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.

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

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.

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 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 investigate the significance of matrix metalloproteinase-9 (MMP-9) and tissue inhibitor of metalloproteinase-1 (TIMP-1) in serum and cerebrospinal fluid for evaluation of severe hand ,foot ,and mouth disease (HFMD) complicated with neurogenic pulmonary edema (NPE).Methods A total of 140 patients diagnosed with HFMD in Henan Children′s Hospital were enrolled and divided into three groups including mild group ,severe HFMD group without NPE ,severe HFMD group with NPE .These severe HFMD patients were also divided into survival group and death group according to the 28-day prognosis .Meanwhile ,50 age-matched healthy children were selected as controls .Serum MMP-9 and TIMP-1 levels were measured in all enrolled children .At the same time ,MMP-9 ,TIMP-1 and ratio of MMP-9／TIMP-1 in cerebrospinal fluid were measured in the severe HFMD group with and without NPE .Quantitative data were compared using one-way analysis of variance , and means comparisons between samples were conducted using LSD-t test .Results Among 140 children with HFMD ,66 were in mild group ,42 in severe HFMD without NPE group ,and 32 in severe HFMD with NPE group .And 50 healthy children were in control group .After 28 days ,14 cases died in severe HFMD groups .MMP-9 , TIMP-1 and MMP-9／TIMP-1 in serum of severe HFMD group with NPE increased significantly greater than those in the other three groups (F＝269 .356 ,121 .301 and 101 .502 ,respectively ,all P ＜0 .05). MMP-9 ,TIMP-1 and MMP-9／TIMP-1 in cerebrospinal fluid of severe HFMD group with NPE were (57 .24 ± 8 .92) μg／L ,(35 .26 ± 8 .14) μg／L and (1 .66 ± 0 .23) μg／L ,respectively ,while those in cerebrospinal fluid of severe HFMD group without NPE were (30 .57 ± 3 .89) μg／L ,(26 .25 ± 0 .32) μg／L and (1 .17 ± 0 .61) μg／L ,respectively .The differences between the two groups were all statistically significant (t＝62 .485 ,37 .680 and 169 .387 ,respectively ,all P＜0 .01).MMP-9 ,TIMP-1 and MMP-9／TIMP-1 in serum and cerebrospinal fluid of death group increased significantly greater than those in survival group ,the difference were statistically significant (all P＜0 .01).The maximum area under curve (AUC) was reached when the MMP9／TIMP-1 ratio in cerebrospinal fluid was 0 .890 (95% CI :0 .801 －0 .978).Conclusions MMP-9 and TIMP-1 may be involved in the pathogenesis of HFMD complicated with NPE .The detection of MMP-9 and TIMP-1 levels may be beneficial for the early diagnosis of severe HFMD with NPE .The imbalance of MMP-9／TIMP-1 ratio can be used as one of the predictors of severe HFMD combined with NPE.

Objective:To evaluate the safety and efficacy of a Zenith fenestrated aortic stent-graft (ZFEN) system in the treatment of short-necked aneurysms.Methods:The clinical data of 5 patients receiving F-EVAR in Zhongshan Hospital, Fudan University from Mar 2018 to Sep 2019 according to the standards of short-necked abdominal aortic aneurysm and COOK ZFEN custom stent were retrospectively analyzed.Results:Patients' average age was (69±11) years old. The time of stent customization was 4-6 weeks. The average maximum diameter of AAA was (60.8±14.0) mm. The average proximal neck diameter was (26.0±2.6) mm and the average length anchoring zone was (6.4±2.2) mm. The rate of technical success was 100%. There was no intraoperative loss of visceral vessels and stent displacement. The meane follow-up time was 6.8 months. The perioperative mortality was 0, and there were no other major complications. There were 3 patients with simple type Ⅱ endoleak and 1 patient with type Ⅲ endoleak. The endoleaks disappeared in 4 patients during follow-up of 3-12 months. 1 patient suffered with type Ⅱ and type Ⅲ endoleak, and the visceral vessels and branching stents were patent in all patients.Conclusion:The short term result of COOK ZFEN stent-graft system was safe and feasible for the treatment of short-necked AAA.

Objective:To discuss the strategies of distal embolic filter protection(DEFP) during excimer laser ablation (ELA) or percutaneous mechanical thrombectomy (PMT) in treatment of peripheral artery disease.Methods:Clinical data of 29 patients undergoing ELA or PMT under the DEFP from Oct 2019 to Aug 2021 were retrospectively collected to analyze the strategies of DEFP and high-risk factors of capturing clinically significant macrodebris.Results:There were 21 males and 8 females, aged (70.3±11.0) years with 32 lesions (29 limbs) including 5 in-stent restenosis (ISR), 10 thrombosis and 17 chronic total occlusion (CTO). The technical success rate of DEFP device release and recovery was 100%. The overall debris capture rate was 77.3% and the macrodebris capture rate was 36.4%. Even with DEFP the distal embolization (DE) incidence was 3.4%. When ELA for CTO with severe calcification or long-segment ISR lesions, the capture rate of macrodebris was as high as 60.0%, and the former was significantly higher than ELA for CTO without high calcification lesions ( P<0.05). Conclusion:ELA or PMT under the DEFP in treatment of peripheral artery disease appears to be of great significance in preventing DE.