Background PKD2-related autosomal dominant polycystic kidney disease (ADPKD) is widely acknowledged to be of milder severity than PKD1-related disease, but population-based studies depicting the exact burden of the disease are lacking. We aimed to revisit PKD2 prevalence, clinical presentation, mutation spectrum, and prognosis through the Genkyst cohort. Study Design Case series, January 2010 to March 2016. Settings & Participants Genkyst study participants are individuals older than 18 years from 22 nephrology centers from western France with a diagnosis of ADPKD based on Pei criteria or at least 10 bilateral kidney cysts in the absence of a familial history. Publicly available whole-exome sequencing data from the ExAC database were used to provide an estimate of the genetic prevalence of the disease. Outcomes Molecular analysis of PKD1 and PKD2 genes. Renal survival, age- and sex-adjusted estimated glomerular filtration rate. Results The Genkyst cohort included 293 patients with PKD2 mutations (203 pedigrees). PKD2 patients with a nephrology follow-up corresponded to 0.63 (95% CI, 0.54–0.72)/10,000 in Brittany, while PKD2 genetic prevalence was calculated at 1.64 (95% CI, 1.10–3.51)/10,000 inhabitants in the European population. Median age at diagnosis was 42 years. Flank pain was reported in 38.9%; macroscopic hematuria, in 31.1%; and cyst infections, in 15.3% of patients. At age 60 years, the cumulative probability of end-stage renal disease (ESRD) was 9.8% (95% CI, 5.2%–14.4%), whereas the probability of hypertension was 75.2% (95% CI, 68.5%–81.9%). Although there was no sex influence on renal survival, men had lower kidney function than women. Nontruncating mutations (n = 36) were associated with higher age-adjusted estimated glomerular filtration rates. Among the 18 patients with more severe outcomes (ESRD before age 60), 44% had associated conditions or nephropathies likely to account for the early progression to ESRD. Limitations Younger patients and patients presenting with milder forms of PKD2-related disease may not be diagnosed or referred to nephrology centers. Conclusions Patients with PKD2-related ADPKD typically present with mild disease. In case of accelerated degradation of kidney function, a concomitant nephropathy should be ruled out.

Abstract. The emerging ISO/IEC 29110 standard Lifecycle profiles for Very Small Entities has at its core a Management and Engineering Guides which is targeted at very small entity (enterprise, organization, department or project) having up to 25 people, to assist them unlock the potential benefits of using standards which are specifically designed to address there needs. The developers of the standard, ISO/IEC JCT1/SC7 Working Group 24 (WG24), recommend the use of pilot projects as a mean to trial the adoption of the new International standard in small organisations. Accordingly an ISO/IEC 29110 pilot project has been established between the Software Engineering group of Brest University and a 14 person company with the aim of establishing an engineering discipline for a new web-based project. This paper details the lessons learned from the pilot project and based on our experiences with using ISO/IEC 29110 we identify a potential deficiency and accordingly propose new process area, "Infrastructure and Support" for include in the future evolution of ISO/IEC 29110 Process Profiles.

The in vitro activity of 8 cephalosporins – cephalothin, cefamandole, cefoxitin, cefuroxime, cefotaxime, cefoperazone, moxalactam and ceftriaxone (Rocephin®) was studied on 109 strains of Neisseria gonorrhoeae and 60 strains of Neisseria meningitidis, isolated from pathological material. Determination of the MICs of these antibiotics by an agar dilution method shows that on N. gonorrhoeae ceftriaxone (geometric mean of the MICs: 0.0008 μg/ml) is the most active, followed by cefotaxime (0.001 μg/ml); cefoperazone (0.008 μg/ml) and moxalactam (0.01 μg/ml) are one-tenth as active; then come cefuroxime (0.05 μg/ml), cefamandole (0.10 μg/ml) and finally cephalothin (0.26 μg/ml) and cefoxitin (0.26 μg/ml). The least susceptibility to penicillin and, to a greater degree, the production of β-lactamase (8 strains) affect the level of susceptibility to these cephalosporins, but the MICs always remain relatively low.

High-quality education helps in finding a job -but student skills heterogeneity and student reluctance to move towards a professional attitude are important barriers to employability. We re-engineered some of the technical courses of a Masters in software development using a Problem-Based Learning (PBL) approach. Although initial results are encouraging, the cost of using PBL must be taken into account. Two aspects are particularly expensive: (i) set-up of the software development practicum, a mid-sized information system and its environment; (ii) screenwriting of problem-based learning scenarios, including procurement of input artefacts.