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- Mazodier, P., et al.
(författare)
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Systemic necrotizing vasculitides in severe alpha1-antitrypsin deficiency
- 1996
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Ingår i: QJM - Monthly Journal of the Association of Physicians. - : Oxford University Press (OUP). - 0033-5622. ; 89:8, s. 599-611
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Tidskriftsartikel (refereegranskat)abstract
- We describe the clinical presentation and outcome in a series of eight patients with systemic necrotizing vasculitis and severe alpha1-antitrypsin (AAT) deficiency followed up at three Swedish hospitals during 1968-92. We also review six other cases reported in the literature during the same period. Diagnosis of severe AAT deficiency was based on the presence of the PiZZ phenotype, or low plasma total trypsin inhibitory capacity, or a low plasma AAT concentration (10-40% of the normal mean value) and presence of the PiSZ- or PiFZ phenotype. The diagnosis of systemic vasculitis was biopsy-verified in all eight patients. Pretreatment laboratory findings, treatment protocol, and outcome were reviewed in each of the 14 patients. Of the eight patients in the Swedish series, six had systemic vasculitis of the microscopic polyangiitis form, one had Wegener's granulomatosis, and another had Henoch-Schonlein purpura. In the series as a whole (n = 14), median age at diagnosis was 48 years (range 44-84), the median number of affected organs was eight, and all 14 patients had skin involvement, and either renal or joint involvement (in most cases both); 71% (10/14) had emphysema; 57% (8/14) had hepatic abnormalities (two having cirrhosis, two fibrosis, and one multiple aneurysms in hepatic arteries); one patient who presented with acute ulcerative colitis developed manifest vasculitic syndrome three years later; and 64% (9/14) died, the major cause of death being renal failure. This syndrome, characterized by multiple organ involvement and fatal outcome, has been underdiagnosed. Physicians should be alert to the presence of the PiZ AAT deficiency gene in patients with systemic vasculitis, especially when the course is progressive or when the patient also has emphysema or cirrhosis. Awareness of those features may aid prompt recognition and enable early treatment.
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- Park, Seoyeon, et al.
(författare)
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The global burden of sudden infant death syndrome from 1990 to 2019: a systematic analysis from the Global Burden of Disease study 2019
- 2022
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Ingår i: QJM Monthly Journal of the Association of Physicians. - : OXFORD UNIV PRESS. - 1460-2725 .- 1460-2393. ; 115:11, s. 735-744
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Tidskriftsartikel (refereegranskat)abstract
- Background Sudden infant death syndrome (SIDS) still remains one of the leading causes of infant death worldwide, especially in high-income countries. To date, however, there is no detailed information on the global health burden of SIDS. Aims To characterize the global disease burden of SIDS and its trends from 1990 to 2019 and to compare the burden of SIDS according to the socio-demographic index (SDI). Design Systematic analysis based on the Global Burden of Disease (GBD) 2019 data. Methods Epidemiological data of 204 countries from 1990 to 2019 were collected via various methods including civil registration and vital statistics in the original GBD study. Estimates for mortality and disease burden of SIDS were modeled. Crude mortality and mortality rates per 100 000 population were analyzed. Disability-adjusted life years (DALYs) and DALY rates were also assessed. Results In 2019, mortality rate of SIDS accounted for 20.98 [95% Uncertainty Interval, 9.15-46.16] globally, which was a 51% decrease from 1990. SIDS was most prevalent in Western sub-Saharan Africa, High-income North America and Oceania in 2019. The burden of SIDS was higher in males than females consistently from 1990 to 2019. Higher SDI and income level was associated with lower burden of SIDS; furthermore, countries with higher SDI and income had greater decreases in SIDS burden from 1990 to 2019. Conclusions The burden of SIDS has decreased drastically from 1990 to 2019. However, the improvements have occurred disproportionately between regions and SDI levels. Focused preventive efforts in under-resourced populations are needed.
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- Ray, J. G., et al.
(författare)
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Venous thromboembolism in association with features of the metabolic syndrome
- 2007
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Ingår i: QJM Monthly Journal of the Association of Physicians. - : Oxford University Press (OUP). - 1460-2725 .- 1460-2393. ; 100:11, s. 679-684
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Tidskriftsartikel (refereegranskat)abstract
- Background: Central obesity, diabetes mellitus, dyslipidaemia and chronic hypertension-features of the metabolic syndrome-have been individually associated with venous thromboembolism (VTE). However, whether each of these factors additively increases the risk of VTE is uncertain. Aim: To determine whether features of the metabolic syndrome independently increase the risk of VTE. Design: Prospective cohort study derived from the Heart Outcomes Prevention Evaluation 2 (HOPE-2) randomized clinical trial. Setting: One hundred and forty-five clinical centres in 13 countries. Methods: We studied 5522 adults aged >55 years with cardiovascular disease or diabetes mellitus. At enrolment, 35% had 0-1 features of the metabolic syndrome, 30% had two, 24% had three and 11% had four. We defined symptomatic VTE as an objectively confirmed new episode of deep-vein thrombosis or pulmonary embolism. Results: VTE occurred in 88 individuals during a median 5.0 years of follow-up. The incidence rate of VTE (per 100 person-years) was 0.30 with 0-1 features, 0.36 with two features, 0.38 with three features and 0.40 with four features of the metabolic syndrome (trend p=0.43). Relative to the presence of 0-1 features of the metabolic syndrome, the adjusted hazard ratio (95%Cl) for VTE was 1.22 (0.71-2.08) with two features, 1.25 (0.70-2.24) with three features, and 1.26 (0.59-2.69) with four features. Discussion: The number of features of the metabolic syndrome present was not a clinically important risk factor for VTE in older adults with vascular arterial disease.
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- Sendi, P, et al.
(författare)
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Spinal epidural abscess in clinical practice
- 2008
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Ingår i: QJM : monthly journal of the Association of Physicians. - : Oxford University Press (OUP). - 1460-2725. ; 101:1, s. 1-12
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Tidskriftsartikel (refereegranskat)
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- Suazo, Matías, et al.
(författare)
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Project Hephaistos – II. Dyson sphere candidates from Gaia DR3, 2MASS, and WISE
- 2024
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Ingår i: QJM Monthly Journal of the Association of Physicians. - 1460-2725 .- 1460-2393. ; 531:1
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Tidskriftsartikel (refereegranskat)abstract
- The search for extraterrestrial intelligence is currently being pursued using multiple techniques and in different wavelength bands. Dyson spheres, megastructures that could be constructed by advanced civilizations to harness the radiation energy of their host stars, represent a potential technosignature, that in principle may be hiding in public data already collected as part of large astronomical surveys. In this study, we present a comprehensive search for partial Dyson spheres by analysing optical and infrared observations from Gaia, 2MASS, and WISE. We develop a pipeline that employs multiple filters to identify potential candidates and reject interlopers in a sample of five million objects, which incorporates a convolutional neural network to help identify confusion in WISE data. Finally, the pipeline identifies seven candidates deserving of further analysis. All of these objects are M-dwarfs, for which astrophysical phenomena cannot easily account for the observed infrared excess emission.
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