Long-term follow-up of Philadelphia chromosome-positive (Ph+) chronic myeloid leukaemia (CML) in children and adolescents managed at a single institution over a 20-year period

Chronic myeloid leukaemia (CML) is rare in childhood. In our Institution we managed 30 consecutive Ph+CML patients aged <18 years, according to our adults’ guidelines. Patients with HLA-identical related donor (RD) underwent stem cell transplant (SCT). Since 1989, patients without RD were systematically treated with -interferon (IFN) (median dosage: 6 MU/day). Of 18/19 evaluable patients, 17 (94.5%) achieved haematologic response (HR), 11/17 (65%) cytogenetic response (CyR), complete (CCyR) in 4 (23.5%). Three patients remain in CCyR, 2 achieved BCR-ABL transcript disappearance. Of 13 patients without CCyR, 5 underwent SCT, 4 switched to STI571, 4 progressed. All patients receiving STI571 in chronic phase (CP) obtained sustained CCyR and 3 a persistent molecular response. 8-year survival among IFN-treated patients, censored or not for subsequent therapies, is 62% and 63%. Overall, 13/30 patients underwent SCT: 5 HLA-identical-RD, 5 matched unrelated donor, 2 mismatched-RD, 1 unrelated mismatched umbilical cord blood. Eight allotransplanted patients (6/6 in 1st CP) are in cytogenetic and molecular remission with 8-year survival of 61% from SCT and 69% from diagnosis. In our 20-year experience, the use of IFN in children without matched RD led to prolonged cytogenetic and molecular responses and long-term survival, without impairing the outcome of subsequent SCT

Investigating fragmentation of gas structures in OB cluster-forming molecular clump G33.92+0.11 with 1000 AU resolution observations of ALMA

We report new, $\sim$1000 AU spatial resolution observations of 225 GHz dust continuum emission towards the OB cluster-forming molecular clump G33.92+0.11. On parsec scales, this molecular clump presents a morphology with several arm-like dense gas structures surrounding the two central massive ($\gtrsim$100 $M_{\odot}$) cores. From the new, higher resolution observations, we identified 28 localized, spatially compact dust continuum emission sources, which may be candidates of young stellar objects. Only one of them is not embedded within known arm-like (or elongated) dense gas structures. The spatial separations of these compact sources can be very well explained by Jeans lengths. We found that G33.92+0.11 may be consistently described by a marginally centrifugally supported, Toomre unstable accretion flow which is approximately in a face-on projection. The arm-like overdensities are natural consequence of the Toomre instability, which can fragment to form young stellar objects in shorter time scales than the timescale of the global clump contraction. On our resolved spatial scales, there is not yet evidence that the fragmentation is halted by turbulence, magnetic field, or stellar feedback.Comment: 24 pages, 18 figures. Accepted to publish on December 04, 2018; updated to arXiv on December 05, 201

The properties of the inner disk around HL Tau: Multi-wavelength modeling of the dust emission

We conducted a detailed radiative transfer modeling of the dust emission from the circumstellar disk around HL Tau. The goal of our study is to derive the surface density profile of the inner disk and its structure. In addition to the Atacama Large Millimeter/submillimeter Array images at Band 3 (2.9mm), Band 6 (1.3mm), and Band 7 (0.87mm), the most recent Karl G. Jansky Very Large Array (VLA) observations at 7mm were included in the analysis. A simulated annealing algorithm was invoked to search for the optimum model. The radiative transfer analysis demonstrates that most radial components (i.e., >6AU) of the disk become optically thin at a wavelength of 7mm, which allows us to constrain, for the first time, the dust density distribution in the inner region of the disk. We found that a homogeneous grain size distribution is not sufficient to explain the observed images at different wavelengths simultaneously, while models with a shallower grain size distribution in the inner disk work well. We found clear evidence that larger grains are trapped in the first bright ring. Our results imply that dust evolution has already taken place in the disk at a relatively young (i.e., ~1Myr) age. We compared the midplane temperature distribution, optical depth, and properties of various dust rings with those reported previously. Using the Toomre parameter, we briefly discussed the gravitational instability as a potential mechanism for the origin of the dust clump detected in the first bright ring via the VLA observations.Comment: Accepted for publication in A&A (10 pages

Reduced expression of frataxin extends the lifespan of C. elegans, N. Ventura et al.

Defects in the expression of the mitochondrial protein frataxin cause Friedreich's ataxia, an hereditary neurodegenerative syndrome characterized by progressive ataxia and associated with reduced life expectancy in humans. Homozygous inactivation of the frataxin gene results in embryonic lethality in mice, suggesting that frataxin is required for organismic survival. Intriguingly, the inactivation of many mitochondrial genes in the nematode Caenorhabditis elegans by RNAi extends lifespan. We therefore investigated whether inactivation of frataxin by RNAi-mediated suppression of the frataxin homolog gene (frh-1) would also prolong lifespan in the nematode. Frataxin-deficient animals have a small body size, reduced fertility and altered responses to oxidative stress. Importantly, frataxin suppression by RNAi significantly extends lifespan in C. elegans

The Radial Distribution of Dust Particles in the HL Tau Disk from ALMA and VLA Observations

Understanding planet formation requires one to discern how dust grows in protoplanetary disks. An important parameter to measure in disks is the maximum dust grain size present. This is usually estimated through measurements of the dust opacity at different millimeter wavelengths assuming optically thin emission and dust opacity dominated by absorption. However, Atacama Large Millimeter/submillimeter Array (ALMA) observations have shown that these assumptions might not be correct in the case of protoplanetary disks, leading to overestimation of particle sizes and to underestimation of the disk\u27s mass. Here, we present an analysis of high-quality ALMA and Very Large Array images of the HL Tau protoplanetary disk, covering a wide range of wavelengths, from 0.8 mm to 1 cm, and with a physical resolution of ~7.35 au. We describe a procedure to analyze a set of millimeter images without any assumption about the optical depth of the emission, and including the effects of absorption and scattering in the dust opacity. This procedure allows us to obtain the dust temperature, the dust surface density, and the maximum particle size at each radius. In the HL Tau disk, we found that particles have already grown to a few millimeters in size. We detect differences in the dust properties between dark and bright rings, with dark rings containing low dust density and small dust particles. Different features in the HL Tau disk seem to have different origins. Planet–disk interactions can explain substructure in the external half of the disk, but the internal rings seem to be associated with the presence of snow lines of several molecules

The VLA view of the HL Tau Disk - Disk Mass, Grain Evolution, and Early Planet Formation

The first long-baseline ALMA campaign resolved the disk around the young star HL Tau into a number of axisymmetric bright and dark rings. Despite the very young age of HL Tau these structures have been interpreted as signatures for the presence of (proto)planets. The ALMA images triggered numerous theoretical studies based on disk-planet interactions, magnetically driven disk structures, and grain evolution. Of special interest are the inner parts of disks, where terrestrial planets are expected to form. However, the emission from these regions in HL Tau turned out to be optically thick at all ALMA wavelengths, preventing the derivation of surface density profiles and grain size distributions. Here, we present the most sensitive images of HL Tau obtained to date with the Karl G. Jansky Very Large Array at 7.0 mm wavelength with a spatial resolution comparable to the ALMA images. At this long wavelength the dust emission from HL Tau is optically thin, allowing a comprehensive study of the inner disk. We obtain a total disk dust mass of 0.001 - 0.003 Msun, depending on the assumed opacity and disk temperature. Our optically thin data also indicate fast grain growth, fragmentation, and formation of dense clumps in the inner densest parts of the disk. Our results suggest that the HL Tau disk may be actually in a very early stage of planetary formation, with planets not already formed in the gaps but in the process of future formation in the bright rings.Comment: Accepted by The Astrophysical Journal Letter

A 1.3 mm SMA Survey of 29 Variable Young Stellar Objects

© 2018 ESO. Reproduced with permission from Astronomy & Astrophysics.Context. Young stellar objects (YSOs) may undergo periods of active accretion (outbursts), during which the protostellar accretion rate is temporarily enhanced by a few orders of magnitude. Whether or not these accretion outburst YSOs possess similar dust and gas reservoirs to each other, and whether or not their dust and gas reservoirs are similar as quiescent YSOs, are issues yet to be clarified.Aims. The aim of this work is to characterize the millimeter thermal dust emission properties of a statistically significant sample of long and short duration accretion outburst YSOs (i.e., FUors and EXors) and the spectroscopically identified candidates of accretion outbursting YSOs (i.e., FUor-like objects). Methods. We have carried out extensive Submillimeter Array (SMA) observations mostly at ~225 GHz (1.33 mm) and ~272 GHz (1.10 mm), from 2008 to 2017. We covered accretion outburst YSOs located at 3σ significance. Detected sources except for the two cases of V883 Ori and NGC 2071 MM3 were observed with ~1″ angular resolution. Overall our observed targets show a systematically higher millimeter luminosity distribution than those of the M ∗ > 0.3 MClass II YSOs in the nearby (400 pc) low-mass star-forming molecular clouds (e.g., Taurus, Lupus, Upp Scorpio, and Chameleon I). In addition, at 1 mm our observed confirmed binaries or triple-system sources are systematically fainter than the rest of the sources even though their 1 mm fluxes are broadly distributed. We may have detected ∼30-60% millimeter flux variability from V2494 Cyg and V2495 Cyg, from the observations separated by approximately one year.Peer reviewe