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Background: Anti-signal recognition protein (anti-SRP) myopathy is a rare idiopathic inflammatory myopathy in children. Herein, a 3-year-old patient with severe anti-SRP myopathy showing a rapidly progressive disease course is presented in order to increase the awareness of pediatricians about idiopathic inflammatory myopathies., Case Presentation: A previously healthy 3-year-old girl presented with progressive symmetrical proximal muscle weakness that caused difficulty in climbing stairs for two months prior to evaluation, and a marked elevation of the serum creatine kinase levels. A skeletal muscle biopsy revealed necrotic and regenerating processes, with mild inflammatory changes. Myositis-specific and associated autoantibodies tested by the immunoblot method were positive for anti-SRP. Pulse corticosteroid, intravenous immunoglobulin, and methotrexate were administered. However, muscle weakness progressed, respiratory distress and dysphagia developed. Rituximab was initiated. While on rituximab treatment, she was able to walk independently and muscle enzymes were within normal range at the 15th month of diagnosis., Conclusion: Early diagnosis of patients with anti-SRP myositis is important to control inflammation and prevent disease progression and complications. To our knowledge, our patient is the youngest case reported in the literature and was successfully treated with rituximab added to conventional therapy., Competing Interests: The authors declare that there is no conflict of interest.

Mowat-Wilson syndrome (MWS) is an autosomal dominant genetic disorder caused by ZEB2 gene mutations, manifesting with unique facial characteristics, moderate to severe intellectual problems, and congenital malformations as Hirschsprung disease, genital and ophthalmological anomalies, and congenital cardiac anomalies. Herein, a case of 1-year-old boy with isolated agenesis of corpus callosum (IACC) in the prenatal period is presented. He was admitted postnatally with Hirschsprung disease (HSCR), hypertelorism, uplifted earlobes, deeply set eyes, frontal bossing, oval-shaped nasal tip, ''M'' shaped upper lip, opened mouth and prominent chin, and developmental delay. Hence, MWS was primarily considered and confirmed by the ZEB2 gene mutation analysis. His karyotype was normal. He had a history of having a prenatally terminated brother with similar features. Antenatally detected IACC should prompt a detailed investigation including karyotype and microarray; even if they are normal then whole exome sequencing (WES) should be done., Competing Interests: Declaration of Conflicting Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s) 2021.)

Motivated by improvements in diffusing wave spectroscopy (DWS) for nonergodic, highly optically scattering soft matter and by cursory treatment of collective scattering effects in prior DWS microrheology experiments, we investigate the low-frequency plateau elastic shear moduli [Formula: see text] of concentrated, monodisperse, disordered oil-in-water emulsions as droplets jam. In such experiments, the droplets play dual roles both as optical probes and as the jammed objects that impart shear elasticity. Here, we demonstrate that collective scattering significantly affects DWS mean-square displacements (MSDs) in dense colloidal emulsions. By measuring and analyzing the scattering mean free path as a function of droplet volume fraction φ, we obtain a φ-dependent average structure factor. We use this to correct DWS MSDs by up to a factor of 4 and then calculate [Formula: see text] predicted by the generalized Stokes-Einstein relation. We show that DWS-microrheological [Formula: see text] agrees well with mechanically measured [Formula: see text] over about three orders of magnitude when droplets are jammed but only weakly deformed. Moreover, both of these measurements are consistent with predictions of an entropic-electrostatic-interfacial (EEI) model, based on quasi-equilibrium free-energy minimization of disordered, screened-charge-stabilized, deformable droplets, which accurately describes prior mechanical measurements of [Formula: see text] made on similar disordered monodisperse emulsions over a wide range of droplet radii and φ. This very good quantitative agreement between DWS microrheology, mechanical rheometry, and the EEI model provides a comprehensive and self-consistent view of weakly jammed emulsions. Extensions of this approach may improve DWS microrheology on other systems of dense, jammed colloids that are highly scattering., Competing Interests: Conflict of interest statement: F.S. is a board member and shareholder of LS Instruments AG.

We study experimentally the origin of heterogeneous dynamics in strongly driven glass-forming systems. Thereto, we apply a well-defined force with a laser line trap on individual colloidal polystyrene probe particles seeded in an emulsion glass composed of droplets of the same size. Fluid and glass states can be probed. We monitor the trajectories of the probe and measure displacements and their distributions. Our experiments reveal intermittent dynamics around a depinning transition at a threshold force. For smaller forces, linear response connects mean displacement, and quiescent mean squared displacement. Mode coupling theory calculations rationalize the observations.

When a particle adsorbs at a liquid interface, the 3-phase contact line geometry depends on the shape of the particle and of the liquid interface. The shape of the contact line is the key to controlling capillary forces among particles, and is therefore a useful means to direct assembly of interfacial particles. We measured the shape of the contact line around millimeter-sized PDMS-coated glass spheres at water/air interfaces with anisotropic shapes. We studied the advancing and receding conditions separately. We focused on interfaces with a cylindrical shape, where the predominant deformation of the meniscus and the contact line both have quadrupolar cos(2ϕ) symmetry. We related the measured magnitude of the quadrupolar deformation to the applied vertical force on the sphere and the interface's deviatoric curvature, D 0 . For modest curvature (D 0 < 0.1 × sphere radius), our results agree with the theoretical prediction for free particles. At higher curvature, the measurements exceed the theory. The theory appears to apply even when there is contact-angle hysteresis, as long as the measured contact angle is used rather than the equilibrium (Young-Dupré) angle. The magnitude of the quadrupolar deformation depends on the applied force. Together, these results show the range of validity of the theory.

In quenched disordered out-of-equilibrium many-body colloidal systems, there are important distinctions between the glass transition, which is related to the onset of nonergodicity and loss of low-frequency relaxations caused by crowding, and the jamming transition, which is related to the dramatic increase in elasticity of the system caused by the deformation of constituent objects. For softer repulsive interaction potentials, these two transitions become increasingly smeared together, so measuring a clear distinction between where the glass ends and where jamming begins becomes very difficult or even impossible. Here, we investigate droplet dynamics in concentrated silicone oil-in-water nanoemulsions using light scattering. For zero or low NaCl electrolyte concentrations, interfacial repulsions are soft and longer in range, this transition sets in at lower concentrations, and the glass and the jamming regimes are smeared. However, at higher electrolyte concentrations the interactions are stiffer, and the characteristics of the glass-jamming transition resemble more closely the situation of disordered elastic spheres having sharp interfaces, so the glass and jamming regimes can be distinguished more clearly.

The angle of contact between a solid surface and a fluid interface plays a key role in wetting and is therefore a focus in studies of a wide range of natural phenomena and fluidic technologies. The contact angle ranges between two values, a maximum (advancing) angle and a minimum (receding) angle. These limiting angles are thought to be properties of the fluids and of the chemistry or topography of the solid. By contrast, we find that the value of the receding angle can be significantly reduced by altering the interface shape. Using millimeter-sized spheres coated with polydimethylsiloxane and pulled through an air-water interface, we observe that the receding angle decreases from 101 ± 1° at a planar interface to as low as 80 ± 1° at saddle- or cylinder-shaped interfaces. The angle decreases smoothly with the deviatoric curvature of the interface (a measure of the shape anisotropy) and is linked to a non-circular contact line.

We measure capillary forces on particles at fluid interfaces in order to assess the key parameters that yield effective stabilizing particles. In our experiments, a millimeter-scale particle is attached to a cantilever, which is used to pull the particle perpendicular to the interface. Simultaneously, we image from the side to measure the cantilever's deflection and thus the pulling force, as well as the height of the particle and the shape of the interface. We find that the peak force on a particle at an interface crowded with other particles is consistently smaller than the force at a clean interface. This result is independent of the difference in fluid mass densities, the material of the target sphere, and the capillary charge of the free particles. We attribute the force reduction to the perturbation of interface shape due to the constraints at the boundaries of the free particles. The results should be helpful in designing particles to stabilize droplets in new oil dispersants or in other applications.