EuCd_{2}As_{2}: A Magnetic Semiconductor.

EuCd_{2}As_{2} is now widely accepted as a topological semimetal in which a Weyl phase is induced by an external magnetic field. We challenge this view through firm experimental evidence using a combination of electronic transport, optical spectroscopy, and excited-state photoemission spectroscopy. We show that the EuCd_{2}As_{2} is in fact a semiconductor with a gap of 0.77 eV. We show that the externally applied magnetic field has a profound impact on the electronic band structure of this system. This is manifested by a huge decrease of the observed band gap, as large as 125 meV at 2 T, and, consequently, by a giant redshift of the interband absorption edge. However, the semiconductor nature of the material remains preserved. EuCd_{2}As_{2} is therefore a magnetic semiconductor rather than a Dirac or Weyl semimetal, as suggested by ab initio computations carried out within the local spin-density approximation.

Transport properties and doping evolution of the Fermi surface in cuprates.

Measured transport properties of three representative cuprates are reproduced within the paradigm of two electron subsystems, itinerant and localized. The localized subsystem evolves continuously from the Cu 3d[Formula: see text] hole at half-filling and corresponds to the (pseudo)gapped parts of the Fermi surface. The itinerant subsystem is observed as a pure Fermi liquid (FL) with material-independent universal mobility across the doping/temperature phase diagram. The localized subsystem affects the itinerant one in our transport calculations solely by truncating the textbook FL integrals to the observed (doping- and temperature-dependent) Fermi arcs. With this extremely simple picture, we obtain the measured evolution of the resistivity and Hall coefficients in all three cases considered, including LSCO which undergoes a Lifshitz transition in the relevant doping range, a complication which turns out to be superficial. Our results imply that prior to evoking polaronic, quantum critical point, quantum dissipation, or even more exotic scenarios for the evolution of transport properties in cuprates, Fermi-surface properties must be addressed in realistic detail.

Severe Form of Salih Myopathy Caused by Combination of Two Heterozygous TTN Mutations.

Salih myopathy is autosomal recessive hereditary early-onset myopathy with fatal cardiomyopathy. It is a rare and heterogeneous form of congenital titinopathies (TTN). Affected children have delayed motor development, normal mental development, and in further course dilated cardiomyopathy. Motor functions have a tendency to improve, but death occurs most often before 20 years of age due to arrhythmias. Our patient is a 2-year-old girl, born in severe perinatal asphyxia, with global hypotonia and poor spontaneous movements. She required immediate endotracheal intubation and mechanical ventilation was initiated without the possibility of cessation. Improvement in her neurological status was not observed. Due to her clinical presentation, we performed genetic testing and a diagnosis of Salih myopathy caused by combination of two heterozygous TTN mutations was confirmed. This case illustrates that Salih myopathy may have severe presentation from birth, with continuous necessity for mechanical ventilation, without any motor improvement.

High-T c Cuprates: a Story of Two Electronic Subsystems.

A review of the phenomenology and microscopy of cuprate superconductors is presented, with particular attention to universal conductance features, which reveal the existence of two electronic subsystems. The overall electronic system consists of 1 + p charges, where p is the doping. At low dopings, exactly one hole is localized per planar copper-oxygen unit, while upon increasing doping and temperature, the hole is gradually delocalized and becomes itinerant. Remarkably, the itinerant holes exhibit identical Fermi liquid character across the cuprate phase diagram. This universality enables a simple count of carrier density and yields comprehensive understanding of the key features in the normal and superconducting state. A possible superconducting mechanism is presented, compatible with the key experimental facts. The base of this mechanism is the interaction of fast Fermi liquid carriers with localized holes. A change in the microscopic nature of chemical bonding in the copper oxide planes, from ionic to covalent, is invoked to explain the phase diagram of these fascinating compounds.

A clinical diagnostic algorithm for early onset cerebellar ataxia.

Early onset cerebellar Ataxia (EOAc) comprises a large group of rare heterogeneous disorders. Determination of the underlying etiology can be difficult given the broad differential diagnosis and the complexity of the genotype-phenotype relationships. This may change the diagnostic work-up into a time-consuming, costly and not always rewarding task. In this overview, the Childhood Ataxia and Cerebellar Group of the European Pediatric Neurology Society (CACG-EPNS) presents a diagnostic algorithm for EOAc patients. In seven consecutive steps, the algorithm leads the clinician through the diagnostic process, including EOA identification, application of the Inventory of Non-Ataxic Signs (INAS), consideration of the family history, neuro-imaging, laboratory investigations, genetic testing by array CGH and Next Generation Sequencing (NGS). In children with EOAc, this algorithm is intended to contribute to the diagnostic process and to allow uniform data entry in EOAc databases.

Unusual behavior of cuprates explained by heterogeneous charge localization.

The discovery of high-temperature superconductivity in cuprates ranks among the major scientific milestones of the past half century, yet pivotal questions regarding the complex phase diagram of these materials remain unanswered. Generally thought of as doped charge-transfer insulators, these complex oxides exhibit pseudogap, strange-metal, superconducting, and Fermi liquid behavior with increasing hole-dopant concentration. Motivated by recent experimental observations, here we introduce a phenomenological model wherein exactly one hole per planar copper-oxygen unit is delocalized with increasing doping and temperature. The model is percolative in nature, with parameters that are highly consistent with experiments. It comprehensively captures key unconventional experimental results, including the temperature and the doping dependence of the pseudogap phenomenon, the strange-metal linear temperature dependence of the planar resistivity, and the doping dependence of the superfluid density. The success and simplicity of the model greatly demystify the cuprate phase diagram and point to a local superconducting pairing mechanism.

Hole pocket-driven superconductivity and its universal features in the electron-doped cuprates.

After three decades of intensive research attention, the emergence of superconductivity in cuprates remains an unsolved puzzle. One major challenge has been to arrive at a satisfactory understanding of the unusual metallic "normal state" from which the superconducting state emerges upon cooling. A second challenge has been to achieve a unified understanding of hole- and electron-doped compounds. Here, we report detailed magnetoresistance measurements for the archetypal electron-doped cuprate Nd2-x Ce x CuO4+δ that, in combination with previous data, provide crucial links between the normal and superconducting states and between the electron- and hole-doped parts of the phase diagram. The characteristics of the normal state (magnetoresistance, quantum oscillations, and Hall coefficient) and those of the superconducting state (superfluid density and upper critical field) consistently indicate two-band (electron and hole) features and point to hole pocket-driven superconductivity in these nominally electron-doped materials. We show that the approximate Uemura scaling between the superconducting transition temperature and the superfluid density found for hole-doped cuprates also holds for the small hole component of the superfluid density in electron-doped cuprates.

Hidden Fermi-liquid Charge Transport in the Antiferromagnetic Phase of the Electron-Doped Cuprate Superconductors.

Systematic analysis of the planar resistivity, Hall effect, and cotangent of the Hall angle for the electron-doped cuprates reveals underlying Fermi-liquid behavior even deep in the antiferromagnetic part of the phase diagram. The transport scattering rate exhibits a quadratic temperature dependence, and is nearly independent of doping and compound and carrier type (electrons versus holes), and hence is universal. Our analysis moreover indicates that the material-specific resistivity upturn at low temperatures and low doping has the same origin in both electron- and hole-doped cuprates.

Spread of Enterobacter cloacae carrying blaNDM-1, blaCTX-M-15, blaSHV-12 and plasmid-mediated quinolone resistance genes in a surgical intensive care unit in Croatia.

The objective of this study was to describe a hospital cluster of NDM-1-producing Enterobacter cloacae infections observed in the surgical intensive care unit (ICU) of a tertiary-care hospital at Pula, Croatia. NDM-1-producing E. cloacae strains isolated from clinical samples were screened by PCR for the presence of carbapenemases. Genetic relatedness of NDM-1-producing E. cloacae strains was determined by multilocus sequence typing (MLST). During the period October 2013 to April 2014, four patients, with overlapping hospital stay in the surgical ICU, developed severe infections caused by E. cloacae demonstrated to produce carbapenemases. According to MLST, all strains belonged to ST133 and were positive by PCR for the blaNDM-1 carbapenemase gene, for blaCTX-M-15 and blaSHV-12 extended-spectrum ß-lactamase (ESBL) genes, and for blaTEM-1 and blaOXA-1 narrow-spectrum ß-lactamase genes. They were negative for other carbapenemases genes including blaOXA-48, blaVIM and blaKPC as well as for AmpC and the armA and rmtB aminoglycoside resistance genes. All strains were positive for the HI2 replicon, suggesting that an IncHI2 plasmid is likely the plasmid carrying the blaNDM-1 gene. Infection control measures were implemented after the first case although they were not effective in avoiding spread of this organism to other patients in the surgical ICU. In conclusion, the evolving epidemiology of NDM-producing micro-organisms and the interspecies diffusion of this resistance mechanism to emerging pathogens such as E. cloacae necessitate the setting up of strong and urgent joint measures to control the spread of NDM carbapenemase especially in the ICU setting.

Single reconstructed Fermi surface pocket in an underdoped single-layer cuprate superconductor.

The observation of a reconstructed Fermi surface via quantum oscillations in hole-doped cuprates opened a path towards identifying broken symmetry states in the pseudogap regime. However, such an identification has remained inconclusive due to the multi-frequency quantum oscillation spectra and complications accounting for bilayer effects in most studies. We overcome these impediments with high-resolution measurements on the structurally simpler cuprate HgBa2CuO4+δ (Hg1201), which features one CuO2 plane per primitive unit cell. We find only a single oscillatory component with no signatures of magnetic breakdown tunnelling to additional orbits. Therefore, the Fermi surface comprises a single quasi-two-dimensional pocket. Quantitative modelling of these results indicates that a biaxial charge density wave within each CuO2 plane is responsible for the reconstruction and rules out criss-crossed charge stripes between layers as a viable alternative in Hg1201. Lastly, we determine that the characteristic gap between reconstructed pockets is a significant fraction of the pseudogap energy.

The rate of quasiparticle recombination probes the onset of coherence in cuprate superconductors.

In the underdoped copper-oxides, high-temperature superconductivity condenses from a nonconventional metallic "pseudogap" phase that exhibits a variety of non-Fermi liquid properties. Recently, it has become clear that a charge density wave (CDW) phase exists within the pseudogap regime. This CDW coexists and competes with superconductivity (SC) below the transition temperature Tc, suggesting that these two orders are intimately related. Here we show that the condensation of the superfluid from this unconventional precursor is reflected in deviations from the predictions of BSC theory regarding the recombination rate of quasiparticles. We report a detailed investigation of the quasiparticle (QP) recombination lifetime, τqp, as a function of temperature and magnetic field in underdoped HgBa2CuO(4+δ) (Hg-1201) and YBa2Cu3O(6+x) (YBCO) single crystals by ultrafast time-resolved reflectivity. We find that τqp(T) exhibits a local maximum in a small temperature window near Tc that is prominent in underdoped samples with coexisting charge order and vanishes with application of a small magnetic field. We explain this unusual, non-BCS behavior by positing that Tc marks a transition from phase-fluctuating SC/CDW composite order above to a SC/CDW condensate below. Our results suggest that the superfluid in underdoped cuprates is a condensate of coherently-mixed particle-particle and particle-hole pairs.

Low bone mineral density for age/osteoporosis in triple A syndrome-an overlooked symptom of unexplained etiology.

UNLABELLED: Triple A syndrome (alacrima, achalasia, adrenal failure, progressive neurodegenerative disease) is caused by mutations in the AAAS gene which encodes the protein alacrima achalasia adrenal insufficiency neurologic disorder (ALADIN). Our investigation suggests that low bone mineral density (BMD) for age/osteoporosis could be a common but overlooked symptom of unexplained etiology in this rare multisystemic disease. INTRODUCTION: The purpose of this study is to evaluate incidence and etiology of BMD for age/osteoporosis, a possibly overlooked symptom in triple A syndrome. METHODS: Dual-energy X-ray absorptiometry (DXA) of the femoral neck, total hip, lumbar spine, and radius, bone turnover markers, minerals, total alkaline phosphatase (ALP), 25-hydroxy vitamin D (25-OHD), 1,25-dihydroxy vitamin D (1,25-OH2D), intact parathyroid hormone (PTH), and adrenal androgens (dehydroepiandrosterone sulfate (DHEAS) and androstenedione) were measured in five male and four female patients. RESULTS: At time of diagnosis, low BMD for age was suspected on X-ray in seven of nine patients aged 2-11 years (not performed in two patients); normal levels of minerals and ALP were found in nine patients and low levels of adrenal androgens in eight patients (not measured in one patient). Reevaluation 5-35 years after introduction of 12 mg/m(2)/day hydrocortisone showed low BMD for age in two children, osteopenia in one, and osteoporosis in six adults. Normal levels of minerals, ALP, PTH, 1,25-OH2D, procollagen type 1, crosslaps, and osteocalcin were found in all patients. Low levels of adrenal androgens were found in all and 25OHD deficiency in six patients. Body mass index was <25 % for age and sex in eight of nine patients. CONCLUSION: Low BMD for age/osteoporosis in our patients probably is not a result of glucocorticoid therapy but could be the consequence of low level of adrenal androgens, neurological impairment causing physical inactivity, inadequate sun exposure, and protein malnutrition secondary to achalasia. Considering ubiquitous ALADIN expression, low BMD/osteoporosis may be a primary phenotypic feature of the disease. Besides optimizing glucocorticoid dose, physical activity, adequate sun exposure, appropriate nutrition, and vitamin D supplementation, therapy with DHEA should be considered.

Risk Factors for Neonatal Sepsis and Method for Reduction of Blood Culture Contamination.

BACKGROUND: False-positive blood cultures findings may lead to a falsely increased morbidity and increased hospital costs. METHOD: The survey was conducted as retrospective - prospective study and included 239 preterm infants (born before 37 weeks of gestation) who were treated in Neonatal Intensive Care Unit (NICU) in Institute for Child and Youth Health Care of Vojvodina during one year (January 1st, 2012 to December 31st, 2012). The retrospective part of the study focused on examination of incidence of neonatal sepsis and determination of risk factors. In the prospective part of the study infants were sub-divided into two groups: Group 1- infants hospitalized in NICU during the first 6 months of the study; blood cultures were taken by the "clean technique" and checklists for this procedure were not taken. Group 2- neonates hospitalized in NICU during last 6 months of the study; blood cultures were taken by "sterile technique" and checklists for this procedure were taken. RESULTS: The main risk factors for sepsis were prelabor rupture of membranes, low gestational age, low birth weight, mechanical ventilation, umbilical venous catheter placement, and abdominal drainage. Staphylococcus aureus and coagulase negative Staphylococcus were the most frequently isolated microorganisms in false-positive blood samples. CONCLUSIONS: Education of employees, use of checklists and sterile sets for blood sampling, permanent control of false positive blood cultures, as well as regular and routine monthly reports are crucial for successful reduction of contamination rates.

Charge order and its connection with Fermi-liquid charge transport in a pristine high-T(c) cuprate.

Electronic inhomogeneity appears to be an inherent characteristic of the enigmatic cuprate superconductors. Here we report the observation of charge-density-wave correlations in the model cuprate superconductor HgBa2CuO(4+δ) (T(c)=72 K) via bulk Cu L3-edge-resonant X-ray scattering. At the measured hole-doping level, both the short-range charge modulations and Fermi-liquid transport appear below the same temperature of about 200 K. Our result points to a unifying picture in which these two phenomena are preceded at the higher pseudogap temperature by q=0 magnetic order and the build-up of significant dynamic antiferromagnetic correlations. The magnitude of the charge modulation wave vector is consistent with the size of the electron pocket implied by quantum oscillation and Hall effect measurements for HgBa2CuO(4+δ) and with corresponding results for YBa2Cu3O(6+δ), which indicates that charge-density-wave correlations are universally responsible for the low-temperature quantum oscillation phenomenon.

In-plane magnetoresistance obeys Kohler's rule in the pseudogap phase of cuprate superconductors.

We report in-plane resistivity (ρ) and transverse magnetoresistance (MR) measurements for underdoped HgBa(2)CuO(4+δ) (Hg1201). Contrary to the long-standing view that Kohler's rule is strongly violated in underdoped cuprates, we find that it is in fact satisfied in the pseudogap phase of Hg1201. The transverse MR shows a quadratic field dependence, δρ/ρ(0)=aH(2), with a(T)∝T(-4). In combination with the observed ρ∝T(2) dependence, this is consistent with a single Fermi-liquid quasiparticle scattering rate. We show that this behavior is typically masked in cuprates with lower structural symmetry or strong disorder effects.

Doping-dependent photon scattering resonance in the model high-temperature superconductor HgBa2CuO4+δ revealed by Raman scattering and optical ellipsometry.

We study the model high-temperature superconductor HgBa(2)CuO(4+δ) with electronic Raman scattering and optical ellipsometry over a wide doping range. The dependence of the resonant Raman cross section on the incident photon energy changes drastically as a function of doping, in a manner that corresponds to a rearrangement of the interband optical transitions seen with ellipsometry. This doping-dependent Raman resonance allows us to reconcile the apparent discrepancy between Raman and x-ray detection of magnetic fluctuations in superconducting cuprates. Intriguingly, the strongest variation occurs across the doping level where the antinodal superconducting gap appears to reach its maximum.

Feedback effect on high-energy magnetic fluctuations in the model high-temperature superconductor HgBa2CuO(4+δ) observed by electronic Raman scattering.

We use electronic Raman scattering to study the model single-layer cuprate superconductor HgBa(2)CuO(4+δ). In an overdoped sample, we observe a pronounced amplitude enhancement of a high-energy peak related to two-magnon excitations in insulating cuprates upon cooling below the critical temperature T(c). This effect is accompanied by the appearance of the superconducting gap and a pairing peak above the gap in the Raman spectrum, and it can be understood as a hitherto-undetected feedback effect on the high-energy magnetic fluctuations due to the Cooper pairing interaction. This implies a direct involvement of the high-energy magnetic fluctuations in the pairing mechanism. All of these effects occur already above T(c) in two underdoped samples, demonstrating a related feedback mechanism associated with the pseudogap.

Hidden magnetic excitation in the pseudogap phase of a high-T(c) superconductor.

The elucidation of the pseudogap phenomenon of the high-transition-temperature (high-T(c)) copper oxides-a set of anomalous physical properties below the characteristic temperature T* and above T(c)-has been a major challenge in condensed matter physics for the past two decades. Following initial indications of broken time-reversal symmetry in photoemission experiments, recent polarized neutron diffraction work demonstrated the universal existence of an unusual magnetic order below T* (refs 3, 4). These findings have the profound implication that the pseudogap regime constitutes a genuine new phase of matter rather than a mere crossover phenomenon. They are furthermore consistent with a particular type of order involving circulating orbital currents, and with the notion that the phase diagram is controlled by a quantum critical point. Here we report inelastic neutron scattering results for HgBa(2)CuO(4+δ) that reveal a fundamental collective magnetic mode associated with the unusual order, and which further support this picture. The mode's intensity rises below the same temperature T* and its dispersion is weak, as expected for an Ising-like order parameter. Its energy of 52-56 meV renders it a new candidate for the hitherto unexplained ubiquitous electron-boson coupling features observed in spectroscopic studies.

Enhancement of the critical temperature of HgBa2CuO(4+δ) by applying uniaxial and hydrostatic pressure: implications for a universal trend in cuprate superconductors.

It is well known that the superconducting transition temperature (T(c)) of cuprate superconductors can be enhanced by varying certain structural and electronic parameters, such as the flatness of the CuO2 planes or their doping level. We determine the uniaxial and hydrostatic pressure derivatives of T(c) in the structurally simple tetragonal compound HgBa2CuO(4+δ) near optimal doping. Our results provide experimental evidence for two further methods to enhance T(c): (i) reducing the area of the CuO2 planes, and (ii) increasing the separation of the CuO2 planar groups. T(c) is found to couple much more strongly to the ratio c/a of the lattice constants than to the unit cell volume. A comparison with prior results for structurally more complicated cuprates reveals a general trend of uniaxial pressure derivatives with T(c).

Ephedrine therapy in eight patients with congenital myasthenic syndrome due to DOK7 mutations.

In congenital myasthenic syndrome with DOK7 mutations ephedrine was reported to be beneficial in single patients. We carried out a small, open and prospective cohort study in eight European patients manifesting from birth to 12 years. Five patients showed limb-girdle and facial weakness, three a floppy infant syndrome with bulbar symptoms and/or respiratory distress. Ephedrine was started with 25 mg/day and slowly increased to 75-100 mg/day. Within weeks after starting therapy an improvement was observed in all patients and clinical follow-up disclosed positive effects more pronounced on proximal muscle weakness and strength using MRC scale. Effects on facial weakness were less pronounced. Vital capacity measurements and repetitive stimulation tests did not improve in the same way as clinical symptoms did. These investigations are appropriate to confirm the diagnosis in case of pathological results, but they might not be appropriate means to monitor patients under ephedrine therapy.