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- Hankeova, S., et al.
(författare)
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Sex differences and risk factors for bleeding in Alagille syndrome
- 2022
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Ingår i: Embo Molecular Medicine. - : EMBO. - 1757-4676 .- 1757-4684. ; 14:12
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Tidskriftsartikel (refereegranskat)abstract
- Spontaneous bleeds are a leading cause of death in the pediatric JAG1-related liver disease Alagille syndrome (ALGS). We asked whether there are sex differences in bleeding events in patients, whether Jag1(Ndr/Ndr) mice display bleeds or vascular defects, and whether discovered vascular pathology can be confirmed in patients non-invasively. We performed a systematic review of patients with ALGS and vascular events following PRISMA guidelines, in the context of patient sex, and found significantly more girls than boys reported with spontaneous intracranial hemorrhage. We investigated vascular development, homeostasis, and bleeding in Jag1(Ndr/Ndr) mice, using retina as a model. Jag1(Ndr/Ndr) mice displayed sporadic brain bleeds, a thin skull, tortuous blood vessels, sparse arterial smooth muscle cell coverage in multiple organs, which could be aggravated by hypertension, and sex-specific venous defects. Importantly, we demonstrated that retinographs from patients display similar characteristics with significantly increased vascular tortuosity. In conclusion, there are clinically important sex differences in vascular disease in ALGS, and retinography allows non-invasive vascular analysis in patients. Finally, Jag1(Ndr/Ndr) mice represent a new model for vascular compromise in ALGS.
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- Juliusson, Gunnar, et al.
(författare)
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Attitude towards remission induction for elderly patients with acute myeloid leukemia influences survival.
- 2006
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Ingår i: Leukemia. - : Springer Science and Business Media LLC. - 1476-5551 .- 0887-6924. ; 20:1, s. 42-47
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Tidskriftsartikel (refereegranskat)abstract
- Combination chemotherapy may induce remission from acute myeloid leukemia (AML), but validated criteria for treatment of elderly are lacking. The remission intention ( RI) rate for elderly patients, as reported to the Swedish Leukemia Registry, was known to be different when comparing the six health care regions, but the consequences of different management are unknown. The Leukemia Registry, containing 1672 AML patients diagnosed between 1997 and 2001, with 98% coverage and a median follow-up of 4 years, was completed with data from the compulsory cancer and population registries. Among 506 treated and untreated patients aged 70-79 years with AML (non-APL), there was a direct correlation between the RI rate in each health region ( range 36-76%) and the two-year overall survival, with no censored observations (6-21%) ( v 2 for trend = 11.3, P < 0.001; r(2) = 0.86, P < 0.02, nonparametric). A 1-month landmark analysis showed significantly better survival in regions with higher RI rates ( P = 0.003). Differences could not be explained by demographics, and was found in both de novo and secondary leukemias. The 5-year survival of the overall population aged 70-79 years was similar between the regions. Survival of 70-79-year-old AML patients is better in regions where more elderly patients are judged eligible for remission induction.
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- Fredriksson, Hasse, et al.
(författare)
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A model of liquid metals and its relation to the solidification process
- 2005
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Ingår i: Materials Science & Engineering. - : Elsevier BV. - 0921-5093 .- 1873-4936. ; 413, s. 455-459
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Tidskriftsartikel (refereegranskat)abstract
- X-ray analysis shows that a liquid is build up of clusters of atoms with a certain number of nearest neighbours. The X-ray analysis shows that 8-11 nearest neighbours surround each atom. Each cluster has a crystal-like structure. Between the clusters there are some free atoms and free electrons. The enthalpy of fusion is according to Richard's rule around the gas constant times the temperature of melting and the heat capacity in the liquid state is normally constant and for some metals lower than that in the solid state. For metals with low melting points it will decrease further with increasing temperature. This behaviour of the metals can be explained by the use of statistical mechanics and by assuming that the clusters, observed by X-ray analysis are rotating around a centre of its mass. The cluster model is applied to explain the diffusion rate in liquid metals. The effect of the experimental set upon measurements of diffusion constants is discussed as well as its effect on crystal growth.
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