Noonan syndrome is an inherited disorder caused by alterations in the RAS-MAPK pathway. There have been several identified genotype–phenotype associations made with respect to congenital cardiac lesions and Noonan syndrome variants, but limited data exist regarding single ventricle disease in this population. Here, we report two patients with PTPN11-related Noonan syndrome and hypoplastic left heart syndrome variants.

This report covers the period July 1996 to June 1999. It has been prepared by the President of the Commission with contributions from the members of the Organizing Committee and Dr. E.M. Corsini. As discussed in Kyoto and decided by the Organizing Committee, the report is meant to be in the “short” version.

A self-consistent analysis of near-UV, HST/FOC images of the elliptical galaxy NGC 4552 is used to show that its central spike has brightened by a factor ˜ 4.5 between 1991 and 1993, and has decreased its luminosity by a factor ˜ 2.0 between 1993 and 1996. A strong UV continuum over the energy distribution of the underlying galaxy is concurrently revealed shortward of λ ˜ 3200 Å by our FOS spectra extending from the near-UV to red wavelengths. Nuclear emission-line profiles of both permitted and forbidden lines are best modelled with a combination of broad and narrow components, with FWHM of ˜ 3000 km s−1 and ˜ 700 km s−1, respectively. Current diagnostics based on the emission line intensity ratios definitely places the spike among AGNs, just at the border between Seyferts and LINERs. This evidence argues for the variable central spike being produced by a modest accretion event onto a central massive black hole (BH), with the accreted material having possibly being stripped from a star in a close fly-by with the BH. In this regard, one has to look at NGC 4552 as the faintest known AGN.

HST images of the E galaxies NGC 1399 and NGC 4552 and of the Sa NGC 2681 were obtained with FOC f/96 through the filters F175W, F220W, F275W and F342W. The most important discovery of these observations is the clear detection of central spikes in NGC 2681 and NGC 4552, that are prominent in each of the FOC images. No spike is detected in NGC 1399. The spikes appear unresolved by the 0″.07 core of the Pre-COSTAR PSF, corresponding to a physical size <~2-3 pc. We estimate V = 16.4 for the spike in NGC 2681 and V = 18.4 for that in NGC 4552, or ~ 107 L ⊙ in both cases. The spikes contribute less than a few percent of the UV flux sampled by IUE. We can offer three options for their nature: a central burst of star formation; effects of stellar interaction in a very dense environment; and a low level AGN. The first two options are currently entertained for the origin of the very dense star cluster at the center of our own Galaxy, whose size and luminosity are comparable to the corresponding limits for the two UV spikes (Genzel et al. 1994). A much fainter central spike (~ 105 L ⊙) is also seen at the center of NGC 205, and appears to be consistent with a modest burst of star formation (Bertola et al. 1995).

Motions of nearby elliptical galaxies reveal a large-scale velocity flow relative to cosmic rest towards the point 1=307±10, b = 9±10. The data are fit best by a two-component flow model. The smaller component is due to Virgo, which induces a velocity at the Local Group of 250 km/s. The main flow is due to a more massive concentration located a distance of 4350±350 km/s towards 1=307, b=9, which induces a local velocity here of 570±60 km/s. This larger component falls off away from the mass concentration roughly as r−1. The Centaurus double cluster and its neighbors are in the direction of the mass concentration but are in the foreground and are falling into it. Galaxy counts, radial velocity surveys, and the motions of nearby spirals are consistent with the above model. The IRAS dipole results are less clear but may also be consistent. There is evidence that the distant mass concentration is non-spherical, with the Centaurus cloud a substantial sub-condensation in the foreground. The formal agreement of the large-scale flow with biased (b=2) cold dark matter is low, but the simple methods used so far to assess this are uncertain. The main weakness of the present data in comparing to theory is the fact that they do not penetrate far enough to show the velocity field on all sides of the mass concentration. Sphericity and total extent of the flow are therefore still unknown.

We have used a new distance estimator for elliptical galaxies to determine the peculiar velocities, with respect to a uniform Hubble flow, of approximately 400 galaxies. The relative distances of five clusters in common with those of Aaronson et al. (1981, 1986), based on the infrared Tully-Fisher relation for spirals, are in good agreement.

We do not see the reflex of the Local Group motion with respect to the microwave background out to recession velocities of 6000 km s−1. Rather, the frame of elliptical galaxies appears to be moving with respect to the microwave background with a velocity of 600 km s−1 towards 1 = 312°, b = +6°. This motion is consistent with a re-analysis of the Rubin et al. (1976) data on the magnitude-diameter relation for ScI galaxies and with the nearby and cluster samples of Aaronson et al. (1982, 1986).

The surface layer of single crystal GaAs that has been bombarded with high energy (2-15 MeV) ions is strained and contains many defects, but it remains crystalline. We have studied the isochronal annealing behavior of this layer using phonon shifts and widths, which were detected by Raman measurements. We do not find any sharp annealing stages to 500 C, at which temperature the phonons have returned to their positions in the unbombarded crystals. These results are consistent with the idea that the phonon shifts are primarily due to antisites and their complexes with other primary defects. However, other extended defects may still be present, and the Raman data show some evidence for this.