[The advantages of 3D imagery in diagnosing and supervising children's and teenagers' scoliosis]. / Intérêt de l'imagerie 3D dans le diagnostic et la surveillance des scolioses de l'enfant et de l'adolescent.

Scoliosis is an abnormal curvature of the spine. One or several curves of more than 10 degrees in the frontal plane can be seen with the rotation of vertebrae in the axial plane, which modifies sagittal curves. In addition to esthetic harm, the morbidity of a scoliosis depends on the extent of the deformation. Treatment, whether it be orthopedic or surgical, is aggressive and never completely cures the condition. At best the deformation will be stabilized at the end of growth. Therefore, it is essential to detect any slight curve and quickly identify any potential progressive form in order to treat it. Visualization of scoliosis in 3D through spine modeling has several advantages at each stage of care. First, with slight curvatures, 3D modeling allows the medical staff to confirm the scoliosis by showing the modification in the three different planes. All curvatures will not progress. Orthopedic treatment is constraining and expensive; only progressive forms will receive it. When the curvature is slight and does not need immediate treatment, 3D modeling at each successive check-up will help detect any sign of likely progression quickly and reliably. Moreover, the medical observation of corset treatment and the preoperative work-up are improved because all 3D parameters of the deformation are accessible. The need for 3D modeling for scoliosis has been known for a long time, but no tool allowing a vertical study with a low level of radiation was available. The EOS imagery system meets these criteria through an optimal analysis of deformations caused by scoliosis.

Reproducibility and repeatability of a new computerized software for sagittal spinopelvic and scoliosis curvature radiologic measurements: Keops(®).

PURPOSE: The purpose of this study was to evaluate the inter- and intra-observer variability of the computerized radiologic measurements using Keops(®) and to determine the bias between the software and the standard paper measurement. METHODS: Four individuals measured all frontal and sagittal variables on the 30 X-rays randomly selected on two occasions (test and retest conditions). The Bland-Altman plot was used to determine the degree of agreement between the measurement on paper X-ray and the measurement using Keops(®) for all reviewers and for the two measures; the intraclass correlation coefficient (ICC) was calculated for each pair of analyses to assess interobserver reproducibility among the four reviewers for the same patient using either paper X-ray or Keops(®) measurement and finally, concordance correlation coefficient (rc) was calculated to assess intraobserver repeatability among the same reviewer for one patient between the two measure using the same method (paper or Keops(®)). RESULTS: The mean difference calculated between the two methods was minimal at -0, 4° ± 3.41° [-7.1; 6.4] for frontal measurement and 0.1° ± 3.52° [-6.7; 6.8] for sagittal measurement. Keops(®) has a better interobserver reproducibility than paper measurement for determination of the sagittal pelvic parameter (ICC = 0.9960 vs. 0.9931; p = 0.0001). It has a better intraobserver repeatability than paper for determination of Cobbs angle (rc = 0.9872 vs. 0.9808; p < 0.0001) and for pelvic parameter (rc = 0.9981 vs. 0.9953; p < 0.0001). CONCLUSIONS: We conclude that Keops(®) has no bias compared to the traditionally paper measurement, and moreover, the repeatability and the reproducibility of measurements with this method is much better than with similar standard radiologic measures done manually in both frontal and sagittal plane and that the use of this software can be recommended for clinical application. LEVEL OF EVIDENCE: Diagnostic, level III.

Dispersive crystal field excitations and quadrupolar interactions in UPd3.

We report inelastic neutron scattering measurements and random phase approximation calculations of the dispersive crystal field excitations of UPd(3). The measured spectra at lower energies agree with those calculated using quadrupolar interaction parameters deduced from bulk and x-ray scattering measurements. The more intense excitations arising from the hexagonal sites were used to obtain exchange parameters which proved to be anisotropic.

Elicited repetitive daily blindness: a new phenotype associated with hemiplegic migraine and SCN1A mutations.

OBJECTIVE: Familial hemiplegic migraine (FHM) is a genetically heterogeneous disorder in which three genes, CACNA1A, ATP1A2, and SCN1A, are currently known to be involved. FHM is occasionally associated with other neurologic symptoms such as cerebellar ataxia or epileptic seizures. A unique eye phenotype of elicited repetitive daily blindness (ERDB) has also been reported to be cosegregating with FHM in a single Swiss family. METHODS: We report an additional family in whom the proband had, in addition to FHM, typical ERDB. In this family and the previously reported Swiss family, the whole coding region of the SCN1A gene was screened after exclusion of mutation in CACNA1A and ATP1A2 genes. RESULTS: We identified two novel SCN1A mutations (c.4495T>C/p.Phe1499Leu and c.4467G>C/p.Gln1489His missense substitutions) in exons 24 and 23, respectively, segregating with the disease in all living affected members. Both mutations were absent from 180 healthy Caucasian controls and were located in an intracellular loop highly conserved throughout evolution. CONCLUSION: We report new clinical data supporting cosegregation of familial hemiplegic migraine and the new eye phenotype of elicited repetitive daily blindness and two novel SCN1A mutations as the underlying genetic defect in two unrelated families. SCN1A encodes the voltage-gated sodium channel Nav1.1 that is highly expressed in the CNS including the retina. This remarkably stereotyped new eye phenotype has clinical characteristics of abnormal propagation of the retinal electrical signal that may be a retinal spreading depression. These results suggest that SCN1A mutations, which alter neuronal brain excitability, may occasionally alter retinal cell excitability.

Morphology of the superconducting vortex lattice in ultrapure niobium.

The morphology of the superconducting flux line lattice (FLL) of Nb comprises gradual variations with various lock-in transitions and symmetry breaking rotations. We report a comprehensive small-angle neutron scattering study of the FLL in an ultrapure single crystal of Nb as a function of the orientation of the applied magnetic field. We attribute the general morphology of the FLL and its orientation to three dominant mechanisms; first, nonlocal contributions, second, the transition between open and closed Fermi surface sheets and, third, the intermediate mixed state between the Meissner and the Shubnikov phase.

Magnetoelastic nature of the dodecagonal anisotropy in holmium metal.

We have investigated the magnetoelastic nature of the dodecagonal anisotropy in the magnetic anisotropy energy (MAE) in the basal plane of the hcp crystalline structure in holmium single crystal. We have proved that the origin of the second harmonic of the hexagonal symmetry in MAE clearly lies on a sixth-order magnetoelastic coupling term. The appearance of a 12-fold anisotropy in MAE in a single crystal having hexagonal symmetry provides a new insight on how the magnetic anisotropy can be modified in a magnetic material with giant spin-lattice coupling.

Dipolar excitations at the LIII x-ray absorption edges of the heavy rare-earth metals.

We report measured dipolar asymmetry ratios at the LIII edges of the heavy rare-earth metals. The results are compared with a first-principles calculation and excellent agreement is found. A simple model of the scattering is developed, enabling us to reinterpret the resonant x-ray scattering in these materials and to identify the peaks in the asymmetry ratios with features in the spin and orbital moment densities.

Determination of the antiferroquadrupolar order parameters in UPd(3).

By combining accurate heat capacity and x-ray resonant scattering results we have resolved the long standing question regarding the nature of the quadrupolar ordered phases in UPd(3). The order parameter of the highest temperature quadrupolar phase has been uniquely determined to be antiphase Q{zx} in contrast to the previous conjecture of Q{x{2}-y{2}}. The azimuthal dependence of the x-ray scattering intensity from the quadrupolar superlattice reflections indicates that the lower temperature phases are described by a superposition of order parameters. The heat capacity features associated with each of the phase transitions characterize their order, which imposes restrictions on the matrix elements of the quadrupolar operators.

Spontaneous symmetry-breaking vortex lattice transitions in pure niobium.

We report an extensive investigation of magnetic vortex lattice (VL) structures in single crystals of pure niobium with the magnetic field applied parallel to a fourfold symmetry axis, so as to induce frustration between the cubic crystal symmetry and hexagonal VL coordination expected in an isotropic situation. We observe new VL structures and phase transitions; all the VL phases observed (including those with an exactly square unit cell) spontaneously break some crystal symmetry. One phase even has the lowest possible symmetry of a two-dimensional Bravais lattice. This is quite unlike the situation in high-Tc or borocarbide superconductors, where VL structures orient along particular directions of high crystal symmetry. The causes of this behavior are discussed.

Observation of a strongly nested Fermi surface in the shape-memory alloy Ni0.62Al0.38.

The Fermi surface topology of the shape-memory alloy Ni0.62Al0.38 has been determined using Compton scattering. A large area of this Fermi surface can be made to nest with other areas by translation through a vector of approximately 0.18[1,1,0](2pi/a), which corresponds to the wave vector associated with martensitic precursor phenomena such as phonon softening and diffuse streaking in electron diffraction patterns. This observation is compelling evidence that these phenomena are driven by the enhanced electron-lattice coupling due to the Fermi surface nesting.

Elicited repetitive daily blindness: a new familial disorder related to migraine and epilepsy.

The authors report a family affected by multiple daily episodes of transient visual loss, elicited repetitive daily blindness (ERDB); the onset was early in life, and the disease followed a benign course. ERDB is associated with childhood epilepsy and familial hemiplegic migraine, apparently segregating as a monogenic, autosomal dominant condition with variable expression. Genetic linkage to CACNA1A was excluded.

Intrinsic behavior of flux lines in pure niobium near the upper critical field.

We report small-angle neutron-scattering (SANS) measurements of flux line properties near H(c2) in an ultrapure sample of niobium with weak pinning of flux in the bulk. These confirm in detail the Abrikosov picture of the flux line lattice to within 20 mK of the upper critical field line. However, it has recently been claimed [X. S. Ling et al., Phys. Rev. Lett. 86, 712 (2001)], on the basis of SANS observations of a disordering of flux lines in niobium, that the flux lattice melts at temperatures clearly separated from the upper critical field line. This discrepancy may possibly arise from differences in sample purity and pinning.

Effects of pond water, sediment and sediment extract samples from New Hampshire, USA on early Xenopus development and metamorphosis: comparison to native species.

In an effort to assess potential ecological hazards to amphibian species in selected regions within New Hampshire, the traditional Frog Embryo Teratogenesis Assay-Xenopus (FETAX), a 14-/21 day tail resorption thyroid disruption assay and >30 day limb development tests were conducted with representative surface water and sediment samples. Two separate sets of samples collected from five sites were evaluated. The primary objectives of the study were to determine if samples were capable of inducing early embryo-larval maldevelopment, to determine if maldevelopment included limb defects, to determine if thyroxine co-administration altered the rates of limb malformation and to evaluate the impact of the samples on growth rates, developmental progress and metamorphic climax. Results from these studies suggested that pond water and sediment extract samples, but not whole sediment samples, from B2, FW, LP and W ponds were capable of inducing abnormal early embryo-larval development. In addition, water samples from B2 and W ponds induced significant abnormal hindlimb development. Some abnormal forelimb development was noted in the tail resorption studies, but not to the same extent as the hindlimbs. Each of the water samples induced appreciable developmental delay, including the paired reference site B1, which could be reversed by the addition of exogenous thyroxine.

Optimization of an exogenous metabolic activation system for FETAX. I. Post-isolation rat liver microsome mixtures.

The developmental toxicity of cyclophosphamide, coumarin, 2-acetyl-aminofluorine (2-AAF), and trichloroethylene (TCE) was assessed with Frog Embryo Teratogenesis Assay: Xenopus (FETAX). Late Xenopus laevis blastulae were exposed to each test material for 96-h in two separate static-renewal tests with and without the presence of five differently induced exogenous metabolic activation systems (MAS). The MAS consisted of Aroclor 1254- (Aroclor 1254 MAS), isoniazid- (INH MAS), phenobarbital- (PB MAS), or beta-naphthoflavone- (beta-NF MAS), or a post-isolation mixture (mixed MAS) of INH-, PB-, and beta-NF-induced rat liver microsomes. Addition of the Aroclor 1254 MAS bioactivated cyclophosphamide, coumarin, 2-AAF, but not TCE. Addition of the PB MAS bioactivated cyclophosphamide, weakly bioactivated coumarin and 2-AAF, but had no effect on TCE developmental toxicity. The beta-NF MAS bioactivated coumarin and 2-AAF, weakly bioactivated cyclophosphamide, but did not alter the developmental toxicity of TCE. Addition of the INH-induced MAS only bioactivated TCE, whereas the post-isolation mixed MAS bioactivated each test material. Based on LC50 and EC50 (malformation) values, embryo growth, and types and severity of induced malformations, each test material was developmentally toxic. Use of post-microsome isolation mixtures from differentially induced rat livers increased the efficacy of the exogenous MAS routinely used by FETAX.

Optimization of an exogenous metabolic activation system for FETAX. II. Preliminary evaluation.

The developmental toxicities of five test compounds including carbon tetrachloride, urethane, phenacetin, parathion, and chloroform, were evaluated using Frog Embryo Teratogenesis Assay--Xenopus (FETAX), with minor modification. Post-isolation mixtures of differently-induced rat liver microsomes (phenobarbital- (PB), beta-naphthoflavone- (beta-NF), and isoniazid- (INH)-induced preparations) were co-cultured directly with X. laevis embryos. Results from these studies suggest that the Aroclor 1254-induced MAS could effectively be replaced by a mixed lot of PB-, beta-NF-, and INH-induced rat liver microsomes. Each of the test materials were found to be developmentally toxic when bioactivated by the mixed MAS.

Evaluation of a reproductive toxicity assay using Xenopus laevis: boric acid, cadmium and ethylene glycol monomethyl ether.

Cadmium (Cd), boric acid (BA) and ethylene glycol monomethyl ether (EGME) were evaluated for reproductive and developmental toxicity in Xenopus laevis. Eight reproductively mature adult male and eight superovulated female Xenopus laevis were exposed to at least five separate sublethal concentrations of each material via the culture water for a period of 30 days. Four respective pairs were mated and the offspring evaluated for developmental effects; an evaluation of reproductive status was performed on the remaining four specimens. Ovary pathology, oocyte count, oocyte maturity and maturation capacity (germinal vesicle breakdown, GVBD) and necrosis were evaluated in the female, whereas testis pathology, sperm count, dysmorphology and motility were studied in the male. Based on this assessment, each test material exerted reproductive toxicity in Xenopus laevis, but with varying potencies. Adult female exposure to Cd and EGME particularly, and to a lesser extent to BA, resulted in transgenerational toxicity to the developing progeny. Further, this model appears to be a useful tool in the initial assessment and prioritization of potential reproductive toxicants for further testing.

Adverse developmental and reproductive effects of copper deficiency in Xenopus laevis.

The effect of copper (Cu) deficiency on the reproduction and development in Xenopus laevis was evaluated, culminating in the development of a defined concentration-response relationship. Separate groups of four adult frog pairs were fed one of three diets for 28 d: (1) low-copper (-Cu); (2) copper supplemented (+Cu); and (3) ASTM standard beef liver and lung (BLL). Embryos collected from frogs administered the -Cu diet had markedly decreased egg masses and viability rates and an increased rate of necrosis when compared to the other dietary treatments. Malformations in -Cu larvae included maldevelopment of the heart, eye, craniofacial region, brain, and notochord. Larvae from adults administered the -Cu diet showed delayed abnormal hindlimb development, characterized as selective reductive deficiencies distal to the femur, with poor cartilaginous development. A U-shaped dose-response curve characteristic of nutritional essentiality was developed for Cu. Overall, these studies indicated that embryos produced from frogs administered a -Cu diet are substantially less viable than embryos from frogs administered a +Cu or copper-adequate (BLL) diet.

Chronic boron or copper deficiency induces limb teratogenesis in Xenopus.

Sets of adult male and female Xenopus laevis were administered a boron-deficient (-B) diet under low-boron culture conditions, a boron-supplemented (+B) diet under ambient boron culture conditions, a copper-deficient (-Cu) diet under low-copper culture conditions, or a copper-supplemented (+Cu) diet under ambient copper culture conditions, for 120 d. Adults from each group were' subsequently bred, and the progeny were cultured and bred. Results from these studies indicated that although pronounced effects on adult reproduction and early embryo-larval development were noted in the -B F1 generation, no effects on limb development were observed. No significant effects on reproduction, early embryogenesis, or limb development were noted in the +B group, irrespective of generation. Highly specific forelimb and hindlimb defects, including axial flexures resulting in crossed limbs and reduction deficits, were observed in -B F2 larvae, but not in the +B F2 larvae. As was noted in the boron-deficiency studies, significant effects on reproduction and early embryo development were observed in the -Cu F1 generation, but not in the +Cu F, generation. Unlike the effects associated with boron deficiency, maldevelopment of the hindlimbs (32 responders, n = 40) was found in the F1 generation.

Novel antihyperglycemic terpenoid-quinones from Pycnanthus angolensis.

Two new compounds, pycnanthuquinone A (1) and pycnanthuquinone B (2), were isolated from leaves and stems of the African plant, Pycnanthus angolensis (Welw.) Warb (Myristicaceae), by bioassay-guided fractionation of an ethanolic extract using a diabetic mouse model. Pycnanthuquinones A and B are the first representatives of a novel terpenoid-type quinone skeleton, and both compounds possess significant antihyperglycemic activity.