Clinical features and surgical treatments of scoliosis in neurofibromatosis type 1: a systemic review and meta-analysis.

BACKGROUND: Neurofibromatosis type 1 (NF 1) is an autosomal-dominant tumor predisposition genetic disease affecting approximately 1 in 3000 live births. The condition could present various manifestations ranging from skin abnormalities to neurological tumors. The musculoskeletal system could also be frequently affected, and scoliosis is the most common orthopedic manifestation. Characterized by the early-onset and rapid progression tendency, NF 1-related dystrophic scoliosis presented discrepancies from idiopathic scoliosis in terms of natural history, clinical features, and management outcomes and thus required special attention. In the current study, the authors conducted a systemic review to outline the body of evidence of the natural history, clinical characteristics, surgical outcomes, and surgical complications of NF 1-induced scoliosis, aiming to provide an elucidative insight into this condition. METHOD: Systemic review and meta-analysis were conducted according to the latest Preferred Reporting Items for Systematic Reviews Meta-Analyses (PRISMA) guidelines. The search was performed in Medline, Embase, and Web of Science Core Collection up to December 27, 2022, using related keywords. Clinical features such as frequencies, segmental involvement, and hereditary information were summarized and described qualitatively. Meta-analysis was conducted using R software and the 'meta' package to yield an overall outcome of efficacy and safety of surgical management, precisely, spinal fusion procedure and growing rods procedure. Corrective rate of Cobb angle, sagittal kyphosis angle, and T1-S1 length post-operative and at the last follow-up was used to evaluate the efficacy, and the occurrence of surgery-related complications was used to evaluate the safety. RESULT: A total of 37 articles involving 1023 patients were included. Approximately 26.6% of the NF 1 patients would present with scoliosis. Patients tend to develop scoliosis at an earlier age. The thoracic part turned out to be the most affected segment. No obvious correlation between scoliosis and genotype or hereditary type was observed. Both spinal fusion and growing rod surgery have shown acceptable treatment outcomes, with spinal fusion demonstrating better performance in terms of effectiveness and safety. The growing rods technique seemed to allow a better lengthening of the spine. The mainstay post-operative complications were implant-related complications but could be managed with limited revision surgery. Severe neurological deficits were rarely reported. CONCLUSION: Scoliosis, especially the subtype characterized by dystrophic bony changes, is a significant orthopedic manifestation of NF1. It has an early onset, a tendency to persistently and rapidly progress, and is challenging to deal with. The current review outlines the available evidence from the perspective of natural history, clinical features, and the treatment efficacy and safety of the mainstay surgical options. Patients with NF1 scoliosis will benefit from a better understanding of the disease and evidence based treatment strategies.

Contributing to the Revolution of Electrolyte Systems via In Situ Polymerization at Different Scales: A Review.

Solid-state batteries have become the most anticipated option for compatibility with high-energy density and safety. In situ polymerization, a novel strategy for the construction of solid-state systems, has extended its application from solid polymer electrolyte systems to other solid-state systems. This review summarizes the application of in situ polymerization strategies in solid-state batteries, which covers the construction of polymer, the formation of the electrolyte system, and the design of the full cell. For the polymer skeleton, multiple components and structures are being chosen. In the construction of solid polymer electrolyte systems, the choice of initiator for in situ polymerization is the focus of this review. New initiators, represented by lithium salts and additives, are the preferred choice because of their ability to play more diverse roles, while the coordination with other components can also improve the electrical properties of the system and introduce functionalities. In the construction of entire solid-state battery systems, the application of in situ polymerization to structure construction, interface construction, and the use of separators with multiplex functions has brought more possibilities for the development of various solid-state systems and even the perpetuation of liquid electrolytes.

Characterization of SNCA Multiplication in Parkinson's Disease: 2 New Cases and Evaluation of the Literature.

Background: Alpha-synuclein (SNCA) copy number variations (CNV) have been certified as a causative mutation in patients with familial and sporadic Parkinson's disease (PD). Case: We report three SNCA duplication cases diagnosed as PD. Through whole-exome sequencing, we identified a de novo 4.56 Mb repeated region in one patient and a 2.50 Mb repeated region in familial PD with two patients. Literature review: In review of previous cases, we suggest that aggressive behavior is more remarkable in CNV4 patients. Meanwhile, frequency of cognition decline and dementia were slightly increased in CNV4 patients. We also illustrate a younger onset age in offspring than parent in familial SNCA multiplication PD cases. No difference was observed in disease duration between parent and offspring generation. Conclusions: Our findings demonstrated the clinical and genetic characteristics in PD with SNCA multiplication and provided strong evidence for genetic anticipation. These results may be instructive for future disease diagnosis and genetic counseling.

[Source Apportionment and Pollution Assessment of Soil Heavy Metal Pollution Using PMF and RF Model: A Case Study of a Typical Industrial Park in Northwest China].

Based on the concentration data of seven heavy metal elements[As, Cd, Cu, Pb, Hg, Ni, and Cr(Ⅵ)] in the surface soil of a typical industrial park in northwest China, the characteristics of heavy metal pollution in the industrial park were analyzed, and the ecological risk and pollution were evaluated using the potential ecological risk index and the index of geo-accumulation. The positive matrix factorization (PMF) model and random forest (RF) model were used for quantitative source analysis, and the emission data of sampling enterprises and empirical data of the source emission component spectrum were combined to identify the characteristic elements and determine the emission source category. The results showed that the heavy metals at all sampling points in the park did not exceed the second-class screening value of construction land in the soil pollution risk control standard for construction land (GB 36600-2018). However, compared with the local soil background values, five elements, excluding As and Cr, were enriched in different degrees, presenting slight pollution and moderate ecological risk (RI=250.04). Cd and Hg were the main risk elements of the park. The results of source analysis showed that the five main sources of pollution were fossil fuel combustion and chemical production sources (33.73%, 9.71%, total source contribution rate of PMF and RF, respectively; the same below), natural sources and waste residue landfill (32.40%, 40.80%), traffic emissions (24.49%, 48.08%), coal burning and non-ferrous metal smelting (5.43%, 0.11%), and electroplating and ore smelting (3.95%, 1.30%). The simulation R2 of the total variable of the two models were above 0.96, indicating that the models could predict heavy metals well. However, considering the actual situation of the number of enterprises in the park and roading density, the main pollution sources of soil heavy metals in the park should be industrial sources, and the simulation results of the PMF model were closer to the actual situation in the park.

Early patellar tendon rupture after total knee arthroplasty: A direct repair method.

BACKGROUND: Patellar tendon rupture after total knee arthroplasty (TKA) is a catastrophic complication. Although the occurrence of this injury is rare, it can lead to significant dysfunction for the patient and is very tricky to deal with. There has been no standard treatment for early patella tendon rupture after TKA, and long-term follow-up data are lacking. AIM: To introduce a direct repair method for early patella tendon rupture following TKA and determine the clinical outcomes and complications of this method. METHODS: During the period of 2008 to 2021, 3265 consecutive TKAs were retrospectively reviewed. Twelve patients developed early patellar tendon rupture postoperatively and were treated by a direct repair method. Mean follow-up was 5.7 years. Demographic, operative, and clinical data were collected. The clinical outcomes were assessed using the Western Ontario and McMaster Universities (WOMAC) score, the Hospital for Special Surgery (HSS) score, knee range of motion, extensor lag, and surgical complications. Descriptive statistics and paired t test were employed to analyze the data. RESULTS: For all 12 patients who underwent direct repair for early patellar tendon rupture, 3 patients failed: One (8.3%) for infection and two (17.6%) for re-fracture. The two patients with re-fracture both underwent reoperation to reconstruct the extensor mechanism and the patient with infection underwent revision surgery. The range of motion was 109.2° ± 10.6° preoperatively to 87.9° ± 11° postoperatively, mean extensor lag was 21° at follow-up, and mean WOMAC and HSS scores were 65.8 ± 30.9 and 60.3 ± 21.7 points, respectively. CONCLUSION: This direct repair method of early patellar tendon rupture is not an ideal therapy. It is actually ineffective for the recovery of knee joint function in patients, and is still associated with severe knee extension lag and high complication rates. Compared with the outcomes of other repair methods mentioned in the literature, this direct repair method shows poor clinical outcomes.

Different squatting positions after total knee arthroplasty: A retrospective study.

BACKGROUND: Total knee arthroplasty (TKA) has been shown to improve quality of life and reduce pain. High-flexion activities such as squatting, kneeling, and floor transfers are mainly listed as demanding tasks. Among them, squatting is an important position. AIM: To provide a new squat position classification and evaluate the different squatting positions of a series of patients after primary TKA. METHODS: From May 2018 to October 2019, we retrospectively reviewed 154 video recordings of the squatting-related motions of patients after TKA. Among the included patients, 119 were women and 35 were men. Their mean age at the index surgery was 61.4 years (range, 30 to 77). RESULTS: The median follow-up was 12 mo (range, 6 to 156 mo). We classified those squatting-related motions into three major variations according to squatting depth: Half squat, parallel squat, and deep squat. The angles of hip flexion, knee flexion, and ankle dorsiflexion were measured in the screenshots captured from the videos at the moment of squatting nadir. A total of 26 patients were classified as half squats, 75 as parallel squats, and 53 as deep squats. The angles of hip flexion, knee flexion, and ankle dorsiflexion all differed significantly among the three squatting positions (P < 0.001). In the parallel squat group, the mean knee flexion angle (°) was 116.5 (SD, 8.1; range, 97 to 137). In the deep squat group, the mean knee flexion angle (°) was 132.5 (SD, 9.3; range, 116 to 158). CONCLUSION: Among the three squatting positions, deep squat showed the highest hip, knee, and ankle flexion angles, followed by the parallel squat. With the improvement of squatting ability, the patient's postoperative satisfaction rate was also significantly enhanced. However, the different squatting abilities of the patients cannot be effectively distinguished from the scoring results (P > 0.05). Our squatting position classification offers a pragmatic approach to evaluating patients' squatting ability after TKA.

False acetabulum is preoperative guidance for Crowe type IV hips on hip reduction without femoral shortening during total hip arthroplasty.

BACKGROUND: We aimed to analyze if the false acetabulum is a good indicator for determining femoral shortening. METHODS: We retrospectively included 102 patients with unilateral Crowe type IV developmental dysplasia who underwent primary total hip arthroplasty from April 2008 to May 2019. Based on the presence of false acetabulum, the 102 hips were further classified Crowe IVA group and Crowe IVB group. Radiographic measurement included the height of greater trochanter (HGT) preoperatively and postoperatively, which reflected the distalisation of greater trochanter (DGT). Harris hip score (HHS), limb length discrepancy (LLD), and complications were collected as clinical evaluation. RESULTS: Sixty hips were classified into Crowe IVA group, and 42 hips were classified into Crowe IVB group. Within Crowe IVA group, the proportion of hips treated with subtrochanteric osteotomy was significantly higher than that in Crowe IVB group (97% vs. 12%) (P < 0.001). The DGT in Crowe IVA group was also greater (67 vs. 32 mm) (P < 0.001). At last follow-up, both two groups obtained excellent clinical scores. There was no significant difference in postoperative LLD between the two groups (P = 0.001). Six dislocations occurred and three patients developed femoral nerve palsy, while all recovered in a year. CONCLUSION: The false acetabulum is a promising and good indicator for determining femoral shortening.

The Influence of Femoral Proximal Medullary Morphology on Subtrochanteric Osteotomy in Total Hip Arthroplasty for Unilateral High Dislocated Hips.

OBJECTIVE: To evaluate the predictive values of femoral proximal medullary morphology for the use of subtrochanteric osteotomy (STO) in unilateral Crowe IV developmental dysplasia of the hip (DDH). METHODS: Ninety four patients with unilateral Crowe type IV DDH (59 hips in STO group and 35 hips in the non-STO group) between April 2008 and June 2019 were enrolled. All patients underwent THA using the Pinnacle acetabular shell, ceramic liner and femoral head, the S-ROM stem with proximal sleeve. Three parameters on the standard anteroposterior hip radiographs were measured: the widths of medullary canals at 20 mm above the center of lesser trochanter (CLT)，20 mm below the CLT and the isthmus. Canal flare index (CFI), metaphyseal canal flare index (MCFI), diaphyseal canal flare index (DCFI) were calculated. A S-ROM femoral stem was used in all patients during total hip arthroplasty (THA). RESULTS: The CFI and DCFI in the STO group were lower than those in the non-STO group. However, there was no statistical difference in MCFI between the two groups. The receiver operating characteristic (ROC) curves shown that DCFI had the highest area under the curve (AUC), at 0.885. This was followed by the CFI, which had an AUC of 0.847. The AUC of MCFI was 0.579. The optimal threshold for DCFI was 1.44, which lead to a sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of 0.771, 0.898, 0.869, and 0.818, respectively. For CFI, the optimal threshold was 3.28, resulting in a sensitivity, specificity, PPV, and NPV of 0.829, 0.729, 0.878, and 0.644, respectively. CONCLUSIONS: The DCFI and CFI may be potent indicators in predicting the use of STO in unilateral Crowe IV DDH. The optimal threshold for CFI and DCFI were 3.28 and 1.44 and had good sensitivity and specificity for predicting the use of STO during THA.

Prognostic nomograms and risk-stratifying systems for predicting survival in patients with resected pT2-4aN0M0 esophageal carcinoma.

BACKGROUND: According to the National Comprehensive Cancer Network (NCCN) guidelines, surveillance or adjuvant chemoradiation is recommended for patients with completely resected pT2-4aN0M0 esophageal carcinoma (EC). Due to this population's variant prognosis, we developed novel nomograms to define the high-risk patients who may need closer follow-up or even post-operative therapy. METHODS: Cases with resected pT2-4aN0M0 EC from the Surveillance, Epidemiology, and End Results (SEER) database and the Sun Yat-sen University Cancer Center (SYSUCC) were enrolled in the study. The SEER database cases were randomly assigned into the training cohort (SEER-T) and the internal validation cohort (SEER-V). Cases from the SYSUCC served as the external validation cohort (SYSUCC-V). Overall survival (OS) and cancer specific survival (CSS) were compared between groups. Multivariate analyses were applied to identify the prognostic factors. Nomograms and risk-classifying systems were developed. The nomograms' performances were evaluated by concordance index (C-index), calibration plots and decision curve analysis (DCA). RESULTS: A total of 2,441 eligible EC cases (SEER-T, n=839; SEER-V, n=279; SYSUCC-V, n=1,323) were included. Age, sex, chemotherapy, lymph node harvested (LNH) and T stage were identified as the independent predictors for CSS. Regarding OS, it also included the prognostic factor of histology. Nomograms were formulated. For CSS, the C-index was 0.68 [95% confidence interval (CI): 0.66-0.71], 0.67 (95% CI: 0.63-0.71) and 0.61 (95% CI: 0.59-0.63) for the SEER-T, SEER-V, and SYSUCC-V, respectively. For OS, the C-index was 0.69 (95% CI: 0.66-0.72), 0.64 (95% CI: 0.59-0.69) and 0.62 (95% CI: 0.61-0.63) for the SEER-T, SEER-V, and SYSUCC-V, respectively. The calibration curves and DCA showed good performances of the nomograms. In further analyses, risk-classification systems stratified pT2-4aN0M0 EC into low-risk and high-risk subgroup. The OS and CSS curves of these 2 subgroups, in the full analysis set or stratified by TNM stage, histology, T stage and LNH categories, showed significant distinctions. CONCLUSIONS: The novel prognostic nomograms and risk-stratifying systems which separated resected pT2-4aN0M0 esophageal carcinoma patients into the low-risk and high-risk prognostic groups were developed. It may help clinicians estimate individual survival and develop individualized treatment strategies.

[Concentrations, Sources, and Health Risks of PM2.5 Carrier Metals in the Beijing Urban Area and Suburbs].

To explore the differences in pollution characteristics, sources, and health risks of PM2.5 carrier metals in urban and suburban areas in Beijing, daily PM2.5 samples were collected from Haidian and Daxing from June to November 2017 and the concentration of PM2.5 and 13 constituent metals were analyzed. The sources of these 13 metal elements were analyzed by positive matrix factorization (PMF), and the health hazards of a subset of 9 metals were evaluated using health risk assessment. The results showed that the concentrations of PM2.5 and 10 metal concentrations in the urban area including Cr, Co, Mn, and Ni were significantly different from those in suburban areas (P<0.05). The source analysis results show four key sources, although their relative contributions vary slightly between urban and rural areas. In urban areas, the main sources are motor vehicles (51.2%), coal burning (19.1%), dust (19.3%), and fuel oil (10.4%); in the suburbs, sources are motor vehicles (47.9%), coal burning (22.6%), dust (20.2%), and electroplating (9.3%). The results of the health risk assessment showed that all metal HQ values in the suburbs were less than 1, and there was no non-carcinogenic risk. Ni and Pb in urban areas, and Cd, Co, Ni, and Pb in suburban areas, do not present a cancer risk, while the R values of As (2.77×10-5), Cd (2×10-6), Co (1.76×10-6), and Cr(Ⅵ) (7.88×10-6) in urban areas and As (8.34×10-6) and Cr(Ⅵ) (4.94×10-6) in suburban areas present some risk of cancer.

Multilayered Graphene-Assisted Broadband Scattering Suppression through an Ultrathin and Ultralight Metasurface.

Here, we present an ultralight multilayered graphene-based metasurface for suppressing specular reflection. With the help of a joint optimization method, dual low-reflection mechanisms including absorption and random diffusion are realized within the same structure, resulting in a remarkable decrease in the backward reflected energy in an ultrabroadband range of 7.5 to 43 GHz (a relative bandwidth of 140.6%). Experiments demonstrate that our design with a thickness of approximately 3.27 mm can maintain excellent antireflection performance over a wide angle range of 0 to 45° for both TE and TM waves. Additionally, as a result of adopting low-density substrates (polyethylene terephthalate and polymethylacrylimide foam) and multilayered graphene films, the proposed metasurface shows the advantage of ultralight weight, thus opening an avenue for a number of engineering applications such as electromagnetic shielding, information security, and electromagnetic compatibility technology. In addition, owing to the natural characteristics (corrosion resistance, bending resistance, etc.) of multilayered graphene films, the proposed metasurface shows enormous potential in some particular application scenarios with harsh conditions.

Histidine-tryptophan-ketoglutarate solution versus University of Wisconsin solution in adult-to-adult living donor liver transplantation: A propensity score matching analysis from mainland China.

ABSTRACT: To compare the difference between University of Wisconsin (UW) solution and histidine-tryptophan-ketoglutarate (HTK) solution in adult living donor liver transplantation (LDLT).This study included LDLT patients at the Liver Transplantation Center of West China Hospital of Sichuan University from November 2001 to June 2018. These patients were classified into 2 groups depending on the use of the different preservation solutions, and the confounding factors between the 2 groups were eliminated by propensity score matching. Finally, the incidence of complications; serum examination at postoperative days 1, 3, 5, 7, 14, 21, and 30; and the overall survival rate of the 2 groups were compared to observe whether there were any differences between the 2 preservation solutions.Of the 298 patients we screened, 170 were treated with UW solution and 128 with HTK solution. After propensity score matching, 106 pairs of patients were selected. In the comparison of the 2 groups, the length of intensive care unit stay in the UW group was significantly longer than that in the HTK group (P = .022), but there was no difference in the total length of hospital stay between the 2 groups (P = .277). No statistically significant difference was observed in the 2 groups in terms of the incidence of complications or postoperative examinations. However, the incidence of early allograft dysfunction in the HTK group was slightly lower than that in the UW group (HTK: UW = 14.1%: 20.7%), although the difference was not statistically significant. In terms of the overall survival rate, the 1, 3, and 5-year survival rates of the HTK group were 85.5%, 70.2%, and 65.1%, respectively, while the 1, 3, and 5-year survival rates of the UW group were 83.1%, 67.2%, and 59.8%, respectively, and there was no significant difference between the 2 groups.In conclusion, our study shows that UW solution and HTK solution are equivalent in perioperative safety, the recovery of transplanted liver function, the occurrence of postoperative complications and overall survival and can be safely and effectively applied in adult LDLT. If economic factors are taken into account, HTK can save costs to a certain extent.