Erythropoietic protoporphyria （EPP） is a rare inherited metabolic disease that often involves skin， blood， and nervous systems， and EPP with the main manifestations of severe liver damage and acute abdominal pain is extremely rare. By reviewing the clinical data and genetic testing results of a patient with EPP， this article discusses the clinical features and pathogenic genes of this disease， in order to improve the understanding of the disease among hepatologists and achieve early diagnosis and treatment.

Myasthenia gravies is a common disease in the clinic. Extended thymectomy is an important way to treat myasthenia gravis. Median thoracotomy, thoracoscopy, and robots are important surgical methods. Da Vinci robot-assisted thoracoscopic surgery is more and more widely used in extended thymectomy, with high surgical safety and good stability. The surgical approach includes intercostal approach, subxiphoid approach, etc. Different surgical approaches have their own advantages, and their surgical effects are different. This article introduces the indications, technical steps, and effects of da Vinci robot-assisted thoracoscopic surgery, analyzes the advantages and limitations of treating myasthenia gravis, and looks forward to its development prospects.

Objective:To investigate the effect of serine/arginine-rich splicing factor 2 (SRSF2) on ferroptosis and its possible mechanism in glioblastoma cells.Methods:The online database of gene expression profiling interactive analysis 2 (GEPIA 2) and Chinese Glioma Genome Atlas were used to analyze the expression of SRSF2 in glioblastoma tissue and its association with patients prognosis. To validate the findings of the online databases, the pathological sections of glioblastoma and non-tumor brain tissues from Tianjin Medical University General Hospital, Tianjin, China were collected and analyzed by using immunohistochemistry. Silencing SRSF2 gene expression in glioblastoma cells by siRNA was analyzed with Western blot. The proliferation index was detected by using CCK8 assay. The rescued experiment was conducted by using expression plasmid of pcDNA3.1(+)-SRSF2. The activity of ferroptosis was assessed by using the levels of iron ions and malondialdehyde in glioblastoma cells and the changes in the ratio of glutathione to oxidized glutathione. The changes of gene expression and differential pre-mRNA alternative splicing (PMAS) induced by SRSF2 were monitored by using the third-generation sequencing technology analysis, namely Oxford nanopore technologies (ONT) sequencing analysis.Results:SRSF2 expression was higher in glioblastoma tissues than non-tumor brain tissues. Immunohistochemistry also showed a positive rate of 88.48%±4.60% in glioblastoma tissue which was much higher than the 9.97%±4.57% in non-tumor brain tissue. The expression of SRSF2 was inversely correlated with overall and disease-free disease survivals ( P<0.01). The proliferation index of glioblastoma cells was significantly reduced by silencing with SRSF2 siRNA ( P<0.01) and could be reversed with transfection of exogenous SRSF2. The levels of intracellulariron ions and malondialdehyde increased ( P<0.05), but the glutathione/oxidized glutathione ratio and the expression of key proteins in the glutathione pathway remained unchanged ( P>0.05). ONT sequencing results showed that silencing SRSF2 in glioblastoma cells could induce a significant alternative 3' splice site change on ferroptosis suppressor protein 1 (FSP1). Conclusion:SRSF2 inhibits the ferroptosis in glioblastoma cells and promotes their proliferation, which may be achieved by regulating FSP1 PMAS.

Objective To analyze the risk factors affecting the prognosis of patients with advanced gastric cancer,so as to provide evidence for identifying high-risk populations and guiding clinical treatment.Methods The clinical data of 282 patients with advanced gastric cancer who admitted to Zhoushan Hospital of Traditional Chinese Medicine from January 2017 to December 2021 were retrospectively analyzed.The survival outcome of patients was obtained by telephone follow-up and regular review.Kaplan-Meier method was used to analyze the overall survival(OS).Univariate and multivariate COX regression models were used to analyze the effects of clinical features on OS in patients with advanced gastric cancer.Results The median OS of 282 patients with advanced gastric cancer was 10.0 months.Univariate analysis showed that first-line chemotherapy,traditional Chinese medicine treatment,body mass index(BMI),Karnofsky performance status(KPS)score,hemoglobin(Hb)and albumin(Alb)were significantly correlated with the survival of patients with advanced gastric cancer(P<0.05).Multivariate COX regression models analysis showed that first-line chemotherapy,BMI,KPS score and Alb were independent risk factors affecting the survival of patients with advanced gastric cancer(P<0.05).Conclusion BMI,Alb,KPS score and first-line chemotherapy are independent risk factors for predicting the prognosis of patients with advanced gastric cancer,indicating nutrition management may be an important way to improve the outcome of patients with advanced gastric cancer.

OBJECTIVE To explore the intestinal absorption characteristics of saikosaponins. METHODS Based on everted intestinal sac model， using accumulative absorption amount （Q） and absorption rate constant （Ka） as indexes， UHPLC-MS/MS technique as a method， the absorption of saikosaponin A， B2， C， D and F from total saponins of Bupleurum chinense （8 g/mL， by crude drug） in the duodenum， jejunum and ileum was detected. RESULTS The correlation coefficients （r） of the regression equations for the absorption of saikosaponins A， B2， C and F in the duodenum， jejunum and ileum were all higher than 0.95， while the r of saikosaponin D in the above intestinal segments was lower than 0.95； compared with the absorption of the same composition in the duodenum， the Q and Ka of saikosaponin A and C circulating in jejunum and ileum for 120 min， as well as the Q and Ka of saikosaponin F circulating in the ileum for 120 min were significantly decreased （P＜0.05）. CONCLUSIONS Saikosaponin A and the other 4 saikosaponins are all absorbed in the duodenum， jejunum and ileum； among them， saikosaponin A， B2， C and F are linearly absorbed， which conforms to the zero-order absorption characteristics， but saikosaponin D shows non- linear absorption.

OBJECTIVE: To establish the finite element model of varus-type ankle arthritis and to implement the finite element mechanical analysis of different correction models for tibial anterior surface angle (TAS) in supramalleolar osteotomy. METHODS: A female patient with left varus-type ankle arthritis (Takakura stage Ⅱ, TAS 78°) was taken as the study object. Based on the CT data, the three-dimensional model of varus-type ankle arthritis (TAS 78°) and different TAS correction models [normal (TAS 89°), 5° valgus (TAS 94°), and 10° valgus (TAS 99°)] were created by software Mimics 21.0, Geomagic Wrap 2021, Solidworks 2017, and Workbench 17.0. The 290 N vertical downward force was applied to the upper surface of the tibia and 60 N vertical downward force to the upper surface of the fibula. Von Mises stress distribution and stress peak were calculated. RESULTS: The finite element model of normal TAS was basically consistent with biomechanics of the foot. According to biomechanical analysis, the maximum stress of the varus model appeared in the medial tibiotalar joint surface and the medial part of the top tibiotalar joint surface. The stress distribution of talofibular joint surface and the lateral part of the top tibiotalar joint surface were uniform. In the normal model, the stress distributions of the talofibular joint surface and the tibiotalar joint surface were uniform, and no obvious stress concentration was observed. The maximum stress in the 5° valgus model appeared at the posterior part of the talofibular joint surface and the lateral part of the top tibiotalar joint surface. The stress distribution of medial tibiotalar joint surface was uniform. The maximum stress of the 10° valgus model appeared at the posterior part of the talofibular joint surface and the lateral part of the top tibiotalar joint surface. The stress on the medial tibiotalar joint surface increased. CONCLUSION With the increase of valgus, the stress of ankle joint gradually shift outwards, and the stress concentration tends to appear. There was no obvious obstruction of fibula with 10° TAS correction. However, when TAS correction exceeds 10° and continues to increase, the obstruction effect of fibula becomes increasingly significant.
Humans ; Female ; Tibia/surgery* ; Finite Element Analysis ; Ankle ; Arthritis ; Fibula/surgery* ; Ankle Joint/surgery*

Objective: To investigate epidemiological characteristics of cancers in Zhoushan City in 2018, so as to provide the evidence for the formulation of cancer control strategies. Methods: The cancer data in Zhoushan City in 2018 were captured from Zhejiang Provincial Information Management System for Chronic Disease Surveillance. The gender- and age-specific incidence of cancers was descriptively analyzed. The cancer incidence was standardized to the data of the Sixth National Population Census in China in 2010 ( Chinese standard population ) and Segi's world standard population. Results: The crude incidence rate, age-standardized incidence rate by Chinese standard population ( ASR China ), and age-standardized incidence rate by world standard population ( ASR world ) of cancers were 468.65/105, 300.12/105 and 232.79/105 in Zhoushan City in 2018, and the cumulative cancer incidence rate was 26.91% among residents at ages of 0 to 74 years, while the truncated-age cancer incidence rate was 431.90/105 among residents at ages of 35 to 64 years. The crude incidence rate, ASR China, and ASR world of cancers were 473.00/105, 285.67/105 and 229.03/105 in men, and 464.43/105, 317.66/105 and 241.30/105 in women in Zhoushan City in 2018, respectively. In addition, the mean age of new cases with cancers in 2018 was ( 61.34±13.19 ) years in Zhoushan City in 2018, and the highest crude cancer incidence was seen in residents at ages of 70 to 74 years ( 1 327.50/105 ). The crude cancer incidence rate was higher in new female cases at ages of 25 to 54 years than in new male cases, and the crude cancer incidence rate was higher in new male cases at ages of 60 to 84 years than in new female cases ( P<0.05 ). The 10 most common cancers included lung cancer, female breast cancer, thyroid cancer, gastric cancer, colorectal cancer, liver cancer, cervical cancer, prostatic cancer, pancreatic cancer and bladder cancer, which accounted for 80.20% of all cancers in Zhoushan City in 2018. Conclusions High cancer incidence was found in Zhoushan City in 2018, and elderly populations are at high risk of developing cancers. The management of lung cancer, female breast cancer, thyroid cancer, gastric cancer and colorectal cancer should be given a high priority.

Objective:To propose a new classification of posterior malleolus fracture to further clarify its pathoanatomy.Methods:Twenty fresh frozen cadaver specimens of normal morphology of lower limbs were selected and dissected and the extent of the tibial insertion of posterior malleolus associated ligaments was measured. At the same time, a retrospective case series analysis was made on the clinical and CT information of 296 patients with posterior malleolus fracture treated at Tongji Hospital of Tongji University from January 2012 to July 2020 or at Karamay Central Hospital from January 2018 to July 2020. The percentage of articular involvement of the fracture, proximal displacement of the posterior malleolus fracture and extent of posterior talar subluxation were measured. A clinically practical new classification system for posterior malleolus fracture was created by correlating posterior malleolus associated ligaments with CT images of posterior malleolus fracture. The new classification included three types: type I was posterior malleolus fracture with only the tibial insertion of inferior transverse tibiofibular ligament involved; type II was posterior malleolus fracture with both the tibial insertions of inferior transverse tibiofibular ligament and posterior inferior tibiofibular ligament involved, which was divided into subtypes IIA and IIB based on the presence of articular cartilage and subchondral bone damage, compression or Die-Punch fragments; type III was posterior malleolus fracture that involved all the tibial insertions of inferior transverse tibiofibular ligament, posterior inferior tibiofibular ligament and posterior tibiotalar ligament, which was sub-classified into subtypes III A and III B according to number of fracture fragments. Anatomic characteristics of the extent of the tibial insertion of posterior malleolus associated ligament, CT imaging parameters for posterior malleolus fracture and corresponding fracture typing were determined. In addition, the new classification system for posterior malleolus fracture was compared with Haraguci classification and Mason classification.Results:Posterior malleolus associated ligaments included the posterior inferior tibiofibular ligament, inferior transverse tibiofibular ligament and posterior tibiotalar ligament from posterolateral to posteromedial tibia. The posterior inferior tibiofibular ligament was attached to the posterolateral tibia and the distance between the highest point of its tibial insertion and the joint line was (45.2±5.6)mm. The inferior transverse tibiofibular ligament was attached to the posterior distal tibia and the distance between the highest point of its tibial insertion and the joint line was (5.5±1.0)mm. The posterior tibiotalar ligament was attached to the posterior colliculus and intercollicular groove of the medial malleolus and the distance between the center of its tibial insertion and the intercollicular groove was (2.5±0.6)mm. Among 296 patients with posterior malleolus fracture, there were 36 patients with type I, 229 with type II (150 type IIA, 79 type IIB) and 31 with type III (11 type IIIA, 20 type IIIB). The percentage of articular involvement of the fracture, proximal displacement of posterior malleolus fracture and extent of posterior talar subluxation in type IIB fracture were significantly greater than those in type II A fracture [23.7(18.6, 28.8)% vs. 18.4(12.7, 21.7)%, 4.1(2.1, 6.0)mm vs. 1.9(0.2, 3.0)mm, 4.7(1.5, 6.2)mm vs. 2.3(1.1, 3.0)mm] (all P<0.01). The proximal displacement of posterior malleolus fracture and extent of posterior talar subluxation in type III fracture were significantly greater than those in type II fracture [7.2(6.0, 8.2)mm vs. 2.7(0.4, 4.0)mm, 10.1(6.0, 15.0)mm vs. 3.1(1.1, 5.0)mm] (all P<0.01). The new classification for posterior malleolus fracture combined the posterior malleolus ligament and injury mechanism of posterior malleolus fracture as compared with Haraguchi classification, which not only further detailed the classification, but also was more practical in clinic for increased the severity of injury was elevated with higher classification level. The new classification was more comprehensive as compared with Mason classification for it mainly added the type of simple-rotation-type posterior malleolus fracture (type IIA of the new classification). Conclusions:In combination with posterior malleolus associated ligaments, injury mechanism and fracture morphology, posterior malleolus fracture is divided into three types. The new classification system more comprehensively describes pathoanatomy of posterior malleolus fracture that contributes to related basic research and clinical diagnosis and treatment.

Objective:To analyze the correlation between the posterior malleolus fracture and fixation and the rotational stability of the ankle and to explore the surgical indications for posterior malleolus fracture aiming to provide the theories for the diagnosis and treatment of disorder.Methods:Twenty fresh frozen cadaver specimens were selected. Further, the extent of the tibial insertion of the posterior inferior tibiofibular ligament (PITFL) and inferior transverse tibiofibular ligament (ITTFL) complex was dissected and measured. Based on the tibial insertion of the ligament complex, the model for the supination-external rotation degree 3 ankle fracture with a posterior malleolar fragment and syndesmosis diastasis was created. Moreover, the area threshold of the posterior tibial insertion of posterior malleolus fracture was biomechanically assessed. The difference of the antirotating ability of the ankle-stiffness between simple posterior malleolus fixation and simple syndesmotic fixation was analyzed statistically.Results:The PITFL and ITTFL were presented in all specimens with relatively broad in PITFL tibial insertion. The PITFL was attached to the posterolateral tibia. The distance between the highest point of the tibial insertion and the articular line was 45.2±5.6 mm, while the ITTFL was attached to the posterior distal tibia. The distance between the highest point of the tibial insertion and the articular line was 5.5±1.0 mm. The width of the tibial insertion of the PITFL and ITTFL complex decreased as the distance from the joint line increased. Biomechanical analysis showed that the threshold of posterior area of posterior malleolus fracture was 1/4S. The stiffnesses of posterior malleolus fixation and syndesmosis stabilization were 0.264±0.080 N·m/° and 0.164 ± 0.061 N·m/°, respectively. The percentage of stiffness restored by posterior ankle fixation was 60.9%±10.2%, which was greater than that by syndesmosis stabilization 37.5%±7.9% ( t=17.09, P<0.001) . Conclusion:The surgical technique for posterior malleolus fracture should consider restoration of the axial and rotational stability of the ankle simultaneously. Posterior malleolus fracture fixation is recommended when the syndesmosis is unstable with the area ratio of posterior tibial insertion of posterior malleolus fracture greater than or equal to 1/4. Syndesmotic fixation is proposed to restore and maintain the rotational stability of the ankle when the syndesmosis is unstable with the area ratio less than 1/4. Regardless of the area ratio, the surgical indications for stable syndesmosis depend on the impact of the posterior malleolus fracture on the axial stability of tibiotalar joint, on the involved articular surface area and on the displacement degree of posterior malleolus fragment.

Objective:To investigate the long-term survival and safety in patients with muscle-invasive bladder cancer (MIBC) who experienced a noninvasive down-staging (≤pT 1)after transurethral resection of bladder tumor (TURBT) plus systemic chemotherapy and received bladder-sparing treatment. Methods:The records of patients with MIBC who underwent maximal TURBT plus systemic chemotherapy-guided bladder-sparing treatment were reviewed retrospectively from Dec 2013 to Dec 2020. Eventually, 22 patients who achieved noninvasive down-staging underwent conservative management. The total patient cohort contained 10 males and 12 females. A majority of patients had single lesion and stage T2 disease. The median age of the patients was 66 years and the median tumor size was 3.0 cm. All patients underwent maximal TURBT to resect all visible diseases and followed by 3-4 cycles platinum-based systemic chemotherapy. After achieving noninvasive down-staging, 14 patients received concurrent chemoradiotherapy, and the other 8 patients underwent surveillance. Overactive bladder symptom score (OABSS) was used to assess the bladder function after treatment.Results:Twelve patients achieved pT 0 and 10 patients were down-staged to cT a-T 1. At a median follow-up of 36.7 months, 90.9%(20/22) patients retained their bladder function successfully. Among the 14 patients who received concurrent chemoradiotherapy, 4 had grade 3 or 4 adverse events. Among the 8 patients who underwent surveillance, 3 had grade 3 or 4 adverse events after systemic chemotherapy.Nine patients experienced tumor recurrence in the bladder, and 2 patients died of bladder cancer. Seven (31.8%) patients experienced Ⅲ/Ⅳ grade complications. The 5-year recurrence-free survival (RFS) and overall survival (OS) in patients achieved pT0 were 66.7% and 100.0%, respectively. The 5-year RFS and OS in patients achieved cTa-T1 were 40% and 72%, respectively. The OABSS score of 20 patients who retained their bladder successfully was (1.00±1.03). Conclusions:MIBC patients who achieved noninvasive down-staging might be candidates for the bladder-sparing treatment with maximum TURBT followed by systemic chemotherapy.The patients who achieved pT 0 might have better prognosis with functional bladder.