A new diffractometer for diffuse scattering studies on the ID28 beamline at the ESRF.

A new diffractometer is now available to the general user community at the ESRF. The new diffractometer is a side station of the high-resolution inelastic X-ray scattering spectrometer on beamline ID28 and is located in the same experimental hutch. Both instruments can be operated simultaneously. The new diffractometer combines a fast and low-noise hybrid pixel detector with a variable diffraction geometry. The beam spot on the sample is 50 µm × 50 µm, where focusing is achieved by a combination of Be lenses and a KB mirror. Wavelengths from 0.5 to 0.8 Šcan be used for the diffraction experiments. The setup is compatible with a variety of sample environments, allowing studies under non-ambient conditions. The diffractometer is optimized to allow a rapid survey of reciprocal space and diffuse scattering for the identification of regions of interest for subsequent inelastic scattering studies, but can also be employed as a fully independent station for structural studies from both powder and single-crystal diffraction experiments. Several software packages for the transformation and visualization of diffraction data are available. An analysis of data collected with the new diffractometer shows that the ID28 side station is a state-of-the-art instrument for structural investigations using diffraction and diffuse scattering experiments.

Environmental risk factors associated with bovine tuberculosis among cattle in high-risk areas.

Our research shows that environmental features are important predictors of bovine tuberculosis (bTB) in British cattle herds in high-prevalence regions. Data from 503 case and 808 control farms included in the randomized badger culling trial (RBCT) were analysed. bTB risk increased in larger herds and on farms with greater areas of maize, deciduous woodland and marsh, whereas a higher percentage of boundaries composed of hedgerows decreased the risk. The model was tested on another case-control study outside RBCT areas, and here it had a much smaller predictive power. This suggests that different infection dynamics operate outside high-risk areas, although it is possible that unknown confounding factors may also have played a role.

TGFß and IL10 have an impact on risk group and prognosis in childhood ALL.

BACKGROUND: Cytokines and their genes have been described to have an influence on incidence and prognosis in malignant, infectious and autoimmune disease. We previously described the impact of cytokine production on prognosis in paediatric standard-risk acute lymphoblastic leukaemia (ALL). PROCEDURE: In this study, we investigated the influence of cytokine gene polymorphisms (TNFα, TGFß, IL10 and IFNγ) on frequency, risk group and prognosis in 95 paediatric ALL-patients. We further report on intracellular production of these cytokines in T-cells. RESULTS: IL10 high-producer-haplotypes were reduced in ALL-patients compared with healthy controls and resulted in a reduced relapse rate compared with low-producer haplotypes. TGFß high-producer-haplotypes were correlated with a high initial blast-count (codon 25: G/G) and were elevated in high-risk ALL-patients (codon 10: T/T). IL10 was positively and IFNγ-production was negatively correlated with initial blast-count. At diagnosis the expression of TNFα and IFNγ was reduced in patients compared with healthy controls. This was more pronounced in high-risk and in T-ALL-patients. CONCLUSION: We conclude that gene-polymorphisms of the regulatory/anti-inflammatory cytokines, TGFß and IL10, but not of the pro-inflammatory cytokines, IFNγ and TNFα, have an impact on prognosis and risk-group of ALL. However, the reduced capacity to produce pro-inflammatory cytokines at diagnosis may serve as another important, functional risk factor. These data may help in further risk stratification and adaptation of therapy-intensity in paediatric patients with ALL.

Room-temperature polar order in [NH4][Cd(HCOO)3]--a hybrid inorganic-organic compound with a unique perovskite architecture.

We report on the hybrid inorganic-organic ammonium compound [NH4][Cd(HCOO)3], which displays a most unusual framework structure: instead of the expected 4(9)·6(6) topology, it shows an ABX3 perovskite architecture with the peculiarity and uniqueness (among all the up-to-date reported hybrid metal formates) that the Cd ions are connected only by syn-anti formate bridges, instead of anti-anti ones. This change of the coordination mode of the formate ligand is thus another variable that can provide new possibilities for tuning the properties of these versatile functional metal-organic framework materials. The room-temperature crystal structure of [NH4][Cd(HCOO)3] is noncentrosymmetric (S.G.: Pna21) and displays a polar axis. DFT calculations and symmetry mode analysis show that the rather large polarization arising from the off-center shift of the ammonium cations in the cavities (4.33 µC/cm(2)) is partially canceled by the antiparallel polarization coming from the [Cd(HCOO)3](-) framework, thus resulting in a net polarization of 1.35 µC/cm(2). As shown by second harmonic generation studies, this net polarization can be greatly increased by applying pressure (Pmax = 14 GPa), an external stimulus that, in turn, induces the appearance of new structural phases, as confirmed by Raman spectroscopy.

Role of disorder in the thermodynamics and atomic dynamics of glasses.

We measured the density of vibrational states (DOS) and the specific heat of various glassy and crystalline polymorphs of SiO2. The typical (ambient) glass shows a well-known excess of specific heat relative to the typical crystal (α-quartz). This, however, holds when comparing a lower-density glass to a higher-density crystal. For glassy and crystalline polymorphs with matched densities, the DOS of the glass appears as the smoothed counterpart of the DOS of the corresponding crystal; it reveals the same number of the excess states relative to the Debye model, the same number of all states in the low-energy region, and it provides the same specific heat. This shows that glasses have higher specific heat than crystals not due to disorder, but because the typical glass has lower density than the typical crystal.

Successful treatment of an infant with CDA type II by intrauterine transfusions and postnatal stem cell transplantation.

Congenital dyserythropoietic anemias are rare hematological disorders leading to ineffective erythropoiesis with chronic anemia, complicated by iron overload. Here we present a remarkable clinical course of an infant with CDA type II who first presented as a severe fetal hydrops, requiring serial intrauterine red cell transfusions. While postnatal transfusion dependency persisted, the patient was successfully transplanted with a myeloablative conditioning regimen and peripheral blood stem cells of a matched donor. We believe that allogeneic HSCT is a reasonable therapeutic approach for patients with very severe CDA, even if only a matched unrelated donor is available.

Structural analysis of potato starch transformation during high-energy ball-milling: Oxygen and humidity content effects.

High Energy Ball-Milling (HEBM) modifies starchs' granule morphology, physicochemical properties, and chemical structure. However, understanding how the HEBM changes the starch chemical structure is necessary to control these modifications. Therefore, this study aimed to investigate the changes in potato starch's long- and short-range molecular order during HEBM at different environmental conditions such as oxygen (Air) and humidity content. Due to the correlation between the starch modification and the energy supplied (Esupp) by the HEBM, Burgio's equation was used to calculate this energy. The starch transformation was followed by X-ray diffraction, Fourier Transform-Infrared Spectroscopy, and Raman spectroscopy. A Principal Component Analysis (PCA) was conducted to reduce the HEBM variables. PAC analysis demonstrated that the different oxygen-humidity conditions do not affect the HEBM of potato starch. Based on the starch chemical structure transformation correlated with Esupp during HEBM, four stages were observed: orientation, modification, mechanolysis, and over-destruction. It was identified for the first time that at low milling energy (<1.5 kJ/g, orientation stage), the glycosidic rings change their orientation, and starch-water interaction increases while the starch's organization reduces. Ergo, the potato starch could be more susceptible to chemical modifications during the first two stages.

Emergence of crystal-like atomic dynamics in glasses at the nanometer scale.

The vibrational dynamics of a permanently densified silica glass is compared to the one of an α-quartz polycrystal, the silica polymorph of the same density and local structure. The combined use of inelastic x-ray scattering experiments and ab initio numerical calculations provides compelling evidence of a transition, in the glass, from the isotropic elastic response at long wavelengths to a microscopic regime as the wavelength decreases below a characteristic length ξ of a few nanometers, corresponding to about 20 interatomic distances. In the microscopic regime the glass vibrations closely resemble those of the polycrystal, with excitations related to the acoustic and optic modes of the crystal. A coherent description of the experimental results is obtained assuming that the elastic modulus of the glass presents spatial heterogeneities of an average size a ~ ξ/2 π.

Physical exercise might influence the risk of oxygen-induced acute neurotoxicity.

OBJECTIVE: Hyperoxia can induce acute neurotoxicity with generalized seizures. Hyperoxia-induced reduction in cerebral blood flow velocity (CBFV) might be protective. It is unclear whether dynamic exercise during hyperoxia can overcome CBFV-reduction and thus possibly increase the risk of neurotoxicity. METHODS: We studied CBFV with both-sided transcranial Doppler with fixed transducer-position and heart rate under increasing hyperoxic conditions in nine professional military oxygen divers. The divers performed dynamic exercise on a bicycle-ergometer in a hyperbaric chamber (ergometries I-III, 21kPa, 100kPa, 150kPa pO2), with continuous blood pressure (ergometries I, II), end-tidal CO2 (PetCO2; ergometry I) being measured. RESULTS: Systolic (CBFVsyst) and diastolic CBFV (CBFVdiast) readings at rest decreased with increasing pO2. During exercise, CBFVsyst and CBFVdiast significantly increased in parallel with increasing pO2, despite reduced flow velocities at rest. ERGOMETRY I: CBFVsyst increased from 65.0 +/- 11.3 cm/second at rest to 80.2 +/- 23.4cm/s during maximum workload (n.s.), diastolic from 14.5 +/- 4.1 cm/second to 15.6 +/- 7.5 cm/s (n.s.). PetCO2 increased from 43.4 +/- 7.8mmHg to 50.0 +/- 7.5mmHg. ERGOMETRY II: CBFVsyst increased from 58.2 +/- 16.5 cm/second to 99.7 +/- 17.0 cm/s (p<0.001), diastolic from 14.0 +/- 10.7 cm/second to 29.4 +/- 11.1 cm/second (p<0.01). ERGOMETRY III: CBFVsyst increased from 54.4 +/-15.0cm/second to 109.4 +/- 22.3cm/s (p<0.001), diastolic from 14.7 +/- 10.4 cm/second to 35.5 +/- 9.3 cm/second (p<0.01). INTERPRETATION: Physical exercise overrules the decrease in CBFV during hyperoxia and leads to even higher CBFV-increases with increasing pO2. A tendency towards CO2 retainment with elevated PetCOz may be causative and thus heighten the risk of oxygen-induced neurotoxicity.

N-terminal prohormone of brain natriuretic peptide: a useful tool for the detection of acute pulmonary artery embolism in post-surgical patients.

BACKGROUND: Acute pulmonary embolism (APE) is an important clinical problem in patients after major surgery and often remains a difficult diagnosis because of unspecific clinical symptoms. Therefore, we investigated the role of N-terminal prohormone of brain natriuretic peptide (NT-proBNP) for the detection of APE. METHODS: In 44 patients with suspected APE referred to the intensive care unit after major surgery, serum NT-proBNP, troponin-I, and D-dimers were measured according to the standard hospital protocol. To definitively confirm or exclude APE, all patients underwent an angiographic CT scan of the thorax. RESULTS: APE was confirmed in 28 and excluded in 16 patients by CT scan. NT-proBNP was significantly (P<0.01) higher in patients with APE [4425 (sd 8826; range 63-35 000) pg ml(-1)] compared with those without [283 (sd 327; range 13-1133) pg ml(-1)]. The sensitivity of the NT-proBNP screening was 93%, specificity 63%, positive predictive value 81%, and negative predictive value 83%. There were no significant (P = 0.96) differences in D-dimers between subjects with and without APE [confirmed APE: 511 (sd 207; range 83-750) µg litre(-1); excluded APE: 509 (sd 170; range 230-750) µg litre(-1)]. Troponin-I levels were not elevated in 32% of the patients with APE. CONCLUSIONS: D-dimer levels are frequently elevated in post-surgical patients and not applicable for confirmation or exclusion of APE. In contrast, NT-proBNP appears to be a useful biomarker for APE diagnosis in the postoperative setting. In the case of NT-proBNP levels below the upper reference limit, haemodynamically relevant APE is unlikely. Troponin-I in contrast is not considered to be helpful.

Heart failure resulting from giant left atrial synovial sarcoma metastasis.

Synovial sarcoma metastasis affecting the heart and infiltrating the mitral valve is a very rare pathology. We report the case of a 44-year-old male treated with chemotherapy for atypical synovial sarcoma of the oral mucosa who presented to our clinic after cardiac decompensation with a presumptive diagnosis of myxoma of the left atrium. A large necrotic tumour positive for CK 22, EMA, CD 99 and BCL-2 but negative for translocation in COBRA-FISH analysis by break-apart probe could be excised and revealed a very rare subtype of synovial sarcoma metastasis arising from the endocard of the left atrium. The tumour was resected and the mitral valve reconstructed through ring annuloplasty.

Mechanical properties of the bovine claw horn during lactation.

Claw horn disorders are one of the main causes of lameness in dairy cows globally. This study aimed to develop material testing techniques to assess changes in the mechanical properties of bovine claw horn (BCH) and to compare these mechanical properties with existing methods of assessing claw horn disorders during lactation. Lameness was also measured through locomotion scoring to assess the clinical significance of changes observed in the scoring for lesions. Experiment 1 used 8 claws collected from four 12 to 18 mo old beef heifers, to develop BCH sample storage methods and techniques to test the mechanical properties of BCH (puncture resistance and elastic modulus). The increase in the moisture content of BCH had a significant negative exponential effect on the elastic modulus of the sole and white line claw horn and a linear reduction in the puncture resistance of BCH. Placing BCH samples in sealed plastic bags and storing them either at 2°C or by freezing samples at -22°C did not alter the dry matter content and, consequently, the mechanical properties of the claw horn tissue. In experiment 2, BCH was collected from 36 lactating dairy cows and mechanical properties were tested using puncture resistance. Puncture resistance of the sole area of the claw horn decreased significantly when hemorrhages in the tested area increased. The puncture resistance of the sole and white line areas decreased at d 160 postpartum when the cows exhibited higher lesion scores and was lower in hind claws that had higher lesion scores when compared with the fore claws. The highest puncture resistance was found at 270 d postpartum, when the animals were at pasture. Puncture resistance was found to be an effective technique for assessing the effect of period of lactation and increasing hemorrhage levels on the mechanical properties and structural strength of bovine claw horn. It was found to be a good method of comparing changes and differences in mechanical properties and structural strength of BCH from the sole and white line areas within each claw and differences between claws. White line BCH consistently had significantly lower puncture resistance compared with the sole. Bovine claw horn with greater levels of hemorrhage or lower puncture resistance, or both, may provide less protection and increase the risk of foreign body penetration, trauma, and secondary infection of the corium of the claw.

Effects of FLIRT on bubble growth in man.

Recompression during decompression has been suggested to possibly reduce the risk of decompression sickness (DCS). The main objective of the current study was to investigate the effects of FLIRT (First Line Intermittent Recompression Technique) on bubble detection in man. 29 divers underwent 2 simulated dives in a dry recompression chamber to a depth of 40 msw (500 kPa ambient pressure) in random order. A Buehlmann-based decompression profile served as control and was compared to an experimental profile with intermittent recompression during decompression (FLIRT). Circulating bubbles in the right ventricular outflow tract (RVOT) were monitored by Doppler ultrasound and quantified using the Spencer scoring algorithm. Heat shock protein 70 (HSP70), thrombocytes, D-Dimers and serum osmolarity were analyzed before and 120 min after the dive. Both dive profiles elicited bubbles in most subjects (range Spencer 0-4). However, no statistically significant difference was found in bubble scores between the control and the experimental dive procedure. There was no significant change in either HSP70, thrombocytes, and D-Dimers. None of the divers had clinical signs or symptoms suggestive of DCS. We conclude that FLIRT did not significantly alter the number of microbubbles and thus may not be considered superior to classical decompression in regards of preventing DCS.

Allogeneic hematopoietic stem cell transplantation in Glanzmann thrombasthenia complicated by platelet alloimmunization.

BACKGROUND: For Thrombasthenia Glanzmann (GT) patients presenting with a severe clinical phenotype due to complete lack of thrombocyte function or increased titres of anti-platelet antibodies hematopoietic stem cell transplantation (SCT) is the only curative therapy. CASE REPORT: We report the case of a 13-month-old boy, presenting with a severe course of GT, who was successfully treated with an HLA-identical sibling bone marrow transplant. SCT was complicated by anti-platelet alloimmunization after platelet transfusion successfully treated with high dosage immunoglobulins (2 g/kg) and partial plasma exchange. CONCLUSION: SCT may be a viable option for selected patients with GT. However, SCT in GT carries its own significant risks, resulting from the development of anti-platelet antibodies. A critical risk-benefit analysis is mandatory prior to SCT.

Artificial granularity in two-dimensional arrays of nanodots fabricated by focused-electron-beam-induced deposition.

We have prepared 2D arrays of nanodots embedded in an insulating matrix by means of focused-electron-beam-induced deposition using the W(CO)(6) precursor. By varying the deposition parameters, i.e. the electron beam current and energy and the raster constant, we obtain an artificial granular material with tunable electrical properties. The analysis of the temperature dependence of the conductivity and of the current-voltage characteristic suggests that the transport mechanism is governed by electron tunneling between artificial grains. In order to understand the nature of the granularity and thus the microstructural origin of the electronic transport behavior, we perform TEM and micro-Raman investigations. Independent of the deposition parameters, TEM measurements show that the dots are constituted of amorphous tungsten carbide clusters embedded in an amorphous carbonaceous matrix. Micro-Raman spectra show two peaks, around 690 and 860 cm(-1) associated with the W-C stretching modes. Higher frequency peaks give information on the composition of the matrix. In particular, we measure a peak at about 1290 cm(-1), which is associated with sp(3) carbon bonds. Furthermore we detect the so-called D and G peaks, at about 1350 and 1560 cm(-1), associated with the vibration modes of the sp(2) carbon bonds. The analysis of the position of the peaks and of their relative intensity suggests that the composition of the matrix is between nanocrystalline graphite and amorphous carbon.

Pulmonary function in children after open water SCUBA dives.

An increasing number of children and adolescents is diving with Self-Contained Underwater Breathing Apparatus (SCUBA). SCUBA diving is associated with health risks such as pulmonary barotrauma, especially in children and in individuals with airflow limitation. As no data has been published on the effects of open-water diving on pulmonary function in children, the objective of this study was to evaluate the effects of SCUBA dives on airflow in children. 16 healthy children aged 10-13 years underwent spirometry and a cycle-exercise challenge while breathing cold air. They subsequently performed dives to 1-m and 8-m depth in random order. Pulmonary function was measured before and after the exercise challenge and the dives. There were statistically significant decreases in FEV1, FVC, FEV1/FVC, MEF25 and MEF50 after the cold-air exercise challenge and the dives. Changes in lung function following the exercise challenge did not predict the responses to SCUBA diving. In 3 children the post-dive decrements in FEV1 exceeded 10%. These children had a lower body weight and BMI percentile. SCUBA diving in healthy children may be associated with relevant airflow limitation. A low body mass might contribute to diving-associated bronchoconstriction. In the majority of subjects, no clinically relevant airway obstruction could be observed.

Concomitant intrauterine growth restriction alters the lipoprotein profile in preeclampsia.

OBJECTIVE: Preeclampsia and intrauterine growth restriction (IUGR) are related conditions. We aimed to characterise common lipid changes. METHODS: Triglyceride and cholesterol levels of patients 24-42 weeks of gestation with IUGR (n = 52), hypertensive IUGR (HIUGR, n = 28), and preeclampsia without IUGR (PE, n = 56) were compared to a control group (CTRL, n = 167). In addition, 60 sera (n = 10 of each pathology IUGR, HIUGR, PE (without IUGR) compared to n = 30 matched CTRL) of severe early onset cases <34 weeks of gestation were chosen and further analysed by ultracentrifugation lipid subfractionation including VLDL, IDL, LDL, and HDL composition. RESULTS: In the full cohort we found low cholesterol in IUGR (p = 0.0405), while triglyceride levels were high in PE (p < 0.0001). Lipid concentrations in HIUGR did not differ significantly from CTRL. In the 60 patients analysed by lipid subfractionation, triglyceride levels were increased in the VLDL subfraction in PE (p < 0.01), however, LDL-bound ApoB and cholesterol levels were lower in IUGR and HIUGR (p < 0.0001 for total cholesterol and p < 0.001 for ApoB in both groups), but not in PE when compared to CTRL. CONCLUSION: Low cholesterol, especially LDL cholesterol levels are a feature of IUGR while high triglyceride levels are a feature of preeclampsia. Increased VLDL-triglycerides suggest a disturbed conversion to LDL in preeclampsia. Of note, the presence of IUGR in hypertensive disorders further alters lipid profiles, which may explain heterogeneous data on lipid values for preeclampsia in the literature. Study groups have to be selected carefully to avoid misinterpretation.

New dynamic diamond anvil cells for tera-pascal per second fast compression x-ray diffraction experiments.

Fast compression experiments performed using dynamic diamond anvil cells (dDACs) employing piezoactuators offer the opportunity to study compression-rate dependent phenomena. In this paper, we describe an experimental setup which allows us to perform time-resolved x-ray diffraction during the fast compression of materials using improved dDACs. The combination of the high flux available using a 25.6 keV x-ray beam focused with a linear array of compound refractive lenses and the two fast GaAs LAMBDA detectors available at the Extreme Conditions Beamline (P02.2) at PETRA III enables the collection of x-ray diffraction patterns at an effective repetition rate of up to 4 kHz. Compression rates of up to 160 TPa/s have been achieved during the compression of gold in a 2.5 ms fast compression using improved dDAC configurations with more powerful piezoactuators. The application of this setup to low-Z compounds at lower compression rates is described, and the high temporal resolution of the setup is demonstrated. The possibility of applying finely tuned pressure profiles opens opportunities for future research, such as using oscillations of the piezoactuator to mimic propagation of seismic waves in the Earth.

Tropical peatland carbon storage linked to global latitudinal trends in peat recalcitrance.

Peatlands represent large terrestrial carbon banks. Given that most peat accumulates in boreal regions, where low temperatures and water saturation preserve organic matter, the existence of peat in (sub)tropical regions remains enigmatic. Here we examined peat and plant chemistry across a latitudinal transect from the Arctic to the tropics. Near-surface low-latitude peat has lower carbohydrate and greater aromatic content than near-surface high-latitude peat, creating a reduced oxidation state and resulting recalcitrance. This recalcitrance allows peat to persist in the (sub)tropics despite warm temperatures. Because we observed similar declines in carbohydrate content with depth in high-latitude peat, our data explain recent field-scale deep peat warming experiments in which catotelm (deeper) peat remained stable despite temperature increases up to 9 °C. We suggest that high-latitude deep peat reservoirs may be stabilized in the face of climate change by their ultimately lower carbohydrate and higher aromatic composition, similar to tropical peats.