Objective To investigate the therapeutic mechanism of Pterocarya hupehensis Skan total flavonoids(PHSTF)for rheumatoid arthritis(RA).Methods Twenty-five male SD rats were randomly divided into normal control group,RA model group,PHSTF treatment(45 and 90 mg/kg)groups,and Tripterygium glycosides(TPG)tablet(10 mg/kg)group(n=5).Except for those in the normal control group,all the rats were subjected to collagen-induced arthritis(CIA)modeling using a secondary immunization method,after which PHSTF and TPG were administered via gavage once daily for 4 weeks.After the treatments,serum levels of TNF-α and IL-1β were measured using ELISA,and ankle joint pathologies were assessed with HE staining;the expression of citrullinated histone H3(Cit-H3),a neutrophil extracellular trap(NET)marker,in the ankle joints was evaluated with immunohistochemistry.In primary cultures of rat peripheral blood neutrophils stimulated with phorbol ester(PMA),the effects of PHSTF(100 and 200 μg/mL)on the expressions of Cit-H3,peptidylarginine deiminase 4(PADI4),neutrophil elastase(NE),and myeloperoxidase(MPO)were examined with Western blotting;immunofluorescence assay was used to observe Cit-H3 expression and NET formation in the cells.Results In the CIA rat models,PHSTF significantly alleviated ankle swelling,decreased serum levels of TNF-α and IL-1β,improved histopathological changes in the ankle joints,and reduced Cit-H3 expression in both the serum and ankle joint cartilage.In the isolated rat neutrophils,PHSTF showed no significant effect on cell viability but strongly inhibited PMA-induced NET release.Conclusion PHSTF can alleviate RA by inhibiting the formation of NETs.

Objective To investigate the therapeutic mechanism of Pterocarya hupehensis Skan total flavonoids(PHSTF)for rheumatoid arthritis(RA).Methods Twenty-five male SD rats were randomly divided into normal control group,RA model group,PHSTF treatment(45 and 90 mg/kg)groups,and Tripterygium glycosides(TPG)tablet(10 mg/kg)group(n=5).Except for those in the normal control group,all the rats were subjected to collagen-induced arthritis(CIA)modeling using a secondary immunization method,after which PHSTF and TPG were administered via gavage once daily for 4 weeks.After the treatments,serum levels of TNF-α and IL-1β were measured using ELISA,and ankle joint pathologies were assessed with HE staining;the expression of citrullinated histone H3(Cit-H3),a neutrophil extracellular trap(NET)marker,in the ankle joints was evaluated with immunohistochemistry.In primary cultures of rat peripheral blood neutrophils stimulated with phorbol ester(PMA),the effects of PHSTF(100 and 200 μg/mL)on the expressions of Cit-H3,peptidylarginine deiminase 4(PADI4),neutrophil elastase(NE),and myeloperoxidase(MPO)were examined with Western blotting;immunofluorescence assay was used to observe Cit-H3 expression and NET formation in the cells.Results In the CIA rat models,PHSTF significantly alleviated ankle swelling,decreased serum levels of TNF-α and IL-1β,improved histopathological changes in the ankle joints,and reduced Cit-H3 expression in both the serum and ankle joint cartilage.In the isolated rat neutrophils,PHSTF showed no significant effect on cell viability but strongly inhibited PMA-induced NET release.Conclusion PHSTF can alleviate RA by inhibiting the formation of NETs.

Objective:To explore the association between chest high resolution CT (HRCT) scoring and prognostic factors of interstitial lung disease (ILD) in patients with anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive dermatomyositis (DM).Methods:The patients with DM admitted to Kailuan General Hospital between January 2017 and December 2019 were included into the study including 13 patients with positiveanti-MDA5 antibody (7 survivors, 6 deaths) and 18 patients with anti-synthase (ARS)-antibody positive. All patients underwent chest HRCT prior to treatment. The consolidation, ground-glass opacity (GGO) and fibrosis were scored to assess HRCT findings. The clinical manifestations were compared between the two groups. Cox regression analysis adjusted for age and sex was used to determine the prognostic factors for anti-MDA5 antibody-related ILD.Results:Compared with ARS patients, glutamyl transferase (GGT) and ferritin levels were significantly higher in MDA5-ILD patients [70.0(37.0, 122.5) vs 21.0(16.5, 33.5), Z=-3.37, P=0.001; 977.0(502.5, 1 366.0) vs 307.1(72.3, 546.9) , Z=-3.44, P=0.001]. The cumulative survival rate was significantly lower in patients with positive anti-MDA5 antibody than in those with positive anti-ARS antibody (100% vs 70%, P=0.001). The DM complicated with acute/subacute interstitial pneumonia (A/SIP) were found to significantly relate to death. There were no significant differences in chest HRCT scoringbetween the survivors and the deceased patients [ HR=1.08, 95% CI(0.95, 1.23), P=0.229; HR=0.97, 95% CI(0.72, 1.30), P=0.814]. Conclusion:Anti-MDA5 antibody is an important index for early diagnosis of DM complicated with acute/subacute interstitial pneumonia (A/SIP). The chest HRCT scoreis is not associated with the prognosis of anti-MDA5 antibody-related ILD patients.

The epidemic of COVID-19 has become the topmost public health threat worldwide. The authors suggested that in addition to strengthening the organization and leadership of the abovementioned work, greater attention be paid to establishing and improving the prevention and control mechanism.Furthermore, special efforts should be given to the safety of the medical workers, by strengthening their infection monitoring and outbreak management. Medical workers in different work areas and positions should be placed under careful protection with due cleaning and disinfection measures. The protection during specimen collection, transportation and medical waste management should also be prioritized. Such special issues in the management of pediatric patients, as allocation of nurses, the screening and management of caregivers, the problem of breastfeeding, and the disinfection of children′s toys and diapers were discussed. This paper also put forward management suggestions for the outpatient department, isolation ward and other key departments, which aim to guide the prevention and control of COVID-19 nosocomial infection in the pediatric outpatient and ward.

The pneumonia caused by the novel coronavirus (2019-nCoV), which began in December 2019, has become the most serious public health problem, threatening people's health and life. This threat is posing a severe challenge on the diagnosis and treatment of 2019-nCoV infection, the prevention and control of hospital cross infection of medical staff. It is suggested that in addition to strengthening the organization and leadership of the abovementioned work, establishing and improving the prevention and control mechanism deserve greater attention. Furthermore, special attention should be given to the safety of the medical staff, strengthening their infection monitoring and outbreak management. Medical staff in different work areas and positions should be placed under careful protection, cleaning and disinfection measures. The protection during specimen collection, transportation and medical waste management should also be prioritized. This paper also put forward management suggestions for the outpatient department, isolation ward and other key departments. These measures are proposed to provide a guidance for the prevention and control of 2019-nCoV nosocomial infection in the pediatric outpatient and ward.

OBJECTIVE: To carry out genetic testing for two families affected with cobalamin C (cblC) and establish a rapid method for the detection of a hotspot pathogenic variant c.609G>A of the MMACHC gene by using a PCR-high-resolution melting curve (PCR-HRM) method. METHODS: Genomic DNA was extracted from peripheral blood samples of the probands and their parents. Potential variants of the MMACHC gene was analyzed by Sanger sequencing. The c.609G>A variant of the MMACHC gene was screened among 100 healthy children with the PCR-HRM method. RESULTS: Sanger sequencing revealed that proband 1 carried compound heterozygous variants c.394C>T and c.609G>A of the MMACHC gene, while proband 2 carried compound heterozygous variants c.482G>A and c.609G>A of the same gene. PCR-HRM analysis of the two probands and the 100 healthy children were consistent with the Sanger sequencing. CONCLUSION c.609G>A is a hotspot pathogenic variant of the MMACHC gene. The diagnosis of cblC may be rapidly attained through detection by PCR-HRM.

OBJECTIVE: To explore the genetic basis for a child with dihydropyrimidase (DHP) deficiency. METHODS: High-throughput sequencing was carried out for the child. Suspected variants were verified by using Sanger sequencing. RESULTS: The proband was found to carry compound heterozygous variants of the DPYS gene, namely c.1468C>T (a missense variant) and c.1339-1363del (a frameshifting variant). CONCLUSION The compound heterozygous variants of the DPYS gene probably underlie the DHP in this child. Above result has enabled genetic counseling and prenatal diagnosis for his parents.

OBJECTIVE: To analyze the molecular etiology of a Chinese child affected with dihydropyrimidinase deficiency. METHODS: Genomic DNA was extracted from peripheral blood samples of the family members. Pathogenic variant was determined by whole exome sequencing and verified by Sanger sequencing. RESULTS: The child was found to harbor homozygous c.905G>A (p.Arg302Gln) variants in exon 5 of the DPYS gene, for which her parents were both heterozygous carriers. CONCLUSION The homozygous c.905G>A (p.Arg302Gln) variants of the DPYS gene probably underlies the dihydropyrimidinase deficiency in the child. Above result has enabled genetic counseling and prenatal diagnosis for this family.
Amidohydrolases/genetics* ; Asian Continental Ancestry Group/genetics* ; Child ; Exons ; Female ; Humans ; Metabolism, Inborn Errors/genetics* ; Mutation ; Pedigree

Objective To investigate the relationship between serum uric acid ( UA) level and brachial?ankle pulse wave velocity ( baPWV) in patients with systemic lupus erythematosus ( SLE) and lupus nephritis (LN)??Methods A total of 110 hospitalized,out?patient and healthy examinees from January 2017 to September 2017 were selected from Kailuan General Hospital??They were divided into three groups:(1)Fifty?five healthy controls were examined at the same time,and those who had no history of hypertension, myocardial infarction and stroke were excluded by physical examination??(2)Thirty?four SLE patients without LN were diagnosed according to the SLE classification standard revised by the American Society of Rheumatology ( ACR) in 1997,excluding those with lupus nephritis??( 3) 21 SLE patients with LN were diagnosed according to the SLE classification standard revised by the American Society of Rheumatology (ACR) in 1997??Pearson correlation coefficient and multivariate linear regression model were used to analyze the related factors affecting baPWV??Results The level of baPWV and the proportion of baPWV (≥1400 cm／s) in SLE without LN group and SLE with LN group were higher than those in healthy control group (all P＜0??05)??In SLE without LN group, baPWV was positively correlated with age, systolic blood pressure (SBP) and total cholesterol ( CHOL) ( r＝ 0??623,0??528,0??402, P＜0??01 or P＜0??05), and negatively correlated with blood uric acid(UA) ( r＝－0??371,P＜0??05),but the correlation was not significant??The correlation between UA and baPWV disappeared after after correction of age,SBP,diastolic blood pressure (DBP) by partial correlation analysis??In SLE with LN group,baPWV was positively correlated with SBP, DBP and serum creatinine ( Cr) ( r＝0??815, 0??725, 0??464, P＜0??01 or P＜0??05)??Multivariate stepwise regression analysis showed that SBP was independently correlated with baPWV in SLE group ( t＝2??54,P＝0??026); UA in SLE group without LN was independently negatively correlated with baPWV(t＝－2??96,P＝0??042); UA(t＝4??24,P＝0??013) and SBP(t＝7??70,P＝0??002) were independently positively correlated with baPWV in SLE group with LN??Logistic regression analysis showed that SLE was a risk factor for baPWV (≥1 400 cm／s),and the OR (95% CI) was 4??31 ( 1??56－11??88),P＝0??005,and there was statistical significance after adjusting for age,SBP,DBP,body mass index ( BMI)??However,UA was not a risk factor for baPWV (≥1 400 cm／s) (P values were 0??163 and 0??519,respectively)??Conclusion The degree of arteriosclerosis in SLE patients is higher than that in normal subjects,and the level of UA in SLE patients may be related to baPWV??

Objective To investigate the correlation between cumulative serum uric acid (cumUA) and brachial-ankle pulse wave velocity (baPWV).Methods Among the workers who participated in the four health check-up of Kailuan Group from 2010 to 2017,subjects who completed one PWV test were selected.The subjects who met the selection criteria were 20 688,subjects who lacked the first three uric acid tests and sex data were excluded.The subjects who had ischemic stroke (excluding lacunar infarction),transient ischemic attack and myocardial infarction were excluded.Decreased subjects were excluded and the extreme value were also excluded,20 295 subjects eventually meet the inclusion criteria and were included for statistical analysis.Stepwise linear regression,multivariate logistic regression and natural spline function were used to analyze the relationship between cumUA and baPWV and the influence of cumUA on baPWV.Results Among 20 295 subjects,the incidence of baPWV ≥ 14 m/s (criteria for judging atherosclerosis) increased with the increase of cumUA.There was significant difference in the incidence of baPWV ≥ 14 m/s (53.07％,54.35％,56.42％,58.41％,61.91％) among different cumUA partition groups (β=0.11,P＜0.01).In stepwise linear regression analysis,after adjusting for other confounding factors,it was found that cumUA was positively correlated with baPWV.In multivariate logistic regression analysis,after adjusting for other confounding factors,the results showed that baPWV ≥aPWVm were all risk factors for the third,fourth and fifth subgroups of cumUA compared with the first subgroup,and the OR05％CI) was 1.35(1.13,1.62) (P=0.01),1.60(1.29,1.97) (P＜0.01) and 2.14(1.64,2.80) (P＜0.01),respectively.Natural spline analysis exhibited a similar J curve relationship between cumUA and increased baPWV.Conclusion CumUA is a risk factor for increased baPWV.