The reaction pp->pppi0pi0 has been investigated at a beam energy of 1.4 GeV using the WASA-at-COSY facility. The total cross section is found to be (324 +- 21_systematic +- 58_normalization) mub. In ...order to to study the production mechanism, differential kinematical distributions have been evaluated. The differential distributions indicate that both initial state protons are excited into intermediate Delta(1232) resonances, each decaying into a proton and a single pion, thereby producing the pion pair in the final state. No significant contribution of the Roper resonance N*(1440) via its decay into a proton and two pions is found
. Ringdén O, Labopin M, Beelen DW, Volin L, Ehninger G, Finke J, Greinix HT, Kyrcz‐Krzemien S, Bunjes D, Brinch L, Niederwieser D, Arnold R, Mohty M, Rocha V, for the Acute Leukaemia Working Party ...of the European Group for Blood and Marrow Transplantation (EBMT) (Karolinska University Hospital Huddinge, Sweden; CEREST‐TC EBMT, Paris, France; University of Duisburg‐Essen, Germany; Helsinki University Central Hospital, Helsinki, Finland; University Hospital, Dresden, Germany; University Hospital, Freiburg, Germany; Medical University Vienna, Vienna, Austria; Medical University of Silesia, Katowice, Poland; University Hospital, Ulm, Germany; Rikshospitalet, Oslo, Norway; University Hospital, Leipzig, Germany; Charité University Hospital, Berlin, Germany; Université de Nantes, Nantes, France; and Hôpital Saint‐Louis, Paris, France). Bone marrow or peripheral blood stem cell transplantation from unrelated donors in adult patients with acute myeloid leukaemia, an Acute Leukaemia Working Party analysis in 2262 patients. J Intern Med 2012; 272: 472–483.
Background. No survival benefit of using blood stem cells instead of bone marrow (BM) has been shown in matched unrelated donor (MUD) transplantation.
Design and methods. In a retrospective registry analysis, we compared the use of blood stem cells (n = 1502) and BM (n = 760) from unrelated donors in patients aged 18–60 years with acute myeloid leukaemia (AML) undergoing myeloablative conditioning between 1997 and 2008. The blood stem cell recipients were older (P < 0.01), had more advanced disease (P < 0.0001) and received less total body irradiation (P < 0.0001) and more antithymocyte globulin (P = 0.01).
Results. Recovery of neutrophils and platelets was faster with blood stem cells (P < 0.0001). The incidence of acute graft‐versus‐host disease (GVHD) was similar, but there was more chronic GVHD in the blood stem cell group hazard ratio (HR) = 1.29, P = 0.02. There were no significant differences in nonrelapse mortality (NRM), relapse incidence and leukaemia‐free survival (LFS) between the two groups amongst patients with AML in remission. In patients with advanced leukaemia, NRM was lower (HR = 0.61, P = 0.02) and LFS was prolonged (HR = 0.67, P = 0.002) when blood stem cells were used. At 3 years, LFS for all patients, regardless of remission status, was 41% for both treatment groups. The outcome was not affected after multivariable analysis adjusted for confounders.
Conclusion. Blood stem cells compared with BM in MUD transplantation for patients with AML in remission resulted in the same rates of LFS. In patients with advanced leukaemia, the blood stem cell group had reduced NRM and improved LFS.
Exclusive measurements of the quasi-free $pn \to pp\pi^0\pi^-$ reaction have
been performed by means of $pd$ collisions at $T_p$ = 1.2 GeV using the WASA
detector setup at COSY. Total and ...differential cross sections have been
obtained covering the energy region $\sqrt s$ = (2.35 - 2.46) GeV, which
includes the region of the ABC effect and its associated resonance structure.
No ABC effect, {\it i.e.} low-mass enhancement is found in the
$\pi^0\pi^-$-invariant mass spectrum -- in agreement with the constraint from
Bose statistics that the isovector pion pair can not be in relative s-wave. At
the upper end of the covered energy region $t$-channel processes for Roper,
$\Delta(1600)$ and $\Delta\Delta$ excitations provide a reasonable description
of the data, but at low energies the measured cross sections are much larger
than predicted by such processes. Adding a resonance amplitude for the
resonance at $m$=~2.37 GeV with $\Gamma$ =~70 MeV and $I(J^P)=~0(3^+)$ observed
recently in $pn \to d\pi^0\pi^0$ and $pn \to d\pi^+\pi^-$ reactions leads to an
agreement with the data also at low energies.
Phys. Rev. C 88, 014004 (2013) An exclusive measurement of the dd -> 3He n \pi 0 reaction was carried at a
beam momentum of p = 1.2 GeV/c using the WASA-at-COSY facility. For the first
time data on ...the total cross section as well as differential distributions were
obtained. The data are described with a phenomenological approach based on a
combination of a quasi-free model and a partial wave expansion for three-body
reaction. The total cross section is found to be \sigma(tot) = (2.89 +-
0.01(stat) +- 0.06(sys) +- 0.29(norm)) \mu b. The contribution of the
quasi-free processes (with the neutron being target or beam spectator) accounts
for 38% of the total cross section and dominates the differential distributions
in specific regions of the phase space. The remaining part of the cross section
can be described within a partial wave decomposition indicating the
significance of p-wave contributions in the final state.
Phys. Lett. B 726 (2013), 187 The presently world largest data sample of pi0 --> gamma e+e- decays
containing nearly 5E5 events was collected using the WASA detector at COSY. A
search for a dark ...photon U produced in the pi0 --> gamma U --> gamma e+e- decay
from the pp-->pp\pi^0 reaction was carried out. An upper limit on the square of
the U-gamma mixing strength parameter epsilon^2 of 5e-6 at 90% CL was obtained
for the mass range 20 MeV <M_U< 100 MeV. This result together with other recent
experimental limits significantly reduces the M_U vs. \epsilon^2 parameter
space preferred by the measured value of the muon anomalous magnetic moment.
Using the production reactions \(pd\to {}^3\mbox{He}\,\omega\) and \(pp\to pp\omega\), the Dalitz plot distribution for the \(\omega \to \pi^+ \pi^- \pi^0\) decay is studied with the WASA detector at ...COSY, based on a combined data sample of \( (4.408\pm 0.042) \times 10^4\) events. The Dalitz plot density is parametrised by a product of the \(P\)-wave phase space and a polynomial expansion in the normalised polar Dalitz plot variables \(Z\) and \(\phi\). For the first time, a deviation from pure \(P\)-wave phase space is observed with a significance of \(4.1\sigma\). The deviation is parametrised by a linear term \(1+2\alpha Z\), with \(\alpha\) determined to be \(+0.147\pm0.036\), consistent with the expectations of \(\rho\)-meson-type final-state interactions of the \(P\)-wave pion pairs.
Cyclophosphamide plus intravenous busulfan has not been compared with cyclophosphamide plus total body irradiation (TBI) in adults with advanced refractory acute myeloid leukaemia before allogeneic ...haemopoietic stem-cell transplantation (HCT). We aimed to assess whether survival of patients receiving ablative intravenous busulfan-based conditioning regimens before a related or volunteer-unrelated donor HCT for refractory acute myeloid leukaemia is not inferior to that of patients receiving an ablative TBI-based regimen.
In this retrospective, multicentre, registry-based study, we obtained data for patients (aged >18 years) with refractory acute myeloid leukaemia in active phase of disease, who had received HCT from an HLA-identical sibling or an unrelated donor after intravenous busulfan plus cyclophosphamide or cyclophosphamide plus TBI conditioning between 2000 and 2012. Data was obtained from the European Group for Blood and Marrow Transplantation registry. The primary endpoints of the study were overall survival and leukaemia-free survival.
We obtained data for 514 patients who had received intravenous busulfan plus cyclophosphamide and 338 patients who had received cyclophosphamide plus TBI. The median percentage of blasts before HCT did not differ significantly between groups (20% range 5-100; IQR 10-32 in the intravenous busulfan plus cyclophosphamide group vs 16% 5-95; 9-33 in the cyclophosphamide plus TBI group; p=0·16). Overall survival at 2 years did not differ between the groups in the univariate analysis (31·2% 95% CI 26·8-35·5 with intravenous busulfan plus cyclophosphamide vs 33·4% 28·1-38·7 wth cyclophosphamide plus TBI; p=0·65). Leukaemia-free survival at 2 years also did not differ between groups (25·0% 95% CI 21·0-29·0 vs 28·4% 23·4-33·5; p=0·47). In multivariable analysis adjusting for differences between both groups, no difference was noted between the two groups in terms of overall survival (hazard ratio HR 0·99 95% CI 0·83-1·20; p=0·95) or leukaemia-free survival (HR 0·97 0·81-1·16; p=0·71). Main causes of non-relapse mortality were graft-versus-host disease (49 10% in the intravenous busulfan plus cyclophosphamide group vs 25 7% in the cyclophosphamide plus TBI group) and infection (36 7% vs 18 5%).
From a practical standpoint, the use of intravenous busulfan plus cyclophosphamide is likely to be a valid and efficient alternative to cyclophosphamide plus TBI conditioning regimen for patients with refractory acute myeloid leukaemia, especially for those transplant centres without access to radiation facilities.
None.
Genetic Drivers of Kidney Defects in the DiGeorge Syndrome Lopez-Rivera, Esther; Liu, Yangfan P; Verbitsky, Miguel ...
New England journal of medicine/The New England journal of medicine,
02/2017, Letnik:
376, Številka:
8
Journal Article
Recenzirano
Odprti dostop
The DiGeorge syndrome, the most common of the microdeletion syndromes, affects multiple organs, including the heart, the nervous system, and the kidney. It is caused by deletions on chromosome ...22q11.2; the genetic driver of the kidney defects is unknown.
We conducted a genomewide search for structural variants in two cohorts: 2080 patients with congenital kidney and urinary tract anomalies and 22,094 controls. We performed exome and targeted resequencing in samples obtained from 586 additional patients with congenital kidney anomalies. We also carried out functional studies using zebrafish and mice.
We identified heterozygous deletions of 22q11.2 in 1.1% of the patients with congenital kidney anomalies and in 0.01% of population controls (odds ratio, 81.5; P=4.5×10
). We localized the main drivers of renal disease in the DiGeorge syndrome to a 370-kb region containing nine genes. In zebrafish embryos, an induced loss of function in snap29, aifm3, and crkl resulted in renal defects; the loss of crkl alone was sufficient to induce defects. Five of 586 patients with congenital urinary anomalies had newly identified, heterozygous protein-altering variants, including a premature termination codon, in CRKL. The inactivation of Crkl in the mouse model induced developmental defects similar to those observed in patients with congenital urinary anomalies.
We identified a recurrent 370-kb deletion at the 22q11.2 locus as a driver of kidney defects in the DiGeorge syndrome and in sporadic congenital kidney and urinary tract anomalies. Of the nine genes at this locus, SNAP29, AIFM3, and CRKL appear to be critical to the phenotype, with haploinsufficiency of CRKL emerging as the main genetic driver. (Funded by the National Institutes of Health and others.).
We report on a high-statistics measurement of the most basic double pionic fusion reaction \(\vec{n}p \to d\pi^0\pi^0\) over the energy region of the \(d^*(2380)\) resonance by use of a polarized ...deuteron beam and observing the double fusion reaction in the quasifree scattering mode. The measurements were performed with the WASA detector setup at COSY. The data reveal substantial analyzing powers and confirm conclusions about the \(d^*\) resonance obtained from unpolarized measurements. We also confirm the previous unpolarized data obtained under complementary kinematic conditions.
An exclusive measurement of the decay eta --> pi+ pi- gamma has been performed at the WASA facility at COSY. The eta mesons were produced in the fusion reaction pd --> 3He X at a proton beam momentum ...of 1.7 GeV/c. Efficiency corrected differential distributions have been extracted based on 13340\pm140 events after background subtraction. The measured pion angular distribution is consistent with a relative p-wave of the two-pion system, whereas the measured photon energy spectrum was found at variance with the simplest gauge invariant matrix element of eta --> pi+ pi- gamma. A parameterization of the data can be achieved by the additional inclusion of the empirical pion vector form factor multiplied by a first-order polynomial in the squared invariant mass of the pi+ pi- system.
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