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Raoul Wallenberg, the courageous Swedish financier and trade executive turned diplomat who saved thousands of Jews in Hungary in 1944, was arrested in January 1945 by the Soviet military counterintelligence, incarcerated in the Lubianka prison in Moscow, and later executed for reasons that remain obscure to this day. Drawing on recently declassified Soviet encrypted cables and a wide array of Sovi
Brottmekaniska processer ar något som intresserat människor i alla tider. Ibland har det varit för att vinna en fördel t ex vid tillverking av flintverktyg och malmbrytning, men oftast for att förhindra mekanisk kollaps. I det senare fallet kan oförsiktighet resultera i spruckna tallrikar eller trasiga fönster, men ocksa i allvarligare saker som brott i flygplansvingar eller fartygsskrov som i värBrottmekaniska processer ar något som intresserat människor i alla tider. Ibland har det varit för att vinna en fördel t ex vid tillverking av flintverktyg och malmbrytning, men oftast for att förhindra mekanisk kollaps. I det senare fallet kan oförsiktighet resultera i spruckna tallrikar eller trasiga fönster, men ocksa i allvarligare saker som brott i flygplansvingar eller fartygsskrov som i vär
För att en utveckling ska anses hållbar måste mål ur såväl sociala, ekonomiska som miljömässiga aspekter vara uppfyllda. Det svenska miljömålssystemet, baserat på Agenda 2030, innehåller 16 miljökvalitetsmål och ett antal etappmål. För genomförande och uppföljning ansvarar åtta myndigheter, av vilka Boverket har ansvar för målet ”En god bebyggd miljö”. Faktorer som har valts att studera är inomhusFor a development to be considered sustainable, social, economic and environmental requirements must be met. The Swedish sustainable development goals, based on Agenda 2030, contain 16 national environmental quality goals. The National Board of Housing, Building and Planning is responsible for achieving the goal "A good built environment". Essential factors chosen for studies are: indoor environme
Background Phenylketonuria (PKU) causes irreversible central nervous system damage unless a phenylalanine (PHE) restricted diet with amino acid supplementation is maintained. To prevent growth retardation, a protein/amino acid intake beyond the recommended dietary protein allowance is mandatory. However, data regarding disease and/or diet related changes in body composition are inconclusive and re
Two young girls without a notable medical history except for asthma presented with an acute toxic encephalopathy with very low serine concentrations both in plasma and cerebrospinal fluid (CSF) comparable to patients with 3-phosphoglycerate dehydrogenase (3-PGDH) deficiency. Clinical symptoms and enzyme measurement (in one patient) excluded 3-PGDH deficiency. Deficiencies in other serine biosynthe
OBJECTIVES:: Vitamin K deficiency (VKD) may cause life-threatening haemorrhages, especially in breast-fed infants with unrecognised cholestasis. Interestingly, hypoallergenic formulas appear overrepresented in reported cases of VKD bleeding (VKDB) in formula-fed infants. We therefore assessed whether the risk of VKD in formula-fed infants with cholestasis is associated with hypoallergenic formulas
Hypomyelination is observed in the context of a growing number of genetic disorders that share clinical characteristics. The aim of this study was to determine the possible role of magnetic resonance imaging pattern recognition in distinguishing different hypomyelinating disorders, which would facilitate the diagnostic process. Only patients with hypomyelination of known cause were included in thi
The disorders of serine biosynthesis are a group of inborn errors of metabolism characterised by congenital microcephaly, seizures and severe psychomotor retardation. Although these disorders are rare the prompt recognition of serine deficiency is important as these disorders are treatable. The diagnosis is based on decreased concentrations of serine in cerebrospinal fluid (CSF). It has previously
In this clinical case, the importance of early detection of Hurler syndrome for outcomes of stem cell transplantation in this patient population is illustrated. Nowadays, children with Hurler syndrome can be treated with stem cell transplantation, but the results largely depend on the moment of treatment. If the procedure is initiated in an early stage of disease, better results can be obtained. T
Deficiency of subunit 6 of the conserved oligomeric Golgi (COG6) complex causes a new combined N-and O-glycosylation deficiency of the congenital disorders of glycosylation, designated as CDG-IIL (COG6-CDG). The index patient presented with a severe neurologic disease characterized by vitamin K deficiency, vomiting, intractable focal seizures, intracranial bleedings and fatal outcome in early infa
Hurler syndrome (HS), the most severe phenotype in the spectrum of mucopolysaccharidosis type I, is caused by a deficiency of the lysosomal enzyme alpha-L-iduronidase (IDUA). At present, hematopoietic stem cell transplantation (HSCT) is the only treatment able to prevent disease progression in the central nervous system, and therefore considered the treatment of choice in HS patients. Because IDUA
Background: Analysis of sialic acid (SA) metabolites in cerebrospinal fluid (CSF) is important for clinical diagnosis. In the present study, a high-performance liquid chromatography-tandem mass spectrometry (HPLC/MS/MS) method for free sialic acid (FSA) and total sialic acid (TSA) in human CSF was validated. Methods: The method utilized a simple sample-preparation procedure of protein precipitatio
As continuous access to energy is necessary for survival, the level of energy stores has a strong effect on fitness and survival. Behaviors by which energy stores are acquired and built up are known as foraging, and this is a very common topic in animal behavior studies. It is especially important for flying organisms to finely tune the level of energy reserves they carry. While most animals will
Many animals store food outside of their own bodies for later consumption, not only birds and mammals, but also invertebrates such as honeybees. I refer to such food storage as hoarding, to distinguish it from internal storage such as in fat deposits. There are two distinct food-hoarding strategies: larder hoarding (all food is stored in one or a few central larders) and scatter hoarding (storage
