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Tuberous sclerosis complex(TSC)is a rare genetic disease that can lead to benign dysplasia in multiple organs such as the skin, brain, eyes, oral cavity, heart, lungs, kidneys, liver, and bones. Its main symptoms include epilepsy, intellectual disabilities, skin depigmentation, and facial angiofibromas, whilst incidence is approximately 1 in 10 000 to 1 in 6000 newborns. This case presents a middle-aged woman who initially manifested with epilepsy and nodular depigmentation. Later, she developed a lower abdominal mass, elevated creatinine, and severe anemia. Based on clinical features and whole exome sequencing, the primary diagnosis was confirmed as TSC. Laboratory and imaging examinations revealed that the lower abdominal mass originated from the uterus. CT-guided biopsy pathology and surgical pathology suggested a combination of leiomyoma and abscess. With the involvement of multiple organs and various complications beyond the main diagnosis, the diagnostic and therapeutic process for this patient highlights the importance of rigorous clinical thinking and multidisciplinary collaboration in the diagnosis and treatment of rare and challenging diseases.
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With the aging of the Chinese society and the population, the incidence of hip fractures in the elderly is increasing significantly. Elderly patients have various basic diseases and decreased organ compensatory capacity, which increase the risks related to surgery and anesthesia, increase the incidence of postoperative complications and mortality, and affect the recovery process of patients. Malnutrition is one of the main causes of hip fractures in elderly patients, and it is also a major factor predicting the prognosis of patients. Elderly patients with hip fractures are considered at high risk of malnutrition. Malnutrition can lead to adverse clinical outcomes, such as increased mortality and complications, prolonged hospital stays, and increased hospital costs. Elderly patients with hip fracture should be routinely screened for nutritional risk. Those with malnutrition or nutritional risk, should be given nutritional support treatment. And conduct assessments and optimizations of nutritional support treatmentby observing the prognosis indicators such as complication rate, mortality, and rehabilitation status. At present, orthopedic surgeons who are the main body of elderly hip fracture treatment do not pay enough attention to the nutritional status of patients. Many elderly hip fracture patients undergo surgery while their malnutrition status has not improved. Therefore, it is important to improve their prognosis that strengthen the perioperative nutritional management of elderly patients with hip fracture. Domestic research on the nutritional status of elderly hip fracture patients started late. Many medical institutions have not carried out routine nutritional screening and active nutritional support treatment for elderly hip fracture patients, and there is also a lack of relevant clinical research and data statistics in the nutritional support and treatment of elderly patients with hip fractures. This article describes the current research status of nutritional risk screening and nutritional support treatment for elderly patients with hip fracture at home and abroad. However, due to differences in ethnicity, lifestyle, religious culture, and eating habits in various regions, foreign research data may not be suitable for domestic patients. Therefore, this article provides a reference for the research on perioperative nutritional screening and nutritional treatment of elderly hip fracture patients, and establishes a nutritional management plan suitable for elderly hip fracture patients in China.
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Objective To summarize and analyze the clinical outcomes of gestational trophoblastic neoplasia (GTN) patients receiving primary treatment at Peking Union Medical College Hospital from 1985 to 2015,and investigate the changes in treatment efficacy between the first and the second 15 years.Methods Clinical data of GTN patient receiving primary chemotherapy at Peking Union Medical College Hospital from January 1985 to December 2015 were retrospectively analyzed.It further compared the therapeutic results and chemotherapy cycles given to GTN patients,according to International Federation of Gynecology and Obstetrics (FIGO,2000) prognostic score system,who were classified to different stages and low-or high-risk groups.Results In total,1 711 GTN patients were included in this study.Comparing the 1985-2000 group and the 2001-2015 group,the results showed that:(1) while the overall complete remission (CR) rate was 93.7％ (1 603/1 711),the CR rate of 2001-2015 group was significantly higher than that of 1985-2000 group [98.4％ (1 155/1 174) vs 83.4％ (448/537),x2=139.353,P＜0.01].This difference was significant between stage Ⅲ and Ⅳ patients,but nonexistent between stage Ⅰ and Ⅱ patients,including low-and high-risk groups.(2) The relapse rate of patients who had been in CR was 2.7％ (43/1 603),with no significant differences between the groups of 1985-2001 and 2001-2015 [3.6％ (16/448) vs 2.3％ (27/1 155),x2=6.867,P=0.142].(3) The overall mortality rate was 2.6％ (44/1 711),which significantly decreased in 2001-2015 group compared to 1985-2000 group [1.6％ (19/1 174) vs 4.7％ (25/537),x2=13.830,P＜0.01].This difference appeared only in high-risk patients with stage Ⅲ disease (x2=9.505,P＜0.01).(4) Fluorouracil was gradually replaced by floxridine in chemotherapy regimens.The total cycles of chemotherapy regimens given to low-risk patients with stage Ⅲ disease significantly decreased in 2001-2015 group,but no statistical difference was shown with patients at other stages.Moreover,the cycles of consolidation treatment were significantly reduced in patients with stage Ⅲ patients.Conclusions GTN patients could obtain satisfactory curative results after appropriate and standard treatment.Peking Union Medical College Hospital has achieved better curative effect in the latest 15 years than before.
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ObjectiveTo explore the change of spino-pelvic parameters following pedicle subtraction osteotomy (PSO) for thoracolumbar kyphosis secondary to ankylosing spondylitis(AS).MethodsTwenty-one AS patients with thoracolumbar kyphosis,who underwent PSO at L1 level from July 2006 to October 2010 in our hospital,were retrospectively reviewed.There were 18 males and 3 females with a mean age of 35.6 years (range,21-53 years).The pre- and post-operative thoracic kyphosis(TK),lumbar lordosis (LL),globe kyphosis (GK),angle of the fusion levels (AFL),sagittal imbalance (SVA),pelvic incidence (PI),sacral slope (SS) and pelvic tilting (PT) were measured.ResultsSignificant differences were observed in terms of the improvement of LL,PT,SS,SVA,GK and AFL (P＜ 0.01).The alteration of LL showed significant correlation with the change of PT (r=0.59,P=0.005),SS (r=0.64,P=0.002),SVA (r=0.49,P=0.025),and AFL (r=0.60,P=0.004).The change of PT exhibited cardinal correlation with the change of SS(r=0.94,P=0.000).The improvement of AFL significantly correlated with the improvement of SS(r=0.61,P=0.003),PT (r=0.59,P=0.005).ConclusionThe change of the sagittal spino-pelvic profile following PSO in AS-related thoracolumbar kyphosis is closely related with the improvement of LL.
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Objective To investigate the risk factors and revision strategies for rod fracture in patients with severe kyphoscoliosis following posterior vertebral column resection (PVCR).Methods Between June 2003 and June 2011,7 patients (4 males and 3 females) who developed rod fractures following PVCR in our institution were retrospectively reviewed.The average age was 24.4 years (range,12-39 years) at the time of primary surgeries.The occurrence times and locations of the rod fractures were recorded and the risk factors for the fractures were analyzed.Results The occurrence times of the rod fractures ranged from 6 to 53 months.Five cases of fracture occurred within 2 years after the primary surgeries,while the other 2 cases occurred more than 4 years after the primary surgeries.Six cases of fracture occurred at levels of osteotomies and 1 case occurred at 1 level below the osteotomy.The potential reasons for rod fractures were listed as follows:(1)residual kyphosis (1 case); (2) residual kyphosis combined with unsteady gait (1 case); (3) residual kyphosis combined with single rod fixation (1 case); (4) residual kyphosis combined with malposition of titanium mesh cage (1 case); (5) residual kyphosis combined with anterior column defect; (6) injury (2 cases).Six patients underwent revision surgeries,5 patients received one-stage combined anterior-posterior approach surgeries,while 1 patient received single posterior surgery.They were followed up for 12 to 22 months (average,18months).At final follow-up,all patients obtained satisfying corrections and graft fusion,without internal fixationrelated complications.Conclusion Rod fractures mostly occur at levels of osteotomies within 2 years after PVCR.Residual kyphosis is the main risk factor for the rod fracture.Injury,anterior column defects,unsteady gait,single rod fixation and malposition of titanium mesh cage also increase risks of rod fractures.