Hereditary angioedema (HAE) is a rare autosomal dominant genetic disorder characterized by recurrent episodes of subcutaneous and submucosal edema, which can be life-threatening in severe cases. Lanadelumab, a recombinant human monoclonal antibody against kallikrein, has been recommended as a first-line option for long-term prophylaxis of HAE. This paper analyzed the clinical data of four patients with HAE diagnosed and treated with lanadelumab for long-term prophylaxis in our department since 2020. It also reviewed relevant literature, summarizing the clinical manifestations of HAE, as well as the efficacy and safety of lanadelumab in treating HAE, to provide therapeutic references for clinicians.

In the era of evidence-based medicine, it is necessary to explore the unique advantages of traditional Chinese medicine (TCM) based on standardized technical methods and operating procedures in order to achieve the modernization and internationalization of TCM and benefit all humanity. The proposal of a three-pronged evidence system combining TCM theory, human experience and experimental evidence marks an important progress in the thinking method of the TCM evaluation system. The multi-evidence body integrated through appropriate methods provides a strong support for the clinical guideline recommendations and evidence-based health decision-making in TCM. Based on the current methodological progress of international evidence synthesis and grading, this paper proposes a novel approach for integrating multi-evidence in TCM: the MERGE framework. The aim is to establish a solid foundation for the development of this methodology and provide guidance for the advancement of evidence-based medicine framework in TCM.

Objective:To analyze the clinical data of patients with end-stage ankle and hindfoot ar-thropathy who underwent tibiotalocalcaneal(TTC)arthrodesis by the same surgeon,explore the short-and mid-term clinical results,complications and functional improvement,and discuss the clinical progno-sis and precautions of TTC arthrodesis.Methods:Retrospective analysis was made on the clinical data of 40 patients who underwent TTC arthrodesis by the same surgeon from March 2011 to December 2020.In this study,23 males and 17 females were included,with an average age of(49.1±16.0)years.All the patients underwent unilateral surgery.The clinical characteristics,imaging manifestations,main diagno-sis and specific surgical techniques of the patients were recorded.The clinical outcomes were evaluated by comparison of the American Orthopaedic Foot and Ankle Society(AOFAS)ankle-hindfoot score and visual analogue scale(VAS)between pre-operation and at the last follow-up.The fusion healing time,symptom improvement(significant improvement,certain improvement,no improvement or deterioration)and postoperative complications were also recorded.Results:The median follow-up time was 38.0(26.3,58.8)months.The preoperative VAS score was 6.0(4.0,7.0),and the AOFAS score was 33.0(25.3,47.3).At the last follow-up,the median VAS score was 0(0,3.0),and the AOFAS score was 80.0(59.0,84.0).All the significantly improved compared with their preoperative corre-sponding values(P＜0.05).There was no wound necrosis or infection in the patients.One patient suf-fered from subtalar joint nonunion,which was syphilitic Charcot arthropathy.The median bony healing time of other patients was 15.0(12.0,20.0)weeks.Among the included patients,there were 25 cases with significant improvement in symptom compared with that preoperative,8 cases with certain improve-ment,4 cases with no improvement,and 3 cases with worse symptoms than that before operation.Con-clusion:TTC arthrodesis is a reliable method for the treatment of the end-stage ankle and hindfoot ar-thropathy.The function of most patients was improved postoperatively,with little impact on daily life.The causes of poor prognosis included toe stiffness,stress concentration in adjacent knee joints,nonunion and pain of unknown causes.

Objective:To examine the effect of short-term regular aerobic exercise on physical fitness of children with pulmonary with atresia ventricular septal defect after radical biventricular treatment.Methods:This was a prospective self pre-and post-control observation study. The subjects performed regular aerobic exercise for 10 days according to the exercise prescription. Body composition measurement and cardiopulmonary exercise test[lung ventilation function, maximum oxygen uptake(VO 2max), maximum oxygen pulse(O 2/HR max), ventilation oxygen uptake efficiency(OUES), exercise load time], 6 min walking distance(6MWD), sports psychometric test, motor function screening test and fitness test, were collected. The changes of test parameters and scale scoring before and after exercise were analyzed and compared. Results:A total of 7 children with PA/VSD after biventricular surgery were enrolled. The age ranged 8.2-16.2 years old, and there were 2 males and 5 females. VO 2max[(1 196.71±395.31)ml/min vs.(1 297.43±425.73)ml/min, P=0.031], O 2/HRmax[(82.43±7.53)ml/beat vs.(91.57±6.95)ml/beat, P<0.001]increased after exercise. The exercise load time was significantly increased compared with that before intervention[(476.43±35.73)s vs.(531.43±45.76)s, P=0.002]. Resting heart rate before exercise( P=0.013) and peak respiration exchange ratio(PeakRER, P=0.021) were significantly lower. Body composition tests suggest weight, intracellular water, protein and muscle content of lower limb were higher( P<0.05). The motor function score was higher than before( P=0.015); the score of sports fear was lower than before( P=0.009). There was no significant difference in lung capacity and 6-minute walking distance before and after exercise( P>0.05). There were no cardiovascular events during the study period. Conclusion:Short-term regular aerobic exercise for children with PA/VSD after biventricular surgery can improve exercise tolerance, increase lower limb muscle content, improve exercise fear and exercise function, and has good safety and feasibility.

Objective:Based on the specific cleavage and non-specific "trans-cleavage" activities of the clustered regularly interspaced short palindromic repeats (CRISPR) and CRISPR-associated protein(CRISPR/Cas13), we established a visually amplification-free rapid detection technique of 2019-nCoV nucleic acid. This technique is easily processed with a low detection limit and good specificity.Methods:According to the 2019-nCoV gene sequence, specific CRISPR RNAs were screened and designed by bioinformatics analysis, and then synthesized as universal signal-strained RNA transcription targets in vitro to establish and optimize the reaction system. Moreover, the 2019-nCoV pseudoviral nucleic acid was used as a standard substance to evaluate the detection limit. A total of 65 positive samples were collected from various 2019-nCoV variants, while 48 negative samples included other clinically common respiratory pathogens, such as influenza A virus, influenza B virus, human parainfluenza virus, Klebsiella pneumonia, etc. All samples were tested by quantitative PCR (qPCR), digital PCR, and the method established in this study. The sensitivity and specificity of the newly established method were analyzed and evaluated. Results:With the newly established technique, the detection time for 2019-nCoV nucleic acid could be minimized to 6 minutes. In addition, the detection limit was 14 copies/μl when assisted by the displaying instrument, whereas it increased to 28 copies/μl with the naked eye. This technique had a sensitivity and specificity of 98.5% (66/67) and 100% (46/46) respectively, showing no statistically significant difference compared to the gold standard qPCR( P=1). Conclusions:This study has successfully established a CRISPR/Cas13a-based visually rapid detection technique for 2019-nCoV nucleic acid. This technique offers the advantages of a simple process, convenient operation, low environmental operating requirements, a detection limit close to qPCR, and a strong potential for on-site testing applications.

OBJECTIVE: To evaluate the early effectiveness of a new minimally invasive plate in the treatment of varus-type ankle arthritis. METHODS: A clinical data of 15 patients with varus-type ankle arthritis who met the selection criteria between March 2021 and October 2021 were retrospectively analyzed. All the patients were treated with medial open-wedge supramalleolar osteotomy and fibular osteotomy. The osteotomies were fixed with the new minimally invasive plate. There were 7 males and 8 females with an average age of 49.8 years (range, 16-71 years). The causes of ankle arthritis included post-fracture deformity in 1 case, sprain in 8 cases, and acquired clubfoot in 1 case; and 5 cases were without obvious factors. The disease duration ranged from 1 to 12 years, with an average of 4.1 years. Comparisons were made between pre-operation and the last follow-up in the Takakura staging, the American Orthopedic Foot and Ankle Society (AOFAS) ankle-hindfoot score, foot function index (FFI), visual analogue scale (VAS) score, tibial anterior surface angle (TAS), tibial lateral surface angle (TLS), and talar tilt (TT). RESULTS: All incisions healed by first intention. All patients were followed up 7-18 months (mean, 12.8 months). At last follow-up, the AOFAS ankle-hindfoot score, FFI, VAS score, and Takakura staging significantly improved when compared with the preoperative ones ( P<0.05). X-ray films showed that the osteotomy healed at 3 months after operation. At last follow-up, TAS significantly increased and TT decreased when compared with the preoperative ones ( P<0.05), and the difference in TLS between pre- and post-operation was not significant ( P>0.05). Complications included 1 case of intraoperative screw breakage and 2 cases of nerve injury of the affected foot. None of the patients complained of significant discomfort at the plate placement during follow-up, and no loosening of the internal fixator occurred. Eleven patients were very satisfied with the effeectiveness, while 4 were relatively satisfied. CONCLUSION The new minimally invasive plate for the varus-type ankle arthritis has good early effectiveness in relieving ankle pain, correcting deformity, improving limb alignment and ankle function, and reducing the incidence of postoperative incisional complications.
Female ; Humans ; Male ; Middle Aged ; Ankle ; Ankle Joint/surgery* ; Osteoarthritis/surgery* ; Retrospective Studies ; Tibia/surgery* ; Treatment Outcome ; Adolescent ; Young Adult ; Adult ; Aged

Objective:To explore the short- and mid-term clinical outcomes of surgical treatment of chronic Lisfranc injuries.Methods:A retrospective analysis was conducted of the clinical and imaging data of the 19 patients with chronic Lisfranc injury who had been treated surgically from April 2013 to September 2020 at Department of Foot and Ankle Surgery, Beijing Jishuitan Hospital. There were 13 males and 6 females, aged from 25 to 58 years (mean, 49 years). The median course of disease before operation was 24 months (from 3 to 312 months). All patients underwent fusion of the 1st, 2nd and 3rd tarsometatarsal joints (TMTJ) plus reduction and fixation or arthroplasty of the 4th and 5th TMTJ by the same surgeon. The clinical outcomes were evaluated by comparison of the American Orthopaedic Foot and Ankle Society (AOFAS) midfoot score, visual analogue scale (VAS) and Lublin score between pre-operation and the final follow-up and by Likert satisfaction scale and post-operative complications as well.Results:The 19 patients were followed up for (66.1±23.2) months (from 12 to 104 months). At the final follow-up, the AOFAS midfoot score was 87 (79, 90), the VAS score 1 (0, 2), and the Lublin score 70 (60, 75), all significantly improved compared with their preoperative corresponding values [51 (44, 63), 4 (4, 6) and 50 (40, 55), respectively] ( P<0.05). Sixteen patients were satisfied with their surgery. Fusion was not healed in 3 cases; plate breakage happened in one case but none of the cases had infection or other complications related to the skin or wound. Conclusion:Fusion of the 1st, 2nd and 3rd TMTJ plus reduction and fixation or arthroplasty of the 4th and 5th TMTJ can lead to good short- and mid-term clinical outcomes in the surgical treatment of chronic Lisfranc injuries.

Objective:To evaluate the clinical and radiographic outcomes of tibiotalocalcaneal arthrodesis via the fibula-preserving anterolateral approach.Methods:From March 2018 to February 2021, 23 patients underwent tibiotalocalcaneal arthrodesis via the fibula-preserving anterolateral approach at the Department of Foot and Ankle Surgery, Beijing Jishuitan Hospital.Of them, 15 were males and 8 females. Their average age at surgery was 54.4 years (range, from 28 to 72 years). There were 11 cases of traumatic arthritis, 2 cases of Charcot arthropathy, 6 cases of neurogenic equinovarus, 2 cases of traumatic equinovarus, and 2 cases of Kashin-Beck disease. Comparisons were made between preoperation and the last follow-up in ankle-hindfoot score of American Orthopedic Foot and Ankle Society (AOFAS), visual analogue scale (VAS), foot function index (FFI), hindfoot alignment angle (HA), hindfoot alignment distance (HD), and hindfoot alignment ratio (HR). The tibia-foot angle on lateral weight-bearing and patient satisfaction at the last follow-up and postoperative complications were documented.Results:This cohort were followed up for an average of 24.7 months (from 12 to 48 months). The AOFAS ankle-hindfoot score (78.2 ± 9.2), VAS [2.0 (0.5, 2.0)], FFI [19.0 (10.5, 35.0)], HA (2.7° ± 5.8°), HD [(0.1 ± 0.8) cm] and HR [44.2 (36.4, 59.2)%] at the last follow-up were significantly improved than the preoperative values [43.4 ± 12.7, 4.0 (4.0, 6.0), 98.0 (60.0, 127.0), 22.0° ± 14.3°, (2.2 ± 1.6) cm and 86.0 (66.3, 100.0)%] (all P<0.05). The tibia-foot angle on lateral weight-bearing was 89.0° (87.1°, 90.4°) at the last follow-up. By the Likert scale, 17 cases were very satisfied with the surgery, 4 cases satisfied and 2 cases tolerate, giving a satisfaction rate of 91.3%(21/23). Infection occurred in one case and nonunion in one. Conclusion:Tibiotalocalcaneal arthrodesis via the fibula-preserving anterolateral approach yields good mid- to short-term clinical and radiographic outcomes, showing a strong capability to correct ankle and hindfoot varus and equinus.

Objective:To evaluate the radiologic and clinical outcomes of supramalleolar dome osteotomy in the treatment of varus-type ankle arthritis.Methods:From June 2018 to December 2019, 13 patients with varus-type ankle arthritis underwent supramalleolar dome osteotomy at Department of Foot and Ankle Surgery, Beijing Jishuitan Hospital. Of them, 13 (5 males and 8 females) were included in the study. Their average age at surgery was 47.2 years (range, from 16 to 65 years). By the modified Takakura staging for arthritis, 3 cases were stage 2, 4 cases stage 3a and 6 cases stage 3b. Comparisons were made between preoperation and the last follow-up in ankle-hindfoot score of American Orthopedic Foot and Ankle Society (AOFAS), foot function index (FFI), visual analogue scale (VAS), Takakura staging, dorsal extension, plantar flexion, range of motion (ROM), tibial anterior surface angle (TAS), tibial lateral surface angle (TLS), and talar tilt (TT).Results:This cohort were followed up for 12 to 29 months (average, 17.2 months). At the last follow-up, AOFAS score (86.5±8.0), FFI (14.5±1.6), VAS score (1.5±1.0), Takakura staging (2.7±1.3), ROM (39.4°±8.0°), TAS (92.4°±6.0°), TT (3.7°±4.4°) were significantly improved than the preoperative values (68.3±14.4, 43.7±3.0, 4.2±1.4, 3.2±0.8, 43.3°±7.6°, 78.2°±8.3° and 7.0°±5.1°), and dorsal extension (10.9°±4.4°) was significantly smaller than the preoperative value (13.6°±5.1°) (all P<0.05). There were no significant differences between preoperation and the last follow-up in plantar flexion (29.7°±4.6° versus 28.5°±5.2°) or TLS (77.8°±4.5° versus 78.1°±5.3°) ( P>0.05). Conclusions:Supramalleolar dome osteotomy has shown promising short-term clinical efficacy in the treatment of varus-type ankle arthritis. It can significantly relieve pain, improve function, substantially correct deformity and retard progression of arthritis, but it may affect dorsal extension.

It is very important for epilepsy treatment to distinguish epileptic seizure and non-seizure. In this study, an automatic seizure detection algorithm based on dual density dual tree complex wavelet transform (DD-DT CWT) for intracranial electroencephalogram (iEEG) was proposed. The experimental data were collected from 15 719 competition data set up by the National Institutes of Health (NINDS) in Kaggle. The processed database consisted of 55 023 seizure epochs and 501 990 non-seizure epochs. Each epoch was 1 second long and contained 174 sampling points. Firstly, the signal was resampled. Then, DD-DT CWT was used for EEG signal processing. Four kinds of features include wavelet entropy, variance, energy and mean value were extracted from the signal. Finally, these features were sent to least squares-support vector machine (LS-SVM) for learning and classification. The appropriate decomposition level was selected by comparing the experimental results under different wavelet decomposition levels. The experimental results showed that the features selected in this paper were different between seizure and non-seizure. Among the eight patients, the average accuracy of three-level decomposition classification was 91.98%, the sensitivity was 90.15%, and the specificity was 93.81%. The work of this paper shows that our algorithm has excellent performance in the two classification of EEG signals of epileptic patients, and can detect the seizure period automatically and efficiently.
Algorithms ; Electroencephalography ; Epilepsy/diagnosis* ; Humans ; Seizures/diagnosis* ; Signal Processing, Computer-Assisted ; Support Vector Machine ; Wavelet Analysis