Background: Anxiety-provoking healthcare procedures require specific child-friendly approaches. Magnetic resonance imaging (MRI) can cause anxiety for children and general anesthesia (GA) is often used. We developed and tested a multi-faceted child-friendly concept, Children Centered Care, for MRI of children without GA.
View Article and Find Full Text PDFPrimary hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome caused by inborn errors of cytotoxicity. Patients with biallelic PRF1 null mutations (encoding perforin) usually develop excessive immune cell activation, hypercytokinemia, and life-threatening immunopathology in the first 6 months of life, often without an apparent infectious trigger. In contrast, perforin-deficient (PKO) mice only develop HLH after systemic infection with lymphocytic choriomeningitis virus (LCMV).
View Article and Find Full Text PDFObjective: Brain arteriovenous malformations (AVMs) in patients with hereditary hemorrhagic telangiectasia (HHT) present different characteristics from sporadic AVMs, and they have lower initial bleeding rates. Conservative management is usually preferred for the treatment of these lesions. In this case study, we present the largest series of HHT patients treated with stereotactic radiosurgery to date.
View Article and Find Full Text PDFAllergic disorders are caused by a combination of hereditary and environmental factors. The hygiene hypothesis postulates that early-life microbial exposures impede the development of subsequent allergic disease. Recently developed "wildling" mice are genetically identical to standard laboratory specific pathogen-free (SPF) mice but are housed under seminatural conditions and have rich microbial exposures from birth.
View Article and Find Full Text PDFViruses can utilize host splicing machinery to enable the expression of multiple genes from a limited-sized genome. Orthobornaviruses use alternative splicing to regulate the expression level of viral proteins and achieve efficient viral replication in the nucleus. Although more than 20 orthobornaviruses have been identified belonging to eight different viral species, virus-specific splicing has not been demonstrated.
View Article and Find Full Text PDFCytomegaloviruses (CMVs) have co-evolved with their mammalian hosts for millions of years, leading to remarkable host specificity and high infection prevalence. Macrophages, which already populate barrier tissues in the embryo, are the predominant immune cells at potential CMV entry sites. Here we show that, upon CMV infection, macrophages undergo a morphological, immunophenotypic, and metabolic transformation process with features of stemness, altered migration, enhanced invasiveness, and provision of the cell cycle machinery for viral proliferation.
View Article and Find Full Text PDFUnicellular organisms that live in marine environments must cope with considerable fluctuations in the availability of inorganic phosphate (P). Here, we investigated the extracellular P concentration-dependent expression, as well as the intracellular or extracellular localization, of phosphatases and phosphate transporters of the diatom . We identified P-regulated plasma membrane-localized, ER-localized, and secreted phosphatases, in addition to plasma membrane-localized, vacuolar membrane-localized, and plastid-surrounding membrane-localized phosphate transporters that were also regulated in a P concentration-dependent manner.
View Article and Find Full Text PDFPathogenic long QT mutations often comprise high phenotypic variability and particularly variants in ANK2 (long QT syndrome 4) frequently lack QT prolongation. We sought to elucidate the genetic and functional background underlying the clinical diversity in a 3-generation family with different cardiac arrhythmias. Next-generation sequencing-based screening of patients with QT prolongation identified the index patient of the family carrying an ANK2-E1813K variant and a previously uncharacterized KCNH2-H562R mutation in a double heterozygous conformation.
View Article and Find Full Text PDFPurpose: To evaluate the need for general anesthesia (GA) in MRI in children aged 4-6 years, using a multi-faceted concept, Children Centered Care (CCC), compared to a standard setup.
Materials And Methods: In this prospective study of 81 children, we developed and tested a multi-faceted concept, CCC, for MRI in children aged 4-6 without GA, comparing it to a standard setup. The CCC included: 1) an interactive app, 2) a trained pediatric team, 3) a children's lounge with a toy-scanner, and 4) a child-friendly multimedia environment in the MRI room.
Object: Navigated transcranial magnetic stimulation (nTMS) combined with diffusion tensor imaging (DTI) is used preoperatively in patients with eloquent-located brain lesions and allows analyzing non-invasively the spatial relationship between the tumor and functional areas (e.g. the motor cortex and the corticospinal tract [CST]).
View Article and Find Full Text PDFAvian bornaviruses are causative agents of proventricular dilatation disease (PDD), a chronic neurologic and often fatal disorder of psittacines including endangered species. To date no causative therapy or immunoprophylaxis is available. Our previous work has shown that viral vector vaccines can delay the course of homologous bornavirus challenge infections but failed to protect against PDD when persistent infection was not prevented.
View Article and Find Full Text PDFAvian bornaviruses are causative agents of proventricular dilatation disease (PDD), an often fatal disease of parrots and related species (order Psittaciformes) which is widely distributed in captive psittacine populations and may affect endangered species. Here, we established a vaccination strategy employing two different well described viral vectors, namely recombinant Newcastle disease virus (NDV) and modified vaccinia virus Ankara (MVA) that were engineered to express the phosphoprotein and nucleoprotein genes of two avian bornaviruses, parrot bornavirus 4 (PaBV-4) and canary bornavirus 2 (CnBV-2). When combined in a heterologous prime/boost vaccination regime, NDV and MVA vaccine viruses established self-limiting infections and induced a bornavirus-specific humoral immune response in cockatiels (Nymphicus hollandicus) and common canaries (Serinus canaria forma domestica).
View Article and Find Full Text PDFObjective: Open conversion after endovascular aortic aneurysm repair (EVAR-c) is performed nonelectively in up to 60% of cases. EVAR-c has been reported to have significantly greater risk of postoperative morbidity and mortality than primary aortic repair, but few data exist on outcomes for symptomatic or ruptured presentations. This study determined outcomes and identified predictors of postoperative major adverse cardiac events (MACEs) and mortality for patients undergoing nonelective EVAR-c compared with nonelective primary aortic repair (PAR) in the Vascular Quality Initiative (VQI).
View Article and Find Full Text PDFBackground: The innovative pneumatic tube system (iPTS) transports one sample at a time without the use of cartridges and allows rapid sending of samples directly into the bulk loader of a laboratory automation system (LAS). We investigated effects of the iPTS on samples and turn-around time (TAT).
Methods: During transport, a mini data logger recorded the accelerations in three dimensions and reported them in arbitrary area under the curve (AUC) units.
We report a case of eosinophilic cystitis in a 7-year-old boy with a history of atopic symptoms, with focus on the radiological findings. He presented with hematuria and dysuria and ultrasonography (US) showed irregular bladder wall thickening resembling a bladder mass. CT urography did not characterize the lesion any further and showed no local or distant spread.
View Article and Find Full Text PDFAcute generalized exanthematous pustulosis (AGEP) is a rare cutaneous eruption that often is a reaction to medications, most commonly antibiotics. Clinically, AGEP closely mimics pustular psoriasis and also is similar to subcorneal pustular dermatosis and IgA pemphigus. For clinicians, it is important to differentiate AGEP from pustular psoriasis.
View Article and Find Full Text PDFBackground: The use of online surveys for data collection has increased exponentially, yet it is often unclear whether interview-based cognitive assessments (such as face-to-face or telephonic word recall tasks) can be adapted for use in application-based research settings.
Objective: The objective of the current study was to compare and characterize the results of online word recall tasks to those of the Health and Retirement Study (HRS) and determine the feasibility and reliability of incorporating word recall tasks into application-based cognitive assessments.
Methods: The results of the online immediate and delayed word recall assessment, included within the Women's Health and Valuation (WHV) study, were compared to the results of the immediate and delayed recall tasks of Waves 5-11 (2000-2012) of the HRS.
Objective: To estimate the effect of sequence on response precision and response behavior in health valuation studies.
Methods: Time trade-off (TTO) and paired comparison responses from six health valuation studies-four US, one Spanish, and one Dutch-were examined (22,225 respondents) to test whether task sequence influences response precision (e.g.
Snake bites are rare events in Germany and are not life-threatening with usually only mild clinical symptoms. The most widespread venomous snake is the common European adder (Vipera berus). Here we present the case of a 53-year-old woman who was bitten by a common adder.
View Article and Find Full Text PDFThe current study examined independent and interactive effects between Apolipoprotein E (APOE) genotype and two types of cognitively-stimulating lifestyle activities (CSLA)-integrated information processing (CSLA-II) and novel information processing (CSLA-NI)-on concurrent and longitudinal changes in cognition. Three-wave data across 6 years of follow-up from the Victoria Longitudinal Study (n=278; ages 55-94) and linear mixed model analyses were used to characterize the effects of APOE genotype and participation in CSLA-II and CSLA-NI in four cognitive domains. Significant CSLA effects on cognition were observed.
View Article and Find Full Text PDFRIG-I-like receptors (RLRs: RIG-I, MDA5 and LGP2) play a major role in the innate immune response against viral infections and detect patterns on viral RNA molecules that are typically absent from host RNA. Upon RNA binding, RLRs trigger a complex downstream signaling cascade resulting in the expression of type I interferons and proinflammatory cytokines. In the past decade extensive efforts were made to elucidate the nature of putative RLR ligands.
View Article and Find Full Text PDFObjective: The optimal role for bare metal stents (BMS) or stent grafts (SG) in femoropopliteal occlusive disease (FPOD) is as of yet undefined. Understanding the clinical consequences of failure can help guide initial treatment decisions. The goal of this study was to define the nature, frequency, and risk factors for adverse clinical events related to BMS and SG failure in FPOD.
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