Objective: To discuss the etiology, pathogenesis, clinical manifestation, diagnosis and therapy of sphenoid wing dysplasia(SWD) associated with neurofibromatosis type Ⅰ(NF-Ⅰ). Methods: We retrospectively reviewed its clinical manifestations, imaging, surgical treatment, complications and postoperative outcome of one NF-Ⅰ patient with SWD. Results: A 14 years-old girl presented with pulsating exophthalmos, loss of vision and café au lait spots. Radiological studies showed right-side orbital enlargement and complete absence of the greater wing of the sphenoid. Titanium mesh was tailored intraoperatively to close the defect as a barrier between the orbital cavity and the cranium and then covered by periosteum.The patient developed postoperative infectious which was controlled by after antibiotic treatment and proper drainage. Proptosis improved significantly after surgery within a month. Ocular pulsation subsided and clinical symptoms improved at 28-month follow-up. Conclusions Sphenoid greater wing dysplasia associated with neurofibromatosis type Ⅰ is a rare inherited autosomal dominant disorders. The treatment should be customized to each patient. Titanium mesh reconstruction is patients with symptomatic sphenoid dysplasia. It can correct the proptosis and pulsating exophthalmos without the risk of bone resorption and recurrence.However, high risk of infection is associated with the procedure.

Objective:To investigate the effects of preoperative pre-rehabilitation on early functional recovery after knee arthroplasty under the multidisciplinary collaboration mode of accelerated rehabilitation surgery.Methods:The clinical data of 51 patients who underwent total knee arthroplasty in the Department of Orthopedics, Jinjiang Hospital from September 2019 to December 2021 were retrospectively analyzed. These patients were divided into an observation group ( n = 24) and a control group ( n = 27). The observation group received pre-rehabilitation before knee replacement surgery, while the control group did not. After completing the admission procedures, patients in the observation group underwent rehabilitation evaluation in the rehabilitation clinic and received individualized rehabilitation training. The control group did not undergo preoperative pre-rehabilitation but underwent the same individualized rehabilitation training as the observation group. The rehabilitation specialist evaluated the patients' rehabilitation scores [hospital for special surgery knee (HSS) score, visual analog scale (VAS) score] at 2 and 5 days after surgery. The main outcome measures included the range of motion (ROM) of the patient's knee joint at 2 and 5 days after surgery, HSS score at 2 and 5 days after surgery, VAS score at 5 days after surgery, the number of days from surgery to discharge, the incidence of postoperative complications, and the rate of outpatient visits after surgery. Results:There was no significant difference in postoperative ROM of the knee joint between the observation and control groups at 2 days after surgery ( P > 0.05). However, there was a significant difference in score of ROM of the knee joint at 5 days after surgery between the two groups [(100.08 ± 7.75) points vs. (88.44 ± 16.09) points, t = 3.34, P = 0.002]. There was no significant difference in HSS score between the two groups at 2 days after surgery ( P > 0.05). However, there was a significant difference in HSS score between the two groups at 5 days after surgery [(62.84 ± 5.78) points vs. (57.09 ± 6.53) points, t = 3.31, P = 0.002]. There was a significant difference in VAS score (exercise) between the two groups at 5 days after surgery [(3.42 ± 1.02) points vs. (5.37 ± 1.15) points, t = -6.39, P < 0.001]. There was no significant difference in the number of days from surgery to discharge between the two groups ( P > 0.05). There was no significant difference in the incidence of postoperative complications between the two groups ( P > 0.05). However, there was a significant difference in the rate of outpatient visits between the two groups [7/17 vs. 1/26, χ2 = 4.45, P = 0.035]. Conclusion:Preoperative pre-rehabilitation in the accelerated rehabilitation surgery model under multidisciplinary collaboration can help improve the early function of patients undergoing total knee arthroplasty, reduce the pain of postoperative rehabilitation, improve the postoperative rehabilitation compliance, and ultimately enhance patient satisfaction with the surgery.

Humans ; Subclavian Artery ; Vertebral Artery ; Angioplasty ; Stents ; Embolism/prevention & control* ; Angioplasty, Balloon/methods*

Objective:To explore the clinical significance of serum Chromogranin A (CGA) level in predicting the prognosis of children with severe hand, foot, and mouth disease (HFMD) and complicating neurogenic pulmonary edema (NPE).Methods:A total of 162 patients with HFMD admitted in our hospital from January 2017 to December 2019 were enrolled in the study; and 40 age-matched healthy children were selected as controls. According to the disease severity and complication the patients were divided into three groups: mild group ( n=88), severe without NPE group ( n=46) and severe with NPE group ( n=28). In 72 severe HFMD patients 16 cases died (fatal group) and 56 cases survived (survival group) within 28 days of hospitalization. The serum CGA, LAC, GLU, WBC, PCT, IL-6, cTnT were measured in all subjects. SPSS 23.0 software was used for data analysis, and the receiver operating characteristic (ROC) curve was used to evaluate the various indicators for predicting the prognosis of severe HFMD combined with NPE. Results:The serum CGA, GLU, LAC, IL-6 and cTnT levels in severe HFMD group with NPE significantly higher than those in the other three group ( H=61.554, 79.031, 86.994, 36.477, 75.021, all P<0.05 ). The serum CGA, LAC, GLU and IL-6 levels in the fatal group were significantly higher than those in survival group ( Z=-6.094, -4.621, -4.283, -5.504, all P<0.05). There was no significant difference in the levels of WBC, PCT and cTnT between the survival group and the fatal group ( P>0.05). The area under the receiver operating curve (AUC) of serum CGA was 0.890 (95% CI: 0.833-0.947) for predicting the prognosis of patients and the best cut-off value was 120.59 μg/L. Conclusion:The detection of serum CGA levels may be beneficial for the early diagnosis of severe HFMD with NPE, and can be used as one of the predictors of death from severe HFMD.

Objective:To evaluate the effect of Rotarex in peripheral arterial disease (PAD).Methods:The clinical data of 90 PAD patients treated with Rotarex from Aug 2018 to Feb 2020 were retrospectively analyzed.Results:Among the 90 patients, 45 patients had atherosclerotic obliterans complicated with acute thrombosis (ASOCAT), 27 patients had graft restenosis or reocclusion, 16 patients had primary or embolism-induced thrombosis, 2 patients had traumatic or iatrogenic arterial occlusion. Except for 2 patients undergoing hybrid surgery, 88 patients underwent endovascular treatment. Two patients died perioperatively. Within 12 months follow-up, 2 patients died, 4 patients underwent major amputation, target arteries of 10 patients were re-stenosed or re-occluded and 5 patients were lost to follow-up. Compared with the preoperative ankle-branchial index (ABI), significant increase was observed in the 12-month ABI (0.80±0.22 vs. 0.43±0.16, P<0.01). The 12-month restenosis/re-occlusion-free rate was 82.7%, and the 12-month major amputation-free survival (MAFS) was 91.6%. Conclusion:For PAD patients, acceptable outcomes can be achieved with reasonable use of Rotarex for debulking, combined with balloon, stent and other techniques to correct the residual lesions.

Objective:To evaluate the feasibility of the screw and plate for clival fixation using a transoral expanded approaches.Methods:The transoral expanded approaches were performed on craniocervical segment specimens obtained from 7 subjects, including transoral approach (TO), transoral with soft (TOP) or hard (TOHP) palate split, mandibulotomy (MO) and mandibuloglossotomy (MLO). The distribution and thickness of soft tissue, the configuration of the vertebral arteries, the distance between the midline and the vertebral arteries, the exposed area of the clivus and cervical spine, and the range of screw angle (the angle between the line from the lower incisor or the central base of the mandible to the exposed area of the clivus and the tangent line of the clivus) were evaluated.Results:The thickness of the soft tissue on the posterior pharyngeal wall above the clival pharyngeal nodules was 3.5±0.6 mm. That on the anterior C 1-C 5 vertebrae was 5.0±0.5 mm. The distances from the bilateral vertebral arteries to the midline was 19.5±1.2 mm at C 1, 2, 14.6±2.7 mm at C 2, 3, 14.0±2.7 mm at C 3, 4, and 13.9±2.7 mm at C 4, 5. For the TO approach, the longitudinal diameter of the exposed clivus was 8.3±3.0 mm. The distance from the lower incisor to the superior margin of the exposed clivus, the lower margin of the exposed clivus, the anterior arch of C 1, the vertebral body of C 2 and C 3 were 104.7±4.3 mm, 99.2±6.8 mm, 81.4±4.3 mm, 75.1±4.0 mm and 68.7±6.5 mm, respectively. Six specimens were exposed to the C 3, while one was exposed to the C 2. For the TOP approach, the longitudinal diameter of the exposed clivus was 18.5±4.8 mm. The distance from the lower incisor to the superior margin of the exposed clivus and the pharyngeal nodules were 107.9±6.7 mm and 104.8±6.7 mm, respectively. For the TOHP approach, the longitudinal diameter of the exposed clivus was 26.3±1.8 mm (the clival length) with distance from the lower incisor to the superior margin of the clivus 112.4±12.6 mm. For the MO/MLO approach, the entire clivus was exposed. The distance from the central base of the mandible to the superior and inferior margin of the exposed clivus and the pharyngeal nodules were 141.8±15.7 mm, 131.0±9.9 mm and 120.5±8.2 mm, respectively. The inferior margin of the exposed cervical vertebra was C 5, 6. The rate of the clival screw placement through anterior occipitocervical fixation using TO, TOP, TOHP, MO and MLO was 0%, 71% (5/7), 86% (6/7), and 100%, respectively. The screw angle was 99.0°±1.8°, 92.6°±7.7°, 92.6°±7.7°, 75.1°±7.7°, and 75.1°±7.7°, respectively. Conclusion:Occipitocervical fixation with clival screw and plate could be conducted in most cases via TOP and TOHP approaches. However, in some cases with small split-mouth or mouth opening limited, smaller clival screw angle caused by basilar impression or basilar invagination, requiring fixation and reconstruction of the lower cervical spine, and the MO/MLO approaches could be still required to achieve the fixation.

The expression of histone lysine-specific demethylase 1 (LSD1) in colorectal cancer cells is increased, and LSD1 is closely related to its occurrence, development, proliferation, invasion and metastasis. LSD1 is a demethylase whose function depends on flavin adenine dinucleoside. It can specifically catalyze the demethylation reaction of histone lysine, and regulate the expression of target genes by reaction of demethyl and dimethyl (H3K4me, H3K4me2, H3K9me, and H3K9me2) at the 4th and 9th positions of lysine H3. Targeted inhibition of LSD1 has been proved to be able to exert significant anti-tumor effect, but since the tumors involve multiple centers and factors, later studies have found that single inhibition of LSD1 cannot completely and effectively kill tumor cells. Moreover, the specificity of the LSD1 catalytic substrate depends to a large extent on the synergistic factors that bind to it and form complexes. The double-target inhibitors based on LSD1 shows more remarkable effect in tumor inhibition. Therefore, finding the combined synergistic factors of LSD1 may provide the basis for the research of multi-target inhibitors.

Objective:To explore the debulking strategy of lower extremity artery lesions.Methods:Retrospectively analyzed the clinical data of 101 patients underwent debulking therapy at Department of Vascular Surgery,Zhongshan Hospital,Fudan University from June 2019 to June 2020.There were 74 males and 27 females,aged (73.2±11.7)years (range:35 to 93 years).There were 31 cases in Rutherford class 3,39 cases in class 4 and 31 cases in class 5. Hypertension occurred in 72 patients. One hundred and forty lesions were treated in 101 patients. Among them, there were 13 lesions(9.3%) in iliac artery,72 lesions(51.4%) in superficial femoral artery,41 (29.3%) lesions in popliteal artery,10 lesions(7.1%) in tibiofibular trunk,and 4 lesions(2.9%) in below the knee artery.Percutaneous mechanical thrombectomy (PMT) was mainly used in acute thrombosis,excimer laser ablation (ELA) was mainly used for chronic in-stent restenosis and chronic stenosis or totally occlusive lesions,while directional atherectomy (DA) was mainly used for short calcified lesions.Results:All of the patients underwent debulking therapy. Eighty-two lesions(58.6%,82/140) were treated by PMT, 56 (40.0%,56/140) were treated by ELA,and 2 (1.4%,2/140) were treated by DA.The ankle-brachial index of the patient was 0.44±0.19 before surgery, 0.87±0.17 immediately after surgery ( t=-16.26, P<0.01), and 0.81±0.20 at 6 months after surgery( t=-14.67, P<0.01),and 0.79±0.15 ( t=-14.12, P<0.01) at 12 months after surgery. At 12 months,the primary patency was 86.1% (87/101),mortality was 5.0% (5/101), freedom from major-amputation survival rate was 93.1% (94/101),and target lesion reintervention rate was 9.9% (10/101). Conclusions:Debulking is feasible and effective to eliminate the arterial contents and maximize the acquisition of lumen.Selection of suitable debulking methods for different segments and lesions would be helpful to improve the technical success and obtain satisfactory results.

Objective:To explore the debulking strategy of lower extremity artery lesions.Methods:Retrospectively analyzed the clinical data of 101 patients underwent debulking therapy at Department of Vascular Surgery,Zhongshan Hospital,Fudan University from June 2019 to June 2020.There were 74 males and 27 females,aged (73.2±11.7)years (range:35 to 93 years).There were 31 cases in Rutherford class 3,39 cases in class 4 and 31 cases in class 5. Hypertension occurred in 72 patients. One hundred and forty lesions were treated in 101 patients. Among them, there were 13 lesions(9.3%) in iliac artery,72 lesions(51.4%) in superficial femoral artery,41 (29.3%) lesions in popliteal artery,10 lesions(7.1%) in tibiofibular trunk,and 4 lesions(2.9%) in below the knee artery.Percutaneous mechanical thrombectomy (PMT) was mainly used in acute thrombosis,excimer laser ablation (ELA) was mainly used for chronic in-stent restenosis and chronic stenosis or totally occlusive lesions,while directional atherectomy (DA) was mainly used for short calcified lesions.Results:All of the patients underwent debulking therapy. Eighty-two lesions(58.6%,82/140) were treated by PMT, 56 (40.0%,56/140) were treated by ELA,and 2 (1.4%,2/140) were treated by DA.The ankle-brachial index of the patient was 0.44±0.19 before surgery, 0.87±0.17 immediately after surgery ( t=-16.26, P<0.01), and 0.81±0.20 at 6 months after surgery( t=-14.67, P<0.01),and 0.79±0.15 ( t=-14.12, P<0.01) at 12 months after surgery. At 12 months,the primary patency was 86.1% (87/101),mortality was 5.0% (5/101), freedom from major-amputation survival rate was 93.1% (94/101),and target lesion reintervention rate was 9.9% (10/101). Conclusions:Debulking is feasible and effective to eliminate the arterial contents and maximize the acquisition of lumen.Selection of suitable debulking methods for different segments and lesions would be helpful to improve the technical success and obtain satisfactory results.

Objective To evaluate the performance of the Quality of Life Instrument for Chronic Dis-ease Nephrotic Syndrome [QLICD-NS(V2.0)] in patients with nephrotic syndrome.Methods A total of 203 patients with nephrotic syndrome (NS) diagnosed in the Department of Nephrology,Affiliated Hospital of Guangdong Medical University from March 2021 to November 2021 were selected for QLICD-NS(V2.0) eval-uation,and the evaluation methods were generalization theory (GT) and item response theory (IRT).The dif-ficulty,discrimination coefficient and information amount of each item were obtained by using Multilog 7.0 software to analyze the grade response model (GRM) of IRT.Results The results of GT showed that the contribution ratio of the global total score in the four domains of QLICD-NS(V2.0) scale was evenly distribu-ted,and the generalization coefficient of the four domains was greater than 0.50.Except for social function,the variance component of the participants in the other three domains were greater than the item variance compo-nent,and the reliability index of each domain was greater than 0.50.The results of IRT showed that the dis-crimination degree of QLICD-NS(V2.0) scale was 0.82.Except for items TNS7 and TNS8,the difficulty coef-ficients of the other items ranged from -3 to 3 and increase monotonically.Conclusion The QLICD-NS (V2.0) scale has good reliability in physiological function and psychological function,and is acceptable in so-cial field and special function.The QLICD-NS(V2.0) scale developed in this study has good performance.