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Influence of potassium chloride on PAH concentration during soot formation studied using laser-induced fluorescence

Previous studies have shown that addition of potassium chloride (KCl) reduces soot concentration and primary particle size. To improve the understanding of this phenomenon, this work focuses on investigating the influence of KCl addition on formation of polycyclic aromatic hydrocarbons (PAHs), assumed to be precursors of soot. The method used is laser-induced fluorescence (LIF), for which differen

Healthcare professionals’ experiences during the initial stage of the COVID-19 pandemic in the intensive care unit : A qualitative study

Background: The COVID-19 pandemic called for rapidly considerable changes in the healthcare system. Healthcare professionals from different departments within the hospital settings were enrolled in the emergency preparedness. This study, therefore, aimed to explore the healthcare professionals’ experiences attending the ICU-preparedness and caring for patients with COVID-19 during the initial stag

A highly invasive malaria parasite has expanded its range to non-migratory birds in North America

Parasite range expansions are a direct consequence of globalization and are an increasing threat to biodiversity. Here, we report a recent range expansion of the SGS1 strain of a highly invasive parasite, Plasmodium relictum, to two non-migratory passerines in North America. Plasmodium relictum is considered one of the world's most invasive parasites and causes the disease avian malaria: this is t

Early life factors in relation to albuminuria and estimated glomerular filtration rate based on cystatin C and creatinine in adults from a Swedish population-based cohort study

BackgroundEarly life factors influence the number of nephrons a person starts life with and a consequence of that is believed to be premature kidney ageing. Thus, we aimed to identify early life factors associated with cystatin C and creatinine-based estimated glomerular filtration (eGFR) rate equations and urine -albumin-to-creatinine ratio after a follow-up of 46–67 years.MethodsThe study includ

Neurometabolic disorders are treatable causes of dystonia

A broad range of rare inherited metabolic disorders can present with dystonia. For clinicians, it is important to recognize dystonic features, but it can be complicated by the mixed and complex clinical picture seen in many neurometabolic patients. Careful phenotyping is the first step towards the diagnosis of the underlying condition and subsequent targeted treatment, further supported by imaging

Myoclonus in childhood-onset neurogenetic disorders : The importance of early identification and treatment

Background In clinical practice, myoclonus in childhood-onset neurogenetic disorders frequently remains unrecognized, because it is often overshadowed by other neurological features. Since treatment can lead to significant functional improvement, accurate phenotyping is essential. To demonstrate the importance of early identification and treatment, we report on four patients with various childhood

Proteomic Discovery and Development of a Multiplexed Targeted MRM-LC-MS/MS Assay for Urine Biomarkers of Extracellular Matrix Disruption in Mucopolysaccharidoses I, II, and VI

The mucopolysaccharidoses (MPS) are lysosomal storage disorders that result from defects in the catabolism of glycosaminoglycans. Impaired muscle, bone, and connective tissue are typical clinical features of MPS due to disruption of the extracellular matrix. Markers of MPS disease pathology are needed to determine disease severity and monitor effects of existing and emerging new treatments on dise

Long-term outcome of Hurler syndrome patients after hematopoietic cell transplantation : An international multicenter study

Mucopolysaccharidosis type I-Hurler syndrome (MPS-IH) is a lysosomal storage disease characterized by multisystem morbidity and death in early childhood. Although hematopoietic cell transplantation (HCT) has been performed in these patients for more than 30 years, large studies on the long-term outcome of patients with MPS-IH after HCT are lacking. The goal of this international study was to ident

Quantification of vitamin B6 vitamers in human cerebrospinal fluid by ultra performance liquid chromatography-tandem mass spectrometry

Since vitamin B6 is essential for normal functioning of the central nervous system, there is growing need for sensitive analysis of B6 vitamers in cerebrospinal fluid (CSF). This manuscript describes the development and validation of a rapid, sensitive and accurate method for quantification of the vitamin B6 vitamers pyridoxal (PL), pyridoxamine (PM), pyridoxine (PN), pyridoxic acid (PA), pyridoxa

A sensitive and simple ultra-high-performance-liquid chromatography-tandem mass spectrometry based method for the quantification of d-amino acids in body fluids

d-Amino acids are increasingly being recognized as important signaling molecules in mammals, including humans. d-Serine and d-aspartate are believed to act as signaling molecules in the central nervous system. Interestingly, several other d-amino acids also occur in human plasma, but very little is currently known regarding their function and origin. Abnormal levels of d-amino acids have been impl

Dried Blood Spot Analysis : An Easy and Reliable Tool to Monitor the Biochemical Effect of Hematopoietic Stem Cell Transplantation in Hurler Syndrome Patients

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

Birth outcomes between 22 and 26 weeks' gestation in national population-based cohorts from Sweden, England and France

Aim: We investigated the timing of survival differences and effects on morbidity for foetuses alive at maternal admission to hospital delivered at 22 to 26 weeks’ gestational age (GA). Methods: Data from the EXPRESS (Sweden, 2004–07), EPICure-2 (England, 2006) and EPIPAGE-2 (France, 2011) cohorts were harmonised. Survival, stratified by GA, was analysed to 112 days using Kaplan-Meier analyses and

Begonia dicressine (Begoniaceae) : A new record for India

Begonia, belonging to the family Begoniaceae, is one of the largest Angiosperm genera with a pan-tropical distribution. Northeastern India shows a high degree of endemism with several endemic species described from the region. Begonia dicressine, so far known from Myanmar, has been recorded for the first time in India from Namdapha National Park. Hence, the present record makes the westernmost dis

Controlling Photoluminescence and Photocatalysis Activities in Lead-Free Cs2PtxSn1−xCl6 Perovskites via Ion Substitution

Lead-free halide perovskites have triggered interest in the field of optoelectronics and photocatalysis because of their low toxicity, and tunable optical and charge-carrier properties. From an application point of view, it is desirable to develop stable multifunctional lead-free halide perovskites. We have developed a series of Cs2PtxSn1−xCl6 perovskites (0≤x≤1) with high stability, which show sw

Automated EW corrections with isolated photons : t t¯ γ, t t¯ γγ and tγj as case studies

In this work we compute for the first time the so-called Complete-NLO predictions for top-quark pair hadroproduction in association with at least one isolated photon (tt¯ γ). We also compute NLO QCD+EW predictions for the similar case with at least two isolated photons (tt¯ γγ) and for single-top hadroproduction in association with at least one isolated photon. In addition, we complement our resul

Anaphora (e62)

Anaphora is a study of 56 classes of vocal multiphonics that explore voiced & voiced; voiced & unvoiced; unvoiced & unvoiced, and; three or more sources. Anaphora was written for the singer Rebekka Uhlig.

Necrotizing enterocolitis and respiratory distress syndrome as first clinical presentation of mitochondrial trifunctional protein deficiency

Background: Newborn screening (NBS) for long-chain 3-hydroxy acyl-CoA dehydrogenase (LCHAD) deficiency does not discriminate between isolated LCHAD deficiency, isolated long-chain keto acyl-CoA (LCKAT) deficiency and general mitochondrial trifunctional protein (MTP) deficiency. Therefore, screening for LCHAD deficiency inevitably comprises screening for MTP deficiency, which is much less amenable

D-amino acid aberrations in cerebrospinal fluid and plasma of smokers

The glutamatergic neurotransmission system and the N-methyl-D-aspartate receptor (NMDAR) have been implicated in smoking and alcohol consumption behavior. Preclinical studies have demonstrated that nicotine and ethanol influence NMDAR functionality, which may have a role in tendencies to consume these substances. Nonetheless, little is known about concentrations of NMDAR coagonists in the cerebros

Neurodegeneration with brain iron accumulation on MRI : An adult case of α-mannosidosis

Case: A 34-year-old woman was referred to our hospital with progressive movement disorders and neurodegeneration with brain iron accumulation and enlargement of the frontal diploe on the MRI. Metabolic testing revealed that she had α-mannosidosis (AMD), a lysosomal storage disorder. Background: AMD is a rare genetic disorder that causes α-mannosidase deficiency resulting in lysosomal accumulation

Vitamin B6 Vitamer concentrations in cerebrospinal fluid differ between preterm and termnewborn infants

BACKGROUND AND OBJECTIVE: Vitamin B6 plays a pivotal role in brain development and functioning. Differences in vitamin B6 homeostasis between preterm and term newborn infants have been reported. The authors sought to investigate whether B6 vitamers in cerebrospinal fluid (CSF) of preterm and term newborn infants are different. METHODS: B6 vitamer concentrations were determined in 69 CSF samples of