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348 results on '"Wang, Shengru"'

9. The Performance of Graduation from Traditional Growing Rods with Apical Control Technique in Patients with Congenital Early-Onset Scoliosis

Author

LI Zhiyi, ZHAO Yiwei, DU You, LI Chenkai, ZHANG Haoran, LIN Guanfeng, YANG Yang, YE Xiaohan, WANG Shengru, and ZHANG Jianguo

Subjects
early-onset scoliosis, traditional growth rods, apical control technique, hybrid technique, graduation performance, Medicine
Abstract

Objective Different from other etiologies of early-onset scoliosis (EOS), congenital early-onset scoliosis (CEOS) is mainly linked to vertebral anomalies, such as formation failures and segmentation failures at the apex segments. So far, there is little research on CEOS patients who have completed traditional growing rods (TGR) treatment, and the initial outcomes of TGR with or without apical control technique (ACT) are different. Therefore, we compared the 'final' results of CEOS patients who completed TGR treatment with or without ACT. Methods We conducted a retrospective study of CEOS patients who completed TGR treatment from 2007—2020. They either had final fusion or were followed up after reaching skeletal maturity. We split the patients into two groups based on whether they had ACT with TGR or not. The ACT-TGR group had apical vertebrectomy/hemivertebrectomy with short fusion and TGR. The TGR-only group had only TGR. We looked at their demographic features, radiographic measurements, and complications. Results This study enrolled 46 CEOS patients, of which 13 patients were in the ACT-TGR group and 33 patients in the TGR group. The ACT-TGR group had a longer distraction interval (1.17 years vs. 0.75 years). The ACT-TGR group had a larger preoperative main curve [87.00(63.50, 98.00)], but the residual curve degrees were comparable between the two groups at the last follow-up (P=0.354). At the last follow-up, the T1-12 and T1-S1 heights were similar between the two groups. The ACT-TGR group had a lower number of implant-related complications per patient (0.77 vs. 1.48). Three patients in the ACT-TGR group underwent final fusion, while 17 patients in the TGR group underwent final fusion (P=0.060). Conclusions Both ACT-TGR and traditional TGR coud effectively correct deformity and preserve spinal growth in CEOS patients. ACT-TGR had a better corrective effect on patients with severe deformity and did not have a significant impact on spinal height. For patients with acceptable correction, spontaneous fusion and without implant failure, retaining the implant and continuing observation could be a strategy for graduating from growing rod treatment.

Published
2023
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10. Progress in the Research of the Spinal Deformity Related to Marfan Syndrome

Author

LIN Guanfeng, WU Nan, WANG Shengru, YANG Yang, and ZHANG Jianguo

Subjects
marfan syndrome, spinal deformity, skeletal abnormalities, correction surgery, Medicine
Abstract

Marfan syndrome(MFS) is an autosomal dominant systemic connective tissue disease. The incidence rate of MFS is about 2-3 per 10 000. Main cause of MFS is FBN1 gene mutation. About 2/3 of MFS patients have spinal deformities, showing symptoms of scoliosis, thoracic lordosis and lumbar kyphosis, severe spondylolisthesis, dural dilatation and pedicle dystrophy. MFS scoliosis develops with age and may continue even after bone maturation. Conservative treatments such as brace are usually ineffective. Surgical treatment of main curve > 40°-45 °is recommended, but due to the special anatomical structure of MFS patients, such surgical complications as dural leakage, failure of internal fixation and revision surgery are not uncommon.

Published
2023
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11. A Case Report of Gorham-Stout Syndrome with Kyphoscoliosis

Author

LI Chenkai, WANG Shengru, LI Ziquan, WU Nan, and ZHANG Jianguo

Subjects
gorham-stout syndrome, kyphoscoliosis, chylothorax, Medicine
Abstract

This article reported the treatment of Gorham Stout syndrome (GSS) with kyphoscoliosis. The patient was an 11-year-old male who was presented with kyphoscoliosis, first developed 4 years ago, accompanied by incomplete paralysis of both lower limbs. The syndrome worsened in the previous year. He had a history of chylothorax, ventilation dysfunction, and osteoporosis. The patient underwent skull traction in another hospital for 7 months, which was ineffective. Finally, he underwent posterior correction, internal fixation, and bone graft fusion (C4-T8) in our hospital. The postoperative clinical outcomes and the posterior correction were satisfactory, with significant improvement in incomplete paralysis of both lower limbs. This article aims to improve the understanding of GSS and provided reference for its diagnosis and treatment through a typical case report and review of previous literature.

Published
2023
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15. Unraveling the genetic architecture of congenital vertebral malformation with reference to the developing spine

Author

Zhao, Sen, Zhao, Hengqiang, Zhao, Lina, Cheng, Xi, Zheng, Zhifa, Wu, Mengfan, Wen, Wen, Wang, Shengru, Zhou, Zixiang, Xie, Haibo, Ruan, Dengfeng, Li, Qing, Liu, Xinquan, Ou, Chengzhu, Li, Guozhuang, Zhao, Zhengye, Chen, Guilin, Niu, Yuchen, Yin, Xiangjie, Hu, Yuhong, Zhang, Xiaochen, Liu, Pengfei, Qiu, Guixing, Liu, Wanlu, Zhao, Chengtian, Wu, Zhihong, Zhang, Jianguo, and Wu, Nan

Published
2024
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19. Rare variant association analyses reveal the significant contribution of carbohydrate metabolic disturbance in severe adolescent idiopathic scoliosis

Author

Wen, Wen, primary, Zhao, Zhengye, additional, Zheng, Zhifa, additional, Zhao, Sen, additional, Zhao, Hengqiang, additional, Cheng, Xi, additional, Du, Huakang, additional, Li, Ziquan, additional, Wang, Shengru, additional, Qiu, Guixing, additional, Wu, Zhihong, additional, Zhang, Terry Jianguo, additional, and Wu, Nan, additional

Published
2024
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24. Expanding the mutation and phenotype spectrum of MYH3-associated skeletal disorders

Author

Zhao, Sen, Zhang, Yuanqiang, Hallgrimsdottir, Sigrun, Zuo, Yuzhi, Li, Xiaoxin, Batkovskyte, Dominyka, Liu, Sen, Lindelöf, Hillevi, Wang, Shengru, Hammarsjö, Anna, Yang, Yang, Ye, Yongyu, Wang, Lianlei, Yan, Zihui, Lin, Jiachen, Yu, Chenxi, Chen, Zefu, Niu, Yuchen, Wang, Huizi, Zhao, Zhi, Liu, Pengfei, Qiu, Guixing, Posey, Jennifer E., Wu, Zhihong, Lupski, James R., Micule, Ieva, Anderlid, Britt-Marie, Voss, Ulrika, Sulander, Dennis, Kuchinskaya, Ekaterina, Nordgren, Ann, Nilsson, Ola, Zhang, Terry Jianguo, Grigelioniene, Giedre, and Wu, Nan

Published
2022
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28. Lengthening Behavior of Magnetically Controlled Growing Rods in Early-Onset Scoliosis: A Multicenter Study

Author

Heyer, Jessica H., Anari, Jason B., Baldwin, Keith D., Mitchell, Stuart L., Luhmann, Scott J., Sturm, Peter F., Flynn, John M., Cahill, Patrick J., Abdulfattah Abdullah, Abdullah Saad, Ahn, Edward, Akbarnia, Behrooz, Akoto, Harry, Albanese, Stephen, Anari, Jason, Anderson, John, Anderson, Richard, Andras, Lindsay, Bauer, Jennifer, Bellaire, Laura, Betz, Randy, Birch, Craig, Blakemore, Laurel, Boachie-Adjei, Oheneba, Bonfield, Chris, Bouton, Daniel, Brassard, Felix, Brockmeyer, Douglas, Brooks, Jaysson, Bumpass, David, Cahill, Pat, Chemaly, Olivier, Cheung, Jason, Cheung, Kenneth, Cho, Robert, Christman, Tyler, Beauchamp, Eduardo Colom, Couture, Daniel, Crawford, Haemish, Crawford, Alvin, Dahl, Benny, Demirkiran, Gokhan, Devito, Dennis, Diab, Mohammad, El Sebaie, Hazem, El-Hawary, Ron, Emans, John, Erickson, Mark, Fabregas, Jorge, Farley, Frances, Farrington, David, Fedorak, Graham, Fitzgerald, Ryan, Fletcher, Nicholas, Floccari, Lorena, Flynn, Jack, Gabos, Peter, Gardner, Adrian, Garg, Sumeet, Gerow, Frank, Glotzbecker, Michael, Gomez, Jaime, Gonda, David, Guillaume, Tenner, Gupta, Purnendu, Halvorson, Kyle, Hammerberg, Kim, Hardesty, Christina, Hedequist, Daniel, Heffernan, Michael, Heflin, John, Helenius, Ilkka, Herrera, Jose, Hogue, Grant, Holt, Josh, Howard, Jason, Hresko, Michael Timothy, Hwang, Steven, Ihnow, Stephanie, Ilharreborde, Brice, Illingworth, Kenneth, Jain, Viral, Jea, Andrew, Johnson, Megan, Johnston, Charles, Jones, Morgan, Karlen, Judson, Karlin, Lawrence, Katz, Danielle, Kawakami, Noriaki, Kelly, Brian, Kelly, Derek, Knapp, Raymond, Koljonen, Paul, Kwan, Kenny, Labelle, Hubert, Lark, Robert, Larson, A. Noelle, Lavelle, William, Lenke, Lawrence, Lew, Sean, Li, Gertrude, Louer, Craig, Luhmann, Scott, Mac-Thiong, Jean-Marc, Mackenzie, Stuart, MacKintosh, Erin, Mangano, Francesco, Marks, David, Marquez, Sanchez, Martin, Jonathan, Martus, Jeffrey, Matamalas, Antonia, Mayer, Oscar, McCarthy, Richard, McIntosh, Amy, McQuerry, Jessica, Mehta, Jwalant, Metz, Lionel, Miller, Daniel, Miyanji, Firoz, Mundis, Greg, Murphy, Josh, Murphy, Robert, Myung, Karen, Nelson, Susan, Newton, Peter, Ede, Matthew Newton, Nguyen, Cynthia, Nunez, Susana, Oetgen, Matthew, Oswald, Timothy, Ouellet, Jean, Pahys, Josh, Palomino, Kathryn, Parent, Stefan, Garcia, Alejandro Peiro, Pellise, Ferran, Perra, Joseph, Phillips, Jonathan, Pizones, Javier, Poon, Selina, Price, Nigel, Ramirez-Lluch, Norman, Ramo, Brandon, Redding, Gregory, Ritzman, Todd, Rodriguez, Luis, Rodriguez-Olaverri, Juan Carlos, Roye, David, Roye, Benjamin, Saiman, Lisa, Samdani, Amer, Perez-Grueso, Francisco Sanchez, Sanders, James, Sawyer, Jeffrey, Sayama, Christina, Schmitz, Michael, Schulz, Jacob, Schwend, Richard, Shah, Suken, Shapiro, Jay, Shufflebarger, Harry, Skaggs, David, Smit, Kevin, Smith, John, Snyder, Brian, Sponseller, Paul, Stephen, George, Stone, Joe, Sturm, Peter, Sukkarieh, Hamdi, Swarup, Ishaan, Szczodry, Michal, Thometz, John, Thompson, George, Tomoko, Tanaka, Truong, Walter, Vialle, Raphael, Vitale, Michael, Vorhies, John, Wall, Eric, Wang, Shengru, Warner, Bill, Weinstein, Stuart, Welborn, Michelle, White, Klane, Wrubel, David, Wu, Nan, Yankey, Kwadwo, Yaszay, Burt, Yazici, Muharrem, and Zhang, Terry Jianguo

Published
2022
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34. Revision Surgery After Spinal Fusion in Adolescent Idiopathic Scoliosis.

Author

Dong, Yulei, Wang, Shengru, Tang, Ning, Zhao, Hong, Yu, Bin, and Zhang, Jianguo

Subjects
ADOLESCENT idiopathic scoliosis, REOPERATION, SPINAL surgery, SPINAL fusion, WOUND infections, KYPHOSIS
Abstract

Study design: Retrospective Aanalysis of a Large Cohort of Cases. Objectives: To explore the rate and cause of revision surgery after spinal fusion in adolescent idiopathic scoliosis(AIS). Methods: The patients diagnosed with AIS who underwent spinal fusion surgery in a single center from 2002 to 2018 were retrospectively analyzed. All the patients were followed up at least 2 years. The causes of revision surgery were analyzed and the incidence of revision surgery was counted. Results: A total of 1816 AIS patients were included in the study. After an average of 8.5 years (range 3-18 years) follow-up, a total of 51 patients underwent 54 revision operations. The overall revision rate is 2.8%. The revision rate of combined approach (anterior and posterior) and anterior approach was 6.6% (8/122), and the revision rate of posterior approach was 2.5% (43/1694). The most common causes of revision were malposition of implants/implants failure (37%), followed by poor wound healing/ infection (23%). Spinal decompensation, adding on and proximal junctional kyphosis (PJK) accounted for 20%. The compensatory curve continued to worsen after selective fusion accounted for 14% and finally the discomfort with the implants accounted for 6%. Conclusions: The overall revision rate of spinal fusion for AIS is 2.8%. The implants and incision problems were the most common causes of revision surgeries. [ABSTRACT FROM AUTHOR]

Published
2024
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41. Identification of novel FBN1 variations implicated in congenital scoliosis

Author

Lin, Mao, Zhao, Sen, Liu, Gang, Huang, Yingzhao, Yu, Chenxi, Zhao, Yanxue, Wang, Lianlei, Zhang, Yuanqiang, Yan, Zihui, Wang, Shengru, Liu, Sen, Liu, Jiaqi, Ye, Yongyu, Chen, Yaping, Yang, Xu, Tong, Bingdu, Wang, Zheng, Yang, Xinzhuang, Niu, Yuchen, Li, Xiaoxin, Wang, Yipeng, Su, Jianzhong, Yuan, Jian, Zhao, Hengqiang, Zhang, Shuyang, Qiu, Guixing, Ikegawa, Shiro, Zhang, Jianguo, Wu, Zhihong, and Wu, Nan

Published
2020
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43. Variants in the SOX9 transactivation middle domain induce axial skeleton dysplasia and scoliosis

Author

Wang, Lianlei, primary, Liu, Zhaoyang, additional, Zhao, Sen, additional, Xu, Kexin, additional, Aceves, Valeria, additional, Qiu, Cheng, additional, Troutwine, Benjamin, additional, Liu, Lian, additional, Ma, Samuel, additional, Niu, Yuchen, additional, Wang, Shengru, additional, Yuan, Suomao, additional, Li, Xiaoxin, additional, Zhao, Lina, additional, Liu, Xinyu, additional, Wu, Zhihong, additional, Zhang, Jianguo, additional, Gray, Ryan, additional, and Wu, Nan, additional

Published
2023
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44. Variants in the SOX9 transactivation middle domain induce axial skeleton dysplasia and scoliosis

Author

Wang, Lianlei, Liu, Zhaoyang, Zhao, Sen, Xu, Kexin, Aceves, Valeria, Qiu, Cheng, Troutwine, Benjamin, Liu, Lian, Ma, Samuel, Niu, Yuchen, Wang, Shengru, Yuan, Suomao, Li, Xiaoxin, Zhao, Lina, Liu, Xinyu, Wu, Zhihong, Zhang, Terry Jianguo, Gray, Ryan S., and Wu, Nan

Subjects
Article
Abstract

SOX9 is an essential transcriptional regulator of cartilage development and homeostasis. In humans, dysregulation of SOX9 is associated with a wide spectrum of skeletal disorders, including campomelic and acampomelic dysplasia, and scoliosis. The mechanism of how SOX9 variants contribute to the spectrum of axial skeletal disorders is not well understood. Here, we report four novel pathogenic variants of SOX9 identified in a large cohort of patients with congenital vertebral malformations. Three of these heterozygous variants are in the HMG and DIM domains, and for the first time, we report a pathogenic variant within the transactivation middle (TAM) domain of SOX9. Probands with these variants exhibit variable skeletal dysplasia, ranging from isolated vertebral malformation to acampomelic dysplasia. We also generated a Sox9 hypomorphic mutant mouse model bearing a microdeletion within the TAM domain (Sox9Asp272del). We demonstrated that disturbance of the TAM domain with missense mutation or microdeletion results in reduced protein stability but does not affect the transcriptional activity of SOX9. Homozygous Sox9Asp272del mice exhibited axial skeletal dysplasia including kinked tails, ribcage anomalies, and scoliosis, recapitulating phenotypes observed in human, while heterozygous mutants display a milder phenotype. Analysis of primary chondrocytes and the intervertebral discs in Sox9Asp272del mutant mice revealed dysregulation of a panel of genes with major contributions of the extracellular matrix, angiogenesis, and ossification-related processes. In summary, our work identified the first pathologic variant of SOX9 within the TAM domain and demonstrated that this variant is associated with reduced SOX9 protein stability. Our finding suggests that reduced SOX9 stability caused by variants in the TAM domain may be responsible for the milder forms of axial skeleton dysplasia in humans.

Published
2023

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