Nitrous oxide fluxes from tropical peat with different disturbance history and management

Peer reviewe

Two-center experience of cannabidiol use in adults with Dravet syndrome

We describe real-world experience with cannabidiol (CBD) in adults with Dravet Syndrome (DS) via GW Pharma early access programme at two UK neurology centres. Adults with genetically-confirmed DS had CBD added to existing therapy, titrated up to 20 mg/kg, as tolerated. The primary outcome measure was percentage reduction in convulsive seizures. Secondary outcome measures included changes in myoclonic seizures, and in cognition and quality of life as assessed by the Caregiver Global Impression of Change (CGIC), and incidence of adverse events (AEs). 18 adults (7 female; median age 27.5 years; range 20–51) were included. Median follow-up was 176 days. In one, another antiseizure drug, clobazam, was introduced during the programme. 3/17 (17.6%) had >30% reduction in convulsive seizures (range: 87.5–100%). AEs occurred in all, the most common being transaminitis (52.9%). Behavioural AEs led to discontinuation in 3/18 (16.7%), including a seizure-free responder. In 7/18, CBD was stopped due to lack of effect. 8/18 continue on treatment. Improvements in CGIC were reported in 41.2% and 47.1% by physicians and families, respectively. 17.6% achieved sufficient reduction in convulsive seizure frequency to qualify for NHS funding. AEs led to withdrawal in only 16.7%. Close monitoring and dose adjustments of other antiseizure drugs were necessary

Non-Stationary Outcome of Alternating Hemiplegia of Childhood into Adulthood

Background Although described as non-progressive, alternating hemiplegia of childhood (AHC) can display a sudden deterioration, anecdotally reported mainly in childhood. Outcome in adulthood is uncertain. Objectives Aim of this study is to describe the long-term follow-up of neurological function in adults with AHC. Methods Seven adults with AHC were included in this retrospective single-center study. Clinical history and previous investigation data were gathered from the review of medical records. Video-documented neurological examination was performed at the last follow-up visit in four out of the seven reported indivisuals. Results Over a median follow-up of 16 years, neurological outcome and trajectories were heterogeneous. All individuals showed new neurological signs or symptoms. Three experienced a serious irreversible neurological deterioration after prolonged quadriplegic episodes and/or status epilepticus in their second or third decade. One patient died at age 29. Conclusions This video-series suggests that AHC in adulthood is not stationary; larger cohorts are needed to identify genotype–phenotype correlations and clinically useful outcome predictors

Mapping peat soil moisture under oil palm plantation and tropical forest in Sarawak

Water table conditions in drained peatlands affect peat decomposition, fluvial carbon and greenhouse gas emissions, and plant growth in oil palm plantations. This study illustrates the spatial heterogeneity of soil moisture profiles in cultivated tropical peat under oil palm plantation and uncultivated secondary forest, using maps. At a study plot under each land use the geographical coordinates of sampling points, tree locations and other features were recorded. Peat soil samples were taken at depths of 0–50 cm, 50–100 cm, 100–150 cm and 150–200 cm, and their moisture contents were determined. Overall, soil moisture content was higher in secondary forest than in oil palm plantation due to land management activities such as drainage and peat compaction in the latter. Significant differences were observed between the topsoil (0–50 cm) and deeper soil layers under both land uses. Soil moisture maps of the study plots interpolated using geographical information system (GIS) software were used to visualise the spatial distributions of moisture content in soil layers at different depths (0–50 cm, 50–100 cm, 100–150 cm, 150–200 cm). Moisture content in the 0–50 cm soil layer appeared to be inversely related to elevation, but the correlation was not statistically significant. On the other hand, there was a significant positive correlation between soil moisture content and the diameters of oil palm trunks. Palm trees with negative growth of trunk diameter were mostly located in subplots which were relatively dry and/or located near drains. The results of this study indicate that soil moisture mapping using GIS could be a useful tool in improving the management of peatland to promote oil palm growth.Peer reviewe

The crosstalk between FGF21 and GH leads to weakened GH receptor signaling and IGF1 expression and is associated with growth failure in very preterm infants.

BACKGROUND: Fibroblast growth factor 21 (FGF21) is an essential metabolic regulator that adapts to changes in nutritional status. Severe childhood undernutrition induces elevated FGF21 levels, contributing to growth hormone (GH) resistance and subsequent linear growth attenuation potentially through a direct action on chondrocytes. METHODS: In this study, we assessed expression of the components of both GH and FGF21 pathways in rare and unique human growth plates obtained from children. Moreover, we investigated the mechanistic interplay of FGF21 on GH receptor (GHR) signaling in a heterologous system. RESULTS: Chronic FGF21 exposure increased GH-induced GHR turnover and SOCS2 expression, leading to the inhibition of STAT5 phosphorylation and IGF-1 expression. The clinical significance of FGF21 signaling through GH receptors was tested in nutritionally driven growth failure seen in very preterm (VPT) infants right after birth. VPT infants display an immediate linear growth failure after birth followed by growth catch-up. Consistent with the in vitro model data, we show that circulating FGF21 levels were elevated during deflection in linear growth compared to catch-up growth and were inversely correlated with the length velocity and circulating IGF1 levels. CONCLUSIONS: This study further supports a central role of FGF21 in GH resistance and linear growth failure and suggests a direct action on the growth plate

Bioremediation of creosote contaminated soil in both laboratory and field scale: Investigating the ability of methyl-β-cyclodextrin to enhance biostimulation

© 2015 Elsevier Ltd. We investigated the bioremediation of 16 polycyclic aromatic hydrocarbons (PAH) in historically creosote contaminated soil using both laboratory and field scale experiments. We found that nutrient amendments and circulation of methyl-β-cyclodextrin (CD) solution enhanced soil microbial degradation capacity. In the laboratory experiment, the degradation of lower molecular weight, 2-3 ringed PAHs was achieved already by circulating nutrient solution and the use of CD mainly increased the desorption and removal of larger, 4-5 aromatic ringed PAH compounds. The 1% CD concentration was most feasible for bioremediation as most of the extracted PAH compounds were degraded. In the 5% CD treatment, the PAH desorption from soil was too fast compared to the degradation capacity and 25% of the total PAH amount remained in the circulated solution. Similar lab-scale results have been generated earlier, but very little has been done in full field scale, and none in freezing conditions. Although freezing stopped circulation and degradation completely during the winter, PAH degradation returned during the warm period in the field test. Circulation effectiveness was lower than in the laboratory but the improved nutrient and moisture content seemed to be the main reason for decreasing soil PAH concentrations. It also appeared that PAH extraction yield in chemical analysis was increased by the CD treatment in field conditions and the results of CD-treated and non-treated soil may therefore not be directly comparable. Therefore, a positive effect of CD on PAH degradation velocity could not be statistically confirmed in the field test. Based on our results, we recommend initiating the bioremediation of PAH contaminated soil by enhancing the microbial degradation with nutrient amendments. The CD seems to be useful only at the later stage when it increases the solubilisation of strongly absorbed contaminants to some extent. More investigation is also needed of the CD effect on the PAH yield in the chemical analysis

Late diagnoses of Dravet syndrome: How many individuals are we missing?

We report new genetic diagnoses of Dravet syndrome in a group of adults with complex epilepsy of unknown cause, under follow up at a tertiary epilepsy centre. Individuals with epilepsy and other features of unknown cause from our unit underwent whole genome sequencing through the 100,000 Genomes Project. Virtual gene panels were applied to frequency-filtered variants based on phenotype summary. Of 1078 individuals recruited, 8 (0.74%) were identified to have a pathogenic or likely pathogenic variant in SCN1A. Variant types were: nonsense (stopgain) in five (62.5%) and missense in three (37.5%). Detailed review of childhood history confirmed a phenotype compatible with Dravet syndrome. Median age at genetic diagnosis was 44.5 years (range 28-52 years). Tonic-clonic seizures were ongoing in all despite polytherapy including valproate. All had a history of fever sensitivity and myoclonic seizures, which were ongoing in two (25%) and three (37.5%) individuals, respectively. Salient features of Dravet syndrome may be less apparent in adulthood, making clinical diagnosis difficult. Regardless of age, benefits of a genetic diagnosis include access to syndrome-specific treatment options, avoidance of harmful drugs, and monitoring for common complications