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Two decades have elapsed since our publication of ‘What kind of illness is anorexia nervosa?’. The question remains whether our understanding of anorexia nervosa and its treatment thereof has evolved over this time. The verdict is disappointing at best. Our current gold standard treatments remain over-valued and clinical outcomes are modest at best. Those in our field are haunted by the constant reminder that anorexia nervosa carries the highest mortality rate of any psychiatric disorder. This cannot continue and demands immediate action. In this essay, we tackle the myths that bedevil our field and explore a deeper phenotyping of anorexia nervosa. We argue that we can no longer declare agnostic views of the disorder or conceive treatments that are “brainless”: it is incumbent upon us to challenge the prevailing zeitgeist and reconceptualise anorexia nervosa. Here we provide a roadmap for the future.

Variants in CLCN4, which encodes the chloride/hydrogen ion exchanger CIC-4 prominently expressed in brain, were recently described to cause X-linked intellectual disability and epilepsy. We present detailed phenotypic information on 52 individuals from 16 families with CLCN4-related disorder: 5 affected females and 2 affected males with a de novo variant in CLCN4 (6 individuals previously unreported) and 27 affected males, 3 affected females and 15 asymptomatic female carriers from 9 families with inherited CLCN4 variants (4 families previously unreported). Intellectual disability ranged from borderline to profound. Behavioral and psychiatric disorders were common in both child- and adulthood, and included autistic features, mood disorders, obsessive–compulsive behaviors and hetero- and autoaggression. Epilepsy was common, with severity ranging from epileptic encephalopathy to well-controlled seizures. Several affected individuals showed white matter changes on cerebral neuroimaging and progressive neurological symptoms, including movement disorders and spasticity. Heterozygous females can be as severely affected as males. The variability of symptoms in females is not correlated with the X inactivation pattern studied in their blood. The mutation spectrum includes frameshift, missense and splice site variants and one single-exon deletion. All missense variants were predicted to affect CLCN4’s function based on in silico tools and either segregated with the phenotype in the family or were de novo. Pathogenicity of all previously unreported missense variants was further supported by electrophysiological studies in Xenopus laevis oocytes. We compare CLCN4-related disorder with conditions related to dysfunction of other members of the CLC family.

Après un bref rappel des bases théoriques de la méthode de mesurage du débit d'un liquide par dilution d'un traceur et de ses limites d'utilisation, les différents traceurs couramment utilisés en rivière et en réseau, tant de distribution d'eau que d'assainissement, font l'objet d'une étude comparée des caractéristiques de mesurage et des coûts. La normalisation, tant internationale que nationale, de cette méthode permet d'éviter le foisonnement de " méthodes simplifiées " dont les résultats, peu fiables, ont souvent conduit à rejeter son utilisation. L'automatisation, surtout pour le diagnostic de réseaux, est possible et des études d'utilisation de nouveaux traceurs, tant de type classique (chimiques, fluorescents ou radiocactifs) que de types " poudreux " et ferromagnétique semblent prometteuses.

Several case reports and open label trials describe olanzapine in the treatment of anorexia nervosa (AN). We report 5 adolescents with AN who received olanzapine in addition to psychotherapy for their eating disorder. Body mass index (BMI) of each case increased while on olanzapine. At doses of 5 mg per day and above, patients reported decreased anxiety around eating, improved sleep, and decreased rumination about food and body concerns. Morning sedation was the most commonly reported adverse effect. Olanzapine appeared to be useful in addition to psychotherapy for these adolescents. This report augments a limited literature on the treatment of this disorder, and an almost nonexistent literature specific to pharmacotherapy for adolescents with AN.

The rarity of identical twins with anorexia nervosa (AN) makes formulating an effective treatment approach for this subset of individuals quite difficult. Clinicians may be applying treatment strategies that have been designed for individuals with an eating disorder. However, these approaches might have to be modified for the treatment of identical twins. The few available case studies have not discussed the challenges a clinician faces when treating identical twins with AN. In this report we describe the presentation of the case of adolescent monozygotic twins who were simultaneously concordant for AN. We discuss their unique presentation and our approach to their treatment.

Major hepatectomy is associated with a high operative risk in patients with cirrhosis. Between June 1983 and December 1991, 33 cirrhotic patients, 31 with good liver function, underwent major liver resection for a large hepatic primary cancer. The hospital mortality rate was 3 per cent; the only death resulted from liver failure after extended right hepatectomy. None of the patients had variceal bleeding during the postoperative period. Survival rates at 1, 2 and 3 years were 66, 43 and 37 per cent respectively. Recurrence was the most common reason for late death. These results suggest that the operative risk of major hepatectomy in cirrhotic patients with a large tumour and good liver function is comparable to that of minor liver resection. Late survival is also similar to that of patients with a small tumour. Cirrhotic patients with hepatic carcinoma and good liver function are suitable for major hepatectomy.

We have identified a point mutation in one α1(I) collagen allele (COL1A1) of a child with the type IV osteogenesis imperfecta phenotype. When compared to parental and control samples, skin fibroblasts of the proband synthesized two populations of type I collagen molecules. One population was normal; the other was delayed in secretion and electrophoretic migration due to post-translational overmodification. Two-dimensional gel electrophoresis of the CNBr peptides demonstrated a gradient of overmodification beginning near the carboxyl-terminal CB peptides. This predicts that the mutation delaying helix formation is near the carboxyl-terminal end of one of the component chains of type I collagen. The mRNA of the patient was probed with overlapping antisense riboprobes to type I collagen cDNA. Cleavage of a mismatch in RNA/RNA hybrids of RNase A allowed the location of the mutation to a 225-base pair region of α1(I) cDNA. The mismatch was not present in RNA/RNA hybrids from either parent. This region of both α1(I) alleles of the patient was isolated by screening a λZAP cDNA library. Sequence determination of both alleles demonstrated a single nucleotide change, G→A, resulting in the substitution of a serine for a glycine at amino acid residue 832. This point mutation occurs in the coding region for α1(I) CB6 and is concordant with the protein data. The finding of a glycine substitution in an α1(I) chain of a patient with the milder type IV osteogenesis imperfecta phenotype requires modification of current molecular models for types II and IV osteogenesis imperfecta.

First short theoretical basis of flow measurement by tracer scattering and feasibility are recalled. Different kinds of usual tracers in river and sewerage waters are compared in respect of measurements features and costs. National and international standardization of such technologies is required to avoid swarming of unreliable reduced process, often leading to believe that tracing is unfitted. Automation is available, mainly in sewers check up, and good luck is allowed for new tracers : soluble (chemical, fluorescent, radioactive) or powdery (ferrimagnetie).Apr?s un bref rappel des bases th?oriques de la m?thode de mesurage du d?bit d'un liquide par dilution d'un traceur et de ses limites d'utilisation, les diff?rents traceurs couramment utilis?s en rivi?re et en r?seau, tant de distribution d'eau que d'assainissement, font l'objet d'une ?tude compar?e des caract?ristiques de mesurage et des co?ts. La normalisation, tant internationale que nationale, de cette m?thode permet d'?viter le foisonnement de " m?thodes simplifi?es " dont les r?sultats, peu fiables, ont souvent conduit ? rejeter son utilisation. L'automatisation, surtout pour le diagnostic de r?seaux, est possible et des ?tudes d'utilisation de nouveaux traceurs, tant de type classique (chimiques, fluorescents ou radiocactifs) que de types " poudreux " et ferromagn?tique semblent prometteuses.

Abstract: A genomic DNA library of the bacterium Bacillus pumilus PLS was constructed and the β-xylosidase gene (xynB) was amplified from a 3-kb genomic DNA fragment with the aid of the polymerase chain reaction technique. The amplified xynB gene was inserted between the yeast alcohol dehydrogenase II gene promoter (ADH2 P ) and terminator (ADH2 T ) sequences on a multicopy episomal plasmid (pDLG11). The xynB gene was also fused in-frame to the secretion signal sequence of the yeast mating pheromone α-factor (MFα1 S ) before insertion between the ADH2 P and ADH2 T sequences on a similar multicopy episomal plasmid (pDLG12). The resulting construct ADH2 P -MFα1 S -xynB-ADH2 T was designated XLO1. Both plasmids pDLG11 and pDLG12 were introduced into Saccharomyces cerevisiae but only the expression of the XLO1 gene yielded biologically functional β-xylosidase. The total β-xylosidase activity remained cell-associated with a maximum activity of 0.09 nkat/ml obtained when the recombinant S. cerevisiae strain was grown for 143 h in synthetic medium. The temperature and pH optima of the recombinant Xlo1 enzyme were 45–50 °C and pH 6.6 respectively. The enzyme was thermostable at 45 °C; however, at 60 °C most of the Xlo1 was inactive after 5 min.

Background. There is no agreement on the management of patients with cirrhosis and recurrent variceal bleeding after failure of medical or endoscopic treatments or both. Portal systemic shunts are highly effective in preventing rebleeding but are associated with a high incidence of chronic encephalopathy. This study compared the results of a slightly modified Sugiura procedure (esophageal transection plus esophagogastric devascularization plus splenectomy) with those of nonselective portal systemic shunts in patients with previous variceal bleeding. Methods. Fifty-four patients were included in this randomized controlled study between January 1984 and April 1989. The major end point was chronic encephalopathy. Secondary end points were recurrent variceal bleeding, survival, ascites, and hepatocellular carcinoma. Results. Twenty-seven patients were assigned to each group. The rate of chronic encephalopathy was significantly (p=0.002) lower after modified Sugiura procedure than after portal systemic shunt. Recurrent variceal bleeding was more frequent after modified Sugiura procedure than after portal systemic shunt, but the difference is not significant. One-, two-, and three-year survival rates were 93%, 81%, and 67%, respectively, in the modified Sugiura group and 78%, 66%, and 39%, respectively, in the portal systemic shunt group (p=0.044). Conclusions. These results suggest that the modified Sugiura procedure is better overall than the nonselective portal systemic shunt in the management of patients with cirrhosis and recurrent variceal bleeding. Although the rebleeding rate is higher after the modified Sugiura procedure, this does not seem to affect mortality in these patients.

Background: Family-based treatment (FBT) is an efficacious outpatient intervention for young people diagnosed with Anorexia Nervosa (AN). To date, treatment to protocol has relied on standard face-to-face delivery. Face-to-face therapy is subject to geographic, temporal and human factors, rendering it particularly susceptible to inequities and disruption. This has resulted in poorer service provision for rural and regional families, and recently a significant challenge to providing face-to-face services during the COVID-19 global pandemic. The present study examines whether FBT for AN can be successfully translated to a digital delivery platform to address these access issues. Method: Forty young people aged 12 to 18 years who meet DSM-5 diagnostic criteria for AN, and live in a rural or regional setting, will along with their family be recruited to the study. Trained therapists will provide 18 sessions of FBT over 9 months via telemedicine to the home of the young person and their family. The analysis will examine treatment effectiveness, feasibility, acceptability, and cost-effectiveness. Discussion: The study addresses the treatment needs of families not able to attend face-to-face clinical services for evidence-based treatment for eating disorders. This might be due to several barriers, including a lack of local services or long travel distances to services. There has been a recent and unprecedented demand for telemedicine to facilitate the continuity of care during COVID-19 despite geographical circumstances. If delivering treatment in this modality is clinically and economically effective and feasible, it will facilitate access to potentially lifesaving, evidence-based treatments for families formerly unable to access such care and provide evidence for the continuity of services when and where face-to-face treatment is not feasible.

No relevant conflicts of interest to declare.

Depuis 2012, la déclaration obligatoire (DO) des mésothéliomes par les médecins (cliniciens, pathologistes) à l’Agence régionale de santé vise à renforcer la surveillance des mésothéliomes (tous sites anatomiques, territoire national) et à améliorer la connaissance des expositions, notamment environnementales à l’amiante. Pour cela, la DO est complétée depuis 2014 par des enquêtes d’exposition professionnelle et environnementale dans 9 régions pilotes. Après plus de 3ans de fonctionnement, l’Institut de veille sanitaire fait un bilan du dispositif de DO et des enquêtes d’exposition.

Les expositions à l’amiante évoluent (professions et secteurs d’activité concernés, expositions environnementales) et leur surveillance est essentielle, notamment pour améliorer les actions de prévention. Les données des systèmes de surveillance des mésothéliomes permettent de mieux connaître les expositions professionnelles. Elles montrent une proportion importante de cas pour lesquels aucune exposition professionnelle à l’amiante n’est identifiée, en particulier parmi les femmes. Pour ces cas, la pertinence et la faisabilité de la surveillance des expositions environnementales a été montrée.

Le Programme national de surveillance du mésothéliome (PNSM) a été mis en place en 1998 par Santé publique France. Parmi ses objectifs figure une meilleure connaissance des expositions professionnelles et extra-professionnelles à l’amiante et aux autres facteurs étudiés.

L’insécurité alimentaire fait partie des facteurs possiblement impliqués dans les associations entre pauvreté et obésité, notamment chez les femmes. L’objectif de cette étude était de décrire les prévalences d’insécurité alimentaire, et d’analyser son association avec la corpulence des femmes recourant à l’aide alimentaire.

Considers military readiness as a moral responsibility of a commander in the United States. Effect of maintaining a constant combat capability on military functioning; Elements of a true esprit de corps; Assessment on an organization's state of readiness by leaders; Requirements of the moral responsibility for readiness.