Objective To summarize the clinical features of the allergic bronchopulmonary aspergillosis (ABPA) in children,in order to improve the understanding for ABPA and make early diagnosis and treatment of the disease.Methods A retrospective study was performed on ABPA patients diagnosed in Department of Respiratory Medicine,Beijing Children's Hospital Affiliated to Capital Medical University from March 2010 to December 2013.The clinical features,laboratory results,image characteristics and the prognosis information were reviewed.Results Eight ABPA cases met the diagnostic criterion.All patients were school-age children (7 years and 2 months to 13 years and 8 months old).Cough (8 cases),productive sputum (8 cases),wheezing (5 cases),fever (4 cases) and hemoptysis (3 cases) were the main clinical features.Six of the 8 patients showed eosinophilia.IgE level was elevated in 7 patients (1.000-5.000 IU/L).All 8 patients were allergic to aspergillus fumigatus,while only 2 cases were positive in sputum culture for aspergillus fumigatus.CT scans showed pulmonary opacities in all 8 cases,while 7 patients had typical central bronchiectasis.Seven patients were treated with the regimen,which included glucocorticosteroid,antifungal agents (voriconazole or itraconazole) and regular bronchoscope.The symptoms of all treated patients relieved,the total serum IgE level and eosinophil cell count decreased spontaneously after the therapy.Conclusions ABPA is rare in children and the clinical features are non-specific.If the patient has elevated total IgE level in serum and eosinophilia,especially in patients with underling diseases,ABPA should be suspected.The positive result of specific antibodies to aspergillus fumigatus and central bronchiectasis on the radiology may give the suggestive diagnosis.ABPA patients generally have good response to the therapy of glucocorticosteroid and antifungal agents.

Objective To investigate the interference role that two kinds of adipokines,adiponectin and leptin,exert to hepatocellular apoptosis in mice with acute liver injury induced by Concanavalin A(ConA).MethodsTwenty-six male BALB/c mice were divided into four groups randomly:ConA group(8 mice),adiponectin group(5 mice),leptin group(5 mice) and control group(8 mice).Mice were sacrificed 8 hours after ConA-treatment for observing hepatic injury and hepatocellular apoptosis.Results Comparison to ConA group,the mice in adiponectin group showed less hepatic injury and lower ALT level and less hepatocellular apoptosis(P

Objective To investigate the clinical features, risk factors, diagnose, treatments and prognosis of patients with hepatitis B virus (HBV)-related liver failure complicated with invasive pulmonary aspergillosis (IPA). Methods A case-control study was performed to retrospectively analyze the clinical data of 43 patients with HBV-related liver failure complicated with IPA. A cohort of 43 patients with HBV-related liver failure complicated with bacterial infection only was analyzed at the same time and another cohort of 43 patients with HBV-related liver failure without any infections served as the control group. Results Compared with the control group, patients with infection had lower platelet counts and prothrombin activity, higher scores of model for end-stage liver disease (MELD), especially the patients with IPA (F=42.43,13.69,14, 22, all P＜0.01). And the similar results were observed in patients with IPA group before and after Aspergillus infection (F= 12.09,14.52,-16.74, all P＜0.01). Furthermore, the annual mortality was 100. 0% in patients with IPA,which was higher than that of patients complicated with bacterial infection only; and both groups were higher than control group of patients without infection. The statistic results showed that patients with longer length of stay, more antibiotics usage, and more invasive procedures were all risk factors of HBV-related liver failure complicated with IPA. Conclusions Conditions of patients with HBV-related liver failure deteriorate rapidly after complicated with IPA and patients have higher mortality and poorer prognosis.Therefore, it will be effective to enhance the recognition of the early clinical manifestations, and make diagnosis timely and treat with potent anti-fungal therapy to improve their survival.

Objective To investigate the correlation between serum cystatin-C (Cys-C) levels and cognitive impairment.Methods A perspective study involving 273 patients in our hospital was performed from 2013 to 2015.They were divided into the cognitive impairment group and the control group.Cys-C measurement results and cognitive impairment assessment scores were collected.Possible influence factors were adjusted,and the correlation between Cys-C levels and mild cognitive impairment was analyzed.Results The distribution of age,hypertension,diabetes mellitus,smoking,drinking,dyslipidemia,and creatinine showed significant differences between the groups at different Cys-C levels (all P＜0.05).There was a significant difference in the Cys-C level between the cognitive impairment group and the control group [(0.727±0.082) mg/L vs.(0.514±0.045)mg/L,t=23.68,P＜0.01].The Cys-C level was negatively correlated with the scores of mini-mental state examination (MMSE) and Montreal cognitive assessment (MOCA) in the cognitive impairment group (r =-0.318 and -0.572,P＜0.05).The incidence of cognitive impairment was elevated with increasing Cys-C levels (x2 =13.12,P＜0.01).Logistic regression analysis showed that high levels of Cys-C (OR=3.298,95％ CI:1.417-7.675,P=0.001),history of diabetes mellitus (OR=7.971,95％ CI:3.036-31.562,P =0.03),education level (OR =2.237,95 ％ CI:1.022-4.896,),smoking (OR =5.692,95 ％ CI:1.060-2.614),drinking (OR=1.227,95％ CI:0.083-1.228),and dyslipidemia (OR=2.267,95％ CI:1.177-4.366) are independent risk factors for cognitive impairment.Conclusions High serum cystatin C levels are closely correlated with the risk of cognitive impairment occurrence.

Objective To explore the safer and more reasonable time of withdrawing nucleoside analogues antiviral therapy in women with chronic HBV infection during immune tolerance period. Methods The patients included in this study were 343 pregnant women with chronic HBV infection who received nucleoside antiviral therapy in Obstetrics and Gynecology Center of 302 Military Hospital of China. According to the time of withdrawing antiviral therapy after delivery, the patients were assigned to P0 group, i.e., withdrawal immediately after delivery, and the patients who stopped the drug 6 weeks after delivery were assigned to P6w group. The patients were compared between these two groups in terms of prevalence of ALT abnormality during pregnancy and postpartum, the peak value and occurrence time of postpartum ALT, and mother-to-child vertical transmission. Results The prevalence of postpartum ALT abnormality was 30.2％ in P0 group and 20.7％ in P6w group (χ2=4.129, P=0.046). Specifically, for the patients with abnormal liver function during pregnancy, the prevalence of postpartum ALT abnormality was 88.0％ and 39.4％ in the two groups respectively (χ2=14.043, P=0.001). While for the patients with normal liver function during pregnancy, the prevalence of postpartum ALT abnormality was 19.4％ and 16.6％ respectively (χ2=0.392, P=0.531). Mother-to-infant HBV transmission was blocked successfully in all the patients in spite of the time of withdrawing antiviral therapy. Conclusions For the pregnant women with chronic HBV infection who received oral nucleoside analogue antiviral agents to interrupt motherto-child transmission, the time of withdrawing antiviral agents did not show significant effect on the prevalence of postpartum liver function abnormality and rate of successful blocking mother-toinfant HBV transmission. However, for the patients with abnormal ALT during pregnancy, it is appropriate to continue the nucleoside analogue antiviral therapy after delivery.

Objective: To observe the therapeutic response of radiosynovectomy with p-32 colloid on hemophilic arthropathy, and to assess the effects of radiosynovectomy with Denver Score on hemophilic arthritis staging. Methods: Radiosynovectomy with p-32 colloid was performed on 326 hemophilic arthritis patients (405 joints) , and recorded bleeding before and after treatment. The MRI performance of 102 joints was evaluated by using Denver scoring system, then was divided into 0-6 and 7-10 groups. Finally, the differences between 2 groups were analyzed. Results: Average pain score of all hemophilic arthritis patients at 6 months, 1, 2 and 3 years post treatment decreased from 3.2±2.4 (n=326) to 1.2±0.6 (n=285, P=0.021) , 1.7±0.5 (n=242, P=0.032) , 2.1±1.1 (n=212, P=0.030) and 2.2±1.6 (n=176, P=0.037) , respectively. The frequency of bleeding in 405 joints at 1, 2 and 3 years post treatment decreased from 15.1±3.6 to 2.1±0.7, 4.3±0.6, and 4.8±0.8 times per year (P<0.01) , respectively; Meanwhile, the proportions of significantly ameliorated joints’ activities were observed as of 68.50% (248/362) , 58.39% (181/310) , 55.67% (162/239) and 42.61% (75/176) , respectively. The frequencies of haemarthrosis at 1 and 2 years post treatment in patients with 0-6 Denver Score (45 target joints) reduced from 13.0±1.9 to 1.3±0.6 (P=0.002) and 3.1±0.9 (P=0.009) times per year, respectively, which also decreased in 7-10 group (57 target joints) from 16.6±2.1 to 3.1±0.9 (P=0.008) and 5.7±1.1 (P=0.004) times per year, respectively. There was no statistical difference between 0-6 and 7-10 groups before treatment in the terms of haemarthrosis frequency (P=0.773) . However, 7-10 group had higher haemarthrosis frequency at 1 and 2 years post treatment compare with 0-6 group (P=0.028 and 0.042, respectively) . Synovial volumes in 29 joints reduced after 6 month when compared with baseline [ (2 362.15±32.41) mm³ vs (3 012.40±39.78) mm³, t=7.621, P<0.001]. Conclusion Radiosynovectomy with p-32 colloid on haemophilic synovitis was a safe and effective procedure. The patients with Denver Score of 0-7 had lower frequency of haemarthrosis.

This study was aimed to investigate the application rrles of Fu-Fang Ku-Shen (FFKS) injection in combination with modern medicines for the treatment of lymphoma in the real world,in order to provide references for rational clinical application of effective medication.Patients diagnosed as lymphoma and treated with FFKS injection from 22 triple-A hospitals in China were enrolled as research objects.Patients' treatment data were extracted from the hospital information system,which was established by the Institute of Basic Research in Clinical Medicine,China Academy of Chinese Medical Sciences,in order to analyze the frequency distribution characteristics of modern medications combined in the treatment.Association rules and Apriori algorithm were used in the analysis of association characteristics of medication combination.The results showed that 3 674 cases fit the inclusion criteria were searched from the data warehouse.In the clinical practice,FFKS injection was frequently combined with moderu medications,such as antibiotics,glucocorticoids,5-HT receptor antagonists,immunomodulatory drags,proton pump inhibitor,chemotherapeutic drugs,and hepatic protector.Its associated modern medication combination showed special rrles,which was in consistency with the related diagnosis and treatment of lymphoma guideline.It was concluded that FFKS injection combined with modern medicine in the treatment of lymphoma in the real world was basically in consistency with related guidelines.It was focused on reducing the side effects of chemotherapy,prolonging survival time,and improving quality of life.It provided references for rational clinical drag application of lymphoma.

Objective: To analyze the clinical characteristics, diagnosis and treatment of bronchopulmonary foregut malformation(BPFM). Method: The clinical manifestations, imaging findings and treatment of 8 patients with BPFM were analyzed retrospectively from January 2006 to May 2016 in Beijing Children′s Hospital. Result: The age of children varied from 2 months to 7 years and 3 children were male while 5 female. Symptoms showed cough in 6 cases, fever in 4 cases, bucking when intaking of fluids or foods in 3 cases, tachypnea in 1 case, wheezing in 1 case, vomiting in 1 case, haematemesis in 1 case Pulmonary signs were decreased breath sounds in 4 cases, phlegm rale in 3 cases, shortness of breath in 2 cases, wheeze in 1 case, and retraction in 1 case. The upper gastrointestinal series showed abnormal fistulous tracts arising from the esophagus or the gastric fundus and extending into the mass in the lung. CT showed pulmonary sequestration and prompted the tube between lung and esophagus. Six children underwent pneumonectomy and esophageal fistula repair. They were discharged and their symptoms were improved. Two cases of children were discharged from a hospital without surgery. Conclusion Bronchopulmonary foregut malformation usually has its onset in early stage of life. The most common symptoms include recurrent pneumonia or bucking when intaking of fluids or foods. CT can demonstrate the bronchopulmonary sequestration and evaluate the communication with the gastrointestinal tract. The upper gastrointestinal series can demonstrate the abnormal tract directly. Pneumonectomy and esophageal fistula repair are the treatment of this disease.

Objective:To analyze the clinical characteristics of eosinophilic lung diseases(ELD) in children to enhance pediatricians′ understanding of ELD.Methods:In this retrospective cross-sectional study, a total of 149 children with ELD were recruited from Beijing Children′s Hospital, Capital Medical University between April 1, 2007 and March 31, 2022.Chi-square test, Fisher′s exact test, Mann-Whitney U test and Kruskal-Wallis test were used to analyze data and conclude clinical characteristics.Spearman correlation was used to analyze the correlation between eosinophils in peripheral blood and bronchoalveolar lavage fluid.Chi-square test and Kappa consistency test were used to compare the differences and consistency in diagnostic results between bronchoalveolar lavage fluid or lung biopsy and eosinophil elevation with chest imaging abnormalities. Results:(1)The isolated lung involvement was mostly caused by allergic bronchopulmonary aspergillosis(9 patients), and other system involvement by idiopathic hypereosinophilic syndrome(89 patients).(2)The main respiratory manifestations included coughing(90 cases, 60.4%) and expectoration(41 cases, 27.5%), while 23.5%(35 cases) of patients had no respiratory symptoms; 50.3% had digestive system involvement, and 40.9% had skin involvement.These were the two most commonly affected organs.(3)Spearman correlation was performed between eosinophils in peripheral blood and bronchoalveolar lavage fluid( r=0.3, P<0.05).Chi-square test was performed to compare ELD diagnosed by bronchoalveolar lavage fluid or lung biopsy with peripheral blood eosinophilia accompanied by abnormal chest imaging( P<0.05).Kappa consistency test(Kappa<0.2) showed poor consistency between the two diagnostic methods. Conclusions:ELD are present in children, and multiple etiologies may be pathogenic.Among children with ELD, the isolated lung involvement is mainly caused by allergic bronchopulmonary aspergillosis.The digestive system and skin are the most commonly affected organs, except for lungs.The correlation between eosinophil levels in peripheral blood and bronchoalveolar lavage fluid is poor.

CRISPR-Associated Protein 9 ; Gene Editing ; CRISPR-Cas Systems/genetics*