Improved filters for angular filter refractometry.

Angular filter refractometry is an optical diagnostic that measures the absolute contours of a line-integrated density gradient by placing a filter with alternating opaque and transparent zones in the focal plane of a probe beam, which produce corresponding alternating light and dark regions in the image plane. Identifying transitions between these regions with specific zones on the angular filter (AF) allows the line-integrated density to be determined, but the sign of the density gradient at each transition is degenerate and must be broken using other information about the object plasma. Additional features from diffraction in the filter plane often complicate data analysis. In this paper, we present an improved AF design that uses a stochastic pixel pattern with a sinusoidal radial profile to minimize unwanted diffraction effects in the image caused by the sharp edges of the filter bands. We also present a technique in which a pair of AFs with different patterns on two branches of the same probe beam can be used to break the density gradient degeneracy. Both techniques are demonstrated using a synthetic diagnostic and data collected on the OMEGA EP (extended performance) laser.

Measurement of Magnetic Cavitation Driven by Heat Flow in a Plasma.

We describe the direct measurement of the expulsion of a magnetic field from a plasma driven by heat flow. Using a laser to heat a column of gas within an applied magnetic field, we isolate Nernst advection and show how it changes the field over a nanosecond timescale. Reconstruction of the magnetic field map from proton radiographs demonstrates that the field is advected by heat flow in advance of the plasma expansion with a velocity v_{N}=(6±2)×10^{5} m/s. Kinetic and extended magnetohydrodynamic simulations agree well in this regime due to the buildup of a magnetic transport barrier.

Nonlinear ablative Rayleigh-Taylor instability: Increased growth due to self-generated magnetic fields.

The growth rate of the nonlinear ablative Rayleigh-Taylor (RT) instability is enhanced by magnetic fields self-generated by the Biermann battery mechanism; a scaling for this effect with perturbation height and wavelength is proposed and validated with extended-magnetohydrodynamic simulations. The magnetic flux generation rate around a single RT spike is found to scale with the spike height. The Hall parameter, which quantifies electron magnetization, is found to be strongly enhanced for short-wavelength spikes due to Nernst compression of the magnetic field at the spike tip. The impact of the magnetic field on spike growth is through both the suppressed thermal conduction into the unstable spike and the Righi-Leduc heat flow deflecting heat from the spike tip to the base. Righi-Leduc is found to be the dominant effect for small Hall parameters, while suppressed thermal conduction dominates for large Hall parameters. These results demonstrate the importance of considering magnetic fields in all perturbed inertial confinement fusion hot spots.

X-ray imaging and radiation transport effects on cylindrical implosions.

Magnetization of inertial confinement implosions is a promising means of improving their performance, owing to the potential reduction of energy losses within the target and mitigation of hydrodynamic instabilities. In particular, cylindrical implosions are useful for studying the influence of a magnetic field, thanks to their axial symmetry. Here, we present experimental results from cylindrical implosions on the OMEGA-60 laser using a 40-beam, 14.5 kJ, 1.5 ns drive and an initial seed magnetic field of B0 = 30 T along the axes of the targets, compared with reference results without an imposed B-field. Implosions were characterized using time-resolved x-ray imaging from two orthogonal lines of sight. We found that the data agree well with magnetohydrodynamic simulations, once radiation transport within the imploding plasma is considered. We show that for a correct interpretation of the data in these types of experiments, explicit radiation transport must be taken into account.

Increased Ion Temperature and Neutron Yield Observed in Magnetized Indirectly Driven D_{2}-Filled Capsule Implosions on the National Ignition Facility.

The application of an external 26 Tesla axial magnetic field to a D_{2} gas-filled capsule indirectly driven on the National Ignition Facility is observed to increase the ion temperature by 40% and the neutron yield by a factor of 3.2 in a hot spot with areal density and temperature approaching what is required for fusion ignition [1]. The improvements are determined from energy spectral measurements of the 2.45 MeV neutrons from the D(d,n)^{3}He reaction, and the compressed central core B field is estimated to be ∼4.9 kT using the 14.1 MeV secondary neutrons from the D(T,n)^{4}He reactions. The experiments use a 30 kV pulsed-power system to deliver a ∼3 µs current pulse to a solenoidal coil wrapped around a novel high-electrical-resistivity AuTa_{4} hohlraum. Radiation magnetohydrodynamic simulations are consistent with the experiment.

Cylindrical implosion platform for the study of highly magnetized plasmas at Laser MegaJoule.

Investigating the potential benefits of the use of magnetic fields in inertial confinement fusion experiments has given rise to experimental platforms like the Magnetized Liner Inertial Fusion approach at the Z-machine (Sandia National Laboratories) or its laser-driven equivalent at OMEGA (Laboratory for Laser Energetics). Implementing these platforms at MegaJoule-scale laser facilities, such as the Laser MegaJoule (LMJ) or the National Ignition Facility (NIF), is crucial to reaching self-sustained nuclear fusion and enlarges the level of magnetization that can be achieved through a higher compression. In this paper, we present a complete design of an experimental platform for magnetized implosions using cylindrical targets at LMJ. A seed magnetic field is generated along the axis of the cylinder using laser-driven coil targets, minimizing debris and increasing diagnostic access compared with pulsed power field generators. We present a comprehensive simulation study of the initial B field generated with these coil targets, as well as two-dimensional extended magnetohydrodynamics simulations showing that a 5 T initial B field is compressed up to 25 kT during the implosion. Under these circumstances, the electrons become magnetized, which severely modifies the plasma conditions at stagnation. In particular, in the hot spot the electron temperature is increased (from 1 keV to 5 keV) while the density is reduced (from 40g/cm^{3} to 7g/cm^{3}). We discuss how these changes can be diagnosed using x-ray imaging and spectroscopy, and particle diagnostics. We propose the simultaneous use of two dopants in the fuel (Ar and Kr) to act as spectroscopic tracers. We show that this introduces an effective spatial resolution in the plasma which permits an unambiguous observation of the B-field effects. Additionally, we present a plan for future experiments of this kind at LMJ.

Effect of Strongly Magnetized Electrons and Ions on Heat Flow and Symmetry of Inertial Fusion Implosions.

This Letter presents the first observation on how a strong, 500 kG, externally applied B field increases the mode-two asymmetry in shock-heated inertial fusion implosions. Using a direct-drive implosion with polar illumination and imposed field, we observed that magnetization produces a significant increase in the implosion oblateness (a 2.5× larger P2 amplitude in x-ray self-emission images) compared with reference experiments with identical drive but with no field applied. The implosions produce strongly magnetized electrons (ω_{e}τ_{e}≫1) and ions (ω_{i}τ_{i}>1) that, as shown using simulations, restrict the cross field heat flow necessary for lateral distribution of the laser and shock heating from the implosion pole to the waist, causing the enhanced mode-two shape.

Magnetized ablative Rayleigh-Taylor instability in three dimensions.

Three-dimensional extended-magnetohydrodynamics simulations of the magnetized ablative Rayleigh-Taylor instability are presented. Previous two-dimensional (2D) simulations claiming perturbation suppression by magnetic tension are shown to be misleading, as they do not include the most unstable dimension. For perturbation modes along the applied field direction, the magnetic field simultaneously reduces ablative stabilization and adds magnetic tension stabilization; the stabilizing term is found to dominate for applied fields > 5 T, with both effects increasing in importance at short wavelengths. For modes perpendicular to the applied field, magnetic tension does not directly stabilize the perturbation but can result in moderately slower growth due to the perturbation appearing to be 2D (albeit in a different orientation to 2D inertial confinement fusion simulations). In cases where thermal ablative stabilization is dominant the applied field increases the peak bubble-spike height. Resistive diffusion is shown to be important for short wavelengths and long timescales, reducing the effectiveness of tension stabilization.

Diagnosing plasma magnetization in inertial confinement fusion implosions using secondary deuterium-tritium reactions.

Diagnosing plasma magnetization in inertial confinement fusion implosions is important for understanding how magnetic fields affect implosion dynamics and to assess plasma conditions in magnetized implosion experiments. Secondary deuterium-tritium (DT) reactions provide two diagnostic signatures to infer neutron-averaged magnetization. Magnetically confining fusion tritons from deuterium-deuterium (DD) reactions in the hot spot increases their path lengths and energy loss, leading to an increase in the secondary DT reaction yield. In addition, the distribution of magnetically confined DD-triton is anisotropic, and this drives anisotropy in the secondary DT neutron spectra along different lines of sight. Implosion parameter space as well as sensitivity to the applied B-field, fuel ρR, temperature, and hot-spot shape will be examined using Monte Carlo and 2D radiation-magnetohydrodynamic simulations.

Magnetic Signatures of Radiation-Driven Double Ablation Fronts.

In experiments performed with the OMEGA EP laser system, magnetic field generation in double ablation fronts was observed. Proton radiography measured the strength, spatial profile, and temporal dynamics of self-generated magnetic fields as the target material was varied between plastic, aluminum, copper, and gold. Two distinct regions of magnetic field are generated in mid-Z targets-one produced by gradients from electron thermal transport and the second from radiation-driven gradients. Extended magnetohydrodynamic simulations including radiation transport reproduced key aspects of the experiment, including field generation and double ablation front formation.

Expanding the clinical spectrum of biallelic ZNF335 variants.

ZNF335 plays an essential role in neurogenesis and biallelic variants in ZNF335 have been identified as the cause of severe primary autosomal recessive microcephaly in 2 unrelated families. We describe, herein, 2 additional affected individuals with biallelic ZNF335 variants, 1 individual with a homozygous c.1399 T > C, p.(Cys467Arg) variant, and a second individual with compound heterozygous c.2171_2173delTCT, p.(Phe724del) and c.3998A > G, p.(Glu1333Gly) variants with the latter variant predicted to affect splicing. Whereas the first case presented with early death and a severe phenotype characterized by anterior agyria with prominent extra-axial spaces, absent basal ganglia, and hypoplasia of the brainstem and cerebellum, the second case had a milder clinical presentation with hypomyelination and otherwise preserved brain structures on MRI. Our findings expand the clinical spectrum of ZNF335-associated microcephaly.

Self-Generated Magnetic Fields in the Stagnation Phase of Indirect-Drive Implosions on the National Ignition Facility.

Three-dimensional extended-magnetohydrodynamic simulations of the stagnation phase of inertial confinement fusion implosion experiments at the National Ignition Facility are presented, showing self-generated magnetic fields over 10^{4} T. Angular high mode-number perturbations develop large magnetic fields, but are localized to the cold, dense hot-spot surface, which is hard to magnetize. When low-mode perturbations are also present, the magnetic fields are injected into the hot core, reaching significant magnetizations, with peak local thermal conductivity reductions greater than 90%. However, Righi-Leduc heat transport effectively cools the hot spot and lowers the neutron spectra-inferred ion temperatures compared to the unmagnetized case. The Nernst effect qualitatively changes the results by demagnetizing the hot-spot core, while increasing magnetizations at the edge and near regions of large heat loss.

Disorders of Microtubule Function in Neurons: Imaging Correlates.

BACKGROUND AND PURPOSE: A number of recent studies have described malformations of cortical development with mutations of components of microtubules and microtubule-associated proteins. Despite examinations of a large number of MRIs, good phenotype-genotype correlations have been elusive. Additionally, most of these studies focused exclusively on cerebral cortical findings. The purpose of this study was to characterize imaging findings associated with disorders of microtubule function. MATERIALS AND METHODS: MRIs from 18 patients with confirmed tubulin mutations (8 TUBA1A, 5 TUBB2B, and 5 TUBB3) and 15 patients with known mutations of the genes encoding microtubule-associated proteins (5 LIS1, 4 DCX, and 6 DYNC1H1) were carefully visually analyzed and compared. Specific note was made of the cortical gyral pattern, basal ganglia, and white matter to assess internal capsular size, cortical thickness, ventricular and cisternal size, and the size and contours of the brain stem, cerebellar hemispheres and vermis, and the corpus callosum of patients with tubulin and microtubule-associated protein gene mutations. Results were determined by unanimous consensus of the authors. RESULTS: All patients had abnormal findings on MR imaging. A large number of patients with tubulin gene mutations were found to have multiple cortical and subcortical abnormalities, including microcephaly, ventriculomegaly, abnormal gyral and sulcal patterns (termed "dysgyria"), a small or absent corpus callosum, and a small pons. All patients with microtubule-associated protein mutations also had abnormal cerebral cortices (predominantly pachygyria and agyria), but fewer subcortical abnormalities were noted. CONCLUSIONS: Comparison of MRIs from patients with known mutations of tubulin genes and microtubule-associated proteins allows the establishment of some early correlations of phenotype with genotype and may assist in identification and diagnosis of these rare disorders.

Mode of delivery in pregnancies complicated by major fetal congenital heart disease: a retrospective cohort study.

OBJECTIVE: To determine the mode of delivery in pregnancies complicated by complex fetal congenital heart disease (CHD). STUDY DESIGN: Five-year retrospective cohort study at a tertiary fetal medicine center (2007 to 2011). Cases of complex fetal CHD (n=126) were compared with 45 069 non-anomalous singleton infants ⩾500 g to determine rates of emergency intrapartum cesarean section (CS), preterm delivery and induction of labor. RESULT: Intrapartum CS is significantly higher in fetal CHD than non-anomalous controls (21% vs 13.5%, odds ratio (OR) 1.7, 95% confidence interval (CI): 1.0 to 2.7; P=0.035), predominantly related to CS for non-reassuring fetal status (OR 2.2, 95% CI: 1.1 to 4.1; P=0.022). Although fetal CHD did not increase emergency CS rates in nulliparous women, CS was significantly increased in multiparous pregnancies (OR 2.4, 95% CI: 1.8 to 4.6; P=0.014). Rates of preterm delivery (OR 3.4, 95% CI: 2.0 to 5.4; P<0.0001) and induction of labor (OR 1.9, 95% CI: 1.3 to 2.9; P=0.001) were higher in the CHD cases. CONCLUSION: Emergency CS is increased in fetal CHD, attributed to a higher rate of CS for non-reassuring fetal status and seen mostly in multiparous women.

Reassessing critical maternal antibody threshold in RhD alloimmunization: a 16-year retrospective cohort study.

OBJECTIVE: To determine the critical maternal antibody threshold for specialist referral in cases of RhD alloimmunization. METHODS: This was a retrospective cohort study, covering a 16-year period at the national tertiary fetal medicine center for management of alloimmunization. Data concerning RhD alloimmunized pregnant women were extracted from an institutional database and maternal anti-D antibody levels were cross-checked with the national reference laboratory. Fetal hemoglobin (Hb) levels were determined only at the first intrauterine transfusion (IUT) and were compared with the pretransfusion maternal anti-D antibody level (IU/mL). Sensitivity, specificity and positive and negative predictive values of maternal antibody thresholds for detecting moderate to severe (Hb ≤ 0.64MoM) fetal anemia were calculated. RESULTS: Between 1996 and 2011, 66 women underwent a first IUT for RhD alloimmunization at our institution. The highest serum anti-D antibody level was extracted for 208 RhD alloimmunized women who did not require IUT during the last 10 years of the study period. The traditional maternal antibody threshold of > 15 IU/mL failed to detect 20% of cases of moderate to severe fetal anemia. The ≥ 4 IU/mL threshold had 100% sensitivity but a 45% false-positive rate. The optimal anti-D antibody threshold for specialist referral in our population was ≥ 6 IU/mL; at this level, no case of moderate to severe anemia was missed and specificity was 61%. Use of this threshold would have eliminated 10% of referrals to our fetal medicine unit without compromising fetal outcomes. CONCLUSIONS: Setting the critical maternal RhD antibody level at >15 IU/mL does not provide sufficient sensitivity. The lower threshold of ≥ 4 IU/mL, though sensitive, is associated with a 45% false-positive rate. In our population, a threshold of ≥ 6 IU/mL minimizes false-positive referrals while maintaining 100% sensitivity for moderate to severe fetal anemia.

Routine obstetric ultrasound services.

Antenatal ultrasonography is widely used in pregnancy to assess fetal growth, wellbeing and anatomy. Although ultrasound screening is now an integral part of routine antenatal care, recommendations for the delivery of obstetric ultrasound vary from country to country. A recent survey of English maternity units reported that 100% of women are offered routine mid-trimester fetal anomaly scanning, in line with expert recommendations. Currently in Ireland, no national guidance exists to inform practitioners on the minimum standards for obstetric ultrasound practice. In 2012, we conducted a structured telephone survey of all 20 maternity units in Ireland (n = 74,377 births). Routine mid-trimester anomaly scanning was offered universally to all women in 7/20 (35%) units, offered selectively to some women in 9/20 (45%) units and not offered to any women in 4/20 (20%) of units. The time allotted for a complete fetal anatomical survey was 10-15 minutes in 4/16 (25%) units, 20 minutes in 7/16 (44%) units and 25-30 minutes in 5/16 (31%) units. Written guidelines on the appropriate management of "soft markers" for fetal aneuploidy were routinely used in 11/16 (69%) of units. In no Irish unit currently, are images from fetal anomaly scanning routinely reviewed by an Obstetrician with an interest in fetal medicine. 19/20 (95%) of respondents believed that a minimum of 2 scans should be offered in routine uncomplicated pregnancies. Improvements in the availability of obstetric ultrasound to pregnant women in Ireland will require increased staffing numbers at both the ultrasonographer and fetal specialist levels. There is a clear need for national guidelines on the provision of routine obstetric ultrasound in Ireland.

Potential role of arthroscopy in the management of inflammatory arthritis.

Despite its advantages in diagnosis, treatment and research, the role of arthroscopy in the management of rheumatic diseases has diminished due to the development of other less invasive means of joint assessment including advances in imaging techniques, e.g. ultrasound and magnetic resonance imaging. However, arthroscopy still provides invaluable information. By direct and precise internal inspection of a joint, arthroscopy allows the collection of synovial membrane samples (biopsies) of excellent quality, notably from the most representative pathological areas. Arthroscopy may also play a therapeutic role in the management of inflammatory arthritis (IA) by providing pain relief (lavage). Here we describe the procedure of knee arthroscopy under local anaesthesia, as well as an in situ visual assessment of synovial inflammation and its correlation with degree of histological and immunological abnormalities. With the emphasis being placed on early diagnosis and treatment initiation in patients with IA and as earlier initiation of targeted biologic therapies becomes more commonplace, the ability to predict which patients will respond to the different therapies available would be invaluable. Assessment of arthroscopic derived synovial biopsies has potential to play an important role in management of early IA in the future.

Recurrent twin-twin transfusion syndrome after selective fetoscopic laser photocoagulation: a systematic review of the literature.

OBJECTIVES: Selective fetoscopic laser photocoagulation (SFLP) is now the treatment of choice for twin-twin transfusion syndrome (TTTS). The incidence of recurrent TTTS following SFLP has been inconsistently reported across different studies. We performed a systematic review of TTTS recurrence following SFLP. METHODS: PubMed and MEDLINE online databases were searched for articles published between 2000 and August 2011, using combinations of the terms 'twin-twin transfusion', 'TTTS', 'laser', 'recur' and 'outcome'. Citations identified in the primary search were screened for eligibility. Studies reporting outcomes from selective SFLP for TTTS in twin pregnancies, which addressed specifically the issue of TTTS recurrence, were included. The primary outcome was rate of TTTS recurrence. Secondary outcomes were therapeutic preference and fetal outcomes in cases of recurrent TTTS. RESULTS: The primary search identified 22 eligible studies that are included in this review (n = 2447 twin pregnancies). Two studies included a minority of non-selective procedures. The published incidence of recurrent TTTS ranged from 0 to 16%. Clinical management was reported in 65.7% (71/108) cases, with repeat SFLP the most commonly performed secondary intervention. Only three studies provided comprehensive outcome data for cases of recurrent TTTS. The overall rate of neurologically-intact survival was 44% (23/52). The data were inadequate to determine the effects of secondary therapeutic approach, placental location or gestational age on perinatal outcome in cases of recurrent TTTS. CONCLUSIONS: The published rate of TTTS recurrence following SFLP in monochorionic twin pregnancies ranges from 0 to 16%. Although limited follow-up data suggest that recurrence is associated with significant perinatal mortality and morbidity, further study is needed. Currently, there are insufficient data available to guide recommendations for clinical management of TTTS recurrence. Future studies on SFLP for TTTS must include details on recurrence rates and provide outcome data specific to the recurrent subset.