x102 10 per 100 ml of water, human adenovirus 41 being the most prevalent serotype. Human adenoviruses were quantified in samples from all 15 surveillance laboratories. Statistical analysis showed no homogeneous linear relation between humanadenoviruses and E. coli, intestinal enterococci or somatic coliphages concentrations in the tested samples when considering all the data together. Significant correlations between human adenoviruses and at least one of the other indicators were observed only when data from individual Laboratories were considered. The quantification of human adenoviruses may provide complementary information in relation to the use of bacterial standards in the control of water quality in bathing water.

Surveillance of adenoviruses and noroviruses in European recreational waters

Exposure to human pathogenic viruses in recreational waters has been shown to cause disease outbreaks. In the context of Article 14 of the revised European Bathing Waters Directive 2006/7/EC (rBWD, CEU, 2006) a Europe-wide surveillance study was carried out to determine the frequency of occurrence of two human enteric viruses in recreational waters. Adenoviruses were selected based on their near-universal shedding and environmental survival, and noroviruses (NoV) selected as being the most prevalent gastroenteritis agent worldwide. Concentration of marine and freshwater samples was done by adsorption/elution followed by molecular detection by (RT)-PCR. Out of 1410 samples, 553 (39.2%) were positive for one or more of the target viruses. Adenoviruses, detected in 36.4% of samples, were more prevalent than noroviruses (9.4%), with 3.5% GI and 6.2% GII, some samples being positive for both GI and GII. Of 513 human adenovirus-positive samples, 63 (12.3%) were also norovirus-positive, whereas 69 (7.7%) norovirus-positive samples were adenovirus-negative. More freshwater samples than marine water samples were virus-positive. Out of a small selection of samples tested for adenovirus infectivity, approximately one-quarter were positive. Sixty percent of 132 nested-PCR adenovirus-positive samples analysed by quantitative PCR gave a mean value of over 3000 genome copies per L of water. The simultaneous detection of infectious adenovirus and of adenovirus and NoV by (RT)PCR suggests that the presence of infectious viruses in recreational waters may constitute a public health risk upon exposure. These studies support the case for considering adenoviruses as an indicator of bathing water quality.