Microglial cell activation and senescence are characteristic of the pathology FXTAS.

Background: Fragile X-associated tremor/ataxia syndrome (FXTAS) is a late-onset neurodegenerative disorder associated with premutation alleles of the FMR1 gene. Expansions of more than 200 CGG repeats give rise to fragile X syndrome, the most common inherited form of cognitive impairment. Fragile X-associated tremor/ataxia syndrome is characterized by cerebellar tremor and ataxia, and the presence of ubiquitin-positive inclusions in neurons and astrocytes.

The Frequency and Reason For Amputations in Electrically Burned Pediatric Patients.

Electrical injuries induce substantial morbidity and mortality. Amputations are often necessary to enable survival and based on tissue nonviability, development of life-threatening infection, or expected nonfunctional outcome. They analyzed occurrence and type of amputations in their institution for electrical and nonelectrical pediatric burns and the number of reconstructive operations.

Obesity alters the in vivo mechanical response and biochemical properties of cartilage as measured by MRI.

Background: Obesity is a primary risk factor for the development of knee osteoarthritis (OA). However, there remains a lack of in vivo data on the influence of obesity on knee cartilage mechanics and composition. The purpose of this study was to determine the relationship between obesity and tibiofemoral cartilage properties.

Relationship between lean body mass and isokinetic peak torque of knee extensors and flexors in severely burned children.

Objective: Severe burns cause hypermetabolic responses and prolonged hospitalization, resulting in loss of body mass and muscle strength. This study aimed to determine whether long-term gains in lean body mass (LBM) after structured exercise programs are functionally meaningful and related to greater muscle strength in severely burned children.

Study Design: LBM and muscle strength were measured at discharge and at 6, 12, 24, and 36 months after burn in 349 children.

Re-evaluation of websites from burn centers in Austria, Germany and Switzerland.

Introduction: Websites serve as information and communication platforms; hence, they are important tools for the self-promotion of hospitals. In 2010, Selig et al. evaluated the online presence of burn centers in Germany, Austria, and Switzerland based on 37 quality criteria.

Presence of Middle Cerebellar Peduncle Sign in Premutation Carriers Without Tremor and Ataxia.

Here we report five cases of male premutation carriers who present without clinical symptoms of the fragile X-associated tremor/ataxia syndrome (FXTAS), but who on MRI demonstrate white matter hyperintensities in the middle cerebellar peduncles (MCP sign) and other brain regions, a rare finding. MCP sign is the major radiological feature of FXTAS; it is therefore remarkable to identify five cases in which this MRI finding is present in the absence of tremor and ataxia, the major clinical features of FXTAS. Subjects underwent a detailed neurological evaluation, neuropsychological testing, molecular testing, and MRI evaluation utilizing T2 imaging described here.

Correlation between invasive and noninvasive blood pressure measurements in severely burned children.

Introduction: Accurate blood pressure monitoring is essential for burn management, with the intra-arterial line method being the gold standard. Here we evaluated agreement between cuff and intra-arterial line methods.

Methods: Data from burned children admitted from 1997 to 2016 were retrospectively reviewed.

Long-term deep-supercooling of large-volume water and red cell suspensions via surface sealing with immiscible liquids.

Supercooling of aqueous solutions is a fundamentally and practically important physical phenomenon with numerous applications in biopreservation and beyond. Under normal conditions, heterogeneous nucleation mechanisms critically prohibit the simultaneous long-term (> 1 week), large volume (> 1 ml), and low temperatures (< -10 °C) supercooling of aqueous solutions. Here, we report on the use of surface sealing of water by an oil phase to significantly diminish the primary heterogeneous nucleation at the water/air interface.

Cryopreservation of infectious Cryptosporidium parvum oocysts.

Cryptosporidiosis in an enteric infection caused by Cryptosporidium parasites and is a major cause of acute infant diarrhea in the developing world. A major bottleneck to research progress is the lack of methods to cryopreserve Cryptosporidium oocysts, thus requiring routine propagation in laboratory animals. Here, we report a method to cryopreserve C.

Estimated versus achieved maximal oxygen consumption in severely burned children maximal oxygen consumption in burned children.

Purpose: In burned children, exercise training increases maximal oxygen consumption (VO max) and can be combined with the nonspecific beta-blocker propranolol to decrease cardiac work. VO max is estimated if indirect calorimetry is not available. We compared measured and estimated VO max in severely burned children treated with or without propranolol to determine the suitability of commonly used formulas in these populations.

Microglia: An Intrinsic Component of the Proliferative Zones in the Fetal Rhesus Monkey (Macaca mulatta) Cerebral Cortex.

Microglial cells are increasingly recognized as modulators of brain development. We previously showed that microglia colonize the cortical proliferative zones in the prenatal brain and regulate the number of precursor cells through phagocytosis. To better define cellular interactions between microglia and proliferative cells, we performed lentiviral vector-mediated intraventricular gene transfer to induce enhanced green fluorescent protein expression in fetal cerebrocortical cells.

Metabolic Patterning on a Chip: Towards in vitro Liver Zonation of Primary Rat and Human Hepatocytes.

An important number of healthy and diseased tissues shows spatial variations in their metabolic capacities across the tissue. The liver is a prime example of such heterogeneity where the gradual changes in various metabolic activities across the liver sinusoid is termed as "zonation" of the liver. Here, we introduce the Metabolic Patterning on a Chip (MPOC) platform capable of dynamically creating metabolic patterns across the length of a microchamber of liver tissue via actively enforced gradients of various metabolic modulators such as hormones and inducers.

Publication trends in spine research from 2007 to 2016: Comparison of the Orthopaedic Research Society Spine Section and the International Society for the Study of the Lumbar Spine.

This study investigated current trends in spine publications of the membership of Orthopaedic Research Society Spine Section (ORS3) and the more global and clinically focused International Society for the Study of the Lumbar Spine (ISSLS). The PubMed database was probed to quantify trends in the overall number of articles published, the number of journals these articles were published in, and the number of active scientists producing new manuscripts. We also evaluated trends in flagship spine journals (, , and ) and in the The total number of active ORS3 and ISSLS authors and articles published have increased over the last 10 years.

Two-in-one: UV radiation simultaneously induces apoptosis and NETosis.

NETosis is a unique form of neutrophil death that differs from apoptosis and necrosis. However, whether NETosis and apoptosis can occur simultaneously in the same neutrophil is unknown. In this paper, we show that increasing doses of ultraviolet (UV) irradiation increases NETosis, which is confirmed by myeloperoxidase colocalisation to neutrophil extracellular DNA.

Chondrogenic Differentiation Processes in Human Bone-Marrow Aspirates Seeded in Three-Dimensional-Woven Poly(ɛ-Caprolactone) Scaffolds Enhanced by Recombinant Adeno-Associated Virus-Mediated SOX9 Gene Transfer.

Combining gene therapy approaches with tissue engineering procedures is an active area of translational research for the effective treatment of articular cartilage lesions, especially to target chondrogenic progenitor cells such as those derived from the bone marrow. This study evaluated the effect of genetically modifying concentrated human mesenchymal stem cells from bone marrow to induce chondrogenesis by recombinant adeno-associated virus (rAAV) vector gene transfer of the sex-determining region Y-type high-mobility group box 9 (SOX9) factor upon seeding in three-dimensional-woven poly(ɛ-caprolactone; PCL) scaffolds that provide mechanical properties mimicking those of native articular cartilage. Prolonged, effective SOX9 expression was reported in the constructs for at least 21 days, the longest time point evaluated, leading to enhanced metabolic and chondrogenic activities relative to the control conditions (reporter lacZ gene transfer or absence of vector treatment) but without affecting the proliferative activities in the samples.

Timing of Laparotomy and Closure in Burn Patients with Abdominal Compartment Syndrome: Effects on Survival.

Background: Survival of burn patients with abdominal compartment syndrome (ACS) is uniformly reported to be poor, averaging just 16% after laparotomy. We hypothesize that better outcomes can be achieved with a strategy of immediate laparotomy and early fascial closure.

Study Design: Patients with burn injury who were diagnosed with ACS between 2005 and 2016 were identified through a search of databases.

Strength and Cardiorespiratory Exercise Rehabilitation for Severely Burned Patients During Intensive Care Units: A Survey of Practice.

Minimizing the deconditioning of burn injury through early rehabilitation programs (RP) in the intensive care unit (ICU) is of importance for improving the recovery time. The aim of this study was to assess current standard of care (SOC) for early ICU exercise programs in major burn centers. We designed a survey investigating exercise RP on the ICU for burn patients with >30% total burned surface area.

Type III collagen affects dermal and vascular collagen fibrillogenesis and tissue integrity in a mutant Col3a1 transgenic mouse model.

Type III collagen is a major fibrillar collagen consisting of three identical α(III)-chains that is particularly present in tissues exhibiting elastic properties, such as the skin and the arterial wall. Heterozygous mutations in the COL3A1 gene result in vascular Ehlers-Danlos syndrome (vEDS), a severe, life-threatening disorder, characterized by thin, translucent skin and propensity to arterial, intestinal and uterine rupture. Most human vEDS cases result from a missense mutation substituting a crucial glycine residue in the triple helical domain of the α(III)-chains.

Development of clinical process measures for pediatric burn care: Understanding variation in practice patterns.

Background: There has been little systematic examination of variation in pediatric burn care clinical practices and its effect on outcomes. As a first step, current clinical care processes need to be operationally defined. The highly specialized burn care units of the Shriners Hospitals for Children system present an opportunity to describe the processes of care.

The Bat as a New Model of Cortical Development.

The organization of the mammalian cerebral cortex shares fundamental features across species. However, while the radial thickness of grey matter varies within one order of magnitude, the tangential spread of the cortical sheet varies by orders of magnitude across species. A broader sample of model species may provide additional clues for understanding mechanisms that drive cortical expansion.

Update on forebrain evolution: From neurogenesis to thermogenesis.

Comparative developmental studies provide growing understanding of vertebrate forebrain evolution. This short review directs the spotlight to some newly emerging aspects, including the evolutionary origin of the proliferative region known as the subventricular zone (SVZ) and of intermediate progenitor cells (IPCs) that populate the SVZ, neural circuits that originated within homologous regions across all amniotes, and the role of thermogenesis in the acquisition of an increased brain size. These data were presented at the 8th European Conference on Comparative Neurobiology.

Isolongifolene attenuates rotenone-induced mitochondrial dysfunction, oxidative stress and apoptosis.

The present study was carried out to investigate the neuroprotective effects of isolongifolene (ILF), a tricyclic sesquiterpene of Murraya koenigii, against rotenone-induced mitochondrial dysfunction, oxidative stress and apoptosis in a cellular model. SH-SY5Y human neuroblastoma cells were divided into four experimental groups (control, rotenone (100 nM), ILF (10 microM) + rotenone (100 nanoM), ILF 10 microM alone treated) based on 3-(4, 5-dimethyl 2-yl)-2, 5-diphenyltetrazolium bromide (MTT) assay. The results of the present study showed that the ILF treatment significantly alleviated rotenone-induced cytotoxicity, oxidative stress and mitochondrial dysfunction in SH-SY5Y cells.

Pharmacological Inhibition of the Pseudomonas aeruginosa MvfR Quorum-Sensing System Interferes with Biofilm Formation and Potentiates Antibiotic-Mediated Biofilm Disruption.

biofilms contribute to its survival on biotic and abiotic surfaces and represent a major clinical threat due to their high tolerance to antibiotics. Therefore, the discovery of antibiofilm agents may hold great promise. We show that pharmacological inhibition of the quorum-sensing regulator MvfR (PqsR) using a benzamide-benzimidazole compound interferes with biofilm formation and potentiates biofilm sensitivity to antibiotics.

Maternal autoimmune antibodies alter the dendritic arbor and spine numbers in the infragranular layers of the cortex.

An association between maternal IgG antibodies reactive against proteins in fetal brain and an outcome of autism in the child has been identified. Using a mouse model of prenatal intraventricular administration of autism-specific maternal IgG, we demonstrated that these antibodies produce behavioral alterations similar to those in children with ASD. We previously demonstrated that these antibodies bind to radial glial stem cells (RG) and observed an increase in the number of divisions of translocating RG in the developing cortex.