Objective:To compare the similarities and differences of macrophage activation syndrome（MAS）combined with systemic juvenile idiopathic arthritis（sJIA）versus with juvenile onset systemic lupus erythematosus（JSLE）.Methods:The clinical data of 48 children with MAS admitted to the Department of Nephrology and Immunology in Children's Hospital of Hebei Province from May 2015 to January 2023 were retrospectively analyzed. The patients were divided into sJIA-MAS and JSLE-MAS group，and the clinical manifestations，laboratory indicators and treatment of the two groups were compared.Results:Among the 48 children（14 males and 34 females）with MAS，the average age of onset was 9.5（3.0，11.8）years. There were 28 cases（11males and 17 females）of sJIA-MAS and 20 cases（3 males and 17 females）of JSLE- MAS. All the 48 children with MAS had fever and hyperferinemia，and the fever with sJIA-MAS was mostly continued fever or remittent fever. Respiratory tract infection was the most common trigger in sJIA-MAS［15 cases（53.6%）］，and disease activity was the most common trigger in JSLE-MAS［13 cases（65.0%）］.Additionally，viral infections（EB virus and cytomegalovirus）were also one of the triggers in MAS［sJIA：7 cases（25%），JSLE：4 cases（20%）］.Compared with JSLE-MAS，the number of days with fever［15.0（12.0，21.0）days vs. 6.0（4.0，9.5）days， Z=-3.812， P=0.001］and the length of hospital stay［29.0（26.3，39.8）days vs.26.0（19.3，30.8）days， Z=-1.958， P=0.049］were longer in sJIA. Compared with JSLE-MAS，ALT［（685.32±561.67）U/L vs.（139.61±124.44）U/L， t=4.973， P=0.001］，AST［784.00（235.25，1 251.25）U/L vs.189.50（53.25，374.08）U/L， Z=-3.283， P=0.001］，CRP［11.48（3.56，28.89）mg/L vs.1.91（0.53，8.98）mg/L， Z=-3.200， P=0.001］，ferritin［32 167.0（12 384.8，65 963.8）μg/L vs.2 003.5（922.5，11 430.0）μg/L， Z=-4.130， P=0.001］，ferritin max/ESR min［1 353.35（355.75，4 342.53）vs.91.92（34.94，291.53）， Z=-4.120， P=0.001］were higher in sJIA.The decrease of CRP was greater in sJIA［80.04（45.64，143.71）mg/L vs.10.20（6.27，25.64）mg/L， Z=-4.433， P=0.001］.Compared with sJIA-MAS，peripheral white blood cell counting［4.05（2.90，7.73）×10 9/L vs.1.56（1.15，3.47）×10 9/L， Z=-3.577， P=0.001］and platelet counting［（162.68±92.19）×10 9/L vs.（110.10±72.99）×10 9/L， t=2.118， P=0.040］were lower in JSLE-MAS. Kidney involvement was more common in JSLE-MAS［10 cases（50%）vs.0 cases（0%）， χ 2=17.684， P=0.001］.There was no significant difference in the incidence of sJIA-MAS and JSLE-MAS meeting the criteria of hemophagocytic lymphohistiocytosis［6 cases（21.4%）vs.5 cases（25.0%）， χ 2=0.084， P=0.772］. Conclusion:Compared with JSLE-MAS，sJIA-MAS is more dangerous and difficult to control，while JSLE-MAS involves more organs，among which the blood system and kidney are more common.

The data of a child with sphingosine phosphate lyase insufficiency syndrome (SPLIS) admitted to Children′s Hospital of Hebei Province on February 4, 2020 were retrospectively analyzed.The child had edema, complicated with ichthyosis, adrenal calcification, and hearing loss from the early infancy.Laboratory examination results suggested a low albumin level, hypercholesterolemia, a high proteinuria level, abnormal liver and renal functions, and hyponatremia.The child gave up treatment and died at home.Whole Exome Sequencing (WES) results showed two hete-rozygous mutations of SGPL1 gene (chr10: 72604336, c.134G>A, p.W45X; chr10: 72629563, c.719G>T, p.S240I). SPLIS is inherited in an autosomal recessive manner.It starts in infancy, and affects the kidney, skin, endocrine, nervous and immune systems.It is suggested that SPLIS patients should take genetic examination.Early diagnosis, appropriate intervention, and vitamin B 6 treatment may relieve some symptoms of SPLIS patients.Adeno-associated virus mediated SGPL1 gene replacement therapy can be a novel cure of SPLIS and is worthy of investigation.

Objective:To study the drug resistance patterns of Bordetella pertussis in vitro, and to know the clinical characteristics of pediatric pertussis and evaluation the treatment outcomes, which may provide references for experiential diagnosis and treatment of this disease. Methods:Nasopharyngeal swabs of the hospitalized children with suspected pertussis in Children′s Hospital, Zhejiang University School of Medicine in 2017 were collected for culture. And the clinical data of the children were collected. The strains were identified by pertussis-specific antiserum agglutination and finally confirmed by mass spectrometry. The drug sensitivity test was performed using the E-test method. The efficacy of therapy with antibiotic was evaluated after two weeks of treatment. Statistical analysis was performed with Mann-Whitney U test and chi-square test. Results:Of 1 029 children, 211 (20.5%) nasopharyngeal swabs were positive for Bordetella pertussis culture, and the isolation rate of the specimens was highest (31.2%, 45/144) in July. Of the 211 pertussis patients, 105 (49.8%) were male and the age were 3.8 (2.2, 6.9) months, 114 (54.0%) were not vaccinated with pertussis diphtheria tetanus mixed vaccine and 192 (91.0%) were prescribed with previous antibiotics. There were 142 (67.3%) children from families with two or more than two children, and 136 (95.8%) of which were the youngest siblings. One hundred and fifty-nine (75.4%) patients had paroxysmal cough and 61 (28.9%) had whooping. The white blood cell counts were higher than 20×10 9/L in 94 (44.5%) patients, and the lymphocyte counts were higher than 10×10 9/L in 97 (46.0%) of patients. The drug susceptibility results showed that 138 (65.4%) strains were against erythromycin, azithromycin and clindamycin with minimum inhibitory concentration (MIC)>256.000 mg/L. The MIC 90 of the isolates to ampicillin, ceftriaxone, cefoperazone/sulbactam, meropenem and trimethoprim/sulfamethoxazole were 0.190 mg/L, 0.190 mg/L, 0.094 mg/L, 0.094 mg/L and 0.750 mg/L, respectively. All strains had a MIC of <0.016 mg/L for piperacillin/tazobactam. After treatment, symptoms were improved in 195(92.4%) patients when they were discharged from hospital. Seventy-six (57.1%) children whose symptoms did not improve after seven-day treatment with macrolides, were prescribed with other antibiotics or other antibiotic with macrolides in combination. Compared with the patients treated with macrolides, more patients treated with cefoperazone/sulbactam or piperacillin/tazobactam had negative nasopharyngeal culture results after two weeks of therapy (46/48(95.8%) vs 46/57(80.7%)), or on day seven (45/46(97.8%) vs 39/47(83.0%)) and on day 14 (45/45(100.0%) vs 41/47(87.2%)) since discharged. The differences were all statistically significant ( χ2=5.50, 5.86 and 6.15, respectively, P=0.019, 0.015 and 0.013, respectively). Conclusions:The majority of children with pertussis do not have whooping, and the resistant rate of Bordetella pertussis to macrolides is high. Further study is needed to evaluate the feasibility and reasonability of cefoperazone/sulbactam and piperacillin/tazobactam in treating pediatric pertussis caused by macrolides-resistant Bordetella pertussis.

The coronavirus disease 2019 (COVID-19) has caused a global pandemic. All people including children are generally susceptible to COVID-19, but the condition is relatively mild for children. The diagnosis of COVID-19 is largely based on the epidemiological evidence and clinical manifestations, and confirmed by positive detection of virus nucleic acid in respiratory samples. The main symptoms of COVID-19 in children are fever and cough; the total number of white blood cell count is usually normal or decreased; the chest imaging is characterized by interstitial pneumonia, which is similar to other respiratory virus infections and infections. Early identification, early isolation, early diagnosis and early treatment are important for clinical management. The treatment of mild or moderate type of child COVID-19 is mainly symptomatic. For severe and critical ill cases, the oxygen therapy, antiviral drugs, antibacterial drugs, glucocorticoids, mechanical ventilation or even extracorporeal membrane oxygenation (ECMO) may be adopted, and the treatment plan should be adjusted timely through multi-disciplinary cooperation.
Betacoronavirus ; isolation & purification ; Child ; Coronavirus Infections ; diagnosis ; pathology ; therapy ; Humans ; Pandemics ; Pneumonia, Viral ; diagnosis ; diagnostic imaging ; etiology ; pathology ; therapy

Betacoronavirus ; isolation & purification ; Child ; Clinical Laboratory Techniques ; Coronavirus Infections ; diagnosis ; drug therapy ; therapy ; Humans ; Pneumonia, Viral ; diagnosis ; therapy

Objective To investigate the expression level of hypoxia-inducible factor-lot (HIF-1ot) in gastric cancer and its relationship with clinicopathological characteristics and prognosis of patients with gastric cancer.Methods From March 3,2011 to September 28,2012,49 patients with gastric adenocarcinoma tissue chips were selected from pathology department of our hospital.They were matched with paracancerous tissues.The expression levels of HIF-1α were measured by immunohistochemistry method in gastric cancer tissues and paracancerous tissues chips.Kaplan-Meier was used to evaluate the progression-free survival (PFS) and overall survival (OS),and the Cox proportional hazards model was used to analyze whether HIF-1α was a prognostic factor.Results The high expression rate of HIF-1α protein in gastric cancer was significantly higher than that in paired para-carcinoma group (42.9％ vs.4.1％,x2 =20.509,P ＜ 0.001).The expression of HIF-1 α protein was related to TNM stage (x2 =4.601,P =0.032),vascular invasion (x2 =6.766,P =0.009) and degree of differentiation (x2 =7.969,P =0.005).Compared with patients with low expression of HIF-1α,the median PFS (16.2 months vs.27.3 months) and median OS (34.8 months vs.43.8 months) were shorter in the patients with high expression of HIF-1 α,and the differences were statistically significant (median PFS:x2 =4.661,P =0.002;median OS:x2 =6.903,P =0.009).The results of single factor analysis showed that overexpre-ssion of HIF-1α was correlated with PFS and OS (PFS:HR =4.461,95％ CI:1.969-10.802,P ＜0.001;OS:HR =3.109,95％CI:1.274-7.588,P =0.013).The results of Cox multivariate analysis showed that overexpression of HIF-1α was the independent risk factor that affected the survival and prognosis of patients with gastric cancer (PFS:HR =4.747,95％ CI:2.175-10.230,P ＜0.001;OS:HR =3.171,95％ CI:1.358-7.404,P =0.008).Conclusion The high expression rate of HIF-1α protein in gastric cancer tissues is significantly higher than that in paracancerous tissues.The expression of HIF-1α is associated with TNM stage,vascular invasion,degree of differentiation in patients with gastric cancer.The high expression of HIF-1α is associa-ted with the shorter median OS and PFS.The high expression of HIF-1α is an independent risk factor for the survival and prognosis of patients with gastric cancer,which is expected to be an independent marker of poor prognosis.

Objective To investigate the effect of flipped classroom based on WeChat and mi-crolecture in medical chemistry experiment course in the context of "internet". Methods The classes were randomly divided into 2 groups, experimental group (flipped classroom teaching, n=97) and the control group (traditional teaching, n=98). Comparison of the chemistry experiment test results were performed with the use of t test between the two teaching groups at the end of the semester to evaluate the experimental teaching method. All statistical processing and analyses were performed with SPSS software (version 12.0). Results The chemistry experiment test score of the experimental group was higher than that of the control group [(77.84±8.22) vs. (73.43±10.14), t=3.341, P=0.008), and the difference was statistically significant. The results of the questionnaire showed that the students in the experimental group generally consider that flipped classroom teaching is better than the traditional teaching in terms of the cultivation of comprehen-sive quality and the teaching effect. Conclusion In the context of "internet", flipped classroom teaching with WeChat microlecture can better mobilize the enthusiasm of students to learn and participate in medical chemistry experiment course, which has been welcomed by students and further suggest good application prospects.

Objective The study aimed to evaluate the short and middle term results in the patients with tetralogy of Fallot(TOF) after one-stage repair and staged repair.Methods A total of 459 TOF younger children less than five-year-old between January 2009 and December 2013 had received surgical repair,including 416 patients by one-stage repair(group Ⅰ)and 43 patients by staged repair(group Ⅱ).Among them,245 were male and 214 were female.The average repair surgical age was 27.8 ranged from 4 to 60 months,average palliation age was 15.4 ranged from 3 to 40 months.23 perioperative and follow-up parameters were assessed including sex,age,weight,preoperative clinic symptom,ratio of McGoon,pulmonary artery index,Z score of pulmonary annulus,cardiopulmonary bypass time,aortic cross-clamping time,type of VSD repair,type of RVOT procedure,Peak RV/LV pressure radio,RV-PA pressure grade,mortality,severely low cardiac output syndrome,hypoxemia,extubation time,ICU time,fellow-up time,left ventricular eject faction,RV index of myocardial performance (MPI),tricuspid annular plane systolic excursion(TAPSE) and pulmonary regurgitation.Results Compared with group Ⅰ,age and weight were significantly lower in patients in group Ⅱ[(19.1 ± 16.4) months vs.(21.1 ± 11.2) months,P ＜ 0.05) and(19.1 ±16.4) kgvs.(21.1 ±11.2) kg,P ＜0.01].The Z score of pulmonary annulus of patients in group Ⅱ were significantly less than those in group Ⅰ (-3.69 ± 2.36 vs.-2.50 ± 1.95,P ＜ 0.01).The ratio of repairing VSD by RV incision and using TAP in patient of group Ⅱ were significantly more than those in group(27/43 vs.71/413,P ＜0.01),(41/43 vs.221/413,P ＜0.01).There was no difference of mortality,complication,extubation time and ICU time bewteen two groups.All patients followed up 12-52 months,there was no difference of LVEF,MPI and TAPSE between two groups.However,the severity of pulmonary regurgitation in patients of group Ⅱ was significant more than those of group Ⅰ (47.6％ vs.32.1％,P ＜ 0.01).Conclusion The early and mid-term results in the the young children patients with TOF after one-stage repair or staged repair were good.Although the prior palliative shunt could promote the development of the hypoplasia pulmonary arteries in the young children patient,it may related to the technique of repairing operations and the postoperative pulmonary regurgitation.

Objective To explore the common pathogens distribution and the drug resistance pattern in vitro from cerebrospinal lfuid culture in children. Methods The results of cerebrospinal lfuid culture in hospitalized children from January 2007 to December 2014 were retrospectively analyzed. Bacteria identiifcation and antimicrobial susceptibility were assayed by Vitek system from Bio Mrieux Company. Some bacterial strains were tested by disk diffusion method. Results Cerebrospinal lfuid from 23099 patients were tested at least one time during research period. 671 strains of bacteria were isolated with positive rate of 2 . 9%, among which there were 579 strains of gram positive bacteria ( 86 . 3%) and 92 strains of gram negative bacteria ( 13 . 7%). The top ifve bacteria were coagulase negative Staphylococcus ( 399 strains, 58 . 9%), Micrococcus ( 37 strains, 5 . 5%), Streptococcus pneumoniae (34 strains, 5.1%), Escherichia coli (32 strains, 4.8%), and Enterococcus faecium (26 strains, 3.9%). The positive rates of cerebrospinal lfuid culture in 8 consecutive years showed a downward trend (χ2?=?10 . 410 , P=?0 . 001 ). The positive rates of coagulase negative Staphylococci showed annual decline trend (χ2?=?31 . 200 , P

Objective To investigate the clinical manifestations and the antibiotics resistance patterns in children with positive blood culture of Escherichia coli. Methods The clinical data of children with positive blood culture of Escherichia coli were retrospectively analyzed from Jan.2007 to Dec.2014. Results In a total of 154774 children who had blood culture in the study period, 8446 children were positive, among whom 408 (4.83%) children were isolated Escherichia coli. The children with the positive blood culture of Escherichia coli mainly were under one year old (51.72%), of which 36.77%was neonates. There were 275 children had underlying diseases, and the most common disease was Leukemia. 199 (48.77%) Escherichia coli strains were producing extended spectrumβ-lactamase (ESBLs) and 85.23%were resistant to ampicillin. All strains were susceptible to meropenem. Conclusions Septicemia caused by Escherichia coli is usually occurred in children with leukemia or in neonates. Since blood infections of Escherichia coil had high rate of ESBLs, the use of carbapenem antibiotics should be cautious.