Search

Before the Song Dynasty, the main processing method of Pinelliae Rhizoma was soup washing. The "new method" in Taiping Huimin Heji JuFang is a processing method that concocted with Ginger,white alum and starter-making.The "Fa Banxia" in the Yuan Dynasty's Yuyuan Yaofang comes from the Taiping Huimin Heji JuFang , and the Fa Wen Banxia, Fa Bai Banxia, and Fa Hong Banxia are the processing methods of patent medicine with a variety of other herbs.Fa Banxia appeared in the Ming Dynasty, and its auxiliary materials were ginger and white alum, and medical formulary began to include formulas containing Fa Banxia. Bencao Gangmu abbreviates the Yuan Dynasty's Fabai Banxia as "Fa Banxia", and is elaborated under the item attached "prescription" item instead of the "treatment". In the literature of Materia Medica, it is recorded that the preparation of auxiliary materials in Fa Banxia increased, including lime, licorice, soap horn, and simple nitro.After Daoguang in the Qing Dynasty, the Fa Banxia in famous medical cases was more used, and at that time, Fa Banxia was Xian Banxia. There are two recipes for Xian Banxia: one is made with seven processes, and the other is soaked in alum licorice water. During the Republican period, Zhang Cigong also pointed out that Fa Banxia was sliced Xian Banxia after rinsing and boiling, while the preparation method of Xian Banxia was Banxia made of ginger and white alum.Ye Juquan pointed out that the so-called "fa" is neither an ancient method nor a new method, questioning the process of repeated immersion in Banxia. After 1949, the questioning of Fa Banxia continued unceasing.Influenced by this, the 1960 edition Beijing Traditional Chinese Medicine Slice Cutting Experience included the method of soaking alum, licorice ,lime water, and was included in the 1963 edition of the Pharmacopoeia of the People's Republic of China .The 1985 version reduced the soaking time and eliminated the soaking process of alum based on the 1963 version, and this method is still used today.

Objective: To observe the association between clinical phenotypes of hypertrophic cardiomyopathy (HCM) patients and a rare calcium channel and regulatory gene variation (Ca 2+ gene variation) and to compare clinical phenotypes of HCM patients with Ca 2+ gene variation, a single sarcomere gene variation and without gene variation and to explore the influence of rare Ca 2+ gene variation on the clinical phenotypes of HCM. Methods: Eight hundred forty-two non-related adult HCM patients diagnosed for the first time in Xijing Hospital from 2013 to 2019 were enrolled in this study. All patients underwent exon analyses of 96 hereditary cardiac disease-related genes. Patients with diabetes mellitus, coronary artery disease, post alcohol septal ablation or septal myectomy, and patients who carried sarcomere gene variation of uncertain significance or carried>1 sarcomere gene variation or carried>1 Ca 2+ gene variation, with HCM pseudophenotype or carrier of ion channel gene variations other than Ca 2+ based on the genetic test results were excluded. Patients were divided into gene negative group (no sarcomere or Ca 2+ gene variants), sarcomere gene variation group (only 1 sarcomere gene variant) and Ca 2+ gene variant group (only 1 Ca 2+ gene variant). Baseline data, echocardiography and electrocardiogram data were collected for analysis. Results: A total of 346 patients were enrolled, including 170 patients without gene variation (gene negative group), 154 patients with a single sarcomere gene variation (sarcomere gene variation group) and 22 patients with a single rare Ca 2+ gene variation (Ca 2+ gene variation group). Compared with gene negative group, patients in Ca 2+ gene variation group had higher blood pressure and higher percentage of family history of HCM and sudden cardiac death ( P <0.05); echocardiographic results showed that patients in Ca 2+ gene variation group had thicker ventricular septum ((23.5±5.8) mm vs. (22.3±5.7) mm, P <0.05); electrocardiographic results showed that patients in Ca 2+ gene variation group had prolonged QT interval ((416.6±23.1) ms vs. (400.6±47.2) ms, P <0.05) and higher RV5+SV1 ((4.51±2.26) mv vs. (3.50±1.65) mv, P <0.05). Compared with sarcomere gene variation group, patients in Ca 2+ gene variation group had later onset age and higher blood pressure ( P <0.05); echocardiographic results showed that there was no significant difference in ventricular septal thickness between two groups; patients in Ca 2+ gene variation group had lower percentage of left ventricular outflow tract pressure gradient>30 mmHg (1 mmHg=0.133 kPa, 22.8% vs. 48.1%, P <0.05) and the lower early diastolic peak velocity of the mitral valve inflow/early diastolic peak velocity of the mitral valve annulus (E/e') ratio ((13.0±2.5) vs. (15.9±4.2), P <0.05); patients in Ca 2+ gene variation group had prolonged QT interval ((416.6±23.1) ms vs. (399.0±43.0) ms, P <0.05) and lower percentage of ST segment depression (9.1% vs. 40.3%, P <0.05). Conclusion: Compared with gene negative group, the clinical phenotype of HCM is more severe in patients with rare Ca 2+ gene variation; compared with patients with sarcomere gene variation, the clinical phenotype of HCM is milder in patients with rare Ca 2+ gene variation.

Objective: To evaluate the impact of individual and combined assessment of age- and sex-specific brachial-ankle pulse wave velocity (baPWV) and pulse pressure (PP) on all-cause mortality. Methods: This study is a prospective cohort study. Individuals participated in the Kailuan Study and completed baPWV measurements between 2010 and 2016 were included in this study. After stratifying by sex, 75th percentile baPWV and PP values for different age group were calculated at five years interval. BaPWV and PP values below the 75th percentile were defined as normal, and those above or equal to the 75th percentile were defined as increased. The participants were allocated to four groups according to their PP and baPWV status: normal baPWV/PP group, high baPWV/normal PP group, normal baPWV/high PP group and high baPWV/PP group. The primary outcome was all-cause mortality during the follow-up period. Cox proportional hazards models were used to explore the impact of individual and combined assessment of baPWV and PP on all-cause mortality events. Results: A total of 39 339 participants were enrolled in this study, aged (49.3±12.8) years, of which 28 731 (73.03%) were males. There were 23 268, 6 025, 6 210 and 3 836 cases in the normal baPWV/PP group, high baPWV/normal PP group, normal baPWV/high PP group and high baPWV/PP group, respectively. The average follow-up duration was (4.98±2.53) years. During the follow-up period, all-cause mortality occurred in 998 individuals. Multivariate Cox regression analysis showed increased risk of all-cause mortality in the high baPWV/normal PP group ( HR =1.27, 95% CI 1.07-1.50), and in the high baPWV/PP group ( HR =1.33, 95%CI 1.08-1.65) compared to the normal baPWV/PP group. Increased pulse pressure alone had no impcat on all-cause death ( HR =1.06, 95% CI 0.87-1.29). Conclusions: The risk of all-cause mortality significantly increases with increased age-and sex-specific baPWV and PP values. BaPWV may be a better predictor of all-cause mortality than PP in this cohort.

Objective: To investigate the representability and etiological diagnostic value of myocardium samples obtained from patients with hypertrophic cardiomyopathy (HCM) by transthoracic echocardiography-guided percutaneous intramyocardial septal biopsy (myocardial biopsy of Liwen procedure). Methods: This study was a retrospective case-series analysis. Patients with HCM, who underwent myocardial biopsy of Liwen procedure and radiofrequency ablation in Xijing Hospital, Air Force Military Medical University from July to December 2019, were included. Demographic data (age, sex), echocardiographic data and complications were collected through electronic medical record system. The histological and echocardiographic features, pathological characteristics of the biopsied myocardium of the patients were analyzed. Results: A total of 21 patients (aged (51.2±14.5) years and 13 males (61.9%)) were enrolled. The thickness of ventricular septum was (23.3±4.5)mm and the left ventricular outflow tract gradient was (78.8±42.6)mmHg (1 mmHg=0.133 kPa). Eight patients (38.1%) were complicated with hypertension, 1 patient (4.8%) had diabetes, and 2 patients (9.5%) had atrial fibrillation. Hematoxylin-eosin staining of myocardial samples of HCM patients before radiofrequency ablation evidenced myocytes hypertrophy, myocytes disarray, nuclear hyperchromatism, hypertrophy, atypia, coronary microvessel abnormalities, adipocyte infiltration, inflammatory cell infiltration, cytoplasmic vacuoles, lipofuscin deposition. Interstitial fibrosis and replacement fibrosis were detected in Masson stained biopsy samples. Hematoxylin-eosin staining of myocardial samples of HCM patients after radiofrequency ablation showed significantly reduced myocytes, cracked nuclear in myocytes, coagulative necrosis, border disappearance and nuclear fragmentation. Quantitative analysis of myocardial specimens of HCM patients before radiofrequency ablation showed that there were 9 cases (42.9%) with mild myocardial hypertrophy and 12 cases (57.1%) with severe myocardial hypertrophy. Mild, moderate and severe fibrosis were 5 (23.8%), 9 (42.9%) and 7 (33.3%), respectively. Six cases (28.6%) had myocytes disarray. There were 11 cases (52.4%) of coronary microvessel abnormalities, 4 cases (19.0%) of adipocyte infiltration, 2 cases (9.5%) of inflammatory cell infiltration,6 cases (28.5%) of cytoplasmic vacuole, 16 cases (76.2%) of lipofuscin deposition. The diameter of cardiac myocytes was (25.2±2.8)μm, and the percentage of collagen fiber area was 5.2%(3.0%, 14.6%). One patient had severe replacement fibrosis in the myocardium, with a fibrotic area of 67.0%. The rest of the patients had interstitial fibrosis. The myocardial specimens of 13 patients were examined by transmission electron microscopy. All showed increased myofibrils, and 9 cases had disorder of myofibrils. All patients had irregular shape of myocardial nucleus, partial depression, mild mitochondrial swelling, fracture and reduction of mitochondrial crest, and local aggregation of myofibrillary interfascicles. One patient had hypertrophy of cardiomyocytes, but the arrangement of muscle fibers was roughly normal. There were vacuoles in the cytoplasm, and Periodic acid-Schiff staining was positive. Transmission electron microscopy showed large range of glycogen deposition in the cytoplasm, with occasional double membrane surround, which was highly indicative of glycogen storage disease. No deposition of glycolipid substance in lysozyme was observed under transmission electron microscope in all myocardial specimens, which could basically eliminate Fabry disease. No apple green substance was found under polarized light after Congo red staining, which could basically exclude cardiac amyloidosis. Conclusion: Myocardium biopsied samples obtained by Liwen procedure of HCM patients are representative and helpful for the etiological diagnosis of HCM.

Objective: To summarize and compare clinicopathological features of Caroli disease and Caroli syndrome. Methods: A total of 21 patients diagnosed with Caroli disease or Caroli syndrome in Beijing Friendship Hospital, Capital Medical University, from January 2015 to December 2018 were included. Through the clinical manifestations and comparative analysis of the differences between different clinical types, the liver pathological features of these patients were described. Results: Of all patients included, 8 were male and 13 were female, and the medium age was 13.5 year old. The initial symptom was fever in 6 cases (28.6%), gastrointestinal bleeding in 6 cases (28.6%) and hepatosplenomegaly in 9 cases (42.8%). Caroli disease accounted for 6 cases (28.6%) and Caroli syndrome 15 cases (71.4%). The total bilirubin [6.7 (4.7, 15.0) vs 16.0(10.9, 33.0)μmol/L] and direct bilirubin [1.3(0.9,6.4)vs 3.5(2.7, 16.2)μmol/L] were significantly lower in Caroli disease group in comparison to those in Caroli syndrome group(both P< 0.05). The hemoglobin [117.0 (106.0, 126.2) vs 85.0 (74.0, 103.0) g/L] and platelet count [286.0 (149.8, 467.5)×10(9)/L vs 76.1(55.0,123.0)×10(9)/L] in Caroli disease group were significantly higher than those in Caroli syndrome group (both P< 0.05). There were 10 patients (47.6%) who underwent liver transplantation. Child-Pugh-Turcotte Score (liver function reserve) were significantly higher than that in the non-liver transplantation group[8.0(8.0, 10.2)vs 5.0 (5.0, 6.0), P< 0.05]. Conclusions: Early symptoms of Caroli disease/Caroli syndrome are atypical and prone to misdiagnosis and misdiagnosis. The diagnosis is usually based on pathology and may be supplemented by laboratory examination and imaging analysis.

Objective: To evaluate the cardiac functional changes in hypertrophic cardiomyopathy(HCM) patients with β-myosin heavy chain gene (MYH7) mutations by three-dimensional (3D) speckle tracking imaging(3D-STI) and conventional echocardiography modalities, and then to explore the potential predictors of adverse cardiovascular events in these patients. Methods: A consecutive series of 192 HCM patients admitted in our center from October 2014 to October 2016 were genetically screened to identify MYH7 mutations in this retrospective study. A total of 43 HCM patients with MYH7 mutations were enrolled. The patients were divided into events group( n= 13) and no event group( n= 30) according to the presence or absence of adverse cardiovascular events(primary and secondary endpoints). All patients were followed up to January 2019 after comprehensive evaluation of 3D-STI, two-dimensional and Doppler echocardiography. The adverse cardiovascular events were recorded. Results: The median follow up time was 1 012 (812, 1 330) days. During follow-up, 13 patients (30.2%) reached endpoints: 6 cases of the primary endpoints(2 cases of sudden cardiac death(SCD), 3 cases of survival after defibrillation, and 1 case of appropriate implantable cardioverter-defibrillator(ICD) discharge); 7 cases of the second endpoints(5 cases of heart failure hospitalization, 1 case of syncope and cardioversion due to supraventricular tachycardia, and 1 case of end-stage HCM). Patients with adverse cardiovascular events had higher prevalence of syncope and risk of SCD, enlarged left atrial volume index(LAVI) and reduced 3D left ventricular global longitudinal train (3D-GLS), as compared to those without adverse events(all P< 0.05). The multivariate Cox regression analysis showed that reduced 3D-GLS( HR =0.814, 95 %CI 0.663-0.999, P= 0.049) was an independent predictor for adverse cardiovascular events. The cutoff value of 3D-GLS≤13.67% was linked with significantly increased risk of adverse cardiovascular events in this patient cohort( AUC =0.753, 95 %CI 0.558-0.948, sensitivity 86%, specificity 69%, P< 0.05). The Kaplan-Meier analysis indicated that the patients with the 3D-GLS≤ 13.67% faced higher risk of death than those with 3D-GLS>13.67%. Conclusion: 3D-GLS is useful on predicting adverse cardiovascular events in HCM patients with MYH7 mutations.

Objective: To investigate the safety and efficacy of transthoracic echocardiography-guided percutaneous intramyocardial septal radiofrequency ablation (PIMSRA) in patients with hypertrophic obstructive cardiomyopathy (HOCM). Methods: Nine HOCM patients with interventricular septal thickness ≥15 mm and ≤25 mm who were treated with PIMSRA between October 2016 to March 2017 in the Hypertrophic Cardiomyopathy Center of Xijing Hospital of Air Force Military Medical University were enrolled,and the clinical data were retrospectively analyzed.Interventricular septum thickness, left ventricular outflow tract diameter and maximum gradient were measured by transthoracic echocardiography immediately after procedure, at 1 month, 3 months and 6 months after operation.Symptoms and New York Heart Association (NYHA) functional class were assessed, and provoked left ventricular outflow tract gradient and exercise time were measured after 6 months. Results: The anterior interventricular septum ((21.5±2.6) mm vs. (24.7±2.7) mm, P< 0.05) and posterior interventricular septum (21.1±2.5) mm vs. (22.6±3.3) mm, P< 0.05) were significantly increased,left ventricular outflow tract diameter was widened ((8.2±3.4) mm vs. (4.8±2.2) mm, P< 0.05), left ventricular outflow tract gradient ((26.8±19.6) mmHg (1 mmHg=0.133 kPa) vs. (83.3±32.4) mmHg, P< 0.05) and mitral regurgitation (2.0±1.9) ml vs. (3.2±3.0) ml, P< 0.05) were significantly decreased immediately after ablation compared with pre-operation values. Anterior interventricular septum, posterior interventricular septum and left ventricular outflow tract gradient further decreased after 1 month ((17.5±2.0) mm vs. (24.7±2.7) mm, P< 0.05; (16.9±2.1) mm vs. (22.6±3.3) mm, P< 0.05; (11.6±4.0) mmHg vs. (26.8±19.6) mmHg, P< 0.05, respectively) compared with values immediately after ablation. Anterior interventricular septum and posterior interventricular septum decreased after 3 and 6 months ((14.8±1.7) mm and (13.4±2.0) mm vs. (17.5±2.0) mm, all P< 0.05; (12.9±1.9) mm and (12.3±2.4) mm vs. (16.9±2.1) mm, all P< 0.05, respectively) compared with values at 1 month after ablation.There were no significantly difference in left ventricular outflow tract gradient at 3 and 6 months post procedure compared with 1 month after ablation (all P> 0.05). Compared with pre-operation, provoked left ventricular outflow tract gradient decreased ((25.5±11.4) mmHg vs. (147.8±58.0) mmHg, P< 0.01), and total exercise time increased ((9.3±1.6) minutes vs. (6.7±1.6) minutes, P= 0.03) at 6 months after operation.The symptoms were disappeared in 5 patients. There were 2 cases with NYHA class Ⅱ and 7 cases with NYHA class Ⅲ before operation,while there were 6 patients with NYHA classⅠ and 3 patients with NYHA class Ⅱ at 6 months after operation ( P< 0.01). Conclusion: Transthoracic echocardiography-guided PIMSRA is a safe and effective new treatment approach for patients with obstructive hypertrophic cardiomyopathy.

Objective: To evaluate the predicting value of the 2014 European Society of Cardiology (ESC) guidelines risk prediction model for sudden cardiac death (HCM Risk-SCD) in Chinese patients with hypertrophic cardiomyopathy (HCM), and to explore the predictors of adverse cardiovascular events in Chinese HCM patients. Methods: The study population consisted of a consecutive 207 HCM patients admitted in our center from October 2014 to October 2016. All patients were followed up to March 2017. The 5-year SCD probability of each patient was estimated using HCM Risk-SCD model based on electrocardiogram, echocardiography and cardiac magnetic resonance (CMR) examination results. The primary, second, and composite endpoints were recorded. The primary endpoint included SCD and appropriate ICD therapy, identical to the HCM Risk-SCD endpoint. The second endpoint included acute myocardial infarction, hospitalization for heart failure, thrombus embolism and end-stage HCM. The composite endpoint was either the primary or the second endpoint. Patients were divided into the 3 categories according to 5-year SCD probability assessed by HCM Risk-SCD model: low risk group<4%,intermediate risk group ≥4% to<6%, and high risk group≥6%. Results: (1) Prevalence of endpoints: All 207 HCM patients completed the follow-up (350 (230, 547) days). During follow-up, 8 (3.86%) patients reached the primary endpoints (3 cases of SCD, 3 cases of survival after defibrillation, and 2 cases of appropriate ICD discharge); 21 (10.14%) patients reached the second endpoints (1 case of acute myocardial infarction, 16 cases of heart failure hospitalization, 2 cases of thromboembolism, and 2 cases of end-stage HCM). (2) Predicting value of HCM Risk-SCD model: Patients with primary endpoints had higher prevalence of syncope and intermediate-high risk of 5-year SCD, as compared to those without primary endpoints (both P< 0.05). (3) Predicting value of HCM Risk-SCD model: The low risk group included 122 patients (59%), the intermediate risk group 42 (20%), and the high risk group 43 (21%). There was a clear trend towards to higher heart rate, higher values of PTF(V1) and plane QRS-T angle, higher left ventricular mass index (LVMI), elevated maximal left ventricular outflow tract pressure gradient (LVOT-PGmax), enlarged left atrial dimension(LAD) and volume index (LAVI), reduced systolic mitral annular velocity (s ' ), and higher late gadolinium enhancement (LGE) volume and mass in patients with high risk of 5-year of SCD, as compared to those with low-intermediate risk (all P< 0.05). Moreover, 5-year SCD probability was positively correlated with heart rate, plane QRS-T angle, LVMI, LAVI, LGE%, and negatively correlated with s ' ( r= 0.161, P= 0.019; r= 0.669, P= 0.001; r= 0.206, P= 0.004; r= 0.284, P= 0.000; r= 0.351, P= 0.000; r= - 0.245, P= 0.001; respectively). (4) LAD, LAVI, e ' and s ' were independent predictors for poor outcomes. HCM patients with LAD≥39 mm, LAVI≥49.6 ml/m(2), e ' ≤6.5 cm/s and s ' ≤6.6 cm/s were more likely to have adverse cardiovascular events (AUC 0.702, 95% CI 0.604 - 0.799, P= 0.001; AUC 0.700, 95% CI 0.567 - 0.833, P= 0.001; AUC 0.716, 95% CI 0.616 - 0.817, P= 0.000; AUC 0.764, 95% CI 0.676 - 0.853, P= 0.000,respectively). Conclusions: The HCM Risk-SCD model is of value in predicting SCD for Chinese HCM patients. The plane QRS-T angle and LGE% are the best predictors of 5-year SCD risk in Chinese HCM patients. Moreover, conventional echocardiographic parameters, including LAD, LAVI, e ' and s ' , are useful to predict adverse cardiovascular events among Chinese HCM patients.

Objective: To identify the potential mutations in a Chinese pedigree with hypertrophic cardiomyopathy (HCM), and to analyze the genotype-phenotype relationship in this pedigree., Methods: Clinical history and physical examinations, electrocardiography (ECG), echocardiography (UCG), cardiac magnetic resonance (CMR) data were obtained from 10 members of a three-generation Chinese family with HCM. A total of 96 genes related to hereditary cardiomyopathy were detected by exon and boarding intron analyses in the proband using second-generation sequencing. Mutations identified in the proband were confirmed by bi-directional Sanger sequencing in the rest 9 family members and 300 healthy controls., Results: Three mutations, including MYBPC3-P1208fs, ANK2-H556R and ANK2-P1974H, were identified in this pedigree. MYBPC3-P1208fs gene mutation was detected in 3 family members (proband, his mother and son), while this mutation was not detected in the rest family members. HCM was diagnosed in the proband and his mother by ECG, UCG and CMR. Son of the proband demonstrated early phenotype of HCM: although UCG and CMR were normal, ECG showed sinus bradycardia and paroxysmal supraventricular arrhythmias as well as ST segment changes. The onset age of HCM diagnosis of the proband and his mother was 42 and 50 years old, presented with palpitation and chest pain, and myocardial fibrosis sign in CMR. Furthermore, we found that left ventricular myocardial fibrosis is related to ECG changes (increasing r wave, ST segment change) in the proband and his mother. No HCM phenotype was evidenced in the 7 family members carrying ANK2-H556R and ANK2-P1974H mutations., Conclusions: Our results show that MYBPC3-P1208fs gene mutation is associated HCM phenotype in this Chinses pedigree. This mutation is associated with myocardial fibrosis and ST changes in HCM phenotype in this pedigree while ANK2-H556R and ANK2-P1974H mutations are not related to HCM phenotype in this family.

The health services management in Taiwan (HSMT) was established at the Qing dynasty of China and has been developed for over 150 years. After the dominion of foreign conturies, the transition of space and time, and the impact of worldwide medical therapy, the HSMT has become a modernized, diversified and unique system.