Orthopedic management of the extremities in patients with Morquio A syndrome.

BackgroundMusculoskeletal involvement in Morquio A syndrome (mucopolysaccharidosis IVA; MPS IVA) contributes significantly to morbidity and mortality. While the spinal manifestations of the disorder have received considerable attention in the literature, there have been few reported studies to date to guide the management of the orthopedic problems associated with the lower and upper extremities.PurposeThe objective was to develop recommendations for the management of the extremities in patients with Morquio A syndrome.MethodsA group of specialists in orthopedics, pediatrics and genetics with experience in the management of Morquio A patients convened to review and discuss current clinical practices and to develop preliminary recommendations. Evidence from the literature was retrieved. Recommendations were further refined until consensus was reached.Results and conclusionsThis present article provides a detailed review and discussion of the lower and upper extremity deformities in Morquio A syndrome and presents recommendations for the assessment and treatment of these complications. Key issues, including the importance of early diagnosis and the implications of medical therapy, are also addressed. The recommendations herein represent an attempt to develop a uniform and practical approach to managing patients with Morquio A syndrome and improving their outcomes

Bright Eyes

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Anomalous interaction of nonlocal solitons in media with competing nonlinearities

We theoretically investigate properties of individual bright spatial solitons and their interaction in nonlocal media with competing focusing and defocusing nonlinearities. We consider the general case with both nonlinear responses characterized by different strengths and degrees of nonlocality. We employ a variational approach to analytically describe soliton properties. In particular, we prove analytically that the interplay of focusing and defocusing nonlocal nonlinearities leads to attraction or repulsion of solitons depending on their separation distance. We then study the propagation and interaction of solitons using numerical simulations of the full model of beam propagation. The numerical simulations fully confirm our analytical results

Detection of sub-shot-noise spatial correlation in high-gain parametric down-conversion

Using a 1GW-1ps pump laser pulse in high gain parametric down-conversion allows us to detect sub-shot-noise spatial quantum correlation with up to one hundred photoelectrons per mode, by means of a high efficiency CCD. The statistics is performed in single-shot over independent spatial replica of the system. The paper highlights the evidence of quantum correlation between symmetrical signal and idler spatial areas in the far field, in the high gain regime. In accordance with the predictions of numerical calculations the observed transition from the quantum to the classical regime is interpreted as a consequence of the narrowing of the down-converted beams in the very high gain regime.Comment: 4,2 pages, 4 figure

Modulation Instability of Ultrashort Pulses in Quadratic Nonlinear Media beyond the Slowly Varying Envelope Approximation

We report a modulational instability (MI) analysis of a mathematical model appropriate for ultrashort pulses in cascaded quadratic-cubic nonlinear media beyond the so-called slowly varying envelope approximation. Theoretically predicted MI properties are found to be in good agreement with numerical simulation. The study shows the possibility of controlling the generation of MI and formation of solitons in a cascaded quadratic-cubic media in the few cycle regimes. We also find that stable propagation of soliton-like few-cycle pulses in the medium is subject to the fulfilment of the modulation instability criteria

Post-rift uplift of the Dhofar margin (Gulf of Aden)

International audienceAn investigation of the sedimentation pattern along the Dhofar margin allows us to describe its late-stage evolution. After the accumulation of a thick post-rift sedimentary succession, two debris flow events occurred at the foot of the slope. The first event, affecting a wide area of the margin, marks a major change in sedimentation. The second event is associated with a shift of sedimentary deposition from the slope toward the basin. This latter debris flow was caused by an uplift phase, and highlights two distinct deformational styles in the eastern and western part of the Dhofar margin. Both events occurred very late in the history of the margin, at least 7.6 Ma after the end of the rifting phase (35-17.6 Ma)

Textile sensors for stab and cut detection

Manufacturers are aiming for more flexible and lightweight protective clothing to increase wearing comfort. A cardigan with a knitted stab-resistant inlay and an alarm system is presented. The stab-resistant inlay is based on a multilayer ultra-high molecular weight poly ethylene (UHMW-PE) fabric. Stab resistance was evaluated according to the standard of the Association of Test Laboratories for Bullet, Stab or Pike Resistant Materials and Construction Standard (VPAM 2011). Furthermore sensors for the detection of cuts and pressure were integrated. Both sensors can trigger alarms if the wearer is attacked. Normal pressure occurring through leaning on a wall or sitting is filtered out and does not trigger an alarm

On the potential for discontinuing atovaquone-proguanil (AP) ad-hoc post-exposure and other abbreviated AP-regimens: Pharmacology, pharmacokinetics and perspectives

According to current guidelines, atovaquone-proguanil (AP) malaria chemoprophylaxis should be taken once daily starting one day before travel and continued for seven days post-exposure. However, drug-sparing regimens, including discontinuing AP after leaving malaria-endemic areas are cost-saving and probably more attractive to travelers, and may thus enhance adherence. AP has causal prophylactic effects, killing malaria parasites during the hepatic stage. If early hepatic stages were already targeted by AP, AP could possibly be discontinued upon return. Pharmacokinetic data and studies on drug-sparing AP regimens suggest this to be the case. Nevertheless, the evidence is weak and considered insufficient to modify current recommendations. Field trials require large numbers of travelers and inherently suffer from the lack of a control group. Safely-designed controlled human malaria infection trials could significantly reduce study participant numbers and safely establish an effective AP abbreviated regimen which we propose as the optimal trial design to test this concept

Order statistics of the trapping problem

When a large number N of independent diffusing particles are placed upon a site of a d-dimensional Euclidean lattice randomly occupied by a concentration c of traps, what is the m-th moment of the time t_{j,N} elapsed until the first j are trapped? An exact answer is given in terms of the probability Phi_M(t) that no particle of an initial set of M=N, N-1,..., N-j particles is trapped by time t. The Rosenstock approximation is used to evaluate Phi_M(t), and it is found that for a large range of trap concentracions the m-th moment of t_{j,N} goes as x^{-m} and its variance as x^{-2}, x being ln^{2/d} (1-c) ln N. A rigorous asymptotic expression (dominant and two corrective terms) is given for for the one-dimensional lattice.Comment: 11 pages, 7 figures, to be published in Phys. Rev.