Proper-motion binaries in the Hipparcos catalogue: comparison with radial velocity data

Context. This paper is the last in a series devoted to the analysis of the binary content of the Hipparcos Catalogue. Aims. The comparison of the proper motions constructed from positions spanning a short (Hipparcos) or long time (Tycho-2) makes it possible to uncover binaries with periods of the order of or somewhat larger than the short time span (in this case, the 3 yr duration of the Hipparcos mission), since the unrecognised orbital motion will then add to the proper motion. Methods. A list of candidate proper motion binaries is constructed from a carefully designed χ2 test evaluating the statistical significance of the difference between the Tycho-2 and Hipparcos proper motions for 103 134 stars in common between the two catalogues (excluding components of visual systems). Since similar lists of proper-motion binaries have already been constructed, the present paper focuses on the evaluation of the detection efficiency of proper-motion binaries, using different kinds of control data (mostly radial velocities). The detection rate for entries from the Ninth Catalogue of Spectroscopic Binary Orbits (SB9) is evaluated, as well as for stars like barium stars, which are known to be all binaries, and finally for spectroscopic binaries identified from radial velocity data in the Geneva-Copenhagen survey of F and G dwarfs in the solar neighbourhood. Results. Proper motion binaries are efficiently detected for systems with parallaxes in excess of ∼20 mas, and periods in the range 1000-30 000 d. The shortest periods in this range (1000-2000 d, i.e. once to twice the duration of the Hipparcos mission) may appear only as DMSA/G binaries (accelerated proper motion in the Hipparcos Double and Multiple System Annex). Proper motion binaries detected among SB9 systems having periods shorter than about 400 d hint at triple systems, the proper-motion binary involving a component with a longer orbital period. A list of 19 candidate triple systems is provided. Binaries suspected of having low-mass (brown-dwarf-like) companions are listed as well. Among the 37 barium stars with parallaxes larger than 5 mas, only 7 exhibit no evidence for duplicity whatsoever (be it spectroscopic or astrometric). Finally, the fraction of proper-motion binaries shows no significant variation among the various (regular) spectral classes, when due account is taken for the detection biases. © ESO 2007.

Déficits immunitaires primaires: diagnostic, prise en charge, quelques perspectives.

Severe primary immunodeficiencies (PID) are rare; their global incidence is comparable to that of childhood leukemia; they include more than 100 different entities. Clinical manifestations are: unusually severe or frequent infections or infections that do not respond to adequate treatment; an increased risk of certain malignancies; sometimes auto-immune manifestations. Delayed diagnosis and management of PID can lead to severe and irreversible complications or to death. PID can become manifest only in the adult; in common variable immune deficiency, the median age at diagnosis is between the 2nd and the 3rd decade of life. PID are often transmitted genetically; recent progresses in molecular biology have allowed more precise and earlier, including antenatal, diagnosis. Molecular treatment of 3 infants with a severe immunodeficiency has recently been achieved in April 2000. Those progresses were mostly based on the study of immunodeficiency databases. We present here the work of a Belgian group specialized in PID; meetings have started in June 1997. This group establishes guidelines for the diagnosis and treatment of PID, adapted to the local situation. The elaboration of a national register of PID is also underway; this has to provide all guaranties of anonymity to patients and families. Such a register already exists at the European level; it has provided the basis for new diagnostic and therapeutic possibilities. The inclusion of Belgian data in this register should allow essential progresses essential for our patients.

Expression of liver-specific member of the 3β-hydroxysteroid dehydrogenase family, an isoform possessing an almost exclusive 3-ketosteroid reductase activity

We have recently characterized two types of rat 3β-hydroxysteroid dehydrogenase/Δ5-Δ4 isomerase (3β-HSD) isoenzymes expressed in adrenals and gonads. In addition, we have cloned a third type of cDNA encoding a predicted type III 3β-HSD protein specifically expressed in the male rat liver which shares 80% similarity with the two other isoenzymes. Transient expression in human HeLa cells of the cDNAs reveals that the type III 3β-HSD protein does not display oxidative activity for the classical substrates of 3β-HSD, in contrast to the type I 3β-HSD isoenzyme. However, in the presence of NADH, type III isoenzyme, in common with the type I isoform, converts 5α-androstane-3,17-dione (A-dione) and 5α-dihydrotestosterone (DHT) to the corresponding 3β-hydroxysteroids. In fact, the type I and the type III isoenzymes have the same affinity for DHT with K(m) values of 5.05 and 6.16 μM, respectively. When NADPH is used as cofactor, the affinity for DHT of the type III isoform becomes higher than that of the type I isoform with K(m) values of 0.12 and 1.18 μM, respectively. The type III isoform is thus a 3-ketoreductase using NADPH as preferred cofactor which is responsible for the conversion of 3-keto-saturated steroids such as DHT and A-dione into less active steroids.