Lambda production in central Pb+Pb collisions at CERN-SPS energies

In this paper we present recent results from the NA49 experiment for $\Lambda$ and $\bar{\Lambda}$ hyperons produced in central Pb+Pb collisions at 40, 80 and 158 A$\cdot$GeV. Transverse mass spectra and rapidity distributions for $\Lambda$ are shown for all three energies. The shape of the rapidity distribution becomes flatter with increasing beam energy. The multiplicities at mid-rapidity as well as the total yields are studied as a function of collision energy including AGS measurements. The ratio $\Lambda/\pi$ at mid-rapidity and in 4$\pi$ has a maximum around 40 A$\cdot$GeV. In addition, $\bar{\Lambda}$ rapidity distributions have been measured at 40 and 80 A$\cdot$GeV, which allows to study the $\bar{\Lambda}$/$\Lambda$ ratio.Comment: SQM proceedings. J. Phys. G: Nucl. Part. Phys.: submitte

A novel missense mutation in Dax-1 with an unusual presentation of X-linked adrenal hypoplasia congenita

A male presented at age 2.2 years with a 6-week history of intermittent vomiting and hyperpigmentation. Investigations showed salt wasting with hyperkalaemia, a grossly impaired cortisol response to ACTH stimulation, elevated renin and ACTH. Family history revealed that two maternal uncles had died soon after birth. A third uncle failed to thrive during infancy but improved with a course of cortisone, then being untreated until further investigation revealed adrenal insufficiency. A fourth uncle died aged 10 days, with urinary salt loss and hypoplastic adrenal glands at postmortem. Molecular studies on the proband, his mother, maternal grandmother, and surviving uncle showed a novel C to G substitution at nucleotide position 794 (missense mutation T265R) in the DAX1 (NR0B1) gene. The proband has responded well to steroid replacement but has proved sensitive to 9α-fludrocortisone treatment, developing hypertension on a dose of 133 μg/m2/day. At 8.8 years he was noted to have testicular volumes of 4 ml, despite no other evidence of secondary sexual development and prepubertal gonadotrophin levels. Novel features of this family include a novel DAX1 mutation, marked variability in age of presentation, hypertension on ‘standard’ doses of 9α-fludrocortisone and mild testicular enla