Objective To optimize the processing technology of Curculigo orchioides Gaertn.Methods On the basis of single factor experiment,the ratio of material to liquid,frying temperature and frying time were taken as the influencing factors,and the comprehensive scores of appearance traits,curculigoside,orcinol glucoside and orcinol gentiobioside were taken as the evaluation indexes.The optimal processing technology was optimized by Box-Behnken response surface method combined with analytic hierarchy process,and the process verification was carried out.Results The optimal process was as follows:the raw C.orchioides pieces were mixed with the same amount of Evodia rutaecarpa juice,placed in a closed container,moistened until the juice was completely absorbed,stir-fried at 130 ℃ for 6 min,taken out and dried at 60 ℃.For each 100 kg of Curculigo,10 kg of Evodia rutaecarpa was used.Evodia rutaecarpa juice preparation method:10 kg of Evodia rutaecarpa was soaked in 12 times the amount of water for 60 min,decocted for 50 min,extracted for 3 times,combined with the filtrate,and concentrated to 100 kg to obtain Evodia rutaecarpa juice.Conclusion The optimal processing technology of Curculigo orchioides Gaertn.is stable and feasible.

Objective:To analyze and summarize clinical phenotypic characteristics and genetic variations in patients with intellectual disability and pathogenic variations of the DYNC1H1 gene across 4 generations within a single family. Methods:Retrospective case analysis.Clinical data of a child with epilepsy and intellectual disability and her family members were collected from the Children′s Medical Center, Peking University First Hospital on December 2019.The child was followed up regularly.DNA was extracted from the peripheral blood of the child′s family members.Then whole-exome sequencing and Sanger sequencing were performed to identify the genetic variation type in the proband and her family members.The relationship between genotype and phenotype was further analyzed.Results:A total of 13 patients across 4 generations in the family had intellectual disability, and the proband also had drug-resistant epilepsy.The variation c. 13556C> A (p.A4519E) of the DYNC1H1 gene was confirmed by gene testing in 8 patients (no blood samples were obtained from the remaining patients). Conclusions:DYNC1H1 gene-related intellectual disability in most previously reported cases are caused by novel variations of this gene.In this study, a large family of 13 intellectual disability patients across 4 generations caused by a pathogenic mutation in the DYNC1H1 gene was summarized.The findings make precise genetic counseling possible for this family and provide a basis for further studies on the relationship between the genotype and phenotype of the DYNC1H1 gene.

Objective:To analyze and summarize clinical phenotypic characteristics and genetic variations in patients with intellectual disability and pathogenic variations of the DYNC1H1 gene across 4 generations within a single family. Methods:Retrospective case analysis.Clinical data of a child with epilepsy and intellectual disability and her family members were collected from the Children′s Medical Center, Peking University First Hospital on December 2019.The child was followed up regularly.DNA was extracted from the peripheral blood of the child′s family members.Then whole-exome sequencing and Sanger sequencing were performed to identify the genetic variation type in the proband and her family members.The relationship between genotype and phenotype was further analyzed.Results:A total of 13 patients across 4 generations in the family had intellectual disability, and the proband also had drug-resistant epilepsy.The variation c. 13556C> A (p.A4519E) of the DYNC1H1 gene was confirmed by gene testing in 8 patients (no blood samples were obtained from the remaining patients). Conclusions:DYNC1H1 gene-related intellectual disability in most previously reported cases are caused by novel variations of this gene.In this study, a large family of 13 intellectual disability patients across 4 generations caused by a pathogenic mutation in the DYNC1H1 gene was summarized.The findings make precise genetic counseling possible for this family and provide a basis for further studies on the relationship between the genotype and phenotype of the DYNC1H1 gene.

Objective To optimize the processing technology of Curculigo orchioides Gaertn.Methods On the basis of single factor experiment,the ratio of material to liquid,frying temperature and frying time were taken as the influencing factors,and the comprehensive scores of appearance traits,curculigoside,orcinol glucoside and orcinol gentiobioside were taken as the evaluation indexes.The optimal processing technology was optimized by Box-Behnken response surface method combined with analytic hierarchy process,and the process verification was carried out.Results The optimal process was as follows:the raw C.orchioides pieces were mixed with the same amount of Evodia rutaecarpa juice,placed in a closed container,moistened until the juice was completely absorbed,stir-fried at 130 ℃ for 6 min,taken out and dried at 60 ℃.For each 100 kg of Curculigo,10 kg of Evodia rutaecarpa was used.Evodia rutaecarpa juice preparation method:10 kg of Evodia rutaecarpa was soaked in 12 times the amount of water for 60 min,decocted for 50 min,extracted for 3 times,combined with the filtrate,and concentrated to 100 kg to obtain Evodia rutaecarpa juice.Conclusion The optimal processing technology of Curculigo orchioides Gaertn.is stable and feasible.

Pulmonary arterial hypertension(PAH)is a complex pulmonary vascular disease characterized by pro-gressive elevation of mean pulmonary artery pressure resulted from the pathological feature of pulmonary vascular re-modeling.Without medical intervention,PAH can eventually lead to right heart failure and death of patients.Up to the present,there are few treatment options for PAH are still mainly function through pulmonary vasodilation.Although these treatments can alleviate symptoms,the prognosis remains poor.In recent years,breakthroughs have been made in understanding the pathogenesis of PAH,thus support the development of new treatment strategies tar-geting at dysregulation of signaling pathways in PAH.This review focuses on five critical pathways and the relevant drugs those entered phase Ⅱ clinical trials and discusses their therapeutic potential,so to provide a basis for future research on targeting therapies for PAH patients.

OBJECTIVE To optimize the honey processing technology of Phellinus igniarius. METHODS The key factors of honey processing technology of P. igniarius （honey-water ratio， the mass ratio of honey-water to P. igniarius， the frying temperature and the frying time） were investigated by orthogonal test combined with analytic hierarchy process to determine the optimal technological parameters， using the internal quality （the contents of ergosterol， protocatechuic aldehyde and protocatechuic acid） and appearance traits as evaluation indexes. RESULTS The optimal process of honey-roasting P. igniarius was to take raw P. igniarius （1 cm3 square block）， add the appropriate amount of auxiliary materials （with 25 kg of refined honey and water for every 100 kg of P. igniarius）， mix well， moisten for 2 h until the auxiliary materials were completely absorbed； put it in a frying container， fry at the frying temperature of 130-140 ℃ for 5 min； take it out， put it in an oven at 50 ℃ for 2 h； take it out， and let it cool. The RSD of the results of three validation experiments was 0.68%. CONCLUSIONS The optimized honey processing technology of P. igniarius is stable and feasible.

OBJECTIVE To optimize the honey processing technology of Phellinus igniarius. METHODS The key factors of honey processing technology of P. igniarius （honey-water ratio， the mass ratio of honey-water to P. igniarius， the frying temperature and the frying time） were investigated by orthogonal test combined with analytic hierarchy process to determine the optimal technological parameters， using the internal quality （the contents of ergosterol， protocatechuic aldehyde and protocatechuic acid） and appearance traits as evaluation indexes. RESULTS The optimal process of honey-roasting P. igniarius was to take raw P. igniarius （1 cm3 square block）， add the appropriate amount of auxiliary materials （with 25 kg of refined honey and water for every 100 kg of P. igniarius）， mix well， moisten for 2 h until the auxiliary materials were completely absorbed； put it in a frying container， fry at the frying temperature of 130-140 ℃ for 5 min； take it out， put it in an oven at 50 ℃ for 2 h； take it out， and let it cool. The RSD of the results of three validation experiments was 0.68%. CONCLUSIONS The optimized honey processing technology of P. igniarius is stable and feasible.

Objective:To design a replaceable corneal scaler and explore its effect on improving the positive detection rate of corneal bacteria and the cure rate of patients with infectious keratitis.Methods:Totally 100 patients with infectious keratitis treated in the Eye&Ent Hospital of Fudan University in Shanghai from June 2020 to October 2021 were conveniently selected as the application object, 50 patients from June 2020 to January 2021 were selected as the control group, and 50 patients from February 2021 to October 2021 were selected as the experimental group. The control group used the traditional corneal scalpel for corneal scraping, and the experimental group used the replaceable corneal scalpel for corneal scraping. The positive detection rate of bacteria, the cure rate of patients as of the date of data collection, the comfort status of patients and the operation difficulty score of medical staff were compared between the two groups.Results:The 28 cases (56%) were positive in the experimental group, and 30 cases (60%) were cured as of the date of data collection; 16 cases (32%) were positive in the control group and 18 cases (36%) were cured as of the date of data collection. The difference between the two groups was statistically significant ( χ2=-2.47, -2.20, all P<0.05). The score of comfort in the experimental group was (83.92 ± 6.40) and that in the control group was (54.04 ± 10.69). The difference between the two groups was statistically significant ( t=17.14, P<0.01). The operation difficulty score of medical staff in the experimental group was (1.46 ± 0.54) and that of medical staff in the control group was (3.24 ± 0.72). The difference between the two groups was statistically significant ( t=-13.50, P<0.01). Conclusions:The use of replaceable corneal scalpel can not only improve the positive detection rate of patients, improve the treatment effect of patients, improve the comfort and satisfaction of patients, but also improve the work efficiency of medical staff and reduce the operational risk of medical staff, so as to improve the real professional value of medical staff.

ObjectiveTo discuss the characteristics of psychological assistance hotline calls and operators' coping strategies of before and after the COVID-19 outbreak， in order to further improve the assistance ability of the psychological crisis hotline. MethodsA retrospective study was conducted on the demographics characteristics， call problems， coping strategies， and call time trends recorded by Changchun psychological assistance hotline information registration platform before the epidemic in Changchun City （January 20， 2019-April 20， 2019） and during the epidemic period （January 20， 2020-April 20， 2020）. ResultsThe differences between gender， age， marital status， location， and occupation type before and during the epidemic were statistically significant （χ²=11.205， 234.240， 152.083， 265.458， 353.385， P<0.01）. The number of different help calls had a statistically significant difference before and during the epidemic （χ²=185.088，P<0.01）. The difference in the number of operators’ different coping strategies before and during the epidemic was statistically significant （χ²=226.810， P<0.01）. Before the epidemic， the main peak of incoming calls was concentrated at 16∶00 to 17∶00， and the secondary peak was concentrated at 22∶00 to 23∶00. During the epidemic， the main peak of incoming calls was also concentrated at 16∶00 to 17∶00， while the secondary peak was concentrated at 10∶00 to 11∶00. ConclusionDuring the COVID-19 epidemic， the number of calls to the psychological assistance hotline was higher than that before the outbreak. The main peak time for calls was the same， and the secondary peak was adjusted from 22∶00 to 23∶00 to 10∶00 to 11∶00. During the epidemic， the number of calls from male， 30 to 39 years old， married， local and staff in Changchun was the most， psychological problems counseling and operator referral strategy were the most before and after the epidemic.

Objective To investigate the changes of left ventricular systolic and diastolic function before left ventricular morphologic changes in obstructive sleep apnea syndrome (OSAS) patients.Methods A total of 111 OSAS patients were divided into left ventricular hypertrophy (LVH) group (n=29) and non-LVH group (n=82).Meanwhile,50 healthy subjects were enrolled as normal control group.Routine echocardiography and two-dimensional speckle tracking imaging (2D-STI) were performed.The differences of conventional echocardiography and 2D-STI parameters were compared among the three groups.The correlations between echocardiography and clinical parameters were analyzed.Results Compared with those of the other 2 groups,left ventricular mass index (LVMI),diastolic thickness of interventricular septum (IVST),diastolic ventricular posterior wall thickness (PWT),left ventricular internal diastolic dimension (LVIDd),the ratio between early diastolic peak velocity of mitral valve and early diastolic velocity of mitral annular (E/e') and left atrial volume index (LAVI) increased (all P＜0.05),and mitral annular early diastolic velocity (e') at interventricular septum and lateral wall decreased in LVH group (all P＜0.05).The mitral annular systolic velocity (s') in LVH group was less than that in normal control group (P =0.013).Compared with those of the other 2 groups,left ventricular global longitudinal systolic strain (S) and early diastolic strain rate (SRE) decreased (all P＜0.05),and the ratio of early diastolic peak velocity of mitral valve to SRE (E/SRE) increased in LVH group (both P＜0.05).Compared with normal control group,systolic strain rate (SRS) decreased in LVH group (P=0.001).S,SRS,SRE in non-LVH group were less than those in normal control group (all P＜0.05),and E/SRE was higher than that in normal control group (P＜0.001).S,E/SRE were independently associated with apnea hypopnea index (both P＜0.05).LVMI was independently associated with mean arterial oxygen saturation (β =-0.299,t =-3.273,P =0.001).Conclusion OSAS can affect the structure and functions of left ventricular independently.The systolic and diastolic functions of left ventricular have been impaired before morphology changed.