Objective To summarize and analyze the clinical phenotypes,hereditary features and treatment and follow-up strategies of different hereditary pheochromocytoma/paragangliomas(PCC/PGL)and related syndromes.Methods Forty-four clinically diagnosed PCC/PGL patients admitted in our hospital from January 2000 to August 2022 were enrolled,and the clinical data of them and their family members were collected.Second-generation sequencing was performed on 43 patients for genetic detection,and Sanger sequencing was applied to verify the mutation of the probands and family members.Results There were 15 patients diagnosed with hereditary PCC/PGL,including 7 cases of von Hippel-Lindau(VHL)syndrome,3 cases of multiple endocrine neoplasia type 2(MEN2),and 5 cases of familial paraganglioma syndrome.Seven VHL syndrome families were diagnosed as VHL2A(c.500G＞A),VHL2B(c.239G＞T and c.444_457del),and VHL2C(c.293A＞G)according to their clinical manifestations.All probands received surgical treatment,and 2 cases of recurrent PCC and the patients with multiple renal cancer also received targeted therapy with sunitinib.Three MEN2 families carried c.1901G＞C,c.1832G＞A,and c.1901G＞A missense mutations,respectively,and were diagnosed with MEN2A clinically.All of them underwent adrenalectomy and thyroidectomy,including one for preventive thyroidectomy.Among the 5 familial paraganglioma syndrome families,4 patients carried SDHB mutations(SDHB:c.343C＞T,c.541-2A＞G,c.575G＞A,c.268C＞T)and 1 patient carried an SDHD mutation(SDHD:c.337_340del).Sporadic retroperitoneal PGL were most common.Conclusion More than 1/3 of PCC/PGL patients carry germline gene mutations,showing obvious genotype-phenotype correlation.Genetic diagnosis technology plays an important guidance role for clinical precision treatment and follow-up,and genetic counseling.

Objective:To investigate the effects of Sacubitril/Valsartan on the recurrence of atrial fibrillation(AF), cardiac structure and function after catheter ablation(CA)of persistent AF.Methods:A total of 180 patients with persistent AF were randomly divided into the treatment group(n=90)and the control group(n=90)before undergoing CA.The control group was given routine perioperative medication according to the guidelines for AF management, and the treatment group was additionally given Sacubitril/Valsartan.Blood pressure, N-terminal pro-B-type natriuretic peptide(NT-proBNP), P-wave dispersion, left atrial dimension, left atrial volume index, mitral regurgitation area, left ventricular end-systolic volume index, end-diastolic volume index and left ventricular ejection fraction(LVEF)were measured at 3, 6 and 12 months after the procedure.Results:Blood pressure was lower in the treatment group than in the control group( P<0.05), but no hypotension or related symptoms occurred.The sinus rhythm maintenance rate was higher in the treatment group than in the control group at 3, 6 and 12 months during the follow-up(80.0%, 72.2%, 78.9% vs.70.0%, 75.6%, 68.9%, χ2=4.866, 6.667 and 4.091, P=0.027, 0.010 and 0.043, respectively). At 6 and 12 months during the follow-up, NT-proBNP, P-wave dispersion, left atrial diameter, left atrial volume index, mitral regurgitation area, left ventricular end-systolic volume index and end-diastolic volume index were lower(all P<0.05)and LVEF was higher( P<0.05)in the treatment group than in the control group. Conclusions:In patients with persistent AF after CA, Sacubitril/Valsartan has favorable effects in maintaining sinus rhythm, reversing cardiac remodeling and improving cardiac function.

Objective To explore the feasibility of 3D printed individualized applicator for the intracavitary HDR-brachytherapy for nasopharyngeal carcinoma.Methods CT scan was performed in 1 case of recurrent rT1 nasopharyngeal carcinoma and 1 case of T2 residual nasopharyngeal carcinoma and the obtained images were transmitted to 3D image processing software.The geometric contour parameters of the nasopharyngeal cavity were obtained and a pipeline was designed to make it close to the recurrent gross tumor volume (rGTV).Individualized cavity applicators were created by using 3D printer.The applicator was inserted into the patient's nasopharyngeal cavity through oral cavity.The source tube and false source were inserted into the preset pipe of the applicator.CT scan was performed again and the images were transmitted to the 3D brachytherapy planning system.Mter delineating the target volume and organ at risk,treatment plan was optimized.After completing the first treatment,the applicator was removed.Before second treatment in a few days,CT scan was reviewed to confirm whether the position was correct.Results When the applicator was inserted into the nasopharyngeal cavity,it could be fully aligned with the nasopharyngeal wall and self-fixed without additional fixation measures.Comparing the location of false source in multiple reviews of CT scan,the error was ≤ 1 mm.No significant discomfort was reported throughout the treatment.In optimized three-dimensional treatment,100％ prescription dose curve included the full rGTV,maximum dose of the brain stem and spinal cord was＜30％ prescription dose.Recurrent patients were given with a prescription dose of DT 40Gy/8 fractions/4 weeks and patients with residual tumors were given with 12Gy/2 fractions/1 week.No tumor recurrence was observed at postoperative 3 months in two cases.Conclusions The 3D printed individualized nasopharyngeal intracavitary applicator has the advantages of self-fixation,accurate location,good repeatability and good patient tolerance.The short-term outcome is effective,whereas its long-term clinical effect and adverse reactions need to be further observed.

PURPOSE: Details of patients hospitalized for asthma exacerbation in mainland China are lacking. To improve disease control and reduce economic burden, a large sample survey among this patient population is indispensable. This study aimed to investigate the clinical characteristics and outcomes of such patients.METHODS: A retrospective study was conducted on patients hospitalized for asthma exacerbation in 29 hospitals of 29 regions in mainland China during the period 2013 to 2014. Demographic features, pre-admission conditions, exacerbation details, and outcomes were summarized. Risk factors for exacerbation severity were analyzed.RESULTS: There were 3,240 asthmatic patients included in this study (57.7% females, 42.3% males). Only 28.0% used daily controller medications; 1,287 (39.7%) patients were not currently on inhaled corticosteroids. Acute upper airway infection was the most common trigger of exacerbation (42.3%). Patients with severe to life-threatening exacerbation tended to have a longer disease course, a smoking history, and had comorbidities such as hypertension, chronic obstructive pulmonary disease (COPD), and food allergy. The multivariate analysis showed that smoking history, comorbidities of hypertension, COPD, and food allergy were independent risk factors for more severe exacerbation. The number of patients hospitalized for asthma exacerbation varied with seasons, peaking in March and September. Eight patients died during the study period (mortality 0.25%).CONCLUSIONS: Despite enhanced education on asthma self-management in China during recent years, few patients were using daily controller medications before the onset of their exacerbation, indicating that more educational efforts and considerations are needed. The findings of this study may improve our understanding of hospital admission for asthma exacerbation in mainland China and provide evidence for decision-making.
Adrenal Cortex Hormones ; Asthma ; China ; Comorbidity ; Disease Progression ; Education ; Female ; Food Hypersensitivity ; Hospitalization ; Humans ; Hypertension ; Inpatients ; Medication Adherence ; Mortality ; Multivariate Analysis ; Pulmonary Disease, Chronic Obstructive ; Retrospective Studies ; Risk Factors ; Seasons ; Self Care ; Smoke ; Smoking

OBJECTIVE:To observe therapeutic efficacy and safety of Shensong yangxin capsule combined with low-dose ami-odarone in the treatment of ventricular tachyarrhythmia. METHODS:In retrospective analysis,106 patients with ventricular tachyar-rhythmia were divided into control group and observation with 53 cases in each group according to drug use. Based on conventional treatment,control group was additionally given Amiodarone tablet (first week:0.2 g each time,3 times a day;second week:0.2 g each time,2 times a day;third week to the end of the treatment:0.2 g each time,1 times a day),orally. Observation group was given Amiodarone tablet (first week:0.2 g each time,twice a day;second week to the end of the treatment:0.2 g each time,1 times daily),orally +Shensong yangxin capsule 1.6 g,3 times a day,orally. Both groups were treated for 8 weeks. Clinical effica-cy,blood pressure,heart rate and electrocardiogram(PR period and QT period)and the cardiac cardioversion time were compared between 2 groups,and the occurrence of ADR was also recorded during treatment. RESULTS:After treatment,total response rate of observation group(94.34%)was higher than that of control group(81.13%),the incidence of ADR was lower than that of con-trol group (3.77% vs. 13.21%) and the cardiac cardioversion time was shorter than that of control group,with statistical signifi-cance(P<0.05). Before treatment,there was no statistical significance in heart rate between 2 groups;after treatment,heart rate of 2 groups were significantly lower than before,and observation group was significantly lower than control group,with statistical significance(P<0.05). There were no statistical significance in blood pressure、PR period and QT period between 2 groups before and after treatment (P>0.05). CONCLUSIONS:Based on conventional treatment, Shensong yangxin capsule combined with low-dose amiodarone have good therapeutic efficacy with rapid response for ventricular arrhythmia,it can significantly improve the heart rate of the patients,and the effect is faster with good safety .

40 patients,20 males and 20 females,with 52 miniscrews were included.The concentration of IL-1β,IL-6 and IL-8 in the gingival crevicular fluid around the miniscrews were measured.The results showed that the concentrations of IL-1β,IL-6 and IL-8 increased during orthodintic tooth movement(P ＜0.05),indicating that orthodontic force had an effect on the cytokines around the miniscrews,which may be related to orthodontic bone resorption.

Objective To study the treatment effect of cinnamaldehyde on Aspergillus fumigatus biofilm(BF) in vitro .Meth‐ods The models of A .fumigatus BF were established in vitro ;the minimum inhibitory concentration(MIC) on A .fumigatus was measured .The crystal violet assay and scanning electron microscopy were employed to determined the treatment effect of A .fumiga‐tus biofilm under varying concentrations of cinnamaldehyde .Results BF models were established successfully in vitro .MIC value of A .fumigatus of cinnamaldehyde was 256 μg/mL ;The biofilm biomass in serially increasing concentrations of cinnamaldehyde(1 MIC ,1/2 MIC ,1/4 MIC)were 0 .81 ± 0 .11 ,1 .13 ± 0 .18 and 1 .59 ± 0 .11 respectively .Compared to untreated control group(2 .18 ± 0 .15) ,difference achieved statistical significance(P<0 .05) .SEM studies revealed the deformity of three‐dimensional structures of biofilms treated with sub‐MICs of cinnamaldehyde .Conclusion Cinnamaldehyde has significant antifungal activity against Aspergil‐lus fumigatus ,sub‐MICs could disrupt the mature biofilm in vitro .

Purpose To investigate the clinicopathologic features, diagnosis and differential diagnosis of primary gastric inflammatory myofibroblastic tumor ( IMT) . Methods Four cases of gastric IMTs were studied by clinicopathologic analysis, immunohistochemistry and in situ hybridization, and the related literature was reviewed. Results In four cases there are two males and two females, age range from 21 to 51 years old, and tumor size ranged from 1. 5 to 6. 5 cm in the greatest dimension. Histologically, these tumors were composed of varied spindle cells and chronic inflammatory cells, in a myxoid or hyalinized stroma. Occasionally, there were calcifica-tion and ossification areas. Most of the spindle cells had bland appearance and a minority of the tumor cells showed mild atypia. One to two mitotic figures were recognized in 10 high power fields ( HPFs) in 1 to 2 patients. Smooth muscle actin staining was observed in all tumors and ALK staining observed in two tumors. One tumor focally expressed CD34. S-100, desmin, CD68, CD117 and DOG1 was negative in all IMTs. The patients were followed up from 24 to 66 months, and none of them had tumor relapsed or metastasis. Conclu-sions Primary gastric IMTs have an intermediate behavior, and a few cases have malignant potential. It should be distinguished from other spindle cell lesions similar to IMT.

Objective To evaluate the in vitro antifungal activity of Cinnamaldehyde in combination with Voriconazole (VRC) against clinically isolated Aspergillus fumigatus strains. Methods According to the Clinical and Laboratory Stan?dards Institute (CLSI) M38-A2 document,the minimal inhibitory concentrations (MIC) of Cinnamaldehyde and VRC alone or in combination against 42 clinical Aspergillus fumigatus isolates were determined by both microdillution method and check?board method respectively. The MIC50, MIC90, MICG and MICs distribution were compared between single drug and both in combination. The concentration-accumulative curve was drawn and fractional inhibitory concentration index (FICI) was cal?culated to evaluate the interaction between two test agents. Results The values of MIC50, MIC90 and MICG were significant?ly decreased (P<0.001) when combination of the two drugs than those of their single use, with their MIC distribution concen?tration-accumulative curves shifted to the left. The value of FICI of Cinnamaldehye-VRC combination ranged from 0.187 5 to 1.5. Sixteen strains (38.10%) of them showed the synergistic effect, 19 strains (45.23%) showed additive effect, and 7 strains (16.67%) showed an unrelated effect, and no antagonist effect on tested Aspergillus fumigatus strains in vitro. Conclu?sion Cinnamaldehye in combination with VRC mainly shows a combined synergic and additive inhibitory effect on Asper?gillus fumigatus isolates, and this combination appears to be more active against the test strains, which are less susceptible to voriconazole.

BACKGROUNDRadiofrequency catheter ablation (RFCA) for atrial fibrillation (AF) has developed rapidly, and is a commonly performed ablation in many major hospitals throughout the world, due to its satisfactory results. The aim of this study was to detect the effect of RFCA on C-reactive protein (CRP), brain natriuretic peptide (BNP), and echocardiograph in patients with persistent and permanent AF.

METHODSA total of 120 patients (71 males, mean age (50.8 ± 12.0) years) with persistent and permanent AF undergoing RFCA under guidance of the Carto merge technique were studied. Left atrial diameter (LAD), right atrial diameter (RAD), left ventricular ejection fraction (LVEF), CRP, and BNP were observed 3, 6 and 12 months after RFCA and compared with results before RFCA. The recurrence of atrial arrhythmias was observed 3 and 12 months after the procedure.

RESULTSCompared with that before RFCA, LAD and RAD decreased and LVEF increased significantly after RFCA. Meanwhile, the levels of CRP and BNP were reduced significantly at 3, 6, and 12 months after RFCA (P < 0.05). In the non-recurrent patients, LVEF was increased significantly compared with the recurrent patients at 3, 6, and 12 months after RFCA (P < 0.05). CRP and BNP levels were decreased significantly in the non-recurrent patients compared with the recurrent patients at 3, 6, and 12 months after RFCA (P < 0.05). After one or two applications of RFCA, during a follow-up of 12 months, 12 patients (10.0%) had AF, 10 patients (8.3%) had atrial flutter, and 5 patients had atrial tachycardia (4.2%).

CONCLUSIONSConversion of AF to sinus rhythm by RFCA, has been shown to reduce LA size and improve LVEF. It can also significantly decrease the levels of CRP and BNP in patients with persistent and permanent AF and reduce the risk of inflammation and developing heart failure.

Atrial Fibrillation ; blood ; diagnostic imaging ; surgery ; C-Reactive Protein ; analysis ; Catheter Ablation ; Echocardiography ; Female ; Humans ; Male ; Middle Aged ; Natriuretic Peptide, Brain ; blood