Clinical study of self-designed Yiqi Huoxue Huayu Decoction combined with western conventional therapy in the treatment of chronic heart failure complicated with atrial fibrillation / 国际中医中药杂志

Objective:To observe the effects of self-designed Yiqi Huoxue Huayu Decoction on cardiac function and serum endothelin (ET-1) and matrix metalloproteinase-9 (MMP-9) levels in patients with chronic heart failure (GHF) complicated with atrial fibrillation.Methods:A total of 100 patients with GHF complicated with atrial fibrillation treated in our hospital from January 2019 to June 2021 were selected as the study subjects, and divided into experimental group and control group according to random number table method, with 50 patients in each group. Patients in both groups were given cardiotonic, diuretic, vasodilator and other conventional treatment, and patients in the experimental group added self-designed Yiqi Huoxue Huayu Decoction. TCM syndrome score, left ventricular end diastolic diameter (LVEDD), left ventricular end systolic diameter (LVESD) and left ventricular ejection fraction (LVEF), serum ET-1 and MMP-9 levels, clinical efficacy, safety and incidence of adverse reactions were observed and compared between 2 groups before and after treatment.Results:The total response rate was 82.0% (41/50) in the observation group and 62.0% (31/50) in the control group, and the difference was statistically significant ( χ 2=4.96, P=0.026). After treatment, the symptoms scores of palpitation, shortness of breath, fatigue, chest and hypochasm pain in the observation group were significantly significantly lower than those in the control group ( t=5.28, 5.29, 5.62, 5.42, P<0.01). After treatment, the LVEDD[(51.23±6.59)mm vs. (55.65±6.17)mm, t=3.46], LVESD[(43.10±4.76)mm vs. (45.99±5.31)mm, t=2.87], serum ET-1[(65.79±8.29)μg/L vs. (79.83±10.08)μg/L, t=7.61], MMP-9 [(175.86±24.81)ng/L vs. (189.49±26.13)ng/L, t=2.68] in experimental group were significantly lower than those in the control group ( P<0.05), while LVEF [(50.01±7.6)% vs. (46.25±6.96)%, t=2.57] was significantly higher than that of the control group ( P<0.05). There were no significant differences in GPT, GOT, UA, SCr levels between both groups before and after treatment ( P>0.05). There were no obvious adverse reactions betewwn both groups. Conclusion:The self-designed Yiqi Huoxue Huayu Decoction can relieve the clinical symptoms, improve the heart function and serum ET-1 and MMP-9 levels in patients with GHF complicated with atrial fibrillation safely.

A preliminary validation of the "lung surface intersegmental constant proportion landmarks" in identifying intersegmental planes during segmentectomy / 中国胸心血管外科临床杂志

@#Objective     To verify the feasibility and accuracy of the "lung surface intersegmental constant proportion landmarks", developed by our center, in identifying intersegmental planes during pulmonary segmentectomy. Methods    We prospectively enrolled the patients who planned to receive thoracoscopic segmentectomy in West China Hospital of Sichuan University and The Third People's Hospital of Chengdu from September 2021 to October 2021. We took a relatively objective and feasible method, intravenous injection of indocyanine green, in identifying intersegmental planes as standard control. We intraoperatively judged the consistency between "lung surface intersegmental constant proportion landmarks" and intravenous injection of indocyanine green in identifying intersegmental planes. We discerned main landmarks of intersegmental plane by the constant proportion segment module, which was built based on the "lung surface intersegmental constant proportion landmarks", as well as distinguished the planes with discrepant fluorescence by peripheral intravenous indocyanine green injection. When the distance between the landmarks determined by the "ung surface intersegmental constant proportion landmarks" and the segmental boundaries displayed by indocyanine green  fluorescence staining was ≤1 cm, the landmarks were judged to be consistent with the planes with discrepant fluorescence. As long as one of the landmarks was judged to be consistent, the method was considered to be feasible and accurate. Results 聽 聽 A total of 21 patients who underwent thoracoscopic segmentectomy were enrolled, with 5 male and 16 female patients. The median age was 55 years, ranging from 34 to 76 years. A total of 11 patients received left-side surgery, while 10 patients received right-side surgery. In the operations of 21 pulmonary segmentectomies, at least one intersegmental landmark determined by the "lung surface intersegmental constant proportion landmarks" was consistent with the intersegmental plane determined by indocyanine green fluorescence staining in each patient. Conclusion 聽 聽The intersegmental landmarks determined by the "lung surface intersegmental constant proportion landmarks" are consistent with that determined by indocyanine green fluorescence staining. The method of "lung surface intersegmental constant proportion landmarks" is feasible and accurate in identifying intersegmental planes during pulmonary segmentectomy.

Perioperative and long-term outcomes of segmentectomy for ground-glass opacity dominant early stage lung cancer: A single-center large-sample retrospective analysis / 中国胸心血管外科临床杂志

@#Objective     To summarize the perioperative and long-term outcomes of ground-glass opacity (GGO) dominant early stage lung cancer patients treated by anatomic segmentectomy. Methods     We collected clinical data of 756 patients from Western China Lung Cancer Database, who underwent intentional anatomic segmentectomy [tumor size (T) ≤ 2 cm, GGO ≥ 50%] in the Department of Thoracic Surgery, West China Hospital, Sichuan University from 2009 to 2018. There were 233 males and 523 females at a median age of 53 (25-83) years including 290 (38.4%) patients of simple segmentectomy and 466 (61.6%) patients of complex segmentectomy. All patients were diagnosed as adenocarcinoma, including 338 (44.7%) patients of minimally invasive adenocarcinoma and 418 (55.3%) patients of invasive adenocarcinoma. Results     The median operative time was 115 (38-300) min, the median blood loss was 20 (5-800) mL, 58 (7.7%) patients had postoperative complications and the postoperative stay was 4 (2-24) days. The median follow-up period was 43.0 (30.1-167.9) months. Five-year overall survival rate was 99.5% [95%CI (98.8%, 100.0%)], 5-year recurrence-free survival rate was 98.8% [95%CI (97.5%, 100.0%)], and 5-year lung cancer-specific survival rate was 100.0%. Conclusion    Anatomic segmentectomy has favorable perioperative outcomes and excellent prognosis in GGO dominant early stage lung cancer patients.

The risk factors for recurrence of peripheral solid small-nodule lung cancer (diameter≤ 2 cm) and the impact of different surgery types on survival: A propensity-score matching study / 中国胸心血管外科临床杂志

@#Objective     To identify the risk factors for postoperative recurrence of peripheral solid small-nodule lung cancer (PSSNLC) (T≤2 cm), and to explore the effects of surgery types on prognosis. Methods     We extracted data from Western China Lung Cancer Database (WCLCD), a prospectively maintained database at the Department of Thoracic Surgery, West China Hospital, Sichuan University, and Surveillance, Epidemiology, and End Results (SEER)  database for peripheral solid small-nodule lung cancer patients (T≤2 cm N0M0, stageⅠ) who underwent surgery between 2005 and 2016. We used univariable and multivariable logistic regression to analyze risk factors for recurrence of PSSNLC. We applied propensity-score matching to compare the long-term results of segmentectomy and lobectomy, as well as the survival of patients from WCLCD and SEER. We finally included 4 800 patients with PSSNLC (T≤2 cm N0M0)(WCLCD: SEER=354∶4 446). We matched 103 segmentectomies and 350 lobectomies in T≤1 cm, and 280 segmentectomies and 1 067 lobectomies in 1 cm<T≤2 cm at a ratio of 1∶4 according to the propensity scores. Results     The results of multivariable logistic regression showed that older age (HR=1.04, 95%CI 1.03-1.05, P<0.001), male (HR=1.60, 95%CI 1.37-1.88, P<0.001), squamous carcinoma (HR=1.65, 95%CI 1.40-1.95, P<0.001), lymph node removed (HR=0.97, 95%CI 0.96-0.99, P<0.001) were risk factors for recurrence after surgery. We found that segmentectomy and lobectomy could reach similar survival in PSSNLC patient with either T≤1 cm or 1 cm<T≤2 cm. Analyses of 1 441 patients with PSSNLC after matching (WCLCD∶SEER=325∶1 116) indicated that the 5-year overall survival rate of the patients in WCLCD was better than that in SEER database ( 89.8% vs. 77.1%, P<0.001). Conclusion     Older age, male, squamous carcinoma, and lymph node removed are the risk factors for recurrence of PSSNLC. Segmentectomy shows similar survival in PSSNLC patient with either T≤1 cm or 1 cm<T≤2 cm N0M0. The patients in the WCLCD shows better survival compared with of the patients in the SEER database.

Anastomotic tuberculosis after carinal resection and reconstruction: A case report / 中国胸心血管外科临床杂志

@#This patient was a 47-year female who underwent carinal resection and reconstruction because of left main bronchial mucoepidermoid carcinoma. She underwent four cycles chemotherapy when recovering from surgery because of subcarinal lymph node metastasis. However, the patient suffered from recurred productive cough and dyspnea during chemotherapy. Bronchoscopic assessment revealed stenosis at the reconstructed carina and left main bronchus five months after surgery. The granulation tissues of the left main bronchus showed no evidence of cancer recurrence. After repeated bronchoscopic resection of granulation tissue combined with bronchial stent placement, the left main bronchial stenosis gradually worsened with granulation tissue growth. Three acid-fast bacilli were found in the granulation tissue harvested ten months after surgery. The reason of postoperative bronchostenosis was confirmed as endobronchial tuberculosis, and antitubercular agents were added. Unfortunately, she had persistent left main bronchostenosis due to irreversible destruction and left pulmonary atelectasis thereafter. Therefore, for the recurring anastomotic granulomas after tracheobronchial reconstruction, the possibility of tuberculosis infection should be considered.

Perioperative outcomes of uniportal versus three-port video-assisted thoracoscopic lobectomy for 2 112 lung cancer patients: A propensity score matching study / 中国胸心血管外科临床杂志

@#Objective     To analyze the perioperative outcomes of uniportal thoracoscopic lobectomy compared with three-port thoracoscopic lobectomy. Methods     Data were extracted from the Western China Lung Cancer Database, a prospectively maintained database at the Department of Thoracic Surgery, West China Hospital, Sichuan University. Perioperative outcomes of the patients who underwent uniportal or three-port thoracoscopic lobectomy for lung cancer during January 2014 through April 2021 were analyzed by using propensity score matching. Altogether 5 817 lung cancer patients were enrolled who underwent thoracoscopic lobectomy (uniportal: 530 patients; three-port: 5 287 patients). After matching, 529 patients of uniportal and 1 583 patients of three-port were included. There were 529 patients with 320 males and 209 females at median age of 58 (51, 65) years in the uniportal group and 1 583 patients with 915 males and 668 females at median age of 58 (51, 65) years in the three-port group. Results     Uniportal thoracoscopic lobectomy was associated with less intraoperative blood loss (20 mL vs. 30 mL, P<0.001), longer operative time (115 min vs. 105 min, P<0.001) than three-port thoracoscopic lobectomy. No significant difference was found between the two groups regarding the number of lymph node dissected, rate of conversion to thoracotomy, incidence of postoperative complication, postoperative pain score within 3 postoperative days, length of hospital stay, or hospitalization expenses. Conclusion     Uniportal video-assisted thoracoscopic lobectomy is safe and effective, and the overall perioperative outcomes are comparable between uniportal and three-port strategies, although the two groups show differences in intraoperative blood loss.

Analysis of preoperative adverse emotion of patients with lung cancer and its effect on postoperative rehabilitation / 中国胸心血管外科临床杂志

@#Objective    To examine the effect of preoperative adverse emotion on rehabilitation outcomes in lung cancer patients undergoing thoracoscopic major pulmonary resection. Methods    We retrospectively analyzed the clinical data of 1 438 patients with lung cancer who underwent thoracoscopic lobectomy and segmentectomy in West China Hospital of Sichuan University from February 2017 to July 2018 including 555 males and 883 females. All patients were assessed by Huaxi emotional-distress index scoring, and were divided into three groups including a non-negative emotion group, a mild negative emotion group, and a moderate-severe negative emotion group. All patients underwent thoracoscopic lobectomy or segmentectomy plus systematic lymph node dissection or sampling. The volume of postoperative chest drainage, postoperative lung infection rate, time of chest tube intubation and postoperative duration of hospitalization were compared among these three groups. Results    There were different morbidities of adverse emotion in age, sex, education level and smoking among patients before operation (P<0.05). Univariate analysis showed that there was no statistical difference in the duration of indwelling drainage tube, drainage volume, postoperative pulmonary infection rate or the incidence of other complications among these three groups, but the duration of hospitalization in the latter two groups was less than that in the first group with a statistical difference (P<0.05). After correction of confounding factors by multiple regression analysis, there was no statistical difference among the three groups. Conclusion    Young patients are more likely to develop bad emotions, women are more likely to develop serious bad emotions, highly educated patients tend to develop bad emotions, and non-smoking patients tend to develop bad emotions. There is no effect of preoperative adverse emotions on the rapid recovery of lung cancer patients after minimally invasive thoracoscopic surgery.

An Overview of the Application of Artificial Neural Networks in Lung Cancer Research / 中国肺癌杂志

Lung cancer is the most common and fatal tumor in the world with limited diagnostic and treatment methods. The development of precision medicine has brought new opportunities for the improvement of diagnosis and treatment of lung cancer. However, various kinds of information required by precision medicine (such as biometrics, clinical test indicators and non-biological environmental background information) are difficult for clinicians to integrate and utilize effectively. With the development of computer technology, artificial neural networks (ANNs), which has the characteristic of high fault tolerance, intelligence and self-learning ability. Its powerful information integration ability can solve many problems in the development and application of precision medicine, which can play a key role in basic research and clinical practice associated with lung cancer. This article reviewed the application of artificial neural network in the field of lung cancer.
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Network pharmacology-based study on material basis and mechanism of Qikui Granules against diabetic nephropathy / 中草药

Objective: This study was designed to explore the "multi-components, multi-targets, multi-pathways" mechanism of Qikui Granules in the treatment of diabetic nephropathy (DN) by network pharmacology, aiming to provide basis for its basic research and clinical application. Methods: The active chemical constituents of Qikui Granules and their related targets against diabetic kidney disease were searched and screened by multiple databases. The network maps of drug-disease-targets were constructed by Cytoscape software. The key targets of Qikui Granules against DN were searched by network topology. The GO and KEGG gene enrichment analysis of the key targets for treating DN were performed by using ClueGO. Results: The network analysis indicated that 67 active chemical components and 212 targets against DN were established from Qikui Granules. A total of 43 key targets were finally screened by network topology analysis. These targets were mainly enriched in 49 significant related pathways such as IL-17 signaling pathway, HIF-1 signaling pathway, TNF signaling pathway and so on. Also, 132 significant related biological processes such as neurotransmitter metabolic processes, positive regulation of small molecule metabolic processes, regulation of blood pressure and oxidoreductase activity were related. Conclusion: This study reveals that the active constituents of Qikui Granules could regulate multiple targets in the pathogenesis of diabetic nephropathy, which provides an important basis for further research on its mechanism of action against DN.

A Novel Mutation of Mitochondrial T14709C Causes Myoclonic Epilepsy with Ragged Red Fibers Syndrome in a Chinese Patient / 中华医学杂志(英文版)

<p><b>Background</b>Myoclonic epilepsy with ragged red fibers (MERRF) syndrome is characterized by myoclonus, generalized epilepsy, cerebellar ataxia, and ragged red fibers (RRFs) in the muscle. T-to-C transition at nucleotide position 14709 in the mitochondrial tRNA glutamic acid (tRNA) gene has previously been associated with maternally inherited diabetes and deafness. However, the association between MERRF and mitochondrial T14709C mutation (m.T14709C) has never been reported before.</p><p><b>Methods</b>Clinical information of a 17-year-old patient was collected; muscle biopsy and next-generation sequencing (NGS) of whole mitochondrial and neuromuscular disease panel were then conducted. Finally, sanger sequencing was carried out to confirm the mutations.</p><p><b>Results</b>The patient presented a typical MERRF phenotype with muscle weakness, epileptic seizure, clonic episodes, cerebellar ataxia, and spinal scoliosis. Muscle biopsy showed RRFs which indicated abnormal mitochondrial functions. NGS of whole mitochondrial gene revealed m.T14709C mutation, confirmed by Sanger sequencing.</p><p><b>Conclusion</b>We present a sporadic patient with typical MERRF presentation carrying the mutation of m.T14709C, which expanded the spectrum of m.T14709C.</p>

A survey on the current development of thoracic surgery in tertiary hospitals of China / 中华外科杂志

Objective@#To study the current development of thoracic surgery in China.@*Methods@#Chinese Society for Thoracic and Cardiovascular Surgery and Chinese Association for Thoracic Surgeons jointly conducted a network survey to directors of thoracic surgery departments in the tertiary hospitals in China from November to December 2018. The contents of the survey included the basic information of the hospital and the status of thoracic surgery department in the hospital. Rank sum test was used to compare the data between different regional hospitals@*Results@#A total of 636 tertiary hospitals participated in the survey. The total number of beds for thoracic surgery departments was 30 646, with M(QR) of 40(20) (range: 3 to 393) for each hospital. The total number of thoracic surgeons was 6 747, with M(QR) of 9(6) (range: 1 to 75) in each hospital. In 2015, a total of 312 425 operations were performed in the 636 hospitals, with M(QR) of 268(484.5) (range: 4 to 8 320) for each hospital. The total number of lung cancer surgeries was 146 601 in 2015, with M(QR) of 100(216) (range: 0 to 6 911) operations in each hospital. The total number of esophageal cancer operations was 67 076, with M(QR) of 40(95) (range: 0 to 1 550) in each hospital. Minimal invasive thoracic surgery was performed in 94.3% (601/636) of the hospitals, with 86.6% (551/636) of hospitals carried out video-assisted thoracoscopic (VATS) lobectomy. Among the hospitals performing VATS lobectomy, 89.3% (492/551) of them started to perform the technique after 2006, and 93.1% (513/551) of them do single-direction thoracoscopic lobectomy. A total of 403 640 VATS lobectomies had been performed until 2015, including 163 682 cases of single-direction thoracoscopic lobectomy. In 2015, 73.74% (108 116/146 601) lung cancer operations and 37.44% (25 110/67 076) of esophageal cancer resections were performed by minimally invasive technique. The development level of hospitals among eastern, middle and Western China was different significantly on number of doctors, number of total operations, number of lung cancer surgeries, proportion of minimally invasive lung cancer surgery, number of esophageal cancer surgeries, and proportion of minimally invasive esophageal cancer surgery (χ2∶7.65 to 60.8, all P<0.05).@*Conclusions@#The discipline of thoracic surgery, especially the minimally invasive thoracic surgery in China is now experiencing a rapid development. The proportion of minimally invasive lung cancer surgery is higher than that of in the developed countries. However, unbalanced development among different regions is still a great challenge in China.

Identification of Seven Novel Mutations in the Acid Alpha-glucosidase Gene in Five Chinese Patients with Late-onset Pompe Disease / 中华医学杂志(英文版)

<p><b>Background</b>Pompe disease is a rare lysosomal glycogen storage disorder linked to the acid alpha-glucosidase gene (GAA). A wide clinical and genetic variability exists between patients from different ethnic populations, and the genotype-phenotype correlations are still not well understood. The aim of this study was to report the clinicopathological and genetic characteristics of five Chinese patients with late-onset Pompe disease (LOPD) who carried novel GAA gene mutations.</p><p><b>Methods</b>Clinical and pathological data of patients diagnosed with glycogen storage disease at our institution from April 1986 to August 2017 were collected, and next-generation sequencing of frozen muscle specimens was conducted.</p><p><b>Results</b>Of the five patients included in the study, the median disease onset age was 13 years, with a median 5 years delay in diagnosis. The patients mainly manifested as progressive weakness in the proximal and axial muscles, while one patient developed respiratory insufficiency that required artificial ventilation. In muscle biopsies, vacuoles with variable sizes and shapes appeared inside muscle fibers, and they stained positive for both periodic acid-Schiff and acid phosphatase staining. Ten GAA gene mutations, including seven novel ones (c.796C>A, c.1057C>T, c.1201C>A, c.1780C>T, c.1799G>C, c.2051C>A, c.2235dupG), were identified by genetic tests.</p><p><b>Conclusions</b>The seven novel GAA gene mutations revealed in this study broaden the genetic spectrum of LOPD and highlight the genetic heterogeneity in Chinese LOPD patients.</p>

Strategies for the prevention and control of bleeding due to vascular injury in thoracoscopic lung surgery / 中华外科杂志

The technique of thoracoscopic lung surgery has gradually matured. Nowadays, thoracoscope is recommended as the most preferred approach for surgical treatment of early stage non-small cell lung cancer in different guidelines. However, there are still some cases of accidential major bleeding due to vascular injury during thoracoscopic lung surgery. The wall of the hilum vessels is relatively thin. These vessels often involve a great portion of the cardiac output blood flow. Once the injury happened, the emergent condition may be life-threatening due to massive blood loss. Therefore, this became an important factor which hindered the development of thoracoscopic lung surgery. In this review, details of the vascular injury in thoracoscopic lung surgery were summarized, including the incidence of vascular injury, commonly injured sites and reasons of the injuries. Among all the cases of thoracoscopic major pulmonary resection, 2.9% to 9.2% may suffer from vascular injury during the operation. The most commonly injuried sites are pulmonary artery and the branches, and this is also the most critical situation during thoracoscopic lung surgery. Hilum adhesion is the most important risk factor for vascular injury. On the one hand, the suction-compressing angiorrhaphy technique was developed for bleeding control and angioplasty. On the other hand, the strategies like pre-control of the pulmonary, cut the bronchus in advance, and fire the bronchus and pulmonary artery together may decrease the incidence of vascular injury in patients with risk factors.

EST-SSR identification, markers development of Ligusticum chuanxiong based on Ligusticum chuanxiong transcriptome sequences / 中国中药杂志

Ligusticum chuanxiong is a well-known traditional Chinese medicine plant. The study on its molecular markers development and germplasm resources is very important. In this study, we obtained 24 422 unigenes by assembling transcriptome sequencing reads of L. chuanxiong root. EST-SSR was detected and 4 073 SSR loci were identified. EST-SSR distribution and characteristic analysis results showed that the mono-nucleotide repeats were the main repeat types, accounting for 41.0%. In addition, the sequences containing SSR were functionally annotated in Gene Ontology (GO) and KEGG pathway and were assigned to 49 GO categories, 242 KEGG pathways, among them 2 201 sequences were annotated against Nr database. By validating 235 EST-SSRs,74 primer pairs were ultimately proved to have high quality amplification. Subsequently, genetic diversity analysis, UPGMA cluster analysis, PCoA analysis and population structure analysis of 34 L. chuanxiong germplasm resources were carried out with 74 primer pairs. In both UPGMA tree and PCoA results, L. chuanxiong resources were clustered into two groups, which are believed to be partial related to their geographical distribution. In this study, EST-SSRs in L. chuanxiong was firstly identified, and newly developed molecular markers would contribute significantly to further genetic diversity study, the purity detection, gene mapping, and molecular breeding.

A Novel AGRN Mutation Leads to Congenital Myasthenic Syndrome Only Affecting Limb-girdle Muscle / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Congenital myasthenic syndromes (CMSs) are a group of clinically and genetically heterogeneous disorders caused by impaired neuromuscular transmission. The defect of AGRN was one of the causes of CMS through influencing the development and maintenance of neuromuscular transmission. However, CMS reports about this gene mutation were rare. Here, we report a novel homozygous missense mutation (c.5302G>C) of AGRN in a Chinese CMS pedigree.</p><p><b>METHODS</b>We performed a detailed clinical assessment of a Chinese family with three affected members. We screened for pathogenic mutations using a disease-related gene panel containing 519 genes associated with genetic myopathy (including 17 CMS genes).</p><p><b>RESULTS</b>In the family, the proband showed limb-girdle pattern of weakness with sparing of ocular, facial, bulbar, and respiratory muscles. Repetitive nerve stimulation showed a clear decrement of the compound muscle action potentials at 3 Hz only. Pathological analysis of the left tibialis anterior muscle showed predominance of type I fiber and the presence of scattered small angular fibers. The proband's two elder sisters shared a similar but more severe phenotype. By gene analysis, the same novel homozygous mutation (c.5302G>C, p. A1768P) of AGRN was identified in all three affected members, whereas the same heterozygous mutation was found in both parents, revealing an autosomal recessive transmission pattern. All patients showed beneficial responses to adrenergic agonists.</p><p><b>CONCLUSIONS</b>This study reports a Chinese pedigree in which all three children carried the same novel AGRN mutation have CMS only affecting limb-girdle muscle. These findings might expand the spectrum of mutation in AGRN and enrich the phenotype of CMS.</p>

Increased Expression of the NOD-like Receptor Family, Pyrin Domain Containing 3 Inflammasome in Dermatomyositis and Polymyositis is a Potential Contributor to Their Pathogenesis / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Dermatomyositis (DM) and polymyositis (PM) are common inflammatory myopathies whose immunopathogenic mechanisms remain poorly understood. The NOD-like receptor family, pyrin domain containing 3 (NLRP3) inflammasome is a type of cytoplasmic multiprotein inflammasome and is responsible for the activation of inflammatory reactivations. Responding to a wide range of exogenous and endogenous microbial or sterile stimuli, NLRP3 inflammasomes can cleave pro-caspase-1 into active caspase-1, which processes the pro-inflammatory cytokines pro-interleukin (IL)-1β and pro-IL-18 into active and secreted IL-1β and IL-18. The NLRP3 inflammasome is implicated in infectious and sterile inflammatory diseases. However, it remains unclear whether it is involved in the pathogenesis of DM/PM, which we aim to address in our research.</p><p><b>METHODS</b>In this study, 22 DM/PM patients and 24 controls were recruited. The protein and RNA expression of IL-1β, IL-18, NLRP3, and caspase-1 in serum and muscle samples were tested and compared between the two groups.</p><p><b>RESULTS</b>The serum IL-1β and IL-18 levels were significantly higher in DM/PM patients than those in the controls by enzyme linked immunosorbent assay (ELISA, DM vs. control, 25.02 ± 8.29 ng/ml vs. 16.49 ± 3.30 ng/ml,P < 0.001; PM vs. control, 26.49 ± 7.79 ng/ml vs. 16.49 ± 3.30 ng/ml,P < 0.001). Moreover, the real-time quantitative reverse transcription-polymerase chain reaction (qRT-PCR) showed that DM/PM patients exhibited higher RNA expression of IL-1β, IL-18, and NLRP3 in the muscle (for IL-1β, DM vs. control, P= 0.0012, PM vs. control, P= 0.0021; for IL-18, DM vs. control, P= 0.0045, PM vs. control, P= 0.0031; for NLRP3, DM vs. control, P= 0.0017, PM vs. control, P= 0.0006). Moreover, the protein expression of NLRP3 and caspase-1 in muscle samples of DM/PM patients were also significantly elevated compared to that in the muscles of the controls.</p><p><b>CONCLUSIONS</b>Our findings demonstrate that the NLRP3 inflammasome is implicated in the pathogenesis of DM/PM. High NLRP3 expression led to elevated levels of IL-1β and IL-18 and could be one of the factors promoting disease progress.</p>

Oculopharyngeal Weakness, Hypophrenia, Deafness, and Impaired Vision: A Novel Autosomal Dominant Myopathy with Rimmed Vacuoles / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Myopathies with rimmed vacuoles are a heterogeneous group of muscle disorders with progressive muscle weakness and varied clinical manifestations but similar features in muscle biopsies. Here, we describe a novel autosomal dominant myopathy with rimmed vacuoles in a large family with 11 patients of three generations affected.</p><p><b>METHODS</b>A clinical study including family history, obstetric, pediatric, and development history was recorded. Clinical examinations including physical examination, electromyography (EMG), serum creatine kinase (CK), bone X-rays, and brain magnetic resonance imaging (MRI) were performed in this family. Open muscle biopsies were performed on the proband and his mother. To find the causative gene, the whole-exome sequencing was carried out.</p><p><b>RESULTS</b>Disease onset was from adolescence to adulthood, but the affected patients of the third generation presented an earlier onset and more severe clinical manifestations than the older generations. Clinical features were characterized as dysarthria, dysphagia, external ophthalmoplegia, limb weakness, hypophrenia, deafness, and impaired vision. However, not every patient manifested all symptoms. Serum CK was mildly elevated and EMG indicated a myopathic pattern. Brain MRI showed cerebellum and brain stem mildly atrophy. Rimmed vacuoles and inclusion bodies were observed in muscle biopsy. The whole-exome sequencing was performed, but the causative gene has not been found.</p><p><b>CONCLUSIONS</b>We reported a novel autosomal dominant myopathy with rimmed vacuoles characterized by dysarthria, dysphagia, external ophthalmoplegia, limb weakness, hypophrenia, deafness, and impaired vision, but the causative gene has not been found and needs further study.</p>

Regulatory T cells in the treatment of autoimmune myositis in mice: efficacy and mechanism / 南方医科大学学报

<p><b>OBJECTIVE</b>To investigate effect of CD4(+) CD25(+) Foxp3(+) Tregs in the treatment of autoimmune myositis (EAM) in mice and explore the possible mechanisms.</p><p><b>METHODS</b>Mouse models of EAM were divided randomly into model group and treatment group, and the latter received infusion of CD4(+) CD25(+) Foxp3(+) Tregs separated from normal mouse spleen by magnetic activated cell sorting. The changes of muscle pathology was observed, and the expression of PD-1 and CTLA-4 in spleen CD4(+) CD25(+) Foxp3(+) Tregs was analyzed using flow cytometry; peripheral blood IL-10 and TGF-β levels were tested using double antibody sandwich ELISA.</p><p><b>RESULTS</b>Compare with the model group, the mice in the treatment group showed significantly reduced muscular inflammatory cell infiltration, increased blood levels of IL-10 and TGF-β (P<0.05), and increased expression of PD-1 and CTLA-4 in spleen CD4(+) CD25(+) Foxp3(+) Tregs (P<0.05).</p><p><b>CONCLUSION</b>CD4(+) CD25(+) Foxp3(+) Tregs reinfusion produces therapeutic effect in mice with EAM by increasing peripheral blood IL-10 and TGF-β levels and PD-1 and CTLA-4 expressions in spleen CD4(+) CD25(+) Foxp3(+) Tregs.</p>

Perioperative and long-term outcome of thymectomy for myasthenia gravis: comparison of surgical approaches and prognostic analysis / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Thymectomy is an established treatment for myasthenia gravis (MG), and video-assisted thoracoscopic surgery (VATS) thymectomy has become an acceptable surgical procedure. This study aimed to compare the results of VATS thymectomy and open thymectomy and to identify the prognostic factors after thymectomy.</p><p><b>METHODS</b>The clinical data of 187 consecutive thymectomies performed between July 2000 and December 2009 were retrospectively reviewed; 75 open thymectomies and 112 VATS thymectomies. Clinical efficacy and variables influencing outcome were assessed by Kaplan-Meier survival curves and Cox proportional hazards regression analysis.</p><p><b>RESULTS</b>The operative blood loss in the VATS group was significantly less than that in the open group ((62.14 ± 55.43) ml vs. (137.87 ± 165.25) ml, P < 0.05). The postoperative crisis rate increased with the severity of preoperative MG and the prescription dose of anticholinesterase. Complete follow-up information of patients more than 12 months after the thymectomy was obtained on 151 cases, 89 cases from the VATS group and 62 cases from the open group, with a mean follow-up period of 59.3 months, range from 12 to 117 months. Complete stable remission (CSR) was the end point for evaluation of the treatment results. The overall five-year CSR rate was 57.5%. Two good prognostic factors were identified; preoperative prescription of anticholinesterase alone (P = 0.035) and non-thymomatous MG (P = 0.003). The five-year CSR rate of the ocular type of MG reached a high level of 67.4%.</p><p><b>CONCLUSIONS</b>Thymectomy can achieve good long-term CSR in MG, and VATS is an ideal alternative method. High-dose prescription of anticholinesterase and the advanced stage by Myasthenia Gravis Foundation of America (MGFA) classification have higher risks of postoperative crisis. Preoperative prescription of anticholinesterase alone and non-thymomatous MG are good prognostic factors. Thymectomy should also be considered for the ocular type of MG.</p>

Dural enhancement detected by magnetic resonance imaging reflecting the underlying causes of cerebral venous sinus thrombosis / 中华医学杂志(英文版)

Dural enhancement detected by magnetic resonance imaging is a common finding in patients with cerebral venous sinus thrombosis (CVST) and is usually interpreted as a change secondary to CVST. We report two cases of CVST with intense and diffuse dural enhancement that resulted from pachymeningitis in one patient and spontaneous intracranial hypotension in another. Pachymeningitis and spontaneous intracranial hypotension were also determined to be the underlying causes of CVST. The clinical data of these two patients are described. In patients with CVST, dural enhancement is not always a secondary change to CVST. It can be a manifestation of the underlying causes of CVST. When diffuse and intense dural enhancement is revealed, sufficient ancillary tests are warranted to rule out other potential pathological changes of the dura mater those can result in CVST.