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- Varsani, H, et al.
(författare)
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Validation of a score tool for measurement of histological severity in juvenile dermatomyositis and association with clinical severity of disease
- 2015
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Ingår i: Annals of the rheumatic diseases. - : BMJ. - 1468-2060 .- 0003-4967. ; 74:1, s. 204-210
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Tidskriftsartikel (refereegranskat)abstract
- To study muscle biopsy tissue from patients with juvenile dermatomyositis (JDM) in order to test the reliability of a score tool designed to quantify the severity of histological abnormalities when applied to biceps humeri in addition to quadriceps femoris. Additionally, to evaluate whether elements of the tool correlate with clinical measures of disease severity.Methods55 patients with JDM with muscle biopsy tissue and clinical data available were included. Biopsy samples (33 quadriceps, 22 biceps) were prepared and stained using standardised protocols. A Latin square design was used by the International Juvenile Dermatomyositis Biopsy Consensus Group to score cases using our previously published score tool. Reliability was assessed by intraclass correlation coefficient (ICC) and scorer agreement (α) by assessing variation in scorers’ ratings. Scores from the most reliable tool items correlated with clinical measures of disease activity at the time of biopsy.ResultsInter- and intraobserver agreement was good or high for many tool items, including overall assessment of severity using a Visual Analogue Scale. The tool functioned equally well on biceps and quadriceps samples. A modified tool using the most reliable score items showed good correlation with measures of disease activity.ConclusionsThe JDM biopsy score tool has high inter- and intraobserver agreement and can be used on both biceps and quadriceps muscle tissue. Importantly, the modified tool correlates well with clinical measures of disease activity. We propose that standardised assessment of muscle biopsy tissue should be considered in diagnostic investigation and clinical trials in JDM.
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| 6. |
- Keller, Annika, et al.
(författare)
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Mutations in the gene encoding PDGF-B cause brain calcifications in humans and mice
- 2013
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Ingår i: Nature Genetics. - : Springer Science and Business Media LLC. - 1061-4036 .- 1546-1718. ; 45:9, s. 1077-
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Tidskriftsartikel (refereegranskat)abstract
- Calcifications in the basal ganglia are a common incidental finding and are sometimes inherited as an autosomal dominant trait ( idiopathic basal ganglia calcification (IBGC)). Recently, mutations in the PDGFRB gene coding for the platelet-derived growth factor receptor beta (PDGF-R beta) were linked to IBGC. Here we identify six families of different ancestry with nonsense and missense mutations in the gene encoding PDGF-B, the main ligand for PDGF-R beta. We also show that mice carrying hypomorphic Pdgfb alleles develop brain calcifications that show age-related expansion. The occurrence of these calcium depositions depends on the loss of endothelial PDGF-B and correlates with the degree of pericyte and blood-brain barrier deficiency. Thus, our data present a clear link between Pdgfb mutations and brain calcifications in mice, as well as between PDGFB mutations and IBGC in humans.
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| 7. |
- Tesson, Sylvie V M, et al.
(författare)
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Integrating microorganism and macroorganism dispersal: modes, techniques and challenges with particular focus on co-dispersal
- 2015
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Ingår i: Ecoscience. - : Informa UK Limited. - 1195-6860. ; 22:2-4, s. 109-124
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Tidskriftsartikel (refereegranskat)abstract
- Whatever their size and the ecosystem they live in, all organisms may disperse at some stage of their life cycle. Dispersal dynamics are to a varying extent dependent on organismal size, life history, ecological niche, survival capacities and phylogeny. Moves towards a synthesis in dispersal ecology have focused primarily on vertebrates and higher plants, yet recent studies suggest that the dispersal of microorganisms and macroorganisms has much more in common than previously assumed. The dispersal of one organism enables co-dispersal for many others, smaller in size. There is an increasing need for a more integrated approach to study dispersal within the context of organismal interactions and their environments. Such an approach is facilitated by recent developments of powerful indirect techniques that enable tracking of microorganisms and macroorganisms over multiple spatial and temporal scales. Likewise, dispersal modelling and theoretical models of the consequences of dispersal can inspire empirical studies across the entire size spectrum. Simultaneously studying the relationships between dispersal of microorganisms and macroorganisms, and accounting for dispersal through time and space, will allow us to better understand the functioning and dynamics of communities and ecosystems, and to make better predictions of future dispersal patterns, changes in biodiversity and connectivity.
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