139 research outputs found

    A precise modeling of Phoebe's rotation

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    Although the rotation of some Saturn's satellites in spin-orbit has already been studied by several authors, this is not the case of the rotation of Phoebe, which has the particularity of being non resonant. The purpose of the paper is to determine for the first time and with precision its precession-nutation motion. We adopt an Hamiltonian formalism of the motion of rotation of rigid celestial body set up by Kinoshita (1977) based on Andoyer variables and canonical equations. First we calculate Phoebe's obliquity at J2000,0 from available astronomical data as well as the gravitational perturbation due to Saturn on Phoebe rotational motion. Then we carry out a numerical integration and we compare our results for the precession rate and the nutation coefficients with pure analytical model. Our results for Phoebe obliquity (23{\deg}95) and Phoebe precession rate (5580".65/cy) are very close to the respective values for the Earth. Moreover the amplitudes of the nutations (26" peak to peak for the nutaton in longitude and 8" for the nutation in obliquity) are of the same order as the respective amplitudes for the Earth. We give complete tables of nutation, obtained from a FFT analysis starting from the numerical signals. We show that a pure analytical model of the nutation is not accurate due to the fact that Phoebe orbital elements e, M and Ls are far from having a simple linear behaviour. The precession and nutation of Phoebe have been calculated for the first time in this paper. We should keep on the study in the future by studying the additional gravitational effects of the Sun, of the large satellites as Titan, as well as Saturn dynamical ellipticity.Comment: 11 pages,15 figures, accepted for publication in A&

    Personalised therapy in follicular lymphoma - is the dial turning?

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    Follicular lymphoma is the most common indolent lymphoma accounting for approximately 20%–25% of all new non-Hodgkin lymphoma diagnoses in western countries. Whilst outcomes are mostly favorable, the spectrum of clinical phenotypes includes high-risk groups with significantly inferior outcomes. This review discusses recent updates in risk stratification and treatment approaches from upfront treatment for limited and advanced stage follicular lymphoma to the growing options for relapsed, refractory disease with perspectives on how to approach this from a personalized lens. Notable gaps remain on how one can precisely and prospectively select optimal treatment for patients based on varying risks, with an anticipation that an increased understanding of the biology of these different phenotypes and increasing refinement of imaging- and biomarker-based tools will, in time, allow these gaps to be closed

    Mutation update for the GPC3 gene involved in Simpson-Golabi-Behmel syndrome and review of the literature

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    Simpson-Golabi-Behmel syndrome (SGBS) is an X-linked multiple congenital anomalies and overgrowth syndrome caused by a defect in the glypican-3 gene (GPC3). Until now, GPC3 mutations have been reported in isolated cases or small series and the global genotypic spectrum of these mutations has never been delineated. In this study, we review the 57 previously described GPC3 mutations and significantly expand this mutational spectrum with the description of 29 novel mutations. Compiling our data and those of the literature, we provide an overview of 86 distinct GPC3 mutations identified in 120 unrelated families, ranging from single nucleotide variations to complex genomic rearrangements and dispersed throughout the entire coding region of GPC3. The vast majority of them are deletions or truncating mutations (frameshift, nonsense mutations) predicted to result in a loss-of-function. Missense mutations are rare and the two which were functionally characterized, impaired GPC3 function by preventing GPC3 cleavage and cell surface addressing respectively. This report by describing for the first time the wide mutational spectrum of GPC3 could help clinicians and geneticists in interpreting GPC3 variants identified incidentally by high-throughput sequencing technologies and also reinforces the need for functional validation of non-truncating mutations (missense, in frame mutations, duplications)

    Charge-radius change and nuclear moments in the heavy tin isotopes from laser spectroscopy: Charge radius of 132^{132}Sn

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    NESTER ACCLaser spectroscopy measurements have been carried out on the neutron-rich tin isotopes with the COMPLIS experimental setup. Using the 5s25p25s^25p^2 3P05s25p6s^3P_0 \rightarrow 5s^25_p6s 3P1^3P_1 optical transition, hyperfine spectra of 126132^{126-132}Sn and 125,127,129131Snm^{125,127,129-131}Sn^m were recorded for the first time. The nuclear moments and the mean square charge radius variation (δ)wereextracted.Fromthequadrupolemomentvalues,thesenucleiappeartobespherical.Themagneticmomentsmeasuredarethuscomparedwiththosepredictedbysphericalbasisapproaches.Fromthemeasured\delta) were extracted. From the quadrupole moment values, these nuclei appear to be spherical. The magnetic moments measured are thus compared with those predicted by spherical basis approaches. From the measured \delta, the absolute charge radii of these isotopes were deduced in particular that of the doubly magic 132^{132}Sn nucleus. The comparison of the results with several mean-field-type calculations have shown that dynamical effects play an important role in the tin isotopes

    Release properties of UCx_x and molten U targets

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    The release properties of UCx_x and molten U thick targets associated with a Nier- Bernas ion source have been studied. Two experimental methods are used to extract the release time. Results are presented and discussed for Kr, Cd, I and Xe

    Integrative analysis of a phase 2 trial combining lenalidomide with CHOP in angioimmunoblastic T-cell lymphoma.

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    Angioimmunoblastic T-cell lymphoma (AITL) is a frequent T-cell lymphoma in the elderly population that has a poor prognosis when treated with cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) therapy. Lenalidomide, which has been safely combined with CHOP to treat B-cell lymphoma, has shown efficacy as a single agent in AITL treatment. We performed a multicentric phase 2 trial combining 25 mg lenalidomide daily for 14 days per cycle with 8 cycles of CHOP21 in previously untreated AITL patients aged 60 to 80 years. The primary objective was the complete metabolic response (CMR) rate at the end of treatment. Seventy-eight of the 80 patients enrolled were included in the efficacy and safety analysis. CMR was achieved in 32 (41%; 95% confidence interval [CI], 30%-52.7%) patients, which was below the prespecified CMR rate of 55% defined as success in the study. The 2-year progression-free survival (PFS) was 42.1% (95% CI, 30.9%-52.8%), and the 2-year overall survival was 59.2% (95% CI, 47.3%-69.3%). The most common toxicities were hematologic and led to treatment discontinuation in 15% of patients. This large prospective and uniform series of AITL treatment data was used to perform an integrative analysis of clinical, pathologic, biologic, and molecular data. TET2, RHOA, DNMT3A, and IDH2 mutations were present in 78%, 54%, 32%, and 22% of patients, respectively. IDH2 mutations were associated with distinct pathologic and clinical features and DNMT3A was associated with shorter PFS. In conclusion, the combination of lenalidomide and CHOP did not improve the CMR in AITL patients. This trial clarified the clinical impact of recurrent mutations in AITL. This trial was registered at www.clincialtrials.gov as #NCT01553786

    Predictive value of PET response combined with baseline metabolic tumor volume in peripheral T-cell lymphoma patients

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    Peripheral T-cell lymphoma (PTCL) is a heterogeneous group of aggressive non-Hodgkin lymphomas with poor outcomes on current therapy. We investigated whether response assessed with PET/CT combined with baseline total metabolic tumor volume (TMTV) could detect early relapse or refractory disease. Methods: From 7 European centers, 140 patients with nodal PTCL who underwent baseline PET/CT were selected. Forty-three had interim PET (iPET) performed after 2 cycles (iPET2), 95 had iPET performed after 3 or 4 cycles (iPET3/4), and 96 had end-of-treatment PET (eotPET). Baseline TMTV was computed with a 41% SUVmax threshold, and PET response was reported using the Deauville 5-point scale. Results: With a median of 43 mo of follow-up, the 2-y progression-free survival (PFS) and overall survival (OS) were 51% and 67%, respectively. iPET2-positive patients (Deauville score ≥ 4) had a significantly worse outcome than iPET2-negative patients (P 230 cm3 and iPET3/4-negative [59%/84%]; TMTV ≤ 230 cm3 and iPET3/4-positive [42%/50%]; TMTV > 230 cm3 and iPET3/4-positive [0%/18%]). Conclusion: iPET response is predictive of outcome and allows early detection of high-risk PTCL patients. Combining iPET with TMTV improves risk stratification in individual patients

    High yield of surveillance in patients diagnosed with constitutional mismatch repair deficiency

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    BackgroundConstitutional mismatch repair deficiency (CMMRD) is a rare autosomal recessively inherited syndrome that is caused by biallelic pathogenic variants of the mismatch repair genes. It is characterised by the development of multiple tumours in the first and second decade of life including brain, gastrointestinal and haematological tumours often resulting in early death. In order to improve the prognosis of these patients, the European collaborative group 'care for CMMRD' developed a surveillance programme in 2014 and established a registry of patients with CMMRD in Paris. The aim of the study was to evaluate the outcome of this programme. MethodsTwenty-two patients with a definitive diagnosis of CMMRD and with at least one follow-up study were selected from the registry. Medical data on the outcome of surveillance were collected from these patients. ResultsDuring a mean follow-up of 4 years, the programme detected eight malignant tumours including three brain tumours, three upper gastrointestinal cancers and two colorectal cancers. Most tumours could successfully be treated. In addition, many adenomas were detected in the duodenum, and colorectum and subsequently removed. Seven patients developed a symptomatic malignancy, including two brain tumours, one small bowel cancer and four haematological malignancies. At the end of the follow-up, 16 out of 22 patients (73%) who participated in the surveillance programme were still alive. ConclusionThe study suggests a beneficial effect of surveillance of the digestive tract and brains.Cellular mechanisms in basic and clinical gastroenterology and hepatolog
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