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- Gennery, A. R., et al.
(författare)
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Treatment of CD40 ligand deficiency by hematopoietic stem cell transplantation: a survey of the European experience, 1993-2002
- 2004
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Ingår i: Blood. - : American Society of Hematology. - 0006-4971 .- 1528-0020. ; 103:3, s. 1152-7
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Tidskriftsartikel (refereegranskat)abstract
- CD40 ligand (CD40L) deficiency causes recurrent sinopulmonary infection, Pneumocystis carinii pneumonia, and Cryptosporidium parvum infection. Approximately 40% to 50% of patients survive to the third decade: long-term survival is unclear. Hematopoietic stem cell transplantation (HSCT) is curative. We present a retrospective analysis of 38 European patients undergoing HSCT for CD40L deficiency in 8 European countries between 1993 and 2002. Donor stem cell source included 14 HLA-identical siblings, 22 unrelated donors, and 2 phenotypically matched parental stem cells (12 T-cell depleted). Of the patients, 34 engrafted and 26 (68%) survived; 3 had autologous reconstitution, 22 (58%) were cured, and 1 engrafted but has poor T-cell immune reconstitution. There were 18 evaluated patients who responded to vaccination. Of the patients, 12 (32%) died from infection-related complications, with severe cryptosporidiosis in 6. Grades 2 to 4 graft-versus-host disease (GvHD) associated with infection occurred in 6 of 12 fatal cases. HSCT cured 58% of patients, 72% of those without hepatic disease. Early T-cell function following whole marrow HSCT may limit cryptosporidial disease, but survival was similar after T-cell-depleted HSCT. Preexisting lung damage was the most important adverse risk factor. Further studies will determine optimal timing and type of HSCT.
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- Kelsall, N. S., et al.
(författare)
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Testing mean-field models near the N=Z line : gamma-ray spectroscopy of the T-z=1/2 nucleus Kr-73
- 2002
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Ingår i: Physical Review C. Nuclear Physics. - 0556-2813 .- 1089-490X. ; 65:4
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Tidskriftsartikel (refereegranskat)abstract
- Excited states in the N=Z+1 nucleus Kr-73 have been investigated using the Ca-40(Ar-36,2pn) and Ca-40(Ca-40,alpha2pn) reactions at 145 and 160 MeV, respectively. gamma rays were detected using the Gammasphere array and events were recorded in coincidence with charged-particle and neutron detectors. The three previously observed bands were extended to high spin, and a new unfavored positive-parity band has been observed. The alignment characteristics and decay properties of the bands are all consistent with large-deformation prolate rotation, with no clear evidence for oblate bands or shape coexistence. This is quite different from neighboring Kr-72,Kr-74, indicating a strong shape-stabilizing role for the valence neutron. The experimental results are compared to extended total Routhian surface, cranked Nilsson Strutinsky, and cranked relativistic mean-field calculations. The results suggest that the paired calculations lack some important physics. Neutron-proton correlations may be the missing ingredient. There is also evidence for an unusual band crossing in the negative-parity bands, which may indicate the presence of T=0 pairing correlations. At high spin all the models can reproduce the experimental data.
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- Lyubimtsev, A. L., et al.
(författare)
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The structure and bonding of Ni3Sn
- 2002
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Ingår i: Journal of Alloys and Compounds. - 0925-8388 .- 1873-4669. ; 340:02-jan, s. 167-172
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Tidskriftsartikel (refereegranskat)abstract
- The electronic structure of Ni3Sn was calculated at ab initio levels for the crystal structure of the low-temperature modification of Ni3Sn refined upon data of single-crystal X-ray diffractometry (P6(3)/mmc, a=5.2950(7), c=4.247(1) Angstrom R=0.0288). The calculations were made with the use of fixed Gaussian (CRYSTAL98 software) and energy-dependent (Stuttgart TB-LMTO-ASA software) basis sets. Difference electron charge density maps were analysed and compared with that of a hypothetical hcp Ni in order to understand bonding in Ni3Sn. It was found that bonding in Ni3Sn has multicentre character with Ni-Sn interaction stronger than Ni-Ni one.
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- Prinzleve, M., et al.
(författare)
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Cocaine use in Europe - a multi-centre study : Patterns of use in different groups
- 2004
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Ingår i: European Addiction Research. - 1022-6877 .- 1421-9891. ; 10:4, s. 147-155
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Tidskriftsartikel (refereegranskat)abstract
- AIM: The study investigates patterns of cocaine powder and crack cocaine use of different groups in nine European cities. DESIGN, SETTING, PARTICIPANTS: Multi-centre cross-sectional study conducted in Barcelona, Budapest, Dublin, Hamburg, London, Paris, Rome, Vienna, and Zurich. Data were collected by structured face-to-face interviews. The sample comprises 1,855 cocaine users out of three subgroups: 632 cocaine users in addiction treatment, mainly maintenance treatment; 615 socially marginalized cocaine users not in treatment, and 608 socially integrated cocaine users not in treatment. MEASUREMENTS: Use of cocaine powder, crack cocaine and other substances in the last 30 days, routes of administration, and lifetime use of cocaine powder and crack cocaine. Findings: The marginalized group showed the highest intensity of cocaine use, the highest intensity of heroin use and of multiple substance use. 95% of the integrated group snorted cocaine powder, while in the two other groups, injecting was quite prevalent, but with huge differences between the cities. 96% of all participants had used at least one other substance in addition to cocaine in the last 30 days. CONCLUSIONS: The use of cocaine powder and crack cocaine varies widely between different groups and between cities. Nonetheless, multiple substance use is the predominating pattern of cocaine use, and the different routes of administration have to be taken into account.
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- Steward, C. G., et al.
(författare)
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Severe pulmonary hypertension: a frequent complication of stem cell transplantation for malignant infantile osteopetrosis
- 2004
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Ingår i: Br J Haematol. - 0007-1048. ; 124:1, s. 63-71
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Tidskriftsartikel (refereegranskat)abstract
- This report describes eight infants who developed acute severe pulmonary arterial hypertension (PAH) at days -2 to +89 after allogeneic stem cell transplantation (SCT) for malignant infantile osteopetrosis (MIOP). They were taken from a total of 28 children (frequency 29%) transplanted for this disease at three institutions between 1996 and 2002. Typical presentations were acute dyspnoea, hypoxia and brady/tachycardia usually in the absence of fever, crepitations or other evidence of infection. Six patients (75%) required assisted ventilation and five (62%) died. There was clinical or pathological evidence of veno-occlusive disease (VOD) in three children, but absence of VOD in the remaining five suggests that a separate disease process may be responsible for the PAH. Responses to nitric oxide (NO), defibrotide (DF), nicardipine and steroids in varying combinations were disappointing. Three children showed sustained improvement after administration of epoprostenol (EP, prostacyclin) in conjunction with NO and/or DF and remain well and free of PAH 25, 31 and 32 months post-transplant. PAH must therefore be excluded in any child who becomes acutely breathless after SCT for osteopetrosis.
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- Fischer, S. M., et al.
(författare)
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Alignment delays in the N = Z nuclei Kr-72, Sr-76, and Zr-80
- 2001
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Ingår i: Physical Review Letters. - 0031-9007 .- 1079-7114. ; 8713:13
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Tidskriftsartikel (refereegranskat)abstract
- The ground state rotational bands of the N = Z nuclei Kr-72, Sr-76, and Zr-80 have been extended into the angular momentum region where rotation alignment of particles is normally expected. By measuring the moments of inertia of these bands we have observed a consistent increase in the rotational frequency required to start pair breaking, when compared to neighboring nuclei. Kr-72 shows the most marked effect. It has been widely suggested that these delayed alignments arise from np-pairing correlations. However, alignment frequencies are very sensitive to shape degrees of freedom and normal pairing, so the new experimental observations are still open to interpretation.
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