| 1. |
- Klionsky, Daniel J., et al.
(författare)
-
Guidelines for the use and interpretation of assays for monitoring autophagy
- 2012
-
Ingår i: Autophagy. - : Informa UK Limited. - 1554-8635 .- 1554-8627. ; 8:4, s. 445-544
-
Forskningsöversikt (refereegranskat)abstract
- In 2008 we published the first set of guidelines for standardizing research in autophagy. Since then, research on this topic has continued to accelerate, and many new scientists have entered the field. Our knowledge base and relevant new technologies have also been expanding. Accordingly, it is important to update these guidelines for monitoring autophagy in different organisms. Various reviews have described the range of assays that have been used for this purpose. Nevertheless, there continues to be confusion regarding acceptable methods to measure autophagy, especially in multicellular eukaryotes. A key point that needs to be emphasized is that there is a difference between measurements that monitor the numbers or volume of autophagic elements (e.g., autophagosomes or autolysosomes) at any stage of the autophagic process vs. those that measure flux through the autophagy pathway (i.e., the complete process); thus, a block in macroautophagy that results in autophagosome accumulation needs to be differentiated from stimuli that result in increased autophagic activity, defined as increased autophagy induction coupled with increased delivery to, and degradation within, lysosomes (in most higher eukaryotes and some protists such as Dictyostelium) or the vacuole (in plants and fungi). In other words, it is especially important that investigators new to the field understand that the appearance of more autophagosomes does not necessarily equate with more autophagy. In fact, in many cases, autophagosomes accumulate because of a block in trafficking to lysosomes without a concomitant change in autophagosome biogenesis, whereas an increase in autolysosomes may reflect a reduction in degradative activity. Here, we present a set of guidelines for the selection and interpretation of methods for use by investigators who aim to examine macroautophagy and related processes, as well as for reviewers who need to provide realistic and reasonable critiques of papers that are focused on these processes. These guidelines are not meant to be a formulaic set of rules, because the appropriate assays depend in part on the question being asked and the system being used. In addition, we emphasize that no individual assay is guaranteed to be the most appropriate one in every situation, and we strongly recommend the use of multiple assays to monitor autophagy. In these guidelines, we consider these various methods of assessing autophagy and what information can, or cannot, be obtained from them. Finally, by discussing the merits and limits of particular autophagy assays, we hope to encourage technical innovation in the field.
|
|
| 2. |
- Al Jebali, Ramsey, et al.
(författare)
-
A helium gas scintillator active target for photoreaction measurements
- 2015
-
Ingår i: European Physical Journal A. Hadrons and Nuclei. - : Springer Science and Business Media LLC. - 1434-6001. ; 51:10
-
Tidskriftsartikel (refereegranskat)abstract
- A multi-cell He gas scintillator active target, designed for the measurement of photoreaction cross sections, is described. The target has four main chambers, giving an overall thickness of 0.103 g/cm(3) at an operating pressure of 2MPa. Scintillations are read out by photomultiplier tubes and the addition of small amounts of N-2 to the He, to shift the scintillation emission from UV to visible, is discussed. First results of measurements at the MAX IV Laboratory tagged-photon facility show that the target has a timing resolution of around 1 ns and can cope well with a high-flux photon beam. The determination of reaction cross sections from target yields relies on a Monte Carlo simulation, which considers scintillation light transport, photodisintegration processes in He-4, background photon interactions in target windows and interactions of the reaction-product particles in the gas and target container. The predictions of this simulation are compared to the measured target response.
|
|
| 3. |
- Barbui, Tiziano, et al.
(författare)
-
Philadelphia-Negative Classical Myeloproliferative Neoplasms : Critical Concepts and Management Recommendations From European LeukemiaNet
- 2011
-
Ingår i: Journal of Clinical Oncology. - 0732-183X .- 1527-7755. ; 29:6, s. 761-770
-
Tidskriftsartikel (refereegranskat)abstract
- We present a review of critical concepts and produce recommendations on the management of Philadelphia-negative classical myeloproliferative neoplasms, including monitoring, response definition, first-and second-line therapy, and therapy for special issues. Key questions were selected according the criterion of clinical relevance. Statements were produced using a Delphi process, and two consensus conferences involving a panel of 21 experts appointed by the European LeukemiaNet (ELN) were convened. Patients with polycythemia vera (PV) and essential thrombocythemia (ET) should be defined as high risk if age is greater than 60 years or there is a history of previous thrombosis. Risk stratification in primary myelofibrosis (PMF) should start with the International Prognostic Scoring System (IPSS) for newly diagnosed patients and dynamic IPSS for patients being seen during their disease course, with the addition of cytogenetics evaluation and transfusion status. High-risk patients with PV should be managed with phlebotomy, low-dose aspirin, and cytoreduction, with either hydroxyurea or interferon at any age. High-risk patients with ET should be managed with cytoreduction, using hydroxyurea at any age. Monitoring response in PV and ET should use the ELN clinicohematologic criteria. Corticosteroids, androgens, erythropoiesis-stimulating agents, and immunomodulators are recommended to treat anemia of PMF, whereas hydroxyurea is the first-line treatment of PMF-associated splenomegaly. Indications for splenectomy include symptomatic portal hypertension, drug-refractory painful splenomegaly, and frequent RBC transfusions. The risk of allogeneic stem-cell transplantation-related complications is justified in transplantation-eligible patients whose median survival time is expected to be less than 5 years.
|
|
| 4. |
- Barbuil, Tiziano, et al.
(författare)
-
Philadelphia chromosome-negative classical myeloproliferative neoplasms : revised management recommendations from European LeukemiaNet
- 2018
-
Ingår i: Leukemia. - : Nature Publishing Group. - 0887-6924 .- 1476-5551. ; 32:5, s. 1057-1069
-
Forskningsöversikt (refereegranskat)abstract
- This document updates the recommendations on the management of Philadelphia chromosome-negative myeloproliferative neoplasms (Ph-neg MPNs) published in 2011 by the European LeukemiaNet (ELN) consortium. Recommendations were produced by multiple-step formalized procedures of group discussion. A critical appraisal of evidence by using Grades of Recommendation, Assessment, Development and Evaluation (GRADE) methodology was performed in the areas where at least one randomized clinical trial was published. Seven randomized controlled trials provided the evidence base; earlier phase trials also informed recommendation development. Key differences from the 2011 diagnostic recommendations included: lower threshold values for hemoglobin and hematocrit and bone marrow examination for diagnosis of polycythemia vera (PV), according to the revised WHO criteria; the search for complementary clonal markers, such as ASXL1, EZH2, IDH1/IDH2, and SRSF2 for the diagnosis of myelofibrosis (MF) in patients who test negative for JAK2V617, CALR or MPL driver mutations. Regarding key differences of therapy recommendations, both recombinant interferon alpha and the JAK1/JAK2 inhibitor ruxolitinib are recommended as second-line therapies for PV patients who are intolerant or have inadequate response to hydroxyurea. Ruxolitinib is recommended as first-line approach for MF-associated splenomegaly in patients with intermediate-2 or high-risk disease; in case of intermediate-1 disease, ruxolitinib is recommended in highly symptomatic splenomegaly. Allogeneic stem cell transplantation is recommended for transplant-eligible MF patients with high or intermediate-2 risk score. Allogeneic stem cell transplantation is also recommended for transplant-eligible MF patients with intermediate-1 risk score who present with either refractory, transfusion-dependent anemia, blasts in peripheral blood > 2%, adverse cytogenetics, or high-risk mutations. In these situations, the transplant procedure should be performed in a controlled setting.
|
|
| 5. |
- Bree, N, et al.
(författare)
-
Shape Coexistence in the Neutron-Deficient Even-Even Hg182-188 Isotopes Studied via Coulomb Excitation.
- 2014
-
Ingår i: Physical Review Letters. - 1079-7114. ; 112:16
-
Tidskriftsartikel (refereegranskat)abstract
- Coulomb-excitation experiments to study electromagnetic properties of radioactive even-even Hg isotopes were performed with 2.85 MeV/nucleon mercury beams from REX-ISOLDE. Magnitudes and relative signs of the reduced E2 matrix elements that couple the ground state and low-lying excited states in Hg182-188 were extracted. Information on the deformation of the ground and the first excited 0+ states was deduced using the quadrupole sum rules approach. Results show that the ground state is slightly deformed and of oblate nature, while a larger deformation for the excited 0+ state was noted in Hg182,184. The results are compared to beyond mean field and interacting-boson based models and interpreted within a two-state mixing model. Partial agreement with the model calculations was obtained. The presence of two different structures in the light even-mass mercury isotopes that coexist at low excitation energy is firmly established.
|
|
| 6. |
- Cederkäll, Joakim, et al.
(författare)
-
Sub-Barrier Coulomb Excitation of ^110Sn and Its Implications for the ^100Sn Shell Closure
- 2007
-
Ingår i: Physical Review Letters. - 1079-7114. ; 98:17, s. 172501-
-
Tidskriftsartikel (refereegranskat)abstract
- The first excited 2+ state of the unstable isotope 110Sn has been studied in safe Coulomb excitation at 2.82 MeV/u using the MINIBALL array at the REX-ISOLDE post accelerator at CERN. This is the first measurement of the reduced transition probability of this state using this method for a neutron deficient Sn isotope. The strength of the approach lies in the excellent peak-to-background ratio that is achieved. The extracted reduced transition probability, B(E2:0+-->2+)=0.220±0.022e2b2, strengthens the observation of the evolution of the B(E2) values of neutron deficient Sn isotopes that was observed recently in intermediate-energy Coulomb excitation of 108Sn. It implies that the trend of these reduced transition probabilities in the even-even Sn isotopes is not symmetric with respect to the midshell mass number A=116 as 100Sn is approached.
|
|
| 7. |
- Clément, E, et al.
(författare)
-
Spectroscopic Quadrupole Moments in ^{96,98}Sr: Evidence for Shape Coexistence in Neutron-Rich Strontium Isotopes at N=60.
- 2016
-
Ingår i: Physical Review Letters. - 1079-7114. ; 116:2
-
Tidskriftsartikel (refereegranskat)abstract
- Neutron-rich ^{96,98}Sr isotopes have been investigated by safe Coulomb excitation of radioactive beams at the REX-ISOLDE facility. Reduced transition probabilities and spectroscopic quadrupole moments have been extracted from the differential Coulomb excitation cross sections. These results allow, for the first time, the drawing of definite conclusions about the shape coexistence of highly deformed prolate and spherical configurations. In particular, a very small mixing between the coexisting states is observed, contrary to other mass regions where strong mixing is present. Experimental results have been compared to beyond-mean-field calculations using the Gogny D1S interaction in a five-dimensional collective Hamiltonian formalism, which reproduce the shape change at N=60.
|
|
| 8. |
- DiJulio, Douglas, et al.
(författare)
-
Coulomb excitation of In-107
- 2013
-
Ingår i: Physical Review C (Nuclear Physics). - 0556-2813. ; 87:1
-
Tidskriftsartikel (refereegranskat)abstract
- The radioactive isotope In-107 was studied using sub-barrier Coulomb excitation at the REX-ISOLDE facility at CERN. Two gamma rays were observed during the experiment, corresponding to the low-lying 11/2(+) and 3/2(-)states. The reduced transition probability of the 11/2(+) state was determined with the semiclassical Coulomb excitation code GOSIA2. The result is discussed in comparison to large-scale shell-model calculations, previous unified-model calculations, and earlier Coulomb excitation measurements in the odd-mass In isotopes. DOI: 10.1103/PhysRevC.87.017301
|
|
| 9. |
- DiJulio, Douglas, et al.
(författare)
-
Electromagnetic properties of vibrational bands in Er-170
- 2011
-
Ingår i: European Physical Journal A. - : Springer Science and Business Media LLC. - 1434-6001 .- 1434-601X. ; 47:2, s. 25-
-
Tidskriftsartikel (refereegranskat)abstract
- Excited states of the nucleus Er-170 have been studied by Coulomb excitation using the GASP gamma-ray detector system at the Laboratori Nazionali di Legnaro. The ground-state band along with a low-lying K-pi = 0(+) band and gamma-vibrational band were populated during the experiment. Based on the measured gamma-ray yields, a set of interband and intraband matrix elements has been extracted using the Coulomb excitation code GOSIA. The resulting E2 matrix elements are compared to collective model predictions.
|
|
| 10. |
- DiJulio, Douglas, et al.
(författare)
-
Excitation strengths in Sn-109: Single-neutron and collective excitations near Sn-100
- 2012
-
Ingår i: Physical Review C (Nuclear Physics). - 0556-2813. ; 86:3
-
Tidskriftsartikel (refereegranskat)abstract
- A set of B(E2) values for the low-lying excited states in the radioactive isotope Sn-109 were deduced from a Coulomb excitation experiment. The 2.87-MeV/u radioactive beam was produced at the REX-ISOLDE facility at CERN and was incident on a secondary Ni-58 target. The B(E2) values were determined using the known 2(+) -> 0(+) reduced transition probability in Ni-58 as normalization with the semiclassical Coulomb excitation code GOSIA2. The transition probabilities are compared to shell-model calculations based on a realistic nucleon-nucleon interaction and the predictions of a simple core-excitation model. This measurement represents the first determination of multiple B(E2) values in a light Sn nucleus using the Coulomb excitation technique with low-energy radioactive beams. The results provide constraints for the single-neutron states relative to Sn-100 and also indicate the importance of both single-neutron and collective excitations in the light Sn isotopes.
|
|
| 11. |
- Diriken, J., et al.
(författare)
-
Coulomb excitation of Ga-73
- 2010
-
Ingår i: Physical Review C (Nuclear Physics). - 0556-2813. ; 82:6
-
Tidskriftsartikel (refereegranskat)abstract
- The B(E2; I-i -> I-f) values for transitions in Ga-71(31)40 and Ga-73(31)42 were deduced from a Coulomb excitation experiment at the safe energy of 2.95 MeV/nucleon using post-accelerated beams of Ga-71,Ga-73 at the REX-ISOLDE on-line isotope mass separator facility. The emitted gamma rays were detected by the MINIBALL gamma-detector array, and B(E2; I-i -> I-f) values were obtained from the yields normalized to the known strength of the 2(+) -> 0(+) transition in the Sn-120 target. The comparison of these new results with the data of less neutron-rich gallium isotopes shows a shift of the E2 collectivity toward lower excitation energy when adding neutrons beyond N = 40. This supports conclusions from previous studies of the gallium isotopes, which indicated a structural change in this isotopic chain between N = 40 and 42. Combined with recent measurements from collinear laser spectroscopy showing a 1/2(-) spin and parity for the ground state, the extracted results revealed evidence for a 1/2(-), 3/2(-) doublet near the ground state in Ga-73(31)42 differing by at most 0.8 keV in energy.
|
|
| 12. |
- Doornenbal, P., et al.
(författare)
-
Enhanced strength of the 2(1)(+) -> 0(g.s.)(+) transition in Sn-114 studied via Coulomb excitation in inverse kinematics
- 2008
-
Ingår i: Physical Review C (Nuclear Physics). - 0556-2813. ; 78:3
-
Tidskriftsartikel (refereegranskat)abstract
- The 2(1)(+) states of Sn-114,Sn-116 were excited in two consecutive experiments by means of Coulomb excitation in inverse kinematics on a Ni-58 target. A precise determination of the reduced transition probability B(E2; 0(g.s.)(+) -> 2(1)(+)) of Sn-114 relative to the well-known 2(1)(+) excitation strength in Sn-116 was achieved by comparing the relative projectile to target 2(1)(+) -> 0(g.s.)(+) decay intensities. The obtained B(E2 up arrow) value of 0.232(8)e(2)b(2) for Sn-114 confirms the tendency of large B(E2 up arrow) values for the light tin isotopes below the midshell Sn-116 that has been observed recently in various radioactive ion beam experiments. The result establishes most clearly the discrepancy between the current B(E2 up arrow) value predictions from large-scale shell-model calculations and the experimental deviation, which commences already for the stable Sn-114 isotope.
|
|
| 13. |
- Ekström, Andreas, et al.
(författare)
-
0(gs)+ -->2(1)+ transition strengths in 106Sn and 108Sn.
- 2008
-
Ingår i: Physical Review Letters. - 1079-7114. ; 101:1
-
Tidskriftsartikel (refereegranskat)abstract
- The reduced transition probabilities, B(E2; 0(gs)+ -->2(1)+), have been measured in the radioactive isotopes (108,106)Sn using subbarrier Coulomb excitation at the REX-ISOLDE facility at CERN. Deexcitation gamma rays were detected by the highly segmented MINIBALL Ge-detector array. The results, B(E2;0(gs)+ -->2(1)+)=0.222(19)e2b2 for 108Sn and B(E2; 0(gs)+-->2(1)+)=0.195(39)e2b2 for 106Sn were determined relative to a stable 58Ni target. The resulting B(E2) values are approximately 30% larger than shell-model predictions and deviate from the generalized seniority model. This experimental result may point towards a weakening of the N=Z=50 shell closure.
|
|
| 14. |
- Ekström, Andreas, et al.
(författare)
-
Coulomb excitation of the odd-odd isotopes In-106,In-108
- 2010
-
Ingår i: European Physical Journal A. Hadrons and Nuclei. - : Springer Science and Business Media LLC. - 1434-6001. ; 44:3, s. 355-361
-
Tidskriftsartikel (refereegranskat)abstract
- The low-lying states in the odd-odd and unstable isotopes In-106,In-108 have been Coulomb excited from the ground state and the first excited isomeric state at the REX-ISOLDE facility at CERN. With the additional data provided here the pi g(9/2)(-1) circle times nu d(5/2) and pi g(9/2)(-1) circle times nu g7/2 multiplets have been re-analyzed and are modified compared to previous results. The observed gamma-ray de-excitation patterns were interpreted within a shell model calculation based on a realistic effective interaction. The agreement between theory and experiment is satisfactory and the calculations reproduce the observed differences in the excitation pattern of the two isotopes. The calculations exclude a 6(+) ground state in In-106. This is in agreement with the conclusions drawn using other techniques. Furthermore, based on the experimental results, it is also concluded that the ordering of the isomeric and ground state in In-108 is inverted compared to the shell model prediction. Limits on B(E2) values have been extracted where possible. A previously unknown low-lying state at 367 keV in In-106 is also reported.
|
|
| 15. |
- Ekström, Andreas, et al.
(författare)
-
Electric quadrupole moments of the 2(1)(+) states in Cd-100,Cd-102,Cd-104
- 2009
-
Ingår i: Physical Review C (Nuclear Physics). - 0556-2813. ; 80:5
-
Tidskriftsartikel (refereegranskat)abstract
- Using the REX-ISOLDE facility at CERN the Coulomb excitation cross sections for the 0(gs)(+)-> 2(1)(+) transition in the beta-unstable isotopes Cd-100,Cd-102,Cd-104 have been measured for the first time. Two different targets were used, which allows for the first extraction of the static electric quadrupole moments Q(2(1)(+)) in Cd-102,Cd-104. In addition to the B(E2) values in Cd-102,Cd-104, a first experimental limit for the B(E2) value in Cd-100 is presented. The data was analyzed using the maximum likelihood method. The provided probability distributions impose a test for theoretical predictions of the static and dynamic moments. The data are interpreted within the shell-model using realistic matrix elements obtained from a G-matrix renormalized CD-Bonn interaction. In view of recent results for the light Sn isotopes the data are discussed in the context of a renormalization of the neutron effective charge. This study is the first to use the reorientation effect for post-accelerated short-lived radioactive isotopes to simultaneously determine the B(E2) and the Q(2(1)(+)) values.
|
|
| 16. |
- Ekström, Andreas, et al.
(författare)
-
Sub-barrier Coulomb excitation of Sn-106,Sn-108,Sn-110
- 2008
-
Ingår i: Frontiers in Nuclear structure, Astrophysics and Reactions - Finustar 2, AIP Conference Proceedings. - : AIP. - 0094-243X .- 1551-7616. ; 1012, s. 296-299
-
Konferensbidrag (refereegranskat)abstract
- The reduced transition probabilities between the first excited 2(+) state and the 0(+) ground state, B(E2; 0(+) -> 2(+)), have been measured in Sn-106,Sn-108,Sn-110 using sub-barrier Coulomb excitation in inverse kinematics at REX-ISOLDE. The results are, B(E2;0(+) -> 2(+)) = 0.220(22),0.226(17), and 0.228(32) e(2)b(2), for Sn-110, Sn-108, and Sn-106, respectively. The results for Sn-106,Sn-108 are preliminary. De-excitation gamma-rays were detected by the MINIBALL Ge-array. The B(E2) reveals detailed information about the nuclear wave function. A shell model prediction based on an effective CD-Bonn interaction in the nu(0g(7/2),2s, 1d, 0h(11/2)) model space using e(eff)(nu) =1.0 e follows the experimental values for the neutron rich Sn isotopes, but fails to reproduce the results presented here.
|
|
| 17. |
|
|
| 18. |
- Emanuel, Robyn M, et al.
(författare)
-
Myeloproliferative Neoplasm (MPN) Symptom Assessment Form Total Symptom Score : Prospective International Assessment of an Abbreviated Symptom Burden Scoring System Among Patients With MPNs
- 2012
-
Ingår i: Journal of Clinical Oncology. - 0732-183X .- 1527-7755. ; 30:33, s. 4098-4103
-
Tidskriftsartikel (refereegranskat)abstract
- PURPOSE Myeloproliferative neoplasm (MPN) symptoms are troublesome to patients, and alleviation of this burden represents a paramount treatment objective in the development of MPN-directed therapies. We aimed to assess the utility of an abbreviated symptom score for the most pertinent and representative MPN symptoms for subsequent serial use in assessing response to therapy.PATIENTS AND METHODSThe Myeloproliferative Neoplasm Symptom Assessment Form total symptom score (MPN-SAF TSS) was calculated as the mean score for 10 items from two previously validated scoring systems. Questions focus on fatigue, concentration, early satiety, inactivity, night sweats, itching, bone pain, abdominal discomfort, weight loss, and fevers.RESULTS MPN-SAF TSS was calculable for 1,408 of 1,433 patients with MPNs who had a mean score of 21.2 (standard deviation [SD], 16.3). MPN-SAF TSS results significantly differed among MPN disease subtypes (P < .001), with a mean of 18.7 (SD, 15.3), 21.8 (SD, 16.3), and 25.3 (SD, 17.2) for patients with essential thrombocythemia, polycythemia vera, and myelofibrosis, respectively. The MPN-SAF TSS strongly correlated with overall quality of life (QOL; r = 0.59; P < .001) and European Organisation for Research and Treatment of Cancer Quality of Life Questionnaire C30 (EORTC QLQ-C30) functional scales (all P < .001 and absolute r ≥ 0.50 except social functioning r = 0.48). No significant trends were present when comparing therapy subgroups. The MPN-SAF TSS had excellent internal consistency (Cronbach's α = .83). Factor analysis identified a single underlying construct, indicating that the MPN-SAF TSS is an appropriate, unified scoring method.CONCLUSIONThe MPN-SAF TSS is a concise, valid, and accurate assessment of MPN symptom burden with demonstrated clinical utility in the largest prospective MPN symptom study to date. This new prospective scoring method may be used to assess MPN symptom burden in both clinical practice and trial settings.
|
|
| 19. |
- Fuchs, David, et al.
(författare)
-
Scoring the Risk of Having Systemic Mastocytosis in Adult Patients with Mastocytosis in the Skin
- 2021
-
Ingår i: Journal of Allergy and Clinical Immunology. - : Elsevier. - 2213-2198 .- 2213-2201. ; 9:4, s. 1705-1712.e4
-
Tidskriftsartikel (refereegranskat)abstract
- BACKGROUND: Mastocytosis in adults often presents with skin lesions. A bone marrow biopsy is necessary to confirm or exclude the presence of systemic mastocytosis (SM) in these cases. When a bone marrow biopsy is not performed, the provisional diagnosis is mastocytosis in the skin (MIS). No generally accepted scoring system has been established to estimate the risk of SM in these patients. OBJECTIVE: To develop a risk score to predict SM in adults with MIS. METHODS: We examined 1145 patients with MIS from the European Competence Network on Mastocytosis Registry who underwent a bone marrow biopsy. A total of 944 patients had SM and 201 patients had cutaneous mastocytosis; 63.7% were female, and 36.3% were male. Median age was 44 +/- 13.3 years. The median serum tryptase level amounted to 29.3 +/- 81.9 ng/mL. We established a multivariate regression model using the whole population of patients as a training and validation set (bootstrapping). A risk score was developed and validated with receiver-operating curves. RESULTS: In the multivariate model, the tryptase level (P < .001), constitutional/cardiovascular symptoms (P = .014), and bone symptoms/osteoporosis (P < .001) were independent predictors of SM (P < .001; sensitivity, 90.7%; specificity, 69.1%). A 6-point risk score was established (risk, 10.7%-98.0%) and validated. CONCLUSIONS: Using a large data set of the European Competence Network on Mastocytosis Registry, we created a risk score to predict the presence of SM in patients with MIS. Although the score will need further validation in independent cohorts, our score seems to discriminate safely between patients with SM and with pure cutaneous mastocytosis. (C) 2020 American Academy of Allergy, Asthma & Immunology
|
|
| 20. |
- Gaffney, L. P., et al.
(författare)
-
Collectivity in the light radon nuclei measured directly via Coulomb excitation
- 2015
-
Ingår i: Physical Review C (Nuclear Physics). - 0556-2813. ; 91:6
-
Tidskriftsartikel (refereegranskat)abstract
- Background: Shape coexistence in heavy nuclei poses a strong challenge to state-of-the-art nuclear models, where several competing shape minima are found close to the ground state. A classic region for investigating this phenomenon is in the region around Z = 82 and the neutron midshell at N = 104. Purpose: Evidence for shape coexistence has been inferred from a-decay measurements, laser spectroscopy, and in-beam measurements. While the latter allow the pattern of excited states and rotational band structures to be mapped out, a detailed understanding of shape coexistence can only come from measurements of electromagnetic matrix elements. Method: Secondary, radioactive ion beams of Rn-202 and Rn-204 were studied by means of low-energy Coulomb excitation at the REX-ISOLDE in CERN. Results: The electric-quadrupole (E2) matrix element connecting the ground state and first excited 2(1)(+) state was extracted for both Rn-202 and Rn-204, corresponding to B(E2; 2(1)(+) -> 0(1)(+)) = 29(-8)(+8) and 43(-12)(+17) W.u., respectively. Additionally, E2 matrix elements connecting the 2(1)(+) state with the 4(1)(+) and 2(2)(+) states were determined in Rn-202. No excited 0(+) states were observed in the current data set, possibly owing to a limited population of second-order processes at the currently available beam energies. Conclusions: The results are discussed in terms of collectivity and the deformation of both nuclei studied is deduced to be weak, as expected from the low-lying level-energy schemes. Comparisons are also made to state-of-the-art beyond-mean-field model calculations and the magnitude of the transitional quadrupole moments are well reproduced.
|
|
| 21. |
- Geyer, Holly L., et al.
(författare)
-
Associations between gender, disease features and symptom burden in patients with myeloproliferative neoplasms : an analysis by the MPN QOL International Working Group
- 2017
-
Ingår i: Haematologica. - : Ferrata Storti Foundation (Haematologica). - 0390-6078 .- 1592-8721. ; 102:1, s. 85-93
-
Tidskriftsartikel (refereegranskat)abstract
- The myeloproliferative neoplasms, including polycythemia vera, essential thrombocythemia and myelofibrosis, are distinguished by their debilitating symptom profiles, life-threatening complications and profound impact on quality of life. The role gender plays in the symptomatology of myeloproliferative neoplasms remains under-investigated. In this study we evaluated how gender relates to patients' characteristics, disease complications and overall symptom expression. A total of 2,006 patients (polycythemia vera=711, essential thrombocythemia=830, myelofibrosis=460, unknown=5) were prospectively evaluated, with patients completing the Myeloproliferative Neoplasm-Symptom Assessment Form and Brief Fatigue Inventory Patient Reported Outcome tools. Information on the individual patients' characteristics, disease complications and laboratory data was collected. Consistent with known literature, most female patients were more likely to have essential thrombocythemia (48.6% versus 33.0%; P<0.001) and most male patients were more likely to have polycythemia vera (41.8% versus 30.3%; P<0.001). The rate of thrombocytopenia was higher among males than females (13.9% versus 8.2%; P<0.001) and males also had greater red-blood cell transfusion requirements (7.3% versus 4.9%; P=0.02) with shorter mean disease duration (6.4 versus 7.2 years, P=0.03). Despite there being no statistical differences in risk scores, receipt of most therapies or prior complications (hemorrhage, thrombosis), females had more severe and more frequent symptoms for most individual symptoms, along with overall total symptom score (22.8 versus 20.3; P<0.001). Females had particularly high scores for abdominal-related symptoms (abdominal pain/discomfort) and microvascular symptoms (headache, fatigue, insomnia, concentration difficulties, dizziness; all P<0.01). Despite complaining of more severe symptom burden, females had similar quality of life scores to those of males. The results of this study suggest that gender contributes to the heterogeneity of myeloproliferative neoplasms by influencing phenotypic profiles and symptom expression.
|
|
| 22. |
|
|
| 23. |
|
|
| 24. |
- Geyer, Holly L., et al.
(författare)
-
The role of sexuality symptoms in myeloproliferative neoplasm symptom burden and quality of life : An analysis by the MPN QOL International Study Group
- 2016
-
Ingår i: Cancer. - : Wiley. - 0008-543X .- 1097-0142. ; 122:12, s. 1888-1896
-
Tidskriftsartikel (refereegranskat)abstract
- BACKGROUNDPatients with myeloproliferative neoplasms (MPNs) including polycythemia vera, essential thrombocythemia, and myelofibrosis, are faced with oppressive symptom profiles that compromise daily functioning and quality of life. Among these symptoms, sexuality-related symptoms have emerged as particularly prominent and largely unaddressed. In the current study, the authors evaluated how sexuality symptoms from MPN relate to other patient characteristics, disease features, treatments, and symptoms. METHODSA total of 1971 patients with MPN (827 with essential thrombocythemia, 682 with polycythemia vera, 456 with myelofibrosis, and 6 classified as other) were prospectively evaluated and patient responses to the Myeloproliferative Neoplasm Symptom Assessment Form (MPN-SAF) and the European Organization for Research and Treatment of Cancer Quality of Life Questionnaire Core 30 (EORTC-QLQ C30) were collected, along with information regarding individual disease characteristics and laboratory data. Sexuality scores were compared with an age-matched, healthy control population. RESULTSOverall, patients with MPN were found to have greater sexual dysfunction compared with the healthy population (MPN-SAF score of 3.6 vs 2.0; P<.001), with 64% of patients with MPN describing some degree of sexual dysfunction and 43% experiencing severe symptoms. The presence of sexual symptoms correlated closely with all domains of patient functionality (physical, social, cognitive, emotional, and role functioning) and were associated with a reduced quality of life. Sexual problems also were found to be associated with other MPN symptoms, particularly depression and nocturnal and microvascular-related symptoms. Sexual dysfunction was more severe in patients aged >65 years and in those with cytopenias and transfusion requirements, and those receiving certain therapies such as immunomodulators or steroids. ConclusionsThe results of the current study identify the topic of sexuality as a prominent issue for the MPN population, and this area would appear to benefit from additional investigation and management. Cancer 2016;122:1888-96. (c) 2016 American Cancer Society. Sexuality problems impact all domains of functionality, depression, microvascular symptoms, and overall quality of life among patients with myeloproliferative neoplasms. These problems correlate with patient age, the presence of cytopenias, transfusion requirements, and common therapies for myeloproliferative neoplasms. See also pages 1804-6.
|
|
| 25. |
- Geyer, Holly, et al.
(författare)
-
Symptomatic Profiles of Patients With Polycythemia Vera : Implications of Inadequately Controlled Disease
- 2016
-
Ingår i: Journal of Clinical Oncology. - 0732-183X .- 1527-7755. ; 34:2, s. 151-
-
Tidskriftsartikel (refereegranskat)abstract
- PURPOSE: Polycythemia vera (PV) is a myeloproliferative neoplasm (MPN) associated with disabling symptoms and a heightened risk of life-threatening complications. Recent studies have demonstrated the effectiveness of JAK inhibitor therapy in patients with PV patients who have a history of prior hydroxyurea (HU) use (including resistance or intolerance), phlebotomy requirements, and palpable splenomegaly. We aimed to determine how these features contribute alone and in aggregate to the PV symptom burden.PATIENTS AND METHODS: Through prospective evaluation of 1,334 patients with PV who had characterized symptom burden, we assessed patient demographics, laboratory data, and the presence of splenomegaly by disease feature (ie, known HU use, known phlebotomy requirements, splenomegaly).RESULTS: The presence of each feature in itself is associated with a moderately high symptom burden (MPN symptom assessment form [SAF] total symptom score [TSS] range, 27.7 to 29.2) that persists independent of PV risk category. In addition, symptoms incrementally increase in severity with the addition of other features. Patients with PV who had all three features (PV-HUPS) faced the highest total score (MPN-SAF TSS, 32.5) but had similar individual symptom scores to patients with known HU use (PV-HU), known phlebotomy (PV-P), and splenomegaly (PV-S).CONCLUSION: The results of this study suggest that patients with PV who have any one of the features in question (known HU use, known phlebotomy, or splenomegaly) have significant PV-associated symptoms. Furthermore, it demonstrates that many PV symptoms remain severe independent of the number of features present.
|
|
