Search Results (5)

Desmoid tumors are rare, deep-seated myofibroblastic tumors with an unpredictable course, ranging from spontaneous regression to infiltrative growth and locally aggressive behavior, but without metastatic potential. Over the past few decades, advances in understanding their natural history, underlying molecular pathways, and patient care priorities have shifted the treatment paradigm from upfront surgical resection to initial active surveillance, with further treatment dictated by continuous disease progression or associated symptoms. However, there are still specific scenarios where surgery continues to play an important role in locoregional treatment and symptom control. This article will focus on current treatment strategies and surgical indications in adult patients with desmoid tumors, emphasizing patient selection, anatomic site-specific considerations, and surgical technique. Understanding the nuanced role of surgery within the growing treatment landscape is key for individualized patient care in a multidisciplinary setting to optimize quality of life and long-term outcomes. Full article

In this study, a Ni-coated carbon fiber reinforced Al-matrix (Ni-CF/Al) composite is prepared utilizing a pressure-free infiltration process. The CFs are coated with a layer of Ni through an electroless plating process, which facilitates the spontaneous infiltration behavior driven by the Al-Ni binary reaction. The spontaneous infiltration process, observed via synchrotron radiation in the direction opposite to gravity, demonstrates a fastest velocity of 31.02 ± 1.08 μm/s. By increasing the infiltration temperature, the interfacial microstructure of the composite can be enhanced, characterized by a reduction in un-infiltrated defects and promoted by the interfacial Al-Ni reaction. Notably, large-size Al-Ni intermetallic compounds (IMCs) at the interface are replaced by fine (Al+Al₃Ni) eutectic structure, given an optimal fabrication temperature of 720 °C. This contributes to a significantly enhanced ultimate tensile strength (UTS) of the composite, reaching a maximum of 135 ± 4 MPa, which is 159.6% higher than that of the matrix. Full article

Background: Autosomal recessive inherited pathogenetic variants in the histidine triad nucleotide-binding protein 1 (HINT1) gene are responsible for an axonal Charcot-Marie-Tooth neuropathy associated with neuromyotonia, a phenomenon resulting from peripheral nerve hyperexcitability that causes a spontaneous muscle activity such as persistent muscle contraction, impaired relaxation and myokymias. Methods: Herein, we describe two brothers in whom biallelic HINT1 variants were identified following a multidisciplinary approach. Results: The younger brother came to our attention for clinical evaluation of moderate intellectual disability, language developmental delay, and some behavioral issues. His elder brother presented mild intellectual disability, hyperactivity, tiptoe walking, and gait ataxia. At first evaluation, motor impairment with frequent falls, pes cavus, and distal hyposthenia with reduced osteotendinous reflexes were found in both. Grip myotonic phenomenon was also noted. Blood tests revealed mildly elevated creatine kinase, and neurophysiology investigations revealed predominantly axonal polyneuropathy. Muscle MRI highlighted fibro-adipose infiltration, prevalent in the lower limbs. Gene panel testing detected a heterozygous HINT1 variant (c.355C>T/p.(Arg119Trp)) on the paternal allele. A further in-depth analysis using Integrative Genomics Viewer and Optical Genome Mapping led us to identify an additional variant in HINT1 represented by a complex rearrangement located in the region 5′UTR-exon 1-intron 1, not previously described. Conclusions: This complex rearrangement could have been overlooked if the clinical picture had not been evaluated as a whole (from a clinical, neurophysiological, and neuroimaging point of view). Neuropsychiatric manifestations (intellectual disability, hyperactivity, etc.) are part of the picture of HINT1-related neuromyotonia. Full article

The high crystal quality of formamidium lead bromide perovskite (CH(NH₂)₂PbBr₃ = FAPbBr₃) was infiltrated in a mesoporous TiO₂ network. Then, high-quality FAPbBr₃ films were evaluated as active lasing media, and were irradiated with a picosecond pulsed laser to demonstrate amplified spontaneous emission (ASE), which is a better benchmark of its intrinsic suitability for gain applications. The behavior was investigated using two excitation wavelengths of 440 nm and 500 nm. Due to the wavelength-dependent absorbance spectrum and the presence of a surface adsorption layer that could be reduced using the shorter 440 nm wavelength, the ASE power dependence was strongly reliant on the excitation wavelength. The ASE state was achieved with a threshold energy density of ~200 µJ/cm² under 440 nm excitation. Excitation at 500 nm, on the other hand, needed a higher threshold energy density of ~255 µJ/cm². The ASE threshold carrier density, on the other hand, was expected to be ~4.5 × 10¹⁸ cm⁻³ for both excitations. A redshift of the ASE peak was detected as bandgap renormalization (BGR), and a BGR constant of ~5–7 × 10⁻⁹ eV cm was obtained. Full article

In this work, the reactive wetting and infiltration behaviors of a newly designed Sn-V binary alloy were comprehensively explored on porous graphite for the first time. It was discovered that 0.5 wt.% addition of V can obviously improve the wettability of liquid Sn on porous graphite and the nominal V contents in Sn-V binary alloys has minor effects on the apparent contact angles wetted at 950 °C. Moreover, the V-containing Sn-V alloys were initiated to spread on porous graphite at ~650 °C and reached a quasi-equilibrium state at ~900 °C. Spreading kinetics of Sn-3V alloy on porous graphite well fitted in the classic product reaction controlled (PRC) model. However, our microstructural characterization demonstrated that, besides vanadium carbide formation, the adsorption of V element at the wetting three-phase contact line spontaneously contributed to the reactive spreading and infiltrating of Sn-V alloys on porous graphite. Meanwhile, the formation of continuous vanadium carbides could completely block the infiltration of Sn-V active solder alloy in porous graphite. Affected by the growth kinetics of vanadium carbides, the infiltration depth of Sn-V alloys in porous graphite decreased at increased isothermal wetting temperatures. This work is believed to provide implicative notions on the fabrication of graphite related materials and devices using novel V-containing bonding alloys. Full article