Objective:To explore the genetic background and clinical features of patients with long QT syndrome type 3 (LQT3).Methods:This retrospective cohort included patients diagnosed with LQT3 at the Department of Cardiology, Renmin Hospital of Wuhan University from January 1998 to December 2022. Patients were categorized into compound type group and single type group based on the presence of a single SCN5A mutation. The two groups were followed up and the differences in baseline characteristics, electrocardiograms, and clinical events between the two groups and probands were compared. Kaplan-Meier curves were used for survival analysis, and the log-rank test was employed to compare the event-free survival rates of first cardiac events between the groups and probands.Results:A total of 97 LQT3 patients were enrolled, including 59 probands. The age at diagnosis was (23.45±19.86) years, with 46 patients (47.4%) being male. Among them, 89 patients were classified as single type group, while 8 patients were classified as compound type group. Genetic testing identified 49 SCN5A mutations, with missense mutations being the majority (91.8%), primarily located in transmembrane regions (40.8%, n=20), interdomain linker regions (28.6%, n=14), and C-terminus (22.4%, n=11). The first cardiac event occurred in 44 patients (45.4%), with an onset age of (13.82±12.50) years. The main trigger was identified as rest or sleep (54.5%, n=24). Compared with patients in single type group, patients in compound type group were younger at diagnosis ((10.35±10.28) years vs. (24.63±20.13) years, P=0.040), had a significantly higher proportion of syncope (87.5% (7/8) vs. 33.7% (30/89), P=0.009), aborted cardiac arrest (62.5% (5/8) vs. 11.2% (10/89), P=0.001), and a lower incidence of event-free survival rates of first cardiac events (12.5% (1/8) vs.58.4% (52/89), log-rank P=0.001). The probands in compound type group had a significantly higher proportion of aborted cardiac arrest comparing to probands in single type group (62.5% (5/8) vs. 17.6% (9/51), P=0.020), while the difference in the incidence rate of event-free survival rates of first cardiac events between the probands in two groups was not statistically significant (12.5% (1/8) vs. 39.2% (20/51), log-rank P=0.08). Conclusion:Compound type LQT3 patients are not uncommon. Such patients are diagnosed at a younger age and exhibit more severe phenotypes, requiring close follow-up and proactive intervention strategies.

Objective:To investigate the differences in clinical and electrocardiographic characteristics between carriers of SCN5A mutations and non-SCN5A mutations in fever-induced Brugada syndrome.Methods:This study is a retrospective cohort study. A total of 263 patients with fever-induced Brugada syndrome who were admitted to Renmin Hospital of Wuhan University from January 2000 to December 2023 were selected. Their clinical manifestations, electrocardiographic characteristics, and major adverse cardiovascular events (MACE) at the time of diagnosis and during the follow-up period were collected. Among them, 200 patients underwent next-generation sequencing. Based on the genetic variation results, after excluding other mutations, they were divided into SCN5A mutation group, non-SCN5A sodium-related mutation group, potassium/calcium mutation group, and no mutation group. Comparisons were made among these groups in terms of their clinical and electrocardiographic characteristics.Results:Among the 263 patients with fever-induced Brugada syndrome, the mean age was (41.9±17.6) years, with 80.6% (212/263) being male. The median follow-up duration was 53.0 months, and 13.7% (36/263) of the patients experienced MACE. The rate of SCN5A mutation was 34.5% (69/200), while the rates of non-SCN5A sodium-related mutations and potassium/calcium-related mutations were 4.5% (9/200) and 3.5% (7/200), respectively. The SCN5A mutation group was younger than the non-SCN5A sodium-related mutation group and the no mutation group (ages were (33.8±14.7), (49.8±11.6), (44.6±15.7) years, respectively, P<0.001). The SCN5A mutation group also had a longer PR interval than the no mutation group ((176.8±32.3) ms vs. (163.9±28.6) ms, P=0.034). The incidence of MACE was higher in the non-SCN5A sodium-related mutation group than that in the no mutation group (55.6% (5/9) vs. 9.1% (9/99), P=0.002). Conclusions:Fever-induced Brugada syndrome patients carrying non-SCN5A mutations exhibit distinct clinical and electrocardiographic characteristics compared to those with SCN5A mutations. These differences warrant attention in clinical practice.

OBJECTIVE: To investigate the early effectiveness of transiliac-transsacral screws internal fixation assisted by augmented reality navigation system HoloSight (hereinafter referred to as "computer navigation system") in the treatment of posterior pelvic ring injuries. METHODS: A retrospective analysis was made in the 41 patients with posterior pelvic ring injuries who had been treated surgically with transiliac-transsacral screws between June 2022 and June 2023. The patients were divided into navigation group (18 cases, using computer navigation system to assist screw implantation) and freehand group (23 cases, using C-arm X-ray fluoroscopy to guide screw implantation) according to the different methods of transiliac-transsacral screws placement. There was no significant difference in gender, age, body mass index, causes of injuries, Tile classification of pelvic fracture, days from injury to operation, usage of unlocking closed reduction technique between the two groups ( P>0.05). The time of screw implantation, the fluoroscopy times, the guide wire adjustment times of each screw, and the incidence of complications were recorded and compared between the two groups. The position of the transiliac-transsacral screw was scanned by CT within 2 days after operation, and the position of the screw was classified according to Gras standard. RESULTS: The operation was successfully completed in both groups. The time of screw implantation, the fluoroscopy times, and the guide wire adjustment times of each screw in the navigation group were significantly less than those in the freehand group ( P<0.05). There were 2 cases of incision infection in the freehand group, and the incision healed by first intention after active dressing change; there was no screw-related complication in the navigation group during operation and early period after operation; the difference in incidence of complications between the two groups (8.7% vs. 0) was not significant ( P=0.495). According to the Gras standard, the screw position of the navigation group was significantly better than that of the freehand group ( P<0.05). CONCLUSION Compared with the traditional freehand method, the computer navigation system assisted transiliac-transsacral screws internal fixation in the treatment of posterior pelvic ring injuries has advantages of improving the accuracy of screw implantation and reducing radiation damage and the time of screw implantation.
Humans ; Retrospective Studies ; Surgical Wound Infection ; Replantation ; Body Mass Index

Objective:To compare the long-term prognosis between open versus closed reduction and internal fixation in the treatment of unstable pelvic fractures.Methods:The data of 402 consecutive patients with unstable pelvic fracture were retrospectively analyzed who had been treated at The First Medical Center and The Fourth Medical Center, PLA General Hospital, and Strategic Support Force Specialty Medical Center from March 2011 to March 2017. This cohort was divided into 2 groups according to the reduction methods. In the open group of 194 cases subjected to open reduction and internal fixation, there were 133 males and 61 females with a median age of 43.0 (30.7, 51.0) years, and 35 cases of type B and 159 cases of type C by the Tile classification. In the closed group of 208 cases subjected to closed reduction and internal fixation, there were 115 males and 93 females with a median age of 45.5 (32.0, 56.0) years, and 40 cases of type B and 168 cases of type C by the Tile classification. The 2 groups were compared in terms of 12-items Short Form Health Survey (SF-12) scores [physical component summary (PCS) and mental component summary (MCS)] at the last follow-up, time from injury to operation, frequency of intraoperative X-ray fluoroscopy, intraoperative and postoperative blood transfusion, operation time, and quality of postoperative fracture reduction.Results:There was no statistically significant difference between the 2 groups in the preoperative general data except for the gender, showing the 2 groups were comparable ( P>0.05). This cohort of 402 patients was followed up for 7.8(6.2, 8.8) years. At the last follow-up, the PCS [49.9 (45.4, 55.4) points] and MCS [53.1 (46.4, 57.6) points] in the closed group were significantly higher than those in the open group [48.2 (41.4, 52.7) and 46.5 (40.6, 53.6) points] ( P<0.05). The closed group incurred significantly shorter time from injury to operation [6 (5, 8) d] and operation time [180 (126, 260) min] than the open group [9 (6, 13) d and 240 (165, 334) min], significantly less intraoperative and postoperative blood transfusion [1.5 (0, 4.0) U] than the open group [5.0 (2.9, 8.0) U], significantly higher frequency of intraoperative X-ray fluoroscopy [104.5 (85.0, 132.0) times] than the open group [21.0 (18.0, 26.0) times], and a significantly higher excellent and good rate of postoperative fracture reduction (92.8%, 193/208) than the open group (86.6%, 168/194) (all P<0.05). Conclusion:In the treatment of patients with unstable pelvic fractures, compared with open reduction and internal fixation, closed reduction and internal fixation can not only significantly shorten the waiting time and operation time of patients, reduce the transfusion during operation, but also achieve better fracture reduction to ultimately improve the quality of life of patients.

Objective:To evaluate the safety and efficacy of cytoreductive surgery plus hyperthermic intraperitoneal chemotherapy (CRS+ HIPEC) in patients with peritoneal carcinomatosis from colorectal carcinoma (CRC PC).Methods:The clinical and follow-up data of 90 consecutive CRC PC patients underwent CRS+ HIPEC in Beijing Shijitan Hospital from January 2015 to June 2018 were collected. Kaplan-Meier method and parallel Log rank test were used for survival analysis. Cox regression model was used for univariate and multivariate analysis.Results:A total of 90 CRC PC patients underwent CRS+ HIPEC, the median age was 53 years (rage: 13 to 81 years), and 51 cases were male, while other 39 were female. The median overall survival (mOS) was 21.9 months (95%CI: 15.7, 28.1). The 1-, 2-, 3-, and 5-year survival rates were 77.8%, 48.6%, 21.1%, and 5.5%, respectively. The incidence rate of serious adverse event (SAE) was 8.9% (8/90). The mortality rate of perioperative period was 2.2% (2/90). Univariate analysis showed the age ( P=0.040), primary tumor site ( P=0.020), preoperative carbohydrate antigen 125 (CA125) level ( P<0.001), peritoneal cancer index (PCI) ( P<0.001), completeness of cytoreduction (CC) ( P<0.001), ascites ( P=0.012) and postoperative adjuvant chemotherapy ( P<0.001) were significantly associated with the OS. Multivariate Cox-analysis identified preoperative CA125 level( P=0.033), CC of 0 to 1 ( P=0.014), and adjuvant chemotherapy postoperative ( P=0.002) were independent prognostic factor for OS. Conclusions:CRS+ HIPEC can improve survival for CRC PC patients with acceptable morbidity and mortality. Stringent patient selection and complete CRS are two key factors for better survival.

Objective:To evaluate the safety and efficacy of cytoreductive surgery plus hyperthermic intraperitoneal chemotherapy (CRS+ HIPEC) in patients with peritoneal carcinomatosis from colorectal carcinoma (CRC PC).Methods:The clinical and follow-up data of 90 consecutive CRC PC patients underwent CRS+ HIPEC in Beijing Shijitan Hospital from January 2015 to June 2018 were collected. Kaplan-Meier method and parallel Log rank test were used for survival analysis. Cox regression model was used for univariate and multivariate analysis.Results:A total of 90 CRC PC patients underwent CRS+ HIPEC, the median age was 53 years (rage: 13 to 81 years), and 51 cases were male, while other 39 were female. The median overall survival (mOS) was 21.9 months (95%CI: 15.7, 28.1). The 1-, 2-, 3-, and 5-year survival rates were 77.8%, 48.6%, 21.1%, and 5.5%, respectively. The incidence rate of serious adverse event (SAE) was 8.9% (8/90). The mortality rate of perioperative period was 2.2% (2/90). Univariate analysis showed the age ( P=0.040), primary tumor site ( P=0.020), preoperative carbohydrate antigen 125 (CA125) level ( P<0.001), peritoneal cancer index (PCI) ( P<0.001), completeness of cytoreduction (CC) ( P<0.001), ascites ( P=0.012) and postoperative adjuvant chemotherapy ( P<0.001) were significantly associated with the OS. Multivariate Cox-analysis identified preoperative CA125 level( P=0.033), CC of 0 to 1 ( P=0.014), and adjuvant chemotherapy postoperative ( P=0.002) were independent prognostic factor for OS. Conclusions:CRS+ HIPEC can improve survival for CRC PC patients with acceptable morbidity and mortality. Stringent patient selection and complete CRS are two key factors for better survival.

Objective:To explore the pathological characteristics and outcomes of elderly patients with community acquired pneumonia (CAP) accompanied by coronavirus disease 2019 (COVID-19).Methods:The diagnosis and treatment process of one elderly patient with CAP accompanied by COVID-19 who was admitted to COVID-19 Treatment Center of Liaoning Province on February 7, 2020 were reviewed. The experience of treatment by analyzing the characteristics of such type of patients during diagnosis and treatment were summarized.Results:A female patient, aged 79 years ald, was admitted to the Center with following features: fever, dry cough, fatigue with dyspnea, scattered moist rales in both lungs, oxygenation index (PaCO 2/FiO 2) of 95 mmHg (1 mmHg = 0.133 kPa), and diffuse interstitial pneumonia in both lungs indicated by chest CT, of which the majority were ground glass-like and fibrous lesions. It was confirmed to be consistent with the feature of severe COVID-19 cases. The patient was successfully cured one month later following anti-inflammatory, anti-viral and high-flow oxygen therapies, homeostasis maintenance of the body, psychological counseling, etc. Accordingly, the treatment experience in CAP combined with COVID-19 in the elderly patients was summarized as follows. In respiratory system, the timing of high-flow oxygen therapy and mechanical ventilation should be seized. As for anti-inflammatory and antiviral therapy, attention should be paid to the treatment of CAP as well as antiviral therapy and symptomatic and supportive therapy. With the progression of the disease, the production of drug-resistant bacteria and the possibility of fungal infection should be paid attention to. For the circulatory system, we should pay attention to the stability of fluid volume and internal environment, and strengthen hemodynamic monitoring and bedside ultrasound to evaluate the cardiovascular capacity-load. In the aspect of the immune system, the selection of the application time of immune-enhancers and glucocorticoids should be paid attention to. In terms of enteral nutrition, early low-fat and high-protein diet is conducive to the recovery of intestinal function and the prevention of bacterial translocation. In addition to the protection of the function of important organs, therapies such as reasonable sedation and psychological intervention should also be used. Conclusions:Elderly patients with CAP accompanied by COVID-19 have complicated conditions and high degree of difficulty in treatment. Comprehensive evaluation of the disease as well as synthetic and effective intervention are the key factors of successful treatment of such patients.

Objective:s To evaluate the impacts of prior surgical scores(PSS) on the clinical efficacy and perioperative safety of cytoreductive surgery(CRS) and hyperthermic intraperitoneal chemotherapy(HIPEC) for pseudomyxoma peritonei(PMP).Methods:From the comprehensive PMP database, we collect the cases treated for the first time by CRS+ HIPEC, to form this study cohort. The clinicopathological features, PSS, CRS+ HIPEC details, overall survival(OS), and serious adverse events(SAEs) are systematically analyzed, to study the correlations between PSS and OS or SAEs.Results:335 PMP cases received standardized CRS+ HIPEC in this study. The median OS is 58.2 months for PSS-0 patients, 63.7 months for PSS-1, and 55.4 months for PSS-2/3, with no statistically significant differences in OS among the different PSS groups(χ 2=0.499, P=0.779). Subgroup analysis by pathologic types also found no statistically significant differences among the different PSS groups. Moreover, no significantly statistical differences are observed in overall SAEs(χ 2=0.625, P=0.722), CRS-related SAEs(χ 2=0.267, P=0.901), and non-CRS-related SAEs(χ 2=0.677, P=0.715), among the different PSS groups. Conclusions:PSS does not pose significant impacts on the efficacy and safety of CRS+ HIPEC for PMP patients at experienced treatment center.

Objective:To evaluate the clinical efficacy and safety of cytoreductive surgery plus hyperthermic intraperitoneal chemotherapy (CRS+HIPEC) for patients with advanced ovarian cancer as the first-line surgical treatment.Methods:The ovarian cancer patients with completed clinical data diagnosed as stage Ⅲ C-Ⅳ according to Federation International of Gynecology and Obstetrics (FIGO) who underwent CRS+HIPEC as the first-line treatment from December 2007 to November 2019 in Beijing Shijitan Hospital were retrospectively analyzed. Survival status was analyzed by using Kaplan-Meier method, and prognostic factors were analyzed by using Cox multivariate regression model. The primary endpoints were median overall survival (mOS) time and median progress-free survival (mPFS) time, and the secondary endpoint was safety in perioperative period. Results:Of 100 patients with advanced ovarian cancer, the median follow-up time was 18.4 months, and 75 (75.0%) patients were alive and 25 (25.0%) patients died, of which the mOS time was 87.6 months (95% CI 72.1-103.1 months), and 1-, 2-, 3-, 4- and 5-year survival rate was 94.1%, 77.2%, 68.2%,64.2% and 64.2%, respectively. Univariate analysis showed that the patients with age≤58 years old ( P = 0.023), Karnofsky≥80 scores ( P = 0.026), ascites ≤1 000 ml ( P = 0.041), peritoneal carcinomatosis index (PCI) score <19 ( P = 0.044) and completeness of cytoreduction (CC) score 0-1 ( P = 0.001) had better prognosis. Multivariate analysis showed that CC score 0-1 was independent prognostic factor, the mortality risk of resectable patients with CC score 2-3 was 3.2 times higher than that in patients with CC score 0-1 ( HR = 3.2, 95% CI 1.4-7.6, P = 0.008), and mPFS time was 23.3 months (95% CI 0-50.7 months) for patients with CC score 0-1. Grade Ⅲ-Ⅳ adverse event rate during perioperative period and mortality rate was 4.0% (4/100) and 2.0% (2/100), respectively. Conclusion:CRS+HIPEC could improve the survival of advanced ovarian cancer patients with good safety.

Objective:This study was to investigate the perioperative safety of cytoreductive surgery (CRS) plus hyperthermic intraperitoneal chemotherapy (HIPEC) for pseudomyxoma peritonei (PMP), and analyze the risk factors of serious adverse events (SAEs).Methods:The occurrences of perioperative SAEs were retrospectively analyzed in 254 PMP patients treated with CRS plus HIPEC. Univariate and multivariate analysis were performed to identify independent risk factors.Results:Among the 272 CRS plus HIPEC procedures for 254 PMP patients, a total of 93 (34.2%) perioperative SAEs occurred, including 26 in infection, 22 in digestive system, 17 in respiratory system, 15 in cardiovascular system, 8 in hematological system, and 4 in urinary system. In terms of severity, the vast majority was grade Ⅲ with 76 cases, followed by grade Ⅳ with 13 cases and grade Ⅴ with 4 cases. Univariate analysis revealed 3 risk factors of perioperative SAEs: HIPEC regimen ( P=0.020), intraoperative red blood cell transfusion volume ( P=0.004), and intraoperative blood loss volume ( P=0.002). Multivariate analysis by logistic regression model analysis revealed that intraoperative red blood cell transfusion volume was an independent risk factor for perioperative SAEs ( OR=1.160, P=0.001). Conclusion:In conclusion, the perioperative safety of CRS plus HIPEC was acceptable. Moreover, intraoperative blood loss volume and red blood cell transfusion volume are expected to be reduced in order to prevent SAEs for PMP patients.