The BiPo-3 detector.

The BiPo-3 detector is a low radioactive detector dedicated to measuring ultra-low natural contaminations of 208Tl and 214Bi in thin materials, initially developed to measure the radiopurity of the double ß decay source foils of the SuperNEMO experiment at the µBq/kg level. The BiPo-3 technique consists in installing the foil of interest between two thin ultra-radiopure scintillators coupled to low radioactive photomultipliers. The design and performances of the detector are presented. In this paper, the final results of the 208Tl and 214Bi activity measurements of the first enriched 82Se foils are reported for the first time, showing the capability of the detector to reach sensitivities in the range of some µBq/kg.

Search for Neutrinoless Quadruple-ß Decay of

We report the results of a first experimental search for lepton number violation by four units in the neutrinoless quadruple-ß decay of ^{150}Nd using a total exposure of 0.19 kg yr recorded with the NEMO-3 detector at the Modane Underground Laboratory. We find no evidence of this decay and set lower limits on the half-life in the range T_{1/2}>(1.1-3.2)×10^{21} yr at the 90% C.L., depending on the model used for the kinematic distributions of the emitted electrons.

Drug screening on Hutchinson Gilford progeria pluripotent stem cells reveals aminopyrimidines as new modulators of farnesylation.

Hutchinson-Gilford progeria syndrome (HGPS) is a rare genetic disorder characterized by a dramatic appearance of premature aging. HGPS is due to a single-base substitution in exon 11 of the LMNA gene (c.1824C>T) leading to the production of a toxic form of the prelamin A protein called progerin. Because farnesylation process had been shown to control progerin toxicity, in this study we have developed a screening method permitting to identify new pharmacological inhibitors of farnesylation. For this, we have used the unique potential of pluripotent stem cells to have access to an unlimited and relevant biological resource and test 21,608 small molecules. This study identified several compounds, called monoaminopyrimidines, which target two key enzymes of the farnesylation process, farnesyl pyrophosphate synthase and farnesyl transferase, and rescue in vitro phenotypes associated with HGPS. Our results opens up new therapeutic possibilities for the treatment of HGPS by identifying a new family of protein farnesylation inhibitors, and which may also be applicable to cancers and diseases associated with mutations that involve farnesylated proteins.

Measurement of the ßß decay half-life of 130Te with the NEMO-3 detector.

We report results from the NEMO-3 experiment based on an exposure of 1275 days with 661 g of (130)Te in the form of enriched and natural tellurium foils. The ßß decay rate of (130)Te is found to be greater than zero with a significance of 7.7 standard deviations and the half-life is measured to be T(½)(2ν) = [7.0 ± 0.9(stat) ± 1.1(syst)] × 10(20) yr. This represents the most precise measurement of this half-life yet published and the first real-time observation of this decay.

[Pulmonary embolism and pregnancy: report of a case; review of the literature]. / Embolie pulmonaire et grossesse: à propos d'une observation; revue de la littérature.

Pulmonary embolism occurring during pregnancy is a rare accident but that still brings about a high mother mortality; it seems to be five to six times more frequent during the pregnancy and the post-partum than for non-parturient women who don't take any estro-progestogens, pulmonary embolism would involve complications for 0.5/1000 pregnancies before delivery. As it presents a lot of diagnostic problems, it is under-estimated. The vascular radiological examinations expose the foetus or embryo to considerable radiation and to a risk of foetal hypothyroidism leading to backwardness. The lung perfusion scanning has the advantage of not injecting iodine but is not specific. Fortunately, some medical examinations such as plethysmography or Doppler echography are safe and can also guide the clinician. As far as therapy is concerned, intravenous heparin is the first intention treatment, it can be replaced subsequently by subcutaneous heparin (low molecular weight heparin). In case of heavy pulmonary embolism endangering the vital prognosis of the patient, in case of clinical or biological resistance to the medical treatment, it could be necessary to perform a pulmonary embolectomy with, if necessary, vena cava interruption with insertion of a mechanical filter.

[Neonatal abstinence syndrome: current and future aspects]. / Le syndrome d'abstinence néonatal: le présent et le futur.

Pregnant heroin-addicted women constitute a major social problem that should not be ignored. Newborns may develop a neonatal abstinence syndrome (NAS). They present with behavioural troubles running a typical clinical course. The level of severity of NAS will be accurately determined, leading to definition of the most appropriate therapy. The best therapeutic formula appears to be paregoric elixir, mixed with phenobarbital if necessary. Least severe cases can be easily controlled by appropriate surrounding conditions. Pharmacological as well as physiopathological effects of opiates are described. Little is known about the long-term effects of opiate exposure; they apparently include frequent instrumental troubles. At the present time, the rapid intervention of a multidisciplinary team is recommended, taking charge of the mothers who should receive methadone in progressively tapering doses.

[Interpreting hypereosinophilia]. / Interprétation d'une hyperéosinophilie.

The diagnosis of a blood hypereosinophilia requires a review of various disease processes such as blood dyscrasias, skin diseases, auto-immune diseases, asthma, parasitic infections, enteropathies, etc. A complete check-up including clinical, laboratory and radiological data allows the identification of an etiology in a majority of cases. Nevertheless, the etiology of hypereosinophilia remains obscure in some cases. Among these unexplained situations, the rare idiopathic hypereosinophilic syndrome requires special attention because of its fatal course due to cardiac involvement which can remain clinically silent for a long period of time. Therefore, the follow-up should include repeated echocardiograms. At the present time, the usual therapy for the most severe cases includes corticosteroids and hydroxyurea.