Objective To investigate the changes of platelet aggregation rate and platelet surface CD 41+CD62P+ expression af-ter clopidogrel discontinuation in the patients with percutaneous coronary interventions (PCI)) .Methods The platelet aggregation rates and platelet surface CD41+CD62P+ in 52 PCI patients with oral clopidogrel for near 12 months and discontinuation soon were measured before clopidogrel therapy(T0 ) ,in 1 week after clopidogrel therapy(T1 ) ,1 week before clopidogrel discontinuation(T2 ) , 1 week(T3 ) and 1 month after clopidogrel discontinuation (T4 ) .Results Compared with T0 ,the platelet aggregation rate and the expression of platelet CD41+CD62P+ at T1 were significantly decreased ,the difference showing statistical significance (P<0 .05) , which at T2 maintained the lower levels ;which at T3 were increased ,which at T4 were recovered to those at T0 .The platelet aggre-gation rates at various time points were (44 .20 ± 18 .36)% ,(25 .38 ± 12 .10)% ,(23 .74 ± 8 .15)% ,(51 .79 ± 10 .55)% and(45 .97 ± 16 .42)% respectively ,and the positive rates of CD41+ CD62P+ were(12 .96 ± 11 .48)% ,(3 .93 ± 3 .33)% ,(4 .72 ± 3 .14)% , (13 .90 ± 10 .38)% and(10 .84 ± 8 .13)% ,respectively .Conclusion In the patients treated with 12-month clopidogrel after PCI ,the platelet aggregation rate and the CD41+CD62P+ positive rate are mildly increased at 1 week after clopidogrel discontinuation and gradually returned to the level before discontinuation at 1 month after clopidogrel discontinuation .

each group were sacrificed, respectively. Distributions of BrdU positive cels and ChAT positive cels were detected by S-P immunohistochemical method. The learning and memory abilities of rats were detected by Morris water maze system.
RESULTS AND CONCLUSION:BrdU positive cels were mainly distributed in the cortex and hippocampus, especialy around the blood vessels, and there was the presence of focal aggregation. A smal amount of BrdU positive cels were observed in the basal ganglia and thalamus as wel as in the ependyma. BrdU positive cels were counted at different time after operation. The number of BrdU positive cels decreased with time, and only a smal number of BrdU positive cels were observed at 60 days after transplantation. The number of ChAT positive cels at different time after transplantation was ranked as folows: neural stem cel transplantation group > model group > sham operated group (P < 0.05). Compared with the model group, the time for searching the platform was significantly lower in the neural stem transplantation group and sham operated group, but the number of crossing the platform was significantly higher in the neural stem cel transplantation group and sham operated group (P < 0.05). The results show that neural stem cels could be transplanted into the rats with vascular dementia, and the cels could survive and migrate in the brain of rats and significantly improve the learning and memory ability. This mechanism may be related to the differentiation and growth of cholinergic neurons in the hippocampus.

Objective To explore the clinical features of chronic granulomatous diseases and Mcleod syndrome caused by continuous X chromosome deletion. Methods The clinical data of two children diagnosed as chronic granulomatous disease and Mcleod syndrome by gene detection were retrospectively analyzed. Results Two males, 4 year 1 month and 1 year 9 month old, were both hospitalized due to persistent pulmonary infections. Both of them had a history of repeated severe infections and BCG vaccine associated lymphadenitis, and were diagnosed as X-linked chronic granulomatous disease for respiratory burst defects and deletion of all CYBB exons. Both of them had retarded motor development, and were diagnosed as DMD for detection of DMD gene exons and muscle speciifc promoter region and exon 1-2 deletion by MLPA. One case was found with obvious echinocytes, the other case showed whole exons deletion of XK gene. Both of them were diagnosed as Mcleod syndrome. Conclusion Continuous X chromosome deletion could lead to combination of Mcleod syndrome, DMD, and X-CGD, which may complicate the condition. Due to the lack of Kx antigen, repeated common blood transfusion can produce relative antibody, which lead to severe hemolytic crisis.

Background:Antibiotics can reduce the efficacy of immunotherapy for melanoma,non-small cell lung cancer and renal cell carcinoma by altering the intestinal microbiome,but their effect in the treatment of gastrointestinal cancer is unclear.Aims:To investigate the prognostic effect of antibiotics on PD-1/PD-L1 inhibitors in the treatment of colorectal cancer(CRC)patients.Methods:The clinicopathological data of 95 patients diagnosed with CRC and treated with immune checkpoint inhibitors(ICIs)from December 2018 to May 2022 at Renmin Hospital of Wuhan University were retrospectively analyzed,and the patients were divided into antibiotic group and control group according to whether antibiotics were used.Risk factors of progression free survival(PFS)and overall survival(OS)were analyzed by univariate and multivariate analysis.Results:Among the 95 patients with CRC,64 were male and 31 were female.The mean age was(60.46±11.82)years.PFS(10.9 months vs.24.8 months,P=0.032)and OS(14.5 months vs.35.5 months,P=0.008)were significantly decreased in antibiotic group than in control group,and one-year survival rate was 56.5%and 78.7%,respectively.Multivariate analysis showed that tumor location and antibiotic use were independent risk factors of PFS(P<0.05),while tumor location,antibiotic use and no radical surgery were independent risk factors of OS(P<0.05).Univariate and multivariate analyses showed that antibiotic administration route,dose were not risk factors of PFS(P>0.05);univariate analysis showed that antibiotic administration route was correlated with OS(P=0.045),however,multivariate analysis showed that antibiotic administration route,infection location were not risk factors of OS(P>0.05).Conclusions:Tumors located in colon and antibiotics applied within 30 days before and after the first dose of ICIs can shorten PFS and OS in CRC patients,while radical surgery can prolong the OS of CRC patients.

Objective To explore the diagnosis and treatment of atypical severe combined immunodeficiency disease (SCID). Methods The clinical data of atypical SCID in 7 children with IL2RG,JAK3,and RAG1 mutations were reviewed and analyzed from September 2012 to June 2017. Results In 7 cases (6 males and 1 female), there were 5 infants, 1 toddler and 1 school-age child. Cases 2, 4, and 6 were classic SCID clinical phenotypes. Cases 1, 3, 5, 7 were atypical SCID clinical phenotypes. Case 6 were diagnosed with Omenn syndrome. Cases 2, 5 were classic SCID immune phenotypes, cases 1, 3, 4, 6, 7 were atypical SCID immune phenotypes, and case 1 had maternal chimera. The next generation sequencing indicated that case 1 had a compound heterozygous JAK3 mutation with c.3097-1G>A/c.946-950GCGGA>ACinsGGT.Cases 2,3,and 4 had IL2RG mutations,with c.865C>T/p.R289X,c.664C>T/R222C,52delG,respectively.Case 5 had JAK3 mutations with c.2150A>G/p.E717G and c.1915-2A>G.Sanger sequencing indicated that case 6 had a RAG1 mutation of complex heterozygosity with c.994C>T/p.R332X and c.1439G>A/p.S480N. Case 7 had homozygous RAG1 mutation with c.2095C>T/p.R699W.Conclusion Under certain conditions,gene mutation can lead to atypical clinical and/or immune phenotypic SCID.

Objective To analyze the clinical characteristics of community-acquired influenza virus pneumonia in hospitalized children and improve the clinicians' understanding level of this disease.Methods Data of 70 cases with community-acquired influenza virus pneumonia admitted to the Respiratory Department and Infectious Disease,Beijing Children's Hospital,Capital Medical University,from November 2009 to April 2018 were collected and the clinical characteristics were analyzed.Results Of the 70 cases,61 cases(89.7％) were discharged after improvement.The median age was 3.5 years old,and 50 cases(71.4％) were 0 to 5 years old.There were 29 cases with severe influenza pneumonia,41 cases with mild influenza pneumonia,3 cases died,and 19 cases (27.1％) had underlying diseases.Sixty-four cases (91.4％) were hospitalized in winter and spring.The first symptoms were mainly fever in 64 cases (91.4％) and cough in 65 cases (92.9％),and temperatures were mostly from 39.1 ℃ to 41.0 ℃.Lung auscultation was dominated by moist rales (30 cases,58.8％) and wheezing (8 cases,15.7％).There were many complications of influenza virus pneumonia,including 19 cases with myocardial injury,11 cases with liver function injury,4 cases with toxic encephalopathy,3 cases with electrolyte disturbance,2 cases with multiple organ failure,2 cases with hemophagocytic syndrome,and 1 case with septic shock.Chest radiographic results reveal bilateral inflammation in 40 children (57.1％),prodominatly in lower lobe lesions (39 cases).The common changes were patchy shadow,interstitial parenchymal lesion,ground glass shadow,and pleural effusion.Forty-seven children (67.1％) were infected by influenza A,and 23 children(32.9％) were co-infected.The percentage of severe cases with underlying diseases (68.4％) was significantly higher than that in children without chronic diseases (31.4％),the difference was statistically significant (x2 =7.830,P =0.005).The increase rate of C reaction protein (CRP) in severe cases (54.3％) was significantly higher than that in mild cases (28.6％),the difference was statistically significant (x2 =4.769,P =0.029).Conclusions Community-acquired influenza virus pneumonia in children mainly occurs in winter and spring.It is more common seen in children under 5 years of age.The main clinical manifestations of community-acquired influenza virus pneumonia are high fever and cough,extrapulmonary complications are more common.Most children have moist rales and showed bilateral inflammation and lower lobe lesions in chest radiography.Children with underlying diseases are more likely to develop severe influenza virus pneumonia.Elevated CRP is associated with severe influenza virus pneumonia.Most patients have a good prognosis,but there are still cases of death.

Objective:To explore the clinical features, follow-up characteristics and prognosis of rheumatic disease complicated with pulmonary arterial hypertension (PAH) in children, and to provide support for its clinical diagnosis and treatment.Methods:A retrospective analysis was conducted on the data of rheumatic 24 patients complicated with PAH hospitalized in the Department of Rheumatology and Immunology, Children′s Hospital Affiliated to the Capital Institute of Pediatrics, Department of Rheumatology and Immunology, Jiangxi Children′s Hospital, Department of Pediatrics Ⅰ, the First Affiliated Hospital of Zhengzhou University and Department of Pediatrics, the Affiliated Hospital of Inner Mongolia Medical University from January 2013 to June 2022.The rheumatic patients complicated with PAH were followed up by telephone on June 30, 2022, and their clinical symptoms, treatment, follow-up, and prognosis data were collected.According to different treatment methods, the patients were divided into different clinical subgroups. The change of PAH was analyzed. The t-test was used for comparison between groups. P<0.05 was statistically significant. Results:A total of 24 cases were enrolled, with 7 males and 17 females.The average onset age of PAH was (10.97±3.79) years old.The median duration of PAH was 6.00 (32.20) months.The average pulmonary artery pressure was (51.71±17.66) mmHg(1 mmHg=0.133 kPa). There were 9 cases of systemic lupus erythematosus, 5 cases of Takayasu′s arteritis, 3 cases of juvenile dermatomyositis, 3 cases of undifferentiated connective tissue disease, 2 cases of systemic juvenile idiopathic arthritis, 1 case of Behcet′s disease, and 1 case of Kawasaki disease.Among 24 cases, the common symptoms were fever (14 cases), fatigue (10 cases) and dyspnea (7 cases). Of the 24 cases, 10 cases were complicated with hydropericardium, 9 cases with valve regurgitation, and 5 cases with decreased systolic and/or diastolic function.Lung changes were observed in 17 cases.Eleven cases were tested for B-type natriuretic peptide (BNP), and the BNP levels were all elevated in them (11 cases), with a median BNP of 3 073 (10 645) ng/L.After the first occurrence of PAH, 12 cases were treated with Methylprednisolone therapy, 10 cases received Cyclophosphamide therapy, and 2 cases who were both systemic lupus erythematosus, underwent blood purification.In the treatment of PAH, 11 cases were treated with pulmonary artery pressure reduction, and 7 of the 11 cases took PAH-targeted drugs.The mean decrease of the average pulmonary artery pressure in children receiving the targeted therapy[(44.80±24.08) mmHg] was significant higher than that in children not receiving the targeted therapy [(16.15±17.25) mmHg] ( t=2.661, P=0.016). Twenty children were reexamined and/or followed up, and the average course of PAH at the telephone follow-up was (36.29±26.67) months.The pulmonary arterial hypertension in 6 cases completely recovered, with median recovery time of 8.00 (13.47) months, but 2 of them died after the complete recovery.The pulmonary arterial hypertension improved in 11 children, 1 of whom died and the remaining children were in stable condition.The pulmonary arterial hypertension worsened in 2 children, 1 of them improved previously but aggravated recently, and the other child did not monitor pulmonary artery pressure and died during telephone follow-up. Conclusions:Rheumatic diseases complicated with PAH are rare and most often diagnosed in severe rheumatic children.It can lead to death, and is commonly accompanied by notably elevated BNP levels.The patients who have early PAH detection, intensive treatment of the primary disease, symptomatic and targeted pulmonary artery pressure reduction show a better prognosis.

Objective:To summarize the clinical features of children with autosomal dominant hyper-IgE syndrome (AD-HIES) and the differential diagnosis of hyper-IgE syndrome and allergic diseases as well.Methods:All clinical data, including general information, clinical features, and genetic changes, from 7 children with AD-HIES who were diagnosed in Beijing Children′s Hospital Affiliated to Capital Medical University from April 2016 to June 2020 were analyzed retrospectively.The diagnostic criteria are based on the National Institutes of Health′s (NIH)′s hyper-IgE syndrome score and combined with the results of gene detection, shown as follows: (1) NIH score over 40, with signal transducer and activator of transcription 3 gene ( STAT3) pathogenic mutation; (2) NIH score between 20 and 40, with reported STAT3 pathogenic mutation; (3) NIH score less than 20 points was excluded. Results:There were 3 males and 4 females.The onset age of 7 cases was within 2 months after birth, and the mean age at diagnosis was 3 years old.All seven cases had recurrent skin or lung infections, with 4 cases having skin and lung infections, 1 case of skin abscesses at the BCG vaccination site, and 2 cases without skin infection suffering from recurrent pneumonia.The mean onset age of skin abscess in 5 cases was 1.5 years, and pus culture of 3 cases were Staphylococcus aureus.Four cases developed bullae and 6 cases had lung infections.Four cases had otitis media, and oral thrush was seen in 4 cases.One case of skin and lung infection developed liver abscess and sepsis.Seven cases had eczema, which was disco-vered in the neonatal period for 6 cases.Four cases had the symptoms of eczema for the first visit.Two cases had food allergy, and 1 case had recurrent wheezing within 1 year old.The serum IgE level and blood eosinophil count in 7 children were elevated.All children had heterozygous pathogenic mutations in STAT3.Six patients had de novo mutations.There were 6 different mutation sites.The 4 mutation sites were reported: c.1145G>A, c.1144C>T, and c. 1699A>G were missense mutations, and c. 1139+ 5G>A was splicing mutation.Two mutation sites had not been reported: c.1031A>C was missense mutation, and c. 2050G>T was nonsense mutation.The pathogenic grade of them were likely pathogenic, and the NIH score of 2 cases were above 40 score, which was consistent with the clinical diagnosis of hyper-IgE syndrome. Conclusions:Eczema is a common and early clinical manifestation of hyper-IgE syndrome, along with elevated IgE levels and eosinophil counts that need to be differentiated from allergic diseases.On the contrary, it often had recurrent skin abscesses or pneumonia, which was prone to bullae.The clinical manifestations of young children were atypical, and genetic testing was helpful for early diagnosis.

Objective:A case of cystic fibrosis admitted in the Respiratory Department of Beijing Children′s Hospital, Capital Medical University in June 2018 and underwent lung transplantation later was analyzed retrospectively.A 10-year-old girl had intermittent productive cough for more than 4 years with clubbed-finger.The lung high resolution CT (HRCT) showed bronchiectasis and mucus impaction, and the nasal sinus HRCT showed sinusitis.She had cystic fibrosis transmembrane conductance regulator ( CFTR) gene complex heterozygous mutation and positive sweat test.The immunoglobulin E (IgE) level and eosinophil count increased, and aspergillus fumigatus-specific IgE was positive.She was diagnosed as cystic fibrosis, allergic bronchopulmonary aspergillosis and sinusitis.Anti-infection, glucocorticoid and symptomatic treatment were given.During the following 21 months, the child had repeated respiratory difficulties and respiratory failure.Her lung function declined.Bilateral lung transplantation was performed in March 2020.She had a good recovery at more than 1 year and 3 months postoperatively.

Primary immunodeficiency diseases (PIDs) refer to immune function decrease and deficiency or immune regulation function imbalance resulted from immune cell or immunomolecular defects caused by single gene mutation.Children with PIDs are prone to develop infectious diseases.Because the immune mechanisms of hosts infected by different pathogens infecting vary, different types of PIDs are relatively susceptible to different pathogens.A number of studies have indicated that some PIDs children are more likely to develop Bacilles Calmétte-Guerin infection, severe tuberculosis and non- Tuberculous mycobacteria severe infection.In this review, several major primary immunodeficiency diseases closely related to the susceptibility of mycobacteria were summarized, including chronic granulomatous disease, severe combined immunodeficiency disease, mendelian susceptibility to mycobacterial disease, and high IgM syndrome, in order to provide a theoretical basis for early identification of such diseases.