Ziquan Li

Objectives: B3GALT6 ‐related disorders are characterized by severe early‐onset spinal deformities requiring surgical corrections but are associated with increased risks of perioperative complications. This study reports the clinical experience and outcomes of selecting the substantially touched vertebra (STV) as the lowest instrumented vertebra (LIV) in spinal surgeries for patients with B3GALT6 ‐related disorders, a group of extremely rare skeletal and connective tissue disorders.Methods: This retrospective study included patients who were molecularly diagnosed with B3GALT6 ‐related disorders and received spinal surgeries for (kypho)scoliosis between 2017 and June 2023. Their medical records were reviewed. We also conducted a systematic literature review to identify (kypho)scoliosis management in patients with B3GALT6 ‐related disorders.Results: We identified a total of four patients. Patient 1 presented with severe kyphoscoliosis and segmentation defects and received a pedicle subtraction osteotomy with short fusion and dual growing rods from T3 to L3. However, coronal imbalance was observed at the 18‐month follow‐up. Genetic testing revealed biallelic disease‐causing variants in B3GALT6 . A revision surgery was successfully performed, with the level of the LIV extended to the STV (L4). The LIV was similarly extended to the STV in the index surgery for subsequent Patients 2 and 3 who received preoperative genetic testing results, and no complication has been observed. Patient 4 underwent preoperative Halo‐pelvic traction to minimize complications, followed by posterior spinal fusion. The curves were successfully reduced without complications. A systematic literature review identified 86 articles reporting (kypho) scoliosis management in 12 of the 63 patients with B3GALT6 ‐related disorders. Limited surgical experience has been reported, with an increased rate of complications, including death.Conclusions: Selecting the STV as the LIV is recommended in spinal surgeries for patients with B3GALT6 ‐related disorders, considering the characteristic joint hypermobility associated with the condition. Additionally, preoperative Halo‐pelvic traction may also be safe and effective. Furthermore, preoperative molecular diagnosis is essential for enabling precision medicine and minimizing complications.

Background: A hemophilic pseudotumor (HPT) is a rare and challenging complication of hemophilia for which there is no classification system that provides uniformity of descriptions or that can be used to guide management. We have developed such a classification based on anatomical site, HPT severity, and corresponding surgical treatment. Methods: The PUMCH (Peking Union Medical College Hospital) classification was developed on the basis of clinical manifestations and imaging features. Extremity and pelvic HPTs were divided into 4 types and 6 subtypes according to anatomical site and whether or not there was destruction of adjacent bone. Associations between the PUMCH classification and surgical treatment, preoperative comorbidities, operative time, intraoperative bleeding, and postoperative complication rates were analyzed. Results: Forty-five patients with 53 HPTs that were treated at PUMCH between December 2005 and October 2021 were included. The mean age at the time of surgery was 35.4 ± 11.9 years, and the median follow-up duration was 60.3 months. Twenty-eight HPTs were classified as type I (13 IA, 7 IB, 8 IC); 3, as type II; 6, as type III; and 16, as type IV. All 20 type-IA and IB HPTs were treated with excision, and the 3 type-II HPTs were treated with curettage and bone grafting. Fourteen type-IV pelvic HPTs underwent excision, 2 of which needed concomitant pelvic reconstruction. Six type-IC HPTs and 1 type-III HPT underwent excision and osseous reconstruction. Amputation was required for 1 type-IC and 3 type-III HPTs. Type-IC HPTs had the longest mean operative time (194.3 ± 28.2 minutes) and the greatest intraoperative bleeding (2,000 mL [interquartile range, 1,100 to 3,000 mL]). Postoperative infection was more common in patients with type-III (50.0%) and type-IC (28.6%) HPTs, but not significantly so. Conclusions: The PUMCH classification is based on the anatomic pathology and surgical strategy for HPTs. The classification of HPTs corresponds to surgical outcomes, and may be helpful for decision-making regarding their surgical treatment. Level of Evidence: Prognostic Level IV. See Instructions for Authors for a complete description of levels of evidence.

Tracheal stenosis and tracheomalacia in patients with congenital scoliosis are serious and rare conditions caused by congenital dysplasia, postintubation injury, trauma, and tracheal tumor. Anesthesia of a child with tracheal stenosis is challenging for anesthesiologists. We describe an 8-year-old female patient developed severe tracheal stenosis and tracheomalacia after growing rod implantation for congenital scoliosis. Comprehensive assessment of preoperative pulmonary function and airway morphology, which can be neglected clinically, should be performed in congenital scoliosis patients.

We numerically investigate the emission of spin wave (SW) packets from an antiferromagnetic (AFM) domain wall (DW) moving at a varying velocity. This SW is analogous to the bremsstrahlung of a charge with variable motion. The SW packets are emitted towards both sides of DW in the DW acceleration/deceleration process. The frequency of this bremsstrahlung-type SW is at sub-terahertz (THz), which is one magnitude lower than that of SW emitted by the Lorentz contraction of DW. Based on the bremsstrahlung-type SW, we proposed a frequency comb with a sub-THz central frequency and a GHz frequency interval by exploiting the back-and-forth DW variable motion. Our work paves a way for developing a spin–orbit-torque -based AFM THz device with a tunable frequency band.

Background: Percutaneous kyphoplasty (PKP) has become an important minimally invasive surgical technique for fracture stabilization and pain relief in patients with vertebral compression fractures. However, intraspinal cement leakage following PKP is a serious postoperative complication that can lead to morbidity and mortality. Case presentation: We describe an uncommon case of epidural leakage of bone cement in an 81-year-old woman who underwent posterior lumbar decompression and fusion from L3–5 4 years prior and had an unremarkable postoperative course. The patient was admitted to Peking Union Medical College Hospital with complaints of muscle weakness and severe low back pain radiating to the left thigh 1 week after PKP of L5 due to an acute osteoporotic compression fracture. Computed tomographic imaging revealed massive leakage of cement into the spinal canal at L5-S1, and therefore, surgical decompression and removal of epidural cement were performed carefully without causing a dural tear. She improved remarkably and no neurologic deterioration was observed in the postoperative period during the one-year follow-up. Conclusions: We present the rare reported case, to our knowledge, of epidural cement leakage after PKP at the segment of internal fixation and discuss the most likely etiologies and preventive measures for this condition.

While the clinical efficacy of aspirin in cerebral thrombosis prevention has been well established, its mechanism of action is still controversial. In an effort to better understand these mechanisms and to identify potential biomarkers, comparative proteomic analysis between 18 patients both pre-aspirin treatment at the time of cerebral thrombotic onset (control group) and post-aspirin treatment (experiment group) was carried out using two-dimensional gel electrophoresis (2-DE) in combination with matrix-assisted laser desorption ionization time of flight mass spectrometry (MALDITOF/ MS). Of the 228 2-DE identified differentially expressed protein spots, 11 proteins showed more than a 1.5-fold difference. Of these, vitamin D-binding protein (DBP) and actin were further examined via Western blot and showed consistent results, with DBP levels significantly increased post-aspirin treatment (114.04 ± 16.69) relative to pre-treatment (66.33 ± 5.61) while actin showed the opposite trend (p < 0.01 for both comparisons). Next, co-immunoprecipitation analysis of DBP and actin showed direct binding. Furthermore, a protein–protein interaction network of DBP and the other differentially expressed proteins was constructed using Ingenuity Pathway Analysis software. These results suggest that DBP acts in the actin scavenge system and consequently the increase in DBP levels correlated with aspirin therapy in cerebral thrombotic patients. These findings also suggest that aspirin may prevent platelet aggregation and thrombosis through the actions of DBP and other DBP related proteins.

Polypropylene (PP)/low-density polyethylene (LDPE) foam composites were prepared by the extrusion and a subsequent post-foaming process in a designed die. Hollow glass beads (HGB) were added to improve the performance of calcium sulfate whisker (CSW)-reinforced PP/LDPE foam composites. The influence of the content of HGB on cell structure and mechanical properties of the as-fabricated PP/LDPE foam composites reinforced by CSW was investigated. It was found that the introduction of HGB could enhance the cell structure of the PP/LDPE foam composites characterized by the reduced mean cell diameter, the increased cell density, and the uniform distribution of cell size. The mechanical properties tests indicated that HGB could significantly improve the compressive properties of the PP/LDPE foam composites in particular.