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- Krag Jakobsen, Ane, et al.
(författare)
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Surgical interventions for nephrolithiasis in ankylosing spondylitis and the general population
- 2015
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Ingår i: Scandinavian Journal of Urology. - : Medical Journals Sweden AB. - 2168-1805 .- 2168-1813. ; 49:6, s. 486-491
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Tidskriftsartikel (refereegranskat)abstract
- Objective. The aim of this study was to estimate rates and type of definitive surgical interventions for nephrolithiasis in Swedish patients with ankylosing spondylitis (AS) compared to the general population. Materials and methods. This national prospective cohort study linked data from Swedish population and healthcare registries. Incidence rates and interventions for nephrolithiasis during follow-up in patients with AS were compared to general population comparator (GPC) subjects. Results. In total, 8572 AS patients were followed for 49,959 person-years and 39,639 matched GPCs were followed for 225,221 person-years. Mean age at study entry was 46 years [interquartile range (IQR) 36-56 years] and 65% were male. In AS patients with a diagnosis of nephrolithiasis during the study period, 29% (72/250) underwent similar intervention for nephrolithiasis compared to 24% (114/466) GPCs (p = 0.21). The incidence rate ratio (RR) in overall AS patients was 2.9 [95% confidence interval (CI) 2.1-3.8] during a median follow-up of 6.2 years (IQR 3.2-8.6 years). With prior diagnosis of nephrolithiasis, the RR for AS patients compared to GPCs was 3.7 (95% CI 1.8-7.7); without prior nephrolithiasis the RR was 2.1 (95% CI 1.5-3.0). Increasing age [odds ratio (OR) 1.02, 95% CI 1.01-1.03], prior nephrolithiasis diagnosis (OR 3.3, 95% CI 1.97-5.62) and atherosclerotic cardiac disease (OR 2.0, 95% CI 1.03-3.91) were identified as predictors of intervention for nephrolithiasis. Conclusions. Patients with AS have an almost three-fold increased risk of surgical intervention for kidney stones, with similar management, compared to the general population.
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- Krag Jakobsen, Ane, et al.
(författare)
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Is Nephrolithiasis an Unrecognized Extra-Articular Manifestation in Ankylosing Spondylitis? A Prospective Population-Based Swedish National Cohort Study with Matched General Population Comparator Subjects
- 2014
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Ingår i: Plos One. - : Public Library of Science (PLoS). - 1932-6203. ; 9:11
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Tidskriftsartikel (refereegranskat)abstract
- Background: Ankylosing spondylitis (AS) is associated with several extra-articular manifestations. Nephrolithiasis (NL) has not been recognized as one of those, however, several factors known to increase the risk of NL are at play in AS patients. The objective was to estimate rates and predictors of NL in Swedish patients with AS compared to the general population. Methods and Findings: We performed a prospective population-based nationwide cohort study based on linkage of data from Swedish registries. 8,572 AS patients were followed for 49,258 person-years (py) and 39,639 matched general population comparators were followed for 223,985 py. Patients were followed prospectively together with comparator subjects from January 2001 through December 2009. The first occurrence of NL during follow-up was the primary outcome. Hazard Ratios (HR) were used to compare these rates adjusting for comorbidities and treatment, and to assess predictors for NL. Mean age at study entry was 46 years (inter quartile range 36-56 years), 65% were males. Based on 250 vs. 466 NL events, the adjusted HR of NL in AS patients was 2.1 (95% CI 1.8 to 2.4). Predictors of NL within the AS group included prior diagnosis of inflammatory bowel disease (IBD) (HR 2.3; 95% CI 1.7 to 3.3), prior diagnosis of NL (HR 16.4; 95% CI 11.5 to 23.4), and patients receiving anti-TNF treatment (HR 1.6; 95% CI 1.2 to 2.1). Male sex was a risk factor for NL both in AS patients and in the general population. Limitations: The risk for residual confounding and inability to study the chemical nature of NL were considered the main limitations of the study. Conclusions: Patients with AS are at increased risk of NL, which may be considered a novel extra-articular manifestation. Previous history of NL, IBD, AS disease severity and male sex were identified as predictors of NL in AS.
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- Laforet, P., et al.
(författare)
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Deep morphological analysis of muscle biopsies from type III glycogenesis (GSDIII), debranching enzyme deficiency, revealed stereotyped vacuolar myopathy and autophagy impairment
- 2019
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Ingår i: Acta Neuropathologica Communications. - : Springer Science and Business Media LLC. - 2051-5960. ; 7:1
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Tidskriftsartikel (refereegranskat)abstract
- Glycogen storage disorder type III (GSDIII), or debranching enzyme (GDE) deficiency, is a rare metabolic disorder characterized by variable liver, cardiac, and skeletal muscle involvement. GSDIII manifests with liver symptoms in infancy and muscle involvement during early adulthood. Muscle biopsy is mainly performed in patients diagnosed in adulthood, as routine diagnosis relies on blood or liver GDE analysis, followed by AGL gene sequencing. The GSDIII mouse model recapitulate the clinical phenotype in humans, and a nearly full rescue of muscle function was observed in mice treated with the dual AAV vector expressing the GDE transgene. In order to characterize GSDIII muscle morphological spectrum and identify novel disease markers and pathways, we performed a large international multicentric morphological study on 30 muscle biopsies from GSDIII patients. Autophagy flux studies were performed in human muscle biopsies and muscles from GSDIII mice. The human muscle biopsies revealed a typical and constant vacuolar myopathy, characterized by multiple and variably sized vacuoles filled with PAS-positive material. Using electron microscopy, we confirmed the presence of large nonmembrane bound sarcoplasmic deposits of normally structured glycogen as well as smaller rounded sac structures lined by a continuous double membrane containing only glycogen, corresponding to autophagosomes. A consistent SQSTM1/p62 decrease and beclin-1 increase in human muscle biopsies suggested an enhanced autophagy. Consistent with this, an increase in the lipidated form of LC3, LC3II was found in patients compared to controls. A decrease in SQSTM1/p62 was also found in the GSDIII mouse model. In conclusion, we characterized the morphological phenotype in GSDIII muscle and demonstrated dysfunctional autophagy in GSDIII human samples. These findings suggest that autophagic modulation combined with gene therapy might be considered as a novel treatment for GSDIII.
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- Vierinen, J., et al.
(författare)
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Space debris observation potential with EISCAT 3D
- 2018
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Ingår i: 2ND URSI ATLANTIC RADIO SCIENCE MEETING (AT-RASC). - : IEEE. - 9789082598735
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Konferensbidrag (refereegranskat)abstract
- We investigate the capabilities of the next generation ionospheric research radar EISCAT 3D (E3D) for space debris observations. We have used the current projected design of E3D as basis of this study. To model the performance of E3D for space debris observations, we have included basic radar equation based error analysis for range and range-rate observations. Because the radar will be multi-static, it is also capable of observing instantaneous three-dimensional vector velocities and positions by observing round-trip range and range-rate between the transmitter and three receiver sites. We have included error estimates for both of the the three-dimensional position and three-dimensional vector velocity observations. To estimate the fraction of total debris that can be observed with E3D, we have used the MASTER model. We have also investigated effects of radio wave propagation. E3D uses a relatively low VHF frequency (233 MHz), which experiences more radio wave propagation effects than more conventional higher frequency space surveillance radars. Our modeling shows that ionospheric ray-bending and group delay are severe enough that these effects need to be modeled in order to determine accurate orbital elements. As EISCAT 3D is an ionospheric research radar, there will be high quality ionospheric electron density measurements that can be utilized for radio propagation modeling.
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